Podcast
Questions and Answers
What is the mainstay treatment for Atypical HUS?
What is the mainstay treatment for Atypical HUS?
- PLex (Plasma exchange therapy) (correct)
- Immunosuppressants
- Eculizumab
- Antibiotics
Bloody dysentery typically resolves within two days.
Bloody dysentery typically resolves within two days.
False (B)
Name one feature of Atypical HUS.
Name one feature of Atypical HUS.
Thrombocytopenia or features of secondary hypertension.
In pre-renal injury, urine output is considered __________.
In pre-renal injury, urine output is considered __________.
Match the following conditions with their characteristics:
Match the following conditions with their characteristics:
What is a common method used to treat edema associated with glomerular disease?
What is a common method used to treat edema associated with glomerular disease?
Salt restriction can be effective in treating edema related to glomerular disease.
Salt restriction can be effective in treating edema related to glomerular disease.
Name one treatment approach for managing edema in glomerular disease.
Name one treatment approach for managing edema in glomerular disease.
In the treatment of edema, it is important to impose a restriction on __________.
In the treatment of edema, it is important to impose a restriction on __________.
Match the following terms with their appropriate actions:
Match the following terms with their appropriate actions:
Which of the following is a type of podocytopathy that does not present with podocytopenia?
Which of the following is a type of podocytopathy that does not present with podocytopenia?
Every patient with minimal change disease (MCD) presents with microhematuria.
Every patient with minimal change disease (MCD) presents with microhematuria.
Name one secondary cause of minimal change disease (MCD).
Name one secondary cause of minimal change disease (MCD).
Focal segmental glomerulosclerosis (FSGS) is associated with a greater than _____ risk of chronic kidney disease (CKD).
Focal segmental glomerulosclerosis (FSGS) is associated with a greater than _____ risk of chronic kidney disease (CKD).
Match the following types of podocytopathies with their associated CKD risk:
Match the following types of podocytopathies with their associated CKD risk:
What percentage of patients present with nephrotic syndrome according to the 75/25 rule?
What percentage of patients present with nephrotic syndrome according to the 75/25 rule?
The most common cause of nephrotic syndrome in adults is Minimal Change Disease (MCD).
The most common cause of nephrotic syndrome in adults is Minimal Change Disease (MCD).
Name one drug that can cause nephrotic syndrome.
Name one drug that can cause nephrotic syndrome.
The most important malignancy associated with nephrotic syndrome is __________.
The most important malignancy associated with nephrotic syndrome is __________.
Which of the following is NOT a drug associated with causing FSGS?
Which of the following is NOT a drug associated with causing FSGS?
Sickle cell anemia is a known cause of secondary FSGS.
Sickle cell anemia is a known cause of secondary FSGS.
Match the following autoimmune diseases with their corresponding association with nephrotic syndrome:
Match the following autoimmune diseases with their corresponding association with nephrotic syndrome:
What gene is associated with autosomal recessive FSGS and what protein does it encode?
What gene is associated with autosomal recessive FSGS and what protein does it encode?
The main treatment for secondary perihilar FSGS that is resistant to immunosuppression therapy is _____ .
The main treatment for secondary perihilar FSGS that is resistant to immunosuppression therapy is _____ .
Match the following genetic forms of FSGS with their characteristics:
Match the following genetic forms of FSGS with their characteristics:
What is the first-line treatment for steroid-resistant nephrotic syndrome in children?
What is the first-line treatment for steroid-resistant nephrotic syndrome in children?
Cyclophosphamide is used to treat steroid-dependent nephrotic syndrome.
Cyclophosphamide is used to treat steroid-dependent nephrotic syndrome.
What is the maximum duration for the treatment with calcineurin inhibitors?
What is the maximum duration for the treatment with calcineurin inhibitors?
___ is characterized by ≥ 2 relapses within 6 months.
___ is characterized by ≥ 2 relapses within 6 months.
Match the following treatments with their associated conditions:
Match the following treatments with their associated conditions:
What urine protein creatinine ratio (PCR) indicates nephrotic syndrome in adults?
