Further studies of the hypoglycemia in children with the exomphalos--macroglossia--gigantism syndrome.

Beckwitli and hiis associates(l) described the following features of a condition subsequently called the exomplialos-macroglossia-gigantism (E IG) syndrome: macroglossia; omphalocoele; visceromegaly; somatic gigantism and neonatal hlypoglycemia. Combs et al.(2) studied two clhildren with this syndrome and demonstrated that lhyperinsulinism and polycytlhemia during infancy were also part of the syndrome. The facial abnormality was emphasized by Irving(3). Wiedemann and his co-workers(4,5) and Hooft et al.(6) suggested that the etiology of this syndrome was a nonprogressive ablnormality of the dienceplhalon. Tlhree studies recently published(7-9) lhave summarized the information currently available in the literature andl have highliglhted the variability of the syndrome. The current paper reports studies carried out in eiglht such clhildren, three of wlhom lhad lhypoglycemia. The clhanging nature of their hypoglycemia is documented, and hypoglycemia in the E1\IG syndrome is discussed.