Medicine Marrow Pg No 775-784 (Nephrology)
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Questions and Answers

Which condition is NOT associated with pulmonary renal syndrome?

  • Diabetes mellitus (correct)
  • Microscopic polyangiitis
  • Granulomatosis with polyangiitis
  • Goodpasture syndrome

    • Goodpasture disease is characterized by antibodies against type-IV collagen.

    True

    What is the recommended maximum duration for the first session of dialysis?

    2 hours

    The main trigger factors for Goodpasture disease include smoking, hydrocarbon exposure, and _____ overload.

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

    Match the HLA types with their associated risk levels:

    <p>DR 2/15 = High risk DR 4 = High risk DR 1 = Low risk DR 7 = Low risk</p> Signup and view all the answers

    What is the treatment option for a patient with a penicillin allergy who has an active sore throat?

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

    Nephrotic syndrome presents with high blood pressure.

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

    What percentage of patients recover from Post-Infectious Glomerulonephritis with prompt treatment?

    <p>99%</p> Signup and view all the answers

    In post streptococcal glomerulonephritis, serum C3 levels are typically _____ in 60% of patients.

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

    Match the following features to the correct syndrome:

    <p>Nephrotic syndrome = Proteinuria is more Nephritic syndrome = RBC cast is present (+)</p> Signup and view all the answers

    Which of the following is NOT a cause of Rapidly Progressive Renal Failure (RPRF)?

    <p>Chronic kidney disease</p> Signup and view all the answers

    Patients with RPGN typically present with normal urine color.

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

    What is one feature of urine examination in RPGN?

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

    A common examination finding in RPGN is __________ puffiness.

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

    Match the investigations with their expected findings in RPGN:

    <p>CBC = Normal Serum Potassium = Raised Serum Creatinine = Raised ABG = Normal/metabolic acidosis</p> Signup and view all the answers

    What is a significant risk associated with performing a biopsy when serum creatinine is significantly elevated?

    <p>Uremic bleeding</p> Signup and view all the answers

    Linear immunofluorescence (IF) is associated only with Goodpasture disease.

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

    What is the procedure to follow if serum creatinine levels are high before performing a biopsy?

    <p>Dialysis, recheck serum creatinine, perform biopsy if decreased.</p> Signup and view all the answers

    Crescentric glomerulonephritis is characterized by the proliferation of parietal epithelial cells with _____ .

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

    Match the following types of immunofluorescence analysis to their descriptions:

    <p>A. Negative IF = No detectable antibodies present B. Diffuse granular IF = Patchy staining typically seen in immune complex diseases C. Linear IF = Antibodies bind along the basement membranes D. Granular IF = Staining that shows scattered deposits of antibodies</p> Signup and view all the answers

    Which of the following is an absolute indication for dialysis?

    <p>Uremic encephalopathy</p> Signup and view all the answers

    Type II glomerulonephritis is the most common in patients younger than 20 years.

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

    What is the primary treatment used for hyperkalemia?

    <p>Calcium gluconate IV</p> Signup and view all the answers

    The presence of linear IgG and C3 in capillary walls indicates __________ syndrome.

    <p>Good pasture</p> Signup and view all the answers

    Match the types of glomerulonephritis with their immunofluorescence findings:

    <p>Type I = Linear IgG + C3 in capillary walls Type II = Immune complex deposition Type III = Low presence of immune complexes</p> Signup and view all the answers

    What is the main treatment approach for this patient?

    <p>Plasma exchange therapy, steroids, and cyclophosphamide</p> Signup and view all the answers

    A patient with Goodpasture syndrome is at risk of recurrence after renal transplant.

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

    What percentage of renal involvement is observed in this patient's condition?

    <p>25%</p> Signup and view all the answers

    The patient presents with _____ and 75% lung involvement.

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

    Match the following conditions with their characteristics:

    <p>Goodpasture Syndrome = No recurrence after renal transplant Alport Syndrome = Post-renal transplant due to A3 defect Type IV RPGN = Condition requiring active management Ongoing pulmonary hemorrhage = A specific treatment requirement</p> Signup and view all the answers

    What is the primary management option for hypertension in patients with pulmonary edema?

    <p>Calcium channel blockers</p> Signup and view all the answers

    PSGN (Post-streptococcal glomerulonephritis) is known to recur.

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

    What type of deposits are observed in the electron microscopy of patients with DPGN?

    <p>Subepithelial camel hump deposits</p> Signup and view all the answers

    The presence of ____ in urine examination indicates possible glomerular damage.

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

    Match the following characteristics with the appropriate investigative techniques:

    <p>Granular IgG + C3 in capillary walls = Immunofluorescence (IF) Subepithelial camel hump deposits = Electron microscopy Exudative neutrophilic capillary infiltration = Histopathology ↑ C3 after 8 weeks = Indication for specific investigation</p> Signup and view all the answers

    What is the most common mode of inheritance for Alport's syndrome?

