{"id":884,"date":"2011-12-31T19:08:51","date_gmt":"2011-12-31T17:08:51","guid":{"rendered":"http:\/\/slanedeti.sk\/cms\/cf-epidemiologia-a-pacientske-registre-2\/201112"},"modified":"2014-01-05T12:07:35","modified_gmt":"2014-01-05T11:07:35","slug":"cf-epidemiologia-a-pacientske-registre-2","status":"publish","type":"post","link":"https:\/\/slanedeti.sk\/cms\/cf-epidemiologia-a-pacientske-registre-2\/884","title":{"rendered":"CF epidemiol\u00f3gia a pacientsk\u00e9 registre"},"content":{"rendered":"

\u010cl\u00e1nok je stru\u010dn\u00fdm preh\u013eadom toho, na \u010do sa v s\u00fa\u010dasnosti zameriava epidemiol\u00f3gia pri cystickej fibr\u00f3ze. Epidemiol\u00f3gia je veda, ktor\u00e1 vyu\u017e\u00edva predov\u0161etk\u00fdm \u0161tatistick\u00e9 met\u00f3dy pre anal\u00fdzu ve\u013ek\u00e9ho rozsahu demografick\u00fdch a klinick\u00fdch \u00fadajov. V\u00fdsledkom s\u00fa rozsiahle datab\u00e1zy a ich \u0161tatistick\u00e9 spracovania, z ktor\u00fdch je mo\u017en\u00e9 pozorova\u0165 r\u00f4zne trendy na \u00farovni celej pacientskej popul\u00e1cie.<\/p>\n

Zo spracovania t\u00fdchto \u00fadajov sa \u010dasto daj\u00fa ur\u010di\u0165 niektor\u00e9 determinanty ochorenia a navrhn\u00fa\u0165 efekt\u00edvnej\u0161ia lie\u010dba.\u00a0 D\u00f4le\u017eitou formou v ktorej s\u00fa tieto \u00fadaje uchov\u00e1van\u00e9 s\u00fa\u00a0 pacientsk\u00e9 registre, na ktor\u00e9 sa tie\u017e zameriava tento \u010dl\u00e1nok. Cie\u013eom je uk\u00e1za\u0165 ak\u00e9 inform\u00e1cie s\u00fa v registroch, v\u00fdznam ich zberu, objasni\u0165 ako registre funguj\u00fa a s\u00fa uveden\u00e9 zdroje prostredn\u00edctvom ktor\u00fdch je mo\u017en\u00e9 \u00fadaje z registrov z\u00edska\u0165. Konkr\u00e9tne \u0161tatistick\u00e9 \u00fadaje, ktor\u00e9 s\u00fa tu uv\u00e1dzan\u00e9 s\u00fa pr\u00edkladmi a vzh\u013eadom na cie\u013e a rozsah \u010dl\u00e1nku nie je z\u00e1merom ich na tomto mieste podrobnej\u0161ie analyzova\u0165.<\/p>\n

Z\u00e1klady a v\u00fdchodisk\u00e1 CF epidemiol\u00f3gie<\/strong><\/h4>\n

<\/strong>Cystick\u00e1 fibr\u00f3za (CF) je vz\u00e1cne, genetick\u00e9, autozom\u00e1lne reces\u00edvne ochorenie. Trp\u00ed n\u00edm pribli\u017ene 50 000 \u013eud\u00ed v Eur\u00f3pe, 30 000 v USA, 3000 v Kanade [1], v mnoh\u00fdch \u010fal\u0161\u00edch krajin\u00e1ch nie s\u00fa dostato\u010dn\u00e9 \u00fadaje o po\u010dte pacientov.
\nOchorenie je sp\u00f4soben\u00e9 mut\u00e1ciou CFTR (cystic fibrosis conductance regul\u00e1tor) g\u00e9nu, s n\u00e1sledn\u00fdm zn\u00ed\u017een\u00edm funkcie CFTR prote\u00ednu. Pod\u013ea typu mut\u00e1cie sa rozozn\u00e1va ve\u013ek\u00e9 mno\u017estvo g\u00e9nov\u00fdch aliel, ktor\u00fdch kombin\u00e1cia vedie k CF sympt\u00f3mom.<\/p>\n

Zast\u00fapenie mut\u00e1ci\u00ed pri neonat\u00e1lnom skr\u00edningu v Braz\u00edlii:
\n<\/em>
\n

