Steroid-Resistant Nephrotic Syndrome

Episode Description:

Steroid-Resistant Nephrotic Syndrome (SRNS) is a complex condition characterized by the failure to respond to standard steroid therapy, often leading to a more severe prognosis. While predominantly affecting children, SRNS can also occur in adults and carries significant implications for kidney function and overall health.

Nephrotic syndrome is marked by severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. SRNS involves genetic mutations affecting podocytes, with key genes including NPHS1, NPHS2, and WT1 playing a crucial role. Immune dysfunction and molecular pathways involving cytokines and growth factors also contribute to SRNS, making it a challenging condition to manage.

Diagnosis involves clinical evaluation, laboratory tests, and genetic screening. Urine analysis, blood tests, and kidney biopsies help confirm the diagnosis and assess the severity of podocyte damage. Genetic testing can identify specific mutations to guide treatment and prognosis, though its availability and cost can be limiting factors.

Treatment strategies for SRNS often involve calcineurin inhibitors like cyclosporine or tacrolimus, along with other immunosuppressive agents such as mycophenolate mofetil and rituximab. Supportive care includes managing edema and hypertension with ACE inhibitors or ARBs, dietary modifications, and lipid-lowering medications.

Despite these measures, many SRNS patients progress to end-stage renal disease (ESRD), requiring dialysis or kidney transplantation. The prognosis depends on the response to therapy and the progression to ESRD, with multidisciplinary care teams playing a crucial role in improving outcomes.

Quiz

1. What is the primary characteristic of Steroid-Resistant Nephrotic Syndrome (SRNS)?

2. Which genes are commonly associated with mutations in Steroid-Resistant Nephrotic Syndrome?

3. Which diagnostic test is crucial for confirming the diagnosis of SRNS?

4. Which class of medication is often used to manage proteinuria and control blood pressure in SRNS patients?

Renes Care Radio is a podcast designed for nephrology professionals, offering in-depth scientific explorations of key topics. It equips kidney care providers with the essential knowledge needed for advanced patient care.

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This podcast is for informational purposes only and is not intended to replace professional medical advice, diagnosis, or treatment.