Cite this page: Arora K. Hereditary hepatic coproporphyria. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/liverhereditaryhepaticcopro.html. Accessed March 21st, 2019.
Definition / general
- Autosomal dominantly inherited hepatic porphyria, due to mutation in coproporphyrinogen oxidase (CPOX) gene (Clin Genet 2012;81:249)
- Causes partial defect of CPOX, sixth enzyme of heme biosynthesis
- Patients may develop acute life threatening attacks with neurovisceral or rarely cutaneous symptoms (Wikipedia: Hereditary Coproporphyria [Accessed 27 October 2017])
- 35 year old woman with rapidly progressive liver cirrhosis (Arch Pathol Lab Med 2002;126:751)