Block 3 - Renal

Question 1

A congenital condition resulting in the absence or underdevelopment of the kidneys. Also presents with pulmonary hypoplasia, oligohydramnios (due to decreased fetal urine excretion and resulting in compression of the fetus), wrinkly skin, twisted fact, and limb defects.

Answer 1

Potter’s syndrome

Question 2

The most common renal congenital condition presenting as fusion of the lower poles of both kidneys. It may rarely cause symptoms, unless compression or occlusion of nearby structures occurs.

Answer 2

Horseshoe kidney

Question 3

A congenital anomaly that is usually unilateral (bilateral disease results in oligohydramnios and Potter sequence). Grossly, the kidneys appear enlarged, irregular, and multi-cystic. Micro: undifferentiated mesenchyme, cartilage, and immature collecting ducts. Not an inherited disease.

Answer 3

Multicystic renal dysplasia

Question 4

An AR congenital disease that presents in both kidneys, resulting from a mutation in the PKHD1 gene. “Spongiform kidneys” and possible liver involvement, leading to congenital hepatic fibrosis (portal hypertension in a child). Multiple ectatic collecting ducts appear radially oriented from the center of the kidney to the surface. The corticomedullary junction is obliterated by the numerous abnormal ducts.

Answer 4

Autosomal Recessive polycystic kidney disease (ARPKD)

Question 5

An AD malformation of both kidneys typically presenting in adults. Results from a mutation in the PKD1 or PKD2 genes. May have hepatic or splenic cysts, Berry aneurysms, colonic diverticulosis, and mitral prolapse. Malformation occurs throughout the entire renal tubule, not just the collecting ducts.

Answer 5

Autosomal dominant polycystic kidney disease (ADPKD)

Question 6

Gene involved in autosomal recessive polycystic kidney disease (ARPKD)

Answer 6

PKHD1

Question 7

Gene involved in autosomal dominant polycystic kidney disease (ADPKD)

(2)

Answer 7

PKD-1 (Polycystin-1) and PKD-2 (polycystin-2)

Question 8

Kidney cysts can commonly be acquired from long-term ___ due to other kidney diseases. >5years. May progress to malignancy.

Answer 8

Acquired cystic disease

Question 9

Single or multiple kidney diseases ranging from 1-10 cm in diameter. Usually present in the subcapsular renal cortex. Commonly an incidental finding at autopsy and they rarely produce symptoms.

Answer 9

simple cysts

Question 10

Essential (primary) hypertension can cause ___ nephrosclerosis. Grossly shows granular, contracted kidneys. Microscopically shows hyaline atherosclerosis.

Answer 10

Benign

Question 11

Malignant hypertension can cause ___ nephrosclerosis. Grossly appears as nodular or flea-bitten kidney. Micro: fibrinoid necrosis of the afferent arteriole and hyperplastic arteriolitis (onion-skinning).

Answer 11

accelerated

Question 12

Secondary hypertension can arise from what renal disease? Increased RAA system results in vasoconstriction and blood volume –> HTN

Answer 12

renal artery stenosis (low BV going into kidney falsely tells body to increase RAA response)

Question 13

___ infarct appears pale and is caused by a mural thrombus from an MI or A-fib, infected valves in endocarditis, complicated AS plaques in the aorta.

Renal infarcts present with sharp flank or abdominal pain and hematuria.

Answer 13

ischemic

Question 14

____ infarct appears as red and is caused by renal vein thrombosis (severe dehydration in infants and septic thrombophlebitis in adults).

Renal infarcts present with sharp flank or abdominal pain and hematuria.

Answer 14

Hemorrhagic

Question 15

Dx? Pale, widespread necrosis of tubular epithelium and calcification. Seen as a complication in obstetric emergencies and DIC.

Answer 15

Diffuse cortical necrosis

Question 16

Key difference between nephritic and nephrotic syndrome?

Answer 16

Nephritic - hematuria

Nephrotic - proteinuria

Question 17

Nephrotic disease with primary GN

Most common cause of nephrotic syndrome in children, due to immune dysfunction and excessive cytokine production, which also results in increased infections and Hodgkin lymphoma. Presents acutely with 4+ albumin, fatty casts in urine, and hematuria in 20% of cases. Responds to steroid treatment, but can progress to focal segmental GN (FSGN). Microscopically, see effacement of foot processes due to cytokine production, WITHOUT immune deposits.

