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child health > congenital renal > Flashcards

congenital renal Flashcards

1
Q

what are the three stages of the development of the kidneys ?

A

pronephros day 22
mesonephros 4th week
metanephros at 5th week , works 4 weeks later

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2
Q

what are the features of renal coloboma syndrome ?

A

hypoplastic kidney

eye disease

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3
Q

what gene is affected in renal coloboma syndrome ?

A

PAX2

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4
Q

what disease is associated with EYA1 mutation ?

A

brachio-oto-renal syndrome

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5
Q

what are the features of brachio-oto-renal syndrome ?

A

hearing deficit
neck fistula
renal anomalies

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6
Q

what are the features of bardet-beidl syndrome ?

A 
obesity 
polydactyly 
hypogonadism 
intellectual disability 
retinitis pigmentosa 
CAKUT
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7
Q

what is used to diagnose CAKUT antenatally ?

A

ultrasound

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8
Q

what is potter syndrome ?

A

associated with a family history of renal agenesis
baby born with low set ears
bilateral polycystic renal disease
or bilateral renal agenesis

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9
Q

what does unilateral renal agenesis look like ?

A

diagnosed using antenatal steroids
compensatory hypertrophy in the contralateral kidney
associated with ipsilateral urogenital malformation

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10
Q

what is the clinical criteria required to make a diagnosis of hypoplastic kidney ?

A

reduction of renal size by 2 SD

exclusion of renal scarring

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11
Q

how is the exclusion of renal scarring made ?

A

using DMSA scan

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12
Q

what is the prognosis of hypoplastic kidney ?

A

increased risk of hypertension

increased risk of chronic kidney disease

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13
Q

what does bunches of grapes appearance on ultrasound indicate ?

A

multicystic dysplastic kidney

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14
Q

what is the prognosis of multicystic dysplastic kidney disease ?

A

no need for surgery
involution of cysts over time
compensatory hypertrophy in contralateral kidney

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15
Q

what are the common features associated with ectopic kidney ?

A

more on the left side
usually still found undescended in the pelvis
usually asymptomatic

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16
Q

what is the most common type of renal fusion anomaly?

A

horse-shoe kidney

17
Q

what chromosomal disease are associated with horse-shoe kidney ?

A

turner syndrome

edwards syndrome

18
Q

what tumor is highly associated with horseshoe kidney ?

A

wilms tumor

19
Q

what is the gold standard for the diagnosis of prostatic urethral valve ?

A

voiding cystourethrogram

20
Q

what is the general management for CAKUT ?

A

use ACE inhibitors
avoid NSAIDs and Aminoglycosides
dietary management

child health (40 decks)

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