Effect of Deoxygenation in Sickle Cell Anemia - Department of

94 Pages·2000·0.4 MB·English

by  David G. Morio

AIMENA LLEC ELKCIS NI NOITANEGYXOED FO STCEFFE
yB
OIROM .G DIVAD
LOOHCS ETAUDARG EHT OT DETNESERP SISEHT A
TNEMLLIFLUF LAITRAP NI ADIROLF FO YTISREVINU EHT FO
FO EERGED EHT ROF STNEMERIUQER EHT FO
ECNEICS FO RETSAM
ADIROLF FO YTISREVINU
6991 TSUGUA
6991 thgirypoC
yb
OIROM .G DIVAD
STNEMGDELWONKCA
yaT-noS-narT regoR .rD ot edutitarg erecnis ym sserpxe ot hsiw I
knaht ot ekil dluow dna sraey owt eseht gnirud em ni tsurt gnivah rof
.snoitseggus ynam sih rof mih
gewzruK .H.U .rD dna okriH .J.R .rD ot seog osla noitaicerppa yM
.eettimmoc ym no gnivres rof
detbedni ma I ,krow siht fo noitelpmoc eht ni ecnatsissa rieht roF
,eH .J.Y .rD ,DhP ,ihcugoN .T.C .rD ,DM ,rethcehcS .N.A .rD ,nworB .R ot
sih dna ,DM ,grebnettoL .R .rD ot edutitarg ym dnetxe ot hsiw osla I .DM
.cinilc llec elkcis rieht morf selpmas doolb gnidivorp rof ffats
,setambal dna sdneirf ym knaht ot ekil dluow I ,yltnatropmi tsoM
sraey owt eseht gnikam rof ,uolihP esruoc fo dna siuL ,KCH ,laeN ,eunaM
.seiromem taerg ,bal eht ni tneps
htlaeH fo setutitsnI lanoitaN eht yb detroppus saw krow sihT
.06094 LH 1OR 7 tnarg hguorht
iii
STNETNOC FO ELBAT
STNEMGDELWONKCA ................................ ...................... iii
SELBAT FO TSIL ................................ ........................ iv
SERUGIF FO TSIL ................................ ...................... iiv
DNUORGKCAB DNA NOITCUDORTNI ................................ ............ 1
noitcudortnI .1.1 ................................ .................. 1
sevitcejbO .2.1 ................................ .................... 2
dnuorgkcaB .3.1 ................................ .................... 4
ecnacifingiS .4.1 ................................ ................. 21
SDOHTEM DNA SLAIRETAM ................................ ................. 51
noitatnemurtsnI .1.2 ................................ .............. 51
rebmahC retemoehrorciM .1.1.2 ................................ .. 51
noisrevnoC egatloV-ot-emiT .2.1.2 .............................. 81
noitisiuqcA ataD .3.1.2 ................................ ........ 12
negyxO dna negyxO ni erusserP laitraP fo slortnoC .4.1.2
noitarutaS ................................ ................... 42
73 ta stnemirepxE .2.2 o OP suoiraV dna C .......................... 72
2
dnuoS fo deepS .1.2.2 ................................ .......... 72
tnemerusaeM ytisocsiV etatS-ydaetS .2.2.2 ...................... 82
noitarbilaC .3.2 ................................ .................. 13
noitaraperP doolB .4.2 ................................ ............ 33
noitaraperP noituloS nibolgomeH .5.2 .............................. 43
noitcarF remyloP fo noitanimreteD .6.2 ............................ 53
YROEHT ................................ ................................ 73
dnuoS fo deepS .1.3 ................................ ............... 73
ytisocsiV etatS-ydaetS .2.3 ................................ ....... 93
STLUSER ................................ ............................... 24
sisylanA doolB .1.4 ................................ ............... 24
amsalP ni snoisnepsuS lleC doolB deR .2.4 ......................... 94
noituloS reffuB etahpsohP ni snoisnepsuS lleC doolB deR .3.4 ...... 35
amsalP ni snoituloS nibolgomeH .4.4 ............................... 75
NOISSUCSID ................................ ............................ 16
sisylanA doolB .1.5 ................................ ............... 16
ygoloehR lleC no noitanegyxoeD fo ydutS .2.5 ...................... 26
kroW rehtruF .3.5 ................................ ................. 56
SECNEREFER FO TSIL ................................ .................... 97
HCTEKS LACIHPARGOIB ................................ ................... 38
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SELBAT FO TSIL
elbaT egap
lleC doolB deR elkciS dna lamroN fo stnemerusaeM ytisocsiV :1.2
.snoisnepsuS ................................ ....................... 33
.stluseR siserohportcelE nibolgomeH :1.4 ............................. 34
.stluseR tnuoC doolB etelpmoC :2.4 ................................ ... 44
.yrammuS CHCM dna siserohportcelE nibolgomeH :3.4 .................... 74
iv
SERUGIF FO TSIL
erugiF egap
doolB deR )SS( elkciS dna )AA( lamroN rof sepahS lacipyT :1.1
.slleC ................................ .............................. 5
laitraP eht neewteb pihsnoitaleR - evruC noitaicossiD :2.1
.negyxO ni noitarutaS eht dna negyxO ni erusserP .................... 7
.citamehcS retemoehrorciM :1.2 ................................ ....... 61
