Sultan Qaboos University medical journal · 2026
Congenital Nephrotic Syndrome in Oman: A 20-year single-centre experience.
СтатьяНазвание и аннотация: Перевод в очереди
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Al Hashmi Muzna, Al Maskari Anisa, Al Saidi Sulaiman, Al Ghaithi Badria, Al Riyami Mohammed, Al Kalbani Naifain
Аннотация
OBJECTIVES: Congenital nephrotic syndrome (CNS) is a rare, severe disease presenting within the first three months of life, with limited prevalence data from Oman and the Gulf region. This study aimed to report 20 years' experience of congenital nephrotic syndrome (CNS) in a single centre in Oman.
METHODS: This retrospective study including children diagnosed with nephrotic syndrome in first 3 months of life, from January 2000 to December 2020 at Royal Hospital, Muscat, Oman.
RESULTS: A total of 26 patients were included in the study, with no gender predominance. Consanguinity was noted in 61.5% of patients and a family history of renal disease was found in 46.2% of patients. The median age at presentation was 19.9 days (range: birth-2 months). Genetic workup was performed in 19 patients of which 73.6% were positive for NPHS1 gene. The median follow-up was 6.3 years (range: 1 month-13 years). Dialysis was initiated in 23.1% of patients, and 15.4% underwent transplantation. The median age at death was 1.5 years (range: 1 month-7 years).
CONCLUSION: Genetic factors remain the predominant cause of CNS in this study's cohort. Despite significant disease course, advances in supportive and specialised care continue to improve outcomes.
METHODS: This retrospective study including children diagnosed with nephrotic syndrome in first 3 months of life, from January 2000 to December 2020 at Royal Hospital, Muscat, Oman.
RESULTS: A total of 26 patients were included in the study, with no gender predominance. Consanguinity was noted in 61.5% of patients and a family history of renal disease was found in 46.2% of patients. The median age at presentation was 19.9 days (range: birth-2 months). Genetic workup was performed in 19 patients of which 73.6% were positive for NPHS1 gene. The median follow-up was 6.3 years (range: 1 month-13 years). Dialysis was initiated in 23.1% of patients, and 15.4% underwent transplantation. The median age at death was 1.5 years (range: 1 month-7 years).
CONCLUSION: Genetic factors remain the predominant cause of CNS in this study's cohort. Despite significant disease course, advances in supportive and specialised care continue to improve outcomes.