    <p>X-Linked</p> Signup and view all the answers

    Ocular manifestations of Alport's syndrome typically appear before the age of 20.

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

    What is the clinical feature that indicates worsening renal function at 10-15 years of age in Alport's syndrome?

    <p>Increased creatinine levels</p> Signup and view all the answers

    The most common type of hearing loss associated with Alport's syndrome is __________.

    <p>sensorineural hearing loss</p> Signup and view all the answers

    Match the following clinical features of Alport's syndrome with their corresponding ages:

    <p>Asymptomatic microhematuria = At birth Proteinuria + Hypertension = At 10 years Increased creatinine = 10-15 years Ocular manifestations = 20 years</p> Signup and view all the answers

    What is a key feature of Thin GBM disease?

    <p>Microscopic hematuria</p> Signup and view all the answers

    Goodpasture's syndrome is associated with a mutation in the COL4 A4/A3 gene.

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

    What disease is classified as an X-linked lysosomal storage disease and has a defect in the α-galactosidase enzyme?

    <p>Fabry's disease</p> Signup and view all the answers

    In Fabry's disease, patients may develop ________ in the skin.

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

    Match the conditions with their features:

    <p>Thin GBM Disease = AD, COL4 A4/A3 mutation Goodpasture's Syndrome = Antibody against α3 Fabry's Disease = Acroparesthesia and angiokeratoma Familial Glomerular Syndromes = Good prognosis</p> Signup and view all the answers

    What is the most common presentation associated with Post Streptococcal Glomerulonephritis (PSGN)?

    <p>Acute proliferative glomerulonephritis</p> Signup and view all the answers

    Post-Infectious Glomerulonephritis (PIGN) is primarily associated with Group A beta hemolytic streptococci.

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

    What is typically the prognosis for Post Streptococcal Glomerulonephritis?

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

    In patients with Post Streptococcal Glomerulonephritis, serum C3 levels are typically _____ in 90% of patients.

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

    Match the following clinical features to their corresponding conditions:

    <p>Cola colored urine = Post streptococcal Glomerulonephritis Rapidly progressive glomerulonephritis = Post Infectious Glomerulonephritis Oliguria = Post streptococcal Glomerulonephritis Pulmonary edema = Post Infectious Glomerulonephritis</p> Signup and view all the answers

    Study Notes

    Pulmonary Renal Syndrome

    • Pulmonary renal syndrome is characterized by RPGN (kidney involvement) and diffuse alveolar hemorrhage (lung involvement).
    • The syndrome is associated with conditions such as Goodpasture syndrome, microscopic polyangiitis, granulomatosis with polyangiitis, and SLE (active disease).
    • RPGN + DAH + Antibody (non-specific) = Anti GBM disease, also known as Goodpasture disease.
    • Goodpasture disease is characterized by RPGN, DAH, and antibodies against the noncollagenous domain of the α3 chain of type-IV collagen.
    • Triggers for Goodpasture disease include smoking, hydrocarbon exposure, fluid overload, and lung infection.

    HLA Association

    • High risk HLA associations for Goodpasture disease include DR 2/15 and DR 4.
    • Low risk HLA associations include DR 1 and DR 7.

    Dialysis Disequilibrium Syndrome

    • The first dialysis session should be less than 2 hours, with subsequent sessions lasting 4 hours.
    • Prolonged dialysis sessions exceeding 2 hours can lead to water movement from the blood compartment to the dialysate compartment, causing decreased blood osmolality, cerebral edema, seizures, and potentially death.

    Treatment Approach

    • Initial checks:
      • ECG: Assess for hyperkalemia, treat with calcium gluconate IV 10 ml over 2-3 minutes.
      • ABG: Check for metabolic acidosis, treat with sodium bicarbonate (NaHCO3) or dialysis.
      • Volume status: Evaluate inferior vena cava diameter using Ultrasound (USG), and use diuretics as needed.
    • Absolute indications for dialysis:
      • Uremic encephalopathy
      • Halitosis, pruritis, pericarditis, bleeding, and gastritis.
    • Relative indications for dialysis:
      • Volume overload
      • Hyperkalemia
      • Metabolic acidosis

    Types of Glomerulonephritis

    • Type I/Goodpasture syndrome: Linear IgG + C3 in capillary walls on immunofluorescence.
    • Type II: Immune complex deposition on immunofluorescence. Examples include Systemic lupus erythematosus (most important cause), Henoch-Schonlein purpura, IgA nephropathy (IPGN), and membranoproliferative glomerulonephritis.
    • Type III (pauci-immune): Low presence of immune complexes on immunofluorescence. Examples include ANCA vasculitis, Wegener's granulomatosis, microscopic polyangiitis.

