Abantu abanesifo se- coronary disease (CAD) beselula banokuba neengxaki zokuzalwa ze-cholesterol, ngokukodwa ukuba isifo senhliziyo esasifanelekile sibonakala sisebenza kwintsapho. Uhlobo oluqhelekileyo lwemfuyo echaphazela i-cholesterol luhlobo lwe-hypercholesterolemia.
I-hypercholesterolemia eqhelekileyo yimizimba ye-genetic syndrome apho amanqanaba e- LDL e-cholesterol aphakanyisiwe ukusuka ngexesha lokuzalwa.
Abantu abane-hypercholesterolemia yentsapho banomngcipheko omkhulu we-CAD ngaphambi kokuqala, isifo sobungozi , kunye nesifo se-artery . Enyanisweni, abantu abaninzi abane- infyoction ye- myocardial abaselula banokufumana le meko.
Ngethamsanqa, ngokunyanzeliswa ngonyango ukunciphisa ama- cholesterol amanqanaba, ingozi yesifo senhliziyo ingancitshiswa kakhulu. Ngenxa yoko, kubalulekile ukuxilonga i-hypercholesterolemia yentsapho ngokukhawuleza kunokwenzeka - kwaye uqinisekise ukuba amalungu omntu kwanalowo mqathango nawo ahlolwe igazi.
Izizathu
I-hypercholesterolemia eqhelekileyo ingabangelwa ziintsilelo ezahlukeneyo zofuzo, ezininzi zazo zichaphazela i-receptor ye-LDL ye-cholesterol. Xa i-receptor ye-LDL ingasebenzi ngokuqhelekileyo, i-LDL ye-cholesterol ayifakwanga ngokufanelekileyo kwigazi. Ngenxa yoko, i-LDL i-cholesterol yakha egazini. Lezi zigaba ze-LDL ze-cholesterol zinyusa kakhulu i- atherosclerosis kunye nesifo senhliziyo.
Ukungaqhelekanga kwemfuyo eyenza i-hypercholesterolemia yentsapho ingayifumana ilifa elivela kubaba, unina, okanye ngabazali bobabini. Abantu abaye bazuza ilifa lokungaqhelekanga kubo bobabini abazali baxelelwa ukuba baxhomekeke kwi-hypercholesterolemia yomndeni. Ifom ye-homozygous ye-hypercholesterolemia yentsapho yindlela enzima kakhulu yesifo.
Ichaphazela abantu abayi-250,000.
Abantu abadla ilifa lomntu ongaqhelekanga kumzali omnye kuphela kuthiwa yi- heterozygous kwi-hypercholesterolemia yomndeni. Leyo ifom ephantsi kakhulu yesifo, kodwa isanda kakhulu kwandisa ingozi yomntu. Phantse omnye kubantu abangama-500 bane-heterozygous family hypercholesterolemia.
Kubantu abaninzi.
Kuye kwachongwa iziganeko ezingaphezulu kwe-1000 ezichaphazela i-LDL i-gene receptor, kwaye nganye yazo ithinta i-receptor ye-LDL ngendlela eyahlukileyo. Ngenxa yesi sizathu, akusiyo yonke i-hypercholesterolemia yohlobo olufanayo. Ubunzima buyahlukahluka kakhulu, kuxhomekeke kuhlobo oluthile lokuguqulwa kofuzo lomntu.
Ukuxilongwa
Oogqirha benza i-hypercholesterolemia yentsapho ngokulinganisa amanqanaba egazi, kunye nokuqwalasela imbali yentsapho kunye nokuhlolwa komzimba.
Iimvavanyo zegazi kubantu abane-hypercholesterolemia yomndeni babonisa izinga eliphezulu le-cholesterol kunye neqondo eliphezulu le-LDL ye-cholesterol. Inqanaba le-cholesterol elipheleleyo nale meko ngokuqhelekileyo likhulu kune-300 mg / dl kubantu abadala, kwaye likhulu kuno-250 mg / dl kubantwana. Inqanaba le-cholestolli ye-LDL ngokuqhelekileyo likhulu kunama-200 mg / dl kubantu abadala, kwaye likhulu kunama-170 mg / dl kubantwana.
