6 курс / Гастроэнтерология / Аутоиммунные_заболевания_печени_в_практике_клинициста_Ивашкин_В

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h 4. 9 ) "- + " " - ! !- "-

4.2." ($& )* +$

"$ ) ($ h ? d – +" " % " -> + ( , & + " " - " ) , , +"- !* ! , >$ + - = !' )$ " -> [Gohlke F. et al., 1996]. ), h/?`_ )-' +"-

% ' !, h-1. , G. Mieli-Vergani (2000) +" )

31 " ) " h/?`_ ! 30 -$ ANA SMA, ! 1 –

-LKM-1, = ANCA, -ASGP-R 0h, +" & '$ ( ($ HLA B8 DR3 -$ CD4/CD8 ! .

" " ! "$! " % ' % % ' -

+" h , $ ) > ' " >; )"- $ = '-> +" ) + + ' . -

( ($ +- )$ ' > " "-

, ! % -$ $, "- . 9 !' “' % ” ?`_, = % -!= > % %" ;$ (e#_?h pp_h), +$,*$ )- "!= ! ;- (- " !"- ! "- +- ' '-> = '-> +" . = "$ "$ > %- %" ; ' $ " = +" " + " =

> +" . & ! ( " ,* % ' -

' ) + $ + ' . $ > ! " + ' '-> +" " +" - +" ; " (> + "-> " > ' "!% >, ' '-

; )" "!,* + " > % . < "$! & (

" + " + " % % + !+ '- -- " ; " ; % ; "- ( + " + " + " ) .

0- " !"- ) &;; ! !+" " +" - " h/?`_ +" " '-. "$ M.P. Manns (1999)

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83

h 4. 9 ) "- + " " - ! !- "-

% "!, ! 40-65% )->. `+ " ! ' " ,' ANA ( 28%), SMA ( 11%), -LKM-1 ( 7%), ; ; +- ( 25%), "- ( 12,5%), "- ; ", N , -ASGP-R ". p * % "- & > % , % ' > ' , +--> $ ! ! + % . b 89% )-> "- ANA SMA +"-, 1:80. ` " + -

" + ANA SMA ; "!, ) ' 4% !' . 9" % , " ! +" HCV-; ( -,-

$ +-, "$ +" ! !-> )-

$> " % "!, " % +"- &+ + . , --LKM-1 +" h-2 $ -, % 33 -> -CYP2D6, +" >" ' % + ` " % "!, '- ) GOR.

-GOR $ $, $ , + ( ; '- $ HCV- ; ( , > -$ $, ' ! 80% )->. e+ +, " +- -GOR, ! $ + ;- ( " $" ) , % + "& +" , " % ) ,- , +" % + ( ,$" "( . " , ' -"-

) -GOR ( " HCV-; ( ,

h.

HCV = !* $ $ +- * , ! ; " % ! 2-% +$, = + !,* + "= " ! " )-- ! ) % . ' 90-> % , " !'-$ % + `, " !" +$ - , ' 40% )-> h-1 80% )-> h-2 +- + -HCV.+ = -$ , ' \ 1-% +$ ! ) -)-> = + =- " ! , )! -+ ( ; ' " ( ; -" = % + "% %- )! . ! 11% + ( , !,* > "- "$ = ! " %"!++- + !' , h ' ,* >! !+" !, " + , ,* > " ( + -- " " , ) , $ + =- " ! + " ( + " ( HCV #<9, '

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85

h 4. 9 ) "- + " " - ! !- "-

"-> !'$> " , +" ' % –

( " HCV-; ( " ^ %" '

+ " ' % " ! !, > " "- Ro/SS-A - ! "- ; "- +" - +" !* -CD8+-; ( .

% +e e ) , , + ' ! 4% )-> _h ` +" = $ )" " ) (-

"-> .

j d d . d +"$ $ $ % "- )"-+ > !$* > + +! " -; % ( "

' -> ) ' , % . -HCV -$ -

$, ! 10–38% )->. + ! ! ! % + % + - % $ ! , !' " ;! (- , " % ) " ; "; " " + .

