Pancreatic exocrine and endocrine function after pancreatectomy for persistent hyperinsulinaemic hypoglycaemia of infancy

Abstract

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 insuYciency is present on long term follow up and development of diabetes mellitus and exocrine failure remain ongoing risks. (Arch Dis Child 1998;79:435-439)

Clinical and genetic heterogeneity in congenital hyperinsulinism

“…This risk increases with age, especially after puberty, and might also be even higher [62]. The exocrine pancreas function might be also altered after subtotal pancreatectomy but this is less frequent and not all patients require supplementation of pancreatic enzymes [15,28]. The ®nal decision for a radical surgical intervention in the diuse form has to be made very carefully and individually for each patient.…”

“…Whereas in focal disease a limited resection of the focal area with preservation of as much pancreas as possible is performed, extension of surgery for diuse disease up to complete pancreatectomy results in an increasing risk of developing diabetes mellitus [15,62,69]. A recent study reported the occurrence of diabetes in 67% of patients after a >95% pancreatectomy [15].…”

Long-term follow-up of 114 patients with congenital hyperinsulinism

“…An improvement in adequate management will be achieved by the recently developed management recommendation by the ENRHI (European Network of Research in Hyperinsulinism) which proposes pancreatic venous sampling prior to surgery (4,15,23,24). If no focal lesion is found during this procedure and a diffuse disease is likely, long-term octreotide treatment has to be considered, especially since after near total pancreatectomy or recurrent operations the risk of developing insulindependent diabetes mellitus is known to be greatly increased, especially after puberty (11,12). At present, the overall incidence of insulin-dependent diabetes is about 27% for all operated patients after a mean follow-up period of 11 years.…”

“…Today, it is known that this might be due to the presence of overlooked focal lesions, especially localized in the head of the pancreas. Besides this, undirected extensive pancreatic resection is associated with a high risk of developing diabetes mellitus (11,12). An alternative drug for long-term treatment is long-acting somatostatin (octreotide) in addition to a carbohydrate-enriched diet and frequent feedings (13).…”

Genetics and Pathophysiology of Hyperinsulinism in Infancy

“…The incidence of these complications (in up to 50% of survivors) has changed little during the past 20 years which indicates the major problems that are faced in managing and treating this condition [3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13]. In addition to genetic disorders, hyperinsulinism in infants can also be present as a transient condition (lasting 1–2 days, or several months) associated with maternal diabetes, rhesus incompatibility, perinatal stresses such as birth asphyxia, maternal toxaemia, or intrauterine growth retardation [14, 15, 16].…”