hrp0084p3-1072 | Hypo | ESPE2015

Failure of Sirolimus Response on Three More Cases with a Diffuse Type of Congenital Hyperinsulinism

al Mutair Angham , Al BALWI Rana , al Otaibi Ahlam , Atawi Mohsen

Background: Congenital hyperinsulinism (CHI) represent a group of clinically and genetically heterogonous disorder that characterized by unregulated insulin secretion by B-cells. It is the most common cause of hypoglycaemia in the neonatal period. Infants with diffuse CHI have homozygous or compound heterozygous mutation in the KATP channel and the majority are unresponsive to standard medical therapy and eventually they need near total pancreatectomy. Recent data showed the e...