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Renal II > Quiz 1 > Flashcards

Quiz 1 Flashcards

1
Q

How does the kidney act as an endocrine organ

A

Secreted erythropoietin, renin and prostaglandins and regulates vitamins D metabolism

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2
Q

What is the mechanism of most glomerular vs tubular and interstitial diseases

A

Tubular is usually immune mediated, tubular and interstitial are usually toxic or infectious

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3
Q

What is azotemia

A

Elevation of BUN and creatinine levels; related to decreased GFR; feature of acute and chronic injury

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4
Q

What is prerenal azotemia

A

Hypoperfusion of kidneys that impairs renal function in absence of parenhcymal damage

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5
Q

What is postrenal azotemia

A

Whenever urine flow is obstructed distal to kidney; relief of obstruction corrects the azotemia

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6
Q

What is uremia

A

When azotemia becomes associated with a constellation of clinical signs and sx; frequently manifest secondary involvement of GI (uremic gastroenteritis), peripheral nerves and heart (uremic fibrinous pericarditis)

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7
Q

What is nephritic syndrome

A

Caused by glomerular dz; dominated by acute onset of either grossly visible hematuria or microscopic hematuria (dysmorphic red cells and red cast cells on urinalysis), diminished GFT, proteinuria and HTN *classic presentation of poststrep glomerulonephritis

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8
Q

What is rapidly progressive glomerulonephritis

A

Nephritic syndrome with rapid decline in GFR (within hours to days)

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9
Q

What is nephrotic syndrome

A

Due to glomerular dz; heavy proteinuria (more than 3.5 gm/day), hypoalbuminemia, severe edema, hyperlipidemia, and lipiduria

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10
Q

What is asymptomatic hematuria or proteinuria usually a manifestation of

A

Subtle or mild glomerular abnormalities

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11
Q

What is acute kidney injury

A

Rapid decline in GFT with dysregulation of fluid and electrolyte balance and retention of met waste product (urea and creatinine); *most severe form -> oliguria or anuria (reduced or no pee); * can result from glomerular, interstitial, vascular, or acute tubular injury

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12
Q

What is chronic kidney dz.

A

Presence of diminished GFR less than 60 mL/min for at least 3 months from any cause, and/or persistent albuminuria

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13
Q

What is the GFR of ESRD

A

Less than 5% of normal; terminal stage of uremia

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14
Q

What are the characteristics of renal tubular defects

A

Dominated by polyuria, nocturia, and electrolyte disorders; results of dz that directly affects tubular structures (nephronophthisis medullary cystic dz) or cause defects in specific tubular fxns (can be inherited - familial nephrogenic diabetes, cystinuria, renal tubular acidosis) or acquired (lead nephropathy)

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15
Q

What are the clinical manifestations of urinary tract obstruction and renal tumors

A

Varied based on location; UTI -> bactetriuria and pyuria (bacteria and leukocytes in urine)

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16
Q

What are secondary glomerular dz

A

Glomerular damage caused by other diseases

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17
Q

What is primary glomerulonephritis or glomerulopathy

A

When kidney is only or predominant organ involved

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18
Q

What are the fluid and electrolyte systemic manifestations of chronic kidney dz

A

Dehydration, edema, hyperkalemia, met acidosis

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19
Q

What are the calcium phosphate and bone systemic manifestations of chronic kidney dz

A

Hyperphosphatemia, hypocalcemia, secondary hyperparathyroidism, Renal osteodystrophy

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20
Q

What are the hematologic systemic manifestations of chronic kidney dz

A

Anemia, bleeding diarrhea is

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21
Q

What are the cardiopulm systemic manifestations of chronic kidney dz

A

HTN, CHF, cardiomyopathy, pulm edema, uremic pericarditis

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22
Q

What are the GI systemic manifestations of chronic kidney dz

A

N/V, bleeding, esophagitis, gastritis, colitis

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23
Q

What are the neuromuscular systemic manifestations of chronic kidney dz

A

Myopathy, peripheral neuropathy, encephalopathy

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24
Q

What are the dermatologic systemic manifestations of chronic kidney dz

A

Sallow color, Pruritus, dermatitis

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25
Q

What are the primary glomerulopathies

A

Acute proliferative glomerulonephritis, rapidly progressive, membranous nephropathy, minimal change dz, focal segmental glomerulosclerosis, membranoproliferative glomerulonephritis, dense deposit dz, IgA nephropathy, chronic glomerulonephritis

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26
Q

What are the systemic dz with glomerular involvement

A

SLE, DM, amyloidosis, goodpasture syndrome, microscopic polyarteritis/polyangiitis, wegener granulomatosis, henoch-schonlein purpura, bacterial endocarditis

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27
Q

What are the hereditary glomerular diseases

A

Alport syndrome, thin basement membrane dz, fabry dz

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28
Q

What does the glomerular capillary wall consist of

A
  • fenestrated endothelial cells
  • glomerular basement membrane (GBM) with a thick lamina densa and thinner (lamina rara interna/externa); NC1 domain important for assembly of collagen into BM (target of abs in anti-GBM nephritis); alpha chain defects -> hereditary nephritis
  • visceral epithelial cells (podocytes)- adhere to lamina rara externa
  • supported by mesangial cells between capillaries; impt in forms of glomerulonephritis
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29
Q

What is the glomerulus permeable to

A

Water, small solutes; impermeable to proteins the size of albumin or larger ; more cationic -> more permeable

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30
Q

What is the visceral epithelial cell important for

A

Maintenance of glomerular barrier function; its slit diaphragm presents a size-selective distal diffusion barrier to filtration of proteins and is responsible for synthesis of GBM components

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31
Q

What is nephrin

A

Transmembrane protein with a large extracellular portion made up of Ig-like domain; extend toward each other and dimerzie across the slit diaphragm; form connections with podocin, CD2-assoc protein and actin cytoskeleton of podocytes

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32
Q

What do mutations in slit diaphragm proteins lead to

A

Nephrotic syndrome

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33
Q

What are the glomerular syndromes

A
  • nephritic: hematuria, azotemia, variable proteinuria, oliguria, edema, HTN
  • rapidly progressive glomerulonephritis: acute nephritis, proteinuria, acute renal failure
  • nephrotic syndrome: proteinuria (>3.5), hypoalbuminemia, hyperlipidemia, lipiduria
  • chronic renal failure: azotemia -> uremia progressive for months to years
  • isolated urinary abnormalities: glomerular hematuria and/or subnephrotic proteinuria
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34
Q

What causes hypercellularity of the glomerular tufts

A
  • proliferation of mesangial or endothelial cells
  • infiltration of leukocytes -> referred to as endocapillary proliferation
  • formation of crescents: accumulations of cells composed of proliferating glomerular epithelial cells and infiltrating leukocytes; occurs following immune/inflammatory injury involving cap walls; plasma proteins leak into urinary space where exposure to procoagulants lead to fibrin deposition; activation of coagulation factors is trigger for crescent formation
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35
Q

What is hyalinosis

A

Accumulation of material that is homogenous and eosinophilic by light micro; hyalin is composed of plasma proteins that have insulated from circulation into glomerular structures; can obliterate cap lumens; usually a consequence of endothelial or cap wall injury and end result of glomerular damage

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36
Q

What is sclerosis

A

Deposition of collagenous matrix; can be confined to mesangial areas (DM) or involve cap loops; can result in obliteration of cap

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37
Q

What are the types of primary glomerulopathies

A

Diffuse: involving all of glomeruli
Global: involving the entirety of the individual glomeruli
Focal: involving only a fraction of glomeruli
Segmental: affecting a part of each glomerulus
Cap loop or mesangial: affecting predominantly cap or mesangial regions

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38
Q

What is the classic example of glomerular injury resulting from local formation of immune complexes (abs reacting to antigens on glomerulus)

A

Membraneous nephropathy

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39
Q

What antigen underlies most cases of primary human membranous nephropathy

A

M-type phospholipase A2 receptor (PLA2R): ab that bind to this leads to complement activation and shedding of immune aggregates from the cell surface to form characteristic deposits of immune complexes along the subepithelial aspect of the BM

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40
Q

What is the pattern of immune deposition on immunofluorescent microscopy of membranous nephropathy

A

Granular rather than linear; reflective of very localized antigen-ab reaction; on light microscopy, BM looks thickened

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41
Q

What are the thiazides diuretics and what are they used to treat

A

Hydrochlorothiazide, metolazone, chlorthalidone; HTN and edema

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42
Q

What are the K+ sparing diuretics and what are they used to treat

A

Na+ channel blockers: amiloride and triamterene
Aldosterone antagonist: spirinolactone and eplerenone
-edema and HTN

