Elsevier

The Journal of Pediatrics

Volume 82, Issue 2, February 1973, Pages 258-262
The Journal of Pediatrics

Original article
Metabolic aspects of the Beckwith-Wiedemann syndrome*

https://doi.org/10.1016/S0022-3476(73)80163-5Get rights and content

Longitudinal metabolic studies on an infant with the Beckwith-Wiedemann syndrome during the first two years of life are reported. The infant was hypoglycemic and hyperinsulinemic. In addition, he showed insulin hyperresponsiveness to glucose and a variety of secretogogues. Therapy with diazoxide abolished the insulin response to glucose but did not suppress the abnormally with basal insulin levels, and the infant remained sporadically hypoglycemic. The additions of Sus-phrine to the diazoxide therapy resulted in suppression of the insulin levels to a normal range, with adequate control of blood glocuse levels.

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There are more references available in the full text version of this article.

Cited by (0)

*

Supported in part by United Cerebral Palsy Research and Educational Foundation, Inc., Grant No. R-232-70, and by the Meeical Research Council of Canada Grants Nos. MA 3037 and MA 2952.

*

Queen Elizabeth II Scientist for Research in Diseases of Children.

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