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URN Biweekly 2 > Congenital and Cystic Diseases of the Kidney- Herrera > Flashcards

Congenital and Cystic Diseases of the Kidney- Herrera Flashcards

1
Q

kidney that didn’t form correctly

A

hypoplastic

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2
Q

kidney that formed correctly but then became smaller

A

atrophy

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3
Q

best thing to do to dx cystic kidney disease

A

IMAGING

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4
Q

associated with bilateral renal agenesis

A

Potter Syndrome

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5
Q

no scarring of kidney and small, never made it to normal size

A

renal hypoplasia

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6
Q

due to migration issues of kidneys and can lead to infections

A

ectopic kidney

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7
Q
A

ectopic kidney

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8
Q

malrotated kidneys and kinking can cause what

A

infection

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9
Q

due to fusion (inferior mesenteric a.)
can lead to infection
propensity to rupture in accidents

A

horsehoe kidney

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10
Q

cystic disease that is almost always unilateral

A

multicystic renal dysplasia

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11
Q

dilation due to backup of urine

A

hydronephrosis

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12
Q

extremely common cystic disease
cortex usually affected

A

simple renal cyst

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13
Q
A

simple renal cyst

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14
Q
A

simple renal cysts

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15
Q
A

simple cysts (remember you can’t really dx with microscopy–the cysts look the same)

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16
Q

unilateral
associated with ureteral atresia
embryologic problem

A

renal dysplasia

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17
Q

NOT premalignant, just embryologic problem

A

renal dysplasia

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18
Q

mesenchyme/cartilage tissue seen on histology

A

renal dysplasia

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19
Q
A

renal dysplasia

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20
Q

multicystic and unilateral

A

renal dysplasia

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21
Q

mesynchyme seen; cysts seen

A

renal dysplasia

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22
Q
A

renal dysplasia (cartilage seen and cysts seen)

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23
Q

cysts and cartilage

A

renal dysplasia

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24
Q

autosomal dominant (AD)
bilateral
HTN, pain, hematuria

A

Adult polycystic renal disease (ADPKD)

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25
Q

what causes HTN in patient in w/ polycystic kidney disease

A

RAAS activated—kidneys underperfused
and cysts constricting renal a.

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26
Q

10-30% of people with this will have aneurysms in brain

A

adult polycystic renal disease (ADPKD)

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27
Q

genes most commonly affected for adult polycystic kidney disease

A

PKD1 and PKD2

28
Q

sometimes associated w/ carcinomas and aneurysms

A

adult polycystic kidney disease (APKD)

29
Q
A

ADPKD

30
Q
A

ADPKD

31
Q
A

ADPKD

32
Q
A

ADPKD

33
Q
A

ADPKD

34
Q
A

ADPKD

35
Q

cysts and fibrosis

A

ADPKD

36
Q

PKD1 gene mutation involved with what being defective

A

cilia

37
Q

mechanoreceptors
controls tubule when it starts expanding to stop
mutated they can’t stop tubule from growing

A

cilia (seen in ADPKD—PKD1)

38
Q

can involve cysts in other organs, not as prominent as the ones in the kidney; mitral valve prolapse

A

ADPKD

39
Q
A

ADPKD

40
Q
A

ADPKD

41
Q

aneurysm in brain think

A

ADPKD

42
Q

best way to diagnose PCKD

A

ultrasound

43
Q

autosomal recessive (AR)
manifestations at birth
a/w congenital hepatic fibrosis

A

childhood polycystic renal disease

44
Q

chromosome 6 mutation
a/w potter syndrome and pulmonary hypoplasia sometimes

A

childhood polycystic kidney disease

45
Q

rare
AR
a/w congenital hepatic fibrosis

A

CPKD (ARPKD)

46
Q
A

ARPKD (CPKD)

47
Q

infant’s kidney

A

ARPKD

48
Q

seen from child’s kidney

A

CPKD (ARPKD)

49
Q

prolonged dialysis use

A

acquired (dialysis-associated) polycystic kidney disease

50
Q

hyperplastic changes in cysts
increased chance of malignancy

A

acquired polycystic kidney disease

51
Q

on dialysis for 3 years, and in 80-90% OF PATIENTS ON DIALYSIS FOR 10 YEARS

A

acquired polycystic kidney disease

52
Q

a hyperplastic change in cyst

A

acquired polycystic kidney disease

53
Q

all the cysts are in the medulla
minor problems

A

medullary sponge kidney

54
Q

cysts in cortical-medullary junction and medulla
significant problems

A

nephronophthisis

55
Q

AR
cilia related disease
childhood/teen onset

A

nephronophthisis

56
Q

AD
not seen w/ US
found on autopsy

A

medullary cystic kidney disease

57
Q

cysts in medulla (collecting ducts)
complications include stones and infections

A

medullary sponge kidney

58
Q
A

medullary sponge kidney

59
Q

AR
childhood/teen onset
NPH1 mutation

A

Nephronophthisis

60
Q

AD
adult onset
MCKD1 mutation

A

medullary cystic kidney disease

61
Q

most common genetic form of ESRD in children

A

nephronophthisis

62
Q

gene responsible for renal form of nephronophthisis

A

NPH1

63
Q

cysts at cortical medullary junction

A

nephronophthisis

64
Q

retinal dysplasia is a/w ______ too

A

medullary cystic disease (AD)

65
Q

cysts at cortico-medullary junction

A

nephronophtisis (group 1 or 2)

66
Q

classical for what

A

nephronophthisis (group 1 or group 2)

67
Q

normal kidneys become atrophic and cysts seen

A

acquired cystic disease

URN Biweekly 2 (15 decks)

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