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29 Cards in this Set

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  • Back
How common are variations/anomalies in urinary system development?
3-4% of newborn infants have it
3 Anomalies of Kidney size, number, or histologic organization:
1. Hypoplastic kidneys
2. Duplications
3. Dysplastic kidney development
What are 2 possible consequences of Hypoplastic kidneys?
1. Small but normal morphology
2. Small and abnormal function
What is the commonly seen feature of Dysplastic Kidney development?
Cysts or Primitive-like Tissue
What is Dysplastic Kidney development the most common cause of?
Multicystic kidneys
What happens if Dysplastic Kidney development is Bilateral?
The fetus will die postnatally.
4 anomalies causing ectopic kidneys or ureters:
1. Pelvic/Lumbar kidney
2. Horseshoe kidney
3. Crossed Renal Ectopia
4. Ectopic Ureter
Which is more common; pelvic or lumbar kidney?
Pelvic - 60%
What causes pelvic/lumbar kidneys?
What side is it associated with?
Failure to ascent to normal position.
-Usually on the left side
What is a discoid?
Fusion of 2 pelvic kidneys because they are close together.
What is Crossed Renal ectopia?
The result of a kidney crossing to be on the same side w/ the other.
What is a Horseshoe Kidney? What syndrome is this associated with?
-Two kidneys have fused poles - usually inferior poles.
-Assoc. w/ Turners Syndrome
What results from Horseshoe kidney?
Failure to ascend b/c the horseshoe U gets stuck on Inferior Mesenteric Artery.
What is often seen with an Ectopic Ureter?
Incontinence - continuous dribbling.
What are 2 forms of Congenital Polycystic Kidney Disease?
1. ARPKD - autosom. recessive

2. ADPKD - autosom. dominant
Which form of PKD is more common?
What 'cystic' kidney disease is less severe than polycystic? Why?
Multicystic - b/c it is usually unilateral and has fewer cysts
What results from the many hundred cysts seen in ARPKD?
Renal insufficiency
What results if ARPKD manifests:
-In Newborns
-In Infants or Children
-In Adults
Newborn: fatal (MOST COMMON)
Inf/Kid: liver problems, HTN, congestive heart failure
Adults: Severe liver fibrosis and portal hypertension.
Where are the cysts located in
ARPKD = cortical only

ADPKD = cortical/medullary
When does ADPKD usually manifest?
Age 30-50
What chromosome is ADPKD associated with?
What 3 associated problems are seen with ADPKD?
-HTN (50%)
-Berry aneurism on ICA (33%)
-Intestinal diverticulosis (80%)
What is another name for Nephroblastoma?
Wilm's tumor
Why is it important to know about Wilm's Tumor / Nephroblastoma?
It is one of the 2 most common neoplasms seen in children (Retinoblastoma is more common)
What are the key features of Wilm's tumor?
-Early onset in medulla, progresses to cortex
-Associated w HORSESHOE kidney
-Assoc w Wt-1 gene on Ch'11p13
-Assoc w other anomalies/synd
What is Hydronephrosis?
Distention of renal pelvis and calices.
What causes Hydronephrosis?
Obstructed urinary flow - usually at ureter-pelvic junction
What is Hydronephrosis the most frequent cause of?
Neonatal abodminal mass