Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

22. CPR Exam 2 > 5. Renal Path 3 > Flashcards

5. Renal Path 3 Flashcards

1
Q

Agenesis of the kidneys is uncommon: bilaterally it is incompatible with life due to oligohydramnios causing potter’s sequence. unilateral agenesis is compatible with life but the kidney will undergo what?

A

hypertrophy and become enlarged, and increased risk of HTN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Hypoplasia is failure of kidneys to develop to normal size, bilateral and unilateral(more common) are both rare, typically due to low birth weight and inc risk for chronic kidney disease, may cause torsion or obstruction of ureter predisposing them to?

A

pyelonephritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What type of kidney is not present at t10-L2 retroperitoneally, but present somewhere along the normal path of the ureter from kidney to bladder?

A

Ectopic kidney

MC site is pelvic brim associated with ureteral abnormalities, infection/obstruction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What type of kidney is common, and caused by fusion of the kidneys producing a continous structure across the anterio of the aorta and IVC, *more commonly fused at lower poles (90%), *get caught by inferior mesenteric artery?

A

Horseshoe kidney

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Renal cysts are common, more than 50% of those over 50 have cysts in the parenchyma, which are often small and asymptomatic and are generally incidental findings but sometimes can become?

A

quite large

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

When comparing unilateral to bilaterial renal cystic disease, what is the major deciding factor between bilateral and unilateral dz?

A

Bilateral are Hereditary/familiar

Unliateral are more commonly acquired

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is an autosomal dominant cystic kidney disease which is a hereditary disorder characterized by multiple expanding cysts of both kidneys that ultimately destroy the renal parenchyma and cause renal failure?

A

AD (adult) polycystic kidney disease (ADPKD)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

AD (adult) polycystic kidney disease (ADPKD) begins in the 30-40s and definitely by age 70, the kidneys are markedly enlarged and may weigh up to 4kg each (15-20 times normal size), the cysts ultimately do what?

A

replace all functional cortex but scattered remnants of nephron units may be seen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

AD (adult) polycystic kidney disease (ADPKD) is an AD inheritance that still needs a ‘second hit’ in both alleles of PKD gene. What percent of people have defective PKD1 gene on chr 16, encodes for polycystin 1 (memebran glycoprotein) and have a higher change of ESRD?

A

85% of patients have the PKD1 on chr16 mutation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

the second mutation occurs in 15% of patients, defective PKD2 gene on chr 4, it codes for polycystin 2 which functions as?

A

Ca2+ permeable cation channel

Note: better prognosis cause lesser chance of ESRD than PKD1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

AD (adult) polycystic kidney disease (ADPKD) occurs in individuals of northern european ancestry. The cysts may be filled with clear, serous fluid or with turbid?

A

red to brown fluid (hemorrhagic fluid)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

The though path of ADPKD is mutations in polycystin 1/2 causes altered tubular cilia and altered calcium flux, causing altered tubular epithelial growth and differentiation, resulting in abnormal ECM, cell proliferation, fluid secretion which results in?

A

Cysts! causing interstitial inflammation and fibrosis as well as glomerular vascular damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

ADPKD is most common in northern europeans and has accelerated progression in black males with sickle cell or HTN, usually presents in 4/5/6th decade with?

A

renal insufficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

AD (adult) polycystic kidney disease (ADPKD) may exhibit abdominal pain due to cyst enlargement and hemorrhage thus leading to what presentation?

A

hematuria (dont confuse with bladder dz!)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

There is often more aggresive/earlier onset in patients who are male with HTN (AA), there is overall an increase in UTI and?

A

nephrolithiasis in these patients

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

There are commonly extra-renal manifestations with AD (adult) polycystic kidney disease (ADPKD); 40% have heptaic cysts, with other organs as well but lower frequency, 4-10% die from subarachnoid hemorrhage due to?

A

ruptured berry aneurysms of the circle of willis (basilar SAH in young patient = ADPKD)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

AD (adult) polycystic kidney disease (ADPKD) presents with mitral valve prolapse 25% of the time and 82% of patients have what disease of the colon?

A

Diverticular disease of the colon

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is an autosomal recessive disease which is the childhood form of ADPKD?

A

AR polycystic kidney disease (ARPKD)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

in most cases AR polycystic kidney disease (ARPKD) is cause by what defective gene on chromosome 6, which encodes a large novel protein fibrocystin-but there are many other mutations?

