Вы находитесь на странице: 1из 495

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2002
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.., .., .., . . -, 2002. 496 .


ISBN 5-8249-0078-7

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ISBN 5-8249-0078-7

. .. .
.., , 2002
-, 2002
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, 2002


............................................................................ 9
I. -..................13
II, - ......17
1. - ..........................18
2. ...........................................26
3. .................................34
4. ...................................................38
III. -

........44
1. - .. 44
1.1. ....................................................45
1.2. .....................................46
1.3.
...........................................47
1.4.
.............................................................48
1.5.
.............................................................49
2. ....................52
2.1. ........................................................................53
2.2. ......................................................56
2.3. ...............................................58
2.4. .............................................59
2.5.
.....................................................................................61
3. ..................................62
4.
..........................................................................................63
5. -
........................................................69
IV. -..............................71
1. , ....................................................................................71
1.1...............................................................................................
.....................................72
1.2. .............................72
1.3..............................................................................................
...........................................................74
1.4..............................................................................................
...........................................................................................79
1.5. - .... 79
2. -........................................81
2.1. ....................................................................................82
3

2.2.
.........................................................................................83
2.2.1. .................90
2.2.2. in vivo...........91

2.3. .................................................................................102
3. -.........................................104
3.1. .................................................................................109
3.2. ,
.......................................................................110
V. -...........112
1. .................................113
2. ..................................115
3. ...................116
4. .............................117
5. ..................................117
6. ....................................118
7. .......................119
8.
-..............................................................119
VI. -.........................121
1. -.... 122
2. -............128
3. -.........................................129
4. ..131
4.1. ... 131
4.2.
.............................................................................131
VII. -


........................................................................133
1. .........................................134
1.1.
..........................................................139
1.2. 141
2.
......................................................................................153
3. -............................................................168
3.1. .....................179
3.2. ........................181
3.3. ....................182
3.4. ................183
4. HELLP-.....................................................................192
4

4.1. HELLP-............................................194
4.2. HELLP-..........195
4.3. HELLP-.............................196
5. - .... 197
5.1. ...................................................197
5.2. ..................................................202
5.3. ................................................................203
6. ..............................................................................204
VIII. .....................................210
1. ......................................211
2. .....................212
2.1. ..........212
2.2. ................215
2.2.1. ..........................216
2.2.2. ...........................................218
2.2.3. .............................................220
2.2.4. ................................221
2.2.5. ........................222
2.2.6.
..................................................................224
2.2.7.
225
2.2.8. ......................................................................226

2.3. -
.........................................................................................227
2.4.
, ..............................229
2.4.1...................................................................................
......................................................................229
2.4.2. ...........................................................230
2.4.3. (
) ................................230

2.5.
....................................................232
2.5.1...................................................................................
.................................................................234
2.5.2...................................................................................
..................236
2.6.
......................................................................238
2.7. ,
........................................................................239
3. .............................240
3.1. ..........................................243
3.2.
...............................................252
5

3.2.1.
..............................................................................................252
3.2.1.1..................................................................................
-1.................................252
3.2.1.2..................................................................................
-6...........................................................................254
3.2.1.3.................................................................................
-12 - ............................................254
3.2.1.4..................................................................................
-10.........................................................................254
3.3.
..........................................................................260
3.4. ..........................264
3.5. .........................................265
3.6. ..................................................269
4. ....................273
4.1.
.....................................................................274
4.2. .......................276
4.3. ..............283
4.4. ........................................285
4.4.1..............................................................................................
285
4.4.2..............................................................................................
....................................................................285
4.4.3..............................................................................................
()...............................285
4.5. .........286
4.5.1......................................................................287
4.5.2. ......................................287
IX. -.... 289
1.
-.................................................................................. 289
2. -...............................290
3. ............................296
4. ...................................300
5.
-........................313
6.
-.....................................................................................330
6.1.
...........................................................................................331
6.1.1..............................................................................................
...........................................................331
6.1.2..............................................................................................
,
Raby(1974)...................................................................................336
6.2.
............................................................................................345
6.2.1.
().......................................................................................345
6.2.2.
()..........................................................................................346
6

6.2.3. - III ()

................................................................................348
6.2.4.

F1+2........................................................................349

6.3. .................350
6.3.1...................................................................................

.............................................................................353
6.3.2...................................................................................
...................................354
6.4.
..........................................................................363
6.4.1. ...............................363
X.
....................................366
XI.
............................................387
1. -......................387
2. II
III -............................................................398
3.
- -
- ......................................406
4.
.......................................408
5.
............................................................410
6.
................................................................410
7.
.............................................................413
8.
..............415
XII.
,
-................................................426
1. ....................................................................................426
1.1. ..........................428
1.2. ...................................430
2. ..............................................437
2.1. ..................................440
3. .................................................441
4. - -...................................444
5. ..............................................444
7

6. .............................................446
6.1. ........................................448
6.2. , ,
...................................................................450
6.3.................................................................451
6.4. ................................452
6.5. /..................................................453
6.6. .......................455
7.
...........................................................455
7.1. ().................................455
7.2.
(rTFPI)......................................................................456
7.3. Vila ........458
7.4. III...........................................462
8. ... 464
9. -.......466
9.1. ..................468
9.2. -.....468
10. -.............469
11....................................................................471
12..............................................................................472
13...........................................................................473
........................................................................................476


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1

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196,251,5

167,448,3

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1,460,53

35,020,0

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292,485,7

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70-120%
70-120%
70-120%

4,0-6,5 /
100-120%
100-150%
150-250%
200-500%
100-150%
150-250%
50-100%
100-200%
35-75%

2.4.
,
III , . (. 19)
.
AT III - 1-, 2-.
AT I l l y
59

.
, III ,
AT III. , -, , -, - ,
AT III
, , - . ,
.

. 20.

60

(. 20).
S,
4-
. S, 40:60. - S. 2-, III, : . ,
S.

2.5.

,
, , ,
.
,
-
. , , PAI,
. , AI
: PAI-1 PAI-2. PAI-1, ,
, 1-2 -
,
PAI-2, PAI-1. , ,
, .
PAI-2, , , PAI-1 PAI - .
, , - AI-1
- ,

, .
61



, - (PAI-1, PAI-2). PAI-2 .
.
PAI-2 . 2- - -
.
, - .

,
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.
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3.

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-
62

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4.

:
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, ,
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. .

,
, .

. .


, .

2 ,
63

.

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.
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.
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,
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(3)

.
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5- .
65

, , , . AT III - . 3- , AT III.

, (PAI). . .

, PAI-2
. .

- ,

, .

. 15-60 . , 24 . . .
XII,
, .

, , -
PAI-2 .
VIII- .
, VII, VIII .

. 66

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, . , , , .
,
.


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.
.
2 , .
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67

2.
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, - , .. );
3.
I I I , ,
() (

-,
);
4.
, (
, , , -
,
);
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.
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. 68

15% . II -III -
( 1-
)
.

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. . , .
.
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5. -



, , , . , : ;
.

.

, ,

69

.
.
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.
,
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,
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, , (HELLP-)


,
-, , ; , / ; ; .
71

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, , . , , , , . , -
. ,
,
; , , .
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.
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1.2.

, 72

-
Neisseria meningitidis

Enterobacteriaceae sp.
Salmonella sp.
Haemophilussp.
Pseudomonas sp.
-
Pneumococcus
Staphylococci
Hemolitic streptococci

Clostridiumsp.

Mycobacterium tuberculosis


III

S
II IV



Aspergillus sp.
Histoplasma sp.
Candida sp.

( )

IX
IX
XI
2

, ,
, HELLP-. , , . , , , ,


, ,
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( , ) .
, , . , , ,
.
, , ( -) .

1.3.
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, . (. 22).
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(),
, ,
(vW), , .
:
, : - 74

. 22.

( )


/ S .
- (t-PA)
1 (AI-1). In vitro

,
IL-1 , .
,
,

,
.

/
, , -
.
75


. -
: , , ; g , , ,
, , , GpIIbllla,
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. :
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76

, , ,
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,
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, ,
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, TFPI.

, ,

.
, V, VII, VIII .
,

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, , VII, IX, XI, XII, III.
: , . ,
, , , II.
, t-PA - (
,
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- , , , ,
), 1-1 PAI-2,
, , ,

.
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,
,

.
, , / ,
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,
, .
, , VII, X, IX, II,
. ,
- ,
, .

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, , ,
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, .

1.4.
.
,
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. , ,
-
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. , , , . .

1.5.
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1. HSV 1
, . ,
, -. . -,
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, , . 79

, .

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, .

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.
.
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.
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30 CD-14
. . , .
, 80

. ,
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), - -
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2. -
, - ,
.

. 23. -

, ,
- .
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,
81

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; 3) .

23.

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-
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) -. - .
, . ,
.
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.

. In
vivo
. . in
vitro , .
, 82

-.
- ,
- .
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, ,
, , , -.
,
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,
. ,
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. , ; , ,
, . in vivo , ( )
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2.2.

, -, - . .
, 83

. , ,

.
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, , ,
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. 24.

, - - - . - , , .
.
,
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().
,,
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84


, . , - . - - 1 (PSGL1). , . - (CD1 la/18, CD1 lb/
18, CDllc/18),
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10


;
;

;
PAI-1

,
;
S

1 -
; -

;

, 1,

-
,
;
.

, (. 10). , ; . , , , III 1- , 1- ( 85

) .
4- ,
S. . -, ,
-,
.
-1 - in vitro,
in vivo . -1 - , .
40- , . -
-1 . -1:
, , ,
. , 1 . -1 . , -1 ,
-1 . ,
, -1 .
- .
- .
--
in vitro , -.
, -, , , , , -, -1 -6.
, -

.
-
: , ; - . -
86

, , , . ,
, , (, ) , , , .
-1 -6 , :
;
,
. ,
,
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, .
-, , PAI-1- .
- . -6, -6
.
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,

. -6, -
.
25.
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, . , , .
87

. 25.
-

, , . . :
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.
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88

.

. .
V. -,
, ( ,
) - (SAPK) - ,
,
.
,
, , 2. . , , .

- PAI-1.
,
. (, HELLP-).
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,
. ,
.
, .
2 2,
, , .
, . in vitro
.
. , .
89


- . , , - .
(.. , , ).
2.2.1.

, , ,
- , . ,

. ,
,
.
, ,
, -, -6.
, , -1, N0, , , . ,
, , , , , ,
, .
, .
4 109 . - , .
.
III,
80% .
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90


- .
12, , 2,
.
.
, .
. Gaut et al ,
, II,
, . ,
.

, (MgSO4)
. , , (I2).
- (
).
, - . - ,
. ,
. .
2.2.2.
in vivo

,
91

, -1,

. , .

(. II).
.
, , ( 12) - .
,

- . I2
,
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. , 1 2
, 2
. ,
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), . 2. , , - .
, , 1 2 .

,
12 .

,
, S III.
,
, , , .
92


.
.
, PAI-1, , ; , PAI-1 .
PAI-2 . PAI-1, .


, , .
-1,
. , , . .
.
, .
, (cFN), (ED-A ED-B),
. ED-A .
, .
, cFN
,
,
.
, cFN,
, cFN II . ,
,
,
cFN .
93

, (VCAM-1) -,
II ,
.
,

( ,
.), ,
.


,
, - . Taylor et al., . I 6-12 .
, Taylor et al., ,
(IGF). , (VEGF, vascular,
endothelial growth factor), . VEGF .
,
, - -1 (-1).
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. ,
,
.
,
. , -1 , , .
-1
, . -1 ( , .). -1
, .
94

NO 80-
, .
NO ,
.
NO , , . ,
. NO
.
,
NO
(Pgl2), ,
, N0 Pgl2 . .
(, , ) Pgl2, NO,
( .) - . , , , ,
, N0 ,
- .
NO ,
N0
. ,
.
,
, , . Dadak et al., .
. Davidge et al. ,
cFN. , .
95


, .
, .
(, , ,
.) ,
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- ( ) . ( ) .
, ( )
. , .
, ,
, , . ,
, , .


,
. , ,
, . A (LpA)
. LpA
, , (-) -100 - . , -
, , , . LpA
.
, ,
. , 96

. .
.
, : , .

. 26.

02 - ,
(), ,
, . -
(22) .
.
(), . GSH - , GSSH - ,
- .

, ,
, , (ROS) - , ,
97

-, - - . , , (22), (1) -
(. 26). .
, .
/
( ) in vivo.
, -, - . .
,
, (- [SOD], -) (, ). ,
, (scavenders) .
( [ ], - -, ) - - ( - ( ) .).
()
(.27). , , .
, ,
. , , .
/
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( ; ) 98

. 27. -
()
,
( LH)
(L-).
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, (
) (LOOH) ().
,
( n+/n+1),
(LOO-, LO-) (- ).

( .)
.
, .
, .

(. ).
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,
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.
99
4*



:
- ;
- .
2. .
3.



.
2.

.
3.

13-
, ,
.


1.
.
2. -

.
3. (PGI2)
.

1.

in vivo in vitro.
2. .
3.
PGI2.


in vitro
1.
II.
2.
PGI2

{ 24
62 ).

1.
II
.
2.
PGI2
2- ,
(48-72 ).


1.
.
2.
-

1.

.
2. ROS
-

in situ.

in vivo. 8-
,
. 8- () in vivo. 8- , 100

12

-2 GI

III

, -2 GPI

-2 GPI

2 GPI

PAI-1

11-

-2 GPI

-2 GPI

-2 GPI,

,
8- . 2 /
8-.
,
.
,
, . -,
, ,
,

-.
101

2.3.
-
() - , / . , , .
- ,
8 .
.
, , . , ,
, , , (
, , );
(, , , , ). ,
. 2%
,
0,2% - .
,
(-) (-). ,
, , . - , -, 5:1. - , , -
.
-,
-.
, - .
Conley
Hartman.
102

. , .
( IgG, IgA,
IgM), in vitro -
, , . .
, (2-GPI, V, X, .
,
. 1983 Harris,
Gharavi . - , IgG
(2-GPI .
, .
V, , .
, , , .
- ,
. , . , ,
. :
2.
, -1-GPI,
, ,
. ,
2.
.
. (32-GPI. , -,
.
103

, , :
1.
;
2.
,
;
3. ,

, .
,
, , .

3. -

: - , , - , ,
- ,
. ,
, .
, ,
XI.
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,
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: ,
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104

.
, - ( , ) :
.
, - - . ,

, ().
,
-. - -,
,
. , , .
-1,
-6 , , , . , . , ELAM, (). , -1
ICAM-1, L-; ,
, , - , , .
, , , . ,
, () ,
() ; ( ).
,
- ,
( ) ().
105

- X,Y,D,E.
-,
.
, ,
. ,
-,
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,
60109/ .

