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Persistent hyperinsulinemic hypoglycemia of infancy (“nesidioblastosis”): Autosomal recessive inheritance in 7 pedigrees

✍ Scribed by Glaser, Benjamin ;Phillip, Moshe ;Carmi, Rivka ;Lieberman, Ester ;Landau, Heddy


Publisher
John Wiley and Sons
Year
1990
Tongue
English
Weight
455 KB
Volume
37
Category
Article
ISSN
0148-7299

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✦ Synopsis


Persistent Hyperinsulinemic Hypoglycemia of Infancy (PHHI) is a rare disease characterized clinically by persistent hypoglycemia with inappropriately elevated circulating insulin concentrations. Here we report on 7 pedigrees including 21 cases. The pedigrees are derived from 3 distinct ethnic groups, and include a very large Bedouin family, and Arab family, and 5 smaller pedigrees of Jewish families all of Eastern European origin. Data obtained from these families and from other families reported in the literature strongly suggest that PHHI is inherited as an autoso-ma1 recessive disorder.