title: [Primary hyperoxaluria: Tunisian experience apropos of 24 pediatric cases].
abstract: We report on 24 children (10 girls) presenting with primary hyperoxaluria. The mean age at diagnosis was 6.3 years (range: 3 months-14.8 years). The mean interval between initial symptom and diagnosis was 1.3 year. The average follow-up period was 22 months (range: 1-60 months). At the time of diagnosis the renal function was normal in 6 children, moderately altered in 1 and severely in 17. During the follow-up the renal function remained stable in 6 patients, improved in 2, deteriorated in 4. The 12 patients with end-stage renal disease at diagnosis remained unchanged. Urolithiasis were present in all patients older than 2 years, and in 1 among the 5 infants. Medullary nephrocalcinosis was observed in 3 patients in whom the renal function was preserved. Diffuse nephrocalcinosis was present in all patients with end-stage renal failure. Improvement of renal function was secondary to stone removal in 2 patients. Extracorporeal shock wave lithotripsy performed in 7 patients was efficient only in 3. In 10 patients oxalate bone disease was correlated with both renal function and dialysis duration, whereas retinal involvement noted in 6 patients was not.
authors.affiliation: Service de pédiatrie, Hôpital Charles Nicolle, Tunis.
authors.forename: A
authors.initials: A
authors.lastname: Kamoun
authors.forename: M
authors.initials: M
authors.lastname: Daudon
authors.forename: A
authors.initials: A
authors.lastname: Zghal
authors.forename: L
authors.initials: L
authors.lastname: Lasram
authors.forename: H
authors.initials: H
authors.lastname: Ben Maiz
authors.forename: C
authors.initials: C
authors.lastname: Belkahia
authors.forename: R
authors.initials: R
authors.lastname: Lakhoua
mesh_terms: D000293:Adolescent; D001847:Bone Diseases / Q000150:complications; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D006960:Hyperoxaluria, Primary / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D007223:Infant; D007668:Kidney / Q000503:physiopathology; D007676:Kidney Failure, Chronic / Q000150:complications; D008096:Lithotripsy; D008297:Male; D009397:Nephrocalcinosis / Q000150:complications; D014416:Tunisia; D014545:Urinary Calculi / Q000150:complications / Q000628:therapy