A/2. Developmental disorders of the kidney, the ureter and the bladder Flashcards

1
Q

Renal agenesis

A
  • (unilateral/bilateral)
  • Agenesis means absent organ due to absent primordial tissue
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2
Q

unilateral renal agenesis symptoms

A

If unilateral, majority of cases are asymptomatic with compensatory hypertrophy of the contralateral kidney;
but abnormalities of the contralateral kidney are
common

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3
Q

Renal hypoplasia

A

Kidney is smaller than normal (hypoplastic), usually structurally normal.

  • Associated with reduced number of functional nephrons, with increased risk of developing kidney failure
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4
Q

DEVELOPMENTAL DISORDERS OF THE KIDNEY

A
  1. Renal agensis
  2. renal hypoplasia
  3. Multicystic
    dysplastic kidney
  4. horseshoe kidney
  5. potter sequence
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5
Q

Multicystic
dysplastic kidney - pathology

A

Ureteric bud fails to induce differenciation of metanephric mesenchyme -> non-functional kidney consisiting of cysts and Connective tissue

  • Predominantly non-hereditary and unilateral; bilateral leads to potter sequence
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6
Q

multicystic dysplastic kidney- it unilateral or bilateral?

A
  • Predominantly non-hereditary and unilateral;
  • bilateral leads to potter sequence
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7
Q

unilateral multicystic dysplastic kidney is it hereditary?

A

predominanly Non-hereditary

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8
Q

Horseshoe kidney def

A

Inferior poles of both kidneys fuse abnormally > as they ascend from the pelvis (during normal embryogenesis)
–>horseshoe kidney get trapped under the inferior mesenteric artery

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9
Q

how is renal function in horseshoe kidney

A

Renal function is usually normal

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10
Q

horseshoe kidney is associated with ?
increases risk for

A
  • hydronephrosis (ureteropelvic junction obstruction)
  • stone
  • infections
  • increased risk of cancer
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11
Q

Potter sequence what is it

A

oligohydramnios
> compression of developing fetus
> limb deformities
> facial anomalies
> compression of chest and lack of amniotic fluid aspiration into fetal lung
> pulmonary hypoplasia

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12
Q

causes of potter sequence

A
  • ARPKD
  • obstructive uropathy
  • bilateral renal agenesis
  • chronic placental insufficiency
  • BILATERAL MULTICYSTIC dysplastic kidney
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13
Q

DEVELOPMENTAL DISORDERS OF THE COLLECTING SYSTEMS AND URETERS

A
  1. Duplex collecting system
  2. Ureteropelvic junction obstruction (UPJ)
  3. Vesicoureteral reflux (VUR
  4. Ureterocele
  5. Posterior urethral
    valve
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14
Q

Duplex collecting
system what is it

A
  • ## Y-shaped bifid ureter
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15
Q

duplex collecting system what is associated with

A

Associated with
* vesicoureteral reflux (VUR)
* and/or ureteral obstruction
* increased risk of UTI

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16
Q

Ureteropelvic
junction obstruction etiology

A
  • Congenital (inadequate canalization)
    or
  • acquired (trauma, fibrosis)
17
Q

Ureteropelvic
junction obstruction presentation

A
  • May be asymptomatic,
  • or present with recurrent UTIs
  • stone disease
  • palpable mass
  • hydronephrosis
18
Q

Ureteropelvic
junction obstruction treatment

A

Pyeloplasty is the treatment of choice,

but if the affected kidney possesses < 10%
of total renal function, nephrectomy should be performed

19
Q

when is nephrectomy indicated in ureteropelvic junction obstruction

A

Pyeloplasty is the treatment of choice,

but if the affected kidney possesses < 10%
of total renal function, nephrectomy should be performed

20
Q

Vesicoureteral
reflux (VUR) definition

A
  • Retrograde regurgitation of urine from the urinary bladder up the ureter and into the collecting system of the kidneys
21
Q

Vesicoureteral
reflux (VUR) is a end result of

A

several anomalies related to the functional integrity of the
* ureter
* bladder dynamics
* and the anatomic composition of the ureterovesical junction (UVJ)

22
Q

Vesicoureteral
reflux (VUR) presents with

A
  • recurrent UTIs,
  • chronic pyelonephritis > may develop CKD (‘reflux nephropathy’)
23
Q

Ureterocele def

A

Cystic out-pouching of the distal ureter into the urinary bladder

24
Q

Ureterocele presentation

A
  • May be asymptomatic
  • or present with recurrent cystitis
  • bladder outlet obstruction; eventually may develop CKD
25
Q

Posterior urethral
valve defintion

A

Membranous remnant in the posterior urethra in males;

it’s persistence can lead to urethral obstruction

26
Q

persistance of posterior urethral valve can lead to

A

urethral obstruction

27
Q

Posterior urethral
valve can be diagnosed prenatally how?

A

can be diagnosed prenatally by
* bilateral hydronephrosis
* and dilated or thickened bladder wall on US

28
Q

Most common cause of bladder outlet obstruction in male infants

A

posterior urethral valve

29
Q

posterior urethral valve is associated with

A

oligohydramnios in cases of severe obstruction

30
Q

DEVELOPMENTAL DISORDERS OF THE URINARY BLADDER

A
  1. Urachus
    anomalies
    * Patent
    urachus
    * Urachal cyst
    * Vesicourachal
    diverticulum

2.Bladder exstrophy

31
Q

Urachus- what is it

A

duct between the fetal bladder and umbilicus

32
Q

Urachus
anomalies are associated with increased risk of

A

associated with :
* UTI and
* increased risk of bladder adenocarcinoma

33
Q

Patent
urachus - what is it

A

Total failure of urachus to obliterate > urine discharge from umbilicus

34
Q

Urachal cyst definition

A
  • fluid-filled cavity lined with uroepithelium, between umbilicus and bladder
  • Cyst can become infected and present as painful mass below umbilicus
35
Q

Urachal cyst etiology

A

Partial failure of urachus to obliterate; fluid-filled cavity lined with
uroepithelium, between umbilicus and bladder

36
Q

complication of urachal cyst and its presentation

A

Cyst can become infected and present as painful mass below umbilicus

37
Q

Vesicourachal
diverticulum - definition

A

Slight failure of urachus to obliterate > outpouching of bladder

38
Q

Bladder exstrophy def

A

Protrusion of the urinary bladder through a defect in the abdominal wall

39
Q

Bladder exstrophy presentation

A
  • Usually present with a wide range of congenital anomalies, affecting the
    1. pelvic bones,
    2. genitals,
    3. reproductive tract,
    4. and gastrointestinal tract