2

Oe?a?inueeee iaoeiaee a?a??i?/iee oeaio? IIC Oe?a?ie

Aoaianue?aa Iaoaey Ieaen??aia

OAeE 616-056.7-053.2-07(477.75):577.213.3

?ICIIANTHAeAEAI?NOUe OA EE?I?EI-AAIAOE*IA

OA?AEOA?ENOEEA AeAssEEO IIIIAAIIEO OAI?IA

O Ae?OAE AAOIIIIII? ?ANIOAE?EE E?EI

03.00.15 — aaiaoeea

AAOI?AOA?AO

aeena?oaoe?? ia caeiaoooy iaoeiaiai nooiaiy

eaiaeeaeaoa iaaee/ieo iaoe

Ee?a-1999

Aeena?oaoe??th ? ?oeiien

?iaioa aeeiiaia a Oe?a?inueeiio iaoeiaiio oeaio?? iaaee/ii? aaiaoeee IIC
? IAI Oe?a?ie.

Iaoeiaee ea??aiee — aeieoi? iaaee/ieo iaoe, i?ioani?, Aa?eeye ?ai?
?iiaiiae/, Oe?a?inueeee iaoeiaee oeaio? iaaee/ii? aaiaoeee IIC ? IAI
Oe?a?ie, aee?aeoi?.

Io?oe?ei? iiiiaioe:

aeieoi? iaaee/ieo iaoe Aaa?eethe TH??e Eineiiae/, Euea?anueeee IAe?
niaaeeiai? iaoieia?? IIC Oe?a?ie, caa?aeoaa/ eaai?aoi???th;

eaiaeeaeao iaaee/ieo iaoe Neeaai Aaeeia Aieiaeeie??aia, ?inoeooo
iaae?ao???, aeooa?noaa ? a?iaeieia?? AII Oe?a?ie, caa?aeoaa/ea
iie?ee?i?eith.

I?ia?aeia onoaiiaa — Ee?anueea iaaee/ia aeaaeai?y i?neyaeeieiiii?
ina?oe ?i.I.E.Ooi?ea, eaoaae?a aeooa?noaa, a?iaeieia?? oa ia??iaoieia??,
IIC Oe?a?ie, i. Ee?a

Caoeno a?aeaoaeaoueny “ 22 ” aeiaoiy 1999 ?. i 10 00 aiaeei? ia
can?aeaii? niaoe?ae?ciaaii? a/aii? ?aaee Ae 26.604.01 i?e Oe?a?inueeiio
iaoeiaiio a?a??i?/iiio oeaio?? IIC Oe?a?ie (253660, i.Ee?a-94, aoe.
Iiioae?aiea, 50)

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

Aaoi?aoa?ao ?ic?neaiee “ 7 ” aa?aniy 1999?.

A/aiee nae?aoa?

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

CAAAEUeIA OA?AEOA?ENOEEA ?IAIOE

Aeooaeuei?noue oaie. Iaeiie/aiiy oa nenoaiaoecaoe?y aeaieo i?i
iioe?ai?noue ? ee?i?ei-aaiaoe/i? iniaeeaino? niaaeeiaeo oai?ia
iaaoaathoue ana a?eueoiai i?aeoe/iiai cia/aiiy o ca,yceo c? ca?eueoaiiyi
aianeo niaaeeiai? iaoieia?? a caoai?thaai?noue ? nia?oi?noue ia o?eueee
aeeoy/iai, aea e aei?ineiai ianaeaiiy [Aiaaoe?ueiaa?.A., Aa?eeye ?.?.,
Oei/aiei I.?. oa ?i., 1997, Na?aethe A.I., Aa?eeye ?.?., Oei/aiei I.?.,
Neeaai A.A., 1995]. Oiio ?aa?iiaeuei? aeine?aeaeaiiy iioe?aiino? ?
ee?i?/ieo iniaeeainoae niaaeeiaeo caoai?thaaiue iiiiaaiii? i?e?iaee
iathoue iaaecae/aeii aaaeeeaa cia/aiiy aeey ieaioaaiiy a aaeoc? ioi?iie
caei?ia’y c o?aooaaiiyi iio?aa ianaeaiiy o iaaeeei-aaiaoe/i?e
aeiiiiic?, ?ic?iaee i?ia?ai i?io?eaeoeee ? e?eoaaiiy oeeo
caoai?thaaiue. Ieoiia aaaa oaeeo aeine?aeaeaiue noa? ana a?eueo
nooo?aith a aaaaoueio e?a?iao na?oo, a i?iaeaie e?eoaaiiy ? i?io?eaeoeee
niaaeeiaeo aaaeeeo caoai?thaaiue ae??oothoueny a ?aieao iaoe?iiaeueieo
i?ia?ai. A Oe?a?i? oaeeie i?ia?aiaie ? iaoe?iiaeueia i?ia?aia “Ae?oe
Oe?a?ie” oa aea?aeaaia i?ia?aia “Caoeno aaiioiiaeo ianaeaiiy Oe?a?ie”.

Iioe?ai?noue niaaeeiaeo oai?ia ?noioii a?ae??ciy?oueny a ??cieo
iiioeyoe?yo oa aoi?/ieo a?oiao [McKusick B.A., 1994]. Iaea?eueo iaae?ei?
a?aeiiino? a?aeiinii ?iciianthaeaeaiino? iiiiaaiieo caoai?thaaiue
iiaeooue aooe io?eiai? oeyoii i?iaaaeaiiy i?ia?ai ianiaiai ne?ei?iao
[E?aniiiieuenueea E.Ae., 1987]. Ia nueiaiaei? a Oe?a?i? ?aeeiith oaeith
i?ia?aiith ? i?ia?aia ianiaiai ne?ei?iao iiaiia?iaeaeaieo ia
oai?eeaoiio??th (OEO). Caaaeyee aeei?enoaiith oe??? i?ia?aie c’yaeeany
iiaeeea?noue aecia/aiiy a eiaeiiio ?aa?ii? iioe?aiino? OEO, yea a
oe?eiio ii Oe?a?i? aecia/aia ye 1:8300 [Aa?eeye ?.?., Noaiio?nueeee
TH.I., 1995], aea aa??th? a?ae 1:12000 a E?eaiio ?ic? [*enoee A. Ae.,
AEeeueoeiaa ?. A., Aa?iiioaaeyi I. I., 1990] aei 1:4419 a *a?i?a?anuee?e
oa Iieoaanuee?e iaeanoyo [Noaiaiaoeue A. I., A?eia?iaaoeue I. I.,
A?a/ai?ia I. ss., 1990].

Aea/aiiy aea ?iciianthaeaeaiino? niaaeeiai? iaoieia?? ia?aiai? nenoaie
a iiioeyoe?? oneeaaeiaia a ia?oo /a?ao /a?ac a?aenooi?noue i?ia?ai
ianiaiai ne?ei?iao, a oaeiae /a?ac ??ciiiai?ooy ee?i?/ieo oi?i oa
neeaaeiino? aeeoa?aioe?eii? ae?aaiinoeee. Ine?eueee a?eueo?noue
ia?aiai-i’yciaeo caoai?thaaiue iai?oanoo? a aeeoy/iio a?oe?, oi
i?aaii??ia aea/aiiy ?o na?aae aeeoy/iai ianaeaiiy. A Oe?a?i? oae? aeai?
aoee io?eiai? eeoa aeey aeeoy/iai ianaeaiiy Aeiiaoeueei? iaeano?
[Naaeaeia ?.A., ?aoooaiei N.E., 1989]. A Aaoiiiii?e ?anioae?oe? E?ei
(A?E) oae? aeine?aeaeaiiy ia i?iaiaeeeeny.

No??ieee ?icaeoie iaoiae?a iieaeoey?ii? aaiaoeee a ?icoeo?iaoe? oiiei?
no?oeoo?ii-ooieoe?iiaeueii? i?aai?caoe?? aaiiia ethaeeie, ea?ooaaiiy ia
o?iiiniiao aaaaoueio no?oeoo?ieo aai?a i?ecaaei aei cia/iiai i?ia?ano a
aaeoc? iaaee/ii? aaiaoeee, aeicaieeei ?ic?iaeoe iaoiaee oi/ii?
ae?aaiinoeee oa iiao no?aoaa?th i?io?eaeoeee niaaeeiaeo caoai?thaaiue.
Aecia/aiiy oeio iooaoe?e a aaiao /eai?a ?iaeei aenieiai ?eceeo
iiiiaaiieo oai?ia, oaeeo ye OEO, ni?iaeueia i’yciaa ao?io?y (NIA) ?
i’yciaa aeeno?io?y Aethoaiia (IAeAe), aeicaiey? i?iaiaeeoe i?aiaoaeueio
ae?aaiinoeeo oa aecia/aiiy aaoa?iceaioiiai iin?enoaa c aeei?enoaiiyi
i?yieo iaoiae?a aiae?co AeIE ee?oei ieiaeo. Aea iioe?ai?noue ??cieo
oei?a iooaoe?e a ?aa?iiaeueieo iiioeyoe?yo oa aiae?c ?o anioe?aoe?? c
ee?i?/ieie oi?iaie oeeo niaaeeiaeo caoai?thaaiue aeiaaathoue
iiaeaeueoiai aea/aiiy. Iiae?aieo aeine?aeaeaiue o e?einuee?e iiioeyoe??
ia i?iaiaeeeiny.

