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OE?A?INUeEEE IAOEIAEE A?A??I?*IEE OeAIO? IIC OE?A?IE

IEOAEEAOeUe Ethaeieea Iao??aia

OAeE.616.37-053.1-0.73.4-8:575.113

AAIAOE*IA EIINOEUeOOAAIIss, AeIIIEIAIAA

Ae?AAIINOEEA OA EE?I?*I? AA??AIOE IA?AA?AO

IOEIA?NOeEAeICO CAEAAEII A?Ae AAIIOEIO

03.00.15 – aaiaoeea

Aaoi?aoa?ao aeena?oaoe??

ia caeiaoooy iaoeiaiai nooiaiy

eaiaeeaeaoa iaaee/ieo iaoe

Eeaa 1999

Aeena?oaoe??th ? ?oeiien.

?iaioo aeeiiaii a ?inoeooo? iaae?ao???, aeooa?noaa oa a?iaeieia?? AII
Oe?a?ie.

Iaoeiaee ea??aiee: aeieoi? iaaee/ieo iaoe, i?ioani?

Ieeeinueea Ia??y Aiae???aia

?inoeooo aenia?eiaioaeueii? ?aae?ieia?? Iaoeiaiai oeaio?o ?aae?aoe?eii?
iaaeeoeeie AII Oe?a?ie, caa?aeoaa/ea eaai?aoi???.

Io?oe?ei? iiiiaioe:

aeieoi? iaaee/ieo iaoe, i?ioani? A?a/ai?ia Ieaia sse?aia, Oa?e?anueea
iaaee/ia aeaaeai?y i?neyaeeieiiii? i?aeaioiaee IIC Oe?a?ie, caa?aeoaa/ea
eaoaae?e;

aeieoi? iaaee/ieo iaoe Oei/aiei Ieueaa ?aai?aia, Oe?a?inueeee iaoeiaee
oeaio? iaaee/ii? aaiaoeee IIC ? IAI Oe?a?ie, caa?aeoaa/ea eaai?aoi???.

I?ia?aeia onoaiiaa

Euea?anueeee iaoeiai-aeine?aeiee ?inoeooo niaaeeiai? iaoieia?? IIC
Oe?a?ie, a?aeae?eaiiy ai?aeai?ieia?? a?iaeaeaii? ? niaaeeiai? iaoieia??,
i. Euea?a.

Caoeno aeena?oaoe?? a?aeaoaeaoueny ” 29 ” aeiaoiy 1999 ?ieo i 10-00
aiaeei? ia can?aeaii? niaoe?ae?ciaaii? a/aii? ?aaee Ae26.604.01. a
Oe?a?inueeiio iaoeiaiio a?a??i?/iiio oeaio?? IIC Oe?a?ie (253660,
Ee?a-94,aoe. Iiioae?aiea, 50).

C aeena?oaoe??th iiaeia iciaeiieoeny o a?ae?ioaoe? Oe?a?inueeiai
iaoeiaiai a?a??i?/iiai oeaio?o IIC Oe?a?ie (i. Ee?a, aoe. Iiioae?aiea,
50).

Aaoi?aoa?ao ?ic?neaii ” 22 ” aa?aniy 1999 ?.

A/aiee nae?aoa?

niaoe?ae?ciaaii? a/aii? ?aaee Naeaciueia A.TH.

CAAAEUeIA OA?AEOA?ENOEEA ?IAIOE

Aeooaeuei?noue i?iaeaie

Aaiaoe/i? i?aeoiaee o oea?ei? iieiioaiiy caei?ia’y ianaeaiiy noathoue
aeaaeaei iioe?aiioeie. Iia? aaiaoe/i? oaoiieia?? oa iaoiaee ae?aaiinoeee
coiiaeththoue iaiao?aei?noue ia?aaeyaeo e oaeineiiaeaiiy a?aeiieo oi?i
i?io?eaeoeee, e?eoaaiiy ? ?aaa?e?oaoe?? niaaeeiaeo caoai?thaaiue.

Ioeiainoeeaeic (IA) – aooiniiii-?aoeaneaia niaaeeiaa caoai?thaaiiy, yea
coiiaeaii iooaoe??th aaia o?ainiaia?aiiiai ?aaoeyoi?iiai a?eea oa
oa?aeoa?eco?oueny o?aaeaiiyi aecie?eiieo caeic aeeoo?ai aaaeeeaeo
i?aai?a, ?aiiueith iai?oanoaoe??th, oyaeeei ia?aa?aii ? aenieith
eaoaeuei?noth. Iioe?ai?noue eiai a Oe?a?i? noaiiaeoue 1:2200
iiaiia?iaeaeaieo [A.ss. ??ciee oa ?io?, 1994].

