Partial elective pancreatectomy is curative in focal form of permanent hyperinsulinemic hypoglycaemia in infancy

A report of 45 cases from 1983 to 2000

C. Crtolle, C. Nihoul Fkt, Dominique M. Jan, M. C. Nassogne, J. M. Saudubray, F. Brunelle, J. Rahier

Research output: Contribution to journalArticle

54 Citations (Scopus)

Abstract

Background/Purpose: Permanent hyperinsulinemic hypoglycaemia in infancy (PHHI)I is a severe disease that leads to brain damage. Since 1989, pathologists have identified 2 different forms of the disease: a diffuse form (DiPHHI) and a focal form (FoPHHI). The purpose of this study was to adapt surgical techniques in case of FoPHHI to cure these infants without risk of diabetes. Methods: All patients with PHHI underwent pancreatic venous sampling (PVS) and elective partial pancreatectomy (EPP). Molecular biology and immunohistochemistry were used to ascertain that FoPHHI was a different disease from DiPHHI. Results: 45 EPPs were performed, guided by PVS and peroperative pathology. The lesions were 17 in the head, 4 in the isthmus, 6 in the body, 15 in the tail of the pancreas. Age at surgery ranged from 25 days to 4 years. Two patients already had been operated on elsewhere, and the focal lesion could be found at second operation. All 45 patients except one, were cured with euglycemia at both fasting and hyperglycaemic tests. Molecular biology has shown a specific anomaly in FoPHHI, which never has been encountered in DiPHHI. Conclusions: PHHI is not a homogeneous disease. In one third of cases, only a small amount of endocrine pancreas is abnormal, and conservative surgery is mandatory. The preand perioperative conditions to point out the focal pancreatic lesion are described.

Original languageEnglish (US)
Pages (from-to)155-158
Number of pages4
JournalJournal of Pediatric Surgery
Volume37
Issue number2
DOIs
StatePublished - 2002
Externally publishedYes

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Congenital Hyperinsulinism
Pancreatectomy
Molecular Biology
Islets of Langerhans
Pancreas
Fasting
Immunohistochemistry
Head
Pathology
Brain

Keywords

  • Hyperinsulinism
  • Partial pancreatectomy
  • Permanent hyperinsulinemic hypoglycaemia in infancy
  • Persistent hypoglycaemia

ASJC Scopus subject areas

  • Surgery

Cite this

Partial elective pancreatectomy is curative in focal form of permanent hyperinsulinemic hypoglycaemia in infancy : A report of 45 cases from 1983 to 2000. / Crtolle, C.; Fkt, C. Nihoul; Jan, Dominique M.; Nassogne, M. C.; Saudubray, J. M.; Brunelle, F.; Rahier, J.

In: Journal of Pediatric Surgery, Vol. 37, No. 2, 2002, p. 155-158.

Research output: Contribution to journalArticle

Crtolle, C. ; Fkt, C. Nihoul ; Jan, Dominique M. ; Nassogne, M. C. ; Saudubray, J. M. ; Brunelle, F. ; Rahier, J. / Partial elective pancreatectomy is curative in focal form of permanent hyperinsulinemic hypoglycaemia in infancy : A report of 45 cases from 1983 to 2000. In: Journal of Pediatric Surgery. 2002 ; Vol. 37, No. 2. pp. 155-158.
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