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בסיס פתולוגי למחלות - רובינס תרגום עברית

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  • TINU 1YGOLOHTAP OT NOITCUDORTNU P ega4

    (:cimetsyS) ( lareneG) . : . :

    : 4

    .1, (' ) " 0052

    : (. )deriuqca ( )cisnirtnI

    . ( ) (. ) '

    . " " .2

    . FC ' . ,

    , . ,

    , , , ' .

    .3

    " . orciM AND " .

    , syarra .

    (snoitatsefinam lacinilC) .4

    . , , ,

    (. )91 (.yrujni llec)

    / xirtam llec- - .

    WEIVREVO : ilumitS suoixoN dna ssertS ot sesnopseR ralulleC egaP 4

    , sisatsoemoh

    , . . " , noitatpada ralullec

    : aisalprepyH yhportrepyH yhportA

    1

  • 2

    tnega suoirujni , - ' , . yrujni llec

    . nruter on fo tniop :

    . ( ) sisorceN . ' sisotpopA

    .AND

    . snoitaretla ralullecbus , . , snoitalumucca ralullecartni (. )

    .

    yrujnI ot sesnopseR ralulleC -- 1-1 ELBAT esnopseR ralulleC sulumitS suoirujnI fo ytireveS dna erutaN

    :snoitatpada ralulleC :ilumits cigoloisyhp deretlA

    noitalumits cihport desaercni ,dnamed desaercnI )senomroh ,srotcaf htworg .g.e(

    yhportrepyh ,aisalprepyH

    yhportA noitalumits ,stneirtun desaerceD

    aisalpateM )lacisyhp ro lacimehc( noitatirri cinorhC

    ;yrujni lacimehc ;ylppus negyxo decudeR noitcefni laiborcim

    :yrujni lleC

    yrujni elbisrever etucA detimil-fles dna etucA

    htaed llec yrujni elbisreverrI )egamad AND gnidulcni( ereves dna evissegorP

    sisorceN

    sisotpopA

    sellenagro suoirav ni snoitaretla ralullecbuS yrujni cinorhc dliM

    snoitacificlac ;snoitalumucca ralullecartnI deriuqca ro citeneg ,snoitaretla cilobateM

    lahtelbus evitalumuc htiw naps efil degnolorP yrujni

    gniga ralulleC

    noitaitnereffiD dna htworG fo snoitatpadA ralulleC egaP 5

    , , , .

    .aisalpateM .

    " . " .

    ' . MCE

  • aisalprepyH egaP 6

    noitatpada ralullec ehT .sllec laidracoym daed dna ,derujni ylbisrever ,detpada ,lamron neewteb spihsnoitaler ehT 2-1 erugiF era stceffe yllareneg ,muidracoym derujni ylbisrever nI .sisorcen cimehcsi si htaed llec fo epyt eht dna ,yhportrepyh si ereh detciped tfel eht ,yhportrepyh laidracoym fo elpmaxe eht nI .segnahc cipocsorcim neve ro ssorg tnerappa ylidaer yna tuohtiw ,lanoitcnuf ylno aera thgil larumsnart eht ,sisorcen gniwohs nemiceps eht nI .)mc 5.1 ot 1 si lamron( ssenkciht ni mc 2 naht erom si llaw ralucirtnev

    htiw deniats neeb evah snoitces esrevsnart eerht llA .noitcrafni laidracoym etuca na stneserper elcirtnev tfel laretaloretsop eht ni emyzne ot eud si niats ot eruliaF .atnegam muidracoym elbaiv sroloc taht etartsbus emyzne na ,edirolhc muilozartetlynehpirt

    .htaed llec retfa egakael

    aisalprepyH cigoloisyhP

    , . ' ' . "

    . AND :

    ' . aisalprepyH lanomroH .1 . ,

    ' . aisalprepyH yrotasnepmoC .2 . , . , ymotcerhpen laretal'

    , "

    . FG .

    " . ( yrotasnepmoc) ' .

    .sititapeh cinorhc ' .

    aisalprepyH cigolohtaP

    . FG

    ' 41-01 " , "

    . . . ,

    . , , ,

    3

  • sesruivmaollipap' snoitcefni lariv .

    Hyhportrepy

    . , .

    , AND . .

    . ( ) " ,

    , sredliubydob ' . . daolkrow

    . "' daolrevo cimanydomeh cinorhc

    . , , '

    ) " . . (.

    divarg a dna )thgir( suretu lamron a fo ecnaraeppa ssorG ,A .ycnangerp gnirud suretu eht fo yhportrepyh cigoloisyhP 3-1 erugiF )tfel( suretu lamron a morf sllec elcsum htooms eniretu depahs-eldnips llamS ,B .)tfel( )gnideelb mutraptsop rof devomer( suretu

    .)thgir( suretu divarg ni sllec pmulp egral htiw derapmoc

    ( ) . , . "

    , , ,

    . (.)

    yhportrepyH fo smsinhaceM

    .

    , (syawhtap noitcudsnart langis) .

    4

  • .yhportrepyh laidracoym gnirud snietorp dna seneg detceles fo noisserpxe eht ni segnahC 4-1 erugiF

    :

    nuj-c, sof-c - rotcaf htworg tsalborbif, 1GFI 1 rotcaf htworg ekil-nilusni, FGT -

    (2nisnetigna, 1niletodne, stsinoga cigrenerda stnega evitcaosaV

    ( hctiws) '

    . , esaPTA , ' ,

    , ( rotcaf citeruirtan lairta )FNA ' , . , , FNA(. )FNA

    .daol cimanydomeh , ( )

    : sreggirt lacinahcem .1

    , 1FGI sreggirt cihport .2 (tnega evitcaosaV)stsinoga cigrenerda , 2

    , " sllec elcsumnon " stnega evitcaosaV FG .

    . "

    . , . .

    . , ,

    .

    Ayhport

    . .

    (. ) , : tcud lassogyrht drohcoton .

    . .

    . .

    5

  • : , daolkrow desaerceD .1

    . , .

    . .

    ( yhporta noitavrened) .2 .

    . ( ) .3 , ,

    .sisorelcorehta

    gniga ot eud si niarb eht fo yhportA .esaesid citorelcsorehta htiw elam dlo-raey-28 na ni niarb eht fo yhportA ,A 5-1 erugiF niarb fo ssol taht etoN .elam dlo-raey-63 a fo niarb lamroN ,B .deppirts neeb evah segninem ehT .ylppus doolb decuder dna

    iclus eht snediw dna iryg eht sworran ecnatsbus .4

    . (sumsaram) , (. aixehcac)

    FNT( ) .aixehcac

    ' , .5 .

    . , (yhporta elines) .6

    . . .7

    " . .

    , .

    . " , RE , . , . .

    yhportA fo msinahceM

    .

    . . : '

    6

  • , (nispehtac' ) .

    yawhtap emosoetorp-nitiuqibU nitiuqibu' " .

    . , -

    (. ) (. ) FNT

    . selucav cigahpotua

    " (. RE, ) .

    laudiser " selucav cigahpotua . sugahpocras , ydob

    . ydob laudiser ' - selunarg nicsufopiL (.yhporta nworb)

    aisalpateM

    . " ( ) ,

    . , (suomauqs)

    .

    7

    ot ranmuloc fo margaid citamehcS ,A .aisalpateM 6-1 erugiFahpose fo noitamrofsnart citsalpateM ,B .aisalpatem suo

    ilehtipe ranmuloc erutam ot )tfel( muilehtipe suomauqs deifit .)aisalpatem tterraB dellac-os(

    laeg mauqs mu arts

    .

    ailic ,

    . " , '

    .

    ( dica cioniter )A .

    . A

    , .

    , . , (. )

    . sugahpose tterrab

    raludnalg ") . (.samonicrac

    .

    . . '

  • aisalpateM fo smsinahceM

    . .

    . MCE FG, " .

    , yilimafrepus FGT ' FG .

