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1

What renal conditions area associated with sickle cell trait

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

2

Horse shoe kidney

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

3

Presentation of ADPKD

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)

4

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

ADPKD

5

Intracranial berry aneuysms are associated with what renal condition

ADPKD

6

Genetic component of ADPKD

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

7

how does the pathology of ADPKD differ from that of ARPKD

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

8

ADPKD differential Diagnosis RCC

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

9

"Tufting of papillary projections"

ADPKD

10

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

ARPKD

11

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

ARPKD. Pulmonary Hyperplasia is secondary to oligohydramnios

12

Clinical presnetation of ARPKD

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)

13

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

ARPKD

14

Buzz word: Fetal lobulation

ARPKD

15

MC cystic disease in children

Multicystic Dysplastic Kidney

16

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

Multicystic Dysplastic Kidney

17

what cystic condition is confined to the medullary pyramids and papillae

Medullary sponge kidney

18

How can you differntiate medullary sponge from PKD

Medullary sponge has intersitial fibrosis

19

Most common genetic cause of ESRD in children and young adults

Nephrophthisis

20

Genetic mutation in ARPKD

Polycystic Kidney Hepatic Disease gene (PKHD1)

21

what type of Nephrophthisis is most common

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

22

what cystic condition has cysts most prominent at the corticomedullary junction

Nephrophthisis

23

Polyuria, polydipsia, cardiac, musculoskeletal, and ocular disease

Nephrophthisis

24

what renal disease causes growth failure in children

nephrophthisis

25

Presnetation of Adult onset medullary cystic kidney disease

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

26

"tubular atrophy interspersed with hypertrophied and dilated tubules"

Adult onset medullary cystic disease

27

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

Acquired cystic renal disease

28

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

acquired cystic renal disease

29

Most common cystic abnormality of the kidney

Simple Cyst

30

Name the nephritic diseae

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

31

Buzz word: Subepithelial humps

Acute proliferative glomerulonephritis

32

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

Acute proliferative glomerulonephritis MCC = Group A strep pharyngitis

33

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

Acute proliferative glomerulonephritis

34

Cresents seen on biopsy

Rapid progressive Glomerular nephritis

35

RPGN gross morphology

kidney is large and pale with petichial hemorrage

36

most common type of GN world wide

IgA nephropathy

37

recurrent symptomatic hematurua after respiratory infection and absence of systemic disease

IgA nephropathy

38

Buzz word: Synpharyngitic macroscopic hematuria

igA nephropathy

39

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

iga nephropathy

40

Renal Disease, Sensorial Hearing loss, Ocular abnormalities

Alport Syndrome "Cant see, cant pee, cant hear a bee"

41

what differnetiates Aloprt Syndrome from IgA nephropathy

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

42

what renal condition is caused by abnormal type IV collagen

Alport Syndrome

43

what renal condition is assoaciated wth Hepatitis C

Membranoproliferative Glomerulonephritis

44

Buzz word: tram tracking

Membranoproliferative Glomerulonephritis

45

what types of eposits are seen in type I memranoproliferative GN

Subendithelual deposits between duplicated membranes

46

what type of deposits are seen in type II membranoproliferative GN

Intramembranous dense deposits = Ribbon like deposits

47

Name the nephrotic diseases

Membranous Nephropathy, Focal Segmental GN, Diabetic nephropathy Minimal change

48

Most commmon casye of adult nephrotic syndrome in adults and elderly

membranous GN

49

What glomerular disease is associated with malignacy

membranous GN

50

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

membranous GN

51

Most common GN leading to ESRD

Focal segmental GN

52

MCC of focal glomerulonephritis

Obesisty and anabolic steroid use

53

what would you see on biopsy of FSG

Hyalin insudation and lipid vacuoles in sclerotic areas

54

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

Diabetic Glomerulonephropathy

55

Buzz word: Nodular glomerulosclerosis or Kinnelstiel Wilson Disease

Diabetic Glomerulonephropathy

56

buzz word: effacement of podocytes, normal appearing glomerular biopsy

Minimal change disease

57

most common casue of acute renal failure

Acute tubular injury (tubular necrosis)

58

what sections of the tubules are sensitive to ischemia and toxins

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

59

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

Acute Tubular injury (tubular necrosis)

