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Pathology > Renal pathology > Flashcards

Renal pathology Flashcards

1
Q

Hartnup disease

A

Autosomal recessive deficiency of neutral amino acid (e.g. tryptophan) transporters in proximal renal tubular cells and enterocytes, which leads to neutral aminoaciduria and decreased absorption from the gut

Pellagra-like symptoms

Tx: high-protein diet and nicotinic acid

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2
Q

Fanconi syndrome

A

Reabsorptive defect in the PCT, leading to increased excretion of amino acids, glucose, phosphate, and bicarb (type II RTA).

Causes: Wilson disease, ischemia, nephrotoxins/drugs

Order of renal tubular defects: Fanconi, Bartter, Gitelman, Liddle (“the kidneys put out FABulous Gliterring Liquid”)

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3
Q

Bartter syndrome

A

Autosomal recessive reabsorptive defect in thick ascending loop of Henle. Affects Na/K/2Cl cotransporter (effects similar to loop diuretics)

Results in hypokalemia and metabolic alkalosis with hypercalciuria

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4
Q

Gitelman syndrome

A

Autosomal recessive reabsorptive defect of NaCl in DCT. Affects similar to thiazide diuretics.

Hypokalemia and metabolic alkalosis without hypercalciuria.

Less severe than Bartter syndrome

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5
Q

Liddle syndrome

A

Autosomal dominant, increased activity of epithelial Na+ channels in distal and collecting tubules.

HTN, hypokalemia, metabolic alkalosis, decreased aldosterone.

Tx: amiloride

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6
Q

Polycystic kidney disease (PKD)

A

Inherited defect leading to bilateral enlarged kidneys with cysts in the renal cortex and medulla

Autosomal recessive form - presents in infants; cysts in kidney and liver; portal HTN (due to hepatic fibrosis)

Autosomal dominant form - presents in young adults; mutation in APKD1/2; cysts in kidney, liver, and brain (berry aneurysms); mitral valve prolapse

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7
Q

Acute tubular necrosis

A

Most common cause of intrarenal azotemia; presents with brown granular casts and hyperkalemia

Etiology may be ischemic or nephrotoxic

  • Proximal tubule and medullary segment of thick ascending limb are most susceptible to ischemic damage
  • Proximal tubule is most susceptible to nephrotoxins
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8
Q

Nephrotoxins

A

Nephrotoxins cause acute tubular necrosis.

  • Aminoglycosides (e.g. gentamicin)
  • Heavy metals (e.g. lead)
  • Myoglobinuria
  • Ethylene glycol (oxalate crystals in urine)
  • Radiocontrast dye
  • Urate (tumor lysis syndrome; PPx with hydration and allopurinol)
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9
Q

Acute interstitial nephritis

A

Drug-induced hypersensitivity involving interstitium and tubules; characterized by oliguria, fever, and eosinophils in urine

Causes: NSAIDs, penicillin, diuretics

May progress to renal papillary necrosis

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10
Q

Renal papillary necrosis

A

Presents with gross hematuria and flank pain

Causes:

  • Chronic analgesic abuse (penacetin/aspirin use)
  • Diabetes
  • Sickle cell
  • Severe acute pyelonephritis
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11
Q

Nephrotic syndrome

A

Glomerular disorders characterized by proteinuria (> 3.5 g/day) resulting in:

  • Hypoalbuminemia (pitting edema)
  • Hypogammaglobulinemia (increased risk of infection)
  • Hypercoagulable state (loss of antithrombin III)
  • HLD and hypercholesterolemia (fatty casts in urine)
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12
Q

Minimal change disease

A

Nephrotic syndrome; most common in children.