-
V11a

, AT III, , S.
, ,
, .
-, ,
(

. -,
,
,
.

(, - ).
-,

-
.

, AT III, S.
TFPI
- , ,
. , TFPI
,
. TFPI -
.
- III. III 80%
. I I I , . ,
III
40-60%. III -
106

. III -. ,
- - , - ,
,
III. ,
III , - .
(-), .
, S , . in vitro ,
, ,
. (, ), .
- - :
Va Villa. , , . .
, ,
- . ,
, - , -.
S
.
, , ,
, -:
,
. , , . , - V, VII
XIII.
, .
107

- .
.
-,
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, . ,
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- .
, . , .
. -, -,
,
- (),
, .
, D ,
- ,
. / -1, -6
1. 1,6
, ,
, .
: ; - ; .
, - . ,
- , , ; XII, IX, VII, 10-20%
.
108


-: , -
. ,
,
.
- .

3.1.
-
-
,
.
. ,
. , , , - .
, ,
, .
, , . , , (
- ),
-1 (1-1).
, , . ( ) , - 1-, 2 .

, , , .
109

3.2. ,

( ) - , . ,

- .
, . ,
,
.
.
: -,
; -,
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. ,
.
-,
2-3
(FPA) F1+2, 60% . ,
.
-,
.
-, , - ,
, , .
,

. , , , .
, 4 ( ),
110

S,
.

, , , . ,
, , -1 ,
- .
in vitro , PAI-1, .
.
,
.
- . - (, , ..)
, ,
-. .

- ,
.

V

-
. , ,
. , , .
, ,
.
, , ,
.
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.
, .
, .
, , . , . ; - , - . - , , .
.

- ,
112

. , , ,
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.
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.
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.
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.
.
.
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,
.
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1.
113

, ,
. , ,
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2-3
, . .
,
.
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: , , ,
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:
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,
.

.
.
.
.

. ( 6-12 ) ,
. 1224 , , ,
.
114

2.
.
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)
. . , , ,

.
, .

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, : , , , ,

. , , .
,
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, ,
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.
,
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, 115

.
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,
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:
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.

3.
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,
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.
, . .
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. .
.
116

4.
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.
. , .
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. .
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5.
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,
. ,
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. 117

, . ,
.
.

, , , - , - , .

.

6.
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, 65%, - 52%,
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,
- 39%,
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(15%),
. .
.
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7.
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.
118



, .

.

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8.
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.
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- 119

,
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.
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.
,
.


.

VI
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.
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. 121

: , , , ,
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.
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,

. 2- , .
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; ,
;
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1.
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, , , 122

, . , , , , , , , , , , ,
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123

, , , - . -
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V, VIII, XIII. ,
.
, . , .
. ,
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. . : 1008-1014, 300-400 /, / 1,
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.
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<16(<400)

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< 4 (100)

1008-1014

=1030

250-400

>900

U/P

=1

>1

U/P

<5
<5

<10

. (>5 /)

. 124

: .
,
. . .

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.
;
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,

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2,
2.
. . (PEEP). -
.
125


. -
, . -
. .
25 .. - . - , . . 2;
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, ,
.
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. .

.

.
.
- , . ,
-.
- 10-15% , . Brain Hourihane ,
- , ,
.
-.

.
, - , , , , . 126

. , , , .
.
.
Brain ., , .
, . -, , . ,
.
-
: , , , , .
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90% , - 65%,
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43%
, - 39%,
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- 17%.
(15%), .
.
-
.
.

.
-
(),
.
15-20% - , ,
, -.
10-15% -
.
-. .
127

- . , . . , , -
.

2.
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- . ,
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15
,
-
-


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HELLP-

,
( , ..),

.
. .
128

, ,
, , , ,
.
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,
, .
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( 95% ),
(95%), ( ) (72%), , , , -
60% .
-, 10% .

3. -
- .

. - .
, , , .
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, (F1+2, ). AT111. , .
, ,
-.
.
, , .
, ,
129

. ,
(, , ). ,
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- ( ) -,
() , .
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, .
,
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. , ,
.
40% . , AT
50% .
F1+2, , ,
-.

,
,
.
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.
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, , 130

, . . 15

, / .

4.

4.1.


. .
. .
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. ,
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, -, .

4.2.

-
,
.

-.
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131

, .
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. , ,
.
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.

VII



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.


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.), (), ,

100%.

, .

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.

,
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1.
()
. , .
1893 Schmorl
. , , , . .. (1896) . J.R.Meyer
1926 .,

. 1941 . P.E.Sterner
C.C.Lushbaugh 8

. 1:8000 1 : 80 000 . , 88 ,
, 44 (50%), 5 . ,
134

, .

, . ,
.

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1 .

; , ;
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.
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135

, ( 2
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, . ,
, .
, ; 20%
. 25%
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.
() ,
.
,
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12 1000 38 . 16.
16

Na, , Ca, Mg, Fe, PO4, Cl, S, Mn, Zn

, , ,
,

, , ,

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E1,E 2 ,F1,F 2

, ,
, ,

6,9-7,15

136

II .
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.
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%, , , ,
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.
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: ;
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.
,
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.
,
,
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,
.
(). .
,
, .

137

.
, .

( , ),
. 1998 Gogola

. , , , . , 60%
. Clark (1995)
19%
.
, ,
,
. ,
, , ; ,
- , , , (. 28).

50% . 50%
-. , I II , . , , , , ,
, grand mal.
, , , - , ,
.

.
, ,
138

. , .

.
,
30 , 8-9 , , . . . , , .
2-4 , .
, , , .
, , ,
-, , .

1.1.

, , , .
, , , . , ,
, . . ,
, , , 139

.

. ,
, .

. , .
. , ,
, , ,
.

, -
. ,
, . , - .
, , -. , , . , , .
, TF- , X, , , , .
, , , .
.
, 140


.
, - .
(
) ,
, .
,
, , ,
. , , , - .

, , .

1.2.

, , , , , , , , , , . - .

. - , , .
, .

,
141

.
.
,
, .
,
, .
, ,
, I . - ( 5 ), (5-10 ). ( 4,5 /), .
, .
.
(X, Y), - (D, ).
III .

( r+k) ( ) .
,
I

.

, ,
. , ,
,
. ( r+k) (
142

ma ) , ,
(. 29, 30).

. 29. Raby
1. ; 2. ; 3. r+k ()=2

. 30. Raby

()



.
.
r : .
, ,

. ,
.
, - . , ,
143

. , , .. ( , , 4
).
,
.

, .
A (%
): A (%
) 1,4 . , .
III, , 300 / 2 /. , .

. , .
,
. , .

,
, .
,
.
, , - .
144

I
, II III

.
.
-
.
,
, , , , , ,
(), (), , , -
.
, ,
, ,
:
1);
2) ;
3) .
,
, -
.
: 1) ( 15 /); 2) ( 25
..); 3) ( 70
. .) ; 4) - ( 350
.. 100% ); 5)
.
- . (2-20 //
), .
. 145

,
.
, -.
, , .

. 25-30%.
,
.
, ,
500-700 . ( ), .
,
,
.
,
, -
.
, , VIII, V, VII, X, XIII, III, ., , , .
3-4
. , ,
(500 ) (5000 4
10 ) , .

, 146

.
, ,
250
000-500 000 100 000-200 000
. ( .).
.
, , - , .
() .
, . , , , , , . (, ) . .
, : , , ; , ,
, , , . , , ( , ; , , , , ,
.
, , . 147

,
. .
, .
60% . , , , -. XIII
.
.
, : ; (. 35); :
, ;
;
, , - ,
, ;
.
1
., 30. 06.03.1992 . 5
.
: 40-41 . . I ,
.
14.03.91, 9- .
- 4- . . . .
170 . 86 . 110 . 44 .
: 5 ,
.
1981 -, 4200.
1983 - , 4000 .
1983 - , /.
1985 - , 4200 .
1991 - .
, - II-III
- , - .
148

, , .
1985 ., 3- ,
, .
530. 120/80 -110/70. ., 78
/, , . , , , . 6 .
7 00 . , 4200 , 52
, 8>8 . , .
7 10 . ,
25x28 . .
150-200 . 1,0 /.

, . , . 30 . 120/90 -130/
100 . ., 98 / , 90/60 . .,
110 /.
8 ,
().
Citol
02.
S.Euphyllini 10,0 /
S. Corglyconi 10,0 /
. 10,0 /
S. Prednisoloni 30 , /
S. Droperidoli 30 /
S. 2% -1,0 /
S.Suprastini2,0 -/ S.
Methacini 0,5 /
S.Dibazoli2,0/
S. Papaverini 2,0 /
, 8-10 . : , , , , . , 18-20 .
, , , .
120/80 . ., 88 /, .
.
149

. 31. .
I -

. 32. . I -
1. ; 2. ; 3,4 -
.

. 33.
1. ; 2.
; 4.

. 34. .
II -

150

: .
. I .
. , ,
/ 500 .
/ / ,
.
: .
:
.
: ,

- (. 31,32,33,34) - /
(F.S-mecm, STAGO France, D- ( ).

I .
- -
.
111 .

, - AT III (Ku-).
: , , .
, , , .
200,0 / 2 .
2500x4 /.
30 / 4 .
1 4 /.
!

, , - -:
.
12.03.91. - ^.
14.03.91.
.
151

. 35.


(, , ) , ( , , , , ). , .
, .
, , - , , :
152

. , , - , .

2.

, , , .
0,2% .

1901 . De Lee , (
) .
1936 . Dieckmann , 25%
-
100 /, .
Jackson Pritchard 1955 . ,
.
Schneider (1955), Seegers (1952), Weiner et al. (1950) Page et
al. (1951). , . , , Chargaff (1945).
Obata, 1919 .

. , (9 ),
.
, , ( , , , , ), . , Brenner et al. (2000),
153

,
, ( V
Leiden, AT I I I , ,
.) . , ,
.
46%
. 21 % . ( 55,6% ), (44% ).
, , (. 17).
17

.
.
-


.
.

()


.
, ,
. . - .
30%

,
,
,
. .
,
.
, , ,

. Placenta praevia.
154

Mo :
, (
). ,
, ( ) 1000 .
,
, . , ,
,
. , .

2/3 . .
,
. ( ). .
, , .
, . ,
- , , , - .

. .
. , ( 5 ), .
, .
70-80% , . ,
.
, , , .

.
155

, .

. . . , , , , . , .
,
.
, ,
.

. 36.

,
- , , .
156

. ,
, , -. - ,
, - (. 36).

. ,

-
,
.
,
,
- , ,
, - , , , - ,
- . ,

, ,
, .
, , .

II III -. , ,
, , r+k
. 157

III
. III
, III (XII, XI,
IX, VII,V ).

. ,
5 ,
.
.

, , , , , +k, . , 50 III
3 .
() I -
.
, ,
, . 25% .
75% , 10%
, .
,
, , . , , , () ,
,
- .

- X Y , 10,24 - 163,6410 3/. 10% ,
158

.
.
, 40% . : ( 30% , - 1,5 / 2,0-3,5 / - 10% ,
1,75 / - 15%
.

- ().


.
,
, .
,
, , 60x10"
6
/. AT III . , AT III 52-76% , , AT III
0,310-0,343 /, AT III. ,

, , .
AT III,

-,
- , ,
AT III.
AT III, , ATIII . ,
-,
159

. ,
-.
()
90x109/ 120109/ 25% , . 75% , .

(, ,
).

20-33% (: 30-50%).

PRP ,
, . ,

- . -
.
, , ,
-
.



, ,
.

- 160

, , .

(II III )
-
.

,
,
.
,
.

. .
AT
III ( ) , 12-27% , , ( ) . . ,
,
, 2,5
, 13-2710-6/. ,
() -

- 161

- 80 000-100 000
, 20 000 /, 5% / 500-1000
(2000) , , 250-500
/, /. II III I
200,0-400,0 (500,0-600,0 ) 10 / .

5001000 /, 1-2 - 25005000 4 15 000-20 000 /.
(150,0-200,0 ),
AT III 54-77%.
15000-30000
/ - X-Y
- , r+k
, (/), 1,5-2 .
II III
, .
-

,

.
,
- I ,
- ,
.

162

,
, . ,
, , .
,
34-36 ,
, , . ( 3-4 ), ,
.
, , ,
, .

4- .
, , , .
. ,
.
. 250 /.
( ). , , , .
,
.
,


1 . , , , - .
60% -. ,
.
163

. , , , .
, . .
, ,
,
. .
.
8%
. . .
. , , .
,
. , , , .

,

. .

, . .
I . , I II

-, , , 164

. .
.
, ,
. , , , . , .

.
.
-, , ( ). ,
Rh ,
.
. 600-700 .
. ,
. .
25-30 /.
,
3:1. .
, , 4-6
.
. .
, ,

, .
. , .
165

. 37.

.
37.
.
, ,
, , , . , .
, 166

.

. 450 /. 300 /,
. 150 / .
, . .
: 100 / 4-6 ( 1 )
. 2
. ,
20000 , 1-2 . (300
1 ) (8000 /3 1 ).
,
. ,
. , . .
, ,
, .
, . . ,
.
, ,
.

. -

. 2-. .
167

3. -
, , : - -
.
1,5 23,2% .
.
5,7 13,4%.
.
. . , , ,
; . ,
677 ,
, . V Leiden, , ( ) , , .
, :
; ,
, , , , , .
, , ,
,
- , . 18 ,
.
,
70% . , ,
168

18
,
( . Brenner ., 2000)

HELLP

A III

S

++
+
+
+

++
++
++
+

+
+
+

APC-R
V Leiden

++
++

++
++

++
++

+
+

+
++

MTHFR C677T

+
+

+
++

++

+
++

G20210A

+
++
++

+
++
++

+
++
+

+
+

++
++
++

:
- ;
+ - ; ++ .

MTHFR C677T , V Leiden, .


( 50%) MTFR
677, , ,
(. 38).

, .
,
- .

, ,
.
,
,
. ,
XIX , . XIX , , .
XX , 169

. 38.

, ,
. ,
.

,

, , .

1953 . F.W. Page (1953) D.
McKay et al. (1953), - .
, ,
. , ,
.
. -, ,

,
,
. , 170

. ,
- - . .
- , . ,
.

.
.
,
, , . , ,
, , .

. ,
, .
- ,
- . - . ,
;
. .
, ,

. , .
, ,
.
,
171

, .

. , .

.
.
. , , . ,
: , , . , .
, ,
.

, .
, (11-15 2),
, (150 3), , , .
, .
; . , . .
172

.

-
. .
,
, ,

,
. ,
, .
- ,
. , ,
- . ,
, , . ,

,
. , 2
, - 8 , .
, , , , , , , , .

, . , , . , , . , .
173


, ,
-
. , .

