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Year : 2022  |  Volume : 5  |  Issue : 2  |  Page : 78-85

Childhood nephrotic syndrome: A single center experience

1 Pediatric Nephrology Center of Excellence; Department of Pediatrics, Collage of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia
2 Department of Pediatrics, Collage of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia

Correspondence Address:
Sherif Mohamed El-Desoky
Pediatric Nephrology Center of Excellence and Department of Pediatics, Collage of Medicine, King Abdulaziz University, Jeddah
Saudi Arabia
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Source of Support: None, Conflict of Interest: None

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Background: Childhood nephrotic syndrome (NS) is the most frequently occurring chronic kidney disease (CKD) among children. Methods: This retrospective review aimed to determine the clinical profile, diagnostic procedures, therapeutic strategies, and outcomes of NS in children <18 years old diagnosed and followed up in the pediatric nephrology unit at King Abdulaziz University Hospital, Kingdom of Saudi Arabia, from 2000 to 2020. Children with an underlying secondary cause for NS were excluded. Results: Five hundred and sixteen children were enrolled into four groups of patients, including steroid-sensitive NS (SSNS, 276 patients), primary steroid resistant (SRNS, 138 patients), secondary SRNS (56 patients), and congenital and infantile NS (46 infants). The age at presentation was 5.29 ± 2.83 years and mean follow-up duration was 4 years. Kidney biopsy was indicated for 174 (33.7%) children, of whom 79 (45.4%) had focal segmental glomerulonephritis, 35 (20.1%) had IgM nephropathy, and 25 (14.3%) had minimal change disease. Seventy (13.5%) patients were found to have positive homozygous variants, chiefly in NPHS1 in 25 (35.27%), NPHS2 in 16 (22.8%), and LAMB2 in 6 (8.5%). Acute kidney injury and kidney failure were most commonly observed in patients with SRNS (P < 0.001), while sepsis and mortality were significantly predominant among patients with infantile NS (P < 0.001). Kidney failure was reported in 8.7% patients. Conclusion: NS in children remains a challenging chronic glomerular disorder with variable outcome. Progression of CKD is seen most commonly in children with SRNS, more so in those with genetic etiology. Patients with congenital NS have the highest mortality.

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