Service interruption on Monday 11 July from 12:30 to 13:00: all the sites of the CCSD (HAL, Epiciences, SciencesConf, AureHAL) will be inaccessible (network hardware connection).
Skip to Main content Skip to Navigation
Journal articles

Complications rénales dans la glycogénose de type 1 : quelles implications pratiques ?

Abstract : Aim. - To investigate risk factors of renal complications in glycogen storage disease type I, in order to identify practical implications for renal preservation. Methods. - A retrospective study of 38 patients with glycogen storage disease type I. Results. - The patients studied were 8.6 years old in average (1.5 to 22 years) and were followed during 7.4 +/- 4.5 years. Hypercalciuria was detected in 23 patients and was related to acidosis (P = 0.028), higher lactate levels (5.9 +/- 3.5 versus 3.7 +/- 1.7 mmol/L; P = 0.013) and smaller height (-2.1 +/- 1.5 SD versus -0.8 +/- 1.5 SD; P = 0.026). Urolithiasis was diagnosed in 7 cases. Glomerular disease (19/38) was more frequent in cases with severe hypertriglyceridemia (P = 0.042) and occurred at an older age (P = 0.007). Microalbuminuria occurred in 15/31 cases; ACE inhibitors were prescribed in only 8 cases. The frequency of renal complications did not differ according to the diet group (continuous enteral feeding or uncooked starch). Logistic regression concluded as risk factors: lactic acidosis for tubular disease and age > 10 years for glomerular disease. Conclusions. - Renal involvement is common in glycogen storage disease type I patients. Tubular abnormalities are precocious, related to lactic acidosis and may be detected by monitoring of urinary calcium. Glomerular hyperfiltration is the first stage of a progressive glomerular disease and is related to age. Practical implications for renal preservation are discussed based on our results and literature. (C) 2015 Association Societe de nephrologie. Published by Elsevier Masson SAS. All rights reserved.
Complete list of metadata
Contributor : Institut Pasteur Tunis Connect in order to contact the contributor
Submitted on : Sunday, October 2, 2016 - 8:02:12 PM
Last modification on : Wednesday, October 28, 2020 - 9:52:04 AM




Amel Ben Chehida, Takoua Bensmaïl, Faten Ben Rehouma, Rim Ben Abdelaziz, Hatem Azzouz, et al.. Complications rénales dans la glycogénose de type 1 : quelles implications pratiques ?. Néphrologie et Thérapeutique, Elsevier Masson, 2015, 11 (4), pp.240 - 245. ⟨10.1016/j.nephro.2014.12.007⟩. ⟨pasteur-01375033⟩



Record views