hereditary coproporphyria (redirected from CPX 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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hereditary coproporphyria

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