Answer 17

Minimal change disease

Question 18

Nephrotic syndrome with primary GN

Disease frequent in Hispanics and blacks. Can be idiopathic or secondary type. Secondary type arises from viral infection, IV rug use, or sickle cell anemia. Presents with non-selective proteinuria, and 75% have hematuria. Prognosis is poor despite treatment and typically progresses to chronic renal failure. Microscopically shows effacement of foot processes and segmental involvement.

Answer 18

focal segmental glomerulosclerosis

Question 19

HIV causes a subtype of focal segmental glomerulosclerosis (FSGS) called ____ glomerulopathy. EM shows collapse of glomerular tufts and tubuloreticular bodies in endothelial cells, which are modified ER.

Answer 19

collapsing

Question 20

Nephrotic syndrome with primary GN

The most common cause of nephrotic syndrome in Caucasian adults. 75% idiopathic/Autoimmune due to Ag against Phospholipase Receptor A2 and IgG4 deposits. Secondary causes are chronic Ag stimulation (Hepatitis, Malaria, Syphilis), malignancy, medication, or systemic disease. Another possible etiology is MAC activation of glomerular cells –> proteases and oxidants –> capillary wall injury –> protein leakage with entrapment of Ab-Ag complexes in the membrane.

Presents as proteinuria >3.5 gm/24hr. 50% have hematuria.

Intermediate prognosis, but many progress to renal failure.

EM shows spikes and domes, subepithelial deposits and foot process efacement.

Answer 20

Membranous glomerulonephritis

Question 21

Nephrotic syndrome with system disease

High serum glucose leads to non-enzymatic glycosylation of the vascular basement membrane leading to hyaline atheriolosclerosis. Glomerular efferent arterial is more affected than afferent, leading to increased filtration pressure, hyperfiltration, and microalbuminuria. ACE-inhibitors slow progression.

Most common cause of end-stage renal disease in the US.

EM shows GBM thickening and sclerosis, effacement of foot processes, and endothelial cell damage.

Answer 21

Diabetic nephropathy

Question 22

glomerular nodules seen in diabetic nephropathy

Answer 22

Kimmelstiel-Wilson

Question 23

Nephrotic syndrome with systemic disease

An uncommon disease that is seen in elderly patients. Associated with hepatomegaly, restrictive cardiomyopathy, multiple myeloma, and long-standing inflammatory diseases like TB, bronchiectasis, and RA. Presents as proteinuria. Very poor prognosis.

Types are AL - light chain - in multiple myeloma and lymphoproliferative diseases

AA- chronic infections

Answer 23

Amyloidosis

Question 24

Nephrotic syndrome with systemic disease that is typically asymptomatic

Answer 24

SLE

Question 25

Nephrotic syndrome with primary GN Two types based on the location of deposits: subendothelial (Hepatitis or HIV) or intramembranous with C3 nephritic factor Primary type: due to immune complex deposition Secondary type: systemic conditions and malignancy

Answer 25

Membranoproliferative GN

Question 26

Nephrotic syndrome with primary GN Subtype of membranoproliferative GN Due to the deposition of circulating immune complexes (SLE, etc), excessive complement activation, or inflammatory reaction. Tx with steroid and immunosuppressives. Most progress to chronic renal disease despite treatment Subendothelial deposits - "railroad" Mesangial proliferation

Answer 26

MPGN type 1, membranoproliferative Subendothelial, mesangiocapillary

Question 27

Nephrotic syndrome with primary GN Subtype of membranoproliferative GN abnormalities resulting in excessive activation of the alternate complement pathway, favoring persistent C3 activation by C3 convertase. Hypocomplementemia. Lamina densa and subendothelial GBM are transformed into an irregular electron dense substance

Answer 27

(formerly )Type 2 MPGN Dense-deposit type

Question 28

Nephritic or nephrotic? Glomerular hypercellularity, type of cell varies Infiltration of inflammatory cells Mild edema, HTN, azotemia due to BUN and creatinine retention, oliguria due to reduced GFR, hematuria (dysmorphic RBCs and RBC casts), proteinuria \<3gm

Answer 28

Nephritic

Question 29

Nephritic syndrome with primary GN Follows group A B-hemolytic strep pharyngitis (strains with M protein) Reduced serum C3 and C4, CRP elevated Occasionally progresses to rapidly progressive GN, but has an excellent prognosis. Also can be caused by staph, meningococci, pneumococci, Measles, mumps, or rubella. Micro: diffuse endothelial and mesangial hypercellularity with neutrophilic (acute) infiltrate. Hump shaped immune complex deposits with extensive foot process effacement and endothelial cell proliferation.

