Literature DB >> 1358034

Incidence of hereditary porphyria cutanea tarda (PCT) in a sample of the Italian population.

L D'Alessandro1, D Griso, G Biolcati, A Macrì, G C Topi.   

Abstract

The determination of the enzymatic activity of URO-D in erythrocytes is the screening method used for differentiation between hereditary and non-hereditary forms of porphyria cutanea tarda (PCT). The aim of the present work was to establish the relative frequencies of the symptomatic and hereditary forms by the determination of the URO-D enzyme in the PCT patients who were regularly treated at the Centre for Porphyrins in our Institute. In the course of this work we also examined the statistical properties of the distributions of both normal and porphyric subjects, so as to be able to suggest values for discriminating between normal subjects and the various types of porphyric subjects.

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Year:  1992        PMID: 1358034     DOI: 10.1007/bf00375795

Source DB:  PubMed          Journal:  Arch Dermatol Res        ISSN: 0340-3696            Impact factor:   3.017


  8 in total

1.  Erythrocyte metabolism. VI. Separation of erythrocyte enzymes from hemoglobin.

Authors:  M A HENNESSEY; A M WALTERSDORPH; F M HUENNEKENS; B W GABRIO
Journal:  J Clin Invest       Date:  1962-06       Impact factor: 14.808

2.  Porphyria cutanea tarda: erythrocyte uroporphyrinogen decarboxylase activity in 471 consecutive patients.

Authors:  M O Doss; M Frank; O Braun-Falco
Journal:  Curr Probl Dermatol       Date:  1991

3.  Familial and sporadic porphyria cutanea: two different diseases.

Authors:  H de Verneuil; G Aitken; Y Nordmann
Journal:  Hum Genet       Date:  1978-10-31       Impact factor: 4.132

4.  [Hereditary and non-hereditary form of chronic hepatic porphyria: different behaviour of uroporphyrinogen decarboxylase in liver and erythrocytes (author's transl)].

Authors:  M Doss; R von Tiepermann; D Look; H Henning; J Nikolowski; F Ryckmanns; O Braun-Falco
Journal:  Klin Wochenschr       Date:  1980-12-15

5.  [Chronic hepatic porphyria with uroporphyrinogen decarboxylase defect in four generations (author's transl)].

Authors:  P A Lehr; M Doss
Journal:  Dtsch Med Wochenschr       Date:  1981-02-20       Impact factor: 0.628

6.  An inherited enzymatic defect in porphyria cutanea tarda: decreased uroporphyrinogen decarboxylase activity.

Authors:  J P Kushner; A J Barbuto; G R Lee
Journal:  J Clin Invest       Date:  1976-11       Impact factor: 14.808

7.  Identification of two types of porphyria cutanea tarda by measurement of erythrocyte uroporphyrinogen decarboxylase.

Authors:  G H Elder; D M Sheppard; R E De Salamanca; A Olmos
Journal:  Clin Sci (Lond)       Date:  1980-06       Impact factor: 6.124

8.  Erythrocyte uroporphyrinogen decarboxylase activity in porphyria cutanea tarda: a study of 40 consecutive patients.

Authors:  J L Held; S Sassa; A Kappas; L C Harber
Journal:  J Invest Dermatol       Date:  1989-09       Impact factor: 8.551

  8 in total

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