Lec 2: Urology & Genitourinary Development Flashcards

0
Q

pronephros/mesonephros

A

fake kidney

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1
Q

mesoderm gives rise to all the layers of genitourinary organs, EXCEPT?

A
adrenal medulla (from ectoderm) 
genital swelling/UG sinus (from endoderm)
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2
Q

mesonephric duct/wolffian duct

A

male parts + trigone

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3
Q

metanephros

A

real kidney

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4
Q

ureteric bud

arises from ______
gives rise to _______

A

arises from mesonephric duct

gives rise to ureter & collecting system

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5
Q

urogenital sinus

A

genitourinary development from the bladder down (excluding gonads & their tubing)

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6
Q

cloaca

A

common chamber into which urinary, genital, and GI tracts converge

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7
Q

potter syndrome

A
Pulomnary hypoplasia 
Oligohydramnios
Twisted face 
Twisted skin 
Extremity defects 
Renal failure
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8
Q

oligohydramnios

A

leads to compression of developing fetus

occurs when fetus does not produce urine

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9
Q

pronephros disappears after?

A

week 4

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10
Q

mesonephros

A

kidney for 1st trimester, then disappears

gives rise to Mesonephric duct

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11
Q

mesonephric ducts consists of?

A

trigone
efferent ducts of testes
epididymus
vas deferens

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12
Q

metanephros

A

kidney after 8 weeks

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13
Q

all kidneys develop from?

A

intermediate mesoderm

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14
Q

step 1 of kidney formation?

A

ureteric bud & metanephros induce each other to create the collecting system

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15
Q

metanephros consists of?

A
nephron 
glomerulus 
proximal tubule 
LOH
distal tubule
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16
Q

utereric bud consists of?

A

collecting ducts
calyces
pelvis
ureter

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17
Q

2 insertions of the utereric bud?

A

cloaca & metanephros

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18
Q

what is the most common cause of abdominal mass in a child?

A

MCKD

multicystic dysplastic kidney

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19
Q

step 2 of kidney formation?

A

kidney begins in sacrum & goes up to chest

roadblcoks: vasculature, malformation (horseshoe), masses

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20
Q

as kidney ascends, utereric bud bifurcates into?

A

calyces
infundibula
collecting ducts

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21
Q

anomalies in collecting ducts can result in?

A

duplicated collecting systems

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22
Q

where is the most common place to find a horseshoe kidney?

A

under the IMA

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23
Q

in a person with a right pelvic kidney, where is the right adrenal located?

