Podcast
Questions and Answers
What are the primary pharmacological treatments for steroid-resistant nephrotic syndrome?
What are the primary pharmacological treatments for steroid-resistant nephrotic syndrome?
IV cyclophosphamide therapy and calcineurin inhibitors.
What dietary considerations should be taken into account for patients with nephrotic syndrome?
What dietary considerations should be taken into account for patients with nephrotic syndrome?
Dietary management should focus on controlling edema and preventing malnutrition.
Which infections are children with nephrotic syndrome particularly susceptible to?
Which infections are children with nephrotic syndrome particularly susceptible to?
Bacterial infections such as peritonitis and cellulitis are common.
What is the role of rituximab in the treatment of nephrotic syndrome?
What is the role of rituximab in the treatment of nephrotic syndrome?
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What are some common complications associated with nephrotic syndrome?
What are some common complications associated with nephrotic syndrome?
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What is the primary indication for renal biopsy in patients suspected of having nephrotic syndrome?
What is the primary indication for renal biopsy in patients suspected of having nephrotic syndrome?
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How is peritonitis diagnosed in patients with nephrotic syndrome?
How is peritonitis diagnosed in patients with nephrotic syndrome?
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What is the likely urine protein dipstick result in a patient with minimal change nephrotic syndrome (MCNS)?
What is the likely urine protein dipstick result in a patient with minimal change nephrotic syndrome (MCNS)?
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Why is psychological support essential for families dealing with nephrotic syndrome?
Why is psychological support essential for families dealing with nephrotic syndrome?
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What is considered the chief pathogen causing infections in children with nephrotic syndrome?
What is considered the chief pathogen causing infections in children with nephrotic syndrome?
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What is the recommended initial steroid treatment dosage for an episode of MCNS?
What is the recommended initial steroid treatment dosage for an episode of MCNS?
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How is sustained hypertension regarded in the context of nephrotic syndrome?
How is sustained hypertension regarded in the context of nephrotic syndrome?
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What is the prognosis for steroid-sensitive nephrotic syndrome compared to steroid-resistant cases?
What is the prognosis for steroid-sensitive nephrotic syndrome compared to steroid-resistant cases?
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What are some common side effects associated with prolonged steroid therapy in nephrotic syndrome?
What are some common side effects associated with prolonged steroid therapy in nephrotic syndrome?
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In patients receiving prolonged corticosteroid therapy, what complication can occur related to hormone levels?
In patients receiving prolonged corticosteroid therapy, what complication can occur related to hormone levels?
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Why is renal biopsy not typically performed in patients likely to have MCNS?
Why is renal biopsy not typically performed in patients likely to have MCNS?
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What is the definition of proteinuria?
What is the definition of proteinuria?
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What role does the urinary protein-to-creatinine ratio play in assessing nephrotic syndrome?
What role does the urinary protein-to-creatinine ratio play in assessing nephrotic syndrome?
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What treatment should be initiated for frequent relapses in nephrotic syndrome?
What treatment should be initiated for frequent relapses in nephrotic syndrome?
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List two types of physiologic proteinuria.
List two types of physiologic proteinuria.
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What laboratory findings are typically monitored in patients with nephrotic syndrome?
What laboratory findings are typically monitored in patients with nephrotic syndrome?
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What is the significance of a 24-hour urine protein excretion greater than 1g/m2?
What is the significance of a 24-hour urine protein excretion greater than 1g/m2?
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What triggers are commonly associated with relapses in patients with MCNS?
What triggers are commonly associated with relapses in patients with MCNS?
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What clinical features are common in minimal change nephrotic syndrome (MCNS)?
What clinical features are common in minimal change nephrotic syndrome (MCNS)?
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What distinguishes idiopathic nephrotic syndrome from secondary nephrotic syndrome?
What distinguishes idiopathic nephrotic syndrome from secondary nephrotic syndrome?
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What is meant by 'urinary remission' in the context of nephrotic syndrome?
What is meant by 'urinary remission' in the context of nephrotic syndrome?
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Define 'steroid-resistant nephrotic syndrome'.
Define 'steroid-resistant nephrotic syndrome'.
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What age group is most commonly affected by nephrotic syndrome?
What age group is most commonly affected by nephrotic syndrome?
