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89 Cards in this Set
- Front
- Back
which has more protein excreted in the urine: nephrotic or nephritic?
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nephrotic
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intrinsic (or primary) kidney disease is normally caused by what?
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Typically is immune-mediated +/- inflammatory cell involvement -->hence, glomerulonephritis
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Hematuria, azotemia, variable proteinuria,
oliguria, edema, and hypertension what syndrome? |
Nephritic syndrome
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3.5 gm/day proteinuria,
hypoalbuminemia, hyperlipidemia, lipiduria what is the syndrome |
nephrotic syndrome
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Azotemia ➙ uremia progressing for
months to years syndrome? |
chronic renal failure
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Acute nephritis, proteinuria, and acute
renal failure syndrome? |
Rapidly progressive glomerulonephritis
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linear, granular, and clumping on immunofloruecence shows what?
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deposition of immune complexes
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What are the 5 types of nephrotic syndromes?
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Minimal change disease
Membranous glomerulopathy Focal segmental glomerulosclerosis (FSGS) Diabetic glomerulosclerosis Amyloid nephropathy |
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Minimal change disease
Membranous glomerulopathy Focal segmental glomerulosclerosis (FSGS) Diabetic glomerulosclerosis Amyloid nephropathy are what type of syndromes? |
nephrotic
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Most common form of nephrotic syndrome in children ?
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Minimal Change Disease (MCD)
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this nephrotic syndrome Tends to be fairly “albumin selective” proteinuria (albumin >>Ig)
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Minimal Change Disease (MCD)
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LM: normal glomerulus
IF: negative EM: effacement of podocyte foot processes (therefore disrupted filtration slits); no immune deposits findings of? |
Minimal Change Disease (MCD)
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Tx for minimal change disease?
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Most experience remission with corticosteroid Tx
Very good response in children Upon withdrawal of corticosteroids, relapses for up to ~ 10 yrs |
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Most common cause of nephrotic syndrome in adults (Caucasians, Asians)
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Membranous Glomerulopathy
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LM: thickened capillary walls; no hypercellularity; silver stain can show spikes along thickened loops
IF: confluent granular staining for IgG, C3 along capillary loops EM: electron dense sub-epithelial immune deposits 4 stages of severity of deposits what are these findings of? |
Membranous Glomerulopathy
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EM: electron dense sub-epithelial immune deposits
found in? |
Membranous Glomerulopathy
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IF: confluent granular staining for IgG, C3 along capillary loops
found in? |
Membranous Glomerulopathy
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LM: thickened capillary walls; no hypercellularity; silver stain can show spikes along thickened loops
found in? |
Membranous Glomerulopathy
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LM: normal glomerulus
found in? |
Minimal Change Disease (MCD)
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IF: negative
found in? |
minimal change disease
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EM: effacement of podocyte foot processes (therefore disrupted filtration slits); no immune deposits
found in? |
minimal change disease
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Scarring of portion of some glomeruli =
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Focal Segmental Glomerulosclerosis (FSGS)
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Heterogeneous group of diseases with different etiologies, pathogenesis, treatments, and outcomes =
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Focal Segmental Glomerulosclerosis (FSGS)
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how many variants based on morphology are there in primary FSGS?
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4
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this variant of FSGS is seen in HIV-assoicated nephropathy... it is very aggressive (ESRD in <1)
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collapsing variant
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most common cause of nephrotic disease in African-Americans?
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FSGS
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Most common presentation: insidious onset of asymptomatic proteinuria with frequent progression to nephrotic syndrome =
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FSGS
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LM: increased ECM, hyalinosis
May be confused with MCD on biopsy as pathologic features are focal and segmental IF: trapping of IG and C3 in sclerotic regions EM: diffuse effacement of foot processes, loss of podocytes and collapse of capillaries with increased ECM finding in |
FSGS
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May be confused with MCD on biopsy ....
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FSGS
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LM: increased ECM, hyalinosis
May be confused with MCD on biopsy as pathologic features are focal and segmental |
FSGS
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EM: diffuse effacement of foot processes, loss of podocytes and collapse of capillaries with increased ECM
findings of |
FSGS
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what are beneficial treatments for FSGS (2)
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Corticosteroids and ACE inhibitors typically beneficial
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Periodic acid-Schiff (PAS) staining shows perihilar areas of segmental sclerosis and adjacent adhesions to Bowman's capsule
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FSGS
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Associated with small vessel disease (sclerosis) throughout body
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Diabetic Glomerulosclerosis (GS)
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Presents as microalbuminuria, progresses to overt proteinuria and perhaps nephrotic syndrome in 10-15 yrs post dx of type 1 or 2 diabetes
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Diabetic Glomerulosclerosis (GS)
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etiology of Generalized increase in BM material synthesis within microvasculature
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Diabetic Glomerulosclerosis (GS)
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LM finding of Diffuse thickening of BM region and proliferation and expansion of mesangium =
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Diabetic GS
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Kimmelstiel-Wilson nodules (PAS- and silver- positive acellular, nodular mesangial sclerosis) =
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Diabetic GS
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Hyaline arteriololar sclerosis =
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Diabetic GS
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~ 30% develop ESRD (leading cause in USA)
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Diabetic GS
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basement membrane (BM) is thickened, and there is an increase in mesangial matrix material.
