6 курс / Гастроэнтерология / Аутоиммунные_заболевания_печени_в_практике_клинициста_Ивашкин_В

51

h 2. ? " '- ) "- ( ""

-" =- - - &;; , ' !* -%" ' % +" . n ' , -300–600 % , " +" . ? " " ; + ( )- )-' > "$, = "$ ! - "-> > ) , , $ + ) '- &;;-, ) "-"-> - +$ % % . , $ )*$ , ' $ !+ "$ ! +" ? d &;; "!% " -- ! " – + .

0- "! ) & %-> + +" > -+ != $ +"$ > % $ + ' % '$ ? d. ? " " -+ " "- +" +"$ ! ,

! ! "$ + ( .

`" "!% > !+ "$ ! +" =- ! " ;-) !' = & " "+ "- - – - # & &, -,* )-' )- "-, -&;; .

! # . + " , +"$ - "! -

% , +" ? d `^ ) , $ ' * , ' $($, ,*$ " ! "!$ " (

; ( D. = "$ )"-" "! ; " "$ +" )$($ [Lindor K.D., Dickson F.R., 1999]. - ? d != , $ + " ' +" !"$ ) D +" )> % + ' . ?" ? d % " " D )-' "! ; - , ) ( )" +" D 25–50 - . c + " " " =- ,, ' ' ) " % > % " "-> ; " .

?" ) ! 1/3 ' )-> ? d ++$ '-> + = $ " ' > ' , ' )! "$ $ +-> + "- . + % !$ + ) '-"! ; " "$ , ' +--$ " ")($.

h 2

53

h 2. ? " '- ) "- ( ""

# # #$. < "!$ + %

) ' +! !, ? d. < " > $> +- $

( " ($ + +" - + ; +" +" %" - " )$ = $ " " ( " ($ + +" + . ( ( & > +- " "$ " "

-$. = "$ ! % > )-> % + "> "$)" , , ' - - ) + )-

. % ) " > " +" = --% + + ' ' ; )" , + "-+-,* % !" > " ! )-> ? d [Kurihara T.et al., 1993]. _"!"% ' ! - &;; .

' ! )-> ? d )! +" !* - "! - $ = '-> !, !. b --> )-> , ' +! !,* + % ' -

$ $ - & "$ + " ' $ ' , ($ " )-' % ) " % " -

= " " ' % + " =$ . 9 =$ !--> +" ' " )! ) % ' ) % + > , + & ! +- % " = ! '$ $ > !'$>.

b )-> "! - $ = " > " &;;

-, = "=$ = " * "-

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

h 2

55

h 2. ? " '- ) "- ( ""

' #

) . /. . 9 ' $ ! $" $ ) % $ + $ + ' // # . =. % " & -" ., % + ., +" . – 1998. – №5. – `.13–18.

% ) 0."., !+ ) ) .'. ! ! - ) $ + ' : $ "!% ! ! -+ % // ". ">. – 1993. – №2. – `.63–68.

Andersson S.G.E., Zomorodipour A., Andersson J.O. et al. The genom sequence of Rickettsia prowazekii and the origin of mitochondria // Nature. – 1998. – Vol.396. – P.133–140.

Balan V., Batts K.P., Porayko M.K. et al. Histological evidence for recurrence of primary biliary cirrhosis after liver transplantation // Hepatology. – 1993. – Vol.18. – P.1392–1398.

Bogdanos D.P., Grasso A., Okamoto M. et al. Double reactivity to E. coli / PDG complex-E2 characterises primary biliary cirrhosis // J. Hepatol. – 2000. – Vol.32– (Suppl.2) – 39A.

Crippin J.S., Jorgensen R.A., Dickson.E.R., Lindor K.D. Hepatic osteodystropy in primary biliary cirrhosis: effects of medical treatment // Am. J. Gastroenterol. – 1994. – Vol.89. – P.47–50.

Gores G.J. Mechanisms of cell injury and death in cholestasis and hepatoprotection by ursodeoxycholic acid // J. Hepatol. – 2000. – Vol.32 (suppl.2). – P. 11–13.

Heathcote E.J., Lindor K.D., Poupon R. et al. Combined analysis of French, American and Canadian randomized controlled trials of ursodeoxycholic acid therapy in primary biliary cirrhosis // Gastroenterology. – 1995. – Vol.108. – A1082.

Joplin R.E., Neuberger J.M. Immunopathology of primary biliary cirrhosis // Eur. J. Gastroenterol. Hepatol. – 1999. – Vol.11. – P.587–595.

Leushner M., Maier K.–M., Schlichting J. et al. Oral budesonide and ursodeoxycholic acid treatment of primary biliary cirrhosis: results of a prospective double-blind trial // Gastroenterology. – 1999. – Vol.117. – P.918–925.

