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- W3022213868 endingPage "749" @default.
- W3022213868 startingPage "731" @default.
- W3022213868 abstract "The porphyrias comprise eight genetic diseases, each corresponding to a specific defect in the pathway of heme synthesis. Four are termed hepatic, because their clinical manifestations are caused by overproduction of heme precursors, mainly, if not entirely, in the liver. In all four, the initial presentation is dominated by abdominal pain and nausea, which may have a neurological basis. Generalized seizures and mental changes also occur. The neurological manifestations may be aggravated by hyponatremia, which is present in many attacks and ranges from moderate to severe. As attacks progress, motor neuropathy becomes prominent. The challenge to the neurologist is to consider the possibility of acute porphyria and obtain appropriate tests before instituting therapy with drugs, such as anticonvulsants, which can intensify the attack with potentially a devastating outcome. With appropriate management, the outlook for an acute attack is good, in most cases with full recovery of neurological function." @default.
- W3022213868 created "2020-05-13" @default.
- W3022213868 creator A5072655119 @default.
- W3022213868 date "2015-01-01" @default.
- W3022213868 modified "2023-09-24" @default.
- W3022213868 title "The Porphyrias" @default.
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