I-hypercholesterolemia ejwayelekile

I-hypercholesterolemia ejwayelekile yinkinga edluliswa emindenini. Kubangela ukuthi izinga le-cholesterol le-LDL (elibi) libe phezulu kakhulu. Isimo siqala lapho kuzalwa futhi singadala ukuhlaselwa yinhliziyo besebancane.

Izihloko ezihlobene zifaka:

• I-hyperlipidemia ejwayelekile ehlanganisiwe
• I-hypertriglyceridemia ejwayelekile
• I-dysbetalipoproteinemia ejwayelekile

I-hypercholesterolemia ejwayelekile iyisifo sofuzo. Kubangelwa ukukhubazeka ku-chromosome 19.

Ukukhubazeka kwenza umzimba ungakwazi ukukhipha i-cholesterol ephansi ye-lipoprotein (LDL, noma embi) egazini. Lokhu kuholela ezingeni eliphezulu le-LDL egazini. Lokhu kukwenza ube sethubeni lokuba nokuncipha kwemithambo ye-atherosclerosis usemncane. Isimo sivame ukudluliselwa emindenini ngendlela evelele ye-autosomal. Lokho kusho ukuthi udinga kuphela ukuthola isakhi esingajwayelekile kumzali oyedwa ukuze uzuze lesi sifo njengefa.

Ezimweni ezingavamile, ingane ingazuza njengefa lozalo kubo bobabili abazali. Lapho lokhu kwenzeka, ukwanda kwezinga le-cholesterol kuba nzima kakhulu. Ingozi yokuhlaselwa yisifo senhliziyo nesifo senhliziyo iphezulu, ngisho nasebuntwaneni.

Eminyakeni yokuqala kungenzeka zingabi khona izimpawu.

Izimpawu ezingenzeka zifaka:

• Amafutha esikhumba anamafutha abizwa ngokuthi ama-xanthomas ngaphezulu kwezingxenye zezandla, izindololwane, amadolo, amaqakala kanye nxazonke zeso
• I-cholesterol ifaka ezinkalweni (xanthelasmas)
• Ubuhlungu besifuba (i-angina) noma ezinye izimpawu zesifo se-coronary artery zingaba khona zisencane
• Ukucinana kwenkonyane elilodwa noma womabili lapho uhamba
• Izilonda ezinzwaneni ezingapholi
• Izimpawu ezinjengokushaywa unhlangothi ngokungazelelwe njengokukhathazeka ukukhuluma, ukwehla ohlangothini lobuso, ubuthakathaka bengalo noma umlenze, nokulahlekelwa ibhalansi

Ukuhlolwa komzimba kungakhombisa ukukhula kwesikhumba okunamafutha okubizwa nge-xanthomas ne-cholesterol deposits esweni (corneal arcus).

Umhlinzeki wezokunakekelwa kwempilo uzobuza imibuzo ngomlando wakho wezempilo nowomndeni. Kungaba khona:

• Umlando oqinile womndeni we-hypercholesterolemia yomndeni noma ukuhlaselwa kwenhliziyo kwasekuqaleni
• Izinga eliphakeme le-LDL cholesterol kubo bobabili noma bobabili abazali

Abantu abavela emindenini abanomlando oqinile wokuhlaselwa yisifo senhliziyo kusenesikhathi kufanele kwenziwe ukuhlolwa kwegazi ukuthola amazinga e-lipid.

Ukuhlolwa kwegazi kungakhombisa:

• Izinga eliphakeme le-cholesterol ephelele
• Izinga eliphakeme le-LDL
• Amazinga ajwayelekile we-triglyceride

Olunye uvivinyo olungenziwa lubandakanya:

• Ucwaningo lwamaseli abizwa ngama-fibroblast ukubona ukuthi umzimba umunca kanjani i-cholesterol ye-LDL
• Ukuhlolwa kofuzo kokukhubazeka okuhambisana nalesi simo

Umgomo wokwelashwa ukunciphisa ubungozi besifo senhliziyo i-atherosclerotic. Abantu abathola ikhophi eyodwa kuphela yesakhi sofuzo esingalungile kubazali babo bangenza kahle ngokushintsha kokudla nezidakamizwa ze-statin.

IZINGUQUKO ZOKUPHILA

Isinyathelo sokuqala ukuguqula lokho okudlayo. Isikhathi esiningi, umhlinzeki uzoncoma ukuthi uzame lokhu izinyanga ezimbalwa ngaphambi kokunquma imithi. Izinguquko ekudleni zifaka ukwehlisa inani lamafutha owadlayo ukuze libe ngaphansi kwama-30% wekhalori yakho ephelele. Uma ukhuluphele ngokweqile, ukunciphisa umzimba kuyasiza kakhulu.

Nazi ezinye izindlela zokusika amafutha agcwele ekudleni kwakho:

• Yidla inyama yenkomo encane, inkukhu, ingulube, newundlu
• Miselela imikhiqizo yobisi egcwele enezinto ezinamafutha aphansi
• Susa amafutha we-trans

Ungehlisa inani le-cholesterol oyidlayo ngokuqeda izikhupha zamaqanda nenyama yezitho zomzimba efana nesibindi.

