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860 Bryrpemnre 6oresmr no.(enr4cony: r 5 r. T. III. Onxororurs. IeMaronorn-fl. Krrannqecra.s 6uo-

xr,rMus. Bo:pacrra 6o.rresnra /flo!.pea. C. I.
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feOTAP-Melua,202l. 416 c. : w. (Cepux <,Bsfrpennne 6oresnra no,[snu4cony>).
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DOI:
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10.33029 /9704-6005-4-PAP
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-2020- r-4t6.
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ISBN 978-5-9704-6005-4 (r. III, pyc.)
ISBN 978-5 -9704-6006- I (o6u., pvc.)
ISBN 978-0 -7 020-7 028-0 (aHm.)
Pyxono4crno <,Bnyrpeuuue 6oresHu no.[enurcouy> flepexr4Jro yxe 22 [eper,r3.rlaHr,rs r,r Ao cr.x flop ocraercs
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EEK 54.1-2-3-4s81

This edition of Chapters 14-13] from Davidsonb hincipla and Prauice of Medicine, 23d Edition by Stuart H. Rdston,
Ian D. Penman, Mark W. J. Strachan, and Richard P. Hobson is published by arrangement with Elsevier Ltd.
.{aHxoe u3tanne, BKJIIoqaIoqee s ce6s raanu [4-131 opuruuaaruoro H3,uaHr,rrr Davidsonb Principles and Prauice
of Medicine,23rd Edition by Stuart H. Ralston, Ian D. Penman, Mark W. J. Strachan, and Richard P. Hobson,
orry6ruronaHo rro couracoBaHnro c Elsevier Ltd.
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@ 2018 Elsevier Ltd. All rights reserved

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coo6qurr
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co6pauo 6onee 1250 Bo[pocoB c iurbrepHarr,rBHbrMlr
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B KHlIre Bnympennue 6oaesuu no ,[seudcony ]I KH]Ire-
cryrHl,Iqe Macleod's C linical Examination (nu4epxan-
uefi yxe l4-e uslabrue), a ratore Pinciples and Practice
of Suryery (Teopun u nparcmuKa xupypzuu) (7 -e wspuue)
rroATBepxraer rrpaBr{lbHocrb raKoro noAxora. Harue
<,ceMericrso>> ny6rlrKarlur? nononnrJrocb r,r3.uaHr{eM
KHr{rr,r Dav idson's Essenlials of Medicine, srrnylllenuofi
rlo MHofoqI,IcJIeHHbIM npocr6au .Iurarereft B Bu,ue
<<KapMaHHoIi> Bepcufl ocHoBHoro Marepuruft KHI,ITII
Davidson's 100 Clinical Cases, a xoroporl co6pauu
crleHaplrr4, B3rrTbre Henocpe.ucTBeHHo r,r3 pa3AenoB
<,Ouucague KrII4HI,IqecKI{x npo6reu>; a raloKe KHI,IT}I
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cr,rcreMHoe onucaHlre Arl$$epeHuualrnori Ar,rarHo-
crvtKvr pa3JruqHbrx clIMnroMoB lr nporrBneHuri. Mu
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yro6Hbrx,rlns Bocnpr{ flrvrs. vt npeKpacHo rrJrJrrocrppr -
poBaHHbrx MaTeplrrlnoB.
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ropAocrr,r 3a npoAoJrxeHr,re cJraBHbrx rpa4lruuft cepa
CrgHru AespucoHa I{ co3AaHue 3HaMeHI,IToft KHuTLI
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10 . ABTOPbI
David H Dockrell MD, FRCPI, FRCPG, FACP
Professor of Infection Medicine, Medical Research
CouncifUniversity of Edinburgh Centre for
Inflammation Research, University of Edinburgh, UK
Emad El-Omar BSc(Hons), MD(Hons), FRCPE'
FRSE, FRACP
Professor of Medicine, St George and Sutherland
Clinical School, University of New South Wales,
Sydney, Australia
Marie Fallon MD, FRCP
St Columba's Hospice Chair of Palliative Medicine,
University of Edinburgh, UK
David R FitzPatrick MD, FMedSci
Professor, Medical Research Council Human
Genetics Unit, Institute of Genetics and Molecular
Medicine, University of Edinburgh, UK
Neil R Grubb MD, FRCP
Consultant in Cardiology, Royal Inflrmary
of Edinburgh; Honorary Senior Lecturer
in Cardiovascular Sciences, University of
Edinburgh, UK
Sally H lbbotson BSc(Hons), MD' FRCPE
Professor of Photodermatology, University of
Dundee; Honorary Consultant Dermatologist and
Head of Photobiology Unit, Ninewells Hospital and
Medical School, Dundee, UK
JAlastair Innes BSc, PhD, FRCPE
Consultant, Respiratory Unit, Western General
Hospital, Edinburgh; Honorary Reader in Respiratory
Medicine, University of Edinburgh, UK
Sara J Jenks BSc(Hons), MRCP, FRCPath
Consultant in Metabolic Medicine, Department
of Clinical Biochemistry, Royal Infirmary of
Edinburgh, UK
Sarah LJohnston FCRP, FRCPath
Consultant Immunologist, Department of
Immunology and Immunogenetics, North Bristol
NHS Trust, Bristol, UK
David EIJones MA, BM, PhD, FRCP
Professor of Liver [mmunology, Institute of Cellular
Medicine, Newcastle University, Newcastle upon
Tyne; Consultant Hepatologist, Freeman Hospital,
Newcastle upon Tyne, UK
Peter l.anghorne PhD, FRCPG, FRCPI
Professorof Stroke Care, Institute of Cardiovascular
and Medical Sciences, University of Glasgow, UK
Stephen M Lawrie MD(Hons), FRCPsych'
FRCPE(Hon)
Professor of Psychiatry, University of Edinburgh, UK
John Paul Leach MD, FRCPG' FRCPE
Consultant Neurologist, Institute of Neuroscience'
Queen Elizabeth University Hospital, Glasgow; Head
of Undergraduate Medicine and Honorary Associate
Clinical Professor, University of Glasgow, UK
Gary Maartens MBChB, FCP(SA)' MMed
Professor of Clinical Pharmacology, University of
Cape Town, SouthAfrica
I
Lucy MackilloP MA(Oxon), FRCP
I
Consultant Obstetric Physician, Oxford University
I
Hospitals NHS Foundation Trust, Oxford; Honorary
(
Senior Clinical Lecturer, Nuffield Department (
of Obstetrics and Gynaecology, University of
I
Oxford, UK
Michael J MacMahon FRCA, FICM' EDIC
I
Consultant in Anaesthesia and Intensive Care,
I
Victoria Hospital,-KirkcaldY, UK I
Rebecca Mann BMedSci, MRCP, FRCPCh
Consultant Paediatrician, Taunton and Somerset
NHS Foundation Trust, Taunton, UK
Lynn M Manson MD, FRCPE, FRCPath
Consultant Haematologist, Scottish National Blood
Transfusion Service, Department of Transfusion
Medicine, Royal Inflrmary of Edinburgh' UK
Sara E Marshall FRCP, FRCPath, PhD
Professor of Clinical Immunology, Medical Research
lnstitute, UniversitY of Dundee, UK
Amanda Mather MBBS, FRACP, PhD
Consultant Nephrologist, Department of Renal
Medicine, Royal North Shore Hospital, Sydney;
Conjoint Senior Lecturer, Faculty of Medicine,
University of SYdneY, Australia
Simon RJ Maxwell BSc, MD, PhD, FRCP, FRCPE'
tr'BPhS, FHEA
Professor of Student Learning Clinical
-
Pharmacology and Prescribing, and Medical
Director, UK Prescribing Safety Assessment, Clinical
Pharmacology Unit, University of Edinburgh, UK
I OaviOAMcAllister MSc, MD, MRCP, MFPH
Wellcome Trust Intermediate Clinical Fellow and Beit
Fellow, Senior Clinical Lecturer in Epidemiology,
and Honorary Consultant in Public Health Medicine,
University of Glasgow, UK
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'12 . ABTOPbI

Simon HLThomas MD, FRCP, FRCPE JulianWhite MB, BS, MD, FACTM
Professor of Clinical Pharmacology and Therapeutics, Head of Toxinology, Women's and Children's
Medical Toxicology Centre, Newcastle University, Hospital, North Adelaide; Professor, University of
Newcastle upon Tyne, UK Adelaide, Australia
Henry G Watson MD, FRCPE, FRCPath John PHWilding DM, FRCP
Consultant Haematologist, Aberdeen Royal Infirmary; Professor of Medicine, Obesity and Endocrinology,
Honorary Professor of Medicine, University of University of Liverpool, UK
Aberdeen, UK
Miles D Wtham PhD, FRCPE
Clinical Reader in Ageing and Health, University of
Dundee, UK

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14 O MEXAYHAPOAHbII4 PEAAKqI'IOHHHff COBET

Saroj Jayasinghe MedhaYRao

Chair Professor of Medicine, Faculty of Medicine,
University of Colombo; Honorary Consultant
Physician, National Hospital of Sri Lanka, Colombo,
Sri Lanka
AL Kakrani
Professor and Head, Department of Medicine, Dr DY
Patil Medical College, Hospital and Research Centre;
Dean, Faculty of Medicine, Dr DY Patil Vidyapeeth
Deemed University, Pimpri, Pune, India
Yasantha Kamath
Senior Professor, Department of Internal Medicine'
MVJ Medical College and Research Hospital,
Bengaluru, Karnataka, India
Professor of Internal Medicine, Principal and Dean,
MS Ramaiah Medical College, Bangalore, India
NRRau
Consultant Physician, Anueraha Medical Centre
and Adarsha Hospital, Udupi, Karnataka; Former
Professor and Head, Department of Medicine,
Kasturba Medical College, Manipal University,
Kamataka, India
Nirmalendu Sarkar
Professor and Head (Retired), Department of
Medicine, Institute of Post Graduate Medical
Education and Research and SSKM Hospital,
Kolkata,India

Piotr Kuna KR Sethuraman

Vice - Chancellor, Sri Balaj i Vidyapeeth University, I.
Professor of Medicine; Chairman, 2nd Department of
Medicine; Head of Division of Internal Medicine, Pondicherry, India P
Asthma and Allergy, Medical University of A
Surendra K Sharma
Lodz, Poland Adjunct Professor, Department of Molecular
n
Medicine, Jamia Hamdard Institute of Molecular o
Pravin Manga
Medicine, Hamdard University, Delhi; n
Emeritus Professor of Medicine, Department of
Director of Reseaiich and Adjunct Professor, F
Internal Medicine, University of Witwatersrand,
Departments of General Medicine and Pulmonary A
Johannesburg, South Africa
Medicine, JNMC, Datta Meghe Institute of Medical n
Ammar F Mubaidin Sciences, Sawangi (M), Wardha Maharashtra, lndia o
Professor of Neurology, Jordan University Hospital,
Ibrahim Sherif T.
Khalidi Medical Center, Amman, Jordan
Emeritus Professor of Medicine, Tripoli University; I
Milind Nadkar Consultant Endocrinologist, Alafia Clinic, F
Professor of Medicine and Chief of Rheumatology and Tripoli, Libya B
Emergency Medicine, Seth GS Medical College and n
Ian J Simpson
KEM Hospital, Mumbai, India o
Emeritus Professor of Medicine, Faculty of Medical
Matthew Ng and Health Sciences, University of Auckland, New n
Honorary Clinical Professor, University of Hong Zealand F
Kong, Tung Wah HosPital, Hong Kong A
SG Siva Chidambaram
Moffat Nyirenda Professor of Medicine, Dean/SPL Officer, n
Professor of Medicine (Global Non-Communicable Perambalur Government Medical College and GHQ, o
Diseases), London School of Hygiene and Tropical Perambalur, India
n
Medicine, UK; Honorary Professor of Research,
Arvind KYaish R
College of Medicine, University of Malawi
Professor and Head, Department of Medicine, Hind A
Tommy Olsson Institute of Medical Sciences, Lucknow; Ex-Professor n
Professor of Medicine, Department of Medicine, and Head of Medicine, KG Medical University, o
Ume6 University HosPital, Sweden Lucknow, India
n
Ami Prakashvir Parikh Josanne Yassallo R
Consultant Physician, Sheth VS General Hospital, Professor of Medicine, Faculty of Medicine HI
Ellisbridge, Ahmedabad; Professor of Medicine and and Surgery, University of Malta; Consultant n
Head of Department of Medicine, NHL Municipal Endocrinologist, Division of Endocrinology, Mater o
Medical College, Ahmedabad, India Dei Hospital, Msida, Malta
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lge o6uapyxuBaerc, B HopMe Onyxonu

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xrq [ruxonporeunoeurfi ropuou, cy6teguruqa o uaccori Xoprorapquroua (100%), nyaurprurfi sanoc

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Ars BeAeHI,ITI Kaxroro [aqlleHTa. Oro rraoxer 6trrr I . Cassidy J., Bissett D., Spence R.A.J. et al. Oxford handbook
oco6enno BEDKHo npu o6cyxgeHLILI nalrueHToB,c peA- of oncolggy, 4th edn. Oxford: Oxford University Press;
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n rpaftue croxnofi KJIrIHlIqecKoir, curyaguu. 2. Dark G.G. Oncolo1y at a glance. Chichester: Wiley-
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3. Hanahan D., Weinberg R.,al The hallmarks of cancen the
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. rrr60p floAxoAqlqero [epBl,IqHoro MeroAa reqeHl'I-fl 4. Tobias J., Hochhauser D. Cancer and its management, 7th
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(American Cancer Society): KluHlrrlecKl{e pexoMeHra-
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oronuredeQQu urcoxxagoco eH)cehr.rNoJEHV xBI^todtrHI,Ic xlqgelrog xl,D{cehr{Jororeu udu oH 'IqII
'nlalenod4eu I,InrIHdocHeJ BEl^toBg?tredeu'suog rtar. 'IHo
-nsefu erDrreh rorenr,r Hr^rorer^rdetr rletrorr xnsodors A
'exdx Bsusoeru?)olr ]r rEHel(QQu[ srog rpHqrudaficus 'EsdeH BuHexEdou ercen ruHeretreduo nftr irJBsoeqffou
Norefr g 'tD{I,IIro>I )E) xI,DIBr '4r.Ilrefn(luo xIiIHiIL?defI -cu oH)KoIlt olt 'nror€mdetr lrru orudo.tuddel, ot,(ggare[
89 r QUO9
64 . SOJIb H nArlrIHATt'tBHAC nOMOUlb
flpu ncrpeue c 6olenun crl'IMyJIoM aKTI'IBauI{'I
crrrrHHono Mo3ra. Te.no xlertor Heo6xoAm"ro A'n-f,
reHepauull floreHuua-
Heirporpanc- HOUI,IUenropoB npI',IBOAIIT K
BbDlorBanrrfl nefipoHa, o6pasonanra-a BBepx K
ra Aeficrst,rs, rotopufi pacnpocrpaHfrercfl'
MrrrrepoB u QYnroruu neftPonon; TAHUIIIO' A TAKXE CTLIMYNUPYCT
CNI'IHHOMO3TOBOMY
. r"p"riro ootoKHa (arccoua) u nepuQepuaecKw Heps' oKa3blBalolul{x
sHcso6oxAeHue nefiporpaHcMl'ITTepoB'
,il o*orroruil, pacr'olaraloxlllxcfl na nepuQepuu ,roprrro" sosAeftcrnue Ha oKpyxarcuue nefiponrr'
u corepxaullx pflr peqelrropoB n neftpoual*roft
ueu6pane;
. cne$u(uusupoSaHHbt pe4enmopos na nepu$epnu',
I Gnnxxofi uosr
[3Becr- Cencopnue ueftpoHu [ocpeAcrBoM IreHrpzulbHblx
cocToslu[x u3 roJIbIX HepBHbIX OKOHqaHLTII' Broporo
6oresue peueflropbl' oronqaHufi o6pasyrot cuHarlc c Hefipouaul'I
HbD( KaK HoIIuIIe[Topbr, r4Jrll cn[HHoro Mo3ra' 3Aecr npo-
MeAl4aropa- 3aAHI'lx
noprl,[Ka B porax
KOTOpbIe aKrI',IBlIpyIoTCS paul}IqHblMl',I
lrcxoAur 3HaqI'ITerbHzuI MoAyr-fluraq 6oresrx
crI'IMyJIoB
B 9nI4-
un. Onn pacfloJlaralorcfl rIpeI'IMyIIIecrBeHHo KaK JloKalruux ueftpoHoB B crIIlHHoM
notr AeftcrsrleM
AepMI,Ice; I'I3 roJIoB-
K 3aA- Mo3re, TaK I{ HI'tcxoA,IIqux nyreft nefipoHoB
. qeHmpalbHozo oKo\qaHufl, KOTOpoe [oAxoAI',IT Ha sToM
nepnrrft uen- Horo Mo3ra' B o6pa6orre 6olenrrx curHzuloB
HeMy pory c[]IHHoro Mo3ra, o6pasyx
ypoBHe [pI,IHIrMaer yracrl{e psr HeftporpaHcMl'IrrepoB'
rpa.nrnuft cI'IHa[c c neftpouauu' nepeAalour]IMl{ aMrrHo-
arpaaaru*arxrrx s ra6.il' 2'2' KHI'IM orHocqr
6oresrre ouylueHl'Ir s rorosHofi Mogr'
curuaroB B cnfiHHoM Mo3re
peqenropbl' yi{acrByloque e o6pa6orxe 6onesttx
Ta6nuqa 2'2' HefiporpaHcMrrrepu u fl

iiln peqenropa KoI,reHraqx'n-

t'roryr 6unu Ca2-'
lzlouuuri xalan Bog6prqarou1rae; npoHt4qaeMble MR KaruoHoB:
AMPA
na- ralra K* e 3aaracnNlocru or crpyKrypbl cy6ueArsuq
lzlouuuti xaual
gor6yr(q"o,,lru; 6norrapyrorcc Mg2+ a cocroeHt4t4 noxoq; 6aoxa'qA
NMDA noreHqtaal]a;
tuoxer 6uru HapyueHa npra vrsureieHrar'r ueu6panuoro
Ca'*, Na. ra K-
npoHt4qaeMble Ann
eos6y)qaoque
KauHarnutfi lulonHutfi xaual fl ocrcraHanfla'lecKne -
r'rHrr'r6rapyot{ne 3a caer BblcBo0oMeHilfi
l-pynna I GPCR floecuHanrueecxrae -
FAMK; npoHr,rqaeMble Anr Na- ra K-
GPCR Arrrasr,rpyor pnA nyrefi nepegaqu chrHanoB; AonrocpoqHoe
l-pynna ll
Bn4tHue'Ha cuHaffruqecKylo eoe6ygnltocru
o6paeoaanue quKrlt4He-
GPCR BeooqrHo, ranna6upyror.rlae; Moryr cHrxarb
l-pynna lll npechHanrhqecKne yMeHburaor
.*Jto ua"rorrrurdio6ocqara;
astcao6ox4enue ffiYraMara
gnn Cl-; 6loxt'tpyo-rcn
lzlourutit xanan B oarorro, rasrr6rpyrol4rae; npoHt4qaeMble
GlyR
xoQeuxot',t
Anq ch
lz'louttutfi xaHan ;;;;;;, r,*rn,,6upyrorqrae B cnilHHoM Mo3re; npoHt4uaer"rure
rAMKA
;";;;;il;"Atnrpv'6rct ot*soAna3enr4HaMu (t,Y119lT.:]:3tlY
-ti',
ffiffi il#;i; vru.rtv"i B Hor'lt4Lienqr4r4' o6utqHo -oxaedea'r
yrr...out." gefi crene Ha AeFrenbHocrb
:lT.::"- ::::1*"""-,
GPCR

Heiponenrugu BocnaJleH[R' yMeHbueHue

Hetipornnunoeute GPCR B ocHoeHoi/ aoe6pxAaror.qrae; ychneHI4e
Beqecrao P Heilponarrvecxoi 6olt't
(cy6craHuraq P) ilccneAoBaHhe
Boi6)or<Aarorqrae; npoBoESTcn KrhHhqecKue
Xolequcroxuuuu CCKRS1 -8 GPCR
AHTATOHNCTOB
Beu'lecrBa P; yvacreyor
CALCRL GPCR Bos6fxqapulhe; 3aMepgor paopyuleHhe
l-eHerhqecru PogcreeuHutfi
KaJlbLlLlTOH14Hy nenTuA

Onuougst Bos6yxqaoque; uoryr 6unu npoHoLlhqenrhBHblMt/

OPl (x) GPCR
,QnuopSrn lz'lirrra6upytotLlue
oP3 (P) GPCR
0-0nAoPSun tzlnrra6rapyroqrae; raKxe sKcnpecc'pyuc""Vttrtutt'ltt,lKneTKaMl,1
ORL-1 GPCR
HoLluqenrun
yMeHbueHue 6onu'
' Bos6yx.qalou{re - ycuneHne 6onra; ranrra6rapyou$ae - Kl4cnora; NMDA N-rrrerun-D-acnaprar;
[lpruevaxrae. AMpA - oit - -
Hoqt4qenruHoButfi (onuouAonoAo6xutfi)
CALCRL peqenrop, no4o6xuri peqenropy *-"q;;;;;;;'dp":5*6,4'"rl
"-"rrio:l;irAfo*criS-rer,rn-4-rasoxcaeonnpon]4oHoBafl
peqenrop.-
Jxarrunox BrqHdorue[ed eedeh rfalcgeE'lelru.rdrg
'xurtgzfu xrllHHe{rcexoHr r urfi xrunedouo xrsHdoJ,g
-ou 'oxEd udu 'urrrrxeQnu 4oxcanunodx xrrgouc,( g
rcsresodusunodx u slesoduccedrodu raru (voudau
gnHnoulrudouoercou guunud s'dar{uduuH) godrc
-rsg u godrco quqg rexox eug 'rflturc gosolrog
reero s €JEoN oJoHHrruc aHgod,(
4rqsr,rhlroJc,( en
EH lrwrtroxcuodu .r,exolr e)K)?r rl,rfIB0I.IJr,IcHaJ
a uh eeu tucHoc 6e Hq u ed n efi
'Joor r 4oHHr,r[c s sorBHJuJ xrlserog
une[edau otunerrucz( x ru[osudu orh 'trcregmsHond
aoHod4eH msndouroc nntIesuDre rodou'rc,lorrrredxoc
rsr,{.wrlJ,c qrog eurflorBsrqorqs ]trcg 'JooI^[ goHHI,Iuc s
ulrrcHodgen unrsHdocHec rcrorctredeu erqdolox' vttsl.:l
-{e[ Hrer,rtrHelou r,oildudesel'ulredor€utran ]rwur€
ersxsfdusr,r.rxe 'soreHJuc nnetradau ta,ilg '(g'1 'cud)
uouod4on xnuruedaQQe xrsHnr,rsdeu xedoluenufioH
BH r{rlereH?) I,rnrqHHo}r u nlwdorualad c rcrorgsrq€rsc
elqdor,ox'HI,I€oHetrB u rqHr,rtrHerilBr,codu'eYodotros
IqHou'HltHrxvf,l"dg rehouDls'sodoruuvaw xrqHhlrrr
-eed rtounerotrrqs c geHBxJ I^to)oro u godrrruuex
orsrtonourrranodu goHHemrlsoIt rcroetrxogoduoc
elrHerBrrcog' er,rHarulcos rcreBrsr 4rlHrorcoc xure
xocs rrtr I,II,IYre0I.{LvrcIJec Nor [oI,IH€xor{ ,i{I,Iftlgo lrusH
-qrmHr,r6'aunedrrxo u osrcqreJemews eoxcehr,rJd(drx
'lqltg€dl'rtaneaaroge€ eMHqlreJl{Imucog'xed'cucuec
B?hoIIDIa'xrl,IHtrorcoc xtDIcehI{HI,IDt xlsHnl,tlreud udu
qJex{Hoos rexow wrfieer{lucHoc BuxcehrrdeQudel
6un eeutucHoc EevcehudeSudeg
'soxureJqlrBHe xlsHtrHouuo
urcoHsurxeQQe oruxe:ruHc r,rJJesudu r,oxolt o.r,n
) -UetrBo xsdo.tox g 'eJ€oH r roHso[oJ s d,ft)r(drc errd
'urog goxcenureuod4en udu rc,r,eBrsBtroll sodo,r,uoned
)flqHlruonuo-d rucceducxe'deru,ldue11'mrHeherr qJcoH
-eurxeQQe uH ren{rru oJh 'xrfl,rHrorcoc xrqse[og xrDrc
-er,nsodx udu rcroler'uueed xedorueled u xede.rrult
-uredlodgeu )oqHsoHco s BuHeHewel,t or,DrcenuQufieu3
'luertodffruc Nrirserog ltrrxcer,unodx x .t lrtrosrdu oJ,It
'soul(r^ruJ,c xrqHsur[ofrunon exlogedgo pr uulrtaducos
s xtatflor.(srceM '4erlfu lrmHoHor r0rr wrsHnodcor[otrx
urcasudu rexow qrcoHhurceruodgeH xrfi Irefruc xnd
-oJo)eH g'orqrcoHhrtrrremrodgan rcleesrseuH ccerrodu
&tlqHHor,r[srx€YE rore 'goHexr eHHeDredsou uH rasJ,o
s oHHegoco 'urog x rcr,oildur,uere ofucrrg ?r^rerclrc
renadeH r€HerefuHeIt u xel'ruxcshr,rdeQudeu xex o.ll,
'rcreuul'3 'soruHJr,rc xrqseuog exrogedgo udu r1ur'av
-sr xrqHsoHco 0u r'rl4H[o B)L}I,IIfIB rIrIIE0IlIIucHo3
ulhe8t.lll,tcHec
99 . 9UO9
' orsrog
Hroxcehr,rreuod4eu c (
sorHer,Ifreu,( Al,rHssotreffccpr xl{)rcehl,rHurnr urrd neuQ
eriIHiIII?hBH teHotregodu axr( orranorsuduun r$^uonnetr
ou u 'rsdoluutren ersHHE[ €H xl,Ifiotr(src{etr 'trI,IHeheu
sotroJer I xHsoH rrxlogedeed rr?urrHalou .Lee.Lced
rr[
-tos socrefiodu xr,r.le wIH€r rr,rHorr rusemruftrf edelt
oII 'urrog euJ,rprducoa €H qrrllrs oHirrreruh€He lfuolt
etsdolox'renrul,firl xex xrx?l' goder"luncuudrod4ex
xutnorelilrfgeos rBsx?o 4rsur,edgo qrrHer r€lr ex]IBr E
'Iqgrxol,ltft elqHilroJl,IlrBucosodu qrrlretrHs kt XYIz,IB
-orcot xHserog xrxcenuuodx udu rcsJ.egodnsulxe
rfrow nnrrodxr,rnr rr)Jerx u xer 'Iqrufiodrce xey
'sor€HJuc xrirseIrog exrogudgo s )oroLDI xIqHIIIIBI,IITJ
urod x csde.r,nu leer,cedeog rsvoJ euHtreucou g 'emlqs
qJ,nlg
roxon urrog go)cer,unodx orn,r,ugeed x llrcoH
-Hoxo[olcedtredu fiur xndoroxen,t runexoltdo.t
oJerflrtroxcun xnrsod,{ a 4unureed BE-cI,I oJh'Nor o
errHexoro[Yedu elrxel qcoresrqoe)crqg' soruHJr,rc
xrirsorog uxrogudgo 4onrredlnefi lnreuxem,{deH
'urcehro adplt 4ex4edx ou 'rqHeserqg sl,ng r,fuox
ruee,lredourE,r reHHesodurrftrHr,rtrHor,ruo u qtrog rBH
-neaoeulrcdeHoJ ru)cehxsodx otn'rcleu.lur,3
'4erauaxedfieed xlrxcanude@udeu ro rscslrr(xxu
raemsHaru,t rBdolox 'Breor{ oJoHgouoJ €rosrc rqHodoJS
oc wrHexondoJ oJorfirtroxcr,IH qHeuerc rennera[aduo
rcreutrorrrgeH elrdon g'nxudfrx(drc nnrsso>rdoxtrou
ulrlttildtr,(trxon ge€rsconuges ilJac ol{mtcorc o)IDIBT
e '(rufirrfuad r€rfltrEoxcun) ereolt oJoHsoroJ 4odox
Ar,rHelflgooc xflrnBroxs ol]firrtr,(trolr renourxs
udrdus
ol{Hne[geduusoseed ro{onr{ rslufuzltoduraH"{.trodudu
IiIITEHJLIS' (1' <,tt1199 1qnUfu3,t[OdUOH> 3UH3s0
7' tWd'XrC)
-eH JlrcoH eoHHorclrhodauamrss atg 'rqueuc€ erIH
-sr?Houilohte lt olqHsruugtox'elqgdorHec IiIHBsosJc
-
roasrc4retronr,reos rorrr,r[s uttog autytttdrroos BH sco[€ tf
'ec,t{erBJ, s pxtrrdou o.rodor,g ulteHodgeH c McuEHI,Ic
I
r,olhedgo ersdor.ox 's)Irrdou oJesJedl uwunodgeu r(dox -
olftrdocnec s rcJorrr.ruBrco['eJoor{ noHHI,Iuc s Extrrd ,
-ou orodors urrenodgon orsnoes€trodou'rsIrBHJu3
s
-i
ltuog
(rxnccehodu) exrogedgo uBHquedrHeh
'rofu(srcrjrotr€os lrHo orsdorox eH
'umuuod4en c utrsc ro rucusee
- urrog eurmducos
roremsHan { vtfll vrrro Hr ror€suhuraa6' rsnuQdoffre u
' (apudad palolat aua? uluo\rcloc) Yuruar [Hr,rHoru[srBx
grsHHesrcEod rxcar,urenoJ r,r d osroelfies )B) eLDIeJ
'rqtrruueuod4ex I ltodoleutronr nulflorulDr,(geoa rc4utn
-orrffsr'rerr{er,(n u'uruBdoruutreltod4eu nltrsseovudor i:
I rceuffiorl,IrsB ')h[VJ vr HvtlTr4IlJ xa) el{)pr 'rqrofor,u
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\.) \
i'.1 |\,
ro'{ o
.-->i
I
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llepegarsnx Aroxucrut
o I
fl
. .............PzX
Narl .7 lrxr
L,\
Narl .8 Nar1.9

