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Pathology > Renal 3 > Flashcards

Renal 3 Flashcards

1
Q

is a hereditary disorder characterized by multiple expanding cysts of both kidneys that ultimately destroy the renal parenchyma and cause renal failure.

A 
AUTOSOMAL DOMINANT (ADULT) POLYCYSTIC KIDNEY 
DISEASE
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2
Q

Inheritance pattern ADKPD

A

autosomal dominant

with high penetrance

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3
Q

ADKPD bilateral or unilateral?

A

Bilateral

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4
Q

PKD1 gene is located on

A

chromosome 16p13.3

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5
Q

Encodes an integral membrane protein named

polycystin-1.

A

PKD1 gene

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6
Q

Polycystin-1 is expressed in tubular epithelial cells,

particularly those of the

A

distal nephron

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7
Q

Located on chromosome 4q21.

A

PKD2 gene

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8
Q

PKD2 gene Encodes an integral membrane protein named _______

A

polycystin-2.

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9
Q

polycystic disease pathogenesis

A

ciliacentrosome complex

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10
Q

Act as mechanosensor to monitor changes

in fluid flow and shear stress.

A

single non-motile primary cilium.

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11
Q

Regulate ion flux and cellular behavior,

including cell polarity and proliferation

A

single non-motile primary cilium.

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12
Q

Gross polycystic kidney disease

A

● Kidneys are bilaterally and large

- External: mass of cysts with no intervening parenchyma

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13
Q

Microscopic polycystic disease

A
  • The cysts may be filled with a clear, serous fluid or
    with turbid, red to brown, sometimes hemorrhagic fluid.
    -The cysts arise from the tubules throughout the
    nephron and therefore have variable lining
    epithelia
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14
Q

clinical features polycystic disease

A
  • asymptomatic (many patients)
  • renal colic
  • dragging sensation
  • insidious onset of hematuria
  • proteinuria, polyuria, hypertension
  • PKD2: older age, later devt of renal failure
  • tend to have extrarenal congenital anomalies.
  • azotemia- no symp
  • Uremia- w/ symp
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15
Q

most common ARPKD

A

Perinatal, neonatal

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16
Q

ARPKD is mostly caused by mutations in the

A

PKHD1 Gene

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17
Q

PKHDA1 gene is located at

A

chromosome region 6p21-p23

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18
Q

PKHDA1 encodes for

A

fibrocystin

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19
Q

Gross ARPKD

A
  • kidneys are enlarged and have a smooth external appearance

- sponge-like appearance

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20
Q

microscopic ARPKD

A

-Cylindrical or less commonly saccular dilation of all
collecting tubules
-The cysts have a uniform lining of cuboidal
cells, reflecting their origin from the collecting ducts.
-In almost all cases, the liver has cysts associated
with portal fibrosis and proliferation of portal bile
ducts.

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21
Q

CLINICAL FEATURES ARPKD

A

-peculiar hepatic injury characterized by bland periportal fibrosis and
proliferation of well-differentiated biliary ductules, now termed congenital hepatic fibrosis
-older children: portal hypertension with splenomegaly

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22
Q

T or F
Congenital hepatic fibrosis sometimes occurs in the
absence of polycystic kidneys or has been reported
in the presence of adult polycystic kidney disease.

A

T

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23
Q

2 categories of CYSTIC DISEASES OF RENAL MEDULLA

A
  1. Medullary Sponge Kidney

2. Nephronophthisis

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24
Q

medullary sponge kidney is restricted to multiple cystic dilatations of the

A

collecting ducts in the medulla

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25
Q

MEDULLARY SPONGE KIDNEY

On gross inspections, the papillary ducts in the medulla are

A

dilated, and small cysts may be

present.

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26
Q

Medullary sponge kidney

-Cysts are lined by

A

cuboidal epithelium or occasionally by transitional epithelium

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27
Q

This group of progressive renal disorders is
characterized by a variable number of cysts in the
medulla, usually concentrated at the
corticomedullary junction.

A

NEPHRONOPHTHISIS

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28
Q

is the cause of

the eventual renal insufficiency

A

cortical tubulointerstitial damage

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29
Q

Three Variants of Nephronophthisis disease complex

are recognized:

A
  1. Sporadic, non-familial
  2. Familial juvenile nephronophthisis (most common)
  3. Renal-Retinal dysplasia (15%)
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30
Q

NEPHRONOPHTHISIS familial forms

A

autosomal recessive traits

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31
Q

most common genetic cause of ESRD in

children and young adults.