() . , , , , -.

. , - , F1+2. , .
, 3 . III, , .

.
r+k , ,
,
.
,
.

.

. -
.

, -

174

(. ). , , , . . ( )
( , ), .
,
, ,
r+k , III
(, , -).
-


.
. -
(32-34 ) ,
,
.
, -
III () ( ). ,

.

-, - . - , .
175

- 110-3, 110-7 ,
-,
8-12 30-45 .
- (I ) -
,
, .. .
- ,
.
-
III , - (I ).
( 22-35 106/)
, AT III
.
, - (II
I II )
- -
. ,
, , -
.
, - .
,

176

, . , - - -
AT III
68-88% ( 79,4+3,1%, <0,05) AT III ,
, AT III . AT III , ,
, AT III, . , AT III 0,382+0,045/ 0,320/ 0,450 / (: 0,300 /).
AT
III ,
AT III , .

() - . -, - - ,
. ,
(),
.


AT III,
.
- , II . , . ,
,
. 177

, .
. , . , (-).
,
. ,
.
.


.
.
, , . , .
. , . .
- .

. . , 40%
20% - .
.

, ,
178

.
, - . .
, , . , , , , ,
, ,

.
,

, . (
20 /), ( 35 /)
III ( 45%).
, , .
,
. , ,
, - .
,
,
, .
, .

3.1.
, ,
,
() 179

. , .
, , ,
, .
, , .
, , Nadji, Sommers, De Wolf et
al. , tunica media ; . , ,
. Brosens et al., Kitzmiller
Benrischke,

, .
( )
.

,
. , .

, .
,

. ,
.
Sheehan Lynch -
; , .
180

3.2.
, ( )
, 1000 2. ,
. , (, ,
, , ,
). , , .
. (,
2) (, NO Pgl2. - , - () . ,
,
.
.
: )
; ) - ; ) . ,

, .
,
. Gaut et al. , ,
II, , . ,
II .
181


, (MgSO^)
.
, , .
- ( ).
,
- . - ,
. ,
- . .
III.

3.3.
, . .

. , () ;

.

, , . , G
1528 ,
182

- .
, .
, ( )
,
. Sattar et al. .
- ().

; , .
, . - , ; , .
,
,
() .
.
, () .
-
.

3.4.

.
,
,
, , , .
183

.
, ,
( 19).
19

- : ,
, , , .
-
18 24 , 0,58.
II 28 .
.
.
.
: V Leiden,
G20210A, .
, , ,
.
.
.

, , , .
,
, , , . , , / , .
, 10

- . ,
,
, .
(50-81
/).
2
. , , 184

II . , . ,
( , , .), .
, , . , -,
, . , , , ., , .
, , ,
, ,
. , , , . MTHFR C677T 4 .
, , .
,
( , ), .
, ( V Leiden, G20210A) .
,
.
, (, , ), 185

(, ) 111
.
( MTHFR C677T, V
Leiden).
2
,, 31 . : 5 . . .
: .
1993 ., ,
32 . 950 , 37
, 4- . 1998 . 26-27
. 1000 2- . 9.12.98.
,
26-27 II .
.
: - 38 ( 20 - 40 ), - 106%,
+/-,- =1/ (Ndo 0,5/), - .
, , 65%, 86% 57%
.
,
, ,
: MTHFR C667T, FVLeiden, PtG20210A.
7 '- MTHFR C667T.
:
-31,52 N -8- 10/.
: 7-8 .
. . . ( MTHFR C677T).
HMF- 0,3 , 4
, , 1 186

, (-3 1-2 ).

, D.
. 34 36
- . 37
.
- : I-II
-- .
D, - . ,


67
.
48 ,
0,3 , 2 . 2 8
, 10 . 67
: 37 . -. .
. I-II. . .
,
,

37 . 2 700
, 47 . - 7/7 .

, 32 27 .
, .
,
,
. 3-
MTHFR C667T ( )
.
, 38 , .
187

3
., 45. 67 : 26 . . I-II . . .
: - . , 1993 ., 32 , . 1994 . 10
. , , 10-15. , 200/100 . .
, . :
: . , .
: . . . . -
( + + +,- + + > 3 / N= 0,5/).
.
- .
: 26 . .
- 140 .
24. I-II.
.
, , , -

: FV Leiden, PtG20210
MTHFR C667T.
FV Leiden. :
.
: .
. - (
-; - - ,
F1+2.
: 29-30 . . I-II .
- . . . FVLeiden ( ).
- ,
- 30 .
188

,
30 .
, 1600 , 2- . 6
5000, - 10 .

V Leiden . , , . ( III )
V Leiden , , .
. ,
,
. , ( 9 ,
7000 , ) , . ,
- .
, , , 2, , ,
. , ,
, .
,
, - .
.
,

,
.

, ,
,
189

. , - (, , , .).
, .
, .
,
, .
( , , , , .) , ..
.
, .

.
20


1. 6 MgSO4x7H2O 100 15-20
.
2. ( 2 / 100
.
3. 4-6 ,
4,8-9,6 /.
4. 24 .
"Pritchard"-
1.4 20%- 1 /.
2. 10 50%-
(5 ) ( 1,0 2% ). , 15 2
/ 20%- (, 1 /).
, 4 /.
3. 4 5 50%- /, , : .
. . . .
4 100 .
4. 24 .

190

, , (MgSO4x7H2O) - , , . , /
24 . ,
.
.

(. 20).
.

4-6 . ,
, .

, ,
, ( , ).

, .

, . ,
(, ) , , ,
, . ,
( , ) .

2,5-5,0 20 .
, ,
, , .
, .
,
191

- ,
.
(
, ) , .
,
, .
AT III,
, .
AT III .
, AT - , , . ,
Pgl2 .
AT III

PGI2 ,
.
4. HELLP-
HELLP- (, , ) Weinstein
1982 . .
- , , , , ,
. , , , , 7
, .
HELLP- . 0,5% 0,9% 15% 26% .
1,0% 4%, 40%. HELLP :
.
, , , , ,
192

, , .
,
, ,
, ( 21).
21
HELLP-


,
,

HELLP- ( ),
, . . , , .
.
- ,
( ) -
( , , ).

,
, .

(

, ..).
35%. , 193

HELLP-, 1,8%, 58-70%.



.

4.1. -
HELLP- .
HELLP- () , .
, HELLP- ,
. - -, , HELLP- , . HELLP-
- ,
- - . TNF-
HELLP-, , , ,
HELLP-, .
. , - in vitro.
,

,
(
, PAI-1 VIII, - AT III ).

PAI-1. , ,
, . ,
, 194

. - ,
, ,
, .
, ,
.
,
HELLP- , , ( ). , , I II

, III - ;
. , HELLP- .
,
( ) , , .
3 . , , - -
.
HELLP- - .
HELLP- , , , . - 38%
HELLP-. , , , , - HELLP- 5%.

4.2.
HELLP-
HELLP-, . , -, 195

,
.
, . , -, , , , , -1. ,
,
2. ,
HELLP- .,/ I2, , , VII, .

, . . , ,
.
, , III ,
-.

4.3. HELLP-
HELLP-
,
. , HELLP- .


(); - .

,
: 6% 10%, 5%, . 196

70 /. 40
, -

100%
, - .

, ,
, .

(4-
12- -10,10,5,5 )
, .
,
.
,
,
-
.

5. -

5.1.
, , 2-3 . De Lee 1901 .
. 1950 . Weiner
, II -.
,
. Weiner (1950)
-, .
, , , , (, , .),
197

-
.
. . .. .

: 14 20
19 , 21 25 14 .
: 13 19, 14-20 14 . ,
4 6, 4 - 27 ,
,
1,5 / 1,85 / (: 2,03,3 /), 85112 75-98 (: 60-70 20-40 ) 6880%, - . (24%) (36%).
52% . ,

- 40-61x10 -3/
36,4% .
24,2% AT III 54-86% (:
100-120%) (81%)
40-60x10-6 / (: 3x10-6 /), .

,
() (), -
. 24,2%
-

( ).
27,3% , 198

() (). -
( ) - ( ).
- , .. .
, ,
. , , , , AT III,
. , (X Y) 163210-3/.
,
, , -
. III
0,320-0,360 / (: 0,300 /), III,
- III (). AT III. AT
III -
AT III. AT III
AT III. ,
- 406010-6/ (: 10 -6/). ',
AT III 54% 119%, 54-72% 12% , 15%
AT III 84-86%.
199

, ,

6% .

(78x10-9/
- 14810-9/).

1x10-3 1x10-4,
.

-, .

19
13 6 14-20 - ,
.
(1,25 / 1,5 /) ,
300,0 .
900,0
1200 ,
.
() 80 000-100 000 / . 20 000-40 000
2 5% - 5,0-10,0 6-8
(, , ). 200

-
-200-400 .

.
,
-
-


.
,
, .


2,25 / 2,75 / .
, . AT III
84% 115%, 0,300 / 0,350 /,
. , .

, .
, (PRP), ()
, ,
.
,
- (II )

201

. ,
.

- , , +k , / ()
.


. 5-10 .
4-5 1000
24-48 . 8
.
(200,0 -400,0 ).
. , . . , .

5.2.

III ;
30%, - 50%.
, ,
, , VIII, V, .
,
, , () .
202

5.3.
.
,
.

.
.
,
. , 46 XX. (69 XXY).
, -
. , 90% . , , .
.
.
,
.
. : , , , - .
, . (- ),
- . .
, , , - ,
. -
203

, , .
. . .
.
-.

, , ,
. , , .

6.
,
()
, - ,
,
, ,
- ( .., 1987; ..,
2000). -

- (
.. .., 1989; Muller-Berghaus G., et al., 1999). , , ,
, - .
()- (), 204

()

() ( .., 1998; .., 1998).
,
, - .
-
.

- , , - , () (, ). ( , , +k, ,
/ ), .. I II III
.
.
- (II III ) , , , -.
,


, ,
, . -
.
-, - 205

-. - -,
,
.
(I )
- (
10x10-6/) (D-E 2-3/),
AT III ( 88%),
, - 60%. .
(II III ) , , (
).
, , .
( 40 -3/), X-Y ,
- 60x10-6 /.
-
( 15-20%).

- , -,
,
. -
,
:
4 (PF 4) - III (), III , . 206

(X-Y) (D-E, D-dimer)


-.
-, - , , - -
, , , .
.
- (III II ) (I ).

-.
-, (II ), . .

.
, .
- (II )
(II III ) , , . - , ,
207


-.


, III. - ,
, .
, 30%
, 11 % ,

. -

87% 38-39 .

- (II ) -
, .

.
(15 000-20 000 /) (150 ICU/) -,
4-5 III,
(), . - :
AT III, , - .
, - 208


. (500-1000 /) (2500-5000 4 ) , , AT
III, .
,
:
, , , -

().
(III ) -
,
, III.

(I II )
- .

VIII


200 ,
. 1743 ,
Henri Francois Le Dran
. 1872 Gross
.
John Collins Warren .
, , . , , . ,
,
, . .
. , : ,
(), , . : . ,

, - .
, ,
. 210

- , , ,
.

1.

. 1967
:
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.
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; )
.
3. : ) ; )
.
4. ( ).
, , 1996 .
: ; , .

, , , .

( 22).
22


(, ,
, ,
)

(
, )

211

,
, ,
, . ,
, , .
. , . 23.
23


:
,

, ..
,
..
, ..

2.
2.1.

.
.
.
212

.
500 , 2000 .
( , ). ,
( ). ( 200 ); . 500-2000 . , , , ..

, , , ,
. , , , , .
( 1000-2000 ),
(, )
. , 40-45%.
,
. 20-25%
,
500 /.
.
. , - , .
, , . ,
, I I I 213

.
. ,
,

.
, ,
, (
) .
( ), .

. , ,
, .
Sheehan Murdoch 1938
.
. ,
.
.
, , , .
.
.

, .

,
.
, ,
, . , .
, ,
.
1000 - 2000
.

214

. : , (500-1500
),
. 2500 ( 600).

.
.
,

. , . ,
,
, , , , . 800 2500 ,
.
, . .

. 1500 .
, (placenta accreta), ,
, -
: , .
, ,
. , , - ,.

2.2.

, 215

. , .
.
2.2.1.
,
(). ,
. . ,
1 ,
. ( , , , , ).
, , . , ,
.

, , ,
, .. .
. (75%) -
. (20%), ,
5 7,5% .
,
, , , . ,
50-80 1 .

.
. 5-10%

.
216

. .
. , , , ,
, .

. ,
,
. 1-1,5 . (, ).
.
20-25% . (10-15%) (
).
20-25% .
, , ,
1 . .

5001000 .
,
, , , .
, 1/22 915% .
. 10- . 34-36 , .
30 34 50%, 200%. , .
, 10- . 18%,
217

.
.
.
.
.
2.2.2.

. . .
, , ,
.
,
. ,
. (. 39).

. 39.

, 20%
, ,
, , .
n.glossopharyngens n.vagus . ,
, , .
218


.
.
-
-
.
20-200 ( 4:1). (,
)
( 24). ,
, (), .
24.
24


( )

a2

1
2

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D2

-
- - . , ,
, ,
.

,
219

.
, .
,
. , , >150 /
40% .
.

- . .
.. - ,
.
, 100%.
2.2.3.
. -, , , .
.

, .
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. . , , , -
.

, ,
.

. , , . .
. ,
220

,
.
.
.
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, 6-
. , 15%.
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.
2.2 .
:
,
, . , .
,
.
. . , 2+ - 2+ . , , 2+ . .

, .

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.
- ,
.

. (, -1, -6, ,
.), .
221


, .
+ .
2.2.5.
, , . , .

- . ,

, , .

222

(. 40). . 10 000 . 2+ , . :
+ (+/2+ - );
: Na/
- 3 Na
,
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, Na+,
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.
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,
- , ,
, 2+ ,
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+ . + .
,
- . , , 15 , . ,
, , .
, , , , .
,
, . 223

. ,
,
, , .
,
,
.
, - .

, 10% ,
-. 15% ,

23 . 20%
, . 30%
,
.
2.2.6.

( ) ,

. , .
. ,
.
.
-.
Shires.
, Na .
. ,
, . 2-4 .
( )
.
224

2.2.7.

() - .
.
,
.
-
. , , .
.
. , (. 25).
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,
.
25

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(
)



, -,
.
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,
. , , .
225
8 - 6578

, - . . , . . ,
, .. . - - .
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, . .. , .
2.2.8.
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, 24-48 , , ,
.
.
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.

,
,

( , ,
).
,
.

2 ( 60 ..) .

.
, II
. 226

. (120-140 /), (50-40 ), .


, , . .
, , ,
, .
, , . III . , , .
. 2 , - . . ,
.
, .

