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.
Miller-Keane Encyclopedia and Dictionary of Medicine, Nursing, and Allied Health, Seventh Edition. © 2003 by Saunders, an imprint of Elsevier, Inc. All rights reserved.

cop·ro·por·phyr·i·a

(kop'rō-pōr-fir'ē-ă), [MIM*121300]
Presence of coproporphyrins in the urine, as in variegate porphyria.
Farlex Partner Medical Dictionary © Farlex 2012

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
McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.

cop·ro·por·phyr·i·a

(kop'rō-pōr-fir'ē-ă)
Presence of coproporphyrins in the urine, as in variegate porphyria.
Medical Dictionary for the Health Professions and Nursing © Farlex 2012

cop·ro·por·phyr·i·a

(kop'rō-pōr-fir'ē-ă) [MIM*121300]
Presence of coproporphyrins in the urine.
Medical Dictionary for the Dental Professions © Farlex 2012
References in periodicals archive ?
AHP is comprised of four subtypes, each resulting from a genetic defect leading to deficiency in one of the enzymes of the heme biosynthesis pathway in the liver: acute intermittent porphyria (AIP), hereditary coproporphyria (HCP), variegate porphyria (VP), and ALAD-deficiency porphyria (ADP).
A molecular, enzymatic and clinical study in a family with hereditary coproporphyria. J Inherit Metab Dis.
Clinically, porphyrias are classified as (a) acute attacks (neuropsychiatric) only, e.g., acute intermittent porphyria (AIP) and ALA dehydratase deficiency; (b) cutaneous (photosensitivity), e.g., porphyria cutanea tarda (PCT), congenital erythropoietic porphyria (CEP), and erythropoietic protoporphyria (EPP); (c) both cutaneous disease and acute attacks, e.g., variegate porphyria (VP) and hereditary coproporphyria (HCP).
In contrast, in AIP, variegate porphyria, and hereditary coproporphyria, urine PBG concentrations are increased as are urine ALA concentrations (Table 2).
J is diagnosed with hereditary coproporphyria (HCP).
[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.
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. J Bioenerg Biomembr 27:215-219.