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STEP 1 Pathoma > Chapter 12 > Flashcards

Chapter 12 Flashcards

1
Q

A Horseshoe kidney would get snuck on what structure?

A

IMA

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2
Q

What is renal agenesis?

A

Absent kidney formation (may be unilateral or bilateral) usually lading to hypertophy of the existing kidney

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3
Q

What does bilateral renal agenesis lead to?

A

Potter sequence- with oligohydramnios with lung hypoplasia, flat face with low set ears, and developmental defects of the extremities

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4
Q

Describe a dysplastic kidney

A

Noninherited, congenital malformation of the renal parenchyma marked by cysts and abnormal tissues

Note that this is usually unilateral but must be dsitignuished from inherited polycystic kidney disease if bilateral

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5
Q

What is this?

A

Polycystic kidney disease- an inherited defect leading to bilateral enlarged kidnyes with cysts in the renal cortex and medulla

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6
Q

How does the AR form of PKD present?

A

Presents in infants as worsening renal failure and HTN or with Potter sequence in newborns

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7
Q

How does the AD form of PKD present?

A

Presents in young adults as HTN due to increasd renin, hematuria. and worsening renal failure

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8
Q

The main associations of PKD are:

A

Berry aneurysm, hepatic cysts, and mitral valve prolapse

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9
Q

What is medullary cystic kidney disease?

A

AD inherited defect leading to cysts in the medullary collecting ducts resulting in parenchymal FIBROSIS leading to shrunken kidneys and renal failure

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10
Q

What is the hallmark of acute renal failure?

A

Acute, severe decrease in renal function developing within days with a hallmark of azotemia (increased BUN and Cr), often with oliguria

Divided into prerenal, postrenal, and intrarenal based on etiology

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11
Q

What are the main causes of prerenal azotemia?

A

Due to decreased blood flo to the kidneys (e.g. cardiac failure, renal steneosis) resulting in decreased GFR< azotemia, and oliguria

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12
Q

What are the main lab findings of prerenal azotemia?

A

Reabsorption of fluid and BUN ensues (serum BUN:Cr > 15);

tubular function remains intact (fractional excretion of sodium (FENa) < 1%)

and urine osmolarity remains > 500 mOsm/kg

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13
Q

What are the main causes of postrenal azotemia?

A

Due to obstruction of the urinary tract downstream from the kidney resulting in decreased outflow and subsequent decreased in GFR with onset of azotemia and oliguria

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14
Q

What are the main lab findings of postrenal azotemia?

A

During early stages of obstruction, increased tubular pressure forces BUN into the blood (serum BUN:Cr >15), FENa remains <1%, and urine osm >500 BUT

with long-standing: serum BUN:Cr <15, FENa >2%, and urine osm <500mOsm/kg

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15
Q

What is acute tubular necrosis?

A

Injury and necrosis of the tubular epithelial cells (most common cause of acute renal failure; intrarenal azotemia)

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16
Q

What are the urinaylsis findings of acute tubular necrosis?

A

Brown, granular casts (necrotic cells lug the tubules and GFR decreases)

serum BUN:Cr <15, FENa >2%, and urine osm < 500

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17
Q

What are the main etiologies of acute tubular necrosis?

A

Etiology may be iscehmic (derceased blood supply resuts in tubular necrosis) or nephrotoxic (toxic agents induce necrosis)

Ischemia- often preceded by prerenal azotemia and the proximal tubule and medullary sgemnt of the thick ascending limb are particularly susceptible to ischemic injury

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18
Q

Describe nephrotoxic damage to the kidneys

A
  • Proximal tubules is most susceptible
  • Amingoglycosides, heavy metals, myoglobinuri (from muscle injury), ethylene glycol, urate (TLS)
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19
Q

________ are used prior to chemo to decrease the risk fo urate-induced ATN

A

Allopurinol (and hydration)

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20
Q

Clinical features of ATN

A

Oliguria with brown, granular casts

Elevated BUN and Cr

Hyperkalemia due to decreased renal excretion with metabolic acidosis

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21
Q

What is acute interstitial nephritis?

A

Drug induced hypersensitivity involving the interstitium and tubules resulting in acute renal failure (intrarenal)

causes include NSAIDS, penicillin, and diuretics

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22
Q

How does acute interstitial nephritis present?

A

oliguira, fever, and rash days-wekeks after starting a drug; eosinophils may be seen in urine

Resolves with drug cessation but may progress to renal papillary necrosis

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23
Q

What are the main causes of renal papillary necrosis?

A

Sickle cell

Severe acute pyelonephritis

Diabetes mellitus

Analgesic abuse

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24
Q
A
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25
Q

What is the basis of nephrotic syndrome?

