David A Weinstein

David A Weinstein

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David A Weinstein

David A Weinstein

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Cornstarch requirements of the adult glycogen storage disease Ia population: A retrospective review.

J Inherit Metab Dis 2019 Aug 15. Epub 2019 Aug 15.

Glycogen Storage Disease Program, Connecticut Children's Medical Center, Hartford, Connecticut.

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https://onlinelibrary.wiley.com/doi/abs/10.1002/jimd.12160
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http://dx.doi.org/10.1002/jimd.12160DOI Listing
August 2019

Complex patterns of inheritance, including synergistic heterozygosity, in inborn errors of metabolism: Implications for precision medicine driven diagnosis and treatment.

Mol Genet Metab 2019 Jul 19. Epub 2019 Jul 19.

Department of Pediatrics, University of Connecticut School of Medicine, Farmington, CT 06030, United States of America; GSD Program, Connecticut Children's Medical Center, Hartford, CT 06106, United States of America.

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http://dx.doi.org/10.1016/j.ymgme.2019.07.011DOI Listing
July 2019

Long-term safety and efficacy of AAV gene therapy in the canine model of glycogen storage disease type Ia.

J Inherit Metab Dis 2018 11 25;41(6):977-984. Epub 2018 May 25.

Glycogen Storage Disease Program, Department of Pediatrics, University of Connecticut School of Medicine, Farmington, CT, 06030, USA.

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http://link.springer.com/10.1007/s10545-018-0199-7
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http://dx.doi.org/10.1007/s10545-018-0199-7DOI Listing
November 2018

Response letter.

J Inherit Metab Dis 2018 11;41(6):915

University of Connecticut Health Center, Farmington, Connecticut, USA.

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http://link.springer.com/10.1007/s10545-018-0249-1
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http://dx.doi.org/10.1007/s10545-018-0249-1DOI Listing
November 2018

Safety issues associated with dietary management in patients with hepatic glycogen storage disease.

Mol Genet Metab 2018 09 18;125(1-2):79-85. Epub 2018 Jul 18.

Section of Metabolic Diseases, Beatrix Children's Hospital, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. Electronic address:

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http://dx.doi.org/10.1016/j.ymgme.2018.07.004DOI Listing
September 2018

Inborn Errors of Metabolism with Hypoglycemia: Glycogen Storage Diseases and Inherited Disorders of Gluconeogenesis.

Pediatr Clin North Am 2018 04;65(2):247-265

Section of Metabolic Diseases, University of Groningen, University Medical Center Groningen, Beatrix Children's Hospital, Groningen, The Netherlands.

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https://linkinghub.elsevier.com/retrieve/pii/S00313955173018
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http://dx.doi.org/10.1016/j.pcl.2017.11.005DOI Listing
April 2018

Aberrant proliferation and differentiation of glycogen storage disease type Ib mesenchymal stem cells.

FEBS Lett 2018 01 3;592(2):162-171. Epub 2018 Jan 3.

Department of Biotechnology and Bioinformatics, College of Science and Technology, Korea University, Sejong, Korea.

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http://dx.doi.org/10.1002/1873-3468.12939DOI Listing
January 2018

Use of Skin Biopsies Among Dermatologists.

Dermatol Surg 2017 Nov;43(11):1348-1357

*Department of Internal Medicine, University of Central Florida College of Medicine, Orlando, Florida; †TriHealth Good Samaritan Hospital, Cincinnati, Ohio; ‡University of Chicago Pritzker School of Medicine, Chicago, Illinois; §Department of Dermatology, University of Cincinnati College of Medicine, Cincinnati, Ohio; and ‖The Skin Cancer Center, Cincinnati, Ohio.

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http://dx.doi.org/10.1097/DSS.0000000000001188DOI Listing
November 2017

Clinical and biochemical heterogeneity between patients with glycogen storage disease type IA: the added value of CUSUM for metabolic control.

J Inherit Metab Dis 2017 09 10;40(5):695-702. Epub 2017 Apr 10.

Section of Metabolic Diseases, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, PO Box 30 001, Groningen, 9700 RB, The Netherlands.

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http://dx.doi.org/10.1007/s10545-017-0039-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5579135PMC
September 2017

Tight metabolic control plus ACE inhibitor therapy improves GSD I nephropathy.

