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
2
Q
Horse shoe kidney
A
90% of cases have fusion of upper pole - patients are usually asymptomatic/normally functioning
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)
4
Q
cysts in the liver, spleen and lungs are assocaited with what renal condition
A
ADPKD
5
Q
Intracranial berry aneuysms are associated with what renal condition
A
ADPKD
6
Q
Genetic component of ADPKD
A
PKD1 (85%) and PKD2- PKD2 presents later than PKD1
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
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
9
Q
“Tufting of papillary projections”
A
ADPKD
10
Q
what renal condition presents with dilated elongated channels at right angles to the cortical surface
A
ARPKD
11
Q
Newborn presents with severe respiratory distress and pulmonary hyperplasia - what renal condition
A
ARPKD. Pulmonary Hyperplasia is secondary to oligohydramnios
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)
13
Q
What cystic disorder is associated with portal hypertension (portal fibrosis)
A
ARPKD
14
Q
Buzz word: Fetal lobulation
A
ARPKD
15
Q
MC cystic disease in children
A
Multicystic Dysplastic Kidney
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
17
Q
what cystic condition is confined to the medullary pyramids and papillae
A
Medullary sponge kidney
18
Q
How can you differntiate medullary sponge from PKD
A
Medullary sponge has intersitial fibrosis
19
Q
Most common genetic cause of ESRD in children and young adults
A
Nephrophthisis
20
Q
Genetic mutation in ARPKD
A
Polycystic Kidney Hepatic Disease gene (PKHD1)
21
Q
what type of Nephrophthisis is most common
A
Familial (juvenile) - becomes clinically evident in childhoood or adolescence.
22
Q
what cystic condition has cysts most prominent at the corticomedullary junction
A
Nephrophthisis
23
Q
Polyuria, polydipsia, cardiac, musculoskeletal, and ocular disease
A
Nephrophthisis
24
Q
what renal disease causes growth failure in children
A
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)