\n\n\n\n\n\n\n\n\n\n\n\n\n
\u00a0Mut\u00e1cia<\/strong><\/td>\nV\u00fdskyt v %<\/strong><\/td>\n<\/tr>\n
\u00a0delta F508<\/td>\n\u00a048,2<\/td>\n<\/tr>\n
\u00a0R1162X<\/td>\n\u00a05,41<\/td>\n<\/tr>\n
\u00a0G542X<\/td>\n\u00a04,5<\/td>\n<\/tr>\n
\u00a03120+1G>A<\/td>\n\u00a04,05<\/td>\n<\/tr>\n
\u00a0G85E<\/td>\n\u00a03,6<\/td>\n<\/tr>\n
\u00a0711+1G>T<\/td>\n\u00a00,9<\/td>\n<\/tr>\n
\u00a0N1303K<\/td>\n\u00a00,9<\/td>\n<\/tr>\n
\u00a0W1282X<\/td>\n\u00a00,45<\/td>\n<\/tr>\n
\u00a0Neidentifikovn\u00e1<\/td>\n\u00a031,99<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<\/div>\n

V\u00fdskyt CF je naj\u010dastej\u0161\u00ed u bielej (kaukazskej) rasy, kde je pribli\u017ene ka\u017ed\u00fd 30-ty \u010dlovek nosite\u013eom CF g\u00e9nu. CF sa ale m\u00f4\u017ee vyskytova\u0165, aj ke\u010f s ni\u017e\u0161ou pravdepodobnos\u0165ou u v\u0161etk\u00fdch r\u00e1s. Pod\u013ea \u00fadajov z USA je nosite\u013estvo g\u00e9nu v hisp\u00e1nskej popul\u00e1cii 1:48, medzi afro-ameri\u010danmi 1:65 a ameri\u010danmi \u00e1zijsk\u00e9ho p\u00f4vodu 1:90. Preto zatia\u013e \u010do pomer novorodencov s CF bol u bielej rasy pribli\u017ene 1:3200, u afro-ameri\u010danov to bolo 1:15 000 a u \u013eud\u00ed \u00e1zijsk\u00e9ho p\u00f4vodu 1:32 000 [3].
\nTaktie\u017e v\u00fdskyt jednotliv\u00fdch mut\u00e1cii (aliel) je \u010dasto odli\u0161n\u00fd medzi r\u00f4znymi popul\u00e1ciami. Napr\u00edklad celosvetovo je najroz\u0161\u00edrenej\u0161ia mut\u00e1cia delta F508. Po\u010det nosite\u013eov delta F508 je 1:200 vo \u0160v\u00e9dsku, 1:143 v Litve, 1:38 v D\u00e1nsku. Naproti tomu v\u00fdskyt delta F508 vo F\u00ednskej popul\u00e1cii je minoritn\u00fd [4].<\/p>\n

<\/strong>N\u00e1rodn\u00e9 CF registre<\/strong><\/h4>\n

Pacientsk\u00e9 registre s\u00fa datab\u00e1zy klinick\u00fdch inform\u00e1ci\u00ed o pacientoch. Tieto registre boli vytvoren\u00e9 pre mno\u017estvo ochoren\u00ed a v mnoh\u00fdch smeroch v\u00fdznamne u\u013eah\u010duj\u00fa medic\u00ednsky v\u00fdskum, osobitn\u00fd pr\u00ednos maj\u00fa v epidemiol\u00f3gii. Je to datab\u00e1za sekund\u00e1rnych \u00fadajov, ke\u010f\u017ee do nej prispievaj\u00fa najm\u00e4 lek\u00e1ri, ktor\u00ed odosielaj\u00fa v \u0161tandardizovanej forme \u00fadaje o pacientoch. V registroch je zaru\u010den\u00e1 anonymita, nie je nijako mo\u017en\u00e9 identifikova\u0165 jednotlivca, tak aby boli \u00fadaje uchov\u00e1van\u00e9 v s\u00falade s pr\u00e1vnymi predpismi na ochranu osobn\u00fdch \u00fadajov a etick\u00fdmi princ\u00edpmi medic\u00ednskeho v\u00fdskumu. CF registre vytvorili a udr\u017eiavaj\u00fa takmer v\u0161etky vyspel\u00e9 krajiny.<\/p>\n