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\fffii7;,-c"u"n,oo",
#
:t&
ErlD l'-
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JT*,
Cruuyn

prac. 2,3. MexaHugr,irur nepu$epuvecxofi ceHcurr43aquu. A ceHcopxurfi HepB, oKaHqr4Baoqlfca Holll4qenropoM B Koxe. 5 -
-
nepneepurecxre Hoquqenropbt oKcnpeccnpyror pagn!4qHbre peqenropbt t4 t4oHHbte KaH€Irlbl, Koropble AeficrByor KaK MeAlaaropbl
2X
6onra.'Oira BKnpqaror HarputeBbte KaHanbt, yqacrByrou.lue B naroTeHe3e BpoxAeHHbtx 6oreeutx cl4HApoMoB; nypuHepruvecxuil
(p2X) peqenrop afleHo3r4HrpraQocQara; qneHbt HaAceMeilcrea iloHHbtx KaH€IIOB c rpaH3ilropHblM peqenropHblM noreHqu€InoM
(transient receptor potential rRP), Koropbte onpeEeneor r43MeHeHi,1r ocMoJlgIlbHocrl,l u reMneparypbl; KilcnoroqyBcrBurenbHble
-
t4oHHbte KaHaIbt, Koropbte onpeAenqtor r4oHbr BoAopoAa; peqenropbl, conprxeHHble c G-6enrou,
Koropble onpeAenflor 6paAu-
peqeniop HefiporpoQuvecxoil rnposnxxuHasst 'l (neurotrophic tyrosine kinase
Kr4H14H, npocramaHAuHbr u a,qeH0314Hrp14Qoc6ar; ra

1), xoropuril o6Hapyxueaer Saxrop pocra HepBoB (nerve growth factor). B aKruBaLlu, 3rt4x peqenropoB nilraHAaMil, l4oHaMh
reuneparypoi (>42"C) yBen[quBaer noreHLl4€Irtbt
-
geficreun, Koropble ycunuBaor 6oreeute chTHaflbl t4
rogopoia [H-] r,r auicoxoil
roiio'ra'o, nepraeepu,recxyro ceHcuiugaqran. C usrueHexrquu no: Bennett D.L., Woods C.G. Painful and painless channelopathies.
Bocnponseogurcfl c pa3peueHun Elsevier flhe Lancet Neurol 2014; 13: 587-599)

NMDA-peqenrop

NR1
Auuttoxucltotut
l-nuqnn (ra 4pyrue xeilpo-
rpaxcurarreput)

fAHfJl|,iM

d
Peryrnqrn
6oneeoro oreera

puc.2.4, MexaHusr,rur qenrparunoil ceHcilTr3aqut4. fl,nr nocrcunanril,{ecxofi aKrnBaqht4 peL{enropa N-raerun-D-acnaprara rpe-
6yercr Haauvre aM[HoKr4c4or rn14qnHa h rnyraMara, Koropbre cBf,3brBaprcg c cy6uegunrqalar,t NR1 u NR2 cooreercrBeHHo; oHt4
ytunuearcr 6oneeure cilTH31br Ha ypoBHe cnuHHoro uosra. HanporuB, r4oHbt MarHill 6roxnpynr aKruBaqurc AaHHoro peqenropa
 'IrcHJ-HEH14v!oU
e
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- EV - dV - - 64 u
(eoedex ercod -
EvBd BrltileEpd vcr4d frcHHomrc8ou soHod4oH oit4r-il8eBd edorxeQ doruahad 4tsu
' ucolrplc.to BEHHA8TCIi\I' :
eod[r/rHe ruog or/E]cl^c-Lo rer(ercaocouc leeeuuxureodrl (aV) O,t -xuQQeoxocrsa) LyAIN
eouod4er emreeed
ruog erercrlcro :dor-ve$ 4rsnnoriuudxcredl (aU Orf (ZttlUOd ZmAOd
uuoq enercrlcr6 LTEHBI frqHHo/l W) OU (tVaUU tyaUf
urhrHlQcuU ueHEuJ.el-oJoE'suog uer'fuyq r€Hev
frcHHoly' W) OU (e'l'eN) yprNCS
PvhHhomilv rvudoron euuemldes
'cJ-cogeuc ueHhomrql\'eodUrudeuu.r'uuog auercrr{cr6 ueHev XrcHHo14 W)OU (O'l'eH) yrrNCS
uubrxl$cuU rBHErlJ-ELoJos'quog
uenfux'euo9 spHqueneilcvodeu'utuueurenodrudg rreHpv {rcHHor4 W) OU k'l'eH) yOwCS
n4rlcoHe'eodUr,r.rour,r.r'uuog auercrlcrg LTEHBv rIrcHHo[4 GU Ort '(1't'BN) y6irCs
(sr,rHBsoUaucEH
HUUJ.OHeO exueg ruhrxi(6 uur) unheilyl (xoueg) xe1
nuog aurruducos eo axtllorehosro 'rqHo.l 'e'Z eht4ugel
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-go udu uwrqHsurewdoQxu s.lrsg .trfuoru endoro) I/tNI,DICehpIJSgel .(fXert neusrclIetronl{e€s NIiIHxorc .
'trI,rHzso[effcc]r erlrHqruJHalqfdJcHu u enndoredogur rsnenaonc,{go oHrrodes eeIrogI{EH utt og uutuudtt
V
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uudotcu ullHufloHco eH vll.rrsel.;x 6oHJBr4[ u 'rsl^rdoH uulrrr/(Iloll Eefflgo g'%05 o[ 0€ ro rerrs?rco3 I,Iro9 a
ro {rr,rHeHolD{ro JorrrsErls eH r}IHBsotrolrocl,I oIqHIIIIBT rlroHHesosurrudene.r Eoxcenlurodx sJcol^reftrerceH orh -
-saru(drcsu u arsHdorudogeu'srog rznHesoourpdoHaJ a
' rlr,.e€e)ou sorra Hor,rrg,{ ru xusotra y ) c LI' vylhrvte d
y
rexcehfiHodx n xodtrnr,rc goserog 4rsudunoured gnu orsHslrB,{trI{guvHkt u e[xceh]IHJ€ eIsHHegJ,caI[dc rSror
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-c)erruHo)'rxrrBunodgnQ xux xIrDIBr'sonodll{uc -er,eN,r,o rqufvwrJ,c erqserog eH 4atrolu uurrxued g
xrraerog xrxcanunodx esrcHlrmlrrog udu o)BHIO :
'eHrces€rr aH BHo urca 'unog fnunudu otlftrgoHco @
sJ,r,rreleduo rqgorh'trI,rHBgo[erccu elqHqlr?ilrelr(drc HrHPHr4vrdereu eDcehrreHeJ I
-r{I{ u eHHdo.r,edoger oHriw{troxgoeH ecs qJuHfourqs 'ruog .r,eeruruc.(
Iqgorh 'Irrlor g qrrorcoc Hexrotr olqlrog c urHoI,IfIBU ernqrog errte oJh 'uonodrleu xrqnnorr{xawodu xurn
rnrHroJcoc oxHarro a mm gnadetu'4tiueaeuogee edr
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sosanHoft 6onuo pe.{Ko BbIqBr.EIor narororuqecKple
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I Axanugu KDoBn
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crf{ae Moxer norpe6orarbcq psr aHarI'I3oB KpoBI'IAJLf,
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npr43HaKaMI,I HacJIe.IIcrBeHHoro HapyueHua o6pa6orrca
6oresrrx rIMnyJIbcoB (cu. ra6n. 2.3).
I KonuqecrBeHHoe ceHcopHoe
I recrupoeaxue
Arq AeranrHofi ouexru xpoHurlecKofi 6olu uo-
xeT rrcIIoJlb3oBarbcg KoJIlIqecTBeHHOe CeHCOpHOe
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6op uucrpyueHToB, npllMeHqeMrrfi n rctunrat{ecxoltr
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ArnoEunun l-unepalresun
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l-loeuruexxas hrlt4 cHhxeHHag TeMneparypHan t{yBcrBurerlbHocrb
recr[-
Pnc, 2.5. llopraruaxoe o6opygoeaxne Anq ceHcopHoro
poBaHilg y nocrenn 6onuxoro
npaKrr{Ke (puc. 2.5). Jlerxoe npI'IKocHoBeHI'Ie K Koxe
c noMolllblo KvIcTvl, TaMnoHa I4JrlI BaTHOTO UapI{Ka
ro3BorseT BbIflBI,ITb HapyIIreHI'Ifl BogrlprtffTvts' JIeTKO-
ro [p]rKocHoBeH]Ifl (atno4nsus'). Ouesxa peaKuLI[
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npoBepKI,I uappueHuft MexaHllqecKofi runepanresuu'
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u xonoAHbIMlI TepM iuIbHbIMI'I BZUII',IKaMI',I [O3BOJIfl eT
onpe.ueJll,Irb HapylreHl,Is re Mneparypnoft 'ryncrou -
reJrbHocrl{. CHa.Iara npoBepslor e.[oponufi flacroK
Koxll, qro6rr ycraHoBI'ITb HopMaJIbHoe oilIyueHl'Ie' a
noroM - nopilKeHHuft Yracrox.
I l4ccneAosaxm Hepexofi npoaoArirocrn
HccleAonaHux nepnuofi npoBoAlIMocrI'I Moryr
IICIIOJIb3OBATbCI, ilJIfl BbIfl BJIEHI'Iq }I KONIIIECTBEH-
soir oqesxu nopaxeHl,Iq KoHKperHbIx HepBoB, KaK
nepu$epuuecKux, TaK I'I IIeHTpilJIbHbIx, a rarcI(e.uJIs
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nefi ponarrvecxotl 6olu. OAnaro .uaHHbIe IlIccJIe.uoBa-
HHs He flo3Bor.fl Ior g$serrunno ouenusarr @yHKuurc
MEJIKI'IX HEPBHbIX BOJIOKOH.
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BroxaAy nepu@epuuecKoro HepBa c noMolllblo
(nanpuuep, I% pac-
BBe.qeHllfl MecrHoro aHecrerl'IKa
TBopa nlrAoKanHa) uoxHo ucnoJrb3oBarb c AI'IarHo-
cruqecxoft ueJIbIo lI AJII orBera Ha Bo[poc' BbI3BaH
nu 6oresoft cI,IH,4poM IropaxeHlleM onpeAeJleHHoro
HepBa IiIIu HepBHoro KopeIIrKa. B clylanx, Kor,[a Boc-
ufunu noxaarurrfi oreK cJlyxar npuvuHoft
n€uIeHI,Ie
6oru (nanpnMep, flpu cAzIEIIeHI,II'I HepBHoro xopeurxa),
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urIoKoKoprI{KoI{roB Moxer rroMoqb yl!{enrrunrr 6olr.
Eroxa.uy nepuQepuuecKoro HepBa raloKe Mo)IC{o uc-
noJrb3oBarb Anfl nporHo3l'IpoBaHlrs s$$exruonocru
6oree paArarzulbHblx Mero.uoB o6es6orl'Isauua (Ha-
nplrMep, a6nilus nepga) npu oHKonorualecKl'Ix 3a-
6orenaHu.flx.
I Cr,tcremb! oqeHKvt 6on4
,{rr loxalu3aul,Iu 6olu, ouenKI'I ee rflxecrll I4
Brufl:g.ufl Ha KarlecrBo XI'I3HI,I 6unu paspa6orasu
pa3nr,rqHble onpocHlIKI,I u IIrK€uIbI. Hau6olee pac-
npocrpaHeHHble I,I3 HI,Ix flepeilucJleHbl s ru6r. 2.4.
Jloxa;rusauus 6oresHx oIuyueHuft Moxer 6urr or-
MerreHa [au]IeHToM Ha cxeMar]IqecKoM uoo6paxeHuu
rera. An.f, oIreHKI,I Tfl)Kecrl{ 6olu ralore ucnonmylor
nep6aarnue, q[cJIoBbIe u noBeAeHqecKue IIrKtuIbI.
Cucreuu nusyansHoft oueHKI,I, ilcnonmylouue pa3-
'4mnernftno xrlserog <rrcoHs[cHorHl,I qrl{renro oxutr -edxqnnodcoJrrotr rrlHodo Drhor c rcJoreufyo ruruc,( -€
-oxgoeH 'lrsrr[ec fxxdelttrou u Anrdellou ol(Hsrer,rfioc .I(
ersHHehedrue 'ugeltedg oJoHr r ro€wr,rHro eHo nJoX
oJe 'erHeI,I[€u qJcorrH€0 'eqwec s senHesumKl,I ev]nvlJl 'orqrog utoxcer,unodx c sorHoulrB[ xocs I rcsu.Itrosodu I(
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u eI,IHBsIqIrExou' t tuseJ,cedBlr' oI,IHoI loHo' edr{Jedeulrlel 'sI,{odIfHHc oJosorog nrod,r,uox oulrorsrcalrl.l(co .
urfl,r NruHesoucoxudu luor.rerr ) qrcoHiluelusrcs,{hderr
irurerx egJceh?x el,IHomlqgou .
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leilHor,orolrec I urHeltflull e]IHehelrgog .
'BrHer{Ireu r,IH€I,tx osJtehE) vH Levuvs. oJocs amqrog
:BIHoI,ITIBU oI,IHoMgo .
qnog oHHer{vrP-)e) 'qJtrHcBrqs re1(trelrc aHoo UtoHtro s
: rcJorrrrsB orqrog goxcanunodx
r^[eh eerrog uno g uulre[J/tw]xor ud11' ulJog vnYrc€.ursxolr
c wHeufrcu Br{Hehorr r{r^[?hEf?o rurulflgo 'rquufinndu
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qrcoHqrorfixrotrod[ )€y'errodtrHuc oJosorog rqJ
-goeu oI{ 'IqHlarrudu $oHsoHco oo ro u{cus?€ orrhr{,tc
->Ioxcu aI,D{zI qrrnnor,( omrug etoHWEHB odogc ud1
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un remudu eH €Jorrox oJe €trJox r,rl.r,r ExtrLrox uxed ro
darut oHsutroH xuHHasJcvod 4rxeuIrg oJa EtrJox 'ooH
uH sJ.esodu.Ieed,(xoseed-o[ rexon eros[x s orqttog c 'mHeserogeego rrrtr rcoelnorl(godr'nreds
xosoreh' denuduell'ndolxe@ ersHd,fuslrrfi u erqHirrru sil,lledxoc lt BfiHerreu oJoHcoJHer [ExI,rEen urcoHsl,t.l
-ufioc e)Dr€J u 'uufroN€ 'runemregf oJe r,r BJHor,I[€[ -xeQQe exre[ro s shor{ou rexon soy[Hcoduo orslflor{
LIIafrxMQ erqHsr,ru,IHJo) ruhorrxs'olcxel,uox lroxod -ou c ilrog l,IJcoHsr,rcuau{n torr,ut?HrlE euneaoducxu6 o
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TEHqIIBI,TIOCOXUCIOUg'SOJHer,rIrB[ Br4nehOU sOJ,eJ,Sr1(e l( rcsJ,rHenudu l,[.rolr 'elrur ruuoxedrss orrHhurr
'logoJnf hrcildo.Jerey nl-uu ou taHehx p€.l.cehpy uyxeho yuscoduo
4urc4euode3 Og-Of ieorvHlu gg eu ewdo$ cevrBdv ge-JS :r4uog foycohr4reuod4ex ByHoho :clHeHuoueE o
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suog ucre,(enuerou aU.J 'qreeBy^ funeilheu rclnotuuoEoou 'ELreJ. Ey\oxC uuog uuheeuueroy
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r1r,lBoxilEU Bcl4og yoeoLroh oyquoyceH'reudelley -olreeHlrx uyxaho uuU ?uyrBI erarucdaeux[ euel;'11
eilHehrLreeAedu u ouugumrqneBd'srcouxeUeleeg :edrey\edBu hdr (oPcs
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rroHr,rxeHHoro HacTpoeHI,IJI HyXHO npoBecrt{ oueHKy
cy[uHJIaJIbHOro pucKa.
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H neKapcrBenurui aHaMHe3, BrITIcHI'ITb Harvqve
B npomnoM sloynorpe6 ler.uir rcuxoaKTI'IBHbIMI{
BeruecrBaMll u nro6rrx g[]r3oAoB $n:lrvecxoro v.ru
ncr4xoJroruFlecKoro laacvJrvrfl.. IJenecoo6pa3Ho raICKe
rlor,IHTepecoBaTbcrl MHeHI'IeM [aUr{eHTa o ToM, qTo
lpoBour,rpyer y Hero 6o:r u KaKoBbI ero oxl'ulaHus or
reqeHl4rl: 6eg nonulraHus [epc[eKTI,tB JIeqeHI{e MO-
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Crparerr.ua, Koropble MoxHo llclloJrb3oBarb 4.lts npe-
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ra6oparopHblx I,I IaHcrpyN{eHTzuIbHbIx raccre.uosaHlalu
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lloxnnute Boouoxnut HapyueHilfl rorunrueuofi
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lulcnonusoearu Bil3yajlbHbte uKaJlbl Mt oqeHKil l,lHTeHcuBHo-
ctu 6ont'
lzlcnorugoaaru QopvalreoeaHHylo oqeHKy xorHilrueHofi
Syrxqru.
lulcnorusosaru reeep6aluuyto oqeHKy.
Tlcnonusoearu Bu3yanbHbte uKanbl AnF 0qeHKil 14HreHcrlBHo-
ctv 6onu.
lzlcnorugosaru uKanbr Mt oqenxlt 6orieaoro noBeAeHuff
lzlcnoausoearu Sopr',laar,rsoaaxl{ylo oqeHKy xorHLnheHoft
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klcnorusoearu reeep6alunyo oqeHKy: Bblpa(eH[, nilqa,
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3-e uqu.; 2010 (Acute pain management: scientific evidence,
l. breathworks-mindfulness.org.uk. I,hnepxer-pecypc .Ers 3rd edn; 2010).
loMepxKrr o6yreur.ra oco3HaHxocrr{ a 6oprbe c 6o11110, 5. npcrc.org. Kpaxar @oprtla onpocHuKa AJrs o{enru 6oln
6oaegnrro I4nr.r crpeccoM. Mar-fi.uura.
2. cuh.org.uk /breathlessness. I,tnQopuaUm r pecypcrr r.r3 7. paintoolkit.org. HucrpyMeHrbr .ulrs caMorroMotu,r flpu
6olrnuqrr Keu6pr,r.uxcxoro yrrxBepc[rtTa no JrelreHlrro Jre.IeHLrLI 6or[.
oau[trs{. 8. palliativecareguidelines.scot.nhs.uk. Perylxpno o6nos-
3. hospiceuk.org. Peclpc 6purancrcu< xoc[ncoB. r.f,eMbre HayqHo o6ocnonaHnrre KllrHr.rqecK[e peKoMeH-
4. mdanderson.org. Kparrorft oupocHtrK oueHKlr BbrpalKex- Ila\fivl.
Hocrn 6olu (rparras Qopna). 9. palliativedrugs.com. flparruvecraa nHQoprvraqu.s o npe-
5. nhmrc.gov.au. Koruerr.ls aHecre3rroJroron u earylrrer naparax, ucnonb3yeMbrx B [[urJr[aruBHofi nonrorun.
uelrrtru*l 6olu Ancrparnm u Hosofi 3erarulrr,l (Australia 10. sign.ac.uk. PexorvreHraqnq SIGN 136
nrrecrori 6orr,r.
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unrn6upyror pea6cop6qvro r.arpus., cnoco6ctnylor Funoroneuuq frnepeoneuun
narpnirypesy npu us6rrrxe HarpI,Ir u o6reua.
Yseru.reHlae norpe6.uenna Harpns BbI3bIBaer rI,I-
,

Cuunrouur Xax4a. Orexr rogutxex.

nepBoJreMr,rrc, Koropzu floBbllrraer noqeuHylo rtep@y-
. foroeoxpyxeHrae Orex 6ptoLuHofi creHxr.
B noroxeHr4l4 croq. OgutLuxa
surc u CKO, a raloKe Ilo.(awuer nupa6orKy peHlIHa sa Cna6ocru
cqer yBenr.rqeH}tfl AocraBKu Harprlq B [JIoTHoe flsrHo.
flpusnaxu Oprocrarurecxar [leprSeprvecxile oreK/.
Oru npoqeccbl 3alycKaror nocJreAoBareJlbHocrb co- rl4noreH3ur. l-loeutLueHue qeHrpanbHoro
6uruft, nporlrBonoJroxHbrx [3MeHeHusM flpu ruIno- Taxnxapgun. BeHo3HoTo AaBneHrlt B
BOJreMr,ru, ycrur,rBarour{x 9KcKpeq}Ilo HaTpI{fl . Cyxocru eo pry. epeMHbrx BeHax.
CnnxeHnuri ryprop Kpennraqnn B rerKilx.
Koxr4. flleepalsrstil autnor.
CruxenneAilype3a. Acqrr.
llorepr vaccur rera. YeerileeHile Maccbr rera,
,Qerrputi, crynop Al- (ttnorga)
KorAa 6 ar,ar,c norpe6aenu-a rI BbIBeAeH[ fl. Harpus.
Hapyrlaercrr, nro6as teHreHIII4fl K II3MeHeHI{Io KoHueH-
KpoBI,I o6H.Ixo KoppeKr[py- Ta6nuqa 4.4. npu.rltrbr runoBoneMull
rpaig:,z,uHarpufl B rrJra3Me il
eTc.fl ocMoTr,rqecKHMr,r MexaHI{3MaM}I, peryJII{pyIoIqI,IMI,I Mexaxusu llpuuepu
soAHHft 6a-nanc. B pesyrrtare HapylIeHI,Ifl 6atrusca
laarpLrs. [porrBJrsrorcq, UIaBHrIM o6pasolr, ]I3MeHeH]I-
Hegocrarornoe HegocraroqHoenocrynrennecnnqeti,
norpe6nexue He4ocraroqHac 3aMecrilTerbHaq
qrvrr.r o6reN,ra BKX, rrro flpuBoAr,rr K rl,rnoBoJleMvfi[.wv HaTpvl, repanilt
rl{flrlepBoJleMuu, a He u3MeHeHusM]r KoHueHTpaIIulI
florepuxarpur Peora,4uapen,HasoracrpaJtbHae
Harpllfl B nJIa3Me KpoBr,r. Krunr,r.recrue npI,I3HaK[ qepe3 xeryAoqHo- acnilpaqrn, nappxHutfi ceuq
r,r3MeHeHlrr o6reua BKX nepevucJleHbl n ra6n. 4.3. xuurevxuri rpaxr
llorepuxarpur
I l'rnoeoneiim '{epe3 Koxy
lpesuepHoenoroorAelreHile,oxoril

fnnonoleuuc onpeAensercr KaK yMeHbIueHlIe llorepu xarpur Tepanrn AuyperuKaMu, rvt4HeparoKoprt4-

o6reua qupKynlrpyrorueft xponu. Hau6oree qacrhIMI,I qepe3 noqKu KoulAHaq HeAocraroqHocru, ry6yrouxrep-
npuqlrHaMlr flBJrflroTcfl rloTepq ruII,I ceKBecTpa\ug. Ha- crrlqilarbHoe ga6oleeanue noqex

rpuftco4epxarrlr{x x}r.qKocrefi uru ocrpar KpoBonore- Bryrperurn Henpoxogrlaocrb Kt4uJeqHnKa, nepilToHt4T,

p.fl, KaK noKa3aHo sra6r. 4.4. cexaecrpaqnR* naHKpearilT, pasuoexeune xaueti

Yueruuexue Ocrpar xpoaonorepn

llarorenea o6ueua xpoan
* f'lpuvuxa ucrolqeHr4e o6ueua qupKynupyrorlefi xpoeu, xorn
florepu Harpnft coAepxaqeft xuAxocru BbI3bIBaIor
o6u.1ee cogepxaHue Harphr 14 BoAbr B opraHu3Me Moxer
r43MeHeHr{rr r @rurrrpaqvu rrarpufl. noqKaM}I v aKr:at- 6uru ropua.nuHbrM unu noBbrueHHbrM.
 6Z_rpDrBU
Z,zEC
9*) LII
0 tgt
0 0
U/quown 'eur/(df u/quo,rn '-lC u/ctuoHn
ctaHatsess oioHHesr,rdilr{e
rlcTeuonur gg .lylquoy\y\ g' f-O'
1.-92
r,(c7suor,r[,] 0t l-go t lylcrror,ly! e-g' ! IldrEH
t(clu 9l'A-9r'Z lylut
J)r 0/ Puer {o3cen 3 Jy I BH arehcpd s
yosouehunucodeg{tcHhrtgg utcoxgedrog
xernuodrysue !r urcoxUlax g euleuHe.Jdo
nrcougadrou aHHseHuexe otqHsoHco .S.t ehnugel
-truornrercudx) eoxreg eulnexdetsoo eH 'rsdosJced
' uuneIrosorrul uutrxeddox prdu fi r{rsHuu.r,xeQQe aerog
r,ftfg r,r e[c^d NoJcr,rE(coc s rcsresuxde[,{
r,{tr(g '(anntruoruox) usod) rirrrEBIII] ]Drrrog ar.rfnux
-deroc 'rsdostced ou, 'qruyrrxo rqg ourqg ol{xo6
' Iqxeulrx H0(I,9o J,eBsr.Ihl4ues,{
u)cahr,rrxuQ r,ru€.(@Hlr eweilgo (91/g oroxo) <lcen
remqrogeH oxrllor rJox 'x()g Er^reir,go Br,rHehures.{
rntr BHsur)eQQ€ oerog eYli.dotff Et4dLeH edosrJ€d
(o.roxcanurouou€l,tQ) oJo)cohr,rHoroeu rue.(Qnu run
-HAgr,rdLfug 'SOH€JdO XIiII{)KUS OHHeHerrX Ulre,{@deu u
ruuefnedgoosod) rr,rHe[soHEJccos rffi dosrced rore
qrBso€qlrollcu Jorros€ou oH oJh'ugodx
Horlol{dHlI
-,hdun Ewotgo euHehurosf a (ewargo oJoHHo['esg
Lo 0t / t oHirrreJlrorlrrgudu) trerr>rr Eom<rrogaH rr.rcoHs
rseodJcxe[ ols yI olrHotres8 'doruuduull 'enel{nerdo
s Istros orr,rHoretredxcEd,(nronqreltdox oHirrEHor,rldol
-odu ur'tEl4Hurdo urcoxtrnx xeJHenr.rdeulto) s r3qrHretr
-educed retr(g eHo or 'e>tuegula'yudtes r,rH Jr.rxdotroc
eH sroo)trux r{[cA '1'y 'cud eH r.rHrqHHorseJ,ctredu
'urrrufiuefrHo) HersorS.{go [r,rNorosouur 4onnexed
-Iqs lruHehorr rldu edoeJ,ced oJoHHasr,rdJ-{Hu dogng
'L',V'UqEL fl OHECITIIO X€)
'xrqHHE[ xrsxdoredoggrr oJe il BrHer,rfrerr €dJor.,{co sor
-u.r,su,{eed
ro Il{or.rse€ KuHotress nrcodoxc u edosrced
dogng '9't 'uger s godosrced xsnnesufu,ft{s rc)ortr
-
-orrnalrlrdu oxodum xrsdoroxeH sercoc e'g'V'vget
s rqHEeE)ou 8)OSOreh oJolrcodos.( xerurodrxelre lr
HrJoxlrax a urcougefuo[ arqHhoilc auntred3 .oN€HH
-e:do s ulrJdeH€ soc?rruo €r r€ilrogerux o:ondensadh
prnarnedsrotrad[ rrrtr srceffles xrirHqrerertrt n
4ud
-orzx aunarufuco[ ooHhoJerrotr qu.rhettcogo
or{HroxgoeH (4auf' xrlu<ruo)ceH eerog) I,IuHEtroIroJ
-.-. UHdIVH8YICOSIIOJ
(.eremdey doerce6) [reorcu uradrer +
Uudorx gildreH + Uudoux gilhquey + Uudourx rnue>r]
reI 0 0 t,ttsHxot c doerced erelyel, uudreg
,91 0 0 eUhdoux uudrex (yo6,g) dosrced lucehrHoroey
0 002 r09 (.eeoxoru-.1) peodrcroU %g
'+pN xr,rdouBx BeoxotUJ-O doerce6
cut sodosrced xuycar,nxor.oeu xndor.oxoH sercog 'g.9 ehraugel
I
I^loH<Ilrorurtr udg 'ot<rcox[llx c ercens el,IHotress
1- $1uBX
eoHqreu,rHrouo[ xrl rcrefgefu 'soJulrofuxere nderou
err)c ehuJonoreu rcou[noruxrotrodll wIu Lkr'fluq eA
9r-9e eUog Hergrrqs \u)g' w\rcdeuo ercou vtyv vuHesauoget
o.rodrco rnreds og 'deNuduEH 'Irfnhu ?rararadu ro rcsJ,eg
-[xdetr0os Holnk{Hrirs JHor.rfrE[ ptrJo)' wtrex u wadJ,es
'rq[oa ruHemr^Jcou runexde[?oIr rrrtr rcqJgsorqrol
! - ru roxoN ytruedel rennouefQnu renneaud.r,,firg
r/,reh seHHouelfuHu seHHesudtlHg
'nuedil
'eruttuQe[ lnronnexed
emh,,ftr - rss o)6ed udtt' uutte dar 4o nnoue.{QHrl UroHH esr.tfu
-rtrs olsfnol/rtou c oHhrlgo 'urcoHgod.tou eutn(xel
qJr,rhelcego r,r IsYos u vud,teg rufiuQetr qil,IHror Jos .
lts[os u uroc uderou qu,IgoH
-erco FrgoJh 'fnur,radu sJ,r4Hedrc^{ [rcoHxor^roos orr .
:BJHEHOUI IO) XTIHSOHCO
us[ raehorrrs rqtros u yudJles eu,rrluootr or,rHehelI'
ouHehou
'rudreH
oJoHHesodrsru@ro BaJcehr,rro) oJetrrgo obl'y oft war^
eoHerlr trcqJu€r{Hc rexon yudleu ruladxcxe B'JeetrEil
ehor^r s rudrer rrr,rfrufuHeIrHo) )E) rl{eds oJ s 'rqtrog
uyudJ,es uutrgdocgeed rr.lnemrssou rehc B0 rcJeEsuh
-ureg.( r{hollt qrcoHirlrruowcg'x€IlqreH?) xlqHhehou
xrqHlrnunr.rc)odu s orr4frgdocgeed ol$ru etrunf, ve>tredl,o
'trcqJpmlqsou rexow [sodx axJ,odosrsc s rqrorcr.Dr Uros
-ehorlr qHesod,{ ex xel onhoJ 'xufrsreHex s rsrHr.rsohor^r
uulrgdocguad lreuserucf rcJeelDKosoduoc o.ln'uhonr
?)OJOU IrrJOdO)C &rO)Sr{H 30-eu rcroBmrssou ueodx
axodosrqc a rirHr,rsohorr rrafiedlHelrxox os'enrdoH g
roBsrsrg oHhlqgo '6y3 ltereJErE)ou trcrorfar 4rsdo.r,ox
'ugodx llxJodosrsr nunul,ued;q 'euodQen s Istros u
rufues mu<detree goy[euHBxeN r.rufresr4J)I8 er4srctrellcs
rcree:KllHo runuxc{uclrerront slcodoxc oll'4onsrel
-pflffi vtfin gollexrI qHoho v}LeI'ffsIJ eH rr{Neuoflolrl,rJ
oxqlroJ vfixe 'yJLeBHeI IeI{ eH 'otrulgBdIt xux '6y3
'rqndoH xuretredu s roreerco oHhrqgo uuwarrosoul,rJ
r,rdu r,rsodx exrodosrsc grudleH rr,rfredrHefrHo)
e)rcruJ
auHesotouccn oqHdotedogeu
112 . KIIhHllt{ECKAf, 6l4OXt4Ml4, t4 META6OIU3M

y{I,tTblBafl , qTo }t3oToHl',Iqecrufi pacTBop HaTpI'If, XJIO-

Ta6nuqa 4.7. Kax npoBoAurb oqeHKy BoAHo'3fleK- puAa MoxeT BbI3bIBarb yllepenurtfi Mera6onuqecKr'Ift
Tponl,trHoro 6anaaca Y
naqHeHTOB
auu.(o3, BO3MOXHO, U3-3a rlpe3MepHoro [ocTyIr]IeHI'Ig
xJroplIIoB, TOrAa KaK <(c6alancuponaHHble pacTBOpbI>'
Sran 1: Kn[Hur{ecKu oqeHurb eoneultlecrui craryc
Hanpl4Mep pacTBOp Xaprrvraua, Moryr BbI3bIBaTb JIer-
a Onpepeluru HalluHue npil3HaKoB Mno- il rhnepBoneurra (cu
KyIO TUIIOHaTpI'IeMI',IIO, TaK KaK oH SBLIeTCS HeMHOTO
ra6r. 4.3)
a llpoeepra-ru exegHeBHylo AuHaMilKy Maccbl rera T4IOTOHUqECKI,IM.