Kungasiza ukukhuluma nodokotela wezokudla ongakunika izeluleko ngokushintsha indlela odla ngayo. Ukwehla kwesisindo nokuvivinya umzimba njalo kungasiza ukwehlisa izinga le-cholesterol yakho.

IMITHI

Uma izinguquko zokuphila zingalishintshi izinga lakho le-cholesterol, umhlinzeki wakho angancoma ukuthi uthathe imithi. Kunezinhlobo eziningana zemithi etholakalayo yokusiza ukwehlisa izinga le-cholesterol egazini, futhi zisebenza ngezindlela ezahlukene. Ezinye zingcono ekwehliseni i-cholesterol ye-LDL, ezinye zinhle ekwehliseni i-triglycerides, kanti ezinye zisiza ukukhulisa i-cholesterol ye-HDL. Abantu abaningi bazoba semithini eminingi.

Izidakamizwa ze-Statin zivame ukusetshenziswa futhi zisebenza kahle kakhulu. Le mithi isiza ukunciphisa ingozi yokuhlaselwa yisifo senhliziyo nokushaywa unhlangothi.

Kubandakanya:

• I-Lovastatin (Mevacor)
• I-Pravastatin (Pravachol)
• I-Simvastatin (Zocor)
• I-Fluvastatin (Lescol)
• I-Atorvastatin (Lipitor)
• I-Pitivastatin (Livalo)
• I-Rosuvastatin (Crestor)

Eminye imithi yokwehlisa i-cholesterol ifaka phakathi:

• Izinhlaka ze-acid-sequestering.
• Ezetimibe.
• Ama-Fibrate (njenge-gemfibrozil noma i-fenofibrate).
• I-Nicotinic acid.
• Ama-PCSK9 inhibitors, afana ne-alirocumab (Okubalulekile) ne-evolocumab (Repatha). Lokhu kumelela isigaba esisha semithi yokwelapha i-cholesterol ephezulu.

Abantu abanefomu elibi lalesi sifo bangadinga ukwelashwa okubizwa nge- apheresis. Kususwa igazi noma iplasma emzimbeni. Izihlungi ezikhethekile zisusa i-cholesterol eyengeziwe ye-LDL, bese i-plasma yegazi ibuyiselwa emzimbeni.

Wenza kahle kangakanani kuncike ekutheni ulandela eduze kangakanani iseluleko sokwelashwa komhlinzeki wakho. Ukwenza ushintsho ekudleni, ukuzivocavoca umzimba, nokuthatha imithi yakho ngendlela efanele kunganciphisa izinga le-cholesterol. Lezi zinguquko zingasiza ukubambezela ukuhlaselwa yinhliziyo, ikakhulukazi kubantu abanefomu elibi lesi sifo.

Abesilisa nabesifazane abane-hypercholesterolemia yomndeni ngokuvamile basengozini enkulu yokuhlaselwa yinhliziyo kusenesikhathi.

Ingozi yokufa iyahlukahluka phakathi kwabantu abane-hypercholesterolemia yomndeni. Uma udla ifa lamakhophi amabili ohlobo lwesici esingalungile, uba nomphumela ompofu. Lolu hlobo lwe-hypercholesterolemia yomndeni aluphenduli kahle ekwelashweni futhi lungadala ukuhlaselwa yinhliziyo kusenesikhathi.

Izinkinga zingafaka:

• Ukuhlaselwa yisifo senhliziyo esemncane
• Isifo senhliziyo
• Unhlangothi
• Isifo semithambo yegazi

Funa ukunakekelwa kwezempilo ngokushesha uma unezinhlungu esifubeni noma ezinye izimpawu eziyisixwayiso zokuhlaselwa yisifo senhliziyo.

Shayela umhlinzeki wakho uma unomlando womuntu siqu noma womndeni wezinga eliphezulu le-cholesterol.

Ukudla okune-cholesterol eningi namafutha agcwele futhi acebile emafutheni angagcwele kungasiza ukulawula izinga lakho le-LDL.

Abantu abanomlando womndeni walesi simo, ikakhulukazi uma bobabili abazali bephethe isakhi sofuzo esingalungile, bangafuna ukululekwa ngofuzo.

Uhlobo II lwe-hyperlipoproteinemia; I-hypercholesterolemic xanthomatosis; Ukuguquguquka kwesisindo se-lipoprotein receptor

• Cholesterol - yini ongayibuza udokotela wakho

• Xanthoma - ukusondela
• Xanthoma emadolweni
• Ukuvaleka komthambo weCoronary

UGenest J, ukuphazamiseka kweLibby P. Lipoprotein nesifo senhliziyo. Ku: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, ama-eds. Isifo Senhliziyo SikaBraunwald: Incwadi Yemithi Yezinhliziyo Nemithambo. Umhla ka-11. IPhiladelphia, PA: Elsevier; 2019: isahluko 48.

URobinson JG. Ukuphazamiseka kwe-lipid metabolism. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 195.