| hepatoerythropoietic porphyria | An autosomal recessive disorder in which there is a deficiency or absence of uroporphyrinogen decarboxylase; results in photosensitivity and excessive hepatic production of 8-and 7-carboxylate porphyrins. (05 Mar 2000) |
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| South African type porphyria | Porphyria characterised by abdominal pain and neuropsychiatric abnormalities, by dermal sensitivity to light and mechanical trauma, by increased faecal excretion of proto-and coproporphyrin, and by increased urinary excretion of d-aminolevulinic acid, porphobilinogen, and porphyrins; due to a deficiency of protoporphyrinogen oxidase; autosomal dominant inheritance. Synonym: protocoproporphyria hereditaria, South African type porphyria. (05 Mar 2000) |
| squirrel porphyria | Porphyria as an apparently normal metabolic state seen in the Florida fox squirrel (Sciurus niger). (05 Mar 2000) |
| d-aminolevulinate dehydratase porphyria | An inherited disorder in which there is a deficiency of porphobilinogen synthase; d-aminolevulinate levels are elevated, leading to neurological disturbances. Synonym: porphobilinogen synthase porphyria. (05 Mar 2000) |
| swine porphyria | Porphyria as a dominant trait seen in swine. (05 Mar 2000) |
| symptomatic porphyria | A form of hepatic porphyria (porphyria, hepatic) characterised by photosensitivity resulting in bullae that rupture easily to form shallow ulcers. This condition occurs in two forms: a sporadic, nonfamilial form that begins in middle age and has normal amounts of uroporphyrinogen decarboxylase with diminished activity in the liver; and a familial form in which there is an autosomal dominant inherited deficiency of uroporphyrinogen decarboxylase in the liver and red blood cells. (12 Dec 1998) |
| intermittent acute porphyria | <gastroenterology, haematology> A group of rare inherited metabolic disorders that result from a disturbance in porphyrin metabolism, causing increased formation and excretion of porphyrin or its precursors. It is caused by hepatic overproduction of d-aminolevulinic acid, which has greatly increased urinary excretion and of porphobilinogen, and some increase of uroporphyrin, due to a deficiency of porphobilinogen deaminase. Clinical features: intermittent acute attacks of hypertension, abdominal colic, psychosis, and polyneuropathy, but with no photosensitivity. It is exacerbated by the ingestion of certain drugs such as; barbiturates). Inheritance: autosomal dominant. (20 Sep 2002) |
| ovulocyclic porphyria | Acute episodic exacerbations of porphyria occurring in the premenstrual period. (05 Mar 2000) |
| erythropoietic porphyria | A classification of porphyria that includes congenital erythropoietic porphyria and erythropoietic protoporphyria. (05 Mar 2000) |
| bridging hepatic necrosis | Area of liver necrosis which bridges adjacent portal areas and central veins; subsequent post-necrotic collapse and fibrosis is likely to result in cirrhosis. (05 Mar 2000) |
| right branch of proper hepatic artery | <anatomy, artery> Right branch of proper hepatic artery; terminal branch of proper hepatic artery supplying right lobe of liver; branch: cystic artery. Synonym: ramus dexter arteriae hepaticae propriae, right branch of proper hepatic artery. (05 Mar 2000) |
| right hepatic artery | <anatomy, artery> Right branch of proper hepatic artery; terminal branch of proper hepatic artery supplying right lobe of liver; branch: cystic artery. Synonym: ramus dexter arteriae hepaticae propriae, right branch of proper hepatic artery. (05 Mar 2000) |
| right hepatic duct | The duct that transmits bile to the common hepatic duct from the right half of the liver and the right part of the caudate lobe. Synonym: ductus hepaticus dexter. (05 Mar 2000) |
| right hepatic veins | Veins draining much of the right lobe of the liver (posterior segments [VI & VII] and part of the inferior anterior segment [V]) which merge to form a single or sometimes double trunk, draining into the right side of the suprahepatic portion of the inferior vena cava (between the superior surface of the liver and the diaphragm); when single, it is the largest vein of the liver. Synonym: venae hepaticae dextrae. (05 Mar 2000) |
| middle hepatic veins | Veins draining the central portion of the liver (the superior anterior segment [VIII] and the left side of the inferior anterior segment [V] of the right lobe and the medial segment [IV] of the left lobe) which join to form a trunk that merges with that of the left hepatic veins about 90% of the time prior to entering the left side of the inferior vena cava. Synonym: venae hepaticae mediae. (05 Mar 2000) |