    . . , A

    . AND sgurd citatsotyc

    .

    htaed llec dna yrujni llec fi weivrevO

    . . ,

    .htaed dna yrujni llec fo noitulove eht ni segatS 7-1 erugiF

    :llec yrujni '

    citamehcS 8-1 erugiF

    a fo noitatneserper eht dna llec lamron

    elbisrever ni segnahc llec elbisreverri dna era detcipeD .yrujni

    ,segnahc cigolohprom ni debircsed era hcihw

    dna segap gniwollof eht nortcele ni nwohs

    -1 erugiF ni shpargorcim si yrujni elbisreveR . 71

    yb deziretcarahc fo gnillews dezilareneg

    sti dna llec eht fo gnibbelb ;sellenagro ;enarbmem amsalp eht

    fo tnemhcated eht morf semosobir

    ;muluciter cimsalpodne raelcun fo gnipmulc dna ot noitisnarT .nitamorhc

    si yrujni elbisreverri yb deziretcarahc

    fo gnillews gnisaercni dna gnillews ;llec eht

    ;semososyl fo noitpursid egral fo ecneserp

    ni seitisned suohproma ;airdnohcotim nellows

    ralullec fo noitpursid dna ;senarbmem raelcun dnuoforp

    8

  • changes. The latter include nuclear codensation (pyknosis), followed by fragmentation (karyorrhexis) and dissolution of the nucleus (karyolysis). Laminated structures (myelin figures) derived from damaged membranes of organelles and the plasma membrane first appear during the reversible stage and become more pronounced in irreversibly damaged cells. The mechanisms underlying these

    changes are discussed in the text that follows.

    ': 1.reversible cell injury / .

    :

    ATP

    2.irreversibly injury and cell death , , . "lethal hit " - . , , '

    ) .( 3 .irreversibly injured cells invariably undergo morphologic changes

    that are recognize as cell death : .

    Figure 1-9 The sequential

    ultrastructural changes seen

    in necrosis (left) and apoptosis

    (right). In apoptosis, the initial changes

    consist of nuclear

    chromatin condensation

    and fragmentation,

    followed by cytoplasmic

    budding and phagocytosis of

    the extruded apoptotic

    bodies. Signs of cytoplasmic

    blebs, and digestion and

    leakage of cellular

    components

    TABLE 1-2 -- Features of Necrosis and Apoptosis Feature Necrosis Apoptosis

    Cell size Enlarged (swelling) Reduced (shrinkage)

    Nucelus Pyknosis karyorrhexis karyolysis Fragmentation into nucleosome size fragments

    Plasma membrane Disrupted Intact; altered structure, especially orientation of lipids

    Cellular contents Enzymatic digestion; may leak out of cell

    Intact; may be released in apoptotic bodies

    Adjacent inflammation

    Frequent No

    Physiologic or pathologic role

    Invariably pathologic (culmination of irreversible cell injury)

    Often physiologic, means of eliminating unwanted cells; may be pathologic after some forms of cell injury, especially DNA damage

    9

  • 01

    , , .

    AND .

    . .

    , ' .

    CyrujnI lleC fo sesua

    . :

    noitavirpeD negyxO.1 . .

    . , .

    . :

    . ' )OC

    . ( .,

    , , ' (. ) ( ) "

    , , ' , stnega lacisyhP. 2

    . sgurD dna stnegA lacimehC .3

    ., , . . , ,

    .' , , :

    . stnegA suoitcefnI .4 . . , ,

    ' . ' snoitcaeR cigolonummI .5

    (. ) ,

    ' ) snoitcaeR citeneG .6 (. ' )S (

    .

    . secnalabmI lanoitirtuN .7 . ,

    . ( ) . , .

    .

  • MyrujnI lleC fo msinahce

    ' , . : , . , .1

    , ,

    . . , .2

    . , ,

    . .

    . .3

    !

    .yrujni llec ni egamad fo setis lacimehcoib dna ralulleC 01-1 erugiF

    :

    PTA o o o

    cigolohprom dna lanoitcnuF 11-1 erugiF ralullecartni desaerced fo secneuqesnoc .yrujni llec gnirud PTA

    o o

    DPTA fo noitelpe

    11

    " PTA PTA .

    , PTA ,

    . revonrut sdipilohpsohp : PTA

    evitadixo o PDA noitalyrohpsohp

    . " PTA yawhtap citylocylG o

    .

  • PTA , , .

    : ' ' %01-%5 PTA

    , ' . -K+aN .1 , , .

    .RE , . .2

    evitadixo , , .noitalyrohpsohp

    PMA PTA " . - . . Hp .

    , ++aC ++aC .3 .

    , RER , PTA .4 . .

    (., 8-1 . ) , . , .5

    . esnopser nietorp dedlofnu ,

    . "

    egamaD lairdnohcotiM

    " . . : "

    sserts evitadixO 2A "

    , (slennahc ecnatcudnoc hgih) "

    .,

    - . ,

    evitadixo - , noitalirohpsohp

    . ,

    C . C ,

    .

    fo ssoL dna muiclac ralullecartni fo xulfnI sisatsoemoH muiclaC

    21

    .

    lairdnohcotiM 21-1 erugiF ( 1.0 ) .yrujni llec ni noitcnufsyd

  • . esaPTA +2gM,+2aC " , '

    .RE

    . '

    : . (PTA )esaPTA o o o

    AND o

    . ) "

    , ( .

    slacidar eerf devired-negyxo fo noitalumuccA

    )sserts evitadixo(

    . " .

    fo secneuqesnoc dna secruoS 31-1 erugiF ,PTA .yrujni llec ni muiclac cilosotyc desaercni enisoneda

    ' . seiceps negyxo evitcaer . ,

    . .sserts evitadixo '

    . " , , , .

    , : ) ' (. ' , , ,

    , ' , .

    :'

    evitcaer fo elor ehT 41-1 erugiF si 2O .yrujni llec ni seiceps negyxo yb )- 2O( edixorepus ot detrevnoc eht ni semyzne evitadixo ,)RE( muluciter cimsalpodne ,enarbmem amsalp ,airdnohcotim si - 2O .losotyc dna ,semosixorep noitatumsid yb 2O 2H ot detrevnoc +2uC eht yb HO ot ecneht dna .noitcaer notneF dezylatac- +2eF/ morf yltcerid devired osla si 2O 2H toN .semosixorep ni sesadixo yllaitnetop rehtona si nwohs .negyxo telgnis ,lacidar suoirujni ot egamad lacidar eerf tnatluseR dna ,snietorp ,)noitadixorep( dipil llec fo smrof suoirav ot sdael AND edixorepus taht etoN .yrujni ot +3eF fo noitcuder eht sezylatac HO gnicnahne suht , +2eF .noitcaer notneF eht yb noitareneg semyzne tnadixoitna rojam ehT ,)DOS( esatumsid edixorepus era enoihtatulg dna ,esalatac esadixorep

    31

  • 41

    ' (. yar-X, VU ) .1 (. )

    (.edirolhcartet nobrac)LCC 4' . .2, . - .3

    " . " ' . , , , RE

    : O2 - - H2O2 - edixorep negordyH - HO snoi lyxordyH -

    . NMP .esadixo HPDAN "

    ( esadixo enihtnax' ) - , .

    , ' .4 (notneF)

    +2eF+H 2O2 -HO + HO + +3eF , (+3eF )cirref -

    (. " . ) ( +2eF )suorref , , " ,(ON )edixO cirtiN .5

    , .ON3- ON2 ( OONO- )noina etirtinyxorep evitcaer ylhgih

    : ,

    (41-1 ) enarbmem fo noitadixorep dipiL .1

    . . HO

    , (noitagaporp) . ,E ,regnevacs noitanimreT.

    snietorp fo noitacifidom evitadixO .2 . , ( ' ) ,

    " . .

    . " AND ni snoiseL .3 (.7 )

    .

    ', , . (.41-1 ) " " - ' .

