title: Short-term effect of captopril on microalbuminuria in children with glycogen storage disease type Ia.
abstract: Early signs of renal dysfunction in glycogen storage disease type Ia (GSD Ia) are glomerular hyperfiltration and proteinuria. In a non-randomized study, the effect of captopril on the improvement of proteinuria in GSD Ia patients with microalbuminuria was investigated. A positive effect has been shown for the insulin-dependent diabetes mellitus patients. Microalbuminuria was defined as albumin/creatinine ratio (mg/mmol) more than 2.5 in spot urine. Nineteen (52.7%) out of 36 patients had microalbuminuria, and 8 patients received captopril at a dose of 1 mg/kg per day. Microalbuminuria was evaluated periodically during the follow-up period. Of the captopril-treated patients, one was lost to follow-up. In the remaining 7 patients, urinary albumin excretion normalized in 3 patients (42.9%) and decreased at least by 50% in another 3 patients (42.8%) after 6 months of treatment. One patient, who was the oldest, did not have any benefit. In untreated patients, only two patients had a decrease in microalbuminuria of more than 50%. Patients with microalbuminuria had significantly higher blood lactate (p < 0.05) and plasma triglyceride (p < 0.01) concentrations and significantly lower blood bicarbonate concentration (p < 0.05) than those patients without it. Additionally, the patients with microalbuminuria had been diagnosed earlier than those without microalbuminuria (p < 0.05). Patients with microalbuminuria have more severe clinical and laboratory findings than those without microalbuminuria. Captopril at a dose of 1 mg/kg per day seems to be effective in at least 50% of GSD Ia patients with microalbuminuria.
authors.affiliation: Division of Paediatric Gastroenterology, Hacettepe University School of Medicine, Ankara, Turkey. haozen@hacettepe.edu.tr
authors.forename: H
authors.initials: H
authors.lastname: Ozen
authors.forename: G
authors.initials: G
authors.lastname: Ciliv
authors.forename: N
authors.initials: N
authors.lastname: Koçak
authors.forename: I N
authors.initials: IN
authors.lastname: Saltik
authors.forename: A
authors.initials: A
authors.lastname: Yüce
authors.forename: F
authors.initials: F
authors.lastname: Gürakan
mesh_terms: D000293:Adolescent; D000419:Albuminuria / Q000188:drug therapy* / Q000209:etiology*; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000008:administration & dosage / Q000627:therapeutic use*; D002216:Captopril / Q000008:administration & dosage / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000150:complications* / Q000188:drug therapy*; D006801:Humans; D007223:Infant; D008297:Male
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors; D002216:Captopril