Sran 2: u3yt{ttrb rpa$ur 6ananca xuAKocr[ B opraHx3ue

r npoBepilrb o6ulrai o6teu nocrynfleHue t4 BblAereHrn xilAKo- I l-unepaonemnq

ai, au'np"gt,gyu1rafi Aenu (e nopr'ae 6arasc noroxurersuutfi Inuepnoleuux o6ycnonaena ns6rrtKoM Harpllfl
Ha -400 Mr, tlro orpa(aer cKpblrble norepil xt4AKocrl4 r{ BOAbI U peAKO BCTpeiIaeTcrI y [auI{eHTOB C HOp-
-800 r',rn u o6paeoeanre BoAbl B npoqecce N4era6orhsN4a uaarnofi Oynxqueft cepAlra lI roqeK, TaK KaK IroqKIl
-400 un).
. npoBepurb coBoKynHoe u3MeHeHrle exeEHeBHoTo 6alaHca o6laAarcr 6olturultrl Bo3MoxHocT,IMI't B oTHoIIIeHI'tlI
xr,4AKocrt4 3a npeAblAytllrle 3-5 Aaefi' yBe.[xrIeHI,Ifl 3KCKpeulIu HaTpl',tg I'1 BOAbI C noMolublo
. Conoctaguru AaHHble rpaSraxa c grnauuroi Maccbl rera h
roMeocTaTl,IqecKnx MexaHu3MOB.
t{t4cTOTO
MhHt4qecKuM BOIeMUqecKuM cTarycoM Aa9 oqeHKil

Sran g, oqexnrb rexyr4rai narororuqecKril npoqecc
t Tlpoar-rarpoBarb norepu4 qepe3 xenyAoqHo-xltueqHstti
rpaKr h xhpypMqecKre ApeHaxil.
. dqenr,trs yBenilqeHile cKpblrblx norepu (uanpruep, npil nilxo-
paAre) ur BHyrpeHHpto ceKBecrpaqup ("rperue
Sran 4: onpegerlurb ypoBeHb MoqeBuHbl u e'leKTpo'll'roB
nna3Me (cu. ra6n. 4.2)
Onpegelraru ypoBeHb Na s nragL'le KaK MapKep orHoc'lTellb-
Horo BoAHoro 6alasca.
OnpeAe,ruaru ypoeeHb K e nnasN,le KaK noKa3arerb 6alanca
eHeKneroqHoro K.
Onp"Aerrrt ypoBeHb HCO; xax npu3HaK HapyLuenran KLl-{P
OnpeAelvnu ypoBeHb MoqeBuHbl 14 KpearhHl4Ha Afle orcrexl4-
Sran 5: ila3Haqurb coorBercrBylol{yKr 3aMecr[re'lb]lyto
repanuto BHyrpl4BeHHblMu pacTBopaMll
IE*eAreero BocnoJlHFTb ocHoBHble norpe6uocru B BoAe
olreKrponilrax (cur. ra6r. 4.5).
.flpnnral,raru Bo BHI4MaHhe oxilAaeMoe nepopaJlbHoe norpe6-
llatorenes
Hau6olee qacrble cl4creMHble HapyureHl'{'I' BbI-
3blBaloulle rr,rrepBoJreMl4lo, repequcrenH s ra6r' 4'8'
flpIa cepae.rHofi HeAocraroqHocrl'I, quppo3e ]I He$po-
TlrqecKoM cI,IHApoMe 3aAepxKa Harplls o6ycloueHa
HeAOCTaToqHOCTbIO rponoo6pauleHl',Ifl Bbl3Bannofi
B
4'5' '
ocHoBHbIM 3a6oreBaHueM, KaK noKa3aHO :Hapwc'
Apyrofi naro@llsuororuqecKl'Ift MexaHI'I3M saleir-
crByercrl npl4 norleqHofi HeAOCraroqHocru, KOrAa
ocHoBHOfi npuunnofi )tser}IqeHl'If O6teua flBlrsercfl
BbrpaxeHHoe cHI{XeHI',Ie cKO, yxyAlIapuee 3KC-
Kpequrc ]/lTrpllfr I',I BOAbI' B TO BpeM'I KaK BTOpI',IqHbIe
KAH'IJIbUEBbIE MEXAHI43MbI I'IMEIOT MEHbIIIEE 3HAIIEHUE'
flaroSusuororlI-fl cllH'upoMa Konna cBfl3aHa c reM,
qro noBbIIrIeHHafl ceKpeuuc zulbAocrepoHa HanpqMFO
t,1
crr{Mynupyer pe a6c op6uuro H.arpus"

.
JreHhe BoAbl 14 naToIloMqecKylo noTepp xl4AKOCTt4'
llpoeoAhru KoppeKqtao o6ueraa nocrynaroqeti B opraHI43M
i Ta6nnqa 4,8, llpu'trnrr rg6urra Harplle u Bogbl

eogur [eclu e/4, o6utqHo HaSHaqaercc B BuAe u3oroHt4HecKoro

Mexaxugu [lPuuePut
5% pacraopa AeKcrposbl (l-nroxosutt)1, Harpt e v Ranvfl Hapyurexre Syxxr4ar,r llepervuoe ga6oreaaHue noeer
B coorBercrBt4t4 c guuauuxoti SreKrporlt4ToB B nra3Me noqeK

Hbre). OAHaKo HeAaBHI'Ie KII'IHI4rIecK[e ]rccJIeAoBaHI'Ifl

llepsurxuti CranApot t KoHua
runepanbAocrePoxu3u*
He rroKa3zlJrrr4 KaKoro-nu6o flpel,IMylr1ecrna unSysuft
veJIoBeKa fipu JIeqeHI{I{ ocrpoll rr{noBoJle-
Bropnvxutfi Xponuuecxan cepAeqHae
affi6yuuua rlrnepanbAocTepoH143M HeAOCTaTOLIHOCTb
nauu. Kpolte toro, 6ruto noKa3aHo,
qro cI{HTerI'rlIecKI'Ie (qr,l. prac. 4.5) L{nPPoe neveuu
HeQPorravecxuti ctlHAPoM
KoJInoI{AbI (nanpuuep, 4excrpanu) xapaKrepl'I3ylorc'I
OHrePonarun c norePei 6erxa
[oBbIIIreHHbIM pI'ICKOM OCTporo noBpexleHl',Ifl [oqeK Hegocrarovtocrb nilTaHfi c
I'I CMepTHocTu y [auueHTOB, HIIXOA.flIUI'IXC' B TqXeIoM lzlpnonarnvecxulti/qurcnuvecxui orex
cocroflHl,lU. Tarclu o6pa:oM' KpICrIL'LIIO1{JIbLIB]LIIOTC',I Cresog noqequora aPrePlta.
onrlrMzurbHblMr{ pacrBopaN{[ A-[q uHoy3uoHHofi Tepa- . Cocronxnn, nepequcneHHbte s srofi ra6nuqe, KpoMe nep-
nuH rrpl4 OCrpofi rlInoBoJIeMI'IU. oAnaro Heo6xoAl',IMbl B14qHoro runepanbAocrepoH143Ma u creHo3a no'{e'lxofi
IloloJrHlrreJrbHble I4ccJIeAoBaHI,IrI AJIfl Bu6opa uau6olee apreplu, o6utqxo conpoBoxgarcTcn reHeparh3oBaHHblMl4
oTeKaM14.
lolxo.Ilfl UIe ro KpI'I CTZUIn OI'IAa B AaHH Oft CIldTy ArryIt4,
 III^IpD{JoII IOIetr?IIgO I,IHO'XErI<IITBH€) XMHqITBI^TUC 'rtasmge[ xlr ruHexorrl,tdu go)hor
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-orrg oHHer^redsoHtro '('pad 'wnd11 'nueorQur.r errHqreJurexeH r,r qJcoHsr.rrxv ' (g'y 'cud'wc) euodQeu
-
-Btrr re u e)olur rcrucoHro ml,ru x) nueorQlrrreuux x€JHor^{Jec xrqHhr,rrc€d s rudreH rl[frgdocaeed
u ulreorQurru€rreV )B) ev>tet'7 rqro)orrrJ ederdou c
wennarenl( oHeersc eusJcr^{etr xI4 'JV radu exoru.r, e
-csedr oJonucuser -4udnu rsdorugulrq'n;oxau? ' Iq[o
g u trroc
4oxxdetrBo goHH?solqg' I,Iul,rterosdeuuJ
odawdoucuodw o?own)nzDt-qndruou ndorungnzu24 . r.rlrHohelr s srod or[HsoHco roredlu uxur,ed[u[,
'nrod6", xerHer^rJec xurflor nweynAdluff suuedol
-fVercou s rcsregodugdocgeed rexon'x€trqrruHe)
g 'Irr rBxrrod.i(r,rtr rnuedel .
xrrHlrrrer^ruc)odu rcre.{dr,rgdoc9ead en redo.lox
'I,Jd.dte]J trrcuh E?mquog lurcongocouc 4on
e 'yud,J;eH r,rl,Irrgdocaeod
IltoHqrer^rucrodu srcen oilmsrogeu ee o)qroJ ro -domdxcxe orlrnemsHand rusrorosrooc rfn (u(c
-ehasro wolrH€xer[ UrrcHH€tr,(xsroxcou'urert {roJ€ / sroltlr 08-0S otr) eHor.rfred s rlrdleH orrHehr,(Hsfuo .
rrY exur,xedrr go)cehr{H}lrx s rcle,{eqroucu oxtrad i4uueneneu xlqHqrpfl uHlrn lrHt?rrog r{du rsm,rodeJ,c
no oH 'xraradfi,rtr 4rygerc o16 'euedgrlren goHqrrux -oxurdoxoxorril ulfl{ r,rrcoHhorurcotreH goHheVdoc
-urre eH exrHHor^rgo r{oHtrodotros-oaeudreu a *11 udu 611y rsdorugruuu 'denrnduen :nr,urerosdeu
-u.r rsHr4r,r.rdu HroHsoHco er,rHohor eo)3ehr,rQfirlellc .
eH roreesl,rHelllgo tlr<do.tox 'rradlug llrr€h oII{fI
-gdocgeed remsHeru('x€IrqrEHEx xrsHqruwr,rcxodu :soJ,HeHou
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- f,dugutuu' rqeedtru*rgogdex ltodoru 9nJlr,la ycl.:eyrr
auHsheu
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'lIHOhex I{wI )ahou
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. Orcyrcrgue npr4eMa AilyperuKoB B nocreAHee BpeMg.
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f-unonarpneuun c runepBolteMuert pa3Mepa KneroK MO3ra --) OTeK TOIOBHOTO
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npI,I3HaKI,I OTeKa TOJIOBHOTO MO3ra, TaK[Ie KaK Orny-
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eu^yer4 e oHrcu eoHcBdy_oeenmr,t!
eQgoxflrc3 qHeouog
tceput4HrytBeocyeJ tceeuilHiltieeocroJ
doreeruy edoreerrye lahreafi
aoxce^ilrouodeexearnoirducceart"Xf y
1X1# eeeUuuuneeocxe_1 ecrPCFBol cHeouog
eoEueouu.lre.r-7y1g
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eouilt xrqdotoyaH xilycehil.toLrodeoH .ioteslqetqs aH rqrHeildee
ersdo-Loxeg
cuU ghHBeoUeucct4 14elruetenoHe:do 6coofnotebxoEoduoc
QlA>tCOSVHnUy 'oerc4odrcced eoycer,ruouodeer eefnoildrccedtodu
rsleeodrueuueg eeeunuerno:tu$c Ev14lJ-EHHByu,tH
eilHelregodu eoHHeHedl"coduced eeuograeg
- Seucn\ cHeouog
BlHeEoueucct4
014ycehIH14lly eourr xulIdU oycuorcoH
gcrbUoeodu + orrdoyl rlodUHrC
r1e.L,fu xrHsuetexrcU unhleeur eralnor{dileilUilhed il
eouilr xr.JIdU uuf S0HEyrlUJOHt4hle8 ouurunSue3 nrodUnug
4erIu xtucuerextgE BvlHeeouogee erqHeilryldrcao,rf,ru -oyuu.t r,ruHeLrueln
:Eour4J xilyquoycsH rqrder euycer,u.rouoreu ,eoee:.cIc u ereueyc ederney nodunr3
BCJ_Aon1,l 'c.lcouercl.o 6pHHeslcy\I tqHxot,{eoe :eylodUsrc
,i*iori -ced e xilfnoi,{ercehri edeud{_.1
nodUrr,lg
10 l-Bct4seg eoruendeQ oycuoycoH eoEndexecuuouox{4
lqr{ol-cr4c foHedoH
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80ut41 x,4yqLroycoH
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uW rcreenp (suur arslr,rueed)
14
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(eu,rercrc 6pHsdoH
ceHquedrHeh,ohUdec,r,lxr.ou) ersuduu6rceeodoedeh
(snorede>iorune,eodUr:our,rt) .rrr.r^ouo.,.6^J.y
(xurcoHnarox e suog) euxcenrtouodeeI
BC100y\N
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uweruande$
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ruendaS 4nxueSafl uHeouog eHHnOCOetru
6r4HguuoreH nr.aaaog oHHmocoenu .lZ.? ehuvgel
68r . EOHl43loduoul4u uaoHuuua ulel4uoEvrsyu
140 . KIll4Hl,lqECKAC EI4OX14M1,19 14 META6OII3M

fluIrlesHe AerepMlrHaHrbr KoHueHTpaqun Tf s nnasMe

(qnnorrnonporer,lH KpoBI{ cnoxHbr, rrocKoJrbKy rrpe3MepHoe norpe6nenue
yrJIeBoAoB, )IC,rpoB H zurKoror-fl Moxer crroco6crsosarr
Tf
i! f :"nx"*ut IloBbllrreHr,rrc ypoBHs
MEXAH}I3MOB.
s ruIasr\,re 3a cqer pa3rutrHbrx

'f ,o"oon,n,o I SHAorerxufi crxrea nrnrAoa

Tpnmnqepug
:4r .. :'
U"g:B,",,,"
Pnc, 4.12. Crpyxrypa rrnonporeuAoB
plr$uqupyror r{x c o6paeonanueu TI, roropue o6r-
e/IlrHrrrorcrr c yceveuuor7 Qopuoft anonunonporel{Ha
B (Ano-B48) no uepe ero cr{Hre3a. KuruevHsrfi XC,
nolyreHnruf u3 [I,Ituta u xeJIqI,I, TaIoKe BcacblBaercrl
qepe3 cneuua,rruuft rpancnoprep Kr,rmevnoft ueu6pa-
Hbr, Ha3brBaeuuft NPClLl. B pesylrrare o6pasyrorca
x[JloMr,rKpoHbr, coAepxauue Tf ]r cJroxHbre s$upu
XC, xoroprre ceKperr,rpyrorcx vepes 6a3oJrarepzrnbHyro
ueu6pauy r mau$aruuecKlre Kanr,rJurflpbr vr [epeHo-
cflrce.B cocyAlrcroe pycJro qepe3 rpygHoft uporor. flpra
nolaAaHr,rlr B xpororor o6pa3yrouuecrr xunoMr4KpoHhr
uo4u$raqrapyrorcs 3a cqer AonoJrHurenrnoro,uo6as-
rerrvfl. anonunoflporeprHoB. Tf s xunoN{r,rKpoHax ru-
.uponr,r3yrorcfl nr,rnoflporeururunasoft , pacno:roxennorl
Ha gHrorenr{u rKaHeBbrx Kan[JurrrpoB. Oro nprno4ur
x nHcso6oxreHr,rrc x[pHbrx Kr,rcJror, Koropbre r{c-
An-s o6pasonauu fl. gHeptutr vtyr
flonb3yrorcrr noKuurbHo
xpauflrcs B Bnue Tf
a uuruuaxvtwr xlEporofi rKaHLr.
Ocraro.rHa.fl peMHaHTHa.s xr{JroMr,rKpoHHaq qacrurla
aKTLIBHO 3UXBaTbIBaeTCf peUenTOpaMI{ nUnOnpOTeI,IHOB
nr,rsrori ruroruocru (JIHII) (LDLR) B rreqeHr.r, Koropbre
pacno3HarorAno-E na ocrarKax JrrrlolporerHor..fux
rroJrHoro BcacbrBaH[s rrurleBbrx Jru[u.qoB rpe6yercx
oKoJro 6-10 v, nogroMy nocle 12-qacoBoro roJroAa-
HI{.s xr,uroMraKpoHbr o6rrqno ne o6uapyu<raBarorcfl B
rura3Me KpoBr4.
OcHosHofi ruulesorl AerepMr4HaHrorl xonuen-
rpa\hvr XC s nrasr,re KpoBr,r r.Brrfl,ercfl norpe6leuue
HACbIUIEHHbIX I{ HEHACbIIIIEHHbX XI,IPHbIX KIICJIOT, KO-
ropbre cHr.rxaror yponsu LDLR (cu. uuxe), rorra KaK
cogepxaquftcq s nuqe XC oKa3brBaer [apa.roKciurbHo
He3HaqlrTeJrbHoe BJrktsHr{e Ha ypoBeHb HaTorrlaKoBoro
XC. PacrurelbHbre creponbr r,r npenaparbr s$$errurnno
uHru6upyor BcacbrBaHlre XC, rocxolbKy oHlr raroKe
yr!{eHbllralor noBTopHoe }Ic[oJrb3oBaHI,Ie XC us xeJr.Iu.
flpu roroAaHr{u neqeHb flBr,sercfl. ocHoBHbrM r{c-
roqHr,rKoM Jrr{n}L[oB B rrrra3Me KpoBLr (puc. 4.1 3). OHa
MOXeT IIOJI) {aTb I{X nyTeM 3aXBaTa, CI{HTe3a I{ npe-
BpauIeH[{ r{3 Apyrux MaKpogJreMenros. Oru Jrvfirurrbr
TpaHcnopTr,rpyroTcrr B Apyrue rKaHtr c floMorubro ce-
KpeUlIu nlrlonporelrHoB oqeHb ur4sxoft nJrorHocrlr,
roroprre 6orarrr TI, Ho orJrr,Frarorc.fl or xr,uoMr,rKpoHoB
TeM, rrTo oHI4 MeHee Maccr,rBHbl I,I coAepxaT noJIHo-
pa3MepHyro MoneKyny Ano B100. floc.ne ceKperlur,r B
KpoBoroK Jrl,Inonporel{Hbl orreHb Husroft [norHocrlr
no.rlBeprarorcq N{era6oru.recxor; o6pa6orxe, aHiuro -
rr,rrrHo xr{noMr4KpoHarra. B peeylbTaTe rl.{Aporusa Tf
nprrroflporeuHoB oqeHb nnsxor? rurorHocrr,r x]rpHbre
KtlcJrorbr srrcno6oxAarorc.s B TKaHr,r u flpeBparqaror
Jrr{[onpore]rHbl oqeHb HI,IsrorT ruoruocrl,I B <(ocraroq-
HbIe)> qaCTI,IUbI, Ha3bIBaeMhIe JIIIIOIIpOTeI,IHaMH npoMe-
xryroqHorl rurorHocrlr. Eomuruncrso nrrnoflporeuHoB
npoMexyroqnorl uornocru 6uc'rpo 3axBarbrBarorcrr
LDLR B neqeHlr, Ho HeKoropue o6pa6arhrBarorcfl
re.IeHo.{HoI7 lr,rna3ofi , Koropafl flpeBpaxlaer rracrl,Iqy
n JIHII 3a cqer yAiureuus TI u 6omuruncrsa BerrlecrB,
rpoue Ano B 100, cro6o4Horo u srepuQlrutrpoBaHHoro
XC. Kara6onusu 6oramrx TI xulouurpoHoB [r Jrr,rflo-
rlporelrHoB oqeHb Hnsroft [rrorHocrr4 Jr]rlolpore]rH-
ilanasoft peryJrrapyerc s Ailo C2 u C3 xa rroBepxHocrr{
3TI,IX qaCTI/tU.
r{acruurr JIHfI crryxar ucro.rHuroN{ XC A;r-s xlerox
ra rraneft (cru. puc. 4. l3). XC JIHfI raHrepHzuru3yercs
c floMoulbro o[ocpeAoBaHHoro peuenropaMu LDLR
sH.qoqlrro3a. .{ocrarra XC no 3ToMy nyr}r cH}rxaer
3Kcnpeccurc LDLR u preHbrrraer cr,tHTe3 Lr aKTr,rBHocrb
orpaHlrrr{Baroqero cKopocrb $epueura An-f, cr.rHTe3a
XC, rugporclrMervJr-rnyrapr4n-rnuKorencltrrerasa A
peAyKra3br ( f M I- KoA-pe4yxrasu ). Apyrun BiDKHbTM
peryn-fl ropoM 3Kcrrpeccuu LDLR sBrsercfl qyBcrBr,r-
reJrbHarr K crepoJraM rrponporer{Hxonneprasa 9-ro
cy6rurlrsuu-KeKclrHoBoro rr4na (PCSK9), Koroparr
pacqennrrer LDLR. BnyrpururerorrHbre KoHrreHrpa-
uuu cno6oAsoro XC noA.{epxflBarorcfl B y3KoM Ar{a-
na3oHe 3a cqer raHrra6upyroqero eogAeficrsus JIHII
Ha gKcrrpeccuro LDLR, ro.rHorl peryJrrrqr{u nepuoAa
noJryBbrBelesus LDLR vepee PCSK9 u yro[ylsr].uu
etepn@uxaquuXC.
eadah (<(sorQud>) nuedgNelr 4oHhoreu) Ijarcsrgo -uer,oduolurn goHodxnnourlxrorc oJeHmeHg LI u)I'IH I
-hemlDr 'LrHeheu eu) sotruufir xeunexde[oc illlDl€llH t
-3X xrIJsJOg 0I{ )x oJoHtrogosc emqrog rr',udugue I
,nrgocorc endolox'11911 xumqrogoH ollaxeaoeedgo x c IV orv '(g 1'y 'ond ltc) (Ug1.) rrcoHrolru 4o)ocns I '
u.r[ogudu o€ '(IV3gV) 1y derdoucuedl 4rsulecce;r aoHueJ.oduouulr 3X wIHeIruo)€H orondeneadh ro IqH t f
-
rjtulttol€slqors3-Olv )Bx oJoHrsostu'€xltr'conedeu ol -etfltalrteo oHlllreJuHlrorrotrxHexr el'DlcenudeQudag I
-ounedgl^tel u oroxcenuQuleuc ro IqtruuuroQcoQ u 3y
gox€I'IH qHaho soH
IrIHhoJeIrx reeltunradu (urconrotru
t4lcoHrouu foveuH qHeho
lqHuoroduouuu UHOU - 6ySCd :eeeuruHne'!
l;g doruehed 4nudernhote$ I8Hq.:ruru o.,ororrcve)-Huerurllg{c o't-6 BeeideeHoxHreroduodu
- -
-oduouuu torr.ro6,ou''rioruehad U'lo"l ieeede6cuedruuheuuderceuox
-
- UUU:(gg1.g ouy doruehad) ,1.orrourlorrr, lquneroduout4lJ llllu \
-xurnheu rvxu :r4rooHrouu {o)oorcs rqsraerod.uounu arcuedeeH uEUH :taJ-soHrouu Iounor'(xeulodu
- -
- eeerv^Ued-Voy:-lyl-f :eeeur4u u,HhoHohou UU :lqloucuv etqudnx -
ieeeurlUad-y *renOeqox-,r,,,O"r^rr-rrra^rcyodUr,,.l - ouv :IO/!V derdoucuedr 4tsu
yXC :euuderceuox drase CXe :tstoucrlv e'qunuex -- iraeroduoul4t'oue y[
arsu,ogoec
- - -
r'rovcenuda$r,ndeu udu
_reccey rlutnorpsrcessc-reoco$r,d-rnraeoueue tecgv/r.vcgv :ccHehou sccrprflouJou rfuon 'eeuuouu,
-
,rqroucr4) ercHdux ersuuogoec e 'ul5ol{rouu roveuH/{ovocrqe r4weHuaroduout4u t4 UUleuodvunour4x/t4lCoHrouu
uceutnosueurqs l
qHeho uy{eHneroououru ,(Exeyr e-nradercerror-roorQe eofroenruuudr uaulgo rauuerceln^co euuderceuox eoduQe
,
royeuH 'eouuutru anxeuedx u rdoucsedr 'ouHBslqcPc8 '01'''ct4d
yrncoHedeu-youeg ,oloJ. e^oJy :roururu erdoucuedr uilu rsxeeexol
U8I.I
a
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il;;il a rv ouvo ol
ueux @r
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euHeucuYQka-X)|(C
eolH14c voJ.elco
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O rexcohr4rEueJodalne 7 ur'uax cuhedvcve {rl>
exuderceuox -ldoucsedr 4rruredgg
exrdetceuox evaercou tseHHoJoUHe
[^no"*]# r JleYsercou uelxe:oExg -+
i tiinuu eyleudu euco[ ::rrrf
1 x rrr,"dr"r",
Q4Y4 anxderuug
.;- . EOHH3aoduoul.tu H soul4ul4u v{eHuogvr3w
142 o KIIAHIA{ECKA9 El,lOXHMl,tq tt META6OJ1143M
Apyroft MeM6paHHbrrl rpaucnoprep (ABCGI). XC,
3axBaqeHHbr fi sru:llu MrureHbKtrMr,r JI B fI, erepra@n -
qlrpyercs Jrerlurr,rHxoJrecreprrHarlr,rJrrpaHc0epa3orU,
3a cqeT qero rIo.IIAep>KfiBaeTcg. rpaAr{eHT 3axBaTa, r,r
qacruqa npeBparuaercs B spelrni c$epuvecrurfi JIBII.
gru JIBII srrcno6ox4aror csori XC B neqeHb Lr Apyrue
rKaHu, Hyxlarcuueca n XC, uepes @aroqnrapnufi
peqerrop B1 (SRB1).
Eelox-nepeHocqr.rK e@npon XC (CETP) B rura3Me
KpoBlI flepeHocr.rr XC Hg JIBfI puu JIHI s nrrronpo-
TeI,IHbI oqeHb ungxoft rrJlorHocrl,r wrut wuroMytKpoHbr
s o6I\,{eH sa Tf. Kor4a ypoBeHb Tf nosHueH, CETP
cuoco6en cHlrxarb ypoBeHb XC s IBfI r4 npeBpa-
qarr JIHfI B <<MiureHbKlre, rurorHbre> qacrlrrlbr JIH[,
Koropbre uoryr 6rrrr 6olee ateporeHHhrMr.r B creHKe
KpoBeHocHoro cocyAa. BUIH xusorHbrx, y Koropbrx
orcyrcrByer CETP, ycroriuznu K arepocK;repo3y.
Yponenr ruruolporer,rAoB B [na3Me KpoBv[ flBrsercs.
ocHoBHbrM uo.ura$nqupyeMrrM Saxropou prrcKa cep-
AeqHo - cocyAracrrx ga6ol esanuh. flonrrurennrre ypoB -
HLI areporeHHblx nunonporel{IoB (r ocuonuou JIHII,
Ho raroKe JlllnonporelrHoB npoMexyroqHorZ rurorHocrr4
rI, no-Br,rAlrMoMy, ocrarKoB xlrJloMrrKpouor) cnoco6-
crByror pa3Burlrro arepocKJrepo3a. flo4rpynna vacrr{rl
JIHfI prueer AorronHrrreJmnrrft 6elox, usnecruuft
KaK anoJrr.rloflpoTer,rH (a), xoropuft roN{ororu.reH
rrfla3MlrHoreny. Kou6uuaqu.fl JIHfl r{ aloJr}rnonpo-
rer.rHa (a) usnecrua KaKJrlrnorporerH (a) (JIn(a)). On
TpaHcrroprr4pyer oKracJreunue @oc@onr{rr]rIhr u c,r.u-
TaeTcq aTeporeHHBrM, nocKoJrbKy ero KoHrleHTpaur{s
B ruIa3Me KpoB[r nBlrs.erc . He3aBucr,rMbru Qarropou
pr4cKa cepAeqHo - cocygrrcrbrx 3a6oreBaHr,rft . flocne
xIIMI{qecKHx uo4u$uxaquft , TaKr,Ix KaK oK}rcJreHr,re,
AnoB-couepxarque nrrnoflporer,rnrr 6onrrue He pa3-
pyualorcq c [ouolquo o6rrrnbD( MexaH]r3Mos. OHr,I sa-
rrycKaror caMonogepxl,IBaroqnft cq socnaautelrurtft
orBer, Bo BpeMfl Koroporo r{x 3axBarbrBaror MaKpo$arur
c o6pasonanlreM rleHl{crbrx KJreroK orrrtnlTerbHoro
- ttut. Ctrcayer y{r,rrbrBarb AonoJrHI,ITeJrbHbre lrcKuDKa-
[pLr3HaKa arepocKrrepor]rqecKr,rx 6lsruer. Oru npo-
ueccbr raroKe oKa3brBalor He6laroupu-arHoe BJII,r.flHIre
Ha $ynxqurc gH.qoreJrr,Ls.
I,I nao6opor, JIBII BbrBoAsr XC rs rxanefi s ne-
qeHb, rAe on uera6olvr3r4pyercfl. r{ gKcKperr,rpyercs
c xeJlqbro. JIBfI rarcre Moryr nporuno4eftcrBoBarb
HeKOTOpbTM KOMIOHeHTaM BOCnZUIT{TeJrbHOrO OTBeTa,
HanpI,IMep SKcnpeCCu}I COCyI]ICTbIX MoneKyn aAre3LI]l
gHAoreJrHeM. CneqonareJrbHo, Hu3Kue yponxu XC
JIBfI, xoropbre tracro conpoBox{arorcq yBerraqeHueM
ypoBHrr Tf, rarcxe noBblrraror plrcK arepocKJrepo3a.
I,lsuepeuu-a ypoBHs nunuroB o6brqHo BbrnorrHsror
no cJreAyroulrM npr{rr}rHaM :
. cKplrHrlHr .qn flepBr{rrHoit ulu ntopnvnoli npo@u-
JIaKTI{Kr,r cep.qeqHo-cocyAr,rcrblx sa6orenanuft ;
. o6cre4oaaHr,re naur{eHroo (u ux poAcrBeHnuron)
c KrurHuqecKI,rMI,I uposBrerliyrflllyr napyrueuuli au-
flr,rrHoro o6ueHa;
. KoHTponb e@QerrunHocrl,r Alrerbr, Maccbr rera yr
JreKapcrBeHHoft repanun.
HapyruenNa Jrlrnu.qHoro o6rrrena traule Bcero Bbr-
.sBrrrroTcrr npu npoBe4eHulr AaHHbrx raccnegonanuft.
I4euepenu-a lposHs o6ruero XC u XC-JIBfI nocle npu-
eMa rruur4 no3BoJrsror oqeHlrrb ypoBeHb XC ne-JIBfI
(paccvnrunaerc.s rar <,o6qurl XC> - <XC ne-JIBfI>),
Ho AJrfl craHr aprv3alq;,z,ur kt3MepeHr,rJr yponHx TI rpe 6y-
ercn o6paserl KpoBr,r nocre l2-qacoBoro roJroAaHufl,
qro no3Borrfler paccqlrrarr XC nHII (XC-nHfI) no
$opuyne <Dp4ana;rr4a:
XC-JIHfl : o6uuft XC - XC-JIB[[ - (Tf /2,2)
uuom/n
V)IYI
XC-JIHII : o6rquft XC - XC-JIBII - (TIl5)
ur/a;r.
KorAa yposHu Tl npenuruaror 4 uiraons /t (350 ur /
al), Ooprrayna craHoBl,Ircs HeAocroBepnofi . I,Isrvrepe-
Hr{e ypoBHs XC se-JIBfI ruru Ano Bl00 nosnorqer
orleHr,rBarb pxcK cep,{eqHo-cocyAtrcrrx sa6orenaHr,rrl
6o-rreero.rHo, ueu XC-JIHII, oco6enno KorAa rroBhr-
rrreHbr ypoBHr,r TI. Bce qarqe ucnorb3yror o6pasqu,
BgqTbre rrocne nprreMa nr,rul[, [oToMy rrTo yposeHr Tf
nocJle npueMa nuul,t sBrfl etcx 6o.nee vyrcrBrlTeJlbHbrM
MapKepoM pr4cKa cepgerrHo-cocyAucrbx sa6oaesaHr,rft .
Teu ne uenee grfl $opl\,ramnoft Auarno crnKnlaurwrus.
fl,rleprpr,rrnuqeprqeMlru u lrcfloJrb3oBanux $opnaylu
tDpugnanrga seo6xoAuna I 2-.racosorl fleprloA roJroAa-
rculre $axroprr, HarrpuMep HeAaBHo [epeHeceHHoe
3a6oneBaHr4e, rrocJre Koroporo ypoBHra XC, JIHfI u
JIBII npeueHHo cHr,DKarorcq flponopq]roHzrnbHo ero
Tsxecru. Pesynrramr, Koropbre Brr,r.flror Ha ocHoBHbre
pellreHr,rf, , TaKr4e KaK Haarulo JleKapcrrenuofi tepanran,
Aororcru 6urr nogTBepxqeHbr c noMorubro noBTopHoro
rr3MepeHr{s.
Yponuu Tf
.racto [oBbrruarorcfl ilpu ox]rpeHr{}r,
c axapHoM Aua6 er e lr I{HcynlrHope3r,rcreHTHocrr{ }r
 'Q7p'vgvt) Bsrcretr ltodtrnuc UlsH[oxJ',(uur ^{vuosrH€HI{I [ov-oHltocor-(e t4
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I uuhedauo erqssacexedeu
| 'ercUadc xrqHHasrcdeyeur neudu
|'eoedrarouu.r'r,1r4r,!eHp 4oxeorahrndeu
I eoHy,,leHeftcHlenac'uHoheu
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174 o rEMATOrIOrrq 14 TPAHCov3hOrOrylg

