Ingxaki abantu abaninzi abanayo kule mihla kukwanda kwamafutha afana ne-cholesterol kunye ne-triglycerides egazini. Kodwa ngamanye amaxesha la manqanaba amafutha, ingakumbi amanqanaba e-triglyceride, aphezulu ngokungaqhelekanga, amaxesha amaninzi ngaphezu kwesiqhelo. Isizathu soku sisenokuba yimeko engaqhelekanga kakhulu yemfuza edluliselwa kubazali. Namhlanje sithetha ngesinye sezifo ezinjalo, i-Familial Hyperchylomicronemia Syndrome, okanye i-FCS ngamafutshane. Nangona esi sisihloko sinzima kancinci, masiqonde ngokulula.
Ngamafutshane, yintoni iFCS?
I-FCS yimeko engaqhelekanga kakhulu yemfuza oyizuza kubazali bakho. Umntu onesi sifo akakwazi ukuqhekeza, okanye ukugaya amafutha, ingakumbi i-triglycerides .
Khawucinge nje ukuba imizimba yethu inefektri encinci eqhekeza amafutha. Ngokwezonyango, le yi-enzyme ebizwa ngokuba yi-lipoprotein lipase (LpL) . Kumntu one-FCS, le 'fektri' ayisebenzi kakuhle, okanye ayisebenzi kwaphela.
Ngenxa yoko, amafutha akukutya esikutyayo, abizwa ngokuba yi-triglycerides, awanakufunxwa ngumzimba kodwa endaweni yoko aqokelelana egazini njengamathontsi amafutha abizwa ngokuba yi-chylomicrons . Oku kuqokelelana kwee-chylomicrons yinto esiyibiza ngokuba yi-chylomicronemia syndrome. Oku kubangela amanqanaba aphezulu kakhulu e-triglyceride egazini.
Kunqabile ukuba umntu omnye okanye ababini kuphela kwisigidi emhlabeni jikelele bachaphazeleke. Idla ngokufunyaniswa ingekafiki iminyaka eli-10, kwaye abanye abantwana bafunyaniswa benale meko kunyaka wokuqala wobomi.
Kutheni oku kusenzeka?
Eyona nto ibangela oku kukuguquka kwezakhi zofuzo. Isizathu sale nto sisiphene kwizakhi zofuzo ezibandakanyeka ekwenzeni i-enzyme ye-lipoprotein lipase (LpL) esithethe ngayo.
Into ebalulekileyo kukuba ukuze ube nesi sifo, kufuneka uzuze esi sifuzo sinesiphene kumama wakho nakutata wakho. Ukuba usizuza kumzali omnye kuphela, awuyi kuba naso esi sifo, kodwa uya kuba 'ngumthwali' waso. Oku kuthetha ukuba unokukwazi ukudlulisela esi sifuzo kubantwana bakho.
I-enzyme ye-LpL iveliswa yipancreas yethu. Umsebenzi wayo ophambili kukuqhekeza ii-chylomicrons egazini kwaye incede umzimba usebenzise amafutha akuyo ukuze ufumane amandla. Ngoko ke xa i-LpL ingasebenzi kakuhle, amafutha awanakuqhekeka kwaye amanqanaba e-triglyceride egazini ayanda.
Zithini iimpawu zoku?
Iimpawu ze-FCS zibangelwa ngamanqanaba aphezulu e-triglycerides egazini. Amanqanaba e-triglyceride yomntu ophilileyo angaphantsi kwe-150 mg/dL. Nangona kunjalo, kumntu one-FCS, eli nqanaba linokudlula i-1,000 mg/dL. Kubaluleke kakhulu ukuqaphela ezi mpawu.
| Uphawu | Ingcaciso elula |
|---|---|
| Intlungu yesisu | Olu lolona phawu luqhelekileyo. Le ntlungu ingaqhelekanga inokuqala esiswini esingaphezulu ize idlulele emqolo. Ngamanye amaxesha inokuba buhlungu kakhulu. |
| I-Pancreatitis | Oku kudla ngokuba yimbangela yentlungu yesisu. I-pancreas sisitho esibalulekileyo ngaphakathi esiswini sethu. Kulapho idumba khona. Iimpawu ezifana nokucaphuka, ukubila, ukuziva ubuthathaka, kunye nokutyheli kwamehlo nolusu nazo zinokuhambisana noku. |
| Utshintsho lolusu | Abanye abantu bakhula imeko ebizwa ngokuba yi -cutaneous xanthomas . Ezi ziingqukuva ezincinci ezingenabuhlungu, ezibomvu-zimthubi, ezibonakala kwiindawo ezifana neempundu, amadolo, kunye neengqiniba. Ezi zenziwe ngamafutha. Ukuba uzibona ezi, isenokuba luphawu lwamafutha aphezulu egazini. |
| Utshintsho emehlweni | I-Lipaemia retinalis yimeko apho imithambo yegazi engaphakathi kweliso ibonakala ngathi yibisi. Oku kubangelwa kukuqokelelwa kwamafutha. Nangona kunjalo, ayilimazi umbono. |
| Ukwandiswa kwesibindi kunye ne-spleen | Ingakumbi kubantwana abancinci, uhlobo oluthile lweeseli ezimhlophe zegazi (ii-macrophages) luzama ukufunxa amafutha angaphezulu emzimbeni. Ezi seli zifunxa amafutha zize ziwabeke esibindini nakwi-spleen, nto leyo ebangela ukuba ezo zitho zomzimba zikhule. |
| Iimpawu zengqondo neze-neurological | Ezinye izigulana zinokuba neemeko ezifana nokudakumba, ukulahlekelwa yinkumbulo, kunye ne-dementia. |
Ifunyaniswa njani kwaye inyangwa njani le nto?
