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

Medicine

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