IA ia?aaaaeii anioe?th?oueny c iaoieia??th eaaaiue, i?ioa a?i yaey?
niaith nenoaiia caoai?thaaiiy c o?aaeaiiyi ianaiia?aae
oeoieiai-eeoeiaiai o?aeoo, a caiaeii – na?oey oa ?ioeo aeeoo?ai
aaaeeeaeo i?aai?a. Oea caoai?thaaiiy neiae ?icaeyaeaoe ye aaaaoia?aiio
ee?i?/io i?iaeaio, ui i?eaa?oa? oaaao iaae?ao??a, aaiaoee?a ? oao?aoe?a
iieaeoey?ii? a?ieia??.

Aaaeeeaei aeinyaiaiiyi o aea/aii? IA ? ea?ooaaiiy aaia o?ainiaia?aiiiai
a?eea (O?AI) o 1989 ?ioe?, ui aeicaieeei ?ic?iaeoe iaoiaee iieaeoey?ii?
ae?aaiinoeee aeaiiai caoai?thaaiiy [J. Riordan et al,1989; J. Rommes et
al, 1989]. Ia nueiaiaeii aeyaeaii iiiaae 750 iooaoe?e oeueiai aaia
[ECCACF, 1997], aea iaeiioe?aiiooth a a?eueoino? ianoaaeaieo iiioeyoe?e
? “iaaei?ia” iooaoe?y delF508 [B.Kerem et al,1989; L.Tsui,1995]. *anoioa
?o aa??th? – ?c i?i?iaeueiei cia/aiiyi o Oo?a//ei? (27%) ? iaeneiaeueiei
o Aeai?? (88%) [I.Handrieser et al, 1990; M.Schwartz et al, 1990;
H.Johansen,1991].

A?aee?eooy aaia IA aoei iia’ycaia c aaeeeeie iaae?yie ia oaeaeeee
i?aeoe/iee ?acoeueoao, aea aeyaeeiny, ui oey aaaeeeaa iiae?y — eeoa
ii/aoie aeiaaiai ? aaaeeiai oeyoo aei iieaaoaiiy aeie? ethaeae, eio?i
no?aaeaeathoue ia ioeia?noeeaeic. Ianooiiei aoaiii, ia iaio cia/iei,
aiiae naia a?aee?eooy aaia, ? inieneaiiy i?ioean?a, yei, a?aeaoaath/enue
a i?aai?ci? iin?y aai?a, ni?e/eiythoue caoai?thaaiiy.

Aaaaoi yei aaoi?e aeacothoue ia ae?aciee ee?i?/iee iie?ii?o?ci ?
??ciiia?ooy aa??aio?a ia?aa?ao IA: a?ae aaaeeeo oi?i ?c eaoaeueiei
eiioeai o ?aiiueiio aeeoeinoa? aei iaeaea oeieeiaeoi? a?aenooiino?
neiioiiaoeee, ca aeiyoeii i?aeaeuaii? eiioeaio?aoe?? aeaeo?ie?o?a iioo
[A.ss.??ciee oa ?i.,1994, I.I.Eai?aiia oa ?i.,1995, A.A. Iaaeia oa
?i.,1992]. Ia oeae /an iienaii i’youe eean?a iooaoe?e, ui i?ecaiaeyoue
aei ii?ooaiiy ooieoe?? oei?eaeiiai eaiaeo. Ee?i?/iee iie?ii?o?ci IA
iia’ycaiee c oeeie ??ciiiai?oieie iooaoe?yie a aai? O?AI [L.Tsui,1995,
L.C.Tsui, P.Durie,1997]. Aea/aiiy iieaeoey?ii-aaiaoe/ieo iniia IA ni?ey?
?icoi?iith eiai iaoiaaiaco ? iie?ioaiith no?aoaa?? e?eoaaiiy [C.Koch et
al,1993]. Ia no/aniiio aoai? aaaeeeaa cia/aiiy ia? aea/aiiy ca’yceo
aaiioeio c oaiioeiiaeie iniaeeainoyie i?iyao caoai?thaaiiy a iiioeyoe??
Oe?a?ie.

?ica’ycaiiy oeeo ieoaiue a Oe?a?i? aeicaieyoue oaeineiiaeeoe ?aiith
ae?aaiinoeeo IA ? cia/ii iie?ioeoe nai?/ania aaeaeaaoia e?eoaaiiy,
aeoeaia aeeniaina?ia niinoa?aaeaiiy oai?eo ae?oae, a oaeiae aaiaoe/ia
eiinoeueooaaiiy c ianooiiei i?iaaaeaiiyi aeiiieiaiai? ae?aaiinoeee a
?ioi?iaoeaieo ?iaeeiao.

Ca’ycie ?iaioe c iaoeiaeie i?ia?aiaie, ieaiaie, oaiaie

Aeena?oaoe?th aoei aeeiiaii a ?aieao aea?aeaaii? iaoeiai-oaoi?/ii?
i?ia?aie “Caoeno aaiioiiaeo ianaeaiiy Oe?a?ie”. Oaia: AeEIO 01.01.01.
/017-92 ? 01. 93. E 020926.

