#agammaglobulinemia

[does-truth-matter]

The CDC has quietly changed who should AVOID the MMR vaccine.

https://www.cdc.gov/vaccines/vpd/mmr/public/index.html

They now state that ANYONE that “Has a parent, brother or sister with a history of immune system problems” should AVOID THE MMR VACCINE!

What exactly is an 'immune system problem?" Every autoimmune disorder.

* Achalasia

* Addison’s disease

* Adult Still's disease

* Agammaglobulinemia

* Alopecia areata

* Amyloidosis

* Amyotrophic lateral sclerosis (Lou Gehrigs)

* Ankylosing spondylitis

* Anti-GBM/Anti-TBM nephritis

* Antiphospholipid syndrome

* Autoimmune angioedema

* Autoimmune dysautonomia

* Autoimmune encephalomyelitis

* Autoimmune hepatitis

* Autoimmune inner ear disease (AIED)

* Autoimmune myocarditis

* Autoimmune oophoritis

* Autoimmune orchitis

* Autoimmune pancreatitis

* Autoimmune retinopathy

* Autoimmune urticaria

* Axonal & neuronal neuropathy (AMAN)

* Baló disease

* Behcet’s disease

* Benign mucosal pemphigoid

* Bullous pemphigoid

* Castleman disease (CD)

* Celiac disease

* Chagas disease

* Chronic inflammatory demyelinating polyneuropathy (CIDP)

* Chronic recurrent multifocal osteomyelitis (CRMO)

* Churg-Strauss Syndrome (CSS) or Eosinophilic Granulomatosis (EGPA)

* Cicatricial pemphigoid

* Cogan’s syndrome

* Cold agglutinin disease

* Congenital heart block

* Coxsackie myocarditis

* CREST syndrome

* Crohn’s disease

* Dermatitis herpetiformis

* Dermatomyositis

* Devic’s disease (neuromyelitis optica)

* Discoid lupus

* Dressler’s syndrome

* Endometriosis

* Eosinophilic esophagitis (EoE)

* Eosinophilic fasciitis

* Erythema nodosum

* Essential mixed cryoglobulinemia

* Evans syndrome

* Fibromyalgia

* Fibrosing alveolitis

* Giant cell arteritis (temporal arteritis)

* Giant cell myocarditis

* Glomerulonephritis

* Goodpasture’s syndrome

* Granulomatosis with Polyangiitis

* Graves’ disease

* Guillain-Barre syndrome

* Hashimoto’s thyroiditis

* Hemolytic anemia

* Henoch-Schonlein purpura (HSP)

* Herpes gestationis or pemphigoid gestationis (PG)

* Hidradenitis Suppurativa (HS) (Acne Inversa)

* Hypogammalglobulinemia

* IgA Nephropathy

* IgG4-related sclerosing disease

* Immune thrombocytopenic purpura (ITP)

* Inclusion body myositis (IBM)

* Interstitial cystitis (IC)

* Juvenile arthritis

* Juvenile diabetes (Type 1 diabetes)

* Juvenile myositis (JM)

* Kawasaki disease

* Lambert-Eaton syndrome

* Leukocytoclastic vasculitis

* Lichen planus

* Lichen sclerosus

* Ligneous conjunctivitis

* Linear IgA disease (LAD)

* Lupus

* Lyme disease chronic

* Meniere’s disease

* Microscopic polyangiitis (MPA)

* Mixed connective tissue disease (MCTD)

* Mooren’s ulcer

* Mucha-Habermann disease

* Multifocal Motor Neuropathy (MMN) or MMNCB

* Multiple sclerosis

* Myasthenia gravis

* Myositis

* Narcolepsy

* Neonatal Lupus

* Neuromyelitis optica

* Neutropenia

* Ocular cicatricial pemphigoid

* Optic neuritis

* Palindromic rheumatism (PR)

* PANDAS

* Parkinson's disease

* Paraneoplastic cerebellar degeneration (PCD)

* Paroxysmal nocturnal hemoglobinuria (PNH)

* Parry Romberg syndrome

* Pars planitis (peripheral uveitis)

* Parsonage-Turner syndrome

* Pemphigus

* Peripheral neuropathy

* Perivenous encephalomyelitis

* Pernicious anemia (PA)

* POEMS syndrome

* Polyarteritis nodosa

* Polyglandular syndromes type I, II, III

* Polymyalgia rheumatica

* Polymyositis

* Postmyocardial infarction syndrome

* Postpericardiotomy syndrome

* Primary biliary cirrhosis

* Primary sclerosing cholangitis

* Progesterone dermatitis

* Psoriasis

* Psoriatic arthritis

* Pure red cell aplasia (PRCA)

* Pyoderma gangrenosum

* Raynaud’s phenomenon

* Reactive Arthritis

* Reflex sympathetic dystrophy

* Relapsing polychondritis

* Restless legs syndrome (RLS)

* Retroperitoneal fibrosis

* Rheumatic fever

* Rheumatoid arthritis

* Sarcoidosis

* Schmidt syndrome

* Scleritis

* Scleroderma

* Sjögren’s syndrome

* Sperm & testicular autoimmunity

* Stiff person syndrome (SPS)

* Subacute bacterial endocarditis (SBE)

* Susac’s syndrome

* Sympathetic ophthalmia (SO)

* Takayasu’s arteritis

* Temporal arteritis/Giant cell arteritis

* Thrombocytopenic purpura (TTP)

* Tolosa-Hunt syndrome (THS)

* Transverse myelitis

* Type 1 diabetes

* Ulcerative colitis (UC)

* Undifferentiated connective tissue disease (UCTD)

* Uveitis

* Vasculitis

* Vitiligo

* Vogt-Koyanagi-Harada Disease

Wonder how many doctors are paying attention?

