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The Role of Human Hemin (Normosang) in Managing Acute Hepatic Porphyria

Acute hepatic porphyria refers to a group of rare metabolic disorders characterized by deficiencies in enzymes involved in the biosynthesis of heme, a component of hemoglobin. These deficiencies lead to the accumulation of porphyrins and their precursors, causing symptoms such as severe abdominal pain, nausea, vomiting, and neurological manifestations.

Understanding Acute Hepatic Porphyria: AHP encompasses four main types: acute intermittent porphyria (AIP), variegate porphyria (VP), hereditary coproporphyria (HCP), and ALA dehydratase deficiency porphyria (ADP). These disorders manifest primarily through neurovisceral symptoms such as severe abdominal pain, nausea, vomiting, and neurological manifestations like seizures and neuropathy. Cutaneous symptoms may also occur, ranging from photosensitivity to blistering skin lesions.

The cornerstone of managing AHP involves minimizing triggers such as certain medicines, hormonal changes, and dietary factors, coupled with symptomatic relief during acute attacks. Historically, therapies like intravenous glucose, hemin, and pain management have been employed, but they often fall short in providing long-term solutions and may have limitations in certain patient populations.

Human Hemin: A Game-Changer in AHP Treatment: Human hemin (supplied under the brand Normosang), a novel therapeutic option, has emerged as a promising breakthrough in AHP management. It is a formulation of heme arginate, a stable heme molecule that directly addresses the enzymatic defects underlying AHP. By providing exogenous heme, Human hemin replenishes the deficient enzyme, thereby restoring heme biosynthesis and mitigating the accumulation of toxic porphyrin intermediates.

The efficacy of Human hemin has been demonstrated in clinical trials, with notable reductions in the frequency and severity of acute attacks among AHP patients. Furthermore, its convenient subcutaneous administration and favourable safety profile make it an attractive option for both healthcare providers and patients.

In addition to Human Hemin, other treatments for acute porphyria attacks may include medications to manage symptoms such as pain and nausea, as well as measures to prevent future attacks, such as avoiding triggers like certain medications, alcohol, and fasting.