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147 Cards in this Set
- Front
- Back
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what collagen type composes most of the GBM
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type 4
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genetic mutations in adult polycystic kidney disease and which has worse prognosis and which is more common
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PKD1 (more common, worse prognosis) and PKD2
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Location of PKD1
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16p13.3
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location of pkd2
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4q21
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which population has a faster acceleration of renal failure in the setting of Adult polycystic kidney disease
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black b/c sickle cell trait
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name 3 extrarenal congenital anomalies in patients with adult polycystic kidney disease
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liver cysts (also rarer spleen, pancreas, lungs), berry aneurysms, mitral valve prolapse
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genetics of childhood polycystic kidney disease
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PKHD1
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chromosomal location of PKHD1
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6p21-23
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Other findings in patients with childhood polycystic kidney disease
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hepatic fibrosis with proliferation of portal bile ducts in children that survive
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what is medullary sponge kidney
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a usually incidental finding of dilated collecting ducts in medulla
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what is nephronophthisis-medullary cystic disease complex
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a group of diseases characterized by cysts at the corticomedullary junction
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name 5 genetic alterations identified with nephronophthisis-medullary cystic disease complex
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two groups: NPH1, 2 and 3 represent the juvenile forms, autosomal recessive; also MCKD1 and 2, autosomal dominant)
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Poststrep glomerulonephritis: clinical presentation, pathogenesis, LM, fluorescence, EM
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Acute nephritis, antibody mediated, LM: diffuse proliferation and wbcs, FM: granular IgG and C3 in GBM and mesangium, EM: subepithelial humps
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Idiopathic RPGN: clinical presentation, pathogenesis, LM, fluorescence, EM
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GN, antiGMB antibody or IC or ANCA associated; LM: proliferation, focal necrosis, crescents, fluoro: linear IgG and C3; granular IgG or IgA or IgM, negative in anca-associated; no dpositis
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Membranous GN: clinical presentation, pathogenesis, LM, fluorescence, EM
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nephrotic syndrome, in situ antibody mediated; diffuse capillary wall thickening, granular IgG and C3, subepithelial deposits
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Minimal change disease: clinical presentation, pathogenesis, LM, fluorescence, EM
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nephrotic; only em finding: loss of foot processes - no deposits
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Focal segmental glomerulosclerosis: clinical presentation, pathogenesis, LM, fluorescence, EM
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nephrotic syndrome, LM: focal and segmental sclerosis and hyalinosis; focal findings on FM: igM and C3; EM: loss of foot processes and epithelial denudation
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MPGN Type I: clinical presentation, pathogenesis, LM, fluorescence, EM
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nephrotic, IC, mesangial proliferation, bm thickening and splitting, FM: IgG + C3, C1q + C4; subendothelial deposits
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Dense deposit disease (MPGN type II): clinical presentation, pathogenesis, LM, fluorescence, EM
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hematuria, chronic renal failure; autoantibody, altnerative complement pathway: mesangial proliferation, bm thickening and splitting; C3 +/-IgG, dense deposits by EM
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IgA nephropathy: clinical presentation, pathogenesis, LM, fluorescence, EM
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recurrent hematuria/proteinuria, focal proliferative GN, mesangial widening; IgA+/-IgG, IgM and C3 in mesangium, EM: mesangial and paramesangial dense deposits
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Chronic Glomerulonephritis: clinical presentation, pathogenesis, LM, fluorescence, EM
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chronic renal failure, hyalinized glomeruli, granular or negative fluor.
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genetic defects in alports
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defect collagen IV; mutation in COL4A5; see ocular, hearing defects as well
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what is thin basement membrane disease
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thinning of bm, hematuria clinically, also traced to chains on collagen IV; seen in women too (as autosomal recessive)
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clinical features of tuberous sclerosis
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epilepsy (with lesions in the cerebral cortex, mental retardation), skin findings and obviously the tumor associations
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Cytogenetic and genetic abnormalities in sporadic papillary ca of kidney
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Cytogenetic: trisomy 7, 16, 17, loss of Y
Genetic: mutated, activated MET, t(X;1)- PRCC oncogenes |
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Cytogenetic and genetic abnormalities in hereditary papillary ca of kidney
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Cytogenetic: trisomy 7
Genetic: muttated activated MET |
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Cytogenetic and genetic abnormalities in sporadic clear cell ca of kidney
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Cytogenetic: translocations of 3;6, 3;8, 3; 11 and del czome 3
Genetic: mutations/hypermeth of VHL |
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cell of origin for oncocytoma and for chromophobe ca
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intercalated cells of collecting duct
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prognosis of chromophobe ca
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excellent compared to clear cell and papillary renal cell
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histologic features of collecting duct carcinoma
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nests of malignant cells within a prominent fibrotic stroma; often hobnailing, very atypical, sometimes intratubular atypia
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VHL gene - what does it encode?