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Diabetic GS
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Renal involvement typical in either AA or AL forms of systemic amyloidosis
Nonselective proteinuria; 60% develop nephrotic syndrome |
Amyloid Nephropathy
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which amyloid is associated with chronic inflammatory process
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AA Amyloid
seen in Amyloid Nephropathy |
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person with nephrotic syndrome that has lots of plasma cells or multiple myeloma, what should you be thinking
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Amyloid Nephropathy
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which amyloid is associated with plasma cell/B cell neoplasm, derived from light chains of Ig molecules (multiple myeloma)
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AL amyloid
seen in Amyloid Nephropathy |
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LM: Congo red stain: apple-green birefringence with polarized light = ?
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Amyloid Nephropathy
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Tx for AA amyloidosis?
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remember this is an inflammatory process so
potent anti-inflammatory agents (e.g., colchicine |
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Tx for AL amyloidosis?
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chemotherapy used to treat multiple myeloma
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T/F
amyloid deposits are not periodic acid-Schiff–positive |
True
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Associated with hematuria, azotemia (elevated BUN), and variable subnephrotic proteinuria & edema
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Nephritic syndrome
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One of most common pediatric kidney diseases
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Acute Post-Infectious GN
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the following are what type of syndromes?
Acute post-infectious glomerulonephritis Membranoproliferative glomerulonephritis ---Type 1 and Type 2 Lupus nephritis IgA nephropathy Anti-GBM glomerulonephritis |
Nephritic Syndromes
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pt has pharygitis, then 2-4 weeks after resolution they have a Type II HS rxn occur affecting the kidney, what do they have
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Acute Post-Infectious GN
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Most often related to nephritogenic strains of group A strep is the etiology of what?
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Acute Post-Infectious GN
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LM: proliferative GN
Early: increase mesangial matrix with increase PMNs Late: mildly hypercellular glomeruli |
Acute Post-Infectious GN
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IF: “lumpy-bumpy” IgG, C3 around capillaries, mesangium
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Acute Post-Infectious GN
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EM: sub-epithelial humps
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Acute Post-Infectious GN
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prognosis for Acute Post-Infectious GN
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Majority improve to baseline within months
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Proliferation of glomerular cells, alteration in GBM, infiltration by WBCs
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Membranoproliferative GN (MPGN)
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Can occur at any age; most frequent in older children and younger adults
More prevalent in underdeveloped countries with high prevalence of chronic infections Often have low levels of complement (C3) |
Type I Membranoproliferative GN (MPGN)
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in Type I Membranoproliferative GN (MPGN) where do immune complexes get deposited? 2
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mesangium
subendothelial capillary walls |
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LM of glomerular crescents
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Type I Membranoproliferative GN (MPGN)
Anti-GBM GN Rapidly Progressive GN |
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Silverstain: doubling ("replication") of GBM...akak tram-tracking
seen in? |
Type I Membranoproliferative GN (MPGN)
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EM: subendothelial and mesangial dense deposits (immune complexes)
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Type I Membranoproliferative GN (MPGN)
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prognosis of: Treatment of underlying disease process (if secondary)
~ 50% patients progress to ESRD |
Type I Membranoproliferative GN (MPGN)
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More pronounced hypocomplementemia
Prognosis typically worse no immune complex deposition... what is this? |
Type II MPGN: Dense Deposit Disease
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Extensive deposition of complement in GBM and mesangial matrix; virtually no Ig (NOT immune complex)
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Type II MPGN: Dense Deposit Disease
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Most patients have IgG autoantibody, the C3 nephritic factor (stabilizes activated C3 convertase (C3bBb)
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Type II MPGN: Dense Deposit Disease
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IF and EM: “ribbon-like” zone of increased density within the GBM and mesangial matrix
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Type II MPGN: Dense Deposit Disease
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Prognosis
Lack of effective treatment with variable progression Some patients develop numerous glomerular crescents and picture of rapidly progressive GN (RPGN) ~ 50% develop CRF within 10 yrs Relatively high recurrence within transplanted kidneys |
Type II MPGN: Dense Deposit Disease
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Of the Glomerular Epithelial cells (visceral cells, or podocytes; parietal cells), which type is the LEAST likely to be involved with glomerular pathology?