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43
Q

What are the carbonic anhydrase inhibitors and what are they used to treat

A

Acetazolamide; urinary alkalization, mountain sickness, glaucoma

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44
Q

What are the loops diuretics and what are they used to treat

A

Furosemide, torsemide, bumetanide, ethacrynic acid; edema and HTN

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45
Q

What are the aquaretics and what are they used to treat

A

Conivaptan and tolvaptan; hyponatremia

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46
Q

What is the osmotic diuretic and what is it used to treat

A

Mannitol; maintains urine flow

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47
Q

What is a natriuretic

A

Substance that promotes excretion of sodium

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48
Q

Which diuretics work on the proximal tubule

A

Osmotic and carbonic anhydrase inhibitors

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49
Q

Which diuretics work on the thin descending loop of Henle

A

Osmotic diuretics

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50
Q

Which diuretics work on the thick ascending loop of henle

A

Loop diuretics

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51
Q

Which diuretics work on the distal convoluted tubule

A

Thiazides

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52
Q

Which diuretics work on the cortical collecting duct

A

Sodium channel blockers and spironolactone

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53
Q

Which diuretics work on the collecting duct

A

Vaptans

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54
Q

What is the MOA of thiazides

A

Sodium chloride channel blocker

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55
Q

What is the MOA of loop diuretics

A

Sodium, potassium, 2 chloride blocker

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56
Q

Where do the K+ sparing drugs work

A

Late distal convoluted tubule and collecting duct

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57
Q

What are the effects of K on the heart

A

Tall t waves, prolonged PR interval, widened QRS interval, flattened P waves, bradycardia, V tach/fib; sinus arrest or nodal rhythm

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58
Q

What are the effects of hypokalemia on the heart

A

Flattened T waves, ST depression, prolonged QT interval, tall U waves, atrial Arrhythmias, v tach/fib

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59
Q

Which diuretics contain sulfa

A

Loop, thiazides

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60
Q

Which diuretics have the greatest amount of diuretic effect

A

Loop

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61
Q

What is the MOA of furosemide

A

Directly inhibits reabosorption of sodium and chloride; indirectly inhibits reabsoprtion of calcium and magnesium; causes increased excretion of water, sodium, potassium, chloride, magnesium and calcium

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62
Q

What is furosemide given to treat

A

Edema assoc with heart failure, hep dz, renal dz; acute pulm edema -> decreases preload; causes rapid dyspnea relief; HTN -> works in patients with low GFR

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63
Q

What toxicities are associated with furosemide

A

Hypokalemia, hyponatremia, hypocalcemia(kidney stones), hypomagnesemia, hypochloremic metabolic alkalosis, hyperglycemia, hyperuricemia (gout), increased chol and triglycerides (atherosclerosis), ototoxicity, hypersensitivity b/c sulfa, dangerous during pregnancy

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64
Q

What are the characteristics of torsemide, bumetanide, ethacrynic acid

A

Torsemide: longer 1/2 life and better oral absorption than furosemide
Bumetanide: more predictable oral absorption
Ethacrynic acid: *non-sulfonamide loop diuretic reserved for those with sulfa allergy

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65
Q

What kind of urine is produced with loop diuretics

A

Isotonic

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66
Q

What forms are available for loop diuretics

A

PO, IV, IM

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67
Q

What drug interactions do loop diuretics hav e

A
  • digoxin
  • ototoxic drugs (gentamicin)
  • potassium-sparing diuretics can counterbalance potassium wasting effects
  • *increases lithium toxicity
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68
Q

Which diuretics cause the greatest sodium loss

A

Loop

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69
Q

What are the effects of hydrochlorothiazide

A

Increases urinary excretion of sodium and water, potassium and magnesium

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70
Q

What are the clinical applications of HCTZ

A

Management of mild-moderate HTN *no effect in patients with low GFR; edema; calcium nephrolithiasis (causes decrease in excretion of calcium) and nephrogenic diabetes insipidus

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71
Q

What toxicities are assoc with HCTZ

A

Orthostatic hypotension, hypokalemia, hypomagnesemia, hyponatremia, hypochloremic met alkalosis, hypercalcemia, hyperglycemia, hyperuricemia, sulf drug hypersensitivity

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72
Q

What are the characteristcis of chlorothiazide, chlorthalidone, metolazone

A

Chlorothiazide: poor oral absorption
Chlorthalidone: longer half life
Metolazone: long acting; CHF

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73
Q

Why do thiazides cause more calcium absorption

A

More reabsopriton in prox tubule because of volume contraction

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74
Q

Is the loss of magnesium greater in loop or thiazides

A

Thiazides

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75
Q

What kind of urine does thiazides produce

A

Dilute

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76
Q

Which diuretic causes the greatest bicarbonate loss

A

Thiazides; impairs distal nephron H+ secretion

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77
Q

When should you not take thiazides diuretics (what time of day)

A

Bedtime

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78
Q

What drug interactions do thiazides have

A

Increases digoxin and lithium toxicity

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79
Q

Which thiazide is used more to reduce CV morbidity and mortality when used for tx of HTN

A

Chlorthalidone: more potent and longer lasting

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80
Q

What are the effects of amiloride

A

Small increase in sodium excretion; blocks major pathway for potassium elimination so K retained; indirectly decreases H, magnesium, and Ca excretion

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81
Q

What are the clinical applications of amiloride

A

Counteracts potassium loss induced by other diuretics in tx of HTN or heart failure; ascites, pediatric HTN

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82
Q

What toxicities are associated with amiloride

A

Hyperkalemia, hyponatremia, hypovolemia, Hyperchloremic metabolic acidosis, dizziness, N/V

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83
Q

What are the effects of spironolactone

A

Blunt as ability of aldosterone to promote Na K exchange in collecting duct by decreasing Na entry through luminal Na channels, decreased basolateral N/K ATPase

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84
Q

What are the clinical applications of spironolactone

A

Counteracts K loss by other diuretics; treatment of primary hyperaldosteronism; reduces fibrosis post MI heart failure; hirsutism; treatment of androgenic alopecia in females

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85
Q

What toxicities are associated with spironolactone

A

Hyperkalemia, amenorrhea, hirsutism, gynecomastia, impotence; tumorigen

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86
Q

What is eplerenone

A

More selective aldosterone antagonist; approved for use in post-MI heart failure

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87
Q

What is the onset of action for spironolactone

A

48 hrs

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88
Q

What are the aquaretics

A

Vaptans

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89
Q

What is the MOA of vaptans

A

Block ADH receptor in collecting duct *non-peptide vasopressin receptor antagonist

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90
Q

What are the clinical applications of conivaptan

A

Treatment of euvolemic and hypervolemic hyponatremia in patients who are hospitalized, symptomatic, not responsive to fluid restriction; *monitor sodium bc too rapid sodium correction -> osmotic demyelination; AD polyistic kidney dz

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91
Q

How is conivaptan administered

A

IV; *substrate of CYP3A4 so inducers and inhibitors of concern; eliminated in feces

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92
Q

What toxicities are associated with conivaptan

A

Orthostatic hypotension, thirst, polyuria, bedwetting

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93
Q

What are the characteristics of tolvaptan

A

Selective V2 receptor antagonist administered orally; *only in hospital; must use 30 days; longer use can be fatal (hepatotoxic)

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94
Q

How do carbonic anhydrase inhibitors work

A

Bicarbonate ion remains in early prox tubule, H+ cycling lost, inhibiting Na/H exchange -> leads to sodium bicarbonate diuretics and hyperchloremic acidosis

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95
Q

What are the uses for carbonic anhydrase inhibitors

A

Urinary alkalinization, metabolic alkalosis, glaucoma, acute mountain sickness

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96
Q

What are the adverse effects of carbonic anhydrase inhibitors

A

Hyperchloremic metabolic acidosis, nephrolithiasis, potassium wasting

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97
Q

How do you administer mannitol

A

IV in large amounts to raise osmolality (in grams)

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98
Q

What are the adverse effects of osmotic diuretics

A

ECV is acutely increased which can exacebate heart failure

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99
Q

What are the uses of osmotic diuretics

A

Prophylaxis of renal failure (keeps fluid in tubule), reduction in intracranial pressure,, reduction in intraocular pressure

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100
Q

How is licorice a. Diuretic

A

Contains glycyrrhizic acid which. Potentiates aldosterone effect

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101
Q

When do you give HCTZ to someone with cirrhosis.

A

If ClCr > 50 mL/min;; add to spironolactone.

