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Caring for a Child with Suspected Sexual Abuse-Juniper Publishers

Opinion

Working in a pediatric setting is both challenging and rewarding experience. To effectively care for the pediatric patients and their families, it is vital for the pediatric nurses to demonstrate critical thinking skills, problem-solving abilities, cultural sensitivity, practical wisdom and sound clinical judgment. Caring for children with suspected sexual abuse requires sound knowledge (clinical and legal), moral decision making, therapeutic communication skills, clinical competencies, and collaborative approach to tackle the challenges posed by these difficult cases in a healthcare setting [1-3]. During my clinical experience at a pediatric setting, caring for a young girl with suspected sexual abuse was both challenging and rewarding experience. Besides abiding by the nursing process, I considered health promotive, curative and rehabilitative aspects to restore health and well-being of the child and family. In this paper, I present the case study of a young child with suspected sexual abuse and share my experience of caring for the traumatized child and her family.

Case History of a Child with Suspected Sexual Abuse

A 4 year 9months old girl presented at the outpatient pediatric unit with the complaints of fever, abdominal pain, itching of genitalia and vaginal discharge for one month. I asked child’s mother about the onset and severity of her symptoms and explored child’s psychosocial history. On performing child’s physical examination, a ruptured hymen and inflamed vagina were noticed. Considering the findings of physical examination, with the help of play therapy I initially developed a rapport with the child. During my communication, I tried to explore from her whether she was ever hurt or touched badly at her private parts. I came to know that each day the child travels from home to school through a school bus in which the child is being bullied by boys who is elder than her. The child also verbalized that the boys in her school bus touch at her private parts which often make her uncomfortable and due to fear she has never shared this with anyone. At this stage, childs mother was shocked and verified that the child has never told her about any such incident.

After undertaking thorough history taking and physical assessment I presented the case in front of my preceptor who was a pediatrician. On hearing the case my preceptor decided to verify the history and physical examination but found the similar findings. This was the time when the mother was prepared by both of us to suspect sexual abuse in her child. The event was quite crucial for the mother and for us being health care professionals because the diagnosis of child abuse holds several legal considerations and ethical issues.

Finally, during this visit child was diagnosed to have vulvo vaginitis, and as per request of child’s mother diagnosis of “child sexual abuse” was not documented in the patient+s file. During this visit child’s high vaginal swab, complete blood count and blood culture and sensitivity samples were sent. As a curative measure, thechild was prescribed oral antibiotics and antifungal topical application. Also, the mother was supported psychologically during this clinic visit. Hospital admission was not advised at that time. Child’s high vaginal swab’s report revealed positive pus cells, gram-negative cocci, gram positive cocci and beta hemolytic streptococci group F that were resistant to the prescribed antibiotics. Child’s blood count revealed neutrophilia and anemia, however, the blood culture was negative.

During that week, the child was brought to the emergency department with the complaint of high-grade fever, abdominal pain, unresolved vaginal discharge and one episode of hematemesis. The child required admission at the inpatient pediatric unit. I observed that child’s mother was extremely annoyed and frustrated because neither child’s primary physician nor the laboratory personnel contacted her to share the abnormal reports of her child. As per mother, the previously prescribed treatment regimen did not work for the child, hence child’s condition got more serious and she ended up into aggravation of symptoms.

I noticed that the child’s mother lost trust on pediatricians and healthcare settings due to no follow-up call from the medical team. At this stage, I tried to maintain my rapport with the child's mother and made her ventilate her feelings. During child's hospital admission, I noticed that the major emphasis of every physician was child's physical treatment (curative aspect) and nobody was looking after the rehabilitative and future preventive measure for this child. I further noticed that almost all attending physicians at the inpatient unit were not paying attention to exploring the perpetrator of the sexual abuse and provide anticipatory guidance to the child's parents.

My Role as a Pediatric Nurse

During child's admission at inpatient unit, I allowed the mother to express her concerns and ventilate her feelings. Child's mother mentioned that she is experiencing confusion because she wants to prevent her child from risks of future abuse but could not think of possible perpetrator of this sexual abuse and possible ways to address this issue. At that stage, I let the mother talk to her child and think  of possible changes in her daily routine. I also encouraged the mother to take her child in confidence and allow her child to express her feelings and experience. I shared a poster with the mother to provide a pictorial understanding of different forms of child abuse, neglect, and maltreatment.

On the second day when I visited the mother she mentioned that the provided material has enabled her to reflect on several aspects of her parenting. Child's mother ventilated that she could think of possible lifestyle changes that might have led to the present condition of her child. Child's mother verbalized that she has neglected her child to some extent after her miscarriage. Mother mentioned that almost 3 months back when she experienced miscarriage she decided to arrange pick and drop for school by a private school bus driver. Mother further shared that on taking her child into confidence the child has reported to her that two boys in her school bus bully her and attempt to insert a pencil in child's vagina which creates discomfort for her

She also acknowledged that her child is picked first and dropped last by the private school bus driver who can also be a perpetrator of this sexual abuse. During that conversation, child's mother also verbalized that she has often seen her brothers-in- law who excessively hug and kiss her child, and let her sit on their lap for hours. Mother mentioned that considering family relations she often feels hesitant to take necessary actions.

In view of above, I explained the mother that now this is a right time to let her child know about “good touch” and “bad touch”, as well as, to pay close attention to her child’s social circle and protection from further abuse. I further encouraged the mother to be confident to hold back her child from possible causes of abuse either they are relatives or strangers.

Child’s mother not only admired the suggestion but also ventilated that previously whenever she used to hear about child abuse and child neglect she used to think that this only happens with children who belong to a low income group and live in families who have low literacy rates. Mother appreciated the provided anticipatory guidance and verbalized that the teaching has made her reflect upon her parenting strategies. Furthermore, mother mentioned that now she strongly feels that she should look for any nearby school for her child, keep an eye on her child's friend circle, aware her child about good touch and bad touch, encourage her child to share her daily life situations openly without any fear of punishment, look after her child's safety needs and be more vigilant.

The above clinical case reveals that when the mother was made aware of possible types and possible sources of child abuse then she reflected on her parenting strategies and social circumstances that were increasing the susceptibility of her child for sexual abuse. Being a pediatric nurse, I realized that establishment of trusting relationship with child's parents, in-depth history taking thorough physical examination, therapeutic communication, and provision of anticipatory guidance hold magical effects on the well-being of traumatized child and family member. My role as patient's advocate, counselor, educator, and communicator enabled child's mother to reflect on their parenting strategies, identify the possible sources and perpetrator of abuse, establish a friendly relationship with her child and take necessary steps towards prevention of subsequent abuse of her child. The presented case scenario also reflected that use of holistic approach (preventive, curative and rehabilitative) is vital to care appropriately for the child with suspected sexual abuse.

To conclude, the presented clinical presentation highlights the crucial role of family-centered approach while caring for children with suspected sexual abuse. The case scenario reflects that as a part of the nursing process, it is imperative that a pediatric nurse establishes therapeutic communication to provide evidence- based, culturally sensitive, context specific and holistic care to restore the well-being of the traumatized child and family members.

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Tympanometry in Neonates- A Comparative Study-Juniper Publishers

Abstract

Objective: Impairment to hearing during childhood may lead to delay in the speech and language skills, making the communication process more difficult. Early identification and rehabilitation can benefit the children. It is necessary to test the hearing using accurate test procedures. Thus, aim of the study was to compare the use of probe tones (226Hz and 1000Hz probe tone) in tympanometry while testing the neonates for hearing before getting discharged from hospital setup.

Method: An inclusion - exclusion criteria was applied in selection of 60 neonates (1-17days). Hearing screening with comparison of 226Hz and 1000Hz probe tone in tympanometry was carried out using a comparative study design. Pressure ranging from + 200 to -400 daPa and a pressure change rate of 600/200 daPa per second was used for testing. Intensity of probe tone was adjusted at 85 dB SPL. Comparison was made for type of tympanogram, ear canal volume, compensated static peak acoustic admittance and tympanometric peak pressure. Sampled paired t test was used to carry out statistical analysis.

Results: 1000 Hz probe tone surpassed the 226Hz probe tone in testing the neonates using tympanometry. A statistically significant difference was observed in all the sub-sections except similar values for ear canal volume.

Conclusion: Many changes occur in the ears of neonates like changes in bone density, mesenchymal loss which has an impact on the mechanical properties of the auditory canal and middle ear system. Thus, 1000Hz probe tone in tympanometry serves a promising tool.

Keywords: Probe tone; Neonates; Immittance audiometry

Abbreviations: DPOAE: Distortion Product Otoacoustic Emissions; SP: Single-peaked tympanogram; TPP: Tympanometric peak pressure; DP: Double-peaked tympanogram; IP: Inverted peaked tympanogram; A: Asymmetric tympanogram; F: Flat peaked tympanogram; Ymt: Compensated static peak acoustic admittance; ECV : Ear Canal volume

Introduction

Normal hearing ability is the main source for development of speech and language skills. Any impairment to hearing during childhood may lead to delay in the speech and language skills, making the communication process more difficult. However, early identification and rehabilitation if given can work for the benefit of the children. Therefore in neonates, it is recommended to carry out a complete hearing evaluation before moving out from the hospital setup post delivery. In addition, it is necessary to focus on the test battery used for testing the hearing of neonates. In accordance with the literature, there still persists a debate on using 226Hz probe tone and 1000Hz probe tone for testing the neonatal hearing.

Tympanometry is an umbrella term encompassing impedance, admittance, and their components. It is defined as mechanical analysis of the auditory system in response to acoustic stimulation. Acoustic immittance measures the acoustic energy transfer that occurs when sound pressure is applied to the tympanic membrane causing its movement. Thus it helps to evaluate the ease or opposition to this sound energy flow within the auditory system. Today, its contribution to clinical diagnosis has aided to better middle ear status diagnosis and has now become a routine part of the audiological test battery approach ranging towards neonates to geriatrics.

In the early years of life up to two years, normal ear tympanic- ossicular system acts differently as mass is the dominating physical feature of the ear. After this age, reaching the adult stage, there is a change in behavior which is controlled by stiffness and is better evaluated using probe frequency of 226 Hz [1]. Thus carrying out conventional tympanometry using 226Hz probe tone at early years of life may not serve of much help. Therefore, condition of the middle year in the early stages of life can be evaluated more expeditiously using high frequency probe tones such as 678 Hz and 1,000 Hz. Conventional tympanometry using 226Hz probe tone in neonates and infants has been used by many audiologists [1-4], although the literature shows that the use of a single frequency is not sensitive enough to detect all cases of middle ear pathology which hinders accurate diagnosis [5 -8].

Tympanometry using high frequencies help to clear up the false-positive screening results that pass off due to pathology in the middle ear or the presence of secretion. Adequate middle ear assessments in the neonatal period results in suitable medical and audiological referrals and can thereby improve the efficacy of newborn hearing screening programs [9]. Evidence from previous literature suggests that the use of a single frequency probe is not highly sensitive enough to identify all cases of middle ear alterations, thereby making diagnosis difficult. Therefore, the purpose of this study was to characterize tympanometry measurements in neonates between 0-17days of age using 2 probe tones.

Materials and Methods

The study was undertaken with the approval of ethical committee formed at Nitte Institute of Speech and Hearing, Mangalore. To conduct the study, following inclusion criteria was considered for the participants of the study:

A. Neonates of 1 to 17 days,

B. Neonates administered and screened for high risk register using Joint Committee on Infant Hearing [10] to rule out possible presence of hearing impairment,

C. Ear canal screened to examine the possible obstacles,

D. Neonates with bilateral Distortion Product Otoacoustic Emissions (DPOAE) present.

A Term of Free and Informed Consent form was furnished to the Parents/caregivers which contained information about the study in very clear and uncomplicated language. Once the parental consent was signed and obtained the study was continued. The study incorporated 60 neonates: 21(35%) female and 39 (65%) . The age ranged between from 1day to 17 days, with an average age of 2.9 days of life. A total of 120 years were evaluated right (100%) and left (100%).

Audiological interview was carried out to begin with the screening assessment, which was followed by visual inspection of the pinna and ear canal, Distortion Product Oto-acoustic Emission testing and tympanometry measurements. Audiological interview in collaboration with information from hospital files was carried out with the parents/caregivers using leading questions, in simple and uncomplicated  language. The purpose was to serve the information regarding neonatal hearing, presence of any upper respiratory tract infection, and high risk indicators for hearing impairment.

Visual inspection of the pinna, ear canal and tympanic membrane was carried out by an experienced clinical audiologist to rule out any disturbances in testing of tympanometry. If any disturbances were encountered the neonate was referred to the Oto-rhino-laryngologist for the further evaluation.

To acquire the tympanometry measurements, Interacoustics AT 235h impedance audiometer was used. Following the specifications of the equipment manual, tympanometry was applied using 226Hz and 1000Hz frequencies, with the pressure ranging from + 200 to -400 daPa and a pressure change rate of 600/200 daPa per second. The intensity of probe tone was adjusted at 85 dB SPL for 226Hz and 1,000 Hz.

Two forty tympanograms were obtained: 114 (95%) with a 226 Hz probe tone, 109 (90.8%) with a 1,000 Hz probe tone. The occlusion encountered during the testing procedure lead to unequal distribution of the tympanograms. In the consequence of occlusion with a 226 Hz and 1000Hz probe tone, the probe was removed from ear and repositioned to restart the testing.

The obtained Tympanogram shapes were categorized as either a single-peaked tympanogram (SP) with maximum tympanometric peak pressure (TPP), a double peaked tympanogram (DP) with two-peak TPP, an inverted peaked tympanogram (IP) with an inverted TPP, an asymmetric tympanogram (A) with a gradual decline of TPP ranging from +200 to -200 daPa and, or a flat peaked tympanogram (F) with no TPP. Similarly, compensated static peak acoustic admittance (Ymt) mentions the maximum peak admittance, where the pressures of the external and middle ear are equal. The maximum admittance peak pressure is expressed in daPa.

With the previous literature [10-12], using 226Hz probe tone SP or DP tympanograms were classified as normal. For 1,000Hz frequency, tympanograms were classified using the Sutton Protocol [13], used for infants upto the age of 4 months. Shadowing this protocol, tympanograms with Ymt>0 and TPP>-200 daPa were considered as normal. Tympanograms with Ymt<0 or TPP < - 200 daPa were considered as abnormal. On classification of tympanograms as either normal or abnormal, the tympanograms with the two different probe tones were compared. In addition, to the literature, single-peaked and double-peaked tympanograms are considered normal, while asymmetric, inverted, and flat tympanograms are considered abnormal [13-16].

Results

Tympanometry was carried out in 60 healthy neonates using 226Hz and 1000Hz probe tone. The tympanometry measures were compared in accordance with type of tympanogram, ECV, Ymt, TPP. The obtained measures for 226Hz and 1000Hz were compared to know the efficacy of the probe tones. Sampled paired t test using SPSS version: 16 was used to obtain a statistically significant difference between the two probe tones.

On comparing the type of tympanograms, 226Hz elicited 71 Single Peaked tympanogram with maximum tympanometric peak pressure and 49 flat peaked tympanogram (F) with no TPP. However, lGGGHz elicited B3 Single peak tympanogram with maximum tympanometric peak pressure, 26 double peak tympanograms with two-peak TPP and llflat tympanogram with no TPP (Figure 1).

Using sampled paired t test it is evident that 1000Hz probe tone is more compatible for neonatal hearing testing then 226Hz probe tone with respect to ECV, Ymt and TPP. However there is a significant difference between the ECV and Ymt. Table 1 shows the statistical differences between the three parameters (Table 1).

Using the 226Hz and 1000 Hz probe tone, the ECV values were almost the same. There was less variation observed using 226Hz and 1000Hz probe tone frequencies. The sampled paired t test revealed no significant difference for the same. However, using 226Hz and 1000Hz probe tone Ymt and TPP were not the same. Sampled Paired t test revealed a significant difference between Ymt and TPP using 226Hz and 1000Hz probe tone in neonates.

A significant difference was observed, when the 226Hz and 1000Hz probe tone frequencies were compared. This indicates that the 1000Hz probe tone surpassed the limitations of 226Hz probe tone in the mass dominating middle ear system of the neonates with better measurements.

Discussion

The results were earned by grouping the right and left ears to facilitate better statistical analysis, as there was no significant difference found between the ears in the previous studies. In the present study a statistically significant difference across two subsections was seen.

It determines compensated static acoustic admittance. For 226 Hz probe tone, the normal range of ECV is between 0.3 and 1.0 mL [17-19]. Literature reports a mean of about 1.5 mm [6,11]. GRASON-STADLER [20] reports, ECV values acquired with a frequency of 678 Hz are 3 times larger than the ECV values obtained at 226 Hz, and that at 1,000 Hz probe tone. This difference can be up to 4.4 times larger. The current study reports that the ECV values obtained using high frequencies are greater with 1000Hz than values obtained with 226 Hz. Consistent with this statement, a significant difference between the results of the 3 frequencies was found, with the mean ECV value at 1,000 Hz greater than the mean values at 678 Hz and 226 Hz [14].

In the present study 226Hz probe tone obtained single and flat peaked tympanograms however; 1000Hz probe tone lead to single, double and flat peaked tympanograms. The possible tympanometry measures leading to differences in the type of peaks could be attributed to the possible presence of cerumen in the ear canal, placement of the probe in the infant ear, or differences in the middle ear system of infants [20]. Similarly, the presence of unequal distribution in number of tympanograms could be due to the presence of occlusion effect. Infants younger than 4 months old have higher chance of obtaining occlusion effect during the testing [11] and a low occurrence of flat tympanograms, indicate possible presence of fluid in the middle ear.

