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.

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 ?
Acute porphyria is a term that includes three similar inherited diseases: acute intermittent porphyria (AIP), variegate porphyria (VP) and hereditary coproporphyria (HCP).
The 3 most common acute porphyrias are hereditary coproporphyria, variegate porphyria, and acute intermittent porphyria, all inherited as autosomal dominant disorders.
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.
Important follow-on work provided the answer to an otherwise puzzling question: why VP and hereditary coproporphyria (HCP) are associated with acute elevations in [delta]-aminolaevulinic acid (ALA) and porphobilinogen (PBG) and thus the acute attack, whereas porphyria cutanea tarda (PCT) is not, even though the enzymatic defect in PCT occurs more proximally in the haem biosynthetic pathway (Fig.
For the diagnosis of AIP, VP, and hereditary coproporphyria (HCP), the 3 most common acute porphyrias, analysis of ALA is not strictly necessary, but the majority of laboratories used the Bio-Rad ALA/PBG Column Test in which ALA and PBG can be analyzed consecutively.
Studies of porphyrin synthesis in fibroblasts of patients with congenital erythropoietic porphyria and one patient with homozygous coproporphyria.
Four porphyrias can present with life-threatening acute neurovisceral attacks: [delta]-aminolevulinic acid dehydratase deficiency porphyria, acute intermittent porphyria (AIP), variegate porphyria (VP), and hereditary coproporphyria (HCP) (1).
Patients with VP have a sharply defined fluorescence emission maximum at an excitation wavelength of 626 [+ or -] 1 nm (15); the presence of this characteristic peak efficiently separates patients with VP from those with AIP, erythropoietic protoporphyria, congenital erythropoietic porphyria, PCT, and hereditary coproporphyria (15, 16).
An acute attack precipitated by AIP is indistinguishable from attacks produced by variegate porphyria or hepatic coproporphyria.
Clinically, the acute porphyrias, which include acute intermittent porphyria, hereditary coproporphyria, and variegate porphyria, present intermittently with neurovisceral signs and symptoms that may be severe, possibly leading to paralysis, and in some cases can be life threatening (4,5).