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DeCS
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Descriptor English:
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Porphyrias, Hepatic
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Descriptor Spanish:
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Porfirias Hepáticas
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Descriptor Portuguese:
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Porfirias Hepáticas
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Synonyms English:
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Hepatic Porphyria
Hepatic Porphyrias
Porphyria, Hepatic
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Tree Number:
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C06.552.830
C16.320.850.742
C17.800.827.742
C18.452.811.400
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Definition English:
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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. |
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Indexing Annotation English:
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do not confuse with PORPHYRIA, ERYTHROHEPATIC
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History Note English:
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2005 (1993)
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Allowable Qualifiers English:
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Record Number:
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30605
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Unique Identifier:
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D017094
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Occurrence in VHL:
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Similar:
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DeCS
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