~shared from Jodi Wilson

[moleculardepot]

Phospho-Btk (Tyr223) Rabbit APC Conjugate

Phospho-Btk (Tyr223) Rabbit APC Conjugate Catalog number: B2018636 Lot number: Batch Dependent Expiration Date: Batch dependent Amount: 10 ug Molecular Weight or Concentration: N/A Supplied as: Solution Applications: a molecular tool for various biochemical applications Storage: 2-8°C Keywords: Bruton tyrosine kinase, Tyrosine-protein kinase BTK, Agammaglobulinemia tyrosine kinase, ATK, AGMX1,…

[drgauravkharya]

Treatment of Primary Immunodeficiency Diseases and Bone Marrow Transplantation

Primary immunodeficiency diseases (PIDDs) are a diverse group of genetic disorders characterized by defects in the immune system. These disorders result in a heightened susceptibility to infections, autoimmune diseases, and malignancies. Effective management of PIDDs is crucial for improving the quality of life and survival of affected individuals. Among various treatment options, bone marrow transplantation (BMT) has emerged as a promising therapeutic approach, offering the potential for a cure for many patients with severe forms of PIDD.

Understanding Primary Immunodeficiency Diseases

PIDDs arise from inherited genetic mutations that impair the development or function of immune cells, such as T cells, B cells, or phagocytes. These conditions are classified based on the specific immune system components affected. Common examples include Severe Combined Immunodeficiency (SCID), X-linked Agammaglobulinemia (XLA), and Chronic Granulomatous Disease (CGD).

SCID, often referred to as "bubble boy" disease, is characterized by the absence of functional T and B lymphocytes, leading to a severely compromised immune response. XLA is a condition where individuals lack functional B cells, leading to an inability to produce antibodies. CGD involves defects in the ability of phagocytes to kill certain bacteria and fungi.

Treatment Approaches

The management of PIDDs involves a combination of supportive care and specific therapies aimed at correcting the underlying immune defect. Traditional treatments include:

1. Immunoglobulin Replacement Therapy:For conditions like XLA, regular intravenous or subcutaneous infusions of immunoglobulin can provide the missing antibodies, thus reducing the frequency and severity of infections.

2. Antibiotic Prophylaxis:Prophylactic antibiotics can prevent infections in individuals with compromised immune systems.

3. Gene Therapy:An emerging treatment option, gene therapy involves correcting the genetic defect responsible for the PIDD. This approach is still under development and has shown promising results in clinical trials for some disorders.

4. Stem Cell Transplantation:Also known as bone marrow transplantation (BMT), this is a definitive treatment for many severe PIDDs.

5. Bone Marrow Transplantation: Bone marrow transplant involves replacing the defective bone marrow or stem cells of an individual with healthy ones from a donor. This procedure can potentially cure or significantly improve the symptoms of PIDDs. The process can be broken down into several key steps:

1. Pre-Transplant Evaluation: Before the transplant, a thorough evaluation is performed to assess the patient's overall health and suitability for the procedure. This includes blood tests, imaging studies, and sometimes a biopsy.

2. Conditioning Regimen:Patients undergo a conditioning regimen, which involves high-dose chemotherapy and/or radiation therapy to eradicate the defective bone marrow and suppress the immune system. This step is crucial to prevent the recipient's body from rejecting the transplanted cells.

3. Stem Cell Infusion: Healthy stem cells, obtained from a compatible donor (usually a sibling or unrelated donor), are infused into the patient. These stem cells migrate to the bone marrow and begin to produce new, healthy blood cells.

4. Post-Transplant Care:After the transplant, patients require close monitoring and supportive care to manage potential complications, such as graft-versus-host disease (GVHD), infections, and organ dysfunction. Immunosuppressive medications are given to prevent the immune system from attacking the transplanted cells.

Outcomes and Considerations

Bone marrow transplantation can be highly effective, offering a potential cure for many severe PIDDs. Success rates vary depending on the specific disorder, the age of the patient, and the compatibility of the donor. Early diagnosis and prompt treatment are critical for optimizing outcomes.

However, BMT is not without risks. Potential complications include GVHD, where the donor's immune cells attack the recipient's tissues, and long-term issues such as endocrine disorders or secondary malignancies. Ongoing research aims to improve the safety and efficacy of BMT, including the development of less toxic conditioning regimens and better methods for matching donors. It is very important to search best BMT centrefor good results.

Conclusion

The treatment of primary immunodeficiency diseases has evolved significantly, with bone marrow transplantation emerging as a potentially curative option for many severe cases. While supportive therapies and gene therapy continue to play important roles, BMT offers hope for a definitive cure and an improved quality of life for affected individuals. Continued advancements in medical research and technology are essential to further enhance the success and safety of these treatments, ultimately providing better outcomes for patients with PIDDs.

[iamnaturalnana]

Who Shouldn't Get Vaccines?

Did your Dr. tell you this ? Avoid vaccination of the MMR if your child has a sibling or parent with an autoimmune deficiency Thanks to Health Freedom Idaho for the list below What exactly is an ‘immune system problem?” Does this include every single auto-immune disorder? *. Achalasia * Addison’s disease * Adult Still’s disease * Agammaglobulinemia * Alopecia areata * Amyloidosis *…

[nathfiset]

What is X-linked agammaglobulinemia and how can cord blood banking help?

I am ready to enroll in cord blood banking NOW and get my special discount!

By clicking on either buttons, you are agreeing to our TOS and disclaimers and will be redirected to an affiliate cord blood banking provider. We might get financial compensation if you sign up with them through our affiliate links. Unlock your special discounts by adding your promo code.CORD300 in the coupon field to get $300 OFF cord blood and tissue banking. OR cord200 to get $200 OFF if you are getting cord blood banking only. I want more information on cord blood banking

 X-linked agammaglobulinemia (XLA) is a rare genetic disorder that affects the body's ability to produce antibodies, leaving individuals vulnerable to recurrent and severe bacterial infections. This rare disorder mainly affects males, with an estimated incidence of 1 in 200,000 births. The condition is caused by mutations in a gene on the X chromosome, which is responsible for producing a protein crucial for the development of certain immune cells. As a result, individuals with XLA have weakened immune systems, making it challenging for their bodies to fight off infections. However, with advancements in medical technology, there is hope for individuals living with XLA. Cord blood banking, a process of collecting and storing umbilical cord blood, has emerged as a potential solution for individuals with XLA. In this article, we will delve into the details of X-linked agammaglobulinemia, its symptoms, and how cord blood banking can help in the treatment and management of this rare disorder.

