USMLE Step 1 Web Prep Abdomen, Pelvis, and Perineum: Part 2

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Abdomen, Pelvis, and Perineum: Part 2

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on!enital Abnormalities "enal a!enesis Failure of one or both kidneys to develop be ause of early de!eneration of the ureteri bud" #!enesis is fairly o$$on in the unilateral for$ but leads to death shortly after birth in the bilateral for$"

"enal #$sts %he for$ation of thin&'alled( fluid&filled ysts fro$ blind tubules( perhaps arisin! fro$ i$proper linka!e bet'een the olle tin! du ts and distal onvoluted tubules"
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on!enital Abnormalities Pelvi# and &orses&oe 'idne$ )elvi kidney is aused by a failure of one kidney to as end" *orseshoe kidney is a fusion of both kidneys at their ends and failure of the fused kidney to as end" Double ureter +aused by early splittin! of the ureteri bud or the develop$ent of t'o separate buds"
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on!enital Abnormalities Patent ura#&us Failure of the allantois to be obliterated" ,t auses ura hal fistulas or sinuses" -e$nants of the allantoi stalk $ay !ive rise to ura hal ysts" ,n $ale hildren 'ith on!enital valvular obstru tion of the prostati urethra or in older $en 'ith enlar!ed prostates( a patent ura hus $ay ause draina!e of urine throu!h u$bili us"
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Pelvi# Dia+&ra!m and ,ro!enital Dia+&ra!m .oth ontain skeletal $us le .oth support pelvi vis era .oth ontain a sphin ter
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lini#al orrelate ,n the $ale( in/ury to the bulb of the penis $ay result in e0travasation of urine fro$ the urethra into the superfi ial perineal spa e" Fro$ this spa e( urine $ay pass into the s rotu$( into the penis( and onto the anterior abdo$inal 'all in the plane deep to 1 arpa fas ia"

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Dee+ Perineal Pou#& -S+a#e. %he deep perineal pou h spa e is the $iddle 3$us le4 layer of the uro!enital diaphra!$" ,t ontains: 1phin ter urethrae $us le & serves as e0ternal sphin ter of the urethra 5eep transverse perineal $us le .ulbourethral 3+o'per4 !land 3in the $ale only4 & du t enters bulbar urethra
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Su+erfi#ial Perineal Pou#& -S+a#e. %he superfi ial perineal pou h is the re!ion belo' the uro!enital diaphra!$ and is en losed by the superfi ial perineal 3+olles4 fas ia" ,t ontains: +rura of penis or litoris & ere tile tissue .ulb of penis 3in the $ale4 & ere tile tissue6 ontains urethra .ulbs of vestibule 3in the fe$ale4 & ere tile tissue6 in lateral 'alls of vestibule ,s hio avernosus $us le & skeletal $us le that overs rura of penis or litoris .ulbospon!iosus $us le & skeletal $us le that overs bulb of penis or bulbs of

vestibule 7reater vestibular 3.artholin4 !land 3in fe$ale only4 & ho$olo!ous to +o'per !land
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Embr$olo!$ of t&e "e+rodu#tive S$stem -+a!e 20%. Adult /emale 8vary( folli les( rete ovarii Indifferent Embr$o Adult 0ale 7onads %estes( se$iniferous tubules( rete testes #ppendi0 of testes

9terine tubes( uterus( )ara$esonephri ervi and upper part of du ts va!ina 5u t of 7artner :esonephri du ts

;pididy$is( du tus deferens( se$inal vesi le( e/a ulatory du t

%able ,,,& #dult Fe$ale and :ale -eprodu tive 1tru tures 5erived Fro$ ;a h 3&2 )re ursor of the ,ndifferent ;$bryo
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Embr$olo!$ of t&e "e+rodu#tive S$stem -+a!e 20%. Adult /emale +litoris <abia $inora Indifferent Embr$o )hallus 9ro!enital folds Adult 0ale 7lans and body of penis =entral aspe t of penis

<abia $a/ora

<abios rotal s'ellin!s

1 rotu$

%able ,,,& #dult Fe$ale and :ale -eprodu tive 1tru tures 5erived Fro$ ;a h 3&2" )re ursor of the ,ndifferent ;$bryo
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/emale Pseudo1Interse2ualit$ +hara teri>ed by havin! ovarian 3but no testi ular4 tissue histolo!i ally and $as ulini>ation of the fe$ale e0ternal !enitalia" ,ndividuals have a 46(?? !enotype" :ost o$$on ause is on!enital adrenal hyperplasia( a ondition in 'hi h the fetus produ es e0 ess andro!ens"
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0ale Pseudo1Interse2ualit$ +hara teri>ed by havin! testi ular 3but no ovarian4 tissue histolo!i ally and various sta!es of stunned develop$ent of the $ale e0ternal !enitalia" ,ndividuals have a 46(?@ !enotype" :ost o$$on ause is inadeAuate produ tion of testosterone and :ullerian inhibitin! fa tor 3:,F4 by the fetal testes" %his is due to a 5a&redu tase defi ien y"
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531"edu#tase 2 Defi#ien#$ +aused by a $utation in the 5B&redu tase 2 !ene that renders 5B&redu tase 2 en>y$e undera tive" Cor$ally ( 5B&redu tase ataly>es the onversion of testosterone to dihydrotestosterone" testosterone -4.
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di&$drotestosterone -D54.
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531"edu#tase 2 Defi#ien#$ %he defi ien y produ es the follo'in! lini al findin!s: 9nderdevelop$ent of the penis and s rotu$ 3$i rophallus( hypospadias( and bifid s rotu$4 and prostate !land" %he epididy$is( du tus deferens( se$inal vesi le( and e/a ulatory du t are nor$al
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om+lete Andro!en Insensitivit$ - AIS6 or 4esti#ular /emini7ation S$ndrome. 8 urs 'hen a fetus 'ith a 46(?@ !enotype develops testes and fe$ale e0ternal !enitalia 'ith a rudi$entary va!ina6 the uterus and uterine tubes are !enerally absent" %estes $ay be found in the labia $a/ora and are sur!i ally re$oved to ir u$vent $ali!nant tu$or for$ation" %hese individuals present as nor$al&appearin! fe$ales( and their psy hoso ial orientation is fe$ale despite their !enotype"
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5$+os+adias 8 urs 'hen the urethral folds fail to fuse o$pletely( resultin! in the e0ternal urethral orifi e openin! onto the ventral surfa e of the penis" ,t is !enerally asso iated 'ith a poorly developed penis that urves ventrally( kno'n as hordee" E+is+adias 8 urs 'hen the e0ternal urethral orifi e opens onto the dorsal surfa e of the penis"

,t is !enerally asso iated 'ith e0strophy of the bladder"

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,ndes#ended 4estes - r$+tor#&idism. 8 urs 'hen the testes fail to des end into the s rotu$" %his nor$ally o urs 'ithin 3 $onths after birth" .ilateral ryptor hidis$ results in sterility" %he undes ended testes $ay be found in the abdo$inal avity or in the in!uinal anal"
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5$dro#ele of t&e 4estes 8 urs 'hen a s$all paten y of the pro essus va!inalis re$ains so that peritoneal fluid an flo' into the pro essus va!inalis" -esults in a fluid&filled yst near the testes"
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Sites of Anastomoses 8et9een t&e Portal and aval S$stems and lini#al Si!ns of Portal 5$+ertension Sites of anastomoses 1" 9$bili us 2" -e tu$

Portal )arau$bili al veins 1uperior re tal veins 3,nferior $esenteri vein4 7astri veins

aval 1uperfi ial veins of the anterior abdo$inal 'all :iddle and inferior re tal veins 3internal ilia vein4

lini#al si!ns +aput $edusa ,nternal he$orrhoids

3" ;sopha!us

=eins of the lo'er ;sopha!eal esopha!us 'hi h drain into vari es the a>y!os syste$ 3pa!e 2114

%able ,,,&3&3