-
DE
Haemin Complex – Mechanism and Use in Porphyria
The haemin complex is an iron-containing compound used in medicine to treat certain metabolic disorders. It works by inhibiting haem biosynthesis.
Add as Preferred SourceThings worth knowing about "Haemin Complex"
The haemin complex is an iron-containing compound used in medicine to treat certain metabolic disorders. It works by inhibiting haem biosynthesis.
What is the Haemin Complex?
The haemin complex is a chemical compound consisting of haemin – the oxidised iron-containing component of haemoglobin – bound to a carrier molecule. In its most common medical form, it is presented as a haem arginate complex, marketed under the trade name Normosang. This active substance is administered intravenously and belongs to the group of agents used in the treatment of porphyrias.
Mechanism of Action
The haemin complex acts through negative feedback inhibition of the enzyme ALA synthase (delta-aminolaevulinic acid synthase), which catalyses the first and rate-limiting step in haem biosynthesis. By inhibiting this enzyme, the excessive production of toxic haem precursors such as delta-aminolaevulinic acid (ALA) and porphobilinogen (PBG) is reduced. These precursors accumulate in the body during certain metabolic disorders and cause severe symptoms.
Medical Use
The haemin complex is primarily used to treat acute episodes of acute hepatic porphyrias (AHP), including:
- Acute intermittent porphyria (AIP) – the most common form
- Hereditary coproporphyria (HCP)
- Variegate porphyria (VP)
- ALA dehydratase deficiency porphyria (ALADP)
These are rare, mostly inherited metabolic disorders in which haem biosynthesis is impaired, leading to acute and potentially life-threatening attacks.
Symptoms of an Acute Porphyria Attack
The symptoms that the haemin complex is specifically used to address include:
- Severe, cramping abdominal pain
- Nausea and vomiting
- Neurological symptoms (e.g., muscle weakness, paralysis)
- Psychiatric symptoms (e.g., confusion, hallucinations)
- Cardiac arrhythmias and high blood pressure
Dosage and Administration
The haemin complex is administered exclusively intravenously (i.v.) in a clinical setting. The standard dosage is 3–4 mg per kilogram of body weight per day, typically over a course of 4 days. The exact dosage is determined individually by the treating medical professional.
Side Effects and Safety Information
Like all medications, the haemin complex can cause side effects. The most common include:
- Reactions at the injection site (e.g., vein inflammation, thrombophlebitis)
- Coagulation disturbances (transient prolongation of prothrombin time)
- Iron accumulation with repeated use
- Rarely: allergic reactions
Due to these potential side effects, careful medical supervision during treatment is essential.
Clinical Significance
The haemin complex is considered the standard therapy for severe acute porphyria attacks when general measures such as carbohydrate loading (glucose infusion) are not sufficiently effective. It can significantly shorten the duration and severity of an attack and prevent life-threatening complications.
References
- European Medicines Agency (EMA): Normosang – Summary of Product Characteristics. EMA, 2023.
- Puy H, Gouya L, Deybach JC: Porphyrias. Lancet. 2010;375(9718):924-937. DOI: 10.1016/S0140-6736(09)61925-5.
- Bonkovsky HL et al.: Acute Porphyrias in the USA: Features of 108 Subjects from Porphyrias Consortium. Am J Med. 2014;127(12):1233-1241. DOI: 10.1016/j.amjmed.2014.06.036.
Building Blocks for a Healthy Life
As an integrated manufacturer, we develop & produce evidence-based, patented, and non-patented formulations. We work exclusively with plant and natural extracts according to the strictest purity & quality standards.
The combination of science & modern technology with the laws of nature creates solutions consistently tailored to humans – for the highest good: health.
Skip product gallery
Subscription available
Floral
For Healthy Oral Flora & Dental Care
Formulated lozenges with Dentalac®, lactic acid bacteria, and Lactoferrin CLN®30 lozenges
Regular price:
22,90 €
Discount
Subscription available
Lactoferrin 60 Capsules
For your universal protection
As one of the most valuable proteins in the body, lactoferrin is a natural component of the immune system.
60 Capsules
Sale price:
50,00 €
Regular price:
59,90 €
Discount
Subscription available
Multivitamin Mineral Complex
Daily basic supply: the complete spectrum of micronutrients
Natural multivitamin complex for the immune system, energy, and central body functions
30 Capsules
Sale price:
27,90 €
Regular price:
29,90 €
Subscription available
Natural Iron
For your iron balance
Specially formulated for your iron balance with plant-based curry leaf iron, Lactoferrin CLN®, and natural Vitamin C from rose hips.
30 Capsules
Regular price:
35,90 €
Most read entries
3 Posts in this encyclopedia categoryMagnesiumcarbonat
Magnesiumcarbonat ist eine anorganische Magnesiumverbindung mit säure-neutralisierender (antazidischer) Wirkung. Erfahre mehr über Vorteile, Einsatzgebiete und Unterschiede zu besser resorbierbaren Magnesiumformen.
Calorie content
The choice of food influences our energy intake. The calorie content varies greatly - some provide little energy, others a lot.
Cologne list
The Cologne List ® is an important initiative in competitive sports that offers athletes guidance in the selection of nutritional supplements.