= ;; " ( % "! % !- ! % % + +"$ $ + " !, ' " +" ( +-" '- + > ' , – +" "! $ " +$

+" % + ` ! !+" $ +" h.

f ' " ; ( " HCV-; ( -" =- !-

!- + +" $ )- "! . <- ' α-\<, "- + ) $ $ $ ! " !- !-> +" ( , = +" !>! , ' % '$ )$ + "$ +" %" "!,* + '- ' ' . + - !' "$ ;!-+ ' ' ' ; +"$ α-\< ! )-> _h ` ' " " ! ! ( , +" & =- " % ' +" )- " " -ASGP-R [Meyer zum Buschenfelde K.-H. et. al., 1993]. - ASGP-R > " "- $ h-1, , "$, %" , " + % + " =$ + ' +" & ) . = "$ " "- +" "! % + + ) !, ! , " + ( "! ' +$ > -+" "! " .

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87

h 4. 9 ) "- + " " - ! !- "-

% > % + " ! . ? & % , ! !'-- +-!, ' ! "$ ! !-> = ! )-> % + + HLA DR3, DR4, DR52 DQ2, . . % +- + , ( "- h "!% ! !- )$.

4.4. $ $,$/ ( , 1

?`-1 – " ! -" ( -% )- , + " = ,* +" !* = * , > " " !,*- $ + $, 100% + " ,! $ % % .

?" ' "$ ?`-1 != ; % % ! AIRE-1 ( ! ! % " %!$" + 1), " + = %+ ' 21- >" -. AIRE-1 +" $ )+ "-> "- -> %-> ! , " + =->) HLA !' !,* > " %!$( ! !-> +"- ( . AIRE-1 "! ) , !,* $ $" > % % * ' =-, + " " - !$" ; ' > ! , ; +- ' . "$ % , ' & ) + ! ;! (- ! +" ' , $ " " +(- ; " . + ! ( AIRE-1, +"$* > " , ?`-1.

9 ' $ " ?`-1 +" " )"- - ' " > + : =- % , !- ! "! ( & " " ; [Perheentupa J., 1996; Betterle C. et al., 1998]. < ) +- + " ' > +"$ ?`-1 +" ) ( .

?`-1 )-' ), "! . < ) + ' % +"$ – =- - , "- = " ( "!,* ' . p * % ! ) % + + "- , $ 4 6 + " , ' +" = $

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89

h 4. 9 ) "- + " " - ! !- "-

"!= , +" ) ( h % [Gerbe-Medhin G. et al., 1997].

- - $ +" + = = !* $+ +!$( “->” ! ( AIRE-1, "- +"$ " " ! ; ( ?`-1, %! != +" ' "$ % !> ! !-> ) ' = [Obermayer-Strauss P. et al., 2000]. e "$ +$ )X$ ' !, ) ( , h "!% ! !- )$.

`>- ! !+" " + +" h " > ?`-1' , $ > , +"$ -> $ '$ " % h.

' #

Betterle C., Greggio N.A., Volpato M. Autoimmune polyglandular syndrome type 1 // J. Clin. Endocrinol. Metab. – 1998. – Vol. 83. – P. 1049–1055.

Boberg K.M., Fausa O., Haaland T. et al. Features of autoimmune hepatitis in primary sclerosing cholangitis: an evaluation of 114 PSC patients according to a scoring system for the diagnosis of AIH // Hepatology. – 1996. – Vol.23. – P.1369–1373.

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Davis G.L. Hepatitis C // Schiff’s diseases of the liver. 8th ed. – Lippincott–Raven. – 1999. – P.793–836.

El–Shabrawi M., Wilkinson M.L., Portmann B. et al. Primary sclerosing cholangitis in childhood // Gastroenterology. – 1987. – Vol.92. – P.1226–1235.

Gohlke F., Lohse A.W., Dienes H.P. et al. Evidence of an overlap syndrome of autoimmune hepatitis and primary sclerosing cholangitis // J. Hepatol. – 1996. – Vol.24. – P.699–705.

Heino M., Peterson P., Kudoh J. et al. Autoimmmune regulator is expressed in the cells regulating immune tolerance in thymus medulla // Biochem Biophys. Res. Comm. – 1999. – Vol. 257. – P. 821–825.

Manns M.P. Viral induction of autoimmunity: mechanisms and examples in hepatology // J. Viral Hepatitis. –1996. – Vol. 4 (suppl.2). – P. 42–47.

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