A

PKHD1 on chr 6

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Mutations in fibrocystin disrupt the collecting tubule and biliary eptihelium differentiation. Grossly, they are slightly enlarged, smooth surface, with numerous small 1-2mm linear/radial arrayed?

A

cysts derived from dilated collecting ducts.

sponge appearance when cut

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

AR polycystic kidney disease (ARPKD) is highly fatal in infancy, childhood form shows smaller cysts at right angle to cortical surface, survivors develop congenital?

A

hepatic fibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

There are four different types of AR polycystic kidney disease (ARPKD), including perinatal, neonatal, infantile, and jevenile, which are the most common and what are the prognoses?

A

Most common perinatal and neonatal - all die relatively quickly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What are the two types of medullary cystic diseases, including nephonopthisis cystic disease and?

A

medullary sponge kidney (benign)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What kidney cysts occur in adults with unknown pathogenesis, found incidentally, scarring is absent, with cystic dilations that consist of cuboidal/transitional epithelium from collecting tubules (benign)?

A

Medullary sponge kidney

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What is a group of progressive diseases that are characterized by variable amounts of cysts in the medulla, typically concentrated at the corticomedullar junction?

A

Nephronophthisis and adult onset medullary cystic disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Nephronophthisis and adult onset medullary cystic disease has 3 forms, sporadic, familial/juvenile (MC-AR), and renal retinal dysplasia which generally present initially with polyuria and?

A

polydypsia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Nephronophthisis and adult onset medullary cystic disease as a group are considered to be the MC *genetic cause of ESDR in?

A

adolescents and young adults

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

in Nephronophthisis and adult onset medullary cystic disease, renal failure occurs secondary to progressive cortical tubulointerstitial damage, what are the two mutations known to cause this disease?

A

MCKD1 and MCKD2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Nephronophthisis and adult onset medullary cystic disease has small kidneys with contracted granular surfaces, large cysts at the cortico-medullary junction and small cysts?

A

in the cortex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

what is a sporadic disorder that can be unilateral or bilarteral and almost always cystic. In unilateral dysplasia may mimic neoplasm and lead to surgical exxploration - excellent prognosis?

A

multicystic renal dysplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

multicystic renal dysplasia is also bilateral which causes renal failure and has a bad prognosis. The kidneys are enlarged, extremely irregular and multi cystic, presence of islands of undifferentiated mesenchyme with cartilage and immature collecting ducts, clinical respents as???

A

adbominal mass

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

What is caused by end stage kidney disease with prolonged dialysis, cortical and medullary cysts that contain clear fluid but may bleed, whose cysts are lined by hyperplastic or flattened tubular epithelium with calcium oxalate crystals?

A

Acquired (dialysis assocatied) cystic dz

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

What is a clinically significant complication in the setting of calcium oxalate stones in a patient receiving dialysis?

A

renal cell carcinoma (7% chance in pts dialyzed for 10+years)***

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

What usually are single or multiple, occur on the renal cortical surface commonly 1-5cm, generally filled with clear fluid, sometime get mistaken for tumors ?

A

Simple renal cysts (benign)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Multicystic renal dysplasia in most cases have an absent ureter, ureteropelvic obstruction or other?

A

lower GU anomalies

rather common mass

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Obstructive lesions of the urinary tract increase chance of infection and to stone formation, and unrelieved obstruction almost always leads to permanent renal atrophy termed?

A

hydronephrosis or obstructive uropathy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Common causes of urinary tract obstructions include congenital anomalies such as posterior urethral valves, urinary calculi, BPH, pregnancy or even?

A

tumors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

What term is used to describe dilation of the renal pelvis and calyces associated with progressive atrophy of the kidney due to obstruction to the outflow of urine?

A

Hydronephrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

What type of obstruction may provoke pain from distention?

A

Acute obstruction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

What type of obstruction can remain silent for long times as the other kidney compensates?

A

Unilateral complete or partial hydronephrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

What type of obstruction causes the inability to concentrate urine (polyuria), distal tubular acidosis, renal salt wasting, renal calculi, and chronic tubulointerstitial nephritis with scarring and atrophy of the papilla and medulla, HTN is common?

A

Bilateral partial obstruction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

What type of obstruction causes oliguria/anuria, incompatible with survivial unless the obstruction is relieved, after removal of block-kidney must undergo diuressis where kidney excretes urine rich in NaCl?

