Charles A Stanley

Charles A Stanley

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Charles A Stanley

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Mechanisms of octanoic acid potentiation of insulin secretion in isolated islets.

Islets 2019 8;11(4):77-88. Epub 2019 Mar 8.

a Division of Endocrinology and Diabetes, The Children's Hospital of Philadelphia , Philadelphia, PA , USA.

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https://www.tandfonline.com/doi/full/10.1080/19382014.2019.1
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http://dx.doi.org/10.1080/19382014.2019.1566683DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6682262PMC
January 2020

Glutamate Dehydrogenase, a Complex Enzyme at a Crucial Metabolic Branch Point.

Neurochem Res 2019 Jan 27;44(1):117-132. Epub 2017 Oct 27.

Department of Biochemistry and Molecular Biology, University of Texas Medical Branch at Galveston, Galveston, TX, USA.

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http://dx.doi.org/10.1007/s11064-017-2428-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5924581PMC
January 2019

Surgical treatment of congenital hyperinsulinism: Results from 500 pancreatectomies in neonates and children.

J Pediatr Surg 2019 Jan 5;54(1):27-32. Epub 2018 Oct 5.

Department of Pediatrics and the Congenital Hyperinsulinism Center, Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.

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http://dx.doi.org/10.1016/j.jpedsurg.2018.10.030DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6339589PMC
January 2019

Glutamate dehydrogenase: Structure of a hyperinsulinism mutant, corrections to the atomic model, and insights into a regulatory site.

Proteins 2019 01 18;87(1):41-50. Epub 2018 Nov 18.

Department of Biochemistry and Molecular Biology, University of Texas Medical Branch, Galveston, Texas.

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http://dx.doi.org/10.1002/prot.25620DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6469361PMC
January 2019

Clinical heterogeneity of hyperinsulinism due to HNF1A and HNF4A mutations.

Pediatr Diabetes 2018 08 1;19(5):910-916. Epub 2018 Mar 1.

Division of Endocrinology and Diabetes, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.

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http://dx.doi.org/10.1111/pedi.12655DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6030428PMC
August 2018

A severe case of hyperinsulinism due to hemizygous activating mutation of glutamate dehydrogenase.

Pediatr Diabetes 2017 Dec 6;18(8):911-916. Epub 2017 Feb 6.

Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.

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http://dx.doi.org/10.1111/pedi.12507DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5545170PMC
December 2017

Seizures and diagnostic difficulties in hyperinsulinism-hyperammonemia syndrome.

Turk J Pediatr 2016 ;58(5):541-544

Acıbadem University Faculty of Medicine, Division of Pediatrics Endocinology, İstanbul, Turkey.

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http://dx.doi.org/10.24953/turkjped.2016.05.014DOI Listing
September 2017

Functional and Metabolomic Consequences of K Channel Inactivation in Human Islets.

Diabetes 2017 07 25;66(7):1901-1913. Epub 2017 Apr 25.

Division of Endocrinology and Diabetes, Department of Pediatrics, The Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA

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http://dx.doi.org/10.2337/db17-0029DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5482088PMC
July 2017

Congenital Hypoglycemia Disorders: New Aspects of Etiology, Diagnosis, Treatment and Outcomes: Highlights of the Proceedings of the Congenital Hypoglycemia Disorders Symposium, Philadelphia April 2016.

Pediatr Diabetes 2017 02 18;18(1):3-9. Epub 2016 Oct 18.

The Children's Hospital of Philadelphia Congenital Hyperinsulinism Center and the Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.

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http://dx.doi.org/10.1111/pedi.12453DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5473026PMC
February 2017

Pharmacological Correction of Trafficking Defects in ATP-sensitive Potassium Channels Caused by Sulfonylurea Receptor 1 Mutations.

J Biol Chem 2016 Oct 29;291(42):21971-21983. Epub 2016 Aug 29.

From the Department of Biochemistry and Molecular Biology, Oregon Health and Science University, Portland, Oregon 97239,

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http://dx.doi.org/10.1074/jbc.M116.749366DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5063981PMC
October 2016

Defining the Phenotype and Assessing Severity in Phosphoglucomutase-1 Deficiency.