Answer 29

Acute proliferative GN (post-infectious GN)

Question 30

Nephritic syndrome with primary GN Most common GN Can be nephritic, nephrotic, or isolated Immune complex deposition in the mesangium Presents in childhood with hematuria, RBC casts, Coke colored urine and diarrhea, and usually follows mucosal infection. Micro: mesangial expansion with segmental sclerosis. "Holly leaf" depositions.

Answer 30

IgA nephropathy aka Berger disease

Question 31

Nephritis syndrome with systemic disease Dx if patient meets 4/11 criteria: malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, neurologic disorder, hematologic (anemia or thrombocytopenia), immunologic, or renal disorder. Low levels of circulating serum complement

Answer 31

SLE

Question 32

Define WHO class of normal SLE with minimal changes on LM

Answer 32

WHO class I

Question 33

Define WHO class of SLE with mesangial GN

Answer 33

WHO class II

Question 34

Define WHO class of SLE with focal proliferative GN

Answer 34

WHO class III

Question 35

Define WHO class of diffuse proliferative GN

Answer 35

WHO class IV

Question 36

Define WHO class of SLE with membranous glomerulopathy

Answer 36

WHO class V

Question 37

Define WHO class of SLE with advanced nephritis

Answer 37

WHO class VI

Question 38

Nephritic syndrome with primary GN Type 1: primary immunologic Type 2: progression of other glomerular diseases Type 3: part of group of systemic diseases

Answer 38

rapidly progressive glomerulonephritis (RPGN) or crescentic nephritic syndrome

Question 39

Nephritic syndrome with primary GN that is a primary immune disease Acute onset with hemoptysis and hematuria. Normal serum complement anti-GBM Ab in serum or tissue. Non-collagenous domain of the alpha 3 chain of collagen IV in lamina dense of GBM. Basement membrane breaks. In situ production of immune complexes in kidney and lung with inflammatory reaction. History of viruses and hydrocarbons

Answer 39

Goodpasture syndrome (a type I RPGN)

Question 40

Nephritic syndrome with primary GN, subtype of RPGN ANCA-associated May be associated with systemic vasculitis (Wegener granulomatosis or microscopic polyangiitis). Some are isolated crescentic GN without involvement in other organs (idiopathic). Poor prognosis despite treatment

Answer 40

Pauci-immune/ANCA-associated (type 3) RPGN

Question 41

Genetic defect resulting in the defective assembly of collagen and the GBM. Absence of a-5 isoform of collagen IV (X-linked); a-3 or a-4 in autosomal inheritance. GM shows irregular thinning and thickening with a "basket-weave" appearance. Rare. Presents with hematuria, mild proteinuria, normal serum complement. More common and severe in males who develop renal failure in early adulthood. Prognosis is variable in female carriers. Nerve deafness and ocular disorders may occur.

Answer 41

Alport's disease

Question 42

Benign familial hematuria that is otherwise asymptomatic. Genetic defect resulting in the defective assembly of collagen and the GBM. Absence of a-5 isoform of collagen IV (X-linked); a-3 or a-4 in autosomal inheritance. GBM shows thinning. Prognosis is good and does not progress to chronic renal failure

Answer 42

Thin basement membrane nephropathy

Question 43

Acute tubular injury can lead to acute tubular \_\_\_\_

Answer 43

necrosis

Question 44

Acute tubular injury results involved injured ____ cells.

Answer 44

tubular

Question 45

Acute tubular injury caused by what? Initially presents with severe cell injury with large cytoplasmic eosinophilic inclusions. Later, total necrosis with calcification.

Answer 45

mercury chloride

Question 46

acute tubular injury cased by what? initially presents with fatty change of epithelium and later necrosis

Answer 46

carbon tetrachloride

Question 47

acute tubular injury caused by what? Presents with ballooning/hydropic degeneration of epithelium of PCT. Calcium oxalate crystals in tubules and urine.

Answer 47

Ethylene glycos

Question 48

Ethylene glycol causes acute tubular injury and ___ \_\_\_ crystals in tubules and urine.

