Medicine Marrow Pg No 765-774 (Nephrology)
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Questions and Answers

What is indicated for transplant in nephrology management?

  • Severe dehydration
  • Low blood pressure
  • If chronic kidney disease (CKD) occurs (correct)
  • Normal kidney function

    • Salt restriction is part of conservative management for nephrology treatment.

    True

    What do decreased levels of C3 and C4 indicate in nephrology?

    Classical complement pathway affected

    The presence of ____ deposits is associated with IgA nephropathy.

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

    Match the following terms with their corresponding conditions:

    <p>C3GN = Alternate complement pathway affected PSGN = Transiently low C3 SLE = Full house effect Light chain deposition disease = IgA &amp; K light chain deposit</p> Signup and view all the answers

    Which demographic is more commonly affected by MPGN and IgA Nephropathy?

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

    More than 50% of patients with IgA Nephropathy present with asymptomatic microhematuria and proteinuria.

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

    What percentage of patients with IgA Nephropathy is expected to develop chronic kidney disease (CKD)?

    <p>33%</p> Signup and view all the answers

    A primary indicator of bad prognosis in MEST-C scoring is the presence of __________ deposits outside the mesangium.

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

    Match the prognosis indicators with their significance:

    <p>Mesangial IgA = Good prognosis Endocapillary hypercellularity = Bad prognosis Crescents = Bad prognosis Segmental sclerosis = CKD development</p> Signup and view all the answers

    What is the most common clinical presentation of IgA Nephropathy?

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

    IgA Nephropathy guarantees a good prognosis.

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

    What type of immunoglobulin is predominantly associated with IgA Nephropathy?

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

    IgA Nephropathy is also known as ___ disease.

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

    Match the following clinical findings with their descriptions:

    <p>Nephrotic syndrome = 35% of patients Chronic kidney disease = 20% with contracted kidneys Rapidly progressive glomerulonephritis = 10% of cases Poor prognosis = 100% cases progress to CKD</p> Signup and view all the answers

    What percentage of childhood nephrotic syndrome is attributed to MCD?

    <p>90%</p> Signup and view all the answers

    Selective proteinuria in MCD means that only albumin and transferrin are lost.

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

    What is the preferred steroid sparing agent for FRNS?

    <p>Oral cyclophosphamide</p> Signup and view all the answers

    About _____% of patients with nephrotic syndrome experience infrequent relapse.

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

    Match the treatments to their corresponding conditions:

    <p>Prednisolone = Initial treatment for nephrotic syndrome Mycophenolate mofetil (MMF) = Best treatment for steroid-dependent nephrotic syndrome Rituximab = Alternative treatment for steroid-resistant cases Oral cyclophosphamide = Steroid sparing agent for FRNS</p> Signup and view all the answers

    Which liver disease is most commonly associated with collapsing FSGS?

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

    Immune complexes generated from IgA are effectively cleared by the liver.

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

    What is the most common glomerular disease associated with cirrhosis?

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

    Damage to the mucosa leads to increased production of ______ that is deficient in galactose.

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

    Match each liver disease with its associated glomerular disease:

    <p>HIV = Collapsing FSGS Cirrhosis = IgA nephropathy Hepatitis B = Membranous nephropathy Hepatitis C = Membranoproliferative glomerulonephritis</p> Signup and view all the answers

    Which antigen is primarily associated with primary Membranous Nephropathy?

    <p>m type PLARA</p> Signup and view all the answers

    The Rule of 1/3 in Membranous Nephropathy indicates that one-third of patients experience spontaneous remission.

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

    What is the key feature observed in Electron Microscopy for Membranous Nephropathy?

    <p>Effacement of podocytes and deposits in subepithelial space.</p> Signup and view all the answers

    The only extrinsic antigen identified in Membranous Nephropathy is the ______ specific antigen.

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

    Match the following immunofluorescence findings with their corresponding types of Membranous Nephropathy:

    <p>IF in a° MN (SLE) = + (IgG, IgM, IgA + C1q, C3) IF in I° MN = + IgG1, G2, G3 = + IgG4 = -</p> Signup and view all the answers

    Which variant of FSGS is associated with the worst prognosis?

    <p>Collapsing FSGS</p> Signup and view all the answers

    Tip Variant FSGS has a poor prognosis similar to that of Classical FSGS.

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

    What specific findings are associated with Collapsing FSGS under electron microscopy?

    <p>Dilated cisternae of endoplasmic reticulum (IFN footprints)</p> Signup and view all the answers

    The lesion location in Tip Variant FSGS is at the tip of the ______.

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

    Match the variant of FSGS with its associated observation:

    <p>Classical FSGS = Podocyte proliferation Tip Variant FSGS = Good prognosis Collapsing FSGS = Complete collapse of glomeruli Secondary perihilar FSGS = Associated with systemic diseases</p> Signup and view all the answers

    What is a characteristic finding in the biopsy of IgM Nephropathy?

    <p>Mesangial matrix expansion</p> Signup and view all the answers

    FSGS is associated with poor prognosis compared to MCD.

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

    What treatment is given at a dosage of 1 mg/kg/day for FSGS?

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

    In FSGS, ______________ material can obliterate glomeruli.

    <p>eosinophilic hyaline</p> Signup and view all the answers

    What is the primary treatment protocol for 70% of patients with membranous glomerulopathy?