J Pediatr 2016 08 17;175:130-136.e8. Epub 2016 May 17.

Hayward Genetics Center, Tulane University School of Medicine, New Orleans, LA; Department of Pediatrics, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands.

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http://dx.doi.org/10.1016/j.jpeds.2016.04.021DOI Listing
August 2016

Histologic and Molecular Profile of Pediatric Insulinomas: Evidence of a Paternal Parent-of-Origin Effect.

J Clin Endocrinol Metab 2016 Mar 12;101(3):914-22. Epub 2016 Jan 12.

Department of Pathology and Laboratory Medicine (T.R.B., A.R.H., L.C., E.D.R.), The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104-4399; Department of Pathology (T.R.B., L.C., E.D.R.), The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania 19104; Division of Endocrinology and Diabetes (K.G., K.E.B., C.M., S.B., C.A.S.), The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104-4399; Department of Pathology (L.M.E.), Northwestern University Feinberg School of Medicine, Chicago, Illinois 60611; Department of Surgery (N.S.A.), The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104-4399; and Departments of Surgery (N.S.A.), Genetics (A.G.), and Pediatrics (C.A.S.), The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania 19104.

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http://dx.doi.org/10.1210/jc.2015-2914DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4803165PMC
March 2016

Perspective on the Genetics and Diagnosis of Congenital Hyperinsulinism Disorders.

J Clin Endocrinol Metab 2016 Mar 23;101(3):815-26. Epub 2016 Feb 23.

Division of Endocrinology, The Children's Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania 19104.

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http://dx.doi.org/10.1210/jc.2015-3651DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4803157PMC
March 2016

Biomarkers of Insulin for the Diagnosis of Hyperinsulinemic Hypoglycemia in Infants and Children.

J Pediatr 2016 Jan 17;168:212-9. Epub 2015 Oct 17.

Division of Endocrinology and Diabetes, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA. Electronic address:

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http://dx.doi.org/10.1016/j.jpeds.2015.09.045DOI Listing
January 2016

Congenital hyperinsulinism in children with paternal 11p uniparental isodisomy and Beckwith-Wiedemann syndrome.

J Med Genet 2016 Jan 6;53(1):53-61. Epub 2015 Nov 6.

Division of Human Genetics, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA Department of Pediatrics, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA.

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http://dx.doi.org/10.1136/jmedgenet-2015-103394DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4740975PMC
January 2016

High Risk of Diabetes and Neurobehavioral Deficits in Individuals With Surgically Treated Hyperinsulinism.

J Clin Endocrinol Metab 2015 Nov 1;100(11):4133-9. Epub 2015 Sep 1.

Division of Endocrinology and Diabetes (K.L., C.A.S., D.D.D.L.), The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104; Department of Pediatrics (K.L., J.R., C.A.S., D.D.D.L.), The Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania 19104; Biostatistics Core (P.R.G.), The Clinical and Translational Research Center, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104; and Department of Surgery (N.S.A.), The Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania 19104.

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https://academic.oup.com/jcem/article-lookup/doi/10.1210/jc.
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http://dx.doi.org/10.1210/jc.2015-2539DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4702456PMC
November 2015

Glutamate dehydrogenase: structure, allosteric regulation, and role in insulin homeostasis.

Neurochem Res 2014 12;39(3):433-45. Epub 2013 Oct 12.

Donald Danforth Plant Science Center, 975 North Warson Road, Saint Louis, MO, 63132, USA.

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http://dx.doi.org/10.1007/s11064-013-1173-2DOI Listing
November 2014

50 years ago in the Journal of pediatrics: Ketotic hypoglycemia.

J Pediatr 2014 Jun;164(6):1310

Division of Endocrinology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.

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http://dx.doi.org/10.1016/j.jpeds.2013.11.028DOI Listing
June 2014

Determination of insulin for the diagnosis of hyperinsulinemic hypoglycemia.

Best Pract Res Clin Endocrinol Metab 2013 Dec 9;27(6):763-9. Epub 2013 Jul 9.