Understanding X-linked agammaglobulinemia and its impact.

X-linked agammaglobulinemia (XLA) is a rare genetic disorder that affects the immune system, specifically the production of antibodies. It primarily affects males and is caused by mutations in the BTK gene, which is responsible for the development and maturation of B cells. Without functioning B cells, individuals with XLA have a severely compromised immune system, making them more susceptible to recurrent bacterial infections, particularly in the respiratory tract and gastrointestinal system. These infections can be severe and potentially life-threatening if left untreated. XLA can have a significant impact on the quality of life of affected individuals, requiring frequent medical interventions and specialized care. Understanding the underlying mechanisms and impact of XLA is crucial for proper diagnosis, management, and support for individuals living with this condition.

The role of cord blood banking.

Cord blood banking plays a crucial role in the possible treatment of X-linked agammaglobulinemia (XLA) and other genetic disorders. Cord blood refers to the blood found in the umbilical cord and placenta after a baby is born. It is a rich source of hematopoietic stem cells (HSCs) which have the ability to differentiate into various types of blood cells, including B cells responsible for antibody production. By preserving cord blood through banking, these valuable stem cells can be stored and potentially used in the future for transplantation or cellular therapies. This means that individuals diagnosed with XLA may have the opportunity to receive a transplant of healthy cord blood stem cells, which could potentially restore the function of their immune system and improve their overall health. Cord blood banking offers hope for families affected by XLA and underscores the importance of this practice in advancing medical treatments for genetic disorders.

What is X-linked agammaglobulinemia?

X-linked agammaglobulinemia (XLA) is a rare genetic disorder that primarily affects males. It is characterized by a deficiency in B cells, a type of white blood cell responsible for producing antibodies that play a vital role in fighting infections. Individuals with XLA have an impaired immune system, making them highly susceptible to recurrent and severe bacterial infections. Symptoms typically manifest in early childhood and may include frequent respiratory infections, chronic diarrhea, and poor growth. XLA is caused by mutations in the BTK gene, which is responsible for producing a protein necessary for the development and function of B cells. Early diagnosis and management of XLA are essential to prevent complications and improve the quality of life for affected individuals. Treatment options include regular intravenous immunoglobulin (IVIG) therapy to provide the necessary antibodies and prevent infections. Additionally, advancements in medical research have shown the potential benefits of cord blood banking in the treatment of XLA and other genetic disorders.

Why cord blood banking matters.

Cord blood banking matters because it offers a potential solution for individuals with X-linked agammaglobulinemia (XLA) and other similar genetic disorders. Cord blood, which is obtained from the umbilical cord and placenta after childbirth, contains a rich source of hematopoietic stem cells (HSCs) that can differentiate into various types of blood cells, including B cells. By storing cord blood, these valuable stem cells can be preserved and used for transplantation in the future. For individuals with XLA, cord blood transplantation offers the possibility of restoring a functional immune system by introducing healthy B cells that can produce the necessary antibodies to fight infections. This innovative approach has shown promising results in improving the long-term health outcomes for individuals affected by XLA and other immune deficiencies. Therefore, cord blood banking plays a crucial role in providing a potential lifeline for those with genetic disorders, offering hope for a healthier future.

How cord blood banking works.

Cord blood banking is a process that involves the collection, processing, and storage of umbilical cord blood for future use. The procedure begins immediately after the birth of a baby, where the umbilical cord is clamped and cut. The cord blood is then extracted from the umbilical cord and placenta, which is a painless and non-invasive procedure that poses no risk to the mother or the newborn.Once the cord blood is collected, it undergoes a series of steps to ensure its viability and long-term preservation. The blood is tested for various infections and diseases to ensure its safety for future use. It is then processed to isolate and concentrate the valuable hematopoietic stem cells (HSCs) present in the cord blood. These HSCs have the ability to differentiate into various types of blood cells, including immune cells like B cells.After processing, the cord blood is cryogenically frozen and stored in a specialized facility known as a cord blood bank. This freezing process, known as cryopreservation, allows the cord blood to be stored for an extended period without losing its therapeutic potential. The cord blood bank ensures that the samples are stored in optimal conditions to maintain the viability and functionality of the HSCs.When needed, the stored cord blood can be retrieved for transplantation. The process involves thawing the cord blood and infusing the HSCs into the patient's bloodstream, where they can migrate to the bone marrow and start producing healthy blood cells. This transplantation can offer a potential cure or significant improvement for individuals with genetic disorders like X-linked agammaglobulinemia, as it enables the introduction of healthy immune cells to support their immune system function.In conclusion, cord blood banking is a valuable resource for individuals with genetic disorders like X-linked agammaglobulinemia, providing them with a potential lifeline for improved health outcomes. By collecting, processing, and storing cord blood, the valuable HSCs contained within it can be preserved and used for future therapeutic purposes. This innovative approach offers hope and possibilities for individuals and their families facing genetic immune deficiencies.

Benefits of cord blood banking.

Cord blood banking offers numerous benefits for both individuals and their families. One of the key advantages is the potential to treat a range of medical conditions and diseases. The hematopoietic stem cells found in cord blood have the remarkable ability to regenerate and replace damaged cells in the body, making them a valuable resource for transplantation. They have been used successfully in the treatment of various blood disorders, such as leukemia and lymphoma, as well as immune system disorders like severe combined immunodeficiency (SCID). Additionally, cord blood banking allows for personalized medicine, as the stored cord blood can potentially be used for future treatments tailored to an individual's unique genetic makeup. Furthermore, cord blood banking provides peace of mind for families, knowing that they have a readily available source of potentially life-saving cells should the need arise.