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protein that is part of a ubiquitin ligase complex involved in targeting other proteins for degradation; it is a tumor suppressor gene and mutations of it allow for both angiogenetic factors (VEGF and TGFb1) and growth factors (ex. ILGF1) to go unchecked
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MET - what does it encode?
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proto-oncogene that when mutated encourages growth
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PRCC - what's its story
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mutations cause a fusion protein that dysregulated mitotic checkpoints causing abnl segregation of czomes
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what can be used to distinguish AL from AA in amyloid deposition
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potassium permanganate can make AA dissolve away (not detectable any longer by congo red) vs. AL lasts
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what is thioflavine T used for?
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it is a dye that binds affividly to beta pleated sheet structures like amyloid and will fluoresce, and thus be positive in amyloid
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how big are amyloid fibrils on EM
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8 um
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most common cause of nephrotic syndrome in adults
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membranous GN
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most common de novo disease in renal transplant recipients
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membranous GN
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common complication of membranous GN
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renal vein thrombosis
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silver stain on membranous GN
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moth eaten around deposits (capillary loop thickening without increased cellularity)
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IF in postinfectious gN
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granular IgG and C3 in mesangium and GBM
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EM in postinfectious GN
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subepithelial humps
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lab findings in poststrep GN
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ASO strep antibody titers, decreased complement, cryoglobulins
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how do MPGN I and II compare on LM
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Similar, MPGN I is more cellular
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what is the fluor for dense deposit disease (MPGN II)
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C3 and IgG - granular in the GBM/mesangium but linear capillary loops
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classic EM findings for MPGN II (dense deposit disease)
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intramembranous deposits
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alternative name for MPGN II
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dense deposit disease
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Silver stain with MPGN I and II
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double contours, tram- tracking
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do MPGN I and II recur in transplant patients
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yes, 90% in II, still a lot in I
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lab findings in MPGN I
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low serum complement (activates complement cascade)
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IF for MPGN I
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C3 in a granular pattern, IgG and early complement (C1q, C4)
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Primary causes of nephrotic syndrome (3)
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minimal change disease, FSGS, membranous GP
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Two examples of secondary causes of nephrotic syndrome
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diabetes, amyloid
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what is the only change in MCD of the kidney
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effacement of foot processes by EM
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What are some secondary causes of MCD in adults (4 categories with specific causes)
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Drugs (Nsaids, lithium); toxins (bee stings, mercury, lead), infections (mono, immunizations); tumors (hodgkin's)
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Population in which idiopathic FSGS is not uncommon
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blacks
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what are 4 symptoms seen in FSGS
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nephrotic, hematuria, HTN and less often renal insufficiency
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what does the term focal in renal pathology refer to
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less than 50% of glomeruli involved
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what does the term diffuse in renal pathology refer to
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greater than 50% of glomeruli involved
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Name 3 clinical settings in which FSGS can occur
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1. solitary kidney; 2. morbid obesity; 3. iv drug use (heroin)
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do steroids help FSGS
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no not many
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does collapsing FSGS have a worse or better prognosis than classical FSGS
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worse
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who gets collapsing FSGS
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increased occurrence in blacks
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what virus is associated with collapsing FSGS
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parvovirus
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histologic features of collapsing FSGS
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collapse of glomerular tuft and proliferation and hyperplasia of glomerular visceral epithelial cells
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what pathology is most commonly associated with HIVAN
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collapsing FSGS with dilation of tubules and casts
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histologic features of membranous GN
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Diffuse thickening of capillary walls of glomeruli produced by sub-epithelial immune deposits and associated basement membrane reaction.
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Four categories of clinical settings in which membranous glomerulonephropathy can occur
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Drugs: Penicillamine, Gold
Infections: Hepatitis B Neoplasms: carcinoma, non-Hodgkins lymphoma Immunologic disorders: SLE, RA, sarcoidosis |
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clinical pathways in which those with membranous GN might progress
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1/3, 1/3, 1/3 remission, status quo, progress to ESRD within 15 years
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silver stain for membranous GN
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Holes, “spikes”, or even double contours in the silver stain, depending on the stage of disease
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IF for membranous GN
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granular IgG and C3
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pathogenesis for membranous GN
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in situ antigens with ab depositing
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Histologic findings in diabetic glomerulosclerosis
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Mesangial matrix increaseing with mesangial cells producing Kimmelstiel-Wilson nodules. Not pathognomonic of diabetes!