    : ' ( egnevacs" )" stnadixoitnA .1

    . :

    A dica cibrocsaE

    . .2 .HO ,

    :

  • . ( edixorp negordyh) .3 :

    (O2H2 + 2O 2O2H2 )H2O2 (O2- + 2H H2O2 + O2 )H2O2 esatumsid edixorepuS

    , esatumsid edixorepus esenagnam ' esatumsid edixorepus-kniz-reppoc

    : " esadixorep enoihtatulg H2O2 + 2 2O2H 2+)remidomoh enoihtatulg( GSSG HSG

    O2H2 + GSSG HSG2 + HO2" " , HSG GSSG

    .

    , . , .

    DytilibaemreP enarbmeM ni stcefe

    .

    . , PTA ,

    (. ) " , , "

    . : '

    noitcnufsyd lairdnohcotiM .1 ,

    . ,

    . , . PTA

    . .

    :

    sdipilohpsohp enarbmem fo ssoL .2

    ,

    ni egamad enarbmem fo smsinahceM 51-1 erugiF : cilosotyc desaercni dna 2O desaerceD .yrujni llec yam tub aimehcsi ni nees yllacipyt era +2aC evitcaeR .yrujni llec fo smrof rehto ynapmocca no decudorp netfo era hcihw ,seiceps negyxo esuac osla ,seussit cimehcsi fo noisufreper .)nwohs ton( egamad enarbmem

    ) (-

    noitalycaer tnedneped-PTA

    51

  • 61

    seitilamronba noteleksotyC .3, . , - .

    . , . - seiceps negyxo evitcaeR .4 : stcudorp nwodkaerb dipiL .5

    deifiretsenu (enitinrac lyca)

    : , , -

    . ,

    , , , : . PTA . dica cielcunobir

    ,sesaND ,sesaNR: . , .snispehtac ,sesadioculg ,sesatohpsohp ,esaetorp

    . ,

    RyrujnI lleC elbisreverrI dna elbisreve

    : PTA AND

    , , , .

    . ) ' , , ( ) , , (. 8-1

    - - . PTA .

    , sdica cielcunobir, , , .elbaemreprepyh PTA

    :

    .1 , , , .2

    (tceffe elppir ) - ' .3 .4

    . ,

    :-

    (PTA , noitalyrohpsohp evitadixo) .1 Hp " , ) .2

    ( . , -

    - , .

    : /

  • . , .- tnatsiser-erutarepmet

    . " ,

    sisorceN dna yrujnI lleC fo ygolohproM

    , . . gal emit .

    ).

    (.61-1 ,

    ,

    .

    , ' .

    .

    21-4

    - , . 06-02

    yrujnI elbisreveR

    segnahc cigolohprom dna lacimehcoib fo gnimiT 61-1 erugiF .yrujni llec ni

    : - .1

    . . , egnahc yttaf .2

    . :

    Mygolohpro

    . (. )

    . , . RE , ,

    . noitareneged ralucav ( egnahc cipordyh) .

    (:71-1 ) ( segnahc larutcurtsartlu) -

    71

  • 1. Plasma membrane alterations '

    blebbing) (, blunting) ( . myelin figures) ( -

    2. Mitochondrial changes small phospholipid-rich amorphous densities 3. Dilation of ER , . 4. Nuclear alterations .

    ecrosoisN

    - . , , .

    . :

    o , autolysis . o ,

    , . .

    Figure 1-17 Morphologic changes in reversible and irreversible cell injury. A, Electron micrograph of a normal epithelial cell of the proximal kidney tubule. Note abundant microvilli (mv), lining the lumen (L). N, nucleus; V, apical vacuoles (which are normal structures in this cell type). B, Epithelial cell of the proximal tubule showing reversible ischemic changes. The microvilli (mv) are lost and have been incorporated in apical cytoplasm; blebs have formed and are extruded in the lumen (L). Mitochondria are slightly dilated. (Compare with A.) C, Proximal tubular cell showing irreversible ischemic injury. Note the markedly swollen mitochondria containing amorphous densities, disrupted cell membranes, and dense pyknotic nucleus

    18

  • , 21-4 .

    Mygolohpro

    : ailihponisoe

    ANR " ailihposab o (81-1 . ) nisoe o

    htiw muidracoyM ,B .muidracoym lamroN ,A .muidracoym eht fo sisorcen cimehcsI 81-1 erugiF etaelcuna cilihponisoe ylgnorts gniwohs ,)erugif fo sdriht owt reppu( sisorcen noitalugaoc erapmoC .elcsum citorcen ot noitcaer ylrae na era muititsretni eht ni setycokueL .srebif laidracoym .erugif eht fo trap rewol eht ni srebif lamron htiw dna A htiw

    . , . , . ,

    . serugif nileym . " : , . spaos muiclac o o serugif nileym o sirbed cilihpoimso o (C71-1 ) , (yffulf) o

    8-1 )AND ,

    (C71-1 .esaND , - sisyloyraK .1 AND (. ) sisonkyP .2

    . . . sixehrroyrak .3

    . ,

    . - sisorcen evitalugaoC, . (. A91-1 ) '

    . , - , . , , ,

    . " " sisorcen evitalugaoC

    .

    91

  • . sisorcen evitcafeuqiL , sisorcen evitcafeuqiL

    . SNC , (. B91-1 ) . , ,

    , " .SUP

    fo ssol htiw ,sisorcen evitalugaoc gnitibihxe tcrafni yendiK ,A .sisorcen evitcafeuqil dna evitalugaoC 91-1 erugiF .erutcetihcra ralubut dna raluremolg fo seniltuo cisab fo noitavreserp htiw tub msalpotyc fo gnipmulc dna ielcun sllec etihw htiw dellif si sucof ehT .noitcefni lagnuf yb desuac yendik eht ni sisorcen evitcafeuqil fo sucof A ,B

    .erutcetihcra lamron eht setaretilbo taht ssecsba laner a gnitaerc ,sirbed ralullec dna

    sisorcen suonergnaG

    02

    . , " , ,

    tew ) " , . (energnag

    . sisorcen suoesaC - ) suoesac

    ,(. 02-1

    (. 2 noitcaer suotamolunarg ) , ,

    . sisorcen taF

    egral a htiw gnul suolucrebut A 02-1 erugiF suoesac ehT .sisorcen suoesac fo aera.yseehc dna etihw-wolley si sirbed

    .

    etuca .

    . seititaercnap . sretse edirecylgirt ,

    , (noitacifinopas taf) , , (. 12-1 )

    .noitcaer yrotammalfni

    htiw sisorcen taf fo icoF 12-1 erugiF saera ehT .yretnesem eht ni noitacifinopas muiclac tneserper stisoped yklahc etihw fo .nwodkaerb dipil fo setis ta noitamrof paos

  • 12

    " , ,

    , . " .noitacificlac cihportsyd .

  • )32 .p( sisorcen dna yrujni llec fo selpmaxE , ,

    . , .

    yrujni cixopyh dna cimehcsI

    . , , .

    . aixopyH .

    . , , , aimehcsI

    . ,

    . " " .

    . : . yretra dne

    , , .

    . , . ( )

    . ( noisufreper) . ,

    . ( noisufreper) .

    . . yrujni noisufreper-aimehcsi

    (. ) : . 22-1 :

    . " PTA- noitalirohpsohp evitadixo- "

    . , PTA "

    . (. ) ( ) "

    . 06 : : .

    , , : PTA- " . sbelb

    - "serugif nileym" " , .

    . , RER- "

    . , .

    . :

    . seitisned suohproma "

  • " . "

    . 04-03 " . ( noisufreper )

    - " ) (.

    " .

    "serugif nileym" .spaos muiclac noitacificlac

    yrujni noisufreper -aimehcsI

    , ,

    . . .

    ? yrujni noisufreper - , " "

    . . esadixo- " "

    . .(sregnevacs )

    )noitisnart ytilibaemrep lairdnohcotim- " . PTA- (

    " " NMP .

    (. 2 ) noisufreper ( )

    . yrujni noisufreper -aimehcsI -, " "

    MgI . noisufreper

    . .

