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Flashcards in Renal - First Aid Deck (220):
1

Pronephros

week 4 kidney then degenerates

2

Mesonephros

functions as interim kidney for 1st trimester and later contributes to the male genital system

3

Metanephros

permanent; first appears in the 5th week of gestation

4

Ureteric bud

derived from the caudal end of the mesonephric duct; gives rise to the ureter, pelvises, calyces and collecting ducts; fully canalized by 10th week

5

Metanephric mesenchyme

ureteric bud interacts with this tissue inducing differentiation and formation of the glomerulus through to the distal convoluted tubule

6

The ureteropelvic junction is the...

last area to canalize and the most common site of obstruction in the fetus.

7

Potter Sequence is...

oligohydramnios leading to compression of the developing fetus leading to limb deformities and facial anomalies (low set ears and retrognathia) and compression of the chest leading to pulmonary hypoplasia.

8

Causes of Potter sequence include...

ARPKD, posterior urethral valves and bilateral renal agenesis. (babies who can't Pee develop Potter)

9

POTTER syndrome is associated with:

Pulmonary hypoplasia Oligohydramnios (trigger) Twisted face Twisted skin Extremity defects Renal failure (in utero)

10

Horseshoe kidney

the inferior poles of both kidneys fuse and as they ascend from the pelvis during fetal development, they get trapped under the inferior mesenteric artery and remain low in the abdomen

11

Horseshoe kidneys have increased risk for...

ureteropelvic junction obstruction, hydronephrosis, renal stones and renal cancer (Wilms tumor).

12

Horseshoe kidneys are associated with...

Turner syndrome.

13

Multicystic dysplastic kidney is due to....

abnormal interaction between the ureteric bud and metanephric mesenchyme. This leads to a nonfunctional kidney consisting of cysts and CT.

14

If multicystic dysplastic kidney is unilateral (most common), then it is generally...

asymptomatic with compensatory hypertrophy of the contralateral kidney.

15

The kidney taken for donor transplantation is...

the left one becasue it has a longer renal vein.

16

The ureters pass....

under the uterine artery and under the ductus deferens.

17

The ureters may be damaged by gynecologic procedures involving...

ligation of the uterine vessels leading to ureteral obstruction or leak.

18

Plasma volume is measured by...

radiolabeled albumin.

19

Extracellular volume is measured by...

inulin.

20

Plasma osmolarity =

290 mOsm/L.

21

The glomerular filtration barrier is composed of...

1. Fenestrated capillary endothelium (size barrier) 2. Fused basement membrane with heparan sulfate (negative charge barrier) 3. Epithelial layer consisting of podocyte foot processes

22

The charge barrier of the glomerular filtration barrier is lost in...

nephrotic syndrome, resulting in albuminuria, hypoproteinemia, generalized edema and hyperlipidemia.

23

Renal Clearance Equation

Cx = UxV/P

24

If Cx < GFR, then there is...

net tubular reabsorption of X.

25

If Cx > GFR, then there is...

net tubular secretion of X.

26

To calculate GFR, use...

inulin because it is freely filtered and is neither secreted or reabsorbed.

27

Normal GFR is about...

100 mL/min.

28

Creatine clearance is an approximate measure of...

GFR but it slightly overestimates GFR becasue creatine is moderately secreted by the renal tubules.

29

Effective renal plasma flow (ERFP) can be estimated using...

para-aminohippuric acid (PAH) clearance because it is both filtered and actively secreted in the proximal tubule. Nearly all PAH entering the kidney is excreted.

30

ERPF underestimates...

the true RPF by about 10%.

31

RBF =

RPF/(1-Hct)

32

FF =

GFR/RPF

33

Filtered load =

GFR x plasma concentration

34

In the kidneys, prostaglandins normally...

dilate the afferent arteriole leading to: -increased RPF -increased GFR (FF remains constant) **NSAIDs block this.

35

In the kidneys, angiotensin II acts to...

constrict the efferent arteriole leading to: -decreased RPF -increased GFR -increased FF **ACE inhibitors block this.

36

Excretion rate =

V x Ux

37

Reabsorption rate =

filtered - excreted

38

Secretion rate =

excreted - filtered

39

At a normal plasma level, glucose is...

completely reabsorbed in the proximal tubule by Na/glucose costransport.

40

Glucosuria begins at...

about plasma glucose of 200. And at 375, all transporters are fully saturated.

41

Normal pregnancy decreases the reabsorption of...

glucose and amino acids in the proximal tubule leading to glucosuria and aminoaciduria.

