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Kidney > Medical Kidney > Flashcards

Medical Kidney Flashcards

1
Q

A 5 year old boy with atopy cane in due to frothy urine and abnormal bleeding. Biopsy of the kidney shows no changes in LM but there is uniform and diffuse effacement of foot processes of the podocytes. There are no deposits on IF. Dx? What are associated diseases or drugs? Tx?

A

Minimal change disease. (MC in children)
Em findings only.
Tx: steroids (steroid-responsive)
Respi infections, atopy/eczema, hodgkin lymphoma
Prophylactic immunization

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2
Q

MC cause of nephrotic syndrome in adults
Labs
Characteristic finding
Assoc drug or diseases

A

Focal segmental glomerulosclerosis (FSGS)
Azotemia present
LM: increased mesangial matrix and deposition of hyaline masses (hyalinosis) leading to obliterated capillary lumina and lipid droplets
Heroin
HIV, sickle cell anemia, secondary event (ex. Iga neohropathy)

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3
Q

A 47 year old man with SLE and frequent headaches came in due to frothy urine. Biopsy of his kidney shows subepithelial deposits along the GBM with spike and dome appearance with effacement of foot processes. On IF, there are granular deposits of Igs and complement along the GBM. Dx? Assoc drugs and diseases?

A

Membranous nephropathy
NSAIDs, penicillamine, captoprio, gold
SLE, infectiond, lung and colon CA and melanoma

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4
Q

Which MPGN comprises of complement activation only? Thru what pathway?

A

MPGN Type II or dense deposit disease
Alternative pathway

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5
Q

What MPGN has immune complex deposition? Complement activation is thru what pathway?

A

MPGN Type I
Classical and alternative

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6
Q

A 7 year old boy who just recovered from cough and colds came in with purpuric skin lesions, abdominal pain and arthralgias. IF of his kidney biopsy would show what? Dx?

A

Henoch-schonlein purpura
Mesangial deposition of IgA

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7
Q

MC type of glomerulonephritis worldwide
Pathology

A

IgA nephropathy (Berger disease)
Deposition of IhA in mesangium (localized to kidneys)

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8
Q

Mc cause of nephritic syndrome in children
Cause?
EM findings?
Labs
Prognosis

A

Post-streptococcal acute glomerulonephritis (PSAGN)
1-4 weeks after pharyngitis or pyoderma with GABHS
Increased anti-streptococcal titers (ASO or anti DNAse B)
Less likely to progress to RPGN in children but more likely in adults
EM: subepithelial humps on GBM
(Camel with beads and hump and coughing)

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9
Q

Pathogenesis of the 3 types of Rapidly progressing glomerulonephritis (RPGN)

A

Type I - antibodies to GBM
Type II - immune complex mediated glomerular injury
Type III - pauci-immune (presence of ANCA)

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10
Q

In this syndrome, antibodies against the alpha3 chain of collagen type IV in alveoli in GBM cause necrotizing hemorrhagic interstitial pneumonitis and RPGN.
Dx? And what type of RPGN?

A

Goodpasture syndrome
Type I

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11
Q

A 10 year old boy came in due to hematuria. On PE, patient is deaf and blind. His uncle also had the same clinical picture. Biopsy of the kidney shows a basket-weave appearnce of the GBM on EM. Dx? Pathology? Prognosis?

A

Alport syndrome
Defective assembly of Type IV collagen
X-linked
90% of males progress to ESRD

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12
Q

A 55 year old hypertensive man came in due to dizziness and frothy urine. His BP was 190/140. Biopsy of his kidney showed fibrinoid necrosis of vessels and onion-skinning. Dx? Pathogenesis?

A

Malignant nephrosclerosis
From malignant hypertension
Endothelial injury—> fibrinoid necrosis —> activation of clotting system (thrombosis) —> release of mitogenic factors from platelets —> hyperplasia of arteriolar smooth muscle

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13
Q

A 56 year old man came in for follow up check up. His BP is 140/90. Crea is elevated. He says he sometimes forgets to drink his medicine. Biopsy of the kidney shows hyaline arteriolosclerosis. Dx? Cause?

A

Nephrosclerosis
Essential hypertension

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14
Q

Special stain for GBM?

A

PAS

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15
Q

What is the pattern of immune deposition on glomerulus on IF?

A

Granular

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16
Q

Thickened basement membrane in membranous nephropathy is due to what?

A

Subepithelial complexes

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17
Q

What is the pattern of anti-GBM antibody induced glomerulonephritis on IF?