60

Biopsy of acute tubular injrut from ethylene glycol

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

61

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

Acute pyelonephritis

62

Buzz word: thyroidization

Chronic pyelonephrits

63

second most common cause of acute kidney injury after pyelonephritis

drug and toxin induced tublointersitial nephritis

64

Initial phase of Acute tubular injury

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

65

Maintainance phase of acute tubular injury

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

66

Recovery phase of acute tubular injury

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

67

How does the infiltrate differe in acute vs chronic tubular nephritis

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

68

what is the most common cause of acute pyelonephritis

Ascending infection (usually E.coli)

69

name the complications of acute pyelonephritis

Papillary Necrosis, Pyonephrosis, Perinephric abscess

70

what population is papillary necrosis normally seen in

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

71

describe the gross morphology of papillary necrosis

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

72

pyonephrosis

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

73

Acute Uric Acid Nephropathy

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

Buzz word: tumor lysis syndrome

Acute Uric Acid nephropathy

75

Chronic urate nephropathy

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

76

what renal conditions are associated with calcium phosphate deposition

Medullary Sponge Kidney and Hypercalcemia and Nephrocalcinosis

77

Buzz word: Bence Jones Protien

Light chain cast nephropathy (myeloma kidney)

78

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

Light chain cast nephropathy (myeloma kidney)

79

what is nephrosclerosis

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

Buzz word: Cortical surface described as grainy leather

Nephrosclerosis (fine even granularity)

81

Athromathous plaque vs fibromuscular dysplasia

fibromuscular dysplasia havs the lumen still in the center

82

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

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

83

Buzz word: Schistocytes

Thrombotic Microangiopathies - result from shearing of red blood cells

84

Most commin Genitourinary tumor in children

Wims (Nephroblastoma)

85

name the benign renal tumors found in adults

Renal Papillary Adenoma, Renal oncocytoma, Angiomyolipoma

86

Name the malignant renal tumors found in adults

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

MCC of ischemia

coronary atherosclerosis (90%)

88

MC Acute coronary syndrome

Angina pectoris

89

angina pectrois is most common in what population

middle aged men and women after menopause

90

most common variant of angina

Stable Angina

91

T/F Stable angina is associated with plaque disruption

False

92

What type of angina is relieved by rest or vasodilator

Stable angina

93

what typically causes unstable angina

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

94

Early response to biochemical response

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

95

loss of myocte contractility happens within what time frome

within 60 seconds

96

early changes are potentially reversible within what time period

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

97

Most MI are what type

Transmural

98

Progression of Myocardial Necrosisi

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

most common cause of death in adults in the US

MI

100

when do gross findings of MI first appear

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

101

infiltrate of abundant neutrophils are seen when (post MI)

1-3 days

102

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

7-10 days

103

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

begins at 2 weeks and is complete at 8 weeks

104

Buzz word: Contraction bands

indicative of reperfusion injury post MI

105

Creatinkin phosphokinase dimer specific for cardiac muscle

CKMB

106

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

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

107

what lab test is most sensitive and specific for myocardial damage

Troponin (T and I)

108

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

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

109

what morphological features are seen with chronic ischemic heart disease

left ventricular hypertorphy and ventricular dilation

110

congenital heart disease occurs when

3-8 weeks gestation

111

major known cause or congenital heart disease

sporadic genetic abnormalities

112

almost all congenital abnormalities predispose for what condition

bacterial endocarditis - abnormalities cause turbulent flowt that can damage endocardium

113

Most common congenital heart condition

Ventricular septal defect

114

what classification of shunt is cyanotic

Right to left

115

what classification of shunt is acyanotic

left to right

116

name the L to R disorders

"all have a D in them" ASD, VSD, PDA, AVDS

117

most common site of ASD

foramen ovale

118

most common congenital heart condition presenting in adulthood

ASD

119

name the R to L disorders

"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

what congenital heart disorder is associated with down syndrome

AVSD

121

4 characteristics of tetrology of fallot

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

122

buzz word: boot shaped heart

tetrology of fallot

123

infant turns blue when crying

tetrology of fallot

124

child squats during exercise

tetrology of fallot - squatting increases venous return to the heart

125

what congenital heart disorder is associated with women with turner syndrome

coartation of the aorta

126

what are the obstructive congenital heart anomalies

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

127

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

coartation of the aorta

128

hypertrophic osteoarthropathy is associated withw hat

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