42

Amino acids are reabsorbed by...

sodium-dependent transporters in the proximal tubule.

43

Hartnup disease is...

a deficiency of neutral amino acid (tryptophan) transporters in the proximal renal tubular cells leading to aminoaciduria.

44

Hartnup disease results in..

pellagra like symptoms.

45

Treat Hartnup disease with...

high-protein diet and nicotinic acid.

46

Fanconi syndrome is...

a reabsorptive defect in PCT

47

Fanconi is associated with...

increased excretion of nearly all amino acids, glucose, HCO3-, and PO43-.

48

Fanconi may result in...

metabolic acidosis.

49

Causes of Fanconi include...

Wilson disease, ischemia and nephrotoxins/drugs.

50

Bartter syndrome is...

a resorptive defect in the thick ascending loop of Henle that affects the Na/K/2Cl cotransporter.

51

Bartter syndrome results in...

hypokalemia and metabolic alkalosis with hypercalciuria.

52

Gitelman syndrome is...

a resorptive defect of NaCl in the DCT. Less severe than Bartter.

53

Gitelman syndrome leads to...

hypokalemia and metabolic alkalosis but without hypercalciuria.

54

Liddle Syndrome is...

increased Na+ reabsorption in the distal and collecting tubules (increased ENaC).

55

Liddle syndrome results in...

HTN, hypokalemia, metabolic alkalosis and decreased aldosterone.

56

Treatment for Liddle syndrome is...

Amiloride.

57

Angiotensin II

-affects baroreceptor function -limits reflex bradycardia -helps maintain blood volume and pressure

58

ANP is released from...

atria in response to increased volume; may act as a "check" on the RAAS; relaxes vascular smooth muscle via cGMP causing increased GFR and decreased renin

59

ADH primarily regulates...

osmolarity; it responds tolow blood volume states

60

Aldosterone primarily regulates...

ECF Na content and volume; responds to low blood volume states.

61

Renin is released from the kidneys in response to...

-decreased BP (JG cells) -decreased Na+ delivery to the macula densa -increased sympathetic tone (Beta-1 receptors)

62

The juxtaglomerular apparatus consists of...

JG cells (smooth muscle of the afferent arteriole) and macula densa (NaCl sensor in the distal convoluted tubule).

63

JG cells secrete...

renin.

64

The juxtaglomerular apparatus defends....

GFR via RAAS.

65

Beta-blockers can decrease BP by...

inhibiting beta1 receptors of the JGA causing decreased renin release.

66

Erythropoietin is released by...

interstitial cells in the peritubular capillary bed in response to hypoxia.

67

The proximal tubule cells convert...

25-OH vitamin D to 1,25 (OH)2 vitamin D which is the active form. 1alpha-hydroxylase is the enzyme.

68

Things that shift K+ out of the cell causing hyperkalemia

Digitalis HyperOsmolarity Insulin deficiency Lysis of cells Acidosis Beta-adrenergic antagonis

69

Things that shift K+ into the cell

-hypoosmolality -insulin -alkalosis -beta-adrenergic agonist (Insulin INto cells)

70

Low Serum Na+

nausea malaise stupor coma

71

High Serum Na+

irritability stupor coma

72

Low Serum K+

U waves on ECG flattened T waves arrhythmias muscle weakness

73

High Serum K+

wide QRS peaked T waves arrhythmias muscle weakness

74

Low Serum Ca2+

tetany seizures QT prolongation

75

High Serum Ca2+

Stones (renal) Bones (pain) Graons (abdominal pain) Psychiatric overtones (anxiety, altered mental status)

76

Low Serum Mg2+

tetany torsades de pointes

77

High Serum Mg2+

decreased DTRs lethargy bradycardia hypotension cardiac arrest hypocalcemia

78

Low Serum Phosphate

bone loss osteomalacia

79

High serum phosphate

renal stones metastatic calcifications hypocalcemia

80

Henderson-Hasselbalch Equation

pH = 6.1 + log(HCO3-/.03PCO2)

81

The predicted respiratory compensation for a simple metabolic acidosis can be calculated using...

the Winters formula. PCO2 = 1.5[HCO3-] + 8 +/- 2 If the measured PCO2 differs significantly from the predicted PCO2, then a mixed acid base disorder is likely present.

