Abantu abane- coronary artery disease (CAD) basencane bangase babe nenkinga engakazalwa nge-cholesterol, ikakhulukazi uma isifo senhliziyo esasifushane singabonakala sisebenza emndenini. Uhlobo oluvame kakhulu lwesimo sezakhi zofuzo oluthinta i-cholesterol yi-hypercholesterolemia yomndeni.
I-hypercholesterolemia ejwayelekile i-genetic syndrome lapho amazinga e- LDL cholesterol ephakanyisiwe kusukela ngesikhathi sokuzalwa.
Abantu abane-hypercholesterolemia emndenini banengozi enkulu ye-CAD ngaphambi kwesikhathi, isifo sohlangothi , nesifo se-artificial . Eqinisweni, abantu abaningi abane- infarction ye-myocardial basencane baqala ukuthola lesi simo.
Ngenhlanhla, nge ukwelashwa okunamandla ukunciphisa amazinga e- cholesterol , ingozi yesifo senhliziyo ingancishiswa kakhulu. Ngenxa yalesi sizathu, kubalulekile ukuxilonga i-hypercholesterolemia yomndeni ngokushesha ngangokunokwenzeka - nokuqinisekisa ukuthi amalungu omndeni womuntu onalesi simo nawo ahlolwa igazi lawo.
Izimbangela
I-hypercholesterolemia evamile ingabangelwa yiziphambeko ezihlukahlukene zezofuzo, eziningi zazo eziphazamisa i-receptor ye-LDL cholesterol. Lapho umamukeli we-LDL engasebenzi ngendlela evamile, i-cholesterol ye-LDL ayisusiwe ngokuphumelelayo kusukela egazini legazi. Ngenxa yalokho, i-cholesterol ye-LDL ikhiqiza egazini. Lezi zinhlobo ze-cholestolol ezingaphezu kwe-LDL ziyasheshisa kakhulu ukuqina kwe-atherosclerosis kanye nesifo senhliziyo.
Ukungahleleki kwezakhi zofuzo okubangelwa i-hypercholesterolemia yomndeni kungathathwa njengefa, unina, noma bobabili abazali. Abantu abazuze ukungafani kwabo bobabili abazali kuthiwa bangabantu abathandekayo ngokweqile kwe-hypercholesterolemia yomndeni. I-homozygous uhlobo lwe-hypercholesterolemia yomndeni yisimo esibi kakhulu sesifo.
Ithinta umuntu oyedwa kubantu abangu-250,000.
Abantu abadla ifuzo elingavamile kusuka kumzali oyedwa kuphela kuthiwa yi- heterozygous ye-hypercholesterolemia yomndeni. Leli fomu elibi kakhulu lesi sifo, kodwa landisa kakhulu ingozi yomuntu yengqondo. Cishe umuntu oyedwa kubantu abangu-500 une-hypercholesterolemia ye-heterozygous yomndeni.
Yilokho abantu abaningi.
Izinguquko ezingaphezu kwezingu-1000 ezithinta i-gene receptor gene zitholiwe, futhi ngamunye wabo uthinta umemukeli we-LDL ngendlela ehlukile. Ngenxa yalesi sizathu, akuwona wonke ama-hypercholesterolemia omndeni afanayo. Ubunzima bungahlukana kakhulu, kuye ngokuthi uhlobo oluthile lokushintshashintsha kwezakhi zofuzo umuntu analo.
Ukuxilongwa
Odokotela benza ukuxilongwa kwe-hypercholesterolemia yomndeni ngokulinganisa amazinga omlomo wegazi, nokucabangela umlando womndeni nokuhlolwa komzimba.
Ukuhlolwa kwegazi kubantu abane-hypercholesterolemia yomndeni kubonisa amazinga amaningi e-cholesterol namazinga aphakeme e-LDL ama-cholesterol. Izinga le-cholesterol eliphelele nalesi simo ngokuvamile likhulu kune-300 mg / dl kubantu abadala, futhi likhulu kunama-250 mg / dl ezinganeni. Izinga le-cholestolol LDL livame kakhulu kunama-200 mg / dl kubantu abadala, futhi likhulu kuka-170 mg / dl ezinganeni.
