renal path flashcards

1
Q

What renal conditions area associated with sickle cell trait

A

Autosomal dominant Polycystic Kidney Disease (associated with accelerated disease in black patients with sickle cell trait) and Real Medullary Carcinoma

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2
Q

Horse shoe kidney

A

90% of cases have fusion of upper pole - patients are usually asymptomatic/normally functioning

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3
Q

Presentation of ADPKD

A

Cysts not present at birth, renal function intact until the 4th or 5th decade when patients become symptomatic. Inscidious onset of hematuria (1st symptom) followed by polyuria and hypertension (Kiddos- HTN and hematuria)

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4
Q

cysts in the liver, spleen and lungs are assocaited with what renal condition

A

ADPKD

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5
Q

Intracranial berry aneuysms are associated with what renal condition

A

ADPKD

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6
Q

Genetic component of ADPKD

A

PKD1 (85%) and PKD2- PKD2 presents later than PKD1

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7
Q

how does the pathology of ADPKD differ from that of ARPKD

A

ADPKD= dilation of all parts of the nephron ARPKD= dilation of the collecting tubules

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8
Q

ADPKD differential Diagnosis RCC

A

RCC R/O based on the fact that ADPKD is bilateral rather than unilateral and the cysts are NONuniform

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9
Q

“Tufting of papillary projections”

A

ADPKD

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10
Q

what renal condition presents with dilated elongated channels at right angles to the cortical surface

A

ARPKD

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11
Q

Newborn presents with severe respiratory distress and pulmonary hyperplasia - what renal condition

A

ARPKD. Pulmonary Hyperplasia is secondary to oligohydramnios

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12
Q

Clinical presnetation of ARPKD

A

Liver and Kidney most often involved (lungs in neonates) , most present before age 20, Hypertension in almost all cases, liver disease predominates in older children and adults (portal hypertension and splenomegaly)

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13
Q

What cystic disorder is associated with portal hypertension (portal fibrosis)

A

ARPKD

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14
Q

Buzz word: Fetal lobulation

A

ARPKD

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15
Q

MC cystic disease in children

A

Multicystic Dysplastic Kidney

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16
Q

what renal condition has a microscopic appearnce discribed as “Disorganized with dilated tubules with cuffs of primative stroma and island of cartilage”

A

Multicystic Dysplastic Kidney

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17
Q

what cystic condition is confined to the medullary pyramids and papillae

A

Medullary sponge kidney

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18
Q

How can you differntiate medullary sponge from PKD

A

Medullary sponge has intersitial fibrosis

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19
Q

Most common genetic cause of ESRD in children and young adults

A

Nephrophthisis

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20
Q

Genetic mutation in ARPKD

A

Polycystic Kidney Hepatic Disease gene (PKHD1)

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21
Q

what type of Nephrophthisis is most common

A

Familial (juvenile) - becomes clinically evident in childhoood or adolescence.

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22
Q

what cystic condition has cysts most prominent at the corticomedullary junction

A

Nephrophthisis

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23
Q

Polyuria, polydipsia, cardiac, musculoskeletal, and ocular disease

A

Nephrophthisis

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24
Q

what renal disease causes growth failure in children

A

nephrophthisis

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25
Q

Presnetation of Adult onset medullary cystic kidney disease

A

Present in 3rd -4th decade with polyuria and polydipsia, may also have hyperuricemia and gout

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26
Q

“tubular atrophy interspersed with hypertrophied and dilated tubules”

A

Adult onset medullary cystic disease

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27
Q

What cystic disease was initially descriebed in patients undergoing dialysis but can also be seen in uremic nondialysized patients

A

Acquired cystic renal disease

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28
Q

cysts filled with straw colored hemorrgagic fluid and often contain calcium oxalate crystals

A

acquired cystic renal disease

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29
Q

Most common cystic abnormality of the kidney

A

Simple Cyst

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30
Q

Name the nephritic diseae

A

Acute proliferative glomerularnephritis, Rapid progressive glomerular nephritis, IgA nephropathy, Alport Syndrome Membranoproliferative Glomerulonephritis

