Background. Primary hyperoxaluria belongs to a group of rare metabolic disorders with autosomal recessive inheritance. It results from genetic mutations of the AGXT gene, which is more common due to higher consanguinity rates in the developing countries. Clinical features at presentation are heterogeneous even in children from the same family; this study was conducted to determine the clinical characteristics, type of AGXT mutation, and outcome in children diagnosed with PH1 at a tertiary referral center in Oman. Method. Retrospective review of children diagnosed with PH1 at a tertiary hospital in Oman from 2000 to 2013. Result. Total of 18 children were identified. Females composed 61% of the children with median presentation age of 7 months. Severe renal failure was initial presentation in 39% and 22% presented with nephrocalcinosis and/or renal calculi. Family screening diagnosed 39% of patients. Fifty percent of the children underwent hemodialysis. 28% of children underwent organ transplantation. The most common mutation found in Omani children was c.33-34insC mutation in the AGXT gene. Conclusion. Due to consanguinity, PH1 is a common cause of ESRD in Omani children. Genetic testing is recommended to help in family counseling and helps in decreasing the incidence and disease burden; it also could be utilized for premarital screening.

Although our pediatric kidney transplant program is a young program it has had successful patient outcomes comparable to international programs. Our study provides evidence that in addition to legal and ethical issues with commercial transplant, it also carries significantly higher morbidity and reduced graft and patient survival.

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Objectives: To determine the spectrum of kidney diseases in Omani children < 13 years of age and to evaluate the complications following kidney biopsy. Methods: This study retrospectively investigated the hospital data of children who underwent kidney biopsies from January 2014 to June 2019 at Royal Hospital, Muscat, Oman. Results: The subjects comprised of 78 children with a median age of 8.0 years (range = 0–13 years). Histopathology showed minimal change disease in 15 (19.2%) children, lupus nephritis in 13 (16.7%), and focal segmental glomerulosclerosis in 13 (16.7%). The most common post-biopsy complications were pain that required analgesia (38; 49.4%) followed by gross hematuria (10; 13.0%). No patient required blood transfusion or surgical intervention. Conclusions: Minimal change disease was the most common histopathological finding in this cohort of Omani children. The records did not mention any major complications following the renal biopsy procedure.

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