Steroid Resistant Nephrotic Syndrome
Diagnosis
- Lack of response to prednisolone therapy for 4 weeks
- Indication for renal biopsy, BBVS
Etiology
- 10 – 20% - Genetic (Mutations in genes encoding podocyte proteins)
Indications for Mutational Analysis
- Congenital Nephrotic Syndrome
- Family History of SRNS
- Sporadic resistance to steroids
- Girls with steroid resistant FSGS.
Adjunctive Therapies
- For steroid resistance (or steroid dependence with serious steroid side effects) include alkylating agents (e.g., cyclophosphamide), calcineurin inhibitors (e.g., cyclosporine, tacrolimus), and renin-angiotensin blockade (ACE inhibitors, ARBs).
- Steroids + statins + Diuretics
ComplicationsZ
- Edema (generalized edema, ascites, pleural effusion).
- Infections (pneumonia, peritonitis, and cellulitis).
- Thromboembolic complications
- Hypovolaemia and Acute renal Failure
- Steroid Toxicity.
Children with nephrotic syndrome are at higher risk for infection with encapsulated organisms, especially Streptococcus pneumoniae, as well as with gram-negative organisms, measles, and varicella.
Prognosis
- Steroid Responsive NS: Good prognosis (MCNS)
- Steroid Resistant NS: Poor prognosis (FSGS).
- Children and adolescents with progressive renal failure may require renal replacement therapy and a renal transplant.
- FSGS may recur after transplant in 30% to 50% of cases.