Porphyria, Hepatic
| Definition: |
Porphyria in which the liver is the site where excess formation of porphyrin or its precursors is found. PORPHYRIA, ACUTE INTERMITTENT and PORPHYRIA CUTANEA TARDA are types of hepatic porphyria. |
| Notes: |
do not confuse with PORPHYRIA, ERYTHROHEPATIC |
| Also Called: |
Coproporphyria, Hereditary,Porphyria, Variegate |
| Previously Indexed: |
Porphyria (1966-1992) |
Porphyria, Hepatic Categories.
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Porphyria Cutanea Tarda - A form of hepatic porphyria (PORPHYRIA, HEPATIC) characterized 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. |
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Porphyria, Acute Intermittent - A form of hepatic porphyria (PORPHYRIA, HEPATIC) characterized by periodic attacks of gastrointestinal disturbances, abdominal colic, paralyses, and psychiatric disorders. The onset of this condition is usually in the third or fourth decade of life. |
Porphyria, Hepatic Definitions and Terms
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