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HIPERCORTICISMO
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Mario Antonio Molina
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UNIVERSIDAD NACIONAL AUTÓNOMA DE MÉXICO FACULT ACULTAD DE ESTUDIOS SUPERIORES SUPERIORE S PLANTEL PL ANTEL IZTACALA HOSPITAL GENERAL ZONA 29 CLÍNICA INTEGRAL II
HIPERCORTICISMO
Anatomía de las Glándulas Suprarrenales
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El #$(+%$* %s $%)%#&*$ !% gl")*)*$&()*(!%s a)&(.a!* #*$ %l l(ga!* "s"al- :"% % %l %s&a!* !% 5*$+*a "(!a $%g"la la %?#$%s(/ !% l*s g%%s !% $%s#"%s&a !% gl")*)*$&()*(!%s0 %l 5GR #"%!% (5('($ l*s %<%)&*s !%l 5GR a)&(.a!* #*$ 5*$+*as s*'$% l*s g%%s !% $%s#"%s&a !% gl")*)*$&()*(!%s- #a$&()(#a!* as> !% la a))(/ s(*l/g()a+%&% (+#*$&a&% !% " (5('(!*$ %!/g%* !% gl")*)*$&()*(!%s0 El $%)%#&*$ !% gl")*)*$&()*(!%s %s&á )*s&(&"(!* #*$ )as( a+(*á)(!*s 6 s% #"%!% !(.(!($ % &$%s !*+((*s <")(*al%s0 El !*+((* !% "(/ !% gl")*)*$&()*(!%s s% l*)al(;a % %l %?&$%+* )a$'*?(l* &%$+(al !% la +*lB)"la0 El !*+((* !% "(/ !%l DNA s% l*)al(;a % la #a$&% +%!(a !% la #$*&%>a 6 )*&(%% "%.% +*lB)"las !% )(s&%>a0 L*s !%!*s !% )() $%#$%s%&a la %s&$")&"$a 'ás()a #*$ la :"% %l !*+((* !% "(/ !%l DNA $%)**)% %s#%)>)a+%&% s%)"%)(as %s#%)>)as !% á)(!*s ")l%()*s0 El !*+((* a+(* &%$+(al #a$&()(#a % la a)&(.(!a! !% &$asa)&(.a)(/ !%l $%)%#&*$ 6 a"+%&a s" %s#%)()(!a!0 La (&%$a))(/ !% l*s $%)%#&*$%s !% gl")*)*$&()*(!%s )* GRE " *&$*s
as lla+a!as )*$$%g"la!*$%s !%l $%)%#&*$ !% %s&%$*(!%s- !(.(!(!*s % )*a)&(.a!*$%s 6 )*$$%#$%s*$%s0 L*s )*$$%g"la!*$%s a)&a s($.(%!* )*+* #"%&%s %&$% l*s $%)%#&*$%s 6 *&$as #$*&%>as ")l%a$%s- 6 #*$ %?#$%s(/ !% a)&(.(!a! %;(+á&()a- )*+* a)%&(lasa * !%sa)%&(lasa !% 5(s&*as- #*$ l*s :"% +*!()a la )*<*$+a)(/ !% l*s ")l%*s/+as 6 la )a#a)(!a! !% &$as)$(#)(/ !% l*s g%%s0 L*s %<%)&*s !% l*s gl")*)*$&()*(!%s s% !%'% #$()(#al+%&% a #$*&%>as s(&%&(;a!as a #a$&($ !%l +RNA !% l*s g%%s % l*s :"% la 5*$+*a %%$)% s"s %<%)&*s0 E!ectos "siolgicos