What urine protein creatinine ratio (PCR) indicates nephrotic syndrome in adults?
The underfill theory is a minor contributor to edema pathophysiology in childhood nephrotic syndrome.
The underfill theory is a minor contributor to edema pathophysiology in childhood nephrotic syndrome.
What are the three primary components that characterize childhood nephrotic syndrome?
What are the three primary components that characterize childhood nephrotic syndrome?
In childhood nephrotic syndrome, hypoalbuminemia leads to a loss of _____ pressure.
In childhood nephrotic syndrome, hypoalbuminemia leads to a loss of _____ pressure.
Match the following conditions with their association with nephrotic syndrome:
Match the following conditions with their association with nephrotic syndrome:
What is the primary activation that leads to sodium and water retention in nephritic syndrome?
What is the primary activation that leads to sodium and water retention in nephritic syndrome?
Minimal Change Disease is unresponsive to steroid treatment.
Minimal Change Disease is unresponsive to steroid treatment.
What is the recommended treatment for ascites in nephritic syndrome?
What is the recommended treatment for ascites in nephritic syndrome?
Low serum albumin is defined as less than __________ g/dL.
Low serum albumin is defined as less than __________ g/dL.
Match the nephrologic conditions with their treatment options:
Match the nephrologic conditions with their treatment options:
What is the most common presentation of glomerular disease?
What is the most common presentation of glomerular disease?
Macrohematuria must always be accompanied by significant proteinuria.
Macrohematuria must always be accompanied by significant proteinuria.
What is the criteria for nephrotic syndrome in adults regarding proteinuria?
What is the criteria for nephrotic syndrome in adults regarding proteinuria?
The _____ is the term used for the rapid decline in kidney function associated with glomerulonephritis.
The _____ is the term used for the rapid decline in kidney function associated with glomerulonephritis.
Match the following conditions with their respective presentations:
Match the following conditions with their respective presentations:
Which condition is the most common cause of adult nephrotic syndrome?
Which condition is the most common cause of adult nephrotic syndrome?
Hypoalbuminemia is a characteristic feature of adult nephrotic syndrome.
Hypoalbuminemia is a characteristic feature of adult nephrotic syndrome.
What is the typical dosage of steroids for treating adult nephrotic syndrome?
What is the typical dosage of steroids for treating adult nephrotic syndrome?
The presence of oval fat bodies in urine is commonly associated with __________.
The presence of oval fat bodies in urine is commonly associated with __________.
Match the following characteristics with the correct condition related to nephrotic syndrome:
Match the following characteristics with the correct condition related to nephrotic syndrome:
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Study Notes
Typical HUS
- Characterized by abdominal pain, bloody dysentery, and resolution in 90-95% cases
- Treatment is conservative
- May progress to HUS in 5-10% cases
Atypical HUS
- Presents with features of secondary hypertension, thrombocytopenia, and renal/endocrine issues
- Treatment includes plasma exchange therapy (PLEX) within 24 hours, eculizumab (costly), immunosuppressants (in complement factor H mutation), renal transplant, and rituximab or eculizumab for post-transplant recurrence
Pre-renal Injury vs. Acute Tubular Necrosis
- Urine output is normal in pre-renal injury and decreased in acute tubular necrosis