Similarly, literature reports, occurrence of single-peaked tympanograms to be predominant [11,20], while other studies indicate a preponderance of double-peaked tympanograms in neonates and infants using 1000Hz probe tone [12,21]. In addition, a higher incidence of single-peaked tympanograms [11,14], followed by flat tympanograms is also quoted [14]. Single-peaked and double-peaked tympanograms are considered normal, while asymmetric, inverted, and flat tympanograms are considered abnormal [13-16]. Literature reports different findings with 226Hz and 1000 Hz probe tones. The uncommon characteristics of the tympanograms obtained from newborns and babies, may be attributed to the physiological differences concerning the ears from newborns and adults [22]. Literature reports high frequency tympanometry provides more detailed information about the state of the mechanics and acoustics of the ear, especially for changes related to the mass factor [23-24], as well as the possibility of middle ear pathology at the initial or final stage. To conclude, 1000Hz probe tone reduces prevalence of flat tympanograms in newborns and 1000 Hz probe tone has also been identified as having greater sensitiveness to identify mild middle ear disorders [25].

The differences observed in obtaining tympanometric curves in neonates can be attributed mainly to normal variations in the subject population and also by the differences in the age of neonates. During growth there are many changes that occur in the ears of neonates, which have an impact on the mechanical properties of the auditory canal. Among the physical changes, differences are seen in the external ear and in the middle ear growth such as enlargement of the external ear, mastoid and middle ear cavity, changes in tympanic membrane orientation and tympanic annulus fusion. Some other variations such as changes in bone density and mesenchynal loss occuring during development could also play a major role. These changes could be related to the acoustic changes influencing the recording of the tympanogram with compensated static peak acoustic admittance [6, 16, 26, 27].

In addition, mass components are larger in high frequency and lower in the low frequency tympanometry [6, 22]. The normal middle ear is primarily dominated by the stiffness of low frequency sounds (226 Hz). In a higher frequency (for instance: 1000 Hz), the relative participation of each anatomical structure is changed and the acoustic admittance measured at the middle ear inlet becomes more predominated by the mass [12].

The admittance measurement in tympanometry indicates changes in the middle ear, literature reports higher mean Ymt values at 1,000 Hz compared to 226 Hz [11,13]. Mean Ymt values equal to 1.06 mmho when evaluating neonates [28]. The results of the current study are in acceptance with the literature. On statistical analysis 1000Hz probe tone obtained higher values of Ymt in comparison to 226Hz probe tone. Low frequency tympanometry in newborns and babies younger than six months has low sensitivity, in other words, the high level of false-negatives.

While comparing the tympanometric peak pressure (TPP) obtained with 226 Hz and 1000Hz. The current study indicated a higher TPP value with 1000 Hz than with 226 Hz. Similar findings have been reported in the literature [3, 6]. Although there were statistically significant differences between the frequencies with respect to TPP, none of the differences were clinically significant. That is, the results for all 2 frequencies were within the normal range [13].

In addition, testing neonates using tympanometry, tympanograms with probe tone of 1000 Hz are reliable, easier to interpret than 226Hz probe tone. It is easy to interpret and more reliable (91%) using 1000Hz than the 226 Hz (35%) probe tone, which shows a significantly better result in the assessment of the middle ear system. Similarly, in infants younger than 3 months of age, 226 Hz probe tone resulted in 58% of false-positive results [25].

The results of the current study are in accordance with the findings in the literature. These findings provide evidence for further obtaining normative values at different ages to adapt clinical practice to the use of high frequency probe tones with young children.

Conclusion

The current study aimed at comparing the two probe tones and its measurements in tympanometry. The neonates were considered as the subjects of the study. The difficulties met by 226Hz probe tone were seen to be surpassed by 1000Hz probe tone in neonatal tympanometry acoustic measurements. Neonates can be well diagnosed using a 1000HZ probe tone using tympanometry. However, further is warranted as the sample size used in current is small.

Informed consent

Informed oral consent was obtained from all individual participants included in the study.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Outcome of 'CDC Kerala' Early Stimulation Model on Neurodevelopment of Late Preterm Infants-Juniper Publishers

Abstract

There is increasing evidence that late preterm babies (Gestational Age 34-0/7wks to 36-0/6 wks) are at increased risk of short term developmental morbidities as compared to term counterparts. Cochrane review had shown that early intervention programs for late preterm infants have a positive influence on motor and cognitive development on short-medium term. Early intervention programs like NIDCAP (Newborn Individualized Developmental Care & Assessment Program), IHDA (Infant Health & Development Program) are best evaluated. Parent based studies have shown positive influence on neurodevelopment outcome. Present study assesses the outcome of parent based early stimulation model on neurodevelopment of late preterm infants by corrected age of 12months.

Keywords: Late preterm babies; Neurodevelopment outcome; Early stimulation; Parent based intervention; Developmental screening tools; Developmental assessment scale for Indian infants

Abbreviations: CDC: Child Development Centre; LP: late Preterm; DASII: Developmental Assessment Scale for Indian Infants; DQ: Deviation Quotient; MeDQ: Mental Deviation Quotient, MoDQ: Motor Deviation Quotient, BSID: Bayley Scales of Infant Development; TDSC: Trivandrum Developmental Screening Chart; DDST II: Denver Developmental Screening Test; A T angles: Amiel-Tison angles

Introduction

The near term terminology has been replaced by late preterm for babies born between34-0/7 to 36-6/7Weeks’ of gestational age. This emphasizes that last 6 weeks of gestation represent critical period of growth and development. Studies have shown that these babies are at three times higher risk for morbidity and mortality than their term counterparts [1,2]. Indian study done by Wagh & Jain reported higher neonatal morbidities in LP babies like need for resuscitation, hypoglycemia, feeding problems, sepsis and hyper bilirubinemia than term controls. They shared concerns of growth and development at 3 months of corrected age in these babies [3]. An inverse relationship between gestational age and risk of developmental delay at 18 months of corrected age was found by Luisa et al. [4].

With better understanding of importance of early enrolment of these babies in early stimulation programmes, their Neurodevelopment outcome has improved [5,6]. Systematic review done by Orton et al. gives account of various early intervention models for these babies [7]. CDC model of early stimulation is parent based, home cantered model which has shown its effectiveness in RCT [8]. Present study assesses effectiveness of this model on Neuro developmental outcome of LP infants.

Materials and Methods

Retrospective, cross sectional analysis of enrolled cases between March & April 2014 which were followed up to 12months of corrected age at CDC, Kerala.

A. Inclusion criteria: LP Infants (Gestational age 34-0/7 to 36- 6/7 weeks) attending CDC Newborn Follow up clinic.

B. Exclusion Criteria: Major congenital anomalies, visual and hearing impairments.

C. Sample Size: 53 late preterm babies.

D. Intervention & monitoring:

i. CDC model of early stimulation (ES) monthly till 12months of Corrected Age

ii. CDC model ES is indigenous, mother oriented program,designed for babies 0-12months, executed by multidisciplinary team. Its effectiveness is proven in RCT across all birth weight groups [8].

iii. Developmental Screening Tools - TDSC, DDST II, AT angles, CDC grading of major motor milestones.

iv. Simultaneous assessment of vision, hearing, lactation and feeding was done.

v. Home program: Hands on training on ES techniques encompassing all developmental domains to mother/ care giver and encouraged to do the same many times at home.

vi. Outcome Measure: Evaluation by DASII at 3-5 months (1st DASII) & 12 months (2ndDASII) of corrected age- Mental age, Motor age & deviation quotients derived. MeDQ, MoDQ value 80 and above were taken as normal as and less than 80 as abnormal.

vii. Developmental Assessment Scale for Indian Infants (DASII) is an Indian standardized adaptation of BSID to assess motor, cognitive development of children up to 30 months of age.

Statistically analyzed with variables - weight, gestational age, National Neonatology Forum (NNF- India) risk factors, parent education and socioeconomic class (Table 1).

Babies with mild and moderate National Neonatology Forum- India risk factors had less abnormal Deviation Quotient.

Results

A. Total 53 babies satisfied inclusion exclusion criteria. 53% (28) were males and 47% (25) were females. 40% (21) babies were normal at 3, 12 months. With respect to socioeconomic class 43% subjects were BPL (Below Poverty Line), 57% were APL (Above Poverty Line). No parent was illiterate.

B. Mother Education- 10th std- 2, 12th std- 25, Graduation- 25, Post graduation- 1

C. Father Education- 10th std- 1, 12th std- 21, Graduation- 27, Post graduation- 4

D. Birth weight & Deviation quotients of DASII- (Table 2)

As the birth weight increases, percentage of abnormal Deviation Quotient decreases

E. As the birth weight increases, percentage of abnormal DQ decreases.

F. All babies with weight > 2.5kg had normal Me, Mo DQ at 12 months.

G. Small for Gestational Age (SGA)/ Appropriate for Gestational Age (AGA) & Deviation Quotients of DASII- (Figure1)

H. SGA babies were 75%. Abnormal DQ were more common than in AGA babies. Improvement in DQ at 12months was more with AGA babies than SGA babies (DQ improvement in SGA- Mo 87.5%, Me 87.5%, AGA Mo 92.5%, Me 100%).

I. Gestational age & Deviation quotients of DASII-

J. 34wk (28%), 35 (34%), 36wk (38%). DQ improvement more as gestational age advances. Improvement in MeDQ at 34 wks- 50%, 36 wks- 100%, MoDQ at 34 wks- 50%, 36 wks- 80%.

K. NNF risk factors & deviation Quotients-

L. Common Risk factors PROM (13 babies), Absent/ reversal EDF 8, Shock 3, Ventilation 3, hypoglycemia 3, Eclamsia 3, Jaundice 3, multiple pregnancy 2.

M. 7 Babies continued abnormal DQs at 12m- reversal/ absent end diastolic flow, prolonged ventilation, IVH grade II- III, low birth weight & PROM.

N. Improvement in Mental Outcome at 12 months- (Figure 2).

O.Out of 18 babies with abnormal MeDQ at 1st DASII, 16 babies normalized by 12 months.

P.94% babies had normal Me DQ at 12m.

Q. Improvement in Motor Outcome at 12 months- (Figure 3).

R.Out of 24 babies with abnormal MoDQ at 1st DASII, 20 Normalized by 12m.

S.89% babies had normal Mo DQ at 12m.

Discussion

Neurodevelopmental outcome in LP infants predominantly depends upon cause of prematurity, SGA/ AGA, associated risk factors [1,2,9]. High percentage of SGA babies was observed in our study, similar to findings of Lackman et al. [10]. With increasing birth weight & gestational age, Neuro developmental outcome of LP infants improved in our study. The same was observed by Schonhaut et al. & Carrie et al. [4,11].

7 Children who continued to have below average DQs at 12m were having severe risk factors. A similar observation was made by Engle et al. [12]. Review of parent based Early Intervention RCTs show positive, clinical, meaningful effects on cognitive and social development of LP infants [7,12,13]. CDC ES model starting from stimulation at NICU, lactation management, multisensory stimulation, activities based on developmental milestones has shown improvement in psychomotor functioning at one year, better parent child bonding in at risk babies [8].

Conclusion

A. Early stimulation pivoting around parent-child in LP babies has positive influence on Neurodevelopmental outcome at 12 months of corrected age.

B. Late preterm is a population at risk, to be monitored.

C. Association between NNF risk factors, primary cause of prematurity and short term Neurodevelopment outcome in LP infants need to address with larger sample size prospective study

Acknowledgement

Rajee Krishnan Ramkrishnan Unnithan, Developmental Therapist, Lekshmi Madhavan Amritham,Preschool Teacher, CDC, Medical College, Thiruvanathapuram.

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Congenital Prepubic Sinus (Type 2 Stephens Variant of Epispadiac Dorsal Urethral Duplication): An Uncommon Anomaly-Juniper Publishers

Abstract

Variants of congenital prepubic sinus have been reported rarely, and because the anatomic features often differ from each other, a consensus concerning the embryology and classification was not achieved yet. Various names including congenital prepubic sinus, sub pubic fistula, prepubic dermoid sinus, and suprapubic dermoid sinus, were used to identify these lesions, and, among the classifications available, none seems to clearly describe this entity. We present a 2-year old boy with a case of epispadiac variant of urethral duplication in which the duplicated urethra presented as a prepubic sinus. We report this uncommon anomaly and review the scattered published reports to improve the global understanding of this uncommon congenital lesion.

Keywords: Urethral duplication; Epispadias; Congenital prepubic sinus; Sinus tract

Introduction

Congenital prepubic sinus (CPS) is a rare anomaly of uncertain etiology. The sinus usually presents as a small tract, commencing on the skin overlying the penis or prepubic area, and extending toward the anterior bladder wall or umbilicus [1,2]. The anatomic and pathologic features of this disorder have been documented, but controversies over its embryologic basis are ongoing.

Case Report

A 2-year-old male child was presented with congenital opening over the dorsal surface of the penis. He was asymptomatic except for occasional clear discharge from the opening. Child was passing urine through the normal urethral opening. Local examination revealed a deformed penis with ventrally hooded prepuce and 8mm midline epispadiac opening over the dorsal surface of the penis. A number 8 infant feeding tube could be easily passed in the opening for a distance of 3cm and could be felt going under the pubic symphysis (Figure 1a & 1b). Fistulography was showing blind ending tract around 2.5cm long going up towards the prevesical space without any communication with bladder (Figure 2a & 2b). At operation, the tract was dissected and was found to be going under the pubic symphysis (Figure 3a & 3b). The tract was traced up to prevesical space, transfixed, ligated and  divided. Specimen (2.5cm sinus tract) was sent for histopathology which showed tract lined by transitional epithelium and focalinflammation (Figure 4).

Discussion

Congenital prepubic sinus is a tract originating in the skin overlying the symphysis pubis, superior to the base of the penis or clitoris, and extending to, but not communicating with, the anterior bladder wall [3]. There are four generally proposed theories for the etiology of Congenital prepubic sinus: First anomaly of abdominal wall closure [4]; and second urethral developmental anomaly, a variant of dorsal urethral duplication [1,2,5-8]. The third theory is that it is a congenital fistula of the primitive urogenital sinus, with three anatomic subtypes depending on the direction of the sinus tract: high, toward the urachal remnant; middle, toward the bladder; and low, toward the prostatic urethra [9]. Fourth theory suggests that it is a remnant of the cloaca [10]. Of all these theories, reports supporting the anomaly of dorsal urethral duplication predominate [1,3,5-8,11]. As these theories cannot explain all the varied presentations of CPS, Tsukamoto et al. postulated recently that CPS may be caused by a  residual cloaca membrane and umbilicophallic groove, and that the depth may determine the position of the end of the sinus tract [12]. However, Huang et al. [1] used an immunohistochemical staining technique of the excised sinus to reinforce the theory of dorsal urethral duplication in a report of five patients with congenital prepubic sinus, when they found the presence of transitional epithelium in the proximal part of the sinus with surrounding smooth muscle bundles [1]. Balster et al. [11] in 2003 also supported this assumption with an immunohistochemical study on the excised sinus tract of a 2-year-old boy with a skin fistula on the dorsal side of the penis [11]. Urethral duplication remains a rare and confusing problem, more so when it presents as a prepubic sinus. Urethral duplication does not represent a uniform entity making it difficult to find an unequivocal and comprehensive classification. Stephens described three types of dorsal urethral duplication according to the anatomy [13]. Type 1 is a complete or incomplete channel that runs parallel to the normal urethra from the glans to the bladder, which may join the urethra or ends blindly. Type 2 is an epispadiac type of channel from the dorsum of the penis to the bladder or one that joins the urethra at some point. Type 3 is a dermoid sinus that simulates an accessory urethra but tracks from the base of the penis in front of the pelvic urethra and bladder behind the pubic symphysis to or towards the umbilicus. We found only 7 reports of 9 cases of epispadiac variant of dorsal urethral duplication in the English literature (Table 1) (6,8,14-18). The similarity of the anatomy of our case to the type 2 variant of the Stephens classification favours the theory that Congenital prepubic sinus is a variant of dorsal urethral duplication. The presence of transitional epithelium in the lining of the sinus in this patient reinforced this theory. Although the tract ended blindly toward the anterior bladder wall, the presence of dorsal chordee, a ventrally hooded prepuce as well as penile torsion supports an epispadiac variant.

Patient with Congenital prepubic sinus should be thoroughly evaluated because of its variable presentation, come to medical attention because of the opening or because of persistent discharge. Diagnosis is mainly clinical but imaging techniques such as micturating cystourethrogram and sinogram could help to outline the direction of the tract, and whether it is blind ending or communicating with the urinary tract. Treatment is individualized depending on the anatomy and severity of the anomaly, and usually consists of excision of the non dominant urethra or sinus tract, usually the dorsal one. Excision is usually curative.

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Intervention Strategies for Successful Breast Feeding: Randomized Clinical Trial-Juniper Publishers

Abstract

Breastfeeding is one of the most natural and beneficial acts a mother can do for her child. There are many possible nipple problems that breastfeeding mothers may encounter. The 7mm nipple length might be a possible screening indicator that would signal the clinician to provide more intensive breastfeeding monitoring (puapornpong, 2013).

Objectives: Comparison of selected nursing interventions like manual technique, rubber band and syringe method on successful breastfeeding pattern among the antenatal mothers.

Methodology: Randomized clinical trial study was conducted among 90 women having nipple problem, 30 in each group i.e., group I(manual technique), group II (rubber band) and group III (syringe method) in the selected government maternity hospital, Pondicherry, India. Samples were selected by simple random sampling. The outcome of study was evaluated by Descriptive and Inferential statistics.

Results: The demographic variables shows that majority of the women 26(86.70%), 23(76.70%) and 23(76.70%) were in the age group of 19-25 in group I, II, III respectively. 13(43.3%) women in group I, 13(43.3%) in group II and 18(60% ) in group III, ware studied up to High School level. Result on breast feeding pattern shows that there was improvement in feeding. Mothers not faced any difficulties for feeding the baby after delivery. Each intervention strategy shows equally good in case of nipple problem.

Conclusion and recommendation: These intervention strategies are very simple and cost effective, so this can be practiced in all settings to correct the nipple problems.