Iaoa ?iaioe

Ioe?ieoe oaiioeiia? i?iyae ci?i a?iioieaaaiaai? nenoaie ? nenoaie
o?aaeaiiy o ae?oae, ui no?aaeaeathoue ia IA, caeaaeii a?ae aaiioeio — c
iaoith iie?ioaiiy i?a- ? iinoiaoaeueii? ae?aaiinoeee oeueiai
caoai?thaaiiy; iioei?coaaoe noaio aaiaoe/iiai eiinoeueooaaiiy ia iniia?
aea/aiiy /anoio iaea?eueo iioe?aieo iooaoe?e aaia O?AI a Oe?a?i?.

Aeey aeinyaiaiiy iinoaaeaii? iaoe aoei noi?ioeueiaaii ianooii?
caaaeaiiy:

1. Aea/eoe ee?i?/i? i?iyae ii?ooaiue a?iioieaaaiaai? nenoaie ? nenoaie
o?aaeaiiy o ae?oae, ye? no?aaeaeathoue ia ioeia?noeeaeic, caeaaeii a?ae
aaiioeio.

2. Aecia/eoe iieacieee o?ce/iiai ?icaeoeo ae?oae, oai?eo ia
ioeia?noeeaeic, caeaaeii iae a?eo, oyaeeino? caoai?thaaiiy ? /ano
anoaiiaeaiiy ae?aaiico.

3. ?ic?iaeoe ?aeiiaiaeaoe?? uiaei oaeineiiaeaiiy aaiaoe/iiai
eiinoeueooaaiiy ? aeiiieiaiai? ae?aaiinoeee a n?i’yo aenieiai ?eceeo
ia?iaeaeaiiy aeeoeie c ioeia?noeeaeicii.

Iaoeiaa iiaecia io?eiaieo ?acoeueoao?a

Aia?oa ia iniia? iiaeeaeaiiai eiiieaeniiai aeine?aeaeaiiy c?iaeaii
ii??aiyeueio oa?aeoa?enoeeo oaiioeiiaeo i?iya?a ci?i a?iioieaaaiaai?
nenoaie ? nenoaie o?aaeaiiy o ae?oae, oai?eo ia IA, caeaaeii a?ae
aaiioeio. O a?oi? aiiiceaio ca iooaoe??th delF508/delF508 a?aecia/aii
iayai?noue oaeeo oneeaaeiaiue, ye iiaaiioi?aen ? ao?ac?y oiiei? eeoee.
Iiaeaii ioe?ieo o?ce/iiai ?icaeoeo ae?oae caeaaeii a?ae aaiioeio, a?eo,
iani-c?inoiaeo ia?aiao??a, oyaeeino? ? o?eaaeino? ia?aa?ao
ioeia?noeeaeico. Iieacaii, ui o ae?oae, oai?eo ia IA, nooi?iue
aeeoaeueii? iaaeinoaoiino? aa??th?oueny iacaeaaeii a?ae iaie?aaoe/iiai
noaoono.

I?aeoe/ia oe?ii?noue io?eiaieo ?acoeueoao?a

Iioeieciaaii noaio aaiaoe/iiai eiinoeueooaaiiy ? aeiiieiaiai?
ae?aaiinoeee ia iniia? aea/aiiy /anoio iaeiioe?ai?oeo iooaoe?e aaia O?AI
a Oe?a?i?. Iieacaii iaiao?aei?noue iieaeoey?ii-aaiaoe/ieo
aeine?aeaeaiue, ni?yiiaaieo ia eeoa ia oai?o aeeoeio, a e ia ??
aaouee?a, oa cae?enithaaieo ?c canoinoaaiiyi i?yii? aeaoaeoe?? iooaoe?e
aaia O?AI ? n?iaeiiai IAe?O- aiae?co.

Aeiaaaeaii aeioe?euei?noue aeei?enoaiiy ?acoeueoao?a
iieaeoey?ii-aaiaoe/ieo aeine?aeaeaiue, i?iaaaeaieo o iiai?e n?i’? i?ae
/an aaiaoe/iiai eiinoeueooaaiiy, ui aeicaiey? cia/ii i?aeaeueoe
ae?aaiinoe/io ?ioi?iaoeai?noue n?iae a?oie aenieiai ?eceeo IA (aei
95,5%). Oea ia? aaaeeeaa iaaeeei-nioe?aeueia cia/aiiy.

O iinoiaoaeuei?e ae?aaiinoeoe? cai?iiiiiaaii canoiniaoaaoe iieacieee
iani-c?inoiaiai ?iaeaeno aeey ioe?iee nooiaiy oyaeeino? ? i?iaiico
ia?aa?ao caoai?thaaiiy, a oaeiae aeey ioe?iee aoaeoeaiino? e?eoaaiiy.