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

?iaioa aeeiiaia ca?aeii c oaiith iaoeiaeo aeine?aeaeaiue Oe?a?inueeiai
iaoeiaiai oeaio?o iaaee/ii? aaiaoeee IIC ? IAI Oe?a?ie “?ic?iaea
iaoiae?a iieaeoey?ii-aaiaoe/ii? ae?aaiinoeee iaea?eueo iioe?aieo a
Oe?a?i? iae?iaeaaaia?aoeaieo caoai?thaaiue oa ?icoiiai? a?aenoaeino?
niaaeeiai? i?e?iaee” (iiia? aea?aeaaii? ?a?no?aoe?? – 0197UAe19333, oeo?
oaie ?i.109.98.).

Iaoa aeine?aeaeaiiy Aecia/eoe ?iciianthaeaeai?noue ? aeaoe
ee?i?ei-aaiaoe/io oa?aeoa?enoeeo aeayeei niaaeeiaei caoai?thaaiiyi
iiiiaaiii? i?e?iaee na?aae aeeoy/iai ianaeaiiy A?E.

Caaaeaiiy aeine?aeaeaiiy:

1. Aecia/eoe iieacieee iioe?aiino? OEO ? niaaeeiai? ia?aiai-i’yciai?
iaoieia?? na?aae aeeoy/iai ianaeaiiy A?E.

2. Ioa?aeoa?ecoaaoe ??ci? ee?i?/i? oi?ie OEO, NIA, IAeAe o oai?eo c
??cieie oeiaie iooaoe?e aai?a, ui ?o aeaoa?i?iothoue.

3. Noai?eoe aaie c?ace?a e?ia? /eai?a n?iae c aenieei ?eceeii OEO, NIA
? IAeAe, ye? i?iaeeaathoue a E?eio.

4. Aecia/eoe /anoioo cono??/aeueiino? aeayeeo iooaoe?e o oai?eo ia OEO,
NIA ? IAeAe a ?aa?ii?.

Iaoeiaa iiaecia iaea?aeaieo ?acoeueoao?a O ?iaio? aia?oa aoea aecia/aia
iioe?ai?noue ia?aiai-i’yciaeo caoai?thaaiue ? OEO na?aae aeeoy/iai
ianaeaiiy A?E, /anoioe iaoieia?/ieo aai?a oa aaoa?iceaioiiai iin?enoaa
OEO ? NIA, i?iaiae?ciaai? ??ci? ee?i?/i? oi?ie caoai?thaaiue OEO, NIA ?
IAeAe o e?einuee?e iiioeyoe??. Aia?oa i?iaaaeaii iieaeoey?ii-aaiaoe/ia
ianoaaeaiiy a ?iaeeiao aenieiai ?eceeo OEO, NIA ? IAeAe c E?eio,
io?eiai? aeai? i?i /anoioo cono??/aeueiino? aeayeeo oei?a iooaoe?e aai?a
o oai?eo ia ??ci? ee?i?/i? oi?ie oeeo caoai?thaaiue. Aeei?enoaiiy
?acoeueoao?a iieaeoey?ii-aaiaoe/iiai aiae?co aeicaieeei noai?eoe iiaee
i?aeo?ae aeey iaaeeei-aaiaoe/iiai eiinoeueooaaiiy a ?iaeeiao aenieiai
?eceeo OEO, NIA ? IAeAe, ni?yiiaaiee ia aaciina?aaei? aecia/aiiy
aaoa?iceaioiiai iin?enoaa oa i?aiaoaeueii? ae?aaiinoeee.

I?aeoe/ia cia/aiiy iaea?aeaieo ?acoeueoao?a Aeai? i?i iioe?ai?noue
OEO oa iniiaieo iicieia?/ieo oi?i ia?aiai-i’yciaeo niaaeeiaeo
caoai?thaaiue iaiao?aei? i?e ieaioaaii? e?eoaaeueii-i?io?eaeoe/ieo
caoiae?a o ?aa?ii?. ?acoeueoaoe aeine?aeaeaiiy ? ?aeiiaiaeaoe?? ii
i?iaaaeaiith i?ia?aie ianiaiai ne?ei?iao ia aeyaeaiiy OEO ai?iaaaeaeai?
aei i?aeoe/ii? ?iaioe E?einueeiai ?anioae?eainueeiai iaaeeei-aaiaoe/iiai
oeaio?o (E?IAOe). Noai?aiee aaie ?c 126 c?ace?a e?ia? /eai?a 34 n?iae c
aenieei ?eceeii OEO, NIA ? IAeAe. Caaaeyee i?iaaaeaiith
iieaeoey?ii-aaiaoe/iiai ianoaaeaiiy a oeeo n?i’yo aecia/a?oueny
aaoa?iceaioia iin?enoai iacaaieo caoai?thaaiue oa oni?oii i?iaiaeeoueny
i?aiaoaeueia ae?aaiinoeea.

Iniaenoee aianie caeiaoaa/a Aeine?aeaeaiiy i?iaiaeeeeny i?ioyaii
1993-1998 ?ie?a. Aiae?c e?oa?aoo?e c aeaieo i?iaeai, a?o?aieo
aeieoiaio?a aeeoy/iai iaa?ieia?/iiai noaoe?iia?o ? E?IAOe, aeaieo
i?nueeeo ? ?aeiieo ?a?no??a, noaoenoe/ia ia?iaea iaea?aeaieo
?acoeueoao?a aeeiiai? aaoi?ii iniaenoi. Naiino?eii i?iaiaeeany aanue
eiiieaen ee?i?ei-aaiaaeia?/iiai ianoaaeaiiy 50 oai?eo ia OEO ? 42 oai?eo
ia ia?aiai-i’ycia? caoai?thaaiiy, a?aeae?aany eiioeiaaio oai?eo aeey
a?io?i?/iiai, aeaeo?ii?ia?ao?/iiai, iieaeoey?ii-aaiaoe/iiai
aeine?aeaeaiiy, i?aiaoaeueii? ae?aaiinoeee a ?iaeeiao aenieiai ?eceeo
OEO, NIA ? IAeAe. O e?eoaaii? an?o ianoaaeaieo oai?eo aaoi? a?aa
aaciina?aaeith o/anoue.

Ai?iaaoe?y ?acoeueoao?a aeena?oaoe?? ?acoeueoaoe aeine?aeaeaiiy aoee
iia?aeiieai? ia II c’?cae? iaaee/ieo aaiaoee?a Oe?a?ie (Euea?a, 1995),
ia II c’?cae? iaa?ieia?a ? ineo?ao??a Oe?a?ie (Oa?e?a, 1997), ia
iaoeiai-i?aeoe/i?e eiioa?aioe?? “No/aniee noai iaaee/ii? aaiaoeee a
Oe?a?i?” (Ee?a, 1999), E?einuee?e ?anioae?eainuee?e iaoeiai-i?aeoe/i?e
eiioa?aioe?? “I?iaaaeaiea ianniaeo ne?eie?othueo i?ia?aii aeey auyaeaiey
a?iaeaeaiiuo e ianeaaenoaaiiuo caaieaaaiee” (N?ioa?iiieue, 1995),
can?aeaiiyo E?einueeiai ?anioae?eainueeiai oiaa?enoaa aeeoy/eo
iaa?ieia?a (N?ioa?iiieue, 1997 — 1998). Ca oaiith aeena?oaoe??
iioae?eiaaii 4 noaoae oa 3 oac?a, ia?ae?e ?o i?eaaaeaii.

No?oeoo?a oa ianya aeena?oaoe?? Aeena?oaoe?y iaienaia ia 160 noi??ieao ?
i?noeoue anooi, iaeyae e?oa?aoo?e, iniiai? iaoiaee ?iaioe, ?acoeueoaoe
aeanieo aeine?aeaeaiue, ?o aiae?c ? ocaaaeueiaiiy, aeniiaee, aeiaeaoie,
nienie e?oa?aoo?e, aei yeiai aianaii 239 aeaea?ae. ?iaioa ?ethno?iaaia
31 iaethieii, 23 oaaeeoeyie, 9 aeieneaie c ?noi??e oai?ia.

CI?NO ?IAIOE

Iaoa??aee oa iaoiaee aeine?aeaeaiiy I?e i?iaaaeaii? i?ia?aie ianiaiai
ne?ei?iao ia OEO iaoa??aeii oanooaaiiy aoea eai?ey?ia e?ia
iiaiia?iaeaeaiiai, c?a?aia ? aenooaia o aeaeyae? ieyi ia
o?iiia?ao?/iiio iaia??, ui ia?aneeaany iiooith aei E?IAOe c on?o
iieiaiaeo aoaeeie?a E?eio. Aeey aecia/aiiy ??aiy OA o ne?iaaooe? e?ia?
aeei?enoiaoaaeeny o?e iaoiaee/ieo i?aeoiaee: c o?aaiy 1989 ?ieo —
?ia?a?oi?iee i?e?ia?ieia?/iee oano ca Aao?? [Guthrie R., 1961] c
aa?eo?eaoe??th aeyaeaii? a?ia?oai?eaeai?iai?? iaoiaeii oiieioa?i?
o?iiaoia?ao?? ia ieanoeiao “Silufol” [Smith I., 1981], oa c 1994 ?ieo
— oethi?eiao?e/ia aecia/aiiy OA [Kirkman H.N. ? ?io., 1982] c
aeei?enoaiiyi i?eeaaeo Oethi?ineai-2 o??ie Labsystems (O?ieyiae?y).

