Document Detail

Renal function in glycogen storage disease type I, natural course, and renopreservative effects of ACE inhibition.
MedLine Citation:
PMID:  19808227     Owner:  NLM     Status:  MEDLINE    
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-nine GSD I patients that visited our clinic were studied. GFR and effective renal plasma flow (ERPF) were measured by means of I(125) iothalamate and I(131) hippuran clearance and corrected for body surface area. Microalbuminuria was defined as >2.5 mg albumin/mmol creatinine and proteinuria as >0.2 g protein per liter. Optimal metabolic control was present when blood glucoses were >3.5 mmol/L, urine lactate/creatinine ratios <0.06 mmol/mmol, triglycerides <6.0 mmol/L, and uric acid concentrations <450 micromol/L.
RESULTS: Quadratic regression analysis showed a biphasic pattern in the course of GFR and ERPF related to age. Microalbuminuria was observed significantly less frequently in the patients with optimal metabolic control compared with the patients with nonoptimal metabolic control. A significant decrease in GFR was observed after starting ACE inhibition.
CONCLUSIONS: This study describes a biphasic pattern of the natural course of GFR and ERPF in GSD I patients, followed by the development of microalbuminuria and proteinuria. Optimal metabolic control has a renoprotective effect on the development of microalbuminuria and proteinuria in GSD I patients. Treatment with ACE inhibitors significantly decreases the GFR, especially in GSD I patients with glomerular hyperfiltration.
Daniëlle H J Martens; Jan Peter Rake; Gerjan Navis; Vaclav Fidler; Catharina M L van Dael; G Peter A Smit
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Publication Detail:
Type:  Journal Article     Date:  2009-10-01
Journal Detail:
Title:  Clinical journal of the American Society of Nephrology : CJASN     Volume:  4     ISSN:  1555-905X     ISO Abbreviation:  Clin J Am Soc Nephrol     Publication Date:  2009 Nov 
Date Detail:
Created Date:  2009-12-07     Completed Date:  2010-02-17     Revised Date:  2013-05-31    
Medline Journal Info:
Nlm Unique ID:  101271570     Medline TA:  Clin J Am Soc Nephrol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1741-6     Citation Subset:  IM    
University Medical Center Groningen, Department of Pediatrics, Hanzeplein 1, PO Box 30 001, 9700 RB Groningen, The Netherlands.
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MeSH Terms
Albuminuria / diagnosis,  epidemiology,  prevention & control
Angiotensin-Converting Enzyme Inhibitors / therapeutic use*
Child, Preschool
Contrast Media / diagnostic use
Disease Progression
Glomerular Filtration Rate
Glycogen Storage Disease Type I / epidemiology*
Infant, Newborn
Iodine Radioisotopes / diagnostic use
Iodohippuric Acid / diagnostic use
Iothalamic Acid / diagnostic use
Kidney / physiology*
Kidney Failure, Chronic / diagnosis,  epidemiology*,  prevention & control*
Regression Analysis
Renal Circulation
Severity of Illness Index
Reg. No./Substance:
0/Angiotensin-Converting Enzyme Inhibitors; 0/Contrast Media; 0/Iodine Radioisotopes; 147-58-0/Iodohippuric Acid; 2276-90-6/Iothalamic Acid

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