Pravdepodobne prv\u00fd register CF pacientov bol vytvoren\u00fd v USA pred viac ako 40 rokmi. Je spravovan\u00fd Cystic Fibrosis Foundation, ktor\u00e1 ka\u017edoro\u010dne vyd\u00e1va verejne dostupn\u00fa spr\u00e1vu (Annual Data Report), sumarizuj\u00facu aktu\u00e1lne \u00fadaje [5]. Americk\u00fd register je z\u00e1rove\u0148 rozsahom najv\u00e4\u010d\u0161\u00ed n\u00e1rodn\u00fd register na svete, ke\u010f\u017ee zah\u0155\u0148a 26 000 pacientov. Z\u00e1kladn\u00e9 \u00fadaje ktor\u00e9 sumarizuje register s\u00fa v\u00fd\u0161ka, v\u00e1ha, pohlavie, genotyp, stav antibiotickej rezistencie, v\u00fdsledky funk\u010dn\u00fdch p\u013e\u00facnych testov, d\u013a\u017eka hospitaliz\u00e1cii, dom\u00e1ce pod\u00e1vanie antibiot\u00edk, pou\u017e\u00edvanie pankreatick\u00fdch enz\u00fdmov, komplik\u00e1cie spojen\u00e9 s CF. V spr\u00e1ve n\u00e1jdeme aj \u010fal\u0161ie \u00fadaje, ako je napr\u00edklad trend po\u010dtu transplant\u00e1cii p\u013e\u00fac.<\/p>\n

Po\u010det transplant\u00e1ci\u00ed p\u013e\u00fac u CF pacientov v USA<\/em>
\n\"201112311744_obr1_transplantacie\"<\/p>\n

Medzi naj\u010dastej\u0161\u00edmi sprievodn\u00fdmi ochoreniami pri CF s\u00fa pod\u013ea US registra osteopatie, diabetes a klinick\u00e9 depresie, pri\u010dom ich v\u00fdskyt sa s narastaj\u00facim vekom zvy\u0161uje od nieko\u013ek\u00fdch percent a\u017e k pribli\u017ene 30 % pri ka\u017edom sprievodnom ochoren\u00ed vo vekovej skupine 35 \u2013 45 rokov. Medzi \u010fal\u0161ie pomerne \u010dast\u00e9 sprievodn\u00e9 ochorenia zara\u010fuje ezofag\u00e1lny reflux a astmu. Spr\u00e1va tie\u017e uv\u00e1dza aj niektor\u00e9 neklinick\u00e9 \u00fadaje, ako druh zdravotn\u00e9ho poistenia, medzi dospel\u00fdmi CF pacientmi \u0161tatistiky dosiahnut\u00e9ho vzdelania, pracovn\u00e9ho zaradenia a rodinn\u00e9ho stavu.<\/p>\n

N\u00e1sledne za\u010dali CF registre zaklada\u0165 \u010fal\u0161ie krajiny. V Nemecku sa za\u010dal projekt zberu demografick\u00fdch a klinick\u00fdch \u00fadajov CF pacientov v roku 1995 ako Cystic Fibrosis Quality Assesment project [6]. Zdrojom \u00fadajov bolo 93 CF centier v Nemecku, pri\u010dom do roku 2005 zahrnul 6835 pacientov.<\/p>\n

V roku 2001 bol zalo\u017een\u00fd \u00cdrsky CF register (The Cystic Fibrosis Registy of Ireland, http:\/\/www.cfri.ie<\/a>). \u00dadaje z \u00cdrska s\u00fa preh\u013eadne spracovan\u00e9 v ro\u010dn\u00fdch spr\u00e1vach, ktor\u00e9 s\u00fa informa\u010dne ve\u013emi hodnotn\u00e9. \u201cUser friendly\u201c str\u00e1nka \u00cdrskeho registra z\u00e1rove\u0148 obsahuje u\u017eito\u010dn\u00e9 inform\u00e1cie o fungovan\u00ed, v\u00fdzname aj finan\u010dnom hospod\u00e1ren\u00ed registra. V roku 2009 zah\u0155\u0148al \u00cdrsky register 1105 pacientov. Z niektor\u00fdch \u00fadajov z v\u00fdro\u010dnej spr\u00e1vy za rok 2009 s\u00fa ni\u017e\u0161ie uveden\u00e9 tabu\u013eky s priemern\u00fdmi hodnotami funk\u010dn\u00fdch p\u013e\u00facnych funkci\u00ed\u00a0a v\u00fdsledky mikrobiologick\u00fdch kultiv\u00e1ci\u00ed.<\/p>\n

