20-2 (congenital) Flashcards
What does congenital renal disease most often result from?
Acquired developmental defect during gestation
Congenital renal anomalies should prompt what?
Evaluation of other organs
– simultaneous development
Prune Belly Syndrome usually occurs in?
Males
What are 4 possible characteristics of Prune Belly Syndrome?
- Bilateral Hydroureteronephrosis
- Vesicoureteral Reflux
- Renal Dysplasia
- Bilateral Cryptorchidism
What are 4 possible characteristics of Prune Belly Syndrome?
- Bilateral Hydroureteronephrosis
- Vesicoureteral Reflux
- Renal Dysplasia
- Bilateral Cryptorchidism
What is Hydroureteronephrosis and with what disease is it almost always present?
Kidney and ureter swell due to obstruction of urine flow
– Prune Belly Syndrome
What is the Vesicoureteral Reflux?
Urine flows in the opposite direction of normal
What is Cryptorchidism?
Testes are intra-abdominally at the level of the iliac arteries
Babies with Prune Belly Syndrome will likely develop what 2 infections and/or need a renal transplant?
UTI
Pyelonephritis
What is a hereditary malignant tumor of the kidneys?
Wilms tumor
When is Bilateral Agenesis of the kidneys seen?
Incompatibile with life – stillborns
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?
It will be enlarged due to adaptation
– glomerular sclerosis may form
If bilateral renal hypoplasia is present, what will present in early childhood?
Renal failure
What are the 3 characteristics of true renal hypoplasia?
- Seen in low birth weight infants
- NO scars
- < 6 lobes/pyramids
What are the 3 characteristics of true renal hypoplasia?
- Seen in low birth weight infants
- NO scars
- < 6 pyramids/lobes
Renal hypoplasia is usually ____
Unilateral
What is a horseshoe kidney and where does it lie compared to the great vessels?
Fusion of the upper or lower poles of the kidneys
– anterior to great vessels
Are ectopic kidneys possible?
Yes
Congenital anomalies of the ureters are only clinically significant if they cause?
Obstruction of urine flow
Describe the grades of the vesicoureteral reflux
1 - into ureter 2 - into pelvis and calyces 3 - mild dilation 4 - dilation and ureteral tortuosity 5 - dilation, ureteral tortuosity, loss of papillary impressions
Leaky valve allows urine to flow in both directions suggests?
Vesicoureteral Reflux (VUR)
What can Vesicoureteral Reflux cause?
Pyelonephritis
Loss of renal function
Abnormal branching of the ureteric bud will cause?
Double/bifed ureters
What is the most common cause of hydronephrosis (swelling) of the kidney?
Ureteropelvic junction obstruction
In early life, males are more often affected with a Ureteropelvic Junction obstruction. What are the symptoms?
Hydronephrosis UTI Hematuria Sepsis Azotemia
In adult life, women are more commonly affected with a Ureteropelvic Junction Obstruction. What are the symptoms?
UTI
Hematuria
Abdominal pain and vomiting
What causes Ureteropelvic Junction Obstructions?
Abnormal smooth muscle bundles or excess collagen deposition
Hydronephrosis, UTI, hematuria in a young male suggests?
Ureteropelvic Junction Obstruction
Where are the 3 normal constrictions of the ureter?
- Ureteropelvic junction
- Crossing external iliac A.
- As ureter traverses bladder wall
Pouch-like invaginations that are usually in the bladder wall, small and asymptomatic
Diverticula
What what predisposes Diverticula to be symptomatic and what can result?
Urinary stasis predisposes to infection, calculi and advanced carcinoma
If a bladder diverticula is congenital, what likely caused it?
Failure of musculature development or tract obstruction in development
Exstrophy of bladder
Bladder develops outside the body due to abdominal wall failure
What is the bladder susceptible to if exstrophy of the bladder occurred?
Chronic infection
Colonic glandular metaplasia
Malformation of the urethral groove causes?
Abnormal opening on the penis
