[Acute intermittent porphyria in the practice of an intensive care specialist].
Acute intermittent porphyria (AIP) is a hereditary disease caused by disordered haem biosynthesis and characterized by paroxysmal exacerbations. It usually manifests in adult women. Provoking factors are pregnancy, alcohol, and "porphyrogenic" drugs. Grave attacks of AIP require urgent hospitalization in intensive care wards, rapid purposeful diagnosis and adequate therapy, determining the prognosis. The number of drugs should be minimized and drugs with a known porphyrogenic effect absolutely ruled out. A 35-year-old patient with the first episode of AIP is described; the disease eventuated in death after 2 months.
Ershov
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D000208:Acute Disease; D000328:Adult; D000428:Alcohol Drinking; D003422:Critical Care; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D011164:Porphyrias / Q000175:diagnosis / Q000401:mortality / Q000628:therapy*; D011247:Pregnancy; D011248:Pregnancy Complications / Q000175:diagnosis / Q000628:therapy; D011379:Prognosis