i Ta6nr,rqa 5.8. KnaccuSuKaqtts tl npt,tqnHbl ep[rpoq]tro3a

A6conprnuri opxrpoqlrro3 Orxocurenuuut& (c nurxuu

o6teuou) opurpoqr,tro3
Feuaroxpur l'loeutLueFl lloeurLUeH

Spr,rrpoqrrapuan IoestueHa B uoprae

Macca
O6rev nnaevut B uoPue Csrxen
KpoB14

[lpuvursr lTepauunue luyperurn

Mrelonpona$eparilBHoe ga6oreeaHile Kypeure
. l/ct uutan norillll/TeM L4t (nepauvnaa npolrQeparuenan nolnqurevun). Oxnperrae
Broputnue 3loynorpe6lerL4e aJlKoToreM
Burcoxni opr4Tponool.4H ra-ga Tunoxcuu maHetT: Cungpov [aric6era
. 6oruLuaq Bbrcora Harq ypoBHeM MopF;
. ga60reeaHne cepAqa ilril nerKnx;
. Telaomo6uHur c BbrcoKrl[,4 cpoAcrBoN/.
llarororuqecxoe noBbtuJeHile ypoBHt Spt4Tponoorl/Ha:
. nogeqHas HeEocraroLlHocru (rragponeQpoe, xracrut, pax);
. Apyrile onlD(onr (renarol,la, 6porxoreuruttT paK, Ml,4oMa l,rarxr, $eoxpovoqu-
ToMa, Te[,4aHTr1o6lacrol.aa voexevxa);
.3K30TeHHa9 Tepanilt TecTocTepoHoM.
SxgorerHoe BBeAeHre 3pt,trponogrilHa:
.IC, noeuruaou.l/e npoil3Bo!ilTenbHocrb y cnoprcl,4eHoB

aroareunrrft 3yA (3yA or KoHraKra c Bo4oft), renaro-
crureHoMer€uluq I,I no.4arpa (L{3-3a 6ucrporo ueta6o-
Jrr,r3Ma epurpoquron).
Eclpr r"ryraqus JAK-2 orcyrcrByer I,LIBHoft Bro-
p[rlHorl npI4qI4HbI HeT, HeO6XOIUUO I'I3MepeHI'Ie Maccbl
Spr,rTpoqr,rroB Ars noATBepxIeHI,ls a6conloTHoro 3pI4-
TpoIIUTO3a, a 3areM AaJlbHeftuue la6oparopHble I{
-
r,rHcrpyMeHTrulbHble uccJIeAoBaHVfl EJI[' rcKrIoqeHI'If
rlrnoKcl{}I I{ IpI{qI,IH HapyIIreH}Ifl ceKpeu[]r 3p]ITpo-
EO3TIIHA.
HeftrponeHur (ra6,r. 5.10). HeftrpolleHllfl uoxet 6rrrr
6eccuunrouHoit, a Moxer npI4BoAI{Tb K reHepzulr{3o-
BaHHoMy cencl4cy. Pucx 6axrepplzlJlbHofi uuQeruuu
cBrI3aH co crelreHblo uellrponenrav, rtpl4 3ToM no-
Ka3areJrra nnxe 0,5x l}ef tt cturalorcrl KpI'ITI'IqecKI'I
nusKuil,rpr. Jluxopa,uKa gBnrlercrl nepBbIM I{ qacro oA}IH-
crBeHHbrM [poqBreHueM uuSeruuu. BosN{oxHH 6om
BropJle, BoKpyr 3a,(Hero lpoxoAa ]r Boc[€L'IeHI',Ie KOXI'I.
Orcyrcrnue nerlrpo@tlroB [puBo.(I,Ir K cerITuueMHI'I u
rrroKy B TeqeHlre HecKoJIbKI,IX qaCOB [pU OTCyTCTBI{I4
He 3aMeAJrI,ITeJIbHo na.Iaroll auru6axrepua,rrHoft re -
pailvkt. JIe.reHI4e o6cyxgaercx sa c. 224.

Yueuuuenue o6tuero'4ncna qllpKyJlllpylouux I lhtilSoneHrq

neftKoullToB Ha3blBaercs neftKofleHuefi. Ona Moxer
6urr o6ycroBJleHa yN{eHbIIreHueM qvlclra Bcex rrrnoB
JIeftKou}ITOB I4JII,I OTTENbHbIX T}INOB I.IIETOK (O6TUTTO
nerlrpo@ruor rutu ruau$oquron). JIeftKoneHI4r Moxer
BO3HI'IKATb }I3ONUPOBAHHO I,IJII B PAMKTIX YN{EHbIUEHI'IS
qlrcJra KIIeToK BCeX TpeX reMaToJlorullecKl{x pocTKoB
(nanqnronenux).
I Heilrponennn
Yuesrmenue .rucna ueftT poSwron (o6rrvno ( 1,5 x
lOe/n, unaercTcs pa3rllquq B 3aB[c]IMocrI{ or Bo3pac-
ra u pacu) Ha3blBaercrl neftrponenuefi. OcnosHHe
npurrr4Hbr netlrponeuulr nepeqncrenrr s ra6r. 5.9 u
Oro yueurureHlre a6coJllorHoro qucJla ltlu@o-
ulrroB MeHee lxl}e/tr. flpuvuHu nepequcreHbl B
ra6:r. 5.9. Heclrorp.a Ha ro qro He3HarII,ITeJIbHoe
cHr,rxeHr{e 'uvcrira luuoouuroB Moxer 6um 6eccuu -
IITOMHbIM, AEOTIqUT KJIETOqHOTO I'IMMYH}ITETA MOXET
rrpr{Becr}I r uu@exuuru (nusnaunrru rpra6auu,
Br{pycaMl4 u Illr,rxo6axrepuruu) l,t npeApacnoJloxeH-
HOCTI'I T IUUOOT4UTTbIM I,I AP}'T}IM 3JIOKAqECTBEHHbIM
nonoo6paso Balat4flM ( oco6euuo reM, Koropble cBssa-
Hbr c BrrpycHuuu lru@ert\nsMvI, TaKIIMII KaK BI'IpyC
Onurrerlsa-Eapp, nupyc rIalulIJIoMbI qeJroBeKa u
Brrpyc rcpneca qeroBeKa 8-ro rlrna). Jluu@oneuur 6es
xaxnx-nu6o orIeBIaAHbIx rrpllquH qacro Bcrpeqaercrl B

Ha pr4c. 5.12. HepeaKo Bcrperlaercr neKapcrBeHHas noxr4rroM Bo3pacTe.

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ff nr.uhoQnuy '(urduueu'r'denrduex) oilmforcodu'(eau
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eoruhoHou\l 'fiy) eHuxxUoy eyloQnuu
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'eoyrlou rIrcHEilouor.4y! t4rrycoh 'I4JOXO'gilhEdOUO gerCAhilJd^dr/X :er,'lggdl .
-14Hodx'614neluhuuou :erueeeuogEe ooHErl]-edoQnUoduor-reuryl . '6e€oygr4dJ'ueueueudeuieg :unhreQx4 .
cruuQoeeg eou14OodqeH
g rruuSoeeg V rctruood.Uq{
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f
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176 . FEMATOIOfU9 U TPAHCOv3nAnOrnfl

Ta6nrnua 5.10. IlexapcroeHHble cpegcrBa' Koropble I OosuHo6unus

I!

F Moryr BbtgblBatr uefirPonexun*

Yseru.reHl4e ql,Icra oo3l,IHo$I'uon 6olee 0,5x lOe/n
[lpuueput
l-pynna o6rr.IHo o6ycnoueuo nH@eruueft (n oco6eHHocrl'1,
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BoonarlrerbHble neHilLll4flrlaMuH, HanpoKceH
sx3err,toft , 6porxualrHot? acruoli, peaKUllsMll Ha JI C),
cpeAcrBa
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Axruapuruuxr Xuuupru, npoKailHal/ilg HbrM HoBoo6pasonanueu (Hanpuuep, nuuSorvrofi)
(cw. ra6,r. 5.9). O6sIvno raKafl 3o3l'Ino$ruur xuxercx
Aurnruneprexsuaxute Kanronpul, oHananpff,
cpeAcrBa nuQegnnuu KpaTKoBpeMennofi.
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nclxorponHblecpeAcTBa MilaHcepilH .Iaercs croftxafl , rlacro KJ'IoHzuIFHzIfl 3o3uuo$ultux'. wa-
rrpr,rMep, [pI,I Mllenonpolu$eparusHrx ga6oreBaHtrsx,
Ilporneouarnpraixute flupuraeraunH, AancoH,
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Myrauuli peqerlropHoll ruposunruHasu (Ha-
B reHax
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rpou6ouurapHoro @axropa pocra c u fi utru c-kit),
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rae O6uIe rO sWC IA UlIpKyn 14 pyIO UILIX
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I fluttOroql4To3
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crux:a6olesaHuft, Ho oHo HepeAKo orMeqaercf, B
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uu rllu@aruqecKux y3JIoB, a raKxe KruHHr{ecKI'Ie
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181 . I4HHVEsUOSVe XUvcSht4JOUOlVW3J BMHShSU lqu[hH]4du
or,(wurcewsoc sJ}lsoroJtrou ofucIsg oIrJoI I HrIHHsIiog
ugodx rr,rneslalrederr ol,IHerefto Iqgorh 'ltretreu atnt I,{I
-ehor s ltudoredoger s rcilulHedx lexon erI{eI{ITElr cr(
usod) neeedgg',r,e,{s'tcJr(cro HeJIlrHs gl4rfloil{lr:rl,es -e
-rooc {odolox g ',(ccex otftrderufiodrude sredgnu n
oHxoIL{ntoreou'uuHugodulceJ rltemIJeHsIrEY Hdu -t
rsHesodulluQufiretrll silsg rfuoru EllerrrrHg eIqHHar -c
-,(dzngo elqgoll1'rerI,IJHB xlqsderulofundeuuru xax rl
B[HersBrqs ffitr'IqHeJ}IrHe eIqIDK?s oeIIog]I€H xlfltr(I '14
-,{ducceducxe'sorufiodrude edogeu NeuHsso€crou IE
-cu c .r,orer,.(eu e)Io{BJ €rHa}Ifieu .{x.todoaHc B '(IqU -
u ggv €[ur rl,IHelreVoduo rrtr roilVerccll €rHeI{IrPrI u
usodv elreedgo 0I4Isu{nod.rude ersunudxc ee u nsodr H
e
rsuu,(fu suHe[e[eduo od,(trofiodu g'qrconurconsoJ
ee n Hsodr
ofut,fttsru,(trrrstttruu en r(9o dtt utrut ettulrudvc tlI
Isulr,(fu rprneretreduo /(d,(follodu rerHlroulss usodl II
nanesuredeu rudoledogelr'erHeuwlltod urlerunodr E
-ude c uraer(Qcuudr,oNeJ rlrtr IiIcc€I,{ 4onder,ufiodrnde -
rlfi'
rrrconllrceltsoc a rcqrudeaorco[d Isgolr, o.ror r
q
Aanefi0c{edtowu iledeu ouHesoileucgo o
'rIIHeheIr Bxl{rx?r B"gOCO rCre'(gedr x
r
rI}rNoHB 4o)Cer,r,ueddovteficolr 4olrexrJ, udu rnnaceuc
xvt yrll u 'usodx rranesutradeu ro rcilru€zxl,o rfuox
( <,rrrore7 ulreretrus J > rautreenne-Ido 4oueoururad
rsJuetrB' denudueu) IsrHoIlII€u endoloxell'uHselrog
uudo:rcu s oHesoducxuQee srrsg oHxrotr orh 'It{sx
-Hnnas.Lctrod ole c'olot<ot,teoseH or€ }Irce'ulfl4 I'rIorHa
-urIE[ c srutrrbgo re.(trerc runaher Iq[orow eIqHsuJuH
-derqre ecs u lrsodx a[Husl'{uadeu eower(duuerl]
El4HesU uedau drtuehodu qrcoHcel lHI
'elneuuufied ^{,{cucuec urn uralteudelxug
rlrouexru>r ulcesudu lexol l on '('-r S I0Z qres 3e uI'IHet
-udgoxureg a 4uL.firc 4rsnnetrxdesrtrou HI'IVo) oxtred
engedx rc.r.eur,edrcs (ao.rufioglttofu 'orftrsedu xex) uu
-odx sorHaHoul,[o) rutleHl'Irar?rHox BeHsruude'ureg
'lrurrsdxcg udu unlrxeQun
raxenslldu ourcehl,IJolror,curonr{wl lll nnexr{dengo
r,Irfrqg uTHepIIIBU OJOHtrO ,( erne e'ego>ty-e.rrsreQ
-nr4ed; I,IH€erog I,II lrllHelrsrodu ururrxcenl'IHulD{ c
nrden( eodl'eutfisolroges scoruseed fiusrctrercous
xsdorox t( 'sodoHotr.to ragodx sorHoulufied 91 eu ou
'uere[eduo en uaodx t,rtanesl4redeu udu sgo>rtr-Brtr
-ereQfir4ed; olqHoelrog Bureuurafi ed runaxedze xcug
'ero)c o.rore.rod olosu,(dx rtor,ureQelne rus'r.er'gfu c
I eonneerec 'Bxesoreh euH€salrogue eor+roudu IJogoc
I rerraerctradu egoxy-PJtrslroQfirged;1 <ueerog
188 o TEMATOI|OT}|9 14 TpAHCOv3l4OrOrUt

Bscrre KpoBn Anq r4ccreAoBaH[q nepeA reMorpaucQysuei . f'lpoBepbre coorBercrBue eruKerKu Ha ynaKoBKe
* . Ygoctoeepbrecb B ToM, r{To ilMeHHo AaHHoMy 14 AaHHbrM xa 6pacnere naLlreHTa.

fl . naqueHry 6yger npoaogurbc, reMorpaHcSysrar. flaxer xpoeu Epacner naqxeHTa

! Mapxrapyfire npo6upxy c o6pasqou rposra,
I pas6opvuao u ror{Ho eanorxure $opMy 3arBKl4 OaMAnaB
I nocre uperru$uKaqux naqueHra. VlMe
V . He sanonxnfire Qopuur u errKerKu 3apaHee.
Ilepenuaaxue xpoau
.Ygocroaepurecb B ToM, qro uMeHHo AaHHoMy
naqneHry 6yger npoeogurbce reMorpaHc$y3r4e. Aara poxAeH[F
. Y6eAurecs B ToM, qro ugexru$rxaqut KaxAoro - YsrxaluHstta #
naKera KpoBr4 coorBercrByer rgexrnQuxaqnn uAeHrh$t4- /I
naq14eHTa. Kaq4oHHblr'l .1.-J
. y6eA[recb, r{ro rpynnbl xpoau AB0 ur RhD
soueo/gotaeo \
ucropan ooIe3Hh \
Kaxgoro naKera KpoBr4 coBMecruMbt c rpynnoi -L<_-
KpoBr! naqueHTa. o_
Ecnu eogt'roxxo,
. f'lpoBepbre Kaxgyo ynaKoBKy Ha HErnuyre noBpex,qeHufi. BcerAa npocure naqheHra caMoro
. f1ph Hanx,{14t4 cotuxexuil xe ncnolusyilre naKer n BepHure ero B orAereHue xa3earu cBoe nMn u Eary poxAeHug.
nepenuBaHut KpoBlr.
. 3anonxure Qoprr,rur, AoKyMeHTupytoque nepenr4BaHue Kaxgoro naKera.

BqAenue Aoxpelrrar{xu
I . 3anvruure B Mcropr4r4 6onegxlr naqxeHra npursuxy nepenuBaHu, KpoBr4,
V Ha3xa.{exxurfi npenapar KpoBx, Aosy, Bce Hexenarenuxure e$$errur u rnr4Hr4Hecxt,ti oreer.

Ha6nlogexr,re
. neperuBaHr4e KpoBu cneAyer npoBoAr4Tb rolbKo rorAa, KorEa 3a naqueHToM Bo3MoxHo opraHu3oBarb ua6nrcAexue.
. AprephanbHoe AaBreHhe, qacrory nynbca r,r reMneparypy rena cneAyer KoHrponilpoBarb Ao nepenuBaHue KpoBu
14 qepes 1 5 MtaH nocne Haqana nepenuBaHhq Kax,qoro naKera KpoBr4.
. Y naLlhexroa, HaxoAgu.I4xcn B cogHaHhu, galuxefiu.ree na6nrcgexue xeo6xogr,rrr,ro ronbKo npil Hall'neutA cuMnroMoB
r,|u npu3HaKax peaKquH Ha nepenuBaHue.
.y naqheHToB e 6eccogxarenbHoM cocroqHr4[ peryngpHo npoeepnfire qacrory nynbca u reMneparypy rera t{epeo
onpegeneHHbre BpeMeHHbre ilHTepBanbr Bo BpeMfl reuorpancSyeuu.
. npilgHaKu naronon4qecKoro KpoBoregeHr4r Bo BpeMq nepenuBaHue KpoBr4 Moryr 6srtu cgqganut c pa3Br4rheM
ABC-cunApor,,ra, Bb13BaHHbrM ocTpofr reMonurrqecxoI peaxqneil.

Puc. 5.15. l-lpoqegypur y nocrerfi 6orunoro Aflf, npoBeAeH[n 6e3onacHofi reuorpancQysuu. Segonacxocrb naLlueHTa 3aBt,tcr4T or
co6noAeHt4f, craHAaprHbrx npoLleAyp 3a6opa o6pa3qoB Anr npoBepKu coBMecruMocrh, nepeflilBaHufl KpoBr4, BeAeHue AoKyMeH-
Taqnu u Ha6flrcAeHus

pa6oraloulllft c nauueuraMl{, 6blr o6y.IeH pacno3- 6yAer noArnepxreHa ra6oparopHblMll AaHHbIMI4 L

