24. ciroza biliara primitiva

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    I. CIROZA BILIARA PRIMITIVA

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    Defnitie Ciroza biliara primitiva (CBP are o etiolo!ie

    ne"#no$"#ta% evol#an& "# "ole$taza"roni"a% &i$tr#"tie pro!re$iva a "telorbiliare intra'epati"e% inamatie portala $ievol#tie fnala $pre "iroza $i in$#f"ienta'epati"a.

    Inamatia intere$eaza "aile biliareintra'epati"e% fin& &en#mita $i "olan!ita

    &i$tr#"tiva ne$#p#rata. Pentr# prima &ata% )al*er raporteaza

    a$o"ierea +ntre CBP $i anti"orpii anti,mito"on&riali (AAM% "are vor f -olo$iti

    #lterior "a mar*eri &ia!no$ti"i.

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    /pi&emiolo!ie Boala e$te ra$p n&ita mai ale$ +n

    pop#latia alba% reprezentan& pana la0 &in "az#rile &e &e"e$ ale "irozei.

    /$te mai ra$pan&ita la -emei%raport#l -emeibarbati fin& &e 21.

    3

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    /tiopato!eneza 4# $e "#noa$te etiolo!ia CBP. 4# $ ,a p#t#t

    evi&entia -a"tor#l initial 5tri!!er6 "e&e"lan$eaza "a$"a&a &e evenimenteim#nolo!i"e.

    Lezi#nile 'epati"e $#nt rez#ltat#l a &o#a-enomene7

    1. Di$tr#"tia ne$#p#rativa a "telor biliare(me&iata &e lim-o"ite. 8n CBP apare o

    "topenie% a&i"a re"erea n#mar#l#i &e"anali"#le biliare interlob#lare% pana la&i$paritia "ompleta.

    . Lezi#ni 'epato"itare in$e &e a"izii biliari

    primari $i $e"#n&ari% "are% +n "on"entratii:

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    CBP $e a$o"iaza "# o $erie &e bolia#toim#ne% pre"#m7 &ermatomiozita% l#p#$#l

    eritemato$% $"lero&ermia% tiroi&itaa#toim#na% poliartrita re#matoi&a. Anomaliile im#nitare intere$eaza ambele

    tip#ri &e im#nitate7A. ;morala. I!M $eri" e$te "re$"#t m#lt% +n

    $er#l bolnavilor "# CBP aparan& #n n#marmare &e anti"orpi. A$t-el% AAM (anti"orpi anti

    mito"on&riali $#nt prezenti la >0&intre "az#ri.B. Cel#lara. ?#nt prezente !ran#loamele $i

    infltrat#l lim-o"itar +n f"at% pre"#m $i

    aner!ia la te$tele "#tanate.=

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    Toate a"e$te rea"tii im#nolo!i"e " "#timp#l la &i$tr#"tia "telor biliare

    interlob#lare $i $eptale. O &ata "# &i$tr#!erea lor% apar "ole$taza%

    fbroza $i% +n fnal% "iroza. Canali"#lele biliare &i$tr#$e% n# a#

    "apa"itatea &e re!enerare% $pre &eo$ebire&e 'epato"ite% "are a# o "apa"itate infnita&e re!enerare.

    Pro9imal &e "anali"#lele biliare &i$tr#$eapare retentia biliara. Ca rez#ltat al a"ti#nii to9i"e a $ar#rilor

    biliare% apar lezi#ni &e tip 5pie"e,mealne"ro$i$62

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    Mor-opatolo!ie

    CBP par"#r!e 'i$tolo!i" patr# $ta&ii%a"e$tora "ore$p#nzan,le #n an#mittablo# "lini"7

    ,?ta&i#l I (portal% "olan!ita.,?ta&i#l II (periportal.,?ta&i#l III ($eptal pre"iroti".,?ta&i#l IV ("iroti"

    @

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    Tablo# "lini" #mate &in pa"ientii &ia!no$ti"ati $#nt

    a$imptomati"i% &ar la toti $#nt prezente$emnele &e "ole$taza7 "re$terea -o$-atazeial"aline $i !ama!l#tamil tran$pepti&aza.

