hrp0089p3-p173 | Fetal, Neonatal Endocrinology and Metabolism P3 | ESPE2018

Outcome of Eight Patients with Congenital Hyperinsulinism (CHI) Studied with 18[F]Dihydroxyphenyl-Alanine Positron Emission Tomography Imaging (18F-DOPA-PET-CT) in Argentina

Tangari Saredo Ana , Flanagan Sarah , Alonso Guillermo , Caceres Juan , Troiano Marina , Bignon Horacio , Bastianello Maria , Graciela Del Rey , Ignacio Bergada

Congenital hyperinsulinism (CHI) results from inappropriate insulin secretion most commonly caused by mutations in the ABCC8 and KCNJ11 genes which encode for the pancreatic β-cells-ATP-sensitive-potassium channel (KATP) subunits SUR1 and KIR6.2 respectively. Diagnosis of CHI is based on the presence of detectable plasma insulin during hypoglycemia, suppressed β-hydroxybutyrate and NEFA. Diazoxide is the major treatment for CHI, sirolimus had also b...