X-linked agammaglobulinemia treatment options.

When it comes to the treatment of X-linked agammaglobulinemia, a primary treatment option is immunoglobulin replacement therapy. This involves regular administration of intravenous or subcutaneous immunoglobulin to compensate for the deficient antibodies in the patient's body. By providing these antibodies, the therapy helps prevent infections and improves overall immune function. Additionally, antibiotics may be prescribed to manage and prevent bacterial infections. It is important for patients with X-linked agammaglobulinemia to receive regular medical check-ups and follow their doctor's guidance to ensure proper management of the condition and minimize potential complications.

The importance of early detection.

Early detection plays a crucial role in the management and treatment of various medical conditions, including X-linked agammaglobulinemia. Detecting the condition early allows for timely intervention and initiation of appropriate treatment options. In the case of X-linked agammaglobulinemia, early detection can help prevent complications associated with recurrent infections and immune deficiencies. Regular medical check-ups, including genetic testing and evaluation of immune function, are essential in identifying the condition early on. By identifying X-linked agammaglobulinemia at an early stage, healthcare professionals can implement strategies such as immunoglobulin replacement therapy and preventive measures to optimize patient outcomes and improve their quality of life. Therefore, emphasizing the importance of early detection becomes crucial in ensuring effective management and timely interventions for individuals with X-linked agammaglobulinemia.

How cord blood can help.

Cord blood, derived from the umbilical cord and placenta after childbirth, contains a rich source of stem cells that can be used for various medical purposes, including the treatment of certain conditions like X-linked agammaglobulinemia. Cord blood banking offers the opportunity to collect and store these valuable stem cells for future use. In the case of X-linked agammaglobulinemia, cord blood stem cells can potentially be utilized for hematopoietic stem cell transplantation. This procedure involves replacing the faulty immune system cells with healthy stem cells from the cord blood, which can then develop into functional immune cells and restore immune function in individuals with X-linked agammaglobulinemia. Cord blood banking provides a potential solution for patients with this condition, offering a viable source of stem cells that can be used in therapeutic interventions and potentially improve their overall health outcomes.

Saving lives with cord blood banking.

Cord blood banking plays a crucial role in potentially saving lives by providing a valuable source of stem cells for medical interventions. The collection and storage of cord blood allow for the preservation of these stem cells, which can be utilized in various treatments and therapies. For individuals with conditions such as leukemia or lymphoma, cord blood stem cells can be used in transplantation procedures to replace damaged or diseased cells with healthy ones, promoting the regeneration of a functioning immune system. Additionally, cord blood banking offers a potential solution for individuals with inherited genetic disorders, as the stored stem cells can be used in gene therapy approaches to correct genetic abnormalities and restore normal cellular function. By harnessing the power of cord blood banking, healthcare professionals and researchers have the opportunity to improve patient outcomes and pave the way for advancements in regenerative medicine.In summary, X-linked agammaglobulinemia is a rare genetic disorder that affects the body's ability to produce antibodies, leaving individuals vulnerable to recurrent infections. Cord blood banking offers a potential solution by providing a source of stem cells that can be used for bone marrow transplantation, potentially curing this condition. While more research is needed, the potential for cord blood banking to improve outcomes for those with X-linked agammaglobulinemia is promising and warrants further consideration. It is important for families to discuss this option with their healthcare providers and make an informed decision regarding cord blood banking for their child's future health.

FAQ

What is X-linked agammaglobulinemia and how does it affect the immune system?X-linked agammaglobulinemia is a genetic disorder that affects the immune system by causing a deficiency in B cells, which are responsible for producing antibodies to fight infections. This results in a weakened immune response and an increased susceptibility to bacterial infections. Patients with X-linked agammaglobulinemia often experience recurrent respiratory tract infections, ear infections, and sinusitis due to the lack of functioning antibodies to combat these pathogens. Treatment typically involves regular infusions of immunoglobulins to provide the necessary antibodies that the body cannot produce on its own.How is X-linked agammaglobulinemia inherited and what are the symptoms associated with this condition?X-linked agammaglobulinemia is inherited in an X-linked recessive manner, meaning the gene mutation is located on the X chromosome. This condition primarily affects males as they have only one X chromosome. Symptoms include recurrent infections, particularly bacterial infections, due to a lack of mature B cells and low levels of antibodies. Patients may experience frequent pneumonia, sinusitis, otitis media, and other respiratory or ear infections. Treatment involves lifelong antibody replacement therapy to manage the immune deficiency and prevent infections. Early diagnosis and management are crucial to improve the quality of life for affected individuals.How can cord blood banking potentially benefit individuals with X-linked agammaglobulinemia?Cord blood banking can benefit individuals with X-linked agammaglobulinemia by providing a source of stem cells for potential future treatment options, such as hematopoietic stem cell transplantation. These stem cells can potentially help restore the immune system function in individuals with this condition, offering a potentially life-saving therapy and improving their quality of life. Additionally, cord blood banking can also benefit other family members who may have similar genetic conditions by providing a source of compatible stem cells for transplantation.What role does cord blood stem cell transplantation play in the treatment of X-linked agammaglobulinemia?Cord blood stem cell transplantation can be a curative treatment for X-linked agammaglobulinemia by providing healthy stem cells that can develop into functioning immune cells, improving the patient's ability to produce antibodies and fight infections. This procedure replaces the defective immune system with a healthy one, potentially offering a long-term solution for individuals with this genetic disorder.What are the potential risks and benefits of utilizing cord blood banking for individuals with X-linked agammaglobulinemia?The potential benefit of utilizing cord blood banking for individuals with X-linked agammaglobulinemia is the potential for hematopoietic stem cell transplantation to replace defective immune cells. However, there are risks associated with the procedure such as graft failure, infections, and graft-versus-host disease. It is important for individuals with X-linked agammaglobulinemia to weigh the potential benefits against these risks and consult with healthcare professionals to make an informed decision.  Read the full article

[alfredo-zauce]

I’m willing to bet fangs-for-the-venom had agammaglobulinemia while alive

🍷 nvrdrnk-wine Follow

so does anyone even like AB blood or…?