A

Diffuse linear pattern (vs. granular in immune complex deposition)

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18
Q

Where are the following molecules deposited in the glomerulus? Cationic, anionic and neutral

A

Cationic- subepithelial
Anionic- subendothelial
Neutral- mesangium

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19
Q

Strains of GABHS in poststreptococcal GN

A

12, 4 and 1 (identified by typing the M protein)

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20
Q

The principal streptococcal anitgenic determinant in most cases

A

Streptococcal pyogenic exotoxin B (speB)

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21
Q

What is the renal autoantigen in primary MGN?

A

M-type PLA2R
Also THSD7A

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22
Q

Variant of FSGS MC seen in HIV patients

A

Collapsing variant

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23
Q

EN finding in type I MPGN?

A

Subendothelial electron-dense deposits

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24
Q

GBM appearance in MPGNs?

A

Tram track or double contour appearance due to splitting of GBM

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25
Q

A circulating autoantibody that allows persistent activation of C3 convertase

A

C3 nephritic factor (C3NeF)

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26
Q

In IgA nephropathy, where are the immune complexes deposited?

A

Mesangium

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27
Q

This is characterized by diffuse thinning of the GBM with hematuria discovered on routine utinalysis. But renal function is normal and prognosis is excellent.

A

Thin basement membrane nephropathy (benign familial hematuria)
Also mutation in collagen type IV

28
Q

MC of acute kidney injury

A

ATI or ATN (old name)

29
Q

4 common gram negative bacteria causing UTI

A

Escherichia coli
Proteus
Klebsiella
Enterobacter

30
Q

MC cause of clinical pyelonephritis

A

Ascending infection

31
Q

It is characterized by infection of tubular epithelial cell nuclei, leading to nuclear enlargement and internuclear inclusions. On EM, inclusions contain virions arrayed in distinctive crystalline-like lattices

A

Polyomavirus nephropathy

32
Q

The only 2 diseases that can also affect the calyces

A

Chronic pyelonephritis
Analgesic nephropathy

33
Q

In chronic pyelonephritis, injury to and scarring in the kidney is most common where?

A

Upper or lower poles where reflux is more common

34
Q

Mc and 2nd mc cause of AKI

A

Pyelonephritis
Drug-induced tubulointerstitial nephritis

35
Q

Alpha chains of type IV collagen affected in Alport syndrome

A

3, 4, and 5

36
Q

A patient diagnosed with lymphoma underwent chemotherapy. He then developed AKI. What is the most likely cause?

A

Acute uric acid nephropathy
- from broken down nucleic acids from killed tumor cells

37
Q

What factors contribute to renal damage in light-chain cast nephropathy (myeloma kidney)

A

Bence-jones proteinuria and cast nephropathy
AL type amyloidosis
Light chain deposition disease (usually kappa in GBM and mesangium)

38
Q

A patient with cirrhosis suddenly developed AKI. What is the most likely cause?

A

Bile cast nephropathy

39
Q

Mc cause of renal artery stenosis

A

Atheromatous plaque

40
Q

What is deficient in TTP?

A

ADAMTS13

41
Q

A 7 year old girl came in due to diarrhea, melena, oliguria and hematuria after eating hamburger from a street vendor. Dx? Causative agent?

A

Typical HUS
E. Coli O157:H7 which produce shiga-like toxins

42
Q

MC inherited deficiency in atypical HUS

A

Factor H
- breaks down the alternative pathway C3 convertase

43
Q

What is a truly hypoplastic kidney?

A

When it shows no scars and has reduced number of renal lobes and pyramids, usually six or fewer

44
Q

This results from development of the metanephros in ectopic foci

A

Ectopic kidney

45
Q

Fusion of which poles is more common in horse-shoe kidney

A

Lower poles (90%)

46
Q

A 45 year old man came in due to hematuria and dragging sensation in the abdomen. He also complains of frequent headaches. On PE there is heart murmur and enlarged liver. Utz showed numerous cysts in the liver and bilateral kidneys. Dx? Mutations?

A

Autosomal Dominant polycystic kidney disease
PKD-1 encoding polycystin 1 (85%)
PKD-2 encoding polycystin 2 - less severe
Need BOTH alleles to be mutated
Both form a protein complex in cilia that regulates intracellular Ca in response to fluid flow

47
Q

An infant was found to have splenomegaly. There was also hematuria. Condition worsened and patient died. On autopsy, there was hepatic periportal fibrosis and proliferation of well diff biliary ductules. Kidneys were enlarged and cut sections show numerous small cysts in cortex and medulla (sponge-like). Micro: cysts were lined by uniform lining of cuboidal cells. Dx, mutation? Origin of cells?

A

Autosomal recessive polycystic kidney disease
PKHD1 encoding fibrocystin important in collecting duct and biliary differentiation
Cuboidal cells are from collecting ducts

48
Q

A 40 year old man underwent WAB CT for executive check-up. Numerous cysts were seen on medulla of both kidneys. Renal function test was normal. Dx? Micro?