A

As a group, nephronophthisis complex

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32
Q

CLINICAL FEATURES OF NEPHRONOPTHISIS

A
  • Polyuria and polydipsia, which reflect a marked defect in the concentrating ability of renal tubules.
  • Sodium wasting and tubular acidosis
  • Some syndromic variants
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33
Q

syndromic variants nephronophthisis

A

Ocular motor abnormalities, retinal dystrophy, liver fibrosis and cerebellar abnormalities.

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34
Q

Nephronophthisis Expected course is ____

A

progression to ESRD in 5 to 10 years

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35
Q

mutated in the juvenile forms of nephronophthisis

A

, NPHP1 to

NPHP11, JBTS2, JBTS3, JBTS9, JBTS11

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36
Q

encode proteins in nephrocystins

A

NPHP1 to NPHP11

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37
Q

Kidneys are small, have contracted granular
surfaces, and show cysts in the medulla, most
prominently at the corticomedullary junction

-small cysts in the cortex

A

nephronophthisis

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38
Q

T or F

In nephronophthisis, glomerular structure is preserved

A

T

39
Q

T or F

Multicystic renal dysplasia can be unilateral or bilateral. The kidney is usually enlarged, extremely irregular, multicystic

A

T

40
Q

Cardinal feature of Multicystic renal dysplasia

A

presence of islands of undifferentiated
mesenchyme, often with cartilage, and
immature collecting ducts.

41
Q

Unilateral renal dysplasia

A

may mimic and lead to surgical exploration and nephrectomy

42
Q

bilateral multicystic renal dysplasia

A

renal failure may ultimately result

43
Q

in acquired cystic disease, the cyts are lined by ______

A

either hyperplastic or flattened tubular epithelium, and often contain calcium oxalate crystals.

44
Q

simple cysts

A

translucent, line by a gray, glistening, smooth membrane, and filled with clear fluid

45
Q

Simple cysts micro

A

single layer of cuboidal or flattened cuboidal epithelium (no clinical significance)

46
Q

T or F

Obstructive lesions of the urinary tract increase susceptibility to infection and to stone formation

A

T

47
Q

T or F

Unrelieved obstruction almost always lead to permanent renal atrophy, termed hydronephrosis or obstructive uropathy

A

T

48
Q

Dilatation of the renal pelvis and calyces associated with progressive atrophy of the kidney due to obstruction to the
outflow of urine

A

Hydropnephrosis

49
Q

when the obstruction is sudden and complete, it leads to

A

mild dilation of the pelvis and calyces and sometimes to atrophy of the renal parenchyma

50
Q

When the obstruction is subtotal or intermittent,

A

progressive dilation ensues, giving rise to hydropnephrosis

51
Q

chronic cases of urinary obstruction

A

cortical tubular atrophy with marked diffuse interstitial fibrosis

52
Q

calculi lodged in the ureters may give rise to

A

renal colic

53
Q

is a useful noninvasive technique of obstructive uropathy

A

Ultrasonography

54
Q

earliest manifestation of bilateral partial obstruction

A

inability to concentrate urine reflected by polyuria and nocturia

55
Q

complete bilateral obstruction of rapid onset:

A

Results in oliguria or anuria and is incompatible with survival unless the obstruction is relieved.

56
Q

T or F

In urolithiasis, men are affected more than women, and the peak age at onset is between 20 and 30 years

A

T

57
Q

characterized by excessive production and excretion of stone-forming substances

A

inborn errors of metabolism such as cystinuria and primary hyperoxaluria

58
Q

4 main types of calculi

A
  • calcium stones
  • Triple stone/ struvite stone
  • Uric acid stones
  • Cystine
59
Q

composed largely of calcium oxalate or calcium oxalate mixed with calcium phosphate

A

calcium stones

-most common

60
Q

composed of magnesium ammonium phosphate

A

Triple stone or struvite stone

61
Q

most important determinant (stones)

A

increased urinary concentration of the stones’ constituents

62
Q

The mechanism of stone formation in this setting involves

A

‘nucleation’ of calcium
oxalate by uric acid crystals in the
collecting ducts.

63
Q

Formed largely after infections by urea-splitting
bacteria (e.g., Proteus and some Staphylococci)
that converts urea to ammonia

A

MAGNESIUM AMMONIUM PHOSPHATE STONES

64
Q

occupying the large
portions of the renal pelvis are frequently a
consequence of infection.

