Primary hyperoxaluria type 1 in Tunisian children
Joint Authors
Khalil, Nourchene
Yusuf, Gharbi
Karoui, Wiem
Abd al-Mawla, Juwaydah
Gargah, Tahar
Lakhoua, Muhammad Rashid
Source
Saudi Journal of Kidney Diseases and Transplantation
Issue
Vol. 23, Issue 2 (30 Apr. 2012), pp.385-390, 6 p.
Publisher
Saudi Center for Organ Transplantation
Publication Date
2012-04-30
Country of Publication
Saudi Arabia
No. of Pages
6
Main Subjects
Topics
Abstract EN
To determine the clinical, biological, and radiological futures of primary hyperoxaluria type 1 in Tunisian children, we retrospectively studied 44 children with primary hyperoxaluria type 1 who were treated in our center from 1995 to 2009.
The diagnosis was established by quantitative urinary oxalate excretion.
In patients with renal impairment, the diagnosis was made by infrared spectroscopy of stones or kidney biopsies.
The male-to-female ratio was 1 : 2.
The median age at diagnosis was 5.75 years.
About 43 % of the patients were diagnosed before the age of five years with initial symptoms dominated by uremia.
Four patients were asymptomatic and diagnosed by sibling screenings of known patients.
Nephrocalcinosis was present in all the patients; it was cortical in 34 %, medullary in 32 %, and global in 34 %.
At diagnosis, 12 (27 %) children were in end-stage renal disease.
Pyridoxine response, which is defined by a reduction in urine oxalate excretion of 60 % or more, was obtained in 27 % of the cases.
In the majority of patients, the clinical expression of primary hyperoxaluria type 1 was characterized by nephrocalcinosis, urolithiasis, and renal failure; pyridoxine sensitivity was associated with better outcome.
American Psychological Association (APA)
Gargah, Tahar& Khalil, Nourchene& Yusuf, Gharbi& Karoui, Wiem& Lakhoua, Muhammad Rashid& Abd al-Mawla, Juwaydah. 2012. Primary hyperoxaluria type 1 in Tunisian children. Saudi Journal of Kidney Diseases and Transplantation،Vol. 23, no. 2, pp.385-390.
https://search.emarefa.net/detail/BIM-293000
Modern Language Association (MLA)
Gargah, Tahar…[et al.]. Primary hyperoxaluria type 1 in Tunisian children. Saudi Journal of Kidney Diseases and Transplantation Vol. 23, no. 2 (Apr. 2012), pp.385-390.
https://search.emarefa.net/detail/BIM-293000
American Medical Association (AMA)
Gargah, Tahar& Khalil, Nourchene& Yusuf, Gharbi& Karoui, Wiem& Lakhoua, Muhammad Rashid& Abd al-Mawla, Juwaydah. Primary hyperoxaluria type 1 in Tunisian children. Saudi Journal of Kidney Diseases and Transplantation. 2012. Vol. 23, no. 2, pp.385-390.
https://search.emarefa.net/detail/BIM-293000
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references : p. 389-390
Record ID
BIM-293000