Priemern\u00e9 hodnoty funk\u010dn\u00fdch p\u013e\u00facnych funkcii v rozdelen\u00ed pod\u013ea vekov\u00fdch skup\u00edn, \u00fadaje z \u00cdrskeho CF registra za r. 2009:<\/em>
\n

\n\n\n\n\n\n\n\n\n\n\n\n
\u00a0Vekov\u00e1 skupina<\/strong><\/td>\n\u00a0Po\u010det vy\u0161etren\u00ed<\/strong><\/td>\nPriemer FEV1 %\u00a0<\/strong><\/td>\nPriemer FVC %<\/strong><\/td>\n<\/tr>\n
\u00a05-9<\/td>\n\u00a0407<\/td>\n\u00a088,4<\/td>\n\u00a092,0<\/td>\n<\/tr>\n
\u00a010-14<\/td>\n\u00a0480<\/td>\n\u00a075,0<\/td>\n\u00a083,5<\/td>\n<\/tr>\n
\u00a015-19<\/td>\n\u00a0460<\/td>\n\u00a067,5<\/td>\n\u00a079,0<\/td>\n<\/tr>\n
\u00a020-24<\/td>\n\u00a0496<\/td>\n\u00a056,7<\/td>\n\u00a072,6<\/td>\n<\/tr>\n
\u00a025-29<\/td>\n\u00a0284<\/td>\n\u00a056,1<\/td>\n\u00a074,2<\/td>\n<\/tr>\n
\u00a030-34<\/td>\n\u00a0172<\/td>\n\u00a054,7<\/td>\n\u00a075,2<\/td>\n<\/tr>\n
\u00a035-39<\/td>\n\u00a085<\/td>\n\u00a054,7<\/td>\n\u00a074,2<\/td>\n<\/tr>\n
\u00a0>40<\/td>\n\u00a062<\/td>\n\u00a057,3<\/td>\n\u00a080,5<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<\/div>\nV\u00fdsledky kultiv\u00e1cii pri CF pacientoch pod\u013ea \u00cdrskeho CF registra v r. 2009:<\/em>
\n
\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n
\u00a0Agens<\/strong><\/td>\nN<\/strong><\/td>\n<\/tr>\n
\u00a0Staphylococcus aureus<\/td>\n\u00a01063<\/td>\n<\/tr>\n
\u00a0Candida sp<\/td>\n\u00a01058<\/td>\n<\/tr>\n
\u00a0Pseudomonas aeruginosa<\/td>\n\u00a02020<\/td>\n<\/tr>\n
\u00a0Norm\u00e1lna flu\u00f3ra<\/td>\n\u00a0656<\/td>\n<\/tr>\n
\u00a0Aspergillus fumagatus<\/td>\n\u00a0415<\/td>\n<\/tr>\n
\u00a0MRSA<\/td>\n\u00a0329<\/td>\n<\/tr>\n
\u00a0Gram pozit\u00edvne koky<\/td>\n\u00a0310<\/td>\n<\/tr>\n
\u00a0Haemophillus influenza<\/td>\n\u00a0248<\/td>\n<\/tr>\n
\u00a0Gram negat\u00edvne bacili<\/td>\n\u00a0210<\/td>\n<\/tr>\n
\u00a0Stenotrophomonas maltophilia<\/td>\n\u00a0172<\/td>\n<\/tr>\n
\u00a0In\u00e9<\/td>\n\u00a0622<\/td>\n<\/tr>\n
\u00a0Celkom<\/strong><\/td>\n\u00a07103<\/strong><\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<\/div>\nVo Ve\u013ekej Brit\u00e1nii CF pacientsk\u00fd register spravuje neziskov\u00e1 organiz\u00e1cia Cystic Fibrosis Trust a jeho v\u00fdro\u010dn\u00e9 spr\u00e1vy s\u00fa taktie\u017e verejne dostupn\u00e9. Britsk\u00fd register je najv\u00e4\u010d\u0161\u00edm v Eur\u00f3pe, ke\u010f\u017ee zah\u0155\u0148a 9029 pacientov [8]. \u00dadaje z Britsk\u00e9ho registra potvrdzuj\u00fa, \u017ee medzi pacientmi je viac chlapcov\/ mu\u017eov ako diev\u010dat\/\u017eien, pri\u010dom tento rozdiel sa postupom veku zvy\u0161uje.<\/p>\n