- Edema is present in acute tubular necrosis, but not in pre-renal injury
- BUN/Creatinine ratio is > 20:1 in pre-renal injury and < 10:1 in acute tubular necrosis
- Fluids are given in pre-renal injury and withheld in acute tubular necrosis
- Tubular injury is present in acute tubular necrosis, but not in pre-renal injury
Podocytopathies
- Glomerular diseases primarily affecting podocytes
Clinical Presentation of Glomerular Diseases
- Asymptomatic
- Macroscopic hematuria
- Nephrotic syndrome
- Nephritic syndrome
- Rapidly progressive glomerulonephritis
- Chronic glomerulonephritis
Podocytopathies with and without Podocytopenia
- Without podocytopenia: Minimal change disease (MCD) with no risk for CKD
- With podocytopenia (risk of CKD present): Focal segmental glomerulosclerosis (FSGS), Membranous nephropathy (MN), and Diabetic nephropathy (DN)
Causes and Presentations of Minimal Change Disease (MCD)
- Secondary causes:
- Drugs: NSAIDs (MCD > MN), Interferon α, Gold (MN > MCD)
- Allergy: Atopic rhinitis, Atopic dermatitis, Asthma, Post-immunization
- Malignancy: Hodgkin's disease, Sezary syndrome, Chronic lymphocytic leukemia
- 100/0 rule: Every patient with MCD presents with nephrotic syndrome, hypertension, acute kidney injury, and absence of microhematuria
Nephrotic Syndrome
- 75/25 rule: 75% present with nephrotic syndrome, 25% have asymptomatic/nephrotic proteinuria without syndrome
- Secondary Causes:
- Drugs: Gold, NSAIDS, d-penicillamine, Captopril, Mercury
- Infections: HBV, Syphilis, Schistosomiasis
- Autoimmune: SLE class V, Rheumatoid arthritis, Sarcoidosis, IgG4 related disease, Autoimmune thyroid disorders, Cystic fibrosis, Bronchiectasis
- Malignancy: Solid organ adenocarcinoma (Colorectal carcinoma is the most important)
- 70/30 rule: 70% present with nephrotic syndrome, 30% are asymptomatic with microhematuria and proteinuria
- Increased risk of thrombosis
- Diseases associated with d-penicillamine: Wilson's disease, Aplastic anemia
- Most common cause of adult-onset nephrotic syndrome: Membranous nephropathy
- Most common cause of NS in children: Minimal change disease
Glomerular Disease - Patterns
- Urine protein creatinine ratio (PCR):
- Adult: ≥3000 mg/g
- Children: ≥2000 mg/g
- Not diagnostic of nephrotic syndrome
- Seen in: Diabetes, 2° FSGS, Amyloidosis
FSGS
- Secondary Causes:
- Drugs: Pamidronate, Anabolic steroids, Heroin, Sirolimus, Dasatinib
- Infections: HIVAN, Parvo B19 virus, EBV, CMV
- Miscellaneous: Sickle cell anemia, Obesity, Congenital cyanotic heart disease
Primary FSGS
- Antigen-associated:
- Soluble urokinase plasminogen activator receptor (SUPAR)
- Cardiotrophin-like cytokine I (CLC-1)
Genetic Forms of FSGS
- Autosomal Recessive: NPHS gene (nephrin)
- Age of presentation: Present at birth
- Histology: Diffuse mesangial sclerosis
- Autosomal Dominant: α-actin 4, TRPC 6
- Age of presentation: Adolescents
Secondary Perihilar FSGS
- Slow progression and good prognosis
- Presents with asymptomatic proteinuria
- Removal of pathological kidney leads to glomerular hypertrophy in remaining nephrons/other kidney, causing 2° Perihilar FSGS
- Resistant to immunosuppression therapy
- Only treatment: Transplantation
Causes of Secondary Perihilar FSGS
- Vesicoureteral reflux (VUR)
- Unilateral/Bilateral renal artery stenosis (RAS)
- Stone, mass
Glomerular Disease - Patterns
- Urine protein creatinine ratio (PCR):
- Adult: ≥3000 mg/g
- Children: ≥2000 mg/g
- Not diagnostic of nephrotic syndrome
- Also seen in: Diabetes (most common), 2° FSGS, Amyloidosis
- Rx: ACE inhibitors, ARBs
Childhood Nephrotic Syndrome
- Characterized by proteinuria, hypoalbuminemia, and edema
- Hypoalbuminemia is caused by urinary loss of albumin
