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hepatic porphyria A category of porphyria that includes porphyria cutanea tarda, variegate porphyria, and coproporphyria.
Synonym: porphyria hepatica.
(05 Mar 2000)
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)
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)
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)
abdomen, acute Clinical syndrome characterised by abdominal pain of great severity associated with other symptoms and signs, usually those of acute peritonitis, which might well be the result of a ruptured abdominal viscus or a similar abdominal catastrophe requiring urgent surgical operation.
(12 Dec 1998)
acute 1. Sharp, poignant.
2. Having a short and relatively severe course.
Origin: L. Acutus = sharp
(18 Nov 1997)
acute abdomen Any serious acute intra-abdominal condition (such as appendicitis) attended by pain, tenderness, and muscular rigidity, and for which emergency surgery must be considered.
Synonym: surgical abdomen.
(05 Mar 2000)
acute abscess A recently formed abscess with little or no fibrosis in the wall of the cavity.
Synonym: hot abscess.
(05 Mar 2000)
acute adrenal crisis <endocrinology> An abrupt life-threatening state which is caused by insufficient production of cortisol by the adrenal gland.
A typical finding in Addison's disease. Individuals who have been taking corticosteroids (glucocorticoids) for a prolonged period of time (weeks to months) are at risk for acute adrenal crisis if the medication is stopped abruptly. For this reason, corticosteroid medication are withdrawn slowly on a diminishing dosing schedule.
Symptoms include low blood pressure (shock), weakness, headache, vomiting, fever chills, tachycardia and sweating.
Treatment includes blood pressure support and intravenous hydrocortisone.
(27 Sep 1997)
acute adrenocortical insufficiency Severe adrenocortical insufficiency when an intercurrent illness or trauma causes an increased demand for adrenocortical hormones in a patient with adrenal insufficiency due to disease or use of relatively large amounts of similar hormones as therapy; characterised by nausea, vomiting, hypotension, and frequently hyperthemia, hyponatraemia, hyperkalaemia, and hypoglycaemia; can be fatal if untreated.
Synonym: addisonian crisis, adrenal crisis, Bernard-Sergent syndrome.
(05 Mar 2000)
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