Rozdelenie pacientov vo Ve\u013ekej Brit\u00e1nii pod\u013ea veku a pohlavia:
\n<\/em>\"201112311744_obr2_pacienti\"
\nPrv\u00fd eur\u00f3psky register CF pacientov bol vytvoren\u00fd v r\u00e1mci projektu EuroCareCF (podrobnej\u0161ie info ni\u017e\u0161ie). EuroCareCF bol \u010dasovo ohrani\u010den\u00fd projekt, ukon\u010den\u00fd v r. 2009. Na eur\u00f3pskej \u00farovni je v s\u00fa\u010dasnosti udr\u017eiavanie registra aktivitou European Cystic Fibrosis Society [9].<\/p>\n

EuroCareCF<\/strong><\/h4>\n

<\/strong>EuroCareCF (http:\/\/www.eurocarecf.eu<\/a>) bol trojro\u010dn\u00fdm projektom financovan\u00fdm Eur\u00f3pskou komisiou, ktor\u00e9ho cie\u013eom bola koordin\u00e1cia viacer\u00fdch sn\u00e1h s cie\u013eom optimalizova\u0165 klinick\u00fd mana\u017ement, v\u00fdvoj nov\u00fdch terapii CF a prispie\u0165 k zlep\u0161eniu kvality \u017eivota pacientov. Jednou s iniciat\u00edv bolo vytvorenie celoeur\u00f3pskeho CF registra na z\u00e1klade spolupr\u00e1ce n\u00e1rodn\u00fdch registrov. Tento register by mal tie\u017e sl\u00fa\u017ei\u0165 ako zdroj \u00fadajov pre glob\u00e1lny register, ktor\u00fd m\u00f4\u017ee by\u0165 vytvoren\u00fd na z\u00e1klade spolupr\u00e1ce EuroCareCF a americkej Cystic Fibrosis Foundation. Do EuroCareCf registra sa zapojilo 35 eur\u00f3pskych kraj\u00edn. Je pochopite\u013en\u00e9 \u017ee vzh\u013eadom na rozdielnos\u0165 n\u00e1rodn\u00fdch \u0161tandardov si vytvorenie celoeur\u00f3pskeho registra vy\u017eiadalo prekonanie mno\u017estva najm\u00e4 technick\u00fdch a metodologick\u00fdch obtia\u017e\u00ed.<\/p>\n

Projekt prebiehal od roku 2006 do 2009 a vych\u00e1dzal z \u00fadajov z n\u00e1rodn\u00fdch registrov zaznamenan\u00fdch do\u00a0 r. 2008. V\u00fdsledky spracovan\u00e9 ako epidemiologick\u00e1 \u0161t\u00fadia boli publikovan\u00e9 v \u010dasopise Journal of Cystic Fibrosis v novembri 2010 [10].<\/p>\n

Po\u010dty diagnostikovan\u00fdch pacientov v n\u00e1rodn\u00fdch registroch niektor\u00fdch eur\u00f3pskych kraj\u00edn,\u010d\u00edsla zah\u0155naj\u00fa aj zomrel\u00fdch pacientov:<\/em>
\n

