CHARLES ALFRED STANLEY

Summary

Affiliation: The Children's Hospital of Philadelphia
Country: USA

Publications

  1. ncbi request reprint Clinical features and insulin regulation in infants with a syndrome of prolonged neonatal hyperinsulinism
    Francis M Hoe
    Divisions of Endocrinology and Neonatology, Department of Pediatrics, Children s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA
    J Pediatr 148:207-12. 2006
  2. pmc Elimination of KATP channels in mouse islets results in elevated [U-13C]glucose metabolism, glutaminolysis, and pyruvate cycling but a decreased gamma-aminobutyric acid shunt
    Changhong Li
    Division of Endocrinology, The Children s Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 283:17238-49. 2008
  3. pmc Extremes of clinical and enzymatic phenotypes in children with hyperinsulinism caused by glucokinase activating mutations
    Samir Sayed
    Clinical Translational Research Center, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
    Diabetes 58:1419-27. 2009
  4. pmc Diazoxide-unresponsive congenital hyperinsulinism in children with dominant mutations of the β-cell sulfonylurea receptor SUR1
    Courtney M MacMullen
    Division of Endocrinology Diabetes, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
    Diabetes 60:1797-804. 2011
  5. pmc The value of radiologic interventions and (18)F-DOPA PET in diagnosing and localizing focal congenital hyperinsulinism: systematic review and meta-analysis
    Björn A Blomberg
    Perelman School of Medicine, University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA, USA
    Mol Imaging Biol 15:97-105. 2013
  6. pmc Parsing ketotic hypoglycaemia
    C A Stanley
    Division of Endocrinology, Children s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19026, USA
    Arch Dis Child 91:460-1. 2006
  7. ncbi request reprint Carnitine deficiency disorders in children
    Charles A Stanley
    Division of Endocrinology, The Children s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA
    Ann N Y Acad Sci 1033:42-51. 2004
  8. ncbi request reprint Preoperative evaluation of infants with focal or diffuse congenital hyperinsulinism by intravenous acute insulin response tests and selective pancreatic arterial calcium stimulation
    Charles A Stanley
    Division of Endocrinology, The Children s Hospital of Philadelphia, Pennsylvania 19104, USA
    J Clin Endocrinol Metab 89:288-96. 2004
  9. ncbi request reprint Molecular basis and characterization of the hyperinsulinism/hyperammonemia syndrome: predominance of mutations in exons 11 and 12 of the glutamate dehydrogenase gene. HI/HA Contributing Investigators
    C A Stanley
    The Children s Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania School of Medicine, 19104, USA
    Diabetes 49:667-73. 2000
  10. pmc Regulation of glutamate metabolism and insulin secretion by glutamate dehydrogenase in hypoglycemic children
    Charles A Stanley
    The Children s Hospital of Philadelphia, PA 19104, USA
    Am J Clin Nutr 90:862S-866S. 2009

Research Grants

  1. International Medical Conference of Congenital Hyperinsulinism
    Charles Stanley; Fiscal Year: 2006
  2. ISLET DYSREGULATION IN INFANTS
    Charles Stanley; Fiscal Year: 2008
  3. Ped Endocrine Career Development in Diabetes Research
    Charles Stanley; Fiscal Year: 2007
  4. MOLECULAR BASIS OF A NEW FORM OF HYPERINSULINISM
    CHARLES ALFRED STANLEY; Fiscal Year: 2010

Collaborators

Detail Information

Publications60

  1. ncbi request reprint Clinical features and insulin regulation in infants with a syndrome of prolonged neonatal hyperinsulinism
    Francis M Hoe
    Divisions of Endocrinology and Neonatology, Department of Pediatrics, Children s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA
    J Pediatr 148:207-12. 2006
    ..To characterize the clinical features and insulin regulation in infants with hypoglycemia due to prolonged neonatal hyperinsulinism...
