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Path 20 Kidney
LECOM Path Ch 20 Kidney
Question | Answer |
---|---|
4 stages of renal failure | Deminished renal reserve, Renal insufficiency, Chronic renal failure, End-stage renal disease |
Progression to renal failure usually ensues when GFR is reduced to | 30-50% of normal |
Acute Nephritic, after infection, Sub-epithelial humps, granular IgG and C3 in GBM, diffuse proliferation | PSGN |
Diffuse nephritis, global hypercellularity, Granular IF | PSGN |
Rapidly progressive glomerulonephritis, Linear IgG and C3 and/or fibrin crescents, proliferative, maybe crescenteric | Goodpasture |
RPGN Type I mediated by | Anti-GBM |
RPGN Type II mediated by | Immune complex |
RPGN Type III mediated by | Pauci Immune |
Type I RPGN that cross reacts with lung BM | Goodpasture |
Elevated titer in Type III RPGN | ANCAs |
Ruptures of the GBM on EM, inflammatory and parietal cell proliferation in Bowman space | RPGN/ cresenteric |
Escape of this into bowman’s space is a leader in crescent development | fibrinogen |
MCC of nephrotic syndrome in children | Minimal-Change Disease |
Nephrotic syndrome Dx with | >3.5g of daily proteinuria |
Membranous Nephropathy mediated by | chronic immune-complex mediated |
Nephrotic, Diffuse thickening of capillary walls, tubular epithelial cells with protein reabsorption droplets, diffuse GBM granular staining, sub-epi deposits on EM | membranous Nephropathy |
Pathognomonic finding of spikes on GBM w. silver stain Dx? | Membranous Nephropathy |
Membranous nephropathy vs Minimal Change disease: difference in onset | Minimal change is selective proteinuria and responds to corticosteroids |
Nephrotic, Diffuse effacement of podocytes on EM, but look normal on light microscopy. Dx? | Minimal-Change Disease |
Most characteristic thing about Minimal-Change disease is | it’s response to corticosteroid therapy |
Effacement of podocytes with normal glomeruli Dx? | minimal change |
MCC of nephrotic syndrome in adults in the US is | Focal Segmental Glomerulosclerosis (FSGS) |
MC location of lesion is FSGS | juxtamedullary glomeruli |
Disease states that can cause FSGS (5) | HIV, IV drug use, Sickle Cell, morbid obesity, loss of renal tissue |
Hylinosis and sclerosis of some viewed glomeruli and only some of the capillaries | FSGS |
Retraction of entire glomerular tuft, proliferation and hypertrophy of glomerular visceral epithelial cells. Dx and most characteristic lesion of? | Collapsing glomerulopathy of FSGS and in HIV-nephropathy |
MC lesion in HIV nephropathy | collapsing variant of FSGS |
Alteration in the GBM, proliferation of glomerular cells, and leukocyte infiltration | membranoproliferative glomerulonephritis (MPGN) |
Other name of Type II primary MPGN | dense-deposit disease |
Type I MPGN is Dx when finding | immune complexes in the glomerulus and activation of both compliment pathways |
Type II MPGN is Dx when finding | decreased serum C3 but normal C1 and C4 |
Is there IgG deposits found in Dense Deposit Disease? | no, only C3 and properdin |
70% of Pts w/ DDD have this circulating antibody | C3NeF |
“Tram-tracking” or duplication of the basement membrane is seen in what disease | MPGN |
Large hypercellular glomeruli, GBM thickened, IgG and C3 in granular pattern in subendothelial location. Dx? | MPGN Type I |
Large hypercellular glomeruli, dense-ribbon like GBM, C3 in basement membrane granular or linear, and circular rings of C3 in mesangium. Dx? | MPGN Type II aka DDD |
Causes of Secondary MPGN (all type I)(7) | SLE, HBV, HCV, HIV, alpha 1 antitrypsin, leukemia or lymphoma |
Frequent cause of recurrent gross or micro hematuria, probably MC glomerulonephritis worldwide | IgA nephropathy |
Which subclass forms Ab in IgA nephropathy | IgA1 |
