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Questions and Answers
What is the primary characteristic of Nephrotic Syndrome?
Which type of Nephrotic Syndrome has no known cause?
Which of the following is a secondary cause of Nephrotic Syndrome?
What is the incidence rate of Nephrotic Syndrome in children per year?
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Which disease is NOT associated with causing Nephrotic Syndrome?
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Which of the following drugs is a known secondary cause of Nephrotic Syndrome?
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Which disease is characterized by hematuria, hypertension, edema, and renal insufficiency?
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What is the classic example of nephritic syndrome?
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What mediates the pathogenesis of glomerulonephritis in nephritic syndrome?
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When does renal involvement in nephritic syndrome typically present?
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What may range from asymptomatic hematuria to acute renal failure in nephritic syndrome?
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What causes lumpy-bump deposits on basement membranes in nephritic syndrome?
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What defines a patient as steroid-dependent?
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What is a potential side effect of cyclophosphamide in nephrotic syndrome treatment?
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Which medication may help maintain remission in children with steroid-dependent or frequently relapsing nephrotic syndrome?
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What is a potential complication of cyclosporine and tacrolimus therapy in children?
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When should cyclophosphamide be withheld in nephrotic syndrome treatment?
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What is the primary characteristic of steroid-resistant patients?
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What is the main reason for the development of edema in patients with the described condition?
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Which symptoms are considered non-specific in patients with the described condition?
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What type of cells are commonly found in the urinalysis of patients with the described condition?
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What is a common presentation in the blood work of patients with the described condition?
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When should a renal biopsy be considered in patients with the described condition?
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Which histopathological type of primary NS is characterized by lesions occurring in some tufts within a glomerulus and sparing of others?
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Which histopathological type of primary NS is associated with proximal convoluted tubules laden with protein droplets and lipids?
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What is the defining abnormality observed in the glomeruli under electron microscope in cases of FSGS?
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At what age range does the onset of Nephrotic Syndrome usually occur?
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'Anasarca' refers to generalized oedema that includes swelling in which areas?
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What is the term for the older name used for Minimal Change Disease?
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Study Notes
Definition and Introduction to Nephrotic Syndrome
- Nephrotic Syndrome (NS) is characterized by:
- Heavy proteinuria (> 3.5 g/day in adults or 40 mg/m2/hr in children)
- Hypoproteinaemia (< 2.5 g/dL)
- Oedema
- Hyperlipidaemia
- NS is 15 times more common in children, with an incidence of 2-3 cases per 100,000 children per year
- There are two types of NS:
- Primary (Idiopathic) NS (90%): no known cause
- Secondary NS (10%): due to either systemic or glomerular causes
Secondary Causes of Nephrotic Syndrome
- Infections:
- Hepatitis B, C
- HIV-1
- Malaria (Plasmodium malariae)
- Syphilis
- Toxoplasmosis
- Drugs:
- Gold
- Penicillamine
- NSAIDs
- Pamidronate
- Interferon
- Mercury
- Heroin
- Lithium
- Systemic diseases:
- Diabetes mellitus
- Sickle cell disease (SCD)
- Goodpasture syndrome
- Amyloidosis
- Systemic lupus erythematosus (SLE)
- Henoch-Schönlein purpura (HSP) nephritis
- Alport syndrome
- Associated with Malignant Disease:
- Lymphoma
- Leukemia
- Melanoma
- Miscellaneous:
- Bee sting allergy
- Metabolic disorders:
- α1-Antitrypsin deficiency
- Fabry disease
- Glutaric acidemia
- Glycogen storage disease
- Congenital syndromes
Clinical Features and Investigations
- Non-specific symptoms:
- Malaise
- Lethargy
- Abdominal or flank pain
- Fever
- Urinalysis:
- RBCs with RBC casts and proteinuria
- Polymorphonuclear leukocytes are common
- Mild normochromic anemia may be present due to hemodilution and low-grade hemolysis
- Serum C3 level is usually reduced
- High ASOT titers
- Renal biopsy is only done if there is:
- Development of acute renal failure
- Absence of evidence for streptococcal infection
- Absence of hypocomplementemia
- Persistence of marked hematuria or proteinuria
- Low C3 level for more than 3 months after onset
Treatment and Complications
- Strict input/output and daily weight monitoring
- Salt restriction and monitor BP
- Oral penicillin for 10 days
- Antihypertensive medications:
- Diuretics
- Angiotensin-converting enzyme inhibitors
- Complications:
- Volume overload
- Heart failure
- Hypertension with hypertensive encephalopathy
- Hyperkalemia
- Hyperphosphatemia
- Hypocalcemia
Nephrotic Vs Nephritic Syndrome
- Nephrotic Syndrome:
- Characterized by hematuria, hypertension, edema, and renal insufficiency
- Classic example: acute poststreptococcal glomerulonephritis
Steroid-Dependent and Steroid-Resistant Patients
- Steroid-dependent patients:
- Relapse while on alternate-day steroid therapy or within 28 days of stopping prednisone therapy
- Steroid-resistant patients:
- Failure to respond to initial daily prednisolone therapy
- Alternative agents for steroid-dependent and steroid-resistant patients:
- Levamisole
- Cyclophosphamide
- Cyclosporine
- Tacrolimus
- Mycophenolate
Histopathological Subtypes of Idiopathic NS
- Minimal change disease (MCD): 85-95% respond to steroid therapy
- Focal segmental glomerulosclerosis (FSGS): 10-20% respond to steroid
- Membranous Nephropathy (MN)
- Membranoproliferative glomerulonephritis (MPGN): 50% respond to steroid
Complications of NS
- Denys-Drash syndrome
- Nail Patella syndrome
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Description
Test your knowledge on Nephrotic Syndrome (NS) in paediatrics with this quiz from GIDEON ROBERT UNIVERSITY. Learn about the definition, characteristics, and incidence of Nephrotic Syndrome in children.