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102
Q

If lithium is the. Cause of DI, what do you treat. It with

A

Amiloride

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103
Q

What are planted antigens

A

Cationic molecules that bind to an ionic components of the glomerulus, DNA, nucleosides, bacterial products, large aggregated proteins

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104
Q

What is an example of a planted antigen causing membranous nephropathy

A

Infants fed cow milk

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105
Q

What pattern does anti-GBM antibody induced glomerulonephritis cause on imunofluorescence

A

Linear

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106
Q

What is goodpasture syndrome

A

When anti-GBM abs cross react with lung alveoli

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107
Q

What is the GBM antigen responsible for classic anti-GBM ab induced glomerulonephritis and goodpasture syndrome

A

NC1 and alpha 3 chain of type IV collagen

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108
Q

What microbial antigens have beeen implicated in glomerulonephritis resulting from deposition of circulating immune complexes

A

Strep, surface antigen of hep B, C, and trep pallium, plasmodium falciparum

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109
Q

What do immune complex formation glomerulonephritis look like on immunofluorescence

A

Granular

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110
Q

What affects the glomerular localization of the antigen, ab or immune complexes

A

-molecular charge and size of reactants: highly cationic antigens tend to cross GBM and reside in subepithelial location; anionic -> subendothelialy and or NOT nephritogenic at all; neutral charge and immune complexes accumulate in mesangium; large immune complexes not nephritogenic because cleared by macrophages and do not enter GBM

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111
Q

What does the location of the ab, antigen, complexes usually indicate about the pathology

A
  • in subendothelial and mesangial -> inflammatory pathology

- subepithelial: non-inflammatory

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112
Q

What are epimembranous deposits indicative of

A

Membranous nephropathy and heymann nephritis

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113
Q

What conditions would you see subendothelial deposits in

A

SLE and membranoproliferative glomerulonephritis

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114
Q

Where would you see deposits with IgA nephropathy

A

Mesangial

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115
Q

When does activation of the alternative complement pathway occur

A

In dense-deposit dz (membranoproliferative glomerulonephritis - MPGN type II) and in C3 glomerulopathies

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116
Q

What are the activities of neutrophils in glomerular injury

A

Release proteases which cause GBM degradation, ROS, and arachnids is acid which contribute to reductions in GFR

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117
Q

What is the role of resident glomerular cells in inflammatory reactions

A

Cause be stimulated to produce ROS, cytokines, chemokines, growth factors, eicosanoids, NO, and endothelin

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118
Q

What makes up the membrane attack complex and what is its function in kidney inflammatory reaction

A

C5b-C9 -> cell lysis. And simulates mesangial cell to produce oxidants, proteases *even in the absence of neutrophils, C5b-9 can cause proteinuria

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119
Q

What is podocytopathy

A

Injury to podocytes (can be caused by HIV)

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120
Q

What diseases can cause loss of podocytes

A

Focal and segmental glomerulosclerosis and diabetic nephropathy

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121
Q

What is normally the key event in causing proteinuria

A

Injury to slit diaphragms (mediated injury or mutation in podocin or nephrin)

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122
Q

What does the GFR have to be reduced to for progression to ESRD to inevitably occur

A

30-50% of normal

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123
Q

What is focal segmental glomerulosclerosis

A

Progressive fibrosis involving portions of some glomeruli develops after many types of renal injury and leads to proteinuria and increasing impairment; can occur even when primary dz was nonglomerular; initiated by adaptive change of the glomeruli; compensatory hypertrophy of remaining glomeruli can maintain function but proteinuria an segmental glomerulosclerosis soon develops; assoc w/ hemodynamic changes (increase in blood flow, filtration and transcap pressure)

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124
Q

What is used to treat progression of glomerulosclerosis

A

Inhibits of renin-angiotensin system

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125
Q

What is tubulointersitial fibrosis

A

*better correlation with decline in renal function with extent of tubulointerstitial damage than with severity of glomerular injury ; causes of tubulointersitital injury - ischemia of tubule segments downstream from sclerotic glomeruli, damage of peritubular cap blood supply, *proteinuria can cause direct injury to and activation of tubular cells which express adhesion molecules and elaborate cytokines that lead to fibrosis

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126
Q

What is nephritic syndrome

A

Glomerular dz characterized by inflammation in glomeruli; *usually presents with hematuria, red cell casts in urine, azotemia, oliguria, and mild-moderate HTN (can have proteinuria and edema but more severe in nephrotic syndrome); can occur with SLE and microscopic polyangiitis but typically characteristic of acute proliferative and exudative glomerulonephritis and crescentic glomerulonephritis *

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127
Q

What is acute proliferative (postinfectious) glomerulonephritis

A

Cluster of dz characterized by diffuse proliferation of glomerular cells associated with influx (exudate) of leukocytes; typically caused by immune complexes

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128
Q

What is post strep glomerulonephritis

A

Occurs after strep infection of pharynx or skin (impetigo); only strep A (most common types 12,4,1); serum complement levels low; granular immune complexes in glomeruli; antigen responsible is SpeB (pyogenic exotoxin); *hump like deposits in subendothelial locations that can dissociate, migrate across GBM and reform on subepithelial side

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129
Q

What is the most frequent presentation of postinfectious glomerulonephritis

A

Nephritic syndrome

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130
Q

What does postinfectious look like on fluorescence

A

Granular IgG and C3 in GBM and mesangium; granular IgA in some cases

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131
Q

What is the most frequent clinical presentation of goodpasture syndrome

A

Rapidly progressive glomerulonephritis

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132
Q

What is the pathogenesis of goodpasture

A

anti-GBM COL4-A3

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133
Q

What is seen on light and fluorescent microscopy for goodpasture

A

Light: extracapillary proliferation with crescents; necrosis
FLuorescence: linear IgG and C3; fibrin in crescents

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134
Q

What is seen on light microscopy for chronic glomerulonephritis

A

Hyalinized glomeruli

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135
Q

What is the most frequent presentation of membranous nephropathy

A

Nephrotic syndrome

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136
Q

What is seen on light microscopy for membranous nephropathy

A

Diffuse cap wall thickening

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137
Q

What is the most frequent presentation of minimal change dz

A

Nephrotic syndrome; unknown pathogenesis - podocytes injury

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138
Q

What is seen on light microscopy of minimal change dz

A

Normal; lipid in tubules

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139
Q

What is the most common presentation of focal segmental glomerulosclerosis

A

Nephrotic syndrome nonnephrotic proteinuria

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140
Q

what does dense-deposit dz (MPGN type II) present as

A

Hematuria; chronic renal failure; autoab -> alternative complement pathway

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141
Q

What does dense-deposit dz look like on fluorescence micro

A

C3 no C1q or C4

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142
Q

What does IgA nephropathy usually present as

A

Recurrent hematuria or proteinuria; unknown path

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143
Q

What does IgA nephropathy look like on light micro

A

Focal mesangial proliferative glomerulonephritis; mesangial widening

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144
Q

What is the morphology of post strep glomerulonephritis

A

Enlarges hypercellular glomeruli caused by neutrophil and monocytes recruitment, proliferation of mesangial and endothelial cells and crescent formation; involves all lobes of glomeruli

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145
Q

What is the clinical presentation of post strep glomerulonephritis

A

Hematuria post sore throat; periorbital edema and some HTN in kids; in adults its more likely to be atypical (sudden appearance of HTN or edema with elevation in BUN)

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146
Q

What is the tx for post strep glomerulonephritis

A

Maintaining sodium and water balance

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147
Q

What is assoc with an unfavorable prognosis of post strep glomerulonephritis

A

Heavy proteinuria with ab GFR

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148
Q

What other infections cause postinfectious glomerulonephritis

A

Staph endocarditis (sometimes produces IgA rather than IgG immune deposits), pneumococcal pneumonia, meningococcemia, hep B and C, mumps, HIV, varicella, mono, malaria, toxoplasmosis

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149
Q

What is rapidly progressive (crescentic) glomerulonephritis

A

Associated with severe glomerular injury but does not denote a specific etiology form; rapid loss of renal fxn with severe oliguria and signs of nephritis syndrome; death can occur within weeks to months if untreated; histo* crescents in glomeruli (proliferation of parietal epithelial cells lining Bowman’s)

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150
Q

What causes rapidly progressive glomerulonephritis (RPGN)

A

Immuno mediated

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151
Q

What types is RPGN broken down into

A
  • anti-GMB ab mediated dz; linear deposits of IgG and C3 in GBM; can cross react and cause goodpasture; plasmapheresis for tx; high prevalence of ppl affected have HLA-DRB1
  • immune complex deposition: postinfectious, lupus, IgA, and henoch-schonlein purpura; granular pattern; plasmapharesis doesn’t help
  • pauci-immune RPGN: lack of anti-GBM abs or immune complexes by fluorescence and EM; have circulating antineutrophil cytoplasmic ab (ANCA) and produce cytoplasmic or perinuclear staining pattern -> vasculitides; can be a part of wegener granulomatosis; most often idiopathic
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152
Q

What is the morphology of RPGN

A

Kidneys enlarged and pale with petechial hemorrhage; crescents; fibrin btw cellular layers in the crescents; EM: ruptures in GBM

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153
Q

What is the clinical manifestation of RPGN

A

Hematuria with RBC casts, moderate proteinuria, variable HTN and edema; in goodpasture, dominated by recurrent hemoptysis or pulmonary hemorrhage; many patients require dialysis or transplant over time

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154
Q

What is the path of nephrotic syndrome

A

Caused by derangement in glomerular cap walls resulting in increased permeability to plasma proteins -> massive proteinuria, hypoalbuminemia, generalized edema, and hyperlipidemia and lipiduria