Keywords: Nursing interventions; Successful breast feeding; Intervention strategies; Antenatal mothers

Introduction

Breast milk is best for the baby, and the benefits ofbreastfeeding will extend well beyond basic nutrition. Breastfeeding is one of the most natural and beneficial acts a mother can do for her child. There is no other single action by which a mother can so impact the present and future health of her baby [1]. Colostrums is the first milk, yellow in color secreted by breast soon after delivery. Dramatic health benefits have been proven to pass from mother to child through breast milk. Breast milk is packed with disease-fighting substances that protect the baby from illness [2]. Breastfeeding currently saves six million of lives each year preventing diarrhea and Acute Respiratory Tract Infections. A breastfed child is 14 times less likely to die from diarrhea, four times less likely to die from respiratory disorders and 2.5 times less likely to die from other infections compared to nonbreastfed infants [3]. Breast feeding the newborn is a satisfying and sometimes anxiety provoking task [4]. The 7mm nipple length might be a possible screening indicator that would signal the clinician to provide more intensive breastfeeding monitoring and support the postpartum mothers. If the nipple measure less than 7mm, it should be cared and corrective measures should be initiated [5].

The abnormalities of the nipple includes long nipple, short nipple, abnormally large nipple, inverted nipple, flat nipple, retracted nipple and cracked or damaged nipple [6-8]. The occurrence of inverted and flat nipples is not uncommon in the practice of newborn care. Although such conditions should not preclude breastfeeding if expert counseling and advice on proper positioning are available, many mothers get frustrated and quit breastfeeding. Nipple problems may lead to a delay in breastfeeding initiation and thus deprive the baby from getting the benefits of colostrums. Inability to attach at the breast causes infrequent suckling and may lead to breast engorgement, and if the mother is not shown how to maintain an adequate supply through expression of milk, the production of milk is likely to decrease. It was estimated that about 10% of pregnant women have inverted or non-projectile nipples, which hinder breast feeding [9]. The National Family Health Survey revealed that the breastfeeding rates in different states were: Tamilnadu- 55.3%, Kerala- 55.4%, Maharashtra- 51.8%, Mizoram- 65.4%, Meghalaya- 58.6%, Orissa- 54.3%, Goa- 59.7% and Assam-50.6%. Breastfeeding rates in Punjab, Uttar Pradesh, Bihar, Rajasthan, Madhya Pradesh, and Delhi were below 40%.

The breastfeeding rate in Karnataka is 45% [10]. Various methods to correct flat and inverted nipples with varying degrees of success and complications have been reported in the literature. Some of them were prenatal exercises like Hoffman's exercise [11], nipple stimulation techniques [12-14] and postnatal use of the Nipplette (Philips Avent, Andover, MA) etc [15]. The best simple method to date had been reported by Kesaree et al. [16] the inverted syringe method to pull out the flat or retracted nipple as well as using breast shells to make the nipple prominent [17].

Objectives

a) To assess the nipple condition in group I, II & III women (mother with manual technique, Rubber band and syringe method) mothers before and after the intervention.

b) To evaluate the effectiveness of strategies on successful breast feeding.

c) To assess the level of satisfaction among the women on the strategies.

d) To associate the post-test level of breastfeeding pattern and level of satisfaction with the selected demographic and obstetrical variables.

Material and Methods

After getting ethical clearance from the concerned authority this study was conducted in the government maternity hospital, Pondicherry All antenatal women with nipple problem who came for delivery were considered as sample for this study. The sample size was 90 women with nipple problems out of which 30 mothers were selected by simple random technique for each three group. All the women were selected based on the inclusion criteria. Reliability of the tool was checked by the inter rater reliability technique and it was found that the tool was reliable. The correlation was calculated by using the formula Cronbach's Alpha for Internal Consistency. The obtained reliability coefficient r=0.9 was highly reliable. Data collection was done in the antenatal wards, those who admitted for safe confinement, willing to participate, had any nipple problem. Oral and written informed consent was obtained from each mother and purpose was explained. Before starting the pre-test the researcher got introduced to all the mothers. First the nipple condition (which type of nipple problem) was assessed. To each group mothers the concern technique was demonstrated (group 1-manual technique, group 2- application of rubber band and group 3-syringe method) and instructed them to repeat the techniques 3-4 times a day for 5 minutes for 7 consecutive days.

The effectiveness of the interventions assessed in the postnatal period with the help of Christi Breastfeeding assessment scale to assess the breastfeeding pattern [18,19]. Inferential and Descriptive statistic was used for analysis (Table 1).

Results

The demography pattern shows that, majority of the women 26(86.70%), 23(76.70%), 23(76.70%) were in the age group of 19-25 years and 13(43.3%), 13(43.3%), 18(60%) had high school level of education, 18(60%), 20(66.6%), 13(43.3%), mothers were from urban area in the group I, II & III respectively. None of the mother in each group 30(100%) had done any type of nipple erection exercise throughout the antenatal period. The obstetrical data revels that 27(90%), 26(86.7%), 28(93.3%) mothers undergone vaginal delivery in the group I, II and III respectively The mean level of breastfeeding pattern was 6.57+1.50 in group I mothers. In group II & III mothers, the mean level of breastfeeding pattern was 6.03+0.76 & 6.00+1.00 respectively. All the mothers in each group were satisfied while feeding the baby. There was no association found between the feeding pattern and to their demographic variables.

Discussion

The mean level of breastfeeding pattern was 6.57+1.50 in group I mothers. In group II & III mothers, the mean level of breastfeeding pattern was 6.03+0.76 & 6.00+1.00 respectively. Dr Kamalendru Chakarabarti, [20] conducted a study on "Managing the nipple problem" among mothers in Kolkata, who came for clinic. She compared the two methods for nipple problem. The standard method of treating nipple problem with syringe technique has some problems. The nipple does not protrude after  syringing in most cases. It has always to be done under medical supervision. In case of rubber band, the nipple stays protruded and though it is always better to done under supervision. Once the mother learns the procedure, she may not require strict supervision.

Jain S [21] "newer innovation in treatment of retracted nipple” in VCSGGMS&RI, Govt. Medical college at Srinagar from march to august 2011, in which 213 women having retracted nipple, 71 in each of the three groups was carried out. Group A (syringe method) Group B (rubber band method) and Group C (sucking by the husband). Highest success rates were seen in group C (vigorous sucking by the husband), being 52, 88 and 94% on days 3, 7 and 14 days respectively. Success rates in group B were lower, being 43.66 and 74% respectively and lowest in group A i.e., 33.47 and 64%, respectively on corresponding days.

Conclusion

Breastfeeding has been important since the beginning of mankind. In approximately 1800 BC Hammurabi's code regulated the behavior and the health of wet nurses. In Sparta, Greece, Spartan women were required to nurse their eldest son. This was the child who was expected to inherit the family name. At other times during history breastfeeding has been seen as something that only lower class or poor people did [21]. Encouraging women to breastfeed presents a major challenge to health care professionals. Despite attempts to increase the number of women choosing to breastfeed, rates of initiation and continuation in many countries remain less than optimal. A focus both on initiating and continuing breastfeeding is important, since many women fail to maintain breastfeeding for recommended period [22]. The study result showed that all the three methods (i.e.) manual technique, rubber band and disposable syringe technique were effective in improving breastfeeding pattern. Although Disposable syringe has improved the breastfeeding pattern for postnatal mothers with nipple problem, Rubber band and manual technique was found to be more effective when compared to Disposable syringe method for successful breastfeeding pattern among postnatal mothers with nipple problem. So this selected technique can be applied as an adjunct intervention by nurses in their  day to day caring the postnatal mothers in hospital and community setting.

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Potential Protective Effect of Sickle Cell Gene Allele on HIV Infection-Juniper Publishers

Short Communication

Both Sickle Cell Disease and HIV Infection have overlapping worldwide distributions, with Africa being the epicenter for these two conditions (Figure 1 & 2) [1,2]. Sickle Cell Disease is a genetic condition affecting chromosome 11 in which homozygous recessive individuals will have significant negative consequences secondary to sickling phenomenon of their red blood cells, while those that are heterozygous have a survival advantage based on a well-described resistance to malaria [3]. The Human Immunodeficiency Virus takes advantage of surface proteins on white cells, resulting in lytic destruction and consequent CD4+ lymphopenia and immunosuppression [4]. Both diseases have significant morbidity and mortality, with limited treatment options. Nevertheless, observations in our pediatric HIV program in Newark, NJ, one of the first epicenters for Perinatal HIV Infection (PHIV), suggested that children with sickle cell disease infrequently were infected with HIV despite being from a high-risk population5 (Table 1) [5]. Current understanding of the interplay of genetic impact on surface membranes supports the idea that genetic variations might impact susceptibility to infectious diseases. Similar to its protective effect against malaria, sickle cell trait may provide protection against acquisition of HIV infection through changes in CXCR4 and CCR5 receptors on CD4+ lymphocytes, possibly blocking viral entry and infection [6]. Potential mechanisms for this disparity might provide better understanding and approaches to HIV infection based on sickle cell status.

The authors have had a long standing hypothesis that the Sickle Cell Gene Allele (SCGA) may confer a protective effect against acquisition and/or progression of HIV infection. Rutgers NJMS in Newark, NJ has long been a center of PHIV care and research since the onset of the HIV pandemic, developing domestic and global clinical, educational and research expertise in PHIV infection leading to the control and subsequent prevention of PHIV in the US. Among our large population of PHIV African American children, we noted a lower than expected occurrence of Sickle Cell Hemoglobinopathy (SCH) suggesting relative protection against PHIV acquisition, and by inference, progression. However, though the data from the Newark PHIV program is suggestive of the potential protective effect of the SCGA, it lacks the patient base needed to achieve sufficient statistical power to confirm this potential association [5]. Sub-Saharan Africa has approximately 64% of the world's population living with HIV, 91% of children under 15 years of age living with HIV, and approximately 10-40% of the world's SCD population [1,7]. The population distribution of individuals in Africa with HbS allele and HIV are seemingly non-overlapping, supporting preliminary data from our pediatric cohort [1,2,5]. This non-overlapping distribution is unexpected, given the high prevalence of both Sickle Cell Disease and HIV in Africa. South Africa, having a low distribution of SCGA has one of the higher concentrations of HIV infectivity in Africa, confirming the significance of these observations. These findings should stimulate further investigation into the molecular basis for this protective effect, which could influence the direction of new therapies (drug, genetic, and immune-based approaches) for the treatment and prevention of HIV.

There are three lines of evidence that suggest Sickle Cell Hemoglobin (SCH) has a protective effect against HIV acquisition/ progression. First, anecdotal observations by investigators at Rutgers NJMS noted the above, which suggested lower transmission rates of PHIV among HIV exposed neonates with SCGA compared with those without [5]. Second, an epidemiological study by Nouraie et al. [8] documented a two-fold lower risk of HIV infection and comorbidity in patients with SCD among African-American adult patient hospital discharges. This effect was observed over a 13-year period in over 400,000 patient records. Further, the authors of this article offered several possible mechanisms for the apparent impact of SCGA on the course of HIV disease including up-regulation of heme oxygenase-1, hypoxia related to the anemia, higher expression of inflammatory cytokines, inhibition of HIV transcription in the presence of reduced iron, as well as use of hydroxyurea in SCD patients. Third, SCGA is already documented to have protective effects against other infectious diseases, such as Malaria. Although this conferred protection occurs by conformational changes in the hemoglobin molecule by the SCGA inhibiting the penetration and proliferation of the Malaria Parasite in RBCs, there may be other characteristics of this gene allele product, expressed on lymphocytes and other white blood cells that may also confer protection against HIV attachment, penetration and/or proliferation [9].

A possible mechanism of protection could exist within the genome of patients with SCGA. Past literature has already elucidated the mechanism by which certain Caucasians of Northern European descent have conferred resistance to HIV [10]. These persons possess a 32-base-pair deletion on the CCR5 white blood cell surface protein, one of two chemoreceptors utilized by HIV- 1 to enter the leukocyte. The CCR5Δ32 mutation found in these individuals results in a dysfunctional surface protein, preventing the virus from entering the host [11]. In the case of individuals with SCGA, the authors further propose that the TRlM5α protein could play a role in the protective effect. This protein disassembles and degrades viral capsid binding, preventing the pre-integration complex from arriving at the nucleus, thus interfering with reverse transcription [12]. A study in 2004 conducted by Stremlau et al. [13] identified TRlM5α as the blocking factor responsible for blocking HIV-1 strains from infecting Old World Monkeys13. Subsequent research has shown that rhesus TRlM5α (TRlM5αrh) appears to be more stable, and thus more potent, than human TRlM5α (TRlM5αhu), lending a reason as to why the majority of humans are immune to HIV-1 infection. Examination into the effect of various polymorphisms in TRlM5αhu revealed differing frequencies of HIV infection among African American cohorts. The TRlM5α 136Q polymorphism-indicating a glutamate at residue 136 in the TRlM5α protein - was identified as possessing a potential protective effect, as it was found at a greater frequency in the High-Risk Exposed Uninfected African American population [14]. Furthermore, the TRlM5α gene locus is found in close proximity to the HBB gene locus on human chromosome 11, lending the possibility that linkage disequilibrium interaction between the two could exist. Because a large proportion of African American individuals possess the SCGA, the authors further propose that a potential link between SCGA and TRlM5α 136Q may explain the lower-than-expected proportion of HlV-infected individuals with the SCGA.

While a recent systematic review concluded that SCD slows the progression of HlV into AlDS, and conversely HlV infection complicates the course of patients with SCD [15], no well controlled study of a relationship between the presence of SCGA and the acquisition or progression of HIV infection has been conducted. Such a study would identify the sickle cell status (SS vs. SC vs. SA vs. AA) in HlV-infected cohorts and, conversely, the HlV status in identified sickle cell cohorts. The population of HlV-infected patients who carry the SCGA would be the target population of a prospective study to determine the impact associated with the comorbidity of HlV infection and SCGA status. The high prevalence of SCGA and HlV infection in the Sub-Saharan African population and the preliminary observations of a possible impact of SCGA status and HlV incidence as well as the progression of HlV-1 disease, justifies further study of the possible interaction between these two significant global health problems. The results of such a study would have a major impact on the management and follow-up of individuals followed in these countries as well as encourage collaboration with and within these resource-constrained areas of the world to address the global health challenges experienced by much of the world's population.

Acknowledgement

Barry Dashefsky MD, Bart Holland PhD, MPH, François Dabis MD, MPH, Nathalie De-Rekeneire MD and Said Aboud MD are thanked for their contributions to this work.

Author Contributions

JO made original observation that SCD appeared in his population of PHIV infants to be underrepresented. HS assisted in recruiting international support for this hypothesis. JM consulted on presence of PHIV in her large cohort of SCD patients. AS provided technical support in data evaluation. KS, GG, JK, RM, PP, AD, OF, and FD provided technical support in data analysis and manuscript preparation.

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Chromosomal Foetal and Placenta Abnormalities Associated with Exomphalos and Umbicinal Hernia-Juniper Publishers

Abstract

Exomphalos is a rare congenital disorder demonstrated by failure of convergence mesoderm segments. Exomphalos is associated with congenital malformations and chromosomal abnormalities of autosomal or sex chromosomes and abnormalities with increased incidence in various systems such as renal and cardiovascular. The morbidity and mortality can be minimized by recognition of specific abnormalities associated with exomphalos development, the corresponding management and treatment strategies.

Conclusion: Exomphalos and umbilical hernias’ may be associated with chromosomal abnormalities in autosomal and sex chromosomes and are related with genetic syndromes such as paternal uniparental disomy (UPD), Pallister-Killian (PKS) and Beckwith-Wiedemann syndrome.

Keywords: Exomphalos; Umbicinal hernia; Chromosomal abnormalities; genetic syndromes; Abnormal karyotype

Introduction

Exomphalos separated in major and minor and could be associated with limb abnormalities, renal defects or caudal midline syndromes. Even though cardiac and renal defects are associated with the occurrence of exomphalos the prevalence is not yet associated with specific chromosomal abnormalities. Exomphalos may be associated with chromosomal abnormalities of both autosomal and sex chromosomes and other syndromes such paternal uni parental disomy (UPD), Pallister Killian (PKS) syndrome Beckwith-Wiedemann syndrome and feto placental chromosomal abnormalities (CMP). At this mini review study we will analyze the combination of exomphalos and umbicinal hernia with specific chromosomal abnormalities and genetic loci associated with the syndromes development.

Umbicinal Hernia and Chromosomal Abnormalities of Autosomal Chromosomes

Umbicinal hernia and exophalos are rare congenital conditions with an incidence of 2-3 cases per 10,000 newborns and are associated with chromosomal abnormalities in foetus and placenta [1,2]. The risk of umbilical hernia and exomphalos varies according to maternal age, gestational age, type of birth and genetic disorders [3,4].

Trisomes 13 and 18 are the most common chromosomal abnormalities associated with the occurrence of umbilical hernia. Snijders et al. [3] determined the appearance of omphalocele in 22.5% of foetuses with trisomy 18, 9.1% of foetuses with trisomy 13, 12.5% of foetuses with other triploid chromosomal abnormalities while 0, 45% of embryos had no chromosomal defects. According to literature the risk factors such as Trisomes 18 or 13 in foetuses with omphalocele is 340 times higher comparing with embryos with no exomphalos during 11th to 14th weeks of gestational age [5,6].

Chen et al. [7] in a study of 89 cases of trisomies 18 revealed 12 cases with omphalocele (13.48%) estimated male to female ratio of 2:1 [7]. Blazer et al studied 18 foetuses with omphalocele and diagnosed 11 cases (61.1%) with chromosomal abnormalities, most cases related to trisomy 18, 21 and one case with fetal karyotype 45 X [6,7].

Calzolari et al. [1,2] analyzed 160 embryos and found 94 cases of umbilical hernia (58.8%) with concomitant chromosomal abnormalities. In 60 cases revealed trisomies 18, 23 or 13, four cases with trisomy 21 and seven with other chromosomal abnormalities [1,2].

Gilbert & Nicolaides [8] studied of 35 foetuses with omphalocele found that 19 had a chromosomal abnormality, 17 of them trisomy 18 and one karyotype 47 XXY [8] while Brant berg in a study of 90 prenatally diagnosed omphalocele revealed 44 cases with chromosomal abnormalities most of them with trisomy 18, 13 and 21 [4,8]. Chromosomal abnormalities were found in majority of foetuses with central and epigastria omphalocele. On the other hand, Van de Geijn analyzed 22 foetuses with omphalocele and found 10 cases with autosomal chromosomal abnormalities, six with trisomies 18 and one with trisomy 13 [8,9].