J Inherit Metab Dis 2017 09 13;40(5):703-708. Epub 2017 Jun 13.

Glycogen Storage Disease Program, University of Florida, Gainesville, FL, USA.

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http://dx.doi.org/10.1007/s10545-017-0054-2DOI Listing
September 2017

Inflammatory Bowel Disease in Glycogen Storage Disease Type Ia.

J Pediatr Gastroenterol Nutr 2017 02;64(2):e52-e54

*Division of Pediatric Gastroenterology, Department of Pediatrics †Glycogen Storage Disease Program, Division of Pediatric Endocrinology, Department of Pediatrics, University of Florida College of Medicine, Gainesville ‡Department of Metabolic Diseases, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.

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http://pdfs.journals.lww.com/jpgn/9000/00000/Inflammatory_Bo
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http://content.wkhealth.com/linkback/openurl?sid=WKPTLP:land
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http://dx.doi.org/10.1097/MPG.0000000000000592DOI Listing
February 2017

50 Years Ago in The Journal of Pediatrics: The Incidence of Neonatal Hypoglycemia-A Completed Survey.

J Pediatr 2017 01;180:74

Glycogen Storage Disease Program University of Florida Gainesville, Florida.

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http://dx.doi.org/10.1016/j.jpeds.2016.08.013DOI Listing
January 2017

Glycogen storage disease type III: diagnosis, genotype, management, clinical course and outcome.

J Inherit Metab Dis 2016 09 22;39(5):697-704. Epub 2016 Apr 22.

Section of Metabolic Diseases, Beatrix Children's Hospital, University of Groningen, University Medical Center Groningen, PO Box 30 001, 9700 RB, Groningen, The Netherlands.

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http://dx.doi.org/10.1007/s10545-016-9932-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4987401PMC
September 2016

BIOPSY 1-2-3 in Dermatologic Surgery: Improving Smartphone use to Avoid Wrong-Site Surgery.

Technol Innov 2016 Sep;18(2-3):203-206

Department of Dermatology, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.

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http://dx.doi.org/10.21300/18.2-3.2016.203DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5218525PMC
September 2016

High Incidence of Serologic Markers of Inflammatory Bowel Disease in Asymptomatic Patients with Glycogen Storage Disease Type Ia.

JIMD Rep 2015 21;24:123-8. Epub 2015 Jun 21.

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

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http://dx.doi.org/10.1007/8904_2015_452DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4582023PMC
September 2015

Disordered Eating and Body Esteem Among Individuals with Glycogen Storage Disease.

JIMD Rep 2015 10;19:23-9. Epub 2015 Feb 10.

Department of Pediatric Endocrinology, University of Florida, Gainesville, FL, USA.

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http://dx.doi.org/10.1007/8904_2014_359DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4501237PMC
July 2015

Evaluation of glycogen storage disease as a cause of ketotic hypoglycemia in children.

J Inherit Metab Dis 2015 May 29;38(3):489-93. Epub 2014 Jul 29.

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

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http://dx.doi.org/10.1007/s10545-014-9744-1DOI Listing
May 2015

In vitro digestion of starches in a dynamic gastrointestinal model: an innovative study to optimize dietary management of patients with hepatic glycogen storage diseases.

J Inherit Metab Dis 2015 May 16;38(3):529-36. Epub 2014 Sep 16.

Post-Graduation Program in Genetics and Molecular Biology, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil.

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http://dx.doi.org/10.1007/s10545-014-9763-yDOI Listing
May 2015

Pregnancy in women with glycogen storage disease Ia and Ib.

J Perinat Neonatal Nurs 2014 Jan-Mar;28(1):26-31

University of Florida, Gainesville, Florida (Ms Ferrecchia); Bard College at Simon's Rock, Great Barrington, Massachusetts (Ms Guenette); UFHealth, Gainesville, Florida (Ms Potocik); and University of Florida College of Medicine, Gainesville (Dr Weinstein).

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http://content.wkhealth.com/linkback/openurl?sid=WKPTLP:land
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http://dx.doi.org/10.1097/JPN.0000000000000017DOI Listing
April 2015

Regression of hepatocellular adenomas with strict dietary therapy in patients with glycogen storage disease type I.

JIMD Rep 2015 12;18:23-32. Epub 2014 Oct 12.