Division of Pediatric Endocrinology, Children's Hospital of Philadelphia, Philadelphia, USA; Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, USA. Electronic address:

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http://dx.doi.org/10.1016/j.beem.2013.06.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4141553PMC
December 2013

Pancreatic surgery in infants with Beckwith-Wiedemann syndrome and hyperinsulinism.

J Pediatr Surg 2013 Dec;48(12):2511-6

Department of Surgery, Children's Hospital of Philadelphia, Philadelphia, PA, USA; Center for Congenital Hyperinsulinism, Children's Hospital of Philadelphia, Philadelphia, PA, USA.

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http://dx.doi.org/10.1016/j.jpedsurg.2013.05.016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4140566PMC
December 2013

The surgical management of insulinomas in children.

J Pediatr Surg 2013 Dec;48(12):2517-24

The Congenital Hyperinsulinism Center and the Department of Surgery at the Children's Hospital of Philadelphia, and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.

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http://dx.doi.org/10.1016/j.jpedsurg.2013.04.022DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4140562PMC
December 2013

Hypertrophic cardiomyopathy in neonates with congenital hyperinsulinism.

Arch Dis Child Fetal Neonatal Ed 2013 Jul 1;98(4):F351-4. Epub 2013 Feb 1.

Division of Endocrinology & Diabetes, Department of Pediatrics, The Children's Hospital of Philadelphia, The University of Pennsylvania School of Medicine, 34th and Civic Center Boulevard, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1136/archdischild-2012-302546DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3683355PMC
July 2013

Accuracy of PET/CT Scan in the diagnosis of the focal form of congenital hyperinsulinism.

J Pediatr Surg 2013 Feb;48(2):388-93

The Congenital Hyperinsulinism Center, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

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http://dx.doi.org/10.1016/j.jpedsurg.2012.11.025DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3597386PMC
February 2013

Measurement of tissue acyl-CoAs using flow-injection tandem mass spectrometry: acyl-CoA profiles in short-chain fatty acid oxidation defects.

Mol Genet Metab 2012 Dec 18;107(4):679-83. Epub 2012 Oct 18.

Division of Endocrinology, Department of Pediatrics, Children's Hospital of Philadelphia and Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1016/j.ymgme.2012.10.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3600647PMC
December 2012

Novel presentations of congenital hyperinsulinism due to mutations in the MODY genes: HNF1A and HNF4A.

J Clin Endocrinol Metab 2012 Oct 16;97(10):E2026-30. Epub 2012 Jul 16.

Clinical and Translational Research Center, The Children's Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania School of Medicine, 3615 Civic Center Boulevard, Abramson Research Center Room 802A, Philadelphia, Pennsylvania 19104, USA.

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http://dx.doi.org/10.1210/jc.2012-1356DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3674296PMC
October 2012

The structure and allosteric regulation of mammalian glutamate dehydrogenase.

Arch Biochem Biophys 2012 Mar 4;519(2):69-80. Epub 2011 Nov 4.

Donald Danforth Plant Science Center, 975 North Warson Road, Saint Louis, MO 63132, USA.

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http://dx.doi.org/10.1016/j.abb.2011.10.015DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3294041PMC
March 2012

Glucokinase activation repairs defective bioenergetics of islets of Langerhans isolated from type 2 diabetics.

Am J Physiol Endocrinol Metab 2012 Jan 27;302(1):E87-E102. Epub 2011 Sep 27.

Department of Biochemistry and Biophysics, University of Pennsylvania, 415 Curie Blvd., Philadelphia, PA 19104-6140, USA.

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http://dx.doi.org/10.1152/ajpendo.00218.2011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3328091PMC
January 2012

The structure and allosteric regulation of glutamate dehydrogenase.

Neurochem Int 2011 Sep 9;59(4):445-55. Epub 2010 Nov 9.

Donald Danforth Plant Science Center, Saint Louis, MO 63132, United States.

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http://dx.doi.org/10.1016/j.neuint.2010.10.017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3135769PMC
September 2011

Two genetic forms of hyperinsulinemic hypoglycemia caused by dysregulation of glutamate dehydrogenase.

Neurochem Int 2011 Sep 2;59(4):465-72. Epub 2010 Dec 2.