Which pathway is activated in IgA nephropathy | alternative compliment pathway |
2 causes of secondary IgA nephropathy | celiac disease and liver disease |
Teen presents with gross hematuria after respiratory infection a week before. Dx and outlook? | IgA nephropathy, variable |
Classic findings in IgA nephropathy | mesangial widening with IgA and C3 in the mesangium |
Hematuria with progression to renal failure, eye disorders Dx? And what else will be found with sensitive testing | Alport Syndrome and hearing deficiency |
Cause of Alport Syndrome | defective collagen IV synthesis; a5 in X-linked variety |
On EM of 2 brothers glomeruli: irregular foci of thickening alternating with attenuation of the GBM, splitting and lamination of the lamina densa “basket weave” appearance. Dx? | Alport syndrome |
2 diseases linked to defective type IV collagen | Alport and Thin Basement Membrane Lesion/Benign Familial Hematuria |
Gross appearance of kidneys in Chronic glomerulonephritis | symmetrically contracted, diffusely granular cortical surface |
Findings in a dialysis kidney (5/6) | arterial intimal thickening, calcifications, calcium oxalate crystals, cystic disease, increased renal adenomas and adenocarcinomas |
Purpuric skin lesions on extensor surfaces of arms and legs and buttock; abdominal pain, GI bleed; non-migratory arthralgias; renal abnormalities | Henoch-Schonlein Purpura |
Mesangial proliferation, IgA and C3 deposition in the mesangium. 2 DDx and what would differentiate | IgA nephropathy or Henoch-Schonlein Purpura; subepidermal hemorrhages and necrotizing vasculitis of small vessels, and GI manifestation |
Arteriolar manifestation of diabetic nephropathy | hylanizing arteriolar sclerosis |
Capillary basement membrane thickening, diffuse mesangial sclerosis, nodular glomerulosclerosis | diabetic nephropathy |
Normal or enlarged kidneys with deposition of AL or AA type chains. Dx? | amyloid kidney |
MCC of acute renal failure | AKI or ATN |
4 types of ATN/AKI: due to | ischemia, Direct toxic injury, Acute tubulointerstitial nephritis from drug hypersensitivity, urinary obstruction |
2 critical events in AKI | tubule cell injury and persistant/severe disturbance in blood flow |
In AKI, loss of cell polarity causes what | abnormal ion transport, increase Na+ to distal tubules, causes vasoconstriction |
What causes luminal obstruction in AKI | detached injured cells |
Patchy tubular necrosis, in short lengths around straight segments of proximal tubule and ascending limb of Loop of Henle. Type of AKI? | Ischemic |
Extensive necrosis along proximal convoluted tubule. Type of AKI | Toxic AKI |
2 areas most susceptible in ischemic AKI | straight portion of proximal tubule and ascending thick limb in medulla |
Area most susceptible to toxic AKI | proximal convoluted tubule |
3 stages of AKI | initiation, maintenance and recovery |
Findings in initiation phase of AKI | slight decline in urine output and rise in BUN |
5 findings of maintenance phase of AKI | sustained decrease in urine to 40-400ml/d; salt/water overload; rising BUN; hyperkalemia; metabolic acidosis |
6 Findings in recovery stage of AKI | increased urine output; loss of large amounts of water, sodium and potassium(hypokalemia); increase infection risk;BUN/Creatinine return to normal |
Acute vs Chronic tubulointerstitial nephritis: interstitial edema, eos and neuts, focal tubular necrosis | Acute |
Acute vs Chronic tubulointerstitial nephritis: mononuclear leukocytes, interstitial fibrosis, widespread tubular atrophy | chronic |
(4 bugs) MCC of UTI (85%) | gram neg bacilli; E.coli; Proteus; Klebsiella; Enterobacter |
MCC of clinical pyelo | ascending infection |
Acute pyelonephritis with reflux: damage is mostly seen where? | upper and lower poles |