  2. pmc Elimination of KATP channels in mouse islets results in elevated [U-13C]glucose metabolism, glutaminolysis, and pyruvate cycling but a decreased gamma-aminobutyric acid shunt
    Changhong Li
    Division of Endocrinology, The Children s Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 283:17238-49. 2008
    ....
  3. pmc Extremes of clinical and enzymatic phenotypes in children with hyperinsulinism caused by glucokinase activating mutations
    Samir Sayed
    Clinical Translational Research Center, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
    Diabetes 58:1419-27. 2009
    ..We report three children with de novo glucokinase hyperinsulinism mutations who displayed a spectrum of clinical phenotypes corresponding to marked differences in enzyme kinetics...
  4. pmc Diazoxide-unresponsive congenital hyperinsulinism in children with dominant mutations of the β-cell sulfonylurea receptor SUR1
    Courtney M MacMullen
    Division of Endocrinology Diabetes, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
    Diabetes 60:1797-804. 2011
    ..Dominant K(ATP) mutations have been associated with diazoxide-responsive disease. We hypothesized that some medically uncontrollable cases with only one K(ATP) mutation might have dominant, diazoxide-unresponsive disease...
  5. pmc The value of radiologic interventions and (18)F-DOPA PET in diagnosing and localizing focal congenital hyperinsulinism: systematic review and meta-analysis
    Björn A Blomberg
    Perelman School of Medicine, University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA, USA
    Mol Imaging Biol 15:97-105. 2013
    ....
  6. pmc Parsing ketotic hypoglycaemia
    C A Stanley
    Division of Endocrinology, Children s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19026, USA
    Arch Dis Child 91:460-1. 2006
  7. ncbi request reprint Carnitine deficiency disorders in children
    Charles A Stanley
    Division of Endocrinology, The Children s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA
    Ann N Y Acad Sci 1033:42-51. 2004
    ....
  8. ncbi request reprint Preoperative evaluation of infants with focal or diffuse congenital hyperinsulinism by intravenous acute insulin response tests and selective pancreatic arterial calcium stimulation
    Charles A Stanley
    Division of Endocrinology, The Children s Hospital of Philadelphia, Pennsylvania 19104, USA
    J Clin Endocrinol Metab 89:288-96. 2004
    ..The combination of ASVS, careful intraoperative histologic analysis, and surgical expertise succeeded in correcting hypoglycemia in 86% of the infants with focal hyperinsulinism...
  9. ncbi request reprint Molecular basis and characterization of the hyperinsulinism/hyperammonemia syndrome: predominance of mutations in exons 11 and 12 of the glutamate dehydrogenase gene. HI/HA Contributing Investigators
    C A Stanley
    The Children s Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania School of Medicine, 19104, USA
    Diabetes 49:667-73. 2000
    ..Based on the three-dimensional structure of GDH, the mutations may function by impairing the binding of an inhibitory GTP to a domain responsible for the allosteric and cooperativity properties of GDH...
  10. pmc Regulation of glutamate metabolism and insulin secretion by glutamate dehydrogenase in hypoglycemic children
    Charles A Stanley
    The Children s Hospital of Philadelphia, PA 19104, USA
    Am J Clin Nutr 90:862S-866S. 2009
    ....
  11. pmc Two genetic forms of hyperinsulinemic hypoglycemia caused by dysregulation of glutamate dehydrogenase
    Charles A Stanley
    University of Pennsylvania School of Medicine, Endocrinology Division, The Children s Hospital of Philadelphia, Philadelphia, PA 19026, United States
    Neurochem Int 59:465-72. 2011
    ..These two novel human disorders demonstrate the important role of GDH in insulin regulation and illustrate unexpectedly important reasons for the unusually complex allosteric regulation of GDH...
  12. ncbi request reprint Hyperinsulinism/hyperammonemia syndrome: insights into the regulatory role of glutamate dehydrogenase in ammonia metabolism
    Charles A Stanley
    Division of Endocrinology, Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Mol Genet Metab 81:S45-51. 2004
    ....
  13. ncbi request reprint Protein-sensitive hypoglycemia without leucine sensitivity in hyperinsulinism caused by K(ATP) channel mutations
    Shannon H Fourtner
    Division of Endocrinology Diabetes, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
    J Pediatr 149:47-52. 2006
    ....
