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 pre- and perioperative conditions to point out the focal pancreatic lesion are described.
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 pre- and perioperative conditions to point out the focal pancreatic lesion are described.
Authors: M-C Nollevaux; J Rahier; J Marchandise; P Thurion; S Godecharles; G Van den Steen; J Jamart; C Sempoux; P Jacquemin; Y Guiot Journal: Am J Physiol Endocrinol Metab Date: 2013-02-12 Impact factor: 4.310
Authors: N Scott Adzick; Diva D De Leon; Lisa J States; Katherine Lord; Tricia R Bhatti; Susan A Becker; Charles A Stanley Journal: J Pediatr Surg Date: 2018-10-05 Impact factor: 2.545
Authors: Björn A Blomberg; Mateen C Moghbel; Babak Saboury; Charles A Stanley; Abass Alavi Journal: Mol Imaging Biol Date: 2013-02 Impact factor: 3.488
Authors: Julie Bendix; Mette G Laursen; Michael B Mortensen; Maria Melikian; Evgenia Globa; Sönke Detlefsen; Lars Rasmussen; Henrik Petersen; Klaus Brusgaard; Henrik T Christesen Journal: Front Endocrinol (Lausanne) Date: 2018-08-22 Impact factor: 5.555