Medicine Marrow Pg No 755-764 (Nephrology)
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Questions and Answers

What is the mainstay treatment for Atypical HUS?

  • PLex (Plasma exchange therapy) (correct)
  • Immunosuppressants
  • Eculizumab
  • Antibiotics

    • Bloody dysentery typically resolves within two days.

    False

    Name one feature of Atypical HUS.

    Thrombocytopenia or features of secondary hypertension.

    In pre-renal injury, urine output is considered __________.

    <p>normal</p> Signup and view all the answers

    Match the following conditions with their characteristics:

    <p>Pre-renal injury = Urine output normal, Edema negative, BUN/Creatinine &gt; 20:1 Acute tubular necrosis = Urine output decreased, Edema positive, BUN/Creatinine &lt; 10:1</p> Signup and view all the answers

    What is a common method used to treat edema associated with glomerular disease?

    <p>Salt restriction</p> Signup and view all the answers

    Salt restriction can be effective in treating edema related to glomerular disease.

    <p>True</p> Signup and view all the answers

    Name one treatment approach for managing edema in glomerular disease.

    <p>Salt restriction</p> Signup and view all the answers

    In the treatment of edema, it is important to impose a restriction on __________.

    <p>salt</p> Signup and view all the answers

    Match the following terms with their appropriate actions:

    <p>Salt restriction = Reduces fluid retention Diuretics = Increases urine production Fluid intake increase = May worsen edema High-protein diet = Not specific to edema treatment</p> Signup and view all the answers

    Which of the following is a type of podocytopathy that does not present with podocytopenia?

    <p>Minimal change disease (MCD)</p> Signup and view all the answers

    Every patient with minimal change disease (MCD) presents with microhematuria.

    <p>False</p> Signup and view all the answers

    Name one secondary cause of minimal change disease (MCD).

    <p>Drugs, allergy, or malignancy.</p> Signup and view all the answers

    Focal segmental glomerulosclerosis (FSGS) is associated with a greater than _____ risk of chronic kidney disease (CKD).

    <p>2/3rd</p> Signup and view all the answers

    Match the following types of podocytopathies with their associated CKD risk:

    <p>Minimal change disease (MCD) = No risk for CKD Focal segmental glomerulosclerosis (FSGS) = &gt;2/3rd CKD Membranous nephropathy (MN) = 1/3rd CKD Diabetic nephropathy (DN) = Risk of CKD present</p> Signup and view all the answers

    What percentage of patients present with nephrotic syndrome according to the 75/25 rule?

    <p>75%</p> Signup and view all the answers

    The most common cause of nephrotic syndrome in adults is Minimal Change Disease (MCD).

    <p>False</p> Signup and view all the answers

    Name one drug that can cause nephrotic syndrome.

    <p>NSAIDS</p> Signup and view all the answers

    The most important malignancy associated with nephrotic syndrome is __________.

    <p>colorectal carcinoma</p> Signup and view all the answers

    Which of the following is NOT a drug associated with causing FSGS?

    <p>Ibuprofen</p> Signup and view all the answers

    Sickle cell anemia is a known cause of secondary FSGS.

    <p>True</p> Signup and view all the answers

    Match the following autoimmune diseases with their corresponding association with nephrotic syndrome:

    <p>SLE class V = Autoimmune Rheumatoid arthritis = Autoimmune Sarcoidosis = Autoimmune Cystic fibrosis = Autoimmune Bronchiectasis = Autoimmune</p> Signup and view all the answers

    What gene is associated with autosomal recessive FSGS and what protein does it encode?

    <p>NPHS gene, which encodes Nephrin.</p> Signup and view all the answers

    The main treatment for secondary perihilar FSGS that is resistant to immunosuppression therapy is _____ .

    <p>Transplantation</p> Signup and view all the answers

    Match the following genetic forms of FSGS with their characteristics:

    <p>Autosomal Recessive = Presents at birth, Nephrin, Diffuse mesangial sclerosis Autosomal Dominant = Presents in adolescents, α-actin 4 or TRPC 6</p> Signup and view all the answers

    What is the first-line treatment for steroid-resistant nephrotic syndrome in children?

    <p>Calcineurin inhibitors</p> Signup and view all the answers

    Cyclophosphamide is used to treat steroid-dependent nephrotic syndrome.

    <p>True</p> Signup and view all the answers

    What is the maximum duration for the treatment with calcineurin inhibitors?

