TY - JOUR
T1 - Partial elective pancreatectomy is curative in focal form of permanent hyperinsulinemic hypoglycaemia in infancy
T2 - A report of 45 cases from 1983 to 2000
AU - Crtolle, C.
AU - Fkt, C. Nihoul
AU - Jan, D.
AU - Nassogne, M. C.
AU - Saudubray, J. M.
AU - Brunelle, F.
AU - Rahier, J.
PY - 2002
Y1 - 2002
N2 - 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.
AB - 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.
KW - Hyperinsulinism
KW - Partial pancreatectomy
KW - Permanent hyperinsulinemic hypoglycaemia in infancy
KW - Persistent hypoglycaemia
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U2 - 10.1053/jpsu.2002.30241
DO - 10.1053/jpsu.2002.30241
M3 - Article
C2 - 11819190
AN - SCOPUS:0036154369
SN - 0022-3468
VL - 37
SP - 155
EP - 158
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
IS - 2
ER -