Patchy interstitial suppurative inflammation, intratubular aggregates of neutrophils, and tubular necrosis Dx? | acute pyelonephritis |
3 complications in Acute Pyelo and their special circumstances | Papillary (coag)Necrosis: in Pt’s with DM and obstruction; Pyonephrosis: complete obstruction; Perinephric abscess: extension through renal capsule |
What finding differentiates kidney from bladder Infx? | leukocyte casts |
Cause of emerging pyelonephritis in transplant Pt’s | polyomavirus |
Only 2 tubulointerstitial diseases that affect the renal calyces | chronic pyelonephrosis and analgesic nephropathy |
2 types of chronic pyelo | reflux and obstructive |
MC type of chronic pyelo | reflux |
Asymmetric scarring of kidney with coarse, discrete corticomedulary scars overlying blunted or deformed calyces, with flattened papilla | chronic pyelo |
What do tubules look like in chronic pyelo | atrophy in some and hypertrophy or dilation in others |
Foamy macrophages with plasma cells, lymphs and PMN cells in large yello/orange nodule in kidney. Not renal cell carcinoma. Dx and w. what Infx? | Xanthogranulomatous pyelonephritis; Proteus infx |
Acute Drug induced interstitial nephritis: common drugs(3) and onset | synthetic penicillin, diuretics, NSAIDS, 15 days after drug exposure |
Kindey injury in acute drug induced nephritis is cause by | IgE and/or cell mediated immune reaction; not the drug ie dose independent |
Kindey interstitium edema and mononuclear infiltration; eos and neuts in clusters with plasma cells, normal glomeruli Dx? | acute drug induced interstitial nephritis |
Analgesic nephropathy is cause by what 2 events, in order | papillary necrosis, then cortical tubulointerstitial nephritis from urine obstruction |
2 drugs causing Analgesic Nephropathy and their action | acetaminophen/phenacetin depletes glutathione causing ROS damage and aspirin inhibits vasodialation by PG |
Normal or slightly small kidneys, cortical atrophy over papillary necrosis which are in various stages Dx? | Analgesic nephropathy |
Cancer associate w/ analgesic pephropathy | transitional papillary carcinoma of the renal pelvis |
Pink/blue tubular casts, angulated and/or fractured, surrounded by macrophages. Dx? | Bence-Jones proteinuria / Myeloma Kidney |
Normal or slightly shrunken kidneys, leather grain on the outside | Benign nephrosclerosis |
Medial and intimal thickening of small arteries in kidney with hyaline arteriosclerosis, microscopic subcapsular scars with sclerotic glomeruli | benign nephrosclerosis |
Pts with malignant HTN with show what type of arterial disease in the kidney | malignant /accelerated nephrosclerosis |
Early malignant nephrosclerosis shows (3) | fibrinoid necrosis of arterioles and small arteries, swelling of vascular intima, intravascular thrombus |
Typical arteriopathy in malignant HTN that causes further narrowing of the lumen | hyperplastic arteriolosclerosis |
“flea bitten kidney” Dx? | malignant nephrosclerosis |
This finding represents an acute event in a person with HTN | fibrinoid necrosis of arterioles |
Interlobular arteries and arterioles with proliferation of concentric smooth muscle cells, collagen, and clear layers Dx? 2 names and what it correlates with | onion-skinning, hyperplastic arteriolitis; renal failure |
Initial cause of HTN with constriction of renal artery is due to | increased plasma renin |
MCC of renal artery stenosis | plaque at the origin |
MC location, age and sex for fibromuscular dysplasia of renal artery | medial thickening, 20-40yo, women, distal or medial portion of the artery |
Typical HUS is most often associated with | consumption of food with Shiga-like toxin |
MCC of TTP | deficiency of ADAMTS13, which regulates vWF |
2 MCC of Atypical HUS | mutation in compliment-regulatory proteins; or acquired endothelial injury |
