This site is intended for healthcare professionals

account_circleSign in
Go to /sign-in page

You can view 5 more pages before signing in

Clinical features

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

  • 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

Related pages

Create an account to add page annotations

Create a free account

Add information to this page that would be handy to have on hand during a consultation, such as a web address or phone number. This information will always be displayed when you visit this page