    Diagnosis Table

    • Type IV/Double Positive (Goodpasture syndrome): Anti-GBM antibody positive, ANCA positive, good prognosis.
    • Type V/Double Negative: Anti-GBM antibody negative, ANCA negative, poor prognosis.

    Post Streptococcal Glomerulonephritis

    • Antibiotics are indicated for active sore throat.
      • Single dose: 1.2 million unit Benzathine penicillin
      • Oral penicillin V: 500mg BD x 5-10 days
      • In penicillin allergy: Erythromycin used.
    • Nephrotic syndrome is characterized by extravascular edema, unlike pulmonary edema.
    • 99% of patients recover in 3-5 days with prompt antibiotic treatment.
    • Microhematuria can persist after treatment but is not significant.
    • Follow-up should include monitoring serum C3 levels, which should normalize after 8 weeks.

    Management of PIGN/IRGN

    • Serum C3 levels are usually low (60% of patients).
    • Biopsy findings: IgA predominant ± C3 or C3 predominant, Garland/rope pattern.
      • Prognosis: Poor.
      • Vancomycin (nephrotoxic).
      • Dialysis.
      • Steroids worsen cellulitis, and other antibiotics do not treat RPGN.

    Nephrotic vs Nephritic Syndrome

    • Onset: Nephrotic syndrome is insidious, while nephritic syndrome is abrupt.
    • Edema: Extravascular edema is prominent in nephrotic syndrome, while intravascular edema is more pronounced in nephritic syndrome.
    • Blood pressure: Normal in nephrotic syndrome, high in nephritic syndrome.
    • Jugular venous pressure: Normal in nephrotic syndrome, raised in nephritic syndrome
    • Proteinuria: More prominent in nephrotic syndrome, less prominent in nephritic syndrome.
    • Hematuria: Less prominent in nephrotic syndrome, more prominent in nephritic syndrome.
    • Red blood cell casts: Absent in nephrotic syndrome, present in nephritic syndrome.
    • Serum albumin: Low in nephrotic syndrome, normal/slightly decreased in nephritic syndrome.

    RPGN and Pulmonary Renal Syndrome

    • RPGN is characterized by rapidly progressive renal failure (RPRF) with diffuse proliferative glomerulonephritis with crescents (histological feature).
    • Causes of RPRF: RPGN, hemolytic uremic syndrome (HUS), severe acute interstitial nephritis (AIN), atheroembolic renal disease.
    • Features: Cola-colored urine (RBC present), decreased urine output, periorbital puffiness, progressive edema (extravascular compartment), raised blood pressure.
    • Investigations: Auscultation: Scattered crepitations.
      • Blood investigations: CBC: Normal; Serum potassium: Normal/raised; Serum creatinine: Raised; Serum Urea: Raised; ABG: Normal/metabolic acidosis.

    Biopsy Information

    • Light microscopy: Crescentric glomerulonephritis, proliferation of parietal epithelial cells with fibrin.
    • Immunofluorescence (IF): Linear IF in type I RPGN.
    • Image analysis (IF):
      • Negative IF
      • Diffuse granular IF
      • Linear IF
      • Granular IF.
      • Note: Biopsy should not be done if serum creatinine is significantly elevated.
      • Procedure: Dialysis, recheck serum creatinine, and biopsy if creatinine decreases.
    • Important associations: Linear IF is associated with Goodpasture disease, diabetes mellitus, light chain disease.

    Medical Case Notes

    • Patient ID: 782
    • Channel: @back_ed8
    • Specialty: Nephrology.
    • Presentation: 75% lung involvement, history of smoking, cough, hemoptysis, 25% renal involvement.
    • Prognosis: Poor.
    • Evaluation: Elevated DLCO (Diffused Lung Capacity for Carbon Monoxide), bilateral diffuse infiltrate on X-ray, diffuse alveolar hemorrhage on CT scan.
    • Conditions Requiring Active Management: Ongoing pulmonary hemorrhage, serum creatinine < 5.5 mg/dL with normal urine output, new cellular crescent (non-fibrous), Type IV RPGN.
    • Treatment: Plasma exchange therapy (PLEX) + steroids + cyclophosphamide for 3-6 months.
    • Definitive treatment: Renal transplant (after antibody negative for 6 months).
    • Note: Goodpasture Syndrome: No recurrence after renal transplant.
    • Alport Syndrome: Post-renal transplant due to A3 defect.
    • Risk: 5% chance of Goodpasture syndrome recurrence.

    Pathogenesis of HTN & Pulmonary Edema

    • NAPIr + plasmin activates ENAC (Epithelial Sodium channel), leading to sodium and water retention, ultimately causing hypertension and pulmonary edema.