  • yrujni lacimehC

    . 9 , : 2 . " .1

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    . .2

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    " , " .

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    -(4lCC )edirolhcartet nobrac . .

    054P " ( 3lCC)

    (. 054P)

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    sedixorep dipil -, .

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    . RER

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    " .

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    (. 32-1 )

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    " .

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    .

    - () " -

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    .

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    .

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    . . - ( ) . ( )

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    .

    : . ,

    -msilobatac lamososyL

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    . .

    .

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    .

    .

    eniuqorolhc Hp-

    . "

    . sitirhtra diotamuehr

    . yhtapoym cixot-

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    .

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    " "

    . 054P-

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    . , , , , sdionasocie . -

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    snoitaretla lairdnohcotiM

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    ,

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    ,

    ( )

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    . ( mn 01 ) . ,

    : : " . , -

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    . etaru ( sdiolakla acniv)

    . -

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    -

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    .

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    , , , . , ME-.

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    .

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    . . , = sucoF.

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    : " . ( 1) . ( 2). ( 3) - ( 4)

    ( 4OP) . . snietorp xirtam eussit evitcennoc ,

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    .

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  • ,slessev doolb fo sllec ,snietorp dna sllec gnitalucric :sesnopser yrotammalfni cinorhc dna etuca fo stnenopmoc ehT 1-2 erugiF .xirtam ralullecartxe eht fo snietorp dna sllec dna

    :1-2

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    - -

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  • :

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    . , ', , 0881s

    . , , , ,

    .

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    . , . 3

    .2-2 .

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    : . , = noitaduxE

    , 020.1 ytivarg , , , = etaduxE .

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    . etaudsnarT = amedE

    .etaduxE . , ( ) etaduxe = suP

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    . .

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    : 3-2

  • Figure 2-3 Blood pressure and plasma colloid osmotic forces in normal and inflamed microcirculation. A, Normal hydrostatic pressure (red arrows) is about 32 mm Hg at the arterial end of a capillary bed and 12 mm Hg at the venous end; the mean colloid osmotic pressure of tissues is approximately 25 mm Hg (green arrows), which is equal to the mean capillary pressure. Although fluid tends to leave the precapillary arteriole, it is returned in equal amounts via the postcapillary venule, so that the net flow (black arrows) in or out is zero. B, Acute inflammation. Arteriole pressure is increased to 50 mm Hg, the mean capillary pressure is increased because of arteriolar dilation, and the venous pressure increases to approximately 30 mm Hg. At the same time, osmotic pressure is reduced (averaging 20 mm Hg) because of protein leakage across the venule. The net result is an excess of extravasated fluid.

    =32 " , =12 " .

    =25 " . . =0. , 50 " ,

    , 30 " . 20 " . , .

    ":"

    : , , , , P , .

    )15 30 .( Immediate transient response .

    , 20 60 , . ,

    .

  • . < -

    :5-2

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    " , amaG-FI, FNT, 1-LI .

    . 42, , 6-4,

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  • :" 4-2

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    .

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    .1-2

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    lailehtodnE eluceloM

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    1-LGSP

    muilehtodne detavitca ot noisehda ,gnilloR X siweL-lylaiS nitceles-E

  • TABLE 2-1 -- Endothelial/Leukocyte Adhesion Molecules Endothelial Molecule

    Leukocyte Receptor Major Role

    (neutrophils, monocytes, T cells)

    ICAM-1 CD11/CD18 (integrins)

    Adhesion, arrest, transmigration (all leukocytes)

    (LFA-1, Mac-1)

    VCAM-1 41 (VLA4) (integrins)

    Adhesion (eosinophils, monocytes, lymphocytes)

    47 (LPAM-1)

    GlyCam-1 L-selection Lymphocyte homing to high endothelial venules

    CD31 (PECAM) CD31 Leukocyte migration through endothelium

    *ICAM-1, VCAM-1, and CD31 belong to the immunoglobulin family of proteins; PSGL-1, P-selectin glycoprotein ligand 1.

    Figure 2-7 Regulation of endothelial and leukocyte adhesion molecules. A, Redistribution of P-selectin. B, Cytokine activation of endothelium. C, Increased binding avidity of integrins

    : 2-7.

    A . , PAF P-Selectin )weibel palade bodies ( .

  • " , , .B . FNT 1-LI : 1-LI FNT , , . nitceleS-E 2-1

    . , , . "

    , 4-ALV 1-MACV : 1-LI FNT .1-caM 1-AFL 1-MACI

    . etaflus nirapeh , , . C

    . snacylgonimasocylg .

    . , , , ,

    , . .

    :sisedepaiD/noitargimsnarT

    ./ , . , ( )

    ( ) .13DC 1-MACEP .

    , enarbmem tnemesab , . . " ,

    1 " , . . 44DC

    ::

    ( 1 epyt ycneicifed noisehda etycokuueL )1DAL . 1-caM 1-AFL , 2

    nitceleS-E , .X SIWEL 2DAL (. )esarefsnart lysocuF

    .

    : :

    .: 42-6 .: 84-42 : , .

    (.E P ) 84 42 , .

    .

  • eht fo evitatneserper era shpargorcimotohp ehT .yrujni etuca gniwollof stneve fo ecneuqes cigolotsih dna citamehcS 8-2 erugiF dna amede fo scitenik ehT .muidracoym detcrafni fo )thgir( setartlifni ralullec )raelcunonom( retal dna )tfel( )cilihportuen( ylrae yradnoces hguohtla ,esnopser tneisnart etuca na sa nwohs si amede ,yticilpmis fo ekas roF .snoitamixorppa era noitartlifni ralullec .rucco osla nac noitartlifni lihportuen dna amede deyaled fo sevaw

    :

    2 - sanomoduesP , - . 4

    . , - . , ytivitisnesrepyh -

    = , .

    , .

    . suonegoxe suonegodne -lymrof-N .

    . . eninoihtem . a5C , . :

    .4B , . .8-LI , , .

    . ( 7 )nietorp-G

    (.3 ) , K3IP , CLP ( PI) K3IP CLP

    . 24cdc/ohR/caR sesaPTG " . sesaPTG

    .

  • .liat gniliart a dna )tfel reppu( muidopolif a gniwohs erutluc ni etycokuel gnivom a fo hpargorcim nortcele gninnacS 9-2 erugiF

    .

    , . gniknil-ssorc

    , , nilosleg, .

    , , , , . =

    , " .2A CKP

    : " . 2A " .tsruB evitadixO - .

    . , , ,

    . ,

    : , LLOT lloT- srotpeceR ekiL(RLT)

    , 01 . .

    , SPL RLT . " ANR, GpC

    secnatsbus ladiciborcim .

    . RCPG .- 7

  • . , , , eninoihtem-lymrof-N : .BTL, E , FAP: , a5C

    , , eninoihteM-lymroF-N

    . . PDG nietorP-G ,

    , , PTG PDG , nietorP-G . CKP , PI CLP

    .tsrub yrotaripser secnatsbus laidiciborcim

    .

    T ", ETANNI KN " , . . EVITPADA

    .

    . , ,

    : . GgI .1

    . cFIR " , 3C .2

    ( ) (. )

    . 1RC

    .3C , nitcenirbif ,)LBM( nitceL gnidniB esonnaM .3

    " nietorp evitcaeR-C, negonirbif .

    .LBM q1C .negonirbif

  • ehT .ilumits tnereffid ezingocer setycokuel fo srotpecer ecafrus llec fo sessalc tnereffiD .noitavitca etycokueL 01-2 erugiF .)sliated rof txet ees( detciped era srotpecer emos ylnO .setycokuel eht fo snoitcnuf eht etaidem taht sesnopser etaitini srotpecer

    " : 3- .

    " .1 ( )

    , : 2 "

    , srotpeceR esonnaM , .

    . enimasotcalag-lyteca-N .

    LDL srotpeceR regnevacS . LDL

    .LDL . ( 81dC/b11dC )1-caM *

    , " .LBM

    , tnemflugne . - tnemflugnE.2

    ( sdopoduesp) , . "

    .- . ,

    . . ,

  • sisotycodne-detaidem-rotpeceR .