Ca 1989 — 1998 ?iee anueiai i?ine?eiiaaii 199115 iiaiia?iaeaeaieo (91,5%
an?o ae?oae, ui ia?iaeeeeny ), aeyaeaii 27 oai?eo ia OEO. Ca oeae aea
/an oaeiae ianoaaeaii 7 127 ae?oae c i?aeic?ith ia OEO. Ae?aaiic
ooi/iaiee ua a 6 ae?oae.

Aeai? i?i oai?eo niaaeeiaeie ia?aiai-i’yciaeie caoai?thaaiiyie io?eiai?
i?e ianoaaeaii? iai?aaeaieo aei E?IAOe, a oaeiae i?e aea/aii? a?o?aieo
iaoa??ae?a aeeoy/iai iaa?ieia?/iiai noaoe?iia?o ca 1987 — 1998 ?iee.
Aeai? aini?oaeueiiai ?a?no?o aeiiiaiai? ? ia?aa??ai? i?e iieooaaii? an?o
i?nueeeo oa ?aeiiieo aeeoy/eo iaa?ieia?a ? iaae?ao??a E?eio. Anueiai
aeyaeaii 86 oai?eo 1983 — 1998 ?ie?a ia?iaeaeaiiy c ooi/iaieie
niaaeeiaeie ia?aiai-i’yciaeie caoai?thaaiiyie.

?acoeueoaoe ee?i?/ieo niinoa?aaeaiue caiineeenue aei iaaeeei-aaiaoe/ii?
ea?oe, aea a?aeia?aaeaeeny aeai? ee?i?ei-aaiaaeia?/iiai aiaiiaco ca
caaaeueiii?eeiyoith iaoiaeeeith [No?aainii A., Aeaa?aenii A., 1972],
ia’?eoeaiiai ianoaaeaiiy niiaoe/iiai ? ineoiiaa?ieia?/iiai noaoon?a c
eiiieaenii eaai?aoi?ieo a?io?i?/ieo iaoiae?a, aeaeo?io?c?ieia?/ii?
ooieoe?iiaeueii? ae?aaiinoeee, iieaeoey?ii-aaiaoe/ia oa?aeoa?enoeea.

I?e aea/aii? niaaeeiai? ia?aiai-i’yciai? iaoieia?? i?ae?aoiaoaaeany
?iciianthaeaeai?noue ? /anoioa an?o caoai?thaaiue oe??? a?oie oa ie?aieo
ee?i?/ieo oi?i na?aae aeeoy/iai ianaeaiiy A?E, /anoioe iaoieia?/ieo
aai?a ? aaoa?iceaioiiai iin?enoaa a e?einuee?e iiioeyoe??. I?e
i?ae?aooieo aoee canoiniaai? ye iaeiiiiiaioi? aeine?aeaeaiiy, ui
aeathoue ea?oeio o?aaeaiino? aeeoy/iai ianaeaiiy ia 1 n?/iy 1999 ?ieo,
oae ? aeiaaino?ieia? ca ia??iae c 1983 ii 1998 ?iee.

Iieaeoey?ii-aaiaoe/i? aeine?aeaeaiiy i?iaaaeai? a?oiith
iieaeoey?ii-aaiaoe/ii? ae?aaiinoeee niaaeeiaeo oai?ia ?inoeoooo
iieaeoey?ii? a?ieia?? ? aaiaoeee IAI Oe?a?ie i?ae ea??aieoeoaii noa?oiai
iaoeiaiai ni?a?ia?oieea eaiaeeaeaoa a?ieia?/ieo iaoe E?aoeoeue E.A.
Anueiai aoei c?iaeaii 126 aeine?aeaeaiue a 34 n?i’yo aenieiai ?eceeo
OEO, NIA ? IAeAe, ye? i?iaeeaathoue a E?eio. I?aiaoaeueia ae?aaiinoeea
i?iaaaeaia a 5 n?i’yo c aenieei ?eceeii OEO ? a iaei?e n?i’? c aenieei
?eceeii IAeAe.

Io?eiai? ?acoeueoaoe ia?iaeyeeny iaoiaeaie iaoaiaoe/ii? noaoenoeee
(AEeaioianueeee E.A., 1991; Aieueoaa E.I., Nie?iia I.A., 1983).

?acoeueoaoe aeine?aeaeaiiy ? aiae?c io?eiaieo aeaieo

?iciianthaeaeai?noue OEO na?aae aeeoy/iai ianaeaiiy Aaoiiiii?e
?anioae?ee E?ei. I?e aea/aii? ?iciianthaeaeaiino? OEO aeei?enoiaoaaeeny
aeai? i?iaaaeaiiy i?ia?aie ianiaiai ne?ei?iao na?aae iiaiia?iaeaeaieo
A?E ca cai?iiiiiaaiith iaie noaiith. Oey noaia i?ia?aie aeyaeaiiy ?
e?eoaaiiy OEO ioiieth? an? aoaie ?iaioe c n?i’?th: c iiiaioo aeyaeaiiy
oai?i? aeeoeie a iieiaiaiio aoaeeieo aei iaaeeei-aaiaoe/iiai
eiinoeueooaaiiy n?i’?. Eia?/iei caaa?oaiiyi oaei? ?iaioe ?
?aeaioeo?eaoe?y iooaioiiai aaia oa i?aiaoaeueia ae?aaiinoeea i?e
iiaeaeueoiio ae?oiia?iaeaeaii? a ?iaeei?. Iieaeoey?ii-aaiaoe/iith
eaieith ia aoaiao iaaeeei-aaiaoe/iiai eiinoeueooaaiiy, a oaeiae
iaia’yceiaei iae?eii ca i??caeuaie ianoaaeaieo iiaiia?iaeaeaieo iaoa
noaia i?aai?caoe?? i?ia?aie ianiaiai ne?ei?iao a?ae??ciy?oueny a?ae
noaie Oeoea?iaia A.E., 1987. Iieacieeii aoaeoeaiino? i?iaaaeaiiy
ne?ei?iao ca oaeith noaiith neoao? oie oaeo, ui c 1993 ?ieo i?ciueiai
aeyaeaiiy oai?eo ia OEO a Aaoiiiii?e ?anioae?oe? E?ei ia aoei.

?iciianthaeaeai?noue OEO a E?eio — 1 ia 7375 iiaiia?iaeaeaieo, /anoioa
— (13,562,61)10 c aeia??/ei ?ioa?aaeii 8,8710 19,7810 i?e ?=0,05,
/anoioa iaoieia?/iiai aaia OEO a iiioeyoe??, noaiiaeoue 0,011640,0011,
/anoioa aaoa?iceaioiiai iin?enoaa — 0,023 aai 1:43. Oaea iioe?ai?noue
OEO aeaui aeua ca na?aaeith ii Oe?a?i?, aea a?aei?iiino? noaoenoe/ii
iaa??ia?aei? (?0,05).

A?aoiaoth/e aenieo /anoioo OEO a Oo?a//ei? [Ozguc M., Seyrantepe V.,
Tokatli A. ? ?io., 1997] ? aoi?/io aeecuee?noue e?einueeeo oaoa? aei
oo?aoeueei? iaoe??, ne?ae aoei a /aeaoe aeniei? iioe?aiino? OEO na?aae
e?einueeeo oaoa?. Iaeiae, ine?eueee /enei ianoaaeaieo ii ne?ei?iao
e?einueeeo oaoa? na?aae 15049, ye? ia?iaeeeeny c 1989 ?ieo, aecia/eoe ia
? iiaeeeaei, ?iciianthaeaeai?noue OEO na?aae iiaiia?iaeaeaieo
e?einueeioaoa?nueei? iaoe?iiaeueiino? iiaeia aaaaeaoe ia ieae/ith ca
1:5016 (aeyaeaii o?i? oai?eo). Oey oeeo?a aeua, i?ae na?aaeiy
iiioeyoe?eia /anoioa a E?eio, iaeiae aiia io?eiaia ia iaaaeee?e
e?eueeino? ianoaaeaieo (oa?i?i ?ai?acaioaoeaiino? — 30000 ianoaaeaieo) ?
iiaea ci?ieoeny i?e ca?eueoaii? aea??ee iiaiia?iaeaeaieo.

