6 курс / Гастроэнтерология / Аутоиммунные_заболевания_печени_в_практике_клинициста_Ивашкин_В
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21
h 1. ! !- % +
$ = ' " ' $ [Czaja A.J., Manns M.P., 1995;
Obermayer-Strauss P. et al., 2000].
$$2 / ( ( , ( 3
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! |
1. ? : |
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= |
+2 |
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!= |
0 |
2. |
` * ' ; ;- f : |
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>3 |
–2 |
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<3 |
+2 |
3. |
9 ( " ($ γ-% )! IgG: |
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- "- ) ' 2 " |
+3 |
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- "- 1,5–2 " |
+2 |
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- "- 1,5 " |
+1 |
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= "- |
0 |
4. |
" ANA, SMA -LKM-1: |
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>1:80 |
+3 |
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1:80 |
+2 |
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1:40 |
+1 |
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<1:40 |
0 |
5. |
< ' : |
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–2 |
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0 |
6. |
< ' HBsAg, -HAV IgM |
–3 |
7. < ' HCV #<9 "!% > % + " +-> "! |
–3 |
|
8. |
< ' -HCV + - " ) % ! ) % |
–2 |
9. |
!- - (6, 7, 8) %- |
+3 |
10. ` +! !,* ! + "- )$ |
+1 |
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11. ?" % + '-> +" + " : |
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–2 |
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+1 |
12. h " ;! : |
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–2 |
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+1 |
13. b+ " ) %$: |
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!='- <35 %, = *- <25 % |
+2 |
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!='- 35–50 %, = *- 25–40 % |
0 |
h 1
22
h 1. ! !- % +
!='- 50-80 %, = *- 40–60 % |
–1 |
!='- >80 %, = *- >60 % |
–2 |
14. h % ' +" : |
|
)!$"- % + + - "- |
+3 |
!+ '- "- |
+2 |
" |
+1 |
+ ' |
+1 |
+ " = = '-> +" |
–1 |
"!% & % ' +" |
–3 |
15. < ' % HLA B8-DR3 DR4 |
+1 |
' " " : |
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+- |
+2 |
' '- |
0 |
! |
–2 |
) " |
–3 |
4 #$$, ,$/)* / , & |
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" #: |
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+"- |
>15 |
"$ - |
10–15 |
#: |
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+"- |
>17 |
"$ - |
12–17 |
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h-1 = " $ ,) " , + ' )-" +" (10–20 + + ! ). \ " "-( "" ' $ ! 43% '-> )-> ' 3 . b ) + ( ) , $ > " "-" !, " + ,, +" & ! 20% >"$ $ $ "-
$ + - ! !+" " .
h-2 ) , $ !* " = (10–15% )-> h)
+" !* ! 2 14 . " - $, 20% )* % ' + ( c " + 4% – `^ . ' )$ > " " ! $ ) - ) > '-% % ' ,. d "" 3- + "-; " "! $ 2 " ' * , ' +" h-1 (! 82%), ' +"-$ >! +" % . h-2 ) " -
! !+" |
; +" + " )-' "- |
( !. |
|
0 +- |
% ' " " %! !- |
23
h 1. ! !- % +
= "!% > " "- $ " '-> + + h -"- '- ! : -ASGP-R, LC-1, SLA, LP.
. 9 ' ! )>-> % ' - > " +"$ $ ) + $ + ' , ,*$ =-+"$ + + % +" (
)$, = ,'$ "!% > % ' >
; " . < )> , ' + ( ; ' % % '
+" h ! !, .
` % % ' ' "$ h +" $ ) -
+ + ' +" "-, > " " !,* $ " + " + " % % + !+ '- (piecemeal)- " , ; " ; % ;-
" ( + " + " + " >, " '
' % ' + ' > (" . 5, 6), ') !' "! , "! !"- + ' , )-' ! ; )" % ! ; " " , ( "" +- ' . d "" )-' ' "- " !$" % ; " "!-$ ; !> ,* + % ' % +" ( - . $ % + ( +" - % " + ' , " =
= " " ; .
1.6. 66 %$2 , &
0 ;; " ( " h )> + " !, ' "
%"!++- ) + ' & % . ` ! -
,' "- >" ' "! - % + , & ( ,!, " % ' " "- % + " +-> "! : HBsAg, HBeAg, -HBc IgM, HBV 0<9, -HCV, HCV #<9, !- - "! e+- "" ( % "! . < )> , ' "! ! % + ` = !* $ $ + * , ! ; " % -! " % +$ = + !,* + "= " ! " )- ! )- % . 0 " ) )! - "$ , + !-
h 1
24
h 1. ! !- % +
# . 5. ! !- % + |
# . 6. ! !- % + |
!+ '- " . # "- |
- " . h + (- |
- + "- "- !+ '- |
- $ -" = % " +- |
- " +- |
' " ; . b' - |
; " . b . × 100. |
% " + ' ' + " >- |
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) " ' + " % " , |
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" + = % +" . b . × 200. |
'$ = + = % " ! -HCV +" +" ! ; " - + " % +$, - ) ! )-> -" = % + "% % )! . = "$ ! 11% + ( , "- !, " "$ h“ ' , ” ! !+" !, " + , ! "->" ( + - " " , ) , $ + =- " ! + " ( + " ( HCV #<9, ' " " > )-> "! - % + ` ! !- +"$ $. ? ' ! 90% (- " HCV-; ( " SMA ANA +"- -1:80 [Czaja A.J., 1998].