University of Pennsylvania School of Medicine, Endocrinology Division, The Children's Hospital of Philadelphia, Philadelphia, PA 19026, United States.

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http://dx.doi.org/10.1016/j.neuint.2010.11.017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3081417PMC
September 2011

Green tea polyphenols control dysregulated glutamate dehydrogenase in transgenic mice by hijacking the ADP activation site.

J Biol Chem 2011 Sep 3;286(39):34164-74. Epub 2011 Aug 3.

Division of Endocrinology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA.

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http://dx.doi.org/10.1074/jbc.M111.268599DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3190766PMC
September 2011

Diazoxide-unresponsive congenital hyperinsulinism in children with dominant mutations of the β-cell sulfonylurea receptor SUR1.

Diabetes 2011 Jun 2;60(6):1797-804. Epub 2011 May 2.

Division of Endocrinology/Diabetes, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

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http://dx.doi.org/10.2337/db10-1631DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3114386PMC
June 2011

Hepatocyte nuclear factor 4α gene mutation associated with familial neonatal hyperinsulinism and maturity-onset diabetes of the young.

J Pediatr 2011 May 24;158(5):852-4. Epub 2011 Feb 24.

Division of Pediatric Endocrinology and Metabolism, Rhode Island Hospital and Brown University, Providence, RI, USA.

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https://linkinghub.elsevier.com/retrieve/pii/S00223476110001
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http://dx.doi.org/10.1016/j.jpeds.2011.01.003DOI Listing
May 2011

Nesidioblastosis no longer! It's all about genetics.

J Clin Endocrinol Metab 2011 Mar;96(3):617-9

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http://dx.doi.org/10.1210/jc.2011-0164DOI Listing
March 2011

A specialized team approach to diagnosis and medical versus surgical treatment of infants with congenital hyperinsulinism.

Semin Pediatr Surg 2011 Feb;20(1):32-7

Division of Endocrinology and Diabetes, The Children's Hospital of Philadelphia, University of Pennsylvania, School of Medicine, Philadelphia, Pennsylvania 19104, USA.

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http://dx.doi.org/10.1053/j.sempedsurg.2010.10.008DOI Listing
February 2011

Rare forms of congenital hyperinsulinism.

Semin Pediatr Surg 2011 Feb;20(1):38-44

Department of General Pediatrics, University Children's Hospital Düsseldorf, Germany.

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http://dx.doi.org/10.1053/j.sempedsurg.2010.10.006DOI Listing
February 2011

Pregnancy in glycogen storage disease type Ib: gestational care and report of first successful deliveries.

J Inherit Metab Dis 2010 Dec 13;33 Suppl 3:S151-7. Epub 2010 Apr 13.

Glycogen Storage Disease Program and Division of Pediatric Endocrinology, University of Florida College of Medicine, PO Box 100296, Gainesville, FL 32610-0296, USA.

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http://dx.doi.org/10.1007/s10545-010-9054-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3800278PMC
December 2010

The hyperinsulinism/hyperammonemia syndrome.

Rev Endocr Metab Disord 2010 Sep;11(3):171-8

The Children's Hospital of Philadelphia, Division of Endocrinology and Diabetes, University of Pennsylvania, School of Medicine, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1007/s11154-010-9146-0DOI Listing
September 2010

Intussusception after pancreatic surgery in children: a case series.

J Pediatr Surg 2010 Jul;45(7):1496-9

Department of Surgery, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1016/j.jpedsurg.2009.09.021DOI Listing
July 2010

Research initiatives in neonatal hypoglycemia.

J Pediatr 2010 May 20;156(5):862-3; author reply 863. Epub 2010 Mar 20.

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http://dx.doi.org/10.1016/j.jpeds.2010.01.048DOI Listing
May 2010

Necrotizing enterocolitis in neonates receiving octreotide for the management of congenital hyperinsulinism.

Pediatr Diabetes 2010 Mar 25;11(2):142-7. Epub 2009 Jun 25.

Department of Surgery, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1111/j.1399-5448.2009.00547.xDOI Listing
March 2010

Characterization and functional restoration of a potassium channel Kir6.2 pore mutation identified in congenital hyperinsulinism.