Department of Radiology, University of Florida College of Medicine, Gainesville, FL, USA.

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http://dx.doi.org/10.1007/8904_2014_344DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4361930PMC
March 2015

Hypoglycemia in Prader-Willi syndrome.

Am J Med Genet A 2014 May 23;164A(5):1127-9. Epub 2014 Jan 23.

Pediatric Endocrinology, University of Florida, Gainesville, Florida.

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http://dx.doi.org/10.1002/ajmg.a.36405DOI Listing
May 2014

Molecular mechanisms of neutrophil dysfunction in glycogen storage disease type Ib.

Blood 2014 May 24;123(18):2843-53. Epub 2014 Feb 24.

Section on Cellular Differentiation, Program on Developmental Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD;

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http://www.bloodjournal.org/cgi/doi/10.1182/blood-2013-05-50
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http://dx.doi.org/10.1182/blood-2013-05-502435DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4007611PMC
May 2014

Thyroid storm following radioactive iodine (RAI) therapy for pediatric graves disease.

Am J Case Rep 2014 14;15:212-5. Epub 2014 May 14.

Division of Endocrinology, Department of Pediatrics, University of Florida College of Medicine, Gainesville, FL, U.S.A.

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http://dx.doi.org/10.12659/AJCR.890366DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4026146PMC
May 2014

Glycogen storage disease type Ia: linkage of glucose, glycogen, lactic acid, triglyceride, and uric acid metabolism.

J Clin Lipidol 2012 Nov-Dec;6(6):596-600. Epub 2012 Aug 30.

Cardiovascular Research Clinic and Lipid Metabolism Laboratory, Human Nutrition Research Center on Aging at Tufts University and Tufts University School of Medicine, Boston, MA 02111, USA.

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http://dx.doi.org/10.1016/j.jacl.2012.08.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3812663PMC
July 2013

Aggressive therapy improves cirrhosis in glycogen storage disease type IX.

Mol Genet Metab 2013 Jun 21;109(2):179-82. Epub 2013 Mar 21.

Division of Pediatric and Adolescent Endocrinology, Glycogen Storage Disease Program, Cleveland Clinic Foundation, Cleveland, OH, USA.

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http://dx.doi.org/10.1016/j.ymgme.2013.03.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3672367PMC
June 2013

Characterization and pathogenesis of anemia in glycogen storage disease type Ia and Ib.

Genet Med 2012 Sep 7;14(9):795-9. Epub 2012 Jun 7.

Glycogen Storage Disease Program and Division of Pediatric Endocrinology, Department of Pediatrics, University of Florida, Gainesville, FL, USA.

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http://dx.doi.org/10.1038/gim.2012.41DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3808879PMC
September 2012

Bone mineral density in glycogen storage disease type Ia and Ib.

Genet Med 2013 5;14(8):737-741. Epub 2012 Apr 5.

Glycogen Storage Disease Program and Division of Pediatric Endocrinology, Department of Pediatrics, University of Florida, Gainesville, Florida, USA.

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http://dx.doi.org/10.1038/gim.2012.36DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3884026PMC
April 2012

Natural history of hepatocellular adenoma formation in glycogen storage disease type I.

J Pediatr 2011 Sep 9;159(3):442-6. Epub 2011 Apr 9.

Glycogen Storage Disease Program and Division of Pediatric Endocrinology, Department of Pediatrics, University of Florida, Gainesville, FL 32610-0296, USA.

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http://dx.doi.org/10.1016/j.jpeds.2011.02.031DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3135733PMC
September 2011

Acoustic accessibility investigation for ultrasound mediated treatment of glycogen storage disease type Ia patients.

Ultrasound Med Biol 2011 Sep 20;37(9):1469-77. Epub 2011 Jul 20.

Philips Research North America, Briarcliff Manor, NY, USA.

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http://linkinghub.elsevier.com/retrieve/pii/S030156291101135
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http://dx.doi.org/10.1016/j.ultrasmedbio.2011.06.004DOI Listing
September 2011

Benefits, motivations, and barriers related to environmental volunteerism for older adults: developing a research agenda.

Int J Aging Hum Dev 2011 ;72(3):189-206

Department of Horticulture, Cornell University, Ithaca, New York 14853, USA.

