Tyrosinemia with acute intermittent porphyria: Aminolevulinic acid dehydratase deficiency related to elevated urinary aminolevulinic acid levels

C. Frederic Strife, Edward L. Zuroweste, Edward A. Emmett, Vincent N. Finelli, H. G. Petering, Helen K. Berry

Research output: Contribution to journalArticlepeer-review

Abstract

A patient who had hereditary tyrosinemia was observed during two illnesses to have characteristics ofacute intermittent porphyria with associated hypertension. Metabolic studies revealed elevated levels of urinary aminolevulinic acid but normal levels of porphyrin metabolites associated with, and possibly explained by, decreased red blood cell activity of the zinc-dependent enzyme, aminolevulinic acid dehydratase. Zinc deficiency could not be directly associated with the diminished enzyme activity. The patient's hypertension appeared to be related to increased urinary excretion of catecholamines and to elevated renin activity in peripheral venous blood.

Original languageEnglish (US)
Pages (from-to)400-404
Number of pages5
JournalThe Journal of pediatrics
Volume90
Issue number3
DOIs
StatePublished - Mar 1977

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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