\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n
\u00a0Krajina<\/strong><\/td>\nPo\u010det<\/strong><\/td>\n<\/tr>\n
\u00a0Belgicko<\/td>\n\u00a0912<\/td>\n<\/tr>\n
\u00a0\u010cesk\u00e1 republika<\/td>\n\u00a0490<\/td>\n<\/tr>\n
\u00a0D\u00e1nsko<\/td>\n\u00a0441<\/td>\n<\/tr>\n
\u00a0Franc\u00fazsko<\/td>\n\u00a04533<\/td>\n<\/tr>\n
\u00a0Holandsko<\/td>\n\u00a0888<\/td>\n<\/tr>\n
\u00a0\u00cdrsko<\/td>\n\u00a0675<\/td>\n<\/tr>\n
\u00a0Nemecko<\/td>\n\u00a04758<\/td>\n<\/tr>\n
\u00a0Portugalsko<\/td>\n\u00a0233<\/td>\n<\/tr>\n
\u00a0Rusko<\/td>\n\u00a0535<\/td>\n<\/tr>\n
\u00a0Rumunsko<\/td>\n\u00a0197<\/td>\n<\/tr>\n
\u00a0Srbsko<\/td>\n\u00a070<\/td>\n<\/tr>\n
\u00a0Taliansko<\/td>\n\u00a04138<\/td>\n<\/tr>\n
\u00a0Turecko<\/td>\n\u00a0481<\/td>\n<\/tr>\n
\u00a0Ukrajina<\/td>\n\u00a096<\/td>\n<\/tr>\n
\u00a0Ve\u013ek\u00e1 Brit\u00e1nia<\/td>\n\u00a08027<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<\/div>\nCelkovo bolo v EuroCareCF registri zahrnut\u00fdch 29 095 pacientov. I\u0161lo o v\u0161etk\u00fdch diagnostikovan\u00fdch pacientov, vr\u00e1tane t\u00fdch \u010do boli zahrnut\u00ed v registri, ale nesk\u00f4r zomreli. Po\u010det zomrel\u00fdch bol 1128 (3.9 %). Niektor\u00e9 krajiny ale poskytli inform\u00e1cie iba o \u017eiv\u00fdch pacientoch. Pri niektor\u00fdch krajin\u00e1ch s\u00fa v\u0161ak v\u00fdznamnej\u0161ie rozdiely v po\u010dte pacientov v registri a skuto\u010dnom po\u010dte pacientov v danej krajine, ke\u010f\u017ee \u00fadaje do registra neposkytli v dan\u00fdch krajin\u00e1ch v\u0161etky CF centr\u00e1. Za ka\u017ed\u00fa krajinu by v\u0161ak po\u010det zaraden\u00fdch pacientov mal by\u0165 nad 50\u00a0 %. Z dan\u00e9ho po\u010dtu bolo 16 479 det\u00ed a 12 616 dospel\u00fdch. V Eur\u00f3pe je teda zatia\u013e prevaha detsk\u00fdch pacientov (57 oproti 43 %).<\/p>\n

Rozdelenie pacientov z CF registrov do vekov\u00fdch skup\u00edn, zahrnut\u00ed s\u00fa iba \u017eiv\u00ed pacienti:
\n<\/em>\"201112311744_obr3_pacienti2\"<\/p>\n

V obdob\u00ed zberu \u00fadajov bol neonat\u00e1lny skr\u00edning (z\u00e1kladn\u00e9 vy\u0161etrenie pre CF kr\u00e1tko po naroden\u00ed) zaveden\u00fd iba vo ve\u013emi malom po\u010dte kraj\u00edn. Z anal\u00fdzy \u00fadajov vypl\u00fdva, \u017ee aj napriek tomu bola v\u00e4\u010d\u0161ina pacientov diagnostikovan\u00e1 v prvom roku \u017eivota.<\/p>\n

Rozdelenie pacientov pod\u013ea veku, kedy boli diagnostikovan\u00ed na cystick\u00fa fibr\u00f3zu:<\/em>
\n\"201112311745_obr4_vek\"<\/p>\n

Trendy vo v\u00fdvoji CF popul\u00e1cie<\/strong><\/h4>\n
Vy\u0161\u0161\u00ed po\u010det diagnostikovan\u00fdch pacientov<\/h5>\n

Vo v\u0161eobecnosti sa po\u010det CF pacientov zvy\u0161uje, pri\u010dom tento trend podporuje jednak vy\u0161\u0161\u00ed po\u010det diagnostikovan\u00fdch pacientov ako aj st\u00e1le sa zvy\u0161uj\u00faca priemern\u00e1 d\u013a\u017eka \u017eivota.<\/p>\n