82

Type I Renal Tubular Acidosis

-defect in ability of alpha intercalated cells to secrete H+ -new HCO3- is not generated leading to metabolic acidosis -associated with hypokalemia -incresaed risk for calcium phosphate kidney stones

83

Causes of Type I Renal Tubular Acidosis

-amphotericin B toxicity -analgesic nephropathy -multiple myeloma -congenital anomalies of the urinary tract

84

Type II Renal Tubular Acidosis

-defect in proximal tubule HCO3- reabsorption leading to metabolic acidosis -associated with hypokalemia -increased risk for rickets

85

Causes of Type 2 Renal Tubular Acidosis

-Fanconi syndrome (wilson dz) -chemicals toxic to proximal tubule (lead, aminoglycosides) -carbonic anhydrase inhibitors

86

Type 4 Renal Tubular Acidosis

-hypoaldosteronism; aldosterone resistance; or K+ sparing diuretics -hyperkalemia impairs ammoniagenesis in the PT -decreased buffering capcity and decreased H+ secretion

87

Presence of casts in the urine indicates that hematuria/pyuria is of...

renal origin (vs. bladder).

88

RBC casts

-glomerulonephritis, ischemia or malignant HTN

89

WBC casts

-tubulointerstitial inflammation, acute pyelonephritis, transplant rejection

90

Fatty casts (oval fat bodies)

-nephrotic syndrome

91

Granular (muddy brown) casts

acute tubular necrosis

92

Waxy casts

advanced renal disease/chronic renal failure

93

Nephrotic Syndrome presents with...

massive proteinuria (> 3.5; frothy urine), hyperlipidemia, fatty casts and edema.

94

Nephrotic Syndrome is associated with...

thromboembolism (hypercoagulable state due to AT III loss in urine) and increased risk of infxn (loss of Igs).

95

Focal Segmental Glomerulosclerosis is the most common cause of...

nephrotic syndrome in African Americans and Hispanics.

96

Focal Segmental Glomerulosclerosis can be idiopathic or associated with...

HIV, sickle cell, heroin, obesity, interferon, and chronic kidney disease.

97

Labs of Focal Segmental Glomerulosclerosis

LM - segmental sclerosis and hyalinosis IF - negative EM - effacement of foot processes

98

Membranous nephropathy is the most common cause of...

primary nephrotic syndrome in caucasian adults.

99

Membranous nephropathy can be idiopathic or associated with...

Ab to phospholipase A2 receptor, drugs, infxns, SLE or solid tumors.

100

Labs of Membranous Nephropathy

LM - diffuse capillary and GBM thickening IF - granular as a result of immune complex deposition EM - "spike and dome" appearance with subepithelial deposits

101

Minimal Change Disease is most common in...

children.

102

Minimal Change Disease may be triggered by...

recent infection, immunization or immune stimulus.

103

Minimal Change Disease may be associated with...

Hodgkin lymphoma.

104

Focal segmental glomerulosclerosis response to steroids

inconsistent; may progress to chronic renal disease

105

Membranous nephropathy response to steroids

poor; may progress to chronic renal disease

106

Minimal Change Disease response to steroids

excellent

107

Minimal Change Disease Labs

LM - normal glomeruli IF - negative EM - effacement of foot processes

108

The most commonly involved organ with systemic amyloidosis is...

the kidney. It is associated with chronic conditions (multiple myeloma, TB, RA).

109

Labs for amyloidosis

LM - congo red stain shows apple-green birefringence under polarized light

110

Type I Membranoproliferative Glomerulonephritis

-subendothelial immune complex deposits with granular IF -"tram-track" appearance due to GBM splitting caused by mesangial ingrowth

111

Type II Membranoproliferative Glomerulonephritis

-intramembranous immune complex deposits (dense)

112

Type I Membranoproliferative Glomerulonephritis is associated with...

HBV and HCV.

113

Type II Membranoproliferative Glomerulonephritis is associated with...

C3 nephritic factor.

114

Diabetic glomerulonephroapthy

-nonenzymatic glycosylation of GBM leads to increased permeability and thickening -nonenzymatic glycosylation of efferent arterioles leads to increased GFR and mesangial expansion

115

Light microscope of diabetic glomerulonephropathy

-mesangial expansion -GBM thickening -eosinophilic nodular glomerulosclerosis (Kimmelstiel-Wilson lesion)

116

Nephritic Syndrome

an inflammatory process that can lead to hematuria and RBC casts in the urine. It is associated with azotemia, oliguria, HTN and proteinuria (<3.5 grams/day).

117

Acute poststreptococcal glomerulonephritis is most frequently seen in...

children adn occurs about 2 wks after group A streptococcal infxn of the pharynx or skin. Resolves spontaneously. Type III HSR.