  14. ncbi request reprint A signaling role of glutamine in insulin secretion
    Changhong Li
    Division of Endocrinology, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 279:13393-401. 2004
    ....
  15. ncbi request reprint Effects of a GTP-insensitive mutation of glutamate dehydrogenase on insulin secretion in transgenic mice
    Changhong Li
    Division of Endocrinology, The Children s Hospital of Philadelphia and Diabetes Center, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 281:15064-72. 2006
    ..This study suggests that GDH functions predominantly in the direction of glutamate oxidation rather than glutamate synthesis in mouse islets and that this flux is tightly controlled by glucose...
  16. ncbi request reprint Genotype-phenotype correlations in children with congenital hyperinsulinism due to recessive mutations of the adenosine triphosphate-sensitive potassium channel genes
    Maria J Henwood
    Division of Endocrinology Diabetes, The Children s Hospital of Philadelphia, Pennsylvania 19104, USA
    J Clin Endocrinol Metab 90:789-94. 2005
    ..These results indicate that some K(ATP) mutations can yield partially functioning channels, including cases of hyperinsulinism that are fully responsive to diazoxide therapy...
  17. ncbi request reprint Clinical and molecular characterization of a dominant form of congenital hyperinsulinism caused by a mutation in the high-affinity sulfonylurea receptor
    Paul S Thornton
    Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    Diabetes 52:2403-10. 2003
    ..2 and trafficks to the plasma membrane, but it had no (86)Rb efflux ion transport activity. These results indicate that hyperinsulinism in this family is caused by a SUR1 mutation that is expressed dominantly rather than recessively...
  18. ncbi request reprint Molecular and immunohistochemical analyses of the focal form of congenital hyperinsulinism
    Mariko Suchi
    Department of Pathology and Laboratory Medicine, The Childrens Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    Mod Pathol 19:122-9. 2006
    ..The combined results from the immunohistochemical and molecular methods indicate that maternal 11p15 loss together with paternal K(ATP) mutation is the predominant causative mechanism of focal hyperinsulinism...
  19. pmc Clinical characteristics and biochemical mechanisms of congenital hyperinsulinism associated with dominant KATP channel mutations
    Sara E Pinney
    Division of Endocrinology Diabetes, The Children s Hospital of Philadelphia, Department of Genetics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    J Clin Invest 118:2877-86. 2008
    ....
  20. ncbi request reprint Prenatal diagnosis and postnatal management of diffuse congenital hyperinsulinism: a case report
    William H Peranteau
    The Center for Fetal Diagnosis and Treatment, The Children s Hospital of Philadelphia, The University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Fetal Diagn Ther 21:515-8. 2006
    ..This case highlights the benefits of the timely in utero diagnosis of hyperinsulinism by mutational analysis...
  21. ncbi request reprint A genome-wide association study identifies KIAA0350 as a type 1 diabetes gene
    Hakon Hakonarson
    Center for Applied Genomics, Abramson Research Center, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    Nature 448:591-4. 2007
    ..These results indicate that KIAA0350 might be involved in the pathogenesis of T1D and demonstrate the utility of the genome-wide association approach in the identification of previously unsuspected genetic determinants of complex traits...
  22. ncbi request reprint Accuracy of [18F]fluorodopa positron emission tomography for diagnosing and localizing focal congenital hyperinsulinism
    Olga T Hardy
    Division of Endocrinology, The Children s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, Pennsylvania 19104, USA
    J Clin Endocrinol Metab 92:4706-11. 2007
    ..This study evaluated the accuracy of 18F-fluoro-l-dihydroxyphenylalanine ([18F]DOPA) positron emission tomography (PET) scans in diagnosing focal vs. diffuse disease and identifying the location of focal lesions...
