Results 251 to 260 of about 25,851 (276)

Endogenous glucose production in type I glycogen storage disease

Metabolism, 1981
The adaptive mechanisms that protect some patients with Type I glycogen storage disease from fasting hypoglycemia were examined in two young adults. Both maintained low normal fasting plasma glucose concentrations even during 3 day fasts; blood lactate concentrations increased during the first 12 hr and then decreased to normal during the second and ...
R C, Powell, S M, Wentworth, I K, Brandt
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GLYCOGEN STORAGE DISEASE: TYPE I-Reply

Archives of Pediatrics & Adolescent Medicine, 1969
To the Editor .—Thank you for giving us the opportunity to reply to Dr. Hockman's letter concerning our article which appeared inThe Journal. Our ophthalmoscopic findings in the patients with GSD, type 1 were not consistent with the classical description of lipemic retinalis.
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Dietary dilemmas in the management of glycogen storage disease type I

Journal of Inherited Metabolic Disease, 2011
AbstractOver the last 50 years, understanding the biochemical bases of glycogen storage disease type I has led to vastly improved survival and health outcomes but the management still centres around an extremely intensive dietary regimen. Patients’ metabolic profiles are really determined by the whole of the diet and it can be very difficult to adjust ...
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Type I glycogen storage disease

Type I glycogen storage disease is a disorder of glucose-6-phosphate breakdown, associated with impaired glycogenolysis and gluconeogenesis. It typically presents in infancy with hypoglycaemia, hyperlacticacidaemia, hypertriglyceridaemia, and hepatomegaly.Provision of a continuous glucose source is the mainstay of treatment, often in the form of ...
Derks, Terry G.J., Schreuder, Andrea B., de Boer, Foekje   +2 more
openaire   +1 more source

Glycogen Storage Disease Type I

2009
David J. Timson, Richard J. Reece, James B. Thoden, Hazel M. Holden, Andrea L. Utz, Beverly M. K. Biller, Eugen-Matthias Strehle, Markus Böhm, Thomas A. Luger, Alexander K. C. Leung, Andrew L. Wong, Markus G. Donner, Dieter Häussinger, Nirmal S. Mann, Marcus Schmitt, Alexander K. C. Leung, William Lane M. Robson, Andrew L. Wong, Manisha Balwani, Ainu Prakash-Cheng*, Robert J. Desnick, Holger Lerche, Ingrid E. Scheffer, Samuel F. Berkovic, Brigitte M. Lobnig, Peter L. M. Jansen, Siegfried Waldegger, Florian Lang, Hugo ten Cate, Hagen Thieme, Michael Weller, Bärbel Schütte, Ronnie Fass, Hideki Kato, Masaomi Nangaku, Hideki Kato, Masaomi Nangaku, Anthony Chang, Pradeep V. Kadambi, Jessy J. Alexander, Pierre Ronco, Hanna Debiec, Christian Hugo, Brigitte M. Lobnig, Brigitte M. Lobnig, John-John B. Schnog, Victor E. A. Gerdes, Jean Francis, David S. Geller, Knut Brockmann, Dong Wang, Darryl C. Vivo, Stefan Kölker, Stefan Kölker, Elardus Erasmus, Lodewyk J. Mienie, John Vissing, Arnold Reuser, Catherine E. Correia, David A. Weinstein, Joseph I. Wolfsdorf, Yoon S. Shin, Reinout P. Hesselink, Maarten R. Drost, Anton J. M. Wagenmakers, Ger J. Vusse, Stefan Kiechl, Jonas Denecke, Ute Schepers, Thomas Kolter, Konrad Sandhoff, Dianna C. Martin, Barbara L. Triggs-Raine, Seda Sancak, Ralf Paschke, Dorin-Bogdan Borza, Sonja Ständer, Heiko Traupe, Michael A. Becker, Hanneke M. Straaten, Leo F. Verdonck, Hugo Ten Cate, Martin Mempel, Dietrich Abeck, Ismat Ghanem, Samer El Hage, Johannes G. Bode, Thomas A. Fleisher, Steven M. Holland, Bennett Myers, David Herrmann, Zaki Kraiem, Yaron Tomer, Terry F. Davies, James G. White, Sylvia Stöckler-Ipsiroglu, Guido Stoll, Klaus V. Toyka, Valéria Lamounier-Zepter, Klaus Ruether   +99 more
openaire   +1 more source

Glycogen Storage Disease Type I

New England Journal of Medicine, 1986
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Peliosis hepatis with type I glycogen storage disease

Journal of Inherited Metabolic Disease, 1991
H, Schmidt, K, Ullrich, H J, von Lengerke, P E, Peters   +3 more
openaire   +2 more sources

Glycogen Storage Disease Type III diagnosis and management guidelines

Genetics in Medicine, 2010
Priya S Kishnani, Stephanie L Austin, Pamela Arn   +2 more
exaly  

Glycogen storage disease type II: enzymatic screening in dried blood spots on filter paper

Clinica Chimica Acta, 2004
Mariana Blanco
exaly  

Hepatocyte Transplantation for Glycogen Storage Disease Type Ib

Cell Transplantation, 2007
Kwang-Woong Lee, Ji-Hyun Lee, Sung Wook Shin   +2 more
exaly