A

Glomerulus dysfunction marked by proteinuria (3.5+g/day) resulting in:

Hypoalbuminemia- pitting edema

Hypogammaglobulinemia- infection risk

Hypercoaguable state due to loss of antitihrombin III

Hyperlipidemia and hypercholesterolemia- may result in fatty casts in urine

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26
Q

What is the most common cause of nephrotic syndrome in children?

A

Minimal Change Disease

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27
Q

What causes Minimal Change Disease?

A

Usually idiopathic but may be associated with Hodgkin lymphoma

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28
Q

How does MCD appear on imaging?

A

H&E- normal glomeruli with normal BM

Effacement of foot processes on EM

Negative immunoflourescence (no immune complex deposition)

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29
Q

How is MCD tx?

A

Good response to steroids

30
Q

What is the most common cause of nephrotic syndrome in Hispanics and AA?

A

Focal segmental glomerulosclerosis (FSGS)

31
Q

What causes FSGS?

A

Usually idiopathic but may be associated with HIV, heroin use, and sickle cell

32
Q

Imaging findings of FSGS

A

Focal and segmental sclerosis of glomeruli

Foot process effacement on EM

Negative IM

33
Q

How is FSGS tx?

A

Poor response to steroids

34
Q

What is the most common cause of nephrotic syndrome in Causasian adults?

A

Membranous nephropathy

35
Q

What causes membranous nephropathy?

A

Usually idiopathic but may be associated with HBV/HCV, solid tumors, SLE, or drugs such as NSAIDS and pencillamine

36
Q

Imaging of Membranous Nephropathy

A

Thick glomerular basement membrane on H&E

Spike and Dome subepithelial deposit on EM
Granular IF due to immune complex deposition

Poor response to steroids

37
Q

A tram-track appearance on H&E due to thickened glomerular BM is classic in what disease?

A

Membranouproliferative glomerulonephritis (will also see a granular IF due to immune complex deposition)

38
Q

What are the types of Membranouproliferative glomerulonephritis?

A

Type I- subendothelial (associated with HBV/HCV)

Type II- associated with C3 nephritis factor (autoantibody that stabilizes C3 convertase, leading to overactivation of complement, inflammation, and low levels of C3)

39
Q

Prognsosis of Membranouproliferative glomerulonephritis?

A

Poor response to steroids- progresses to chronic renal failure

40
Q

How does DM cause kidney damage?

A

High serum glucose leads to nonezymatic glycosylation of the vascular basement membrane resultin in hyaline arteriolosclerosis (the glomerular efferent arteriole is more affected than the affarent arteriole, leading to a high glomerular filtration pressure- leads to microalbuminuria)

41
Q

DM induced kidney damage eventually progresses to __________

A

Nephrotic syndrome marked by sclerosis of mesangium with formation of Kimmelstein-Wilson nodules

42
Q

What is the most commonly affected organ with systemic amyloidosis?

A

Kidneys (amyloid deposits in the mesangium resulting in nephrotic syndrome)- Congo red shows apple-green birefrigence

43
Q

Describe the basis of nephritic syndrome

A

Glomerular dysfunction marked by glomerular inflammation with:

limited proteinuria (<3.5g/day)

Oliguria and azotemia

Salt retein with periorbital edema and HTN

RBC casts and dysmorphic RBCs in urine

44
Q

Describe PSGN

A

Nephritic syndrome that arises after GAS infection of the either the skin or pharynx presenting 2-3 WEEKS after infection as hematuria (cola colored urine), oliguria, HTN, and priorbital sweeling (usually in children)

45
Q

What causes rapidly progressive glomerulonephritis?

A

Nephritic syndrome that progresses to renal failure within weeks-months due to crescent formation in Bowman space of glomeruli, composed of FIBRIN and MACROPHAGES

46
Q

The immunoflourecence pattern with rapidly progressive glomerulonephritis can help make a diagnosis of the underlying cause. Explain

A

Linear (anti-BM Ab)- Goodpasture syndrome

Granular- PSGN most common or diffuse proliferative glomerulonephritis

Negative IF- Wegener (c-ANCA), microscopic Polyangiitis, and Churg-Strauss syndrome

47
Q

What is the most common nephropathy worldwide?

A

IgA Nephropathy (Berger Disease)- IgA immune complex deposition in the emsangium of glomeruli presenting in childhoood as episodic gross or microscopic hematuria with RBC casts following mucosal ifnections (e.g. gastroenteritis)

48
Q

What is Alport Syndrome?

A

Inherited defect in type IV collagen (X-linked) resulting in thinning and splitting of the glomerular BM and presenting as isolated hematuira, sensory hearing loss and ocular issues

49
Q

How does cystitis present?