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What are some associated symptoms to assess for in a child with nephrotic syndrome?
What are some associated symptoms to assess for in a child with nephrotic syndrome?
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What role do podocytes play in the pathogenesis of nephrotic syndrome?
What role do podocytes play in the pathogenesis of nephrotic syndrome?
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Study Notes
Proteinuria and Nephrotic Syndrome
- Proteinuria is defined as the presence of protein in the urine.
- Proteinuria can be physiological (caused by fever, exercise, stress, or seizures) or pathological (caused by glomerular or tubular disease).
- Orthostatic proteinuria is an exaggerated form of physiological proteinuria.
- Pathological proteinuria is further categorized into non-nephrotic range and nephrotic range proteinuria based on spot urine protein/creatinine ratio and 24-hour urine protein excretion.
- Diagnostic tests for proteinuria include urine dipstick test (detects albumin only), spot urine protein/creatinine ratio, and 24-hour urine protein collection.
Pathological Causes of Proteinuria
- Glomerular proteinuria: Increased permeability of glomerular capillaries allows large molecules like albumin to pass into the urine. Causes may include nephrotic syndrome, glomerulonephritis, and reflux nephropathy.
- Tubular proteinuria: Defective tubular reabsorption of low-molecular weight proteins like B2 microglobulin. Causes include Fanconi syndrome, tubulointerstitial nephritis, and acute tubular necrosis.
Evaluation of Proteinuria
- Renal function tests (RFTs)
- Albumin levels
- Total serum protein
- Renal ultrasound
- Urinary protein electrophoresis
- Serology for glomerular diseases (ANA, hepatitis serology, complement levels C3, C4)
- DMSA scan
- Renal biopsy
Management of Proteinuria
- Treatment depends on the cause (etiology).
- Angiotensin-converting enzyme inhibitors (ACEIs) are recommended.
- 30-40% reduction in proteinuria and control of hypertension are treatment goals.
- Vitamin D analogs (like calcitriol) can reduce proteinuria in adults with chronic kidney disease (CKD) by 15-20%.
Nephrotic Syndrome
Epidemiology
- Nephrotic syndrome (NS) is a common renal disorder in developed countries, with rates of 20-40 per million people.
- In India, the estimated rate is 90-100 per million population.
Definition
- NS is a clinical syndrome characterized by heavy proteinuria (greater than 40 mg/m2/hr or >1g/m2/24hr).
- Hypoalbuminemia, generalized edema, and hyperlipidemia are also characteristic features.
- Hematuria (typically microscopic), hypertension, and elevated urea levels may be seen.
Important Terms
- Idiopathic NS: The underlying disease mechanism is unclear.
- Secondary NS: Secondary to other systemic diseases (e.g., lupus, HSP, hepatitis B).
- Urinary remission: Urinary albumin is undetectable or low for a few days.
- Relapse: Recovery from initial symptoms is followed by a return to previous symptoms and heavy proteinuria.
- Frequent relapse: Multiple relapse in a year or 6 months.
- Steroid dependent NS: Relapse despite steroid therapy.
- Steroid resistance NS: Persistent proteinuria despite high-dose steroid therapy.
- Congenital NS: NS presenting in the first three months of life.
Pathogenesis of Nephrotic Syndrome
- Genetic abnormalities,
- Immune dysfunctions.
- Circulating factors
- Podocytes and anti-proteinuric agents.
Clinical Features of Nephrotic Syndrome
- Onset is usually between 2 and 6 years of age.
- More common in boys (60-70%).
- Typically insidious and presents with swelling around the eyes (periorbital edema) that is worse in the morning but resolves during the day.
- Swelling may spread to involve other parts of the body, including the face, extremities, and abdomen.
- Could be associated with upper respiratory tract infections (URTIs).
- Some symptoms include: Abdominal pain, fever, vomiting, rash, organomegally, lymphadenopathy are less common.
Evaluation for Nephrotic Syndrome
- Examination to detect associated infection, physical findings.
- Assessment of systemic disease features (fever, rash, joint pain, organomegally, lymphadenopathy) is important, as is monitoring of blood pressure.
- Patients with massive edema may occasionally have elevated blood pressure. Blood pressure often normalizes when edema subsides.
