Podcast
Questions and Answers
What is the primary cause of nephrotic syndrome?
What is the primary cause of nephrotic syndrome?
What is the cut-off value for serum albumin levels to diagnose nephrotic syndrome in children?
What is the cut-off value for serum albumin levels to diagnose nephrotic syndrome in children?
Which of the following is NOT a common cause of nephrotic syndrome?
Which of the following is NOT a common cause of nephrotic syndrome?
What is the initial treatment for nephrotic syndrome?
What is the initial treatment for nephrotic syndrome?
Signup and view all the answers
What percentage of steroid-resistant nephrotic syndrome cases have mutations in podocyte-associated genes?
What percentage of steroid-resistant nephrotic syndrome cases have mutations in podocyte-associated genes?
Signup and view all the answers
What is the unit of measurement for proteinuria in the diagnosis of nephrotic syndrome?
What is the unit of measurement for proteinuria in the diagnosis of nephrotic syndrome?
Signup and view all the answers
Which of the following glomerular lesions is commonly associated with nephrotic syndrome in children?
Which of the following glomerular lesions is commonly associated with nephrotic syndrome in children?
Signup and view all the answers
What is the incidence of nephrotic syndrome in children per year?
What is the incidence of nephrotic syndrome in children per year?
Signup and view all the answers
What is the term for patients who do not achieve complete remission within 4-6 weeks of initial treatment?
What is the term for patients who do not achieve complete remission within 4-6 weeks of initial treatment?
Signup and view all the answers
What is the primary protein lost in the urine in nephrotic syndrome?
What is the primary protein lost in the urine in nephrotic syndrome?
Signup and view all the answers
Study Notes
Nephrotic Syndrome: Pathophysiology, Treatment Options, Complications, and Diagnostic Criteria
Nephrotic syndrome (NS) is a rare condition that affects approximately 1-3 per 100,000 children per year. It is characterized by severe proteinuria, hypoalbuminemia, and/or the presence of edema. The pathophysiology of NS involves a severe alteration in the glomerular filtration barrier, which allows large quantities of protein, mostly albumin, to freely pass in urine. The condition is diagnosed in children when proteinuria exceeds 40 mg/m² per hour, and serum albumin levels are below 3.5 g/dL.
Pathophysiology
The primary cause of NS is a severe alteration of the glomerular filtration barrier, leading to the loss of large quantities of proteins, mainly albumin, in urine. Various glomerular lesions are associated with NS, including minimal change disease in children, focal and segmental glomerulosclerosis in adults with African ancestry, and membranous glomerulopathy in Caucasian adults. Other causes include infections, extrarenal or systemic diseases, and drug-induced nephrotoxicity.
Treatment Options
The initial treatment for NS is a course of daily oral prednisolone/prednisone (PDN) for 4-6 weeks. Patients who do not achieve complete remission within this timeframe are considered to have steroid-resistant nephrotic syndrome (SRNS). In 10-30% of SRNS cases, mutations in podocyte-associated genes can be detected. If a genetic cause is identified, specific therapies may be considered.
Complications
Nephrotic syndrome complications are driven by the magnitude of proteinuria or hypoalbuminemia or both. The most common clinical presentation of nephrotic patients is edema, which should be differentiated from generalized edema of heart failure and liver cirrhosis. Complications of NS include thromboembolic complications, acute kidney injury, and infections.
Diagnostic Criteria
The diagnosis of NS is based on laboratory data, specifically proteinuria over 3.5 g/day in adults or 40 mg/m² per hour in children, in association with a low serum albumin (<3.5 g/dL). A low serum albumin is also required for diagnosis.
Pediatric Considerations
In pediatric patients with SRNS, a kidney biopsy is recommended, except in cases of known infection. Genetic testing is also recommended, as mutations in podocyte-associated genes can be detected in 10-30% of SRNS cases. The treatment of steroid-resistant nephrotic syndrome is challenging due to its heterogeneous etiology, frequent lack of remission by further immunosuppressive treatment, and severe complications including the development of end-stage kidney disease and recurrence after renal transplantation.
Studying That Suits You
Use AI to generate personalized quizzes and flashcards to suit your learning preferences.
Description
Learn about Nephrotic Syndrome, a rare condition characterized by severe proteinuria, hypoalbuminemia, and edema. Understand the pathophysiology, treatment options, complications, and diagnostic criteria for this condition. Discover the challenges of diagnosing and treating steroid-resistant nephrotic syndrome in pediatric patients.
