Lec 2: Urology & Genitourinary Development Flashcards
pronephros/mesonephros
fake kidney
mesoderm gives rise to all the layers of genitourinary organs, EXCEPT?
adrenal medulla (from ectoderm) genital swelling/UG sinus (from endoderm)
mesonephric duct/wolffian duct
male parts + trigone
metanephros
real kidney
ureteric bud
arises from ______
gives rise to _______
arises from mesonephric duct
gives rise to ureter & collecting system
urogenital sinus
genitourinary development from the bladder down (excluding gonads & their tubing)
cloaca
common chamber into which urinary, genital, and GI tracts converge
potter syndrome
Pulomnary hypoplasia Oligohydramnios Twisted face Twisted skin Extremity defects Renal failure
oligohydramnios
leads to compression of developing fetus
occurs when fetus does not produce urine
pronephros disappears after?
week 4
mesonephros
kidney for 1st trimester, then disappears
gives rise to Mesonephric duct
mesonephric ducts consists of?
trigone
efferent ducts of testes
epididymus
vas deferens
metanephros
kidney after 8 weeks
all kidneys develop from?
intermediate mesoderm
step 1 of kidney formation?
ureteric bud & metanephros induce each other to create the collecting system
metanephros consists of?
nephron glomerulus proximal tubule LOH distal tubule
utereric bud consists of?
collecting ducts
calyces
pelvis
ureter
2 insertions of the utereric bud?
cloaca & metanephros
what is the most common cause of abdominal mass in a child?
MCKD
multicystic dysplastic kidney
step 2 of kidney formation?
kidney begins in sacrum & goes up to chest
roadblcoks: vasculature, malformation (horseshoe), masses
as kidney ascends, utereric bud bifurcates into?
calyces
infundibula
collecting ducts
anomalies in collecting ducts can result in?
duplicated collecting systems
where is the most common place to find a horseshoe kidney?
under the IMA
in a person with a right pelvic kidney, where is the right adrenal located?
in the normal spot
step 3/4 of kidney formation?
as kidney ascends, it gains and loses blood supplies: iliacs–>aorta
when the vessels do not involute along the way, you get?
multiple arteries/veins
15% have 2 or more arteries
at 30 days, the cloaca becomes split by?
UG sinus
anorectal canal
UG sins grows and absorbs part of the Wollfian Duct distal to ureteric bud….& becomes the
trigone
the connection between the WD and UB moves…?
laterally & becomes the ureteral orifice
insertion of WD into US moves…?
medially & becomes the ejaculatory duct
most common pediatric anomalies?
vesicoureteral reflux UPJ & UVJ obstructions ectopic ureters duplications ureterocele
ectopic ureter
ureter that does not insert into the trigone
more common in girls
weigert-meyer rule
in duplicated systems, the upper pole is ectopic
usually the upper pole is obstructed
patient presents as “continuously wet”
if due to an ectopic ureter, where is it located?
distal to bladder neck
3 mo old comes in with hydronephrosis. what are the potential causes?
UPJO Megaureter VUR Ureterocele Urinary Obstruction (Posterior Urethral Valves) Misread (MCDK/ARPKD)
around week 5…?
genital ridges form next to developing mesonephros & create the primitive sex chord
around 6 weeks?
paramesonephric (mullerian) ducts form lateral to the nephric ducts
SRY
sex determining region of the Y chromosome
with SRY, primitive sex cord becomes?
without SRY, primitive sex cord becomes?
sertoli cells
genital ridge become testis
ovarian follicles
with SRY, sertoli cells…?
secrete MIS (mullarian inhibiting substance) & paramesonephric duct involutes
without SRY, MIS?
does not regress –>
wollfian regression & Mullerian development
week 8-10 (males with SRY)
leydig cells develop (testosterone development)
week (females without SRY)
Mullerian duct develop into fallopian tubes, uterus, upper 2/3 vagina
5-alpha reductase converts?
testosterone to DHT
DHT causes UG sinus differentiation into external genitalia
genital tubercle
penis
clitoris
genital swellings
scrotum
labia majora
uretheral folds
penile urethra
labia minora (folds remain separate)
urogenital groove
lumen of penile urethra
vestibule
male and female genitals are morphologically indistinguishable until?
the 7th week
male: female analogs
prostate
skene’s gland
male: female analogs
prostatic utricle
vagina & uterus
male: female analogs
postatic urethra
entire urethra
male: female analogs
vas deferens and ejaculatory duct
gartner’s duct
male: female analogs
scrotum
labia majora
male: female analogs
penis
clitoris
male: female analogs
cowper’s glands
bartholin’s glands
male: female analogs
gubernaculum
round & ovarian ligament
male: female analogs
testis
ovary
hypospadias (percent at different points)
anterior: 50%
middle: 30%
posterior: 20%
what is an undescended testis called?
cryptorchidism
most common pediatric urologic procedure? second most common?
circumcision
orchidopexy
testicles start & end where?
upper abdomen
descend through retroperitoneum to inguinal canal
testicles descent passes through?
processus vaginalis
what guides the testicles descent?
gubernaculum
failure of the processus vaginalis to close results in?
communicating hydrocele or hernia
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
orchiopexy before puberty reduces risk for?
cancer
what is linked to cryptorchidism?
deficiency in gonadotropins
congenital adrenal hypoplasia
inborn error in metabolism
pediatric emergency
variable presentation
MC, GC, sex hormone dysfunction
deficiencies that lead to CAH?
21 OH (95%) 11B-OH (5%) 3B=rare
what can cause a build up of aromatase?
obesity (fat cells produce aromatase)
aromatase converts testosterone to estradiol
2 day old male w/CAH presents with vomiting…
salt wasting
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
effectiveness of therapy is determined by?
17 OH levels
46XX
2/2 Maternal Androgens/tumors
danazol (testosterone) for endometriosis
Progestatoinal androgens
aromatase deficiency
androgen insensitivity
most common cause of 46XY DSD
spectrum from complete external feminization to ambigious genitalia to infertile male
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
complete gonadal dysgenesis
45 XO
turner’s syndrome
klinefelter’s syndrom
XXY
(non disjunction during meiosis)
low T
high LH & FSH & estradiol
gynecomastia
increased breast cancer risk
effectiveness of therapy is determined by?
.
what can cause a build up of aromatase?
obesity (fat cells produce aromatase)
aromatase converts testosterone to estradiol
2 day old male w/CAH presents with vomitting…
salt wasting
what can cause a build up of aromatase?
.
