A Grimberg, RJ Ferry, A Kelly, S Koo-McCoy, K … - Diabetes, 2001 - Am Diabetes Assoc From the Division of Pediatric Endocrinology (AG, RJF, AK, SK-M., LB, CAS), the Children's Hospital
of Philadelphia, Philadelphia, Pennsylvania; the Division Endocrinology (KP), University of
Chicago, Chicago, Illinois; the Division of Endocrinology and Metabolism (BG), Hebrew ... Cited by 76 - Related articles - BL Direct - All 7 versions
RJ Ferry… - The Journal of pediatrics, 2000 - Elsevier From the Division of Endocrinology & Diabetes, Department of Pediatrics and Department of
Radiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; the Division
of Endocrinology, Hadassah Medical Center, Hebrew University, Jerusalem, Israel; the ... Cited by 47 - Related articles - BL Direct - All 8 versions
- ►nih.gov H Huopio, F Reimann, R Ashfield, J … - Journal of Clinical …, 2000 - Am Soc Clin Investig ATP-sensitive potassium channels play a major role in linking metabolic signals to the exocytosis
of insulin in the pancreatic β cell. These channels consist of two types of protein subunit: the
sulfonylurea receptor SUR1 and the inward rectifying potassium channel Kir6.2. ... Cited by 118 - Related articles - BL Direct - All 9 versions
- ►endojournals.org CA Stanley, PS Thornton, A Ganguly, C … - Journal of Clinical …, 2004 - Endocrine Soc INFANTS WITH CONGENITAL hyperinsulinism frequently present in the neonatal period with
symptomatic hypoglycemia, which may cause seizures or permanent brain damage. In the
past, these infants were believed to have a disturbance in pancreatic development ... Cited by 58 - Related articles - BL Direct - All 4 versions
- ►endojournals.org A Kelly, D Ng, RJ Ferry Jr, A Grimberg, S Koo- … - Journal of Clinical …, 2001 - Endocrine Soc Mutations of glutamate dehydrogenase cause the hyperinsulinism/hyperammonemia syndrome
by desensitizing glutamate dehydrogenase to allosteric inhibition by GTP. Normal allosteric activation
of glutamate dehydrogenase by leucine is thus uninhibited, leading us to propose that ... Cited by 37 - Related articles - BL Direct - All 6 versions
- ►endojournals.org I Giurgea, K Laborde, G Touati, C Bellanne- … - Journal of Clinical …, 2004 - Endocrine Soc Congenital hyperinsulinism (CHI) is related to two main histological pancreas anomalies: focal
adenomatous hyperplasia and diffuse ß-cell hypersecretion. Pharmacological tests to measure
acute insulin responses (AIR) to peripheral iv injections of glucose, calcium, and ... Cited by 33 - Related articles - BL Direct - All 4 versions
- ►diabetesjournals.org SG Straub, KE Cosgrove, C Ämmälä, RM Shepherd, … - Diabetes, 2001 - Am Diabetes Assoc From the Institute of Molecular Physiology and Department of Biomedical Science (KEC,
RMS, REO, PDB, JCC, MJD), University of Sheffield, Western Bank, Sheffield, and the Institute
of Child Health (MS, KJL, AA-G.), London, UK; the Division of Endocrinology & ... Cited by 46 - Related articles - BL Direct - All 4 versions
J Rahier, Y Guiot, C Sempoux - Arch Dis Child Fetal Neonatal Ed, 2000 - sur1.org The syndrome of persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI) was de- scribed
more than 40 years ago by Mc Quarrie.1 Despite the inordinate amount of interest in this
syndrome, the pathogenesis of the disease has not yet been completely elucidated. For ... Cited by 66 - Related articles - View as HTML - BL Direct - All 7 versions
- ►endojournals.org MJ Henwood, A Kelly, C MacMullen, P Bhatia, A … - Journal of Clinical …, 2005 - Endocrine Soc Congenital hyperinsulinism (HI) is most commonly caused by recessive mutations of the pancreatic
ß-cell ATP-sensitive potassium channel (K ATP ), encoded by two genes on chromosome
11p, SUR1 and Kir6.2. The two mutations that have been best studied, SUR1 g3992-9a ... Cited by 31 - Related articles - All 4 versions
- ►nih.gov [PDF] V Verkarre, JC Fournet, P De Lonlay, MS … - Journal of Clinical …, 1998 - pubmedcentral.nih.gov Congenital hyperinsulinism, or persistent hyperinsulinemic hypoglycemia of infancy (PHHI),
is a glucose metabolism disorder characterized by unregulated secretion of insulin and profound
hypoglycemia. From a morphological stand- point, there are two types of ... Cited by 152 - Related articles - BL Direct - All 7 versions