    . . 3

    , , . )sIOR tnuhs etahpsohponom-esoxeh "

    (. HPDAN , esadixO HPDAN " IOR

    . -2O - . 7 esadixO HPDAN

    , . , ,

    IOR . (11-2 B ) ON . ,

    .

    . " 2O2H , , "

    OPM. OPM LCOH .

    )noitanegolah , . ( .

    edilaH-OPM-2O2H (. ) OPM

    . HO, . )esadixO enoihtatulG / eslataC "

    !(.1

    . ", , IPB = nietorp gnisaercnI ytilibaemreP ladiciretcaB

    , .

    enimaoculg-lyteca-N-dica-cimarum .

    . , , nietorP cisaB rojaM

    . , , grA , , snisnefeD

    (. ) . , ,

    , 5-4 Hp , .

  • eht no srotpecer ot b3C dna cF fo gnidnib dna tnemhcatta sevlovni )muiretcab ,.g.e( elcitrap a fo sisotycogahP ,A 11-2 erugiF selcitrap detsegni fo noitcurtsed yb dewollof ,seloucav citycogahp htiw semososyl fo noisuf dna ,tnemflugne ,enarbmem etycokuel ,B .seussit ralullecartxe otni desaeler eb yam stnetnoc elunarg ,sisotycogahp gnirud taht etoN .semososylogahp eht nihtiw .selcisev citycogahp nihtiw setaidemretni negyxo evitcaer ladiciborcim fo noitcudorP

    laiciborcim ,

    : - IOR - . -

    ! .

    :

  • yrujnI decudnI-etycokueL fo selpmaxE lacinilC -- 2-2 ELBAT cinorhC etucA

    sitirhtrA emordnys ssertsid yrotaripser etucA

    amhtsA noitcejer tnalpsnart etucA

    sisorelcsorehtA amhtsA

    esaesid gnul cinorhC sitirhpenoluremolG

    noitcejer cinorhC yrujni noisufrepeR

    kcohs citpeS

    sitilucsaV

    .

    : " . = gnideeF gniruD noitatigrugeR - = sisotycogahP detartsurF -

    , , , .

    , = esaeleR cixototyC - . slatsyrC etarU

    .( ) -

    . " ,

    : , ,

    1DAL: .

    , 2DAL .

    emordnyS ihsagiH-kaidehC: .

    , , ( ) , . doolB "

    . sraemS . T

  • esaesiD suotamolunarG cinorhC: ladiciborcim .

    . esadixO HPDAN dekniL-X

    (.xohp19pg) 2

    (.xohp76p xohp74p) . , :

    , .

    snoitcnuF etycokueL ni stcefeD -- 3-2 ELBAT

    tcefeD esaesiD

    citeneG

    snirgetni 81DC/11DC fo niahc 1 ycneicifed noisehda etycokueL

    fo sisehtnys rof deriuqer esarefsnart lysocuF 2 ycneicifed noisehda etycokueL )nitceles rof rotpecer( edirahccasogilo detalylais

    tsrub evitadixo desaerceD esaesid suotamolunarg cinorhC

    )tnenopmoc enarbmem( esadixo HPDAN deknil-X

    )stnenopmoc cimsalpotyc( esadixo HPDAN evissecer lamosotuA

    metsys 2O 2H-OPM tnesbA ycneicifed esadixorepoleyM

    gnikcod enarbmem ellenagro ni devlovni nietorP emordnys ihsagiH-kaidhC noisuf dna

    deriuqcA

    ,sispes ,ycnangilam ,setebaid ,yrujni lamrehT seicneicifedonummi

    sixatomehC

    noisehdA sutillem setebaid ,sisylaidomeH

    ,setebaid ,sispes ,aimena ,aimekueL noitirtunlam ,setanoen

    ytivitca ladiciborcim dna sisotycogahP

    :

    . , , ' , ( 1-LI ,FNT ) , ,

    .

    . .

    , , .

    . , , :

    . - , - . " ateB-FGT

  • . FNT ( ") ) "

    (. ,

    :

    ( sninik, ) . 1 . "

    ( ) . ( , ) -

    . ,/, , : . ( , , )

    " . 2 . , sniniK,

    . . 3 (.ON IOR) ( )

    . " . 4 .

    . , . 5

    . ) . - , . 6

    ( ninikydarb esaninik) " ( ) ( " " " )"

    (. . . 7

    .sllec lailehtodne ,CE .noitammalfni fo srotaidem lacimehC 21-2 erugiF

  • senima evitcaosaV

    . .

    . .

    : ., , .1 (.6 ) .2 .a5C a3C snixotalyhpanA .3 . .4 (.P ecnatbuS) .5 .8-LI,1-LI .6

    ! . esahp tneisnart etaidemmI

    . .1H "

    htiw deniatS .eussit laititsretni eht ni dna slessev doolb dnuora sllec tsam gniwohs mutnemo fo daerps talf A 31-2 erugiF taf htiw deniats selubolg taf era serutcurts der ehT .)elprup ro eulb krad( selunarg llec tsam eht yfitnedi ot niats citamorhcatem ).AM ,retsecroW ,loohcS lacideM sttesuhcassaM fo ytisrevinU ,onjaM .G .rD fo ysetruoC( .niats

    (5-enimatpyrtyxordyh ). .

    . , niffamorhcoretne , , PDA, ,

    . - , . EgI FAP "

    . . FAP

  • .

    . 02 . EVITPADA ETANNI

    , , .

    . 9C-1C .

    3 3C :

    4C 2C , ( GgI MgI) 1C .esatrevnoC 3C b2b4C

    , (SPL, ' ) " b3C " 3C . ,

    . esatrevnoC 3C bBb3C . bB B ( )

    . .1C : 3C esatrevnoC 3C 3

    ./ b3C a3C :

    esatrevnoC 5C (esatrevnoC 3C ) b3C 9C . 9C-6C b5C, a5C 5C

    , CAM . !rorrE

  • : , CAM "

    C4a C5a C3a: anemonehP ralucaV .1

    )snixotalyhpanA . a5C(. sixalyhpana

    .

    , a5C : . 2 . ,

    , , ( )b3Ci b3C: . 3 .

    " 5C 3C

    . . etaduxE , :

    ...

    !rorrE

    ot sdael syawhtap tnereffid yb tnemelpmoc fo noitavitcA .metsys tnemelpmoc eht fo snoitcnuf dna noitavitca ehT 41-2 erugiF tnemelpmoc rehto dna 3C fo stcudorp nwodkaerb yb detaidem era metsys tnemelpmoc eht fo snoitcnuf ehT .3C fo egavaelc

    ni debircsed era tnemelpmoc fo noitaluger dna noitavitca eht ni spets ehT .)CAM( xelpmoc kcatta enarbmem eht yb dna ,snietorp . 2-2 xoB

  • , "

    . , :

    : esatrevnoC 5C esatrevnoC 3C .I b3C FAD

    " , : " .1C ( HNI1C )rotibihnI 1C

    fo rotibihnI enarbmeM 95DC CAM .sisyl evitcaeR

    : . 3C . , susotamehtyrE supuL cimetsyS , 4C 2C

    .- CAM

    .airessieN , : , airunibolgomeH lanrutcoN lamsyxoraP

    . - 95DC FAD , PI

    . .

    amedE citoruenoignA yratidereH HNI1C , . " ,

    niniK 2C = 2C . 2C 4C 1C , nierkillak HN1C, .

    . 21

    .

    ", snegoniniK evitcaosaV . snierkillaK

    , ninikydarB . . ,

    :

    "( 21 )namegaH .1 .enarbmem tnemesab

    (.a21 rotcaf / rotavitca nierkillakerp )21 .2 .nierkillak nierkillakerp .3

  • ( 1 )namegaH . 5C . . .

