Create
Learn
Share

Pathology 2 - Final - Part 2

rename
davidwurbel7's version from 2016-08-08 16:05

Glomerular Diseases

 

Question Answer
All glomeruli are abnormalDiffuse Glomerulonephritis
Only a few glomeruli are abnormalFocal Glomerulonephritis
Fibrosis involving only a segment of the involved glomerulusFocal Segmental Glomerulosclerosis
Hyperplasia of one of the glomerular cell types with or without inflammatory cell infiltrateProliferative Glomerulonephritis
Thick GBM due to immune complex deposition with no proliferative changeMembranous Glomerulopathy
Thick GBM and hypercellular glomeruliMembrano-Proliferative Glomerulonephritis
Proliferation of parietal epithelial cells and mononuclear cell infiltrate form crescent-shape in Bowman’s spaceCrescentric Glomerulonephritis
Conditions that manifest with increased GBM permeabilityNephrotic Syndrome
Conditions that manifest with inflammatory damage to glomeruliNephritic Syndrome
The amount of protein loss in urine is less than 3.5 gNephritic Syndrome
The amount of protein loss in urine is 3.5 g or moreNephrotic Syndrome
Patients complain of smoky colored or coca colored urine. One of the significant findings is presence of RBC castsNephritic Syndrome
The inflammatory damage seen in Nephritic Syndrome is due to these cellsNeutrophils
Clinical finding include, Proteinuria, Hematuria, Azotemia, RBC cast, Oliguria and Hypertension (PHAROH)Nephritic Syndrome
Nephritic syndrome that arises after infection of the skin or pharynx by Nepritic strains of Group A Beta hemolytic streptococci. Sometimes can also arise after infection with non-streptococcal organisms as wellPoststreptococcal Glomerulonephritis (PSGN)
The most common glomerular disease worldwideIgA Glomerulopathy
Is characterized by deposition of IgA immune complex in the mesangium of glomeruli. Most common presentation is nephritic. Occurs following a mucosal infection (respiratory or GIT)IgA Glomerulopathy
Increased mucosal synthesis of IgA and its decreased clearanceIgA Glomerulopathy
IgA Glomerulopathy can be a part of this diseaseHenoch-Schonlein Purpura
Production of antibodies directed against basement membrane. The antibodies are against the non-collagenous component of type IV collagen. Antibodies are directed against BOTH the glomerular and pulmonary capillary basement membranes.Goodpasture's Syndrome
Goodpasture's Syndrome produces this pattern on immunoflourenceLinear Pattern
Treatment for this includes plasma exchange, steroids and cytotoxic drugs (cyclophosphamide, prednisone)Goodpasture's Syndrome
This is the most common type of glomerulopathy in patients with SLEDiffuse Proliferative Glomerulonephritis
Formation of antibodies to DNA. Formation of DNA-antiDNA immunecomplexes. Deposition of ICs diffusely, usually subendothelial. Activation of complement pathwayDiffuse Proliferative Glomerulonephritis
The most common cause of death in patients with SLE is thisChronic Renal Failure
This classification of SLE shows diffuse proliferative GNClass IV
This classification of SLE shows diffuse membranous GNClass V
SLE Class IV has this type nephropathy presentationNephritic Presentation
SLE Class V has this type nephropathy presentationNephrotic Presentation
Negative IF. Associated with C-ANCAWegener granulomatosis
Negative IF. Associated with P-ANCAMicroscopic polyangitis
Negative IF, Associated with P-ANCA. Granulomatous inflammation, asthma, eosinophiliaChurg-Strauss Syndrome
ANCA associated RPGN are also known as this due to the absence or scarcity of Ig and C3Pauci Immune GN
A malignant form of nephritic syndrome, in which progressive loss of kidney function occurs within weeks or months following the primary insultRapidly Progressive Glomerulonephritis
This is histologically characterized by formation of crescent between Bowman’s space and glomerular tuft due to deposition of fibrin proliferation of parietal cells infiltration by monocytes and macrophages in the Bowman’s capsuleRapidly Progressive Glomerulonephritis
Formation of this is an extremely bad prognostic signCrescents
A rare X linked dominant disorder caused by a defect in type IV collagen. Defect is in the synthesis of alpha 5 chain of type IV collagen in GBM. Results in thinning and splitting of GBMAlport’s Syndrome
Autosomal dominant disorder. Extremely thin GBM. Normal renal function. Presents as: Mild proteinuria and persistent microscopic hematuriaThin BM Disease
memorize

