Inkinga abantu abaningi abanayo kulezi zinsuku ukwanda kwamafutha afana ne-cholesterol kanye ne-triglycerides egazini. Kodwa ngezinye izikhathi la mazinga amafutha, ikakhulukazi amazinga e-triglyceride, aphezulu ngokungavamile, izikhathi eziyizinkulungwane ngaphezu kokuvamile. Isizathu salokhu kungaba yisimo esingavamile kakhulu sezakhi zofuzo esidluliselwa kubazali. Namuhla sikhuluma ngesinye salezi zimo, i-Familial Hyperchylomicronemia Syndrome, noma i-FCS ngamafuphi. Nakuba lesi kuyisihloko esiyinkimbinkimbi, masisiqonde kalula.
Kalula nje, iyini i-FCS?
I-FCS yisimo esingavamile kakhulu sofuzo osithola kubazali bakho. Umuntu onalesi simo akakwazi ukugaya amafutha, ikakhulukazi ama-triglycerides .
Ake sithi imizimba yethu inefektri encane ephula amafutha. Ngokwezokwelapha, lokhu kuyi-enzyme ebizwa ngokuthi i-lipoprotein lipase (LpL) . Kumuntu one-FCS, le 'fektri' ayisebenzi kahle, noma ayisebenzi nhlobo.
Ngenxa yalokho, amafutha ekudleni esikudlayo, abizwa ngokuthi ama-triglycerides, awakwazi ukumuncwa ngumzimba kodwa kunalokho aqoqana egazini njengamaconsi amafutha abizwa ngokuthi ama-chylomicrons . Lokhu kuqongelela kwama-chylomicrons yilokho esikubiza ngokuthi i-chylomicronemia syndrome. Lokhu kubangela amazinga aphezulu kakhulu e-triglyceride egazini.
Akuvamile ukuthi umuntu oyedwa noma ababili kuphela kwabayisigidi emhlabeni wonke bathinteke. Ngokuvamile kutholakala ukuthi banalesi sifo ngaphambi kokuba bafinyelele eminyakeni eyi-10, kanti abanye abantwana batholakala benaso ngonyaka wokuqala wokuphila.
Kungani lokhu kwenzeka?
Imbangela eyinhloko yalokhu ukuguquka kwezakhi zofuzo. Isizathu salokhu ukukhubazeka kwezakhi zofuzo ezihilelekile ekwenzeni i-enzyme ye-lipoprotein lipase (LpL) esikhulume ngayo.
Into ebalulekile ukuthi ukuze uthole lesi sifo, kufanele uthole lesi sakhi sofuzo esingalungile kumama wakho nakubaba wakho. Uma usithola kumzali oyedwa kuphela, ngeke usithole lesi sifo, kodwa uzoba 'umthwali' walesi sakhi sofuzo. Lokhu kusho ukuthi unamandla okudlulisela lesi sakhi sofuzo ezinganeni zakho.
I-enzyme ye-LpL ikhiqizwa yi-pancreas yethu. Umsebenzi wayo oyinhloko ukuphula ama-chylomicron egazini nokusiza umzimba ukuthi usebenzise amafutha akuwo ukuze uthole amandla. Ngakho-ke uma i-LpL ingasebenzi kahle, amafutha awakwazi ukuqhekeka futhi amazinga e-triglyceride egazini akhuphuka kakhulu.
Ziyini izimpawu zalokhu?
Izimpawu ze-FCS zibangelwa amazinga aphezulu e-triglycerides egazini. Amazinga e-triglyceride omuntu ophilile angaphansi kuka-150 mg/dL. Kodwa-ke, kumuntu one-FCS, leli zinga lingadlula u-1,000 mg/dL. Kubaluleke kakhulu ukuqaphela lezi zimpawu.
| Isibonakaliso | Incazelo elula |
|---|---|
| Ubuhlungu besisu | Lolu uphawu oluvame kakhulu. Lobuhlungu obuhlala buqala esiswini esingaphezulu budlulele emhlane. Ngezinye izikhathi bungaba bukhulu kakhulu. |
| I-Pancreatitis | Lokhu kuvame ukuba imbangela yobuhlungu besisu. I-pancreas iyisitho esibalulekile ngaphakathi esiswini sethu. Yilapho ivuvukala khona. Izimpawu ezifana nokucanuzela kwenhliziyo, ukujuluka, ukuzizwa ubuthakathaka, kanye nokuphuzi kwamehlo nesikhumba nakho kungahambisana nalokhu. |
| Izinguquko zesikhumba | Abanye abantu baba nesimo esibizwa ngokuthi ama-cutaneous xanthomas . Lawa amaqhubu amancane angenabuhlungu, abomvu-aphuzi, avela ezindaweni ezifana nezinqe, amadolo, kanye nezindololwane. Lawa akhiwe ngamafutha. Uma uwabona, kungaba uphawu lwamafutha aphezulu egazini. |
| Izinguquko emehlweni | I-Lipaemia retinalis yisimo lapho imithambo yegazi engaphakathi kweso ibonakala sengathi inobisi. Lokhu kubangelwa ukunqwabelana kwamafutha. Kodwa-ke, akulimazi ukubona. |
| Ukwanda kwesibindi kanye ne-spleen | Ikakhulukazi ezinganeni ezincane, uhlobo oluthile lweseli elimhlophe legazi (ama-macrophage) luzama ukumunca amafutha amaningi emzimbeni. Lawa maseli amunca amafutha bese ewafaka esibindini nasesinqeni, okwenza lezo zitho zomzimba zikhule. |
| Izimpawu zengqondo nezezinzwa | Ezinye iziguli zingase zibe nezimo ezifana nokucindezeleka, ukulahlekelwa yinkumbulo, kanye nokuwohloka komqondo. |
Lokhu kutholakala futhi kwelashwa kanjani?
