What is Steroid Resistant Nephrotic Syndrome (SRNS)?
Steroid Resistant Nephrotic Syndrome (SRNS) is a type of
nephrotic syndrome in children that does not respond to standard corticosteroid therapy. Typically, these children do not achieve remission of proteinuria after 4-6 weeks of daily prednisone treatment, necessitating alternative therapeutic approaches.
What are the Causes?
SRNS is often linked to
genetic mutations affecting the proteins that maintain the glomerular filtration barrier in the kidneys. These mutations can be either inherited or occur sporadically. Additionally, certain types of
glomerulonephritis, such as focal segmental glomerulosclerosis (FSGS), are commonly associated with SRNS.
What are the Symptoms?
The symptoms of SRNS in children include severe
proteinuria, hypoalbuminemia, edema, and hyperlipidemia. Unlike steroid-sensitive forms, these symptoms persist despite corticosteroid treatment. Other symptoms may include fatigue, poor appetite, and infections due to low immunoglobulin levels.
How is SRNS Diagnosed?
Diagnosis typically involves a combination of clinical evaluation and laboratory tests. Persistent proteinuria despite corticosteroid therapy raises suspicion for SRNS. A renal biopsy may be required to determine the underlying histopathology, such as FSGS or minimal change disease. Genetic testing can help identify specific mutations responsible for SRNS.
What are the Treatment Options?
Treatment for SRNS is more challenging and often involves a multi-modal approach. Immunosuppressive agents such as calcineurin inhibitors (e.g.,
cyclosporine and tacrolimus), mycophenolate mofetil, or rituximab are commonly used. Supportive care includes managing edema with diuretics, controlling blood pressure, and reducing proteinuria with angiotensin-converting enzyme (ACE) inhibitors or angiotensin II receptor blockers (ARBs).
What is the Prognosis?
The prognosis of SRNS varies widely. Children with genetic forms of SRNS often have a more guarded prognosis, with many progressing to chronic kidney disease (CKD) or end-stage renal disease (ESRD). Early and effective treatment can improve outcomes, although many patients may eventually require
renal replacement therapy such as dialysis or a kidney transplant.
What Ongoing Research Exists?
Research is ongoing to better understand the genetic basis of SRNS and to develop targeted therapies. Newer agents, such as selective endothelin receptor antagonists and biologics, are being explored in clinical trials. Advances in
genomics and personalized medicine hold promise for more effective and tailored treatment strategies in the future.
How to Support Families?
Managing SRNS can be stressful for families. Providing comprehensive education about the disease, treatment options, and potential outcomes is crucial. Psychological support, including counseling and support groups, can help families cope with the emotional and practical challenges of managing a chronic illness.
