coproporphyria

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 porphyria" >acute intermittent porphyria can occur.

cop·ro·por·phyr·i·a

(kop'rō-pōr-fir'ē-ă), [MIM*121300] Presence of coproporphyrins in the urine, as in variegate porphyria.

coproporphyria

An AD condition that primarily affects ♀, which is often latent until puberty Clinical2⁄3 of Pt are asymptomatic; Sx occur as acute attacks of photosensitivity or mental dysfunction, precipitated by alcohol, barbiturates, diphenylhydantoin, estrogens, griseofulvin, mephenytoin, meprobamate, progestins, sulfonamides, valproic acid, and other drugs Lab ↑ coproporphyrin III in feces, accumulation in the liver; during attacks, coproporphyrin, porphobilinogen, δ-aminolevulinic acid–ALA are ↑ in urine Management Morphine, phenothiazine, chlorpromazine; if no improvement in 24 hrs, IV ferric heme to suppress ALA synthase activity

cop·ro·por·phyr·i·a

(kop'rō-pōr-fir'ē-ă) Presence of coproporphyrins in the urine, as in variegate porphyria.

cop·ro·por·phyr·i·a

(kop'rō-pōr-fir'ē-ă) [MIM*121300] Presence of coproporphyrins in the urine.