Nephrotic Syndromes

Question Answer
These types of casts are seen with nephrotic syndromeFatty Casts
Under polarized light, oval fat bodies demonstrate this appearanceMaltese Cross Appearance
Clinical findings include Hypoalbuminemia, Edema, Lipid abnormalities and Proteinuria (HELP)Nephrotic Syndrome
The most common cause of nephrotic syndrome in childrenMinimal Change Disease (MCD)
The most common cause of nephrotic syndrome in patients with HIV, sickle cell disease or heroin addictFocal Segmental Glomerulosclerosis (FSGS)
The most common cause of nephrotic syndrome in adultsMembrano-Proliferative Glomerulonephritis (MPGN)
Glomeruli appear normal under light microscope. EM shows loss (effacement) of VEC foot processes leading to increased glomerular permeabilityMinimal Change Disease (MCD)
Usually idiopathic; may be associated with Hodgkins lymphomaMinimal Change Disease (MCD)
Often preceded by a respiratory infection. Immune dysfunction leads to the release of cytokines. The cytokine mediated damage to visceral epithelial cells causing loss of negative charge of the GBM. Resulting in selective proteinuria (albumin but not globulin)Minimal Change Disease (MCD)
Treatment with Corticosteroids. Prognosis is excellent with majority have a complete recoveryMinimal Change Disease (MCD)
Light microscopy: Normal glomeruli. Lipid accumulation in proximal tubular cells; Immunofluorescence: Negative; Electron microscopy: Effacement of epithelial (podocyte) foot processes due to fusion or lossMinimal Change Disease (MCD)
Treatment - Short term high dose steroids, ACEi. Prognosis - chronic renal failure within one year without treatmentFocal segmental glomerulosclerosis (FSGS)
Clinical presentation as patients typically present with nephrotic syndrome and Non selective heavy proteinuria (both albumin and globulin)Focal segmental glomerulosclerosis (FSGS)
Light microscopy - Focal and segmental sclerosis and hyalinization of glomeruli (due to trapping of protein in mesangium); Immunofluroscence: Negative; Electron microscopy: Fusion of foot processes of VEC with Focal detachment of visceral epithelial cellsFocal segmental glomerulosclerosis (FSGS)
The most common cause of nephrotic syndrome in Caucasian adultsMembranous glomerulopathy
Usually Idiopathic or secondary to Drugs ( captopril, penicellamine, NSAIDs), Infections: (HBV, syphilis, plasmodium malariae), Malignancy: carcinomas (colon and lung), NHL, Autoimmune disease: SLE (nephrotic presentation)Membranous glomerulopathy
Caused by deposition of immune complexes in the subepithelial zone of glomerular capillaries resulting in thick glomerular BMMembranous Glomerulopathy
Clinical presentation is slow onset of nephrotic syndrome in otherwise healthy patient with nonselective proteinuriaMembranous Glomerulopathy
Any adult presenting with nephrotic syndrome (Membranous glomerulopathy) should be suspected of having thisMalignancy or SLE
Light microscopy: Diffuse GBM thickening due to subepithelial deposits. Silver stain / Electron microscopy: subepthelial deposits in a spike (extension of GBM around deposits) and dome (deposits in the GBM) pattern.Also known as “Hair on end". Immunofluorescence: Granular patternMembranous Glomerulopathy
Characterized by: thickening of Glomerular basement membrane and Proliferation of mesangial cells due to immune complex deposition resulting in splitting of GBM into two layers. This results in “Tram track appearance”Membranoproliferative glomerulonephritis (MPGN)
The most common type of MPGNMembranoproliferative glomerulonephritis (MPGN) Type I
Caused by deposition of immune complex in the subendothelial zone and mesangium. Immune complexes activate the complement pathway resulting in cellular proliferation and injuryMembranoproliferative glomerulonephritis (MPGN) Type I
Membranoproliferative glomerulonephritis (MPGN) associated with HBV and HCV infections, leukemia, lymphomaMembranoproliferative glomerulonephritis (MPGN) Type I
Characterized by electron-dense intramembranous deposits. This is a pathognomonic for this condition. Nature of deposits not knownMembranoproliferative glomerulonephritis (MPGN) Type II
Associated with presence of C3 nephritic factor (C3NeF). It is an autoantibody that binds to C3 convertase. Prevents degradation of C3 converatase. Causing sustained activation of C3, inflammation. Resulting in very low C3 levelsMembranoproliferative glomerulonephritis (MPGN) Type II
Autoantibody that binds to C3 convertaseC3 Nephritic Factor (C3NeF)
Initially manifests as microalbuminuria that later results in nephrotic syndromeDiabetic Glomerulopathy
The most common cause of chronic renal failure in the USADiabetes Mellitus
This is diagnosed by Microscopic findings: Afferent and efferent hyaline arteriolosclerosis. Diffuse glomerulosclerosis. Nodular masses develop in the mesangial matrix. These are referred to as Kimmelstiel-Wilson NodulesDiabetic Nephropathy
Nodular masses developing in the mesangial matrix in Diabetic NephropathyKimmelstiel-Wilson Nodules
Amyloid deposits in the mesangium results in nephrotic syndromeRenal Amyloidosis
memorize