🕷 blood-is-the-strife Follow

i’ll drink it if there’s nothing else but it kinda blows

🦇 battybrained Follow

guys there is literally no discernible difference between blood types. blood is blood its all the same

🍷 nvrdrnk-wine Follow

you don’t even know how wrong you are

🥀 sanguine-dreaming Follow

Umm. I like AB? Maybe it has something to do with me being type AB before dying but it’s my favourite.

⚰️ draculas-left-cainine Follow

omg. OMG. GUYS. WHAT IF YOUR FAVORITE BLOOD TYPE IS JUST WHAT WOULDVE BEEN COMPATIBLE WITH YOUR BLOOD TYPE BEFORE VAMPIRIFICATION?????

THATS WHY WE ALL LOVE O NEGATIVE. IT ALL MAKES SENSE!!!!

☠️ crypt-ic Follow

HOLY SHIT

🧛🏻‍♂️ fangs-for-the-venom Follow

hate to ruin your theory but i don’t like o neg

🍷 nvrdrnk-wine Follow

what the fuck is wrong with you

[helthcareheven]

VitaImmuno pH4: Intravenous Immunoglobulin Potency

Introduction Human immunoglobulin, also known as intravenous immunoglobulin or IVIG, is a therapeutic preparation of pooled human immunoglobulins obtained from the plasma of healthy donors. It contains a balanced mixture of immunoglobulin G antibodies against various infectious agents. IVIG therapy aims to replace the missing antibodies in patients with primary or secondary immunodeficiencies. This article discusses the composition, mechanism of action, indications and administration of pH4-adjusted IVIG.

Composition IVIG contains mainly immunoglobulin G (IgG) antibodies extracted from donated human plasma by fractionation processes. It consists of over 95% intact IgG molecules with traces of IgA and IgM. The immunoglobulins in IVIG have specific pathogen-directed antibodies against bacteria and viruses that infect humans. The IgG antibodies are polyclonal, which means they contain a wide variety of antibodies that react against various microorganisms. The pH4-adjusted formulation of IVIG has a neutral pH between 4-5 to reduce the risk of acute kidney injury.

Mechanism of Action Human Immunoglobulin (pH4) for Intravenous Injection works through multiple mechanisms to boost the immune system. It replaces the missing antibodies in immunodeficient patients by providing passive immunization against pathogens. The exogenous IgG antibodies bind to Fc receptors on phagocytes, B cells, and other immune cells to facilitate opsonization and elimination of infectious agents. IVIG also modulates the immune system by inhibiting pro-inflammatory cytokines, complement activation, and Fc receptor-mediated phagocytosis. These immunomodulatory effects help control autoimmune and inflammatory conditions.

Indications IVIG therapy is recommended for treatment and prevention of infections in patients with primary immunodeficiencies like common variable immune deficiency, X-linked agammaglobulinemia and hypogammaglobulinemia. It is also a first-line treatment for certain autoimmune diseases like Kawasaki disease, Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy. IVIG reduces symptoms and relapses in these conditions by dampening pathological autoantibody production and cytotoxic immune responses.

Administration IVIG is administered intravenously through a peripheral or central line at regular intervals. The dosage varies depending on the indication and ranges between 300-600 mg/kg given every 3-4 weeks or every 2-6 months for replacement therapy in primary immunodeficiencies. Frequent dosing is required initially for treatment of autoimmune and inflammatory conditions. IVIG is usually well-tolerated but some common adverse effects include headache, nausea, fever, chills and abdominal pain which can be managed symptomatically. Rare complications include thromboembolic events, aseptic meningitis and acute kidney injury. Careful infusion with hydration and dose monitoring helps minimize risks.

Mechanism of Action As described earlier, IVIG exerts its immunomodulatory and anti-inflammatory effects through multiple mechanisms. A major mechanism involves modulation of the Fc portion of IgG molecules. IVIG engages inhibitory Fcγ receptors (FcγRIIB) on immune cells, decreasing their activation threshold. This suppresses pro-inflammatory cytokine release and phagocytosis. IVIG also competes for binding to activating Fcγ receptors, further inhibiting immune cell activation. At the same time, exogenous IgG provides opsonization of pathogens via activating FcγRs to enhance elimination.

Additionally, IVIG binds specifically to certain cytokines and complement factors to inhibit their activity. It neutralizes interleukin (IL)-1, IL-6, tumor necrosis factor (TNF)-α and interferon (IFN)-γ which play a key role in chronic inflammation. IVIG also downregulates expression of adhesion molecules and secretion of chemokines involved in immune cell trafficking to sites of inflammation. By blocking complement activation at the C3 convertase level, IVIG prevents membrane attack complex formation and tissue damage in autoimmune diseases. These diverse suppressive mechanisms help control excessive immune responses and inflammation in various conditions treated with IVIG.

Efficacy and Clinical Trials The efficacy and safety of IVIG has been validated through extensive clinical research over the past few decades. In primary immunodeficiencies, long-term IVIG therapy greatly improves survival by preventing life-threatening bacterial infections. For Kawasaki disease, a randomized controlled trial showed IVIG plus aspirin led to a significantly lower coronary artery aneurysm rate compared to aspirin alone.

Some notable clinical trials in other conditions include a trial demonstrating that IVIG prevented relapse in Guillain-Barré syndrome; a large trial confirming clinical improvement and reduced relapses with IVIG versus placebo in chronic inflammatory demyelinating polyneuropathy; and trials establishing efficacy of IVIG in idiopathic thrombocytopenic purpura and multifocal motor neuropathy. Ongoing research continues to explore new potential uses of Human Immunoglobulin (pH4) for Intravenous Injection in various autoimmune and inflammatory diseases.

