Vanhu vane chirwere chetachiona (CAD) vachiri vaduku vanogona kunge vane dambudziko rekuzvarwa necholesterol, kunyanya kana chirwere chepfungwa chisati chapera chinoratidzika chinomhanya mumhuri. Rudzi rwakawanda kupfuura mamwe ose rwemageneti anokonzera cholesterol ndeyemhuri hypercholesterolemia.
Familial hypercholesterolemia is genetic syndrome umo LDL cholesterol mazinga akakwirira kubva panguva yekuzvarwa.
Vanhu vane mhuri ye hypercholesterolemia vane njodzi yakakura yeCAD isati yasvika, chirwere chepakati , uye chirwere chetachiona chirwere . Zvechokwadi, vanhu vazhinji vane chirwere chemyocardial muzera remakore vanotanga kuva nemamiriro aya.
Nenzira inofadza, nechisimba kuporesa kuderedza cholesterol , hutachiwana hwechirwere chemoyo hunogona kuderedzwa zvikuru. Nokuda kwechikonzero ichi, zvakakosha kufungidzira mhuri ye hypercholesterolemia nokukurumidza sezvinobvira - uye kuve nechokwadi kuti nhengo dzemhuri dzomumwe munhu ane chirwere ichi vanewo ropa remidziyo yakaedzwa .
Zvinokonzera
Fedal hylescholesterolemia inogona kukonzerwa nehutano hwakasiyana-siyana hunokonzerwa nehutachiona, huzhinji hwahwo hunobata mukana weLDL cholesterol. Apo LDL receptor isingashandi zvakajairika, LDL cholesterol haina kubviswa zvinobudirira kubva muropa. Nokudaro, LDL cholesterol inovaka muropa. Izvo izvi zvinodhaka zvinonzi LDL cholesterol zvinowedzera kukurumidza atherosclerosis uye nehutano hwehutano.
Maitiro anokonzerwa nehutano hwemhuri anonzi hypercholesterolemia anogona kupiwa nhaka kubva kuna baba, amai, kana vabereki vose. Vanhu avo vakagara nhaka yekusasikirwa kubva kuvabereki vose vari kunzi homozygous for family hypercholesterolemia. Nzira ye homozygous yemhuri inonzi hypercholesterolemia inorwara zvakanyanya zvechirwere chacho.
Inobata mumwe mu 250,000 vanhu.
Vanhu vanogara nhaka yegeni risina kunaka kubva kune mumwe mubereki mumwe chete vanonzi heterozygous yemhuri hypercholesterolemia. Ichi chimiro chisina simba chechirwere ichi, asi ichiri kuwedzera zvikuru moyo wemunhu. Inenge imwe muvanhu 500 ine heterozygous family hypercholesterolemia.
Ndiyo vanhu vazhinji.
Zvimwe zvinopfuura 1000 zvakasiyana-siyana zvinokonzerwa neLDL receptor gene zvakaonekwa, uye chimwe nechimwe chazvo chinokanganisa mutambi weLDL zvakasiyana zvakasiyana. Nokuda kwechikonzero ichi, havasi vose vehukama hwe hypercholesterolemia dzakafanana. Kuoma kunogona kusiyana zvakasiyana, zvichienderana nerudzi rwemhando yakasiyana-siyana yezvekuberekwa kwemunhu munhu.
Kuziva
Vanachiremba vanoita kuti chirwere chemhuri chiratidze hypercholesterolemia kuburikidza nekuyera ropa, uye kufungisisa nhoroondo yemhuri uye kuongorora mumuviri.
Kuyera ropa muvanhu vane mhuri ye hypercholesterolemia inoratidza huwandu hwekushanda kweklesterol uye yakakwirira yeLDL cholesterol mazinga. Zvose zvinokonzerwa necholesterol nemamiriro ezvinhu aya kazhinji zvinopfuura 300 mg / dl muvanhu vakuru, uye inopfuura 250 mg / dl muvana. I-LDL cholesterol iyo inowanzopfuura 200 mg / dl muvanhu vakuru, uye inopfuura 170 mg / dl muvana.
Triglyceride mazinga kazhinji haasi kunyanya kukwirira kune vanhu vane chirwere ichi.
Ani naani ane mhuri ye hypercholesterolemia inogona kunge ine hama dzave nemamiriro ezvinhu. Saka nhoroondo yemhuri yepakutanga chirwere chemwoyo chinogona kuva chikonzero chakasimba chechiremba kuti afungisise izvi.
Fedal hylescholesterolemia inogonawo kukonzera hutano hwemafuta huripo kuti huve nepamusoro pemakumbo, mabvi, pamwe netononi, uye kumativi ose e-cornea emeso. Aya mafuta aripo anonzi xanthomas . Cholesterol inowanikwa pamawere , anonzi xanthelasmas , anowanzonziwo. Pese apo murwere ane xanthomas kana xanthelasmas, kuongororwa kwemhuri ye hypercholesterolemia kunofanira kupinda pakarepo mupfungwa yechiremba.
Kuzvidzivirira kwehutano hwemhuri inonzi hypercholesterolemia kunogona kuitwa kana iLDL cholesterol mazinga akareba kwazvo, mazinga e triglyceride akajairika, uye nhoroondo yemhuri inoenderana. Kana kana xanthomas kana xanthelasmas varipo, kuongororwa kunogona kuonekwa sekutaura kwakakwana. Kuongorora zvirwere kunogona kubatsira (asi kazhinji hazvidiwi) mukuita kuti kuongororwa, uye kunogona kubatsira zvikuru pamagariro ekupa mazano ekuberekwa.