I"6% % la <")(/ !% )as( &*!as las )Bl"las !%l )"%$#*0 Las #$()(#al%s )*s%)"%)(as +%&a'/l()as !% la s%)$%)(/ !% gl")*)*$&()*(!%s * s" a!+((s&$a)(/ s% !%'% a a))(*%s !($%)&as !% %sas 5*$+*as % las )Bl"las0 Alg"*s %<%)&*s (+#*$&a&%s s* #$*!")&* !% $%s#"%s&as 5*+%*s&á&()as #*$ la (s"l(a 6 %l gl")ag/0 Las $%s#"%s&as l(#*l>&()as !% las )Bl"las g$asas a las )a&%)*la+(asACTH 6 5*$+*al !% )$%)(+(%&*- s% a&%a % a"s%)(a !% gl")*)*$&()*(!%s0 E!ectos meta#licos
L*s gl")*)*$&()*(!%s %s&(+"la a la )a$'*?()(asa !% <*s<*%*l#($".a&*- la gl")*sa 474<*s
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g$as*s 6 gl()%$*l 5a)(a la )($)"la)(/0 C*&$('"6% al +a&%(+(%&* !% " a#*$&% a!%)"a!* !% gl")*sa al )%$%'$*0 E!ectos cata#licos $ antiana#licos%
T(%% %<%)&*s )a&a'/l()*s 6 a&(aa'/l()*s % l*s &%(!*s l(<*(!% 6 )*"&(.*- %l +s)"l*- la g$asa #%$(
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)*+* *&$*s +%!(a!*$%s !% la (a+a)(/0 Es*s %.%&*s s* +%!(a!*s #*$ "a s%$(% )*+#l%a (&%$a))(*%s )* +*lB)"las !% a!5%s(/ !% l%")*)(&*s )* +*lB)"las s*'$% s"#%$)(%s %!*&%l(al%s :"% s* (5('(!as #*$ l*s gl")*)*$&()*(!%s0 L*s )a+'(*s s* +á?(+*s a las 7 5*$as 6 s% !(s(#a % 2 5*$as0 Ta+'(B (5('% la <")(/ !% l*s +a)$/
s&()*s 6 *&$as )Bl"las #$%s%&a!*$as !% a&>g%*s- !(s+("6% la )a#a)(!a! !% la )Bl"la !% $%s#*!%$ a l*s a&>g%*s 6 l*s +(&/g%*s % l*s +a)$/
El s>!$*+% !% C"s5(g s% #$*!")% )*+* $%s"l&a!* !% la %?#*s()(/ )$/()a al %?)%s* !% gl")*)*$&()*(!%s0 E la +a6*$>a !% l*s )as*s s% !%'% al )*s"+* !% gl")*)*$&()*(!%s &%$a#B"&()*s % !*s(s al&as- 6 % +%*$ #$*#*$)(/ a l%s(*%s &"+*$al%s 5(#*sa$(as- a!$%al%s * % *&$as l*)al(;a)(*%s %)&/#()as0 Ha6 !*s &(#*s !% s>!$*+% !% C"s5(g %l %!/g%* 6 %l %?/g%*0 El C"s5(g %!/g%* s% )a$a)&%$(;a #*$ "a #$*!"))(/ !% )*$&(s*l % %?)%s* !%&$* !%l *$ga(s+*- % &a&* :"% %l C"s5(g %?/g%* s% #$%s%&a )*+* )*s%)"%)(a !% "
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a !% l*s )as*s0 La )a"sa +ás <$%)"%&% !% %s&% s"'&(#* %s la 5(#%$s%)$%)(/ 5(#*sa$(a !% ACTH J #*$ " a!%*+a )*$&()*&$*#* %&(!a! :"% s% !%*+(a %<%$+%!a! !% C"s5(gJ0 2J la <*$+a (!%#%!(%&% !% la ACTH- :"% )*$$%s#*!% al 3 a 2 !% l*s )as*s 6 :"% s% !%'% "s"al+%&% a a!%*+as a!$%al%s- 6 % +%*$ #$*#*$)(/- al )a$)(*+a a!$%al- a la 5(#%$#las(a a!$%al +a)$**!"la$ * +()$**!"la$ #(g+%&a!a #$(+a$(a0 Etiología ) Síndrome de Cus(ing dependiente de ACTH*