HaBaTb 3Haqr,rTeJrbHyro noTepro KpoBr4 Ha paHHr,rx
cTaAuqx lI Harr[HaTb rerreHue Ao pa3BuTI4rI rIroKa.
EoruHuurr AonxHhr rlMerb MecrHbre rrporoKonbr A.rtrr
JIeqeHI{rI MaCCI,IBHbIX KpOBOTerIeHVil, kt BeCb KJII{HI'I -
qecKuft nepcoHan AonxeH 3HaTb r{x coAepxaHpre.
ClaxeHna.s pa6ora r{ KoMMyHr{KarIrrr Heo6xoAr{Mbr
Anrr npeAoTBpaxleHr,rrr He6naro[p[rrTHoro KJr]rHH]re-
cKoro llcxoAa, HeofITI4MaJI bHoit vnv HenoAxoA.f, tuefi
npaKTuKI,I nepeJrlrBaHH.q KpoBu r,r floTepu KoMrroHeH-
TOB. cBeXe3aMOpOXeHHyrO nna3My CneAyeT BBOApTTb
KaK qacrb nepBoHatriubHori uHoy3r4oHHoft Tepaflurr
B COOTHOIXEHI'IU, NO KPAftHEIl MEPE, I:2 C KOHIIEH-
TpaToM 3pr.{Tpour,rToB Ao lonyqeHLrrr pe3ynbTaroB
Koarynsur4oHHbrx npo6. flpu
pasrurr,rH y rrarllreHra
KpoBoTeqeH[s cneAyeT BBoAr{Tb cBexe3aMopoxeHHylo
rrna3My r,r KoHrIeHTpaT 3pr,rTpoulrToB B cooTHorxeHllH
1:l ao rex rrop, [oKa 3OOeKT]rBHocrb reqeHr,rq He
He 6yAer paccMorpeH Bonpoc o qelecoo6pa3Hocrr,r
r,rcfroJrb3oBaHus Kpr,ronpeuunr{Tara. flocne ocraHosKu
KpoBoreqeH vfl Aurb}],eirruee nepenuBaHr{e cBexe3a-
MOpOXeHHOft nlaSllhr AOnXHO 6rrrr OCnOSaHO Ha
.rlaHHbrx aHaJrlr3oB : [oKa3aHr{eM .unrr TpaHcoy3ulr
sBJrflercrr 3HaqeHlre IIB u/uru AqTB s I -5 pa3 Bbrrre
HOpMbr Arq craHAaprHofi AosH cBexe3aMopoxeHHofi
nJra3Mbr (15-20 Mn/Kr). Kpuonpequrr{rar cneAyer
Ha3HaqaTb, ecnu ypoBeHr $u6puuoreHa rra.{aer Hr,rxe
1,5 r/n Korlr.recrso rpoM6our{roB cneAyer noMep-
xlrBarb Ha ypoBHe Bbrrrre 50x 10e/-rr; vro6u ) {ecrb
BPEMfl AOCTABKI,I, CICAYET 3AKA3bIBATb TPOM6OUHTbI,
ecnll KpoBoTeqeHI,Ie flpoAonxaeTcrl I,I KoJII{qecTBo
rpoM6our,rroB Hr4xe 100x l0e/n. I4cnorrgosaHue
KOMnOHeHTOB KpOBr,r npu MaCCr,rBHbrX KpOBOTeqeHr,rqX
cyMMr,rpoBaHo B Ta6Jr. 5.17, a ocHoBHbre MoMeHTbr
rpaHcoy3r,roJrorurr B ra6n. 5.20.
-
 oHHOsudrIHs 8/S
ierHeueeU eoxeoxee eoHquedrxeh
-
flgfi larHeueBU eoHsueuderde - !t ivrodUuuc-ccedrcuU grsHdoreduuced 4ndrco - 9[69
'creeodurveddox oyruuoxgoeu IeoU
- 4erev tr 'xur,rh)read xrsxxouer{Qcued.u.cou xrqdrco udu euuanar u euuBgoveucgo 'gt'g'ry I(II
w
'noUodoucr,ry o7o69 I oruedaronoJncro qrltheHspH . ell
'ueody er4Heer4uedeu srnredxadg o
'u[hedi-quMQHr ueeoJBho ccxHodorci(su:r,rxar, [oHuIdJ soHEJdo cxQBdJoHauHgcl . osr
'qt €mPy [FH6r4r-yIVoduaH'gymrcvo . 'IqJ,
'haody u!4Hes[uadeu aucou h rz-g euH€hsr e oHhtqgg .
prctlocdrona c €oHfleau*?'xrxJeu erHetha(edsou eodtco ,rrcoxUr,rx are^dJedeg qrc
|
-da
e)
andoH I nnveuerf eoHeoHsa goHquEdrxeh sudeneq o Ueh
-'H;; e
- ."{'uxl.er, [oH[ dr soHerdo ouQedoxa.u+ed eruHuouBg . e14Hemrqaou
Je1(:
'r4sody sBlcoc {tlsoee: ereeodurodrxoy o
J }{o
,rHorourJ/evmstb lodr,co rdg
I emI
t4t'{EhhHhUO 4arhe-rsulc*ox ee Bcqrx.Ledgg .
l,'tr,'ltqHHeeoequouc140H/4nrqHHesoeguoucn ccrlt.'ll,1mserco 'orrueder oIxsoyrelQHr4 h oc4uedaJ.oHoJuc)ro q-Lelq{. uu
nnece oc or,cona n8ody cilHeshuedeu er4Hauevao e ouus moHHoH t- '(g 'u"r 'wc) onhxaQun olxsuendarxEg BH r4r4Hed6othr gH
-onetaeH s cJ.^Hdes :Ar,{oJ,crc ol^HHoheAOHu t,t t,laod] re)eu crBHC . xdu cr4sJ.cIgu edLyauc oJoyod[m r4npyrlougrlrHE ouueder qlerq{.
'naod)i BuHe€ 'eed^xtr q1BsodHuod!.rol . -?6
-fiuedeu olndorPdogeu e ueodx Bc{em€AHdes3 haeedgo crreedur6 o 'yrhoy{ enueHe I!4tn9o 'ehHEa
I,DI
'.!{/e rtnt 9rO (undQexnue) -ououcct4 ooyoohr4rrr.txoxg !HxHr4dyc [rcHHor4hBufupox']VOnr.co
H!4ueHadUE hacesa ar{eeucoxHodg t4vvt vMHoLouAJ 4ouexrr rdg o -nrceneoc ouft{qrenfl4edrl{n eH frgcd,uli'eodx rquui(dJ et4xereuadrp *tl
'dee4eu,{gau eedar, uorleMgsrec <u[heHeeH . qrudoreou'xaody auHeaoualrcc!4 eoycahr]ouorgodxnn srrxuourtg e
'onueder oritrxorer{QHr n oruede.rcH€Jr,1c)ro srEhel{ o ' 14t,,lPh t4H t4ue !4 nlqHHeSOEqUOUCT4gH/r4r1rq HHeSO€Sr.rolrJar
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'naod)l or,iHea[uedeu srnredxedg o noHHoHanereH s cr(HdoE :^r,{arcuc olxxouelQxr n ueodx releu sru*g .
nr4hxead nelcahttJdsuue cBuexBI xsodx HhnHrEb crhxeoHln uEHquE]rde.DEg
-He
IOI
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'cueuuox nun qxodU'nnesarouu: uur uuexardguu-r poxsreuderde eurueeedoodrosg . eU
'oJor,,{hxdauoc r€Bh HaHanor,r nuh xetbxedeou hsodx rgyBlJ .
I
I LcnheHxnErHoy BeHquBtrdeDaBg
€H ,"odr rrre"
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I r4sodv sorHeHounov
I xratnoilegeerooco{qino},{ou c nodtsrr,rc-3glt rrrr,ey o
eU
@
i furcer'urodeeuor:xe'neeucoxuodg o
.UrnecodAQ errreHeeH peedl!4[ !4r4HexhHc !4du
:hrun OO[< aHBodA es eedf,nU qreerxdeEtou
I aExhxeed ueycehHJdeuue cBuexBI :Br4hEeuideJ-erey/eeadrtaV rrndorruoyl o
'edoarced o:ovc€hr4Jouo!4er4Q otr4eAQH!4 a/B grehpfi e
I4aody BxHesruadeu euHeuouro e oil4s y{oHHgHewereH s
aaH cr^Hd€s :^nercnc olIHHol4eISHr4 u neodl rexeu eruug .
olgv ewararxc ou qlro[uraxr$asoceH
| 'reodx eleypu oJoHquhseduer arxeeurede;_1 o eEr
ewelcrc ou qroolxrareflsoceH eH e]rHedeotfou
IaOEV
efneht,lrorHe4heu ee
qrEloiugeH h oqJ.codoyc femqHo,.'r c oner(QHr,r qJ.raoHgoeoa .
:-uo!,{Erehedeu crEV .
atnBh norHeuheu ee :oVeH 'l3^Ercs^h Bgec omodox moHque$o I
cl.euoiuqeH n oc.r.codoyc 4emrueur c ore[SHr4 cu-haoHpogog o leH rHonhEu t.r oHcur4gErc eltHBorcoc 'Co Z-] eH eual tcdfuedeuyler ans
.oHHoufrn a/s Jn g 1 xrneuaQdoux hrcoag . -emrqsou ,rur4 C" ge< pyuedoxr4u oysuor sc.reeuolugex erxeuheu r( nuca
quHc/lt{e InyJeU eeuuorroJ eeg Euhxeod UEHHoxeIOcHedr BeHqurdgao
PhIHshuedy ffi evvedoxIlJ'
I ot{geuc oyqrrol reeholrrs Exlrxeed
I
'r4sod, eh,Hrue pH a*HHecuuee'elHeuurhed ,"rr#:"'ffXiTt[X?"6?1iffiffiflS;.o$,i .
'euodoulay ornhEdfuEc cJ,uueveduo u 'shHexlqu
fuorceh '!['ecsu^u fuoIceh 'euorceholrxs 'drowco Iredrcreg q!4HuoutqE .
xsodx exHgsxuedeu qrnreclxedu
^dfupdouner
eyJ€r, {oHtsAdr 'xphmtcn 'xBrcoy s cuog 'ehxHeiluedy '^h,lu y r4sody |qsr4uhdu 'cxodts
'cueuuoy 'ar4eHeloui4r t,![rt4 srexetdeuuJ cEHcuP[deJ-de 'chvdeyrxE1'goHEo 'BytsBdoxt4U.
untueed [oHHoxEAOcHedr lodrco loHxoneoE rrBHerduy'q$.oru$ltC
68 I. ]4HHVS3UOSVe XUXCAhI4JOUOIVI,II:|J 614Hith3u tCulthHhdu
190 r fEMATO,lOfng U TPAHCOy3UOnOrUA
Hbre nonoxeHl'i
t ]lt#1,:tii,:;,",nf
. OrpaHrqrrelbHag crpareTilq neperilBaHiln opr4Tpot-lt4ToB
(Hb <70 rh) e$Qernaena He MeHbue, veu ru6eparuHar
crparerr4, (<100 r/n).
. EorsrrtilHcreo coo6qerrti B cxeMax reMoHaAgopa, TaKrx
SHOT, ornocsrcs r our6xau B npoqecce nepelrilBaHilg
KpoB14.
o HecNlorpq Ha ro qro rpancQyeraonno-TpaHcMt4co4BHbte
urSexqru cBrerorcl cepueanoil npo6aeuofi Anl naqileHroB,
KOTOpbTM npoBoA9T nepeflflBaHhe KpoBr4, oHr4 BcTpeqaoTc9
peAKo.
. Y naqueHroe c rpaeuofi iln4 oxoTaMu il y naqueHroB, nepe-
HecuJr4x onepaTuBHoe BN,reuJaTeIbcTBo, HeT AoKa3aTeJtbcTB
Toro, qro uHQyern aru6yr,,rrHa qeroBeKa ilnu Apyrilx Konno-
r4AHbrx pacTBopoB cHuxaer plcK cMepTil no cpaBHeHt4o c
rnQysuei Kpr4craJrlor44Hbtx pacrBopoB.
I nepeflr4BaHue KpoBt/ B HoqHoe BpeMc peKoMeHAoBaHo ronbKo
B SKCTpeHHbrX CnyqaCX
['lpr,trreraxne. SHOT cepuesHbte pucKt4 nepentaBaHrr KpoBt4.
- penapaLlil14,QHK (eronoeurg, gayuopy6raqran,
Xruuorepanuq
- sro rrpr,rMeHeHlre JIC Ars neqe-
Hrs 3roKaqecrBeHHbrx oflyxoJreft (ra6l. 5.21). Mnorue
reMo6JlacTo3br qyBcTBl,merbHbr K Bo3Aeft ctsurc xuMlr-
oTepaleBTI4rIecKI,Ix flpelapaToB, II no9ToMy XI,IMI,IO-
Tepanr4f, flBilserc . ocHoBorZ JreqeHr,rfl 6omruuncrsa
onyxoJreft cr{creMbr KpoBr,r. Cyrqecrryer MHoxecrBo
JIC, roroprre 4eftcrryror, roBpexlas AHK puu na-
pyrrrafl rcrretoqgrrft Mera6om,rsM TaKr4M o6pasou, uro
aKTrIBUpyIOTC.fl eCTeCTBeHHbre MeXaHI{3MbI alo[To3a
(Haupurrrep, TP53), rr KJrerKa yMupaer. Hecuorpx na
To rITo paKoBrre rterrcu 6olee rryBcrB[TeilbHbl, x[M]Io-
Tepa[Hs B 3HaqurerbHoft crenenu necnequ$uvna
u y6unaer KaK HeKoropble 3AopoBhre KIIerKlr, TaK rr
3roKaqecrBeHHbre KJrerKr,t. Oro npuroAr,IT K qacrbrM
no6o.rHbrM e$@erranr JreqeHu.s, TaKr4M KaK ilpexo.r.fl-
rrlarr He.4ocTaToqHocTb KocTHoro Mo3ra, BocntureHue
cJrr43rrcrbD( o6oro.rer rr 6ecruroAl,Ie. floA4epxcrnaroulax
Tepanurl naurreHToB, [onyqarcqux xtrM]roTepailyfio,
uMeeT perlaroqee 3HaqeHue AJIrr npeoAoileHlr.f, sTlrx
no6o.rHrrx s@Oerros. Lluenno sra rrogepxr{Barouil"fl
Tepaflvrr, BK[IotIafl npenaparbl KpoBu, anru6uoruwt,
flporrrBorp[6rosrre npeflaparbr, $arropu pocra
npoTllBopBoTHhre cpeAcTBa, [o3BoJrrrJra reMaTonoraM
AOCTr,rrrb Har4llyqml,Ix Bo3Mo)rcrbrx pe3yJIbTaToB B r,rH-
TeHcrrBHorZ xlrMr4orepannu (uartpuuep, npu JreqeHr,ru
ocrprrx lefixosor).
OcnosHrre npl,IHrlr{nhr x}IMl,Iorepafluu BKrroqaror
xou6unupoBaHue HecKonbKlrx HeB3alrMoAeftcrnyo-
uruxJIC no cxeMe, roropax y6unaer $rarcupoBaHHyro
.qonro 3roKaqecrBeHHbrx KJreroK nprr onpeAelennoft
Ta6nrqa 5.21. Ilprauepur ul,rpoxo tacnonbsyeubtx
i rpynn nporlBoonyxoneBbtx npenapaToB
B reMaTororult
Aaxunupyoque npenaparbr
KaK
CLuueator .qByxqenoqeqHyro,QHK sa cver 4o6aenenun
aaxurunoi rpynnu (quxroQoc$auug, uerQarau,
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Y Aerefi qaue Bcero [opaxaercrl celegenxa' Mac-
cI{BHOe yBenI4qeHI,Ie CeJIe3eHKI',I MOXer IIpI',IBeCTI',I K
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cg ceKBecrpa\uv c IToflBJIeHI'IeM crrllbHoro 6oreso-
ro cuH.4PoMa BCJIeACTBI{e pacTqxeHl4fl ee KancyJIbI.
Y uyxrulaH na6lroAaercrl TaKoe ocJIoxHeHI'Ie, KaK
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c ceploBrl.[Hox:rero.IHofi aneuuefi [apBoBl'{pycoM
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3r{rr sp}rrpoll}IroB. 9ro ntrgtrsaer rsxenylo aHe-
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lIecTBO peTI.II(yJIOUI4TOB CHr{XeHO.
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Ta6nr,rqa 5.39. CepnogugHoKreroqHae aHem[f,
npra 6epeuextocrn
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MeHHocrlr: cnegyer uuSopurpoearb o BoSMoxHocrul
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. Craryc BaKqxHaqxu: AonxeH 6u[u o6HoereH Ao Hacrynne-
Hrn 6epelaeunocru.
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(5 urhyr-) Ao HacrynneHilt Bo BpeMe 6eperaeuuocru.
14
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hxru6uropur Afl{D: npneu cfleAyer npeKparurb Ao Hacrynne-
nnn 6epelaennocrr.
. fleroqnas runepreH3xr: cneEyer l4culottt4Tb Ao HacrynrleHufl
6epeueuuocru.
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HeAOCTaTOqHOCTI4, qTo npilBoAt4T K npeSKnaMnchu 14
3aAepxKe enyrpr,ryrpo6noro pa3Btlrilf nloAa.
o Aqeruncantaqt4noBae Kltcrora (AcnrpuN') 75 ur: cregyer
ecei 6epelaennocrn.
npilH14Marb Ha nporcxeH14t4
. IlpoQranarrnxa rpou6oo6pa3oBaH[f, nocre poAoB: BCe
xeHu.l,rHbt c cepnoBilAHoKneroqHoi aHeuueil AonxHbl nony-
qaru HMI B TeqeHile He MeHee 1 0 gneil nocre ecrecrBeHHo-
ro poAopa3peueHl4fl 14 B TeqeHhe 6 ueg nocne KecapeBa
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pracxa BTe (cr'l. ra6r. 5.65).
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Hyro coBMecrttMocrb KpoBh no peeyc-Qarropy tr Kell-arrn-
reny. Kpoeu AonxHa 6uru orpuqareruHoil e orHoLUeHilil
qr4ToMerajloB14pyca.
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cepnoBlrlHorneroqnoft aneuraeft [oBbIIIreH placK
ocroxnenrafi , cBg3aHHbIx c 6epeueuuocrblo, BKIIIo-
qaq 6oresHenHrtfi rpll3, IInaueHTapHyIo HeAocra-
roirHocrb u rpou6os (ra6,r. 5.39).
IlafioparopHue n nHcrpyweHTanbHbe
uccneAoBaHuf,
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HerqxeJroe reqeHl,Ie - ypoBeHb reMorJIo6,IHa O6bIqHo
cocraBJuler orono 60-80 r/n. B Masxe KpoBI'I c paHHero
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 218 o IEMATOIIOTH9 n TPAHCOy3UOIi,OIUA

Ta6nnqa 5.40. fluarxocrl,rqecK[e rp]lrep]tlt Ta6nuqa 5.41. fl eqexrae 6onruroi 6era-ranacceuuu

i 6era-ralracceunn i
Sonsuac 6era-raaacceura (rouoourorcr) flpo6neua lleqeHue
. Tgxe,]aq n4noxpor,4Har aHeMr4r. Spurponosrnvecxar Allorerras TI-CK or qeroBe-
. npr43HaKr rrxenotZ op/Tpot-lrrapHoir, gucnnaalt. HeAocraro'{Hocrb qecKoro lerixoqrrapHoro
. Opurpo6racros. aHTrTeHcoBi,4ecrul,,roro 6para
. OrcyrcrBue !4ni{ 3HaqilTelbHoe yMeHblxeHile Kort4qecrBa iln11 cecrpbt.
revomo6rra A. flepell,tealre KpoBil Anq
o fioeutueriturri ypoBeHb reraomo6rra F. noMepxaH/e ypoBHe
. AaHHbre o rotvl, ero o6a poplrrelr ilN/eor Manyo rarracceN/ilro Hb >100 r/n.
@orileaas Krlcrora 5 lar/cyr
Maran 6era-ralacceMlr, (rerepoorarorul)
. IerKat
fleperpysxa xene3oM Tepanrar npenaparaMh xer$a
aHeN/ilF. npoTrlBonoKa3aHa.
. MrxpoqrarapHble runoxpol,4Hure eprrpoqrrur (6es ' Xerarnpyoqar repan/e An,
xereeogeQuqrra). BbrBeAeHrr xere3a
. HeiaHoro.l4cleHHble MilueHeBhAHbte KnerKil.
. ToqeqHaq 6asoQluran. Cnaexoueraaun, CnreHerrovilq; cra. ra6n.5.37
. flosurLuernaq Qpaxr4,rn rer',tomo6nxa A, Bbl3blBalol4Elt MeXaHl,l'{eCKUe
npo6neuu, qpe3MepHae
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e reuorpaxcQysunx
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TOnbKO npr,r He3ooeKrr.rBHorl repanlr[ xeJre3oM Jrer-
Ta6nraqa 5.42. Anearuas B noxnJrou Boapacre
KoIl Mr,rKpour,rrapHori aHeMfivt. fouoslrrorrr (6olrmax
ra.nacceuux) ly6o ne cuoco6Hbr cr4HTe3upoBaTb reMo-
rro6HH A, ru6o s ry.uxeM cJr) {ae uoryr o6pasoBbrBarb ' CpeAHufi ypoBeHb reuomo6uua: cHr4xaerc, c Bo3pacroM y
naqueHToB o6oero nola, Ho ocraercq B npegenax HopMarb-
oqeHb He6orbilroe ero KoJrlrqecTBo; nocne [epBbrx Hurx sHaqeHilfi. lzlcruHHoe cHuxeHile ypoenn reraomo6rua,
4-6 uec xr,I3Hu y HLrx pa3BxBaercs rflxeraq rpaHc- KaK npaBilno, cBc3aHo c Haruqr4eM KaKoro-To ga6oreeaHhq.
r Axelaus Hr4KorAa He Moxer cqilTarbct (BaphaHToM HopMbtD B
Sysuonuo saBtrcuMas. rlrfloxpoMHa s. a:aeMvfl . iluar -
noxilJloM BGpacTe.
HOCTr4qeCKr,re Kpr,ITepr,ru cyN{Mr,rpoBaHu n ra6,r. 5.40. . CrlanroMbt: uoryr 6unu He3HaqilTerbHbtMt4 il pa3B!4Barbct
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eBr'eHuA (ogurLura uoreru) r qepe6palsasre cun,lnrouur (rolo-
Ileqenne u npo$nnaKrnKa BoKpp(eHr4e u anaua).
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Cu. ra6l. 5.41. y
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xoprroHa Nrs, aHurvrsa lHK Ha paHHux cporax 6epe- BogpacroM t4aia HanAguA 4pyrux aa6oleeaauil n ae nenn-
MeHHOCTT4, qTo6br cAenaTb BO3MOXHbTM rIpepbIBaHLIe orcc HagexHbtMil noKa3arerfl Mr4 geSuqura xellega.
6epeueHxocru. Oro o6creAosaHr{e qenecoo6pasuo . Han6olee pacnpocrpaHeHHag npr4quHa geQraqrara xenesa:
ToJrbKoB ToM cJlyr{ae, ecJI}I u3BecrHo, vro o6a po4rrelx KpoBonoTepg il3 XenyAoqHo-Kt4uJeqHoro TpaKTa.
. Hau6olee pacnpocrpaHeHHas npilvuHa geQr,rqura BrrraMilHa
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npepbrBaHlre 6epeueuxocrvr EJrs. Hlrx Aolycrr.rMo. xpoHileecKoro arpo$uvecroro racrpilra Bo3pacraer B
noxhjroM Bo3pacTe.
I furuOa-ranaccenlrffi o llcuxoHeepolorilgecKr4e cuMnroMbt, cBqgaHHbte c 4e$lrqrarov
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paclpocrpaHeuo n IOro-Bocro.rnorl AsuH. B xpoMo-
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coue 16 ecrb ABa JroKyca arboa-reHa, lI [osroMy y npil orcyrcrBr4r4 reMaroaomrecxux Hapyueumri.
KaxAoro qeJroBeKa ecrb rrerbrpe a:l.rerrfl a.urQa-reua. r AHe[auq xpoHr4qecKoro ga6oreeaHlR: gacro Bcrpeqaercs B
. Eclra oArrH LI3 HI,IX orcyrcrByer, KJIuHI,I.recK[ ga6o- noxfiloM Bo3pacre us-sa pacryqefi pacnpocrpaHeHHocl4
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esrcHum<rrog I eeox4 eu yttrvleled Isnur,udll
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-uBH' urcorreesoroguc a runuur..ed anxcer.uQudroar
rcroruhar rJO'eooxger NoHtruoreuvrr rtoxcer,unodx
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xou6nHauurc pr{Tyr(crrMa6a c 6o;ree Mqrrori xr,rMrro-
repanueft: 6en,(auycruHoM r{Jrr{ nepopuurbHbrM xJro-
pau6yuurorrl. HeAasHo norrBr.rJrocb 6oree arruaHoe
aHrr.rreJro rrporr{B CD20 2-ro rr.rrra o6uuyryeyrvla6,
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Krerrur XJIJI 3aBr.rcqr or flaroJroruqecKorl u nocro-
sttuoit flepeAarlu CLIIH,UIoB qepe3 nylb B-rcrerOqHoro
perlenropa (B-cell receptor BCR). JIC, roroprre lro-
-
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Ka3brBaror MHoroo6eruaroulue pe3ynbrar u. kl6pytuttu6
uuru6upyer rupo3r{HK}rHasy Epyrona, a uaetatucu6
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l46pyr unn6 v vle ruwrclr6 :aperucrpupoBaHbr Arq
reqeHu-s peqr{AlrBoB XIflI, Ho, qro oco6eHuo BiDKHo,
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c M),rauuqMu TP53 Ha scex crailt4flxuBce qaule craHo-
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6yluuauu. Jlyrenax repafiur Moxer flc[oJrb3oBarbcfl
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sa6oresaxu s. t4rryr or un$exqnoHHbrx ocloxnenuft .
Ha srDKusaeMocrb BJrnfl ror nporHocrlrqecrue oco6eu-
.Hocrr4 reftxosa, B qacrHocrlr MyraUuoHHbrfi craryc
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s$(f err. Y naquenroB, rroJry{arcul[x xr,rMr,rorepan[rc
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arpeccuBHyro -rnu$ouy sHcoroft crerreHr{ 3JroKaqe-
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craprue 60 rer;
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lou lerlxoqlrroB, qacro flpeBbrrrrarcrlvrM 400 x l}e / L
Xaparrepnue KJrerKlr - 6olrurue luu$ouuru c
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(aHrurena nporr,rB CD52) [pr,r Brryrpr{BeHHoM BBeAe-
lluu6tut e@@errunen flpr,rMepHo y 90% tauueHroB c
T-nponuuSour{rapHbrM refi xosoM.
ayro-
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LuHeIro erB)rrr rentrodes,fifxery) excud oJo)6r,rH
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l-epunxaruausrfi qeHrp B-xrtero,{xuril Sorruryn

Ta6nnqa 5.52. flepecmorpeHmaq MexAynapognan
i uxana oqeHKlr nporHo3a npu MuenoA[tcnn?cT1,r9€-
cxnx cutrgpouax*
Maxrufixaq A$$epexrnsril
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cocyA
Kareropur Cymua Meguana Cpox Ao nporpeccu-
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Mocrr4r ner y 25% naquexroe,
Kopa
TET
llaparop-
Oqenu <1,5 B,B Her TI4KEIIIbHUI'
xugxufi 30Ha

Hrsxui >1 ,5-3 5,3 10,8

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roqruri Kpoaeuocubre cocyAbr
-
Bucoxufi >4,ffi 1,6 1,4 SQ$epexruuri
- rurrl$aruvecxrfi cocyA
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errcoxuI
Pnc. 5.25. Cxeua qnroapxureKroHr4Kr4 nnuQaruvecroro y3na.
*
Cucreua oqeHKh nepecMorpeHHoil Mexgyxapo4xofi PagluqHure nonyagqun nuuQoquroa H€uoARTCn B pa3Hbrx
3oH€x y3ra: B-xlerru a Qonnuxyrax, T-merxu B napa-
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-
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9 rraec y narlueHToB c BbrcoKrrM pLrcKoM, r.{ B BenuKo-
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rl,IcroJloruqecKoro IIccJIeA oBaHvrfl. 6uontata, KaK gro
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(FDOI). Eortrrur,trrcrno mauQou r{Meror B-Mero.rHoe
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y3na rroKa3aHa Ha pnc. 5.25.
[prr . Orryxolr,r BbrcoKoft crerreHlr 3noKaqecrBeHHocrl4
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JAtTCfl MeEJIeHHO, Moryt CyIqeCTBOBaTb B TeqeHUe
MHOTI,IX MeCrrIIeB AO IOCTaHOBKII AI{arHO3a I'I, KaK
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filcrororn.recKr,rM npusuaxou JIX flHrrfl.ercs HaJrvr-
.rr,re Kreror Pug-Illrepu6epra (n Poccrau ux na3brBaror
r.rrerKaMr{ Eepe3oBcKoro-Illrepu6epra .
- Ilpuu. ped.):
Kpy[Hbrx 3JroKaqecrBeHHbrx nuu$ouAHux KJreroK
B-rcrrero.rHoro [poucxoxleuur (pNc. 5.26). Onu uacro
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ra6,r. 5.54.
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i Xo4xxraxa (xnaccra$uraqus enn Ap6op) nplr orcyrcrBlll,I 3a6OJIeBaHvIfl vUrII Mofyr orpilKarb
Cragun OnpeAenexue
NHSrurrrpaqlllo HeqeHI,I. flpuanaru o6crpylquu
uoryr 6urr BbI3BaHbI y3naMI,I B Boporux fleqeHu.
llopaxenre lttl$arravecxotZ eouut (l) uln
ogHot7
. flosrrlrreHubtir yposeHb JIaKTarAerI,IAporeHa3bI flB-
ogHoro oKcrpanruQaruvecxoro opraHa unil rxanu (1.)

tfleTc'. ue6raronpusTHblM flporHocTuqecxuu Qar-

llopaxeuue EBy)( ilnLt 6olee rur',rSarurecxnx sox (ll),
ropoM.
unu oKcrpanilM$arnvecxoro opraHa, unl4 TKaHU no
. PenmeeruoepaQua opzanoe zpydnoil Ktemrcu Moxer
oIHy cropoHy (eutu:e rrn Hraxe) graaSparvut (ll.)
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Kaxaaq craAr4s AononHl4TerbHo noppa3genserct Ha 3BoJrfler U.{enru0prul{poBarb y3JIbI, CBfl3aHHbIe C
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llorepn vaccur rena >10%, npo$ysrute norut, nr,rTb craA}Irc 3a6oneBaHuq LI orcnexl4Barb 3ooeKr
nnxopa,qKa JreqeHrlfl (pnc.5.27).
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K nurr,,tQarurvecKtaM crpyKrypaM orHocer nura$aruvecxne
y3rbr, cene3eHKy, TvtMyc, Kortbqo Barugefiepa, anneHAuKc t4 Hrrrb xr,rpypruqecKl4 I{,JII{ C IOMOIUbIO r{pecKoxHofi
neftepoeur 6nRulru. nyHKTIHOHH Oit 6:1lOmC11^]1l nOA peHTreHOrOrUqecKI',IM
KoHrponeM (puc. 5.28).

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err71nou o6pasoBaHhu Ha MoMeHT o6paqeHrn K Bpavy. B
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r{ro orpaKaer
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Tnxecru Ypoaext Klunuqecxan KapruHa
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Ocnoxuenue repanuu QamoPanu
cBepTilBaHue KpoBu
no 1986 r. KoHueHrparu $arropoB cBeprbIBaHI'trI
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naur4eHrbl 3apaxuul{cb BIr4l u BHpycoM renarura B
14
z C. flauueHrbl c reuo$u.nuefi, [oJIy{aBIIrue o6'r-
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rro.tlBep ranuc b IIHaKTIIB alJlll{' Bldpy CoB, floqrr{ Bc e
uuQuuuponaHbl BI{pycoM renarl4ra C,y 80-90% ecrr
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rera rro craHAapraM B reMaroJrorurr (British Committee
for Standards in Haematology).
2. cibmtr.org
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KocrHoro uosra (Intemational Bone Marrow Transplant
Registry).
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i Ta6nuqa 7.5, [Ipr.ruepu Sapmarorenerxqec(rx pa3nnvufi, cnoco6uux lt3meHurb orBer Ha reKapcrBeHHoe cpeAcrBo

fexerr,{ecxufi npenaparu
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npoKahHaMuA 4o 50% B HeKoropbrx nonynell4ex
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uera6olu3tvtotr,t.
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nonynequ14 Eaponur,
fl oesrueHHsri pr4cK roKcrleHocru npu ceepx6urcrpora r,lera6oarasue (3%
eeponefiqee, 40o/o x(treneil Ceeepuoti ASpnxu)
Oxucrenre (CYP2C18) l1poryaHua Csuxeuue eQ$errnaHocru nph MeMeHHori rpancQoprrlaqtalt a aKrtaeHutfi
laera6orrr y naqr4eHroB c 3aMeAfleHHbrM t'/era6ortagr',4otr,4
OxucreHue (CYP2C9) Bap$apnn lzlseecrHo, vro noruuopQusM CYP2C9 Brr4rer Ha eur6op 4osrpoeox

Oxrcnerue (CYP2C19) Kronrporper CHrxeHue aKruBHocl4 Sepraerra npilBoAl4r x ocra6rerulto aHr/arperaHr-

roro e$Qerra
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vera6oruguou
HLA-B-1502 [HLA (human Kap6avaeenrr-r FoeurLueHue pilcKa rnxelbrx nopaxenutr xoxra (uanprraep, crH4pova Cru-

- rero-
leucocyte antigen)
aeqecx,4ri rer)rxoqrrapnuril
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(HalaHoro Bbrue B HeKoropbrx asilarcKr4x crpauax)
AHTilTEH

AeSuqnr Cyxcaueronun ilogn4 flogaelerue uHaKruBaqr4il npenapara npilBoAr4T K Anr4renbHoMy napanilqy

nceBAoxoIt4HScTepa3br (cyxquunrxoruu) h rHorAa x grrreruuoi ocraHoBKe AbrxaHilr, vro rpe6yer npoBeAeHr4q
ncryccreeHHoft BeHTilnsqun nerKilx Ao rex nop, noKa npenapar ue 6yger
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Oapuaxoguxauruxa

AeSrqur mtoxoso-6- fl penaparur-oxrcfiurefl , Pilcx reiaorilsa npu ge$raqure G6PD

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(G6PD)
Ocrpan unrepuilrrrlpyroqae
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floruraopQr,rsr,,rSLC0181
B TOM 9r4cJre npOTr4BO-
uarnpnilnure cpe4crBa
(Hanpnraep, xnopoxrH,
npuuaxuH)
IlC, rHgyqrpyoque floeurlxeFrue pilcKa pagBr4l4fi ocrporo npilcryna nopSupnu
aKrilBHocrb Seplaerra
Crarrsur l-loeuruleFrile pilcKa pa3Brlr4r pa6gorr,tr,tonr,rsa

HLA-8.5701 A6axaeup
florur,ropQr,rsrur fl oeutu:euue pilcKa KoxHbrx peaxquil il MnepLiyBcrBkrrerbHocrr4
[lornuopQrsu HLA-B-5801 Alronypuuol floeururenue pucKa noqBneHil, cbrnil y xaHbcKrlx rrrafiqee
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KilTailqeB

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Sarropa renarolllrroB npenaparoB
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Klerxu HER2-noroxilrerbHbrx Tpacrysyua6 noeurLueHile gyBcrBilTenbHocrr x uHru6npyrcqer'ay eosgeracreraro Ha pocr
onyxorei voroqsotT xe.ne3br AeneHue paKoBbrx xrerox-ulrueHei
t,r

[HER (human epidermal growth

factor receptor peqenrop
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pocra venoeexa]
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. qeccax, rreM o6brirHbre $apuaqenrrvecKr,re lpeflaparbr,

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ll Sasa ycTylaeT MecTo HoBbrM MlrIIreHgM, TaKr{M KaK cJrox-
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oTBeTOM, 9aCTO C CypporaTHbrMr4 KnilHilqeCKUMt4 KOHeqHbtMt4
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. llpoao4rrcn nocre roro,
-
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penra6eruuocril reqeHuq
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Ta6nuqa 7.15. Horsre repaneBrxqecKue anbrepHartaBbt rpaAtaquoH]tbtm npenapaTaM

