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 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

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

coproporphyria

[kop′rōpôrfir′ē·ə]
Etymology: Gk, kopros + porphyros, purple
a rare autosomal-dominant metabolic disorder in which large quantities of nitrogenous substances, called porphyrins, are excreted in the feces. Attacks, with varying GI and neurological 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.

coproporphyria

An AD condition that primarily affects ♀, which is often latent until puberty Clinical23 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.

coproporphyria (kop´rōpôr´fir´ēə),

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.

coproporphyria

porphyria marked by excessive excretion of coproporphyrin, chiefly in the feces.
References in periodicals archive ?
J is diagnosed with hereditary coproporphyria (HCP).
The other 2 acute porphyrias arise from deficiencies later in the pathway: coproporphyrinogen oxidase in hereditary coproporphyria (HCP) and protoporphyrinogen oxidase in variegate porphyria (VP).
1] The acute porphyrias that are inherited in an autosomal dominant pattern include acute intermittent porphyria (AIP), variegate porphyria (VP) and hereditary coproporphyria, while acute porphyria due to 6-aminolaevulinic acid (ALA) dehydratase deficiency is an autosomal recessively inherited condition.
Catatonia was first described by Karl Kahlbaum in 1874 and has as an aetiology neurological disorders (encephalopaties, tumours, degenerative, central pontine myelinolysis, locked-in syndrome), psychiatry (hysteria, neuroleptic malignant syndrome, schizophrenia, psychosis, drug withdrawl), infectious diseases (AIDS, malaria, neurosyphilis), metabolic ones (Addison's, diabetic ketoacidosis, hepatic or renal failure, poisoning), but also inherited neurometabolic disorders such as homocystinuria, coproporphyria, Tay-Sachs disease.
KEY WORDS: Hereditary coproporphyria, Polyneuropathy, Neuropathic pain, Photosensitivity.
Hereditary coproporphyria is usually latent before puberty.
Acute hepatic porphyrias (acute intermittent porphyria (AIP), variegate porphyria (VP) and hereditary coproporphyria (HCP)) are inherited disorders of the heme biosynthesis pathway, which usually cause life-threatening acute attacks consisting of severe abdominal pain, nausea, vomiting, acute neuropathy, muscle weakness, even mental disturbances.
The acute hepatic porphyrias are autosomal dominant conditions with low penetrance and include acute intermittent porphyria, hereditary coproporphyria and variegate porphyria.
Molecular abnormalities of coproporphyrinogen oxidese in patients with hereditary coproporphyria.
A diagnosis of hereditary coproporphyria was eventually established.
The 3 most common acute porphyrias are hereditary coproporphyria, variegate porphyria, and acute intermittent porphyria, all inherited as autosomal dominant disorders.
Studies of porphyrin synthesis in fibroblasts of patients with congenital erythropoietic porphyria and one patient with homozygous coproporphyria.