    .a5C

    negoninik nierkillak .4 -( 1 )21 /

    .ninikydarb

    " ninikydarb gnitrevnoc nisnetoigna " . esaninik (. ECA )emyzne

    ( 21 ) namegaH " enarbmem tnemesab

    21 (. ) .a21

    : 2 .

    , , . ( a2 ) .

    .4 "( 2 ) .

    ))RAP( srotpeceR detavitcA esaetorP RCPG (. ,

    . , ,

    1-RAP , , :

    nitceleS P . 2 ON FAP

    " 21a metsys citylorbif / .

    . , : anemonehP ralucsaV

    , , " rotavitcA negonimsalP . ,

    stcudorP tilpS nirbiF .

    .3C 3C

  • namegaH .

    : , a5C, a3C,

    . a5C .

    (. ) .1: a5C a3C

    . . 2 . nierkillaK, nimsalP. 3

    : 4 , a21 = rotcaF namegaH

    .sninik evitcaosav niniK . rotcaf namegaH " nierkillak ,

    . . X , , . . metsys citylonirbiF . . .

    . " . " ,

  • Figure 2-15 Interrelationships between the four plasma mediator systems triggered by activation of factor XII (Hageman factor). Note that thrombin induces inflammation by binding to protease-activated receptors (principally PAR-1) on platelets, endothelium, smooth muscle cells, and other cells.

  • (: )86-68 , snibboR

    setilobatem dica cinodihcarA :snidnalgatsorp ,snixopil dna seneirtokuel , " , "

    . , - -

    " , - sdiocotua .

    . . , 02 , - . -( AA) : ,

    .(a5C) , , (1

    +2aC )- (2 (.

    - - (3 .

    : AA- (4

    ) " - sdionasocie o snietorp-G (.

    - , .

    . ( 2-XOC DIASN)

    (. ) sesanegyxopil o

    ) 2-XOC-( ) 1-XOC - yawhtap esanegyxoolcyc : . AA- (

    , ciseglarepyh : - 2EGP o ,

    . .-

    .- , tsam - 2DGP o .- - 2FGP o esatehtnys nilcycatsorp - ( nilcycatsorp) 2IGP o

    , - 2IGP .1FGP , 2IGP .

    enaxobmorht -( enaxobmorht) 2AxT o : - 2AxT , esatehtnys

  • 2BxT- .- .

    .

    1-XOC- , "" 2-XOC, , ) ,

    . (

    3- - yawhtap esanegyxopil -5 - ( OL-5) esanegyxopil-5: .

    . - ETEH

    - ) 1TLsyC- " .

    . . 2TLsyC-( 1

    - 4BTL o .

    - - ( ) 4ETL- 4DTL, 4CTL o ) - ,

    , - , (.

    ) - AA- - snixopil, , , :(

    . .

    esanegyxopil-21 " -( 4B- 4A )4BXL, 4AXL o . 4ATL

    ( ) : , .

    .

    : . AA- - snivloser , . "

    .nivloser- ,

  • :

    TABLE 2-4 - Inflammatory Actions of Eicosanoids Action Metabolite Vasoconstriction Thromboxane A2 , leukotrienes C4 , D4 ,

    E4

    Vasodilation PGI2 , PGE1 , PGE2 , PGD2

    Increased vascular permeability Leukotrienes C4 , D4 , E4

    Chemotaxis, leukocyte adhesion Leukotriene B4 , HETE, lipoxins

  • :syawhtap dionasocie- -

    . - . ( gnitalyteca) - o 2-XOC- , - srotibihni 2-XOC o

    1-XOC- .

    .srotibihni

    . nicahtemodni , (sDIASN) - o . , sDIASN- OL-5 -

    . o (.2TLsyC- 1TLsyC) o

    :sdiocitrococulg - srotibihni murtceps-daorB - , noitaluger-nwod o

    ON )SONi-( FNT, 1-LI) - , 2A , 2-XOC

    (.esahtnys

    , 1 nitrocopil - noitaluger-pu o . " AA-

    . - . " "

    teletalp- rotcaf gnitavitca(FAP)

    . - FAP

    . EgI " , .

    B . , A . . -: .-

    FAP gnitavitcani ' " . nietorp-G FAP

    : FAP- . sesalordyhlyteca

    . / , , (tsam- ) , : -

    . .- .- 0001-001 ) , -:

    (.

  • (. ") . .- .tsrub evitadixo . " sdionasocie

    FAP : (. )

    senikomehc dna senikotyc , " , "

    . . . .

    . " . : 1-LI- FNT

    FNT- FNT FNT : ) T " nixotohpmyl . " 1-LI (.nixotohpmyl

    , , , - " . :

    ( noitavitca lailehtodne) - sdionasocie, FG, , ,

    gnimirp, , , ON- .

    . . , ( 6-LI )snoitcaer esahp-etuca

    , , evaw-wols , ,

    , noisnetopyh(: FNT )kcohs citpes niportocitroc

    FNT. Hp- , . "

    aixerona- , aixehcac- FNT .

  • senikomehc ( - 01-8)

    syc- . 02- 04 . :

    ) -( senikomehc : ) CXC , " , 8-LI: , (

    .FNT- 1-LI-

    etyconom . syc- - ( senikomehc : ) CC nietorp yrotammalfni esahporcam, nixatoe, (1-PCM )nietorp tnatcarttaomehc

    (. deterces dna desserpxe llec T lamron dna detaluger )SETNAR-( 1-PIM )1. , , ,

    . nixatoe ,

    : , syc- -( senikomehc : ) C . . nitcatohpmyl

    : , eniklatcarf - C3XC " -

  • " , - ; T .

    , . nietorp-G : .

    , 1-VIH- - srotpecer-oc- 5-RCC- 4-RCXC .

    :

    .

    .

    .MCE- "

    ON- , , )

    " - , (.

    (.rotcaf gnixaler devired-lailehtodne: ) .

    ( , ) -

    , - , PMG " .

    3 (.esahtnys edixo cirtin )SON " eninigra-L- ON :

  • -( )SONn, ( )SONe , ,

    .ON

    " -( elbicudni )SONi .( -NFI, FNT ) - ON: ON

    , ,

    . tsam

    . selunev yrallipactsop- "gnillor"- ON-

    . ON

    - , sisorelcsorehta- ON

    .noisnetrepyh

    : . , ON

    .- ON- (1

    - ON (2 .

    . ON (3

    (. ) SONi (4

    . , ON ,

    setycokuel fo stneutitsnoc lamososyl . .

    :

    , ', , , -( yradnoces) .- , - ,

    , , - ( yramirp ) , ) , (, )

    (.3 , , G . -

    , - ( ) .

    :

    Hp- , - - .

  • tnemesab, : - - . , , , enarbmem

    snixotalyhpana , 5C- 3C . - . - o - - , , , o

    . , ,

    " .

    - nispyrtitna-1: , esaetorpitna . nilubolgorcam-2

    (.ycneicifed nispyrtitna-1- )

    negyxo-lacidar eerf devireds, -

    .

    .HPDAN

    . ( HO) ( 2O2H) , (OS) -

    - . ON

    , (8-LI ) , . . "

    - . .

    :

  • . - : . o- , noitadixo enihtnax o

    .

    . nispyrtitna-1a - esaetorpitna .MCE-

    (., ) - :

    . - nimsalpolurec . - gnirrefsnart . - esatumsid edixorepus .- - - esalatac .- - - esadixorep enoihtatulg

    . "

    seditpeporuen

    , sdionasocie- senima evitcaosav- ,

    A ninikoruen- P ecnatsbus .

