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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

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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
what causes HTN in patient in w/ polycystic kidney disease
RAAS activated---kidneys underperfused and cysts constricting renal a.
26
10-30% of people with this will have aneurysms in brain
adult polycystic renal disease (ADPKD)
27
genes most commonly affected for adult polycystic kidney disease
PKD1 and PKD2
28
sometimes associated w/ carcinomas and aneurysms
adult polycystic kidney disease (APKD)
29
ADPKD
30
ADPKD
31
ADPKD
32
ADPKD
33
ADPKD
34
ADPKD
35
cysts and fibrosis
ADPKD
36
PKD1 gene mutation involved with what being defective
cilia
37
mechanoreceptors controls tubule when it starts expanding to stop mutated they can’t stop tubule from growing
cilia (seen in ADPKD---PKD1)
38
can involve cysts in other organs, not as prominent as the ones in the kidney; mitral valve prolapse
ADPKD
39
ADPKD
40
ADPKD
41
aneurysm in brain think
ADPKD
42
best way to diagnose PCKD
ultrasound
43
autosomal recessive (AR) manifestations at birth a/w congenital hepatic fibrosis
childhood polycystic renal disease
44
chromosome 6 mutation a/w potter syndrome and pulmonary hypoplasia sometimes
childhood polycystic kidney disease
45
rare AR a/w congenital hepatic fibrosis
CPKD (ARPKD)
46
ARPKD (CPKD)
47
infant's kidney
ARPKD
48
seen from child's kidney
CPKD (ARPKD)
49
prolonged dialysis use
acquired (dialysis-associated) polycystic kidney disease
50
hyperplastic changes in cysts increased chance of malignancy
acquired polycystic kidney disease
51
on dialysis for 3 years, and in 80-90% OF PATIENTS ON DIALYSIS FOR 10 YEARS
acquired polycystic kidney disease
52
a hyperplastic change in cyst
acquired polycystic kidney disease
53
all the cysts are in the medulla minor problems
medullary sponge kidney
54
cysts in cortical-medullary junction and medulla significant problems
nephronophthisis
55
AR cilia related disease childhood/teen onset
nephronophthisis
56
AD not seen w/ US found on autopsy
medullary cystic kidney disease
57
cysts in medulla (collecting ducts) complications include stones and infections
medullary sponge kidney
58
medullary sponge kidney
59
AR childhood/teen onset NPH1 mutation
Nephronophthisis
60
AD adult onset MCKD1 mutation
medullary cystic kidney disease
61
most common genetic form of ESRD in children
nephronophthisis
62
gene responsible for renal form of nephronophthisis
NPH1
63
cysts at cortical medullary junction
nephronophthisis
64
retinal dysplasia is a/w ______ too
medullary cystic disease (AD)
65
cysts at cortico-medullary junction
nephronophtisis (group 1 or 2)
66
classical for what
nephronophthisis (group 1 or group 2)
67
normal kidneys become atrophic and cysts seen
acquired cystic disease

URN Biweekly 2 (15 decks)

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