hrp0098p2-346 | Late Breaking | ESPE2024

Dynamic changes in exocrine pancreatic function after near total pancreatectomy in CHI Patients with KATP channel genes variants

Beka Elpiniki , Cheng Lauren , Cunjamalay Annaruby , Alhusaini Fatemah , Amin Rakesh , Peters Catherine , Dastamani Antonia

Background: Compound heterozygous or homozygous recessive variants within the ABCC8 and KCNJ11 genes represent common genetic aetiologies for Congenital Hyperinsulinism (CHI). These variants are associated with a severe diffuse form of the disease, often refractory to medical management, necessitating near-total pancreatectomy. Post-surgery, patients may develop diabetes mellitus (DM) and pancreatic exocrine insufficiency (PEI), necessitating...