20-2 (congenital)

Question 1

What does congenital renal disease most often result from?

Answer 1

Acquired developmental defect during gestation

Question 2

Congenital renal anomalies should prompt what?

Answer 2

Evaluation of other organs

– simultaneous development

Question 3

Prune Belly Syndrome usually occurs in?

Answer 3

Males

Question 4

What are 4 possible characteristics of Prune Belly Syndrome?

Answer 4

1. Bilateral Hydroureteronephrosis
2. Vesicoureteral Reflux
3. Renal Dysplasia
4. Bilateral Cryptorchidism

Question 5

What are 4 possible characteristics of Prune Belly Syndrome?

Answer 5

1. Bilateral Hydroureteronephrosis
2. Vesicoureteral Reflux
3. Renal Dysplasia
4. Bilateral Cryptorchidism

Question 6

What is Hydroureteronephrosis and with what disease is it almost always present?

Answer 6

Kidney and ureter swell due to obstruction of urine flow

– Prune Belly Syndrome

Question 7

What is the Vesicoureteral Reflux?

Answer 7

Urine flows in the opposite direction of normal

Question 8

What is Cryptorchidism?

Answer 8

Testes are intra-abdominally at the level of the iliac arteries

Question 9

Babies with Prune Belly Syndrome will likely develop what 2 infections and/or need a renal transplant?

Answer 9

UTI

Pyelonephritis

Question 10

What is a hereditary malignant tumor of the kidneys?

Answer 10

Wilms tumor

Question 11

When is Bilateral Agenesis of the kidneys seen?

Answer 11

Incompatibile with life – stillborns

Question 12

Unilateral Agenesis of a kidney is compatible with life if no other issues are present. How will that one kidney that is present likely look and what may develop in it?

Answer 12

It will be enlarged due to adaptation

– glomerular sclerosis may form

Question 13

If bilateral renal hypoplasia is present, what will present in early childhood?

Answer 13

Renal failure

Question 14

What are the 3 characteristics of true renal hypoplasia?

Answer 14

1. Seen in low birth weight infants
2. NO scars
3. < 6 lobes/pyramids

Question 15

What are the 3 characteristics of true renal hypoplasia?

Answer 15

1. Seen in low birth weight infants
2. NO scars
3. < 6 pyramids/lobes

Question 16

Renal hypoplasia is usually ____

Answer 16

Unilateral

Question 17

What is a horseshoe kidney and where does it lie compared to the great vessels?

Answer 17

Fusion of the upper or lower poles of the kidneys

– anterior to great vessels

Question 18

Are ectopic kidneys possible?

Answer 18

Yes

Question 19

Congenital anomalies of the ureters are only clinically significant if they cause?

Answer 19

Obstruction of urine flow

Question 20

Describe the grades of the vesicoureteral reflux

Answer 20

1 - into ureter
2 - into pelvis and calyces
3 - mild dilation
4 - dilation and ureteral tortuosity
5 - dilation, ureteral tortuosity, loss of papillary impressions

Question 21

Leaky valve allows urine to flow in both directions suggests?

Answer 21

Vesicoureteral Reflux (VUR)

Question 22

What can Vesicoureteral Reflux cause?

Answer 22

Pyelonephritis

Loss of renal function

Question 23

Abnormal branching of the ureteric bud will cause?

Answer 23

Double/bifed ureters

Question 24

What is the most common cause of hydronephrosis (swelling) of the kidney?

Answer 24

Ureteropelvic junction obstruction

Question 25

In early life, males are more often affected with a Ureteropelvic Junction obstruction. What are the symptoms?

Answer 25

``` Hydronephrosis UTI Hematuria Sepsis Azotemia ```

Question 26

In adult life, women are more commonly affected with a Ureteropelvic Junction Obstruction. What are the symptoms?

Answer 26

UTI Hematuria Abdominal pain and vomiting

Question 27

What causes Ureteropelvic Junction Obstructions?

Answer 27

Abnormal smooth muscle bundles or excess collagen deposition

Question 28

Hydronephrosis, UTI, hematuria in a young male suggests?

Answer 28

Ureteropelvic Junction Obstruction

Question 29

Where are the 3 normal constrictions of the ureter?

Answer 29

1. Ureteropelvic junction 2. Crossing external iliac A. 3. As ureter traverses bladder wall

Question 30

Pouch-like invaginations that are usually in the bladder wall, small and asymptomatic

Answer 30

Diverticula

Question 31

What what predisposes Diverticula to be symptomatic and what can result?

Answer 31

Urinary stasis predisposes to infection, calculi and advanced carcinoma

Question 32

If a bladder diverticula is congenital, what likely caused it?

Answer 32

Failure of musculature development or tract obstruction in development

Question 33

Exstrophy of bladder

Answer 33

Bladder develops outside the body due to abdominal wall failure

Question 34

What is the bladder susceptible to if exstrophy of the bladder occurred?

Answer 34

Chronic infection | Colonic glandular metaplasia

Question 35

Malformation of the urethral groove causes?

Answer 35

Abnormal opening on the penis