J Biol Chem 2010 Feb 23;285(9):6012-23. Epub 2009 Dec 23.

Center for Research on Occupational and Environmental Toxicology, Oregon Health and Science University, Portland, Oregon 97239, USA.

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http://dx.doi.org/10.1074/jbc.M109.085860DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2825395PMC
February 2010

Regulation of glutamate metabolism and insulin secretion by glutamate dehydrogenase in hypoglycemic children.

Am J Clin Nutr 2009 Sep 22;90(3):862S-866S. Epub 2009 Jul 22.

The Children's Hospital of Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.3945/ajcn.2009.27462AADOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3136010PMC
September 2009

Sulfonylurea receptor 1 mutations that cause opposite insulin secretion defects with chemical chaperone exposure.

J Biol Chem 2009 Mar 16;284(12):7951-9. Epub 2009 Jan 16.

Center for Research on Occupational and Environmental Toxicology, Oregon Health & Science University, Portland, Oregon 97239, USA.

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http://dx.doi.org/10.1074/jbc.M807012200DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2658088PMC
March 2009

Neurological aspects in hyperinsulinism-hyperammonaemia syndrome.

Dev Med Child Neurol 2008 Dec;50(12):888

Division of Endocrinology/Diabetes, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, USA.

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http://dx.doi.org/10.1111/j.1469-8749.2008.03149.xDOI Listing
December 2008

Untangling the glutamate dehydrogenase allosteric nightmare.

Trends Biochem Sci 2008 Nov 24;33(11):557-64. Epub 2008 Sep 24.

Donald Danforth Plant Science Center, Saint Louis, MO 63132, USA.

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http://dx.doi.org/10.1016/j.tibs.2008.07.007DOI Listing
November 2008

Exendin-(9-39) corrects fasting hypoglycemia in SUR-1-/- mice by lowering cAMP in pancreatic beta-cells and inhibiting insulin secretion.

J Biol Chem 2008 Sep 17;283(38):25786-93. Epub 2008 Jul 17.

Division of Endocrinology, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA.

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http://dx.doi.org/10.1074/jbc.M804372200DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3258866PMC
September 2008

Poor specificity of low growth hormone and cortisol levels during fasting hypoglycemia for the diagnoses of growth hormone deficiency and adrenal insufficiency.

Pediatrics 2008 Sep 11;122(3):e522-8. Epub 2008 Aug 11.

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.

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http://dx.doi.org/10.1542/peds.2008-0806DOI Listing
September 2008

Clinical characteristics and biochemical mechanisms of congenital hyperinsulinism associated with dominant KATP channel mutations.

J Clin Invest 2008 Aug;118(8):2877-86

Division of Endocrinology/Diabetes, The Children's Hospital of Philadelphia, Department of Genetics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA.

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http://dx.doi.org/10.1172/JCI35414DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2441858PMC
August 2008

Destabilization of ATP-sensitive potassium channel activity by novel KCNJ11 mutations identified in congenital hyperinsulinism.

J Biol Chem 2008 Apr 4;283(14):9146-56. Epub 2008 Feb 4.

Center for Research on Occupational and Environmental Toxicology, Oregon Health and Science University, 3181 S.W. Sam Jackson Park Road, Portland, OR 97239, USA.

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http://dx.doi.org/10.1074/jbc.M708798200DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2431039PMC
April 2008

Hyperinsulinism in infancy and childhood: when an insulin level is not always enough.

Clin Chem 2008 Feb 21;54(2):256-63. Epub 2007 Dec 21.

The Children's Hospital of Philadelphia, Division of Endocrinology, Philadelphia, PA, USA.

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http://dx.doi.org/10.1373/clinchem.2007.098988DOI Listing
February 2008

Accuracy of [18F]fluorodopa positron emission tomography for diagnosing and localizing focal congenital hyperinsulinism.

J Clin Endocrinol Metab 2007 Dec 25;92(12):4706-11. Epub 2007 Sep 25.

Division of Endocrinology, The Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, Pennsylvania 19104, USA.