Answer 48

calcium oxalate

Question 49

Acute ___ is typically caused by infection with the patient's own fecal flora (gram - bacilli), ascending UTI or descending infective endocarditis. Micro: small abscesses with interstitial inflammation and papillary, tubular necrosis. Pyonephrosis, perinephric abscess, pyelonephritic scar

Answer 49

pyelonephritis

Question 50

Chronic ____ is interstitial inflammation with scarring of the renal parenchyma, calyces, and pelvis. Reflux nephropathy or chronic obstructive type Marked blunting of calyces with hydronephrosis and hydroureter. Micro: "thyroidization of the kidney." Interstitial fibrosis and mononuclear infiltration. Glomeruli usually spared but some may be sclerotic. Gross: irregular scarring: broad depressed areas of cortical fibrosis overlying a dilated calyx. Bilateral and asymmetrical.

Answer 50

pyelonephritis

Question 51

acute ___ interstitial nephritis presents with fever, rash, rapidly increasing creatinine, and eosinophilia. Urinalysis: proteinuria, hematuria, and pyuria with eosinophils Tubulitis and tubular necrosis

Answer 51

drug-induced (non-specific)

Question 52

Nephropathy caused by \_\_\_\_. Presents with renal insufficiency, HTN, anemia, CRF. Papillary necrosis, hematuria, proteinuria, and flank pain. Increased risk of urothelial carcinoma.

Answer 52

analgesics and NSAIDs

Question 53

Tubulointerstitial nephritis that occurs acutely after chemotherapy. Presents with related symptoms and stones in the calyces, pelvis, ureter, and bladder.

Answer 53

Urate nephropathy

Question 54

Non-specific diagnosis of stones in the kidney

Answer 54

urolithiasis

Question 55

Renal stones Most common type, seen in adults. Caused by idiopathic hypercalciuria, hypercalcemia, and Crohn's

Answer 55

Calcium oxalate or calcium phosphate

Question 56

renal stones Second most common Caused by infection with proteus, which alkalinizes the urinary tract and produces staghorn calculi.

Answer 56

ammonium magnesium phosphate

Question 57

renal stones Radiolucent stones produced in an acidic environment. Commonly in gout or cancer patients treated with chemotherapy.

Answer 57

uric acid

Question 58

Renal stones Rare but seen in children. Produced in an acidic environment and in genetic cystinuria.

Answer 58

cysteine

Question 59

benign renal tumor common, \<3cm, well-circumscribed incidental finding at autopsy

Answer 59

renal adenoma

Question 60

benign renal tumor associated with tuberous sclerosis. Hamartoma

Answer 60

angiomyolipoma

Question 61

benign renal tumor small, firm, gray located in pyramids, no clinical significance

Answer 61

renal fibroma

Question 62

benign renal tumor large, eosinophilic cells filled with mitochondria and central scarring

Answer 62

oncocytoma

Question 63

benign renal tumor mesenchymal tumor in children

Answer 63

mesonephric blastoma

Question 64

aggressive renal tumor 80% of all renal tumors in adults Generally asymptomatic. "The great mimic." The classic triad: hematuria, palpable abdominal mass and dull flank pain. Initial presentation is usually due to metastasis. Gross: hemorrhage, necrosis, vascular invasion. Invasion of renal veins. Ectopic hormone production aka paraneoplastic syndrome causes varied presentations: polycythemia (EPO), HTN (renin), hypercalcemia (PTH-like), Cushing syndrome (ACTH), etc. Many predisposing factors 40% of patients die Types: clear cell (shown below), papillary, or other

Answer 64

Renal cell carcinoma

Question 65

type of renal cell carcinoma

Answer 65

clear cell carcinoma

Question 66

Type of renal cell carcinoma

Answer 66

papillary carcinoma

Question 67

Aggressive renal tumor that occurs unilaterally in children. Micro: primitive, dark, small blue round cells Primitive glomeruli and tubules

Answer 67

Wilms tumor

Question 68

\_\_\_\_ epithelium

Answer 68

urothelial aka transitional

Question 69

Aggressive tumor Aggressive behavior increases with tumor size Large and multiple, become less papillary and more sessile, more atypical, increased mitotic activity, invasive, and metastatic. Invades muscularis propia, prostate, rectum, vagina, or retropreitoneum Metastasizes by lymph or blood Types: squamous cell, mixed, or adenocarcinoma

Answer 69

Urothelial (transitional) cell tumor

Question 70

Infection that increases the risk of squamous cell urothelial carcinoma Micro: parasite eggs

Answer 70

Schistosoma hematobium (schistosomiasis) in Japan and Egypt