[omkarpatel]

Human Immunoglobulin (pH4) for Intravenous Injection is Estimated to Witness High Growth Owing to Rising Prevalence of Neurological and Immunodeficiency Diseases

Human immunoglobulin (pH4) is a blood plasma derived product administered intravenously for treatment of various neurological and immunodeficiency diseases. It contains antibodies which help strengthen the body's natural defenses against infectious agents. The rising incidences of immune deficiency disorders like agammaglobulinemia and common variable immunodeficiency have inflated the demand for immunoglobulin therapy. The global Human Immunoglobulin (pH4) for Intravenous Injection Market is estimated to be valued at US$ 17.5 billion in 2023 and is expected to exhibit a CAGR of 16% over the forecast period 2023-2031, as highlighted in a new report published by Coherent Market Insights. Market Opportunity: Rising prevalence of neurological and immunodeficiency diseases is estimated to witness high growth. Neurological disorders pose a significant healthcare burden globally and afflict millions worldwide each year. conditions like Guillain-Barre syndrome and chronic inflammatory demyelinating polyneuropathy require intravenous immunoglobulin as a mode of treatment. According to the World Health Organization, over 1 billion people suffer from some form of neurological disorder. The annual cost of neurological conditions in the U.S. alone is estimated to be over $800 billion. Thus, the growing cases of neurological diseases will spur demand for immunoglobulin therapy, acting as a high impact rendering driver for the market.

Porter’s Analysis Threat of new entrants: Low barrier to entry due to availability of raw material and manufacturing process knowledge. However, high capital requirements for production facilities and strong brand loyalty towards existing players limits threat. Bargaining power of buyers: Moderate bargaining power due to presence of substitutes. However, specialized production process results in inelastic demand. Bargaining power of suppliers: High dependence on plasma suppliers limits bargaining power. Suppliers can charge higher prices or refuse supply during shortages. Threat of new substitutes: Low threat as IgG products have no close substitute for treatment of certain immune disorders and deficiencies. Substitution requires regulatory approval and clinical trials. Competitive rivalry: Intense competition between major players. Companies compete based on product quality, reliability of supply, price and brand reputation. SWOT Analysis Strength: Established production facilities and regulatory approvals. Diversified product portfolio to treat range of conditions. Weakness: High capital intensity of production and vulnerability to plasma supply disruptions. Stringent regulatory norms increase compliance costs. Opportunity: Expanding patient population and increased diagnosis rate in developing regions. New clinical indications can boost revenue streams. Threats: Price controls and government reimbursement policies. Stringent import and export regulations across countries. Key Takeaways The global Human Immunoglobulin (pH4) for Intravenous Injection market is expected to witness high growth. The market is dominated by North America owing to high prevalence of immunodeficiency and autoimmune disorders. Rising healthcare spending and presence of major players in the US and Canada will drive the region's market share. Europe is the second largest regional market led by countries such as Germany, UK and France. Higher acceptance of IgG therapies and supportive national health strategies supplement the regional growth. Key players operating in the Human Immunoglobulin (pH4) for Intravenous Injection market are CSL Behring, Grifols, Baxter, Octapharma, Kedrion, CNBG, Hualan Bio and BPL. These players acquire plasma from approved donors and fractionate to produce standard and specialized IgG formulations.

[12nb34]

📆 Jan 2023 📰 Exposed seronegative: Cellular immune responses to SARS-CoV-2 in the absence of seroconversion 🗞 Frontiers

Determining which antigens are targeted in SARS-CoV-2 ESNs provides insight into mechanisms of response. T-cells targeting the replication-transcription complex (RTC) of SARS-CoV-2 were described by Swadling et al. (2022) in ESNs (7). The RTC is comprised of the RNA polymerase NSP12, a co-factor NSP7, and the helicase NSP13 (37). Its expression early in the SARS-CoV-2 replication cycle makes the RTC a target for rapidly-induced T-cell responses (7). The authors identified fivefold-higher RTC-specific T-cell responses in ESNs compared to unexposed controls. Furthermore, cellular immunity in ESNs preferentially targeted the RTC over structural proteins compared to seropositive individuals. However, the authors did not assay cellular responses to other NSPs.

In a study of six ESN sexual partners of HSV-2-infected individuals by Posavad et al. (2010), T cell responses in ESNs were skewed towards peptides expressed early in the virus replication cycle, whereas HSV-2 seropositive individuals more frequently generated responses to structural proteins present in virions. The authors speculated that this skew in ESNs reflected early T-cell engagement with infected cells before the production of infectious virions. Together, these data support a model whereby rapid T-cell responses targeting early translated NSPs may prevent infection from gaining a foothold.

To prevent infection before seroconversion, a rapid cellular response appears critical. Chandran et al. (2021) assayed weekly nasopharyngeal swabs and blood samples from HCWs, and demonstrated that SARS-CoV-2 specific T-cell proliferation can occur before PCR positivity (42). These rapid responses may originate from pre-existing, cross-reactive T-cells specific for human coronaviruses (HCoVs). Cross-recognition of SARS-CoV-2 by HCoV-specific T-cells has been widely described (43–50), and T-cells from COVID-19 convalescents preferentially target conserved epitopes over SARS-CoV-2-specific epitopes (49). HCWs display higher levels of HCoV-specific T-cells than community controls (28), which may contribute to the abundance of ESNs amongst HCWs. The activation of cross-reactive T-cells by related viruses has been termed ‘heterologous immunity’ (51). This is distinct from autologous viral infection in that neutralising antibody responses to the heterologous virus may be suboptimal, allowing cellular memory to dominate.

The RTC is highly conserved between SARS-CoV-2 and HCoVs (7). Tetramer staining of T-cells with an HCoV-HKU1 homologue of the RTC component NSP7 showed strong responses in SARS-CoV-2 ESNs. Swadling et al. (2022) suggested that prior exposure to HCoV-HKU1 generates cross-reactive T-cells specific for NSP7, enabling rapid abortion of SARS-CoV-2 infection (7). A study of camel workers in Saudi Arabia identified both CD4+ and CD8+ responses to Middle-East Respiratory Syndrome coronavirus in four highly-exposed seronegative individuals, suggesting that the ESN phenomenon may be common to other human-infective coronaviruses.

