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Porphyrias, Hepatic

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Definition: A group of metabolic diseases due to deficiency of one of a number of LIVER enzymes in the biosynthetic pathway of HEME. They are characterized by the accumulation and increased excretion of PORPHYRINS or its precursors. Clinical features include neurological symptoms (PORPHYRIA, ACUTE INTERMITTENT), cutaneous lesions due to photosensitivity (PORPHYRIA CUTANEA TARDA), or both (HEREDITARY COPROPORPHYRIA). Hepatic porphyrias can be hereditary or acquired as a result of toxicity to the hepatic tissues.  do not confuse with PORPHYRIA, ERYTHROHEPATIC   
Examples Coproporphyria, Hereditary; Porphyria, Acute Intermittent; Porphyria Cutanea Tarda; Porphyria, Hepatoerythropoietic; Porphyria, Variegate; Protoporphyria, Erythropoietic
Other names Hepatic Porphyria; Hepatic Porphyrias; Porphyria, Hepatic

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012

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