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31
Q

Buzz word: Subepithelial humps

A

Acute proliferative glomerulonephritis

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32
Q

7 year old Patient presents with painless gross hematuria 3 weeks after having a sore throat

A

Acute proliferative glomerulonephritis MCC = Group A strep pharyngitis

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33
Q

Biopsy shows hypercellularity due to infiltration and proliferation of endothelial , mesangial, and epithelial cells

A

Acute proliferative glomerulonephritis

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34
Q

Cresents seen on biopsy

A

Rapid progressive Glomerular nephritis

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35
Q

RPGN gross morphology

A

kidney is large and pale with petichial hemorrage

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36
Q

most common type of GN world wide

A

IgA nephropathy

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37
Q

recurrent symptomatic hematurua after respiratory infection and absence of systemic disease

A

IgA nephropathy

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38
Q

Buzz word: Synpharyngitic macroscopic hematuria

A

igA nephropathy

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39
Q

Biopsy shows mesangial exapansion without significant involvement of capillary walls or lumina

A

iga nephropathy

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40
Q

Renal Disease, Sensorial Hearing loss, Ocular abnormalities

A

Alport Syndrome “Cant see, cant pee, cant hear a bee”

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41
Q

what differnetiates Aloprt Syndrome from IgA nephropathy

A

will always have microscopic hematuria with Alport syndrome. Microscopic hematuria will be abscent between illnesses in IgA nephropathy

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42
Q

what renal condition is caused by abnormal type IV collagen

A

Alport Syndrome

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43
Q

what renal condition is assoaciated wth Hepatitis C

A

Membranoproliferative Glomerulonephritis

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44
Q

Buzz word: tram tracking

A

Membranoproliferative Glomerulonephritis

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45
Q

what types of eposits are seen in type I memranoproliferative GN

A

Subendithelual deposits between duplicated membranes

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46
Q

what type of deposits are seen in type II membranoproliferative GN

A

Intramembranous dense deposits = Ribbon like deposits

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47
Q

Name the nephrotic diseases

A

Membranous Nephropathy, Focal Segmental GN, Diabetic nephropathy Minimal change

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48
Q

Most commmon casye of adult nephrotic syndrome in adults and elderly

A

membranous GN

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49
Q

What glomerular disease is associated with malignacy

A

membranous GN

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50
Q

Biopsy shows spikes (progression of basement membrane) near subepithelial deposits and no increase in cellularity

A

membranous GN

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51
Q

Most common GN leading to ESRD

A

Focal segmental GN

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52
Q

MCC of focal glomerulonephritis

A

Obesisty and anabolic steroid use

53
Q

what would you see on biopsy of FSG

A

Hyalin insudation and lipid vacuoles in sclerotic areas

54
Q

Biopsy shows hyaline arteriolosclerosis and basement membrane thickening of tubules and glomerular capillaries

A

Diabetic Glomerulonephropathy

55
Q

Buzz word: Nodular glomerulosclerosis or Kinnelstiel Wilson Disease

A

Diabetic Glomerulonephropathy

56
Q

buzz word: effacement of podocytes, normal appearing glomerular biopsy

A

Minimal change disease

57
Q

most common casue of acute renal failure

A

Acute tubular injury (tubular necrosis)

58
Q

what sections of the tubules are sensitive to ischemia and toxins

A

straight portions of the proximal tubule and medullary thick ascending loop of henle

59
Q

Biopsy shows eosinophilic hyaline casts and pigmented granular casts (muddy brown casts)

A

Acute Tubular injury (tubular necrosis)

60
Q

Biopsy of acute tubular injrut from ethylene glycol

A

ballooning and hydrophobic or vacuolar degeneration of proximal convoluted tubules. Calcium oxide crystals in tubular lumen

61
Q

what condition causes intertubular aggregates of neutrophils and neuetrophilic tubulitis with neutrophil casts

A

Acute pyelonephritis

62
Q

Buzz word: thyroidization

A

Chronic pyelonephrits

63
Q

second most common cause of acute kidney injury after pyelonephritis

A

drug and toxin induced tublointersitial nephritis

64
Q

Initial phase of Acute tubular injury

A

lasts 36 hours - period when patient exposed to ischemia/toxin and parenchymal injury is developing but not yet established.

