Hereditary coproporphyria

Hereditary coproporphyria (HCP ) is an acute, hepatic ( liver -related) metabolic disorder selected from the group of porphyria. These diseases affect the synthesis of porphyrins such as heme, the red blood pigment. HCP shows symptoms mainly in the nervous system and internal organs ( visceral neuro ), more rarely, sun sensitivity.

The HCP is a congenital disorder formation of red blood pigment hemoglobin, caused by the partial loss of the enzyme coproporphyrinogen oxidase ( CPO, sixth enzyme of the heme formation chain). Due to the reduced activity of the enzyme occurs to the accumulation of precursors ( coproporphyrins ) in the liver and in the feces. The HCP is autosomal dominant. Symptoms, triggering factors and treatment acute intermittent porphyria are similar to those of (AIP ), with the difference that in some cases sunlight is not tolerated.