\u0160ir\u0161ie mo\u017enosti CF diagnostiky viedli po\u010das posledn\u00fdch 10 rokov k tomu, \u017ee bolo mo\u017en\u00e9 diagnostikova\u0165 v\u00e4\u010d\u0161\u00ed po\u010det pacientov. Z\u00e1sadn\u00fdmi zmenami boli v\u00e4\u010d\u0161ia dostupnos\u0165 genetick\u00e9ho testovania, zavedenie neonat\u00e1lneho skr\u00edningu vo vyspel\u00fdch krajin\u00e1ch a tie\u017e zv\u00fd\u0161en\u00e9 povedomie o CF viedlo k testovaniu \u0161ir\u0161ieho okruhu os\u00f4b. Neonat\u00e1lny skr\u00edning znamen\u00e1, \u017ee by mala by\u0165 diagnostikovan\u00e1 ve\u013ek\u00e1 v\u00e4\u010d\u0161ina novorodencov, ktor\u00ed maj\u00fa CF. Na druhej strane bol tie\u017e diagnostikovan\u00fd v\u00e4\u010d\u0161\u00ed po\u010det pacientov vo vy\u0161\u0161om veku. Jedn\u00e1 sa najm\u00e4 o pacientov s menej \u010dast\u00fdmi a spravidla aj menej z\u00e1va\u017en\u00fdmi mut\u00e1ciami. Predpoklad\u00e1 sa, \u017ee v niektor\u00fdch krajin\u00e1ch m\u00f4\u017ee by\u0165 st\u00e1le vysok\u00fd po\u010det nediagnostikovan\u00fdch pacientov. To sa vz\u0165ahuje najm\u00e4 na chudobnej\u0161ie krajiny, \u010di u\u017e vo v\u00fdchodnej Eur\u00f3pe, ako aj inde vo svete.<\/p>\n

Predl\u017eovanie priemernej d\u013a\u017eky \u017eivota<\/h5>\n

Trend neust\u00e1leho predl\u017eovania priemernej d\u013a\u017eky \u017eivota je po\u010das posledn\u00fdch desa\u0165ro\u010d\u00ed evidentn\u00fd,\u00a0 v\u00fdznamn\u00fd a pozorovan\u00fd vo v\u0161etk\u00fdch krajin\u00e1ch. V s\u00fa\u010dasnosti je predpokladan\u00fd priemern\u00fd vek do\u017eitia CF pacientov v Kanade 46.7 roka [11]. Zatia\u013e \u010do trend zlep\u0161enia je zrejm\u00fd vo v\u0161etk\u00fdch krajin\u00e1ch, st\u00e1le pretrv\u00e1vaj\u00fa nemal\u00e9 rozdiely medzi jednotliv\u00fdmi krajinami. S predl\u017euj\u00facim sa priemern\u00fdm vekom do\u017eitia s\u00favisia \u010fal\u0161ie zmeny v CF popul\u00e1ci\u00e1ch, ako je zvy\u0161ovanie po\u010dtu dospel\u00fdch pacientov, n\u00e1rast incidencie sprievodn\u00fdch ochoren\u00ed (osteopor\u00f3za, diabetes). V r. 2009 st\u00e1le preva\u017eovali v Eur\u00f3pskej CF popul\u00e1cii deti, ale predpoklad\u00e1 sa, \u017ee tento pomer sa obr\u00e1ti.<\/p>\n

Vy\u0161\u0161ia \u00famrtnos\u0165 \u017eien<\/h5>\n

Celkovo bolo v s\u00fabore eur\u00f3pskeho CF registra 53 % chlapcov\/mu\u017eov. Tieto \u00fadaje s\u00fa konzistentn\u00e9 s in\u00fdmi, dlh\u0161ie zn\u00e1mymi zisteniami o prevahe chlapcov a mu\u017eov v CF popul\u00e1cii. Pomer pohlav\u00ed pri naroden\u00ed je v CF popul\u00e1cii rovnak\u00fd ako pri nie-CF (s miernou prevahou chlapcov). Zatia\u013e \u010do v celej popul\u00e1cii je vy\u0161\u0161ia \u00famrtnos\u0165 u chlapcov, pri CF popul\u00e1cii je to u diev\u010dat. Preto sa pomer v neprospech \u017eien s postupom veku zv\u00e4\u010d\u0161uje, pribli\u017ene o jeden percentu\u00e1lny bod v ka\u017edej nasleduj\u00facej vekovej skupine pri rozdelen\u00ed po 5 rokov. Pr\u00ed\u010diny tohto javu nie s\u00fa v s\u00fa\u010dasnosti dostato\u010dne vedecky presk\u00faman\u00e9, aj ke\u010f boli formulovan\u00e9 niektor\u00e9 hypot\u00e9zy, ktor\u00e9 by to mohli vysvet\u013eova\u0165. Je zn\u00e1me, \u017ee vy\u0161\u0161ia \u00famrtnos\u0165 u \u017eien nast\u00e1va v obdob\u00ed puberty, \u010do viedlo k predpokladom \u017ee pohlavn\u00e9 horm\u00f3ny m\u00f4\u017eu ma\u0165 vplyv na priebeh ochorenia. Tie\u017e sa zd\u00e1 \u017ee v niektor\u00fdch krajin\u00e1ch je priemern\u00fd vek ur\u010denia CF diagn\u00f3zy u diev\u010dat vy\u0161\u0161\u00ed, ako u chlapcov, pri\u010dom taktie\u017e d\u00f4vod nie je dostato\u010dne objasnen\u00fd. In\u00e9 \u0161t\u00fadie zase nazna\u010duj\u00fa, \u017ee \u00famrtnos\u0165 sa v s\u00fa\u010dasnosti medzi pohlaviami vyrovn\u00e1va a v bud\u00facnosti by mali by\u0165 rozdiely men\u0161ie, resp. bli\u017e\u0161ie priemeru v nie-CF popul\u00e1cii.<\/p>\n

