Pancreatic exocrine and endocrine function after pancreatectomy for persistent hyperinsulinaemic hypoglycaemia of infancy
a Department of Paediatrics and Child Health, University
of Leeds, Leeds, UK, b Department of Radiology, University of Leeds, c Department of Paediatric Surgery, General Infirmary
at Leeds, Leeds, UK
Correspondence to: Dr A Cade, Cystic Fibrosis Unit, Children's Day Hospital, St James' University Hospital, Beckett Street, Leeds LS9 7TF, UK.
Accepted 21 April 1998
AIM
To evaluate long term detailed pancreatic
endocrine and exocrine function in children with persistent
hyperinsulinaemic hypoglycaemia of infancy (PHHI) after 85-95% pancreatectomy.
METHODSSix 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.
RESULTSPancreozymin-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).
CONCLUSIONSClinical 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.
© 1998 by Archives of Disease in Childhood
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