Cellular immunity is able to clear SARS-CoV-2 infection in isolation; patients with X-linked agammaglobulinemia who cannot produce antibodies eventually clear SARS-CoV-2 infection, and mount higher magnitude CD8+ T-cell responses to SARS-CoV-2 compared to immunocompetent individuals (54). However, in Wang et al. (2021) the magnitude of the SARS-CoV-2-specific CD4+ T-cell response was twice as high in infected individuals compared to ESNs. This casts doubt on their role in protection against infection.

[sbgridconsortium]

A look into specific interactions between Bruton’s Tyrosine Kinase (BTK) and its inhibitors

Note: This publication highlight is part of the SBGrid/Meharry Medical College Communities Project, focused on science education and demonstrating how structural biology and preclinical science connect to medicine.

Non-receptor tyrosine kinases (nRTK) are a subgroup of tyrosine kinases that are responsible for the phosphorylation of proteins. As the name suggests, tyrosine kinases work by transferring a phosphate group from ATP to the tyrosine residue of a protein. Non-receptor refers to the group of tyrosine kinases found within the cytosol of the cell, unlike receptor kinases, which are embedded into the cellular membrane. nRTKs are involved in many cell functions such as regulating cell growth and proliferation. They also play critical roles in immune system regulation. One specific nRTK that is involved in propagating signals from B cell receptors is known as Bruton’s tyrosine kinase (BTK). BTK’s importance was discovered when it was revealed that mutations in the Btk gene, the gene that encodes BTK, leads to the development of X-linked agammaglobulinemia (XLA), an immunodeficiency disease. BTK also plays essential roles in many diseases such as mantle cell lymphoma, chronic lymphocytic leukemia, Waldenstrom macroglobulinemia, small lymphocytic lymphoma, marginal zone lymphoma, and chronic graft-versus-host disease, just to name a few. These factors combined have made BTK inhibition a target of several drug therapies aimed at treating B cell malignancies. These therapies include the first in class BTK inhibitor, Ibrutinib, and the second-generation inhibitors Acalabrutinib, Zanubrutinib and Orelabrutinib. Although all of these therapeutics have seen success in clinical applications, specific interactions between the drugs and BTK are not well understood. 

Above: Front and back views of BTK/Acalabrutinib complex (PDB: 8FD9)  CC BY SBGRID.

In this work, SBGrid member Amy Andreotti and colleague David Lin reported the first structure of BTK in complex with Acalabrutinib. They also report a structure of BTK with Tirabrutinib, another second-gen BTK inhibitor that, at the time of this publication, is in clinical use in Japan and Taiwan but not yet FDA approved. When comparing their BTK/Acalabrutinib complex structure with a previously reported structure of BTK/Ibrutinib complex, the authors noted several regions where structural differences occur based on evaluation of RMSD values. Comparisons between BTK/Acalabrutinib and BTK/Tirabrutinib reveal large conformational differences in the activation loop that the authors attribute to the kinase undergoing dynamic fluctuations when bound to the drug, after further investigation of the previously reported structure of BTK/Tirabrutinib complex. Along with broad structure characterization, the authors also identified a few key residues that interact with the inhibitors. Combined, their work shows the need for further probing into how these drugs interact with BTK in order to fully examine how these inhibitors bind. 

Read more in PLOS One 

- KeAndreya Morrison, Meharry Medical College

[moleculardepot]

Phospho-Btk (Tyr223) Antibody APC Conjugate

Phospho-Btk (Tyr223) Antibody APC Conjugate Catalog number: B2018597 Lot number: Batch Dependent Expiration Date: Batch dependent Amount: 10 ug Molecular Weight or Concentration: 76.28 kDa Supplied as: Solution Applications: a molecular tool for various biochemical applications Storage: 2-8℃ Keywords: Bruton tyrosine kinase, Tyrosine-protein kinase BTK, Agammaglobulinemia tyrosine kinase, ATK,…

[tumimmtxpapers]

SARS-CoV-2 spike antibody concentration in gamma globulin products from high-prevalence COVID-19 countries are transmitted to X-linked agammaglobulinemia patients

CONCLUSION: As the prevalence of COVID-19 infections rises, detection of SARS-CoV-2 spike IgG in commercial IVIG products increases and is then transmitted to the patient. Future studies are needed to investigate the neutralizing capabilities of SARS-CoV-2 IgG and whether titer levels in IVIG remain consistent as the incidence of infection and vaccination rates in the population changes. http://dlvr.it/Smgbmt

[worldwidewi]

Tips to boost the immune system to medical issues for world travelers

These days every individual loves to roam all across the world extensively, and increasingly so. Approximately, 20 and 70% of the 50 million people from the industrialized world visiting the emerging world report illness associated with their travel. Moreover, many illnesses are mild, 1 to 5% of returned travelers become ill enough to seek medical attention, and 1 in 100, 000 succumb to a travel‐related disease (TRD).

Those travelers who already have underlying medical conditions, and diseases attained during travel may lead to more severe significance compared to healthy travelers. 2–5 also, contingent on the essential condition there may be diminished immunogenicity and clinical efficacy of vaccinations. Live conical vaccines, such as that for yellow fever, may produce sickness. On the other hand, medical issues for world travelers are the biggest issues and that hampers the roam moments.

The immune deficiencies that inspiration travel can be separated into several groups:                        

•             Humoral immune deficiency with primary or secondary hypo‐ or agammaglobulinemia, eg, due to the use of rituximab, multiple myeloma, chronic lymphatic leukemia, or nephrotic syndrome; the biggest medical issues for world travelers.