Hyaline changes in arterioles (pathognomonic) and tubular BM thickening |
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the three diseases with nephritic syndrome and low complement
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post infectious, SLE, MPGN (II?)
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The two disease states with nephritic syndrome and normal complement
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igA and antiGBM (goodpasture's)
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IF and EM in postinfectious GN
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IF: granular IgG and C3
EM: subepithelial humps (recall immune complex etiology) |
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3 common lab findings in SLE patient
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+ANA, +dsDNA and low complement
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30 year old Asian man with recurrent hematuria following a flu-like illness - what disease?
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IgA (berger's disease) nephropathy
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IF in IgA nephropathy
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IgA, C3 and less IgG in mesangial region
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Em for IgA nephropathy
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mesangial and paramesangial deposits
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what systemic, self-limited illness resembles IgA nephropathy on biopsy
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Berger's disease
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pathogenesis of MPGNI
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immune complex, with low complement, mesangial thickening and extension into capillary loops
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which glomerulonephritides have deposits and where?
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subepithelial: post infectious and membranous
subendothelial: MPGN1 Mesangial: IgA Not specified: RPGN (if IC), MPGN II (DDD) |
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RPGN can be divided into two (or three) etiologies what are they
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Goodpasture's (antiGBM COL4 A3 antigen); idiopathic (anti-GBM IC; ANCA assoiated)
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If you see crescents what four pathologies should you think of
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SLE, postinfectious, IgA, antiGBM/RPGN
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in whom would one find collapsing FSGS (two prominent populations)
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blacks, HIV
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Findings of class II lupus nephritis
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mesangial proliferative lupus nephritis
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Findings of class III lupus nephritis
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focal proliferative lupus nephritis
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findings of class IV lupus nephritis
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diffuse proliferative lupus nephritis
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findings of class V lupus nephritis
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membranous lupus nephritis
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describe lupus anticoagulant syndrome
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caused by anticardiolipin or antiphospholipid antibodies and produces a thrombotic microangiopathy, can find fibrin thrombi in kidneys
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Causes of membranous glomerulopathy (5 categories with specific causes)
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Primary (75%) are idiopathic. Secondary are seen with autoimmune/collagen vascular disease (ex lupus, RA), drugs (gold, penicillamine, NSAIDs), infection (hep B or C), tumors (carcinomas including breast, lung, GI)
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histologic features of thrombotic microangiopathies
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thrombi, mucoid intimal edema, endothelial injury
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Differential diagnosis of thrombotic microangiopathy of the kidney (6)
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HUS, TTP, malignant HTN, scleroderma, antiphospholipid/anticardiolipin antibody syndrome, pre-eclampsia etc.
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causes of papillary necrosis
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NSAIDs, DM, acute pyelonephritis
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4 patterns of calcineurin nephrotoxicity
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isometric tubular vacuolization, hyaline arteriolopathy, striped fibrosis with tubular atrophy and thrombotic microangiopathy
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acute antibody mediated rejection in kidney 3 criteria
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1. peritubular capillary distribution of C4d, 2. donor-specific antibodies in recipient, 3. acute tissue injury (PMNs in capillaries)
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cresents with granular IF/IC disease: (4 diseases)
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lupus, MPGN, postinfectious, IgA
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cresents with linear IF
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antiGBM, goodpasture's if involves lungs
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cresents with minimal/pauci-immune
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Most common causes of cresents in adults: most associated with ANCA+, Wegener's, microscopic polyangiiis, Churg-Strauss, renal-limited puaci=immune cresenteric GN
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another "name" for TRIs
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IFN footprints
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two diseases in which TRIsare most commonly encountered
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HIV, lupus, (and if treated with IFN)
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most common light chain in amyloid
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lambda
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most common light chain in LCDD
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kappa
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hallmark histologic feature of acute cellular rejection in kidney
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interstitial inflammation across tubular membranes/tubulitis (along with endovasculitis)
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HCV is associated with which glomerulonephritides (3)
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MPGN, cryoglobulinemic GN (similar to MPGN), membranous
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what is the cryoglobulin in HCV-associated cryoglobulinemic GN
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mixed - monoclonal igMkappa with polyclonal iGG
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spikes on silver stain mean which disease
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membranous
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causes of ATN
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ischemic, toxic (drugs, metals, solvents, radiation, contrast, osmotic agents like mannitol), etc.