  23. ncbi request reprint Hyperinsulinism in infancy and childhood: when an insulin level is not always enough
    Andrew A Palladino
    The Children s Hospital of Philadelphia, Division of Endocrinology, Philadelphia, PA, USA
    Clin Chem 54:256-63. 2008
    ..HI is characterized by dysregulated insulin secretion, which results in persistent mild to severe hypoglycemia. The various forms of HI represent a group of clinically, genetically, and morphologically heterogeneous disorders...
  24. ncbi request reprint A novel susceptibility locus for type 1 diabetes on Chr12q13 identified by a genome-wide association study
    Hakon Hakonarson
    Center for Applied Genomics, Abramson Research Center, The Children s Hospital of Philadelphia, PA 19104 4318, USA
    Diabetes 57:1143-6. 2008
    ..03 x 10(-10)) and confirmed in two additional cohorts. Here we describe the results of testing, in these additional cohorts, 23 loci that were next in rank of statistical significance...
  25. ncbi request reprint Glutaminolysis and insulin secretion: from bedside to bench and back
    Andrea Kelly
    Department of Pediatrics, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
    Diabetes 51:S421-6. 2002
    ..These findings provide a foundation for defining pathways of basal secretion and AASIS, augmenting our understanding of beta-cell function...
  26. pmc Exendin-(9-39) corrects fasting hypoglycemia in SUR-1-/- mice by lowering cAMP in pancreatic beta-cells and inhibiting insulin secretion
    Diva D De Leon
    Division of Endocrinology, Department of Pediatrics, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 283:25786-93. 2008
    ..Our findings indicate that exendin-(9-39) normalizes fasting hypoglycemia in SUR-1(-/-) mice via a direct effect on insulin secretion, thereby raising exendin-(9-39) as a potential therapeutic agent for K(ATP) hyperinsulinism...
  27. ncbi request reprint Poor specificity of low growth hormone and cortisol levels during fasting hypoglycemia for the diagnoses of growth hormone deficiency and adrenal insufficiency
    Andrea Kelly
    Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Division of Endocrinology Diabetes, 8416 Main Building, 34th and Civic Center Boulevard, Philadelphia, PA 19104, USA
    Pediatrics 122:e522-8. 2008
    ....
  28. pmc Follow-up analysis of genome-wide association data identifies novel loci for type 1 diabetes
    Struan F A Grant
    Center for Applied Genomics, Abramson Research Center, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
    Diabetes 58:290-5. 2009
    ....
  29. ncbi request reprint Regulation of leucine-stimulated insulin secretion and glutamine metabolism in isolated rat islets
    Changhong Li
    Division of Endocrinology, Children s Hospital of Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 278:2853-8. 2003
    ..These mechanisms provide an explanation for hypoglycemia caused by mutations of GDH in children...
  30. pmc Mechanism of hyperinsulinism in short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency involves activation of glutamate dehydrogenase
    Changhong Li
    Division of Endocrinology, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 285:31806-18. 2010
    ..These studies indicate that SCHAD deficiency causes hyperinsulinism by activation of GDH via loss of inhibitory regulation of GDH by SCHAD...
  31. ncbi request reprint The hyperinsulinism/hyperammonemia syndrome
    Andrew A Palladino
    The Children s Hospital of Philadelphia, Division of Endocrinology and Diabetes, University of Pennsylvania, School of Medicine, Philadelphia, PA 19104, USA
    Rev Endocr Metab Disord 11:171-8. 2010
    ..The HI/HA syndrome provides a rare example of an inborn error of intermediary metabolism in which the effect of the mutation on enzyme activity is a gain of function...
  32. ncbi request reprint A specialized team approach to diagnosis and medical versus surgical treatment of infants with congenital hyperinsulinism
    Andrew A Palladino
    Division of Endocrinology and Diabetes, The Children s Hospital of Philadelphia, University of Pennsylvania, School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Semin Pediatr Surg 20:32-7. 2011
    ....
  33. ncbi request reprint Histopathology of congenital hyperinsulinism: retrospective study with genotype correlations
    Mariko Suchi
    Department of Pathology and Laboratory Medicine, The Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA
    Pediatr Dev Pathol 6:322-33. 2003
    ..We conclude from this study that nearly 90% of HI cases can be classified into either a diffuse or a focal form. However, a small percentage of cases represented a diagnostic challenge...