65
Q

Maintainance phase of acute tubular injury

A

Uremia, salt and water overload, rising BUN, HYPERkalemia, metabolic acidosis

66
Q

Recovery phase of acute tubular injury

A

diuretic phase large amount of salt and water lost, HYPOkalemia becomes a problem

67
Q

How does the infiltrate differe in acute vs chronic tubular nephritis

A

Acute: Leukocyte infiltration (mainly neutrophils and eosinophils) Chronic: inflammation mainly monocytes

68
Q

what is the most common cause of acute pyelonephritis

A

Ascending infection (usually E.coli)

69
Q

name the complications of acute pyelonephritis

A

Papillary Necrosis, Pyonephrosis, Perinephric abscess

70
Q

what population is papillary necrosis normally seen in

A

complication of acute pyelonephritis seen in people with diabetes, sickle cell disease, urinary obstruction, also seen in NSAIDs

71
Q

describe the gross morphology of papillary necrosis

A

tips or distal 2/3 of the pyramids have areas of gray/white to yellow necrosis

72
Q

pyonephrosis

A

suppurative exudatecannot drain (due to obstruction) and fills the renal pelvis, calyces, and ureter with pus

73
Q

Acute Uric Acid Nephropathy

A

precipitation of uric acid in renal tubules and development of acute renal failure - seen in leukemic patients undergoing chemotherapy (cancer cell is killed and uric acid us released- tumor lysis syndrome)

74
Q

Buzz word: tumor lysis syndrome

A

Acute Uric Acid nephropathy

75
Q

Chronic urate nephropathy

A

Gouty nephropathy- monosodium urates deposit in the distal tubules and collecting ducts and interstitium and form birifringent needle like cysts

76
Q

what renal conditions are associated with calcium phosphate deposition

A

Medullary Sponge Kidney and Hypercalcemia and Nephrocalcinosis

77
Q

Buzz word: Bence Jones Protien

A

Light chain cast nephropathy (myeloma kidney)

78
Q

biopsy descrived as Angulated and tubular casts surrouned my macrophages (some of them multinucleated)

A

Light chain cast nephropathy (myeloma kidney)

79
Q

what is nephrosclerosis

A

Sclerosis of renal arterioles and small arteries usually in the setting of HTN that results in parenchymal ischemia and glomerulosclerosis that ultimately shrinks the kidney

80
Q

Buzz word: Cortical surface described as grainy leather

A

Nephrosclerosis (fine even granularity)

81
Q

Athromathous plaque vs fibromuscular dysplasia

A

fibromuscular dysplasia havs the lumen still in the center

82
Q

inflammation in the setting of cortical infarct is a response to what

A

Inflammation is in response to the necrosis NOT the cause of the necrosis

83
Q

Buzz word: Schistocytes

A

Thrombotic Microangiopathies - result from shearing of red blood cells

84
Q

Most commin Genitourinary tumor in children

A

Wims (Nephroblastoma)

85
Q

name the benign renal tumors found in adults

A

Renal Papillary Adenoma, Renal oncocytoma, Angiomyolipoma

86
Q

Name the malignant renal tumors found in adults

A

Clear cell renal cell carcinoma, Papillary renal cell carcinoma, Chromophobe renal cell carcinoma, Collecting duct (Bellini duct) carcinoma, renal medullary carcinoma, urothelial carcinomal or the renal pelvis

87
Q

MCC of ischemia

A

coronary atherosclerosis (90%)

88
Q

MC Acute coronary syndrome

A

Angina pectoris

89
Q

angina pectrois is most common in what population

A

middle aged men and women after menopause

90
Q

most common variant of angina

A

Stable Angina

91
Q

T/F Stable angina is associated with plaque disruption

A

False

92
Q

What type of angina is relieved by rest or vasodilator

A

Stable angina

93
Q

what typically causes unstable angina

A

disruption of a plaque and superimposed partial thrombus, and probably embolis or vasospasm (or both)