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155
Q

What causes the edema in nephrotic syndrome

A

Reduced intravascualr colloid osmotic pressure; also retention of sodium and water due to compensatory secretion of aldosterone mediated by hpovolemia enhanced renin secretion and reduction in secretion of atrial peptides

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156
Q

What is a highly selective vs poorly selective proteinuria

A

Highly: low weight proteins (albumin, transferrin)
Poorly: higher weight globulins and albumin

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157
Q

How does the lipid appear in the urine of people with nephrotic syndrome

A

Free fat or oval fat bodies (lipoprotein resorted. By tubular epithelial cells and then shed along with injured tubular cells)

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158
Q

What are nephrotic patients vulnerable to

A

Infection especially staph and pneumococcal b/c loss of Igs in urine; thrombotic and thromboembolic complications also common (loss of anticoagulants); renal v thrombosis is consequence particularly in membranous nephropathy

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159
Q

What is nephrotic syndrome caused by in the US in diff ages

A

Younger than 17: primary kidney lesion

Adults:systemic dx

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160
Q

What are the most frequent systemic causes of nephrotic syndrome

A

Diabetes, amyloidosis, SLE; also caused by drugs(NSAIDs, penicillamine and heroin), infections, malignancy (carcinoma, lymnphoma) and bee sting allergies, hereditary nephritis

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161
Q

What are the most common primary lesions of the kidney that cause nephrotic syndrome

A

Minimal change dz (most common in kids), membranous glomerulopathy(most common in older adults), and focal segmental glomerulosclerosis

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162
Q

What are the most common causes of secondary membranous nephropathy

A
  • drugs: penicillamine, gold, NSAIDs (esp in RA patients)
  • malignant tumors (carcinomas of lug and colon and melanoma)
  • SLE
  • infections :hep b,c, syphilis, schisto, malaria
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163
Q

What antigen is implicated in neonatal membraneous nephropathy

A

Endopeptidase

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164
Q

What HLA is membranous nephropathy linked to

A

HLA-DQA1; caused by abs to renal autoantigen mostly phospholipase A2 receptor

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165
Q

What is the main Ig deposited in membranous nephropathy

A

IgG4

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166
Q

What do you see on silver stain with membranous nephropathy

A

Spikes from GBM

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167
Q

what is the major determinant of ECF osmolality

A

Sodium

168
Q

How can you estimate ECF osmolality

A

Doubling sodium concentration

169
Q

Differences in what pressure cause movement of fluid btw ICF and ECF

A

Only osmotic

170
Q

What is the formula for starling forces

A

Pc-Pi - osmotic cap - osmotic interstitial

171
Q

What happens when you add hypotonic NaCl to ECF

A

Osmolities of ECF and ICF lower and volume of both increased

172
Q

What happens when you add hypertonic NaCl to ECF

A

Osmolalities of ICF and ECF increase; volume of ECF increases by ICF decreases

173
Q

How do you calculate anion gap

A

Sodium - (chloride + bicarbonate)

174
Q

What is the effect of vasocontrictors (symp, angiotensin II, and endothelin on GFR)

A

Decreases

175
Q

What is the effect of vasodilators on the GFR

A
  • prostaglandins: no change/increase
  • NO: increase
  • bradykinin: increase
176
Q

What is the effect of ANP/BNP on GRF

A

Increases it; no change on RBF

177
Q

Where are the alpha 1 receptors in the kidney

A

Afferent arterioles

178
Q

What is the effect of sympathetic stimulation on the kidney

A

Decreases hydrostatic pressure and GFR decreases salt excretion

179
Q

How is renin stimulated

A

By beta receptor stimulation

180
Q

What is the most effected part of the kidney by sympathetic

A

Proximal tubule

181
Q

What signals do the kidneys receive in response to volume expansion

A

Decreases sympathetic, increased ANP BNP, inhibition of ADH, decreased renin and aldosterone
* increased excretion of sodium

182
Q

What factors are important for renin secretion

A

Perfusion pressure sympathetic n activity and delivery of NaCl to macula densa

183
Q

What are the functions of angiotensin II

A

Stimulation of aldosterone, arterial vasoconstriction, stimulates ADH and thirst, enhances NaCl reabsorption

184
Q

How does aldosterone work

A

Reduces NaCl excretion by stimulating its reabsorption by thick ascending loop, distal tubule and collecting duct; increases number of NaCl synporters and Na channels in principle cells in late distal tubule and collecting duct and Na/k pump on basolateral side , stimulates H secretion through H ATPase in apical membrane of alpha intercalated cells

185
Q

How is Na absorbed in first half of proximal tubule

A

With bicarbonate, in second half absorbed with Cl

186
Q

Which part of the loop of henle absorbs Na

A

Only ascending

187
Q

Which part of the loop of henle is impermeable to water

A

Ascending

188
Q

What does th early distal tubule reabsorb

A

Na Cl via NCC symporter and ca , impermeable to water * thiazides

189
Q

Does the proteinuria in membranous nephropathy respond well to corticosteroids

A

no

190
Q

what is minimal change dz

A

relatively benign; diffuse effacement of foot processes of visceral epithelial cells *detectable only by EM in glomeruli that appear normal via light micro; *most frequent cause of nephrotic syndrome in children

191
Q

People with what disease are at increased risk for minimal change dz

A

non-hodgkins lymphoma

192
Q

does the proteinuria of minimal change dz respond to corticosteroids

A

yes

193
Q

what are the clinical features of minimal change dz

A

renal fxn remains good; massive proteinuria; no HTN or hematuria; *characteristic is dramatic repsonse to corticosteroids

194
Q

what is primary focal segmental glomerulosclerosis

A

most common cause of nephrotic syndrome in adults; HTN and hematuria with proteinuria

195
Q

what are the classification and types of focal segmental glomerulosclerosis

A
  • primary dz
  • in association with HIV, heroin addiction, sickle cell or obesity
  • as a secondary event
  • component of adaptive response to loss of renal tissue (unilateral renal agensis or acquired causes)
  • uncommon inherited forms; mutations in genes encoding for slit diaphragm
196
Q

how does focal segmental glomerulosclerosis differ from minimal change disease

A

in FSGS - higher hematuria, reduced GFR and HTN; proteinuria is nonselective; poor response to corticosteroids; progression to kidney dz with ESRD

197
Q

what is the hallmark of the pathogenesis of FSGS

A

damage of epithlial cells; hyalinosis and sclerosis occur from entrapment of plasma proteins in hyperpermeable foci and increased ECM deposition

198
Q

what is the genetic basis for FSGS

A
  • NPHS1 on chrom 19q13 encodes nephrin
  • AR FSGS - caused by mutations in NPHS2 which encodes podocin *steroid resistant nephrotic syndrome of childhood onset
  • AD caused by mutation in alpha actinin 4 (encodes podocyte binding protein)
  • TRPC6 -> increases calcium influx into podocytes
199
Q

People of african descent with what mutation are at risk for FSGS

A

APOL1

200
Q

what is collapsing glomerulopathy

A

variant of FSGS; characterized by collapse of entire glomerular tuft with or w/o FSGS lesions; characteristic feature is proliferation and hypertrophy of glomerular visceral epithilal cells *most characteristic lesion of HIV nephropathy = poor prognosis (more common in blacks)

201
Q

what are the morphologic features of HIV associated nephropathy

A

collapsing variant of FSGS; striking focal cystic dilation of tubule segments filled with proteins; tubuloreticular inclusions within endothelial cells (EM) -> modification of ER induced by circulating IFNa

202
Q

what is membranoproliferative glomerulonephritis type I and II

A

type I: deposition of immune complexes containing IgG and complement
type II: dense deposit dz; activation of complement is most impt factor; belongs to group of disorders called C3 glomerulopathies

203
Q

what are membranoproliferative glomerulonephritis also called

A

mesangiocapillary glomeruloneprhtiis (because proflieration is mostly in mesangium)

204
Q

what is the morphology of MPGN

A

glomeruli are large and hypercellular; glomeruli have lobular appearacne due to proflierating mesangial cells and increased mesangial matrix; GBM is thickened and shows double contour or tram track appearance (in silver or PAS stains) caused by duplication of BM
*type I characterized by subendothelial electron dense deposits IgG and C3 deposited in granular pattern

205
Q

what are the clinical features of MPGN

A

most present with nephrotic syndrome and a component of nephritic syndrome (hematuria); not improved by any treatment

206
Q

how do you calculate interstitial fluid levels

A

ECF-plasma

207
Q

what are normal sodium levels

A

136-146

208
Q

in what type of patient would you be careful administering NSAIDS in terms of kidney

A

HTN, renal steonsis, patient on diuretics; NSAIDS will inhibit prostaglandin production

209
Q

what does vasodilation of the afferent artieriole cause

A

increased RBF, GFR and hydrostatic pressure; caused by prostaglandins, bradykinin, NO, dopamine, ANP

