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[The hepatic porphyrias].

Authors: J, Schenk;

[The hepatic porphyrias].

Abstract

Among the different types of hepatic porphyrias, acute intermittent porphyria (AIP) and the group of chronic hepatic porphyrias (CHP) are most frequently seen in Europe. Both diseases are supposed to be inherited, but clinical manifestation mostly occurs following endogenous and especially exogenous stimulation. While recurrent attacks of abdominal and neuropsychiatric symptoms in AIP are frequently precipitated by therapeutic doses of commonly used drugs, the gradual development of porphyria cutanea tarda (PCT) from a clinically non-apparent type of CHP seems to be most commonly due to excessive and prolonged intake of alcohol. The characteristic and with regard to the laboratory findings important pathobiochemical features, the clinical symptoms, and the present therapeutic concepts of both disorders of hepatic porphyrin metabolism are discussed. The prognosis of hepatic porphyrias will be determined above all by early diagnosis of the metabolic disorder as well as by a profound medical information of the patient to avoid strictly all well-known exogenous factors which may initiate or exacerbate the disease.

Keywords

Adult, Male, Liver Cirrhosis, Porphyrins, Drug-Related Side Effects and Adverse Reactions, Carboxy-Lyases, Coproporphyrinogen Oxidase, Middle Aged, Enzymes, Hepatitis, Hydroxymethylbilane Synthase, Alcoholism, Intensive Care Units, Porphyrias, Liver, Skin Manifestations, Chronic Disease, Humans, Uroporphyrins, Ferrochelatase, Metabolism, Inborn Errors, Diet Therapy, Bloodletting

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selected citations
These citations are derived from selected sources.
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
0
Average
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