Amazinga we-Triglyceride ngokuvamile awaphakanyiswa ngokukhethekile kubantu abanalesi simo.
Noma ubani one-hypercholesterolemia yomndeni kungenzeka ukuthi abe nezihlobo ezinesimo. Ngakho umlando womndeni wezifo zakuqala zezinhliziyo kungaba yindlela eqondile yokuthi udokotela abhekane nalokhu kutholakala.
I-hypercholesterolemia ejwayelekile ingahle ibangele ukuthi izici ezinamafutha zikhuphuke ezizungeze izimbongolo, amadolo, ama-tendon, kanye nokuzungeze i-cornea yamehlo. Lezi zinhlamvu ezinamafutha zibizwa nge- xanthomas . I-cholesterol ibeka kumajwabu amehlo, okuthiwa i- xanthelasmas , nawo avamile. Noma nini lapho isiguli sine-xanthomas noma i-xanthelasmas, ukuxilongwa kwe-hypercholesterolemia yomndeni kufanele kungene ngokushesha engqondweni yodokotela.
Ukuxilongwa ngokucophelela kwe-hypercholesterolemia yomndeni kungenziwa uma amazinga e-LDL ama-cholesterol ephezulu kakhulu, amazinga e-triglyceride ajwayelekile, futhi umlando womndeni uhambisana. Uma kukhona i-xanthomas noma i-xanthelasmas, ukuxilongwa kungacatshangwa kahle. Ukuvivinya izifo kungaba usizo (kodwa ngokuvamile akudingekile) ekwenzeni ukuxilongwa, futhi kungasiza kakhulu ngezinhloso zokuthola ukwelulekwa kwezakhi zofuzo.
Isifo se-vascular esibangelwa i-hypercholesterolemia yomndeni iqala ngesikhathi seyingane. Ngakho abantwana emindenini enalesi sifo kufanele bahlolwe njalo ngamazinga aphakeme e-LDL ama-cholesterol aseneminyaka engu-8 ubudala. Uma amazinga e-cholesterol awo ephakanyisiwe, ukwelapha ngeziqu kufanele kubhekwe ngokuqinile.
Xhumanisa Izifo Zenhliziyo
Ngaphambi kokutholakala kwezidakamizwa ze- statin , izifo zesifo senhliziyo ngaphambi kwesikhathi zaphezulu kakhulu kubantu abane-hypercholesterolemia yomndeni nasezihlotsheni zabo. Esifundweni esikhulu esenziwa phakathi neminyaka yama-1970 (ngaphambi komthetho), amaphesenti angu-52 yezihlobo zesilisa zeziguli ezine-hypercholesterolemia enesifo senhliziyo eseneminyaka engama-60 (uma kuqhathaniswa nengozi ekulindeleke engamaphesenti angu-13), kanti amaphesenti angu-32 wezihlobo zabesifazane babe nesifo senhliziyo eneminyaka engama-60 (uma kuqhathaniswa nengozi ekulindeleke yamaphesenti angu-9. Lesi sifundo senza ikhaya lendabuko yalesi simo.
Ukwelapha
Ukuthuthukiswa kwezidakamizwa ezinamandla, "izizukulwane zesibili" zashintsha ukuphathwa kwe-hypercholesterolemia yomndeni. Ngaphambi kokutholakala kwalezi zidakamizwa ezinamandla, ukwelashwa kwalesi sifo kudinga ukusebenzisa imithi eminingi, kufaka phakathi izidakamizwa zesimiso esincane "esesizukulwane sokuqala" esincane. Ngenkathi le ndlela eminingi yezidakamizwa yanciphisa ingozi yeziguli ze-cardiovascular, ukwelashwa kungaba nzima ukubekezelela futhi kwaba nzima ukuphatha.