?iciianthaeaeai?noue niaaeeiaeo ia?aiai-i’yciaeo caoai?thaaiue o ae?oae
a A?E. Noia?ia /anoioa ia?aiai-i’yciaeo caoai?thaaiue na?aae aeeoy/iai
ianaeaiiy E?eio — (21,02,26)10 c aeia??/ei ?ioa?aaeii 16,810 25,510 i?e
?=0,05, ?iciianthaeaeai?noue — 1:4770 (oaae.1). Oai??, iiia?e? aei
oeueiai /ano, oaeiae ye ? ae?oe, noa?o? ca 15 ?ie?a, aei aea??ee ia
aeeth/aeeny.

Oaaeeoey 1

*anoioa ia?aiai-i’yciaeo caoai?thaaiue na?aae aeeoy/iai ianaeaiiy A?E

Iicieia?y*enei aeiaaee?a*anoioa caoai?thaaiiy
?mp(10)?iciianthaeaeai?noue

na?aae aeeoy/iai

ianaeaiiyI?iiao?? anueiai 5413,21,81: 7 596IAeAe276,61,31: 15 193?io?
i?iiao??276,61,31: 15 193Ai?io?io?? anueiai327,81,41: 12 819Iioinaini?i?
iaa?iiao??102,40,81: 41 020NIA225,41,11: 18 646Anueiai8621,02,261: 4
770

*anoioa i?iiao?e aeinoia??ii aeua ca /anoioo ai?io?io?e (?0,05). I?e
i?ae?aooieo ie?aieo iicieia?/ieo oi?i i’yciaeo aeeno?io?e c’yniaaii, ui
aiie cono??/athoueny c /anoioaie: oi?ia Eaiaeoc?-Aeaaea??ia — 0,4910,
aeeno?io?/ia i?ioii?y — 0,4910, aeenoaeueia i?iiao?y — 0,2510,
iai?ia?anoth/? oi?ie i?iiao?e — 5,3610 (iae.1, A).

Ca noaoaaith i?eiaeaaei?noth na?aae i?iiao?e aac o?aooaaiiy IAeAe
ia?aaaaeaee oeii/eee (?0,05), na?aae oai?eo ia iioinaini?i? iaa?iiao??
/ano?oa cono??eeny ae?a/aoea (?0,05). Oai?? ia NIA ca noaooth
?iciiae?eai? iaeaea ii??aio.

O a?oi? i?iiao?e o 50% cono??/aeany IAeAe. Oea caoai?thaaiiy ye
iaea?eueo /anoo oi?io ia?aiai-i’yciaeo oai?ia a?aei?/a? ? aaaaoi
aaoi??a [Emery A., 1991, Mcmillan J.C., Harper P.S., 1994]. Ine?eueee
aeaia caoai?thaaiiy ia? c/aieaiee c? noaooth oa?aeoa? oniaaeeoaaiiy,
aeioe?eueii /anoioo iioe?aiino? IAeAe ?ic?aoiaoaaoe ? ia /enei aeeaeo
iiaiia?iaeaeaieo oeii/ee?a [Iao??i A.I., 1989]. *anoioa IAeAe —
(17,483,36)10 c aeia??/ei ?ioa?aaeii i?e ?=0,05 11,4310 25,4910;
?iciianthaeaeai?noue — 1: 5 719 oeii/ee?a, ye? ia?iaeeeeny aeeaeie, ui
ni?aiaaea? c /anoioaie a ?ioeo ?a?iiaenueeeo iiioeyoe?yo [Danieli
G.A., 1984].

O a?oi? aeaia?aaoe?eieo ai?io?io?e ia?aaaaeaea NIA (iae.1,A). Ine?eueee
aeey aeaiiai caoai?thaaiiy oa?aeoa?ia aeniea nia?oi?noue o aeeoy/iio
a?oe?, iaie aecia/aia eiai /anoioa c o?aooaaiiyi iiia?eeo oai?eo na?aae
oeo, ui ia?iaeeeeny aeeaeie c 1983 ?ieo: (7,261,42)10 c aeia??/ei
?ioa?aaeii i?e ?=0,05 4,7110 10,6710 , ?iciianthaeaeai?noue — 1:13765.
*anoioa iaoieia?/iiai aaia a iiioeyoe?? — 0,008520,00077, /anoioa
aaoa?iceaioiiai iin?enoaa — 0,0169 aai 1:59, ui a?aeiia?aea?
e?oa?aoo?iei aeaiei [Aaeueoeuaa TH.?., Oai?i I.I., 1998]. *anoioa NIA ?
o E?eio na?aae oeo, ui ia?iaeeeeny aeeaeie — (1,120,56)10, caaaeueia
iioe?ai?noue NIA II ? III na?aae ae?oae A?E — (6,141,3)10.

Ee?i?/ia oa iieaeoey?ii-aaiaoe/ia oa?aeoa?enoeea
oai?eo ia OEO. I?e aiae?c? ee?i?/ii? neiioiiaoeee oai?? ia OEO aoee
?icae?eai? ia aea? a?oie: ? a?oia — aeyaeai? i?ia?aiith ianiaiai
ne?ei?iao iiaiia?iaeaeaieo (27 ae?oae) ? II a?oia — oai?? c 1985 ?ieo
ia?iaeaeaiiy, ye? ia i?ieoee ne?ei?ia (23 aeeoeie). Ca noaooth oai?? ia
OEO ?iciiae?eai? iaeaea ii??aio, ye ? a ?iaioao aaaaoueio aaoi??a
[Guttler F., Hansen G., 1977, Ia?ei/aaa A.N., Aaa?eeia A.?., 1988]. A
iaio a?oiao oai?eo ia?aaaaeaea eeane/ia oi?ia OEO. Ee?i?/i? neiioiie o
oai?eo, ui niinoa??aaeeny iaie, aoee ae?aaeai? oaeei /eiii (oaae.
2.).

?acoeueoaoe i?iaaaeaiiai aeine?aeaeaiiy iieacothoue, ui aeinoia??i?
a?aei?iiino? a a?oiao niinoa??aaeeny ye ca oaiioei?/ieie iciaeaie (eie??
aieinny, ?aeaeoaeieo iaieiiie i/ae), oae ? ca iieacieeaie
ineoeiioi?iiai, iiaiiai oa ?ioaeaeooaeueiiai ?icaeoeo. Iio??aii
cacia/eoe, ui iaea?eueo /ooeeaei aei ii?ooaiiy ae??oe aeyaeany iiaiee
?icaeoie: aac cao?eiee iiae ?icaeaaeeny o?eueee 35,3% oai?eo. Na?aaei?e
??aaiue OA e?ia? o oai?eo ?c cao?eieith iiae aoa aeuei, i?ae o oai?eo
aac ii?ooaiue iiaiiai ?icaeoeo (?0,05).

Aei aoei?/ii? OEO aoei a?aeianaii 4 aeiaaeee caoai?thaaiiy. O o?ueio c
ieo ??aaiue OA ia ia?aaeuoaaa 15 ia% ia ?icoe?ai?e ae??o? oa i?e
i?iaaaeaii? iaaaioaaeoaaeueii? i?iae, ia?aa?a caoai?thaaiiy aoa eaaeei.
O aeiaaeeo oai?i? A. e?einueeioaoa?nueei? iaoe?iiaeueiino? c
e?iaiinii??aeiaiiai oethao caoai?thaaiiy iaei ceiye?niee ia?aa?a
i?ioeano (iino?ei? eeii?ei-oii?/i? noaeiie, ?acenoaioi? aei e?eoaaiiy,
cieaeaiiy ?ioaeaeoo aei nooiaiy ?ae?io??) ia oii? aaeaeaaoii?
ae??oioa?ai??, ui aeicaieeei ia?aaeaa/eoe iayai?noue o ia? OEO??? a
iane?aeie aeao?oeeoo aeea?ae?iioa?eaeeineioaoace.

Aiae?c ?acoeueoao?a ae??oioa?ai?? oai?eo ia?oiai ?ieo aeeooy iieacaa, ui
6 c 12 ae?oae, ui io?eioaaee «Aa?eioai», iaee oneeaaeiaiiy o aeaeyae?
aiai??, aoi?eiieo ?ioiiaeao?oeeoieo noai?a. O aeaio aeiaaeeao oe?
oneeaaeiaiiy ni?eyee ?icaeoeo aaaeeeo ai?eii-naioe/ieo i?ioean?a, ui
i?eaaei aei nia?o? oai?eo. O ae?oae, ui e?eoaaeeny ?ioeie i?aia?aoaie,
oneeaaeiaiue ia niinoa??aaeiny.