Lindor K.D., Dickson E.R. Primary biliary cirrhosis // Schiff’s diseases of the liver. 8th ed. – Lippincott–Raven, 1999. – P.679–692.

Lombard M., Portmann B., Neuberger J. et al. Cyclosporin A treatment in primary biliary cirrhosis: results of a long-term placebo-controlled trial // Gastroenterology. – 1993. – Vol.104. – P.519–526.

Ludwig J. Idiopathic adulthood ductopenia: an update // Mayo Clin. Proc. – 1998. – Vol.73. – P.285–291.

Miloshevski M., Seraphimovski V., Trajanovski M. et al. Long-term ursodeoxycholic acid therapy in the patients with primary biliary cirrhosis // J. Hepatol. – 2000. – Vol.32 (suppl.2). – 157A.

Papatheodoridis G.V., Deutsch M., Hadziyannis E. et al. Ursodeoxycholic acid for primary biliary cirrhosis: final results of a 12-year prospective randomised controlled trial // J. Hepatol. – 2000. – Vol.32 (suppl.2). – 40A.

Poupon R.E. Management of primary biliary cirrhosis resistant to UDCA therapy // J. Hepatol. – 2000. – Vol.32 (suppl.2). – P.19–20.

Poupon R.E., Bonnard A.–M., Chretien Y. et al. Ten-year survival in ursodeoxycholic acid treated patients with primary biliary cirrhosis // Hepatology. – 1999. – Vol.29. – P.1668–1671.

Str%ter J, M ller P. Pathogenesis of primary biliary cirrhosis: CD95-induced apoptosis at last? // Eur.J.Gastroenterol.Hepatol. – 1999. – Vol.10. – P.539–543.

Van Hoogstraten H.J.F., Hansen B.E., Van Buuren H.R. et al. Prognostic factors and long-term effects of ursodeoxycholic acid on liver biochemical parameters in patients with primary biliary cirrhosis // Hepatol. – 1999. – Vol.31. – P.256–262.

h 2

57

h 3. ? " '- " "!,* > %

+" = ! ! !, - &;; % '$ )-$.

3.1. . ,$/

$ " +" " ?`_ ' ! .

?" <o9, ( ($ "- ) , $ 70% !' , $

)-> ?`_ $ 2,4 7,5%. `^ +" )$

+"$ " ' & % +$ 6,3 100 000 [Olsson R. et al., 1991]. "$, & - ( , - !, ) ?`_, !'- $ = % - % ) + % '$ ! ( ( +-- )$ ' + " " ! 20–30% + ( . ?-!, ?`_ " ' $ " = , ' ? d, + ! ' % ! ; , ' ?`_ $ $ $ ) ' -> +" ' " + ( + ' `^ .

3.2. . &

e %$ ?`_ . ' + (-> &- + % ' > ; " " " , $ ) "$ "! $ ; ( , % ' $ +" " + = !- !- " ( .

0 . $ ( ($ ?`_ <o9 %

! +" + =$ + % ' " +"$ ' " ' !, ! + "- " ) " , ) "--> '-> = '-> . _" ' $ ) "$ $ = != +" ' (- ( + "=$ +$ ) " % " , !* % ;- )" !. 0"!% -$ ' !, + "-

h 3

59

h 3. ? " '- " "!,* > %

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

, $ )*$ + ) " "! 3-% + !- ( " > % ) " !, " , ! - +" . ? = )- + , ' "- " "! ! ! "-> , )-> ?`_ " ' , $. )" , %-$ !)- " "! ;- ( & + % ?`_ ! !, .

' 2 #$# / . +" ' + % ?`_ +" $ - ; " .

, $ + $ !' ) . ) ' -$ ( ($ ?`_ % + +-

HLA A1, B8, DR3, DR2, DR6, DRw52a. b = -$ $ $ HLA DRB1*1301, DQA1*0103 DQB1*0603.

= " -= %"!++ )-> ?`_, +--> + DRw52a, + " , )-, %- +

& - + "= , $ " , ' ! )-

> +" =$ .

. $* "$ ) %- + % +" "= , $ % +- "!,* " !- !-> " ( + % ?`_. e % + ) "! $ " ! > % ' > !'$ ' % %! " % ! . , % + " ' -> - % + + HLA (B8, DR3 ".) ( "- " '- !- !- )$, ' + ' .

< "!$ %! " % ! +" ?`_ ,' , :

• % + "% % )! , " +" ) ;"- ( IgM;

•+- - "- ! (ANA, SMA, pANCA ".);

•+-!, ( " ( , ( " ! "!,* > -" '->

) "-> !-> + ;

h 3