118

Acute poststreptococcal glomerulonephritis presents with...

peripheral and periorbital edema, dark urine and HTN. -increased anti-DNase B titers -decreased complement levels

119

Acute poststreptococcal glomerulonephritis labs

LM - glomeruli enlarged and hypercellulr IF - "starry sky" granular appearance "lumpy bumpy" due to IgG, IgM and C3 deposition along the GBM and mesangium EM - subepithelial immune complex humps

120

Rapidly progressive (cresenteric) glomerulonephritis results from several disease processes:

1. Goodpasture syndrome: type II HSR, Abs to GBM and alveolar BM leading to linear IF 2. Granulomatosis with polyangiitis 3. microscopic polyangiitis

121

Rapidly progressive glomerulonephritis Labs

LM and IF - crescent-moon shape consisting of fibrin and plasma proteins with glomerular parietal cells, monocytes and macrophages

122

Rapidly progressive glomerulonephritis presents with...

hematuria/hemoptysis. -PR3-ANCA/c-ANCA -MPO-ANCA/p-ANCA

123

Diffuse proliferative glomerulonephritis is due to either...

SLE or MPGN. This is the most common cause of death in SLE.

124

Labs of diffuse proliferative glomerulonephritis

LM - "wire looping" of capillaries EM - subendothelial and sometimes intramembranous IgG based immune complexes often with C3 deposition IF - granular

125

IgA Nephropathy (Berger) often presents with...

a URI or acute gastroenteritis. Episodic hematuria with RBC casts. Seen with Henoch-Schonlein purpura.

126

IgA Nephropathy Labs

LM - mesangial proliferation EM - mesangial immune complex deposits IF - IgA based immune complex deposits in the mesangium

127

Alport Syndrome is due to...

a mutation in type IV collagen leading to thinning and splitting of the glomerular basement membrane; usually X-linked

128

Alport syndrome presents with...

glomerulonephritis, deafness and eye problems.

129

Kidney stones can lead to...

hydronephrosis and pyelonephritis.

130

Kidney stones present with...

unilateral flank tenderness colicky pain radiating to the groin hematuria

131

All kidney stones are radiopaque except...

Uric acid stones with are radiolUcent.

132

Calcium phosphate stones precipitate at...

increased pH.

133

Calcium oxalate stones precipitate at...

decreased pH.

134

Calcium stones are promoted by...

hypercalciuria.

135

Oxalate crystals can result from...

ethylene glycol (antifreeze), vitamin C abuse or Crohn's.

136

Treatments for recurrent calcium stones includes...

thiazides and citrate.

137

Most common presentation of kidney stones:

calcium oxalate stone in a pt with hypercalciuria and normocalcemia

138

Urine crystals of calcium stones appear...

envelope or dumbell shaped.

139

Ammonium magnesium phosphate stones urine crystals appear....

as a coffin lid.

140

Ammonium magnesium phosphate stones area also known as...

struvite.

141

Struvite stones are caused by...

infection with urease + bugs (Proteus mirabilis, Staph, Klebsiella) that hydrolyze urea to ammonia leading to urine alkalinization.

142

Ammonium magnesium phosphate stones can form...

staghorn calculi taht can be a nidus for UTIs.

143

Ammonium magnesium phoshpate stones precipitate at...

increased pH.

144

Uric acid stones precipitate at...

decreased pH.

145

Risk factors for Uric acid stones

-decreaed urine volume -arid climates -acidic pH

146

Uric acid stones are visible on...

CT and ultrasound but not X-ray.

147

Uric acid stones have a strong association with....

hyperuricemia (gout). They are often seen in disease with increased cell turnover like leukemia.

148

Treatment of uric acid stones

alkalinization of the urine.

149

Uric acid stones urine crystals appear as...

rhomboid or rosettes.

150

Cystine stones precipitate at...

decreased pH.

151

Cystine stones urine crystals appear....

hexagonal.

152

Cystine stones are mostly seen in...

children secondary to cystinuria. Can form staghorn calculi. Positive sodium nitroprusside test.

153

Treatment for Cystine stones

alkalinization of urine and hydration

154

Hydronephrosis is...

distention/dilation of the renal pelvis and calyces. Usually caused by urinary tract obstruction; other causes include retroperitoneal fibrosis and vesicoureteral reflux.

155

Hydronephrosis only impairs renal function if...

it is bilateral or the pt only has one kidney.