    8n -ormele $imptomati"e &eb#t#l e$tein$i&io$.

    ;lterior apar pr#rit#l% i"ter#l% -ati!abilitatea%pi!mentarea melani"a a te!#mentelor%

    9antela$ma% $teatoreea%'epato$plenome!alia $i malab$orbtiavitaminelor % A $i D ($in&rom 'emora!ipar%t#lb#rari &e ve&ere% reri o$oa$e% -ra"t#ri

    $pontane% o$teoporoza.

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    Dia!no$ti" ?in&rom#l "ole$tati" 7 "re$" -o$-ataza

    al"alina% !ama!l#tamiltran$"repti&aza%bilir#bina $eri"a +n ambele "omponente%a"izii biliari $eri"i (a"il "oli" $i lipi&ele

    $eri"e (mai ale$ "ole$terol#l. Im#nolo!i"7 "ara"teri$ti"a e$te "re$terea

    AAM (anti"orpi anti mito"on&riali la #n titr#mai mare &e 1:>% "'iar% &in $ta&i#l

    a$imptomati" la ,

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    /vol#tie CBP evol#eaza pro!re$iv% $#praviet#irea

    me&ie +n -azele &ia!no$ti"ate a$imptomati"fin& &e pe$te 1> ani% iar la "ele$imptomati"e &e apro9imativ @ ani.

    O &ata "# aparitia "irozei $i a vari"elore$o-a!iene% pro!no$ti"#l &evine mairezervat.

    O po$ibila $ta&ializare "lini"a a CBP e$te7

    , a$imptomati"a, $imptomati"a ani"teri"a, $imptomati"a i"teri"a

    , "iroza.1>

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    Tratament

    Tratament#l CBP e$te -o"alizat pe treiobie"tive terape#ti"e7

    1. Mani-e$tarile &atorate "ole$tazei

    . Pro"e$#l im#n

    3. Ciroza 'epati"a propri#,zi$a

    11

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    1.Tratament#l mani-e$tarilor "ole$tati"e,Pr#rit#l e$te $in&rom#l "el mai $#paratorE $e

    +n"ear"a ameliorarea l#i "#7 "ole$tiramina39:!zi% -enobarbital la pr#rit#l re-ra"tar% iarpe termen $"#rt F anti'i$tamini"ele%-ototerapia "# #ltraviolete% anta!oni$tiiopia"eelor $i "orti"o$teroizii. La -ormele la"are tratament#l me&i"amento$ e$teinef"a"e% $e poate tenta pla$ma-ereza.

    ,?teatoreea $e re"e prin $"a&erea

    !ra$imilor &in alimente $i tratament#l&ef"it#l#i &e vitamine lipo$ol#bile.,O$teo&i$trofa (o$teoporoza% o$teomala"ia $e

    trateaza prin $#plimentare &e "al"i# $i

    vitamina D.1

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    .Tratament#l pro"e$#l#i a#toim#n,A"il #r$o&eo9i"oli" (;DCA 1>,1=m!*!

    "orpzi e$te "el mai ef"ient tratament% maiale$ +n $ta&iile I $i II. ;DCA $ea&mini$treaza pe rata +ntre!ii vieti.A"il #r$o&eo9i"oli" e$te me&i"ament#l

    &e ele"tie +n terapia CBP. In #ltima vremepro!no$ti"#l CBP $,a ameliorat o&ata "##tilizarea pe termen l#n! a ;DCA.

    3.Tratament#l "irozei /$te i&enti" "# "el &e$"ri$ anterior. Tran$plant#l 'epati" e$te o alternativa

    terape#ti"a la CBP "la$a C'il&,P#!' B $i C.13