Podcast
Questions and Answers
What is the primary characteristic of Nephrotic Syndrome?
What is the primary characteristic of Nephrotic Syndrome?
- Low cholesterol levels
- High blood pressure
- Heavy proteinuria (correct)
- Low white blood cell count
Which type of Nephrotic Syndrome has no known cause?
Which type of Nephrotic Syndrome has no known cause?
- Systemic NS
- Infection-induced NS
- Primary (Idiopathic) NS (correct)
- Secondary NS
Which of the following is a secondary cause of Nephrotic Syndrome?
Which of the following is a secondary cause of Nephrotic Syndrome?
- Goodpasture syndrome
- Lymphoma (correct)
- Diabetes mellitus
- Heavy proteinuria
What is the incidence rate of Nephrotic Syndrome in children per year?
What is the incidence rate of Nephrotic Syndrome in children per year?
Which disease is NOT associated with causing Nephrotic Syndrome?
Which disease is NOT associated with causing Nephrotic Syndrome?
Which of the following drugs is a known secondary cause of Nephrotic Syndrome?
Which of the following drugs is a known secondary cause of Nephrotic Syndrome?
Which disease is characterized by hematuria, hypertension, edema, and renal insufficiency?
Which disease is characterized by hematuria, hypertension, edema, and renal insufficiency?
What is the classic example of nephritic syndrome?
What is the classic example of nephritic syndrome?
What mediates the pathogenesis of glomerulonephritis in nephritic syndrome?
What mediates the pathogenesis of glomerulonephritis in nephritic syndrome?
When does renal involvement in nephritic syndrome typically present?
When does renal involvement in nephritic syndrome typically present?
What may range from asymptomatic hematuria to acute renal failure in nephritic syndrome?
What may range from asymptomatic hematuria to acute renal failure in nephritic syndrome?
What causes lumpy-bump deposits on basement membranes in nephritic syndrome?
What causes lumpy-bump deposits on basement membranes in nephritic syndrome?
What defines a patient as steroid-dependent?
What defines a patient as steroid-dependent?
What is a potential side effect of cyclophosphamide in nephrotic syndrome treatment?
What is a potential side effect of cyclophosphamide in nephrotic syndrome treatment?
Which medication may help maintain remission in children with steroid-dependent or frequently relapsing nephrotic syndrome?
Which medication may help maintain remission in children with steroid-dependent or frequently relapsing nephrotic syndrome?
What is a potential complication of cyclosporine and tacrolimus therapy in children?
What is a potential complication of cyclosporine and tacrolimus therapy in children?
When should cyclophosphamide be withheld in nephrotic syndrome treatment?
When should cyclophosphamide be withheld in nephrotic syndrome treatment?
What is the primary characteristic of steroid-resistant patients?
What is the primary characteristic of steroid-resistant patients?
What is the main reason for the development of edema in patients with the described condition?
What is the main reason for the development of edema in patients with the described condition?
Which symptoms are considered non-specific in patients with the described condition?
Which symptoms are considered non-specific in patients with the described condition?
What type of cells are commonly found in the urinalysis of patients with the described condition?
What type of cells are commonly found in the urinalysis of patients with the described condition?
What is a common presentation in the blood work of patients with the described condition?
What is a common presentation in the blood work of patients with the described condition?
When should a renal biopsy be considered in patients with the described condition?
When should a renal biopsy be considered in patients with the described condition?
Which histopathological type of primary NS is characterized by lesions occurring in some tufts within a glomerulus and sparing of others?
Which histopathological type of primary NS is characterized by lesions occurring in some tufts within a glomerulus and sparing of others?
Which histopathological type of primary NS is associated with proximal convoluted tubules laden with protein droplets and lipids?
Which histopathological type of primary NS is associated with proximal convoluted tubules laden with protein droplets and lipids?
What is the defining abnormality observed in the glomeruli under electron microscope in cases of FSGS?
What is the defining abnormality observed in the glomeruli under electron microscope in cases of FSGS?
At what age range does the onset of Nephrotic Syndrome usually occur?
At what age range does the onset of Nephrotic Syndrome usually occur?
'Anasarca' refers to generalized oedema that includes swelling in which areas?
'Anasarca' refers to generalized oedema that includes swelling in which areas?
What is the term for the older name used for Minimal Change Disease?
What is the term for the older name used for Minimal Change Disease?
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.