 <\/p>\n

[1] Wiki CF, Epidemiology http:\/\/www.wikicf.com\/wiki\/back-basics\/epidemiology<\/a>, Nov 2011
\n[2] Frequency of 8 CFTR gene mutations in cystic fibrosis patients in Minas Gerais, Brazil, diagnosed by neonatal screening, Perone et al., Brailian Journal of Medical and Biological Research, 43 (2010), 134-138
\n[3] Comparison of the clinical manifestations of cystic fibrosis in black and white patients, Hamosh et al., The Journal of Pediatrics, Vol 132, Issue 2, 255-259
\n[4] Cystic Fibrosis mutation ?F508 in Finland: other mutations predominate, Kore J et al., Human Genetics, Vol 85, No 4, 413-415,
\n[5] Cystic Fibrosis Foundation, Patient Registry Annual Data Report 2009, http:\/\/www.cff.org\/UploadedFiles\/research\/ClinicalResearch\/Patient-Registry-Report-2009.pdf<\/a>
\n[6] From registry to quality management: the German Cystic Fibrosis Quality Assassment project 1995 – 2006, European Respiratory Journal, 2008, 31, 29-35
\n[7] Cystic Fibrosis Registry of Ireland,\u00a0 Annual Report 2009, http:\/\/www.cfri.ie\/docs\/annual_reports\/CFRI2009.pdf<\/a>, Nov 2011
\n[8] Cystic Fibrosis Trust, CF Registry, http:\/\/www.cftrust.org.uk\/aboutcf\/publications\/cfregistryreports<\/a>, Nov 2011
\n[9] European Cystic Fibrosis Society, ECFS Patient Registry, http:\/\/www.ecfs.eu\/projects\/ecfs-patient-registry\/project<\/a>, Nov 2011
\n[10] Cystic fibrosis across Europe: EuroCareCF analysis of demographic data from 35 countries, Mehta G, Macek M, Mehta M, Journal of Cystic Fibrosis, Vol 9 (2010), S5-S21
\n[11] Cystic Fibrosis Canada, Canadian Cystic Fibrosis Patient Data Registry Report 2009, http:\/\/www.cysticfibrosis.ca\/assets\/files\/pdf\/CPDR_ReportE.pdf<\/a> , Nov 2011<\/div>\n","protected":false},"excerpt":{"rendered":"

Článok je stručným prehľadom toho, na čo sa v súčasnosti zameriava epidemiológia pri cystickej fibróze. Epidemiológia je veda, ktorá využíva predovšetkým štatistické metódy pre analýzu veľkého rozsahu demografických a klinických údajov. Výsledkom sú rozsiahle databázy a ich štatistické spracovania, z … Continued<\/a><\/p>\n","protected":false},"author":7,"featured_media":1235,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"kt_blocks_editor_width":"","footnotes":""},"categories":[3],"tags":[192,193],"class_list":["post-884","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-lieky-a-liecba","tag-epidemiologia","tag-pacientske-registre"],"yoast_head":"\nCF epidemiol\u00f3gia a pacientsk\u00e9 registre - Slan\u00e9 deti<\/title>\n<meta name=\"description\" content=\"\u010cl\u00e1nok o epidemiol\u00f3gii v cystickej fibr\u00f3ze ako aj o n\u00e1rodn\u00fdch registroch cystickej fibr\u00f3zy.\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/slanedeti.sk\/cms\/cf-epidemiologia-a-pacientske-registre-2\/884\" \/>\n<meta property=\"og:locale\" content=\"sk_SK\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"CF epidemiol\u00f3gia a pacientsk\u00e9 registre - 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