?acoeueoaoe i?iaaaeaiiai aeine?aeaeaiiy cai?iaaaeaeaii a i?aeoeeo ?iaioe
iaaeeei-aaiaoe/ii? eiinoeueoaoe?? ?inoeoooo iaae?ao???, aeooa?noaa ?
a?iaeieia?? AII Oe?a?ie, a?aeae?eaiiy iaaeeoeeie ieiaea. Iaoa??aee
aeena?oaoe?? aeei?enoaii o iaoiaee/ieo ?aeiiaiaeaoe?yo “Ae?aaiinoeea,
e?eoaaiiy oa i?io?eaeoeea ioeia?noeeaeico”, Iaeana, 1999. Aeioe?eueiei ?
?oi? iiaeaeueoa ai?iaaaeaeaiiy a e?eoaaeueii-ae?aaiinoe/io ae?yeuei?noue
niaoe?ae?ciaaieo a?aeae?eaiue ? aaiaoe/ieo oeaio??a, ui iaaeathoue
aeiiiiiao oai?ei ia ioeia?noeeaeic.

Iniaenoa o/anoue aeena?oaioa

Aeena?oaoe?eia ?iaioa ? iniaenoei o?oaeii aaoi?a. Naiino?eii cae?eniaii
aeia?? ?ai?acaioaoeaii? a?oie ae?oae, oai?eo ia ioeia?noeeaeic ??ciiai
nooiaiy oyaeeino?. Ocaaaeueiaii ? i?iaiae?ciaaii ?acoeueoaoe ee?i?/iiai
? iieaeoey?ii-aaiaoe/iiai aeine?aeaeaiiy ia?aii? a?oie ae?oae.
I?iaaaeaii aio?iiiiao?e/ia ianoaaeaiiy niinoa?aaeoaaieo ae?oae, iiaeaii
eiai ioe?ieo a ia?oeaioeeyo ? ca iani-c?inoiaei ?iaeaenii. I?iaaaeaii
iaaeeei-aaiaoe/ia eiinoeueooaaiiy ?iaeei aenieiai ?eceeo IA. Naiino?eii
ii?aoeueiaaii ?acoeueoaoe ?iaioe, cae?eniaii noaoenoe/iee aiae?c
io?eiaieo aeaieo ? i?aeaioiaeaii oaeno aeena?oaoe??. ?ic?iaea, aiae?c ?
?ioa?i?aoaoe?y iaoa??aeo, aeeeaaeaiiai a aeena?oaoe??, iaeaaeeoue
aaoi?o.

Ai?iaaoe?y ?acoeueoao?a aeena?oaoe??

?acoeueoaoe i?iaaaeaii? ?iaioe aoei ii?eethaeiaii o aeiiia?ae? ia II
c’?cae? iaaee/ieo aaiaoee?a (i. Aeia-Aoa, 1991), VI c’?cae?
Oe?a?inueeiai oiaa?enoaa aaiaoee?a ? naeaeoe?iia??a ?i. I.?. Aaaeeiaa
(Iieoaaa, 1992), O c’?cae? aeooa??a-a?iaeieia?a Oe?a?ie (Iaeana, 1996),
4-io Iaoe?iiaeueiiio eiia?an? c ioeia?noeeaeico (Iineaa, 1998), II
c’?cae ooec?ao??a ? ioeueiiiieia?a Oe?a?ie (Ee?a,1998)

Ioae?eaoe??

Ii oai? aeena?oaoe?? iioae?eiaaii 11 ?ia?o. O oiio /ene? 4 aeo?iaeuei?
noaoo? (AEo?iae i?aeoe/aneiai a?a/a, 1997; Iaae?ao??y, aeooa?noai oa
a?iaeieia?y, 1998, 1999; ECCACF Newsletter, 1996).

Ianya oa no?oeoo?a ?iaioe

Aeena?oaoe?eia ?iaioa, aeeeaaeaia ia 150 noi??ieao iaoeiiieno,
neeaaea?oueny ?c anooio, iaeyaeo e?oa?aoo?e, 4 ?icae?e?a aeanieo
aeine?aeaeaiue, aiae?co e ocaaaeueiaiiy aeanieo aeine?aeaeaiue,
aeniiae?a, nieneo aeei?enoaieo aeaea?ae. Oaeno aeena?oaoe?? ?ethno?iaaii
25 oaaeeoeyie oa ?enoieaie. Nienie aeei?enoaieo aeaea?ae i?noeoue 183
iacae.

INIIAIEE CI?NO

Ia’?eo oa iaoiaee aeine?aeaeaiiy

Aeey ?ica`ycaiiy iinoaaeaieo caaaeaiue iaie aoei ianoaaeaii 111 ae?oae,
oai?eo ia ioeia?noeeaeic, a?eii iae 3 i?nyoe?a aei 15 ?ie?a ?c 100
?iaeei.