210
Q

what does vasoconstriction of the afferent arteriole cause

A

decrease in RBF, GFR, and hydrostatic pressure

211
Q

what does vasodilation of the efferent arteriole cause

A

increase in RBF, decrease in GFR, increase in hydrostatic pressure

212
Q

what does vasoconstriction of the efferent arteriole cause

A

decreased RBF, increased GFR, decreased hydrostatic pressure

213
Q

what leads to renin secretion

A

symp stimulation, decreased NaCl to macula densa, vasoconstriction of afferent arteriole

214
Q

what inhibits renin

A

angiotensin II and ADH

215
Q

what does ang II do

A

increase thirst, increase Na reabsorption in PT; increase ADH and aldosterone

216
Q

what do principle cells do

A

sodium and water reabsorption and potassium secretion

217
Q

which hormones regulate water permeability

A

ADH ANP BNP in late distal tubule and collecting duct

218
Q

what causes a decrease in ADH

A

ethanol

219
Q

what causes secondary MPGN

A

SLE, hep B and C; endocarditis; HIV; schisto; alpha antitrypsin deficiency; malignant dz; Type I

220
Q

what causes dense deposit dz

A

excess activation of alternative complement pathway; decreased C3 but normal C1 and 4; low factor B and properdin (alternative pathway); C3 and properdin deposited in glomeruli but no IgG; C3 nephritic factor (C3NeF) binds C3 convertase and protects it from inactivation

221
Q

what is the morphology of dense deposit dz

A

characterstic on EM -> permeation of lamina densa of GBM by ribbon-like electron dense material; C3 not in dense deposits, but in BM and mesangium (mesangial rings)

222
Q

what are the clinical features of dense deposit disease

A

primarily affects kids and young adults; mixed nephritic and nephrotic; poor prognosis; recurrence in transplant

223
Q

what is IgA nephropathy (berger dz)

A

presence of promiment IgA deposits in mesangial regions; recurrent hematuria *most common type of glomerulonephritis worldwide; normally isolated dz but can present in a systemic disorder of children (henoch schnolein purpura); secondary IgA neprhopathy in liver nad intestinal dz

224
Q

what is the pathogenesis of IgA nephropathy

A

defect in normal formation or attachment of galactose-containing sugar chains called O-linked glycans to the hinge region of IgA -> aberrently glycosylated IgA1 deposited in glomeruli or forms comlexes with IgG abs and deposit in mesangium; can activate complement via alternative pathway (C3 no C1q or C4)

225
Q

what diseases are associated with an increased risk of IgA nephropathy

A

Celiac, liver dz (defective clearance of IgA complexes)

226
Q

what is the classic immunofluorescent picture of IgA nephropathy

A

IgA in mesangium

227
Q

what are the clinical features of IgA nephropathy

A

hematuria lasting for several days and then returns; slow progression to renal failure (increased risk of progression -> proteinuria, HTRN and extent of glomerulosclerosis); recurrence in transplant

228
Q

what is hereditary nephritis

A

group of familial renal diseases assoc with mutations in collagen genes that manifest primarily with glomerular injury *alport syndrome and thin basement membrane lesion (benign familial hematuria)

229
Q

what is alport syndrome

A

hematuria with progression to chronic renal failure accompanied by nerve deafness and various eye disorders (lens dislocation, posterior cataracts and corneal dystrophy) *X-linked (males- fully syndrome; femalse - hematuria) but also AR

230
Q

what is the pathogenesis of alport syndrome

A

mutations in collagen IV; mutations in collagen IV alpha 5 chain (COL4A5) assoc with ESRD at earlier age

231
Q

what is the morphology of alport syndrome

A

EM - irregular foci of thickening alternating with thinning and splitting and lamination of lamina densa = prodcues basket weave appearance

232
Q

what are the clinical features of alport syndrome

A

gross or microscopic hematuria; proteinuria can develop later

233
Q

what is thin basement membrane lesion or benign familial hematuria

A

common; manifested by asymptomatic hematuria; diffuse thinning of GBM; excellent prognosis; caused by mutations in alpha 3 or 4 chains of collagen IV; AD or AR

234
Q

does chronic glomerulonephritis have to come from an acute phase

A

no

235
Q

what is the morphology of chronic glomerulonephritis

A

kidneys are symmetrically contracted and have granular cortical surfaces; cortex is thinned and incrase in peripelvic fat

236
Q

what is henoch schonlein purpura

A

childhood syndrome consisting of pupuric skin lesions (extensor surfaces and butt), ab pain and intestinal bleeding and arthralgias with renal abnormalities (hematuria, nephritic/nephrotic syndrome); onset often follows resp infection; excellent prognsosis

237
Q

what is the pathognomoinc feature of henoch schonlein purpura

A

deposition of IgA and sometimes IgG and C3 in mesangial region

238
Q

what is fibrillary glomerulonephritis

A

fibrillar deposits in mesangium and capillary walls that resemble amyloid but do not stain with congo red; deposition of IgG (4) C3 and Igk and Iglambda light chains; nephrotic syndrome, hematuria and progressive renal insufficiency

239
Q

what are goodpasture, microscopic polyagiitis, and granulomatosis with polyangiitis all characterized by

A

foci of glomerular necrosis and crescent formation

240
Q

what is essential mixed cryoglombulinemia

A

IgG-IgM complexes induce cutaneous vasculitis, synovitis adn proliferative glomerulonephrtiis (MPGN type I) (assoc with Hep C)

241
Q

what causes acute tubular injury

A

ischemia (ischemic ATI) or direct toxic injury to tubules (neprhotoxic ATI) or combo - mismatched blood transfusions and other hemolytic crises causing hemoglobinuria and skeletal m injuries causing myoglobinuria

242
Q

what are the results of tubule cell injury

A

increased sodium delivery to distal tubules which incites vasoconstriction via tubuloglomerular feedback; injured cells detach fromm and cause luminal obstruction, increased intratubular pressure, and decreased GFR; can also cause intersitital edema, increased intersistial pressure

243
Q

which portions of the kidney are extremely vulnerable to ischemic and toxic injury

A

straight portion of proximal tubule and thick ascending limb in renal medulla

244
Q

what is the difference in morphology of ATI caused by mercuric chloride and carbon tetrachloride and ethylene glycol

A

mercuric: injured cells contain acidophilic inclusions; later become necrotic, are desquamated at the lumen and undergo calcification
carbon tetrachloride: accumulation of neutral lipids in injured cells
ethylene glycol: ballooning and hydropic or vacuolar degeneration of proximal convoluted tubules; calcium oxalate crystals form in tubule lumens

245
Q

what are that patterns of injury in ischemic and toxic ATI

A

ischemic: patchy necrosis with short lengths damaged; straight proximal tubule and ascending lim most vulnerable
Toxic: extensive necrosis along proximal convoluted tubules
*both types: lumens of distal convoluted tubule and collecting duct contains casts

246
Q

what are the phases of ATI

A
  • initiation: about 36 hrs; slight decline in urine output with rise in BUN
  • Maintenance: sustained decrease in urine output, salt and water overload, rising BUN, hyperkalemia, met acidosis, can overcome it
  • Recovery: increase in urine volume; hypokalemia becomes a problem; increased vulnerability to infection
247
Q

what are tubulointerstitial nephritis

A

involves inflammatory injury to tubules and interstitium and manifested by azotemia

248
Q

what are some causes of tubulointersitial nephritis

A

-acute/chronic bacterial pyelonephritis, acute hypersensitivity, analgesics, heavy metals, lead, cadmium, urate nephropathy, nephrocalcinosis, acute phosphate nephropathy, hypokalemic, oxalate neph, chronic UTI, mutliple myeloma, sjogren, sarcoidsosis, balkan nephropathy, nephronophthisis medullary cystic dz complex

249
Q

what are useful markers in distinguishing acute from chronic tubulointerstitial nephritis

A

edema , eosinophils and neutrophils in acute form; fibrosis and tubular atrophy in chronic

250
Q

how can tubulointerstitial diseases be distinguished from glomerular disease

A

-absence of nephritic or nephrotic syndrome; presence of defects in tubular function (can’t concentrate urine, metabolic acidosis, etc)

251
Q

what is pyelonephritis

A

inflammation affecting the tubules, interstitium, and renal pelvis; acute (usually UTI) and chronic(more complex)

252
Q

when is hematogenous spread of infection to the kidneys more likely to occur

A

in presence of ureteral obstruction and in debilitated patients

253
Q

how do microbes move from the bladder to the kidneys

A
  • urinary tract obstruction: can’t flush out microbes; ie: BPH, tumors, stones, or neurogenic gladder dysfunction caused by DM or SC injury
  • vesicoureteral reflux: incompetence of vesicoureteral valve
  • intrarenal reflux: most common in upper and lower poles of kidney where papillae have flattened or concave tips; demonstrated by voiding cystourethrogram
254
Q

what is acute pyelonephritis

A

suppurative inflammation of the kidney caused by bacterial or viral (polyomavirus) *histo: intratubular aggregates of neutrophils, tubulitis and tubular necrosis; starts in tubules and can extend to intersititum where it produces abscesses that destroy the tubules; extensive dz can affect glomeruli (esp fungal)