A

Bilateral complete obstruction

43
Q

What affects 5-10% of americans, usually unilateral (80%), men affected more than women, during ages 20-30?

A

Urolithiasis/Nephrolithiasis

44
Q

The most important determinant of Nephrolithiasis is an increased urinary concentration of the stones constituents such that it exceeds their solubility in urine, aka ?

A

supersaturation

45
Q

Kidney stones become symptomatic when they enter the ureter and cause intense pain (renal colic), ulceration and bleeding of ureter mucosa or?

A

obstruction of urinary flow

46
Q

What is the most common type of stone, associated with hypercalciuria and with or without hypercalcemia, but when both are present, it is a result of hyperparathyroidism which can be a result of progressive kidney failure?

A

Calcium Oxalate stones (calcium and oxalate)

47
Q

What type of kidney stone caused by urea splitting bacteria (proteus/staph) forms the largest kidney stones called staghorn calculi- occupying renal pelvis?

A

Struvite stones (staghorn caused by infection)

48
Q

What type of stone is common in patietns who have hyperuricemia such as gout or leukemia, forming a LOW (acidic) urinary pH, you need to alkalinize the urine to solubulize the stone and pee it out (give bicarb)?

A

Uric acid stones (radiolucent unlike Ca stone)

49
Q

What type of stones are formed with genetic disease like cystinuria that prevent reabsorption of proteins from the lumen, *form at low / acidic urinary pH?

A

Cystine Stones

50
Q

What is the most common benign renal neoplasm, which like most other b9 lesions rarely cause clinical problems and are discovered incidentally, and are small (less than cm) but sometimes like oncocytomas or angiomyolipomas can measure 10cm+?

A

Renal papillary adenoma

51
Q

Renal papillary adenoma is defined by a size of being less than 1.0cm, any neoplasms greater than 1cm with adenoma histomorphology are usually classified as?

A

low grade renal cell carcinoma

52
Q

Renal papillary adenomas are common incidentally found during autopy 40% in 70y/o+, RCC is relatively uncommon, grossly you can see a cortical, discrete, yellow-grey which may be?

A

small and multiple

53
Q

On LM Renal papillary adenoma shows complex, branching papillomatous structures, cells are cuboidal- no atypia, thin fibrovascular cores, these lesions have cytogenic features of?

A

Trisome 7/17

54
Q

What neoplasm has a strong association with tuberous sclerosis (AD loss of TSC1/2), present in adults, middle aged, F>M?

A

Renal angiomyolipoma

55
Q

Renal angiomyolipoma are benign neoplasms consisting of vessels, smooth muscle, and fat from the perivascular epitheliod cells, sometimes they may rupture and have massive hemorrhage so initial presentation may be?

A

shock secondary to massive retroperitoneal and or intra-abdominal hemorrhage

56
Q

What is an eptihelial neoplasm/benign tumor composed of large eosinophillic cells with small round benign looking nuclei that have large nucleoli? (**massive amounts of mT)

A

renal oncocytoma

57
Q

renal oncocytoma arise from type A intercalated cells of renal cortical collecting ducts, typically present in adulthood, rarely familial (but if it is its bilateral), Clinically ddx with RCC and non-clear cell, it has rare?

A

invasion/metastasis

58
Q

renal oncocytoma are typically mahogany brown and well circumscribed, may be quite large (1o-15cm) may look like a RCC, but often has a central?

A

stellate scar

59
Q

On LM, renal oncocytoma has abundant acidophilic granular cytoplasm and may show alveolar, nesting, tubular or?

A

solid pattern

**EM packed with mT

60
Q

What can one expect on LM in renal angiomyolipoma?

A

thick waled blood vessels (angio)
smooth muscle (myo)
fat (lipoma)

61
Q

What is an adenocarcinoma of the kidney that is unusual but MC adult kidney cancers seen in 6-8th decade, 2:1 MtoF?

A

Renal cell carcinoma RCC

62
Q

Renal cell carcinoma RCC are mainly idiopathic with some familial mutations to consider, arise from renal tubular epithelium, what is the greatest risk factor, and others including obesity, HTN, estrogen, asbestos?

A

SMOKING CIGARETTES

63
Q

in Renal cell carcinoma RCC the poles are most commonly affected and 4% are hereditary, AD, seen in younger patients. What syndrome causes most hereditary RCCs, causing bilateral RCC and cysts with cerebellar involvement!?