?iciiae?e ca noaooth ? a?eii: oeii/ee?a — 62, ui noaiiaeei 56%, a
ae?a/aoie — 49 – 44% a?aeiia?aeii. Ae?oae aei 1 ?ieo — 57 (51,4%), o
a?oe? 1-3 ?iee — (18%), 4-7 ?ie?a — 23 (20,7%), 8-12 ?ie?a — 9 (8,1%),
i?ney 14 ?ie?a — 2 (11,8%). Ca aoi?/iei neeaaeii niinoa?aaeoaaieo ?iaeei
iaoe??ioe ?iciiae?eyeeny o oaeee niin?a: 86 (77.4%) — oe?a?ioe?, 5
(4,5%) — ?in?yie, 2 (1,8%) — iieaeaaaie, a 18 ae?oae — a?ae ci?oaieo
oetha?a i?ae oe?a?ioeyie ? ?in?yiaie.

On?o oai?eo aoei i?aeaeaii eiiieaeniiio ee?i?/iiio, eaai?aoi?iiio ?
ooieoe?iiaeueiiio ianoaaeaiith, ui aeeth/aei ieceo niaoe?aeueieo
iaoiae?a – AeIE-aiae?c, aiae?c oei?eae?a iioiai? ??aeeie,
?aioaaiieia?/ia ianoaaeaiiy i?aai?a a?oaeii? ee?oeie;
aeaeo?iea?ae?ia?ao?th, aoiea?ae?ia?ao?th, ni??ia?aoeth, OCAe-
ae?aaiinoeeo i?aai?a /a?aaii? ii?iaeieie, aeine?aeaeaiiy ia?oeeaeueii?
iai?oae eenith a e?ia?. Ioe?ieo nooiaiy oyaeeino? caoai?thaaiiy
i?iaiaeeee ca oeaeith Oaaoiaia-A?anoeeaea, ca?aeii c iiaeeo?eaoe??th
A.?a/einueeiai ? I.Eai?aiiaa [1985].

Iieaeoey?ii-aaiaoe/i? aeine?aeaeaiiy, yeeie aoei ioiieaii oai?eo ae?oae,
?oi?o aaouee?a ? nean?a, cae?enieea a?oia iieaeoey?ii? ae?aaiinoeee
niaaeeiaeo oai?ia ?inoeoooo Iieaeoey?ii? a?ieia?? ? aaiaoeee AI Oe?a?ie
(ea??aiee a?oie eaiaeeaeao a?ieia?/ieo iaoe E.A. E?aoeoeue). AeIE-aiae?c
aeeth/aa aecia/aiiy naie iooaoe?e o 7, 10, 11 aeciiao aaia O?AI:
delF508, 1677 delTA (10 aecii), R553X, G551D (11 aecii),
R334W,1154insTC, del5491 (7 aecii), a oaeiae n?iaeiee aiae?c iie?ii?oieo
AeIE – ia?ea??a, u?eueii c/aieaieo ?c aaiii IA o nenoaiao (KM-19/Pstl
XV2C TGg1 ? J3. 11Pst). ?aeaioeo?eaoe?y iooaoe?e cae?enithaaeany ca
aeiiiiiaith iie?ia?acii? eaioethaiai? ?aaeoe?? neioaco AeIE (IOe?)
[J.Kenneth et al,1990], ?c ianooiiith ?aeaioeo?eaoe??th iayaiino? aai
a?aenooiino? a?aeiia?aeieo iooaoe?e ca aeiiiiiaith aeaeo?ioi?aco.
Niaoeeo?/i?noue IOe? aecia/aeany ia?ith ie?aii?aeia??a, eiiieaiaioa?ieo
e?ioeyi oeaieoth/eo ae?eyiie aiie?o?eaoe??. IOe? i?iaiaeeee ia
oa?iioeeeea?? “Perkin Elmer” (o??ia “Cetus”, NOA) ca noaiith,
cai?iiiiiaaiith Salki [R.K. Saiki et al,1988]. Ca ?acoeueoaoaie
AeIE-aiae?co a 57,2% aeiaaee?a aeyaeaii oae? iooaoe?? aaia O?AI: delF508
– 56,3%; R553X – 0,45%; G551D – 0,45%, ? oai?eo ae?oae aoei iiae?eaii ia
o?e aaiioeiia? a?oie: ia?oa – ?c aaiioeiii delF508/delF508, ae?oaa –
delF508/x ? o?aoy – x/x.

Ae?aaiic i?aeoaa?aeaeoaaany i?aeaeuaiith eiioeaio?aoe??th oei?eae?a
iioo, ui noaiiaeea a na?aaeiueiio ii a?oi? 96,38+3,87 iiieue/e.
Aecia/aiiy eiioeaio?aoe?? aeaeo?ie?o?a o iio? aeeiioaaeiny ca
aeiiiiiaith i?eiea?i?iiaiai aeaeo?ioi?aco ca iaoiaeii A?aniia ? Eoea,
[L.Gibson, R.E Cooke,1959]. I?aeaeuaiiy eiioeaio?aoe?? oei?eae?a iioiai?
??aeeie a?aecia/aeiny o an?o aaiioeiiaeo a?oiao (oaaeeoey 1).

