hereditary coproporphyria (redirected from CPO deficiency)

coproporphyria /cop·ro·por·phy·ria/ (-por-fir´e-ah) any of various types of porphyria characterized by elevated levels of coproporphyrin in the body.

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hereditary coproporphyria  (HCP) a hepatic porphyria due to a defect in an enzyme involved in porphyrin synthesis, characterized by recurrent attacks of gastroenterologic and neurologic dysfunction, cutaneous photosensitivity, and excretion of coproporphyrin III in the feces and urine and of δ and porphobilinogen in urine.

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hereditary coproporphyria.

See coproporphyria.

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coproporphyria [kop″ro-por-fir´e-ah]

any of various types of porphyria characterized by elevated levels of coproporphyrin in the body.

hereditary coproporphyria a hepatic porphyria transmitted as an autosomal dominant trait, characterized biochemically by constant excretion of coproporphyrin III in the feces and intermittent urinary excretion of coproporphyrin, α-aminolevulinic acid (ALA), and porphobilinogen (PBG). The condition is usually asymptomatic, but acute attacks resembling those of acute intermittent porphyria can occur.

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hereditary coproporphyria

Metabolic disease An AD porphyria caused by a 50% ↓ in coproporphyrinogen oxidase activity Clinical Neurologic dysfunction, photosensitivity Lab ↑ Fecal protoporphyrin. See Porphyria.