A

Von Hippel Lindau VHL syndrome

64
Q

What is an AD disorder with mutations in FH gene (fumarate hydratase) causing cutaneous and uterine leiomyomata with an aggressive type of papillary carcinoma with icnreased risk for metastatic spread?

A

Hereditary Leiomyomatosis and RCC syndrome

65
Q

What is AD caused my mutations in MET proto-oncogene, resulting in many bilateral tumors with papillary histology?

A

Hereditary papillary carcinoma

66
Q

What is the MC type of RCC, 95% sporadic, 98% of familial caused by a loss on chr 3 where the VHL gene is, causing unliateral tumors?

A

Clear Cell carcinoma

67
Q

Clear Cell carcinoma form from proximal tubular epithelium presenting with large clear or granular cells, bright yellow-grey- white spherical mass, they are likely to invade the renal vein and may go to the heart, causes what if it is on the left?

A

varicocele

68
Q

What type of RCC is multifocal in origin with a better prognosis, less common, associated with Trisomy 7, 16, 17 and loss of Y in males (MET proto oncogene), associated with dialysis associated cystic disease?

A

Papillary carcinoma

69
Q

On LM in Papillary carcinoma, they are cuboidal or columnar epithleium arranged in papillary pattern that came from distal convoluted tubules, there is little strom but is highly vascularized, with what type of cells in the core?

A

foam cells are common in the core

70
Q

What type of RCC is uncommon and has a pale eosinophilic cytoplasm, nuclear halos, arising from type B intercalated cells of renal cortex collecting ducts?

A

Chromophode renal carcinoma

71
Q

Chromophode renal carcinoma have excellent prognosis but may be hard to differentiate from?

A

oncocytoma (grow from intercalated cells as well)

72
Q

What type of carcinoma occurs in young patients due to translocations at the TFE3 gene and have cells with clear cytoplasm with papillary architecture in the medulla?

A

Xp11 translocation carcinoma

73
Q

In sporadic papillary carcinoma, it is one lesion associated with trisomy 7,16,17 Loss of Y and mutated/activated MET, What is seen in hereditary papillary carcinoma?

A

multiple lesions due to trisomy 7 and mutated MET

74
Q

In sporadic clear cell carcinoma there are deletions on chr3, loss of VHL, with one lesion only, in hereditary which accounts for 4% of clear cell carcinomas, what can be seen?

A

MULTIPLE lesions due VHL loss

75
Q

RCC is often asymptomatic, discovered by CT or MRI for nonrenal cause, may reach 10cm before symptoms occur. What is the common triad of symptoms?

A

Hematuria** (50%) microscopic only
Costovertebral pain (20%)
Palpable flank mass (10%)

76
Q

RCC tend to reach large sizes and metastasize before local signs and symptoms, then generalized non specific symptoms occur such as malaise, weakness, fever and?

A

weight loss

77
Q

25% of RCC patients have evidence of metastatic disease at the time of initial diagnosis to lung bone LN, liver, adrenal and brain. RCC is known as a great mimic for what?

A

it has many paraneoplastic syndromes ***

78
Q

RCC has paraneoplastic syndromes such as polycythemia, hypercalcemia, HTN, cushings, leukemoid rxn, feminization/ masculization and?

A

amyloidosis

79
Q

Major factors in prognosis include stage (NOTE: spreads hematogenously not via LN) and histologic type; chromophobe is good prognosis, papillary and clear cell are average and what are worse?

A

collecting duct
sarcomatoid
medullary

80
Q

RCC typicall travels up the inferior vena cava to the heart, where it usually extends from the IVC into?

A

the wall of the right atrium

81
Q

RCC clear cell type aka hypernephroma, shows optically clear cytoplasm and sharply delineated cell membrane, clear cytoplasm is due to glyocgen and?

A

lipid accumulation

PAS+, OilRedO+, Sudan Black +

82
Q

What is a defining characterist of chromophobe renal cell carcinomas along with thewell defined cell membranes, faintly granular cytoplasm?

A

perinuclear clear halos!

83
Q

What rare type of RCC is composed of spindle cells simulating a mesenchymal neoplasm, and if this component arises in any RCC it implies poor prognosis?

A

Sarcomatoid renal cell carcinoma

84
Q

What rare type of RCC shows branching tubules lined by highly atypical cuboidal cells?