- Sign: White transverse line on the fingers
- Edema pathophysiology: Underfill theory > Overfill theory
- Hypoalbuminemia leads to decreased capillary oncotic pressure
- Fluid shifts from capillaries to interstitium
- Periorbital puffiness and pedal edema (extravascular edema)
- Decreased effective circulatory volume
- Secondary RAS activation
- Release of aldosterone
- Retention of Na+ and water (intravascular edema)
Steroid-Resistant Nephrotic Syndrome (SRNS)
- Proteinuria persists despite full dose of steroids for 4 weeks
- First indication for biopsy in children
- Treatment: Calcineurin inhibitors (DOC): Tacrolimus, Cyclosporine
Relapse of Nephrotic Syndrome
- Proteinuria after 1 month of steroid:
- 25%: No further relapse
- 25%: Infrequent relapse (steroids tapered over 4 weeks during remission)
- 50%: Frequently relapsing nephrotic syndrome (FRNS)
- ≥ 2 relapses within 6 months
- ≥ 4 relapses within 1 year
- Steroid-sparing drugs (to prevent steroid toxicity):
- Oral cyclophosphamide 2 mg/kg/day x 12 weeks + steroid x 2 weeks
- Oral levamisole
Steroid-Dependent Nephrotic Syndrome (SDNS)
-
2 consecutive relapses during tapering of steroid or within 14 days after stopping steroid
- Treatment: MMF 1200 mg/m²/day, Rituximab 375 mg/m²/day x 4 doses
Nephrology - Telegram Channel
- Overall theory: Seen in nephritic syndrome
- Pathogenesis: β hemolytic streptococci
- Release plasmin, activate epithelial sodium channel (ENAC), causing primary RAS activation
- Na+ and water retention (intravascular edema)
Management of Nephrotic Syndrome
- Minimal Change Disease (MCD):
- Prevalence: 85-90%
- Treatment: Steroid responsive (95%), biopsy not helpful
- Focal Segmental Glomerulosclerosis (FSGS):
- Genetic form: Unresponsive to steroids
- Treatment: Renal transplantation
Risk of Thrombosis in Nephrotic Syndrome
- Low serum albumin (2 g/dL)
- Dosage:
- 20% albumin: 20 g / 100 mL
- Dose: 0.5 g/kg
Edema Manifestations in Nephrotic Syndrome
- Ascites (moderate to severe):
- Treatment: Paracentesis (ascitic fluid tapping)
- Often a cause of death if untreated
- Possible cause: Pneumococcal peritonitis
- Pleural effusion (mild):
- Pericardial effusion (rare):
- Symptoms: Disproportional dyspnea
- Diagnosis: Echocardiogram (echo)
Other Conditions in Nephrotic Syndrome
- Membranous nephropathy (MN):
- IgA nephropathy (rare):
Presentations of Glomerular Diseases
- Asymptomatic
- Macrohematuria
- Nephrotic syndrome
Indications for Biopsy
- Presentation:
- Asymptomatic urine abnormalities (most common presentation):
- Proteinuria (>500 mg/24 hrs)
- Microhematuria (≥3 RBC/HPF)
- Proteinuria alone (>1 g/24 hrs)
- Macrohematuria: On urine analysis
- 40/5/1 rule:
- ≥240: Dysmorphic
- ≥25: Acanthocytes
- ≥1: RBC cast
- Significant proteinuria
- 40/5/1 rule:
- Asymptomatic urine abnormalities (most common presentation):
Nephrotic Syndrome
Criteria
- Nephrotic-range proteinuria
- Adult: >3.5 g/24 hrs
- Children: >50 mg/kg/day
- Hypoalbuminemia
Adult Nephrotic Syndrome
- Characteristics
- Proteinuria: Urine PCR ≥ 3000 mg/day
- Edema
- Hypoalbuminemia
- Causes
- Membranous nephropathy (most common)
- Focal segmental glomerulosclerosis (FSGS)
- Minimal change disease (MCD) (15%)
- IgA nephropathy
- Membranoproliferative glomerulonephritis (MPGN) (rare)
- Amyloidosis
###Â Management of Adult Nephrotic Syndrome
- Biopsy to rule out secondary causes (paraneoplastic syndromes)
- Treatment:
- Steroid: 1 mg/kg/day x 4 months
- If no response: SRNS (most common: FSGS)
Microscopic Images
- Microscopic Image A: Shows maltese crosses in polarized light (seen in Fabry's disease)
- Microscopic Image B: Shows oval fat bodies
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