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http://dx.doi.org/10.2190/AG.72.3.bDOI Listing
September 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

Antibodies to CBir1 are associated with glycogen storage disease type Ib.

J Pediatr Gastroenterol Nutr 2010 Jul;51(1):14-8

Division of Pediatric Gastroenterology, Department of Pediatrics, USA.

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http://dx.doi.org/10.1097/MPG.0b013e3181c15f78DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2893276PMC
July 2010

Refractory ulcers: the role of tumor necrosis factor-alpha.

J Am Acad Dermatol 2010 Jul;63(1):146-54

College of Medicine, University of Florida, Gainesville, Florida, USA.

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http://dx.doi.org/10.1016/j.jaad.2009.08.004DOI Listing
July 2010

Chromosomal and genetic alterations in human hepatocellular adenomas associated with type Ia glycogen storage disease.

Hum Mol Genet 2009 Dec 16;18(24):4781-90. Epub 2009 Sep 16.

Division of Medical Genetics, Duke University Medical Center, Durham, NC 27710, USA.

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http://dx.doi.org/10.1093/hmg/ddp441DOI Listing
December 2009

A detailed characterization of the adult mouse model of glycogen storage disease Ia.

Lab Invest 2009 Sep 6;89(9):1032-42. Epub 2009 Jul 6.

Department of Pathology, Immunology and Laboratory Medicine and The Program for Stem Cell Biology and Regenerative Medicine, University of Florida College of Medicine, Gainesville, FL 32610-0275, USA.

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http://dx.doi.org/10.1038/labinvest.2009.64DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3132560PMC
September 2009

Vascular dysfunction in glycogen storage disease type I.

J Pediatr 2009 Apr 21;154(4):588-91. Epub 2008 Dec 21.

Division of Pediatric Endocrinology and Glycogen Storage Disease Program, Department of Pediatrics, University of Florida, Gainesville, FL 32610-0296, USA.

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https://linkinghub.elsevier.com/retrieve/pii/S00223476080095
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http://dx.doi.org/10.1016/j.jpeds.2008.10.048DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3607442PMC
April 2009

Use of modified cornstarch therapy to extend fasting in glycogen storage disease types Ia and Ib.

Am J Clin Nutr 2008 Nov;88(5):1272-6

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

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3808112PMC
http://dx.doi.org/10.3945/ajcn.2008.26352DOI Listing
November 2008

Perioperative management of patients with glycogen storage disease type Ia.

Plast Reconstr Surg 2008 Jul;122(1):42e-43e

Mount Sinai Medical Center, University of Florida College of Medicine, Gainesville, Fla, USA.

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http://dx.doi.org/10.1097/PRS.0b013e31817746daDOI Listing
July 2008

Pregnancies in glycogen storage disease type Ia.

Am J Obstet Gynecol 2008 Jun 1;198(6):646.e1-7. Epub 2008 Feb 1.

Department of Pediatrics, Beatrix Children's Hospital, University Medical Center Groningen, the Netherlands.

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http://dx.doi.org/10.1016/j.ajog.2007.11.050DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3812528PMC
June 2008

Glycogen storage disease type IIIa presenting as non-ketotic hypoglycemia: use of a newly approved commercially available mutation analysis to non-invasively confirm the diagnosis.

J Pediatr Endocrinol Metab 2008 Jun;21(6):587-90

Division of Pediatric Endocrinology, University of Missouri-Columbia School of Medicine, Columbia, MO 65212, USA.

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June 2008

Necrotic foci, elevated chemokines and infiltrating neutrophils in the liver of glycogen storage disease type Ia.

J Hepatol 2008 Mar 28;48(3):479-85. Epub 2007 Dec 28.

Section on Cellular Differentiation, National Institute of Child Health and Human Development, NIH, Bethesda, MD 20892-1830, USA.

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http://linkinghub.elsevier.com/retrieve/pii/S016882780700644
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http://dx.doi.org/10.1016/j.jhep.2007.11.014DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2744599PMC
March 2008

Liver transplantation in children with glycogen storage disease: controversies and evaluation of the risk/benefit of this procedure.

Pediatr Transplant 2008 Mar;12(2):137-45

Division of Pediatric Gastroenterology, Department of Pediatrics, University of Florida College of Medicine, Gainesville, FL 32610-0296, USA.

