Abstract
Porphyria-like symptoms are common in hereditary tyrosinemia (HT). This is due to inhibition of δ-aminolevulinate (ALA) dehydratase (D) by succinylacetone (SA). We present our experience with hemiarginate (HA) for porphyria crises in two pts with HT. Both showed low activity of erythrocyte ALA-D and sustained high urinary excretion of ALA and SA. The symptoms started at 18 mo (Pt 1) and 3 yrs (Pt 2) with irritability, drowsiness, vomiting, hypertension, and peripheral neuropathy. ALA excretion increased up to 15 times the upper normal limit. When intravenous HA was given (for 4 d), the pts became alert within the first day, painless and normotensive within 3 d, and the neuropathy vanished within 2 wks. Urinary ALA excretion decreased within 24 h. In all, 10 relapses occurred and were treated with HA. In Pt 1 prophylactic HA twice a wk for up to 9 mo prevented recurrences. No side effects of HA were noted. Conclusion: HA is a safe and effective therapy for porphyria crises of HT.
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