A?e, o yeiio aia?oa ae?aaiinooaaany ioeia?noeeaeic, eieeaaany a?ae 1
i?nyoey aei 14 ?ie?a ? ii a?oi? a na?aaeiueiio noaiiaea 19,15+3,18
i?nyoey.

Oaaeeoey 1

Na?aaei? aaee/eie eiioeaio?aoe?? aeaeo?ie?o?a iioo (iiieue/e) o ae?oae,
oai?eo ia ioeia?noeeaeic, caeaaeii a?ae oyaeeino? caoai?thaaiiy ?
aaiioeio

A?oie ca

aaiioe-

iii Caaeia?eueiee

ia?aa?a Na?aaeiuei-

oyaeeee

ia?aa?a Oyaeeee

ia?aa?a Na?aaei?

cia/aiiy

aeaeo?ie?oo 1. delF508/

delF508

n = 44

101,0 + 1,0

106,9 + 6,6

101,6 + 4,9

103,0 + 3,92. delF508/x

n = 36 83,9 + 12,8 84,8 + 6,0 93,9 + 4,9 89,0 + 3,83. x-/-x

n = 30 76,5 + 8,1 93,3 + 5,2 99,2 + 7,1 94,8 + 4,4

A?eueo?noue niinoa?aaeoaaieo ae?oae iio?aieee aei ee?i?ee a ia??iae
caaino?aiiy a?iioieaaaiaaiai i?ioeano (?o ianoaaeoaaee o?eueee
oi?iaeiaae oeueiai ia??iaeo).

Ca aeaieie ee?i?ei-aaiaaeiae/iiai aiaiiaco, on? oai?? ae?oe aoee
ia?iaeaeai? a?ae caei?iaeo aaouee?a. Na?aaei?e a?e aaouee?a aei iiiaioo
iieia?a: iaoe – 24,7 ?ie?a, aaoueei – 26.5 ?ie?a.

O aeine?aeaeoaaieo oai?eo ae?oae ia?o? iciaee caoai?thaaiiy aeyaeyeeny:
a?ae ia?iaeaeaiiy — o 53 ae?oae (47,7%); o a?oe? aei 3-o i?nyoe?a — o 33
(29,7%), aei iaeiiai ?ieo — o 20 (18%). Oaeei /eiii, o a?eueoino? ae?oae
(106 oai?eo, 95%) ia?o? ee?i?/i? iciaee caoai?thaaiiy ?icaeaaeeny aaea
ia ia?oiio ?ioe? aeeooy.

A?aeiia?aeii aei aeaieo ee?i?/ii? ea?oeie e ia’?eoeaiiai aeine?aeaeaiiy,
ci?oaia oi?ia ioeia?noeeaeica niinoa??aaeany o 88 ae?oae (79,3%); o 8
aeiaaeeao (7,2%) – eaaaiaaa oi?ia, o 15 aeiaaeeao (13,5% %) – eeoeiaa
oi?ia (oaaeeoey 2).

Oaaeeoey 2

?iciiae?e oai?eo ia ioeia?noeeaeic ca oi?iith caoai?thaaiiy

caeaaeii a?ae aaiioeio

O i ? i a del508/delF508 del F508/ x x / xO n ue i a i
caoai?thaaiiye?euee. %e?euee. %e?euee %e?euee. Ci?oaia 38 86,4 30 81,1
20 66,6 8879,3 Eaaaiaaa– 3 8,1 5 16,7 8 7,2 Eeoeiaa 6 13,6 4 10,8 5
16,7 1513,5 Onueiai 44 39,6 37 33,4 30 27,0 111

Ci?ie o noai? a?iioieaaaiaai? nenoaie ia ia?oiio ?ioe? aeeooy iinoaee o
88% ae?oae. Na?aaei?e a?e i?iyao ?ani??aoi?iiai neiae?iio noaiiaea 5,3
i?nyoey. O 22 ?c oeeo ae?oae (23,4%) ?ani??aoi?iee neiae?ii iinoaa aei
ia?oiai i?nyoey aeeooy. Ia?oeie eiai i?iyaaie aoei eaaea iieaoeeoaaiiy
(aac aai ia oii? ?ani??aoi?ii? a??onii? ?ioaeoe??) ?c iinooiiaei
?icai?oaiiyi oa?aeoa?ieo ee?i?/ieo i?iya?a oai?iae: caoyaeiee aai
iino?eiee i?enooiiiiae?aiee eaoaeue c aeaaeith, ai?eiith aaaeei
a?aeoa?eoaaiith iie?ioith, aieia? o?eie a eaaaiyo, ui caa??aathoueny
i?ney e?ea?aeaoe?? aino?eo yaeu.