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microhematuria without significant proteinuria
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thin basment membrane, alports and igA nephropathy
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double counters - think which glomerular disease
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MPGN (mesangial cells grow between layers of GBM)
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IF of DDD
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C3 only
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clinical findings in DDD
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proteinuria, hematuria and depressed serum C3
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what is Fabry's disease
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x-linked lysosomal storage disease resulting from alpha-galactosidase A deficiency
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Fabry's disease by EM
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zebra bodies (intracytoplasmic lamellated, myelin-like bodies with periodicity of 6-8 nm)
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clinical findings in fabry's disease (7)
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acroparesthesias, angiokeratomas, hypohidrosis, corneal and lenticular opacities. progressivve renal, cardiac and cerebrovascular disease
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what is fibrillry GN
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adults with hematuria, proteinuria and renal insufficiency; mesangial proliferation/endocapillary proliferation, cresents; randomly oriented fibrils 16-24 nm in mesangium, BM by EM; these are immune deposits; IF is positive for IgG, C3, kappa and lambda
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is fibrillary GN steroid responsive or no
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no
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associated underlying conditions for secondary amyloid
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RA (most common), psoriasis, ankylosing spondylitis, TB, crohn's, infections, IVDA, familial Mediterranean fever
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in secondary amyloidosis what is the precursor protein
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serum amyloid A (SAA), acute phase reactant of liver
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which is more common - primary or secondary amyloidosis
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primary in US (AL)
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HSP shows what on renal biopsy
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proliferative GN (mesangial cresent) with dominant staining for IgA (think systemic IgA)
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what infectious is HUS associated with
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E coli 0157:H7 and shigella dysenteriae
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name three conditions/diseases that secondary FSGS has been associated with
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hiv, heroin abuse, loss of renal mass
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gene mutated in adult PKD
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PKHD1, czome 6; encodes fibrocystin (regulates epithelial cell proliferation and adhesion)
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common cause of acute interstitial nephritis
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NSAIDs
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NSAID-induced injury patterns (4)
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AIN, MCD, membranous, papillary necrosis
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most common cause of nephrotic syndrome in adults
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membranous
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most common cause of nephrotic syndrome in adult blacks
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FSGS
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papillary necrosis arises in what four clinical settings
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obstructive pyelonephritis, sickle cell anemia, diabetes and analgesic abuse
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wegener's granulomatosis affects which vessels (small, medium or large) and what organs (3)
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small
kidney, lung, URtract |
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what key lab is found in wegeners
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cANCA (cytoplasmic, indirect IF, target is proteinase-3 within primary granules of neutrophils/monocytes)
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wegeners in kidney - histologic appearance
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necrotizing, crecentic GN (pauci-immune)
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contract microscopic polyangiitis vs. wegeners
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like wegener's, microscopic polyangiitis is a necrotizing/cresentic GN that is pauci-immune
unlike wegener's MPA is not granulomatous, has a wider tissue distribution (kidney, skin/mucosa, lungs, brain heart, GI and muscle) most associated with pANCA (p is perinuclear which is artifactual) |
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what is churg strauss (renal findings, clinical findings and lab (1) finding)
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small vessel vasculitis, associated with pauci-immune necrotizing/crescentic GN with granulomatous inflammation and eosinophils; history of asthma and eosinophilia; pANCA+
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common cause of acute interstitial nephritis
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NSAIDs
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NSAID-induced injury patterns (4)
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AIN, MCD, membranous, papillary necrosis
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most common cause of nephrotic syndrome in adults
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membranous
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most common cause of nephrotic syndrome in adult blacks
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FSGS
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papillary necrosis arises in what four clinical settings
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obstructive pyelonephritis, sickle cell anemia, diabetes and analgesic abuse
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wegener's granulomatosis affects which vessels (small, medium or large) and what organs (3)
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small
kidney, lung, URtract |
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what key lab is found in wegeners
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cANCA (cytoplasmic, indirect IF, target is proteinase-3 within primary granules of neutrophils/monocytes)
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wegeners in kidney - histologic appearance
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necrotizing, crecentic GN (pauci-immune)
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contract microscopic polyangiitis vs. wegeners
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like wegener's, microscopic polyangiitis is a necrotizing/cresentic GN that is pauci-immune
unlike wegener's MPA is not granulomatous, has a wider tissue distribution (kidney, skin/mucosa, lungs, brain heart, GI and muscle) most associated with pANCA (p is perinuclear which is artifactual) |
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what is churg strauss (renal findings, clinical findings and lab (1) finding)
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small vessel vasculitis, associated with pauci-immune necrotizing/crescentic GN with granulomatous inflammation and eosinophils; history of asthma and eosinophilia; pANCA+
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most common cause of renovascular HTN in young women
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fibromuscular dysplasia (developmental disorder of medium sized muscular arteries), sausage string deformity by imaging that is not at aortic ostia
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basic lesion of diabetic nephropathy
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thickening of BM with nodular formation (kimmelstiel-wilson)
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