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Suggested pathobiochemical development of chronic hepatic porphyrias

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Summary

A working hypothesis concerning the development of acquired chronic hepatic porphyrias in liver diseases is proposed, based on biochemical differentiation of these metabolic disturbances. A stepwise progression appears to exist from the secondary porphyrinurias through the asymptomatic chronic hepatic porphyrias of the Types A, B, and C to porphyria cutanea tarda. It is attempted to diagnose chronic disturbances in hepatic porphyrin synthesis while they are still clinically inapparent.

Zusammenfassung

Eine Arbeitshypothese zur Entwicklung erworbener hepatischer Porphyrien bei Lebererkrankungen wird anhand einer biochemischen Differenzierung dieser Stoffwechselstörungen aufgestellt, die stufenweise Übergänge von sekundären Porphyrinurien über asymptomatische chronische hepatische Porphyrien Typ A, B und C bis zur Porphyria cutanea tarda erkennen ließ. Es ist anzustreben, die Diagnose einer chronischen hepatischen Porphyrinsynthesestörung im klinisch noch inapparenten Stadium zu stellen.

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  1. Abteilung für Klinische Biochemie am Hygiene-Institut der Universität Marburg a. d. Lahn, Germany

    Manfred Doss

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  1. Manfred Doss
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Doss, M. Suggested pathobiochemical development of chronic hepatic porphyrias. Klin Wochenschr 49, 941–942 (1971). https://doi.org/10.1007/BF01485221

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