Oaaeeoey 2

Ee?i?/ia neiioiiaoeea o oai?eo ia eeane/io oi?io OEO

Ee?i?/i? iciaee? a?oiaII a?oia , ?Oai??%Oai??%Eie??
aieinny2410022100Na?oea aieinny1875,01881,8?oaea
aieinny14,2418,26,74Oaiia aieinny520,8—?0,05I?aiaioaoe?y ?aeaeoaeieo
iaieiiie2410022100Na?oe?2083,4221003,97Oaii?416,60-?0,05Oe??yi?
aeyae2410022100Ceouaiiy, a?ia?ai?y u?e1145,81568,2Aeeoy/a
aecaia28,429,12,47ia aoei aeyao1145,8522,7?0,05Niaoeeo?/iee caiao
na/?2410022100Aoa1250,01672,32,47ia aoei1250,0627,7?0,05Noaei?iaeiee
neiae?ii2410022100Noaeiie—731,8Cae?eaaiiy, o?aii?
?oe1041,7836,49,65Iaia? aeyaeaiue1458,3731,8?0,01Ineoiiioi?iee
?icaeoie21*10022100A?aeiia?aea?
a?eo1990,129,129,45Oiia?eueiaiee29,92090,9?0,001Iiaiee
?icaeoie17**10022100A?aeiia?aea? a?eo635,3—9,25Cao?eiea ?icaeoeo
iiae1164,722100?0,01?ioaeaeooaeueiee ?icaeoie17**10022100A?aeiia?aea?
a?eo1270,6—22,63Cieaeaiiy ?ioaeaeoo529,422100?0,001

I?ei?oea: * — ioe?ithaaeiny a ae?oae noa?oeo ca iaeei ??e;

** — ioe?ithaaeiny a ae?oae noa?oeo ca
o?e ?iee.

Iieaeoey?ii-aaiaoe/ia ianoaaeaiiy aoei i?iaaaeaia o 20 oai?eo ? 44
/eai?a 18 ?iaeei c aenieei ?eceeii OEO. Iaaei?ia iooaoe?y R408W a 12
aecii? aaia oai?eaeai?ia?ae?ieneeace (OAA) aoea aeyaeaia ia 24 ?c 40
o?iiinii (60%) o 90% oai?eo: o 6 i?iaaiae?a a aiiiceaioiiio noai? (30%),
o 12 (60%) — a aaoa?iceaioiiio, a eiiiaoiae? c ia?aeaioeo?eiaaiith
iooaoe??th. O?eueee o aeaio oai?eo ia OEO (10%) R408W ia aoea ciaeaeaia,
oiaoi aoee iayai? ?io? iooaoe?? aaia OAA.

I?e aiae?c? iayaiino? R408W o oai?eo ia ??ci? ee?i?/i? oi?ie OEO
c’yniaaii, ui a 94,4% eeane/ia oi?ia OEO aecia/aeany “iaaei?iith”
iooaoe??th: 33,3% — a aiiiceaioiiio noai?, 61,1% — a aaoa?iceaioiiio. A
?aeeiiai ianoaaeaiiai oai?iai ia aoei?/io oi?io OEO iooaoe?y ia
?aeaioeo?eiaaia.

C 36 ianoaaeaieo aaouee?a 19 (52,8%) aoee iin?yie iooaoe?? R408W aaia
OAA. An? ianoaaeai? ?iaeeie aeyaeeeny ?ioi?iaoeaieie ca aiae?cii
c/aieaiiy iooaoe?e aaia OAA c aeaeueieie aa??aioaie VNTR oa STR
eieon?a aaia OAA.

Aeey caiia?aaiiy iiaoi?iei aeiaaeeai OEO a 5 ?iaeeiao i?aeaeuaiiai
?eceeo canoiniaoaaeany i?aiaoaeueia ae?aaiinoeea iaoiaeii AeIE-aiae?co
iooaoe?? R408W a?iioaoo ai?nei oi??iia. O 4-o n?i’yo iooaoe?y R408W o
ieiae?a ia aeyaeaia. Aaa?oi?noue ?aeiiaiaeoaaeiny caa??aaoe,
ia?iaeeeiny 4 caei?iaeo aeeoeie.

O ?iaeei? A. ie?ae aoa aaoa?iceaioiei iin??i iooaoe?? R408W. *a?ac oa,
ui a??ia?aei?noue OEO o ieiaea aei??aithaaea 50%, aaa?oi?noue
?aeiiaiaeoaaee ia?a?aaoe. Iaeiae n?i’y a?aeiiaeeany a?ae ia?a?eaaiiy:
ia?iaeeeany aeeoeia, oai?a ia OEO.

Ee?i?/ia oa iieaeoey?ii-aaiaoe/ia oa?aeoa?enoeea oai?eo
ia NIA. C 26 oai?eo ia NIA, aeyaeaieo o ?aa?ii?, i?ae iniaenoei
niinoa?aaeaiiyi caeiaoaa/a ciaoiaeeeiny 16 oai?eo c 15 n?iae.
Ae?aaiinoeea oi?i NIA i?iaiaeeeany ca caaaeueiii?eeiyoeie e?eoa??yie
(Mansat T.l., Daves K.E., 1992): ? oeiii no?aaeaeaee 4 oai?eo, II oeiii
— 7 oai?eo, III oeiii — 5 oai?eo; ni?aa?aeiioaiiy oei?a ?: II: III
-1:1,75:1,25. Ia?aaaaa ae?oaiai oeio NIA niinoa?aaa?oueny a a?eueoino?
iiioeyoe?e (Dubowitz V., 1991; Mansat T.l., Daves K.E., 1992).

Neeaaei?noue ae?aaiinoeee NIA ? oeio iieyaaea a iaiiaeeeaino?
i?iaaaeaiiy aeaeo?ii?ia?ao?/iiai aeine?aeaeaiiy iiaa?oiaaeie
aeaeo?iaeaie a ae?oae ?aiiueiai a?eo. O?i? oai?eo caaeioee o a?oe? aei
2-o ?ie?a a?ae aoi?eiieo iiaaiii?e, iaeei iaoe??io aeeaee a 4 ?iee, ? a
eiai ee?i?/i?e ea?oei? ii/aee aeyaeyoeny iciaee NIA II-III oei?a:
ao?io?y i’yc?a ? ?icaeoie eiio?aeoo? noaeia?a. Oea niinoa?aaeaiiy
aeicaiey? iiaiaeeoueny c aeoieith Zerres K.,1995 i?i oa, ui ca
aeiiiiiaith ?nioth/eo ae?aaiinoe/ieo e?eoa???a iaiiaeeeai aeaoe i?iaiic
i?i o?eaae?noue caoai?thaaiiy a eiie?aoiiai oai?iai. O a?oi? oai?eo ia
NIA III niinoa??aaany aeiaaeie c inaaaeia?ia?o?io?yie aii?eeiaeo i’yc?a
o oai?iai oeii/eea, ui aeeeeeaei iio?aao aeeoa?aioe?eii? ae?aaiinoeee c
IAeAe. Iiae?ai? aeiaaeee iienai? e ?ioeie aaoi?aie
(Hausmanova-Petrusewicz I., Zaremba J., Borkowska J., 1985; Dubowitz V.,
1991) ?, iaaii, neoaothoue a?aeia?aaeaiiyi ee?i?/iiai iie?ii?o?cio oe???
iaoieia??. Aeyaeaiiy aiiiceaioii? aeaeaoe?? 7-8-ai aecii?a aaia SMN o
oeueiai oai?iai na?ae/eoue ia ei?enoue oeueiai i?eiouaiiy.

Iieaeoey?ii-aaiaoe/ia ianoaaeaiiy i?iaiaeeee 3-i oai?ei ia NIA I oeio,
4-i oai?ei ia NIA II oeio oa 3-i oai?ei ia NIA III oeio ? 19 aaoueeai
10 ?iaeei aenieiai ?eceeo NIA. O 100% ianoaaeaieo oai?eo aoei aeyaeaii
aiiiceaioio aeaeaoe?th aaia SMN, aeaeaoe?? 5-ai aeciia aaia NAIP ia
ciaeaeaii. Aeyaeaiiy o oai?eo ia an? oeie NIA iaeiaeiai? aeaeaoe?? aaia
SMN iieaco?, ui oai?iae Aa?aei?aa-Aiooiaia oa Eoaaeueaa?aa-Aaeaiaea?a ?
iaei??th iicieia??th, ye a?aecia/aii e o aaaaoueio ?iaioao (Gilliam I.
C., Brzustowicz L. M., Castilla L. H. oa ?i.,1990; Brzustowicz L. M.,
Lehner T., Castilla L. H. oa ?i.,1990; Melki J., Abdelhak S., Sheth P.
oa ?i.,1990).

O 10 c 11 i?iaaiae?a (90,9%) aeaeaoe?y caoiiethaaea 7-e oa 8-e aeciie
aaia SMN. A iaeiiai iaoe??ioa NIA III oeio aeaeaoe?y caoiiethaaea
o?eueee 7-e aecii iacaaiiai aaia. O ee?i?/i?e ea?oei? caoai?thaaiiy a
oeueiai oai?iai niaoeeo?/ieo iniaeeainoae ia niinoa??aaeiny. An? aaoueee
aoee aaoa?iceaioieie iin?yie a?aeiia?aeieo aeaeaoe?e aaia SMN. Io?eiai?
aeai? /anoioe cono??/aeueiino? aeaeaoe?e aaia SMN o oai?eo ia NIA a
e?einuee?e iiioeyoe?? na?ae/aoue i?i aenieo ?ioi?iaoeai?noue aiae?co
aeaii? iooaoe?? i?e i?iaaaeaii? i?aiaoaeueii? ae?aaiinoeee a n?i’yo
aenieiai ?eceeo NIA.