May be associated with Hodgkin lyphoma (cytokine release disrupts podocytes)

Effacement of foot processes results in loss of albumin but not immunoglobulin

Excellent response to steroids (unique among nephrotic syndromes)

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13
Q

Focal segmental glomerulosclerosis (FSGS)

A

Nephrotic syndrome

Hispanics and African Americans

HIV, heroin use, sickle cell disease

Like MCD, FSGS involves effacement of the foot process

Unlike MCD, FSGS does NOT respond well to steroids. Progresses to chronic renal failure

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14
Q

Membranous nephropathy

A

Nephrotic syndrome

Caucasian adults

Hepatitis B or C, solid tumors, SLE, drugs (e.g. NSAIDs, penicillamine)

Immune complexes deposit below the podocytes (subepithelial deposits) to produce granular IF and “spike and dome” appearance on EM

Progresses to chronic renal failure

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15
Q

Membranoproliferative glomerulonephritis (MPGN)

A

Mixed nephrotic/nephritic syndrome

“Tram-track” appearance on H&E and granular IF

  • Type I MPGN - subendothial immune complex deposits; associated with HBV and HCV
  • Type II MPGN - intramembranous immune complex deposits; associated with C3 nephritic factor (leads to overactivation of complement and inflammation)

Progresses to chronic renal failure

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16
Q

Effect of diabetes on the kidneys

A
  1. Nonenzymatic glycosylation of vascular basement membrane
  2. Hyaline arteriosclerosis (especially glomerular efferent arteriole)
  3. Hyperfiltration injury leads to nephrotic syndrome (sclerosis of mesangium with Kimmelstiel-Wilson nodules)

PPx: ACE inhibitors

17
Q

Effect of systemic amyloidosis on the kidneys

A

Amyloid deposits in mesangium, resulting in nephrotic syndrome

18
Q

Post-streptococcal glomerulonephritis (PSGN)

A

Nephritic syndrome

Presents 2-3 weeks after infection with nephritogenic strain of GAS (M protein virulence factor)

Immune complex deposition (granular IF) due to IgG, IgM, and C3 deposition; subepithelial “humps” on EM

Increased anti-DNase B. Decreased complement.

Usually resolves, but 25% of adults progress to rapidly progressive glomerulonephritis

19
Q

Rapidly progressive glomerulonephritis (RPGN)

A

Nephritic syndrome

Progresses to renal failure in weeks to months

Crescents in Bowmn space (comprised of fibrin, C3b, and macrophages)

Etiology of RPGN determined by IF pattern:

  • Linear (anti-basement membrane antibody) - Goodpasture syndrome
  • Granular (immune complex deposition) - PSGN or diffuse proliferative glomerulonephritis
  • Negative IF (pauci-immune) - Wegener granulomatosis (c-ANCA), microscopic polyangitis (p-ANCA); Churg-Strauss (p-ANCA and granulomas/asthma/eosinophilia)
20
Q

IgA nephropathy

A

Nephritic syndrome

Usually follows mucosal infection (e.g. gastroenteritis)

IgA deposits in mesangium

21
Q

Alport syndrome

A

Nephritic syndrome

X-linked defect of type IV collagen

Isolated hematuria, sensory hearing loss, ocular disturbances

22
Q

Calcium oxalate/calcium phosphate nephrolithiasis

A

Most common type of nephrolithiasis.

Envelope/dumbbell shaped crystals

Idiopathic hypercalciuria, hypercalcemia.

Ethylene glycol (antifreeze), vitamin C abuse, and Crohn disease promote formation of oxalate crystals.

Calcium phosphate crystals precipitate at alkalinic pH. Calcium oxalate crystals precipitate at acidic pH

Tx: HCTZ (Ca-sparing diuretic), citrate

23
Q

Ammonium magnesum phosphate (struvite) nephrolithiasis

A

Coffin lid-shaped crystals

Infection with urease-positive organisms (e.g. Proteus, Staph or Klebsiella) that hydrolyze urea to ammonia

Staghorn calculi (nidus for UTIs) precipitate at alkalinic pH

Tx: Surgical removal of staghorn calculi, eradication of pathogen

24
Q

Uric acid nephrolithiasis

A

Radiolucent (unique among stones); rhomboid-shaped crystals

Hyperuricemia (leukema, myeloproliferative disorders, gout)