•             Cellular immune deficiency, eg, due to HIV contagion or immune‐suppressive therapy;

•             General immune commotion due to defective barriers such as skin or mucosal disorders, or a condensed gastrointestinal acid barrier;

•             Other conditions that reason for a higher risk of infection for example diabetes, malignancies, pregnancy, functional as plena/splenectomy, cardiovascular prostheses, hematologic stem cell transplantations, accompaniment disorders, and older age (>60 years).

As the region changes the immune system is intertwined, and immune deficiency is often of a combined type.6

Literature and many endorsements exist on the HIV‐infected medical issues for world travelers in whom the degree of immune compromise can be enumerated by measuring CD4+ lymphocytes.4,7,8 Little evidence and fewer endorsements are obtainable with respect to transplant patients, and even less with respect to other forms of immune suppression. Moreover, no well‐validated laboratory measures are accessible that quantify the degree of immune suppression in these patients.

This analysis focuses on travel‐related health risks for dissimilar groups of travelers with original medical conditions who visited our website to know more about the medical issues for world travelers.

[mcatmemoranda]

[aw-hawkeye-no]

Almost all of pre-serum!Steve’s ailments can be explained by one underlying condition and I have RECEIPTS

So from CA:TFA and and CA:TWS, we can determine that pre-serum!Steve had, at least, the following issues:

Asthma 

Scarlet fever

Rheumatic fever

Heart arrhythmias, heart “trouble”, 

Chronic fatigue

Weight loss, poor growth

Partial deafness

Stomach ulcers

Pernicious anemia

Recurrent sinusitis, bronchitis, and pneumonia

Scoliosis, fallen arches

Poor eyesight

“Nervous trouble of any sort”

Now just from this list, it looks like Steve had just a myriad of issues and should never have been allowed out of the house (which is probably true). But in medicine you typically don’t like to give patients more problems than they have -- you try to explain as many symptoms as you can with one condition. 

Last night I fell into a research hole, and I think I can explain (almost) all of Steve’s woes with a single underlying primary immunodeficiency. The two most likely candidates would be Common Variable Immunodeficiency (CVID) or X-Linked Agammaglobulinemia (XLA). There’s a lot of overlap between these conditions, but I think that CVID is more likely in Steve’s case because inflammatory conditions and autoimmune disease are a common component. 

CVID is a primary humoral immunodeficiency defined by a decrease in antibodies (specifically IgG, although IgA and IgM concentrations are decreased as well). Decreased antibodies predisposes you to recurrent bacterial (and occasionally viral) infections. Patients diagnosed with CVID also frequently have concurrent autoimmune diseases or inflammatory conditions. 

So, how does CVID explain the poor sick waif that was pre-serum Steve Rogers?

Recurrent bacterial infections 

Recurrent otitits media/interna (ear infections) can physically damage the ear drum, inner ear bones, or even the vestibulocochlear nerve, leading to permanent deafness.  

Recurrent sinusitis, bronchitis, and pneumonia are extremely common in CVID patients. Respiratory infections are actually one of the hallmarks of the disease.

Recurrent bronchitis can lead to bronchiectasis, where the walls of the bronchi/bronchioles (lower airways) are thickened from inflammation. This is present in up to 76% of CVID patients. Bronchiectasis is a form of chronic obstructive pulmonary disease, and symptoms are very similar to asthma (wheezing, difficulty breathing, and coughing).

Predisposition to streptococcal infections. Both scarlet and rheumatic fever are complications of strep infections that lead to disseminated disease that can affect heart, joints, skin, and brain. 

Recurrent gastrointestinal infections, including Helicobacter pylori. Helicobacter infections were found in 49% of CVID patients with GI upset, and were more likely to be symptomatic than patients without an immunodeficiency. H. pylori is known to be associated with gastric ulcers. Chronic infection can also lead to atrophic gastritis (found in 79% of CVID patients with H. pylori), which is important since atrophic gastritis causes...

Pernicious anemia

Pernicious anemia is caused by a deficiency in Vitamin B12. Atrophic gastritis, defined as chronic inflammation of the gastric mucosa and replacement of parietal cells with fibrous tissue, leads to decreased absorption of dietary Vitamin B12 and is a common cause of pernicious anemia. 

Atrophic gastritis is a complication of chronic H. pylori infection. However, it can also be a primary autoimmune disease, and has been seen in CVID patients. 

Symptoms of pernicious anemia include: 

Cardiac issues including arrhythmias, murmurs, angina, and eventually congestive heart failure. 

Chronic fatigue and exercise intolerance

Irritability (I know this isn’t listed as one of Steve’s pre-serum conditions, but c’mon, he was such a little shit)

Neurologic signs that include tremors, cold hands and feet, peripheral nerve damage that can lead to deafness and blindness

CVID, like other primary immunodeficiencies, is actually associated with other primary autoimmune and inflammatory diseases. The working theory is that when one part of the immune system is depressed, other portions get ramped up. Pernicious anemia and chronic fatigue could be components of a primary autoimmune disease. There have also been several cases of CVID patients having allergic asthma, so it is possible that Steve did have asthma (although I like the theory that he had chronic obstructive pulmonary disease that was misdiagnosed as asthma).

But Michelle, you may say, if Steve had such a severe immunodeficiency why didn’t he die of tuberculosis like his mother? Well, my friend, patients with CVID and XLA are actually not more likely to contract TB than the general population. This is because CVID and XLA are problems with B-cells and decreased antibodies, while the most important component of fighting mycobacterial infections are T-cells and secretory proteins called interferons. It is possible that Steve was latently infected with TB, and may have become symptomatic later in life when another illness put his entire immune system under stress, but it is also possible that he was able to avoid infection to begin with. 

TL;DR, Steve Rogers had common variable immunodeficiency that led to recurrent infections and a boatload of problems. This has been an egregiously long post from your local medicine nerd. 

*Edit/Disclaimer: I’m not a human medical doctor, I’m a veterinarian. All of this info comes from peer-reviewed journal articles, and I am really proud of the case I put together here, but please take it all with a grain of salt if applying it to your own medical issues/history.