    Management

    • Basic investigations: RFT: mild impairment; Urine examination: ↑ RBC, Albumin: 1+/2+, WBC casts.
    • Specific investigation:
      • Indications: ↑ C3 > 8 weeks (Likely C3 GN), recurrent episodes (PSGN does not recur).
      • Findings:
        • Histopathology: DPGN (Diffuse proliferative GN), exudative neutrophilic capillary infiltration > mesangial proliferation.
        • Immunofluorescence (IF): Granular IgG + C3 in capillary walls > mesangium (Starry sky pattern) during clinical phase. Only C3 (mesangial) during recovery phase.
        • Electron microscopy: Subepithelial camel hump deposits (Lumpy bumpy deposits).

    Treatment

    Sign Rx
    Hypertension Calcium channel blockers (CCBs): Amlodipine: 2.5-5 mg BP charting hourly
    Pulmonary edema Diuretics: Torsemide: 5-10 mg
    Hyperkalemia (Can cause cardiac complication) ECG monitoring (Check for tall T waves) Serum K⁺ levels checked regularly

    Familial Glomerular Syndromes

    Alport's Syndrome

    • Hereditary Nephritis with mutation in Collagen IV (COL4A5), most commonly a deletion.
      • Table of Skin BM and Affected Chain: | Skin BM | Seen in | |---|---| | α1 - α1 - α2 | Birth | | α3 - α4 - α5 | GBM, Cochlea, Ocular BM | | α5 - α5 - α6 | Skin BM |
    • Inheritance: | Mode of inheritance | Affected chain | |---|---| | X-Linked (80%) | α5 | | AR (15%) | α3/α4 | | AD (5%) | α3/α4 |
    • Clinical features:
      Timeline Features
      Birth Asymptomatic microhematuria
      10 yrs Proteinuria + HTN
      10-15 yrs Creatinine ↑
      15 yrs ENT manifestation: SNHL low frequency (most common) High frequency
      20 yrs Ocular manifestations: Anterior lenticonus
      25-30 yrs Oil droplet appearance on slit lamp
      30 yrs ESRD in X-linked, carriers have mild disease, 40 yrs ESRD in AR, mild disease in AD

    Post Streptococcal Glomerulonephritis

    • Post streptococcal Glomerulonephritis (PSGN):

      • Demographic: Immunocompetent child, Male > Female, Age: 2-7 years, Predisposing infection: Streptococcus (Sore throat < Skin infection)
      • Etiology: Group A beta hemolytic streptococci (GABHS), Strains 1, 2, 4, 12 → Sore throat, Strains 47, 49, 55, 57 → Skin infection
      • Incubation period (IP): Sore throat: 7-10 days, Skin infection: 2-4 weeks
      • Presentation: Acute proliferative glomerulonephritis (most common), ↓ C3 (In 90%)
      • Prognosis: Good (No risk of CKD)

    • Post infective Glomerulonephritis (PIGN)/Infection related glomerulonephritis (IRGN):

      • Demographic: Immuno deficient child, Male > Female, Predisposing factors: Diabetes mellitus
      • Organism: MRSA: methicillin-resistant Staphylococcus aureus
      • Presentation: Rapidly progressive glomerulonephritis (RPGN), ↑ C3 (In 60%)
      • Prognosis: Poor prognosis

    HLA Associations in Glomerular Diseases

    • HLA DR4, HLA DR1
    • Antigens:
      • NAPIr (Nephritis associated plasmin type 2 receptor)
      • SPES (Streptococcal pyogenic exotoxin B)
    • Antibodies: Anti DNAase B (After preceding skin infection)

    Clinical Features

    • Acute presentation (within 1-2 days):
      • Triad: Cola colored urine (Hematuria), Hypertension, Oliguria (RFT impairment)
    • Other features:
      • Pulmonary edema, Hyperkalemia, Diastolic BP fluctuation → ↑ risk of PRES/hypertensive encephalopathy, Seizures.

    Thin GBM disease

    • Autosomal dominant, COL4 A4/A3 mutation.
    • Features: Microscopic hematuria (~5 years), Good prognosis.

    Fabry's Disease

    • X-linked lysosomal storage disease.
    • Defect: α-galactosidase.
    • Features: Acroparesthesia, Angiokeratoma, Accumulation in vessel: Heart, cerebrovascular, cornea verticillata.
    • Investigation:
      • LM, IF: Normal
      • E/m: uniformly thin.

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    Description

    This quiz explores Pulmonary Renal Syndrome, focusing on its characteristics such as RPGN and diffuse alveolar hemorrhage, alongside associations with various diseases like Goodpasture syndrome. Additionally, it discusses risk factors and HLA associations relevant to this syndrome. Test your knowledge on these critical medical concepts!

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