A

Collecting duct carcinoma

85
Q

What carcinoma originates from the urothelium of the renal pelvis and may be multiple and 50% of patients have concomitant bladder tumors, associated with analgesic and balkan (tubulointerstitial) nephropathy?

A

urothelial (transitional cell) carcinoma of the kidney

86
Q

urothelial (transitional cell) carcinoma of the kidney is usually smll when discovered, may block urinary outflow and lead to palpable hydronephrosis and flank pain, infiltration of the wall of pelvis/calyces is frequent with what clinical symptom?

A

Painless Hematuria (general poor prognosis)

87
Q

What is the biggest risk factor for Urothelial carcinoma of the pelvis?

A

SMOKING

88
Q

What tumor is common in asians>whites> blacks, 4th most common childhood malignancy with peak between 2-5 years, discovered as a large abdominal mass with pain, microscopic hematuria, and HTN?

A

Wilms Tumor (5-10 percent are bilateral)

89
Q

10% of patients associated with specific types of congenital malformations or familial syndromes, chr11 abnrl, WT1 mutations or loss/2nd hit. What syndrome has wilms tumors 30-44% of the time along with wilms, aniridia, genital problems and retardation?

A

WAGR (chr11-WT1 mutation)

90
Q

What syndrome which has wilms tumors 90% of the time also presents with gonadal and renal tumors, associated with chr11?

A

Denys-Drash Syndrome (chr11 WT1 mutation)

91
Q

What syndrome is a non-WT1, poss WT2 syndrome with IGF 2?

A

Beckwith-Wiedemann Syndrome

92
Q

If not one of the three syndromes, wilms tumors are previously health children with no congenital abnormalities or syndromes, 10% have WT1 mutation and 10% have?

A

B-catenin mutation

93
Q

What syndrome is most commonly observed genitourinary malformation in affected males =undescented testes whereas females may be affected with streak gonads or uterine malformations?

A

WAGR

94
Q

What syndromic wilms tumor will most commonly have gonadal tumors too?

A

Denys-Drash

95
Q

What syndromic wilms tumor will have hemihypertrophy as well as macroglossia?

A

Beckwith Wiedemann Syndrome

96
Q

Wilms tumors on LM are associated with nephrogenic rests (precursor lesion)- 25-40% are unilateral and 100% bilateral- meaning look out for that other kidney cause its probs bad too! What are the two types of histomorphology?

A 
Typical triphasic (favorable)
Anaplastic (unfavorable)
97
Q

Typical triphasic has blastemal (small blue cells densely packed), epithlial tubules, and stromal cells (spindle shaped), the majority have all three cell types (triphasic) monophasic variants therefor may present diagnostic difficulties. There is no significant?

A

anaplasia

98
Q

Anaplastic histo (bad) may be focal or diffuse with extreme cellular pleomorphism and atypia, focal anaplasia doesnt always mean poor prognosis, diffuse does. Anaplasia tends to be associated with p53 mutations and resistance to?

A

chemotherapy

99
Q

Wilms tumors grossly are well circumscribed, tan to gray color, large expansil tumor usually in what part of the kidney?

A

The lower pole of the kidney

100
Q

What is the most critical prognostic element for wilms tumors of the kidney?

A

whether there is presence or absence of diffuse anaplasia (BAD)

101
Q

What are the four MC malignant neoplasms of infancy?

A

leukemia
retinoblastoma
neuroblastoma
Wilms tumor

102
Q

Are the following characteristics favorable or unfavorable for neuroblastomas?
Stage 1,2A,2B,4S
<18months
Scwannian stroma and ganliocytic different.
mitosis-karryorrhexis < 200/5000cells
Hyperdiploidy
TRkA expression present

A

Favorable

103
Q

Are the following characteristics favorable or unfavorable for neuroblastomas?
Stage 3/4
older than 18months
Scwannian stroma and ganliocytic differentiation not present
mitosis-karryorrhexis >200/5000cells
near diploid (segmental chromosomal losses w chromothripsis)
MYCN amplified
Chr 1p loss
Chr 11q loss
TRKB expression
Mutations of neuritogenesis genes

A

BAD- Unfavorable

104
Q

Metastasis to the kidney is uncommon and often a terminal even, frequently multifocal and bilateral seen from lung, melanoma, breast, GI and ?

A

Pancreas

22. CPR Exam 2 (20 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions