Pediatrics Flashcards
What does cranial urogenital sinus become
bladder and pelvic urethra, continous with allantois
what does Caudal urogenital sinus become
phalic urethra and distal vagina
What does allantois become
Urachus and medial umblical ligament
What is the detection rate of bladder prenatally on US?
depends on time
50% at 10 weeks- concurrent with start of urine production
78% 11 weeks
88% 12 weeks
100% 13 weeks
Bladder empties Q 15-20 mins. So if u cant see it try again. Maybe its empty. In 1st trimested will be 6-8 mm.
classify prenatally detected bladder anomolies
Dialated with obtruction( congenital urethral strictures, ant and post urethral valves, Urethral atresia, obstructing syringocele, sarcococcygeal teratoma, sacral myelomeniogecle, rectum anomlies
no obstruction: Prunne belly syndrome, neurogenic bladder, congenital megacystitis
Nondialated: clocal and bladder exstrophy, bladder hypoplasia, bladder agensis
classify post-natally detected bladder abnormalities
Urachal ( patent urachus, umblical urachus sinus, urachal cyst, vesicourachal diverticulum
What are the different types of bladder diverticulum detected post-natally?
Priamry paraureteral (congenital) , secondary paraureteral( often bc of obstruciton, weakening of bladder wall by sx or infection ) Iatrogenic, caused by infravesical obstruction or congenital defect
what is the gold standard for dx of bladder diverticula in peds?
VCUG
what are the different types of bladder duplication and what anomolies are they associated with
Complete or incomplete.
coronal or saggital. associated with abnormalities of external genitalia and lower GI tract. Saggital duplication is most common
what is the tx of nephrogenic adenoma in peds?
TUR + ibuporfen and TMP SMX
What is the treatment of eosinophilic cystitis in peds?
TUR = Bx of lesion then CS, Antihistamines, =/- abx. observation in very young neonates can be done
What 3 steps must occur for normal sexual differentiation?
1- Chromosomal sex at fertilization
2- Gonadal differentiation
3- differentiation of internal duct and ext genitalia
What gene is the Testis determining factor? complete name?
SRY( sex-determining region Y- gene)
Where is the SRY gene located?
distal portion of the short arm of The Y chromosome
name a gene that is involved in sexual determination and is associated with aniridia?
WT1-
associated with WAGR(wilms tumor, aniridia, Genitourinary findings and retardation)
Upto what age is the embryo bipotential sex determination?
6 weeks
Describe the journey of the primordial germ cells 3-5 weeks gestation?
at 3 weeks they develop and migrate along the wall of the yolk sac to the fetus, reaching their destination at 5 weeks at the gonadal ridge
what causes the indifferent gonad to become a testicle?
SRY gene
What is the first endocrine product formed by the testis? function?
MIS(mullerian inhibiting substance)/ AKA anti-mullerian hormone at 7-8 weeks, causes regression of ipsilateral mullerian structures
What is required for ovarian organogenesis
abscence of SRY gene
duplicate copies of at least one, yet unidentified x chromosomal locus
fetal ovaries undergo intense (……) proliferation to reach 20 million cells by (…) weeks GA.
Mitosis, GA
What cells formed in the testis at 9 weeks GA produce testosterone?
leydig cells
What promotes the production of testosterone in utero?
1- initially, autologus
2- placental hcg
3- LH from pituitary
what are 3 functions of testosterone in embryo?
1- virilization of wolfian structures( local/adjacent effect)
2- virilization of urogenital sinus (systemic)
3- virilization of genital tubercle (systemic)
What is the role of DHT in sexual development?
external virillization
sexual maturation at puberty
converted from testosterone in the urogential sinus , prostate and external genitalia by 5-AR
what effect does estrogen have on female differentiation of internal or external genitalia
none
how does the presence of estrogen effect the development of male genitalia?
Blocks MIS and associated with male reproductive tract abnormalities
Internal genital analogues for male and female Indifferent stage, male, female gonad mullerian duct wolfian duct mesonephrons
Indifferent stage, male, female
gonad M-testis, F- ovary
mullerian duct F- fallopian tube, uterus, upper 2/3 vagina
wolfian duct M-vas deferens, seminal vesicle
mesonephrons M- epididymis
what causes wolfian duct to involute?
absense of testosterone
When do penile formation, elongation and testicular decent occur?
formation : starts at 10 weeks, complete at 13
elongation 3rd trimester
testicular decent: 3rd trimester
