Pediatric Renal Transplantation in Oman: A Single-center Experience.

OBJECTIVES: This study sought to report 22 years experience in pediatric kidney transplantation in Oman. METHODS: Electronic charts of all Omani children below 13 years of age who received a kidney transplant from January 1994 to December 2015 were reviewed. Data collected included patient demographics, etiology of end-stage kidney disease, modality and duration of dialysis, donor type, complication of kidney transplantation (including surgical complications, infections, graft rejection) graft and patient survival, and duration of follow-up. RESULTS: During the study period transplantation from 27 living related donors (LRDs), 42 living unrelated donors (LURDs), also referred to as commercial transplant, and one deceased donor were performed. The median age at transplantation was nine years for both groups. The most common primary diagnosis was congenital anomalies of the kidney and urinary tract in 32.8% of patients followed by familial nephrotic syndrome in 20.0% and polycystic kidney disease in 18.5%. Almost half the patients were on hemodialysis before transplantation, 35.7% were on peritoneal dialysis, and 14.2% received preemptive renal transplantation. Children who received LURD kidneys had high surgical complications (42.8%) compared to the LRDs group (17.8%). Five patients from LURDs group had early graft nephrectomy and four patients developed non-graft function or delayed graft function. In addition, patients in the LURDs group had a higher incidence of hypertension and acute rejection. Graft and patient survival were both better in the LRDs than the LURDs group. CONCLUSIONS: 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.

C3 Glomerulopathy and post-infectious glomerulonephritis define a disease spectrum.

BACKGROUND: Post-infectious glomerulonephritis (PIGN) usually follows a benign course, but few children have an atypical, severe presentation, and these exceptional cases have been linked to the dysregulation of the complement alternative pathway (CAP). There is a considerable overlap in the histopathological features of PIGN and C3 glomerulopathy (C3G), which is also associated with CAP dysregulation but has a poorer outcome. We hypothesized that PIGN and C3G define a disease spectrum, and that in the past there may be some children with C3G who were misclassified with PIGN before C3G was described as a separate disease entity. METHODS: Children with PIGN (n = 33) diagnosed between 1985 and 2010 who underwent a renal biopsy due to their unusual course were reviewed and of them, 8 were reclassified into C3G based on the current classification criteria. Outcome was based on the degree of proteinuria, C3 level, and renal function at follow-up. RESULTS: Sixteen (72.7%) children with typical PIGN recovered completely as compared to only 2 (25%) with C3G. Of note, children with "typical" PIGN had a more severe disease course at onset; however, the outcome at last follow up was favorable. CONCLUSIONS: Our results support the hypothesis that PIGN and C3G form a disease spectrum and have different long-term clinical implications and management strategies.

Primary hyperoxaluria type 1 in 18 children: genotyping and outcome.

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.