coproporphyria

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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cop·ro·por·phyr·ia (kpr-pôr-fîr-)

n.

The presence of coproporphyrins in the urine, as in variegate porphyria.

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coproporphyria

[kop′rōpôrfir′ē·ə]

Etymology: Gk, kopros + porphyros, purple

a rare autosomal-dominant hereditary metabolic disorder in which large quantities of nitrogenous substances, called porphyrins, are excreted in the feces. Attacks, with varying GI and neurologic symptoms, may be precipitated by certain drugs, including barbiturates, sulfonamides, and steroids. Patients are often helped by a high-carbohydrate diet. Also called hereditary coproporphyria. See also acute intermittent porphyria, coproporphyrin, porphyria.

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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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coproporphyria (kop´rōpôr´fir´ē),

n the presence of an abnormal concentration of coproporphyrin in the urine. Normal values range from 70 mg to 250 mg/day. An increased amount of coproporphyrin III occurs in the urine in clinical lead poisoning, exposure to lead without clinically apparent symptoms, infections, malignant disease, alcoholic cirrhosis, after ingestion of small amounts of ethanol, and normally in some individuals.

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coproporphyria

porphyria marked by excessive excretion of coproporphyrin, chiefly in the feces.

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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