Literature DB >> 839332

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

C F Strife, E L Zuroweste, E A Emmett, V N Finelli, H G Petering, H K Berry.   

Abstract

A patient who had hereditary tyrosinemia was observed during two illnesses to have characteristics of acute 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.

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Year:  1977        PMID： 839332     DOI： 10.1016/s0022-3476(77)80701-4

Source DB:  PubMed          Journal:  J Pediatr        ISSN： 0022-3476            Impact factor:   4.406


  6 in total

1.  Type I hereditary tyrosinaemia: presentation of 11 cases.

Authors:  T Coşkun; I Ozalp; N Koçak; A Yüce; M Caglar; R Berger
Journal:  J Inherit Metab Dis       Date:  1991       Impact factor: 4.982

2.  Homotransplantation of the liver in a patient with hepatoma and hereditary tyrosinemia.

Authors:  R O Fisch; E R McCabe; D Doeden; L J Koep; J G Kohlhoff; A Silverman; T E Starzl
Journal:  J Pediatr       Date:  1978-10       Impact factor: 4.406

3.  Hereditary tyrosinemia and the heme biosynthetic pathway. Profound inhibition of delta-aminolevulinic acid dehydratase activity by succinylacetone.

Authors:  S Sassa; A Kappas
Journal:  J Clin Invest       Date:  1983-03       Impact factor: 14.808

4.  Hereditary tyrosinaemia type I: a long-term study of the relationship between the urinary excretions of succinylacetone and delta-aminolevulinic acid.

Authors:  H Schierbeek; G J Beukeveld; H van Faassen; F J van Spronsen; K Bijsterveld; E E Venekamp-Hoolsema; B G Wolthers; G P Smit
Journal:  J Inherit Metab Dis       Date:  1993       Impact factor: 4.982

5.  Peripheral neuropathy as the presenting feature of tyrosinaemia type I and effectively treated with an inhibitor of 4-hydroxyphenylpyruvate dioxygenase.

Authors:  T C Gibbs; J Payan; E M Brett; S Lindstedt; E Holme; P T Clayton
Journal:  J Neurol Neurosurg Psychiatry       Date:  1993-10       Impact factor: 10.154

6.  New type of hepatic porphyria with porphobilinogen synthase defect and intermittent acute clinical manifestation.

Authors:  M Doss; R von Tiepermann; J Schneider; H Schmid
Journal:  Klin Wochenschr       Date:  1979-10-15
  6 in total

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