156

Hydronephrosis leads to...

compression atrophy of the renal cortex and medulla.

157

Renal cell carcioma originates from...

the proximal tubule cells leading to polygonal clear cells filled with accumulated lipids and carbs.

158

Renal cell carcinoma is most common in...

men 50-70 yrs old.

159

Increased incidence of renal cell carcinoma with...

smoking and obesity.

160

Renal cell carcinoma manifests clinically with...

hematuria, palpable mass, secondary polycythemia, flank pain, fever and weight loss.

161

Renal cell carcinoma can invade...

the renal vein and then the IVC and spread hematogenously. Metastasizes to the lung and bone.

162

Renal cell carcinoma is associated with gene deletion on...

chromosome 3 (sporadic or inherited as von Hippel-Lindau syndrome).

163

Renal cell carcinoma is also associated with...

paraneoplastic syndromes (ectopic EPO, ACTH, PTHrP)

164

Renal cell carcinoma is resistant to...

chemo and radiation. Need to resect or use immunotherapy.

165

Renal oncocytoma is...

a benign epithelial cell tumor that has large eosinophilic cells with abundant mitochondria without perinuclear clearing

166

Renal oncocytoma presents with...

painless hematuria, flank pain and abdominal mass.

167

Treatment for renal oncocytoma

nephrecotmy

168

Wilms tumor (nephroblastoma)

most common renal malignancy of early childhood; contains embryonic glomerular structures

169

Wilms tumor presents with...

huge, palpable flank mass and/or hematuria

170

Mutations of Wilms tumor

loss of function mutations of tumor suppressor genes WT1 or WT2 on chromosome 11

171

Wilms tumor may be part of either...

Beckwith-Wiedemann syndrome or WAGR complex (Wilms tumor, Aniridia, GU malformation and mental Retardation)

172

The most common tumor of the urinary tract system is...

transitional cell carcinoma.

173

Transitional cell carcinoma presents with...

painless hematuria (no casts) which suggest bladder cancer.

174

Transitional cell carcinoma is associated with problems in your....

Pee SAC: P - phenacetin S - smoking A - aniline dyes C - cyclophosphamide

175

Chronic irritation of the urinary bladder leads to...

squamous metaplasia leads to dysplasia and squamous cell carcinoma. Presents with painless hematuria.

176

Risk factors for squamous cell carcinoma of the bladder include:

-Schistosoma haematobium infxn (middle east) -chronic cystitis -smoking -chronic nephrolithiasis

177

Acute infectious cystitis presents as...

suprapubic pain, dysuria, urinary frequency adn urgency.

178

Risk factors for acute infectious cystitis

-femal gender (short urethra) -sexual intercourse ("honeymoon cystitis") -indwelling catheters

179

Causes of Acute Infectious Cystitis

-E. coli -Staph saprophyticus (seen in sexually active young women) -Klebsiella -Proteus mirabilis (urine has ammonia scent) -adenovirus (hemorrhagic cystitis)

180

Lab findings for acute infectious cystitis

-positive for leukocyte esterase -nitrates appear for gram-neg organisms -sterile pyuria and negative urine cultures suggest N. gonorrhea or Chlamydia

181

Pyelonephritis affects...

the cortex with relative sparin of glomeruli/vessels.

182

Acute pyelonephritis presents with...

dysuria, fever, CVA tenderness, nausea/vomiting. Often with white cell casts in urine.

183

Causes of pyelonephritis include:

-ascending UTI (E. coli often) -vesicoureteral reflux -hematogenous spread to the kidney

184

CT of pyelonephritis shows...

striated parenchymal enhancement.

185

Risk factors for pyelonephritis

-indwelling catheter -urinary tract obstruciton -DM -pregnancy

186

Complications of acute pyelonephritis

-chronic pyelonephritis -renal papillary necrosis -perinephric abscess

187

Chronic pyelonephritis is the result of...

recurrent episodes of acute pyelonephritis. Typically requires predisposition to infxn such as vesicoureteral refulx or chronic stones.

188

Findings of Chronic Pyelonephritis

-coarse, asymmetric corticomedullary scarring, blunted calyx -tubules can contain eosinophilic casts resembling thyroid tissue

189

Drug-induced interstitial nephritis is...

pyuria and azotemia occuring after administration of drugs that act as haptens inducing hypersensitivity. Nephritis typically occurs 1-2 wks after certain drugs (diruretics, penicillins, sulfonamides, rifampin) but months after NSAIDs.

