Primary hyperoxaluria (PH) is a rare autosomal recessive inherited metabolic
problem. It is a heterogeneous disease with variable age of onset and variable prognosis. The
clinical presentations range from renal failure during infancy to asymptomatic cases during
adulthood. To determine the pattern of PH in a group of Libyan children at Tripoli children's
hospital. We reviewed the medical records of all children diagnosed as PH from 1994 up to
2007 in nephrology unit at Tripoli children’s hospital. 36 patients (17 male and 19 female)
diagnosed as primary hyperoxaluria during the study period. Their age of presentation was 3.9
± 3.7 years with an equal sex distribution. 25 of 36 patients have family history of renal
problems, 21 of 36 patients were production of consanguineous marriage. 17 of 36 patients
were originally came from Al-Jabel gharbi. The patients presented commonly with recurrent
urinary tract infections (UTI), renal stone and hematuria. 33 of the 36 patients had abnormal
songraphic pattern, which include renal stone, and medullary nephrocalcinosis. Renal failure
found in 3 of 36 patients at presentation while 4 patients developed renal failure later on
during fellow up after mean interval of 7 years. Primary hyperoxaluria is not uncommon
disease in our society and it causes chronic renal failure in children.

Keywords: primary hyperoxaluria, renal stone, renal failure, nephrocalcinosis, Libya.

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