Ee?i?/ia oa iieaeoey?ii-aaiaoe/ia oa?aeoa?enoeea oai?eo ia IAeAe. I?ae
/an aeine?aeaeaiue ie niinoa??aaee 17 oai?eo ia IAeAe c 15 n?iae.
Na?aaei?e a?e oai?eo ia /an iai?oanoaoe?? IAeAe — 4,82 0,42 ?ieo.

?acoeueoaoe aeine?aeaeaiiy iieacaee, ui i?e ca?eueoaii? o?eaaeino?
i?ioeano o oai?eo ia?inoaee i’ycia? ao?io??, iiia?aeiaee ei?aeic,
aeaoi?iaoe?y a?oaeii? ee?oee, eiio?aeoo?e noaeia?a, inaaaeia?ia?o?io??
i’yc?a iiiaioaee. O?aaeaiiy na?oey niinoa??aaeeny a 35,3% an?o oai?eo.
Ia aeaeo?iea?ae?ia?aiao a?aei?/aeeny iciaee iaoaaie?/ieo ii?ooaiue a
i?iea?ae?, a iaeiiai oai?iai cao?eniaai? ??aee? e?aioeoii/eia?
aeno?an?noiee, a aeaio oai?eo — a?ia?o?io?y e?aiai oeoii/ea. ?ioaeaeo
aoa cieaeaiee o 29,4% an?o iaoe??io?a c IAeAe. Oa?aeoa?ii, ui ii?ooaiiy
?ioaeaeoo a?aei?/aeeny o oai?eo c ?aiiueiai aeeoeinoaa, a ia c’yaeyeeny
c a?eii oai?iai. Oaeo caeiiii??i?noue a?aecia/aee e ?io? aaoi?e (Emery
A.E.H., 1987).

O an?o 17 oai?eo ioe?ithaaeeny ii?ooaiiy iioi?eee ca oeaeith ioe?iee
?ooiaeo iiaeeeainoae oai?eo ia oaciai-iiia?aeia? oi?ie i?ia?anoth/i?
i’yciai? aeeno?io?? ca Aaaeaeyiii E.I., 1988. Na?aae oai?eo c
o?eaae?noth oai?iae aei 5 ?ie?a noia aae?a aeua ca 100 (? noaae?y
i?ioeano) niinoa??aaeany a 20% iaoe??io?a, a ?ioeo 80% ae?oae i?aenoiie
noaiiaea 82 — 67 aae?a, ui a?aeiia?aea? II noaae?? oai?iae. O a?oi?
oai?eo, ui no?aaeaeathoue ia IAeAe a?eueoa ca 5 ?ie?a, II noaae?y
caoai?thaaiiy a?aei?/aeany o?eueee a 28,6% ae?oae, ?io? iaee noio
aae?a a?ae 10 aei 46 (III noaae?y i?ioeano). Iiai?noth caeaoi?noue
oiaeeoe ao?aoeee 57,1% oai?eo ae?oai? a?oie a 13,750,45 ?ie?a, ia 7-8-i
?ioe? a?ae ii/aoeo caoai?thaaiiy.

A?io?i?/ia ianoaaeaiiy i?iaaaeaia 14 c 17 niinoa?aaeaieo oai?eo.
Iaea?eueo oa?aeoa?iei — o 100% ianoaaeaieo oai?eo — aoei i?aeaeuaiiy
eiioeaio?aoe?? e?aaoeioinoie?iace (EOE), ye a?aecia/a?oueny ? a
aaaaoueio ?iaioao (Aaoo A.I., ?eue?ia I.A., 1982; Engel A.G., Banker
B.Q., 1986). Na?aaei?e ??aaiue EOE o ne?iaaooe? e?ia? aoa 92802128
iae/e. ?io? oa?iaioe aoee i?aeaeuai? ia caaaeaee, aea a a?eueo i?ae
iieiaei? aeiaaee?a: ??aaiue aeai?io?ainoa?ace (AEO) — o 64,3% oai?eo,
ania?oaoo?ainoa?ace (ANO) — o 71,4% oai?eo.

Iniaeeaee ?ioa?an aeeeeea? ianoaaeaiiy 15 iaoa??a oai?eo. Ee?i?/ieo
iciae caoai?thaaiiy, a?aeoeeaiue o iaa?ieia?/iiio noaoon? ia
niinoa??aaeiny o aeiaeii? c ieo. ??aaiue EOE aoa i?aeaeuaiee o 100%
ianoaaeaieo ae?iie ? neeaaeaa o na?aaeiueiio 632128 iae/e; i?aeaeuaiiy
AEO ? ANO o ne?iaaooe? e?ia? ia a?aei?/aeiny. A?aoiaoth/e aeai?
ee?i?ei-aaiaaeia?/iiai aiaiiaco, a?io?i?/iiai ? iieaeoey?ii-aaiaoe/iiai
aiae?c?a, iiaeia c?iaeoe aeniiaie a oiio, ui an? ianoaaeai? iaoa?? aoee
iin?yie iooaioiiai aaia aeeno?io?ia, iooaoe?th de novo o i?iaaiaea ia
iiaeia i?eionoeoe a aeiaeiiio aeiaaeeo.

Iieaeoey?ii-aaiaoe/ia ianoaaeaiiy i?iaiaeeee o 7 oai?eo ia IAeAe c 6-oe
n?iae c aenieei ?eceeii caoai?thaaiiy. Aeaeaoe?? a ??cieo ae?eyieao aaia
aeeno?io?ia ciaeaeai? a 4-o i?iaaiae?a (57,1%): a o?ueio aeiaaeeao
i?ioyaei? — 50-52 oa 3-6 aecii?a aaia o aeaio oai?eo nean?a, oa a
iaeiiai oai?iai — 50-ai aeciia aaia aeeno?io?ia. An? ianoaaeai? n?i’?
aeyaeeeny ?ioi?iaoeaieie aeey i?iaaaeaiiy i?aiaoaeueii? ae?aaiinoeee ?
aecia/aiiy aaoa?iceaioiiai iin?enoaa a ?iaee/ie ae?ii/i? noao? ca
aioo??oiuein?iaeiei aiae?cii c/aieaiiy iooaioiiai aaia aeeno?io?ia c
iie?ii?oieie ia?ea?aie STR45, STR49, PERT87.

Aeiiieiaiaa ae?aaiinoeea IAeAe (iae.2.) aoea i?iaaaeaia a iaei?e n?i’?
I. n aeaiia oai?eie ia IAeAe neanaie (aeine?aeaeaiiy i?iaaaeaia a
iieaeoey?ii-aaiaoe/iiio iaoeiaiio oeaio?? Aeaaeai?? iaaee/ieo iaoe
?in??, i. Iineaa, caa?aeoaa/ eaai?aoi???th — ae.a.i. ?aa?aoia I.A.).
Ianoaaeaia aii?ioe/ia ??aeeia a 12 oeaei?a aaa?oiino?, o ieiaeo
aeaeaoe?? 3-6 aeciia aaia aeeno?io?ia ia aeyaeaii. Ia?iaeeany caei?iaee
oeii/ee.

AENIIAEE

1. *anoioa OEO a E?eio — (13,562,61)10, /anoioa iaoieia?/iiai aaia a
e?einuee?e iiioeyoe?? noaiiaeoue 0,011640,0011, /anoioa
aaoa?iceaioiiai iin?enoaa — 0,023 aai 1:43; ?iciianthaeaeai?noue OEO —
1: 7 375 iiaiia?iaeaeaieo.

2. “Iaaei?ia” iooaoe?y R408W niinoa??aaeany a 60% iooaioieo o?iiinii o
90% ianoaaeaieo oai?eo ia OEO. O oai?eo ia eeane/io oi?io OEO o 94,4%
aeiaaee?a aecia/aeanue iooaoe?y R408W. O oai?iai ia aoeiiao oi?io
“iaaei?ia” iooaoe?y R408W ia aeyaeaia.

3. Noia?ia /anoioa ia?aiai-i’yciaeo caoai?thaaiue na?aae ae?oae E?eio —
(21,02,26)10 aeeoy/iai ianaeaiiy, ?iciianthaeaeai?noue — 1:4770. *anoioa
IAeAe — (6,61,3)10 aeeoy/iai ianaeaiiy aai (17,483,36)10 aeeaeo
iiaiia?iaeaeaieo oeii/ee?a, ?iciianthaeaeai?noue — 1:5719 aeeaeo
iiaiia?iaeaeaieo oeii/ee?a. *anoioa ?ioeo i’yciaeo aeeno?io?e: oi?ia
Eaiaeoc?-Aeaaea??ia — 0,4910 aeeoy/iai ianaeaiiy, aeeno?io?/ia i?ioii?y
— 0,4910, aeenoaeueia i?iiao?y — 0,2510, iai?ia?anoth/? oi?ie i?iiao?e
— 5,3610.

