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Pancreatic exocrine and endocrine function after pancreatectomy for persistent hyperinsulinaemic hypoglycaemia of
A Cade1, M Walters, J W Puntis
1Department of Paediatrics and Child Health, University of Leeds, UK.
Insights
Children with persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI) undergoing major pancreatectomy show long-term subclinical pancreatic insufficiency. Risks of diabetes mellitus and exocrine failure persist despite initial blood glucose control.
Area of Science:
- Pediatric endocrinology
- Gastroenterology
- Surgical outcomes
Background:
- Persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI) is a rare cause of neonatal hypoglycemia.
- Subtotal pancreatectomy is a treatment option for PHHI, but its long-term effects on pancreatic function are not fully understood.
Purpose of the Study:
- To evaluate long-term detailed pancreatic endocrine and exocrine function in children with PHHI after 85-95% pancreatectomy.
- To assess the risk of developing diabetes mellitus and exocrine insufficiency post-surgery.
Main Methods:
- Retrospective follow-up of six children with PHHI post-pancreatectomy (0.9-12.7 years post-resection).
- Clinical assessment, endocrine function tests, pancreatic MRI, and direct/indirect exocrine function tests.
- One non-operated PHHI child included for comparison.
Main Results:
- Pancreozymin-secretin stimulation tests revealed deficiency in 4/6 children; 1 required enzyme supplements.
- Pancreolauryl tests were abnormal in 2/3 children.
- One child developed insulin-dependent diabetes; two required diazoxide for normoglycemia.
- MRI showed no significant pancreatic remnant regrowth.
Conclusions:
- Despite initial blood glucose control, subclinical pancreatic endocrine and exocrine insufficiency is common long-term after major pancreatectomy for PHHI.
- Ongoing risks of diabetes mellitus and exocrine failure exist in these patients.
Aim:
To evaluate long term detailed pancreatic endocrine and exocrine function 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. Standard endocrine assessment, pancreatic magnetic resonance imaging (MRI), and detailed direct and indirect tests of exocrine pancreatic function were performed.
Results:
Pancreozymin-secretin stimulation test results were normal in only one child, borderline in two, and deficient in four, one of whom requires daily pancreatic enzyme supplements. Pancreolauryl tests performed in three children were borderline in two and abnormal in the other. Only one child had low faecal chymotrypsin values. One child developed insulin dependent diabetes at 9 years and two children at 1.0 and 13.3 years require diazoxide to maintain normoglycaemia. MRI showed no major regrowth of the pancreatic remnant after resection (n = 5).
Conclusions:
Clinical evidence of endocrine or exocrine dysfunction has developed in only two patients to date, but detailed pancreatic function testing suggests subclinical deficiency in all but one of our patients with PHHI. Although 95% pancreatectomy results in postoperative control of blood glucose, subclinical pancreatic insufficiency is present on long term follow up and development of diabetes mellitus and exocrine failure remain ongoing risks.