The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.
Orpha Number: 73423
A rare disease caused by the ingestion of unripe Blighia sapida fruits. It is a serious intoxication that is frequent in certain countries in the Caribbean and Western Africa. In contrast, it is rare in France and other Western countries. Intoxication leads to toxic hypoglycaemia and inhibition of neoglucogenesis. The hypoglycaemia is caused by the effect of hypoglycin A, which is found in the arils.
The clinical manifestations are severe (coma, convulsions, delirium, toxic hepatitis, acute dehydration and a state of shock) and may lead to death.
Management and treatment
Treatment is symptomatic (administration of glucose, IV or per os ).
Although the intoxication is generally benign or has a favourable outcome, ingestion of large doses may lead to death: ackee fruit intoxication lead to 29 deaths in 1998 and more than 50 in 2001.
Visit the Orphanet disease page for more resources.
Source: GARD Last updated on 05-01-20
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