Na?aaei?e a?e i?iyao eeoeiaiai neiae?iio noaiiaea 2,1+0,3 i?nyoey. O 38
aeiaaeeao (37%) eeoeiaee neiae?ii ?icii/aany a?ae ia?iaeaeaiiy. O 16
ae?oae (14,4%) caoai?thaaiiy iai?oanooaaei iaeii??aei ?eaonii.

C-iii?ae oneeaaeiaiue caoai?thaaiiy aeeaaiinoiaaii: niiioaiiee
iiaaiioi?aen (3,6%), aoaeaeoac (5,4%), na?oeaai-eaaaiaaa iaaeinoaoi?noue
?c ?icaeoeii eaaaiaaiai na?oey (26,1%) ? eiai aeaeiiiainaoe??th (4,4%),
a?e?a?iee oee?ic ia/?iee (2,7%). O ia??iae? niinoa?aaeaiiy o 6,7%
a?aecia/aeeny iciaee aino?iai neioneoa, 3,3% ae?oae iaee iieeiic
iiniaeo oiae?a, 5,6% – aaeaii?aee II ? III nooiaiy.

Aeey eiiieaenii? ioe?iee o?ce/iiai ?icaeoeo aeei?enoiaoaaee ye
ia?oeaio?euei? noaiaea?oe, oae ? iani-c?inoiaee ?iaeaen (IC?),
?ic?iaeai? I.M. Tanner, ui aeicaieya ioe?ieoe iieacieee iane o?ea o
ca’yceo c ?inoii (ii?iith aaaaea?oueny IC? a?ae 110 aei 90 a?aenioe?a)
[Iaco??i A.A. ? ?i.,1985; I.M. Tanner et al, 1966].

Iani-c?inoiaee ?iaeaen ?ic?aoiao?oueny o oaeee niin?a:

(oaeoe/ia aaaa)/(?aeaaeueia aaaa a?aeiia?aeii aei c?inoo ? noao?) o
100%.

A?aenoaaaiiy o o?ce/iiio ?icaeoeo aoei aeyaeaii o 87 (78,4%) ae?oae
noiniaii aaae e o 72 (64,8%) noiniaii c?inoo (iani-c?inoia? iieacieee
ieae/? ca 10 ia?oeaioee?a). I?e oeueiio ae?aci? ii?ooaiiy (iaio ye 3
ia?oeaioeey) noiniaii aaae aeyaeaii o 58,6% oai?eo, noiniaii c?inoo – o
37,7%. Na?aaei? iieacieee aaae o an??? a?oi? noaiiaeee 12,72+1,85
ia?oeaioee?a, c?inoo — 25,0+3,1 ia?oeaioee?a. Iani-c?inoiaee ?iaeaen aoa
ieae/ee ca ii?io ? aei??aithaaa 84,3% o an??? a?oie.

?aioaaiieia?/ia ea?oeia caeaaeaea a?ae o?eaaeino? oai?iae, oyaeeino?
noaio ? oace caoai?thaaiiy. Iaea?eueo oa?aeoa?iith aoea caaaeueia
aio?caiaoici?noue (68%), a oaeiae ii?aeiaiiy ae??aiiaoeueicieo caeooo?a
?c noiauaiiyi no?iie a?iio?a, e?eueoeaiiae?aieo ? e?i?eieo ooai?aiue,
e?no (60%) ia oii? aeainoi?iiiueiai aeeooc?eiiai eaaaiaaiai ?enoiea.
?aioaaiieia?/iee ?iaeaen o na?aaeiueiio ii a?oi? aei??aithaaa 12,20+0,64
aaeai.

Aeine?aeaeaiiy ooieoe?? ciai?oiueiai iiaeeoo o a?eueoino? oai?eo ae?oae
(14 oai?eo – 85%) aeyaeei eiia?iiaaio oi?io aaioeeyoe?eieo ii?ooaiue, a
o aeaio ae?oae — ?ano?oeoeaio oi?io. O 28% ae?oae iieacieee ooieoe??
ciai?oiueiai iiaeeoo aoee a ii?i?. Aaioeeyoe?eio iaaeinoaoi?noue I-II
nooiaiy aoei aeyaeaii o o?ueio oai?eo, III nooiaiy – o 4-o ae?oae, IV
nooiaiy – o 3-o ae?oae. Aiae?c aeaieo aeaeo?iea?ae?ia?aie aeyaea o
a?eueoino? oai?eo (86%) ii?ooaiiy iaoaaie?/ieo i?ioean?a o i?iea?ae?
a?iiene/iiai oa?aeoa?o; aoei oaeiae neioniao oao?ea?ae?th (72,4%),
ia?aaaioaaeaiiy i?aaeo a?aeae?e?a na?oey (30,6%). Aoiea?ae?ia?ao?y
aeyaeea o 10 (10,2%) oai?eo a?ia?o?io?th i?iea?aea i?aaiai oeoii/ea, o
42%) oai?eo — cieaeaiiy nei?ioiino? i?iea?aea.