94
Q

Early response to biochemical response

A

within seconds - lactate levels rise and ATP falls (due to lact of oxygen and cessation of aerobic metabolism)

95
Q

loss of myocte contractility happens within what time frome

A

within 60 seconds

96
Q

early changes are potentially reversible within what time period

A

20-30 minutes - ischemia > 1 hour causes damage to cardiac microvasculature

97
Q

Most MI are what type

A

Transmural

98
Q

Progression of Myocardial Necrosisi

A

Necrosis begins in small zone of myocardium beneath the endocardial surface in the center of the ischemic zone. VERY NARROW ZONE OF MYOCARDIUM BENEATH THE ENDOCARDIUM IS SPARED FROM NECROSIS DUE TO DIFFUSION OF OXYGEN FROM THE VENTRICLE

99
Q

most common cause of death in adults in the US

A

MI

100
Q

when do gross findings of MI first appear

A

12-24 hours: Dark Mottling of infarct and central pallor

101
Q

infiltrate of abundant neutrophils are seen when (post MI)

A

1-3 days

102
Q

well developed phagocytosis of dead myoctes occurs when (post MI)

A

7-10 days

103
Q

MI scar begins to form at ____ and is complete at ____

A

begins at 2 weeks and is complete at 8 weeks

104
Q

Buzz word: Contraction bands

A

indicative of reperfusion injury post MI

105
Q

Creatinkin phosphokinase dimer specific for cardiac muscle

A

CKMB

106
Q

when does CKMB appear in the blood, peak, and return to baseline

A

appears 2-4 hours after MI, peaks at 24 hours and returns to baseline at 36

107
Q

what lab test is most sensitive and specific for myocardial damage

A

Troponin (T and I)

108
Q

when does troponin appear in the blood, peak, and return to baseline

A

appears 2-4 hours after MI, peaks at 48 hours, and persists for 10-14 days post MI

109
Q

what morphological features are seen with chronic ischemic heart disease

A

left ventricular hypertorphy and ventricular dilation

110
Q

congenital heart disease occurs when

A

3-8 weeks gestation

111
Q

major known cause or congenital heart disease

A

sporadic genetic abnormalities

112
Q

almost all congenital abnormalities predispose for what condition

A

bacterial endocarditis - abnormalities cause turbulent flowt that can damage endocardium

113
Q

Most common congenital heart condition

A

Ventricular septal defect

114
Q

what classification of shunt is cyanotic

A

Right to left

115
Q

what classification of shunt is acyanotic

A

left to right

116
Q

name the L to R disorders

A

“all have a D in them” ASD, VSD, PDA, AVDS

117
Q

most common site of ASD

A

foramen ovale

118
Q

most common congenital heart condition presenting in adulthood

A

ASD

119
Q

name the R to L disorders

A

“All have a T in them” Tetrology of fallot, transposition of the great vessels, tricupsid atresia, patent truncus arteriosus, total anomolous venous connection/reutrun

120
Q

what congenital heart disorder is associated with down syndrome

A

AVSD

121
Q

4 characteristics of tetrology of fallot

A

1: VSD 2: Overriding Aorta 3: RVH 4: Pulmonic stenosis

122
Q

buzz word: boot shaped heart

A

tetrology of fallot

123
Q

infant turns blue when crying

A

tetrology of fallot

124
Q

child squats during exercise

A

tetrology of fallot - squatting increases venous return to the heart

125
Q

what congenital heart disorder is associated with women with turner syndrome

A

coartation of the aorta

126
Q

what are the obstructive congenital heart anomalies

A

congenital aortic stenosis, congenital pulmonic stenosis, coarctation of the aorta

127
Q

congenital heart disease: bp in upper extremity elevated and low in the lower extreity

A

coartation of the aorta

128
Q

hypertrophic osteoarthropathy is associated withw hat

A

Right to left shunts - clubbing fo fingers (tips of fingers expand and blunt)