title: Histochemical, clinical, and in vitro beta cell responses in a neonate with persistent hyperinsulinaemic hypoglycaemia of infancy.
abstract: 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).
authors.affiliation: Department of Chemical Pathology, Chinese University of Hong Kong, Shatin, New Territories, People's Republic of China. nspanesar@cuhk.edu.hk
authors.forename: N S
authors.initials: NS
authors.lastname: Panesar
authors.forename: C W
authors.initials: CW
authors.lastname: Poon
authors.forename: C T
authors.initials: CT
authors.lastname: Liew
authors.forename: G W
authors.initials: GW
authors.lastname: Wong
authors.forename: N M
authors.initials: NM
authors.lastname: Hjelm
mesh_terms: D015056:1-Methyl-3-isobutylxanthine; D002478:Cells, Cultured; D003981:Diazoxide / Q000627:therapeutic use; D004232:Diuretics; D005947:Glucose; D006801:Humans; D006946:Hyperinsulinism / Q000151:congenital* / Q000188:drug therapy / Q000601:surgery; D006965:Hyperplasia; D007003:Hypoglycemia / Q000151:congenital* / Q000188:drug therapy / Q000601:surgery; D007150:Immunohistochemistry; D007231:Infant, Newborn; D007328:Insulin / Q000032:analysis; D007515:Islets of Langerhans / Q000737:chemistry / Q000473:pathology; D008297:Male; D010180:Pancreatectomy; D010726:Phosphodiesterase Inhibitors; D049993:Sodium Chloride Symporter Inhibitors / Q000627:therapeutic use; D013004:Somatostatin / Q000032:analysis / Q000627:therapeutic use; D017211:Treatment Failure
chemical_list: D004232:Diuretics; D007328:Insulin; D010726:Phosphodiesterase Inhibitors; D049993:Sodium Chloride Symporter Inhibitors; D013004:Somatostatin; D005947:Glucose; D003981:Diazoxide; D015056:1-Methyl-3-isobutylxanthine