2.3. -

,
. . . . ,
. , , , .
. ,

227

. . , .

2-.
. , 12 2 ,
. , , .
, -: , , . - . ( ) (
) -. ,
,
. , - , - -
- . - ,
, .
, , , -, , - . , , , ,
-.
- :
, . 228


. -
, , .

2.4.
,

, , , ,
.
, -. ,
. - , 500,0
( 1000,0 ) - .
( , , ).
,
, .
.
: ) ; ) ; ) ( ).
2.4.1.
229

( ). (
), . -
, , (
) , (, : ). , . ,
, .
2.4.2.
, 1000 , ,
(, .), , .
:
1) ( );
2) ;
3)
.

: , , ,
( ),
, . <100 . ., > 120 /.
. .
2.4.3. (
)

:
1.
.
2. , ,
230

( ) .
3. ,
, , ,
.
4.
. ,
.
,
. .
,
, , ,
.
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, , , ), , ,
. , ,
.
1/21/4 ,
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, .
, ,
.

-, .

-
231

.

/ .

. , ,
/. , - (, ,
) , . ,
. .

2.5.

,
.
, ( , ), . .
:
1.
.
2.
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.
3.
( )
.
4.
, .
232

5.
(
4% )
. - .
- , (0,25 /).
, .

. ,
, , , , .
26




-
:
1.
2. 40 1
3. 0,25 /,
4. 1 200 2 20
5.
1.
( )
6.

, , , (
). ,
placenta accreta, , (
). , , , a. hypogastrica. :
placenta percreta (Dubois, 1997).
,
233

. ,

, . 26 .
2.5.1.


.
, . , .
, . , .
, .

. , . 0,9% .
(, ) . 30%
(1,5 ) .
- ( 70 000 40 000). : . ,
, . ,
(, ) (. ). :
234

1. (t

Dextran-70 >8 , Dextran-40 - 3 ) ,


, .
2.
,
. ,
. ,
.
-
.
3. - ( 4,4-5,7)

.
(
) . 46 , ( 7,2-7,9)
. ,
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,
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,
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.
.
( ) 50-75% . () , . , 2
, 10 10% .
(
). .
235

- ,
, III.
, ,
. .
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6-10 . . ;
>12 . . .
, , ,
.
, :
- :
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) ( );
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) ;
) (
- , - );
2. :
60-100 /;
;
( );
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;
;
.
2.5.2.

, , .
, . , .

236

.
, : - ,
.
, .

, , ,
, .
.
, . ,
,
. 0,5-1 /,
. , -, ,
.
,
, - 5-10 . , - , 12 . .
, , . 1 5 / / .
, (<50
/). , ,
200-500 / .
- , . ( ,
), (, ) .

-. ,

. 237

, , , .

2.6.

, , , . :
1.
- ;
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,
;
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.
. .
3-4 .
: ,
, .
,
.
. , . 100% , . 100 8,4% 10
.
,
:
0,5-1,0
0,6
500
500 500
10% .
238


10 10% 0,5 . .
.

2.7. ,


. , , ,
. , ,

. , - , : , . , , .

, , , :
1.
.
, ,
.
2.
.
.
,

.
.
3.

. ,
. , ,
.
, , 239

. 27.
27

.
. :
1. >
90 . . .
2, >0,5 //
3.
.
.
.
.
-
.


.
.
.
.
.
.

( 5%
)

.
.

.

. ,
, ,
, , , ,
.

.



(
< 30000/
, , ,
).
F.
.
G.
,


(,

).

3.
- ,
,
.
,
, , 1992 . American College of Chest Physicians () Society of
240

Critical Care Medicine (SCCM) . , ,


,
(, , , .)-
28.
28
, ,
(SIRS)
,
:
1) 38" 36'
2) 90 /
3) 20 2 32 . .
4) 12 000/3, 400/3 10%


, SIRS.
:
1) 38' 36'
2) 90 /
3) 20 0 2 32 . .
4) 12 000/ 3, 400/3 10%

/SIRS
, , .
(
) ,
. - :
90 .., 40 . .

(SIRS-)
-
,
, {
) , .
, ,
, ,
, .


(Centers for Disease Control, CDC) 13-
, 10 139%- .
241

,
, (rs ).
. 20-25 ,
.
- , (} , (E.coli, Klebsiella, Enterobacter. Pseudomonas
Seirata), (Bacteroides). , 29
,
, %

0,5-1,0

: ; -

0,5-85,0
<10

1-4

<1

1-4

1-2


()

<1

30

< 6

-
-
-
-

-
-
-
-

242

(Staphilococcus, epidermidis, Pneumococcus.


Staphilococcus aureus, Streptococcus viridam).
.
.
. (, ), , (. 29).
,
, : 8 10%.
( ), (, , , ), ,
.
, 8-10%.

(. 29,30).

3.1.
. ,
.
50%
, .
, , . Faecalis.

.

, , (. 41).
40% .
, . 243

. 41.

:
, , -1, -6; II
(NO), . , , . ,

CD 14 .
,
-. ,
CD 14. ,
.
()
.
.
CD 14 .
, , .
S. aureus pyogenes , . , , ,
244

. ,
(TSST-1) . -
, .
, . , ,
.
.
. S. pyogenes - - , . -1 .

E.coli, Klebsiela, Enterobacter, Serratia. , Pseudomans spp., Proteus, Citrobacter,
Xantomonas, Aeromonas, Salmonella, Shigella. Pseudomonas spp. Klebsiella.
. ,
.
() (. 42).

. 42.
245

, 1x106 .
. , , .
R.Westphal (1975) , , 15-20% ,
.
. , , , , ,
,
.
, .
. ,
, -
.
1894 . G. Sanarelli
24 .
, . , (1916-1924 .), , .
coli, 24 , ( , , ). G. Schwartzman
1928 ., . typhosis, 24
, ,
.

24 -, 246


( , ,
, , , ).
-, . , , - ,
-.
. 3 ,
- CD14, (SR) (32(CD11/ CD 18) - .
SR, ,
, CD 11/
CD 18 CD 14
, ,
. CD14 CD11/ CD18
-
CD 14 - .
1000 .
CD 14 - , , ,
, . , CD 14 - , . , CD 14
,

. 2000 (TLR).
TLR -1
NFKB.
NFKB
IFKB ,
. CD 14 TRL , TRL, MyD88. - IRAK-, - TRAF6. TRAF6
NFKB-IFKB. NFKB
-1 (. 43).
247

. 43.
-

(32- ,
- , - (CD11,
b,c,d ) - (CD18). CD1 la/ CD18
- -- . CD11/ CD18
. 3- , iC3b.
CD1 lc/ CD 18 .
CD 11/ CD 18 NFKB , -,
.
. .
, , ,
.
248


. . . ,
. ( ), ( )
, , .
, , .
. , .

. , . ,
,
, - 3
.
, ,

. XII ,
. , .
, VII ,
.
,
. : , , 3 ,
. ,
249

3-5 ()
, .
.
, , ,
.
, .
-, . ,
. , .

- . , ,
, , -,

-. ,

.
(200-500 /)
2-24
.
10% , , , . .
, , V, VII, VIII, XII, XIII, , .
, , 250

, , ,
,
. -.
, , XII ,
, .

.
,

,
, .

, .


-
-.

. , ,
, -, . , -
.

-
. , - , . , . , , (, ) (). -
251

(, , -) .
, ,
.
,
, . , . ,
.
, ,
,

.

3.2.

,
. , (. 44). .
, . ,
, .
3.2.1.

3.2.1.1. -1
17 ;
317- ,
. - 252

. 44.

, 26 . . , - CD120 CD120b.
,
, NFKB.
- . , .
-1 - -1 -1(3,
. -1 (3 -1.
-1(3
-1(3- (ICE). -1(3 - , ,
- 1 .
-1. I 80
. -1 -ip.
253

,
.
3.2.1.2. -6
-6 , , -, , . -6 gpl30,
-11, , -1.
- gpl30 -6 gp 130/
gpl30 . -6 , - , , -, 1- .
, -6
I -1.
3.2.1.3. -12-
-12 . -12 - . -12
, , .

, -, -.
II
, IgG.
3.2.1.4. -10
-10 18 .
-10 -, -,
. .
-10 , -1, -6 -12. -10 ; II
, , .
254

, in vivo, TNF, IL-1, IL-6 IL-10. , . ,


, TNF - , ; 90 . TNF IL-6 IL10, IL-1(3, IL-6 IL-10, TNF, . , IL-1(3, IL-6 , , IL-10
,
TNF,
TNF .
TNF IL-ip ,
LD100- . IL-10 , , . IL-10
, IL-10 - . IL-6 , .
TNF
. TNF
F1+2 612 TNF.
TNF , - t-PA, , PAI-1, , ,
.
, TNF
, ,
aHTH-TNF- - , F1+2
- . , . coli, a-TNF-
255

, . ,
, a-TNF-
, ..
- tPa,
-2- (). , TNF , .
in vivo IL-la , , ,
-. TNF ,
IL-1, .. tPA,
PAI-1.
, , ,
. coli TNF, IL-1.

IL-6 in vivo. IL-6 -
F1+2 -,
. , -IL-6-
, . IL-6, ,
,

in vitro.
IL-10

(TF) . , IL-10 (500 /)
, (500 /), ,
.
IL-10 (25 /) ,
. coli (4 /). IL-10 IL-10
, , .
256

, . coli, TNF IL-1


, . ,
. , TF
VII/VIIa .
TF-
;
, . , , , . , C/S, , , .
TNF () , ,
. , TNF , . ,
TNF ,
PAI-1, , . TNF , . IL-1
, ;
IL-6, , .
: 1) , ; 2) , ,

. ,
, , ,
.
257

,
.

- . , , , , . , , , , , . ,
.

- .
, ,
,
, , , ,
, ,
, . , . .
, ,
. (NO).
TNF-a ,
,
- ,
258

, , , , , . , TNF-a N0,
. NO .

, .
.
, ,
. , , ,
.
- - , .
,
; ,
- ().
, . .
.
, ,
. (CARS), -4, -10,
-13, (3, , -a -1, - . , . - ,
.
9,
259

3.3.


. .
, . () 5 : 1)
; 2) ;
3) ; 4) ; 5) . 45,46.

. 45.

I . ,
, , . , , . , .
, .
, .
II
. 260

. 46.

.

, . - - ,
. , . 261

. .

, . , , ,
,
-,
, , . .
IV .
, (CARS). CARS .
- V
- ,
.
.

, ,
.
,
, .
( ), - .
,
, .
, -
.
. ,
. . -
.
262

-
, , , , , - , . ,

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.
,
. , (, , , .)
, -,
, .
, , ,
. , , - ,
, , , . .

20 40 .
.
, .
,
, ,
.
,

, . , , .
.
263

3.4.
, ( 70 ..);
:
, ( 500 ) ,
- (, , , ), , .
, ,
, ( ), .
, . , , , ( ), ( 50%),
( 13%) ( 94%).
- , ,
.
, .
, ,
, 39-40 . , ,
. : 4-6 , . - , . 612 . ,
, ; .
(40-60/20-0 ..). , , 264

,
.
, . ()

.
() ,
() . () ,
.

3.5.
(, , ).
, , - , . , ,
, ,
, , . ,

,

.

: 1)
4 (
) ; 2) ( ) ; 3) ; 4) , , ; 5) -
; 6) ( ).
() .
265

,
.
,
, ,
.
, . , . ,
, , ,
. ,
.

, , , (
20 ).
, . ,
, , . ,
, .
.

.
. , - , , .

.
, , . ( )
( ).
, .
266

, . , , ,
, ,
.
.

. ,
, (),

.

( , , ), , .
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. ,
, ,
,
, .
. () . ,

. . , , .
,
.

,
267

.
,
. , 12-24 , .
47.

. 47.
268

3.6.
:
- 90 . .;
- 30 /;
2 - 60 . .;
;
;
;
.
:
A.
150-200 /
, (
, , (,
.), , , , ).
. ,
2 60 . ., , .
:
15 /, 25 ,
()
70 . . , -
350 . . 100% , 2 45 .
., .
.
B. :
1) 2 / 6 ;
2) 1,5 /, 1 / / 8 ;
3) 900 / 8 ;
4) -
.
. .
4-6 , ,
, , , , , .
269

. , . .
.
( 0,5-1,0 /). .
. ,
.
3. , .
, .
, .
:
, -
. ,
, - . , 25% ,
, .
.
.
,
,
, .
,
- AT III .
,
-
. .

, 270

. ,
,
, ,

( ). ,
.
, - .

-
(). ,
, ,
, , . , (TFPI).

. .
-, , , .
.
:
1) .
2) ,
.
3) .
:
) ;
) ,
/,
;
) , ,
, , (BPI:
bactericidial permeability increasing protein). BPI -
,
;
, ,
.
271


:
A)
, , .
--
,
, . - .
,
-- .
) .
B)
,
.
-1- (-IRA),
.
(POF) . POF -. - , ,
POF.
POF
, -6-.
POF .
POF , , : ,
POF -1 ; -
, . , POF , , . In vitro , POF

CD1la, CD1lb, CD11 CD18.
, , ,
. POF
.
272

, (, .), .. (PgE). , - PgE. ,


PgE .
,
( -)
.

4.
, , ,
, - . , 70%. ,
, .
, . , . ,
,
.

, .
, . ,
.
,
: ,
. ,
. -273

, ,
.

4.1.

50 , . . , , .
, .
, , . 0,6-10% . ,
; 0,04-0,2% . 0,001% . 15%
. 70% , ,
.
4- .
20% , 1% .
. ,
. - ,
.
-
10-50 100 000 . 100-500 .
, , , , . , - .
. 274

IgE- ,
G.
, . , . , - , , .
, , . : ,
, . .
- .
. , - , .
( ) , . ,
.
. , , , ,
. .
.
- .
.
. Laurent
. , .
,
, , . . Barbier .
275

, -, .
. , . - -1 -.
,
,
.
. , .
.. . : ,
. 5-10% ,
.
,
. , , -, . Smith (1985) ,
29 , 50 .
, . .
, . . ,
. ,
. - . 31
.
32.

4.2.
I 276


, / . I
.
31
,

1 -5 10 000

10
100 000

10 2667 (500
)

0,9%

47 024 10

32

- ,
, ;

-
, -


,
,
.
,


. , . .
, . :
.
,
277

.
6-8
, , ,
CD 4+ -.
, :

IgE-,
, .

. 48.

CD 4+ - Th2 (. 48).
IgE- -. IgE Th2 -278

, . , Th2 : -3, -4, -5 - (GM-CSF). -


IgE -4, .
IgE- . ,
110 Fc- . IgE
, /
. IgE - FceRI. / IgE.
.
,
, .
, IgE-FceRI (. 49).

. \

. 49.
279


.
.
.

