Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

renal > congenital anomalies > Flashcards

congenital anomalies Flashcards

1
Q

renal agenesis results when

A

ureteric buds do not develop OR the primordia (stalks of buds) of ureters degenerate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

renal agenesis also called

A

renal hipoplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

why is there kidney development failure in renal agenesis/ hipoplasia

A

failure of buds to penetrate metanephrogenic blastema

= no nephrons are induced by the collecting tubules to develop from blastema

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

causes of renal agenesis/ hipoplasia

A

probably has a multifactorial cause

clinical evidence suggests polycystic kidneys (many cysts) could be a cause

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

types of renal agensis

A

unilateral renal agenesis (absence of 1 kidney)

bilateral renal agenesis (absence of both kidneys)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

unilateral renal agensis

A

1/1000 births, more common in males
non fatal
causes no symptoms
usually present kidney undergoes hypertrophy and compensates for missing kidney
should be suspected in children with a single umbilical artery

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

bilateral agenesis

A

1/3000
fatal
associated with Potter syndrome (20% of cases caused by bilateral agenesis)
associated with oligohydramnios (little/ no urine excreted into amniotic cavity)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

potter syndrome characteristics

A 
wide set eyes + palpebronasal folds (epicanthic folds)
ears are low set
nose is broad and flat
chin is receding
limb clubbing/ anomalies
respiratory anomalies
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

common cause of death by bilateral renal agenesis

A

death shortly after birth due to pulmonary hypoplasia (small/ underdeveloped lungs) = respiratory insufficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

ectopic kidneys

A

one or both kidneys in abnormal position
non fatal
most located in pelvis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

types of ectopic kidneys

A

pelvic kidneys
crossed renal ectopia
unilateral fused kidney

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

which congenital anomalies can result in kidneys fusing together

A

pelvic kidneys: form a discoid/ pancake kidney
crossed renal ectopia: 90% are fused
unilateral fused kidney: developing kidneys fuse after they leave pelvis
horseshoe kidney: usually fused at lower poles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what percentage of crossed renal ectopia cases have fused kidneys

A

90%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

horseshoe kidney

A

very rare: 0.2%
non fatal
associated with turner syndrome (7% of cases have horseshoe kidney)
usually no symptoms

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what percentage of turners syndrome have horseshoe kidney

A

7%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what anomaly is associated with turners syndrome

A

horseshoe kidney

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

what anomaly is associated with potter syndrome

A

bilateral renal agenesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

what percentage of potter syndrome have bilateral renal agenesis

A

20%

19
Q

malrotated kidney

A

hilum faces anteriorly: retains embryological position
hilum faces posteriorly: kidney rotated too much
hilum faces laterally: rotation in wrong direction
often associated with ectopic kidneys

20
Q

what other anomaly are malrotated kidneys associated with

A

ectopic kidneys

21
Q

duplications of urinary tract

A

non fatal
common
result from abnormal division of ureteric bud

22
Q

how are duplications of urinary tract created

A

incomplete division IN A DIVIDED KIDNEY of ureteric bud = bifid ureter

complete division IN A NON DIVIDED KIDNEY of ureteric bud = double kidney with bifid ureter or separate ureters

23
Q

ectopic ureter

A

non fatal
pee cannot be stored in bladder (pee will exit whenever)
differs from male and females

24
Q

differences of ectopic ureters for male and females

A

in males: ureter inserts into urethra, ductus deferens, prostatic utricle, OR seminal gland

in females: ureter inserts into bladder, urethra, vagina OR cavity of vagina

25
Q

cause of ectopic ureter

A

ureter not incorporated into the trigone between the openings of the ureter in the posterior part of bladder
- instead carried caudally with mesonephric duct + incorporated into middle pelvic portion of vesicle part of urogenital sinus

26
Q

cystic kidney disease

A

autosomal recessive polycystic kidney disease
OR
multicystic dysplastic kidney disease

27
Q

autosomal recessive polycystic kidney disease

A

fatal w/o dialysis and kidney transplant
1/20000
kidneys contain many small cysts = renal insufficiency
death occurs shortly after birth

28
Q

what disease is associated with pulmonary hypoplasia

A 
autosomal recessive polycystic kidney disease (25%)
potter syndrome (100%)
29
Q

what percentage of autosomal recessive polycystic kidney disease is associated with pulmonary hypoplasia

A

25%

30
Q

mutation of what gene causes most polycystic kidney and congenital hepatic fibrosis

A

PKHD1 gene

31
Q

mutation of PKHD1 gene causes

A

polycystic kidney and congenital hepatic fibrosis

32
Q

multicystic dysplasia kidney disease

A

mostly non fatal (75% survive)
abnormal development of renal system
unilateral in 75% of cases
fewer cysts that in autosomal recessive polycystic kidney disease

33
Q

which disease has more cyst formation within kidneys
a) autosomal recessive polycystic kidney disease
OR
b) multicystic dysplasia kidney disease

A

A) autosomal recessive polycystic kidney disease has more cyst formation which causes renal insufficiency

34
Q

cause of multicystic dysplasia kidney disease

A

dysmorphology: abnormal development of renal system

35
Q

old hypothesized cause of cyst formation in multicystic dysplasia kidney disease

A

old reason: failure of ureteric bud derivatives to join tubules derived from metanephrogenic blastema

36
Q

new hypothesized cause of cyst formation in multicystic dysplasia kidney disease

A

new reason: cystic structures are wide dilations of parts of continuous nephrons (especially the loops of henle)

37
Q

difference in cyst frequency and size for both cystic kidney diseases

A

ARPKD: most cysts, small in size

MDKD: least cysts, vary in size (from mm to cm)

38
Q

probable embryological basis of defect: unilateral renal agenesis

A

absence of ureteric bud

= no reciprocal induction of metanephrogenic blastema by ureteric bud = no kidney on one side

39
Q

probable embryological basis of defect: divided kidney + bifid ureter

A

divided ureteric bud

= caused growth of 2 ureter stems

40
Q

probable embryological basis of defect: pelvic kidney

A

divided ureteric bud

= can grow around preliminary blood supply vessel (common iliac artery) and prevent ascension

41
Q

probable embryological basis of defect: malrotation of kidney

A

complete division of ureteric bud

= allows kidney to rotate the opposite way (laterally instead of medially)

42
Q

probable embryological basis of defect: double kidney (supernumerary kidney)

A

complete division of ureteric bud

= 2 ureteric buds that induce the formation of metanephrogenic blastema = 2 kidneys on one side

43
Q

probable embryological basis of defect: fused kidneys (crossed renal ectopia)

A

migration of one kidney to the other side

= both kidneys on one side are pushed together and fuse

44
Q

probable embryological basis of defect: discoid kidney (pelvic kidney)

A

fusion of kidneys

renal (3 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions