The Beckwith-Wiedemann syndrome in four African infants.
Four cases of Beckwith-Wiedemann syndrome are reported. The clinical, pathological, radiological and laboratory findings in the syndrome are reviewed. The risk of intraabdominal malignancy in both the complete and incomplete forms is stressed and the consequent importance of early diagnosis emphasised. Early recognition of hypoglycaemia in infants with an omphalocele is also advocated. It is considered that the findings in one case indicate possible hyperresponsiveness of insulin secretion to i.v. glucose. The incidence of the syndrome is estimated to be 1/15000 births.
D001506:Beckwith-Wiedemann Syndrome / Q000453:epidemiology / Q000473:pathology*; D006801:Humans; D007231:Infant, Newborn; D009549:Nigeria