:
1- 2- . - ,
.
. 1, 2-.
1
, 2- ,
. 2-
(N0).
2-. 2- , . ^ . ,
.

. 1-
() . . ,
2- .
- - . . 2-,
, .
, (, , ), .
.
.
. 2,
280

. . . 5- 5-
(5-). 5 : 5- , 5-- , . 5- 4 .
4 4, ( 4, D4, 4),
. 4 , -
, , . 4

.

(), .
, -
. , , -1, -3, -4, -5,
-6 - . , .
- , . MIP - 1 I-1.
, ,
, , , , . 33.
, , ,
.
,

281

33


4, 4, 4

D4

4, 4, D4

,
.

.
.
.
, , - -.

, .
, 10 50% ,
. , , .
-
-. ,

V, VIII , . - , ,

. , .
282

4.3.

. ,
.
.
, , . : , , , , , , ..
( 34).
34
,

, , ,
,

, ,

, ,

,

, ,
,

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,

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, ,

,
,
,

,
,
,

5-30 . .
: ,
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, . 283

, , , . - , , , .
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88%

47%

, ,

33%

, , ,

30%

46%

56%

15%

16%

6%

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36

II

III

IV


, , .
.
-. 35. 36.
284

4.4.



.
, 15 .
, .
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,
( )
, , ,
.
, , .
4.4.2.
.
, ,
. . .
4.4.3. ()
- , 1-.
, ,
, , , . 285

4
1-. .
, , .

4.5.

4.5.1.
.
. -, , ,
. , , .

, ,
, .
, ,
, . - ,
30 . ,
. .
, .
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37. ,
.
,
.
15 .
286

37




100% .
, , . .
1:1000
0,3-0,5 . ,
. 10-15 .
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: (25-50 ) (300 ) 4 /. ,
.
. 100 - 1
30-60 .
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.) 0,25-0,5 15 .
. ,
2000 . ( 500 ). -
400 500 , 5% .
, .
. 287


.
. (0,25 ).
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38

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,


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IX

-
1.
-
, , , ,
.
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, 3,8% 10:1, vacutainer.

. ,
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).

, ,
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.. , , , (14-16 ) ,
- .
289

, ,
, 4-5 , - 30
- , 2
.
2 ,
. -20" . (platelets rich plasma
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,
( +5) ,
. , .
, (), , , .

2. -
,
-
, (. 50). ,
,
,
. , , - , :
,
. 290

. 50. -

, - .

, . ( F1+2) , , , , .
, ,
, ,
. , , ,
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291


,
.
() -. , , 50-75% . .
, ,
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, , .
-,
. ,
.
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, ,
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. , , ,
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V, VIII, IX XI). ,
l/ . -,
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50-60% , -.
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,
.
. ,
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, : (
/ )
10 ( ), 292

.
10 , , .
. / , , , 1 . , , , . , VIII:C , VIIIC, ,
VIII;C -, .

. 51.

/ 85100% -. ,

, ,
. - , - , (. 51). ,

, - , -
, , , 293

, , , . .
, - ELISA-
- .

. 52. /

. 53. D-

, , D-. D , , 294

,
XIII, (. 51). D-, , ,
- X, Y, D ,
(.
52,53). D- - -6/22,
- , D-. . , ,
D- .

, , , : D-
F1+2 ( 90% ), III ( 89%), ( 88%) ( 75%). ,
,
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-6/22
.
, (- )
.
. ,
, , . ,
, .
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, - . ,
. X
X.
, , , - ,
.
295

, ,
,
, . - , - -
.
, , . , , ,
.
D- : - - .

3.
, ,
, -.
,
. III
(AT III).
F1+2 .

A (FPA),
- III,
- ().
, F1+2 ELISA-
. , F1+2
, FPA (. 54).
296

. 54. F1+2

AT III
. AT III,
. AT III
, .
AT III ,
, , , AT III.

, ,
- () 4
(PF4) - .
FPA - , , . FPA . FPA '
- . Fl+2, FPA ELISA-. ,
, :
F1+2.
, ,
F1+2 FPA
; AT III
; .
297

, FPA in vitro . , FPA ( 3


5 ),
. F1+2 (90 )
, FPA. , F1+2,
in vitro , FPA.
. , , - , . . ,
, , .

.
.
, , 2- 2-.
, , , .


-2- () 2-- (ELISA).
in vivo.

.
, -, (
), -, (2-).
, 298

, 2- , -
, ( 2).
, , , . , , ( , ), , , ,
. ,
.
,

. 4 (PF4) - (-TG) ;
. . , PF4 -TG , , , : (, , , , .). , ,
, PF4 P-TG .
,
, ,
.
(, , .).
, , ,
. ,
,
, , , ,
299

. R.Bick
et al. 1998 .
(. 39).
39

-
( I)
:
F1+2


D-
( II)
: D
(X.Y-)
- ()
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:
AT III
^-
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:



( IV)
:

I, II, III , , - IV.

4.
,
300

.
. ,
,

-
;
-.
, - ,
-.
,

, -,
.

, -.
:
, .
, : , , ()
.
- . , , , , .

(,
).

, , 301

- .
,
- .
-
, ,
,
.
-,
,

, , , , - . -
. ,
-
,
- .
- . . , .
(III II ) - .
, ( , , , , ()) (
, ). 302

.
, ,
. ,

-, (I ), .
-.
, ,
, .
. . , .
/ ( ),
- . , , ,
( Raby). - .
, (II III ) , (- ).

.
,
-, ,
.
.
,
303

,

.

, .
VIII IX
. -

- ,
( ) .


( FVIII FIX)
. ,
-

-.
, Lee White,
, , . . , , , . , , .
, ,
.


304


(, ,
.).
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-, .
, - ,
. ,
.
- , , , .. . , , , ,
, . , ,


- .
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, . , , , . ( )
, -
. ,
. , -
, .

-
305

.
, -
- (..
- ).
( ) , . Raby .

F1+2
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.
, -
, (, , ,
.), ( ).


.
, , . ,
-
. - , .

( ,
,
306

..).

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. .

-, - ,
.
,
,
.
,
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, ,
, - (. 40).
.
,
. , ,
. -

.
- , 307

40
,
-

-

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-

-
-

- / , , D-Dimer min - (- )

- -
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,
-
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()
() /

-
RM/

-
, ,
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Lpa

-
ATIII,PrC<PrS,aPrCR -/(+) -

( ) - >
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- I II - . , -
,

.
308


, , ,

.

-
I
,
- ,
X Y
,
-
- D , D-Dimer.


- 11III .

, , ,
-.
, ,
,
- .
()
-

.
, ,
, - , - , . , ,

-.
, 309

41

-

-
-
-

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-
-

, III

-
-
-

-


- /
,

-
-

-
- (/)
-
-

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310


, II


, I

-
( Ivy, Lee-White, , ,
./, r+k, , .,
,

)
-
( )
- ,

-
-
AT III
- (+)
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- max, / (+)
-
(, )
-
( ,
)
- AT III
()AT HI
-
- ,

- (+)(-)
- > (XY) >(DE)
- D-Dimer min
- /(+)(+)

-.

, , (. 41).
,
-,
, -,
, , XY .
,
, , .

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D
, -.
. , ,
.
, . , , -.
-
, , . 311


, ,
. .
, . ,
, . , , .
,
,

.
-

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, .
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, .
.

-
.
312

,
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5.

-

-, .


.
, ,
,
,
.
, - , ,
. .
. , ,
, .


: 1) , (I ) ; 2)
313

, () -
(II-III ); 3) , - (I ).
,
( ),
, .
- ,
,
.
-
, , , . , , - , , -.

, , ,
.
. - , VIII GP Ib/IX .
, , , , ,

.
GP Ilb/IIIa, , - .
. 2
2,
.
, ,
in vitro 314

.
,
- - (p-TG) 4 (PF 4),
(Wu Hook) (. 55).

. 55.
4 -

, in vivo.
in vitro : 1)
; 2)
( ); 3)
; 4) ; 5) ; 6) ; 7) ; 8)
, , , , , , ; 9) - 3 (PF 3); 10) 4 () (PF4); 11) - (- TG);
12) ..

315

( 250-300 109/).
( ) ( ).

.
(, , ,
)
.
- - ,
.
,
. , .

Ivy,

1 (40 . .), .

. Ivy 1-6 , - 1 3 .

, ( ).
,
,
51 I111.
6-8 .

, - () . ,
316

-
, ,
.
, ,
. -,
, , , , (, , ).
-
,
, , - ((3- , 4 ). (3 2010-12 - 3510-12 /, 200x10-12
/, ,
- 250x10 -12 / .
4
410-12 - 610-12/. , (3.


.
, , .

( , , , , , , ).
( 110-3 ), .
,
, ( 1x10-5 , 110-4 ),

( 1x10-7 M, 1x10-4 ).
317

. 56 .



,
, , (. 57)
318

. - (t)
.

. 57. :
1- () ; 2 - ; 3
- ()

- , .
,
, , : - ( Urn
-); - , ,

319

42 .
42



30-50%
30-40%

60-80% ( )

10-20%

tnn

25-30

tK,

30-45

Za,

60-75'

Za2

60-75"
30-45-


. , , (1x10-3 lxl0-5 M)
. ,
(lxlO-7 1x10-5), (1x10-4 ) ,
, , , - .
( ) t , .
t. ,
.

, (. 58)
.

42.
,
, , 80-85, t , ,

320

. ,
, ( ), , . , -
(. 59).

. 59.
-

. 60.


-
( 1x10 -3 ),
( 110 -5 110'-7, , )
(. 60). ( , 321

, , -
)

- , , ,
(.61).

. 61.
(II , -)

.

.
:

,
, ..
( , , , 4
, ). ,
, ,
.
322

,
( , ) .

,
, , . . , , ,
, () ,
.
( Wu Hook, I975), , . ,
, , , ,
.

, , -
, .. -
,
.

( , ).
( )
() 100-150 , 20 0,25-0,5 24-48 ,
, ,
- ( , , ,
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323

( (), ,
.) ,
(, .).
.
,
, , -
(110-3, 110-5) (1x10-4 ),
2
, 300-1500
, 60100%, .


, . (300-500 ) 48 . 62.

. 62.
0,5 48
(I ) -


( 2- )

50 . 324

-
(. 63). ,
,
( , ), .
,

-. ,
. ,
.

5 6

. 63.
50 / (I )
-

1 - 110' 3 ; 2 - 1x105 ; 3 - 1x10 7 ; 4 - ; 5 -


1x10" ; 6 - 1x104 ; 7 -

, , , , . - lxlO-4 , - 1x10-7 (1x10-5 ),


-110-4.
(. 64).
,
, , 80-100% . 10-15% (
30%).
325

. 64.
250 VIC/ (
- )
.


,
. , ( 110 -3, 1x10-4 ),
30%; ( 1-5, 1x104
) - ,
( 1x10-7, 1x10-4 ) -
().

. , ,
, ,
. , ( )
( ) .
, .
, , . -
326

,
.
, , , . I (
1,01 0,1), , , - .
: (), ,
, , .
, .
,
( , , )
- .
, ,
, , , , . , -
( ).
(, ) ,
.
,
,
, , .
, .

.
, ,
, -, 4 () - ; . 327

. ( )
, ,
: - (- ) - , , ( 10 ) . ( )
(1x10-5
), -
.
( )
lxl0-5 M
, , - 14; -
-
- 14.
; .

; .VIII
.VIII .. -.


().

,
. .
. ,
, , . (328

, : , ()
2.

(6-8 )
.

, , .
. ( 10-15%), ( 20%) (lxl0-3 M). 3001500 /. 2 , . (300-500 ) 24-48 , ( 2 ) .
,
(, , , ),

, - -
. :

,
(110 -7 110-5). - .
( 25-30%). (), ,
-, -, -, 329

. , ,
. ( , , , , ), (, ), ( .), ,
.
, ,
.

6.
-
(
) 37 - , , , .

.
..,

.
() Claus . , . , .
Merskey,

, .
-

.
330

Mancini (1996) Laurel.


- , .
.
.
( ). (
)
( ), .

- (II-III )
( - ) (I ) -.

(
.) - .
-
, ,
(
) -.
75-100 / 3-4 /.

6.1.

6.1.1.
, , . 65.
()
0,025 12 (5 /
331

. 65.

7,35). II, V, VIII, IX, X, XI XII,


, (FXI FXII). (PF-3)
(), , FXII, FXI, FIX, FVIII ., FVII FXIII (
: 60-70 ).

( ) 0,025 1 2 (5 /) - , .. ,
.
,
, ,
, , FVII
FXIII.
, .
332

50-
.

, .
,
.
-, .. 37.
( ), , ,
. , (1974) .
Lee-White
: ( )
. 3 45" 30 .
, ,
,
4-8 37" 8-15 . , .

. 66.

Quick (1966), (. 66), ..


333

, : , ,
, , , Mg2+
1++-, . ,
. .
1935
.
,

II, V, VII, X.
. (III II ) -, . (
) ,
. , .
() .
10-12 20-30 , .
( III
, ), .
.
. - FII, FVII, FX - FV.
,
:
) ,
100% ( II III );
334

)
( II III -
);
) () -
,
(

,
1,0.), -
FVII, ,

(, . FVII
);
) (MHO)
,
. MHO
.
()
(Bothrops
atrox), (. 67), ,
.
,
(
),
.
16-22
.

. 67.

335

6.1.2. ,
Raby (1974)
,
(I, 29%).
.
,
. 40- .

. 50-

.
.
, 37" ,
0,25 0,1 1,29% 12 (
(3,8%)). , . .
, ,
(. 68).

, ,
-
.
-
, . ,
69.
: ,
, ,
(PRP - plasma rich platelet) , ( '
plasma poor platelet).

200 g 500-800 / 5-10 , . 336

. 68.
- ( 1 ); -
( 1 20 ); -

. 69.
1 - ; 2 - ; 3 -

() - 3000 / (2000 g) 10 .
68,69, 70.
(. 70 71).
- 1
, ,
.
-

20 .
337

. 70.
(7500 ) - (5 000 ) /
1 - ; 2 - 7 500 / ( 30 ); 3 - 7 500
/ ( 2 ); 4 - 7 500 / ( 4 ); 5 - 5 000
/ ( 30 ); 6 - 5 000 / ( 2 ).

. 71.
15 000 / (5 000 8 )

1 - ; 2 - 2 5 000 /; 3 - 1 - ;
4 - 3- ; 5 - 7- ; 6 - 12 .

. - , .
r+k -
.
- - , , - .
Em - . - , , : (lOOma/100-ma). (),
, (Em/k).
338

.
, .. , (PRP) () .
- (III ),
, , -,
Raby (
). , , .

. 72. ()
-
1 - , (PRP) ; 2 -
() ; 3- , (PRP) ; 4-
() .