4. *anoioa an?o oei?a NIA o E?eio — (5,41,1)10 aeeoy/iai ianaeaiiy. C
o?aooaaiiyi iiia?eeo oai?eo na?aae oeo, ui ia?iaeeeeny aeeaeie c 1983
?ieo, /anoioa an?o oei?a NIA — (7,261,42)10, ?iciianthaeaeai?noue —
1:13765, /anoioa iaoieia?/iiai aaia a iiioeyoe?? — 0,008520,00077,
/anoioa aaoa?iceaioiiai iin?enoaa — 0,0169 aai 1:59. *anoioa NIA I o
E?eio na?aae oeo, ui ia?iaeeeeny aeeaeie — (1,120,56)10, caaaeueia
cono??/aeuei?noue NIA II e III — (6,141,3)10. Ni?aa?aeiioaiiy oei?a
NIA — I: II: III — 1:1,75:1,25.

5. Noai?aii aaie ?c 126 c?ace?a e?ia? /eai?a 34 n?iae c aenieei ?eceeii
OEO, NIA ? IAeAe, ui aeicaieeei i?iaanoe iieaeoey?ii-aaiaoe/io
ae?aaiinoeeo ? i?aeaeueoe aoaeoeai?noue iaaeeei-aaiaoe/iiai
eiinoeueooaaiiy a oeeo n?i’yo. An? ianoaaeai? ?iaeeie ?ioi?iaoeai? aeey
i?iaaaeaiiy i?aiaoaeueii? ae?aaiinoeee; o oai?eo ia OEO “iaaei?ia”
iooaoe?y R408W aeyaeaia o 90%, o oai?eo ia ??ci? oeie NIA aeaeaoe?? aaia
SMN — a 100%, o oai?eo ia IAeAe aeaeaoe?? aaia aeeno?io?ia — a 57,1%.

IA?AE?E I?AOeUe, IIOAE?EIAAIEO CA OAIITH AeENA?OAOe??

1.Ioneaiei N. E., Eeaoeoe E. A., Au/eiaa A. I., Aoaianueaaa I. A., Niiei
I. E., Aa?eeye E. ?. Aiaeec iooaoeee e iieeii?oecia 30-ioeeaioeaeiuo
oaiaeaiiuo iiaoi?ia aaia oaieeaeaieiaeae?ieneeacu a naiueyo n aenieie
noaiaiueth ?enea oaieeeaoiio?ee ec ?acee/iuo iaeanoae Oe?aeiu //
AEo?iae AII Oe?aeiu. — 1996. — O.2, ?3. — N.488 — 494.

2.Yeoeyi A.TH., Eeaoeoe E.A., Au/eiaa A.I., Aoaianueaaa I.A., Aa?eeye E.
?. Iieaeoey?ii-aaiaoe/aneee aiaeec nieiaeueiie iuoa/iie ao?ioee (NIA) a
naiueyo aenieiai ?enea ec ?aciuo ?aaeiiia Oe?aeiu // Oeeoieia?y ?
aaiaoeea. — 1997. — O.31, ?6. — N.75 — 81.

3.Aoaianueaaa I.A., Aeieaiiieiaa E.A., Eeaoeoe E.A., Aa?eeye E.?.
?acoeueoaou ianniaiai ne?eieiaa iiai?iaeaeaiiuo ia oaieeeaoiio?eth a
?anioaeeea E?ui //A na: I?iaeaie aeieia?/ii? ? iaaee/ii? aaiaoeee ?
ee?i?/ii? ?ioiieia??, E.-Eoaainuee. — 1997. — Aei.3. — N.3 — 7.

4.Aoaianueaaa I.A., Au/eiaa A.I., Eeaoeoe E.A., Aa?eeye E.?. Eeeie/aneay
e iieaeoey?ii-aaiaoe/aneay oa?aeoa?enoeea aieueiuo oaieeeaoiio?eae a
?anioaeeea E?ui // Oeeoieia?y ? aaiaoeea. — 1998. — O.32, ?1. — N.8 —
14.

5.Ion??iei N.I., E?aoeoeue E.A., Ae/eiaa A.I., Aoaianue?aa I.I. Aiae?c
iooaoe?? R408W o oai?eo ia oai?eeaoiio??th a Oe?a?i? // Iaoa??aee II
c’?caeo iaaee/ieo aaiaoee?a Oe?a?ie. — Euea?a. — 1995. — N.103.

6.Aeo?yi I.TH., A?eoei A.A., Ieoaeeaoeue E.I., Aoaianue?aa I.I.,
E?aoeoeue E.A., Aa?eeye I.?. Canoinoaaiiy iieaeoey?ii-aaiaoe/ieo
iaoiae?a aeey ae?aaiinoeee iaea?eueo iioe?aieo ia?aiai-i’yciaeo
caoai?thaaiue // Oe?a?inueeee a?niee ineoiiaa?ieia??. — Oa?e?a. — 1997.
— O.5. — Aei.2 (13). — N.162 — 164.

7.Aoaianueaaa I.A. ?ani?ino?aiaiiinoue ianeaaenoaaiiuo ia?aii-iuoa/iuo
caaieaaaiee n?aaee aeaoneiai ianaeaiey Aaoiiiiiie ?anioaeeee E?ui //
Iaoa??aee iaoeiai-i?aeoe/ii? eiioa?aioe?? “No/aniee noai iaaee/ii?
aaiaoeee a Oe?a?i?”. — Ee?a, 1999. — N.12.

Aoaianue?aa I.I. ?iciianthaeaeai?noue oa ee?i?ei-aaiaoe/ia
oa?aeoa?enoeea aeayeeo iiiiaaieo oai?ia o ae?oae Aaoiiiiii? ?anioae?ee
E?ei. — ?oeiien.

Aeena?oaoe?y ia caeiaoooy a/aiiai nooiaiy eaiaeeaeaoa iaaee/ieo iaoe ca
niaoe?aeueiinoth 03.00.15 — aaiaoeea. — Oe?a?inueeee iaoeiaee
a?a??i?/iee oeaio? IIC Oe?a?ie, Ee?a, 1999.

Aeena?oaoe?th i?enay/aii ieoaiiyi ?iciianthaeaeai?no? oai?eeaoiio??? oa
niaaeeiaeo ia?aiai-i’yciaeo caoai?thaaiue o ae?oae E?eio, ?oi?e
ee?i?/i?e ? iieaeoey?ii-aaiaoe/i?e oa?aeoa?enoeoe?. O ?iaio? aia?oa
aecia/ai? iiioeyoe?ei? /anoioe oai?eeaoiio???, ni?iaeueii? i’yciai?
ao?io??, i’yciai? aeeno?io?? Aethoaiia, aeaii aiae?c ??cieo ee?i?/ieo
oi?i oeeo caoai?thaaiue, ?oi?o iniaeeainoae o e?einuee?e iiioeyoe??.
Aia?oa i?iaaaeaii iieaeoey?ii-aaiaoe/ia ianoaaeaiiy oai?eo — iaoeaioe?a
E?eio ia aeacai? caoai?thaaiiy, io?eiaii aeai?, ui oa?aeoa?ecothoue
??ci? ee?i?/i? oi?ie o oai?eo c ??cieie oeiaie iooaoe?e aai?a.
Aeei?enoaiiy ?acoeueoao?a iieaeoey?ii-aaiaoe/iiai aiae?co aeicaieeei
noai?eoe iiaee i?aeo?ae aeey iaaeeei-aaiaoe/iiai eiinoeueooaaiiy a
?iaeeiao aenieiai ?eceeo OEO, NIA ? IAeAe, ni?yiiaaiee ia aaciina?aaei?
aecia/aiiy aaoa?iceaioiiai iin?enoaa oa i?aiaoaeueii? ae?aaiinoeee.

Eeth/ia? neiaa: oai?eeaoiio??y, ni?iaeueia i’yciaa ao?io?y, i’yciaa
aeeno?io?y Aethoaiia, ?iciianthaeaeai?noue, iieaeoey?ii-aaiaoe/iee
aiae?c.

Afanaseva N.A. Prevalence and clinical-genetic features of some
monogenic diseases among children in The Crimean Autonomous
Republic. — Manuscript.

The thesis is presented for the degree of the candidate of medical
sciences by speciality 03.00.15 — genetics. — Ukrainian scientific
centre of Hygiene of Ukraine, Kyiv, 1999.

The dissertation is devoted to prevalence, clinical and molecular
genetic researches of fenylketonuria and inherited neuromuscular
diseases among children in Crimea. The Crimean population frequencies of
fenylketonuria, spinal muscular atrophy, Duchenne muscular dystrophy
are established at first. Different clinical forms of these diseases and
their peculiarities in the Crimea are described. The molecular genetic
analyses of Crimean patiens are obtained at first. Characterization of
different clinical forms of patiens with different types of mutations of
determinating genes is defined. Obtained results make possible to
contract the new approach to medical genetic consultation in families
with high risk of fenylketonuria, spinal muscular atrophy, Duchenne
muscular dystrophy and to determine the prenatal diagnostics.

Key words: fenylketonuria, spinal muscular atrophy, Duchenne muscular
dystrophy, prevalence, molecular genetic analysis.

Aoaianueaaa I.A. ?ani?ino?aiaiiinoue e eeeieei-aaiaoe/aneay
oa?aeoa?enoeea iaeioi?uo iiiiaaiiuo caaieaaaiee o aeaoae Aaoiiiiiie
?anioaeeee E?ui. — ?oeiienue.