255
Q

what are the 3 complications of acute pyelonephritis

A
  • papillary necrosis: seen mainly in diabetes, sickle cell, and urinary tract obstruction; tips of pyramids have areas of gray-white to yellow necrosis (coagulative)
  • pyonephrosis: seen when there is total obstruction; suppurative exudate unable to drain and fils renal pelvis, calcyes and ureter with pus
  • perinephric abscess: extension of suppurative inflammation through renal capsule into perinephric tissue
256
Q

what does a pyelonephritic scar suggest

A

ascending infection and vesicoureteral reflux as the pathogenesis of the dz

257
Q

Who are the most common people to get acute pyelonephritis

A

catheterized, pregnant women, first year of life (males), females, preexisting renal lesions, DM, immunosuppression

258
Q

The presence of what on urinalysis suggests renal involement

A

leukocyte casts

259
Q

what is polyomavirus nephropathy

A

latent infection activated upon immune suppression; chracterized by infection of tubular epithelial cells leading to nuclear enlargmenet and intranuclear inclusions composed of virions in crystalline like lattices on EM; reduce immunosuppression to treat

260
Q

what is chronic pyelonephritis

A

disorder in which chronic inflammation and scarring involve the calyces and pelvis only one that does this(

261
Q

What are the two forms of chronic pyelonephritis

A
  • reflux nephropathy: more common form; occurs early in childhood as a result of superimposition of urinary infection on congenital vesicoureteral reflux and intrarenal reflux
  • chronic obstructive pyelonephritis: recurrent infections on obstructive lesions lead to repeated bouts of renal inflammation and scarring
262
Q

what is the morphology of chronic pyelonpehritis

A

irregularly scarred; if b/l, asymmetric (in glomerulonephritis -> symmetric); coarse discrete corticomedullary scars overlying dilated, blunted, or deformed calcyces and flattened papillae

263
Q

what is xanthogranulomatous pyelonephritis

A

rare form of chronic pyelonephritis; accumulation of foamy macrophages intermingled with plasma cells, lymphocytes, PMN, and occassional giant cells; associated with proteus infections and obstruction; produce large yellowish orange nodules that can be confused with RCC

264
Q

what is a cause of renal HTN in kids

A

reflux nephropathy

265
Q

what can some people with pyelonephritic scars develop

A

secondary FSGS; poor prognosis

266
Q

what drugs most commonly cause acute drug-induced interstitial nephritis

A

methicillin, ampicillin, rifampin, diuretics, NSAIDs and alopurinol and cimetidine

267
Q

what are the clincial features of drug induced interstitial nephritis

A

fever, eosinophilia, rash, and renal abnormalities (hematuria, mild proteinuria, leukocyturia including eosinophils)

268
Q

what is the pathogensis of drug induced nephritis

A

hypersensitivity; not dose related; IgE mediated or T cell mediated Type IV reaction (delayed hypersensitivity); drugs function as haptens and bind plasma membrane of tubular cells

269
Q

how do you calculate clearance

A

urine concentration * urine flow rate/ plasma concentration

270
Q

what is the affect of hypoalbuminemia on serum calcium

A

decreases it without affecting the ionized calcium level; if serum albumin normal, clinical decisions based on ionized calcium levels

271
Q

what is the calculation for corrected calcium

A

total calcium + .08 (4 - serum albumin)

272
Q

what are the effects of calcitonin

A

lowers blood calcium by inhibiting calcium absorption in intestines, inhibiting osteoclast activity, and inhibiting renal tubular cell reabsorption of caclium; also inhibits phosphate reabsorption (like PTH)

273
Q

what are the effects of PTH on the kidney

A

acts in distal nephron to increase calcium reabsorption and inhibits phosphate reabsorption in proximal tubule

274
Q

what stimulates the formation of vitamin D3 (calcitriol) in kidney

A

PTH

275
Q

where is most of the calcium reabsorbed in the kidney

A

proximal tubule

276
Q

what are some causes of hypercalcemia

A

primary hyperparathyroidism, malignancy, thiazide diuretics, milk-alkali syndrome, immobilization syndrome, granulomatous dz, familial hypocalciuric hypercalcemia; vitamin D intoxication

277
Q

how do you manage acute hypercalcemia

A
  • ECF volume replacement with .9% saline
  • furosemide
  • if does not respond to saline diuresis especially if secondary to malignancy -> *bisphosphonates
  • calcitonin
  • glucocorticoids
  • hemodialysis
278
Q

what is true hpocalcemia and what are some causes

A

only when ionized calcium reduced; hypoPTH; vit D defi; chronic kidney dz; familial hypocalcemia, pseudohypoparathyroidism; rhabdo; acute pancreaitits; septic shock

279
Q

how do you manage hypocalcemia

A
  • in emergency situation (seizures, tetany, hypotension, arrhythmias): IV calcium
  • chronic, mild: oral calcium supplements +/- vit D
  • hypoparathyroidism: calcium and vit D supplements
280
Q

what does FGF-23 do to phosphorous

A

increases renal excretion

281
Q

what effect does vit D3 have on phosphorous

A

increases intestinal absorption

282
Q

what effect does insulin have on phosphorous

A

shifts phosphate into cells

283
Q

where is most of the phosporous reabsorbed

A

proximal convoluted tubule; sodium dependent transporters; regulated by FGF-23 and PTH

284
Q

what are the sx of hyperphosphatemia

A

signs of hypocalcemia; tissue ischemia or calciphylaxis (vascular calcification or necrosis); renal osteodystrophy

285
Q

how do you manage hyperphosphatemia

A

acute: saline diuresis

in ESRD: reduce dietery intake/intestinal absorption via phosphate binders

286
Q

how do you treat renal osteodystrophy

A

caused by hyperparathyroidism secondary to hyperphosphatemia combined with hypocalcemia; treat with calcium and vit D supplements

287
Q

what is refeeding hypophosphatemia

A

cause of death in starving people/anorexics as hexokinase phosphorylates glucose taken into cells

288
Q

how do you manage hypophosphatemia

A
  • moderate: no therapy
  • persisitent: oral phosphate
  • severe (<1 mg/dL): IV phosphate
  • frequently also hypokalemic and hypomagnesemic so must also be corrected
289
Q

where is magnesium found

A

ICF

290
Q

where is most of the magnesium absorbed

A

ascending limb driven by NK2Cl transporter

291
Q

what kind of patients do you see hypomagnesemia in

A

ICU; result of: nutrition, diuretics, albumin, aminoglycosides, PPI

292
Q

what are the sx of hypomagnesemia

A

weakness, tremor, seizures, vertical and horizontal nystagmus; nonspecific T wave changes; prolonged Qt interval;; premature ventricular contractions; torsade de pointes; v fib; enhanced digitialis toxicity; hypokalemia and hypocalcemia

293
Q

how do you treat hypomagnesemia

A

oral or IV replacment

294
Q

how do you get hypermagnesemia

A

ESRD, massive intake (epsom salt), magnesium infusion (given for pre-eclampsia/eclampsia)

295
Q

what are the sx of hypermagnesemia

A

if less than 3.6 - asymptomatic
if 4.8-7.2: nausea, flushing, HA, lethargy; diminished DTR
if 7.2-12: somnolence, hypocalcemia, absent DTR, hypotension, bradycardia, ECG changes
if > 12: muscle paralysis; flacid quadriplegia, apnea, resp failure, complete heart block and cardiac arrest

296
Q

how do you treat hypermagnesemia

A
  • if normal renal function: stop administration and add loop or thiazide diuretic
  • reduced renal function: as above but add saline infusion
  • ESRD: dialysis
297
Q

what do a small percentage of patients with analgesic nephropathy develop

A

urethelial carcinoma of the renal pelvis

298
Q

what are the NSAID-associated renal syndromes

A

acute kidney injury (ischemia), acute hypersensitivity interstitial nephritis, acute intersitital neprhtisis and minimal change dz, membraneous nephropathy

299
Q

what is acute uric acid nephropathy

A

caused by precipiation of uric acid crystals in renal tubules leading to obstruction of nephrons and acute renal failure; more likely in ppl with leukemias or lymphomas who are undergoing chemo (drugs kill tumors which produce uric acid)

300
Q

what is chronic urate nephropathy

A

gouty nephropathy;occurs in patients with hyperuricemia; deposit in distal tubules and collecting ducts; form needle-like crystals; evoke a monocyte response called a tophus

301
Q

which disorders are associated with hypercalcemia

A

hyperPTH, multiple myeloma, vit D intoxication, met cancer, excess calcium intake

302
Q

what are the causes of papillary necrosis

A

DM, analgesic nephropathy, sickle cell, obstruction (more in males)

