Renal Pathology Flashcards by Dr. Acula

Light/Immunoflourescence/Electron Microscopy Findings in Minimal Change Disease

Only EM finding: foot processes (podocyte) effacement

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Disease: Associated w/ dense subendothelial immune complex deposits, granular IF, “tram-track” apparance

Type 1 Membranoproliferative Glomerulonephritis (MGN) – nephritic/nephrotic; GBM splitting by mesangial ingrowth

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Disease: associated w/ C3 nephritic factor, intramembranouse dense deposits

Type 2 Membranoproliferative Glomerulonephritis (MGN) – C3NF stabilizes C3 convertase (C3bBb) –> low serum C3

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Average adult kidney weight

150g

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What cell is the major glomerular barrier to protein loss in the urine?

Podocytes

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This congenital renal disease is associated with increased risk later inlife for developing 2ndary FSGS due to overwork of the nephron

Unilateral renal agenesis –> overwork of nephron –> progressive glomerular sclerosis

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Disease: X-linked dominant type IV collagen mutation –> thinning and splitting of GBM; EM: “basket-weave” appearance

Alport Syndrome – Nephritic Syndrome; “can’t see (retinopathy, lens dislocation), can’t pee (glomerulonephritis), can’t hear a bee (sensorineural deafness)

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Markedly reduced intrauterine urine production leads to ___

Potter sequence = Low urine production –> oligohydraminos –> compression –> low-set ears, small receding chin, beak-like nose, abnormally bent lower extremities

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Renal hypoplasia definition and frequent association

Histologically normal kidney w/ less than 6 lobes; frequently oligomeganephronia (enlarged glomeruli)

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Horshoe kidney associated pathologies

Renal infection (Pyelonephritis) – ureter obstruction
Renal stones
Hydronephrosis – ureter obstruction

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Congenital Disease: Histo: undifferentiated tubules and ducts, mantles of undifferentiated mesenchyme (SmM and cartilage)

Renal Dysplasia – aplastic, multicystic, diffuse cystic, obstructive forms

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MCC of an abdominal mass in newborns?

Unilateral multicystic renal dysplasia = renal enlargement by multiple cysts –> irregular mass

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What part of the nephron does it affect?

-Adult AD PKD
-Autosomal Recessive PKD

–AD PKD = Anywhere – Glomerulus, PT, DT, CD

–AR PKD = Collecting Duct

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Earliest, most common presentation of AD PKD

Hypertension

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MCC of Neophrotic syndrome in:

-Caucasian Adults
-American Blacks
-Children

Caucasian Adults = Membranous Nephropathy

American Blacks = FGSG

Children = MCD

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Disease: EM: “spike and dome” appearance, subepithelial deposits

Membranouse Nepropathy = nephrotic presentation of SLE, diffuse capillary and GBM thickening, MCC in Caucasian adults

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MCC of 2ndary nephrotic syndrome and end-stage renal disease in US

Diabetes –> Diabetic Glomerulonephropathy = Eosinophilic nodular glomerulosclerosis: mesangial expansion, GBM thickening

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Disease: Associated w/ Kimmelstiel-Wilson lesions

Diabetic Glomerulonephropathy

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Disease: Associated w/ effacement of podocyte foot processes in EM, but normal glomerulus in light microscopy and IF studies.

Minimal Change Disease – selective proteinuria (albumin)

–Corticosteroids –> remission w/ intermittent relapse

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Disease: Associated with VHL tumor suppressor gene deletion on chromosome 3

Renal Cell Carcinoma

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Disease: Risk factors include Schistosoma haematobium infection (Middle ease), chronic cystitis, smoking, chronic nephrolithiasis

SCC of Bladder = chronic irritation –> squamous metaplasia –> dysplasia –> SCC
—Presents with painless hematuria

Disease: Associated w/ deletion of WT1 tumor suppressor gene

WAGR syndrome = Wilms tumor, Aniridia, Genital abnormalities, mental and motor Retardation

Disease: Associated w/ mutations of WT1 tumor suppressor gene

Denys-Drash syndrome = Wilms tumor, progressive renal (glomerular) disease, male pseudohermaphroditism

Disease: Associated w/ mutations in WT2 gene cluster, particularly IGF-2

Beckwith-Wiedmann syndrome = Wilms tumor, neonatal hypoglycemia, muscular hemihypertrophy, organomegaly (including tongue)

Disease: Classically presents w/ painless hematuria, multifocal "field defect" (recurring) tumors, risk factors include: naphthylamine, azo dyes (hair salon), cyclophosphamide or phnacetin

