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[Hereditary coproporphyria. 7 cases].

A Jaeger, J D Tempe, F Geisler

La Nouvelle Presse Medicale

|November 15, 1975

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Hereditary coprophyria is a rare hepatic porphyria. This study details two fatal cases, highlighting peripheral paralysis and respiratory failure, and identifies new family cases.

Area of Science:

Biochemistry
Genetics
Hepatology

Background:

Hereditary coproporphyria, identified in 1955, is a rare hepatic porphyria.
It shares clinical similarities with acute intermittent porphyria, but with rarer neurological manifestations.
The biochemical lesion remains elusive, though its hereditary basis is established.

Observation:

Patients exhibit massive urinary and fecal excretion of coproporphyrins.
Excretion of porphobilinogen and delta-amino-levulinic acid is only slightly elevated.
Adverse drug reactions, particularly to barbiturates, are noted, similar to acute intermittent porphyria.

Findings:

The authors report two cases of hereditary coproporphyria presenting with peripheral paralysis and respiratory failure.

Related Experiment Videos

One of these cases was fatal.

Family studies identified three additional cases in one family and two latent cases in another.

Implications:

This research contributes to understanding the clinical spectrum and genetic transmission of hereditary coproporphyria.
Early identification and management of affected families are crucial.
Further research into the biochemical basis is warranted to improve diagnostic and therapeutic strategies.