    <p>Modified Ponticelli regimen</p> Signup and view all the answers

    Match the following conditions with their biopsy findings:

    <p>IgM Nephropathy = Mesangial matrix expansion Focal Segmental Glomerulosclerosis (FSGS) = Focal and segmental involvement of glomeruli Minimal Change Disease (MCD) = Normal histology Chronic Kidney Disease (CKD) = Obliteration of glomeruli</p> Signup and view all the answers

    ACE inhibitors and ARBs are only used for symptomatic patients with podocytopathies.

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

    Name the two medications prescribed during the modified Ponticelli regimen.

    <p>Steroids and oral cyclophosphamide</p> Signup and view all the answers

    The use of __________ is recommended if there is no response to the Ponticelli regimen.

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

    Match the following terms with their descriptions:

    <p>Normal glomerular capillaries = Thin walls Membranous glomerulopathy = Thick capillary walls with foot process effacement ACE inhibitors = Medications for controlling blood pressure Oral cyclophosphamide = Cancer treatment used in nephrology</p> Signup and view all the answers

    What is the principal immunofluorescence finding in Type 1 MPGN?

    <p>IgG + IgM + C3</p> Signup and view all the answers

    Type 2 MPGN is associated with a 80% recurrence post transplant.

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

    What appearance do deposits have on electron microscopy in MPGN?

    <p>Train track appearance</p> Signup and view all the answers

    MPGN is also known as __________ Glomerulonephritis.

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

    Match the types of MPGN with their characteristics:

    <p>Type 1 MPGN = 80% recurrence Type 2 MPGN = 100% recurrence</p> Signup and view all the answers

    Study Notes

    Membranoproliferative Glomerulonephritis (MPGN)

    • MPGN is also known as mesangiocapillary glomerulonephritis.
    • MPGN is a rare disease.
    • MPGN Type 1, also known as classical MPGN, features IgG, IgM, and C3 deposits in the capillary wall and mesangium.
    • 80% of MPGN Type 1 cases experience recurrence after a transplant.
    • MPGN Type 1 can be associated with plasma cell dyscrasias, infections like Hepatitis C, infective endocarditis, leprosy, and malaria, shunts like ventriculoperitoneal shunts, autoimmune diseases like SLE, Sjogren's, and scleroderma, and malignancies.
    • MPGN Type 2, also known as C3GN, features C3 deposits with or without IgG in the capillary wall and mesangium.
    • 100% of MPGN Type 2 cases experience recurrence after a transplant.
    • MPGN Type 2 can be associated with chronic lymphoid leukemia (CLL), acquired partial lipodystrophy of the face, and optic nerve drusen.
    • Electron microscopy reveals a "train track" or "double contour" appearance due to deposits in the subendothelial and mesangial areas.

    IgA Nephropathy

    • IgA nephropathy is the most common glomerular disease.
    • It is also known as Henoch-Schönlein nephritis or Berger's disease.
    • IgA nephropathy is characterized by IgA deposits in the mesangium.
    • It can present with hematuria, proteinuria, and even nephrotic syndrome.
    • It typically progresses to chronic kidney disease (CKD).

    Focal Segmental Glomerulosclerosis (FSGS)

    • FSGS can be classified into different histological variants.
    • Classical FSGS is the most common variant.
    • Secondary perihilar FSGS is another variant.
    • Tip variant FSGS is characterized by lesions at the tip of the glomerulus and has a good prognosis.
    • CollapsingToolbar FSGS is the most severe variant and often leads to end-stage renal disease.
    • Collapsing FSGS is associated with HIV infection, heroin use, and interferon (IFN) α.
    • Collapsing FSGS has a worse prognosis than other FSGS variants.

    Membranous Nephropathy (MN)

    • MN is characterized by thickening of the glomerular basement membrane.
    • It is associated with antibody-mediated immune complex deposition.
    • The immune complexes are usually located in the subepithelial space, leading to a "spike and dome" appearance under electron microscopy.
    • MN can be either primary or secondary.
    • Primary MN is associated with antibodies against phospholipase A2 receptor (PLA2R).
    • Secondary MN is usually triggered by other conditions such as lupus nephritis or infections.
    • MN may spontaneously remit, progress to end-stage renal disease, or have stable disease with continued proteinuria.
    • Transplantation can be a treatment option for MN, but recurrence is possible.

    Minimal Change Disease (MCD)

    • MCD is a common cause of nephrotic syndrome in children.
    • It is characterized by podocyte effacement under electron microscopy.
    • The pathogenesis involves changes in podocyte proteins, particularly those related to the slit diaphragm.
    • MCD commonly relapses, with upper respiratory tract infections (URTI) often triggering relapses.
    • Treatment of MCD involves corticosteroids, but some cases are steroid resistant or steroid dependent.
    • Treatment for MCD with frequent relapses includes steroid-sparing agents such as cyclophosphamide and mycophenolate mofetil (MMF).

    Other Points

    • Mucosal injury can be a contributing factor to glomerular diseases.
    • Cirrhosis can lead to immune complex deposition in the glomeruli due to deficient clearance of immune complexes by the liver.
    • Spondyloarthritis can be linked to glomerular diseases through an immune response.
    • Polymorphic alleles may have a role in the development of FSGS.
    • IgM nephropathy is a rare glomerular disease resembling MCD but with a worse prognosis.
    • Anti-phospholipase A2 receptor (PLA2R) antibodies are a significant biomarker for MN.

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    Description

    This quiz explores the types, characteristics, and associations of Membranoproliferative Glomerulonephritis (MPGN). It covers the intricacies of MPGN Type 1 and Type 2, including their clinical implications and recurrence after transplantation. Test your knowledge on this rare kidney disease and its underlying causes.

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