303
Q

when can accumulations of calcium phosphate occur in the kidney

A

people prepping for colonoscopy

304
Q

how does multiple myeloma cause kidney damage

A
  • bence jones proteinuria and cast nephropathy: Ig light chains directly toxic to epithelial cells; also combine with urinary glycoprotein (tamm-horsfall protein) under acidic conditions to form large tubular casts that obstruct the lumen
  • amyloidosis of AL type formed from free light chains (usually lambda)
  • light chain deposition disease: light chains (kappa) deposit in GBM and mesangium causing glomerulopathy
  • hypercalcemia and hyperuricemia
305
Q

what is suggestive of bence jones proteinuria

A

light chain proteinuria

306
Q

what is bile cast nephropathy

A

caused by hepatorenal syndrome; bile casts form in distal nephron; yellowish-green to pink; reversibility depends on severity of liver dz

307
Q

what is the morphology of nephrosclerosis

A

kidneys are either normal or moderately reduced in size; cortical surface has granularity; loss of mass due to cortical scarring and shrinking; histo: narrowing of lumen caused by hyalinization; fibroelastic hyperplasia; patchy ischemic atrophy (foci of tubular atrophy and interstitial fibrosis and glomerular alterations)

308
Q

what people with nephrosclerosis are at increased risk for developing renal failure

A

AA, severe blood pressure elevation, ppl with a second underlying dz (esp DM)

309
Q

what is the pathogenesis of malignant nephrosclerosis

A

vascular injury -> fibrinoid necrosis -> activation of coagulation factors; platelets cause hyperplasia of smooth m of vessels *markedly elevated levels of plasma renin

310
Q

What is the morphology of malignant nephrosclerosis

A

petechial hemorrhages (flea bitten appearance); onion skinning of interlobular arteries (also called hyperplastic arteriolitis) - correlates with renal failure

311
Q

what are the qualifications for malignant HTN

A
  • systolic >200 diastolic >120
  • papilledema
  • retinal hemorrhages
  • encephalopathy
  • CV abnormalities
  • renal failure
312
Q

Is fibromuscular dysplasia more common in men or women

A

women in younger age groups

313
Q

what is the morphology of renal artery stenosis

A

reduced in size and shows diffuse ischemic atrophy; no arteriosclerosis except maybe in contralateral kidney

314
Q

what are the thrombotic microangiopathies

A

thrombotic thrombocytopenic purpura and hemolytic uremic syndrome

315
Q

what is the outcome of thrombotic microangiopathies

A

excessive activation of platelets which deposit in capillaries; consumption of platelets leads to thrombocytopenia and the resulting thrombi create flow ab that shear red cells producing hemolytic anemia

316
Q

what is typical vs atypical HUS

A

typical: associated with consumption of food contaminated with EHEC
atypical: inherited mutations of complement regulatory proteins; acquired causes of endothelial injury (antiphospholipid ab, complications of pregnancy or OCP, scleroderma, HTN, chemo, radiation)

317
Q

what mutation is TTP associated with

A

ADAMTS13 (metalloprotease that regulates vWF)

318
Q

what is the initiating event in HUS vs TTP

A

HUS: endothelial injury
TTP: platelet aggregation

319
Q

who does atypical HUS most often affect

A

adults; most commonly deficient in factor H (breaks down C3 convertase); Factor I and CD46 also mutations seen;

320
Q

what other conditions does atypical HUS arise with

A
  • antiphospholipid ab syndrome: follows a chronic course
  • pregnancy: grave prognosis
  • systemic sclerosis or HTN
  • chemo
  • radiation
321
Q

Is there a better outcome with typical or atypical HUS

A

typical

322
Q

what are the manifestations of TTP

A

pentad: fever, neuro sx, microangiopathic hemolytic anemia, thrombocytopenia, renal failure; seen more in women younger than 40 *CNS involvment is dominant feature *treat with plasmapharesis

323
Q

what do cholesterol crystals look like

A

rhomboid clefts

324
Q

what are the most common abnormalities seen with sickle cell nephropathy

A

hematuria and diminished concentrating ability (hyposthenuria); the hyperosmolarity dehydrates red cells and increases teh concentration of sickled cells (reason why even those with sickle trait are affected)

325
Q

what can happen as a result of ACE inhibitors

A

decrease in GFR which will increase serum creatinine; preserves kidney function; <30% increase ok if no hyperkalemia

326
Q

what are sx of hypervolemia

A

weight gain, edema, bounding pulse

327
Q

what can cause hypovolemia

A

hypoaldosteronism, adrenal insufficiency, heart failure, cirrhosis, anaphylaxis, sepsis, pregnancy, third spacing, neprhotic syndrome

328
Q

how do you calculae plasma osmolality

A

2*Na plasma + glucose plasma/18 + BUN plasma /2.8

329
Q

what is normal plasma osmolality

A

285-295

330
Q

which drugs cause increase vs decrease in ADH

A

increase: morphine, nicotine, cyclophosphamide
decrease: alcohol, clonidine, haloperidol

331
Q

what are the sx of hyponatremia

A

SALT LOSS mnemonic

Stupor/coma, anorexia, lethargy, tendon reflexes decreased; limp mm; orthostatic hypotension, seizures, stomach cramping

332
Q

how do you treat hyponatremia

A
  • severe sx: hypertonic NaCl followed by fluid restirction or vaptan
  • Moderate sx: vaptan or hypertonic NaCl followed by fluid restriction
  • no or min sx: fluid restriction
333
Q

what happens to the brain with hyponatremia

A

acute: increased water
chronic: decreased water

334
Q

how do you calculate the amount of 3% NaCl to give to a hyponatremic patient

A

patient weight in kg * desired correction rate

335
Q

what are the sx of hypernatremia

A 
TRIP
Twitching, tremors, hyperreflexia
Restlessness, irritable, confusion
Intense thirst dry mouht, decreases urine output
Pulm and peripheral edema
336
Q

how do you tx hypernatremia

A
  • if hypovolemic: isotonic saline
  • in other patients, use hypotonic IV solutions
  • correct over 48 hrs at .5 mEq/L/hr
337
Q

how do you calculate water deficit

A

.6 *body weight in kg (1-140/sodium)

338
Q

what is the effect of hypokalemia on the heart

A

tachycardia

339
Q

what is the effect of hyperkalemia on ECG

A

high T waves

340
Q

what helps internalize potassium

A

insulin, beta 2 agonist, aldosterone deficiency, alpha blockers, alkalosis, hypoosomolarity

341
Q

what are the causes of hypokalemia

A

GRAPHIC IDEA

GI losses, renal tubular acidosis, aldosterone,paralysis, hypothermia, insulin excess, cushing

342
Q

what are the sx of hypokalemia

A

skeletal m and smooth m weakness (ileus and constipation); hypotension, arrhythmias; polyuria

343
Q

what are causes of hyperkalemia

A

Renal dz, excessive intake, drugs, factitious (hemolysis), addisons, tissue release, acidosis, beta antagonists, insulin def

344
Q

what are the sx of hyperkalemia

A

-bradycardia, peaked T waves, flattened P wave, prolonged P-Q interval, widened QRS, sine wav; weakness

345
Q

what drugs are given for hyperkalemia

A

renin blockers (NSAIDS, aliskiren), ACEI, aldosterone inhibitors (spironolactone), amiloride, triamterene, trimethroprim

346
Q

how do you tx hyperkalemia

A
  • antagonize cardiac affects - IV calcium
  • redistribute K into cells: insulin and glucose OR beta agonist OR bicarb (but generally not recommended)
  • facilitate K elimination: K losing diuretic, mineralocorticoid, cation exchange resin, dialysis
347
Q

when does diffuse cortical necrosis usually occur

A

after obstetric emergency (abruptio placentae), septic shock or extensive surgery

348
Q

what do truly hypoplastic kidneys show

A

no scars and reduced number of renal lobes and pyramids

349
Q

what are the classifications of renal cysts

A
  • polycystic kidney dz (ar and ad)
  • medullary cystic dz (medullary sponge kidney and nephronophthisis)
  • multicystic renal dysplasia
  • acquired (dialysis) cystic dz
  • localized renal cysts
  • renal cysts in hereditary malformation
  • glomerulocystic dz
  • extraparenchymal renal cysts
350
Q

what are the features of AD polycystic kidney dz

A

large multicystic kidneys, liver cysts, berry aneurysms, mitral valve prolapse; hematuria, flank pain, UTI, renal stones, HTN; renal failure at 40-60 y/o

351
Q

what are the features of AR polycystic kidney dz

A

enlarged, cystic kidneys at birth; hepatic fibrosis is a complication; death in infancy or childhood

352
Q

what are the features of medullary sponge kidney

A

medullary cysts on excretory urography; hematuria, UTI, recurrent renal stones; benign dz

353
Q

what are the features of familila juvenile nephronophthisis

A

AR; corticomedullary cysts; shrunken kidneys; *salt wasting, polyuria, growth retardation, anemia; progressive renalfailure begining in childhood

354
Q

what are the features of adult onset medullary cystic dz

A

AD; corticomedullary cysts and shrunken kidneys; salt wasting, polyuria; chronic renal failure beginning in adulthood