  34. ncbi request reprint Blood glucose control during selective arterial stimulation and venous sampling for localization of focal hyperinsulinism lesions in anesthetized children
    Giovanni Cucchiaro
    Children s Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    Anesth Analg 99:1044-8, table of contents. 2004
    ....
  35. ncbi request reprint Central nervous system hyperexcitability associated with glutamate dehydrogenase gain of function mutations
    David M Raizen
    Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, USA
    J Pediatr 146:388-94. 2005
    ..To describe seizure phenotypes associated with the hyperinsulinism/hyperammonemia syndrome (HI/HA), which is caused by gain of function mutations in the enzyme glutamate dehydrogenase (GDH)...
  36. ncbi request reprint Congenital hyperinsulinism: intraoperative biopsy interpretation can direct the extent of pancreatectomy
    Mariko Suchi
    Department of Pathology and Laboratory Medicine, The Children s Hospital of Philadelphia and University of Pennsylvania School of Medicine, 5203 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA
    Am J Surg Pathol 28:1326-35. 2004
    ..However, a small number of cases with unusual histology warrant caution when performing frozen section evaluation...
  37. ncbi request reprint A multidisciplinary approach to the focal form of congenital hyperinsulinism leads to successful treatment by partial pancreatectomy
    N Scott Adzick
    Department of Surgery, and The Center for Hyperinsulinism, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    J Pediatr Surg 39:270-5. 2004
    ..Although these 2 forms of HI are clinically identical, focal HI can be cured surgically. The authors reviewed their experience with partial pancreatectomy for focal HI...
  38. pmc Comparative genetic analysis of inflammatory bowel disease and type 1 diabetes implicates multiple loci with opposite effects
    Kai Wang
    Center for Applied Genomics, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Hum Mol Genet 19:2059-67. 2010
    ....
  39. pmc Expression, purification and characterization of human glutamate dehydrogenase (GDH) allosteric regulatory mutations
    Jie Fang
    Division of Endocrinology, The Children s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA
    Biochem J 363:81-7. 2002
    ....
  40. ncbi request reprint Familial leucine-sensitive hypoglycemia of infancy due to a dominant mutation of the beta-cell sulfonylurea receptor
    Sheela N Magge
    Division of Endocrinology, Children s Hospital of Philadelphia, Abramson Research Center, Room 802, 3615 Civic Center Boulevard, Philadelphia, Pennsylvania 19104 4318, USA
    J Clin Endocrinol Metab 89:4450-6. 2004
    ..2 in COSm6 cells demonstrated partially impaired ATP-dependent potassium channel function. Leucine-sensitive hypoglycemia in this family was found to result from a dominantly expressed SUR1 mutation...
  41. ncbi request reprint Diagnosis and localization of focal congenital hyperinsulinism by 18F-fluorodopa PET scan
    Olga T Hardy
    Division of Endocrinology, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    J Pediatr 150:140-5. 2007
    ..To assess the accuracy of 18F-fluoro-L-dihydroxyphenylalanine ([18F]-DOPA) PET scans to diagnose focal versus diffuse disease and to localize focal lesions in infants with congenital hyperinsulinism...
  42. ncbi request reprint Mechanisms of Disease: advances in diagnosis and treatment of hyperinsulinism in neonates
    Diva D De Leon
    University of Pennsylvania, Philadelphia, PA, USA
    Nat Clin Pract Endocrinol Metab 3:57-68. 2007
    ..The diagnosis and management of children with congenital hyperinsulinism requires a multidisciplinary approach to achieve the goal of therapy: prevention of permanent brain damage due to recurrent hypoglycemia...
  43. ncbi request reprint Hypoglycemia in the neonate
    Charles A Stanley
    Division Chief of Endocrinology Diabetes, Children s Hospital of Philadelphia, Professor of Pediatrics, University of Pennsylvania School of Medicine, 34th Street and Civic Center Blvd, Philadelphia, PA 19104, USA
    Pediatr Endocrinol Rev 4:76-81. 2006
    ..diffuse disease. Disease-specific diagnoses and treatments are therefore essential for effective management of the various forms of neonatal hyperinsulinism...