A

in the normal spot

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24
step 3/4 of kidney formation?
as kidney ascends, it gains and loses blood supplies: iliacs-->aorta
25
when the vessels do not involute along the way, you get?
multiple arteries/veins 15% have 2 or more arteries
26
at 30 days, the cloaca becomes split by?
UG sinus | anorectal canal
27
UG sins grows and absorbs part of the Wollfian Duct distal to ureteric bud....& becomes the
trigone
28
the connection between the WD and UB moves...?
laterally & becomes the ureteral orifice
29
insertion of WD into US moves...?
medially & becomes the ejaculatory duct
30
most common pediatric anomalies?
``` vesicoureteral reflux UPJ & UVJ obstructions ectopic ureters duplications ureterocele ```
31
ectopic ureter
ureter that does not insert into the trigone more common in girls
32
weigert-meyer rule
in duplicated systems, the upper pole is ectopic | usually the upper pole is obstructed
33
patient presents as "continuously wet" if due to an ectopic ureter, where is it located?
distal to bladder neck
34
3 mo old comes in with hydronephrosis. what are the potential causes?
``` UPJO Megaureter VUR Ureterocele Urinary Obstruction (Posterior Urethral Valves) Misread (MCDK/ARPKD) ```
35
around week 5...?
genital ridges form next to developing mesonephros & create the primitive sex chord
36
around 6 weeks?
paramesonephric (mullerian) ducts form lateral to the nephric ducts
37
SRY
sex determining region of the Y chromosome
38
with SRY, primitive sex cord becomes? without SRY, primitive sex cord becomes?
sertoli cells genital ridge become testis ovarian follicles
39
with SRY, sertoli cells...?
secrete MIS (mullarian inhibiting substance) & paramesonephric duct involutes
40
without SRY, MIS?
does not regress --> wollfian regression & Mullerian development
41
week 8-10 (males with SRY)
leydig cells develop (testosterone development)
42
week (females without SRY)
Mullerian duct develop into fallopian tubes, uterus, upper 2/3 vagina
43
5-alpha reductase converts?
testosterone to DHT DHT causes UG sinus differentiation into external genitalia
44
genital tubercle
penis clitoris
45
genital swellings
scrotum labia majora
46
uretheral folds
penile urethra labia minora (folds remain separate)
47
urogenital groove
lumen of penile urethra vestibule
48
male and female genitals are morphologically indistinguishable until?
the 7th week
49
male: female analogs prostate
skene's gland
50
male: female analogs | prostatic utricle
vagina & uterus
51
male: female analogs | postatic urethra
entire urethra
52
male: female analogs | vas deferens and ejaculatory duct
gartner's duct
53
male: female analogs | scrotum
labia majora
54
male: female analogs | penis
clitoris
55
male: female analogs | cowper's glands
bartholin's glands
56
male: female analogs | gubernaculum
round & ovarian ligament
57
male: female analogs | testis
ovary
58
hypospadias (percent at different points)
anterior: 50% middle: 30% posterior: 20%
59
what is an undescended testis called?
cryptorchidism
60
most common pediatric urologic procedure? second most common?
circumcision orchidopexy
61
testicles start & end where?
upper abdomen descend through retroperitoneum to inguinal canal
62
testicles descent passes through?
processus vaginalis
63
what guides the testicles descent?
gubernaculum
64
failure of the processus vaginalis to close results in?
communicating hydrocele or hernia
65
risk factors for cyrptorchidism
``` advanced maternal age maternal obesity breech presentation c-section coke during pregnancy pre-term birth low birth weight family history hypospadias ```
66
orchiopexy before puberty reduces risk for?
cancer
67
what is linked to cryptorchidism?
deficiency in gonadotropins
68
congenital adrenal hypoplasia
inborn error in metabolism pediatric emergency variable presentation MC, GC, sex hormone dysfunction
69
deficiencies that lead to CAH?
``` 21 OH (95%) 11B-OH (5%) 3B=rare ```
70
what can cause a build up of aromatase?
obesity (fat cells produce aromatase) | aromatase converts testosterone to estradiol
71
2 day old male w/CAH presents with vomiting...
salt wasting
72
CAH treatment goals
supply deficient hormone supress pituitary ACTH secretion (hence adrenal androgens & clinical virilization) permit normal gonadal development correct salt-water loss or hypertension in complicated forms
73
effectiveness of therapy is determined by?
17 OH levels
74
46XX
2/2 Maternal Androgens/tumors danazol (testosterone) for endometriosis Progestatoinal androgens aromatase deficiency
75
androgen insensitivity
most common cause of 46XY DSD spectrum from complete external feminization to ambigious genitalia to infertile male
76
5alpha-reductase deficiency
46 XY that ranges from female to ambiguous small phallus/large clitoris UG sinus (convergence vagina/urethra) labial fusion blind ended vagina high T:DHT ratio
77
complete gonadal dysgenesis
45 XO | turner's syndrome
78
klinefelter's syndrom
XXY (non disjunction during meiosis) low T high LH & FSH & estradiol gynecomastia increased breast cancer risk
79
effectiveness of therapy is determined by?
.
80
what can cause a build up of aromatase?
obesity (fat cells produce aromatase) | aromatase converts testosterone to estradiol
81
2 day old male w/CAH presents with vomitting...
salt wasting
82
what can cause a build up of aromatase?
.