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Parietal Cells
**Only a factor in Crescentic GN** (Rapid Progressive GN) |
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A 12-year-old girl presents with periorbital, abdominal, and ankle swelling. She had been in good health until several months ago when she gained some weight and noted swelling in her legs. A chest X-ray shows bilateral pleural effusions without evidence or lung disease. A urinalysis reveals heavy proteinuria (8g/24 hour); there is no hematuria. A percutaneous needle biopsy of the kidney reveals no morphologic abnormality via light microscopy. Which of the following best describes the most likely medical condition responsible for these findings?
A. Amyloid nephropathy B. Focal segmental glomerulosclerosis C. Hereditary nephritis D. Membranous glomerulopathy E. Minimal change disease |
Minimal Change Disease
**NO MORPHOLOGIC CHANGE ON LIGHT MICROSCOPY** ***THIS IS ON THE EXAM*** |
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A 30-year-old man with a history of smoking suddenly develops oliguria, hematuria, and hemoptysis. Serologic studies reveal antibodies to the glomerular basement membrane. The following pathologic feature is present in the renal biopsy:
1. Crescents in the urinary space 2. Leukocyte infiltrate in the glomerulus 3. Mesangial cell proliferation 4. Thickening of the GBM 5. Thrombi in the glomerular capillaries |
1. Crescents in the urinary space
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An 8-year-old girl presents with headaches, dizziness, and malaise. Physical examination reveals facial edema. Her blood pressure is 180/110 mm Hg, and a 24-hour urine reveals oliguria, hematuria, and 2.7g protein. A kidney biopsy shows electron-dense sub-epithelial humps. What would be the most likely additional finding in this girl?
1. Kidney biopsy with no appreciable findings by light microscopy and immunofluorescence 2. Bilateral nerve deafness 3. Serology positive for nephritic factor 4. A history of antecedent pharyngitis 5. Progression to chronic renal failure despite aggressive corticosteroid therapy |
4. A history of antecedent pharyngitis
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Renal Biopsy is indicated in most cases of Nephrotic/Nephritic Syndrome
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Nephrotic Syndrome
**Exclusion** = Minimal Change Dz in Children |
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Woman; African-American
Clinical: S/Sx’s of nephritic syndrome, SLE ANA-positive with anti-dsDNA Ab’s LM: wire-loop thickening of GFB |
Lupus Nephritis
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African American woman with oliguria and hematuria presents to your clinic. Blood work shows ANA positive and Anti-dsDNA antibodies.
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Lupus Nephritis
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LM: wire-loop thickening of GFB
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Lupus Nephritis
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20 yo man presents with hematuria, proteinuria, oliguria, azotemia
-Hx of resp or GI infection LM: mesangial proliferation IF: IgA staining in mesangium |
IgA Nephropathy
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Patients with IgA Nephropathy normally have a history of: (2)
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GI infection
Respiratory Infection |
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35 yo Male
-Clinical: S/Sx’s of nephritic syndrome -Rapidly progressive renal impairment/failure -Hemoptysis - History of smoking |
Goodpasture's Syndrome
**W/O hemoptysis= Anti-GBM Disease** |
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LM: presence of crescents in most of the glomeruli
-Proliferation of parietal epithelial cells, with compression and eventual obliteration of the glomerular capillary tuft (severely impairs GFR) |
RPGN
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-Young person
Asymptomatic hematuria Morphologic: uniform thinning of GBM |
Thin Basement Membrane Disease
(Hereditary) |
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Most common cause of hereditary hematuria
Clinical: Asymptomatic hematuria Etiology: mutations in genes coding for alpha chains of type IV collagen Morphology: EM shows uniform thinning of GBM |
Thin Basement Membrane Disease
(Hereditary) |
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-Shrunken kidney with diffuse granular cortical surface
-Cortex is thinned -LM shows many/most glomeruli replaced by eosinophilic acellular material, arteriolar sclerosis, tubular atrophy, renal osteodystrophy (calcification and ossification of renal parenchyma secondary to compensatory hyperparathyroidism for attempted maintenance of normal serum Ca levels) |
Chronic Glomerulosclerosis
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**Man / boy**: hematuria with progression to renal impairment/failure; nerve deafness, cataracts
(Fracturing of GBM) Woman / girl: hematuria only |
Alport Syndrome
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Genetics of Alport Syndrome?
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X-linked recessive
(Women only have hematuria) |
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Man / boy: hematuria with progression to renal impairment/failure; nerve deafness, cataracts
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Alport Syndrome
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-LM shows many/most glomeruli replaced by eosinophilic acellular material, arteriolar sclerosis, tubular atrophy, renal osteodystrophy
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Chronic Glomerulosclerosis
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