  44. ncbi request reprint Effects of hypoglycemia on developmental outcome in children with congenital hyperinsulinism
    Linda Steinkrauss
    Department of Endocrinology, The Children s Hospital of Philadephia, PA 19104, USA
    J Pediatr Nurs 20:109-18. 2005
    ..Early recognition and control of hypoglycemia are essential for preventing developmental delay in these children...
  45. ncbi request reprint Necrotizing enterocolitis in neonates receiving octreotide for the management of congenital hyperinsulinism
    Pablo Laje
    Department of Surgery, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Pediatr Diabetes 11:142-7. 2010
    ..The aim of this report is to present a series of patients treated at our institution in which we observed this association, and review the current related literature...
  46. ncbi request reprint Multiple ectopic lesions of focal islet adenomatosis identified by positron emission tomography scan in an infant with congenital hyperinsulinism
    William H Peranteau
    Department of Surgery, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    J Pediatr Surg 42:188-92. 2007
    ..It highlights the ability of PET to successfully identify focal lesions, including ectopic tissue, responsible for hyperinsulinemic hypoglycemia...
  47. ncbi request reprint Newborn screening by tandem mass spectrometry for medium-chain Acyl-CoA dehydrogenase deficiency: a cost-effectiveness analysis
    Laura N Venditti
    Department of Genetics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
    Pediatrics 112:1005-15. 2003
    ....
  48. ncbi request reprint Intussusception after pancreatic surgery in children: a case series
    Pablo Laje
    Department of Surgery, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    J Pediatr Surg 45:1496-9. 2010
    ..Postoperative intussusception (POI) has been described after a wide variety of pediatric surgical procedures, but it has not been reported as a complication after pediatric pancreatic resections...
  49. ncbi request reprint Insights into the structure and regulation of glucokinase from a novel mutation (V62M), which causes maturity-onset diabetes of the young
    Anna L Gloyn
    Diabetes Research Laboratories, Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford OX3 7LJ, United Kingdom
    J Biol Chem 280:14105-13. 2005
    ..Elucidating such mechanisms leads to a deeper understanding of the GCK glucose sensor and the biochemistry of beta-cells and hepatocytes...
  50. ncbi request reprint The structure of apo human glutamate dehydrogenase details subunit communication and allostery
    Thomas J Smith
    Donald Danforth Plant Science Center, 975 North Warson Road, St Louis, MO 63132, USA
    J Mol Biol 318:765-77. 2002
    ..These results support the previous proposal that purines regulate GDH activity by altering the dynamics of the NAD binding domain. Finally, a possible structural mechanism for negative cooperativity is presented...
  51. pmc A novel KCNJ11 mutation associated with congenital hyperinsulinism reduces the intrinsic open probability of beta-cell ATP-sensitive potassium channels
    Yu Wen Lin
    Center for Research on Occupational and Environmental Toxicology, Oregon Health and Science University, Portland, Oregon 97239, USA
    J Biol Chem 281:3006-12. 2006
    ..Our results also highlight the critical role of the Kir6.2 slide helix in determining the intrinsic open probability of KATP channels...
  52. ncbi request reprint Advances in diagnosis and treatment of hyperinsulinism in infants and children
    Charles A Stanley
    J Clin Endocrinol Metab 87:4857-9. 2002
  53. ncbi request reprint Evolution of glutamate dehydrogenase regulation of insulin homeostasis is an example of molecular exaptation
    Aron Allen
    The Donald Danforth Plant Science Center, 975 North Warson Road, St Louis, Missouri 63132, USA
    Biochemistry 43:14431-43. 2004
    ....
  54. ncbi request reprint A mutation in the TMD0-L0 region of sulfonylurea receptor-1 (L225P) causes permanent neonatal diabetes mellitus (PNDM)
    Ricard Masia
    Department of Cell Biology and Physiology, Washington University School of Medicine, St Louis, MO 63110, USA
    Diabetes 56:1357-62. 2007
    ....