Chirwere chevascular chinokonzerwa nehutachiona hwemhuri chinotanga muhuduku. Saka vana mumhuri dzine chirwere ichi vanofanira kugara vachiongororwa nepamusoro yeLDL cholesterol madiki vachiri vaduku ava nemakore masere. Kana cholesterol yavo yakasimudzwa, kurapa nemasimoni kunofanira kunyatsoongororwa.
Link to Cardiovascular Disease
Isati yasvika, kuwanikwa kwemishonga yemasimoni , chiitiko chepfungwa dzisati dzasara chirwere chakanga chakakwirira kwazvo kuvanhu vane mhuri ye hypercholesterolemia uye nehama dzavo. Muchidzidzo chikuru chakaitwa muma1970 (isati yasvika), 52 muzana yehama dzehama dzevarwere vane hypercholesterolemia yemhuri vaiva nechirwere chemwoyo pazera remakore makumi matanhatu (zvichifananidzwa nechinotarisirwa chekutaridzika 13 muzana), uye 32 muzana yehama dzechikadzi dzakanga dzine chirwere chemwoyo pazera remakore makumi matanhatu (zvichifananidzwa nechinotarisirwa chinotarisirwa ne9 muzana.Chidzidzo ichi chakakonzera mhuri yemamiriro ezvinhu aya.
Kurapa
Kubudirira kwezvakawanda, "chizvarwa chechipiri-chizvarwa" mishonga yemishonga yakashandura utungamiri hwemhuri hypercholesterolemia. Pamberi pekuwanikwa kwemishonga iyi ine simba, kurapwa kwechirwere ichi kwakada kushandisa kushandiswa kwemishonga yakawanda, kusanganisira iro rinotonyanya kukosha "chizvarwa chokutanga" mishonga yemishonga. Kunyange zvazvo iyi nzira yakasiyana-siyana yekushandisa zvinodhaka yakaderedza varwere vehutano hwehutano, kurapwa kungave kwakaoma kushivirira uye zvaive zvakaoma kutarisira.
Nekuvandudzwa kwechipiri chechizvarwa chechipiri-statvastatin -atorvastatin (Lipitor), rosuvastatin (Crestor), kana simvastatin (Zocor) - nzira yekurapa mhuri ye hypercholesterolemia yakashanduka. Kurapa ikozvino kwakatanga nehuwandu hwemazana, wechipiri-chizvarwa chechirwere chechirwere. Iyi mishonga inowanzoita kuderedza kukuru kweLDL cholesterol, uye inogonawo kubereka shrinkage chaiyo ye atherosclerotic plaques .
Zvidzidzo izvo zvakatarisa kuwedzera mamwe madhiragi (kunyanya, Ezitimibe / Vytorin ), kusvika kuchizvarwa chechizvarwa chechipiri chakakundikana kuratidzira chero humwe huri nani huri nani mukugadzirisa zvipatara. Saka, kune varwere vane heterozygous family hypercholesterolemia (iyo "mikukutu" maitiro), nhamba yepamusoro, chirwere chisina kukodzera-mishonga nechipiri-generation statins yakaramba ichirapa kurairwa.
Muna 2015, FDA yakagamuchira boka idzva rezvinodhaka- PCSK9 inhibitors-kune varwere vane hypercholesterolemia yemhuri. Apo imwe yemishonga iyi yakawedzerwa kune chimiro, zvinoderedza zvinoshamisa muLDL cholesterol mazinga zvinogona kuwanikwa. Basa rePCSK9 inhibitors mukurapa heterozygous family hypercholesterolemia iye zvino haisi pachena, sezvo mishonga yemakirini inotarisirwa kuratidza kuti kusanganiswa uku kunovandudza zvido zvekurapwa zvichiri kuitika. Kunyange zvakadaro, mishonga iyi ine simba ine simba inogona kuwedzerwa kumazamu epamusoro-soro kana LDL cholesterol ichiramba yakasimudzirwa zvakanyanya pamutemo wechirongwa chete.
Kunyange zvazvo cholesterol yakakwirira inowedzera hutachiwana hwehutano hwemwoyo kune vanhu vane mhuri ye hypercholesterolemia, zvimwe zvepfungwa dzepfungwa zvinoramba zvichiri kukosha zvakare. Saka chinhu chinonyanya kukosha pakurapa kwavo ndechokutonga nechisimba zvose zvimwe zvinokonzera ngozi kune chirwere chepfungwa, kunyanya kusvuta, kuneta, kusava nekuita muviri, uye kukwira kweropa .
Homozygous Form
Muvatachiona vane homozygous (yakaoma) maitiro emhuri ye hypercholesterolemia, mungozi yemwoyo yakanyanya kwazvo zvokuti chirwere chinotyisa chinokurudzirwa pakarepo apo mamiriro acho akawanikwa. Nemhaka yekumhanya kwakanyanya mucholesterol inowanikwa kune ava varwere, mazano aripo zvino ndeyokutanga kurapa nezvose zvakanyanya-dose statins uye PCSK9 inhibitor.
Kunyangwe nerudzi urwu rwemishonga ine utsinye, asi dzimwe nguva cholesterol inoramba yakakwirira. Mune zviitiko izvi, kurapwa ne apheresis kunogona kudiwa kuti uwane cholesterol mazinga pasi.
Summary
Maziviro e hypercholesterolemia chirwere chakakomba chinokonzerwa neklesterterol metabolism. Vanhu vane mhuri ye hypercholesterolemia vanoda kurapwa kwechisimba kuderedza cholesterol maitiro uye kudzora mamwe mamiriro evanhu vane ngozi, kuitira kuti kuderedza dambudziko rekushaya chirwere chepfungwa chisati chavapo. Izvo zvakakosha zvikuru kuti nhengo dzemhuri dzavo dziongorwe nekuda kwechiitiko ichi.
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