Hughes et al. [10] studied 30 foetuses with omphalocele and found 13 cases who had chromosomal abnormalities. Most of the foetuses had trisomy 18, trisomy 13, two had a trisomy 21 and one Turner syndrome while Hwang & Kousseff [11] in a study of 93 cases of umbilical hernia found in 37 cases with chromosomal abnormalities, including trisomy 19, 18, 11, 21 and trisomy 13. Nicolaides et al. [6] in a study of 116 foetal of omphalocele revealed 42 cases (36.2%) of identified autosomal chromosomal abnormalities. Most of examined foetuses had trisomy 18, six had trisomy 13 [12].

Additionally, Eydoux et al. [13] in the one study of 46 embryos with omphalocele found that 12 cases  (26.1%) had chromosomal abnormalities and seven trisomy 18, trisomy 13 while Hsu et al. [14] studied 24 neonates with omphalocele and reveal six cases (25%) with chromosomal abnormalities such as trisomy 18 and 13 [14].

Even if several studies revealed the correlation between umbilical hernia and trisomy 21; Torfs et al. [15] found only one case of trisomy 21 among 2979 newborns with omphalocele and concluded that trisomy 21 is not predispose of increased risk for umbilical hernia. Mastroiacovo et al. [16] revealed seven cases of trisomy 21 among 8560 cases of umbilical hernia. At this study the ratio was 1/1200 which was significantly higher than 1 /425,000 of general population, suggesting that trisomy 21 increases the risk of umbilical hernia in fetus.

Umbicinal Hernia and Sex Chromosomal Abnormalities

Besides autosomal chromosome abnormalities exomphalos and umbicinal hernia can also be associated with chromosomal abnormalities of sex chromosomes such as monosomy 45 X and trisomies 47 XXY or 47 XXX [17-19]. In prenatal screening is important besides abnormalities of autosomal chromosomes to identify chromosomal abnormalities of sex chromosomes as possible key factors of umbilical hernia development.

Saller et al. [17] reported the presence of monosomy 45 X in a fetus with omphalocele while Govaerts et al. [18] identified a case of umbilical hernia in fetus with monosome 45 X and polyhydramnion by ultrasound analysis during gestational age of 22 weeks. Goldstein & Drugan [19] suggested that umbilical hernia in patients with Turner syndrome may be due to expression of some localized genes of X chromosome in early pregnancy. Several reports suggested that chromosomal abnormalities are more common in combination with omphalocele [20,21] while the cysts of umbilical cord increase risk of aneuploidy occurrence, mainly of trisomy 18 in fetuses with omphalocele [21]. Cysts umbilical related omphalocele usually are pseudo cysts and allantoid vesicles [21].

Syndrome Pallister-Killian (PKS) and Umbicinal Hernia

Pallister-Killian (PKS) Syndrome is a malformation characterized by mosaicism and tetrasomia of 12p genetic region. The PKS has the clinical features of local alopecia, severe mental retardation, seizures and frequent occurrence of diaphragmatic hernia while in some cases it may be associated with omphalocele. Tejada et al. [22] have reported a case of PKS with ultrasonographic features such as polyhydramnion, umbilical hernia and short length diagnosed with tetrasomia of 12p confirmed in cell cultures of skin fibroblasts.

Paternal Uniparental Disomy (UPD) of Chromosome 14 and Umbicinal Hernia

Paternal uniparental disomy (UPD) has been reported to be associated with multiple abnormalities such as thoracic hypoplasia, ribs abnormalities, laryngomalacia, hypertrophic cardiomyopathy and mental retardation [23]. Papenhausen et al. [24], Cotter et al. [25] and Kurosawa et al. [26] reported the correlation of umbilical hernia with occurrence of UPD of chromosome 14. Towner et al suggested that prenatal diagnosis of abdominal defect associated with increased nuchal translucency or skeletal abnormalities and coexistent of UPD of chromosome 14 [27].

Several abnormal characteristics are associated with UPD on different chromosomes. For example, genetic mapping on chromosome 11 and paternal UPD connected to Beckwith Wiedemann syndrome [28,29]. UPD of chromosome 15 has also been associated with the syndromes Prader Willi and Angelman [30] as well as the UPD of chromosome 16 may also associate with abnormal phenotypes [31,32].

Micro duplication of Genetic Region 15q11

According to literature referred a case report of a foetus with exomphalos, increased nuchal translucency and normal karyotype. The ultrasound analysis revealed exomphalos with micrognathia and tetralogy of Fallout. Although the karyotype was normal molecular karyotype revealed micro duplication of 408 kb in chromosomal region of 15q11.2.

Beckwith-Wiedemann Syndrome (BWS) and Exomphalos

BWS caused by impairments and deficiencies occurred in the chromosomal region of 11p15 [33-35]. This genetic region comprises the genes involved in the cell cycle, development and tumour suppression. The chromosomal location of 11p 15 is organized into two subareas comprising the genes 1GF2 and H19 and another centromeric region includes genes CDKN1C (Kinase Inhibitor 1C),  KCNQ1 (subfamily Q, potassium voltage-gated channels) and KCNQ1OT1 [34,35].

Mutations in CDKN1C gene (known as p57Kip2) demonstrated at 5% of patients with BWS [36]. Patients with mutations CDKN1C gene have a typical phenotype of BWS, with a very high frequency of exomphalos. The appearance of exomphalos associated quite strongly with the syndrome as 65% of patients with BWS may appear exomphalos [35,36].

Exomphalos occurrence is more frequent in patients with mutations in the genes KCNQ1OT1 and CDKN1C. If methylation status of KCNQ1OT1 and H19 genes is normal then sequencing analysis of CDKN1C is important especially in patients with family history of exomphalos [35,36].

Fetoplacental Chromosomal Abnormalities (CMP) and Exomphalos Development

The effects of chromosomal abnormalities in placental and foetus (CPM) are under investigation due to assess the participation of these genetic elements in exomphalos development [37]. CPM aberrations associated with growth retardation, and foetal death. The fetoplacental chromosomal abnormalities may lead to emergence of a different number of chromosomes of placenta and the foetus even if the karyotype is normal. These foetuses may reveal normal growth [38] or intrauterine growth restriction outcome that may result in intrauterine foetal death [39]. Drikos et al studied case of an infant diagnosed prenatally with exomphalos with intestinal contents from the 14th week of gestational age. Prenatal diagnosis by choral villi sample detected pseudomosaicism of placenta (mos45X/46XY), while amniocentesis on the 19th week of pregnancy revealed normal karyotype. This is the first reported case of placental pseudo-mosaicism (mos45X/46XY) combined with exomphalos [38].

Trisomy 2 of the placenta associated with growth retardation and abnormal clinical characteristics at birth, while the CPM on the sex chromosomes does not usually have any adverse effect on embryo development [39]. The pseudo-mosaicism of placenta may also be associated with the occurrence of umbilical hernia and various phenotypic abnormalities. In a clinical case showed non mosaic trisomy 22 in the chorionic villus, mosaic trisomy 22 in amniocytes and normal karyotype of lymphocytes showed abnormal foetal characteristics of reminiscent Goldenhar syndrome. The foetal mosaicism appears to be the likely explanation for Goldenhar syndrome [39,40].

Conclusion

Exomphalos and umbicinal hernia can quite often associate with disorders of the gastrointestinal and central nervous system seems to be predominated such as in cases of Beckwith- Wiedemann and abnormal karyotype syndromes.

Exomphalos may be associated with limb abnormalities or caudal midline syndromes. Although cardiac and renal abnormalities seem to be associated with the occurrence of exomphalos the prevalence is not apparently associated with chromosomal abnormalities. Such disorders may be associated with chromosomal defects at autosomal and sex chromosomes and other syndromes such as paternal uniparental disomy (UPD), Pallister-Killian (PKS) and Beckwith-Wiedemann syndromes. The determination of the associated malformations can lead to conscious prenatal counselling. This provides parents reliable and clear information's in order to determine the continuation of pregnancy.

Compliance with Ethical Standards

Funding: This mini review has been written without support of any fund.

Disclosure of Potential Conflicts of Interest

The author declares have no conflict of interest.

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Congenital Urethral Polyps: A Rare Cause of Bladder Outlet Obstruction in Children-Juniper Publishers

Abstract

Polypoid mass in the prostatic urethra is uncommon but potentially represents a wide spectrum of different entities, ranging from congenital malformations, benign polyps, premalignant disorders to various malignancies. Congenital urethral polyps are rare entity in infancy. They are congenital benign tumors. Most patients present with acute retention, hematuria or intermittent bladder outlet obstruction. The treatmentof choice is transurethral endoscopic ortransvesical resection. Histological study confirms the diagnosis and theprognosis is excellent. We report a case of an 18 month's old boy who presented with acute urinary retention. There was history of dribbling of urine and excessive crying during micturition. Imaging and endoscopic studies confirmed the presence of a polypoid pedunculated lesion at the bladder base arising from posterior urethra. It was excised by cystostomy because of an unsuccessful cystoscopic removal attempt. Although this is considered a benign lesion with no previous reports of recurrence or malignant behavior, it produces dramatic urinary symptoms in the pediatric population with a wide differential diagnosis. Imaging and endoscopic findings may suggest a malignancy and are not sufficient to render a precise diagnosis, which can only be made by pathologic examination.

Keywords: Polyps; Urethra; Hematuria; Congenital; Urinary retention

Introduction

Congenital urethral polyps cause a variety of symptoms in children. John Hunter is credited with the first documented case of urethral polyp, and Thompson reported the first case in a patient. Congenital urethral polyps are rare with only sporadic reports of a small series of cases [1]. Posterior urethral polyps in children are uncommon, and anterior urethral polyps are even rarer. Nevertheless, anterior urethral polyps are exceptionally reported in the literature [2-5]. These polyps are congenital and occur usually in boys; the average age of presentation is 5.2 years. Polyps in boys mostly arise from the posterior urethra, are usually proximal to the membranous urethra, and present usually as a single tumor, only rarely as multiple separate masses. This benign pathology is supposed to represent a developmental error in the invagination process of the submucosal, glandular portion of the inner zone of the prostate gland [6]. These Congenital urethral polyps are the most common benign mesodermal tumors of the urinary tract. Such lesions can also be called prostatic urethral polyps (in boys), fibroepithelial polyps of the urethra, or benign urethral polyps. Congenital urethral polyps more frequently occur in males and in the posterior urethra; this entity is very rare in females. Congenital urethral polyps should not be confused with the polyps of botryoid sarcoma [7]. The embryologic basis for urethral polyps is not clear; however, it is possible that they may arise from mesonephric remnants [8]. These lesions are benign, pedunculated tumors that arise in the region of the verumontanum in males or mid-urethra in females. In early infancy, they usually cause urethral obstruction. However, in older boys, the main presenting features include hesitancy, diminished stream, incomplete emptying, urinary retention, and sudden painful interruption of urinary stream, dysuria, hematuria, UTI, VUR, and enuresis.

Case Report

An 18-month-old boy presented with complaint of urinary retention and dribbling of urine for 3 days. Clinical examination revealed no abnormalities. Laboratory assessments were unremarkable, except for 4-5 red blood cells on urine analysis. Cystourethrography (VCUG) revealed normal findings and ultrasonography (USG) showed a round, mobile mass, 22x9mm in size at the bladder neck. Cystourethroscopy confirmed the presence of a posterior urethral polyp extending from posterior urethra into the bladder. An attempt to remove it endoscopically was unsuccessful, because the polyp had a smooth surface, a tense wall and was floating in the bladder Therefore, it was excised by cystostomy. Pathologic gross findings consisted of polypoidal greyish white soft tissue measuring 1.5x1x1cm. Micro- section shows a polyp covered by transitional epithelium which is locally ulcerative, other areas the epithelium is mildly hyperplastic. The stroma is composed of vascular fibro-connective tissue and shows a dense inflammatory infiltrate comprising of neutrophils, lymphocytes, eosinophils and histiocytes (Figure 1).

Discussion

Congenital polyps of the male urethra are pedunculated lesions that arise as a defective protrusion of the urethral wall [1]. They occur more often in children and in patients present with bladder outflow obstructions [9]. The investigation of choice is urethrocystoscopy. An ultrasound of the urinary bladder and posterior urethra may reveal a polyp. Histologically, the polyps are composed of vessels, muscle and, less frequently, nerves and glands covered by urothelium. There is some controversy over their embryological origins [7-9]. Downs postulated that the polyps resulted from a defective protrusion of the urethral valve [1] . Kuppusami & Moors [10] suggest that metaplastic epithelial changes had occurred secondary to the estrogen released during gestation. They found similarities between the urethral polyp and verumontanum histology, which consisted of smooth muscle and small glands lined by transitional cell epithelium [10]. Stephens [11] postulated that the polyp is most likely a congenital lesion. Barrie & Simms [12] believe that the polyp is a response to urinary tract infections; however, most such inflammatory polyps do not occur in children [11]. The absence of glandular elements within the fibrous polyps of most patients distinguishes them from the verumontanum. These polyps arise from the vestiges of Muller's tubercle and then fail to regress [1]. A precise history, physical examination, and uroflowmetry patterns in toilet-trained children can strongly suggest urethral polyps in children. Urinary tract ultrasonography and micturition cystourethrography are the most important examinations for this diagnosis; however, they are not always successfully diagnostic. The final diagnosis must be confirmed by direct video urethrocystoscopy with minimal irrigation flow. Transurethral resection of the urethral polyps is the standard firstline of management [13]. However, when the polyp length is larger than 3cm, with diameter of 1cm or more, it becomes displaced into the bladder, and open transvesical removal may be an acceptable alternative.

Conclusion

In all children who present with sudden interruptions of their urinary flow and no obvious bladder or urethral stones, urethral polyps must also be kept in mind as a differential diagnosis. The final and definite diagnosis of polyps is made based on the appearance on urethrocystoscopy. The endoscopic resection of the polyp is the treatment of choice; however, transvesical resection is a safe option in large bladder polyps.

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Unilateral Complete Ureteral Duplication with Impacted Stone at Ectopic Opening of Upper Moiety Ureter in Posterior Urethra and Simultaneous Bladder Stone: A Rare Entity-Juniper Publishers

Abstract

Complete ureteral duplication with ectopic upper moiety opening into posterior urethra is rare anomalies. This is due to development of two ureteric bud from mesonephric duct. Development of stone in this moiety is rare event. Here we are describing a 12 year old child who presented with left lumbar mass and dysuria. He had left complete duplex ureter with impacted stone at ectopic opening of upper moiety ureter in posterior urethra, simultaneous bladder stone and hydronephrotic non-functioning upper renal moiety. Combined (cystourethroscopy and open surgical) approach was used for management of this case.

Keywords: Ectopic ureter; Duplex renal moiety; Stone in duplex kidney

Introduction

Duplex system can be describes as two pelvicaleceal system with either single lower ureter or double ureter draining into bladder or outside the bladder (ectopic) [1,2]. Duplex system with double ureter and ectopic opening of upper pole ureter into proximal urethra (ectopic) is rare anomalies compare to bifid ureter [1]. Stone formation in duplex system is potential complication [1,3-5]. Combined approach should be used to deal such situation in resource poor setting.

Case Report

Twelve year old male child presented with complain of colicky pain with mass in left lumbar region and dysuria since 5 month. No history of fever, hematuria or trauma to abdomen. Examination revealed single 10cm x 8cm nontender cystic ballotable mass in left lumbar region. Complete blood count was normal, urea 32mg%, creatinine 0.6mg% Ca 9mg% uric acid 5.6mg% PO4 5.3mg%, urine microscopy 25 pus cells/HPF, urine culture no growth, Plain X-ray shows two radiopaque shadow in pelvis (Figure 1), USG shows Right kidney Normal, Left upper moiety gross hydronephrotic, left ureter dilated till lower end, lower moiety normal, Stone in urinary bladder (1.5cm) and Left VUJ (1.6cm). Intravenous urogram (IVU) and nuclear imaging suggestive of left upper moiety nonfunctioning (Figure 2 & 3), left lower moiety normal. Right kidney normal.

During cystourethroscopy one stone was impacted at ectopic opening of ureter and projecting into posterior urethra in curvilinear fashion and another stone in urinary bladder, stone at ectopic opening was disimpacted and pushed backed into ureter and child was catheterized. Open left upper pole nephrouretectomy with removal of ureteral stone and cystolithotomy was performed.

Figure 4 (specimen) showing stone of urinary bladder (upper) and impacted stone of ectopic ureter (red arrow). Post operatively child recovers well and on 6 month follow up child was well.

Discussion

Duplex system with double ureter with ectopic opening of upper moiety ureter into posterior urethra in male child is rare anomalies [1,6]. In duplex system lower moiety is usually good functioning and upper moiety having ectopic opening of ureter is hydronephrotic and poor or nonfunctioning [7,8], as in our case. Ureteral duplication with ectopic upper moiety ureter in male child is usually asymptomatic. These duplex systems are vulnerable for urinary tract infections and urolithiasis. Urinary calculi are often due to relative stasis of urine but may occur due to factors unrelated to the duplication. Development of stone in upper moiety ureter may be due to either obstruction, recurrent infection or other factor. Treatment of nonfunctioning upper moiety is surgical removal [7].

Retrieval of large stone per urethrally is difficult task in setting where lithotripter is not available. In this case we had to disimpact and push backed the stone into the ureter so that we could be able to remove stone by open surgery

Duplex moiety with impacted stone in lower end of upper moiety ureter can be dealed with combined approach (cystoscopy and open) in resource poor setting.

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Obstructive Fibrinous Tracheal Pseudomembrane: A Very Rare and Life-Threatening Complication of the Endotracheal Intubation-Juniper Publishers

Abstract

Obstructive fibrinous tracheal pseudomembrane is a rare complication associated with endotracheal intubation. We report the case of a 10-year-old boy hospitalized for a severe abdominal trauma. The boy remained intubated for 4 days. After extubation he started to have stridor and acute respiratory distress so a reintubation was necessary. After 24 hours, an elective extubation was performed and the  boy presented stridor and dyspnoea with no improvement with medical treatment. A fibrinous mobile membrane was seen during a flexible bronchoscopy. The pseudomembrane was removed and the patient remained asymptomatic. The knowledge and an early diagnosis of this pathology is very important due to be a life-threatening complication.