Initiating event in TTP vs HUS | TTP: platelet aggregation by vWF multimers; HUS: endothelial injury |
What is usually present and causative in the majority of TTP Pts? | antibodies to ADAMTS13 |
Child Diarrhea then Sudden onset of bleeding, hematemesis, melena, severe oliguria, hematuria, microangiopathic hemolytic anemia, thrombocytopenia and HTN Dx? | typical HUS |
MC deficiency in atypical HUS | factor H, compliment regulatory protein |
How to differentiate TTP and HUS? | normal ADAMTS13 in HUS |
Better outcome typical or atypical HUS? | typical |
Fever, neurologic symptoms, microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. CNS involvement is a dominant feature | TTP |
Massive necrosis after an obstetrics emergency is usually limited to where? Dx? | cortex; diffuse cortical necrosis |
Renal infarcts are usually of what type | white |
Wedge shaped area of gray white color, depressed scar on cortex Dx? | renal infarct |
Adult Polycystic Kidney Disease: Inheritance, cyst locations, outcome | AD; large multicystic kidneys, liver cysts, berry aneurysms; chronic renal failure at 40-60yo |
Childhood Polycystic Kidney Disease: Inheritance, cyst locations, outcome | AR; enlarged, cystic kidneys at birth; variable, death in infancy or childhood |
Medullary Sponge Kidney: Inheritance, cyst locations, outcome | none; medullary cysts on excretory urography; benign |
Familial Juvenile Nephronopthisis: Inheritance, cyst locations, outcome | AR; corticomedullary cysts, shrunken kidneys; progressive renal failure starts in childhood |
Adult-Onset medullary cystic disease: Inheritance, cyst locations, outcome | AD; Corticomedullary cysts, shrunken kidneys; chronic renal failure in adulthood |
Simple Cysts: Inheritance, cyst locations, outcome | none; single or multiple cysts in normal sized kidney; benign |
Acquired Renal Cystic Disease: Inheritance, cyst locations, outcome | none; Cystic degeneration in end-stage kidney disease; Dialysis dependant |
MC mutation in ADPKD | PKD1 |
Better or worse prognosis with PKD1 mutation in ADPKD? | worse |
Gene affected in ARPKD | PKHD1 |
Bilaterally Enlarged kidneys with smooth external appearance; numerous small cysts on section with dilated elongated channels at right angles to the cortex Dx? | ARPKD |
Extra-renal disease with ARPKD | liver cysts with portal fibrosis |
Extra-renal disease with ADPKD | cysts in the liver, intracranial berry aneurysm, mitral valve prolapse |
Multiple cystic dilations of the collecting ducts in the medulla | Medullary sponge kidney |
MC malignant kidney tumor in adults | renal cell carcinoma |
MC malignant kidney tumor in children | Wilms tumor |
On autopsy small tumor with the cortex, pale yellow, discrete, well circumscribed nodules, complex branching cuboidal cells Dx? | Renal Papillary Adenoma |
Angiomyolipomas are present in 25-50% of Pts with | tuberous sclerosis |
Clinical importance of angiomyolipoma? | likes to spontaneously hemorrhage |
Solitary tumor in kidney, tan/brown, well-encapsulated, large eosinophilic cells, small round benign nuclei, lots of mitochondria. Dx? And what cell they come from | Oncocytoma; intercalated cells |
Most significant risk factor for renal cell carcinoma | tobacco |
MC type of renal cell carcinoma | Clear Cell Carcinoma |
Loss of VHL on 3p leads to what tumor | hereditary and sporadic Clear Cell Carcinoma |
Trisomy 7 leads to what renal tumor | sporadic and hereditary Papillary carcinoma |
Mutated/activated MET leads to what renal tumor | sporadic and hereditary Papillary carcinoma |
Renal tumor with pale eosinophilic cells, perinuclear halo, in solid sheets. Dx? | Chromophobe renal Carcinoma |
Renal tumor: nest of malignant cells enmeshed within a prominent fibrotic stroma. Typically in the medulla | Collecting duct / Bellini Duct carcinoma |