ABSTRACT

Introduction: Total pancreatectomy (TP) markedly improves quality of life in children with chronic pancreatitis (CP), but results in brittle, insulinopenic diabetes. TP with IAT (TPIAT) may preserve insulin secretion. Minimal data are available regarding TPIAT in pediatric patients (Pts).

Methods: Between 2009-2016, TPIAT was performed in 13 Pts (7 boys) with median age 10.3 yrs (range 7-17 yrs). Six (46%) had a PRSS1 (protease, serine, 1) mutation, 2 (15.4%) had a CFTR (Cystic fibrosis transmembrane conductance regulator) mutation, 2 (15.4%) had combined CFTR/SPINK1 (serine protease inhibitor, Kazal-type, 1) mutations. All were euglycemic prior to TPIAT (normal fasting glucose and HbA1c; normal response to mixed meal tolerance test in a subset). Islet cells, isolated after TP, were infused in the portal vein. Pts were kept on an insulin infusion for (average) 6.8 days, then switched to MDI, with tight glycemic control.

Results: Six months after TPIAT, 5 Pts (38%) did not require insulin (HbA1c 5.5-6.1%), 3 Pts (23%) were on basal insulin only (0.03-0.35 U/Kg/day), with HbA1c of 5.5-6.7%, and 5 Pts (39%) required basal/bolus insulin therapy (0.5-1 U/Kg/day, median 0.6) with a HbA1c of 6.8-10.2%, median 7.6. Insulin requirements did not correlate with BMI-SDS (r=0.18) or number of islets/kg infused (r=-0.21). Pts with PRSS1 mutation had borderline lower (p=0.05, t-test) insulin requirements (0.1 U/kg/day) than Pts with CFTR mutation (0.46 U/kg/day). Ten Pts (77%) discontinued pain medication with complete pain resolution within 3 months. Complications including pyloric stenosis, intraabdominal adhesions, and gastroparesis occurred in 3 Pts; Spontaneous/exercise-induced hypoglycemia in 2 Pts.

Conclusions: TPIAT is an effective treatment for CP in children and adolescents. Within 6 months, it provided pain resolution in 77% and allowed good glycemic control with no insulin or low dose basal insulin in 61% of patients in this series.