Introduction

Damage of the airways caused by intubation is usually associated with mechanical trauma caused by the endotracheal tube (ETT). Stridor occurs in approximately 1-16% of the patients, but in 4-8% of these children the extubation fails and an urgent reintubation is needed. The most common causes are laryngeal or tracheal edema. The symptoms are usually presented within 1-4 hours after extubation and are usually resolved in under 24 hours. Stridor can persist in spite of the treatment with nebulized epinephrine and steroidal therapy or with repeated extubation failures. In such cases, a fiberoptic bronchoscopy and differential diagnosis of common causes including subglottic stenosis, vocal cord damage or subglottic/tracheal granulomas are all required [1].

In recent years an infrequent cause of extubation failure has been highlighted; obstructive fibrinous tracheal pseudomembrane (OFTP) with a similar presentation of stridor post-extubation. The knowledge of this pathology and its early diagnosis is extremely important as this is a potentially life-threatening complication [2].

Case Report

We present a case of a previously well 10-year-old boy who was admitted to the Pediatric Intensive Care Unit after severe abdominal trauma. The patient was under hemodynamic instability with an hemorrhagic shock caused by an hepatic and pancreatic laceration. His trachea was intubated with an appropriately sized 7-mm oral cuffed ETT, that passed easily into the trachea. A caudal pancreatectomy was performed. The child remained intubated and was on ventilatory support for 5 days. The patient had no fever or suggestive signs of infection in repeated blood testing. The blood culture and tracheal aspirates were negative. The chest x-ray was normal. A few hours after the extubation, the boy started to have severe stridor and acute respiratory distress and a reintubation was necessary. There were no early complications with the reintubation and the patient improved immediately. Repeated ETT suction revealed no secretions. After 24 hours, an elective extubation was performed. Inspiratory stridor and dyspnea started shortly after extubation with normal SpO2. The cause of the respiratory distress was thought to be laryngeal edema and this was treated with inhaled budesonide and intravenous steroidswith no improvement.

The symptoms became progressively worse and blood gas results showed increased PCO2, so a flexible bronchoscopy was performed. In the trachea, a white, fibrinous mobile membrane was seen two centimeters below the subglottis in an anteroposterior and transverse position. This caused an intermittent complete obstruction of the airway during the inspiration (Figure 1). A rigid bronchoscope was introduced and the pseudomembrane was introduced and the pseudomembrane was removed.

The patient remained asymptomatic after extubation without difficulty breathing or stridor. The control bronchoscopy showed a normal airway size with a mild erythematous circumferential area. Secretions or other inflammatory symptoms were not observed. 21 days after admission the child was discharged home asymptomatic with no further complications.

Discussion

OFTP is a very rare and life-threatening complication of the endotracheal intubation. The first case was described in 1999 [2]. Birch described the first pediatric case in the year 2005. A 8-year-old that developed stridor in the first 24 hours after extubation for dental surgery with general anesthesia [3]. The real incidence is unknown as endoscopy to review airway injury after intubation is not a routine process. In 2011 a short series was reported in which a total of 24 adult patients were described and a review of the literature was made[4]. It is usually not considered a complication after a bronchoscopy in Pediatric Intensive Care Unit (PICU) patients or in Neonatal Intensive Care Unit (NICU) patients. However, a recent retrospective study performed over a 10 year period describes OFTP in 1.4% of PICU or NICU patients who had symptoms or clinical signs after extubation [1]. The hypothesis is that the pseudomembrane formation is caused by an ischemic injury of the tracheal mucosa and submucosa, which causes ulceration and necrosis. Finally, a fibrinous exudate and an infiltration of polymorphonuclear neutrophils can be observed, causing a functional stenosis [5]. The tracheal ischemia due to cuff pressure injury of the ETT has been suggested as the etiology, nevertheless it has been reported in children intubated with no tracheal cuff [1,3]. It is also associated with traumatic intubation or inappropriately large ETT. The OFTP can occur in patients within a short time of intubation; however the average time of previous intubation is 37 hours. The clinical presentation consists of stridor and a different grade of dyspnea that typically occurs before 24 hours postextubation in the pediatric patients. In adults this can occur 10-15 days later [4]. An important difference between OFTP and laryngeal edema is that OFTP does not respond to medical treatment. Differential diagnosis includes staphylococcal tracheobronchitis with tracheal pseudomembranes. In these cases, cultures could be positive, sepsis could be a clinical presentation and the lesion might not necessarily be localized to the site of the cuff or in the subglottis area. Bronchoscopy is used to diagnose OFTP. The typical endoscopic findings are circumferential membranes firmly attached to the trachea that move (or not) in the airway with the respiratory cycle. It can collapse the airway completely. It can be an annular or flapping septum. The lesion is located in the subglottis and the first tracheal rings. The rest of the airway is normal. Standard treatment includes rigid bronchoscopy and removal of the tracheal membranes. However, a flexible bronchoscopy with forceps or mechanical removal with a tracheal balloon can also be useful. There is no sequela. In some cases described in adults the condition fully resolved after expectoration with spontaneous membrane removal [5].

Conclusion

In conclusion, in patients with clinical symptoms of stridor and dyspnea after extubation that does not improve with medical treatment it is important to think about the formation of a tracheal pseudomembrane that causes airway obstruction. This obstruction can be intermittent or positional and can be life-threatening. Pediatric pneumologists, intensivists and otorhinolaryngologists should know this pathology for early bronchoscopic diagnosis and treatment.

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Diagnosis and Treatment of Supraventricular Tachyarrhythmia in Pediatric Population: a Review Article-Juniper Publishers

Abstract

Supraventricular tachyarrhythmia (SVT) is the most common tachyarrhythmia in pediatric patients and accounts for more than 90% of pediatric arrhythmias. As many as 16 different mechanisms of pediatric SVT exist; we focused in this review article on the three most common groups of SVT mechanisms in pediatrics: atrioventricular re-entry tachyarrhythmia (AVRT), atrioventricular nodal re-entry tachyarrhythmia (AVNRT), and a trial tachyarrhythmia (AT). Many methods have been implicated in the diagnosis of SVT like full history taking regarding onset and frequency of palpitation, syncope, chest pain, dispend, heart failure, drug intake and physical examination. Children with suspected SVT must be subjected to resting 12-lead surface ECG and ECG during the attack. Echocardiography must be done for detection of any structural heart diseases. Moreover, recent techniques like Electro physiologic study (EPS) is the most definitive diagnostic procedure. Many therapeutic strategies like drugs and radiofrequency ablation (RFA) have been succeeded to provide a significant reduction of the risk of SVT, but RFA offers the prospect of cure of the arrhythmia and avoidance of drug-associated side effects. It is often becoming the first line of therapy for many children with SVT, as it has a relatively low morbidity and mortality, and it results in a low rate of recurrence of SVT.

Keywords: SVT; Children; Diagnosis; Treatment

Core Tip: This manuscript discusses the most common types, recent strategies in diagnosis and management of Supraventricular tachycardia in pediatric age group.

Introduction

Supraventricular tachyarrhythmia (SVT) is the most symptomatic pediatric arrhythmia resulting from an abnormal mechanism that requires structures in the heart above the bifurcation of the bundle of His for its continuation. Clinical symptoms of SVT are variable; ranging from asymptomatic or minor palpitations to more severe manifestations [1]. Theincidence of SVT has been documented to affect between 1 in 250to 1 in 25,000 children [2].

Variable mechanisms ofSVT exist; we concentrated on the three commonest forms occurring in children which are: atrioventricular re-entry tachyarrhythmia (AVRT), atrioventricular nodal re-entry tachyarrhythmia (AVNRT), and a trial tachyarrhythmia (AT) [3].

Most Common Types of Svt in Pediatrics

Atrioventricular re-entrant tachyarrhythmia (AVRT) or accessory pathway re-entry tachyarrhythmia.

An accessory pathway (AP) is a microscopic bridge of muscle connecting atrium and ventricle that bypasses the normal electrical insulation of the AV ring. The impulse spreads down either via the atrioventricular (AV) node or the AP and then up the other A re-entrant unconventional circuit is formed, [4] (Figure 1).

AVRT is the commonest type of SVT in the pediatric population forming two-thirds of the affected children, then comes the atrioventricular nodal reentry tachyarrhythmia (AVNRT) and the trial tachyarrhythmia (AT) [5]. AVRT is more common in males, but its incidence decreases with age. On the other hand, the incidence of AVNRT and a trial tachyarrhythmia is increased with age [6].

Atrioventricular nodal reentry tachyarrhythmia (AVNRT)

The AV node is composed of "slow" pathway and "fast" pathway. AVNRT happens when an ante grade impulse is blocked at one pathway (usually the fast pathway), resulting in conduction over the other (usually the slow pathway). After the impulse is travelled down via the slow pathway, the fast pathway is now unblocked and the impulse can spread retrograde via the fast pathway. So, a re-entrant circuit is formed, [7] (Figure 2).

AVNRT accounts for 15% of cases of pediatric SVT, mostly present over the age of 5 years and almost completely absent in infants. Females were more than males in cases diagnosed as AVNRT or AT [6]. Atria tachyarrhythmia (AT). AT is mostly due to abnormal automaticity, may be formed by remnant embryonic cells with automatic qualities, causing abnormal impulses that come away from normal sinus node but still within the atria. Although it is a rare condition, but still the third common mechanism of tachyarrhythmia occurring in children, if remains undetected can result in a dilated cardiomyopathy [7].

History and clinical presentations of Svt in pediatrics

The clinical presentation varies with the age of the patient; generally, it differs in infancy from that in an older child. In infancy most attacks occur before 6 months of age, and may often less than 4 months. They may be undetected or associated with pallor The parents may take their child to the doctor with a complaint of poor feeding, or vomiting. The rapid breathing or ashen color may not be observed by them. By the time, the pediatrician sees them: there is usually significant cardio-respiratory distress. The infant will often have signs of heart failure. Occasionally frank circulatory shock will be present [8]. Occasionally, a young patient may have palpitations, chest or abdominal pain, occasionally syncope, particularly with exertion may occur, but the presence of congestive heart failure beyond infancy is extremely uncommon [9](Table 1).

Physical Examination of Svy in Pediatrics

Following the history, the physical examination should be performed: unexplained hypothermia, with poor perfusion, a picture of sepsis but with negative cultures. In the older infants, irritability associated with vomiting and sometimes diarrhea (uncommon). In approximately 20% of infants, SVT is detected during the routine examination without symptoms [10].

Auscultation should be done to reveal the presence of structural heart disease, heart rate and rhythm should be noted. Further evaluation will depend on the severity and frequency of symptoms, the age of the child, and the presence of structural heart disease. Infants with SVT may have a heart rate range from 220 to 300 BPM. Older children generally have slower rates which may count from 180 to 240 Bpm. Untreated SVT can result in congestive heart failure (CHF) within 24 to 48 hours. Termination of the attack by vagal maneuvers may suggest a reentrant tachyarrhythmia involving AV node [5].

Diagnostic investigations of Svt in pediatrics

Recording a 12- Lead Electrocardiography (ECG) at rest should be done and examined for any abnormal rhythm, delta waves, abnormal QT interval, sinus tachyarrhythmia, or any sign of underlying structural heart disease. [11]. ECG can clearly diagnose about 80% of AVNRT and AVRT, but incorrectly categorize approximately 20% of cases of SVT; therefore, the ECG cannot serve as the sole means for determining the mechanism of tachyarrhythmia [12].

Another diagnostic tool is worn by the patient named Holter monitor. It can record continuous ECG tracing for 24 to 48 hours,having the same idea as classic ECG wires and electrodes but it is a portable device that provides information about symptoms that can occur during the day [7].

In selected patients with occasional complaints (i.e. less than two episodes of SVT per month) and associated with disabling symptoms (i.e. hemodynamic instability) can be diagnosed by implantable loop recorders. If the clinical history is not enough or other measures have failed to identify the SVT mechanism, then recordings and stimulation can be done via Tran esophageal a trial loops for these patients for diagnosis or to provoke paroxysmal tachyarrhythmia [13].

The possibility of structural heart disease should be excluded by Echocardiography examination in children with identified attacks of SVT, which is not possible to be detected via physical examination or resting 12-lead ECG [14].

Other investigations can be done to exclude other causes included in the differential diagnosis of SVT Chest x-ray with lateral and anteroposterior views is done to diagnose cardiomyopathy and CHF. Laboratory tests which include; serum electrolytes to diagnose any imbalance leading to abnormal cardiac rhythm, complete blood cell count (CBC) with differential to exclude infections or anemia, also, screening of toxic substances, thyroid function tests, and arterial blood gas can be helpful [5].

The most accurate diagnosis can be done with the electrophysiological study (EPS). It is used for clear classification of different mechanisms of SVT Also, EPS combined with catheter ablation can be used as a definitive long-term therapy [7].

Management of Svt in Pediatric Age Group

The dealing with SVT can be done in two ways: acute termination of the attack of tachyarrhythmia and prevention of recurrences (chronic therapy). The acute treatment begins with documentation of the arrhythmia with a 12 lead ECG, assessment of hemodynamic status and recognition of the arrhythmia mechanism. Chronic therapy is based on the SVT mechanism, the patient's age, and the frequency and patient's complaint during the attack as well as access to medical care and sophistication of the patient caretaker (Table) [15].

Acute management of Supraventricular tachyarrhythmia

For the aerodynamically tolerated episode, vagal physical maneuvers are to be used first. The patient may be taught to do Valsalva maneuver (the most potent physical maneuver), induce vomiting, dive the face in the iced water for 10-20 seconds, or carotid sinus massage may be done, an ocular pressure is not recommended for children. These physical maneuvers are potent in terminating re-entry SVT; mostly AVRT and to less extent AVNRT [11].

An ultra-short-acting drug (Adenosine) is highly effective in terminating AV node-dependent SVT, but it is of limited value in the diagnosis of AT. Vagal maneuvers and adenosine produce transient AV node block and result in sudden termination of SVT in any tachyarrhythmia involving AV node but not in AT [16].

The use of Beta blockers or long-acting calcium channel blockers is of value especially for patients with frequent a trial or ventricular premature beats which may act as a cause of recurrence of SVT but they must be used with great caution as they may potentiate hypotension [11].

In an aerodynamically unstable child with severe hypotension, electrical cardio version by synchronized DC shocks is the treatment of choice. It is a safe and effective procedure in the majority of patients [2].

Long term (chronic) management of Supraventricular tachyarrhythmia

The decision to initiate chronic therapy in infants and children is based on symptoms, efficacy rates, safety issues, expected response to therapy and frequency of episodes. The range of therapeutic options aimed at treating disorders of heart rhythm has expanded tremendously over the past decade; the complexity of actions produced by ant arrhythmic agents occasionally leaves the clinician frustrated by attempted at predicting the clinical results [2].

A child with SVT should be viewed globally while putting a long-term therapeutic plan, as this may affect him through his lifelong. A pharmacologic therapy is used to provide symptom relief. The usual selection of drugs (in order) is propranolol, verapamil and amiodarone. Dioxin is especially considered in cases with AVNRT, while amiodarone in combination with propranolol or/ and verapamil is effective in cases with AT [11].

Radiofrequency ablation (RFA) using the traditional radiofrequency energy to heat and destruct the site of origin of the SVT mechanism has been more widely applied in children since the 1990s [3]. It is now considered as the first line of management for many children with SVT and offering the prospect of cure of the arrhythmia and avoidance of drug-associated side effects. Success rates for ablation are as high 95% and characterized by low morbidity, low mortality, and a low rate of SVT recurrence [17] . RFA may have some complications resulting from the vascular access (i.e. hematomas, perforation of the aorta, deep venous thrombosis), or during catheter manipulation (i.e. alular damage, perforation of the coronary sinus or myocardial wall), or inaccurate ablation (resulting in i.e. AV node block, myocardial perforation, coronary artery spasm, transient ischemic attacks)[18] . Successful elimination of SVT substrate results in improving the pediatric quality of life regarding physical, emotional, social, school and psychosocial functions [19].

In conclusion, SVT is a common pediatric age group problem. Many methods have been implicated in the diagnosis of SVT like detailed history taking and physical examination. ECG and echocardiography must be done to children suspected to have SVT Electro physiologic study (EPS) is a recent technique for definitive diagnosis. RFA is a definitive therapy for many children with SVT However, further studies are mandatory in order to provide more novel therapeutic agents for complete protection of SVT and decreasing the complications of already used management procedures.

Acknowledgement

The author is grateful for Deanship of Scientific Research (DSR), Taibah University and Medina, Saudi Arabia for support.

Author Contributions

Abo Hadeed H.M.A. did the literature review and analysis, wrote the paper; drafting and critical revision and editing, and final approval of the final version.

Open-Access

This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and The use is non-commercial. See: http://creativecommons.org/ licenses/by-nc/4.0/

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Neonatal Land Transport-Juniper Publishers

Summary

Owing to good perinatal care (prenatal detection of problems and timely transport of pregnant women), critically ill newborns are usually delivered in large centres (with tertiary perinatal care provided) where they can be given intensive care and therapy. When this is not the case, a critically ill newborn must be transported to a centre with a higher level of perinatal care. The less time it takes from identifying a problem to transpor to a higher level centre, the better the chances for a better outcome, even though the transport of newborns is still tied to higher morbidity and mortality rates than in utero transport. This is especially the case with extremely preterm and very preterm neonates.

The successful medical transport of such high-risk and sensitive population requires a highly specialized personnel and appropriate equipment. In ideal conditions, the neonatal transport team is a link in the system of neonatal care followed by an intensive care unit where critically ill newborns are treated (with the right personnel, equipment and subspecialists for diagnostics and therapy when needed).

