Other Names for this Disease
- Coproporphyria hereditary
- Hereditary coproporphyria porphyria
- Porphyria hepatica coproporphyria
- Porphyria hepatica II
autosomal dominant form of liver (hepatic) porphyria that is very similar to acute intermittent porphyria, although it is usually a less severe disease. It results from low levels of the enzyme responsible for the sixth step in heme production - coproporphyrinogen oxidase. This enzyme speeds the conversion of coproporphyrinogen to protoporphyrinogen. In coproporphyria, the porphyrin precursors porphobilinogen and amino-levulinic acid (ALA) accumulate, as well as the formed porphyrin coproporphyrin. This leads to abdominal pain, neuropathies, constipation, and skin changes. Treatment is dependent on the symptoms.Hereditary coproporphyria is an
Last updated: 11/11/2010
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- Learning about Porphyria. National Human Genome Research Institute (NHGRI). 2010; http://www.genome.gov/19016728. Accessed 11/11/2010.
- DeLoughery TG. Porphyria, Hereditary Coproporphyria. eMedicine. 2009; http://emedicine.medscape.com/article/205374-overview. Accessed 11/11/2010.
- Hereditary Coproporphyria (HCP). American Porphyria Foundation. 2009; http://www.porphyriafoundation.com/about-porphyria/types-of-porphyria/HCP. Accessed 11/11/2010.
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