Abstract

Neonatal diabetes mellitus (NDM) with hypoinsulinism is an uncommon condition with an estimated incidence from 1 in 3,00,000 to 4,00,000 live births [1]. Diabetes mellitus with hyperinsulinism due to Berardinelli Seip Congenital Lipodystrophy( BSCL) presenting in the neonatal period is even rarer [2]. We report a child with neonatal onset BSCL having mutation of the AGPAT 2 gene diagnosed at the age of 42 days

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