Diseases

Hereditary Coproporphyria

An autosomal dominant porphyria that is due to a deficiency of COPROPORPHYRINOGEN OXIDASE in the LIVER, the sixth enzyme in the 8-enzyme biosynthetic pathway of HEME. Clinical features include both neurological symptoms and cutaneous lesions. Patients excrete increased levels of porphyrin precursors, 5-AMINOLEVULINATE and COPROPORPHYRINS.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

An autosomal dominant porphyria that is due to a deficiency of COPROPORPHYRINOGEN OXIDASE in the LIVER, the sixth enzyme in the 8-enzyme biosynthetic pathway of HEME. Clinical features include both neurological symptoms and cutaneous lesions. Patients excrete increased levels of porphyrin precursors, 5-AMINOLEVULINATE and COPROPORPHYRINS.

MeSH Record

Classification

Broader headings

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MeSH Record

Synonyms

3 entry terms
  • Coproporphyria, Hereditary
  • Coproporphyrinogen Oxidase Deficiency
  • Deficiency, Coproporphyrinogen Oxidase

MeSH Record

Aspects Covered

35 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, congenital, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.

MeSH Record

History Note

2005; use PORPHYRIA, HEPATIC 1993-2004

MeSH Record

Previous Indexing

  • Porphyria (1965-2004)
  • Porphyria, Hepatic (1993-2004)

MeSH Hierarchy

Tree Numbers

  1. C06w.552w.830w.074w
  2. C16w.320w.850w.742w.074w
  3. C17.800w.827w.742w.074w
  4. C18.452w.811w.400w.074w

AMA Style

References

  1. National Library of Medicine. Hereditary Coproporphyria. Medical Subject Headings (MeSH). 2026. Unique ID D046349. http://id.nlm.nih.gov/mesh/2026/D046349
  2. Hereditary Coproporphyria. In: Wikipedia. https://en.wikipedia.org/wiki/Hereditary_coproporphyria
  3. Hereditary Coproporphyria. In: Wikidata. https://www.wikidata.org/wiki/Q823140