b )-> " 40 )> !"$
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25 |
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h 1. ! !- % + |
-" ' % ( "! + |
( , ,'$ ) |
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–9 . - |
-" ' = , " - |
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; "" ; "" , = α1-" + ! -+$$ ,'$ + ' % % >" α1-" + -' . "-> !'$> -)> +"$ ;; " ( % -%- % + , $ " % , , > " "--" =- ! % ' % .
1.7. 1 "
! = +"$ & " + % '$- + "- + + % ' !, ! !+" !, " + ,, " %$% $ , $ % ,- " "-. > > $ +" h ,' $ "! -" ) ( , !' !,* > -! +-> . ?$ ' , h +" - = .
! & / $" )
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! |
" #$ |
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• -" =- +- |
• ` +- ! " |
• + ' |
- |
-" =- |
) ) "- |
• < ! +" %" - |
! !, |
"- $ |
" |
• ` 3–9 " |
• ` <3 " |
• ` ≥10 " |
• γ-% )!- <2 " |
• -" =$ ( +- |
• ` ≥5 " + |
• ? " + "- |
$ |
γ-% )!- ≥2 " |
% + |
• ? "- % + |
• - |
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• <- ( "" |
! )!$"- |
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• 0 + "- |
"- |
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( "" " '- |
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+ * |
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h 1
?" + " -) " != +" % &-; +" + % +"$= + ) '- &;; ! +" ' ! !,* " "-
26
h 1. ! !- % +
. \ " "- " "- ( " (- , ".), "$ ) - !, , >!= + " $ $ )- +" !* $=-+ , " ,* $ +" +" .
` ( , +- $ &;; ! !+" !-
$ - + ) ' % $ " "-) ! " )$, &, +" $,* ) +" 6-" + +!" ) ,* +" ;-
" ,. f ' h +" = .
4+ $" /
%$, $ |
%$ # , |
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$ |
?" : |
?" : |
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60 |
% – 1-$ $ |
30 % – 1-$ $ |
40 |
% – 2-$ $ |
20 % – 2-$ $ |
30 |
% – 3-$ 4-$ $ |
15 % – 3-$ 4-$ $ |
20 |
% – + "= ,*$ |
10 % – + "= ,*$ |
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+" – 50 % + $ |
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+ 1- |
' ( ) & #: |
) & #: |
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+ + ! |
) " |
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+ " |
( +$ |
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' $ +!> |
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" "$ % + "$ |
+ " +" |
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! %- " |
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) >- '$ +" " " !, &;;-: 5- 10- $$ -= )-> ; -! !+" $ 94 90% . -+ ' ) > ' >, " % ' > % - % ' > +" )$, +$,* +" ) +" + " , ) , $ ! 31%. ? " ! !+" " )$ -+ ) + + ' $( '$ % % ' > +" -
27
h 1. ! !- % +
h, + - – " %!$" ' ) > ' -) " = 1 " 6 .
!' ' &;; "-> > -
! !+" M.P. Manns (1999) " ! +- - +" 150 % ! , ' +" = $ !* -- ! ' ' - + ) '-> &;; .
! ' % &;; + >$ + " +" +" , $ $ + +- - '$ "!% > ! !+" " . * +, $ -
$,* $ - ) " -' % !- % , +" + " % > $ > - = , ' ! % )" + % '- > > , !' !,* > + " = + ' +" h."- $ +" "-
" ! ( , ) > ' " |
|
; '- |
$ ( + " ! h-1, |
h-2 |
[Alvarez F. |
et al., 1999]. |
|
|
,' % 1–1,5 %/ % ) !, "-
+ , " " +$ ' % % ' "- ; ( " " , >"$,* $ + "= ,*+" 2,5–10 % ( ; ; – 50 % '- " [Kanzler S. et al., 1996].
? " +- +" $ $ +" " " % +$ +% %, ) ,* % - ;; - , " "- " ( + " ( 15 " - +"- ) - - + ) '- &;; . ?" + " " +" +" + " 90% "= $ + ' ,- > ( " ($> ! + %- ; (-.+" "-> $> + - $ &;;-)! = ) > ' " ! )--> ) ( "" ; " "- + " - !-[Danielson A., Prytz H., 1994].