Urothelial Carcinoma

Characteristics of Flat vs. Papillary pathway of Urothelial Carcinoma

``` Flat = develops as high grade-flat tumor, early p53 mutations Papillary = develops as low-grade papillary tumor, progresses to high grade, no early p53 mutations ```

Disease: arises from a urachal remnant, cystitis glandularis, or exstrophy

Urinary adenocarcinoma (usually bladder) - -Urachal remnant = at dome of bladdre - -Cystitis glandularis = columnar metaplasia - -Exstrophy = congenital failure failure to form caudal portion of anterior abdominal and bladder walls

Disease: Hamartoma of blood vessels, smooth muscle, and adipose tissue

Angiomyolipoma -- increased frequency in Tuberous Sclerosis

Where does Renal Cell Carcinoma arise?

Epithelial cells of proximal kidney tubules

RCC can cause a varicocele on which side?

Left -- L spermatic vein leads drains into L renal artery (can be blocked in RCC) --R spermatic vein drains directly into IVC

This type of stone is treated with HCTz

Calcium oxalate/phosphate

Kidney stones of these 2 substances can form staghorn calculi

Ammonium magnesium phosphate = classical staghorn Cystine

Infection w/ urease positive organisms (Proteus, Klebsiella) can lead to this kind of nephrolithiasis

Ammonium magnesium phosphate -- forms in alkaline urine

This kind of kidney stone can't be seen in X-ray

Uric acid

Gene products affected in AD PKD vs. AR PKD

AD PKD = polycystin 1, 2 = Ca signalling normally inhibiting tubular growth AR PKD = fibrocystin = regular cell differentiation, proliferation and adhesion in collecting ducts

What congenital kidney disease is associated w/ berry aneurysms (family hx of death my aneurysm), hepatic cysts, and mitral valve prolapse?

AD PKD

Disease: Autosomal dominant defect leading to parenchymal fibrosis and shrunken kidney

Medullary Cystic Kidney Disease = cysts in medullary collecting ducts --> worsening renal failure

Which 2 parts of the nephron are most susceptible to ischemic damage?

1. Proximal tubule | 2. Medullary segment of TAL

Acute Tubular Necrosis causes what acid/base change?

1. Increased Anion gap metabolic acidosis (decreased excretion of organic acids (K-H antiport) 2. Hyperkalemia (decreased renal excretion)

Disease: IF microscopy shows linear pattern

Goodpasture syndrome (Type 2 hypersensitivity) = anti-basement membrane (type IV collagen) antibody; associated w/ HLA-DRB1

What diseases are associated with granular pattern in IF microscopy?

PSGN or Diffuse proliferative glomerulonephritis = immune complex deposition

In what disease does IgA immune complex deposit in mesangium of glomeruli?

``` IgA Nephropathy (Berger Disease) = most common nephropathy worldwide ---Childhood presentation of hematuria w/ RBC casts ```

What nephrotic syndrome has good response to steroids?

Minimal Change disease (MCD) | ---Poor response = FSGS, Membranous nephropathy, MPGN

What is the first change in the kidney in Diabetes Mellitus?

Nonenzymatic glycosylation of vascular basement membrane --> hyaline arteriolosclerosis (affecting efferents more than afferents) --> hyperfiltration injury --> microalbuminemia

What is 'thyroidization' of the kidney and when do you see it?

Atrophic tubules containing eosinophilic proteinaceous material resembling thyroid follicles - --Seen in Chronic Pyelonephritis - --May have waxy casts in urine

What is the most common cause of acute intrarenal azotemia?

Acute Tubular necrosis

What kind of acute renal failure? Urine osm = 500+ FENa = less than 1% BUN/Cr = 20+

Pre-renal azotemia = low renal blood flow --> low GFR | --> more reabsorption of fluid and BUN --> high ratio, low FENa

What kind of acute renal failure? Urine osm = less than 350 FENa = 2%+ BUN/Cr = less than 15

Intrinsic renal failure = Usually Acute Tubular Necrosis = patchy necrosis --> debris --> obstruction --> low GFR --> impaired BUN and Na reabsorption; --classic brown granular/epithelial casts

Urine osm, FENa, and BUN/Cr changes in Early vs. Long-standing Post-renal azotemia

Post-renal azotemia - -Early stage obstruction = decreased outflow --> forces BUN back into blood (high BUN/Cr ratio), normal tubular function (FENa less than 1%, high urine Osm) - -Long-standing obstruction = tubular damage --> impaired BUN and Na reabsorption (low BUN/Cr ratio, high FENa), inability to concentrate urine (low Urine osm)

When do you see fatty casts ("oval fat bodies")?

Nephrotic syndrome, where lipids are passes along with protein ---"Maltese cross" sign

When do you see WBC vs RBC casts?

RBC Casts = glomerulonephritis, malignant HTN | WBC Casts = tubulointerstitial inflammation, acute pyelonephritis, transplant rejection

3 Types of Urinary Incontinence and their causes

1. Stress Incontinence = urethral hypermobility or intrinsic sphincter deficiency; sneezing, lifting 2. Urgency Incontinence = overactive bladder; detrusor instability 3. Overflow Incontinence = incomplete emptying; detrusor underactivity