uroporphyria

uroporphyria

 [u″ro-por-fir´e-ah]
porphyria with excessive excretion of porphyrins.

uroporphyria

/uro·por·phyr·ia/ (-por-fir´e-ah) porphyria with excessive excretion of uroporphyrin.

uroporphyria

[yoo͡r′ōpôrfir′ē·ə]
Etymology: Gk, ouron + porphyros, purple
a rare genetic disease characterized by excessive secretion of uroporphyrin in the urine, blistering dermatitis, photosensitivity, splenomegaly, and hemolytic anemia. Corticosteroid ointments may be helpful for the skin lesions; splenectomy may be necessary to alleviate the hemolytic anemia. Most patients die from hematologic complications before they reach middle age. See also porphyria.

uroporphyria

porphyria with excessive excretion of uroporphyrin.
References in periodicals archive ?
Uroporphyria in mice: thresholds for hepatic CYP1A2 and iron.
CYP1A2 is essential in murine uroporphyria caused by hexachlorobenzene and iron.
There is evidence that some of the toxicity of these pHAHs may be directly due to CYP1A activity; for example, CYP1A2 knockout mice are resistant to liver damage and uroporphyria when exposed to TCDD (Smith et al.
Protection of the CYP1a2 null mouse against uroporphyria and hepatic injury following exposure to 2,3,7,8-tetrachlorodibenzo-p-dioxin.
CYP1a1(-/-) male mice: protection against high-dose TCDD-induced lethality and wasting syndrome, and resistance to intrahepatocyte lipid accumulation and uroporphyria.
Cyp1a2(-/-) mice also appear to be completely protected from hepatic uroporphyria caused by dioxin, hexachlorobenzene, and iron overload, although metabolism of these chemicals does not seem to be involved in the mechanism of their toxicity (Sinclair et al.
Uroporphyria produced in mice by iron and 5-aminolaevulinic acid does not occur in Cyp1a2(-/-) null mutant mice.