E?)%s(.a #$*!"))(/ !% ACTH !% *$(g% 5(#*sa$(* +()$*a!%*+a +ás <$%)"%&% :"% a)$*a!%*+aJ * !% CRH !% *$(g% 5(#*&alá+()*0 Más <$%)"%&% % +"%$%s0 E?)%s(.a #$*!"))(/ !% ACTH * !% CRH %)&/#()*- g%%$al+%&% a #a$&($ !% Ca$)(*+a #"l+*a$ !% )Bl"las % a.%a *a& )%llJ T"+*$ )a$)(*(!% '$*:"(al- " *&$*s &"+*$%s !%l &(+*- #á)$%as " *.a$(*- )a$)(*+a +%!"la$ !% &($*(!%s000J0 • •
QD%'% s*s#%)5a$s% % #a)(%&%s <"+a!*$%s )* )"a!$* )*s&(&")(*al % 5(#*#*&as%+(a ) Síndrome de Cus(ing no dependiente de ACTH
Ia&$*gB()* #*$ a!+((s&$a)(/ !% )*$&()*(!%s %?/g%*s )a"sa +ás <$%)"%&%J % %s&*s )as*s- las !%&%$+(a)(*%s !% )*$&(s*l % sag$% 6 *$(a s* 'aas0 O$(g% s"#$a$$%al A!%*+a )a"sa s"#$a$$%al +ás <$%)"%&% % a!"l&*sJ Ca$)(*+a )a"sa %!/g%a +ás <$%)"%&% % la (
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Las !(.%$sas )a"!as !%l S>!$*+% !% C"s5(g s% #"%!% ag$"#a$ % !*s )a&%g*$>as !%#%!(%&%s !% la ACTH % (!%#%!(%&%s !% Bs&a0 Las )a"sas !%l s>!$*+% !% C"s5(g !%#%!(%&% !% la
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E la )l>()a %l s>!$*+% !% CRH %)&/#()a %s (!(<%$%)(a'l% !%l s>!$*+% !% ACTH %)&/#()a0 S( %+'a$g*- !%s!% %l #"&* !% .(s&a '(*:">+()* s% ()$%+%&a las )*)%&$a)(*%s #las+á&()as !% CRH * s"#$(+(!asJ 6 la s%)$%)(/ !% la ACTH %s&(+"la!a #*$ la CRH s% #"%!% s"#$(+($ )* g$a!%s !*s(s !% !%?a+%&as*a * as> % %l s>!$*+% !% ACTH %)&/#()aJ0 Alg"as .%)%s l*s &"+*$%s * 5(#*sa$(*s #$*!")% CRH 6 ACTH %)&/#()as0 '% Tumores suprarrenales
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s"#$a$$%al * #a$&()(#a&%0 La s%)$%)(/ !%l %s&%$*(!% a)*&%)% !% +a%$a al%a&*$(a 6 %#(s/!()a- 6 #*$ l* g%%$al * s% s"#$(+% )* !%?a+%&as*a0 E l*s )a$)(*+as s"#$a$$%al%s %s <$%)"%&% la s*'$%#$*!"))(/ !% #$%)"$s*$%s a!$/g%*s- l* )"al $%s"l&a % 5($s"&(s+* 6 .($(l(;a)(/ !% las +"%$%s a!"l&as- * !% l*s (,*s !% a+'*s s%?*s0 P*$ *&$a #a$&%- % l*s a!%*+as s"#$a$$%al%s la #$*!"))(/ !% #$%)"$s*$%s a!$/g%*s %s&á $%la&(.a+%&% l(+(&a!a0 P*$ &a&*- las +a(<%s&a)(*%s )l>()as !% Bs&*s )*$$%s#*!% #$()(#al+%&% a las !%l %?)%s* !% )*$&(s*l0 S% !%s)**)% la $a;/ #*$ la )"al s% !%sa$$*lla a!%*+as s"#$a$$%al%s- #%$* s% 5a %)*&$a!* +"&a)(*%s a)&(.a!*$as !% l*s $%)%#&*$%s #a$a l*s