A

Medullary sponge kidney
Cysts are lined by cuboidal or occasionally transitional epithelium - these are dilated collecting ducts in the medulla

49
Q

Most common genetic cause of ESRD in children and young adults
Mutation?

A

Nephronophthisis
NPHP1 to NPHP11 (encode nephrocystins)
JBTS2,3,9,11 (in juvenile form)
These proteins are found in the primary cilia
NPHP2 (inversin - mediates left-right patterning during embryogenesis)

50
Q

A 10 year old boy complains of polydipsia and polyuria. A relative was also known to have this. WAB CT showed numerous cysts in the medulla of the kidney. Renal biopsy shows the cysts were predominantly in the corticomedullary junction and these were lined by cuboidal to flat epithelium. The cortex had widespread atrophy and interstitial fibrosis. Dx? Prognosis?

A

Familial juvenile nephronophthisis (MC subtype)
Others: sporadic and renal-retinal dyaplasia
Cysts MC in corticomedullary junction
ESRD in 5-10 years due primarily to cortical tubulointerstitial damage

51
Q

A 21 year old male came in due to hematuria. Utz showed a cystic left kidney. Cysts ranged from a few mm to cm. What would you advise the patient? Dx? Prognosis? Expected histo findings?

A

Multicystic renal dysplasia
Nephrectomy of left kidney is curative
Bilateral disease will result in failure
Cysts lined by flattened epithelium. Islands of undifferentiated mesenchyme, often with cartilage and immature collecting ducts.

52
Q

A 59 year old man on dialysis complained of hematuria. Renal utz showed numerous cysts in the kidney. Dx? Cause? Prognosis?

A

Acquired cystic disease
Dialysis-Obstruction of tubules by oxalate crystals or fibrosis
100-fold risk of developing RCC

53
Q

What is the most common renal stone? What are the other types?

A

MC: calcium stones (calcium oxalate +/- calcium phosphate)
Triple stones or struvite stones (magnesium ammonium phosphate)- by bacteria like proteus that convert urea to ammonia —> largest stone “staghorn calculi”
Uric acid stones- those with gout and leukemias (due to high cell turnover rate)
Cystine- genetic defect in renal reabsorption of amino acids including cystine

54
Q

Which type of renal stonw form the largest stones - “staghorn calculi”

A

Triple stones or struvite stones

55
Q

What enhances stone formation in urine?

A

Deficiency in inhibitors of crystal formation in urine

56
Q

A 25 year old man was paranoid about a 1 cm mass in his kidney. Renal biopsy was done which showed a cortical mass composed of complex, branching, papillomatous structures with numerous complex fronds. Dx? Prognosis?

A

Renal papillary adenoma
Cut off of 3 cm (1.5 cm in WHO) separates benign from metastasizing but there were reports that smaller tumors have metastasized

57
Q

A 12 year old girl came in due to seizures, and was reported to have intellectual disability. A whole body scan showed a mass in the left renal area. What is the most likely dx?

A

Angiomyolipoma
- vessels, smooth muscle and fat originating from perivascular epithelioid cels
Present in 25-50% of patients with tuberous sclerosis (LOF mutations in TSC1 or TSC2)

58
Q

A tumor is composed of large eosinophilic cells having small, round, benign-appearing nuclei with large nucleoli. Dx? The cells arise from?

A

Oncocytoma
Intercalated cells of collecting ducts

59
Q

Importance of detection of mutation in NPHS1 or NPHS2 in FSGS

A

Mutations in these genes tend to be resistant to immunotherapy

60
Q

Pathogenesis of dense deposit disease

A

Circulating C3 nephritocenic factor (C3NeF)
Familial: complement factor H-related (CFHR) gene mutations

61
Q

Pathogenesis of IgA neohropathy

A

Abnormally glycosylated IgA1 contributes to formation of anti-glycan antibodies or some viruses or bacteria express galNac—> complexes attach to fibronectin in ECM or type IV collagen in mesangium—> mesangial hypercellularity

62
Q

Which type of inflammatory cell is most characteristic of chronic pyelonephritis?

A

Plasma cells

63
Q

Results from a long standing renal infection that may form a mass-like lesion which is well-circumscribed with variegated tan to brown surface. May mimic an RCC radiographically.

A

Xanthogranulomatous pyelonephritis

64
Q

Usual nephrotoxic drugs causing papillary necrosis in analgesic nephropathy

A

Aspirin —> block vasodilation —> ischemia
Phenacetin —> acetaminophen
NSAID

65
Q

What is the infectious agent in most renal abscesses?

A

Staphylococcus aureus

Kidney (2 decks)

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