    .SNP- SNC- , seditpeporuen ninikyhcat-

    P ecnatsbus. ' P ecnatsbus " , ,

    . , -

    .

    srotaidem rehto , , ,

    . :

    . , - (1

    , 1 rotcaf decudni-aixopyh " . , FGEV

  • . - (2

    , " - dica ciru : . . -

    - ( tuog) .( )

    noitammalfni etuca fo srotaidem lacimehc fo yrammus (. )

    , . ,

    .oviv ni

    .ON- , , , - , (a5C- a3C )snixotalyhpana,

    .P ecnatsbus- FAP, E- D, C ,

    .( 4BTL) , a5C . , , - etuca - FNT- 1-LI

    .stnatcaer esahp

    . . - ON

    :

    noitammalfnI etucA fo srotaideM fo yrammuS - 5-2 ELBAT noitcA

    ecruoS rotaideM ralucsaV rehtO sixatomehC egakaeL

    dna enimatsiH ninotores

    ,sllec tsaM steletalp

    - +

    niaP - + etartsbus amsalP ninikydarB

    nietorp amsalP a3C revil aiv

    tnemgarf cinospO - + )b3C(

    ,noisehda etycokueL + + segahporcaM a5C noitavitca

    morf ,sllec tsaM snidnalgatsorP enarbmem sdipilohpsohp

    etaitnetoP rehto srotaidem

    ,niap ,noitalidosaV - revef

  • TABLE 2-5 - Summary of Mediators of Acute Inflammation Action

    Mediator Source Vascular Leakage Chemotaxis Other

    Leukotriene B4

    Leukocytes - + Leukocyte adhesion, activation

    Leukotriene C4 , D4 , E4

    Leukocytes, mast cells

    + - Bronchoconstriction, vasoconstriction

    Oxygen metabolites

    Leukocytes + - Endothelial damage, tissue damage

    PAF Leukocytes, mast cells

    + + Bronchoconstriction, leukocyte priming

    IL-1 and TNF Macrophages, other

    - + Acute-phase reactions, endothelial activation

    Chemokines Leukocytes, others

    - + Leukocyte activation

    Nitric oxide Macrophages, endothelium

    + + Vasodilation, cytotoxicity

    TABLE 2-6 - Role of Mediators in Different Reactions of Inflammation Vasodilation Prostaglandins

    Nitric oxide

    Histamine

    Increased vascular permeability Vasoactive amines

    C3a and C5a (through liberating amines)

    Bradykinin

    Leukotrienes C4 , D4 , E4

    PAF

    Substance P

    Chemotaxis, leukocyte recruitment and activation

    C5a

    Leukotriene B4

    Chemokines

    IL-1, TNF

    Bacterial products

    Fever IL-1, TNF

    Prostaglandins

  • snidnalgatsorP niaP

    ninikydarB

    lamososyl egahporcam dna lihportueN egamad eussiT semyzne

    setilobatem negyxO

    edixo cirtiN

    noitammalfni etuca fo semoctuo , .

    , . , . , ,

    : 3

    . - ( noituloser etelpmoc) (1 , ,

    , . , ,

    , , ( )

    scitahpmyl. (.)

    -( tnemecalper eussit evitcennoc yb gnilaeh) (2 ,

  • , aruelp) .

    , , ( muenotirep

    . noitazinagro . .

    (3, - o

    )*( : .

    , (ainomuenp) . )*( ,

    . (.)

    (. ) o

  • noitammalfni etuca fo snrettap cigolohprom, ,

    .

    noitammalfni suores

    (. noisuffe ) , .

    noitammalfni suonirbif , ,

    .- , ( )

    , segninem: . ( etaduxe) " .

    . " . -

    , ( noituloser) , .

    ( noitazinagro)

    )sdnarts . (

  • noitammalfni tnelurup ro evitaruppus ,

    . ,

    )cinegoyp ( iccocolyhpats )

    .siticidneppa etuca (. , ,

    . cinegoyp . :

    . o . o , o

    . (.: ) "

    sreclu , ,

    . noitareclu. :

    . , , o . , - o

    .

    . - NMP , ,

    .

  • noitammalfni etuca fo yrammus :

    , , .

    " , (. ) sregnassem dipil,

    " " .

    .

    . - , . ,

    " (: )

    - - . , "

    (. etaduxe) ,

    . snoitcnuj-

    . ( romut) ( rolac) , (robur)

    " ( ) - . ,

    " " . ,

    (.rolod) ,

    noitammalfni cinorhc , ( ) .

    .

  • , . ", -

    . sisolucrebut, sisorelcsorehta, sitirhtra diotamuehr

    noitammalfni cinorhc fo sesuac :

    , illicab elcrebut - . , ( )mudillap amenopert

    . ytivitisnesrepyh epyt deyaled

    (. )noitcaer suotamolunarg

    - ( ) - - - acilis etalucitrap o

    .sisocilis

    - sisorelcsorehta o . "

    . - - .susotamehtyre supul- sitirhtra diotamuehr:

    serutaef cigolohprom , ,

    : ,

    . , - . " - " - "

    . ( )

    noitartlifni llec raelcunonom .

    lailehtodneoluciter ) :

    eussit- , (. metsys

    , ( ) . segahporcam

    (. segahporcam raloevla) ( setycoitsih sunis) , .

    eussit- . egahporcam eussit ,

    , , " egahporcam .

  • 84 . , ) " .

    (. :" . -

    .KN- T ( -NFI ) . - .

    , , .

    , .

    ( / ) - : " .

    - . ( ) .

    :

    -PCM ) , o .ytivitisnesrepyh-deyaled (. 1

    .a5C o (.-FGT- devired teletalp - FGDP )FG o . o .- o

  • . -

    .seuqalp suotamorehta

    . " -

  • :

    (.) . .

    :

    - ( , ) (. )MCE

    (. , ) (.FG) ,

    . , , .

    : - , , "

    , citylonirbif

    . T . dica ciru . , ,

    ) , : ( A: )

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

    noitammalfni cinorhc ni sllec rehto

    - , - . ( ) o

    .

    ( 1-LI, FNT) o .

  • : , -

    srotalumits-oc T

    T . T , ( 21-LI)

    , B . , (-NFI ) .

    CPA,

    , , sisenegonagro diohpmyl . sretnec lanimreg

    .sitirhtra diotamuehr

    . EgI - .

    , nietorp cisab rojam . nixatoe , -

    .

    . - tsam

    (.IRcF )EgI cF- - tsam- EgI- - o

    . AA

  • . / , . , .

    . tsam- - o , -

    (. sitileymoetso ) " :

    . T .

    noitammalfni suotamolunarg ,

    - (.dioilehtipe) , (. ) ,

    amolunargohpmyl, esaesid hctarcs-tac, sisodiocras, (sisolucrebut) :

    sisoillyreb, citocym , silihpys, sisollecurb, ysorpel, elaniugni .

    , , - : (.

    . , , ,

    , , , - .

    dioilehtipe- - . " " ,

    . , ,

    sllec tnaig dioilehtipe .

    02, , (m 05-04 ) ) ( )

    . (. epyt-ydob ngierof

  • :

    foreign-body granuloma - " - , : , , "

    . epithelioid -giant cells ) , .(

    immune granuloma - , .

    .

    T , . , IL-2

    T -IFN- epithelioid -giant cells. . tubercle

    -. , . .

    :

    o " ) acid-fast .(

    o ) .( o ) .(

    -sarcoidosis.

    TABLE 2-7 -- Examples of Diseases with Granulomatous Inflammations Disease Cause Tissue Reaction Tuberculosis Mycobacterium

    tuberculosis Noncaseating tubercle (granuloma prototype): a focus of epithelioid cells, rimmed by fibroblasts, lymphocytes, histiocytes, occasional Langhans giant cell; caseating tubercle: central amorphous granular debris, loss of all cellular detail; acid-fast bacilli

    Leprosy Mycobacterium leprae

    Acid-fast bacilli in macrophages; non-caseating granulomas

    Syphilis Treponema pallidum

    Gumma: microscopic to grossly visible lesion, enclosing wall of histiocytes; plasma cell infiltrate; central cells are necrotic without loss of cellular outline

    Cat-scratch disease

    Gram-negative bacillus

    Rounded or stellate granuloma containing central granular debris and recognizable neutrophils; giant cells uncommon

  • noitammalfni ni scitahpmyl . -

    , .

    . , ( scitahpmyl)

    ) enarbmem tnemesab, snoitcnuj (.

    - . -

    , . - " snoitcnuj .