. 73.
()
1 - , (PRP); 2 - ().

339

, ( , ,
3 (PF3) ..) (. 72,73). -
, .

. 74.
-
1 - , (PRP) ; 2 -
() ; 3 - , (PRP) ; 4 -
() .

. 75.
()
( )
1 - , (PRP); 2 -
(). < 1 ..

, .
340

.
, ,
,
.

- , (. 74,75,76).

. 76.
- (
)
1 - , (PRP); 2 - ().


:
: - ;
, - .
= 1,01, . .
Raby (1974). (r+k=) . , , ,
341

. 77. ,
(PRP) () (,) ()
1 - ; 2- (=3,7 ..);
3 - (=, 12 ..).

. 78. Raby
1 - -; 2 -
; 3 - r+k ()>+ ().

. 79. Raby
1 - ; 2 - ; 3 -
r+k /r+k < 2; 4 - r+k /r+k >1,5.

342

.
,
- ( , , , ..).
, -
,
.
.
, - . r+k r+k (-).
r+k 1,5-2 (. 78)
( , , ..)
, ,
. ,
(2-3 ) ,
(. 78,79). , -
.
. -
(III ) () , , ,
. .
.

( 100-200 1 ). , .

( ),
343

( ) 1,29% .
( 3-10 ) ) (. 80).

,
(, , ..). (. 81).

. 80. .
1. ; 2.

( 500 /)

. 81.


, .
. , (
- ).

344

. ,
. , ,
, .

6.2.

6.2.1.
()
Godal H.C. Abidgaard U. (1966)
, ,
, ( ).
: 9:1. 15 3000 / 4". 1 0,15 50% 37 5 .
,
.
- no Niewiarowski S. Gurewich
V.(1971)
: - ,
.
. 5 10 75
1% - 1:5, 1:10,1:20, 1:40,1:80. 1% -
.
1:5,1:10,1:20,
1:40,1:80. , - . 0,2 - -
0,2 0,2 .
30 5 37.
345

; , ,
.
: , .
+, - ++,
- +++, -
- ++ ( 2- ), +++++ (
5- ).
() ,
Largo R. . (1976)
, : 1) 100
() , 2) 100 , 3) 100
. - 50
, , 37 - 10 . 6 ,
( ) .
+++ -
, ++ -
.
- :
- .
, .
6.2.2.
()
,
( ,
, ), (), .
() - , - -
, ( 346

X-Y; ).
Merskey , 1966
/
() .
2,0
, 20 3600 . , 37" 30 1500 / - 10 .
, . , -20.

, , , .
() Hamger .,
1970
()
/ () Hawiger ., 1970, , /.
, - ,

(, .)

.
( , , ) , /
0,05 /.

, , .
347

, ,
,
, . 56" . .
-
-
, (- ).
- - ( ,
, , -
.). -
.
A (FPA)
(FPB)

-
.
FPA FPB
, FPB ( FPA)
.

, .. , ,
- III (), - ((3TG), 4 (pF4),
F1+2
. (492 ) .
6.2.3. - III
()

,
AT III .
348

III ( ), . ,
AT III , , . .
,
.
, :
- ;
- -;
- ;
- ;
:
- ;
- ;
- ;
- , ;
- , ;
- ;
- .
.
( )
.
1,450,410 -6/ 0,85 3,2x10-6/.
.
6.2.4.

F1+2
, , . ,
, Va, ++ . , Va, Ca2+ . , 349

: F1+2.
F1+2 - .
F1+2 ,
( ). , F1+2
( ), .
, F1+2
, . :
- ;
- ;
- ;
- ;
- ;
- ;
- , .
AT III, , F1+2 .

F1+2. , F1+2 . F1+2 ,
.
F1+2 :
1. .
2. .
3. .
4. .

6.3.


.
(PC) 2
Agkislrodon contortix, Prota, PC
. PC , ,
, . PC
350

. PC. PC. 30 4% .
(APC-R) PC FVa
FVIIa. PC. PC 2,4-4,0 .. APC-R FV . FV Leiden PC . APC-R
Leiden. PC . S - .
XIII
. FXIII .
-
FVIII:Rco ( )
FVIILRag.
, , , , .. -.
II, VII X , ,
. ,
-, .
( ) ,
.
, .
VIII; IX; XI; XII
351

,
VII; II; V X - - .
, X
-
VII ,
X .

(. ..)
. , . , .

( III; , ; 2-, 2-,
; ; -1-; VIII:RAg; XIII, 2- ;
XII; ; II .) Maucini (1965) Laurel, .

, .


-, .
1:1, . , : New
Oxford Test ( FVIII
FIX 0,5 FVIII FIX) BetesdaTest ( FVIII
FIX FVIII FIX
.
( III; , 2-, 2-352

, -1-, 2-; ;
, .) .. , ,
, -.

(405 ) , .
- ( 1:1 1:160)
. -
.
6.3.1.


(, Mancini, 1966).
(. ).
Kovalsky (1959) ,

-.
: 1-, 2-, 2-, (). 2-
,
-, .
. - () -
- - .
() - (),
, ,
.
, 353

-.

,

, , .

,
.
- , .
6.3.2.

.
.
,

.
(ELISA), - , .

(), (ELISA), IgC, , . ( R.L. Bickeial .)
43.
43

36% IqG
17% 1
14% IgA
33%

(32GP1,
-,
-2G1 -. -d2G1-,
354

. -2G1 , .
ELISA ( ) ,
.
, , , . ,
,
. 2GP1 . 2GP1.
, IgG IgM .
mpl GPL.
mpl 1 IgG. ( 60 mpl U/ml 80
GPL), (20-80 GPL 20-60 mpl U/ml)
( 20 GPL mpl U/ml). 10 GPL mpl U/ml . IgG IgM 90%,
75%.
- , ,
.
, ,
,
-2G1 - .
R. Bick . (. 44).
-
. , 1990
. ; 1997
- 355

44
.
( , , ,
.)
,

-AKA(lgG, IgA, IgM)


-BA(dRWT)
-
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,
(
).

- (IgG, IgA, IgM)


- (IgG, IgA, IgM)
- (IgG, IgA, IgM)
- (IgG, IgA, IgM)
- (IgG, IgA, IgM)

. .. (. ..).
(. 82). I , - . , , ;
(dPT), (dRVVT), .
- , ,
, /, ; .
II - ,
-.
.
.
, ,
-.
III - ,
(
). - ,
-
.
( , ) , 356

1. -
-
-
- (dPT)
- (dRVVT)

2.
,

3.



:
-
-
-

4.
. 82.
:
, ,
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-, , , .
, , ,
. ,
,
:
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. , , , ;
.
,
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dRVVT,
23-27 ,
357

,
. ; IgG, IgM BA. dRVVT
.
() . . dRVVT
, , . ,
dRVVT , ,
4 ( ) , . , (BioData Corporation) 100 / 4 . , , 95 / 4 ,
,
- dRVVT . , , dRVVT , , (Bell-Alton), 4 ,
. , , , , -1 ., , 2- () .
.

, , 32GP1 ,
, , , ELISA-.
(, , 358

, , (32GP1) ( ) . ,
7% /
( 1). 15% , ( 2), 15% ( 3) 22% 5. 44 .
,
.
- .
. , :
1) :
- ;
- ;
-
;
-
;
- ;
- ;
- .
2) dRVVT :
- ;
- V;
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- .
3)
:
- ;
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-
;
- ;
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4) :
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. 359

. 84.
(dRVVT)

( )
.
II .
; .
,
, 4:1. 360

.
, ,
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() 5 .
, , ,

1:1. ( 4:1) , , , ..
- , .
,
1:1 ( ) ( ).

,
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.
-

.

, -, , - (), F1+2 (. 45).
, ,
, .



-
. ,
,

, .
361

45


.
.

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, -.

- .

.


. .
.

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.
, .

.


/,
III , -,


. , .

;
; ;
; ;
; .

, .
.
I I I . , . 362


, ,
, .
:
1. .
2. .
3.
.

6.4.

().

(X Y). ,

, , .
, ,
-.
- (FM-) -, : ().
6.4.1.

.
. 1,5-2
. - ( ) .
,
363

, 2-3 .
, 0,1-0,8 /.
,
- .


.
:, ,

.

.
-
,
. ,
III .
Raby (1974) . , , ,

. ( ).
, +k -
+ - I,
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364

. , .
, , ,
. .
.
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. , -,
.






. ,
.
, ,
, ( ), ..).
, ,

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, , ,
.
. ,
-
,
.
, ,
, . , 366

.
: , , - -. VIII , VII .
, 20-30% , VII 1-15%.

- .
,
. ,
, II III .
, - .
, ( -)
( , , ).
- ,
,
- .
-
- , (, , ) .


( )
, .
367

-
.
.

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.

, ,
.
,
. , , . , . .
,

, . , - ( , , ) .
() ( )
(
). (, ).
( ), ,
368


, . , , , () ,
.
, ,
. , , .
, . , (2+- 2), .
, - , ,
, , ,
.

.
2-3
2,
. ,
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, , .
369

.
,
, , ,
.
. ,
.
, .
, - 12- . 5,5
18- 0,9 /.
2,0 - 3,0 /, .., , .
II, IX, VII, X, XI XII .
.

. , 12-24 , 11, VII X 20-23%, IX - 14-40% .
, 12- ,

, , , VIII .
, ( ), ,
, . , 370

. , ,
.
, , , , .
. , V VIII , (. 85).

. 85.

, , .
- ,. ,
II, VII, IX X
, , 2-12 . XI , XII - 9-14 , ,
. XI .
371

V VIII

,
34-36 .
,
V VIII

.
. - II, VII, IX, X, XI, XII, .
,
,

(. 86, 87, 88).

. 86.

- ; -

, V VIII , .

,
372

. 87.
()
()

- ; -

. 88. r+k

- ; -

.
.

,
( ,
).

.
373

. 89.

3- (. 89), II , ,
. ,
. 3 , - 4 ,
. ,
,
.
(XIII)
10 2 ,
. XIII
. XIII , , 3- .
XIII . , . , ,
, .

( ), .. , . III, 1-, 2-, -1 .
374


I I I (75%).
.. , - , . , ,
III.
,
III (. 90). , III , S
. III
, -,
.

. 90. III


, .
,

, , . - . ,
375

,
.
, ,

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,
. ,
( ) .

, . , ,
. , ,
.
,
. . .
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II III - . , (. 91). ,
III.
,
.

.
, .
376

. 91. -

, ,
1-, 2-,
, .
III,

. II I , .

O
,
.
, ,

, , ,
.
, .

. , ,
377

,
, .
, 21,2% .

,
.
, , ,
,
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, . , ,
,
( ) .

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, (),
- .
,
, ,
.

, ,
378


- - .

. , , , , ,

,
, . , ( III
II -).

. , , , , ( I -).

( ) -. , , II - III . ,
.
, III
. . - -.

( ) ,
.
.
. .
379

,
,
.
-
. ,

. ,
.

III . - .
II
-III - -
, ,
. , , II III -. , II III III II -.
III - (. 92).
: , ().


.
,
, . , , :
X Y . 380

. 92. -

. , ,
,
,
,
, . , : , ,
.
, ,

.
.
, , ,

.
381

, . ,
.
, -,

. I , .
, ( I I
) ( I I I ).

, , ,
. , , ( ) ,
.


,
.
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.
( )
, , .
382


(. 93).

. 93.
,

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-

-
,
. ( I II
). ( I I
).

, , .


. , , -
III .
383

III , , .
III
. III
. -.
III
. ,
.
,
, III
. - , , .

- .
, , . .
, , .
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- . .
,
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,
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.
384

,
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.
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, .
, .
.
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, -. ,
-

.
,


, .
, ,
.

,
.
,
, .
,
, 385


.
. .

.
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.

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.

XI


1. -
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; 3) ; 4) ; 5)
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.
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-
, , 387
13

. , .
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,
( ).
, .
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.
(, F1+2), (),

, .

,
. (, , , (, ), , , (). () ( 20
).


, () .


,
-,
388

.

( , ),
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, .. (10 00020 000 / ) 20 000
/ ,
. -
.
, .
( ) : 2 , 30 4-5
.

.
25 000 -30 000 / , 10 00020 000 / ,
.

-, . ,
0,05-0,15 /
. , 3-4 , , .
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. 389



, -.

, -,
.
,
,
,
.
, ,

,
, .
- , - , . -,
.
, I I I .

I I I , . - 390


.
, .

-, II III -,
. - . , , -,
. ,
( () ()). III.
, III, 30%.
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- III, ,
X , . , 5
, 100 , .

.
- . ,
,
, .
, . , , 4 000 50 000 , - 3500-4500 .
,
391


,
, .
III. AT III 1:1,
180-200 . - III , ,
. ( 24 ),
60 : , 1 AT III 1 , (0,01 /)
750 .
-
, ,

,
.
.

.

. , -, , , .

- ,
.
392

,
.

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( ),
.
. ,
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. ,
2-2,5
. . , . . , .

- . 15 000-20 000 / -.
10 000 12
0,1 /,
393

. .

2-4 ,
. :
, , . 1,5-2- . 2-3
120 ,
.
0,4-0,8 /,
.

,
, .
,
0,5-2 , , .
,
,
. 100 , . , . 100 /
,
100 /
.
. , 2
-
III.


-. ,
, , . 394


, , .

, . ,

. . ,
. .
. ,
,
-,
, .
,
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-,
.


, , , , .
- .
395

,
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.
,

.
,
.
III , .
-
, . : ,
1,5-2,0
500-1 000 / (12 000-24 000 /) 2 500-5 000 4 (15 000-30 000 /).
2
30
.
.
, , , ,
. ,
500-1000 / , 2 500-5 000 4 .
,
, .
, , .
396

. 94.
-


(. 94).
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,
, , .

,
.
,
-
.
, , AT III - (). ,
, ,
AT III
, 397

.
,
-, , ,

,
-,
150,0-200,0 AT
III, 48-72 , .., 3-4 .
, 1 000 / 5 000 4
,
.
.

2.
II III
4

.
- (II ) , (. 95). , 80 000-100 000 5%-
. , -
,
. 20 000-40 000 2
5% 5,0- 10,0 6-8 .
398

. 95.

-
(II-III ) , , . AT III ,
.
, ,

I-II .
, ,
, .
( . 96).



. 100
399

. 96.

000 5%-10,0 . , ,
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-
( ), , , AT III.


,
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-, -

.

-
400

, , . ,


, (. 95, 96).