R

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Moxoxaoxanuxbre aHTlrrera
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aure rncnorsr (PHK)
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l-exxan repanur
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Tepanun crBonoBbrMn KnerKaMr
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cHr{xeHr,re a6coruoruoro pr{cKa ua l%, Xora neprax
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KoHKperHoro [auueHTa 6olruee 3Har{eHI,Ie I,IMeer
a6corrorsufi pucr. To ecrr Korr,rqecrBo naulleHroB,
KorophrM HyxHo upofiru reqeHue (number needed
to be treated), 1rls toro .Iro6u uonyql,ITb [oJIE3y y
oAHoro nau[eHTa (no cpannexuto c [Jrarre6o), co-
craBHT 100 .rerosex. .flaxuufi upr{Mep sBJrsercs
unnlocrpauueft roro, KaK Kpy[HbIe Knl,IHr,rqecKue
I.ICCJIe.qOBaHtr.s HOBbTX [peUapaTOB Moryr.ueMOHCTpI{-
poBaTb cTaTr,rcTr,rqec Ku 3HaqL{Moe }r BleqaTJrsroulee
cHI,IXeHr,re oTHOCr,rTeJrbHOrO pI{CKa, U ilpl1l STOM 3a
gTuM Kpoercs BecbMa cxpouuuft pealrnuft Krrr{Hr{-
,{ecxllft pe3ynbrar.
I Oqexxa pexra6enuxocru ne.{exm
Hoarre JIC,{acro 6onee s$@eKTr.rBHbr no cpaBHe-
Hlrlo co craH.rlaprHoft repanueft, xo o6u.{Ho flpeBoc-
xo.rlsr ee no crol,rMocru. Ero.{xer 3.rlpaBooxpaHeHr,rs B
rax.uoft crpaHe orpaHuqeH, r{To He [o3Bonger 3aKy-
[arb Bce HoBbre [peflaparbr. Taxuu o6pasou, cJrox-
nue SuuaxcoBbre perrJeHr,rs AoJrxHbr flpnHr,rMarbcs
c yrreroM npr.rHrlr,Ifla eru.recxofi cflpaBeAnnBocrr,t.
OcHosHuM noAxo.uoM sBnfler c f, aHaru3 gKoHoMr,rse -
cxoft sS$exu.tBHocrn, B xo.ue Koroporo [poBoErircs
cpaBHeHr,re oTHocr,rTeJrbHbrx 3aTpaT r,r pe3yJIbTaToB
pasauvuoft .uesreJrbHocrr,r. AHarusH gxoHoMu'{ecxoft
eQQexrunnocru o6u.rHo Bbrpaxanr B BI,I.(e coor-
HOUIEHI'IC, B KOTOPOM 3HAMEHATEJIb _ 9TO IIPUPOCT
3.U,OpOBbs, a qucIHTeIb
- 9TO pacxo.tlbl, noTparreHHhle
Ha npupocr 3.rlopoBbq. OcHosHas npo6leua cocrour B
rolr,t, vto6u co[ocTaBHTb cToHMocTb BMeIIIaTeJIbCTB C
pa3HbIMI,I KrI,rHI,rqecKHMI,I ucxo,rlaMu. O.uga ueroAI,IKa
3alrlnqaeTcg B cpaBHlITeJIbHoM pacr{eTe oxr,rAaeuoft
flpororuKl,ITenbHocrr,r x],r3Hl,r c [o[paBKoft Ha ra.recrso
xr,r3Hlr (un.uexc QAL! na @one leveHr,rq HoBbrM rtpe-
 .XU}INB/fu oHsod^ wogqr€Hor{tr€H €H cclrEogodu Hedlc osJ,c -od
I,IC XI{)TCOhI,IHI{ID{
xrqHHeuoveduo s gusrcgov soroxorodu axlogudeed -Hr,rmqrog s u uwuxwoocddced u uwlqHxolrc rcrol os
g slgeoglceM ox)Igr roxow sorcurBrlrlouc xlqHrcew - rrsr uxHorro olrxcohr,rwosoxeoxBwduQ ur,e ec g
t4o
'HorpsxoEB roBslqg BXX
wouHohorsudu c lsluwox grqHsuuxoy'ruueueltudu
oJor{qrrB)rolr rftr onadgovo er,rHotbKodhd ooxcHl{rluEow BEJecs on 'rsdBuedu UHsoHlotsgru{sedc wtsdolox c cs
endolox'tqledeuedu or ox.rror rouhollols WqHquuxou 'uuusdel J,dEEHErc 'xuslodl-g iercedeoe oJogolr rrE srt
xE) EVJoJ'xruH?goHco xIqHHorBc uH eHudlc s HHah srcoHHarr ol(soxuHr4Eo rucoH (,LfVQ) HHEU:I< r,IrcoH EFI
-BH0BH S.r,rSg r;fuOW srSdO,rOX '3y enuHUeOdUd.loUled -suolllxuoEodu e uxeugndu olt''gcroEu,Ihc'xndorg rco
-E€ ocs rcrorusn€Ex^ woHcruHor,rlt€H s :(drrfwdoQ -oe leoHrodu ccJeBroV Ursdolox Bn 'I4t{owods BVoudau -r
HrqHqrEHoun€H UrrxcHuludg'dewuduuw) drr[ltdotp emqHor^{ oHHesrcoIn^c xrsdoJox srcoHqlfou{xuoEodu uo
guuuaoVeuccu xuxcohI,IHuID{ XIqHHBV EH IsHEsoHco soJ
UnHqrreHor,rrruH Uogorr won '3y xocuuc gr,rxludx eau '
-og .lrxdevoc drudltdorp grsuueelcdexou grqHqraroy or{hrqgo Hlulsr[eed o,le 'xtrsdeu-og 'r,cew xnwueerf rc
'CU xrqsoH or4Husooqrouc[ BH wecodueo ou rusemad oxqroxcoH qrco Bor,rrruHp oJor€ [ oxuuug 'gl'L'tgBL -e
laswuHudu N dur{ndoQ gnuueelcduxeu UrqHsuzxou g Hexsdro voxvou,lo.r,6'(a1y|/u.r,coueulxoQQe 't[
BC
lavee' 3y orr,rHohEHeEH ou r,rufrEtrHowoxed eHHsr€xor Uoxcohr,rwoHoxe eureue) osrcohux uu yoxauduou c
leuenlugedsgd u /fir4J,[rou or[Hsr€xou ressr,rudlc LrH0r,rx [xodc x r,Bdr,ee xlqHquerl4Huouou nrcoHsur -x
-rsg 'ouugEdu v€x 'uuur(fu u.1,6 '(ruueuuoulqs xuvw -xaQQe Er,IHomoHrooc rHeI,IluO@eox sl,ulur'ccud -[
eocdfcsd ur.rHeheucogo r4 EsreEosox^d oHeod^ Bu omKol { 'uglcEedc enHHolfl^[oHox€c la ls.tudJse olqHquel SB:
Breruwo) guuamed ruHedveHs oJor[ov(Eoucou rrE) -uHrouoE rqHrcosou uucg '(r.ldenc) (0D I,I (eseodore -c
c[HeHBdxoosgdEo rsdoxEoHow oxolpr E 'tqdlcoc ouxc ooHqrEoYu) <1> f[orew eHo0Eu€I,IE s I,IHoux BsrcohBx -el
-Hr,rlluuew u rersarrufl duQ' ruuocdeu {}rxcHr{[r{Eox{ uluaunuipQ€ox ouHesogquoucu rgoc s roehollols guu
gr,rmtrBur.r{ n gumdulc ruYoxs xrsdolox surcoc e '(ts.l -uHE rore 'rur{ohou oJoH.rdstrHerc euoQ uu u wor,Bdsu
-Heuesr,ru>r€ xu uvu) CU ou rqroruwo) ucrorowu rduHei
-xodM xrorcr{I,IrluEoH osrcHumsuog g' r,rHrrBYrroltroxod llygTeoduuuoU OOO O0 reuusercoc rBdrBe rrc
-il
olqHquou{HuouoE ucrolgslqaygar uuu vcruEoss'urflor{ -oneuyrerdu rodou xlr o1h 'oJor r4xHB€oHco BH guHeheu eogoH BO
-ou go)rcHr,rrru[oxl suHBsoducHeuuQ ue uwrqHHosrc +uaodrcxeuu$ eH rodExenoved u6 '1'lygTsodeuuoV 669 gy e0I
rugBJcoc rrcoxsuueQ$e t4ovcehhvloHov€ €t4t BHB oth -vt
-rosro 'uwuneJdo oHsod^ noHqr€)ou BH orc€h
'roBhpHeo orh 'sodBur/ou OOO gi E xersdrBe xrqHqugJ.r4xuouou
'uusrcuav e},
rdu ,ttVO t'O orqHcuelr4Hr/ouoU ree€r4houcego g elrHohou oJh
uosc qrusoHcogo Hogocouc quqg HoxuoE hedg 'Bg.rc 'IUosrqs v rcEox!4du cfiHexgdxoospdVo illoBugo E lcrcunoHove
rc
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(sodeuuroU OOO I L crcor'tnorc) AIVO 8'l= 9'0
'ruHohor ntrfi s eur(dE J,oruduEsa ousuercorcowec tauu norxeuhrS$eor c EUoJ xrcHqueluuuouoE udr q.uaxodu A
eoledeuedu xrqr{er(m{owoxod o*rgogewudu x ruHBsBx fuxeilhsu fu'rovcehurcutercexUedc rgcuoEeou E gr4Hghou . pa
-ouogurodu enHsrer{trt,tguEHu sler{u r/fuolt'fdowttdu i(sodeuuoU OOOZ qlcol\holc) A'1Vg t' l= /'O v'rol 'gc
x .ATqdoIo) .I4WBIHoI,ITIEU I,IWISHqUAVIO AMKoI,{ Yur.uu -xeuhu$Qeox c reu xrqHqueJlHuouoU xIsU gfiHehgi g qJ4x ro
fungrhpu,furoycenilrcilr.grcexUedc recuoegou ouH€hgrr o
! BII]
-oed slusodueoruodu .l,(lolrr os Hr,rfrgyrrowoxod'rc€Bg
:orh 'IUoerqe
UoHguorEoBxoE Bcuolrlolonu wosrcehEx lsHel'uHufuo YEI
v ruEoxrdu u 'r4rcoHquogBJ.xed euuexs rrceeodu ngou
s.r,rs g .l[.row u urcoHsrtrxetpQe goxcehulroHoxe ulehI 'ur4HBsoVeuccr,r eEox e ercxxeh,(uou'nrcotaleeguxqe erqE[dv fit
809 ccroruusErcoc orcEh oxEHVO'uwrqHHosrcohsx re,{dr4ueuon cuHexpdxooesdus ilrcBugo s rcufloHove '(%Og OH
oouog r,r ultnueedgooHr4Eo oorrog Br,rHohBHr?H sJEuoE c orrxexgsdc 0u %0r) qrcoxrdenc o,{lngo reaxnxc r (cen 7 I '(g
roleJowou u nehsds x ruHeeoged.l ex e 'tataltum{ewoxod c ouxexgpdc ou cen g;) ur4HBsguogBe crHE€odr,iccedJodu oE -s
cr,{ede egHUgdc repeuFr,iuee,{ oHquelhhBH€ (g) euxexeu eoso11
r,exdoEoc ser,cvoaoMd'xrqHr{EE rcxufnorowr,r sor,rrEHE r}l
oJoxcohr,rrur {orcuc oucou soJdoucxe gouudfu orqr{Huc BloHmol l4ovouceg oHhrqgo eorxeilhgu Uex ;. euxer,er e st
o:odorov eucou'ul,teIQxr enxxeendLfu e grqxrucgtrtgxg'Uo-t -ol
-I{uEI{'Bgl,cEogoMd erxcanugutrx s IqHohoIHg lltaued
E eodguuoE O00g crconuorc 'gr4Hoheu ooeoH :g or.1Hghgu r
-or gor{EorHowuxr,rVow ou }rrrrrBtrHowoxed eurouyAl BU
:uiluBdil spxquedodeu 'UoJ s sod
-BuJoU OOO; qrcov\r,rolc 'or4Hghe[, eoxrdeuxp$ :[ gfiHohorl o
gI
[rheEHouroved erHedUeHg I :uvmuv trorcuor. Bved crxeheu gEorev\ seU roreehxepdc Bll
aEor 7 oercoxsuerr4xuoUodu ulrxgEoEeuccr4 noxcehhHr4rrv E 'g
'(ufuowou rdoxcHr,rluEon ur,rHEsosrcHom
-desoc u cesodoEs rsHedxo &fuumHr,r HrrqHqreHourrsH urconeruxeQS" -q
Ioxcah[]roHove-ov]rHnux e]rueHv'gl'Z ehxugel
lf
I .E
.Lerrslcernl(co xu ur,rHuludgoxureg a'dewuduuu)
r.00 0 hl4HSHVdXOOgVdue S rl.l3$txu3Httrt n grctfSdc xtqHH3srgdDtSu ut4H3tnvdEo Sl4Hvsodt4u^Jtd
302 r HAflIIE)I(AUIIIE Ktll4Ht4t{ECKl4E HA3HAI{EHH9
HasHaqeHile npenaparoB
B Kfl t4HNLreCKol4 npaKTilKe
i{'
ffiWr%w*r'
HasHaqeHue JIC Aorxno ocHoBbrBarbcrr Ha paqlo-
HrurbHoM noAxoge no pflIy BonpocoB (cu. ra6l. 7. l).
I Jlocraxoaxa Ararxosa
B u.ueare Ha3HaqeHlre AonxHo ocHoBbrBarbcs Ha
loATBepx.qeHHoM Ar,rarHo3e, Ho n pea;rrnofi xr{3Hr,r
MHOrI{e Ha3HaqeHlr.f, AeJrarorcfl c yr{eroM BeporrTHo-
crlr Bo3MoxHbrx Ar.rarHosor (Hanpuuep, Ha3HaqeHrre
uuru6uropoB nporoHHofi nouurr rrplr AlrcKoM@opre
sa rpy4nuorl, Bo3HlrKaroueM [ocJre npr{eMa nrauru).
I OnpegereHxe qenefi reqeHrt
Ilelu le.reHuq o6rr.rHo rroHqrHhr, oco6esso
xorAa neo6xoArrMo ug6anutr naqlleHTa or cllMnro-
uon (uanpuMep, rrpu 6orflx, TorrrHore, sanopax).
B pa4e cny{aeB uerb neqeHvlfl He crorb orIeBIlAHa
An,anallr{eHron, oco6eHHo KorAa oHa 3aK;Iloqaercrl
n npoSu-naKrr,rKe 6yayrqux co6uruft (uanpuuep,
Ha3HaqeHr,re nnru6uropon AfIO !.r,fr cHl,z.xelp.vls.
v Allrr yBeJlr,rqeHu.fl npo.4on-
pr,rcKa rocfrlrTuurlr3atJvrvt
xr{TeJrbHocr vr xvr3Hur rtpu xpoHuqecxorl cepAe.IHoIU
HeAocraroqnocru). Bpavu AoJrxHbr r{Merb qerKoe
npeAcTaBJreHue o ueJrflx JreqeHr,rq, coHracHo KoropbrM
oHra 6yayr oqeHuBarb ero ycnerrrHocrb r4rru 6esycneur-
Hocrb. Baxso raKxe y6eAurrcs ToM, qro rrarlr{eHT,
B
Anf, KOTOpOTO HaMeqeHbr eTU UeJrU, pa3AeJrrreT Lrx c
BparroM (xooneparunuocrr).
I Bur6op repaneBrrqecKoro nogxoga
[nr unorux KrrlHr,rqecK[x cr,rryauuri ueAnxa-
MeHTo3Has r ep ar.vfl. He rrBJrrrerc fl. o6 s.sare rrrrorl.
flpuurauax Bo BHuMaHr4e rroreHrluuubHyro ilonb3y Ir
BPEA, BPAqU AO'DKHbI YI{I'ITbIBATb, OTBEqAET IU MEI-I",-KA-
MeHTO3Harr TepAflI,Ig VrHTepeCaM rralllreHTa r,r flBIIfleTcfl
rr.r xaxoft-ro o.qprH MeroA neqeHr,rrr 6onee npe4nouru-
TeJIbHbIM, qeM Apyrlre MeToAbI I4iII{ OTCyTCTBI,Ie BMeIIra-
reJrbcrBa (Harpuuep, Suslrorepauprs, rrcr.rxorepar:'vs,
xupypruqecKoe BMernarenrcrno). Ouenra coorHo-
rrreHr,rfl IloJrb3hr n Bpe4a qacTo Bbr3brBaeT cJroxHocTr4
vr 3aBncvrr or p.fl.[a $arropon, prMerouux orHorrreHr,re
K rrarlr{eHTy, ero sa6oresaHr,rro r{ Ha3HaqaeMoMy npe-
napary (ratu.7.17).
i MeAuKaMeHTo3xoi repanuu
. Tcxectu ga6oregaHr4g t/It4 ct/I/nroMa
. eQOercruanocrb npenapara
. Tqxecru noreHqilarbHbrx no6oqxurx gSQeKroB
. BepoqrHocru Bo3Hr/KHoBeHuq no6oqnurx eS$erroe
. OQQerrileHocrb Apyrilx npenaparoB u HeMe.q,4KaMeHTogHbrx
i,4eToAoB reqeHilF
. Segonacrocrb Epyrilx npenaparoB t1 Hei/eEilKal/eHTogHbrx
MeTOAOB reqeHilfl
I Bu6op npenapara
flo nan6oree pacnpocrpaHeHHbrM noKa3aHu.flM
(nanpnuep, caxapnrrft Aua6er 2-ro rnna, 4enpeccux)
npr,r Ha3HaqeHr,ru ecrb Bbr6op us xecxomroorJlC, vacro
npe4craBrsnulra( pa3Hbre xnaccrr JIC. Hecuorpa na ro
1ITO Bpaqu qacro 3HaroT peKoMeHIarlr{r,r IIo pauuoHllrlb-
HOrvry nu6opy flpenapaTa Anq cpe.uHecrarlrcT[rlecKoro
narllreHTa, rrM Bce paBHo xax4uri pas neo6xoAul,ro
nprlHl,rMarb perxeHl,Ie o6 aAexnatrocrlr AaHHoro Bbr-
6opa arq KaxAoro KoHKperHoro uarlueHra. Ha nrr.;
6op npavou fpeflapara Moryr BJrr,rflTb onpeAeneHHbre
Sarropu.
Bcacuaauue
flaqueury Moxer ygo6uee urorarb oAH[ neKap-
crneHHne $opMhr, rreM Apyrue, rur6o ecrn y nauueHTa
lrMeerc, pBora, To Moxer Bo3HI,IKHyrb norpe6nocrr r
lapeHTepiurbHoM BBe.qeHr{x npenapaTa.
Pacnpegenenue
Pacnpe4e.uerue flpeflapara B TKaHrrx Moxer onpe-
Aersrb nu6op flpenapara (nanpuuep, Terpaqr,rKrrr,rHbr
u pn$aunnq[H HaKarurr{Barorc.fl B xerrrl{, a JrI,rHKoMr{-
rllrH r{ KJr}rHAaM}rq}tr
- n xocrrx).
Meru6onusu
flpn rmr<urrx ga6onenanlrsx neqeHu creAyer ns6e-
rarb nplrMeHeHr,rr r{HTeHcusHo Mera6or[3r,rpforuro(cff
flpeflaparoB (nanpnuep, orr]rorrlHbre aHamrerurur).
Buaegenue
Y naqnenroB c HapyrrreHneu @ynrc1nlr noqeK rrplr
r.aruttuut iulbrepHarllBbl cJreAyer u36erarr npllMe -
HeH}rf, npenaparoB, BbrBe,{eH[e Koropbrx 3aB]rc]rr oT
ypoBHs no.reqHoft eKcKpeqllu (xanpnuep, A[roKctrH,
AMHHOUII,IKOSI,LUH).
-olr 'der{udluH) tlxuraHrxo)uwdeQ goHHoHeI lou ]ImI '(uunoldeurr
rnduoluex tH
xrirmrxou,( rueneroun.l 4ou<redfucor IIII1,I Vl1ldI,,JfaY r,rdu sHotr s eeud g eH € 'qHe[ a eed 1 mldu
evthvrrer' dertuduen] rturxeQQe I{I4)oehI,II^I€HI,I[o) -oHr,rour 61y dor,r,rgulrra' dev'rr,rdueu) ruuenexudu IO
-ewdeQ NtsHrruduoJelrgaH NrsHHsxdes[otr eerog rtogocouo I^uD{JeIr eelrog }Ilfl,l rranegevuradlr 4ol^[oxo -0
rruirg rexon rHeI{fIBIr Evlox 'etrloJ oHHegoco He)(Bs r^lorcodu c IsJ,eduuadu sredugna rer([arc nur'edg ud
troxtrox grsHxodo.t co uioxel'I,Imor.Il{trox9oen adalu ou
oM H AhOU e tHonn eu s JJ/IH H ax4doa ndu
ee qrusofur4J oHHertren u eredeuedu Iseotr Ilto)cI,IH c
eI{HehoII irrSHI,IhBH re,{[orc xer.eds 'ornauedu xe;4 '( u xer,'or,fitrduen xan
1 1' 1' rger no) onxeao[edcouo
tctB0il ouHesodtu! srpsosrcgetrowtless ilJoI I ersdolov '3y ruueuenudu b
oJoHHBJehoc runenanrudu <LIEJo9oI,I HIDKror unudg -3
'exI,IH IilH€cuuo'qlego[orc Jl{orc
a ueJJiaailoN u ees ruq H H oe tcd eveuxes
v{rsdorox'rpIu€soduootr evtrxed edoqtsu udu ltloxtrou
xtc,, HGtu ote u ov@ H o u H.eln e ds totredu
orqHsoHco'sJ,usotrodeugo oxx,{u'}Ircol^lutroxgoeH
udu'orr(dorox'utlfier'IdoQuu 4ous[eJuHlrouoV Ne 'el,lce UIoHiITB[xHodg tldu as os 'Bxr'otrfuex -
-uner,rtrou c onnowedgoHtro rHel.'w{dellcxe )€) qrus Iq
oJoser ecodglsu uurrxedQ goHHa)Icrll{o uut ulreu udtt
-udr,ewcced r,e,(trerc eI,IHeh€H€€H oogollr rI{HohBHegH
dolexorgoued['u-$ Hoh€HE€H quslg Jexon olqHoorog
oJoHirreHourl€d I4n€[Ilrlsptdu c rlr,rarcrosJooc g
'r{Isso)3HI,I[O Eo)cehllHordeuu: c wurHeI,I[€II 'uuuedel- x NeIlHse
-€)orrogurodl uwt WoI,IHso€)ox s.tlvq .tr{..Iorl ung
quirg Joxow oH €YJox{H 'srHoullerr o.ronredxrtox rrtr
xctld-uesuou ol,IHemoHrooc nIqHlIIrEI llIr[o ro€retrc 6 U H e eO UOge e enln u le n tlu ox
EIsdolox 'r'lrxed 4ramn trueg oH 'sorHel,Ifiett notrcl,Ih s
Numqlrog u0 I,IuHetrolrgeH I^loxoeh]Ircl4.r,ercantredc eH '(urog goHqlrl{c udu uuQdolat 'tlouuucueluueu e
-
erirHHesoHco'oturtegoduootr ou plurletrHerato;red .roreg udu lrolwrerredeu' dertuduus) ruuuaerogso I{rcox -
-
qresosrcresJooo Hexlro[ e.tedeuedu doqtsg
-nl.egudeud urer,[Voaeuodg'aolxetptp€ xllD{cehl{c)or -rr
rcsrlelqs eg 'I,Igemul.,rl-]IH€xr BH eusJcl]retreos eoHhor
BnHesouogee qtcov$t
-emotr <Ir€sl,Iheucego le[,{g redorox'rEu[redurelrnox
goeurr4o.tcr( oJa shl,Irco[ efifflolrroseou'eredeuedu '€JHoJ€ o:onnonlnreQnn
(runuaodueor ltnxad) rrananevuudu s.LcoHledx u
I{rcoHqrerusr,car{r' tSolteereroutredu I4II}I 4OHrrOsepI uH
enreudu gocouc ',(eotr sr,edglss Hex[oY hedg
rcir.rusrrsoHco exroro[ rudolox' uuuedoJo)I,LtoI'I9I'IrHe
crHeeodneoE e]Iurxed doqrg I edogrsa udu dolxeQ UlqHtrusoho aalroglleH 016
'$'7'ugw 'wc) uuuedsr 4onnearcdoler dogt<e 6wHeseuo9ee unoH Hegoco
€H reBI,Irs uI,IJoHeJ BtsJox '4I,IrIBfuuc xr,rxcer'I4HI4IDI olr
' (goltrce goHirlruIlxHodg c aolueurluu .{ uutrduxoser,c
-clrh eomqrogeH rcreonrr uHaweds rfi{emrorceH )
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y q$osr4hy!r4r4duco8 :t4J.coHholpJ-couaH roHhehou Ht4lt/808d
uru ruduuedu qresoduourogerow r,rHehorr r,rJooHgoc g
vcr4d r4rcHHomrc8ou <- yohou r4r4hyH^Q oilHoxr4Hc :CgllH - -ouc rDrHefro rftr sorcer xrqHr?s>retrB le^{alcefndc e11 -
BDoQO€ ojoycohr4cyot ut41t4s0Bd ycild XtcHHamtceou ,
<- Br4eillIogeroy! OTIHOXI4HC : Ht4rrt ilQOOr' UOTIOHEdUOdU
'(uneneu ecoddufi zdu'deltuduun) runuaodulnfm
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Br,rJ.rEeBd vcud rrcHHomrc8ou <- BneiluogBJ-e,I ouHexilHc
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-BJ.coJ.do ur,4lilseed rcud 4rruuemrreou <- eodoruehedodeg
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Bcraexr: Fitzpatrick J'E" Morelli J.G' Dermatology secrets plus,
sth edn. philadelphia: Elsevier lnc.; 2016; Lawrence
color atlas and text or ohvsical slsns in dei;aiEbiv. r".,i*'w;ir;i'ibbl;'ilo1s9_s c.M., cox N.H.
transmitted diseases anb Ato^s, +rr"eq;. d;,il;,
Eisevier rnc.; zoroivoorejr.i., r-ri.n [.,
aair;d fr.b:, [ii
, et at. A,as or sexua,y
v"ri.eila pneumonia rn an rmmunocompetent
adult' can Med Assoc J 2012; fia(l: t02+; aor;g;" J.1., s;iafi;r-i.v:d;;, K.o., et ar. Dermatorogy essentiars. phiraderphia:
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Hueli .rarue 6rrsanr npexAeBpeMeHHbre poAbr,
qaue poxlaroTc, He.qoHoIueHHbIMI,I.
Ta6nuqa 8.9. eruonoruqecKue Qarroput nHeBMo-
i lfl,il,t Bo BpeM, 6epemexxocrn
Eaxrepuaruxure
. Streptococcus pneumoniae . Haemophilus influenzae
. Mycoplasma pneumoniae , Staphylococcus aureus
, Legionella
Brapycxure
. Bupyc rpunna . Vaicella zoster (enpyc
eerpnroi ocnur)
flpegcraanexo no Marepnarau Lim W., Macfarlane J., Colt-
horpe C. Pneumonia in pregnancy. Thorax 2001; 56:
398-405.
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flpegcraanexo no Marepilanau Ateka-Barrutia O,, Nelson-Piercy C. Connective tissue disease in pregnancy. Clin Med 2013;
131:58G-584.
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340 r EEPEITIEHHOCTb

2. Royal College of Obstetricians and Gynaecologists.

Thromboembolic disease in pregnancy and the puerperium: Be6-cafir
acute management. Green top guideline. London: RCOG; H auuoHa,'I bHoe orne,'leH l{e
I . npeu.ox. ac.uk/mbrrace-uk.
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344 . flYEEPTATHbIM NEPUOA H NOAPOCTKOBHfi BO3PACT

Heo6xoAr,rMo flpe.qnpuHsrb p.s.q maroB, Koropbre rrpu- Ta6nraqa 9.3, Knro,{esure noattlt4r, no KoropbtM oqe-
BeAeHhr B ra6r. 9.2. fleprurrr ruaroM flBrrfl,ercfl, pas- HfiBaercn roroBHocrb Monofioro qenoBeKa nepefirn
R
pa6orKa rrpr{HrlurroB KoHcynbrr{poBaHr,r.s MonoAex}r B crlcreuy Meflnqr4HcKoro na6nrcgexun Bapocnbtx
u o6yvexue,UaHHbrM npuHrlr4naM MeAr{rltrHcKoro
nepcoHzura. 3areu Heo6xoAlluo paspa6orarr c[creMy 1. Onucureaer ceoe ga6oregaHile, ero Blt4nHile Ha ggopoBbe il
BbIflBJIeHUfl [arIr{eHTOB, HyXAaK)uIUXCS B nepexoAe r,r3 nporHoS
2. l1ornuaer qenb HaaHaqeHiln u eQQerru npenaparoB
Ta6nuqa 9,2, Ocxosxue eneMeHrbt npu paspa6orre 3. floruuaer qenil Ha3HaqeHiln r eQQerrut npoBoAxMoro
JteqeHil9
i nporpauMbr MeAr,rqnncxoro xa6nngennn grlf, nepe-
xoAHoro nepuoAa 4, SHaer ocHoBHbrx MeAhquHcKt4x corpyAHt4KoB ra rx QyHrqnr
{Dopuupoaaxue crpareru[ nepexoAHoro nep[oAa CauoctoRtenaxoctu
. Pagpa6orra crparen4,4 c yqeroM o6parnorZ cBe3r/ or 1. Moxer coBepuarb Br4gr4rbr B KnilHilKy no,trulnorHocruo
MOnOAexr'l caMocTorTenbHo
. O6yveHne peanil3al.ll4t4 gatrori crparerilu r/egilLlt4HcKoro
2. 3Haer, KaK sanilcarbcfl na npreu/cxoppeKmpoBarb BpeMe
B143t4Ta
nepcoHaIla
3. f'loHnuaer npilHr-{ilnbt xoH$rgeaqnaluxocru
Orcnexueaxue x MoHr4TopupoBaHue 4. Moxer gaKa3brBaTb noBTopHbre peqenrbt Ha npenaparbt
. Paspa6orra npoqecca BbtnBreHils naqr4eHToB 5. llputruuaer xexoropyn/nolHylo orBercrBeHHocrb 3a npreM
. Oopuupoaanue cucreM orclexilBaHun t4HAt4Br4AyanbHoro npenapaToB 14 ,rHbre BilAbt re9eHilg
6. Sxaer, rge MoxHo noryer4rb noMol{b
nporpecca
. BsrcqeHre 3anporpaMMr4poBaHHoro nepexoAHoro nepr4oAa B Sgopoaue u o6par xusxn
cilcreMy oKa3aHrln ueguqmncrori noMou.lt4
1 .'ilorruaer BOKHocrb co6ntoAeHilF guerur, SreuvecKhx Harpy-
forosxocru K nepexogHoMy nepuogy sox, reveHnn ey6oe
2. lloHnuaer BpeA KypeHlar, aJ]KoTorF, HapKorr4KoB
. OnpeAeneHne noAxogeu.{ero MeAyepexAeHur Mfl oKa3aHlB 3. l]oxurr,mer npo6neMbr ceKcyaJ]bHoro gAopoBbr, 6epelaeuxo-
noMorJlr,l BSpocnbrM
crn, uH$erqrfi, nepegaBaeMbx nonoBbtM nyreM
. OpraHil3aqilf, gHaKoMcrBa c xolaangoi rrlegrqrtcxofi noMol{t4
83pOCIbrM floacegxeanae aKTi,rBHocrb
r flucuueHxoe uxQopuupoaaxhe o npoBeAeHru nepaorl
1 . Cavoo6cnyxneanue/npuroroBlteHue egbt
cogvecrHofi KoHcyrbraqr4!,l
2. CavocrosrerbHoe nepeABrxenue/nepees4ur
flnaxupoaarue nepexogHoro nepuoAa 3. l1oesgxn, HoqeBK!4 BAan[ or AoMa
4. Iurorur, Bbrnrrarbr, SuHaHcoaan HegaBilcr,4Mocrb
. O6ecneqeHue o6parHofi cBesil MexAy neguarpurecxoil
rouaxAoi u xouaH4ofi MeAlLlt4HcKoro na6logenun mpocnbx BpoQeccroranurure Sarropur
. Onpe4eneHhe norpe6xocrr B npoMulfrorHofi KoHcynbraqrit4 1. Texy4ee u 6ygyqee o6pasoeaHre/unnHile ea6oreaalvte Ha
t f'loAroroBKa nucbMeHHoro Meguqr4HcKoro conpoBoxAeHilg: KapbepHbre nnaHbt
- ,[uarHos 2. floceqaeuocrb 14 ycneBaeMocrb B uJKore
- Texyqee reqeH[e 3. Onur pa6orbr 14 Aocryn K npoQopuexraqrr
- ,Qannure aHaMHega 4. Bxeyve6Har AeqrerbHocrb
r 3a6aarospeMeHHaq ornpaBKa cooreercreyoqefi
5.
14 r4HTepecbt
Pacxpurue ur$opuaquu ulxonuroi agunHrcrpaquu/
ruQopuaquu pa6orogarero
. flpeAocraerenne Heo6xoguuoi rx$opuaqru il nolAepxKa
llcuxocoqnar uxure Saxropur
Ilepexog B cr4creMy Me4uqytHcKoro xa6nn4exnR B3pocnbtx
1. Cauoyeaxenue/yeepernocru e ce6e
. OprauusaqnR nepeoi xoucyluraqtr 2. Bocnpunne csoero reralcaMooqeHKa
. CoeNrecrxurrl pae6op nepegaaaer',1oil nrQopri,laqrr 3. Oftror.reHilc co cBepclHt/KaMu
o BurssreHile nilqHbx onaceHrti r rpeeor y MonoAoro qeroBeKa 4. llo4gepxxa aKTilBHocl4 B cor]ilarbHbtx cernx, o6qerrn c
. BurscueHre npr4ophreroB 3AopoBbq y MonoAoro qeroBeKa ceuueri, 6rusrnx ornoueHufi c ApysbrMl4
. O6Hoerexile MeAuLl4HcKoro pe3toMe u nrraHa oKaaaHuc Heor- 5, Crparerrara npeoAoneHue rpy4xocrei
roxlrori noMotlrl
flepexogxufi nepxoA
l4rrerpaqur I cxcreMy MeAltrytHcKoro xa6nngenrr aapocnbx
1. flouurrraer KoHqenqillo nepexoAHoro nepiloga
. Konrarcr c neAuarpoM r,1 noATBepx4eHhe nepexoga 2. Comacex c nlaHoM nepexo4a
. [1ovonlu Mono.qoMy qenoBeKy B non] .{eHilil Aocryna K ApyMM 3. Bilsurbr K BpaqaM e nepexo4Hurri nepraog (e KnvHuKy pie
[/eA[qilHcKr4M ycnyraM naqileHroB nepexogHoro aospacra)
. llpogolxeune ilHgr4Br4gyanbHoro ruraHa na6lrcgenun c 4. lloceqeHre epavei rs Me4yqpexgeHun 4nn espocrulx (npra
yverou norpe6Hocrei uoaogoro eeroBeKa neo6xoguuocrr)
. Tlory,{eHile o6parnofi cBngt4 or Mono4btx ao4ei o cocroflB- 5. floryraer nepBrqHyp MeArqxHoryro noMollb, o6paulaercn K
uJeMce nepexoAe
ApyMM CneqranucTaM caMocTonTerbHo
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oru.lugeud le,(amgocouc'Bleuoxc .r,cod J,gesuhuragf uc<rr?HuheH He)Kffotr troxede11'lcedeoa 4Isuoxrcodtrou
unode.lco.lcel rngodr( eI,IHemIqsoII'sotruoeorul ldorr, Ir wore€ e'vusesedaoc oJosoro[ troudeu rehoIIDIs
fxr,ogzdrra rr{dur,{nnrc'uIrorde3 ntlarol uu lefalc4etr 'Loueffid or4Hehar s J,u[oxcuodu ccerlodu rore 'IqHar
-eoa Holrrdo.I guhor^dur,{lru.r,corfxnrruoQ u 'xexnur e -etreduo oH oxJeh etroxedo[ totodc elqHqlrPhlurllo
e ra[UIII uruuxremr eHodercoJcer eorsllc .r,eddunfi ^ulr,c
enorrdo.r oJatnoild[s]IHl,IeJollr uxrogedlsa oI,IHehI,I[ aBHHouo{ugEH
-eg,( goxlahsrpn A 'noenQourr znoltdo.r o,rolnoildur ot0u30de8 I Bt4H0u0{u9EH
-,{lrtu.t cor,{xurroQ u enol^tdo,t olarnoildue}rHl,Ierolr oJovcohhdlE[uau
^r401cr43 lqNarcils 8n
euHomKogoscna (oueedgoo)hexc e)olur) ra,{dunftttu,rc Uoxodau t,tr[osrodu HaxuoU uUloy
sYedaho orosc s 4rsdo.r,ox 'lrocrfi^telr€lounr enoltdor
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o rrunel^IdoQnu rungodtroll' (<rnfievudoQnu rensrer
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 346 o nyEEpTATHHfi nEproA il noApocTKoBb!fi BOSPACT