Amanqanaba e-Triglyceride aqhelekanga ngokukodwa kubantu abaneli meko.
Nabani na onomdla we-hypercholesterolemia yintsapho inokuba neentsapho ezinesimo. Ngoko imbali yentsapho yezifo zesifo senhliziyo isisifo esinamandla ukuze ugqirha ukuba acinge ngale ngxaki.
I-hypercholesterolemia eqhelekileyo inokubangela ukuba iziqhamo ezinamafutha zihlakulele ezijikelezileyo, iidolo, kunye neetononi, kunye neenxa zonke zaso. Ezi ntlawulo ezinamafutha zibizwa ngokuthi yi xanthomas . I-cholesterol idilesi kwiibophe , ezibizwa ngokuba yi- xanthelasmas , ziqhelekile. Nanini na isigulane sinexanthomas okanye i-xanthelasmas, ukuxilongwa kwe-hypercholesterolemia yomndeni kufuneka kufike ngokukhawuleza kwengqondo yengcali.
Ukuxilongwa ngokuxhomekeka kwe-hypercholesterolemia yentsapho kunokwenziwa ukuba amazinga e-LDL ye-cholesterol aphezulu kakhulu, amanqanaba e-triglyceride aqhelekileyo, kwaye imbali yentsapho iyahambelana. Ukuba ngaba i-xanthomas okanye i-xanthelasmas ikhona, ukuxilongwa kunokuqwalaselwa ngokuchanekileyo. Ukuvavanya i-Genetic kunokunceda (kodwa ngokuqhelekileyo akuyimfuneko) ekwenzeni ukuxilongwa, kwaye kunokuba luncedo kakhulu ngenjongo yokucebisa ngezofuzo.
Isifo se-vascular esibangelwa yi-hypercholesterolemia yintsapho iqala ngexesha lobuncinci. Ngoko abantwana kwiintsapho ezile ngxaki kufuneka zihlolwe rhoqo kwizinga eliphezulu ze-LDL ze-cholesterol ezincinci ezineminyaka ezisibhozo. Ukuba izinga labo le-cholesterol liphakanyisiwe, unyango lweemimiselo kufuneka luqwalaselwe ngamandla.
Isiqhagamshelo kwiSifo soGawulayo
Ngaphambi kokufumaneka kwezidakamizwa ze- statin , iziganeko zesifo se-cardiovascular premature ziphezulu kakhulu kubantu abane-hypercholesterolemia kunye neentsapho zabo. Kwisifundo esikhulu esenziwa ngonyaka wama-1970 (phambi koo-statins), iipesenti ezingama-52 zesigulane esinezigulane ezine-hypercholesterolemia sasinesifo senhliziyo esiphantsi kweminyaka engama-60 ngo-60 ubudala (xa kuthelekiswa nomngcipheko okulindeleke wepesenti ezili-9.
Unyango
Ukuphuhliswa kwezilwanyana ezinamandla, "isibini-generation generation" izidakamizwa zitshintshe ulawulo lwe-hypercholesterolemia. Ngaphambi kokufumaneka kwezi zidakamizwa ezinamandla, unyango lwalesi sifo lufuna ukusetyenziswa kwamachiza amaninzi, kubandakanywa nezidakamizwa zesiganeko esincinci "sokuqala". Nangona le ndlela yokusetyenziswa kwezidakamizwa yayinciphise ingozi yezilwelwe zengqondo, inyango ingaba nzima ukunyamezela kwaye yayinzima ukuyilawula.
