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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
lyrdyhflus- gninosiop edirolhc cirucrem:
, , . PTA ' .
, esadixo emorhcotyc- - . .-noitalirohpsohp evitadixo
. .2
054P " . . RES-
" , " .
" :
-(4lCC )edirolhcartet nobrac . .
054P " ( 3lCC)
(. 054P)
- RES- . noitadixootua
dipil . sedixorep
sedixorep dipil -, .
yrujni llec revil decudni-4lCC: :
, - RES
. RER
yttaf- . (. 42-1 )gninosiop 4lCC fo revil
" .
RES- .
(. 32-1 )
- . - )lonelyT( nehponimatecA . , noitadinoroculg & noitaflus "
- . 054P " " , HSG . HSG HSG . -
. 5-3 ,
, " . "
)62.p( sisotpopA
"
. AND- .
. (.2-1 9-1 ) ,
.
sisotpopa fo sesuaC
". " " 2791 , ,
. , . AND- ,
. :
: . , , -
" htaed llec demmargorp" . .
. , : -
, .
, ailehtipe tpyrc- - .
etuca- - . . esnopser yrotammalfni
.FG . - - T "
. : :
: - AND- . . AND-
,
- .
- .
. - . "
.
: : ( ME- ) :
. , , : , . :
. 2- . gnibelb :seidob citotpopa- sbelb cimsalpotyc
.
: " ,
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. noitacificlac cihportsyD srotaitini.
selcisev xirtam , mn 002 .
: " . ( 1) . ( 2). ( 3) - ( 4)
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: . , = noitaduxE
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, sisatS .3 03 51 sisats .
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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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A siweL X siweL . = - . T , , :
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3 2
81DCa11DC 1AFL . , CPA ,
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. b3Ci ,
.
. 44DC etaflus nirapeh snietorpocylg ekil-nicuM .
.1-2
seluceloM noisehdA etycokueL/lailehtodnE -- 1-2 ELBAT
lailehtodnE eluceloM
etycokueL eloR rojaM rotpeceR
)setycohpmyl ,setyconom ,slihportuen( gnilloR X siweL-lylaiS nitceles-P
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
:
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(. ) . , ,
, 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 . -
, :
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. "
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.)
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.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
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.
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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