Ukuxilongwa
Ngenxa yokuthi kuyisimo esingavamile, ngezinye izikhathi kungathatha isikhathi ukuthola ukuxilongwa okunembile. Uma unezinhlungu zesisu eziqhubekayo, ukuhlaselwa yi-pancreatitis okuphindaphindiwe, kanye namazinga aphezulu e-triglyceride egazini lakho, udokotela wakho angase asole i-FCS.
Ukuhlolwa okulandelayo kwenziwa ukuqinisekisa lesi sifo:
- Ukuhlolwa kwegazi le-triglyceride yokuzila ukudla: Lokhu kuhlolwa kwenziwa ngemva kokuzila ukudla amahora amaningana. Uma izinga le-triglyceride liphakeme kune-750 mg/dL, kungaba yi-FCS. Ngezinye izikhathi isampula yegazi ingabonakala sengathi ilubisi.
- Ukuhlolwa kwezinga le-lipase: Kunikezwa umjovo okhethekile (i-heparin) ukuze kulinganiswe ukuthi ingakanani i-enzyme ye-LpL ekhiqizwa ngumzimba.
- Ukuhlolwa kofuzo: Lena yindlela kuphela yokuqiniseka ngokuphelele. Ingahlola ukuthi i-gene enephutha izuzwe njengefa kubazali bobabili.
- I-CT scan: Lokhu kusiza ukubona ukuthi kukhona yini umonakalo ezithweni zomzimba ezifana ne-pancreas nesibindi.
Izindlela zokwelapha
Ukudla kuyindlela eyinhloko yokwelapha i-FCS, futhi kukhona nomuthi omusha osanda kuvunyelwa.
Into ebaluleke kakhulu: Imithi efana nama-statin nama-fibrate, avame ukunikezwa i-cholesterol, ayisebenzi ku-FCS. Ngoba ukuze leyo mithi isebenze, i-enzyme ye-LpL esikhulume ngayo ekuqaleni idinga ukusebenza kahle emzimbeni.
Ukudla kwe-FCS
Lena yingxenye ebaluleke kakhulu yokuphatha. Kubalulekile ukusebenzisana nodokotela wakho kanye nochwepheshe wezokudla ukuze uthuthukise lolu hlelo lokudla.
- Nciphisa amafutha: Inani lamafutha adliwayo ngosuku kufanele libe ngaphansi kwamagremu angu-20.
- Yeka ukuphuza utshwala ngokuphelele: Utshwala bukhulisa amazinga e-triglyceride nakakhulu.
- Nciphisa ushukela olula nama-carbohydrate: Gwema izinto ezifana neziphuzo ezinoshukela namaswidi.
- Izinto okufanele uzidle: Imifino, izithelo, ubhontshisi, okusanhlamvu okuphelele, amaqanda amhlophe, imikhiqizo yobisi engenamafutha, kanye nenyama engenamafutha.
- Izithasiselo zamavithamini: Udokotela wakho angase ancome ukuthi uphuze amavithamini ancibilikayo emafutheni njengevithamini A, D, E, kanye ne-K, okudingekayo emzimbeni ukuze unciphise amafutha.
Umuthi omusha
U-Oletarsen (Tryngolza)Kungumuthi omusha owavunywa ngoDisemba 2024. Kungumjovo ojovwa ngaphansi kwesikhumba kanye ngenyanga. Kalula nje, usebenza ngokunciphisa ukukhiqizwa komzimba kweprotheni ebizwa ngokuthi i-apoC-III, esiza amafutha ukuthi aqoqeke egazini. Ungafunda kabanzi ngalokhu kudokotela wakho.
Izinselele zokuphila ne-FCS
I-FCS yisimo esihlala isikhathi eside esingaba nomthelela omkhulu empilweni yomzimba neyengqondo.
- Ubunzima bokudla: Ukudla ngaphandle nokuya emaphathini kungaba nzima kakhulu.
- Imiphumela yengqondo: Ubuhlungu obuvamile, ukwesaba ikusasa, ukukhathazeka, kanye "nenkungu yobuchopho" kungase kwenzeke.
- Umthelela empilweni yomphakathi kanye nasemsebenzini: Ukulaliswa esibhedlela njalo kungenza kube nzima ukugcina ubudlelwano bomphakathi kanye nomsebenzi.
Ngakho-ke, kubaluleke kakhulu ukusebenzisana eduze nethimba lakho lezokwelapha futhi, uma kudingeka, funa ukwesekwa ngumeluleki wezempilo yengqondo.
Umlayezo Wokuya Nawe Ekhaya
- I-FCS yisimo esingavamile sofuzo esibangela ukuthi umzimba ungakwazi ukuphula amafutha (ama-triglycerides).
- Lokhu kubangela amazinga aphezulu kakhulu e-triglyceride egazini, futhi izimpawu eziyinhloko ubuhlungu obukhulu besisu kanye nokuvuvukala kwe-pancreas (i-pancreatitis).
- Ingxenye eyinhloko yokwelashwa ukulandela ukudla okuqinile okunqunyelwe amafutha . Utshwala kufanele bugwenywe ngokuphelele.
- Imithi ejwayelekile ye-cholesterol ayisebenzi kulesi simo, kodwa kukhona umuthi osanda kwethulwa.
- Ukuphatha lesi simo kuyinselele yokuphila konke, ngakho-ke kubalulekile ukuthola ukwesekwa udokotela wakho, isazi sokudla okunempilo, kanye nomndeni.
- Uma wena noma othile emndenini wakho enalezi zimpawu, bona udokotela wakho ngokushesha bese ucela iseluleko.
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