Clinical presentation, histopathology, and treatment outcomes of steroid-resistant nephrotic syndrome in children presenting in a tertiary care hospital in Pakistan

Introduction: Nephrotic syndrome (NS) is one of the most common glomerular diseases in children. Steroid-resistant nephrotic syndrome (SRNS), although represents a small fraction of NS is a challenging condition to manage. The study aims to review the clinical signs and symptoms, histopathology, and treatment outcomes of SRNS in children. Methodology: This cross-sectional study was conducted in the Pediatric nephrology department of Shifa International Hospital from 1st September 2019 to February 2022. All children 1 to 18 years old fulfilling the criteria for SRNS patients were included. Their age of presentation, signs, symptoms, clinical findings, laboratory data, and histopathological findings on renal biopsy were noted. Outcomes like complete remission, partial remission, and chronic kidney disease were recorded. Results: A total of 67 (25.8%) SRNS patients were included, mean age was 4.3 + 3.09 years. FSGS 21 (31.3%) was the most common, and MCD was seen in 9 (13.4%). Classic signs and symptoms of edema, weight gain, and abdominal distension were present in the majority. Oliguria, hematuria, and hypertension were significantly associated with a diagnosis of SRNS (p<0.05). In the laboratory workup, the mean albumin was 2.03 + 0.6 gm/l, the mean cholesterol 385.3 + 149 mg/dl, the mean serum creatinine was 2.1 + 1.16 mg/dl, and the mean protein creatinine ratio was 7.7 + 4.2. CKD and ESRD developed in 10 patients, and mortality was reported in 10 patients (P<0.005). Conclusion: SRNS comprises a significant number of cases of NS resulting in ESRD with high morbidity and mortality.