    . , . ,

    , (. sitinedahpmyl) ( sitignahpmyl) -

    - ( selcillof) aisalprepyh- .

    . aisalprepyh

    .sitinedahpmyl , evitcaer ,

    , . aimeretcab- , , .

    , sitigninem, sitidracodne . , segninem,

    .sitirhtra citpes-

    noitammalfni fo stceffe cimetsys

    cimetsys esnopser esahp etuca

    " (.SRIS )esnopser yrotammalfni

    . SPL

    :esnopser esahp etuca-

    . , 4 - " , negoryp

    :. -

    1-LI ( snegoryp suonegoxe) SPL

    ,(snegoryp suonegodne )FNT-

    , 2EGP , . AA

  • tniop-tes- , PMAc

    " , , sDIASN. . -

    , .

    . kcohs taeh-

    , , - snietorp esahp-etuca , (nietorp evitcaer-C) PRC: .

    " " (.AAS )nietorp A diolyma mures-

    (. )FNT- 1-LI-( )6-LI ,

    . AAS- PRC o . ,

    , A nietorpopilopa AAS, esnopser esahp-etuca o , .

    , .

    , (xuaeluor) o noitatnemides etycorhtyre) RSE .

    .SPL ( etar

    , snietorp esahp-etuca-

    :( AAS )

    . sisodiolyma- o laidracoym- PRC- o

    sisorelcsorehta . noitcrafni

    PRC )noitcrafni- - , (. ,

    .noitcrafni laidracoym-

    - " . , - 000,001-000,04- , 000,02-000,51

    snoitcaer diomekuel (. )= : (. )

    ) loop evreser citotimtsop- o - (.FNT- 1-LI

    (. )

  • , o . FSC .

    . - ailihportuen

    - spmum, sisoelcunonom suoitcefni ) .

    ( selsaem namreG

    , revef yah, amhtsa laihcnorb) (. sisotycohpmyl )

    , ( snoitatsefni citisarap

    , eaisttekcir, , revef diohpyt) . ailihponisoe

    ( , aozotorp

    (. ainepocuel)

    . o , , o

    .

    (.srogir) o . ( sllihc) o . o . o . ( esialam) o

    SPL- ( sispes) - sispes .1-LI- FNT ,

    FNT .

    : (. CID ) -

    . , "( FT )rotcaf eussit FNT- SPL o " - o

    . niludomobmorht-( IPFT )rotcaf eussit ,

    . sisenegoenoculg-

    " ON ,

    - CID . ,

    . kcohc citptes -

    SPL . -

  • ) , . (

    ( SDRA) emordbys ssertsid yrotaripser tluda - o . , "

    . . o

    noitammalfni evissecxe ro evitcefed fo secneuqesnoc :

    . / - o. ytinummi etanni-

    , .

    - - - o . -

    , :

    , . - , sisorelcsorehta ,

    ".rellik tnelis" , . ,

    .MCE ,

  • 5

    , ( SPM) (. )

    - , . ecnatsbus dnuorg- .

    etaflus narapeh ,etaflus natamred SPM- . etaflus nitiordnohc dna ,etaflus natarek

    , .

    , . ,

    . 7 1- , SPM SPM- .

    . deknil-x , , - ( -1- 1 SPM )

    , . . -

    , , SPM, aenroc- " ", . , ,

    . .

    , :

    . ( ) , , , , ,

    . , ,

    , " . - ,

    . , evitisop-SAP, , .SNC- SPM ,

    , - " , , ( ) . kcip-nnamein

    - - - . SPM-

    - . , - .

    , relruh . , 7

    . - 1- , H 1 SPM , . SPM

    , . 42-6 6 " . SPM

    , 2 SPM , . - , 01 aenroc- , (deknil-x ) relruh-

    .

  • ()

    . ,

    . ,

    , - , .

    . , (. 71-5 )

    " -6- , -1- - 6- , (.)

    (. - PDU) - 000,01 , ( 001 )

    " . - 4,1- , .

    4- - 1- . ,

    , . emyzne gnihcnarbed - , . "

    . "

  • Figure 5-17 Pathways of glycogen metabolism. Asterisks mark the enzyme deficiencies associated

    with glycogen storage diseases. Roman numerals indicate the type of glycogen storage disease associated with the given enzyme deficiency. Types V and VI result from deficiencies of muscle and

    liver phosphorylases, respectively. (Modified from Hers H, et al: Glycogen storage diseases. In Scriver CR, et al [eds]: The Metabolic Basis of Inherited Disease, 6th ed. New York, McGraw-Hill, 1989, p.

    425.)

    , -12 , .

    , . , - :

    : , . ,

  • . , , , - 6- (. 81-5 )

    ( 1 , ekreig nov ) (.7-5 ) -

    , emyzne gnihcnarbed- " .

    . , , , (: )

    . , ,

    ( ) : . - ) ( kfp) , (5 ' )

    . ( 7 ,

    .

    .selcsum lateleks dna revil eht ni msilobatem negocylg lamron fo amehcs deifilpmiS ,poT 81-5 erugiF

    .msilobatem negocylg ni devlovni semyzne citapeh fo ycneicifed detirehni na fo stceffE ,elddiM lateleks ni negocylg ezilobatem taht semyzne eht ni ycneicifed citeneg a fo secneuqesnoC ,mottoB

    .selcsum

  • - ) (

    - branching enzyme . .

    , ) 2 , ( ,

    ) .( 4) ' ( , , ,

    .

    Figure 5-19 Pompe disease (glycogen storage disease type II). A, Normal myocardium with

    abundant eosinophilic cytoplasm. B, Patient with Pompe disease (same magnification) showing the myocardial fibers full of glycogen seen as clear spaces. (Courtesy of Dr. Trace Worrell,

    Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX.)

    - .

    TABLE 5-7 -- Principal Subgroups of Glycogenoses Clinicopathologic

    Category Specific TypeEnzyme

    Deficiency Morphologic

    Changes Clinical Features

    Hepatomegalyintracytoplasmic accumulations of

    glycogen and small amounts of

    lipid; intranuclear

    glycogen

    Hepatic Type Hepatorenalvon Gierke

    disease (type I)

    Glucose-6-phosphatase

    Renomegalyintracytoplasmic accumulations of

    glycogen in cortical tubular epithelial cells

    In untreated patients: failure

    to thrive, stunted growth,

    hepatomegaly, and renomegaly.

    Hypoglycemia due to failure of

    glucose mobilization,

    often leading to convulsions.

    Hyperlipidemia and

    hyperuricemia resulting from

    deranged glucose

  • TABLE 5-7 -- Principal Subgroups of Glycogenoses Clinicopathologic

    Category Specific TypeEnzyme

    Deficiency Morphologic

    Changes Clinical Features metabolism;

    many patients develop gout and skin xanthomas.

    Bleeding tendency due to

    platelet dysfunction.

    With treatment most survive and

    develop late complications,

    e.g., hepatic adenomas

    Myopathic Type McArdle syndrome

    (type V)

    Muscle phosphorylase

    Skeletal muscle only

    accumulations of glycogen

    predominant in subsarcolemmal

    location

    Painful cramps associated with

    strenuous exercise.

    Myoglobinuria occurs in 50% of

    cases. Onset in adulthood (>20

    years). Muscular exercise fails to

    raise lactate level in venous blood.

    Serum creatine kinase always

    elevated. Compatible with normal longevity

    Mild hepatomegaly

    ballooning of lysosomes with

    glycogen, creating lacy cytoplasmic

    pattern

    Miscellaneous Types

    Generalized glycogenosisPompe disease

    (type II)

    Lysosomal glucosidase

    (acid maltase)

    Cardiomegalyglycogen within

    sarcoplasm as well as

    membrane-

    Massive cardiomegaly,

    muscle hypotonia, and

    cardiorespiratory failure within 2 years. A milder adult form with

    only skeletal muscle

    involvement, presenting with

    chronic myopathy

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    ni segnahc ot traeh

    (sisonorhco)

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