Ein Fall von kongenitaler Porphyrinurie mit Koproporphyrin in Harn und Stuhl

Bergh, A. A. Hijmans van den; Regniers, R.; Müller, Marie

doi:10.1159/000195235

Cited by 11 publications

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“…These were named coproporphyrin I and uroporphyrin I, the former having four methyl and four propionic acid groups, the latter eight carboxyl groups. Several instances of the same disease have since been studied by Fischer and Duesberg (5), and also Van den Bergh (6), in which identical porphyrins were excreted. However, one instance was reported by each in which coproporphyrin III clinically indistinguishable types of idiopathic porphyrinuria was presented.…”

mentioning

confidence: 95%

Concerning the Naturally Occurring Porphyrins

Watson¹

1935

J. Clin. Invest.

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mentioning

confidence: 95%

Concerning the Naturally Occurring Porphyrins

Watson¹

1935

J. Clin. Invest.

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“…Porphyria cutanea tarda. Dunklere Haare des Nackenhaaran− satzes (waren vor der Erkrankung grau).Diese 1928 von Hijmas van den Bergh[49] beschriebene seltene akute Porphyrie hat in 20 % der Fälle neben den Allgemeinsymp− tomen der akuten Porphyrie der PCT vergleichbare Hautverände− rungen[19]. Die autosomal dominante Erkrankung beruht auf Punktmutationen des Koproporphyrinogenoxidase−Gens.…”

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Die kutanen Porphyrien

Köstler¹,

Wollina²

2005

Akt Dermatol

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EinleitungDen Porphyrinkrankheiten liegt eine enzymatische Störung der Synthese der Porphyrinogene und des Häms zu Grunde. Nach dem primären Expressionsort des Enzymdefektes können die Porphyrien in erythropoetische und hepatische Formen unter− teilt werden [1], aus klinischer Sicht wird zunehmend in akute und nicht akute Porphyrien klassifiziert [2 ± 4] (Tab. 1).Die nicht akuten Porphyrien sind durch Hautsymptome charak− terisiert, bei der PV und HK können zusätzlich zu abdominellen, neurologischen und psychischen Symptomen Hautveränderun− gen auftreten, die AIP und die Doss−Porphyrie weisen aus− schließlich Allgemeinsymptome auf. AbstractPorphyrias are metabolic disorders caused by defects of enzymes of the hem− or porphyrin synthesis. Six out of the eight known diseases also have cutaneous manifestations which may be of di− agnostic relevance. Very rare disorders are congenital erythro− poietic porphyria and hepatoerythropoietic porphyria which re− semble each other. Both are associated with severe skin lesions in the sun−exposed areas. Erythropoietic protoporphyria (inci− dence 1 : 100,000) has always to be considered in differential di− agnosis of light−induced skin diseases. There are new insights in trigger factors and associated disorders of porphyria cutanea tar− da (PCT) (~1 ½ of the adult population affected) like hepatitis C virus infection or HFE−gene mutations that may have an impact on treatment regimens like phlebotomy and/or chloroquine. He− reditary coproporphyria and porphyria variegata are rare in Ger− many. They may cause PCT−like skin lesions, neuropsychiatric and gastrointestinal symptoms. The diagnosis of porphyria is pri− marily based on pattern recognition of metabolites in urine, faeces and blood. Übersicht 138Dieses Dokument wurde zum persönlichen Gebrauch heruntergeladen. Vervielfältigung nur mit Zustimmung des Verlages.

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Studies on Porphyria

Turner¹

1938

Arch Derm Syphilol

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Ein Fall von kongenitaler Porphyrinurie mit Koproporphyrin in Harn und Stuhl

Cited by 11 publications

References 0 publications

Concerning the Naturally Occurring Porphyrins

Concerning the Naturally Occurring Porphyrins

Die kutanen Porphyrien

Studies on Porphyria

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