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http://dx.doi.org/10.1111/j.1399-3046.2007.00803.xDOI Listing
March 2008

Neutrophilia and elevated serum cytokines are implicated in glycogen storage disease type Ia.

FEBS Lett 2007 Aug 16;581(20):3833-8. Epub 2007 Jul 16.

Section on Cellular Differentiation, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892-1830, USA.

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http://dx.doi.org/10.1016/j.febslet.2007.07.013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2553720PMC
August 2007

Urinary hepcidin in congenital chronic anemias.

Pediatr Blood Cancer 2007 Jan;48(1):57-63

Division of Hematology/Oncology, Children's Hospital, Boston, Massachusetts, USA.

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http://dx.doi.org/10.1002/pbc.20616DOI Listing
January 2007

Impaired intestinal iron absorption in Crohn's disease correlates with disease activity and markers of inflammation.

Inflamm Bowel Dis 2006 Dec;12(12):1101-6

Center for Inflammatory Bowel Disease and Divisions of Gastroenterology and Nutrition, Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA.

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http://dx.doi.org/10.1097/01.mib.0000235097.86360.04DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2788427PMC
December 2006

Increased scavenger receptor class B type I-mediated cellular cholesterol efflux and antioxidant capacity in the sera of glycogen storage disease type Ia patients.

Mol Genet Metab 2006 Nov 14;89(3):233-8. Epub 2006 Jun 14.

Section on Cellular Differentiation, Heritable Disorders Branch, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA.

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http://linkinghub.elsevier.com/retrieve/pii/S109671920600180
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http://dx.doi.org/10.1016/j.ymgme.2006.05.002DOI Listing
November 2006

Assessment of ozone effects on nitrate export from Hubbard Brook Watershed 6.

Environ Pollut 2006 May 7;141(1):8-21. Epub 2005 Oct 7.

Boyce Thompson Institute for Plant Research, Tower Road, Ithaca, NY 14853, USA.

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http://dx.doi.org/10.1016/j.envpol.2005.08.030DOI Listing
May 2006

Hepatic glycogen synthase deficiency: an infrequently recognized cause of ketotic hypoglycemia.

Mol Genet Metab 2006 Apr 6;87(4):284-8. Epub 2005 Dec 6.

Division of Pediatric Endocrinology, University of Florida College of Medicine, Gainesville, USA.

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http://dx.doi.org/10.1016/j.ymgme.2005.10.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1474809PMC
April 2006

Mutation frequencies for glycogen storage disease Ia in the Ashkenazi Jewish population.

Am J Med Genet A 2004 Aug;129A(2):162-4

Dor Yeshorim, The Committee for the Prevention of Jewish Genetic Diseases, Brooklyn, New York, USA.

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http://dx.doi.org/10.1002/ajmg.a.30232DOI Listing
August 2004

Glycogen storage diseases.

Rev Endocr Metab Disord 2003 Mar;4(1):95-102

Diabetes Program, Division of Endocrinology, Charles A Janeway Medical Firm, Children's Hospital Boston, Boston 02115, MA, USA.

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March 2003

Effect of continuous glucose therapy with uncooked cornstarch on the long-term clinical course of type 1a glycogen storage disease.

Eur J Pediatr 2002 Oct 31;161 Suppl 1:S35-9. Epub 2002 Jul 31.

Division of Endocrinology, Children's Hospital Boston, 300 Longwood Avenue, Boston MA 02115, USA.

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http://dx.doi.org/10.1007/s00431-002-1000-2DOI Listing
October 2002

Glycogen synthase deficiency (glycogen storage disease type 0) presenting with hyperglycemia and glucosuria: report of three new mutations.

J Pediatr 2002 Jun;140(6):781-3

Children's Hospital Dartmouth and Dartmouth Medical School, Manchester, New Hampshire.

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http://dx.doi.org/10.1067/mpd.2002.124317DOI Listing
June 2002

Modeling changes in red spruce carbon balance and allocation in response to interacting ozone and nutrient stresses.

Tree Physiol 1991 Jul-Sep;9(1_2):127-146

Ecosystems Research Center, Cornell University, Ithaca, NY 14853, USA.

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http://dx.doi.org/10.1093/treephys/9.1-2.127DOI Listing
July 1991