Ngokuthuthukiswa kwe-statins -atorvastatin (Lipitor) yesibili esinamandla ngokwengeziwe, i-rosuvastatin (Crestor), noma i-simvastatin (iZocor) -indlela yokwelapha i-hypercholesterolemia yomndeni ishintshile. Ukwelashwa sekuqale ngomthamo ophezulu wesidakamizwa esisodwa esisodwa, sesizukulwane sesizukulwane sesizukulwane. Lezi zidakamizwa ngokuvamile zikhiqiza ukunciphisa okukhulu kwamazinga e-LDL cholesterol, futhi zingase zikhiqize i-shrinkage yangempela yama- atherosclerotic plaques .
Izifundo ezibukezile ukwengeza izidakamizwa ezengeziwe (ikakhulukazi, u- Ezitimibe / Vytorin ), kuya ku-statins yesizukulwane sesibili zihlulekile ukukhombisa ukuthuthukiswa okungeziwe emiphumeleni yomtholampilo. Ngakho-ke, ezigulini ezine-hypercholesterolemia (i-hypercholesterolemia) ye-heterozygous yomndeni (ifomu "elibi"), isisindo esiphakeme, ukwelapha okukodwa kwezidakamizwa kanye nesimiso sesizukulwane sesizukulwane sesibili sishiye ukwelashwa okunconywayo.
Ngo-2015, i-FDA ivumile iklasi elisha lezidakamizwa- i- PCSK9 inhibitors- kubagula abane-hypercholesterolemia yomndeni. Uma enye yalezi zidakamizwa ingeziwe kwisimiso, ukunciphisa okuphawulekayo kwamazinga e-LDL cholesterol kungatholakala. Indima ye-PCSK9 inhibitors ekwelapheni i-hypercholesterolemia ye-heterozygous yomndeni okwamanje ayifuni kahle, ngoba izilingo zomtholampilo zihlose ukubonisa ukuthi le nhlanganisela ithuthukisa imiphumela yomtholampilo eseqhubekayo. Noma kunjalo, lezi zidakamizwa ezintsha ezinamandla zingase zengezwe emazingeni amancane aphezulu uma i-LDL i-cholesterol isalokhu iphakanyisiwe kakhulu kwi-statin therapy yedwa.
Nakuba amazinga e-cholesterol aphakanyiswe kakhulu ukwandisa ingozi yesifo senhliziyo kubantu abane-hypercholesterolemia yomndeni, ezinye izinto ezibangelwa yizinhliziyo zengqondo zihlala zibalulekile futhi. Ngakho isici esibucayi ekwelapheni kwabo ukulawula ngokuhlukumezeka zonke ezinye izifo zabo ezingozini zezinhliziyo, ikakhulukazi ukubhema, ukukhuluphala, ukungazivocavoca, nokucindezelwa kwegazi okuphakeme .
Ifomu le-Homozygous
Kulezi ziguli ezine-homozygous (severe) uhlobo lwe-hypercholesterolemia yomndeni, ingozi ye-cardiovascular is high kangangokuba ukwelashwa okunonya kakhulu kunconywa ngokushesha uma isimo sesitholakala. Ngenxa yokuphakama okukhulu kwe-cholesterol ebonwe kulezi ziguli, izincomo zamanje zizoqala ukwelashwa nazo zombili izitezi eziphezulu kanye ne-PCSK9 inhibitor.
Ngisho nangalolu hlobo lwezokwelapha eziyingozi, ngezinye izikhathi amazinga e-cholesterol ahlala ephakeme. Kulezi zimo, ukwelashwa nge- apheresis kungadingeka ukuletha amazinga e-cholesterol phansi.
Isifingqo
I-hypercholesterolemia ejwayelekile yinkinga ebalulekile yefa ye-cholesterol metabolism. Abantu abane-hypercholesterolemia emndenini badinga ukwelashwa okunonya ukuze kunciphise amazinga e-cholesterol nokulawula ezinye izici zengozi yomzimba, ukuze kuncishiswe ingozi ye-heart disease ngaphambi kwesikhathi. Kubaluleke kakhulu ukuthi amalungu omndeni wabo ahlolwe lesi simo.
Imithombo:
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