To investigate long-term clinical and lived impacts of subtotal pancreatectomy for diffuse congenital hyperinsulinism (HI) as reported by affected families.
Approach:
Key Findings:
71% of participants underwent subtotal pancreatectomy before two months of age (24 out of 34).
Only 9% (3 out of 34) had normal glucose status at discharge.
At follow-up, 44% (15 out of 34) reported diabetes, 41% (14 out of 34) pancreatic insufficiency, and 24% (8 out of 34) ongoing medication for hypoglycemia.
Continuous glucose monitoring showed suboptimal time in range regardless of diabetes status.
Caregivers reported high stress and mental health challenges during hospitalization and post-discharge.
Interpretation:
Subtotal pancreatectomy may be necessary for managing severe hypoglycemia in diffuse HI, but it is not curative and leads to lifelong management challenges.
Limitations:
Small sample size for qualitative interviews.
Potential selection bias in participant recruitment.
Conclusion:
The study reports the long-term impacts of subtotal pancreatectomy on patients with diffuse HI and their families.