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
Q

step 3/4 of kidney formation?

A

as kidney ascends, it gains and loses blood supplies: iliacs–>aorta

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25
Q

when the vessels do not involute along the way, you get?

A

multiple arteries/veins

15% have 2 or more arteries

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26
Q

at 30 days, the cloaca becomes split by?

A

UG sinus

anorectal canal

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27
Q

UG sins grows and absorbs part of the Wollfian Duct distal to ureteric bud….& becomes the

A

trigone

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28
Q

the connection between the WD and UB moves…?

A

laterally & becomes the ureteral orifice

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29
Q

insertion of WD into US moves…?

A

medially & becomes the ejaculatory duct

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30
Q

most common pediatric anomalies?

A
vesicoureteral reflux 
UPJ & UVJ obstructions 
ectopic ureters 
duplications 
ureterocele
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31
Q

ectopic ureter

A

ureter that does not insert into the trigone

more common in girls

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32
Q

weigert-meyer rule

A

in duplicated systems, the upper pole is ectopic

usually the upper pole is obstructed

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33
Q

patient presents as “continuously wet”

if due to an ectopic ureter, where is it located?

A

distal to bladder neck

34
Q

3 mo old comes in with hydronephrosis. what are the potential causes?

A
UPJO
Megaureter
VUR
Ureterocele
Urinary Obstruction (Posterior Urethral Valves) 
Misread (MCDK/ARPKD)
35
Q

around week 5…?

A

genital ridges form next to developing mesonephros & create the primitive sex chord

36
Q

around 6 weeks?

A

paramesonephric (mullerian) ducts form lateral to the nephric ducts

37
Q

SRY

A

sex determining region of the Y chromosome

38
Q

with SRY, primitive sex cord becomes?

without SRY, primitive sex cord becomes?

A

sertoli cells
genital ridge become testis

ovarian follicles

39
Q

with SRY, sertoli cells…?

A

secrete MIS (mullarian inhibiting substance) & paramesonephric duct involutes

40
Q

without SRY, MIS?

A

does not regress –>

wollfian regression & Mullerian development

41
Q

week 8-10 (males with SRY)

A

leydig cells develop (testosterone development)

42
Q

week (females without SRY)

A

Mullerian duct develop into fallopian tubes, uterus, upper 2/3 vagina

43
Q

5-alpha reductase converts?

A

testosterone to DHT

DHT causes UG sinus differentiation into external genitalia

44
Q

genital tubercle

A

penis

clitoris

45
Q

genital swellings

A

scrotum

labia majora

46
Q

uretheral folds

A

penile urethra

labia minora (folds remain separate)

47
Q

urogenital groove

A

lumen of penile urethra

vestibule

48
Q

male and female genitals are morphologically indistinguishable until?

A

the 7th week

49
Q

male: female analogs

prostate

A

skene’s gland

50
Q

male: female analogs

prostatic utricle

A

vagina & uterus

51
Q

male: female analogs

postatic urethra

A

entire urethra

52
Q

male: female analogs

vas deferens and ejaculatory duct

A

gartner’s duct

53
Q

male: female analogs

scrotum

A

labia majora

54
Q

male: female analogs

penis

A

clitoris

55
Q

male: female analogs

cowper’s glands

A

bartholin’s glands

56
Q

male: female analogs

gubernaculum

A

round & ovarian ligament

57
Q

male: female analogs

testis

A

ovary

58
Q

hypospadias (percent at different points)

A

anterior: 50%
middle: 30%
posterior: 20%

59
Q

what is an undescended testis called?

A

cryptorchidism

60
Q

most common pediatric urologic procedure? second most common?

A

circumcision

orchidopexy

61
Q

testicles start & end where?

A

upper abdomen

descend through retroperitoneum to inguinal canal

62
Q

testicles descent passes through?

A

processus vaginalis

63
Q

what guides the testicles descent?

A

gubernaculum

64
Q

failure of the processus vaginalis to close results in?

A

communicating hydrocele or hernia

65
Q

risk factors for cyrptorchidism

A
advanced maternal age 
maternal obesity 
breech presentation 
c-section 
coke during pregnancy 
pre-term birth 
low birth weight 
family history 
hypospadias
66
Q

orchiopexy before puberty reduces risk for?

A

cancer

67
Q

what is linked to cryptorchidism?

A

deficiency in gonadotropins

68
Q

congenital adrenal hypoplasia

A

inborn error in metabolism
pediatric emergency
variable presentation
MC, GC, sex hormone dysfunction

69
Q

deficiencies that lead to CAH?

A
21 OH (95%)
11B-OH (5%)
3B=rare
70
Q

what can cause a build up of aromatase?

A

obesity (fat cells produce aromatase)

aromatase converts testosterone to estradiol

71
Q

2 day old male w/CAH presents with vomiting…

A

salt wasting

72
Q

CAH treatment goals

A

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
Q

effectiveness of therapy is determined by?

A

17 OH levels

74
Q

46XX

A

2/2 Maternal Androgens/tumors

danazol (testosterone) for endometriosis
Progestatoinal androgens
aromatase deficiency

75
Q

androgen insensitivity

A

most common cause of 46XY DSD

spectrum from complete external feminization to ambigious genitalia to infertile male

76
Q

5alpha-reductase deficiency

A

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
Q

complete gonadal dysgenesis

A

45 XO

turner’s syndrome

78
Q

klinefelter’s syndrom

A

XXY
(non disjunction during meiosis)

low T
high LH & FSH & estradiol

gynecomastia
increased breast cancer risk

79
Q

effectiveness of therapy is determined by?

A

.

80
Q

what can cause a build up of aromatase?

A

obesity (fat cells produce aromatase)

aromatase converts testosterone to estradiol

81
Q

2 day old male w/CAH presents with vomitting…

A

salt wasting

82
Q

what can cause a build up of aromatase?

A

.