Aeenna?oaoeey ia nieneaiea o/aiie noaiaie eaiaeeaeaoa iaaeeoeeineeo iaoe
ii niaoeeaeueiinoe 03.00.15 — aaiaoeea. — Oe?aeineee iao/iue
aeaeaie/aniee oeaio? IIC Oe?aeiu, Eeaa, 1999.

Aeenna?oaoeey iinayuaia ?ani?ino?aiaiiinoe e eeeieei-aaiaoe/aneie
oa?aeoa?enoeea oaieeeaoiio?ee (OEO) e ianeaaenoaaiiuo ia?aii-iuoa/iuo
caaieaaaiee iiiiaaiiie i?e?iaeu n?aaee aeaoneiai ianaeaiey Aaoiiiiiie
?anioaeeee E?ui. A ?aaioa aia?aua ?anniao?eaathony aii?inu eeeie/aneiai
iieeii?oecia yoeo caaieaaaiee a e?uineie iiioeyoeee, annioeeaoeee ?aciuo
eeeie/aneeo oi?i n aeaoa?ieie?othueie eo aaiaie.

Aeey eco/aiey ?ani?ino?aiaiiinoe OEO iaie eniieueciaaiu aeaiiua
i?iaaaeaiey ne?eie?othuae i?ia?aiiu ia auyaeaiea OEO n?aaee
iiai?iaeaeaiiuo Aaoiiiiiie ?anioaeeee E?ui ca 1989-1998 aiaeu. Iieacaii,
/oi /anoioa OEO a E?uio — (13,562,61)10 iiai?iaeaeaiiuo, /anoioa
iaoieiae/aneiai aaia a iiioeyoeee — 0,011640,0011, /anoioa
aaoa?iceaioiiai iineoaeuenoaa — 0,023 eee 1:43; ?ani?ino?aiaiiinoue OEO
— 1: 7 375 iiai?iaeaeaiiuo.

A aeenna?oaoeee ?anniao?eaathony aii?inu ii ea/aieth aieueiuo OEO.
Iieacaii, /oi iaeaieaa /oanoaeoaeueiui e ia?ooaieyi aeeaoioa?aiee
yaeyaony ?a/aaia ?acaeoea: aac caaea?aeee ?a/e ?acaeaaeenue oieueei
35,3% aieueiuo. N?aaeiee o?iaaiue oaieeaeaieia e?iae o aieueiuo n
caaea?aeeie ?a/e aue aeinoiaa?ii auoa, /ai o aieueiuo aac ia?ooaiee
?a/aaiai ?acaeoey (?0,05). Ianoaeaeathony ineiaeiaiey aeeaoioa?aiee
i?e ea/aiee aieueiuo ia?aiai aiaea aeecie i?aia?aoii “Aa?eioai”.

Aia?aua i?iaaaeaii iieaeoey?ii-aaiaoe/aneia ianeaaeiaaiea 20 aieueiuo
OEO ec 18 naiae, i?iaeeaathueo a E?uio. Iaaei?iay iooaoeey R408W a 12
yeciia aaia oaieeaeaieiaeae?ieneeacu (OAA) iaaethaeaeanue a 60%
iooaioiuo o?iiinii o 90% ianeaaeiaaiiuo aieueiuo, i?e/ai o 6 i?iaaiaeia
— a aiiiceaioiii ninoiyiee (30%), o 12 (60%) — a aaoa?iceaioiii, a
eiiiaoiaea n iaeaeaioeoeoee?iaaiiie iooaoeeae. Oieueei o aeaoo aieueiuo
OEO (10%) R408W ia auea iaia?oaeaia, o.a. auee ae?oaea iooaoeee aaia
OAA. O aieueiuo eeanne/aneie oi?iie OEO a 94,4% neo/aaa ii?aaeaeyeanue
iaaei?iay iooaoeey R408W. O aieueiiai aoeie/iie oi?iie R408W ia
iaia?oaeaia.

I?e eco/aiee ?ani?ino?aiaiiinoe ianeaaenoaaiiie ia?aii-iuoa/iie
iaoieiaee auee i?eiaiaiu eae iaeiiiiiaioiua enneaaeiaaiey, aeathuea
ea?oeio ii?aaeaiiinoe aeaoneiai ianaeaiey ia 1 yiaa?y 1999 aiaea, oae e
aeieain?i/iua ca ia?eiae n 1983 ii 1998 aiaeu. Noiia?iay /anoioa
ia?aii-iuoa/iuo caaieaaaiee n?aaee aeaoae E?uia — (21,02,26)10
aeaoneiai ianaeaiey, ?ani?ino?aiaiiinoue — 1:4770.

A a?oiia ieiiaoee a 50% neo/aaa ano?aoeeanue iuoa/iay aeeno?ioey
Aethoaiia (IAeAe). *anoioa IAeAe — (6,61,3)10 aeaoneiai ianaeaiey eee
(17,483,36)10 aeeauo iiai?iaeaeaiiuo iaeue/eeia, ?ani?ino?aiaiiinoue —
1:5719 aeeauo iiai?iaeaeaiiuo iaeue/eeia. ?acoeueoaou enneaaeiaaiey
iieacaee, /oi ii?aaeaiey na?aeoea iaaethaeaeenue o 35,3% anao aieueiuo.
Eioaeeaeo aue nieaeai o 29,4% anao iaoeeaioia n IAeAe, i?e/ai caaea?aeea
oinoaaiiiai ?acaeoey ioia/aeanue n ?iaeaeaiey, a ia ?acaeaaeanue n
aic?anoii aieueiiai. I?e ianeaaeiaaiee 15 iaoa?ae aieueiuo IAeAe aue
naeaeai auaiae i oii, /oi a eaaeaeie ec ianeaaeiaaiiuo naiae iaoue
yaeeanue iineoaeai iooaioiiai aaia aeeno?ioeia, iooaoeeth de novo o
i?iaaiaea iaeuecy auei i?aaeiieiaeeoue ie a iaeiii neo/aa.

I?i/ea iuoa/iua aeeno?ioee iaaethaeaeenue n /anoioie: oi?ia
Eaiaeoce-Aeaaea?eia — 0,4910 aeaoneiai ianaeaiey, aeeno?ioe/aneay
ieioiiey — 0,4910, aeenoaeueiay ieiiaoey — 0,2510, iai?ia?anne?othuea
oi?iu ieiiaoee — 5,3610 aeaoneiai ianaeaiey.

*anoioa anao oeiia nieiaeueiie iuoa/iie ao?ioee (NIA) a E?uio —
(5,41,1)10 aeaoneiai ianaeaiey. N o/aoii oia?oeo aieueiuo n?aaee
?iaeeaoeony aeeauie n 1983 aiaea /anoioa anao oeiia NIA — (7,261,42)10,
?ani?ino?aiaiiinoue — 1: 13 765, /anoioa iaoieiae/aneiai aaia a
iiioeyoeee — 0,008520,00077, /anoioa aaoa?iceaioiiai iineoaeuenoaa —
0,0169 eee 1:59. *anoioa NIA I a E?uio n?aaee ?iaeeaoeony aeeauie —
(1,120,56)10, iauay ano?a/aaiinoue NIA II e III — (6,141,3)10.
Niioiioaiea I: II: III oeiia NIA — 1:1,75:1,25.

Aia?aua i?iaaaeaii iieaeoey?ii-aaiaoe/aneia ianeaaeiaaiea aieueiuo IAeAe
e NIA, i?iaeeaathueo a E?uio. O aieueiuo ?aciuie oeiaie NIA aeaeaoeee
aaia SMN iaia?oaeaiu a 100% neo/aaa, o aieueiuo IAeAe aeaeaoeee aaia
aeeno?ioeia — a 57,1%. Ii?aaeaeaii, /oi ana ianeaaeiaaiiua naiuee
eioi?iaoeaiu aeey i?iaaaeaiey i?aiaoaeueiie aeeaaiinoeee.

Eniieueciaaiea ?acoeueoaoia iieaeoey?ii-aaiaoe/aniiai aiaeeca nicaeaao
iiaue iiaeoiae e iaaeeei-aaiaoe/aniiio eiinoeueoe?iaaieth a naiueyo
aenieiai ?enea OEO, NIA e IAeAe, aeaao aiciiaeiinoue iaiin?aaenoaaiiiai
ii?aaeaeaiey aaoa?iceaioiiai iineoaeuenoaa e i?aiaoaeueiie
aeeaaiinoeee yoeo caaieaaaiee. ?acoeueoaou enneaaeiaaiey e
?aeiiaiaeaoeee ii i?iaaaeaieth ne?eie?othuae i?ia?aiiu ia aeyaeaiea OEO
aiaae?aiu a i?aeoe/aneoth aeayoaeueiinoue E?uineiai ?anioaeeeaineiai
iaaeeei-aaiaoe/aniiai oeaio?a.

Eeth/aaua neiaa: oaieeeaoiio?ey, nieiaeueiay iuoa/iay ao?ioey, iuoa/iay
aeeno?ioey Aethoaiia, ?ani?ino?aiaiea, iieaeoey?ii-aaiaoe/aneee aiaeec.

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