Congenital renal pathoma 12/21 Flashcards

1
Q

Formation of stones and infections are related with ……… (disease)

A

Horseshoe kidneys.

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2
Q

Hydronehprosis is associated with …….. (disease)

A

Horseshoe kidneys.

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3
Q

Oligohydramnios causes pulmonary hypoplasia by mechanism: ……….

A

Due to the lack of normal alveolar distention by aspirated amniotic fluid.

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4
Q

I. Horsehoe kidney. Which part connected?

A

lower poles

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5
Q

What congenital is the most common?

A

Horsehoe kidney.

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6
Q

I. Where is located horsehoe kidney? (abnormal)

A

abnormally located in the lower abdomen

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7
Q

I. Horsehoe kidney. Under what vessel?

A

Inferior mesenteric artery
Kidneys got cought by this vessel when it ascents from pelvis to abdomen and get stucked.

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8
Q

II. Renal agenesis. what is it?

A

Absent kidney formation

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9
Q

II. Renal agenesis. unilateral or bilateral?

A

may be both

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10
Q

II. Renal agenesis. Unilateral. Leads to what?

A

Hypertrophy of the existing kidney

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11
Q

II. Renal agenesis. Unilateral. What physio process in other kidney?

A

Hyperfiltration that increases risk of renal failure later in life.

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12
Q

II. Renal agenesis. Bilateral. Leads to what?

A

Oligohydramnios

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13
Q

II. Renal agenesis. Bilateral. How is called disease and what manifestation?

A

Potter sequence.
Oligohydramnios with lung hypoplasia, flat face with low set ears, developmental defects of the extremities.

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14
Q

What diseases is impatible with life?

A

II. Renal agenesis. Bilateral –> POTTERS SEQUENCE

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15
Q

Potter sequence.
Oligohydramnios with lung hypoplasia, flat face with low set ears, developmental defects of the extremities.?

A

II. Renal agenesis. Bilateral.

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16
Q

Hyperfiltration that increases risk of renal failure later in life.?

A

II. Renal agenesis. Unilateral.

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17
Q

Hypertrophy of the existing kidney?

A

II. Renal agenesis. Unilateral.

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18
Q

III. Dysplastic kidney. Inheritance?

A

Noninherited

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19
Q

III. Dysplastic kidney. definition?

A

Congenital malformation of the renal parenchyma

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20
Q

III. Dysplastic kidney. how is characterized morphologically?

A

Congenital malformation of the renal parenchyma characterized by CYSTS AND ABNORMAL TISSUE (eg cartilage)

21
Q

III. Dysplastic kidney. which side?

A

Usually unilateral;

22
Q

III. Dysplastic kidney. What need to do if bilateral?

A

must be distinguised from inherited polycystic kidney disease

23
Q

must be distinguised from inherited polycystic kidney disease?

A

III. Dysplastic kidney. bilateral

24
Q

IV. PKD. Inheritance?

A

Inherited

25
Q

IV. PKD. what side affected?

A

bilateral

26
Q

IV. PKD. Morphology?

A

Bilateral enlarged kidneys with cysts in renal CORTEX AND MEDULLA

27
Q

IV. PKD. AR inheritance. what population?

A

Infants

28
Q

IV. PKD. AR inheritance. Associated with what?

A

Congenital hepatic fibrosis –> leads to portal hypertension and hepatic cysts.

28
Q

IV. PKD. AR inheritance. symptoms?

A

In infants as worsening renal failure and hypertension. New borns may be present with potters sequence

29
Q

In infants as worsening renal failure and hypertension. New borns may be present with potters sequence?

A

IV. PKD. AR inheritance.

30
Q

Congenital hepatic fibrosis –> leads to portal hypertension and hepatic cysts.?

A

IV. PKD. AR inheritance.

31
Q

IV. PKD. AD inheritance. What population?

A

Young adults

32
Q

IV. PKD. AD inheritance. Symptoms?

A

in young adults as hypertension (due to increased renin), hematuria and worsening renal failure.

33
Q

in young adults as hypertension (due to increased renin), hematuria and worsening renal failure.?

A

IV. PKD. AD inheritance.

34
Q

IV. PKD. AD inheritance. mutations?

A

APKD1 and APKD2 gene.

35
Q

IV. PKD. AD inheritance. how develops cysts

A

over time

36
Q

IV. PKD. AD inheritance. associated with what?

A

berry aneurysms -> SAH., hepatic cysts, mitral valve prolapse.

37
Q

berry aneurysms -> SAH., hepatic cysts, mitral valve prolapse?

A

IV. PKD. AD inheritance.

38
Q

APKD1 and APKD2 gene?

A

IV. PKD. AD inheritance.

39
Q

V. Medullary cystic kidney diseases. Inheritance?

A

Inherited

40
Q

V. Medullary cystic kidney diseases. what type of inheritance?

A

AD

41
Q

V. Medullary cystic kidney diseases. morphology?

A

defect leading to cysts in the medullary collecting ducts

42
Q

V. Medullary cystic kidney diseases. presentation?

A

parenchymal fibrosis results in shrunken kidney and worsening renal failure.

43
Q

parenchymal fibrosis results in shrunken kidney and worsening renal failure.?

A

V. Medullary cystic kidney diseases. (AD)

44
Q

defect leading to cysts in the medullary collecting ducts?

A

V. Medullary cystic kidney diseases. (AD)

45
Q

III. Dysplastic kidney. Unilateral - symptoms?

A

Asymptomatic

46
Q

III. Dysplastic kidney. Bilateral - symptoms?

A

Early, severe renal insufficiency due to absence of functional renal tissue

47
Q

III. Dysplastic kidney. UW definition of morphology.

A

Noncommunicating cysts intervening with dysplastic tissue