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http://dx.doi.org/10.1210/jc.2007-1637DOI Listing
December 2007

Congenital hyperinsulinism associated ABCC8 mutations that cause defective trafficking of ATP-sensitive K+ channels: identification and rescue.

Diabetes 2007 Sep 15;56(9):2339-48. Epub 2007 Jun 15.

Center for Research on Occupational and Environmental Toxicology, Oregon Health and Science University, Portland, OR 97239, USA.

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http://dx.doi.org/10.2337/db07-0150DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2225993PMC
September 2007

A mutation in the TMD0-L0 region of sulfonylurea receptor-1 (L225P) causes permanent neonatal diabetes mellitus (PNDM).

Diabetes 2007 May 22;56(5):1357-62. Epub 2007 Feb 22.

Department of Cell Biology and Physiology, Washington University School of Medicine, St. Louis, MO 63110, USA.

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http://diabetes.diabetesjournals.org/cgi/doi/10.2337/db06-17
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http://dx.doi.org/10.2337/db06-1746DOI Listing
May 2007

Diagnosis and localization of focal congenital hyperinsulinism by 18F-fluorodopa PET scan.

J Pediatr 2007 Feb;150(2):140-5

Division of Endocrinology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA.

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http://dx.doi.org/10.1016/j.jpeds.2006.08.028DOI Listing
February 2007

Prenatal diagnosis and postnatal management of diffuse congenital hyperinsulinism: a case report.

Fetal Diagn Ther 2006 12;21(6):515-8. Epub 2006 Sep 12.

The Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, The University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1159/000095664DOI Listing
January 2007

Mechanisms of Disease: advances in diagnosis and treatment of hyperinsulinism in neonates.

Nat Clin Pract Endocrinol Metab 2007 Jan;3(1):57-68

University of Pennsylvania, Philadelphia, PA, USA.

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http://www.nature.com/articles/ncpendmet0368
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http://dx.doi.org/10.1038/ncpendmet0368DOI Listing
January 2007

Hypoglycemia in the neonate.

Pediatr Endocrinol Rev 2006 Dec;4 Suppl 1:76-81

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.

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December 2006

The diagnosis of ectopic focal hyperinsulinism of infancy with [18F]-dopa positron emission tomography.

J Clin Endocrinol Metab 2006 Aug 9;91(8):2839-42. Epub 2006 May 9.

London Center for Pediatric Endocrinology and Metabolism, Hospital for Children National Health Service Trust, London WC1N 3JH, United Kingdom.

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http://dx.doi.org/10.1210/jc.2006-0455DOI Listing
August 2006

Protein-sensitive hypoglycemia without leucine sensitivity in hyperinsulinism caused by K(ATP) channel mutations.

J Pediatr 2006 Jul;149(1):47-52

Division of Endocrinology/Diabetes, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

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http://dx.doi.org/10.1016/j.jpeds.2006.02.033DOI Listing
July 2006

Effects of a GTP-insensitive mutation of glutamate dehydrogenase on insulin secretion in transgenic mice.

J Biol Chem 2006 Jun 30;281(22):15064-72. Epub 2006 Mar 30.

Division of Endocrinology, The Children's Hospital of Philadelphia and Diabetes Center, Philadelphia, Pennsylvania 19104, USA.

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http://dx.doi.org/10.1074/jbc.M600994200DOI Listing
June 2006

Green tea polyphenols modulate insulin secretion by inhibiting glutamate dehydrogenase.

J Biol Chem 2006 Apr 13;281(15):10214-21. Epub 2006 Feb 13.

Donald Danforth Plant Science Center, 975 Warson Road, St. Louis, MO 63132, USA.

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http://www.jbc.org/lookup/doi/10.1074/jbc.M512792200
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http://dx.doi.org/10.1074/jbc.M512792200DOI Listing
April 2006

A novel KCNJ11 mutation associated with congenital hyperinsulinism reduces the intrinsic open probability of beta-cell ATP-sensitive potassium channels.

J Biol Chem 2006 Feb 6;281(5):3006-12. Epub 2005 Dec 6.

Center for Research on Occupational and Environmental Toxicology, Oregon Health and Science University, Portland, Oregon 97239, USA.

