Results 301 to 310 of about 67,076 (344)

Dietary management of Type I glycogen storage disease

Journal of the American Dietetic Association, 1984
The most commonly recognized type of glycogen storage disease (von Gierke's disease) results from deficient glucose-6-phosphatase activity. This enzyme is the last step in the release of free glucose from the liver into the circulation. Thus, the most prominent and life-threatening complication in the illness is severe and often prolonged hypoglycemia,
Harry L. Greene, Cathy C. Folk
openaire   +3 more sources

Glycogen storage disease type I in Tunisia: An epidemiological analysis

Journal of Inherited Metabolic Disease, 2008
SummaryObjective: Analysis of epidemiological data concerning GSD I in Tunisia. Subjects and methods: All the cases diagnosed as GSD I between 1992 and 2005 in a paediatric department recruiting all the metabolic diseases referred from the North of Tunisia were reviewed. Individual data (sex, socioeconomic and educational background, geographic origins,
H. Ben Turkia, Hatem Azzouz, Neji Tebib, Wafa Cherif, A. Ben Chehida, S. Abdelmoula, M. Ben Dridi   +6 more
openaire   +3 more sources

Ischemic stroke in an adult with glycogen storage disease type I

Journal of Clinical Neuroscience, 2010
Glycogen Storage Disease Type I (GSD-I) is a metabolic disorder characterized by deficiency of glucose-6-phosphatase resulting in ineffective glycogen metabolism to glucose. These patients frequently have hyperlipidemia, among many other metabolic derangements. There is no consensus regarding the risk of developing atherosclerosis.
Stanley Tuhrim, Bradley N. Delman, J.M. Goulart, J.Y. Yoo, Kathryn F. Kirchoff-Torres   +4 more
openaire   +3 more sources

Dental management of a patient with glycogen storage disease type I

The Journal of the American Dental Association, 1985
Patients with glycogen storage disease type I (GSD I) have varied medical problems, including a bleeding diathesis characterized by a prolonged bleeding time. A case of a patient with GSD I having a history of medical problems, some of which were related to oral and dental bleeding, was presented. The patient was cariesfree and had generalized moderate
Edward C. Marshall, Stephen A. Ralls, Stephen A. Ralls   +2 more
openaire   +3 more sources

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 ...
Samuel M. Wentworth, Richard C. Powell, Ira K. Brandt   +2 more
openaire   +3 more sources

The use of alanine, free carnitine and IGFBP-1 as potential biomarkers for glycogen storage disease type I

Molecular Genetics and Metabolism, 2022
H. Saavedra, A. Yu, DF Rodriguez-Buritica   +2 more
semanticscholar   +1 more source

Glycogen storage disease type I: diagnosis, management, clinical course and outcome. Results of the European study on glycogen storage disease type I (ESGSD I)

European Journal of Pediatrics, 2002
J. Rake, G. Visser, P. Labrune, J. Leonard, K. Ullrich, P. G. Smit   +5 more
semanticscholar   +1 more source

[Advances on the management of renal lesion in glycogen storage disease type I].

Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology, 2021
W. Wu, J. S. Wang
semanticscholar   +1 more source

Glycogen storage disease type I patients with hyperlipidemia have no signs of early vascular dysfunction and premature atherosclerosis.

NMCD. Nutrition Metabolism and Cardiovascular Diseases, 2021
J. Schmitt, Michael Wurm, K. Schwab,, U. Spiekerkoetter, L. Hannibal, S. C. Grünert   +5 more
semanticscholar   +1 more source

Onconephrology: The intersections between the kidney and cancer

Ca-A Cancer Journal for Clinicians, 2021
Mitchell H Rosner, Kenar D Jhaveri, Mark A Perazella   +2 more
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