    <p>2 years</p> Signup and view all the answers

    ___ is characterized by ≥ 2 relapses within 6 months.

    <p>Frequently relapsing nephrotic syndrome (FRNS)</p> Signup and view all the answers

    Match the following treatments with their associated conditions:

    <p>MMF = Steroid-dependent nephrotic syndrome Rituximab = Steroid-dependent nephrotic syndrome Oral cyclophosphamide = Steroid-sparing drug Levamisole = Steroid-sparing drug</p> Signup and view all the answers

    What urine protein creatinine ratio (PCR) indicates nephrotic syndrome in adults?

    <p>≥3000 mg/g</p> Signup and view all the answers

    The underfill theory is a minor contributor to edema pathophysiology in childhood nephrotic syndrome.

    <p>False</p> Signup and view all the answers

    What are the three primary components that characterize childhood nephrotic syndrome?

    <p>Proteinuria, Hypoalbuminemia, Edema</p> Signup and view all the answers

    In childhood nephrotic syndrome, hypoalbuminemia leads to a loss of _____ pressure.

    <p>capillary oncotic</p> Signup and view all the answers

    Match the following conditions with their association with nephrotic syndrome:

    <p>Diabetes = Most common condition Focal segmental glomerulosclerosis (FSGS) = Secondary cause Amyloidosis = Another secondary cause ACE inhibitors = Treatment option</p> Signup and view all the answers

    What is the primary activation that leads to sodium and water retention in nephritic syndrome?

    <p>Renin-angiotensin system activation</p> Signup and view all the answers

    Minimal Change Disease is unresponsive to steroid treatment.

    <p>False</p> Signup and view all the answers

    What is the recommended treatment for ascites in nephritic syndrome?

    <p>Paracentesis</p> Signup and view all the answers

    Low serum albumin is defined as less than __________ g/dL.

    <p>2</p> Signup and view all the answers

    Match the nephrologic conditions with their treatment options:

    <p>Minimal Change Disease (MCD) = Steroid responsive Focal Segmental Glomerulosclerosis (FSGS) = Renal transplantation Membranous nephropathy (MN) = Supportive care IgA nephropathy = Varied based on severity</p> Signup and view all the answers

    What is the most common presentation of glomerular disease?

    <p>Asymptomatic urine abnormalities</p> Signup and view all the answers

    Macrohematuria must always be accompanied by significant proteinuria.

    <p>False</p> Signup and view all the answers

    What is the criteria for nephrotic syndrome in adults regarding proteinuria?

    <p>Nephrotic range proteinuria is greater than 3500 mg/24 hrs.</p> Signup and view all the answers

    The _____ is the term used for the rapid decline in kidney function associated with glomerulonephritis.

    <p>Rapidly progressive glomerulonephritis</p> Signup and view all the answers

    Match the following conditions with their respective presentations:

    <p>Nephritic syndrome = Acute glomerulonephritis Chronic kidney disease (CKD) = Chronic glomerulonephritis Asymptomatic urine abnormalities = Proteinuria and microhematuria Macrohematuria = Presence of dysmorphic RBCs</p> Signup and view all the answers

    Which condition is the most common cause of adult nephrotic syndrome?

    <p>Membranous nephropathy</p> Signup and view all the answers

    Hypoalbuminemia is a characteristic feature of adult nephrotic syndrome.

    <p>True</p> Signup and view all the answers

    What is the typical dosage of steroids for treating adult nephrotic syndrome?

    <p>1 mg/kg/d x 4 months</p> Signup and view all the answers

    The presence of oval fat bodies in urine is commonly associated with __________.

    <p>nephrotic syndrome</p> Signup and view all the answers

    Match the following characteristics with the correct condition related to nephrotic syndrome:

    <p>Focal segmental glomerulosclerosis = Common form of nephrotic syndrome Minimal change disease = Seen in children Amyloidosis = Associated with systemic disease Membranoproliferative glomerulonephritis = Rare cause of nephrotic syndrome</p> Signup and view all the answers

    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

    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

    ### 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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    Test your knowledge on typical and atypical Hemolytic Uremic Syndrome (HUS), as well as the differences between pre-renal injury and acute tubular necrosis. This quiz covers symptoms, treatment options, and diagnostic criteria associated with these conditions. Perfect for medical students and healthcare professionals!

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