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http://dx.doi.org/10.1074/jbc.M511875200DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1479853PMC
February 2006

Clinical features and insulin regulation in infants with a syndrome of prolonged neonatal hyperinsulinism.

J Pediatr 2006 Feb;148(2):207-12

Divisions of Endocrinology and Neonatology, Department of Pediatrics, Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1016/j.jpeds.2005.10.002DOI Listing
February 2006

Molecular and immunohistochemical analyses of the focal form of congenital hyperinsulinism.

Mod Pathol 2006 Jan;19(1):122-9

Department of Pathology and Laboratory Medicine, The Childrens' Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, PA, USA.

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http://dx.doi.org/10.1038/modpathol.3800497DOI Listing
January 2006

Effects of hypoglycemia on developmental outcome in children with congenital hyperinsulinism.

J Pediatr Nurs 2005 Apr;20(2):109-18

Department of Endocrinology, The Children's Hospital of Philadephia, PA 19104, USA.

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http://dx.doi.org/10.1016/j.pedn.2004.12.009DOI Listing
April 2005

Central nervous system hyperexcitability associated with glutamate dehydrogenase gain of function mutations.

J Pediatr 2005 Mar;146(3):388-94

Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, USA.

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http://dx.doi.org/10.1016/j.jpeds.2004.10.040DOI Listing
March 2005

Genotype-phenotype correlations in children with congenital hyperinsulinism due to recessive mutations of the adenosine triphosphate-sensitive potassium channel genes.

J Clin Endocrinol Metab 2005 Feb 23;90(2):789-94. Epub 2004 Nov 23.

Division of Endocrinology/Diabetes, The Children's Hospital of Philadelphia, Pennsylvania 19104, USA.

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http://dx.doi.org/10.1210/jc.2004-1604DOI Listing
February 2005

Evolution of glutamate dehydrogenase regulation of insulin homeostasis is an example of molecular exaptation.

Biochemistry 2004 Nov;43(45):14431-43

The Donald Danforth Plant Science Center, 975 North Warson Road, St. Louis, Missouri 63132, USA.

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http://dx.doi.org/10.1021/bi048817iDOI Listing
November 2004

Carnitine deficiency disorders in children.

Ann N Y Acad Sci 2004 Nov;1033:42-51

Division of Endocrinology, The Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA.

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http://doi.wiley.com/10.1196/annals.1320.004
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http://dx.doi.org/10.1196/annals.1320.004DOI Listing
November 2004

Medications used in the treatment of hypoglycemia due to congenital hyperinsulinism of infancy (HI).

Pediatr Endocrinol Rev 2004 Nov;2 Suppl 1:163-7

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.

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November 2004

Congenital hyperinsulinism: intraoperative biopsy interpretation can direct the extent of pancreatectomy.

Am J Surg Pathol 2004 Oct;28(10):1326-35

Department of Pathology and Laboratory Medicine, The Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, 5203 34th Street & Civic Center Boulevard, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1097/01.pas.0000138000.61897.32DOI Listing
October 2004

Familial leucine-sensitive hypoglycemia of infancy due to a dominant mutation of the beta-cell sulfonylurea receptor.

J Clin Endocrinol Metab 2004 Sep;89(9):4450-6

Division of Endocrinology, Children's Hospital of Philadelphia, Abramson Research Center, Room 802, 3615 Civic Center Boulevard, Philadelphia, Pennsylvania 19104-4318, USA.

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http://dx.doi.org/10.1210/jc.2004-0441DOI Listing
September 2004

Hyperinsulinism/hyperammonemia syndrome: insights into the regulatory role of glutamate dehydrogenase in ammonia metabolism.

Mol Genet Metab 2004 Apr;81 Suppl 1:S45-51

Division of Endocrinology, Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1016/j.ymgme.2003.10.013DOI Listing
April 2004

A multidisciplinary approach to the focal form of congenital hyperinsulinism leads to successful treatment by partial pancreatectomy.

J Pediatr Surg 2004 Mar;39(3):270-5

Department of Surgery, and The Center for Hyperinsulinism, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1016/j.jpedsurg.2003.11.019DOI Listing
March 2004