A

staghorn calculi

65
Q

Common in individuals with hyperuricemia, such
as patients with gout, and diseases involving rapid
cell turnover, such as the leukemias and patients
undergoing chemotherapy

A

uric acid stones

66
Q

urine of pH below ___ may predispose to uric

acid stones

A

5.5

67
Q

T or F

uric acid stones are radiolucent

A

T

68
Q

Caused by genetic effects in the renal
reabsorption of amino acids, including cystine,
leading to cystinuria

A

cystine stones

69
Q

urolithiasis most favored sites

A

Renal calyces and pelvis and in the bladder

70
Q

most common malignant tumor is

__________ followed by _______

A

renal cell carcinoma; Wilms tumor

71
Q

Small, discrete adenomas arising from the renal

tubular epithelium

A

renal papillary adenoma

72
Q

Benign neoplasms

A

A. Renal Papillary adenoma
B. Angiomyolipoma
C. Oncocytoma

73
Q

Malignant neoplasms

A

A. Renal cell carcinoma

B. Urothelial carcinoma of the renal pelvis

74
Q

● These small tumors are less than 1.5 cm in diameter
● They are present invariably within the cortex and
appear grossly as pale yellow-gray, discrete, and
well-circumscribed nodules

A

Renal papillary adenoma

75
Q

microscopic appearance of renal papillary adenoma

A

cells are cuboidal to polygonal in shape and have regular, small central nuclei, scanty cytoplasm, and no atypia

76
Q

Consists of vessels, smooth muscle, and fat originating from perivascular epithelioid cells

A

Angiomyolipoma

77
Q

● The clinical importance of angiomyolipoma is due

largely to their susceptibility to

A

spontaneous hemorrhage

78
Q

disease caused by loss-of-function
mutations in the TSC1 or TSC2 tumor suppressor
genes.

A

tuberous sclerosis

79
Q

● Epithelial neoplasm composed of large eosinophilic
cells having small, round, benign-appearing nuclei
that have large nucleoli.

A

Oncocytoma

80
Q

Oncocytoma thought to arise from the

A

intercalated cells of collecting ducts

81
Q

Oncocytoma gross appearance

A

Tumors are tan or mahogany brown, relatively

homogenous, and usually well encapsulated with a central scar in one-third of causes.

82
Q

T or F

Most renal cancer is sporadic, but unusual forms
of autosomal dominant familial cancers occur,
usually in younger individuals.

A

T

83
Q

CLASSIFICATION OF RENAL CELL CARCINOMA:

A
  • Clear cell carcinoma
  • Papillary carcinoma
  • Chromophobe carcinoma
  • Xp11 Translocation carcinoma
  • Collecting duct (bellini duct) carcinoma
84
Q

most common type of renal cell carcinoma

-made up of cells with clear or granular cytoplasm and are non papillary.

A

Clear cell carcinoma

most: sporadic but can be familial

85
Q

associated with VHL syndrome, there is loss of sequences on the short arm of chromosome 3

A

Clear cell carcinoma

86
Q

characterized by a papillary growth

pattern

A

Papillary carcinoma

87
Q

most common cytogenic abnormalities in papillary carcinoma are

A

Trisomies 7 and 17 and loss of Y in male patients in the sporadic form, and trisomy 7 in the familial form.

88
Q

T or F

Unlike clear cell carcinomas, papillary
carcinomas are frequently multifocal in
origin

A

T

89
Q

composed of cells with prominent cell
membranes and pale eosinophilic
cytoplasm, usually with a halo around
the nucleus.

A

Chromophobe carcinoma

90
Q

● genetically distinct (uncommon) subtype of
renal cell carcinoma.
● It often occurs in young patients

A

Xp11 Translocation carcinoma

91
Q

defined by translocations of the TFE3 gene

A

Xp11 translocation carcinoma

92
Q

How do we differentiate it with papillary CA?

A

In contrast with the cytoplasm of papillary CA, Xp11 translocation CA has a clear cytoplasm.

93
Q

Histologically these tumors are
characterized by malignant cells forming
glands enmeshed within a prominent
fibrotic stroma, typically in a medullary
location.

A

Collecting duct (bellini duct) carcinoma

94
Q

most reliable clinical feature of renal cell carcinoma

A

hematuria

Pathology (31 decks)

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