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Archives of Disease in Childhood - Fetal and Neonatal Edition 1998;79:F141-F144; doi:10.1136/fn.79.2.F141
Copyright © 1998 BMJ Publishing Group Ltd & Royal College of Paediatrics and Child Health.
Arch Dis Child Fetal Neonatal Ed 1998;79:F141-F144 ( September )

Histochemical, clinical, and in vitro beta  cell responses in a neonate with persistent hyperinsulinaemic hypoglycaemia of infancy

N S Panesar,a C W Poon,a C T Liew,b G W K Wong,c N M Hjelma

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 beta  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 beta  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 beta  cell hyperplasia overwhelming the paracrine regulatory mechanism(s).

Keywords: persistent hyperinsulinaemic hypoglycaemia of infancy; somatostatin; in vitro cell studies; negative immunohistochemistry; steric hindrance


© 1998 by Archives of Disease in Childhood

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