, – - *- ! !+" ", +"$ - " + % +" !%" "=$. >-% $ + +" ; " ( ;-
h 1
28
h 1. ! !- % +
( ' "!$ +!"-> ! . +-
$ '$ ! !-> ) - "-
& + "-> .
# #. ?$ " + ( + ' +" h +"$, $ &;; , + " ' % !" '$ = - " ( , -" =- + ) '- &;; " ( ' " + :+ " , " " % + " , X$ $ + *- " % " , ) , + . )-'
$ ,'$ =$ != +" + (
( "" , $ $ " +" " + -
! +" %" " )$ +$, " "-
> ' + " ! + ' )-> -
( "" ' |
. - - $ , ' |
" +$ |
' 2 +" = , + " |
, +"- |
|
= ) |
- , + ( = ,' $ |
|
=$ " + ( [Czaja A.J., 1997; Manns M.P., 1999]. ?" % " + ( ) % +"$ -: 5- $$ -=-
+"- 90%, >$ ! !'- = " (- , ( !, " '- " 0 80%. # " (-- ! )-> h-1, ) HLA DR3-+->, " + " ! '$ " + " + ( . `-
# . 7. -= )-> h +" ' ; |
'$ +" |
29
h 1. ! !- % +
! !'-, ' + " + ($ ! !+" -$ " +$ +" " * "= , +$ !+ " " ( h [Salcedo M. et al., 2000].
1.8. 9( ( " &
< '- h + > +" % : 5- $$ -=-$ 50%, 10- $$ – 10% [Roberts S.K. et al., 1996].= "$ +" "-> > ! !+" +-$ &;; " " ' )$ (" .7). ? - A.J. Czaja (1997), 20- $$ -= )-> h ; " + +"- 80%.
' #
-. . ! ! - % + // # . =!" . % " & " ., % + ., +" . – 1998. – №5. – `.47–56.
) . /. ., !+ ) ) .'. ! ! - % + // # . =!" . % " & " ., % + .,+" . – 2001. – №2.
% ) 0."., !+ ) ) .'. ! ! - ) $ + ' : $ "!% ! ! -+ % // ". ">. – 1993. – №2. – `.63–68.
Alvarez F., Berg P.A., Bianchi F.B. et al. International autoimmune hepatitis group report: review of criteria for diagnosis // Hepatology. – 1999. – Vol.31. – P.929–938.
Alvarez F., Ciocca M., Carnero-Velasco C. et al. Short-term cyclosporin induces a remission of autoimmune hepatitis in children // J. Hepatol. – 1999. – Vol. 30. – P. 222–227.
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., Aadland E., Jahnsen J. et al. Incidence and prevalence of primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis in a Norwegian population // Scand. J. Gastroenterol. – 1998. – Vol.33. – P.99–103.
Czaja A.J. Autoimmune hepatitis // Handbook of Liver Disease. – 1998. – P.63–83.
Czaja A.J. Drug therapy in the management of type 1 autoimmune hepatitis // Drugs. – 1999. – Vol. 57. – P. 49–68.
Danielson A., Pritz H. Oral budesonide for treatment of autoimmune chronic active hepatitis // Alim. Pharm. Ther. – 1994. – Vol.8. – P.585–590.
Doherty D.G., Donaldson P.T., Underhill J.A. et al. Allelic sequence variation in the HLA class II genes and proteins on patients with autoimmune hepatitis // Hepatology. – 1994. – Vol.19. – P.609–615.
Johnson P.J., McFarlane I.G. Meeting report of the International Autoimmune Hepatitis Group // Hepatology. – 1993. – Vol.18. – P.998–1005.
h 1
30
h 1. ! !- % +
Lohse A.W., K gel M., Meyer zum B(schenfelde K.H. Evidence for spontaneous immunosupression in autoimmune hepatitis // Hepatology. – 1995. – Vol.22. – P.381–388.
Manns M.P. Viral induction of autoimmunity: mechanisms and examples in hepatology // J. Viral Hepatitis. –1996. – Vol. 4 (suppl.2). – P. 42–47.
Manns M.P. Autoimmune hepatitis // In: Schiff’s diseases of the liver. 8th ed. - Lippincott-Raven. – 1999. – P.919–935.
Meyer zum B(schenfelde K.-H., Hoofnagle J., Manns M.P. Immunology and liver // Dordrecht: Kluwer Academic Publishers. – 1993. – P.277–283.
Obermayer–Strauss P., Strassburg C.P., Manns M.P. Autoimmune hepatitis // J. Hepatol. – 2000. – Vol. 32 (suppl. 1). – P. 181–197.
Roberts S.K., Therneau T.M., Czaja A.J. Prognosis of histological cirrhosis in type 1 autoimmune hepatitis // Gastroenterology. – 1996. – Vol. 110. – P. 848–857.