ALA Dehydratase Deficiency Porphyria Medication
- Author: Smeeta Sinha, MD; Chief Editor: Emmanuel C Besa, MD more...
Specific treatments for acute attacks of ALAD deficiency porphyria (ADP) include heme replacement with intravenous hematin and carbohydrate loading with intravenous dextrose.[11, 12] Hematin, the ferric hydroxylated form of heme, should be administered within 24 hours for severe ADP attacks, or for mild symptoms that fail to improve after 24 hours.
Metalloporphyrin agents reduce morbidity and prevent complications: Attacks of porphyria may progress to irreversible neuronal damage. Hemin therapy is used to prevent an attack from causing neuronal degeneration; it is not effective in repairing neuronal damage.
Previously known as hematin (hematin is a term used to describe chemical reaction products of hemin and sodium carbonate solution). Enzyme inhibitor derived from processed RBCs and an iron-containing metalloporphyrin. Decreases production of ALA synthetase, thus decreasing ALA levels. DOC for acute attacks of ALAD deficiency porphyria (ADP).
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