The composition of transport teams varies according to the transport type (by land, air or water) and referral organization (one way, two ways, medical teams specialized in neonatal transport); however, education, competence and equipment including the necessary medical supplies should be appropriate for the level of care. Also, basic stabilization measures before transport and the necessary medical and technical procedures before and during transport are the same in all the types of transport mentioned. Special procedures (administration of surfactants, 'cooling', administration of prostaglandins) should be performed by an experienced team well familiar with the application techniques, monitoring methods and possible complications (and how to deal with them).

The peculiarity of neonatal transport are surgical patients with congenital anomalies who require specific procedures (such as gastric emptying, ventilation, certain body positioning etc). Any transfer, as well as any medical procedure should be properly documented (pretransport documentation, newborn's condition at the transport team arrival, performed interventions, the course of transport, any complications, transport time etc).

Assessing the right transport indications, maintenance of competence and keeping the transport team properly equiped make neonatal transport a significant segment of operations of any healthcare facility.

Introduction

Away from the secure and well supplied neonatal intensive care unit (NICU), with special equipment and limited personnel, and often uncertain and unpredictable transport course, a transport team works in conditions far from ideal. During transport, a newborn is exposed to numerous harmful noxae - noise, vibrations, acceleration or deceleration, temperature instability, all of which could additionaly aggravate an already difficult condition. The treatment outcome of the transported newborn depends on a number of factors, some of which can be measured - acidosis levels, retained carbon dioxided levels, blood pressure, glucose in blood levels, and temperature. Maintaining the neonate's organism homeostasis is thus of vital importance, but sometimes very difficult to achieve during transport.

In view of all above mentioned factors, neonatal transport is a risk for a newborn as well as for a transport team [1-3]. The first transport incubator constructed for 'the care of weak and premature infants' was used in 1900 by Dr. Joseph De Lee (Chicago Lying-In Hospital). The incubator was used to transport 'these weak children from remote parts of town and suburbs' [4].

The regionalization of perinatal care in the entire world, which started during the 1970's, contributed to the agglomeration of ill neonates in the centres that could provide diagnostics and treatment for most of neonatal diseases. Directly resulting from regionalization, the number of transports of critically ill neonates increased, to centres where they are taken care of and diagnostical and therapeutical procedures fully observed, either in utero, before birth (applying corticosteroids, for instance), or during and directly following birth (nCPAP, surfactant). Deregionalization that is under way in developed countries implies creation of intensive care units in various healthcare facilities (and ample investments into equipment and personnel) with staff training and highly developed transport network. The aim is to bring each and every newborn, should there be a need, to tertiary care with appropriate diagnostics and treatment.

Transport could elevate the risk of mortality and aggravate the newborn's illness. Ill neonates that are transported from a lower to a higher level centre of perinatal care usually have a less successful outcome that the ones born in a high level care centre; even the newborns that are transported between the same high level care centres have a worse outcome than the neonates that were not transported. This is the reason why, when assessing potential neonatal problems, in utero transport is the best solution [1,2].

Even though regionalization, deregionalization and even grading of neonatal departments, that is a division of NICU into different categories (primary, secondary, tertiary) have been carried out, there is still a certain number of newborns in need of intensive care and therapy that are born in primary and secondary care settings. These are most often the reasons:

A. Unforeseen or unidentified perinatal circumstances that do not allow for the mother to be transported - no time for in utero transport;

B. Unidentified prenatal condition that is life threatening for a newborn;

C. The pregnant woman's (obstetrician's) wish to deliver the baby in a certain facility;

D. Some other reason;

E. Neonatal transport from secondary or tertiary care NICU to a higher level.

Modes of Neonatal Transport

Cocnerning the type of vehicle and other options, the transport can be made by land or air, and also on water. Transport vehicles used for land transport can be specialized emergency medical vehicles, rarely standard emergency medical vehicles with the addition of a mobile incubator; air transport implies the use of special aeroplanes and helicopters for the transport of patients, but standard aeroplanes and helicopters can be used as well with the additon of mobile incubators. Waterway transport makes use of vessels that can be fitted with an incubator. The advantages of land transport are as follows:

A. The lowest cost

B. Mostly not affected by the weather conditions

C. Better fitted to patients

D. Vehicles interior can be customized (dedicated exclusively to neonatal transport)

E. Land transport allows (with appropriate equipment) for the transport of several patients simultaneously (two mobile incubators).

Shortcomings of land transport are:

A. Slowness

B. The need to secure the incubator stability and to bring in all the necessary equipment (2).

Generally speaking, critically ill neonatal transport (apart from in utero transport and the so-called 'intra-hospital' transport, which are segments in their own right) can be organized as

A. two ways transport and

B. one way transport.

These two transport types (mostly) differ according to who organizes and who performs the transport:

A. Two ways transport: usually organized and carried out by a higher lever healthcare facility team. (It is a common practice that teams from facilities with Illb or Illc perinatal care levels organize two ways transport of critically ill newborns; the advantages are the presence of competent personnel, appropriate equipment and the immediate treatment).

B. One way transport: by a healthcare facility team organizing the transport into a facility of a higher level care. (In most cases, there is no specialized transport team, it is formed ad hoc with basic transport equipment).

C. By a specialized team for neonatal transport: this kind of transport is characteristic of countries with vast territories and small population (such as Australia), where the predominant mode of transport is by air. These teams are specially trained and supplied so they can start and administer therapy en- route [5].

D. By teams for emergency medical assistance who perform neonatal transport along their regular duties (critically ill neonatal transport occurs sporadically) without the presence of a neonatologist or specially trained neonatal transport physician on board.

Two Ways Land Transport of Critically Ill Newborns

To ensure timely, safe and beneficial transport, the following conditions should be met:

A. Appropriate transport team composition;

B. Required competencies and skills of transport team members ;

C. Required transport equipment:

D. Required drugs and medical supplies;

E. Accurately assessed transport indications;

F. Choosing the optimal time (newborn's age and condition) for transport.

Concerning the composition of the critically ill neonatal transport team, its permanent members can be:

A. A physician, a nurse (compared to the modes of transport, physicians and nurses can be specially trained for neonatal transport or carrying out the transport by merely monitoring the patient).

B. A registered nurse, a respiratory therapist (This transport team composition is characteristic of countries where the costs of transport are very high if a physician is on board, so it is more cost effective having a respiratory therapist in the team - with a physician 'on line'.

C. A nurse (trained to monitor vital parameters and perform basic cardiopulmonary resuscitation (CPR), while consulting with a physician 'on line' in case of any other problems).

D. Driver (In some countries drivers are also trained nurses).

In the case of land transport, the physician makes the decision about the transport speed and whether to call for police escort in order to ensure undisturbed ride without speeding or slowing down. If the newborn is stabilized before transport, there is almost no need for high speed driving. If it is possible to plan transport ahead, it is best to avoid the time of traffic rush hour. Should the newborn's condition worsen during transport or a certain medical procedure must be performed (reintubation, tenting or mere auscultation), the transport vehicle should be stopped so that the procedure can be properly performed.

The transport of ill newborns is a demanding task which requires specific equipment and competencies; each transport should be planned so that the risk of complications en-route is minimized ; it is much more difficult and involves higher risk to intervene during transport (intravenuous cannula insertion, endotracheal intubation) than performing these in a regional hospital. These are the reasons why certain conditions and procedures must be met before transport takes place [6,7].

Condtions and procedures before transport:

Stabilization of an Infant

Before each transport, the condition of the newborn must be stabilized as much as possible and the therapy administered [7,8]. Then the following measures are [5,9]:

Respiratory stabilization

Many diseases in newborns manifest through respiratory problems, even in cases when the primary disease is not respiratory. Neonatal respiratory reserves are relatively small and without adequate control respiratory problems may become significant. It is of vital importance to ensure enough oxygenation and ventilation (with appropriate pCO2) before transport. If there is a need for respiratory support by using nasal CPAP or for intubation and assisted ventilation, respiratory support should be provided in such a way that there is enough even in case of condition aggravation during the transport. If it is uncertain that the newborn will be able to breathe spontaneously, the infant should be intubed before transport and adequate ventilation secured. Whenever it is possible, acid-base status should be checked before transport.

Circulatory stabilization

Neonatal circulation is rather unstable and many diseases due to which the newborns are transported are accompanied by different digrees of circulatory insufficiency This is especially the case with newborns having respiratory problems (Respiratory Distress Syndrome, Meconium Aspiration Syndrome), heart diseases and serious infections. Newborn's circulation state can be assessed by measuring arterial blood pressure. Invasive blood pressure measurement is the safest and most reliable option; it is also possible to use a cuff, but it is less reliable. It is compulsory to check the blood pressure values before transport and to compare them with normal values for that age and gestational age. The occurence of metabolic acidosis refers to circulatory insufficiency and must be treated before transport. Newborns with unstable circulation, who are given intravenous therapy or endotracheal intubation should have at least two vascular lines open [10].

Temperature stabilization

It is important for a newborn to have normal temperature before and during transport. Hypothermia will increase respiratory problems as well as the risk of other complications. Overheating, especially of asphyctic newborns may increase the risk of cerebral disease. (This is why therapeutic hypothermia is planned for before transport).

Metabolic stabilization

It is necessary to start with dextrose infusion before transport, and the enteral food should be evacuated (by emptying the stomach) in order to prevent aspiration of gastric contents during transport. Normal need for glucose is equal to the solution of 10% dextrose with the volume of 3,6ml/kg/h (6mg/kg/min glucose).

Stabilization of the Central Nervous System (CNS)

Some severely ill newborns have convulsions. It is of vital interest to secure metabolic needs of CNS before transport, especially by avoiding hypoglycemia. It is necessary to stop the seizures prior to transport. Some authors report higher grade and frequency of ICH in transported neonates [11].

Infection treatment

At slightest suspicion, adequate antibiotics therapy should be administered prior to transport. Obtaining samples for microbiological analyses is beneficial before starting with antimicrobial therapy [12,13].

Surfactants administration prior and during transport

Administration of surfactant prior the transport is safe. The newborn should be given time to stabilize after surfactant was administered. Administration of surfactant does not influence the frequency of transport of low birth weight neonates into centres with higher levels of care and treatments [14-17].

Administration of prostaglandins during transport

In spite of adverse effects of prostaglandins (hypotension, vasodilatation, redness, heart rhythm disorder, convulsions, apnea, hypoventilation, frequent bowel movement, diarrhoea, pyrexia), its administration is not contraindicated to most congenital heart defects (except for total anomalous pulmonary venous return). The recommendation for patients who are administered prostaglandins during transport is to be electively intubated for timely therapy of sudden apnea. Every facility should have its own protocol or prostaglandins administration during transport [18-21].

Cooling during transport

Therapeutic hypothermia that is started within six hours after birth has as a result better neurodevelopmental outcomes at term neonates with moderately severe and severe Hypoxic Ischemic Encephalopathy. The largest number of these neonates are born in facilities where NICU are not properly equipped for cooling; with the addition of transport time, it is quite possible that therapy will start late, so that neuroprotective effect of cooling should be initiated even during transport, optionally by passive cooling. Every facility that transports neonates should have its own protocol on this procedure concerning the necessity of a systematic approach[22,23].

Communication with regional facilities, medical documentation etc

Prior to transpott a regional healthcare facility should be contacted to ensure stabilization of the newborn (especially in the case of surgical diseases, congenital defects, etc). One should obtain a list of the required documents to be given to the transport team (X-ray records, laboratory analyses results). It is important to note whether it is necessary for mother's blood to be taken [23].

Transport team

The required competencies (theoretical and skills) of the transport team members:

A. Physician: Beside theoretical knowledge of pediatrics- neonatology and perinatology, they must be skilled at: nasogastric tube placement, catheterization of urinary bladder, rectal tube placement, peripheral venuous cannulation, central venuous cannulation (and of umbilical vein), endotracheal intubation, pleural space drainage, surfactant administration, application of respirator, aspirator and defibrillator. They should be excellent with techniques of manual ventilation and CPR. Beside theoretical and practical knowledge, they must be able to recognize and act immediately upon any change in the neonate's condition during transport as well as anticipate potential problems.

B. Nurse: Beside competence and skills, they are responsible for all the apparatus to be in working order, they must know in detail how to assemble all transport devices, check the contents of transport medical supplies (expendable supplies and drugs). They are skillful at performing CPR.

C. Driver: is responsible for the transport vehicle, gas and power supply to all machines and devices. They should be trained to repair any potential defects during transport and finds the best transport route (through rush hour, extreme weather conditions).

Necessary equipment

Mobile incubator with a transport ventilator

Device for the applicatin of nitric oxide

Portable ecmo

Cooling device

Oxygen hood

Monitor (ECG, respiratory, pCO2, pO2)

Pulse oximeter

Body temperature measuring device

O2 concentration measuring device

Blood pressure measuring device (by cuff or artery blood pressure)

Reanimation balloon with masks (in different sizes)

Intubation equipment

Air way (different sizes)

Aspirator

Injectomats

Stethoscopes (for the physician and the nurse)

Before placing the equipment into the transport vehicle, it must be inspected. Its working order and contents are checked. Following the equipment, all connections are checked (power, medical gases) and their state of operation. Drugs and expendable medical supplies container is inspected before and after it was brought into the vehicle. All the equipment used during transport must be inspected and serviced timely and regularly. There has to be a nurse responsible for releasing/taking complete and working equipment. Medications that are prepared for transport have to be properly labeled and packed, and there has to be a person responsible for this. Transport equipment and applied medications and medical supplies should be recorded in their own documents.

If any device is not working properly, it should be detected in time, duly noted and repaired [23].

lt is vital that transport team is very well familiar with monitoring equipment and ablr to manage in situations when this equipment is unreliable [12].

Medications that are required for transport (in appropriate solution, with precisely stated concentration, i.e. the amount of drug per unit of volume):

Adrenalin

Adenosine

Antibiotics

Aqua redestilata (pro injectione)

Bensedin

Calcium

Dexamethason

Dopamin

Dobutamin

Diuretics

Fentanyl

Flormidal (Midazolam)

Dextrose 10%, 5%, 12,5%, 50%

Glucagone

Heparin

Hydrocortison

Konakion (vitamin K)

Lidocaine

NaCl 0,9% , NaCl 3% normal saline, 3% saline

NaHCO3 8,4% bicarbonate

Phenobarbiton

Prostaglandin

Surfactant

Expendable medical supplies required for the transport (plastic and suture material).

Documentation

Accurate and proper record keeping is an important segment of neonatal transport. If there is no possibility for a telephone conversations to be recorded - and even if there is, a written form should be filled, containing the following items [24-27]:

A. When, where from and who makes the call (name and surname of that person - physician or nurse)

B. General information about the patient to be transported (name and surname, sex, date and hour of birth, place of birth)

C. Discription of the disease along with therapy and diagnostic procedures results; current diagnosis

D. Assessment of the emergency of transport

E. Instructions to the caller - potential diagnostic and therapy procedures to be performed before the transport team arrives

F. Contact person's name and telephone number that transport team can contact if needed

G. Assessment of time from the moment of making the telephone call to the transport team's arrival (which is told to the person making the call).

Following the transport team arrival, the physician (or nurse) who had made the call (or treated the newborn until the transport team arrival) validates the medical documents stating the patient's condition by signing them when the team has arrived. It is useful to hand over to the transport team the copy of work-ups, X rays, blood type of the newborn and mother, important information about pregnancy - diseases, an antibody titer etc. Transport sheet should contain the name and surname of the physician, the nurse and the driver who carried out the transport [9,11].

Disinfection during transport

Disinfection during transport is very important in order to prevent spreading multi-resistant germs from one facility to another.

Parents' consent prior to transfer

Prior to transport parents must be fully acquainted with the information of their child's condition and the purpose of transfer. With different congenital malformations it is important to explain to parents the type and kind of defect, the options of treatment and the risks. One should be very careful about giving prognosis concerning that transfer itself does not guarantee survival and healing. Parents should sign a written consent for the transport and special procedures (transfusion of blood products, or surgery if necessary) [28-30].

It is of great importance for parents to be able to see and touch their child, and if possible, take photographs or get the newborn's foot print before transport. If parents cannot be transported together with their newborn, the regional hospital is obliged to give them information about the place and possibilites of staying with their child (accommodation for mothers, a hotel etc).

Most NICU that newborns are transferred to have brochures with the information about the department, its address, telephone number, that should be given to parents before transport. Parents need to meet the people in charge of the transport (know the name and surname of the physician and nurse). If it is assessed that the newborn is critically ill with an unfavourable prognosis, the team may talk to parents about potential naming and christening the child, etc [31-33].

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Thoracoscopic Esophageal Atresia Repair First Ever Pakistanian Study, Early Learning Curve-Juniper Publishers

Introduction

Esophageal atresia with or without fistula is rare congenital anomaly occurring In 1/3000-5000 of neonatal population [1] which is traditionally repaired by posteriolateral thoracotomy& still a standard approach, but this approach is associated with musculoskeletal morbidity and later in life scoliosis, chest wall deformity & nerve damage [2-4]. With the advancement in minimal invasive paediatric procedures & high definition technology and looking into consideration of these morbidities it become possible to perform technically demanding & complex procedures thoracoscopicaly. In relation to this first time in 1999 isolated esophageal atresia done in male infant of 2 month which was repaired thoracoscopicaly [5]

For last 10 years after first repair refinement's in technique & feasibility in thoracoscopic repair of esophageal atresia led to adoption of this technique in many institutions world wide [1]

The objective of study is to do esophageal atresia thoracoscopicaly going through few basic steps of learning curve to make it possible for achievement of final thoracoscopic repair

Material and Methods

This is retrospective study conducted from June 2015 to May 2016 at Peoples University of medical & health sciences Nawabshah Sind, Pakistan & 11 patients included for thoracoscopic esophageal atresia repair Detailed pre-operative assessment done in all patients, fullterm with type C esophageal atresia weighing greater than 1.5 kg was selected. Neonates with major cardiac anomalies on echocardiography & having GI anomalies were excluded. Preoperative assessment of gap between two atretic ends were roughly assessed by plain x-ray of cervical & thoracic spine in lateral view with large bore tube in situ in esophagus.The distance between tube & expected carina level at T4 measured and if it is more than two vertebrae expected to be larger gap between two ends (Figure 1) Data collected included newborn age & weight at the time of surgery, operative time, mechanical ventilation required, days of hospitalization, time to start first feed & post-op complications.