Risk factors include low urine volumes and acidic pH

Tx: hydration, alkalinization of the urine (potassium bicarbonate), allopurinol

25
Q

Cystine nephrolithiasis

A

Hexagonal shaped crystals

Children; associated with decreased reabsorption of cysteine

Staghorn calculi (like ammonium magnesium phosphate stones)

Tx: hydration and alkalnization of urine

26
Q

Chronic renal failure

A
  • Uremia (nausea, anorexia, pericarditis, platelet dysfunction, encephalopathy with asterixis, deposition of urea crystals in skin)
  • HTN
  • Hyperkalemia with metabolic acidosis
  • Anemia (decreased EPO production by renal peritubular interstitial cells)
  • Hypocalcemia and hyperphosphatemia (decreased 1-alpha-hydroxylation by proximal renal tubule cells)
  • Renal osteodystrophy due to secondary hyperparathyroidism, osteomalacia (decreased mineralization), and osteoporosis (metabolic acidosis)
  • Dyslipidemia (hypertriglyceridemia)
  • Waxy casts

Increases risk for RCC

27
Q

Renal cell carcinoma

A

Hematuria, palpable mass, flank pain

Paraneoplastic syndromes: EPO, renin, PTHrP, ACTH

Left-sided varcocele due to involvement of left renal vein

Loss of VHL (3p) tumor suppressor gene

Sporadic tumors - single tumor in upper pole of kidney; adult male smokers

Hereditary tumors - bilateral tumors, young adults.

28
Q

Von Hippel-Lindau disease

A

Autosomal dominant disorder associated with inactivation of VHL gene

Hemangioblastoma of the cerbellum and bilateral renal cell carcinomas

29
Q

WAGR syndrome

A

WAGR: Wilms tumor, Aniridia, Genital abnormalities, mental and motor Retardation

Deletion of WT1 tumor suppressor gene (11p13)

30
Q

Denys-Drash syndrome

A

Wilms tumor, progressive glomerular disease, male pseudohermaphroditism

Mutation of WT1 tumor suppressor gene (11p13)

31
Q

Beckwith-Wiedemann syndrome

A

Wilms tumor, neonatal hypoglycemia, muscular hemihypertrophy, organomegaly (including tongue)

Mutations in WT2 gene cluster

32
Q

Urothelial (transitional cell carcinoma)

A

Arises from urothelial lining of renal pelvis, ureter, bladder, or urethra

Risk factors: cigarette smoke, naphtylamine, azo dyes, long-term cyclophosphamide or phenacetin use

Painless hematuria

Two pathways: flat (high-grade, early p53 mutation), papillary (low-grade, late p53 mutation)

Tumors are often multifocal and recur (“field defect”)

33
Q

Squamous cells carcinoma of the bladder

A

Risk factors:

  • Chronic cystitis (older woman)
  • Schistosoma hematobium infection (young Egyptian male)
  • Long-standing nephrolithiasis
34
Q

Adenocarcinoma of the bladder

A

Etiologies:

  • Urachal remnant (tumor develops at dome of bladder)
  • Cystitis glandularis (chronic inflammation of bladder)
  • Exstrophy (congenital failure to form caudal portion of anterior abdominal and bladder walls)
35
Q

Diffuse proliferative glomerulonephritis

A

Nephritic syndrome. Etiology of rapidly progressive glomerulonephritis

Sub-endothelial deposits of IgG and C3

“Wire-looping” of capillaries

Most common caused of death in SLE

36
Q

Metabolic alkalosis with low urine Cl-

A

Often associated with volume depletion (increased reabsorption of bicarb in the proximal tubule)

Responds to saline infusion (repletes Cl- and volume)

Etiologies: vomiting, contraction alkalosis, previous thiazide use, cystic fibrosis

37
Q

Metabolic alkalosis with high urine Cl-

A

Often associated with volume expansion and hypokalemia

Resistant to saline infusion therapy

Etiologies: hyperaldosteronism, severe hypokalemia, current diuretic therapy, Bartter’s syndrome or Gittelman syndrome, aminoglycoside toxicity

Pathology (17 decks)

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