Uxilongo
Ngenxa yokuba yimeko engaqhelekanga, ngamanye amaxesha kungathatha ixesha ukufumana uxilongo oluchanekileyo. Ukuba unentlungu yesisu engapheliyo, ukuhlaselwa yi-pancreatitis ngokuphindaphindiweyo, kunye namanqanaba aphezulu e-triglyceride egazini lakho, ugqirha wakho unokurhanela i-FCS.
Kwenziwa ezi mvavanyo zilandelayo ukuqinisekisa isifo:
- Uvavanyo lwegazi lwe-triglyceride yokuzila ukutya: Olu vavanyo lwenziwa emva kokuzila ukutya iiyure ezininzi. Ukuba inqanaba le-triglyceride liphezulu kune-750 mg/dL, lisenokuba yi-FCS. Ngamanye amaxesha isampuli yegazi ingabonakala ngathi lubisi.
- Uvavanyo lwenqanaba le-lipase: Kunikwa inaliti ekhethekileyo (i-heparin) ukulinganisa ukuba ingakanani i-enzyme ye-LpL eveliswa ngumzimba.
- Uvavanyo lwemfuza: Le yindlela yokuqiniseka nge-100%. Ingajonga ukuba imfuza enesiphene ifunyenwe ngabazali bobabini.
- I-CT scan: Oku kunceda ukubona ukuba kukho umonakalo kwizitho ezifana nepancreas nesibindi.
Iindlela zonyango
Ukutya yeyona nto iphambili kunyango lwe-FCS, kwaye kukwakho neyeza elitsha elivunyiweyo kutshanje.
Eyona nto ibalulekileyo: Amayeza anjenge-statins kunye ne-fibrates, adla ngokunikezelwa kwi-cholesterol, awasebenzi kwi-FCS. Kuba ukuze loo mayeza asebenze, i-enzyme ye-LpL esithethe ngayo ngaphambili kufuneka isebenze kakuhle emzimbeni.
Ukutya kwe-FCS
Le yeyona nxalenye ibalulekileyo yolawulo. Kubalulekile ukusebenzisana nogqirha wakho kunye nengcali yesondlo ukuze uphuhlise esi sicwangciso sokutya.
- Nciphisa amafutha: Ubungakanani bamafutha asetyenziswayo ngosuku kufuneka bube ngaphantsi kwama-20 grams.
- Yeka ukusela utywala ngokupheleleyo: Utywala bunyusa amanqanaba e-triglyceride nangakumbi.
- Nciphisa iiswekile kunye neekhabhohayidreyithi ezilula: Ziphephe izinto ezifana neziselo ezineswekile kunye neelekese.
- Izinto zokutya: Imifuno, iziqhamo, iimbotyi, iinkozo ezipheleleyo, amaqanda amhlophe, iimveliso zobisi ezingenamafutha, kunye nenyama engenamafutha.
- Izongezo zevithamini: Ugqirha wakho unokucebisa ukuba uthathe iivithamini ezinyibilikayo emafutheni ezifana neevithamini A, D, E, kunye neK, ezifunekayo emzimbeni ukuze kuncitshiswe amanqatha.
Amayeza amatsha
U-Olezarsen (Tryngolza)Liyeza elitsha elamkelwe ngoDisemba ka-2024. Liyeza elifakwa phantsi kolusu kanye ngenyanga. Ngamafutshane, lisebenza ngokunciphisa ukuveliswa kweproteni emzimbeni ebizwa ngokuba yi-apoC-III, enceda amafutha aqokeleleke egazini. Ungafunda okungakumbi ngale nto kugqirha wakho.
Imingeni yokuphila ne-FCS
I-FCS yimeko yobomi bonke ekufuneka ilawulwe. Inokuba nefuthe elikhulu kwimpilo yomzimba neyengqondo.
- Ubunzima bokutya: Ukutya ngaphandle nokuya kwiipati kunokuba nzima kakhulu.
- Iziphumo zengqondo: Iintlungu ezihlala zisenzeka, uloyiko lwekamva, ixhala, kunye "nenkungu yobuchopho" zinokwenzeka.
- Impembelelo kubomi bentlalo kunye nomsebenzi: Ukulaliswa esibhedlele rhoqo kunokwenza kube nzima ukugcina ubudlelwane bentlalo kunye nomsebenzi.
Kungoko ke, kubaluleke kakhulu ukusebenzisana ngokusondeleyo neqela lakho lezonyango, kwaye ukuba kuyimfuneko, funa inkxaso yomcebisi wezempilo yengqondo.
Umyalezo Wokuya Ekhaya
- I-FCS yimeko engaqhelekanga yemfuza ebangela ukuba umzimba ungakwazi ukuqhekeza amafutha (ii-triglycerides).
- Oku kubangela amanqanaba aphezulu kakhulu e-triglyceride egazini, kwaye iimpawu eziphambili ziintlungu zesisu eziqatha kunye nokudumba kwe-pancreas (i-pancreatitis).
- Eyona nto iphambili kunyango kukulandela ukutya okungqongqo kakhulu okunemilinganiselo engqongqo yamafutha . Utywala kufuneka buphetshwe ngokupheleleyo.
- Amayeza e-cholesterol aqhelekileyo awasebenzi kule meko, kodwa kukho iyeza elitsha elisandul’ ukungeniswa.
- Ukulawula le meko ngumceli mngeni wobomi bonke, ngoko ke kubalulekile ukufumana inkxaso kugqirha wakho, ingcali yesondlo, kunye nosapho.
- Ukuba wena okanye umntu osapho lwakho unezi mpawu, bonana nogqirha wakho ngokukhawuleza kwaye ucele ingcebiso.
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