Linda Ernst

Summary

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

Publications

  1. doi request reprint Congenital Hypoglycemia Disorders: New Aspects of Etiology, Diagnosis, Treatment and Outcomes: Highlights of the Proceedings of the Congenital Hypoglycemia Disorders Symposium, Philadelphia April 2016
    Diva D De Leon
    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
    Pediatr Diabetes . 2016
  2. pmc Clinical presentation and management of children with diffuse and focal hyperinsulinism: a review of 223 cases
    Katherine Lord
    MD, Division of Endocrinology and Diabetes, The Children s Hospital of Philadelphia, 3615 Civic Center Boulevard, Abramson Research Center Room 802A, Philadelphia, Pennsylvania 19104
    J Clin Endocrinol Metab 98:E1786-9. 2013
  3. pmc Determination of insulin for the diagnosis of hyperinsulinemic hypoglycemia
    Diva D De Leon
    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
    Best Pract Res Clin Endocrinol Metab 27:763-9. 2013
  4. pmc GLP-1 receptor antagonist exendin-(9-39) elevates fasting blood glucose levels in congenital hyperinsulinism owing to inactivating mutations in the ATP-sensitive K+ channel
    Andrew C Calabria
    Division of Endocrinology, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
    Diabetes 61:2585-91. 2012
  5. 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
  6. doi request reprint A case report of rapid progressive coarctation and severe middle aortic syndrome in an infant with Williams syndrome
    E Kevin Hall
    Division of Cardiology, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Congenit Heart Dis 4:373-7. 2009
  7. pmc Neonatal diabetes and congenital malabsorptive diarrhea attributable to a novel mutation in the human neurogenin-3 gene coding sequence
    Sara E Pinney
    Division of Endocrinology and Diabetes, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    J Clin Endocrinol Metab 96:1960-5. 2011
  8. doi request reprint Multiple ruptured cerebral aneurysms in a child with Takayasu arteritis
    Suresh N Magge
    Department of Neurosurgery, Children s Hospital of Philadelphia and the Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    J Neurosurg Pediatr 1:83-7. 2008

Collaborators

Detail Information

Publications8

  1. doi request reprint Congenital Hypoglycemia Disorders: New Aspects of Etiology, Diagnosis, Treatment and Outcomes: Highlights of the Proceedings of the Congenital Hypoglycemia Disorders Symposium, Philadelphia April 2016
    Diva D De Leon
    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
    Pediatr Diabetes . 2016
    ....
  2. pmc Clinical presentation and management of children with diffuse and focal hyperinsulinism: a review of 223 cases
    Katherine Lord
    MD, Division of Endocrinology and Diabetes, The Children s Hospital of Philadelphia, 3615 Civic Center Boulevard, Abramson Research Center Room 802A, Philadelphia, Pennsylvania 19104
    J Clin Endocrinol Metab 98:E1786-9. 2013
    ..Congenital hyperinsulinism (HI) occurs in two distinct histologic forms: diffuse and focal. Distinguishing between them is essential because a pancreatectomy is curative for focal HI and palliative for diffuse HI...
  3. pmc Determination of insulin for the diagnosis of hyperinsulinemic hypoglycemia
    Diva D De Leon
    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
    Best Pract Res Clin Endocrinol Metab 27:763-9. 2013
    ....
  4. pmc GLP-1 receptor antagonist exendin-(9-39) elevates fasting blood glucose levels in congenital hyperinsulinism owing to inactivating mutations in the ATP-sensitive K+ channel
    Andrew C Calabria
    Division of Endocrinology, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
    Diabetes 61:2585-91. 2012
    ..Our findings have two important implications: 1) GLP-1 and its receptor play a role in the regulation of fasting glycemia in K(ATP)HI; and 2) the GLP-1 receptor may be a therapeutic target for the treatment of children with K(ATP)HI...
  5. 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...
  6. doi request reprint A case report of rapid progressive coarctation and severe middle aortic syndrome in an infant with Williams syndrome
    E Kevin Hall
    Division of Cardiology, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Congenit Heart Dis 4:373-7. 2009
    ..We report the case of a newborn infant who had a rapidly progressing diffuse form of arteriopathy that required two surgeries and one percutaneous balloon dilation within the first 2 months of her life...
  7. pmc Neonatal diabetes and congenital malabsorptive diarrhea attributable to a novel mutation in the human neurogenin-3 gene coding sequence
    Sara E Pinney
    Division of Endocrinology and Diabetes, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    J Clin Endocrinol Metab 96:1960-5. 2011
    ..The aim was to describe the clinical presentation and to characterize the genetic mutation present in a child with congenital malabsorptive diarrhea and neonatal diabetes...
  8. doi request reprint Multiple ruptured cerebral aneurysms in a child with Takayasu arteritis
    Suresh N Magge
    Department of Neurosurgery, Children s Hospital of Philadelphia and the Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    J Neurosurg Pediatr 1:83-7. 2008
    ..Aneurysmal subarachnoid hemorrhage is a rare presentation of Takayasu arteritis. To the authors' knowledge, this is the youngest reported patient with Takayasu arteritis to present with a ruptured cerebral aneurysm...

Research Grants2