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STEP 1 > Congenital Kidney Quiz > Flashcards

Congenital Kidney Quiz Flashcards

1
Q

A rare kidney diseases characterized by tubular damage and interstitial fibrosis.

SHRUNKEN Kidneys

A

Medullary Cystic Kidney

AD

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2
Q

Which 2 congenital renal diseases are inherited?

A

PKD

Medullary Cystic Kidney

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3
Q

Which congenital renal disease is associated with Potter Syndrome

A

AR-PKD

Bilateral Renal Agenesis

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4
Q

Which congenital renal disease has lower fused poles?

A

Horeshoe Kidney

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5
Q

Which congenital renal disease causes kidney Hypertrophy/Hypersecretion?

A

Unilateral Renal Agenesis?

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6
Q

What 3 associations are found in ADPKD?

A

BERRY ANEURYSM
Mitral valve prolapse
Hepatic/Cystic Fibrosis

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7
Q

What two gene mutations lead to AD-PKD?

A

PKD1

PKD2

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8
Q

Which congenital renal disease has Kidney fibrosis?

A

Medullary Cystic Kidney

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9
Q

List two Autosomal Dominant congenital renal diseases?

A

AD-PKD

Medullary Cystic Kidney

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10
Q

Which congenital renal disease has cartilage (or abnormal tissue) in the kidneys?

A

Dysplastic Kidney disease

non-inherited

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11
Q

Which 2 congenital renal diseases lead to HTN?

A

AR-PKD

AD- PKD

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12
Q

Young adult with hematuria, flank pain, recurrent UTIs, and/or kidney stones

Possible h/o or FHx of:
murmur (MVP)
Hepatic problems due to cysts

Enlarged Kidneys Bilaterally

A

AD–Polycystic Kidney Disease

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13
Q

Horseshoe kidney gets caught where?

A

IMA

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14
Q

Increased Renin
Hematuria
young adult

A

AD-PKD

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15
Q

Portal HTN → ascities, paraumbilical/epigastric veins dilated, esophageal varices, Hepato and/or splenomegaly

Hepatic Fibrosis → anechoic cysts/spots on US, multiple cysts

Bilaterally enlarged cystic kidneys → proteinuria, hematuria, or oligouria

PULMONARY HYPOPLASIA → respiratory distress

A

AR-PKD

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16
Q

oligohydraminos
lung hypoplasia
flat face
low ears

A

Bilateral Renal Agenesis

AR-PKD

17
Q

PKHD1 codes for

A

Fibrocystin (in kidney and liver)

*AR-PKD

18
Q

Clinical findings associated with

Cysts in the medullary collecting tube

A

Medullary cystic kidney

19
Q

Uretic bud fails to induce differentiation of metanephric mesenchyme
Consists of cyst and connective tissue
Unilateral/bilateral
POTTERS

A

Dysplastic Kidney (Multicystic)

20
Q 
Associated with 
Hydronephrosis
Renal stones
Turner syndrome
Trisonomies 13, 18, 21
A

Horseshoe Kidney

21
Q

↑ Risk of Renal Cancer

A

Horseshoe Kidney

22
Q

Posterior urethral valves only seen in

A

MALES

never females

23
Q

Bilateral hydronephrosis
THICK or DILATED wall bladder on US
Bladder outlet obstruction MOST COMMON
(+/- oligohydraminos in fetus)

A

Posterior urethral valves

24
Q

Reccurent UTIs in a young male with a thick bladder wall

A

Posterior urethral valves

25
Q

Bifurcation of one uretic bud or 2 uretic buds connecting to blastema.
Y shaped bi-fid ureter

A

Duplex collecting system

*causes vesicuroectal reflux & Pyelonephritis

26
Q

Highly associated with vesicoureteral reflux

uretera, obstruction, ↑ risk of UTIs

A

Duplex collecting system

27
Q

Little girl with reccurrent UTIs and flank pain

A

vesicoureteral reflux

28
Q

POTTER is associated with
AR-PKD &
Dysplastic Kidney disease

It stands for

A 
Pulmonary hypoplasia
Oligohydraminos
Twisted face
Twisted skin (Low ears, Flat face)
Extremity defects
Renal failure
29
Q

Inadequate canalization of the ___ is the most common cause of unilateral fetal hydronephrosis.

A

ureteropelvic junction

the connection site between the kidney and the ureter,

STEP 1 (88 decks)

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