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Published on: July 3, 2013
Long-term prognosis of idiopathic nephrotic syndrome in children
Sare Gülfem Özlü1, Gülay Demircin, Nazan Tökmeci
1Department of Pediatric Nephrology, Dr Sami Ulus Childrens and Maternity Hospital , Ankara , Turkey and.
Insights
Idiopathic nephrotic syndrome (INS) in children is often steroid-responsive, particularly minimal change disease (MCD). However, focal segmental glomerulosclerosis (FSGS) indicates steroid resistance and potential chronic kidney disease (CKD).
Area of Science:
- Pediatric Nephrology
- Internal Medicine
- Clinical Research
Background:
- Idiopathic nephrotic syndrome (INS) is a significant kidney disorder in children.
- Understanding prognostic factors is crucial for managing INS outcomes.
Purpose of the Study:
- To analyze demographic, clinical, and laboratory data of children with INS.
- To identify factors influencing the clinical prognosis of pediatric INS patients.
Main Methods:
- Retrospective analysis of 372 children with INS diagnosed between 1990-2008.
- Evaluation of demographic, clinical, laboratory findings, treatment protocols, and prognosis.
Main Results:
- 80.4% of patients were steroid-responsive; 19.6% were steroid-resistant.
- Focal segmental glomerulosclerosis (FSGS) was linked to steroid resistance, while minimal change disease (MCD) showed higher steroid sensitivity.
- Complete remission rates were 96% for steroid-sensitive and 46.6% for steroid-resistant cases; 15% of steroid-resistant patients developed chronic kidney disease (CKD).
Conclusions:
- Steroid therapy response and intercurrent infections are key prognostic indicators in pediatric INS.
- Early identification of steroid resistance, especially in FSGS cases, is vital for preventing CKD progression.
Background:
To investigate the demographic, clinical and laboratory data of the children with idiopathic nephrotic syndrome (INS), and to determine prognostic factors that affect the clinical outcome of the patients.
Methods:
Medical charts of 372 patients diagnosed to have INS and followed up at least 5 years between January 1990 and December 2008 were evaluated, respectively. After initial demographic, clinical and laboratory findings of the patients were documented, therapeutic protocols, prognosis and prognostic factors were investigated.
Results:
299 of the patients (80.4%) were steroid responsive and 73 (19.6%) were not. Focal segmental glomerulosclerosis (FSGS) was observed in 57%, minimal change disease (MCD) in 20.6% and diffuse mesengial proliferation in 21.9% renal biopsy materials. Steroid sensitivity was higher in patients with MCD and under the age of five years. Resistance to steroids was higher in children with FSGS. Complete remission was achieved in 96% of patients who were sensitive to steroids and in 46.6% who were resistant. 15% of patients who were steroid resistant developed chronic kidney disease (CKD).
Conclusion:
Intercurrent infections and response to steroid therapy are the most important factors affecting the prognosis of the disease.
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