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(3anyCKaOT" nOrlOEOe
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npqAcraTBnbHoi x6ne3bt
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t{
a suqx[xe eonn[Kynocrr4Mynr4pyor4r4fi ropMoH Bo3Aefi-
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reK[ xenroro rena, crl4Myrlt4pyf, BHpa6orKy nporecrepoHa. B orBer Ha noreuHyr3r4pyroul;ft
rdprvrox -er*, re*, raKxe B He6onb-
tlJl4x Kolll4qecrBax npoAytlt4pyDr aHAporeHbl (He noKa3aHo), 5 y Myx\.r4H JrtorehH;stapyoqr,ir ropn,lox aosgeficrByei ;.
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cruquaflbHble KnerKh J]efiAura' crl4Myrlhpyf, Bbtpa6orKy recrocreboH;. ooanrxynocruMynxpyrou1ufi il;6-
ropMoH u recrocrepoH Aefi-
crByror Ha KnerKfi Cepronu, cruuynnpyf, cnepr4aroreHeo. Bsero r,'is smith n.C ruLtter's
ol,stitiicJa.o iinecotogy, 2nd edn. phila-
delphia: Saunders, Elsevier, lnc.; 200g
-ou oJo)Er 4l,Isrctrorco[ xlqHqlrBilrlHeroll oI]IHBI^II,IH
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u urcoHsrs,,(c)oc Isr lorgodu rcrorrncrodu or,cedeos
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BHOI^IC II,IVOXCI,IOdII rilH€HeOU rUnOde U)IhOr )
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uEoff erqEo[onr 'froHoxEH 'rscoduos xr4 oulflol/(cederHu
srugnr,{^{trodu urcoHgocouc u fircoHqwrgelic IoHqrBH
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utrom erqtroron alcedeos vrosoxlcod[ou neH[oorr g
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u lorercodes utrorr ontrouon xE) oJor edelr o11
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oroudemreru) ro wolnorercro'elcedeoa wourcox ud11
' ys.Lcrr.re€, oJoser ouQzdlou e:lHod sresocqrrorr crr
ouxolt ercedtos oJoHJcox u)Heno rltoxnor rr[''ercod
,(xulresutr rerlrtrer^re€ oxxur gotrI4oxr.rudo)o)orm r^ro
-udll uvtyulJBserog€€ HI IIqHqIreJI,IIrBUcos uIltH)cohIrH
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^ nklcgocouc'xlrHrrt,tr eH J,ol(alc4o[0os rsHoltdor 4urn
er.rHesotrorcgo rcsruaogedror roxon'rlrtroxcuodu on
eunesedeoc oosotrou funenorr'r,(yuore x urcA 'enedrc
LFA t VfiJOlIOlt8l4o 14 BUnOIVHV EVHqWHOl,thyXlO
ou qroruo?xou 4nutredc oeIrroremrqsedu runenora
-lo xnsrdutrnetc g'7 €H r^roh eerog 'ruuerruir,ceu ora
urods Jorer luHorr rnnesadeoc oJosorou 4oxxdantt
[o11 'u[or,rdeu oJonleJdogfu frur'un r$^roHHed oollog r
J,ta[ost,tdu rt,tHed n<o sJ,connanedr,coduced n rrrHguflr
r,rrcoHhouqgou ) r[rrHevuel'(emsned reeufJ,ceH
eunegedeoc eosouou Brror Utocc?r[ [oHHomrqsou c
UoJeE' ^,() elrel ecce]\r u (4atewnod I u.r,zldeg(u ottsh
-un) eeuruene UrqHUoI^[ac Jorrurs r,rHouoJc 4omqrog
I
s uarev rfineaedeoc oJosorou rnsornrfucuH lcedeos
BH 'Hoo€ueutr 4rxodum lrHoho rorerlr ruHeaodeoc
oJosoroil uxodc eHHsIrBndoH oH 'goxllhqlrq-v\ lrtIII
LeItr zI u xehosev rIrE rer 11 oudeltudu roErsumoc
(uuuewdgo)r,r(og e) ruueaedeoc broaorou Errph?H
rcudeos Ur,rH['edO '(9'6 'cud) edennel orulexuQuc
-cen rol(esrorrclt 'rfiHegedeoc oJoso[ou Eofideu s
xumutroxcuodu'4unenefi0fi xr{xcenreuQ r}ilresod
-n.r,nelt[xo[ Brrtr oxrrrxudrr {o)coh}rHr,rrx g 'exgotf
?H rqcoros urced J,oreHr,rnen r emod xohzxc laftarc
orodo,r,ox eucou ')ehutr r}IHeh}Ireg.{ c rcreEHI,IhBH
euHegedeoc oosoroil Eo)rrhlrrpw 6'uufiefdlcnert
rcror€Hr{heH oHhngo elcod l,trcodorc oxt,til EH 'Iqcor
-os elqsoxgolr.raJ,ced rorgHl,rhgH'noorox olirHholron
rcroIBsI,Is0Bd xru f 'Ercod exumEHI,IV ou soxI,IhgIrBI^l
rorcxedouo flxhose[ ulrJ,useed lroxcenueuQ g
'HWe,(es)ctredu onnolelcotr oHhrsgo oH'Iqlot<orc eJ,ced
-oos orosoxrcodEou mrre[asoil rr &rllHorru(rno runenelu
-e7 'eodorxeQ xrsn&fu,srfxourroc nor,rHn,rua c ercrer
e 'ruouH€sodooc nn{cohl,IeuQ c nHeerac nIHeHeI [0rI
elrxcehl,IJolroxllcu I,I elilHqlreHor{fior{e' ruHercodea
rxeneudu entcehr'f,Iolfoxllcu I{ oIqHiIIrpHoI,I[on€ oIDIod
-fim oorrog u os'lror,rueaedeoc wrqsororr c orqHHuersc
'trI,IHoHerrofi emtcenueuQ oxqlroJ oH rgoc s ro€holnls
'etrofideu oronleldegfu ro er,rhr,rruo s 'lcedeos {rnsox
-modloll'ercedoos ltrosoxrcodtrou s nrqnostrorrgBu
' r{trI,IHeHoruou }tu)cohl,IJolroxucu l,t ltuxcerru.loroQ
- don' ltnxc o hl,IJolrolr g .roilalcgo collc rI,IHe HeIlt€]r
errHirreHor rdo1 'o6gE us osdewudu rcleeslnnruea(
r}{Husedeoc oJosofou Eoudou g eHurfcuu rufoiltrod11
'7 u 1 elcod aodoueQ :nsugotrouoHurfcnu.{xrogudne
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u IIN€xI{Hhoho[trBH goHe.IodtrHe BterHI,Ic euner,uroe,(
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u r,tulrufgo oll{HelrsoHalc' golrfxurroQ olnr,raeed
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no6xoeoe Orcyrcreyer Pe4rue, An4HHbre nnou.Fflb, qeM y B3pocnbr) pacnpocTpaHrloqt4ece
u np9Mbre Bolrocbl rpy6ure u 3aBrfrbre
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l-exuraaun ,[ony6eprarxure MOTJJOHKe KpaCHeeT 14 MOIUOHK
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pacnpocrpaHelor4uec,
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Be!.eHvrs. Nrs. ux 6y4yu1ero 3AopoBbs u 6raronoryrua.
Y uoloAexu rrorrBJr.f,ercfl 6omure Bo3MoxHocreil !.ttg
SopuynuponKu rr{rrHbrx uerefi, oxu yrenflrcr 6omure
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npexHee 3HaqeHlre. K rouy npeueHr,r, KorAa MoroAbre
nIoAI,I BbIXOAqT U3 nOApOCTKOBOTO BO3paCTa, OHI{, KaK
lpaBI,IJIo, craHoBqrcq 6onee [poHI{uarenbHbIMI,I rI
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lepcneKTlrBe. I,Iuesuo pa3Burrae erNx 6oree 3peJrbrx
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ur,roHl,IpoBaHufl B paMKax Mo,uen[ [peAocTaBJreHl,lfl
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npaBrrJro, cql,ITaercs Hopuoir .qag xax.qorl craI'ilvr
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nnft n gMour,roHzurbsoft n nclrxoJroruqecxor? c$epe
Jre)rorr B ocHoBe noAxo.qon, ueo6xoguMbrx,urq reqeHurr
xpoHurrecK[x sa6orenaHuft y uoapocrxor crapruerl
nospacruoft rpyrrflbr ]r MoJro.uexu.
'B0oHI/'IEHB l,IrcoHHegoco il uu?roE erlHqlrorlrHlroilo[
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-rqs elclBr € 'Susoher eeln(xel u eened rcoems}Itros
- odu' eoule uv lpt4>tce:.finur:r rcla,(duIrfndoQ oxlen
4odo.rox a'(oltscuu eoHilrerutroaoduoc) €xcfiulqs
r€)cHr,Ifiuvelt rcle.{.dultdoQ 4er,eda xrsrcodes eI{He[
-orug€H vou Bxesoroh oJo[oror{ ovosodeu
' rerufu c oJoxcohl,IHlalrx
o.ronledxno) rI,IHo[oIrg€H IqcHsoIH ecs qru[^cao
oHhr,Iu mer,edg
rorroatou orh'IDII'IHUTX 4OUUeAodue
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e[otadoIr ltontroxodeu s BTHoI,ITIBI u eheds er'edrcs
resdeu orceh 'uHer,leds omqrog rcle,{.gedl finadr,cs
1lore ruE 'orusBdu x€x 'u 'ltfitruelrtvod nfiuogc oc
.lrrous rJrirrcodeu 4uedeu €H rrEoxudu ouusgo ule['
'BrHOurI€U 4er,COUgedr,ou lilHerlo u rl'IHesotrorcgo
xIqHHS['eoeuw?H€ 4ercoHHegoco xlqsehollt) YuHeIl
-etreduo rrtr exx€r E 'wolHol,trtgu u r{oheds d.firelt
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lgrr 9!
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fhHaheHe xilvocrge
g ucteBxilHc sxesod4
ucroeFilueed ovquovceH
vemleeU x {omoHo{ [ BursrrBedY
ruhedruehHov uuHeeedeoc o.Loeouou oU
:hmrqv! xrcHlauovc omileeed oHcueuupdeu
suHBeedeoc oJosouou oU BercehHeE?un ro
screeeuhrluee^ oHHUoJ.cou srhedrHehxoy
ercBdeoe doue$ 4nr9oU
noeovrcodEou I omtce %Oe el rueod4 'ouoxuulcxy
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r4r4HohEHE xrcucodee roeJl4lcoU il 'vem^eeU
,{ onnegoco 'gt4HBeedeoc oJoeoLIou
oucou scJ-oext4ttc odrctsg qrcoHerluv
'rorr gL BH
{amoHot ['reu ;; eu t,'leuUedc
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e vemleaU I ucrilEoxndu rlcoHEt4rve vuu
'uttHBeedeoc oJoEollou
Uondeu e erauelc Brcod edel'l ou regr
-cBdeoe EEoHc t4 ercBdeoe novcroU I uoJ"o
-Ext,1Hc 'oErcohHaUEU,'t omtcE
I crcoHet4lvv
elcedeoe oreuUedc uusexnrcoU oU Ht,tt x{rtt
l{ 'eden r4eur4edv ou 'crercedeos rexof!
'ercedeoe v\oe
-ovrcodUouEUcreesilhilueel(s.tcouerrxy -oHxnBHllHPlrV
pudernenwoy pnnefdusnuexy
orcedeos nosovrcod
-Uou s uriHoHovr8u auvcahhrtll,lxol,tg 't'6 ehhugel
6?0 . Bt1HVgoueUCCl4 3HHqrVrH3WAdrCHl4 t4 SlqHdorvdogvu
-soHercf BIII aI,IHoh€He oam{o}nrus reonu xlsucodtl
yvt t/^v&efr>Ksdru( ltoxcnllrlutren s eJ,cudeos oJosol
-rcod[ou ?rHoI4rI?U rufierqrfcHox rennuadeg
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'EJc?deos olenEadc sunxo0t ,( n
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udII
B 'e€1l€IIoHoN E HI,ItflHox,{ u rnnueed€oc oJosoro[
troudeu a xehosoE,{ tsl^{ugelcouoc &tolfer€o?xou )oDL
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r,rr{Jorroruu 4oueesdec eusrclfclo s v{orerl g
' rr,rHeso[ercgo oJemlIoHqrsr
rr,rH eEosodu .lofgedl e n (needeQcnedJ,Iruneufiru -l
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udu alcedeos v{oaoxJ,codtroil s O[n t,trcoHsI'IDIE {oH
-Homlilsou al4HaItsrng'Erelroxc Srcod o:onsucHau{t
rfl edg os ul^[BrcBrgoerco oJol^l3eerr'rugedIsu' rlIJEN!
goHJco) elnelntdeQoeu olqrcoHslalxu oneraorcfgo
ercedeos nosoxJ,cod[ou s Olfl rnsodf oI'IHemIqs
-ou 'xB1 '6't 'nger g eHeaodul{ltr(c 4elrerecexou
xrqnledxnox :rtft xvfiHahelre xlsHcHedeQed o ruIIBv{
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350 . llYEEPTATHblt4 flEPUOA n flOAPOCTKOBb[4 BOSPACT

KOTOpbIe, Ha ero B3uIgA, 3acnyxr,rBaloT BHUMaHI{fl TeJIbHoe BpeM.fl , .{ro6H 3a.qarb xox$ugenq}raJlbHbre
(ra6,r. 9.5). MHorue MoJroAbre nro.{u c xpoHr,rqecKr,rMr,r Bonpochl MoJIo.4oMy [arI[eHTy, a TaroKe Bonpocbr IIo
3a6oneBaHufl Mr,r [puxoA.sr Ha flepByro KoHcynbraur,rn o6pasy xll3Hpr, rdrII,I yAocroBepurbcfl B ToM, qro oHI,I
n au6ylaropHoe oTAeJreHve Ng. B3pocJrbrx co cBorrMr,r naoryr 6urr xouOuaenuralbHo 3a,qaHbr Ha cneAyrouleM
po,rurensMr,t. I{ea ec oo6pa3Ho BhrAeJrr,rrb Ao[oJrHr{ - BrI3UTe.

Ta6nrar{a 9'5' oco6exxocrra c6opa aHaMHesa n $uauvecxoro o6cnegoearhc naqueHra npta ero nepexoAe Hs neAua-
i TpurqecKoro MeAyqpexAeH[n B c[creMy MeAu{xHcxoro xa6nngexun B3pocnbtx
Oco6exxocru aHaMHeaa ulunn o6cnegoaaaua O6ocxosaxue
Atauxe3*
Ocnoeuoe/raauurSecrrapyoqee sa6oreeaHne ra nogpo6xuri Oqexxa oco6exxocrei y rnxecru ga6oreBaHr4n, a raKxe paHee npoBo-
aHanrl3 ero TeqeHr|c AuBueroce leqeHue r ero sQQerruaxocrr
Ceraer7nurri aHaMHea Muorue xpoHilrecKue ga6oneBaHhq, BogHuKaqu.lile B AercKoM Boopacre,
. Pncytox TeHearoTt4qecKoro ApeBa Bcex qreHoB ceMbkl r4Meor cepbeaHbe TeHerugecKue r ceuefiHure npuer4Hbt, Koropbte Heo6xo-
nepBoil creneHr4 pogcrBa h qrreHoB cei/br,1 BTopoti AilMO nphHt4MaTb B paceeT
creneH!4 pogcrBa npu HatMerM ga6oreeaHufi

Teryqar MeAr4KaMeHrosHa, Tepanilr, paHee npoBoAhMae Muorue npenapaTbt HagHayaprcn MilTenbHo; KoHqeHTpaqilh prAa neKap-
Tepanilt cTBeHHbrxnpenaparoB Hy)ffio KoHTponupoBaTb; pgA neKapcTBeHHbx npe-
naparoB uoxer o6lagprb reparoTeHHbtM geficraraeur
flrc6ure panee npoBegeHHbte BMeLlarerbcrBa, B ToM r{ilcne Oqexxa npoBoAr4BrJJt4xcq MeroAoB ,reqeHul u nx esQerrnanocla
xilpyprilqecKre
l1y6eprarHurri craryc/nauaro uencrpyaqrari liouoraer oqeHrTb rflxecTb ga6oaeeaHrs u narrepHbt pocra.
Heo6xogruo nHQoprurrpoearb naqheHTa o cocrogHt4r4 penpogyrruanofi
clcreMbr 14 ySHaTb m oxhAaHil9 no nnaHhpoBaHr4o ceMbu
Coquaruxurri aHaMHeg l1oeaorser uJupe oqeH[Tb BlvaHne 3AopoBbF naqueHra Ha rheHyto xr43Hb
. Tlonyvaer o6pasoaaHre unn palotaer? r QrHaHcoaoe noroxeHile.
. Foryvaer coorBercrByoulue luroru/noco6ne? fleffercs KocBeHHbtM noKa3arereM Tnxecru ga6oreeaHr4q I onpe4enger
. ly'veer Srnancoaure vflA Apyrne coqilaJtbHbte npo6leuur? MexaH[3Mbt noAAepxKt4, yro raKxe noMoTaer B oqeHKe reKyu.l4x norpe6-
r Xreer c pogrrenrun/orge.nuno? xocrefi naquerra
llogpo6nurrl paccnpoc Irc6ure gpynre casgaHHsre/He cBqgaHHbte c ga6oleeanneM cilMrrroMbt !,,tlt4
npo6aeuur
Orgltqecxoe o6cne4oaatue
Pocr, aec, paceer ilHAeKca Maccbt rera XorR uologurx logei vacro conpoBoxAaor poAfirenr, npoBogt4Tb
M ocMoTp xejtaTenbHo pa3AenbHo, BocnonBoBaBuJt4cb Bo3MoxHocTbto KoH-
O6qrarZaxalre rr,torra (npu aeo6xogruocrra) QugeHqraluHo o6cygraru qacrHbte Bonpocbt, raKte KaK loloBbte napr-
OqeHxa ny6epralroro craryca Hepbr, ceKcyanbHae aKn/BHocrb, ynorpe6leHre HapKol4KoB k atnotote
O6qnfi ocuorp naqheHra
* Bo epe_un nepeofi
KoHcynbraLlrln cneAyer oqeHnrbHanr4Hr4e y naqreHTa yBepeHHocrr4 KOMneTeHTHOCTI4 B npilH9THr4
14

peuexufi, cnoco6nocrr corrauJarbcr c npeMaraeMbrM nnaHoM Aeficreufi. Flpn xanuvrau couxexufi Ha oror cqer cne4yer
BbrgcHHTb, rro 6yger npuHuMarb KntogeBbte peueHr4s.

po6neru bt Mefl il Ltt4 HcKoro

[1
Ha6nlofleHilfl B noflpocTKoBoM
nept4oAe
ll po6neua npuBepxeHHocl4 neqeHilo
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oH oTpaxaeT, HacKoJrbKo naqueHT peanbHo npu-
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cneAyer yKa3aHr,rflM Bpaqa. ,{annurl TepMr{H noA-
pa3yMeBaeT TaKxe napTHepcTBo Ir coTpyAHr,rqecTBo
MEX4Y IAUUEHTOM I,I YXAXI,IBAIOqI,IM 3A HI,IM JII{UOM.
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352 O IIYEEPTATHbII4 NEP]4OA U NOAPOCTKOBHfi BO3PACT

Orconqauue ma1,tuuot 9. 7
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. fipe4ocrasrsilre rerryo, KoHKperHyro I nonHyp
uxSopuaqro c BbrcoKrlM pNCK0M fqnfl xN3H14
. TloAgepKH/Te BKnaI naqileHTa B nprlHFrl4e peureHur o
IEqEHilil floApocrxosbrfi Bo3pacr y pflra rr,ru coqeraercrl c
. npeAocraeure naqreHry AocraroqHo BpeMeHu, qto6ut ou vor OCO6EHHOCTfl MI,I IIOBEAEHI'IS, I,IMCIOUI}IMI,I BbICOKI{fi
SaAaTb cBot4 Bonpocbr
. pr,rcK.qnq xu3Hr{. 9ro cle.uyer uMerb B B}r,4Y, co6rlpafl
Burcrparearire AoBeprrenbHbte orHouJeHrr c naqileHToM
aHaMHe3 y flauueHra, rlepexoA.srqero I,I3 fleI-uaTpu-
,ls6erafire npeAy6exAexui
qecKoro yqpex.qeHu.E B MeAytrpe)qeHile Mfr B3poc-
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o ToM, KaK OHa BTUFeT Ha UCXOAbT Sa6OneBaHU'
. Yqururearite oco6eHHocl4 reqeHilfi B pa3Hbtx grHl4qecKl4x t4 xpoHuqecKuMr,t 3a6oneB aHvtflMvt, 6olee crurongu r
coluanbHbrx fpynnax noBeAeHr4ro, yrpoxaloueMy I,Ix 3.qopoBbK), u lIMeIoTcf
. ly's6eraire Heo6terrueHocrvl B npvrr+ruv MeAUL$4HcKrlx
.uaHHbIe o B3ar,rMocBrI3I,I Mex.qy [JIoxI{MlI vcxo]JiaM.u
peLuexril (npegmveperHorZ ltnu HenpeAHauepeHxori)
. Y,{hruraafire pacoBbre/orHr4qecKue u F3brKoBbte oco6eHFiocl4
6oresHeft I{ [oBe.ueHlreM, coflpflxeHHblM c pI,IcKoM
naqueHToB .IIJIfl XLI3HI/I, B UOApOCTKOBOM nepuo.qe.
. YAenRfr re AonorHurerbHoe Bpel/f, npeoAoneHr4ro KynbrypHbrx
6apuepoe Ta6nuqa 9.8, C6op aHaMHesa y naq[eHroB nog-
. Burscxsilre y naqileHTa ero orHouJeHile K npoBoAr,rMoMy pocTKoBoro Bo3pacra: noBeAeHHe, conpnxeHHoe
IeYeHI4lO, OxuAaHl,lf, oT HeTo Ll lqnbrypHbE HOpMbl, KOTOpble E
c aurcoxoi creneHbp pncxa (HEADS)
Moryr BoriTr4 c Hr4M B npornaogericrere
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lqnbrypHoM ypoBHe . OrHorLreHrR ' Pa6ora no Aor/y
. Boerexaire naqueHTa B npoqecc reqeHr4f,, noaururailre ero . Coqra.nbHag no4AepxKa
aKrilBHocrb r pacunpnrire ero BogMoxHocrr,l
r A4anrupyilre o6tscHeHuR K ypoBHro noHtaMaHiln naqileHTa O6pasoaaxue

Oqenure npuBepxeHHocrb repanxu

. Lllxora . Kapuepa

. . OxgalaeHut . Ynueepcrtet
Hanpnrayro: . Onurr pa6orur . OilHaHcoBbte npo6neMbt
- flo rucry noBTopcrorqr4xce peqenroB (4nn PO HenpilMe-
Hr4Mo, TaK xax 6oauuan ,racru flC BbrAaercg 6espeqen- Anruexocrr
rypHo, a xounuorepHurfi yHer He Be4erca,
- I1puu. pe4.) . CBepcrHt4Kt4, noAil, Ha Koro- r Ol43t1qecKa9 aKTrtBHocrb
- [10 m6oparopHbrM noKaoareneM pbrx MoxHo nonoxilTbcg cnopT
- flo roxqerrpaqun flC
o KocBeHHo:
14

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- 3a4afire Bonpocbr naqheHry: "KoTAa aur B nocnegHufi pag
C 16-rerHero Bospacra npu
npilHt4Manu ceoti npenapar?", qacro Bbt 3a6btBaflil
"llar He4eecnoco6Hocrur
nptaHffTb cBou npenaparur na eroil Hegele?,
Hapxoruxu
Burpepxxa npeAcraBreHa c pa3peuJeHur Auepuxaxcxoro
Koflnegxa npo$unarrurecxofi ueAuqrxur. Medication . Cilraperbr r arKororb: . E$peqenrypHbre
Adherence: lmproving Health Outcomes Time Tool: A cKorbKo, KaK Hacro? npenaparbr
Resource from the American College of Preventive Medicine.
201 1 . t'loryveno ns https://webmail2.tst.nhs.uk/go/www. Pauuox nltTaxun
acpm.org/?MedAdhereTTProviders. . BaxHbre KoMnoHeHrbr pa- . KoQe[H (xannrrr)
qroxa (xaluqri, BilTaMrH D) . y\iacTfie B nbFHKax, nnoxag
Jlureparypubr e AaHHbre rrocJre.uHero BpeMeHr,r . Bec nepeHocr4Mocrb anKoron,
aeMoHcTplrpyroT, qTo .[BycTopoHHff.fi KoMMyHuKarIu.s
MexAy [auueHToM r,r BpaqoM rroBbrrrraeT yIoBJreTBopeH- Cerc
HOCTb JIEqEHI,IEM, YIYI{IIIAET NOHI,IMAHI,IE NAUIIEHTOM
. CeKcyanbHae osa6oqeHHocrb . KoHrpaqenqilr (uegrxauex-
cBoero cocTosHu.fl, ero npuBepxeHHocTb JIeqeHI4Io I,r
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cHI/DKaeT plICKr,r He6raronpr4flTHbrx co6brTr,Ift , cBrI3aH- Con

HbIX C npOBO,{UMbrM IerreHUeM. . llpogorxurerbHocrb cHa I Llacrbre npo6p(qeHut

Moro.qrte Jrro.qu, flepexo.r1f,rrlrre B cr,IcreMy Me,uH-

o TpyAHocrr4 c 3acbrnaHheM . Parxee npo6p<gexre
u,rHcKoro na6rrc.uenus B3pocJrbrx, [o-pa3HoMy [pe.u- Cyuqugaruxure HaKnoHHocrr4
cTaBJIfl IOT Ce6e ngalruoorHorreHLr.fl Mexay [aIIueHToM . Eenpeccm . BbtcoKr4fi pilcK y nogpocr-
r,rBpatroM r,( 6olee cruronHbr K coBMecrHofi pa3pa6orKe . HacrpoeHre KoB c t4HBElnfiAHocrbo
IIJIaHa JIeqeHI{q, rITO B UTOre ynyquaeT UX [pI,IBepXeH- hs Segal T.Y. Adolescence: what the cystic fibrosis team
Hocrb reparr]rr,r B AoJrrocpoqHofi flepcneKrr,rBe. needs to know. J R Soc Med 2008; 101(Suppl 1): 15-27.
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 354 O TIYEEPTATHbIfi NEPNOA r NOAPOCTKOBbIF BO3PACT

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un$exrluu, sussaHHoft Br4pycoM Enureftua-Eapp, caxapHoFo Ava6era npor{3orrmo B AercKoM BoSpacre.