Technique

Baby placed in modified prone position with right side elevated at 45 degree with tracheal intubation in all cases & no attempt was done for single lung ventilation. In first four cases single small incision of 2-3 cm like we do in VATS given below the tip of scapula at 5th intercostal space. 5mm scope put from top of incision & two working 3mm instruments places directly from mid of incision, lung retraction done with 3mm fan retractor from bottom of incision .The aim of this technique was to see videoscopic view of internal anatomy and to assess difficulties while doing few of steps video-asisted like azygos vein ligation & fistula dealing. Rest of procedure completed by open method. In next 7 cases three port technique was used consisted of camera port of 5mm just below tip of scapula, right port of 5mm In mid axillary line two space above camera port and the last 3mm port placed two space below in 6th or 7th intercostal space behind posterior axillary line (Figure 2).

Lung collapse was done with Co2 insufflations with flow of 1lit/min & pressure kept between 5-8 mmhg. The first step in all cases was to deal azygos, which was accomplished by hook electrode easily after opening the pleural membrane. Next step was to ligate with fistula (Figure 3) which was done with 5mm of Grena plastic clips passed through right upper port (Figure 4)Next was to identity the upper pouch, which was mobilized by hook in right hand and grasper in left hand to pull pouch inferiorly to achieve adequate length (Figure 5) Once the good length is achieved posterior layer of two ends was done intracorporeal by 5/0 non-absorable in 2 cases and vicryl in rest of cases. After completing posterior layer trans anastomotic tube placed & anterior layer of esophagus repaired (Figure 6) Chest drain placed in all cases.

Results

Initial 4 cases out of llcases underwent video assisted like Uniportal VATS, which were later on converted to complete the procedure &7 cases, were successfully done thoracoscopicaly. Mean age at the time of surgery was 3 days because most of patients coming late in our hospital & mostly they are diagnosed postnatally. Weight of patients at the time of surgery ranges between 1.5-3.5kg in the initial case as including the video assisted cases the operative time was bit longer between 120 to 150 minutes, which later on improved in last two cases was under 120 minutes. Mechanical ventilation required in 4 cases &rest of patients were fine post operatively and only required oxygen support for few days.

Contrast swallow done in all case on day 5 but in 2 cases early contrast was done suspecting leak out of which one case had a confirmed leak, which was healed conservatively. First feed was started on day 3 in 4 cases through nasogastric tube which were clinically fine, in 5 cases feed started on day 5 after contrast swallow & remaining two case feed started lately after 6 days as these were on mechanical ventilation.

Postoperative complications encountered in 3 cases out of which one had leak, one had stricture & last one had reflux. Leak was managed conservatively, stricture improved on dilatation & gastro esophageal reflux managed on medical therapy. Days of hospitalization were variable ranging from 8 days to 16 days. There was one mortality in our series that patient presented late after 7 days & died because of sepsis.

Discussion

According to IPEG & EUPSA survey thoracoscopic esophageal repair is a world wide accepted procedure in more than 65 developed centers that are attempting but many aspects of EA management are lacking consensus they recommend establishment of an EA registry at the end of survey [6,7] .

In few Asian countries & Middle East thoracoscopic repair of esophageal atresia has been started but they are at initial learning stage & they have found same results as develop centers [8-10]. In Pakistan it is first ever study done which is going to be reported in order to follow the develop centers doing advance paediatric minimal invasive surgery. Details regarding advantage of thoracoscopic repair have been extensively studied like superior visualization, Cosmesis, identification of fistula & avoidance of musculoskeletal morbidities [1,11]. In our study in the initial four cases we have adopted new technique like Uniportal VATS to learn the videoscopic anatomy & to do procedure keeping the safety of patient in mind. Regarding age, weight & associated anomalies our selection criteria was safe as we have excluded cases with low birth weight, prematurity & major cardiac association are absolute contraindication to thoracoscopic approach as Rothenberg et al also described in his studies [12].

Azygos vein ligation or sparing is an option, which in our study in all case dealt with coagulating hook, but in few studies azygos sparing technique have been done claiming lesser edema at esophageal anastomotic site & prevention of recurrent fistula formation [8,9,13]. Fistula ligation can be done in different ways like suture ligation, titanium clips but we have done with Grena 5mm plastic clips which is having secure locking mechanism in front but there is no statistical significant difference in all techniques [1, 11,14].

The most difficult part of surgery is esophageal anastomosis intracorporeally which is technically demanding, the technique is same like open but the first stich is difficult one to apply because of apart ends, we have the same feelings especially in long gap where we have applied stays before applying first intracorporeal stich [8,11]. The operative time is variable in our study which was more in initial cases because of technical difficulty & small working space, the same duration of operative time was seen in other studies with their initial learning curve experiences.

Although the number of patients in our series is small we cannot conclude issues related to post operative complication like leak & anastomotic narrowing in our cases was only 10% each but different comparative studies have found these complication are lesser in thoracoscopy group versus open group [15,16].

Recently study by borruto et al with Meta analysis shows no difference in open & thoracoscopic group but the only advantage is to prevent major thoracotomy [17].

Conclusion

Thoracoscopic repair of esophageal atresia with TEF is feasible, but is technically challenging& demanding.Our current experience is on quite limited number of patients and there is considerable learning curve required till the perfection of procedure. There is need to make refinements in the steps of the complex paediatric laparoscopic procedures to make it easy for surgeons wanting to adopt this procedure. Take home message is that a multi center program should be started in our part of world that should be guided by experienced surgeons from developed centers& secondly advance suturing skills should be learned before starting this procedure.Avoidance of thoracotomy is a major advantage and is proven benefit in the recovery of patient.

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Iron Deficiency: Beyond Anemia-Juniper Publishers

Abstract

Iron deficiency is most common nutritional disorder affecting at least one third of world population. Iron is crucial to biologic functions, including respiration, energy production, DNA synthesis, and cell proliferation. Impaired brain development and cognitive, behavioral and psychomotor impairment are worrisome manifestation of iron deficiency. Studies have demonstrated that some of the changes occurring during period of brain growth spurt (<2 years age) may be irreversible. Association of iron deficiency with febrile convulsion, pica, breath holding spells, restless leg syndrome and thrombosis is increasingly being recognized. Impaired cell-mediated immunity and bactericidal function are generally noted in iron deficiency however, the findings are inconsistent

Keywords: Iron deficiency anemia; impaired Cognition; Breath holding spells; Pica Febrile convulsion; Thrombosis; Infections

Introduction

It is estimated that more than 1.5 billion people affected globally. One third of the world population suffers from Iron deficiency anemia of which 90% live in developing third world. Iron is the most important component of hemoglobin. Iron deficiency is the most common cause of nutritional anemia and commonly occurs during period of increased requirement e.g. in infancy, adolescence pregnancy and during lactation especially among the people with poor socioeconomic status due to inadequate intake of dietary iron, infestation, infections, and malabsorption Anemia is just one manifestation of iron deficiency. Pallor is seen over the face, palm, nail and tongue. The child may like to eat inedible objects such as clay and mud (pica). The child may not be playful and active because of easy fatigability Anemic children are susceptible to develop frequent infection. Iron deficiency during early life has been seen shown to slow neurodevelopment, cause attention deficit hyperactivity disorders, reduce learning capacity, and predispose to development of febrile convulsion. Iron deficiency result in to conduct disorders, significantly lower scholastics performance, reduces cognitive performance, breath holding spells and papilledema.

Pathophysiology

Iron plays an essential role in the synthesis of hemoglobin. It is unique in that it takes up and releases oxygen with no energy expenditure. Large amounts of the iron are recycled daily from the breakdown from the destroyed red cells. Dietary iron occurs in two forms. Heme iron from animal proteins is better absorbed than non heme iron. Non heme iron is obtained from plant foods and vegetables and is absorbed in the ferrous form mostly in the duodenum and to a lesser extent in the jejunum and proximal ileum. Absorbed iron from the intestinal lumen is transported with a divalent metal protein (DMT) across the mucosal border. From the enterocyte the iron reaches the plasma by the specific transporter proteins called ferroprotein. The primary mechanism of iron homeostasis is regulated by hepcidin.

Hepcidin level decreases in iron deficiency and increases in inflammation and iron excess. The elevated hepcidin blocks the ferroprotein and limits the mobilization of iron in to the plasma. Iron circulates the blood bound to the transferring. Bone marrow erythroblasts have receptors for the iron transferrin complex and the iron complex enters the cells by the endocytosis for the hemoglobin synthesis. The absorption of iron is regulated by iron deficiency (absorption increases), inflammation and iron repletion (absorption decreases), mediated by hepcidin.

Over 90% of the dietary iron for infants and young children are non heme iron. Only 10% of dietary non heme iron is absorbed. The absorption of non heme iron is highly altered by the dietary factors. Food rich in Vitamin C like orange juice, meat and fish enhance iron absorption; calcium, phosphate, tannin in tae and bran decreases the absorption. Breast milk iron, present in low concentration is well absorbed; cow milk contains high level calcium and phosphorus that interfere with iron absorption [1-5].

Stages of iron deficiency

First stage: A decrease in concentration of serum ferritin.

Second stage: Hb concentration is normal or the normal range, low serum iron concentration and transferring saturation, increase in total iron binding capacity.

Third stage: Decrease Haemoglobin associated with low MCV & MCH and high RDW (Figure 1).

Iron and Brain

Behavioral and cognitive dysfunction are must worrisome manifestation of iron deficiency. Recent research has revealed that anemia is late manifestation of iron deficiency, brain deficiency occurs even with normal level of hemoglobin, as iron is most important to red blood cells over all other organs including brain. The biological basis of the behavioral and cognitive development delays due to abnormalities in neurotransmitter metabolism, decreased myelin formation and alterations in brain energy metabolism [6].

IDA and Infants < 2 years age

In Infants adverse effects of iron deficiency on behavior are special concern because the latter part of brain spurt coincides with the period in which iron deficiency anemia is most prevalent (6-24 month). Observational studies have suggested that iron deficient children have lower IQ scores, decreased attentiveness and lower scores on tests of academic performance compare with non anemic controls. These studies indicates that iron deficiency anemia in infancy, perhaps of particular severity and chronicity, has irreversible cognitive impairment [7].

IDA in Children > 2 years Age

Observational studies in children over 2 years have reported poorer cognition and school achievement in iron deficient children. Adolescent girls are prone to develop iron deficiency because of poor dietary intake along with increased iron requirement related to rapid growth and menstrual blood loss and are at greater risk of cognitive impairment [8].

Preventive Trails

Two recent trials reported beneficial effects of iron therapy in infancy. Developmental and behavioral benefits from iron supplementation in infancy.

IDA and Pica

The word Pica is derived from latin root meaning magpie, a bird capable of eating a variety of things. Lanzkowsky define Pica as "a perversion of appetite with persistent and purposeful ingestion of non nutritive substances like Pica includes geophagia (dirt or clay ingestion) tricophagia (hair ingestion), amylophagia (starch ingestion) and pagophagia (ice ingestion). It is a well documented feature of iron deficiency anemia in children.

IDA and Breath Holding Spells (BHS)

The low hemoglobin cause rapid cerebral anoxia due to decreased oxygen carrying capacity of blood that in turn lead to Breath holding spells. Anemic children being irritable may be more predisposed to breath holding spells. Iron therapy should more remarkable therapeutic beneficial in controlling the spells in children with evidence of iron deficiency. Iron has role as a cofactor in catecholamine metabolism in central nervous system. Clinical profile and hematological associations of BHS as result of interaction of cerebral erythropoietin, nitric oxide and interleukin 1 [10-13].

IDA and Febrile Seizure

Febrile seizures are the most common type of seizures, occurring in 2-5% of all children. Kebrinsky et al studied the role of iron in febrile seizures and they reported significantly increased incidence of iron deficiency in non seizures compared to seizure group. Iron deficiency may increase frequency of febrile convulsion. Exact etiology of febrile seizures is not known however depend on metabolism of several neurotransmitters, enzyme activities and cerebral erythropoietin has been postulated as causative factor for seizures [14].

IDA and Stroke

Iron deficiency anemia is significant risk factor for stroke in otherwise healthy young children. This increased incidence of thrombotic complications in IDA due to various factors. Increased level of erythropoietin in IDA has been incriminated to have a possible role in stimulating megskaryopoesis, resulting in thrombocytosis. Recently, Bilic & Bilic [5] have reported that amino acid sequences homology of thrombopoietin and erythropoietin may explain the thrombocytosis in children with iron  deficiency anemia. In addition to the increased thrombotic risk associated with high platelet count, other possible mechanism suggested is decreased in antioxidant defense in iron deficiency anemia result in to increased oxidative stress, prone to develop platelet aggregation. Reduced deformability and increased viscosity of microcytic red blood cells in iron deficiency may contributory by affecting blood flow patterns within the vessels. Furthermore, anemic hypoxia secondary to iron deficiency could precipitate situations of increased metabolic stress ( i.e. infection ) at risk area of brain supplied by end arteries, such as the basal ganglia, thalamus and hypothalamus resulting in stroke [15,16].

IDA and Restless Leg Syndrome (RLS)

Restless leg syndrome is characterized by repeated aphasic involuntary muscles contractions. It is mostly reported in adult patient and largely under reported from pediatric population. Although most of the cases with RLS are idiopathic or hereditary; decreased brain iron content and metabolism can lead to RLA. Iron deficiency state may precipitate RLS in as much as 25-30% of people. MRI studies have demonstrated decreased iron content in substantia nigra and red nucleus. Fatigue decrease productivity and decrease learning capacity with iron deficiency [17-20].

Iron and Infection

Iron is required for normal immune function, cell differentiation and growth. Iron is also required for peroxide generating mechanism, cytokine production and myeloperoxidase function in neutrophil. Impaired cell-mediated immunity and bactericidal function are generally noted in iron deficient children; however, the findings are inconsistent. Impaired immunity result into repeated infection [21-25].

Iron and Temperature Regulation

Iron deficiency anemia more readily become hypothermic and have depress thyroid function.

Iron and Growth

Iron deficiency anemia result into impaired growth [26].

Celiac Disease

Malabsorption of iron and result into iron deficiency anemia [27].

Prematurity

Preterm baby due to low iron store and more to growth result into iron deficiency anemia.

Vitamin A and iron deficiency anemia

Deficiency of Vitamin A Limit Mobilization of Iron from Its Stored Site hence Causing Iron Deficiency Anemia [25].

Hook worn infestation

Having hook worn infestation in children decrease level of blood hence increasing iron demand.

Conclusion

Being most common nutritional disorder, it is imperative to recognize effects and long term consequences of iron deficiency Though anemia is common, iron deficiency state without anemia is largely under-recognized. Studies have reported lower cognitive scores even in children with iron deficiency without anemia. Irreversible cognitive impairment has been reported in children who experienced iron deficiency during period of critical brain growth (<2 years of age). Iron deficiency anemia is highly prevalent in India (reported 55.7% to 85.1% in different states in NFHS-3) and much larger population having iron deficiency without anemia; hence it is critical to recognize the cognitive impairment and treat early [28,29].

Point to be Remember

• Iron Deficiency is most common preventable nutritional deficiency in the world, especially among infants and young children.

• Prevalence of iron deficiency anemia among children under 5 year of age has been estimated to be 75% in India.

• Iron deficiency has adverse effect on physical, mental, emotional, cognitive performance.

• The role of iron deficiency in precipitating febrile convulsion breath holding spell, hyper cyanotic blue spell and infection.

• Iron deficiency has been frequent association oftendency of pica. Pica is common symptoms which predisposes to consumption of lead resulting in plumbism.

• Long term consequences of iron deficiency are poor growth & development, depressed immune function and behavioral changes.

• Serum ferritin along with C-reactive protein serves as best indicator of body iron store.

• The committee of nutrition of the American Academy of Pediatrics recommends the hemoglobin <11 gm/dl and serum ferritin <10 mcg/L as diagnostic of iron deficiency anemia in presence of normal CRP.

• The absorption of iron is regulated by iron deficiency (absorption increases), inflammation and iron repletion (absorption decreases), mediated by hepcidin.

Acknowledgement

The authors wish to thank B.J Medical College Civil Hospital Ahmadabad for support this work.

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The Role of Surgical Ligation of Patent Ductus Arteriosus in Very Preterm Infants: A Mini Review and Case Report-Juniper Publishers

Abstract

Patent ductus arteriosus (PDA) in the very low birth weight premature newborn (VLBWPN) is currently treated with medical therapy or by surgical ligation. The objective of this mini-review and brief case report is to highlight the potential side effects of medical therapy (indomethacin or ibuprofen) including bronchopulmonary dysplasia (BPD), necrotizing enterocolitis (NEC), intraventricular hemorrhage (IVH), bowel perforation, pulmonary edema and bleeding, retinopathy, thrombocytopenia, and decrease in renal function and present an example of a patient frequently encountered in the neonatal intensive care unit that dramatically improved with prompt surgical intervention. Secondary objective is to support a more proactive approach toward early surgical ligation and highlight factors in VLBWPN who might be at the greatest risk and might benefit from earlier intervention. Currently PDA ligation is generally performed in the cases in which indomethacin or ibuprofen medication is unsuccessful or contraindicated. Given the side effects of indomethacin or ibuprofen medical therapy, it is important to recall the risks of nonoperative management of VLBWPN and to consider earlier utilization of surgical ligation.