- Urine analysis: 3-4+ proteinuria, protein/creatinine ratio.
- Complete blood count with microscopy and urine culture.
- Blood tests: Measurement of urea, creatinine, electrolytes, proteins, and cholesterol, serum albumin, and C3 complement levels are vital.
- Ultrasound of abdomen and chest imaging.
- Other blood tests like Monteux test for tuberculosis should be considered.
- Renal biopsy is typically not needed for minimal change nephrotic syndrome (MCNS).
Treatment of Initial Episode of Nephrotic Syndrome
- Prednisone: 2 mg/kg/day or 60 mg/m2/day in divided doses for 6 weeks.
- Prednisone dosage can be tapered by reducing the dose over the course of 6 weeks.
- More concentrated doses are given during alternative days for more 6 weeks..
- Monitor blood pressure.
- Adjust dietary sodium and fat intake.
- Provide psychosocial support to the family.
Subsequent Course
- Majority of patients with MCNS (minimal change NS) will have frequent relapses, which may be precipitated by URTIs.
- Patients may be classified as having infrequent relapses, frequent relapses, or steroid-dependent relapses.
- Relapses can be treated with a course of prednisone until proteinuria resolves, followed by tapering over 4 weeks.
- Repeated steroid treatment for frequent relapses can lead to unacceptable side effects.
Treatment of frequent relapses and steroid dependence
- Patients often develop serious steroid toxicity.
- Side effects include features like obesity, hirsutism, striae, hypertension, impaired glucose tolerance, cataracts, and growth retardation.
- Alternate long-term prednisone therapy might be considered in severe cases.
Steroid Sparing Agents
- Levamisole, cyclophosphamide, mycophenolate mofetil, cyclosporine, tacrolimus, and rituximab are commonly used steroid-sparing agents.
Steroid Resistant Nephrotic Syndrome Management
- 3-6 doses of pulse methylprednisolone (20-30 mg)
- IV cyclophosphamide (500-750 mg/m2 monthly)
- Calcineurin inhibitors (for two to three years)
- Rituximab (375 mg/m2)
General Care
- Dietary management to control edema.
- Prevention and treatment of infections.
- Psychological support for the patient and family.
- Avoid non-traditional or hazardous medications.
Complications
- Infections, Hypovolemia, acute kidney injury, Thromboembolism, hyperlipidemia, urinary loss of low-molecular weight proteins, hypoproteinemia, and malnutrition.
- TB flare-ups may occur in patients on immunosuppressive therapies.
1- Infection
- Children with nephrotic syndrome are more susceptible to bacterial infections, especially if they are on immunosuppressive therapy.
- Common infections include peritonitis, cellulitis, empyema, bone/joint infections, sepsis, and tuberculosis.
- Usual pathogens include Streptococcus pneumoniae and staphylococci.
Causes of Infection in NS
- Steroid and immunosuppressant use
- Edema as a site for bacterial growth
- Urinary loss of immunoglobulins, factor B, and transferrin
Peritonitis
- Mostly seen in patients who have relapses with ascites.
- Symptoms include; abdominal pain, fever, vomiting.
- Diagnosis is made through peritoneal fluid analysis. Confirmation is done by presence of neutrophils (75-100/mm3).
- Treatment includes Parenteral antibiotics such as third-generation cephalosporins for 7–10 days.
Stress Dose Corticosteroids
- In patients who have received >1 mg/kg/day of prednisolone for more than two weeks within the past six to twelve months.
- Patients undergoing surgery and serious illnesses may need a dose of corticosteroids.
- Hydrocortisone is given at 30–100 mg/m2/day in divided doses and then tapered as the condition improves.
Outcome of Nephrotic Syndrome
- Patients with steroid-sensitive NS typically have an excellent prognosis with complete resolution of symptoms and no residual renal dysfunction within 14-20 years of onset.
- Steroid-resistant NS patients have a higher risk of developing progressive kidney disease.
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Description
This quiz focuses on the key concepts of proteinuria and nephrotic syndrome, exploring its definitions, causes, and diagnostic tests. Understand the differences between physiological and pathological proteinuria and their implications on kidney health. Test your knowledge on the various types of proteinuria and their clinical significance.