!$*+% !% C"s5(g (!%#%!(%&% !% ACTH (!")(!* #*$ al(+%&*0 E %s&*s #a)(%&%s %l #B#&(!* (5('(!*$ !% la 5*$+*a gas&$*(&%s&(al GIPJ %s&(+"la s%)$%)(/ !% )*$&(s*l a )a$g* !% " a!%*+a s"#$a$$%al "(la&%$al0 La %?#$%s(/ a*$+al !% $%)%#&*$%s #a$a %l GIP % las )Bl"las !%l &"+*$ s"#$a$$%al #%$+(&% a Bs&as $%s#*!%$ a la (g%s&(/ !% al(+%&* )* " ()$%+%&* % %l )AMP 6 la s"'s(g"(%&% #$*!"))(/ !% )*$&(s*l0 E% Hiperplasia micronodular #ilateral
D(s+("6% las )*)%&$a)(*%s !% la ACTH- 6 %l )*$&(s*l * s% s"#$(+% )* g$a!%s !*s(s !% !%?a+%&as*a0 F% Hiperplasia macronodular #ilateral
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Cuadro clínico
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A +%"!* &a+'(B %?(s&% debilidad muscular - a&$('"('l% a "a +(*#a&>a %s&%$*(!% #$*?(+al !% %?&$%+(!a!%s s"#%$(*$%s % (<%$(*$%s% ()l"s* !% l*s +s)"l*s gl&%*sK % l*s #a)(%&%s )* s>!$*+% !% C"s5(g #*$ ACTH %)&/#()a- la +(*#a&>a #"%!% ll%ga$ a s%$ +"6 (&%sa- ag$a.a!a #*$ la 5(#*#*&as%+(a <$%)"%&% % %s&*s #a)(%&%s0 D% (g"al <*$+a s% #"%!% *'s%$.a$ estrías cutáneas !% )*l*$ $** .(*lá)%*- s*'$% &*!* % a'!*+%- )a!%$as- +a+as 6 )a$a (&%$a !% l*s '$a;*s- a?(las 6 +"sl*s s* +ás <$%)"%&%s % (&%sas % l*s #a)(%&%s /.%%sJ0
La #(%l %s a 6 <$ág(l- a&$/)a- #*$ #B$!(!a !% &%(!* g$as* s"')"&á%*- 6 las %:"(+*s(s 6 l*s 5%+a&*+as a#a$%)% %s#*&á%a+%&% * &$as &$a"+a&(s+*s +>(+*s0 Las 5%$(!as )()a&$(;a l%&a+%&% 6 s% #"%!% *'s%$.a$ á$%as !% acantosis nigricans. E l*s )as*s :"% )"$sa )* s%)$%)(/ a"+%&a!a !% ACTH %?(s&% aumento de pigmentación cutánea - s(+(la$ al !%s)$(&* % la %<%$+%!a! !% A!!(s*- :"% s"%l% s%$ +ás (&%sa % l*s #a)(%&%s )* #$*!"))(/ %)&/#()a0 S* <$%)"%&%s la oligomenorrea * la amenorrea 6- s( %?(s&% 5(#%$a!$*g%(s+*- #"%!% *'s%$.a$s% seborrea- acné- hirsutismo 6 s(g*s !% .($(l(;a)(/ % )as*s g$a.%s0 La HTA moderada- )* $%(a 'aa 6 )* #$%s(*%s !(as&/l()as s"#%$(*$%s a 943++ Hg- %s 'as&a&% 5a'(&"al0 P$*'a'l%+%&% %s&á $%la)(*a!a )* "a %?#as(/ !%l .*l"+% %?&$a)%l"la$ #*$ $%&%)(/
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