Introduction

Patent ductus arteriosus (PDA) is a serious condition frequently seen in the neonatal intensive care unit in premature low-birth-weight infants. The reported incidence of PDA among premature babies less than 28 weeks and 1000 grams is as high as 70%, and the rate of spontaneous closure in very low birth weight premature newborns (VLBWPN) is about 34% [1]. Patent ductus arteriosus (PDA) in preterm infants is an important entity to diagnose since it is well established to be associated with increased mortality and morbidities such as bronchopulmonary dysplasia (BPD), necrotizing enterocolitis (NEC), and intraventricular hemorrhage (IVH); however, a causal relationship has not been definitively established [2]. Patent ductus arteriosus, which leads to significant hemodynamic, pulmonary, gastrointestinal, cerebrovascular, and retinal problems, can be medically or surgically treated; although, there has been an ongoing debate on which method should be preferred [1]. However, studies show that PDA closure rates with medical treatment are still low in VLBWPN. Failure of medical intervention occurs in 40% to 50% of VLBWPN necessitating surgical intervention [3,4]. In patients with a bleeding diathesis, necrotizing enterocolitis (NEC) or where nonsteroidal anti-inflammatory drugs (NSADs) are contraindicated surgical intervention is the only available option [4]. Given the side effects of indomethacin or ibuprofen medical therapy such as necrotizing enterocolitis, bowel perforation, pulmonary edema and bleeding, retinopathy, thrombocytopenia, and decrease in renal function [1] it is prudent to seriously consider an alternative to nonoperative management of VLBWPN.

Case Report

Patient was a fourteen day old female born premature at 24 weeks gestation. She was born via caesarian section due to preterm premature rupture of membranes and fetal intolerance to labor with a birthweight of 490 grams. She was noted to have Apgar scores of 5 and 8 and required immediate neonatal resuscitation with intubation for respiratory distress syndrome (RDS). She subsequently was admitted to the neonatal intensive care unit (NICU) for closer monitoring. At the time of consultation, she was tolerating feeds and weighted 560 grams but continued to require mechanical ventilation with inability to wean Fi02 (upper 40%). A transthoracic ECHO cardiogram was performed in the immediate postpartum period and found to be consistent with a large PDA with left to right shunting.

The patient subsequently underwent attempted medical therapy for closure of PDA with two courses of indomethacin. Repeat transthoracic echocardiogram on day of life eleven was found to be consistent with persistent moderate PDA with continued left to right shunting with a gradient of 25-30mmHg across the PDA. Other pertinent past medical history was significant for questionable germinal matrix bleed, extreme prematurity and respiratory failure. Other imaging included serial daily chest x-rays consistent with RDS with bilateral fluid overload that was unresponsive to Lasix.

Surgery was performed in the NICU; the patient was positioned in the right lateral decubitus position and all pressure points were padded. The left chest was prepped and draped. A posterior lateral thoracotomy was performed with Bovie electrocautery just inferior to the tip of the scapula and blunt dissection was preformed down to the subcutaneous tissues; the latissimus dorsi muscle was divided. The serratus muscle was then retracted anteriorly and the third interspace was identified. The electrocautery was further used to divide the intercostal muscles and the left chest was entered. The aorta, recurrent laryngeal nerves, subclavian artery, and the ductusarteriosus were identified; grossly normal anatomy was noted. Dissection was then carried out inferiorly and superiorly to the patent ductus arteriosus after the overlying pleura was opened. A test clamp was then preformed which she tolerated well. A medium clip was applied to the ductus, and it was noted to occlude completely. The lung was reinflated and the ribs reapproximated “with two 3-0 interrupted Vicryl sutures. Likewise the muscles were reapproximated and finally the skin was closed in a subcuticular fashion. The postoperative chest x-ray showed the clip in good position and the lungs adequately reexpanded. The immediate postoperative course was uneventful and the postoperative day one chest X-ray revealed dramatic resolution of bilateral pulmonary infiltrates (Figure 1). The Fi02 was quickly weaned to the low 30s without difficulty. The neonate resumed feedings on postoperative day one.

Discussion

Surgical PDA ligation is generally utilized after medical therapy with indomethacin or ibuprofen has been unsuccessful at closure. Thoracotomy (eventually done bedside in the neonatal unit to avoid transport) has been the standard of care for treating large, symptomatic, or persistent PDAs since its inception in 1938 [3]. In a study by Trust et al, they found for infants under 800 g, the failure rate for PDA closure with indomethacin was found to be as high as 40%-50% [5]. In addition, indomethacin treatment in VLBWPN has numerous severe side effects such as necrotizing enterocolitis, bowel perforation, pulmonary edema and bleeding, retinopathy, thrombocytopenia, and decrease in renal function [1]. Furthermore, other studies have clearly shown that the incidence of NEC and the duration of TPN were significantly decreased in premature infants with early surgical ligation of symptomatic PDA that was refractory to medical treatment [6].

As highlighted by Weisz et al, it is important to note that many of the observational studies on the treatment of PDA do not adequately account for confounding by indication [7]. In other words, it may be that PDA ligation is a surrogate marker for increased illness severity, as “sicker” infants may be more likely to be referred for ligation and therefore surgical ligation is generally not considered until medical treatment has failed or was contraindicated [7]. Studies to date have inadequately addressed this confounding by indication.

The results from previous studies by Grosfeld et al and Cassady et al support an opinion, that VLBWPN should undergo early surgery as the treatment of choice [8,9]. The defined interval from failed medical management in the VLBWN to surgical ligation has not been defined and is likely multifactorial in nature. Therefore, a brief period of conservative medical management for VLBWN with PDA may be a reasonable approach, but without rapid resolution and success with drugs, surgical closure of PDA, generally assured and complete with one attempt, remains the gold standard of treatment and should not be delayed.

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Strategies for Increasing Immunization Rates Among Children and in Low to Middle Income Countries-Juniper Publishers

Abstract

The article reviews the importance and what strategies are effective in improving immunization rates in children and adults. How can we help improve immunization rates in the U.S. and in LMICs? Do strategies to improve childhood vaccination rates work? [1,2] reported the importance of immunizations as an important childhood preventive health maintenance program towards improving childhood survival.

This is an important feature for childhood health for two reasons:

Reported immunizations as the most successful public health prevention program for preventing childhood diseases and untimely illnesses or death in childhood. The WHO highly recommends safe monitoring and administration of vaccinations. In conclusion [1] found mostly a low level of evidence to support each strategy for improving immunization rates among children in LMICs and there is some low level of evidence for a combined effect among strategies as follows: information handouts, health education with reminder cards at facilities, immunization outreach with and without household incentives, home visits, and integration of immunization with other services [4]. Found some significance in single-method interventions, but found a combined effect more promising to increase vaccine rates. Further well conducted RCTs are needed.

Background Summary

Human papillomavirus (HPV) is the most common sexually transmitted infection in the United States with approximately 79 million Americans are infected with HPV both men and women alike. Each year an estimated 14million people become newly infected. Not all 100 types of HPV cause pathological disease,

however some do and can lead to genital warts, different types of cancers (i.e., cervical, vulvar, vaginal, penile, anal, and oropharyngeal). There are approximately 27,000 adults affected by HPV associated cancer annually and approximately 360,000 people experience genital warts. HPV vaccine is the primary prevention for HPV associated diseases. HPV vaccine rates like others in a series tend to drop by the time of the last dose by about 20%. Overall HPV vaccination rates are low at 40% for girls and 22% for boys [1,2].

Reported the importance of immunizations as an important childhood preventive health maintenance program towards improving childhood survival.

This is an important feature for childhood health for two reasons

Direct reduction in disease through immunization and

It allows time during visit to review for other treatable conditions [1].

In a Cochrane review studied interventions for improving coverage of childhood immunizations in low and middle-income countries (LMICs) updated from prior review in 2011 [3], reported immunizations as the most successful public health prevention program for preventing childhood diseases and untimely illnesses or death in childhood. The WHO highly recommends a safe monitoring and administration of vaccinations. Vaccines typically have a high safety profile and side effects are minimal, if at all, mostly site redness, fatigue, and tenderness for a couple days [4].

Study Purpose

Completed a systematic review to evaluate the effectiveness of interventions or strategies for increasing HPV vaccination rates and [1,2] also completed a review to examine interventions or strategies for improving vaccination rates in childhood in LMICs. According to the [5] in the U.S. many immunization rates are overall fairly high, but gaps still exist and there is still room for improvement timeliness at the recommended age is essential for proper coverage. In 2011 in the U.S., 4 cases of rubella, no cases of diphtheria, 36 cases of tetanus, and no polio cases existed at the time. Vaccines in a series were noted to be a problem such as DTaP from 19-35 months of age only 84.6% of the children had completed the four dose series in 2011 [5]. In cases of outbreaks, it is often found that it may exist in an unimmunized person or a person at risk due to illness or immunocompromise. Flu vaccination rates in health care workers and the elderly population overall are less than 70% [5]. Sustainable strategies for improving vaccination rates to reduce vaccine preventable diseases remain an important role in our health care prevention plan.

Research Question

The research question is clear what strategies are effective in improving immunization rates in children and adults? How can we help improve immunization rates in the U.S. and in LMICs? Do strategies to improve childhood vaccination rates work?

Research Design

Studies reviewed included randomized controlled trials (RCT) [1], non-RCTs, controlled before after studies, and interrupted time series conducted in LMICs involving children age 0-4 years, caregivers, and healthcare providers in a Cochrane Review meta-analysis [4]. Studies included RCTs, quasi-experimental or observational studies all quantitative data.

Sample Size and Selection

Studies reviewed included randomized controlled trials (RCT) [1], non-RCTs, controlled before after studies, and interrupted time series conducted in LMICs involving children age 0-4 years, caregivers, and healthcare providers. Two authors independently reviewed studies for eligibility. Study participants were over 1,692 [4] Located 2,569 studies in the primary search and included 34 studies in the review that met inclusion criteria.

Measuring Instruments

Used RR for dichotomous data and planned to report costs with a mean difference (MD) [1], but no studies reported this type of data. Confidence intervals (CI) for all measures [4]. Utilized a forest plot of selected results of intervention studies measuring series initiation of >1 dose of HPV vaccine and additional results is shown in an additional forest plot.

Statistical Analyses

Consisted of 14 studies (10 cluster RCTs and 4 individual RCTs) all met inclusion criteria. Study locations included states in the U.S. (i.e. Georgia) and other countries such as (i.e., Ghana, Honduras, India, Mali, Mexico, Nicaragua, Nepal, Pakistan, and Zimbabwe) [1]. One study had a unclear risk of bias and 13 had high risk of bias. Interventions or strategies utilized were as follows: community based health education (3 studies), facilitybased health education (3 studies), household incentives (three studies), regular immunization, outreach sessions (1 study), home visits (1 study), and supportive supervision (1 study), and information campaigns (1 study), and integration of immunization services with intermittent prevention treatment of malaria (1 study). There was a moderate level of evidence to support health education at town meetings or at home (risk ratio (RR) 1.68, 95% confidence interval (CI) 1.09 to 2.59).

Low-certainty, evidence that facility-based health education plus vaccine reminder cards may improve vaccination rates (RR 1.50, 95% CI, 1.21-1.87). Household monetary incentives have little or no effect on vaccine rates. Regular immunization outreach may improve vaccine rates with low level of evidence (RR 3.09, 95% CI, 1.69-5.67). Immunization outreach when combined with household incentives may improve vaccine rates with low level of evidence (RR 6.66, 95% CI, 3.93-11.28). Home visits to identify non-vaccinated children and health clinic referrals for immunization updates may improve vaccine rates with low level of evidence (RR 1.22, 95% CI, 1.07 to 1.39). Integration of immunizations with services had low level of evidence to improve vaccine rates (RR 1.92, 95%, CI 1.42-2.59).

[4] Included a study of mostly girls 70.6%, n=24 but included adolescents and young adults regarding HPV vaccination rates. Sixteen studies (47.1%) included looked at intervention effects on more than on e vaccine including HPV vaccine while 18 (52.9%) evaluated the intervention impact only on HPV vaccine coverage. Interventions included vaccine requirement for school attendance, patient reminder and recall systems, patient education, community-based interventions implemented in combination, provider assessment and feedback, provider reminders, healthcare system-based interventions, vaccine program in schools, reducing out of pocket costs. Most interventions demonstrated an increase in vaccine rates. Patient education when used alone as little evidence to demonstrate effect but in combination with other intervention has more success. Reminder and recall (i.e., text, mail, phone, e-mail etc.) with patient education demonstrated the most single effect.

Conclusion

[1] Found mostly a low level of evidence to support each strategy for improving immunization rates among children in LMICs and there is some low level of evidence for a combined effect among strategies as follows: information handouts, health education with reminder cards at facilities, immunization outreach with and without household incentives, home visits, and integration of immunization with other services. Further well conducted RCTs are needed. [4] Found some significance in singlemethod interventions but found a combined effect more promising to increase vaccine rates. More studies were recommended to integrate in multi-type facilities and adapted into practice on a wide scale.

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Gullian – Barre Syndrome Variant with Unilateral Facial Weakness-Juniper Publishers

Abstract

It is a postinfectious polyneuropathy due to alteration of protein component of myelin (p2 neurotogenic peptide) leading to demyelination because of autoimmune mechanism. Neurological manifestation begins after 2 to 4 weeks of viral or bacterial infection. Clinical expression includes an acute onset symmetrical ascending weakness (both proximal & distal) with unilateral facial weakness  and respiratory weakness and autonomic dysfunction. The diagnosis depends on clinical picture, electrophysiological findings and CSF examination. Immunotherapy is the main stay of treatment. IVIG & Plasmaphrersis done within 2 to 4 weeks of symptoms onset is recommended. Treatment is warranted in non ambulatory patient (Modified Hughes GBS disability scale). The patient who hav not responded to initial IVIG treatment may benefit from second course of IVIG. General supportive care includes cardiorespiratory care, physiotherapy, nutritional management, management of neuropathic pain, bladder–bowel care and prevention of deep vein thrombosis.

Keywords: Acute inflammatory demelinating polyradiculoneuropathy (AIDP); Acute motor axonal neuropathy (AMAN); Acute flaccid paralysis; Clinical neurophysiology; Immunotherapy

Abbreviations: AIDP: Acute Inflammatory Demelinating Polyradiculoneuropathy; AMAN: Acute Motor Axonal Neuropathy; NCS: Nerve Conduction Study; IVIg: Intravenous Immunoglobulin

Introduction

Gullian – Barre syndrome is also known as acute inflammatory demyelating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN). AIDP is the predominant subtype in North America & Europe while AMAN is commonly reported subtype in Asia including India & Central & South America. About 65% of children report preceding upper respiratory tract & gastrointestinal tract infection. Immunopathogenesis involve molecular mimicry & formation of cross reacting antiganglioside antibodies. The GBS has several variants depending upon distribution of motor, sensory, cranial, autonomic or cebellar involvement variant, among them AIDP is the most common [1]

Discussion

Clinical expression

Muscle pain, difficulty while walking or refusals to walk are often the first presenting symptoms (50%)

Distal limb weakness which ascending & symmetrical (20%) Areflexia.

Respiratory muscle weakness & facial weakness (20%)

Sensory symptoms including painful parathesia, backache & meningismus (50% -80% )

Transient bladder involvement can occur in few children.

Approximately 25% develop respiratory insufficiency requiring artificial ventilation & 75% have autonomic dysfunction. The course is monophasic in most of the children. 80 % reach maximum severity within 2 weeks & 97 % in 4 weeks.This phase is followed by a relatively static ‘plateau phase’ ranging from 2 days to 6 months before recovery begins [1].

Diagnosis

The diagnosis of GBS is clinical & is supported by a few investigations. Characteristic CSF finding are of Albumino- Cytological dissociation i.e. a combination of elevated CSF protein & normal cell count. Nerve conduction studies help in diagnosis of different sub types of GBS. In early phase of GBS motor & sesory nerve conduction study (NCS) is normal. In such situation diagnosis supported by prolonged F waves latencies. NCS abnormalities tend to peak by 2 weeks of illness. Children with GBS should be managed in PICU during initial phase (Table 1) [2,3].

Clinical neurophysiology

Earliest abnormalities is a drop in the amplitude of the evoked muscle action potential & conduction blocks. Marked slowing of nerve conduction can be recorded in about 50% patients. Reduced compound motor unit potential amplitude is the most frequent finding. Absence or prolongation of F wave is common. Proximal blocks can be detected by measuring F wave. Conduction studies improve slowly over a period of several months. Spontaneous fibrillation may detected on electromyography during recovery phase after 2 or 3 weeks [1].

Pathogenesis

AIDP is multifocal noninfective inflammatory process causing demyelinaton or axonal degeneration of peripheral nerves. ADIP is generally due to T cell mediated immune myelin damage. Increased incidence of several axonal degeneration in GBS following C. jejuni infection is known with more severe involvement. The mechanism of axonal damage is different molecular mimicry due to shared epitopes with gangliosides [1] (Figure 1).

Prognosis

Recovery is common & often complete in the majority.Mortality is now ≤ 5%. Most of the death in childhood are due to preventable respiratory complication. Acute axonal neuropathy, with good pgognosis.Acute axonal and sensory axonal neuropathy generally causes poor prognosis. Miller Fisher variant in which cerebellar signs, cranial nerves are involved also has a poor prognosis. The disabilities included foot drop, pes cavus and postural tremor & persisting weakness of the hands [1].

Treatment

Supportive symptomatic treatment is the mainstay of therapy in the majority. The child should be closely observed in hospital, objective assessment of respiratory function, regular measurement of vital capacity is performed. Ventilatory support should be considered if there is evidence of respiratory insuffiency [3,4].

Dysphagia if present necessitates nasogastric feeding.

Chest and limb physiotherapy should be initiated early and carried out carefully. Bladder and bowel function should be attended to.

Plasmapheresis has been shown to be effective in decreasing severity and shortening the non ambulatory phase. No significant complication ensue fron well conducted plasmapheresis. Several paediatric studies support the results.

Intravenous immunoglobulin (IVIg) is atleast as effective as Plasma exchange. IVIg is now become the preferred treatment due to ease of administration. Dose of IVIg 0.4 g/ kg body weight daily given on five successive days or two successive doses of 1 g/kg may be given active treatment of impending/ respiratory failure is imperative. Indication of immunotherapy includes a hughes GBS disabilities scale ≥3 or when patient is unable to walk unaided for 10 meters [5-7].

Conclusion

Acute flaccid paralysis in children is a medical emergency. AFP is a clinical syndrome with array of differential diagnosis.The common causes of AFP are Gullian – Barre syndrome, Anterior horn cell myelitis and Acute transverse myelitis. Rapid evolution of the weakness can lead to respiratory failure. Hence a child with AEP Should be managed in PICU in the initial few days. Immunotherapy is a main stay treatment.

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