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2591 pages added, reviewed or updated during the last month (last updated: 16/4/2021)


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  • presentation may be with sudden death, seizures, encephalopathy or liver failure
  • average age of presentation is 15 months
  • presentation is often in winter and autumn when the prevalence of intercurrent infections is highest
  • in previously undiagnosed cases there is a 25% mortality at first presentation; 25% have significant neurological morbidity at first presentation
  • during crises there is often hepatomegaly secondary to fatty infiltration
  • presentation may be as a Reye's like illness
  • a late sign is hypoglycaemia
  • tendency for decompensation decreases with age with increased glycogen stores
  • adults have been diagnosed with MCAD - they have never presented symptomatically but have a strong desire to eat when hungry and not miss meals

Last reviewed 01/2018

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