Hematin in Acute Porphyria Neuropathy

Abstract

Porphyria is a hereditary or acquired metabolic disease in which 'haem' synthesis is impaired and increased amounts of porphyrin and porphyrin precursors. Acute intermittent porphyria (AIP) is a rare disease that sometimes occurs with severe clinical symptoms and causes neurophsyciatric, neuromusculer, autonomic disfunction because of porphobilinogen and delta-aminolevulinic aciduria accumulation due to porphobilinogen deaminaz deficiensy. AIP should not be forgotten while making differential diagnosis of polyneuropathy. In this article, a case is presented who had acute symptoms and developed respiratuary failure in a few hours and was died because of pulmoner hemorrhage during hematin treatment.