Context (archaeology)

Pancreas

Biology

Hyperinsulinism

Endocrinology

Insulin

Environmental health

Paleontology

Pediatrics

Physics

Pancreatectomy

Congenital hyperinsulinism

Optics

Insulin resistance

Population

Internal medicine

Medicine

Gastroenterology

Incidence (geometry)

Diabetes mellitus

The Journal of Clinical Endocrinology & Metabolism

Context The management of congenital hyperinsulinism (CHI) has improved. Objective To examine the treatment and long-term outcome of Finnish patients with persistent and transient CHI (P-CHI and T-CHI). Design A population-based retrospective study of CHI patients treated from 1972 to 2015. Patients 106 patients with P-CHI and 132 patients with T-CHI (in total, 42 diagnosed before and 196 after year 2000) with median follow-up durations of 12.5 and 6.2 years, respectively. Main Outcome Measures Recovery, diabetes, pancreatic exocrine dysfunction, neurodevelopment. Results The overall incidence of CHI (n = 238) was 1:11 300 live births (1972-2015). From 2000 to 2015, the incidence of P-CHI (n = 69) was 1:13 500 and of T-CHI (n = 127) 1:7400 live births. In the 21st century P-CHI group, hyperinsulinemic medication was initiated and normoglycemia achieved faster relative to earlier. Of the 74 medically treated P-CHI patients, 68% had discontinued medication. Thirteen (12%) P-CHI patients had partial pancreatic resection and 19 (18%) underwent near-total pancreatectomy. Of these, 0% and 84% developed diabetes and 23% and 58% had clinical pancreatic exocrine dysfunction, respectively. Mild neurological difficulties (21% vs 16%, respectively) and intellectual disability (9% vs 5%, respectively) were as common in the P-CHI and T-CHI groups. However, the 21st century P-CHI patients had significantly more frequent normal neurodevelopment and significantly more infrequent diabetes and pancreatic exocrine dysfunction compared with those diagnosed earlier. Conclusions Our results demonstrated improved treatment and long-term outcome in the 21st century P-CHI patients relative to earlier.

Long-Term Outcome and Treatment in Persistent and Transient Congenital Hyperinsulinism: A Finnish Population-Based Study