Histochemical, clinical, and in vitro
cell responses in a
neonate with persistent hyperinsulinaemic hypoglycaemia of infancy
a The immunohistochemistry data were
presented at the 16th International Congress of Clinical Chemistry,
London, 8-12 July 1996 and the case was presented at the 9th Asian
Congress of Paediatrics, Hong Kong, 23-27 March 1997. Department of Chemical Pathology, The Chinese
University of Hong Kong, Shatin, New Territories,
Hong Kong, People's Republic of China, b Anatomical and
Cellular Pathology, c Department of Paediatrics
Correspondence to: Dr Panesar. Email: nspanesar{at}cuhk.edu.hk
Accepted 11 February 1998
When treatment with diazoxide and somatostatin for persistent
hyperinsulinaemic hypoglycaemia of infancy failed, subtotal pancreatectomy was performed on a neonate on day 41. The pancreatic tissue was saved and used for immunohistochemical and cell culture studies. The initial immunohistochemistry of
cells for insulin was
negative, using a 1 in 200 dilution of insulin antiserum, but positive
results were obtained with an increased concentration of the antiserum.
The insulin to somatostatin cell ratio in islets of
Langerhans was about 1:1, with no somatostatin cells outside the
islets. Glucose stimulated insulin secretion in a concentration
dependent manner in vitro. Isobutyl methyl xanthine doubled insulin
secretion, but lithium had no effect. The glucose stimulated insulin
secretion was inhibited by somatostatin, epinephrine, and in the
absence of Ca2+.
In view of the normal in vitro responses of
cells to
various secretory analogues, the lack of responsiveness to somatostatin analogue before pancreatectomy may not have been due to deficiency or
resistance to somatostatin, but to
cell hyperplasia overwhelming the paracrine regulatory mechanism(s).
© 1998 by Archives of Disease in Childhood
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