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Baker-Winegrad syndrome

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A syndrome occurring in infants usually before 6 months of age after ingesting food containing fructose or after infections, fatigue, fasting, and stress; severe hypoglycemia (fits, seizures, coma) and acidosis frequenly fatal or requiring hospitalization. Nausea and vomiting do not occur after fructose ingestion. At older age no aversion to sugar and sweets. The child is not emaciated; frequently obese and hepatomegalic. Both sexes affected.

Bibliography

R. A. Chambers, R. T. Pratt RT:
Idiosyncrasy to fructose. The Lancet, London 1956, 2: 340.
L. Baker, A. I. Winegrad:
Fasting hypoglycemia and metabolic acidosis associated with deficiency of hepatic fructose 1, 6-diphosphatase activity.
The Lancet, London, July 4, 1970, 2 (7662): 13-16.
M. De Pra, E. Laudanna:
[Baker-Winegrad disease (hepatomegaly, hypoglycemia during fasting, hyperlactacidemic metabolic acidosis, hepatic fructose-1-6-diphosphatase deficiency). Presentation of the 1st Italian case and pathogenetic hypothesis]. Article in Italian.
Minerva pediatrica, Torino, December 31, 1978, 30 (24): 1973-786.

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