CHRONIC RENAL DISEASE IN TYPE I GLYCCGEN STORAGE DISEASE
نویسندگان
چکیده
منابع مشابه
Renal function in glycogen storage disease type I, natural course, and renopreservative effects of ACE inhibition.
BACKGROUND AND OBJECTIVES Renal failure is a major complication in glycogen storage disease type I (GSD I). We studied the natural course of renal function in GSD I patients. We studied differences between patients in optimal and nonoptimal metabolic control and possible renoprotective effects of angiotensin converting enzyme inhibition. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS Thirty-ni...
متن کاملPsychosocial functioning in youth with glycogen storage disease type I.
OBJECTIVE To assess the quality of life and psychosocial functioning among pediatric patients with Glycogen Storage Disease (GSD) types Ia and Ib. METHODS Thirty-one youth with GSD types Ia and Ib and 42 healthy controls participated. Quality of life ratings from the GSD types Ia and Ib sample were compared with a previously reported clinical comparison sample. Children completed measures of ...
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Glycogen storage disease type I (GSDI), an inborn error of carbohydrate metabolism, is caused by defects in the glucose-6-transporter/glucose-6-phosphatase complex, which is essential in glucose homeostasis. Two types exist, GSDIa and GSDIb, each caused by different defects in the complex. GSDIa is characterized by fasting intolerance and subsequent metabolic derangements. In addition to these ...
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STUDIES of haptoglobin, 1-3 group-specific component,3,4 and numerous other products of hepatic synthesis59 have shown that liver homografts permanently retain their original metabolic specificity after transplantation. Consequently, liver transplantation has been regarded for some years4 as a potentially decisive way to treat those inborn errors of metabolism which result partly or completely ...
متن کاملGlycogen storage disease type I: laboratory data and diagnosis.
A study of 20 cases of glycogen storage disease type I has shown differences from the classical picture. Hyperuricemia was observed in fewer than half of the cases. All patients had increased triglycerides in serum, but fewer than two thirds had increased concentrations of total cholesterol. There was a consistent increase of aminotransferases in serum. Many textbooks discuss hyperuricemia, lac...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 1987
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-198704010-01038