80 000-200 000 , 40 000-60 000 .
, , 300,0 600,0 .
, -
- .. .
5%
0,5-2,0 6 .
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500-1 000 , /,
, 2 500-5 000 4 ( , 12 000-24 000 15 000-30 000 ). . , I-II -

: , , , r+k, ,
1,5-2
, ,
. 401

,
.
, 30
.
2
,
200,0-300,0 .
( 2-4 ), - .
,
III. , . AT III
. AT III . AT III 54% 77%, AT III - .
AT III,
96-129%,
135-143%.
() . ,
,
-, ,
,
.
II - 402

,
.


AT III
-0,330-0,453 /,
AT III. ? II -
AT III ( 70%)
,
.
200,0-300,0
AT III,
98-119% 0,055-0,125 / . 3-5
AT III .
15
000-20 000 /
, , , .
-,
, ,
.
0,050-0,085 /,
, . , , -
.
, , ,
-
, . II 403

I ,
- .
,

,
, -.


, ,
. , , ,
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-. , 8-12
.
. ,
- .
,
-.
-

.
( 4 )
404

2,5005,000 4 .
10,000 - 15,000 / . .
, -
,
-.

.

AT III ,

, II -.
,

.


.
, .

.
,
- , II -.
-
405

, , , ,
, , , .

3.

- -
-
,
, .
- -
, -
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( 0,75 - 225 /, 0,25-0,5 48 )
15,000 /
.
, 5,000 8
(15000 /) 0,3 (7 500
AntiXa ). 75-150 /,
406

- 175-225 /. , ,
0,25-0,5 48 .
( , , ..)
, . 2 -
, - , .
- , ,
. ,
15-25% . , 1,10-7
. 0,25 0,5 48 , ,


.

, .
.
- .

.
- ,
407

. , ,
- . 0,3 / (7,500
AntiXa ) , .
,
. ()
. AT III (98-114%), , .
38-44%, ,
, . - -. 5,000
8 .
1-2 ,
, .

4.

-
.
, ,
, .
, 20-24 (0,25-0,5 48 ).

,
.
408

20-22 ,
, .

, 48-60%, 75-80%.
, , II III -

.

. 97.
-
,
AT III



,
. - 20-23% (<0,05), - 2 (<0,01), - - 25% (<0,05),
- 21%
(<0,05). , . 55,8 5,1% ( 97).
409

, (
) .

5.

, 16 , .
. 400 48 3 (4) ,
.
90% (80%),
.
.
, 32%,
10 . ,

, , .
, ( , 2-3 ) ,

.

6.


80 410

: , . 5000
(7500) 3 (2) ( 15000 )
3 (4) 0,3 (7 500 AntiXa ) 1-2 . ; 1-2
; - 3-4
(. 98).

. 98. ()
-



, . , , .
. ( 1-2 ) .
,
III, , .
.
, - ,
AT III 110-125%, .. 25-42% . - 411

(
1 -2 ),
2 .
AT III, . 0,08-3,125 / .
: , 200,0 48-72 2-4 0,3 (7500 ). 8 ,
. , 12-24
, - ,

. . -15,000 / ,
8-12 . ,
( ) 2-3 .
,
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.
, ,

412

. . -

,
.
-

-. - , ,
.
,
79% 38-39
.

7.



.

. -
5-7 .
. .

3 (PF 3), 413

- -
. AT III
42,40,10~6 / 68-72%.
. 15 000 / (5
000 8 ) , . :
, . AT III 2833% .
, , , , .

-
, ()
.

- .
15 000 / 0,063 - 0,120 /,

.
. , . , - 414

,
.

. ,

-
. .

21,1% - 20,7% - . 81,8%. ,

79% .


.

8.


. , , 2, , . , ,
, ,
, , , 5 ,
. ( 50 ) , ( 30000 /),
.
, -. , 415


.
(0,05 /) (1 /)
,
(20 /) 2+.
, , .
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, .

, ( ), ,
.
,
,

.
, ,
, . , , , , .
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(, , .). .
AT III, , .
416


AT 111
.
.
,
.

, .
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AT III , , 4 .
- .
AT III , ,
-. ,
, , - .

, , , . - . .
,
, AT I I I ( = - 0,535). ,
417

, ,
. AT III ( ) AT I I I . , 150,0-200,0 , -.
, AT III
,
-.
- ,
,

4- (PF-4) , AT III.


.
- (I ),

, -,
, .
- .
AT III,
10,000-20,000 / ,
.

- , . (
4 ) , .
AT III 65-80% ,
418

AT III ,
, , -.

AT III. ,

, -. , , AT III
-,
, , , AT III, - AT III, , AT III. , AT III ,
, -

, , 4- (PF4) , . - .
-
() ,
. ,
2 AT III. , ,
, .
, , -
.

,
, 419
14

.
,
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, .
,
, .

. ,
20 - 0,25-05 48 ,
.
,
. , , . ,

,
.
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( 75 225
),
. , 420

.
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, ,
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-
.
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( ).
, .

( )
( ),
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, . 75-100
.

0,25-0,5 48
. 421

,
- , , .
. ,

.

-
.
() ().
,
. , - II - III .
4
AT I I I - . , ,
. -
200,0-400,0 48-72 3-4
, , .
,
, - .
, ,
,
, 422


( , ).

, .
,
, .
-
.
(15,000-20,000 /) (150 ICU/)
-, 4-5 III ,
() (D-E - ), .
- :
AT III, , - .
, -,
. (500-1,000 /) (2,500-5,000 4 ) , , AT
III, .

-,
-
- ,
:
1.
( ),
423

(
);
2.
(,
,
,
.);
3.
,


;
4.


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.
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,
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-.
,
,
.
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.
424

,

,
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.
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-
, .


,
, .

XII

,

-
1.
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. . .
, . , . . ,
,
, (110 30 ). - - , .
0,5% ,
.

.
.
: , - . III,
, .
5-17 (
5400 ). III 426

- . (--- D--- ). - ( 24 ).
III
, .
, . -, 5000 III. -, -. -, -
(TFPI). TFPI , . ,
, . .
.
, , , , , . 99.

. 99.

, III. ,
- ,

427

III.
I I I - - ( ),
-. III
(58,9%).
,
- , III,
.

1.1.

. , . - , , , , .
-
- 4 .
15-30 , 2-6
.
.

5 000-10 000 , .
, , . ,
. ,
- , -.
, - in vitro. 10
2,0 ( 10 000 )
, . ( 20 30 ).
2,5 .
428

,

. : 2-3 . ,
. 5000-10000

900 / 10-15 //.
, -.
. 100 1 , 50 10 .
, .
, : ,
.

. 100.


. , , , 1 (. 100). 429

. - ,
, (, , , ,
) - PF4
- .
. , , ,
, ,
AT III , .

, , . , . , . , , ,
, . , .
-
, ,
.
. ,
, ( 2)
.

. ,
PF4
.

1.2.
- ,
.
430

.
,
.
, .
.

5% , 30% ..
. . 3-4
, , (
100 000/. .
, , , , . 6-12 . , - () 1- - - II - .
11.
I , (
), ( 10-30%), , ,
;

(.46).
46
- 1-
:

'" - 10-30% .

: - .
,

II - .
.
Fc-
II;
431

Fc-
, II;
, FcRII , ,
II;

:
) -
II;
) ,
,
, II.

(GP) Ib-IX, , , FcRII-
--4 .
GP Ib-IX FcRII
. ,
,
FcRII-, GP Ib-IX.
,
II: , , PF4; PF4.
.
FcRII-

-4-, , ,
. ,
,
PF4 . , , , .
FcRII .
- .

, HS, PF4. , in vitro
aHTn-FcRII-.
432

, ,
II ,
(. 101):
A)
-
;
) -4, , ( ), ,
, -4 ;
B)
---4 ;
) ,
.

. 101. -
II

- .
50%
. , , . II 47.

II,
. 433

47
-
II
:
1. : 50%
.
2. .
3. , .
4. .

(PAT),
, II
()
(0,5 / ), (10,0 /), . , -
. , ,
; ,
.
; -
,
.

(>90%),
. ()
, .
.
, (SRA)
,
. , , .

, , PAT,
(, , ) .
434

- (HIPA)
- . , , , .
- IgG II .
4/ ELISA ,
;
. , IgG IgGl>IgG2>IgG3. , , PAT, ,
II , PF4 (11-8, -
2 (NAP-2)).
, , - ,
- , .

. , - ,
, . , .
, II
, , . ,
II.
: II
,
, II . , , , , :
, Fc-. II :
435

1.
,
, .
2.
, :
)
() ().
, II (
) , ;
)
, ;
) 5-
.

II .
, , II
, . II 48.
48
II
1. .
2. ,
,
II.
: 0,4 /, 0,15 /.
1,5-3 .
3. /, 20-30 /
12 .
4.
S.

II. , ,
,
. II.
, .
, .
,
.
, , , . , 436

, S. , ,
:
) S;
) , 5-6 , .
, .
,
( ).
, , , .


in vitro: , .

(OrglOl 72) . Org 10172
(Lomoparan, Orgaron) in vitro
10% .
,
-
.
:
( -),
, , , ,
, ,
, .

2.
() (. 49) 1/3
. 1000 10000
5 .

, , . III,

. --.
/
437

49

1:1, 1:4, 1,9:1 (, ) 3,9:1 . ,


.
4
,
. , . ,
.
X,
.
TFPI, , , VII . , 30% 438

, TFPI
70% . , , (. 102).

. 103. -

, , :

;
;
;
439

;
1-2
;
.

2.1.
90%. 190270 , 3 .
18-28 , -8-12
. , , . 0,5 %
.
.
, :
(), (), (), (),
(, ),
(-), (, ), (), (). -, .
- 2,0:1,
-3,1:1, 3,7:1.
4 , ,

(, -)
.
- 4,1 , 3,7
, - 2,8 .
, AT III, 50.
, ,
- , .
. ,
Gazzaniga (1995) 440

50
, ATIII
1. TFPI
2. - II
3.
4.
5.
6.

20 /
5,2% , - 0,9%; - 0,5%, - 0,5%.
,
, ,
. ,
I , , , .
51.
51

/ ()


-.
.

F1+2

.

.


-,
.

.

3.

,
441

. , , .

. (
1714) . ( G ) ( D, F). .
: 60 D , 30 F , N- F
. , ,
, 25-30% 3-0 . .
,
, Org
31540/SR90107A (C)rg315) - .
Org315 III, ,
. ,
,
, .
, . Org315,
. , AT I I I ,

AT I I I . AT - Org315 .
( , 1,
) AT III,
.
. , , Org315 : TFP1

(.51).
442

AT I I I .

AT III, AT - .
AT I I I 500 . ,
AT I I I
AT III. ,
, ,
, .
,
,
(, - //). , ( ) 100%
, 76% - , (0%) Org315.
Org315 . ELISA- , ,
PF4-renapHH.
52
Org 315
100%


,
1 -3
15-20

Org315 (. 52), , (1
/)
.
, 24
. ,
, F1 +2 .
443

4. - -
-, -
. , . - , , ,
AT III - . - -- II , , AT III.

.

: , , , . , () .
Orgl0172 (), , ,
--.
- ( PPS, SP-54, PZ-68
) . , -,
MF701 (Opocrin Laboratories, Corlo, Italy) 370 (). , , .

5.
:
-
. , III. ,
- .
,
4 .
444

. 104.

. 104.

: ; -; ;
, - .
- , .
- - , 65 .

. -
, , VIII , , .
- -, .

, , - . ,
- , .
- - 0,1 / 8 . - 1,3% . ,
- .

III . ,
445


.
- - LY806303. , -57.
, . - .
,
. , ,
, .

6.
- ( , ), .

GPIb . GPIIbllla, .

2. ,
.
, /
.
. ,
. , ,
446

. ,
, , ,
, , ,
.
, .
100 - - GPIIb, ,
. 5 2 , 2- . 2, , . (GPIalla, GPIV, GPVI), .

,
. GPIIbllla. PgG2 PgH2 , , , .
105.

. 105.
1 - ; 2 - ; 3 - , ;
4 - 1 ; 5 - ; 6 - ; 7
- 11111; 8 - .

447

6.1.

. , .
250 500 . , 500 (Bayer),
500 100 , 50
100 (Lannacher), 325 (A&S
Pharmaceuticals). - .
.
529
,
.
--1 - ,
-2 - :
(, ). -1
-2 72
, .
: , - - . ,
529 -1
516 -2. .
(15-20 ), -1, -2. , ,
-1 -2 50-100 . ,

, . ,
-1, , ( 10%) .
, 2 -
- 1, - -
-1, -2.
, ,
2.
448

,
10-
. :
,
( , , ,
- , .).
,
- ,
(PAF).
- .
-1 . . , N- 600 .
53
-

()
()

(/)

-1

100

24-48

-2

650

3-4

50-81
3000-5000

. -, (1500 )
- .
.
500 .

(. 53). 54.
.
30-40 , - 1
. 3-4 . , ,
. 449

54



(/)
75

75

160

50

160

40-50%. , , , ,
. -1 , .
15-20 .
, . - ( ), ,
. .
. 48 , - 2 . .
- : 0,1-0,15
. 10 . , -,
.
, .

6.2. , ,

(, ), ().
, - .
450


.
. .
,
. , , . , , .

in vitro, - . - , . PARIS I PARIS II - .

6.3.
. , -
. ,

- ,
.
:
(2,),
G-- ,
(P2Y,). , ,
,
(2). , .
, 451

2 , .
90% , per os,
.
1-3 250 .

2-3 . 98% , .
- 2-- -
.
24-36 . ( 1% , 5% .). 250 .
, .
- .
, (75
500 ), .
. - . 2 400
48 . 500-100
30%, - 40-60%.

6.4.
,
, .
. (, ).
, ,
.

, , . 452

.
. , .

6.5. llb/llla

/
, ) . / 2+- / , .
/ ,
400-411 3- . ,
, , , RGD-. /
,
,
- . /
.
,
- .

/ .
/: ,
.

, Fab-
/ . 7 (PeoIIpo Lilly-Centrocor, ). (7 Fab)
48 . / : RGD-
KQAAGDV (---- -) - .
(av
3 )
-1 (-1 CDllb/CD18a).
.
- ,
/ 453

1:1,5. 15-30 . 65% , . 24


50-60%.

(EPIC).

. (EPILOG, CAPTURE) , .
, /
, (,
COR Therapeutic).
800 . , Ilb/IIIa,
.
/ RGD. 2,5 . 4 30%.
, 11b/
111a (Hoffman La Roche). / - (Searle).
. . , , , , . , / .
(, )
.
2 ,
. , RGD-. 495 . .
0,15 mg// 4
2,5 , 97%. 1,6 .
454

6.6.
,
. - - ( - 3 ).
- .
.
( ),
. .

, . .

7.

7.1. ()

, , ,
.

,
.
(purpura fulminans). .
, - . , ,
, .
( , ) Gruber ., 1998, in vivo
. S.
.
455

,
, .

