hereditary 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.

he·red·i·tar·y cop·ro·por·phyr·i·a

an inherited (autosomal dominant) disorder of a deficiency of coproporphyrinogen oxidase, resulting in overproduction of porphyrin precursors leading to neurologic disturbances and photosensitivity.

hereditary coproporphyria

hereditary coproporphyria

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