Pediatric Kidney and Urinary Tract Flashcards

1
Q

In fetal development, the kidneys are _________. As maturation proceeds, more parenchyma grows to even out the dips.

A

lobular

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2
Q

What is the incidence of unilateral and bilateral renal agenesis?

A

Unilateral: 1/1000
Bilateral: 1/5000 (lethal)

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3
Q

Renal ectopia results from __________.

A

failure of the kidney(s) to rise out of the pelvis

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4
Q

Adult polycystic kidney disease can result from mutations in PKD1 or PKD2 (polycystin proteins). _______ develops more slowly.

A

PKD2

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5
Q

_________ mutations are common in autosomal dominant PKD.

A

De novo (25%)

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6
Q

Autosomal dominant polycystic kidney disease typically presents in _______________.

A

the fourth decade of life with chronic flank pain and hematuria; chronic hypertension in the fifth decade

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7
Q

Only _______ percent of ADPKD cases progress to ESRD.

A

50

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8
Q

What are some associations with ADPKD?

A
Hepatic cysts
Mitral valve prolapse
Diverticulosis
Cerebral aneurysms
Pancreatic cysts
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9
Q

What protein is typically defective in ARPKD?

A

PKHD1 (fibrocystin)

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10
Q

Autosomal recessive polycystic kidney disease often presents as ___________.

A

hypertension in the first few years of life

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11
Q

________ is dilations of collecting tubules, while ______ is dilation of the entire nephron.

A

ARPKD; ADPKD

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12
Q

___________ is the most common cause of abdominal mass in a newborn.

A

Multicystic dysplastic kidney

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13
Q

The most common kidney tumor present at birth is ____________. It can even be detected via prenatal sonogram by displaying the “ring sign.”

A

congenital mesoblastic nephroma

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14
Q

Wilms tumor is also known as __________.

A

nephroblastoma

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15
Q

Wilms tumor accounts for _____ percent of kidney tumors.

A

80

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16
Q

Why aren’t Wilms tumors biopsied?

A

Because they are very frangible and easily puncture. Leakage of the fluid upstages the patient.

17
Q

Rhabdomyoblastic differentiation (presence of skeletal muscle) is seen in _____ percent of Wilms tumors.

A

10

18
Q

What is anaplasia?

A

Large, bizarre mitoses due to p53 mutations

19
Q

What is the phenotype of Beckwith-Weidemann syndrome?

A

Gigantism
Macroglossia
Abdominal wall defects

20
Q

What is WAGR?

A

Wilms tumors
Aniridia
Genitourinary malformation
Retardation

21
Q

WAGR results from ________.

A

deletion of 11p13 (including PAX6 and WT1)

22
Q

Which kind of PKD presents with larger cysts?

A

Autosomal dominant

23
Q

What causes multicystic dysplastic kidney?

A

Abnormal induction of the metanephric blastema by the ureteric bud

24
Q

Congenital mesonephric blastoma is associated with trisomy _________.

A

11

25
Q

What three tissues does Wilms tumor typically recapitulate?

A

Blastemal
Stromal
Epithelial

26
Q

Beckwith-Weidemann syndrome is caused by ________ abnormalities.

A

imprinting

27
Q

ADPKD has __________ penetrance.

A

near 100%