190

Drug-induced interstitial nephritis is associated with...

fever, rash, hematuria adn CVA tenderness.

191

diffuse cortical necrosis is...

acute generalized cortical infarction of both kidneys likely due to a combination of vasospasm and DIC.

192

Diffuse cortical necrosis is associated with...

obstetric catastrophes and septic shock.

193

The most common cause of intrinsic renal failure is...

acute tubular necrosis. Death most often occurs during the initial oliguric phase.

194

Key finding of acute tubular necrosis

-granular "muddy brown" casts

195

3 stages of Acute Tubular Necrosis

1. inciting event 2. maintenance phase - oliguric; lasts 1-3 wks; risk of hyperkalemia; metabolic acidosis 3. recovery phase - polyuric; BUN adn serum creatinine fall; risk of hypokalemia

196

Ischemic acute tubular necrosis is secondary to...

decreased RBF (hypotension, shock, sepsis, hemorrhage, CHF). Results in death of tubular cells that may slough into tubular lumen.

197

Nephrotoxic Acute Tubular Necrosis is secondary to...

injury resultin gfrom toxic substances (aminoglycosides, radiocontrast, lead, cisplatin), crush injury (myoglobinuria), hemoglobinuria.

198

Pathogenesis of renal papillary necrosis

sloughing of renal papillae leads to gross hematuria and proteinuria; may be triggered by

199

Renal papillary necrosis is associated with...

DM acute pyelonephritis chronic phenacetin use sickle cell anemia/trait

200

In the normal nephron, BUN is...

reabsorbed (for countercurrent multiplication) but creatitine is not.

201

Acute kidney injury is defined as...

an abrupt decline in renal function with increased creatinine and increased BUN over a period of several days.

202

Prerenal azotemia occurs as a result of...

decreased RBF leading to decreased GFR. Na/water and urea are retained by the kidney in an attempt to conserve volume so BUN/creatinine ratio is increased.

203

Intrinsic renal failure is generally due to..

acute tubular necrosis or ischemia/toxins; less commonly due to acute glomerulonephritis.

204

Intrinsic renal failure demonstrates...

patchy necrosis leading to debris obstructing the tubule and fluiud backflow across the necrotic tubule. This decreases GFR. BUN reabsorption is impaired (decreased BUN/creatinine ratio).

205

Urine in intrinsic renal failure has...

epithelial/granular casts.

206

Postrenal azotemia is due to..

outflow obstruction (stones, BPH, neoplasia, congenital anomalies). Develops only with bilateral obstruction.

207

Consequences of Renal Failure

MAD HUNGER MA: Metabolic Acidosis D: Dyslipidemia H: Hyperkalemia U: Uremia N: Na/H20 retention G: Growth retardation/developmental delay E: Erythropoietin failure (anemia) R: Renal osteodystrophy

208

Uremia is a clinical syndrome marked by:

increased BUN and creatinine -nausea/anorexia -pericarditis -asterixis -encephalopathy -platelet dysfunction

209

Renal Osteodystrophy is...

failure of vitamin D hydroxylation, hypocalcemia, and hyperphosphatemima leading to secondary hyperparathyroidism. Causes subperiosteal thinning of bones.

210

Within renal osteodystrophy, hyperphosphatemia also independently...

decreases serum calcium by causing tissue calcifications.

211

Autosomal dominant polycystic kidney disease

-innumerable cysts causing bilateral enlarged kidneys ultimately resulting in destruction

212

ADPKD presents with...

flank pain, hematuria, HTN, urinary infxn and progressive renal failure; typically in adults.

213

ADPKD has mutation in either...

PKD1 (on chr 16) or PKD 2 (on chr 4).

214

ADPKD is associated with...

berry aneurysms, mitral valve prolapse and hepatic cysts.

215

Autosomal recessive polycystic kidney disease

infantile presentation; significant renal failure in utero can lead to Potter sequence

216

ARPKD is associated with...

congenital hepatic fibrosis.

217

Concerns of ARPKD beyond the neonatal period include...

HTN, portal HTN, and progressive renal insufficiency.

218

Medullary cystic disease is...

an inherited disease causing tubulointerstitial fibrosis and progressive renal failure with the inability to concentrate urine. Shows shrunken kidneys on ultrasound.

219

Simple renal cysts are typically found in...

the outer cortex filled with ultrafiltrate. Verry common/majority of all renal masses; found incidentally.

220

Complex renal cysts have...

solid components as seen on CT and require follow-up/removal due to increased risk of RCC.