publications
Article
Complications rénales dans la glycogénose de type 1 : quelles implications pratiques |
Abdelhak Sonia, Azouz Hatem, KAABACHI Naziha, Tebib Neji, 2014 |
Nephrologie & Therapeutique, 11(2015), 2014.12.007, 240-245, Décembre 2014 |
Résumé
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.
Aim. – To investigate risk factors of renal complications in glycogen storage disease type I, in order toidentify 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 during7.4 4.5 years. Hypercalciuria was detected in 23 patients and was related to acidosis (P = 0.028), higherlactate levels (5.9 3.5 versus 3.7 1.7 mmol/L; P = 0.013) and smaller height (2.1 1.5 SD versus0.8 1.5 SD; P = 0.026). Urolithiasis was diagnosed in 7 cases. Glomerular disease (19/38) was morefrequent 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 ofrenal complications did not differ according to the diet group (continuous enteral feeding or uncookedstarch). Logistic regression concluded as risk factors: lactic acidosis for tubular disease and age > 10 years forglomerular disease.