  55. pmc Congenital hyperinsulinism associated ABCC8 mutations that cause defective trafficking of ATP-sensitive K+ channels: identification and rescue
    Fei Fei Yan
    Center for Research on Occupational and Environmental Toxicology, Oregon Health and Science University, Portland, OR 97239, USA
    Diabetes 56:2339-48. 2007
    ..Our study identifies a group of CHI-causing SUR1 mutations for which the resulting K(ATP) channel trafficking and expression defects may be corrected pharmacologically to restore channel function...
  56. pmc Destabilization of ATP-sensitive potassium channel activity by novel KCNJ11 mutations identified in congenital hyperinsulinism
    Yu Wen Lin
    Center for Research on Occupational and Environmental Toxicology, Oregon Health and Science University, 3181 S W Sam Jackson Park Road, Portland, OR 97239, USA
    J Biol Chem 283:9146-56. 2008
    ..Our studies suggest the importance of Kir6.2 subunit-subunit interactions in K(ATP) channel gating and function and reveal a novel gating defect underlying CHI...
  57. ncbi request reprint Green tea polyphenols modulate insulin secretion by inhibiting glutamate dehydrogenase
    Changhong Li
    Donald Danforth Plant Science Center, 975 Warson Road, St Louis, MO 63132, USA
    J Biol Chem 281:10214-21. 2006
    ..These results demonstrate that EGCG, much like the activator of GDH (BCH), can facilitate dissecting the complex regulation of insulin secretion by pharmacologically modulating the effects of GDH...
  58. ncbi request reprint The diagnosis of ectopic focal hyperinsulinism of infancy with [18F]-dopa positron emission tomography
    Khalid Hussain
    London Center for Pediatric Endocrinology and Metabolism, Hospital for Children National Health Service Trust, London WC1N 3JH, United Kingdom
    J Clin Endocrinol Metab 91:2839-42. 2006
    ..The focal form of the disease can be cured if the focal lesion can be localized accurately and completely resected with surgery...
  59. ncbi request reprint Medications used in the treatment of hypoglycemia due to congenital hyperinsulinism of infancy (HI)
    Khalid Hussain
    The London Centre for Paediatric Endocrinology and Metabolism, Great Ormond Street Hospital for Children NHS Trust WC1N 3JH and the Institute of Child Health, University College London, WC1N 1EH United Kingdom
    Pediatr Endocrinol Rev 2:163-7. 2004
    ..The drugs used in the medical therapy for congenital hyperinsulinism are diazoxide, octreotide, glucagon and nifedipine...
  60. ncbi request reprint Structural studies on ADP activation of mammalian glutamate dehydrogenase and the evolution of regulation
    Soojay Banerjee
    Donald Danforth Plant Science Center, St Louis, Missouri 63132, USA
    Biochemistry 42:3446-56. 2003
    ..This suggests that there was some selective advantage of the antenna itself and that animals evolved new functions for GDH through the addition of allosteric regulation...

Research Grants4

  1. International Medical Conference of Congenital Hyperinsulinism
    Charles Stanley; Fiscal Year: 2006
    ..unreadable] [unreadable] [unreadable]..
  2. ISLET DYSREGULATION IN INFANTS
    Charles Stanley; Fiscal Year: 2008
    ..The results of this research will provide essential information for improving the diagnosis and treatment of HI children and for understanding the basis of insulin regulation in normal humans. ..
  3. Ped Endocrine Career Development in Diabetes Research
    Charles Stanley; Fiscal Year: 2007
    ..The Program is strongly supported by access to a superb range of institutional resources, including the CHOP GCRC and the UPenn DERC. Request is made for 2 Appointee slots in this Program each year. ..
  4. MOLECULAR BASIS OF A NEW FORM OF HYPERINSULINISM
    CHARLES ALFRED STANLEY; Fiscal Year: 2010
    ....