A

Dysuria, urinary frequency, urgency, and suprapubic pain with systemic signs of fever usually absent

50
Q

What is sterile pyuria?

A

The presence of pyuria (10+ WBC/hpf and leukocyte esterase) with a negative urine culture- suggests urethritis due to Chlamydia trachomatis or Neisseria Gonorrhoeae

51
Q

What are some findings of chronic pyelonephritis?

A

Interstitial fibrosis and atrophy of tubules due to multiple bouts of acute pyelonephritis (commonly due to vesicoureteral reflux in children or obstruction (BPH, cervical carcinoma)

Leads to cortical scarrign with blunted calyces

52
Q

What is the classic microscopic finding of chronic pyelonephritis?

A

Thyroidization of the atrophic tubules

53
Q

One consequence of chronic renal failure is uremia. How might this present?

A

Increased nitrogenous waste rpoducts in blood result in N/V, anorexia, pericarditis, platelet dysfunction, encephalopathy with asterixis, and deposition of urea crystals in skin

54
Q

Angiomyolipoma- hamartoma comprised of blood vessels, smooth muscle, and adipose tissue (increased frequency in tuberous sclerosis)

A
55
Q

How might a renal cell carcinoma present?

A

With a triad of hematuria, palpable amss, and flank pain

Fever, weight loss, or paraneoplastic syndrome (e.g. EPO, renin, PTHrP, or ACTH) may also be present

rarely may present with ledt-sided varicocele

56
Q

How does a renal cell carcinoma apepar grossly and microscopically?

A

Clear cell type shows a clear cytoplasm (most common subtype)

57
Q

What causes renal cell carcinoma?

A

loss of VHL (3p) tumor suppressor gene, which leads to increased IGF-1 (promotes growth) and increased HIF transcirption factor (increased VEGF and PDGF)

58
Q

Renal cell carcinoma may be hereditary or sporadic. Explain.

A

Sporadic- classically in males, 60+ yo, as a single tumor in the UPPER pole of the kidney (risk: cigs)

Hereditary- arise in young adults and are often bilateral (Von Hippel Disease)

59
Q

In addition to increasd risk of renal cell carcinoma, mutation of VHL also carries a risk of _______

A

hemangioblastoma of the cerebellum

60
Q

How is renal cell carcinoma staged?

A

T- based on size and involvement of the renal vein

N- spread to retroperitoneal lymph nodes

61
Q

What is a Wilms Tumor?

A

Malignant tumor comprised of blastema (immature kidney mesenchyma), primitive glomeruli and tubules, and stromal cells (most common malignant renal tumor in children with an avg. age around 3)

62
Q

What is the most common cause of Wilm Tumor?

A

90% Sporadic

63
Q

What are some syndromes involving Wilms Tumors?

A

WAGR Syndrome- Wilms Tumor, Aniridia, Genital Abnormalities, and mental and motor Retardation

Denys-Drash Syndrome- Wilm Tumor, progressive renal (Glomerular) disease, and male pseudohermphroditism

Beckwith-Wiedemann syndrome

64
Q

What is Aniridia?

A

is the absence of the iris, usually involving both eyes. It can be congenital or caused by a penetrant injury. Isolated aniridia is a congenital disorder which is not limited to a defect in iris development, but is a panocular condition with macular and optic nerve hypoplasia, cataract, and corneal changes.

65
Q

What causes WAGR Syndrome?

A

deletion of WTI tumor suppressor gene (11p13)

66
Q

What causes Denys-Drash Syndrome?

A

MUTATIONS (not deletions) of WT1

67
Q

Describe Beckwith-Wiedemann syndrome

A

Syndrome of WIlm tumor, neonatal hypoglycemia, muscular hemihypertrophy, and organomegaly (including tongue)

68
Q

What causes Beckwith-Wiedemann syndrome?

A

mutations in WT2 gene cluster (11p15.5), particularly IGF-2

69
Q

Describe a urothelial (transitional cell) carcinoma

A

Malignant tumor ariisng from the urothelial lining of the renal pelvis, ureter, bladder, or urethra (most commonly in the bladder)

70
Q

What are the risk factors for urothelial carcinoma?

A

Cigs

Naphthylalmine

Azo dyes

long-term cyclophosphamide or phenacetin use

71
Q

What are the two main types of urothelial carcinoma?

A

Flat- develops as a high-grade flat tumor and then invades; associated with early p53 mutations

Papillary- develops as a low-grade papillary tumor that progresses to a hgh-grade papillary tumor and then invades; not associated with p53 mutations

72
Q

What are the main risk factors for squamous cell carcinoma of the bladder?

A

chronic cystitis (older women), Schistosoma hematobium infection (Egyptians), and long-standing nephrolithiasis

STEP 1 Pathoma (18 decks)

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