.margaiD kcolB CVT deifilpmiS :2.2 ................................ ... 91
.puteS retrevnoC egatloV-ot-emiT :3.2 ................................ 02
.erusaeM CVT lacipyT :4.2 ................................ ............ 22
negyxO dna negyxO ni erusserP laitraP fo slortnoC :5.2
.noitarutaS ................................ ........................ 52
.edortcelE negyxO cihpargoraloP a rof evruC citsiretcarahC :6.2 ...... 72
.tnemirepxE llaB gnillaF a rof tuptuO lacipyT :7.2 ................... 03
fo noitcnuF a sa retaW dellitsiD ni dnuoS fo deepS :8.2
.erutarepmeT ................................ ....................... 23
lacirdnilyC )b( dna diulF dednuobnU )a( a ni llaB gnillaF :1.3
.ebuT ................................ .............................. 93
6 sronod dna )SA( 5 ot 1 sronod :seliforP noitcarF remyloP :1.4
.)SS( 01 ot ................................ ........................ 84
OP .sv ytisocsiV tnerappA :2.4 ,sronod SA )b( ,sronod AA )a( :
2
.sronod SS )c( dna ................................ ................. 05
OS .sv ytisocsiV tnerappA :3.4 ,sronod SA )b( ,sronod AA )a( :
2
.sronod SS )c( dna ................................ ................. 15
)b( ,sronod AA )a( :noitcarF remyloP .sv ytisocsiV tnerappA :4.4
.sronod SS )c( dna ,sronod SA ................................ ...... 25
OP .sv ytisocsiV tnerappA :5.4 ,sronod SA )b( ,sronod AA )a( :
2
.sronod SS )c( dna ................................ ................. 45
OS .sv ytisocsiV tnerappA :6.4 ,sronod SA )b( ,sronod AA )a( :
2
.sronod SS )c( dna ................................ ................. 55
iiv
)b( ,sronod AA )a( :noitcarF remyloP .sv ytisocsiV tnerappA :7.4
.sronod SS )c( dna ,sronod SA ................................ ...... 65
OP .sv ytisocsiV tnerappA :8.4 dna ,sronod )SA( ,sronod AA )a( :
2
.sronod )SS( ................................ ....................... 85
OS .sv ytisocsiV tnerappA :9.4 ,sronod SA )b( ,sronod AA )a( :
2
.sronod SS )c( dna ................................ ................. 95
)b( ,sronod AA )a( :noitcarF remyloP .sv ytisocsiV tnerappA :01.4
.sronod SS )c( dna ,sronod SA ................................ ...... 06
noitcarF remyloP .sv ytisocsiV tnerappA :stiF evruC tseB :1.5
.amsalP ni bH )c( dna ,SBP ni CBR )b( ,amsalP ni CBR )a( ........... 66
:noitcarF remyloP .sv ytisocsiV tnerappA :tiF evruC tseB :1.5
.SBP dna amsalP ni CBR SA ,SS ................................ ...... 76
iiiv
loohcS etaudarG eht ot detneserP sisehT fo tcartsbA
eht fo tnemllifluF laitraP ni adirolF fo ytisrevinU eht fo
ecneicS fo retsaM fo eergeD eht rof stnemeriuqeR
AIMENA LLEC ELKCIS NI NOITANEGYXOED FO STCEFFE
yB
OIROM .G DIVAD
6991 ,tsuguA
yaT-noS-narT regoR :namriahC
gnireenignE dna ,scinahceM ,gnireenignE ecapsoreA :tnemtrapeD rojaM
ecneicS
lacigoloehr eht stceffa taht esaesid a si aimena llec elkciS
depoleved neeb sah retemoehrorcim llab A .sllec doolb der fo seitreporp
llams yrev fo seitreporp lacigoloehr eht erusaem ot yrotarobal siht ni
02( seititnauq m fo stceffe ehT .snoisnepsus llec doolb der elkcis fo )l
fo seitreporp lacigoloehr eht no "muirbiliuqe" ta noitanegyxoed ydaets
suogolotua ni tircotameh % 52 ta dednepsuser sllec doolb der elkcis
nibolgomeH .derusaem era noituloS reffuB etahpsohP ni dna amsalp
yb deniatbo ,amsalp suogolotua ni tircotameh % 52 ta snoitulos
enarbmem fo stceffe eht etarapes oT .deiduts osla era ,noitacinos
dna tiart elkcis ,remylop S nibolgomeh eht fo taht morf seitilamronba
laminim evah sllec doolb der tiart elkciS .desu era doolb llec elkcis
ytisned laminim a tibihxe noitalupop rieht dna seitilamronba enarbmem
.ytienegoreteh
laitrap fo noitcnuf a sa deziretcarahc era seitreporp lacigoloehR
oN .noitcarf remylop dna ,noitarutas negyxo ,negyxo ni erusserp
fo noitcnuf a sa dettolp si ytisocsiv nehw nees si noitalerroc tnerappa
xi
x
,dnah rehto eht nO .negyxo ni noitarutas ro negyxo ni erusserp laitrap
dna ytisocsiv noisnepsus llec neewteb dnuof si pihsnoitaler raenil a
tiart elkcis ni % 03 fo eulav lacitirc a ot pu noitcarf remylop
eulav lacitirc siht evobA .stneitap llec elkcis ni % 55 dna slaudividni
.nees si stneitap llec elkcis ni ytisocsiv ni esaercni prahs a ,% 55 fo
wohs dna edicnioc sllec doolb llec elkcis dna tiart elkcis rof atad ehT
remylop ot pu detaler ylraenil era noitcarf remylop dna ytisocsiv taht
.% 55 fo eulav noitcarf
dna gnidnatsrednu ruo otni thgisni wen sreffo yduts sihT
doolb der elkcis fo seitreporp lacigoloehr eht fo noitaziretcarahc
gnissessa rof retemarap doog a eb yam ytisocsiv taht dnuof si tI .sllec
eht gnitaulave rof dna aimena llec elkcis ni ytireves lacinilc eht
.stnega lacigolocamrahp fo stceffe
x