- Ceprotin, Baxter.
Ceprotin . Ceprotin
.
,
.
,
, , .
.
, , -
. ,
.

7.2.
(rTFPI)
TFPI- LACI- (- ) . 1-- TF/VIIa/ TF/VIIa//,
, , ,
(. 106). TFPI
.
TFPI

( );
TFPI ,
TFPI 6-8 .
456

. 106. TFPI

, in vivo 3 TFPI: 8085%


; 10% 3% . TFPI 42000 3
.
TF/
FVIIa// 2 :
1) TFPI ;
2) 1 TFPI Vila TF/VII/.
III , , - TFPI
.

TFPI ( TFPI I II , TFPI, 3 ) -, . , TFPI
. TFPI ,
. TFPI , TF- , , , , ,
, .
TFPI,
,
. t-PA- 457

rTFPI. ,
rTFPI .
, rTFPI
,
24- TFPI . ,
,
VIIa-TF. , rTFPI
, .

7.3. Vila


V11a . 1970 IX/VIII,
. ( )
. 10-15%
IX/VIII -
VIII.
-
, ,
50-60%. , ,

Villa.
V11a ,

.
VII .
VII ,
N- Gla-,
, - -
, . -
Gla - .
458

.
- ,
Gla- - .
VII .
VII
.
- , ,
, VII. VII
,
, VII .
, Vila , X.
, , ,
.
, IX, V, VIII
. , , pVIIa X IX.
V11a
: V11a.
V11a
1978 ,
Villa.
, , V11a - .
V11a, 10
V11a .
VII NovoNordisk.
V11a (NovoSeven)
1996 , - 1999 , - 2000
, .
pVIIa
V11a.
- . ,
V11a

459

-, pVIla
9 - ,
, 35
, 50%.
pV11a.

Vila pVIla.
VII -
. VII 13
8 . VII
,
VII, , . . , pVII

.
,
VII 152153. pVII , ,
.
pVII -
pVII.
.
100%.
pVIla Q-ce , ,
. - NovoSeven - 1,2 , 2,4 , 4,8 pVIla .
pVIla 2,45 , . pVIla
34,5 //, - 93,6 /. NovoSeven 55 56.
pVIla NovoSeven .

VII . ,
- VII.
pVIla , . , , (,
460

55

NovoSeven

17 /

35 /

70 /
36,1

//

30,3

32,4

MRT

3,3

3,3

3,4

Vss

98,1

109,6

119,9

AUC

//

11,0

25,9

53,3

t 1/2

2,6

2,7

2,8

56

NovoSeven" ( )

//

32,4

36,6

MRT

3,5

2,9

11/2

2,8

2,5

Vss

110,0

103,5

AUC

//

29,8

22,3

), - 10% . V11a .
1985 , pV11a . , 50%
, NovoSeven
50-100 / . ,
- NovoSeven .
pVIIa , V11a
. , pVIIa 1, Villa ,
.

V11a

-.

461

, - , .
. NovoSeven
,
.
NovoSeven .
, . ,

, ,
.

7.4. III
III
-, III , .
III 1980
. AT III . ,
1 , , , .
. V111a, IXa, V11a,
,
,
AT III
, , ,
AT III
/.

AT III.
AT III . 462

, AT III , - .
(6-)
. 5-10% .
,
, ,
, . AT III .
,
. 155.
, .
AT III . .
AT III (,
Behringwerke)
AT III.
AT III
. AT
III . AT III
AT III , . , AT III , .
AT III -
60
10 . - .
III. (Kabi Pharmacia AB), Hyland. -. 10 , 500 ME .
Throbate-III (Bayer) - . 10 20 , 500 1000 .
III 463

.
.
,
(8,5 1,2 ) 100% 96 ( 66,59 U/).
39
- .
.
-.
,
. ,
, -. , , -.
, . ,
, .

8.

: , . , , . ,
,
.
. - .
8 . 1-, 464

92% , .
3,5 S-1-. -
, 12,2% . , , 21 1-.

III, V11a, 2- ,
.

750 . 2
. 2-

.
.

, .
-
(), (), ().
(Bayer), (-), , . :
. . , ,
. ,
, X
. , ,
.
,

.
- .
, , , , , , . -
30
500 000 ( ) .
1 000 000 .
10-12 .
,
. . , , 465
16 - 6578

. .
,
. - . (1-2 ), , . 40 000-60 000 .
- , 58 6500 .
. , , - . 1 1/6 . 50 000 ,
, 50 000 . 300 000 .
, - , ,
, , . AT III ( 70%)
- .

9.
-
() - ,


,
. , , , .
VIII 70% . ,
6 ,
-18 1 .
1 . 450
45% 290-300 . 466

65 , - .
37. 30 .
1-6 24 .
.
II, V, VII, X, XIII
.
,
. II 2-3 , , - 10-40% . 10-20 / .
V , 5 , - 10-15%
. 10-20 / .
VII - 2-6 ,
5-10% . 10-20 /, - 5 / 4-6 .
X
10-15% . 10-20 /, - 5 / 12 . XI
30% ,
, V X.
XIII - - 6-10
, - 15% .
,
III . , , .

,
, .
, . - .
- , , , .

, . , - , .
467

,
. -,
(10-15 / ).
1,5
1 /, , .
,
III . , ,
-, , .

9.1.
, () .
-. -
, . .
.
, .

,
1,1 % 1982 1,5/1 000 000 1997 .
. , 1992 .
0,03%,
2,7/1 000 000.

9.2.
-
.
468

. 0,9% ;
15 / . ,
.
(120%), (124%), ( 34,8),
75,71%.
45-50
/. , 5%
, 10% , 300-500 , , . . .
,
(5%) .
.
, . , . (136%),
(142%). III 119%. 15%. ,
. , ,
, .

10.
-

- ,
. , .
469


. , . 50-70
. -
. 22.

. ,
(7-10 ),

1 , 4. 72
.
. ,
2- .

: I III,
. .

.

.

- . ,

50x109 100x109/. .
.

42% -, - 54% .
3 . .

. 470

2-10% . , , , ,
40%. .
,
. , , .

. , .

11.

4, (10-20 ) . , VIII, XIII, . 18 ;
1 . , -
.

80 VIII (.. ), 250 , 20-30% XIII 40-70% .

; 10-20
, . , 6 , .
:
- , ,
XIII . .
, - 471

. , ,
. , , .
.

12.
1970
, .

.
, , -1-6 .

- ,
,
.
1,5 /
, 2-6
.
, .
, , VIII. VIII ,
VIII- .
In vitro, ( ),
.
.
(,-), tPA
.
.
, ; ,
.
472

, ,
.

13.
.

. , ,
, . .
, . .
, : , , . , VIII,
VIII.
-: /
,
, , .
(ASFA)
, . , ;
, , ; , , , - , .
- 473

, :
- ;
- -;
- ;
- ;
- , , ;
- ( ,
, , ).
0,5-3
1-4 . ,
,

. , .
.
, ,
. 40 / . ,
, , .
1 1,5 , 3000 4500 .
1000 ,
; , ,
, (5% )
1:1.
IgM,
.
.
, (,

.

, , .
474

( ), , . 28% ,
:
- - : ,
, , , ,
, ;
- ;
- (
);
- ( 2,5% );
- : , , ,
, , , ;
- ;
- , ,
, .


1.

..
.
. ., 1989, 8, . 43-46.
2. .., .., ..

. ,
1992, 3, . 7-11.
1. .., .. //. -1998.-9. . 10-11.
3. ..
.// .... ....-., 1992,25 .
4. ..

.// . . ... ..., ,
1987, . 22
5. ..

() .//
. . ......, .,2000, - 22 .
6. . .

.//. .
.... . .- ., 1992, 26 .
7. ..
,
.//. . . , , 1998, 137 .
8. ..
,
.//. .... . . - ., 1998,20 .
9. .. (, ,
). - .: - , 1979. - 352 .
10. .., .. // . . -1986.
-4. - . 32 - 35.
11. . . // . - . - .:
, 1979. - . 14 - 26.
12. .. -
// . . .
. - 1977. - . 2. - . 3-13.
476

14. .., .. ,
.// III . . " ,
-. ", 1997, .21-22.
15. .., .., .., ..
. - .: , 1995, 243 .
16. .., .., .. .
.-, 1980.-313.
17. .., I I . . II . . -1987. - 11.-.
138-142.
18. .., .., .. .
. - : - . -, 1992 - 176 .
19. ..
.//....
. . . ., ., 1998. - 24 .
20. .. . - .:
-, 1998. -56 .
21. .. . - .:
, 1989,385 ,
22. .. - .-.: -, 2000. - 148 .
23. .. : .
.. - . , 1985 - .2
24. .. . - .:
, 1980.-336 .
25. ..// . . - 1983. - . 55. - 12. -'- 125.
26. .. // : .
. II . , 1983. - . 223 - 225.
27. .. / .
.. . - : , 1985. - . 25. - .
527-528.
28. .. // : III . -. - .. 1985. -.183-184.
29. ., ., // . . -1993 -7.-.
58-61.
30. ., .., .II. . //. . 1997. - . 69. - 2. - . 35 - 37.
31. ., .., .. .
(-): , . ., 1989.-23 .
32. ., . II.
. - .: -, 1999. - 224 .
477

33. .., .., . .//. .


. - 1988.- 1.-. 23-29.
34. .., .., .. . //. . 1988.- 11.-. 7- 12.
35. .., .. //. . -1992. 4. -. 3-5.
36. .., .. // . .
. - 1997. - . 124. - 9. - . 346 -347.
37. .., .., .., ..
,

. // III . . "
, -. ", , 1997, .
16-17.
38.

. .//.. . . - ., 1999. -105 .
39. ..

.// . .. . . - .,
2000. - 24 .
40. .. III
-, .//
. .... . . . -., 1984. - 19 .
41. .. , ,

.// .... . . .
., ., 1990,22 .
42. .. .//,
1986. - .26. - 9.-. 116-118.
43. .. .//.., 1993.-.65. - 10. . 101 -105.
44. .., ., .
. - : -, 1989. -240.
45. ... :
?// . .,-1998.-5.-.52-54.
46. .. - . ., 1977.
47. .. // . . - 1983. - 7. - . 3 - 8.
48. .. // . . - 1989. - 7. - . 3 - 8.
49. .. - .:
, 1990.-. I-432 .
50. .. . - .: , 1994. -32.
478

51. ... .., .. .


. - 1997. - 5. - . 12 - 14.
52. .. .., ..
// . . - 1984 - 6 - . 3-9.
53. ..
.// . ... .. . . - , 1997. - 65 .
54. O.K., .., .. .// .
. - 1983. - N 8. - . 50 - 52.
55. O.K., .., ..
: , ,
. - : , 1985. - 286 .
56. O.K., .., .. . //
. -., 1987. -.
181.
57. ..
. // . .... ..., .,
2000,-23.
58. ..
.//
, 1993, 6, .67-68.
59. .. II . - . - : , 1979.
-. 129-133.
60. .. // . . -1983. - 8. - . 29 - 34.
61. .. II
: . .
-. - 128. - : - . . -, 1984. - .
15-22.
62. . . 5'
.// . ... . .
.-. 1987. -24.
63. .., .., .. . - : , 1994. - 256 .
64. ..
.
. -. - 1987,83-84.
65. .. ./
/ .... . . , , 1987,36 .
66. - .. .., ..

, .// . . -1987,
12,3-5 .
479

67. .., . . .- .: , 1989, . 256.


68. .. //, . . -1988 - 5. - .321 - 325
69. .. //, . . -1994. - 2. - . 136-155.
70. .. //. . . -1994. - 4. -334.
71. .. //, . . -1996. - 1. - . 1 -5.
72. .., .., .. . // .
. -1983. - 8. - . 3-7.
73. ..
. . , 2000.-364 .
74. .., .., .. . - ,
1985.-112.
75. .. (
). - : . 1983.-221 .
76. .. -:
, 1991.-302
77. . .
.// . .... . . - ., 2000. - 24.
78. ..
.// ... . . . , ., 1990,20 .
79. ..

.// ... . . - .,
1992. - 130.
80. ..
.// . ... . . - ., 1998. - 22.
81. ..

.//
. .... . . . - ., 1989.
82. .., .., .. .
. , 1990,
2, .26-29.
83. .., .. //. . -1991.-
11.-. 22-25.
84. .., .. //. . -1983. 8. - . 45 - 49.
85. .., .., .. ,
. - .:
, 1989.-320.
480

86. .., ..
. . .
, 1981, . 63-64.
87. . .
.// . ....
. . . - ., 1982. - 24 .
88. ..

1-11 ., .... . . . ., 1987,
22 .
89. ..

, // . . . . 1985. -8. -. 50-54.
90. .. - :
. .... - . . - .. 1986.
91. .. . - .: , 1993. -159.
92. .. . - .. :, 1998.
-191.
93. . ., .., . . II . - , 1982. -. 18-19.
94. .. // , . . - 1988. - . 69. - 5. - ,
383 - 386.
95. .., .., .. . // ,
. . - 1997. - 5. - . 68.
96. ..
.//
, . .. , .. , , 2000. - 35-38.
97. .., ..
.// ., 1998.-262.
98. .. ,


.// . .... . . . - .,1981. - 35 .
99. ..
.//., , 2000, 343 .
100. .., ..

.//
1997,1, .38-41
481

101. . ., . .
- .// 1985, 10,
.71-77.
102. ..
. // . . .-1997.- 1 -.29-31.
103. .. . - .: ,
1970-304 .
104. .. // . . -1986. -2. -. 56-82.
105. ., .., .. : , 1988. - 148 .
106. ., .. // . .,1984. - 4.
-. 68-72.
107. ..
// . -1986. -. 17, 2, -. 56-82.
108. . . ,

.
109. . . ,
- (
). // . . ... .
. . - , 1997. -37.
110. .., .., . // . .
. -1996. - 4. - . 17 - 20.
111. .., ., .. // . .
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112. .., .. // . . -1991. .: . 25 - 26.
113. .. . //. . .... . .,
1992, 43.
114. .. , ,
. // . ...
. . . , -,1986.-23.
115. ..
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116. ., ..
, . - .,
, 1977.
117. ., .., ..
,
. //
, 1974, 11,1-5.
482

118. .. ,

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119. .. .//
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120. .. . -., ,
1988. - 288 .
121. .. . -, ,
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122. .., .. // . . -1996. -2. . 32-34.
123. B.C., .. ..
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124. .. . - .: ,
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125. .., ..
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126. .. .// ., 1996.-201.
127. .. // . . -1985. - 8. -. 1012.
128. .., .., .. . //
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129. .., .., .. // .
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132. .., .., . // .
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133. .., ., .. //.
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135. .. . .: , 1977.-311.
136. .. -
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