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Renal > Day 13 6/1/15 > Flashcards

Day 13 6/1/15 Flashcards

1
Q

Hydronephrosis

A
  • dilation of the renal by pelvis by urine

- ureteropelvic junction obstruction is most common cause of pediatric hydronephrosis

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2
Q

Hydroureter

A

-dilation of ureter by urine

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3
Q

Ureteropelvic Junction Obstruction

A
  • obstruction between kidney and ureter
  • most common cause of pediatric hydronephrosis
  • obstruction is more common in boys (M>1)
  • left side is involved in 67%
  • s&s: abdominal mass, pain, UTI
  • coexists with other congenital abnormalities ~50% of patients
  • 10% show ipsilateral reflux
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4
Q

Ureteral Duplication

A
  • most common renal abnormality
  • 2 ureters ipsilaterally enter bladder
  • s&s: failure to toilet train, continuous drip incontinence
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5
Q

Vesicoureteral Reflux

A

-urine traveling back up the ureter upon contraction of the detrusor of the bladder, usually resulting in hydroureter

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6
Q

Ureterocele

A
  • cystic dilation of terminal intravesical ureter

- can be obstructive if orifice is stenotic or ectopically located and can cause reflux

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7
Q

Urachal Remnant

A
  • urachus connects the dome of the fetal bladder to the allantois in the umbilical ligament
  • can cause pain and retraction of umbilicus during micturition
  • can form cyst (closed), sinus (open at one end), or fistula (open at both ends)
  • may leak out of umbilicus
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8
Q

Megalocystis (Megacystis)

A
  • chronic abnormal distension of bladder by urine due to bladder outlet obstruction
  • may cause prune belly in babies (eagle-barrett syndrome)
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9
Q

Posterior Urethral Valves

A
  • abnormal congenital obstructing membrane loated in posterior male urethra
  • results from abnormal insertion of mesonephric duct on the cloaca
  • causes abnormal development of all upstream structures due to chronic increased intraluminal pressure
  • most common cause of bladder outlet obstruction in boys only
  • s&s: poor urine stream, UTI, incontinence in older boy
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10
Q

Bladder DIverticulum

A

-outpouching of bladder mucosa through a weakness in muscular wall

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11
Q

Hypospadias

A
  • orifice of the penile urethra at location along the ventral aspect of the penis
  • results from abnormal fusion of urogenital folds from androgen insufficiency
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12
Q

Chordee

A
  • fibrous band causing penis to curve toward its location

- usually associated with hypospadias (orifice of penis in wrong spot)

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13
Q

Epispadias

A

-location of uretheral opening on the dorsal aspect of the penis (top)

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14
Q

Exstrophy

A

-exposure of the bladder mucosa due to absence of the abdominal wall

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15
Q

Consequences of Fetal Urinary Tract Obstruction or Renal Agenesis

A
  • inability excrete urin
    dec. amniotic fluid (oligohydramnios)
  • less room for fetal movement (Potters sign)
  • loss of lung development
  • amnion nodosum (nodules of squamous cells on amniotic membrane)
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16
Q

Renal Agenesis and Hypoplasia

A
  • failure of metanephric diverticulum or its early degeneration
  • unilateral more common
  • L is more likely absent
  • complete renal agenesis is lethal
17
Q

Renal Dysplasia

A

-abnormal metanephric tissue of the kidney with cysts and heterotopic tissues such as cartilage due to pleuripotent potential of renal cells

18
Q

Renal Ectopia

A
  • failure of kidney to rise out of the pelvis or to rotate medially
  • may result in ureteral obstruction
  • kidneys may be discoid in shape
19
Q

Horseshoe Kidney

A
  • kidneys fuse together
  • 90% of time lower lobes fuse
  • often ectopic and fail to rotate medially
  • inc. incidence of urolithiasis
20
Q

Cystic Kidney Diseases

A
  • autosomal dominant (adult) polycystic kidney disease
  • autosomal recessive (infantile) polycystic kidney disease
  • multicystic dysplastic kidney disease
21
Q

Autosomal Dominant Polycystic Kidney Disease

A
  • adult
  • mutations in PKD1 or PKD 2
  • PKD 2 is slower
  • 25% of patients have no family hx
  • near 100% penetrance
  • presents in 4th decade with flank pain and hematuria
  • HTN and chronic renal failure in 5th decade of life
  • only 50% progress to end stage renal disease
  • associated with: hepatic cysts, mitral valve prolapse, diverticulitis, cerebral aneurysms, pancreatic cysts
22
Q

Autosomal Recessive Polycystic Kidney Disease

A

-due to PKHD1 which encodes fibrocystin (fibrosis of liver)
-manifests as HTN in first few years of life
-diminished urine concentrating ability and renal insufficiency
-growth retardation 25% of time
progresses to renal failure requiring dialysis or transplantation
-histology: cysts are dilated collecting tubules

23
Q

Multicystic Dysplastic Kidney

A
  • most common cause of an abdominal mass in the newborn period
  • most common cystic malformation of the kidney in infancy
  • often associated with ureteral or ureteropelvic atresia
  • affected kidney is nonfunctional: asymptomatic and will involute over time
  • results from abnormal induction of the metanephric blastema by ureteral bud
24
Q

Congenital Mesoblastic Nephroma

A
  • most common kidney tumor at birth to 6 months of age
  • can be detected on US with ring sign
  • bland spindle cells infiltrate
  • complete resection is most important prognostic factor
  • ETV6-NTRK3
  • identical translocation is found in congenital fibrosarcoma
  • trisomy 11 is also frequently found
25
Q

Wilms Tumor (Nephroblastoma)

A
  • most common malignant kidney tumor of childhood
  • presents 4-6 years of age as a solitary abdominal mass
  • treated by resection of chemotherapy (before or after)
  • classic histology is triphasic (blastemal, epithelial, stromal)
  • rhabdomyoblastic (skeletal muscle)
  • anaplasia (unfaborable histology)
26
Q

Beckwith-Weidemann Syndrome

A
  • phenotype includes gigantism, macroglossia and abdominal wall defects
  • due to imprinting abnormalities on chromosome 11p15.5
  • 5-7% develop Wilms tumor as well as other embryonal tumors
27
Q

WAGR Syndrome

A
  • Wilms tumor
  • Aniridia
  • GU malformation
  • mental Retardation
  • deletion of 11p13 includes PAX6 and WT1
  • 30% of these pts develop Wilms tumor

Renal (14 decks)

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