355
Q

what are the features of multicystic renal dysplasia

A

not inherited; irregular kidneys with cysts of variable size; renal failure if b/l; curable if unilateral

356
Q

what are teh features of acquired renal cystic dz

A

cystic degeneration in ESRD; hemorrhage, erythrocytosis, neoplasia are complications; dependence on dialysis

357
Q

what are the features of simple cysts

A

not inhertied; single or multi cysts in normal sized kidneys; microscopic hematuria; benign

358
Q

what is the pathogenesis of AD polycystic kidney dz

A

mutations in PKD1 and 2; PKD1 encodes polycystin 1 expressed in tubular epithielial cells *most common mutation; PKD2 (polycystin 2) Ca cation channel; less severe than PKD1 mutations
*thought to be defect in cilia sensing calcium which causes a defect in intracellular calcium

359
Q

in whom is progression of polycystic kidney dz accelerated in

A

blacks (esp sickle cell), males, and in the presence of HTN

360
Q

what do patients with APKD die of

A

coronary or HTN heart dz, infection, ruptured berry aneurysm

361
Q

what is the pathogensis of AR polycystic KD

A

mutations in PKHD1 (expressed in kidney, liver and pancreas) - encodes for fibrocystin - localized to cilium of tubular cells

362
Q

what are the types of medullary cystic dz

A

medullary sponge kidney, nephronophthisis and adult onset medullary cystic dz

363
Q

what is the cause of renal insufficiency in nephronophthsis and adult onset medullary cystic dz

A

cortical tubulointerstitial damage

364
Q

what are the variants of nephronophthisis

A

sporadic, famililal juvenile (most common), and renal-retinal dysplasia
*most common genetic cause of ESRD in children and young adults

365
Q

what do ppl with nephronophthisis present with first

A

polyuria and polydipsia; tubular acidosis and sodium wasting; some syndromic forms can have extrarenal manifestations (ie: joubert syndrome)

366
Q

what is the pathogensis of juvenile nephronophthisis

A

mutations in NPHP1-11; (encode nephrocystins)NPH2 encodes inversion (L-R patterning)

367
Q

what mutations are seen in adult onset medullary cystic dz

A

MCKD1/2

368
Q

what is the morphology of nephronopthsis

A

kidneys are small, have granular surfaces and cysts in medulla

369
Q

what is the characteristic histo of multicystic renal dysplasia

A

islands of undifferentiatedd mesenchyme, often with cartilage, and immature colecting ducts

370
Q

what is multicystic renal dysplasia often associated with

A

ureteropelvic obstruction, ureteral agenesis or atresia, and anomalies of lower urinar ytract

371
Q

what are people with acquired cystic dz at risk for

A

RCC

372
Q

how can you tell a cyst from a tumor

A

cysts are smooth, avascular, and give fluid rather than solid signals on US

373
Q

what are the causes of urinary tract obstruction

A

congenital:posterior urethral valves, uretrhal strictures, meatal stenosis, bladder neck obstuction
stones, BPH, tumors, inflammation, sloughed papillae or blood clots, pregnancy, uterine prolaspe, functional disorders

374
Q

what is hydronephrosis

A

dilation of renal pelvis and calyces associated with progressive atrophy of the kidney due to obsruction to outflow of urine

375
Q

how do you dx obstructive uropathy

A

US

376
Q

what occurs after relief of complete urinary tract obstruction

A

diuresis

377
Q

what are the types of urolithiasis

A

calcium oxalate, triple stones or struvite stones (magnesium, ammonium, phosphate), uric acid stones, cystine

378
Q

what are causes of calclium stones

A

hyperoxaluria, hyperuricosuria, hypocitraturia

379
Q

what causes magnesium ammonium phosphate stones

A

infection by urea splitting bacteria (proteus and staph); form the largest stones *staghorn calculi

380
Q

what are teh features of uric acid stones

A

ppl with hyperuricemia or acidic urine; radiolucent*

381
Q

what population of people tend to have angiomyolipomas

A

patients with tuberous sclerosis; can spontaneously hemorrhage

382
Q

what is an oncocytoma

A

epithelial neoplasm composed of eosinophilic cells that arise from intercalated cells of collecting ducts

383
Q

who is RCC more prominent in

A

males in 6-7th decade

384
Q

what is the most significant risk factor for RCC

A

smoking

385
Q

what is hereditary leiomyomatosis and renal cell cancer syndrome

A

AD; mutation of FH which expresses fumarate hydratase; characterized by cutaneous and uterine leiomyomata and an aggressive type of papillary carcinoma

386
Q

what is hereditary papillary carcinoma

A

AD; multi b/l tumors with papillary histo; mutations in MET

387
Q

what is birt-hogg-dube syndrome

A

AD; mutations in BHD which expresses folliculin; skin (fibrofolliculomas, trichodiscomas, and acrochordons), pulmonary (cysts or blebs) and renal tumors

388
Q

what is clear cell carcinoma

A

most common type; nonpapillary; loss of short arm of chrom 3 (harborbs VHL)

389
Q

what is papillary carcinoma

A

trisomies 7 and 17 and loss of Y in male patients in the sporadic form; trisomy 7 in famililal form (MET - encodes RTK for hepatocyte growth factor); frequently multifocal in origin

390
Q

what is chromophobe carcinoma

A

cells with prominent cell membranes and pale eosinophilic cytoplasm with a halo around the nucleus; multi chrom losses and extreme hypodiploidy; grow from intercalated cells of collecting ducts adn have excellent prognosis compared with clear cell and papillary

391
Q

what is Xp11 translocation carcinoma

A

occurs in young patients and defined by translocations of TFE3 gene located at Xp11.2 with a number of partner genes -> results in overexpression of TFE3; clear cytoplasm with papillary architecture

392
Q

what is collecting duct (bellini duct) carcinoma

A

arise from collecting duct cells in medular; several chrom losses; malignant cells form glands enmeshed within prominent fibrotic stromg; *medullary carcinom is similar seen in patients with sickle cell trait

393
Q

where do clear cell carcinomas most likely arise from

A

proximal tubule epithelium - usually as solitary unilateral lesions; yellow color as a result of prominent lipid accumulation

394
Q

where do papillary carcinomas arise from

A

distal convoluted tubules; multifocal and b/l; hemorrhagic and cystic; composed of cuboidal or columnar cells; form cells common; psammoma bodies can be present

395
Q

what are the clinical features of RCC

A

costovertebral pain, palpable mass, hematuria; *polycythemia, hypercalcemia, HTN, hepatic dysfuctnion, feminization or masculinization, cushing, eosinophilia, leukemoid rxns, and amyloidosis

396
Q

what are the most common sites of mets of RCC

A

lungs and bones; LN, liver, adrenal and brain

397
Q

what syndrome is associated with increased risk of urothelial carcinomas of renal pelvis

A

Lynch and analgesic nephropathy

398
Q

what is teh prognosis for urothelial carcinomas of renal pelvis

A

not good; infiltrate wall

399
Q

which causes of HTN cause an icrease in volume

A

low-renin essential HTN; primary hyperaldosteronism

400
Q

what is the most common cause of hydronephrosis in infants and children

A

ureteropelvic junction obstruction; more common in males (in adults, more common in women)

401
Q

how do you treat HTN in someone with renal dz

A

ACEI or ARB

402
Q

what does potassium do to BP

A

lowers it

403
Q

what is captopril

A

ACEI; side effect: cough and angioedema

  • enalapril: prodrug
  • benazepril and lisinopril have longer half lives
404
Q

what is losartan

A

nonpeptide angiotensin II receptor antagonist; side effects: hypoglycemia, hyperkalemia and cough

  • valsartan: Not a prodrug
  • candesartan: irreversible binding
405
Q

what are the adverse effects of aliskiren

A

increase in creatine phoshokinase, increased BUN, increased serum Cr; hyerkalemia

406
Q

what medications work well in blacks for HTN

A

calcium channel blockers and diuretics OR beta blockers, ACE inhibitors and ARBS added to a diuretic

407
Q

who should you not give ACE inhibitors or ARBs to

A

sexually active girls

408
Q

when should you not give drugs interfering with angiotensin II

A

renal failure with b/l renal stenosis

409
Q

when is ACEI contraindicated

A

pregnancy

410
Q

how are bladder cancers defined

A

invasion of muscularis propria

411
Q

what is a cystocele

A

when uterine prolapses and brings the bladder with it and the bladder protrudes from the vagina

412
Q

what is the most common cause of hydronephrosis in infants and children

A

ureteropelvic junction obstruction; more common in males (in adults, more common in women)

413
Q

what is ormond dz

A

idiopathic sclerosing retroperitoneal fibrosis

414
Q

what is the most common congenital anomaly of the bladder

A

vesicoureteral reflux

415
Q

when do you see acquired diverticula of teh bladder

A

prostatic enlargement

416
Q

what can bladder diverticula lead to

A

infection, calculi, rarely carcinoma which tend to be more advanced in stage

Renal II (3 decks)

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