nocne rpaHcrulm,HTa\uvti 6omruuHcrso B3pocnblx Ha I{ccaeAosaHils y [oApocrKoB c ciltapHuu,qua6erou
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pr,rcK AaHHoro ocJroxHe *rus.. P ucx Bo3HI,IKHoBeHIa.fi I4SMepgJII{ ypOBeHb DITOKO36I B KpOBI{ I{ BHOCI{,II}I B
rrocrrpaHcnnaHraur,roHHux .nuu$onporu$epatur- AHeBHUK caMoKoHTpoax Suxrunnble pesynbrarll.
nux ga6olena*nfi sasncur or Blula rpaHcnnaHTaul,Il,t: Eruo rarcre noKagaxo, qro reqeHr,re car€pHoro AIta-
rryMynsTr{BHaff rlacToTa nocTTpaHcnnaHTaIIuoHHbIx 6em uorcro yJryqIuilTB npu co6rtoAeHl{}r [pI{HIII{noB
rr,ru$onporu@eparrouux ga6oresaHufi n te.{eHI,Ie [porpaMMhr rrepexoAa K caMocTorTeJIbHoMy KoHTpoJIIo
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adherence: Making it simple. MedGenMed 2005;7: 4.
2. Crowley R., Wolfe L, Lock K., McKee M. Improving the
transition between paediatric and adult healthcare: a sys-
tematic review. Arch Dis Child 2011:96:548-553.
3. Segal T.Y. Adolescence: what the cystic fibrosis team needs
to know. J R Soc Med 2008; l0l(Suppl l): l5-27.
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2003; (Cexmuxan nypnypa) Forbes C.D., Jackson W.F. Clinicalmedicine, 3rd edn. Edinburgh: Mosby, Elsevier lnc.; 2004; (Bexosnan
rpo$uvecxan neaa) Mosti G. Compression and venous surgery for leg ulcers. Clin Plast Surg 2012; 39: 26$-280
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4. eugms.org. O6ulecrno repl4arpl,Iqecrort nre.unqunrr
Enponeftcroro corosa (European Union Geriatric Medicine
Society) : rrccJle.uoBaH[rl, AoKyMeHrbI c I,I3noxeHI,IeM
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6. knowledge.scot.nhs.uk/effectiveolderpeoplecare.aspx.
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IrpaKTr{Ke s o6racr[ reqeHl{q u pea1wtna\nw ocla6-
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cra6urHocru no3br y noxlulblx nloAeft.
8. qfracture.org. Kamryrerop ptlcKa [epeJIoMoB, Barrvl4vr-
suponauuuri AJrg Hac en enlts Beauro 6pur anuu.
Bxarcqaer 6olee rurporufi cnerrp QarropoB pficKa, qeM
FRAX.
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noMoB: MoxeT r{cflonb3oBaTbcg 4rs. pacqeTa pI,IcKa B
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uenefi. AHK o6pasyerxpoMocoMbr r/r H€xoAlrrcr BrApe Korra Hlrrrr AsoftHoft ctrupaJru AHK <pacxoAqrcfl>,
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uoxer 6uTb yrpaqeHa I,IJIlr, Hao6opoT, Ao[oJrHr4TeJrbHo
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nrouur geQrqura o-1 -arrrrpnnc[Ha rBJrs]orcn p$ynbraroM
geicrarn SepueHra Ha HopManbHbre rKaHu
Bapuarrur sa6oneeaxrn
. M-aapilaHt: ecru y qeroBeKa ecrb HopManbHbre T6Hbr
SERP/NA 7 n o-1 -axrurpuncun eurpa6aruraaerce B Aocraror{-
HoM KorhqecrBe, roBoptr, HTo y raKoro HenoBeKa M-aapraHr.
. Ar,rarHo3, Kor.ra eMy 6uro 3 roAa, noroMy rITo oH He
S-eapilaxr: MfraLlne p.Glu264Val npilBoAr4r K cHr4xeHhrc
ypoBHe 14 o-1-arrurpuncrHa nprMepHo Ao 40% Hopuur.
, Z-aapnaar: Myraq[, p.Glu342Lys npr4BoAr4r K sHavrrer]bHoMy
cHr4xeHulo ypoeHn o-1 -arrurpilnc14Ha.
, PiZ: y rngei,
xoropure RBrf,rorcn roMogurorHbrMu no
MyraLUr4 p. Glu342 Lys, uoxer Ha6rrcgarucn geQuqrr
o-1-aHrurpuncrHa 14 cBf,gaHHbE c Ht4M chMnroMbr.
. P|ZS: y rnAefi, y Koropbrx uMeerce KoMnreKcHas rerepoor4-
rorHa, Myraque p.Glu342lys u p.Glu264Val, uoryr 6uru npo-
qBreHuc ga6oreeaHuR, B HacrHocl4 ecnr oHLl rqprr, oAHaKo
sril nposBreHilr o6urqHo MeHee BbrpaKeHbr
Kesr - ura,qruufi 6par. Bce 6a6yuxn u reryruK[r
xr{Bhr r{ 3gopoBbr. B ceuefisou aHaMHBe yxaaauuft
B
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pr,rc. I 1.8), npn KoropoM AJIfl pa3Bl{r}rs sa6oresaHus
xeo6xorulto, .ITo6u o6a arurers rena 6u;ru MyraHT-
HbrMu. Eorusofi qenoBeK HacJre.rlyer rro orHoMy My-
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KOTOpbIe, CJIe.UOBareJrbHO, sBJrsrOTCs 3,UOpOBbIMI,I
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HOM nOKOJIeHLIU, TaKIM O6paSOU, COCTOIHI,Ie MO-
xeT Bo3HI,tKHyTb COBepIlIeHHO HeOXlrIaHHO.
Ayrocouno-peqeccr,rBHoe sa6oaesaHr,re Bo3Hr{Kaer
upu6rr,rsureJrbHo y I us 4 Aereft, po)KreHHbrx or Hocr,r-
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KoropLrx susrorcs Hocr{TeJreM reHa, cocrawner 25%,
a Bepof,THocrb roro, vro pe6enox 6es sa6oaesaHug
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(pegxo)
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sa6oresaHufi, o6clxraeuhrx B .qpyrux DraBil( KHr{ru,
rplrBe,qeHhr a ra6r. I 1.4.
X- cuenne x noe H acne$o BaH ne
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(cu. pr.rc. I 1.8).
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.flrcueuua (m6n. 11.6). Ero poAlrrenr{ 3ano4o3pr{nr
Mor caMocrosreJrbHo xollr,rrb t,r s ceMeftHoM aHaMHBe
6uru yxasaHrns HaraHHoe sa6oresaHr,re: asaa 9aaapaa
no Marepr,rHcxofi auuur,r crpaAaJr ur,rogucrpoQueft
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ponuxofi AHK = 0,5:1
+ - Aerequr e AHK naqnexra
Coorxouexne
IIHK naqxeHTa/KoHr-
Ponuxoi lHK = 1,5:1
+ AynnuKaqxs a IHK naqnexra
CoorHouJeHt4e
lHK naqheHTa/KoHT-
ponuxoi,4HK = 1:1
+ HOpMa
AoloJrHr,rrenbHbre MoAplouurlpylouue @axropu (re-
Herr,rqecKr,re $arcropu, $arropu oKpyxalouefi cpe.urt
lrJrlr cToxacTl,rqecKr4e oarropu).
Haroneq, cnyqaftHble pe3yJlbrarhl npe.ucraBrqlor
co6orl .qeneuurc unv I.yr,IruKaulllo B reHe I{JIH reHux,
KOTOphIe CBfl3aHbr C QeHOTUnOU I4JII,I pPICKOM, He I,IMe-
IollllrMr,r orHo[reHl,Ls K xuuo6aM nauueHTa. Hanpnuep,
ecnu np}I Marpr4KcHofi cpaBHI,ITeJIbHOtlt renouHofi
ru6puguaaunlr rro noBoAy ynacrnenuoft orcr€Irlocru
o6uapyxuaaercfl .ueneuus B reHe BRCAI, ero 6yAer
cqr{Tarbcfl cJry{arlHbrM pe3ynbraroM.
l4eyuent,te reHoB
AmnnnQrKar{}rr reHoB: nor[Mepa3Hae
IloluuepasHaq rlerIHarl peaKIII,Iq npeAcraBJlser
co6oft @yn4arvrenrarrsufi MeroA na6opaTopHblx
r,rccJre.qoBaHuri, n xo,ue KoToporo BblnorHflercq aM-
muouraqras qeneBbrx wacrKoB reHoMa qeJIoBeKa,
KOTOpbre lrcloJrb3ylorcs. NrA AaJrbHeftrnero aHalIH3a,
qAIIIC BCETO.UNg CEKBEH}IPOBAHUS IHK.
IIPU NOJII{.
Mepa3Hofi rlenHoft peaKu[u BblnoJlHeercs uuKruqe-
cKoe I,r3MeHeHlre reMlepaTypbl: [oBTopHbIe UI4KJIbI
HarpeBaH[fl r,r oxjrax.ueHr,rf, o6ec[equBaloT HcxoAHoe
pa3AeJreHr,re AByxrlenoqesuorl .{HK ua ABe oTAeJIbHbIe
uenu (npouecc, Ha3brBaeMufi 4euarypaqreft). Kaxuaq
r,r3 noJryrreHHux ueneft cJrlucur uarpuqeft Ha rIocJIe-
AyrouleM 9Tane peflnHKaul{}r, Bo BpeMq Koroporo A.llf,
orxlrra B KoHxpetrofi o6racrr4 reHoMa I,lcnoJlb3ylorcfl
npaftuepu. Taxre quxrbl HarpeBaHu t f oxnaxreuut u
AeHarypaqlr u f p ennuxagl,rl,I MHoroKparHo [oBrop.f, lor-
c.fl, B pe3ynbTaTe qero IIpoHcxoAuT gKcnoHeHu[aJIbHM
aunluSurarlr4s AHK Mex.qy caircav,u npatluepa
(puc. ll.ll).
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Puc. 11.12. Cexaexupoeanue no MeroAy Cexrepa, or{eHb ulr4poKo r,rcnon$yorJ.leecn a [HK-guarHocruKe. B gaxnou MeroAe npu-
MeHrorcq Sparuexru ,QHK, aunnuQuqupoBaHHbre npu noMorll4 noruuepaexori qenxoil peaKqrlr H coorBercrByrorlr4e reHy,
xoropufi npeAcraBnner t4Hrepec. Peaxqun ceKBeHilpoBaHue npoBoAtr c noMou{bp xotrr6uxaqufi AHTO u gn-gesoxcu-gHTO
(EIATO, EATTO, AAITO u p4l-TO) c $nrcopecqexrxoft uerxofi, Koropble BKnoqaorce Bo BHoBb cr,lHresr4poBaHxyto [HK, vro
npt4BoAt4r K repMuHaLltlt4 qenu a srofi roqxe. 3areu npoAyKru peaKquu noABepraprcn KanunntpHoMy snerrpoSopesy, n Qpar-
MeHrbt pasnurrHoro pa3Mepa o6xapyxueaor npu noMor{n na3epa, B pesynurare ororo nonyqa}or xpoMarorpaMMy nocneAoBa-
TerbHocrr,r, Koropag coorBercrByer qeneeoil nocneAoBarenuxocru,[HK

Ta6nuqa 11.9. IlpeuruyulecrBa r HeAocrarK[ ceKBexnpooaHlln qenoro reHoMa, ceKBeHupoBaH[n qenoro eK3oMa
I lt naxenei rexos
AHarus llpe[uyu{ecrsa Hegocrarxn
llonxorexouxoe Cal,lurri noaHurfi aHalrg reHoMa ug cyqecrByolLl4x, Eorce goporoe cooAaHue il xpaHeHile AaHHbtx.
coKBoHupo- Eolee paaxol,repHurri oxaar reHoB, qro no3Borfler Eyger o6napyrr<uBarb Mr4rlrr,loHbr BapilaHroB e Hexogt/pyloqefi
BaHll8 nyHUJe OnpeAenilTb KoII4qecrBeHHb€ orMoHeHilg. !HK, nmepnperaque Koropbrx uoxer 6uru sarpygHureaunori.
(Whole-genome lloreHqnaruHo Moxero6uapyxuaaru Bce reHHbre Conpoeoxgaercfi BbrcoKr4M pr4cKoM BbrsBnexrR cryvafinurx
sequencing) Myraqru,BK,'loqaeuHrpoHHbreMyraqilx pesynbraroB.
He6oruuas my6rxa cHt,1TbrBaHilff (uaroe rorrrecrBo cHrlTbr-
eaxrfi), caegoeareJ'lbHo, MeroA r/eHee vyBcrBilTeJreH il B
uexuuefi creneHu cnoco6eH BbrrBrqrb MoaalaqugM
lloaxogxsouxoe fleueele, qeM norHoTeHoMHoe ceKBeHhpoBaHhe. Menee paaxouepxuril oxaar TeHoMa, creAoBarelrbHo, TpyAHee
coKBeHrpo- Ananng xe orpaHuHhBaercg reHaMu, Ms Koropbrx o6xapyrr<unu KorvqecrBeHHbre oTMoHoHr4r.
BaHxe rcBecrHa cnoco6xocru BbrsbrBarb saAaHHo€ Mexee nonnuril aaanns (1-2Vo rexoua), qeM npil norHoreHoM-
(Whole-exome sa6oreeaxue. HoM ceKBeHupoBaHuu.
sequencing) O6napyxuaaerc, MeHbuJee Kon!,rHecrBo BapuaFrToB, Eoaee auEoxufi pr4cK nonyHeHhs cryrailrurx p$ynbraroB no
qeM np[ noIHoreHoMHoM ceKBeHupoBaH[u, cneAoBa- cpaBHeHno c HanpaBneHHbtM ceKBeHr4poBaHueM reHoB
TenbHo, nerlle npoBecTLr r4HrepnpeTaq[o.
Soree mldoxoe ceKBoHr4poBaHue, egM npt,! nonHo-
reHoMHoM ceKBeHupoBaHh[, lT0 noBbrrraeT qyBcTBrl-
TenbHocTb r,r BoSMoxHocTb Bbr9BIeHu, Mo3at4qrcMa
llaxenr rexoa SxoHoiau,{ecxal e$Serruarocru. BbrnBrqer ronbKo Bapuallr4r4 B Tex reHax, MF Koropbrx cBrsb
Ovexu my6oxoe ceKBeHilpoBaHile, yBenuquBapu.lee c ga6oaeeaHileM yxe ugBecrHa.
BeposrHocrb BbtrBreHufl Mogaul.lugMa. Cloxxo go6aalRru B naHerb HoBbre reHbt no Mepe hx
O6Happ<reaercn MeHbulee Kol'rueecrBo BapilaHToB, o6rappxenun
cneAoBarenbHo, nerlle npoBecTu r4HTepnpeTaqt4rc
AaHHblx.
f'locxorury aHanus orpaHilqeH u3BecrHbrMrl reHaMn,
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npeAcraBneoLLlre co6oi nocnegoBarerbHocr4 r43 zl-1 6 gr,rHy-
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oAr4H HyKn6OI4A.
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AHK.
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r.[4lo, a Kpacurenb orlennreTcr.
B cHere cuHTegr4pyercn HoBae qenb, cocroqulag ug
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cepht4 oluroHyKneo'n4AHbx soHAoB.
3arer,r cunrmupyercg eu.p qAHa qenb, Ho oHa cMeu.leHa Ha
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Rpoqecc noBTopflercn HecKonbKo pag (B cucreMe KoMnaHr4t4
SOUD 5 quuoe), B p$ynbTare qero noryeapr nepexpbtBalo-
quecn ua6aoxbr, Koropbre aHanu3upytor u onp€IleJupr
cocrae qeaeeofi nocIeAoBaT€IbHocI,,l
l4oxxoe nonynpoBoAHlrKoeoe cexBeHxposaHne
. npu BxntoHeHun HyKn€onlAA B pacryqyo qenu,QHK aucao-
6oxAaercr noH BoAopoAa, xoropurfi uroxno o6Hapyrruru no
usMeHeHuto pH pacraopa. Buneo6oxgexue uoHoB BoAopoga
sBrnercn ocxoaoi uoHHoro nonynpoBoAHr4KoBoro
coKBeHUpOBaHU9.
o KaxAuri aMnnu$h4,lpoaaxxui xnacrep,QHK pacnoaaraercn
HaA nonynpoBogHr4KoBbrM oJreMeHToM, Koropbti no3Boneer
o6napyxuaaru ugMeHeHue a pH pacraopa.
. Knacrep,(HK npouuraaor B cMecu, e xoropoi coAepxfficf,
ronbKo oAr4H run HyKneoryAa.
. Ecnl B cMecu npncyrcrByer npaBnnbHbri Hyxleorug, KoMMe-
uexrapHuri cneAyoqeMy ocHoBaHuto Ha Marpuqe !HK, ox
BcrparBaercf, B qenoqKy c aurcao6oxgeHueM hoHa BoAo-
poAa, roropuri o6Happxueaerca no usuexenun pH.
. exeou). Koxlurft
['lpn xaarquu roMononuMepa (nocaegoaareauHocrb hg AByx
unu 6oree oAuHaKoBbrx xyxaeorugoe) 6yger onpegenrrucn
yMoHbuJeHue gHaeeHns pH, nponopquoHarbHoe Kon4qecrBy
uAeHDlqHbX HyMeOTUAOB B nOCreAOBaTeIbHOCll4
. Bupaenueanue u udeumuQurca4un eapuaxmoe. flpu
noMorqlr cneur4anbHorc nporpaMMHoro o6ec[e-
qeHI,IS npoBoIgTC' aHaJIH3 CqI'ITaHHLIX nOCne.UO-
BaTeJrbHocTeft u cpaaueHr,re noJryqeHHhrx,uaHHLrx
c sTiuloHHbrrrr rua6ronou. Sror npoqecc r,r3BecTeH
KaK <BbIpaBHI,IBaHIiIe>, ILJIII (KapTI,IpOBaHI,Ie>. He-
cMorpc Ha ro sro rcHoM qeJroBeKa colepxur 3 uapl
ocHoBaHl/tft , BbIpaBHHBaHlre no3BoJlff er xeo6H.Iafi -
HO rOqHO Oflpe.(enr,rTb reHoMHoe [polrcxoxleHr,re,
ecJrr{ crr}rrlrBaeMbrft yqacroK cocror{T uz25 ulu6o-
nee HyKJIeorI,uoa. Bapnanrbr BbtsBlqrcTcc npu Ha-
Irquu paeruvufi Me)rqy cqr{TaHHbrM r{ 3T€uroHHbrM
reHoMoM. Hanpuuep, ecJIH B noJIoBIiIHe cqUTaHHbIX
B o[pe.ueneHHoM [oJroxe-
nocJle.qoBareJrbHocrefi
Hr,IU O6HapyXXBaerCq HyKrreoTI,I.U, OTnI,IqHbIft OT
TaKOBOTO B gTUUIOHHOM reHOMe, BepOffTHO, I,IMeeTCfl
reTepo3l,IroTHrls 3aMeHa ocHoBaHlrr. Koru.recrno
cqurHsaHl,Ifi, BbIpoBHeHHbIx B 3a.qaHHofi ToqKe,
Ha3hrBaercs ury6r,rHoft HJrr,r oxBaroM. tleu 6omrue
rrry6una cqlrbrBaHl,rs, TeM ToqHee onpe4ensrorc.E
Bapr,raHrLr. Tena ne MeHee B rIeJroM lurq noryqeHr,Lfl
AlrarHocT[]IecKI{ 3HagI,IMbIX pe3yJIbTaTOB AOCTaTOq-
Hofi cqr,rraercs rny6r,rHa 30 u 6onee c.rurhrnaxufi.
Buecro roro qro6br e.qr,rHoBpeMeHHo ceKBeHHpo-
Barb roJrbKo ogay ne6olblrryn nocne.uoBareJrbHocrb
AHK, NGS nosnorser aH€urr,r3r,rpoBarb corHr{ rhrcrq
qelorreK AHK s o.uHoM gKc[epr,rMeHTe r{ [o3roMy
qacro raloKe Ha3brBaercfl rexsoloruefi MHoxecrBeH-
Horo [apaJrJleJrbHoro ceroeHr{poBaHl,Lg. Conpeueuuoe
o6opyroBaHile Nrfl NGS nosnorfl er ceKBeHr,rpoBarb
Becb reHoM qeJIoBeKa 3a I .qeHb npr,r cTor,rMocTI,I, [p]r-
6ansurearHo paBHort 1000.uoruapoB CIIIA.
Saxaat npu ceKBennpoBannu Hoaoro
noKoneHnf,
Hecuorpa na ro qro reflepb lrMeercfl Bo3MoxHocrb
lpoBo.(r{Tb ceKBeHI,IpoBaHI,Ie Bcero reHoMa B o.uHoM
SKCnepI{MeI{Te, OHO He BCerIa.gB[reTCq OITTI{MaJIbHbIM
I{c[oJlb3oBaHueu NGS. 3axnarupu NGS orHocrrc.s x
coocax(eHl{p uerenoft o6racrr,r r€HoMa r,r Moxer 3a-
TparI{BaTb oT HecKoJIbKI{X TeHOB.UO HeCKOJIbKX)( COTeH
reHoB, cB.sgaHHblx c 3a,uaHHbIM Qexorunou (nauelr
reuon), oK3oHbr Bcex r,r3BecrHrrx KoAr{pyro[Iux reHoB
(sxsoru) r,uru gK3oHbr Bcex KolllrpyorurD( reHoB,.rrJt Ko-
TopbD( I{3BecrHa cBs3b c sa6oresaHueu (rurunr.recrclft
I,I3 srllx
noAxo.uoB HanpaBJIeHHoro
noBTopHoro ceKBeHr,rpoBaHr,rs xMeer pflI npelrlryqecrB
Iir HeAocrarKoa (cu. ra6r. I 1.9). lar npuueueuus NGS
c o[T[rMaJIbHofinoln:oft,Elg [aq]IeHTa Ba)KHo, qro6u
KlrHHlrulIcT xopouo IoHI,IMaJI, xaroft TecT nyqlxe Hc-
loJrb3oBarb B KoHKperHoft xruH[.recxofi curyaquu.
flpofinenu, cBf,ganHue c npnneueHnen
Texilonornfr ceKaeililpo&ailnn HoBoro
noKoneHnf,
Ienounue rexHoJrorlru cloco6Hbr npoll3Becrr,r ne-
peBopor B nparcru.recxoft Me.uuqnHe, u 6olee 6ucrpoe
u 6oree AerueBoe ceKBeHupoaauue.[HK yneauuraaer
Bo3MoxHocrlr,quarHocrI,IKI,I, reqeHr{s u flpo@uamu-
rcr ga6oneBaHufi. Teu ne ueHee reHoMHbre rexHoJrorur.r
He JII{IUeHLI He.qOCTaTKOB, OAHI,IM l{3 KOTOpbD( SBIgeTCf,
xpaHeHlle orpoMHono KoJII{qecTBa IaHHbIX, [OJryrIeH-
BBSTqII{hA'SOTHOJBOd €srCehr,ruox OJOmqHer^r refgefu
Ho f)qroxcou 'SCN r^[eh 'ersemev trorew grqHHe[
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111 ruueeodl,IHesroc l^toalcefrllfuru4edu ltnneoncg
' (urnr,oenr{H sJcoHhr,ru{etrfi
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utruloercrdn ororddE rr,rHersegotr u Bfilaelrr-a( oJo otr
em{JoorfifH oJonder,nel^teru^o, oJoID(Bx uHHorEEgotr
ndg 'needexurou ulr.r,curv( ndu 4oxr,elt 4onurettced
-oorQ c sotrI,IJ,oerx.,(H m,rHehorDrs oJoHqrerBso[e[c
-olr Krtr rsttudJ,un srod reefuu redorox '(nder,ceror
anHHuaodfifru@uruxe rcrorrHer^rudu lrodor,ox udu
'SgN ro eunuuro a) yg[' en(xeror^r BuHhehouenoHYo
rctol(esrouclr oweJclrc Hor€ g 'secuorcsolg curced
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s rcto,(eqlfoucu effiEh ecg auHegodl,IHesxec eosdrrdx
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r,rrcoHJc?h s'godorsou xnHtruJoarx^Hudt nncnuucxe
srusodr,rJ,coHJeutr rorrosoou aH SCN 'o.ror elrody
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'unertetrng-Brr,Isxeg rvnodtrHuc xux eI,DrBr'BJHr,rr
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n -ou 'eHeJ o Urr,rH?Ho xrrHhoJercotr raH o,t codu ceH ,( oJ,rt
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xllH s u'uquo grrHqreruhuHe t,I rxeds rcrolf,gedl,
uxrogudeud xlr yvY o)BHtro 'gonrJ,rdorrE xrqHxorc
nrneuglundu lreilu rcqu,Igotr or)Kor r oJor€ I{Helrerc
gonueretreduo g'elrHeserrogee reusrrerrs redorox
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nmr) Eoufo ruHersBrirs rlri soJ,Hurdes xlsHsrrcI^IdoH
rlrHolrmo{n slegodurdoc qrcoHxol^loos qrrrrg BHxrotr
cgn l( 'xrquHetr rq€89 ersHufdx erxeJ rreenrugud
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nn ogedgo nff Uolu,rtroxgoex'ullfieNdoQnu runenedx
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9Tr,r nper,rMyrrlecrBa, ceKBeHr4poBaHr,re III floKoreHu.fl
Bepo.trrHo, s dJrwxafirueM 6yAyrrleM 3aMeHr{T NGS.
Bnzgy rryranlrrlbr B TepMr{Hax NGS u ceKBeHr,rpoBaHt4fl,
III nororenlr.fi 9Tr4 TexHoJloruu Bce qarqe o6o3Har{aror
KaK <<MaCCOBOe napaJUIeJIbHOe CeKBeHI,rpOBaHlre )>.
leHourvrxa B KnilHilqecKor4 npaKTilKe
I HepBHO-nCffil,lt{eCKOrO
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I paSB[Tr,rt
Hecuorpa Ha ro qro peAKr,re sa6orenauu.a, no
o[peAeneHI,IIo, BbISBJUIK)TC' HeqaCTO, B COBOKyflHOCTI,I
n Belnxo6puraum oHlr 3apenrcrpr,rpoBaHbr npr,rMep-
Ho y 3 MrH qeroBeK,6oruuuucroo r,r3 Koropbrxp.eru.
NGS usMeHr,rJro Bo3MoxHocrr,r ArrarHocrlrKn peAKr,rx
sa6oresaHuit. Ec:nu paHee Mbr orpaHr,rqr,rBzurr{cb rro-
cneAoBaTeJIbHbIM aHzulu3oM oTIeJIbHbIX reHOB H .4Jtfi
o[pe.4eneHu.fi Ha[paBJreHr{fl reHeTlFlecKoro uccJleAoBa-
nng 6ru neo6xo4nrrr rurunuqecrcrrl Auarnos, to celtvac
NGS nossonqer BbrnoJrHurb r,rccJre.qoBaHr4e HecKoJrb-
Kr,rx reHoB B oAHoM sKcnepuMeHTe. 3ro uoraro cgenarr
IryIeM a}la-JWr3A [aHeJrr,r reHoB, Krrr4HuqecKoro gK3oMa
lrJrl{ gK3oMa (cu. ra6,r. I 1.9). luaruocrr,rqecKas rIeH-
uocrr NGS nplr paccrpoftcrBax HepBHo-ncuxrcrecKoro
pasBurus. weJwlr.tw:m,cb npr{MepHo ta 30%. YcraHos-
rreHvte reHerr,rqecKofi npupo4u pe.uKoro sa6oresaHus
MOXeT AaTb CeMbflM OTBeTbI Ha IIX BOnpOCbI, nO3BOJI_rreT
loJrytII,ITb r,tHQopuaqurc o [porHo3e lr npeAocraBJuer
BO3MOXHOCTb [O3HaKOMr,rTbCq C ApyIUMI{ IIaIIr4eHTaMr,r
c AaHHbrM ga6orenanueM pr flonytrurr neo6xo4ul.ryro
[oAAepxKy. Eoree roro, reHer]rqecKoe LIccJreAoBa-
Moxer.uarb rleHrryro uu$opuaqurc Nrs, Tex nap,
Hr,re
Koropbre rrJraHr,rpyror Aanrxeft ruue 6epeueHHocrr.r tr
n 6y4ylqeu xorgr ) {ecrb pe3ynbrarbl upeHara,'IbHoro
TecTupoBaHI,Iq.
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nufi onpe4e rrsror c n seaulvlo.qeft crBlreM psAa reHoB
u Sarropon oKpyxaro{eft cpe4u. B erofi curya\nu
reHerr,rqecKl,rrl eKfla.u B pa3Br,rrr,re sa6oresaHu-fl Ha3br-
Baercfl EoJrr,IreHnuu. .(o neAaBHero BpeMeH[ flporpecc
B BrISBIeHLIII reHeTlIqeCKUX I,I3MeHenrUl, XOrOprre
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6rur o.reHr He3HaqurerbHblM, oAHaKo Bce u3MeHr,I-
Jrocb c [oflBJreH]reM [oJIHoreHoMHbrx uccne.uosaHl,Ift.
, Genome-wide association study o6uvno BKrroqaer B
ce6.fl reHoruurrpoBaHlre MHorotrucreuuux (>500 000)
reHerr,rqecKlrx MapKepon (o4uouyueorlr.rlHbrx rro-
mruop@usuon), xoropue pacnoJroxeHbr no BceMy
reHoMy s 6omurorl rpymre ;rrcAefi c sa6oresaHuer.{
r,r B KoHTpomnoft rpynne. Cparuenre reHorunoB
oAHoHyKrreorr,rAHhrx uorul,rop0rE3MoB y rrarlr{eHroB
c sa6oresaHr,reM r,r y npe4craBlrrelefi xonrpomnolt
rpynnbr no3Borfl er r{reHTr{$uquponarr o6nacru reHo-
Ma r,r, cre,(oBareJrbHo, reHbl, .qJlfl KoTophlx xapaKTepHa
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sa6oresaHr,I.f,.
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flpeuaramxa.s reHerrrqecKarr AuarHocrr{Ka Moxer
flpoBoAr{Tbc.fl B cI} {asx npeAlonaraeMoro BbrcoKo-
ro pucKa reHerl,IqecKoro sa6oaegaHl,Ig y rrJroga, npr{
onpeAeneHHbrx pe3ynbrarax ynbrpa3Byronoro/6uoxu-
Ml4alecKoro cKpI,IHI,rHra rlr6o npn Haluqilil or.f,rolleH-
noro ceuefinoro aHaMHe3a. Ha nporxxeHr{r,r MHorr,rx
rer ocxosoft npeHar€IrrbHorl AuarnocrvtKtr nBlnfliorcs.
I{HBa3I{BHbIe TeCTbI, B qaCTHOCT}I aMHUOIIeHTe3 lI
6uoncus. BopclrH xopr4oHa. B ro xe BpeMs oHlr Bce
qalle BbITecHrIIoTcg HeuHBa3rIBHbIM I{CCJIeAOBaHI4eM
6ecrc[ero.rHoft $era-nrnorl IHK, Koropa.f, o6paeyercx
r rpoQo6racrrx flJrarleHru, o6napyxuBaercs. B KpoB[
Marepr,r c 4-S-fisele.nu 6epeueuuocrlr r,r coxpanflercfl.
B AocraroqHoM AJLS rrccJreAoBaHlr.E Koruqecrse Ao 9-fi
6epeueHHocrn.
He.qeJrr,r
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BeHr,rpoBaHlre u KoJII{qecTBeHHoe oilpe.ueneHr,Ie
xpoMocoM-cneuuQuqHHx nocneAosaremHocterl
6ecrueroqHoft $eraarnofi AHK c r{cnoJrb3oBa-
HIaeu NGS c ue,rrbro BbrflBJreHlrfl rpucol.ruu 13, 18
ulu 21. To.rnocrr HeI,IHBa3uBHoro flpeHarrurbHo-
fo TecTupoBaH.ufl B .u{arHocTr{Ke aHeyrrJror,Lquu,
cnequ@uuHolt iltts. 6epeuennocru, npu6llrxa-
erc.fl K 98%. Jloxtoorpr{rrarenbHbre pe3yJrbrarbr
uoryr 6urr non) {eHhI B cJly{aqx, KorIa KonI,Iqe-
ctso 6ecK,rero.rHoft Seralruort .{HK c.rruruxol.r
ua.no (nosnaoxHo, r{3-3a paHHeft 6epeuenHocrr,r
lIJII,r BbrcoKoro r{HAeKca Maccbr teta uarepu) ustu
KorAa aHeyrrnola.Ilyrfl Bo3HlrKJra sa 6olee [o3.rHrrx
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2. decipher.sanger.ac.uk
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Hr{3MOB C KOMMeT{Tapr{f,Mn, neftcxas 6asa aaHHux pe.uKrrx sa6oaenaHuft.