Aeey aiae?co aeaieo canoiniaaii iaoiae ia?aeiiiai noaoenoe/iiai
oii?yaeeoaaiiy, aeenia?n?eiee, ei?aeyoe?eiee, aeene?ei?iaioiee ?
?aa?an?eiee iaoiaee. Aeey iiaiiai aeyaeaiiy aieeao aaiioeio ia i?iyae
ee?i?/ieo iciae caoai?thaaiiy ie aaeaeeny aei ?ioaa?aeueieo iieaciee?a.
Aeey ?oiuei? ioe?iee canoiniaoaaeany aaoi?nueea iaoiaeeea I.TH.
Aioaiiiiaa [I.Aioaiiiia, 1991]. Oii?yaeeoaaiiy cae?enithaaeiny ia
ia?niiaeueiiio eiii’thoa?? a iaeao? “Statistica” [I.Ieioea? oa ?i.,1991;
A.Iaeneiia oa ?i.,1983; A.Eae?i,1980; I.Aioaiiiia, 1991].

?acoeueoaoe aeine?aeaeaiiy oa ?o iaaiai?aiiy

A?iioieaaaiaa? ci?ie aeii?iothoue o ee?i?/i?e ea?oei? ioeia?noeeaeico,
aecia/athoue eiai ia?aa?a ? i?iaiic. C iaoith aea/aiiy ca’yceo i?ae
oeiii iooaoe?? ? ee?i?/ieie aa??aioaie i?io?eaiiy ?ani??aoi?iiai
i?ioeano iaie aoei aea/aii iniaeeaino? o?aaeaiiy a?iioieaaaiaai? nenoaie
o ae?oae caeaaeii a?ae aaiioeio. O a?oi? aiiiceaio a?aecia/a?oueny ?aiiy
iai?oanoaoe?y caoai?thaaiiy, o 62% ae?oae ia?o? iciaee caoai?thaaiiy
iinoaee a?ae ia?iaeaeaiiy, o o?aoeie ?c ieo aoee i?iyae oeiiaiai
?ani??aoi?iiai neiae?iio (nooee eaoaeue a?aioe?, i?enooiiiiae?aiee,
iiaeaeiee eieethoiiiae?aiee, ?c aaaeei a?aeie?aiethaaiith aeaaeith
iie?ioith). O 38% aeiaaee?a iciaee caoai?thaaiiy c’yaeeeny o 6-i?ny/iiio
a?oe?. Ia ia?oiio ?ioe? aeeooy o 94% ae?oae ?icaeioany ?ani??aoi?iee
neiae?ii. Na?aaei?e a?e eiai i?iyao noaiiaeoue o a?oi? 3,2+0,76 i?nyoey.
Na?aaeiy aaee/eia PaO2 – ia?aiao?a, yeee iaeae?aci?oa a?aeaeca?eaeth?
eeniao?ainii?oio ooieoe?th i?aai?cio, aei??aithaaea 61,1+2,81 ii ?o. no.
O oe?e a?oi? caaino?aiiy a?iioieaaaiaaiai i?ioeano aoee iae/ano?o? e
i?io?eaee iaeaaae/a (ia c?acie iiaaiii?? o 84%). Aeey ?aioaaiieia?/ii?
ea?oeie aoee oa?aeoa?i? caeoooy eaaaiaai? oeaieie (a?ia??ioeyoe?y),
iineeaiiy ? aeaoi?iaoe?y a?iioinoaeeiiiai ?enoiea, ina?aaeeia?, ??aeoa
naaiaioa?i? ? /anoeia? ?io?eueo?aoe. ?aioaaiieia?/iee ?iaeaen ca
A?anoeeaeii aei??aithaaa o oe?e a?oi? 10,5 aaea. O 16% ae?oae
a?iioieaaaiaaee i?ioean a?aeaoaaany ?c aoyaoaaiiyi a?iio?a ??ciiai
eae?a?o, ia c?acie a?iio?oo.

Oaaeeoey 3

Nooi?iue aeeoaeueii? iaaeinoaoiino? ae?oae, oai?eo ia ioeia?noeeaeic,

caeaaeii a?ae aaiioeio

Nooi?iue aeeoaeueii? iaaeinoaoiino? Aiiiceaioe ca delF508/delF508
Aaoa?iceaioe ca delF508/ x x / x

AeI 0 no. –26,34,2AeI I no. 12,925,016,7 AeI II no. 25,834,029,1 AeI
III no. 58,031,350,0 AeI IV no. 3,33,0–

sse aeaeii c oaaeeoe? 3, c?noaaeaiiy ee?i?/ieo ? ooieoe?iiaeueieo
iieaciee?a o a?oi? delF508/delF508 aeyaeei o 2/3 ae?oae AeI-III no.
(Z=2,69, p

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