Ngokuphuhliswa kwe-statins -atorvastatin (i-Lipitor) yesibini-esinamandla kakhulu, i-rosuvastatin (Crestor), okanye i-simvastatin (iZocor) -indlela yokunyanga i-hypercholesterolemia yentsapho ishintshile. Unyango ngoku uqalwe ngesantya esiphezulu sesicatshulwa esisodwa, sesizukulwana sesizukulwana sesibini. Ezi zi yobisi zivelisa ukunciphisa okukhulu kwizinga le-cholesterol ye-LDL, kwaye kunokuvelisa i-shrinkage yangempela ye- atherosclerotic plaques .
Izifundo ezithe zajonga ukongeza izidakamizwa ezongezelelweyo (ngokukodwa, i- Ezitimibe / Vtorin ), ukuya kwii-statins yesizukulwana sesibini ziye zahluleka ukubonisa naluphina uphuculo olongezelelweyo kwiziphumo zeklinikhi. Ngoko, kwizigulane ezine-heterozygous familial hypercholesterolemia (ifomu "elibi"), i-dose ephakamileyo, unyango olulodwa lonyango kunye nesigqirha sesizukulwana sesibini luye lwahlala lucetyiswa unyango.
Ngo-2015, i-FDA ivume iklasi elitsha lamachiza-i- PCSK9 inhibitors- kwizigulane ezine-hypercholesterolemia. Xa elinye leziyobisi longezwa kwi-statin, ukunciphisa ngokuphawulekayo kumanqanaba e-cholesterol ye-LDL kufumaneka. Indima ye-PCSK9 inhibitors ekunakekeleni i-heterozygous familial hypercholesterolemia okwangoku ingabonakali, ekubeni izilingo zonyango zijolise ekuboniseni ukuba olu nhlanganisela luphucula iziphumo zekliniki eziqhubekayo. Sekunjalo, ezi zonyango ezinamandla zingenakongezwa kwimimiselo ye-dose ephakamileyo ukuba i-LDL i-cholesterol ihlala iphakanyisiwe kakhulu kwi-anti-static treatment kuphela.
Nangona iinqwelo ze-cholesterol eziphakanyisiweyo zandisa kakhulu ingozi yesifo senhliziyo kubantu abane-hypercholesterolemia yomndeni, ezinye izinto ezinobungozi be-cardiovascular remain still important. Ngoko umbandela obaluleke kakhulu kunyango lwabo ukulawula ngokugqithiseleyo zonke ezinye iingozi zentliziyo yesifo, ngakumbi ukutshaya, ukukhuluphala, ukungabikho kokuzivocavoca kunye nokunyuswa kwengcinezelo yegazi .
Ifomu leHomozygous
Kwizigulane ezine-homozygous (ezinzima) uhlobo lwe-hypercholesterolemia yentsapho, ingozi ye-cardiovascular is high kangangokuthi unyango olushushu lucebisa ngokukhawuleza xa imeko ifunyaniswa. Ngenxa yokuphakama okukhulu kwe-cholesterol ebonwe kwezi zi gulane, iziphakamiso zangoku ziza kuqaliswa unyango kunye zombini wezonyithi eziphezulu kunye ne-PCSK9 inhibitor.
Nangona lolu hlobo lonyango olunefuthe, ngamanye amaxesha, amazinga e-cholesterol ahlala ephakamileyo. Kule meko, unyango nge- apheresis kunokufuneka ukuba ivelise amaqondo e-cholesterol.
Isishwankathelo
I-hypercholesterolemia eqhelekileyo yintlupheko ezuze ilifa le-cholesterol metabolism. Abantu abane-hypercholesterolemia yentsapho badinga unyango olunefuthe lokunciphisa amazinga e-cholesterol kunye nokulawula ezinye izinto ezinobungozi benhliziyo, ukwenzela ukunciphisa umngcipheko wesifo senhliziyo esesifushane. Kubaluleke kakhulu ukuba amalungu abo entsapho ahlolwe le meko.
Imithombo:
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