PANCREATIC EXOCRINE AND ENDOCRINE FUNCTION AFTER PANCREATECTOMY FOR PERSISTENT HYPERINSULINEMIC HYPOGLYCEMIA OF INFANCY

Aim-To evaluate long term detailed pancreatic endocrine and exocrine f unction in children with persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI) after 85-95% pancreatectomy. Methods-Six children with PHHI between 0.9 and 12.7 years after pancreatic resection underwent clinical and investigative follow up at 1.0 to 14.9 years of age. One child with PHHI who had not had pancreatectomy was also assessed. Stan dard endocrine assessment, pancreatic magnetic resonance imaging (MRI) , and detailed direct and indirect tests of exocrine pancreatic functi on were performed. Results-Pancreozymin-secretin stimulation test resu lts were normal in only one child, borderline in two, and deficient in four, one of whom requires daily pancreatic enzyme supplements. Pancr eolauryl tests performed in three children were borderline in two and abnormal in the other. Only one child had low faecal chymotrypsin valu es. One child developed insulin dependent diabetes at 9 years and two children at 1.0 and 13.3 years require diazoxide to maintain normoglyc aemia. MRI showed no major regrowth of the pancreatic remnant after re section (n = 5). Conclusions-Clinical evidence of endocrine or exocrin e dysfunction has developed in only two patients to date, but detailed pancreatic function testing suggests subclinical deficiency in all bu t one of our patients with PHHI. Although 95% pancreatectomy results i n postoperative control of blood glucose, subclinical pancreatic insuf ficiency is present on long term follow up and development of diabetes mellitus and exocrine failure remain ongoing risks.