Sonke sithetha nge-cholesterol ngalo lonke ixesha, kwaye sixhalabile ngayo, akunjalo? Kodwa khawucinge ngayo, ngaba wakha weva ngesifo esizuzwe njengelifa apho amanqanaba e-cholesterol aphezulu ngokungaqhelekanga ukususela ebuntwaneni, mhlawumbi kwanasebuntwaneni? Namhlanje siza kuthetha ngesifo esingaqhelekanga kodwa esibaluleke kakhulu. Esi sibizwa ngokuba yi-Homozygous Familyal Hypercholesterolemia (HoFH). Nangona eli gama liyinkimbinkimbi kancinci, masiliqonde ngokulula.
Yintoni iHomozygous Familyal Hypercholesterolemia?
Ngamafutshane, esi sisifo semfuza esenza kube nzima emizimbeni yethu ukususa i-cholesterol "embi", oko kukuthi, i-LDL (i-Low-Density Lipoprotein) cholesterol, egazini lethu. Sonke siyazi ukuba i-cholesterol yinto efana ne-wax efunekayo kwiiseli zethu. Umsebenzi we-LDL cholesterol kukuthwala le cholesterol emzimbeni wonke ngegazi.
Kodwa xa amanqanaba e-LDL ephezulu kakhulu, le cholesterol eyongezelelweyo iqala ukuqokelelana kwimithambo yegazi enika igazi entliziyweni yethu, imithambo yegazi. Ekuhambeni kwexesha, ezi ntsalela zinokuvala imithambo yegazi, zinqumle igazi kunye neoksijini eya entliziyweni, kwaye zonyuse umngcipheko wokuhlaselwa yintliziyo .
Esi sifo sizalwa naso. Oku kuthetha ukuba unokuba ne-cholesterol ephezulu ukususela ebuntwaneni. Esi sisifo esinzulu esingenakunyangwa kwaye kufuneka silawulwe ubomi bakho bonke.
Ukuba ayinyangwa, amadoda anale meko anokuba nesifo sentliziyo xa ekwiminyaka yawo engama-40 kunye nabafazi abakwiminyaka yawo engama-50. Kungoko ke, kubaluleke kakhulu ukukwazi oku kunye nokufuna unyango olufanelekileyo.
Yintoni isizathu soku?
Esi sisifo esibizwa ngokuba sisifo semfuza. Enyanisweni, ufumana esi sifo kuphela ukuba ufumana iikopi ezimbini zemfuza ezingasebenzi kakuhle, enye evela kumama wakho enye evela kutata wakho .
Ngokwesiqhelo, isibindi sethu sisusa i-cholesterol ye-LDL engaphezulu egazini. Isibindi sine-'LDL receptors' ekhethekileyo yoku. Ezi zifana neemagnethi ezibamba i-cholesterol ziyisuse egazini. Kodwa kumntu onesi sifo, ngenxa ye-gene enesiphene esiyizuzileyo, ezi receptors azisebenzi kakuhle. Isiphumo kukuba amanqanaba e-cholesterol anyuka ngokungalawulekiyo.
Usenokuba ukhe weva nge-'Heterozygous' Familyal Hypercholesterolemia. Kulapho i-gene enesiphene ifunyanwa khona ngumzali omnye kuphela. Le meko ayinzima njenge-'Homozygous'.
Zithini iimpawu zesi sifo?
Kukho iimpawu eziphambili ezininzi ezinokusetyenziswa ukuchonga esi sifo. Masizijonge kwitheyibhile.
| Uphawu | Inkcazo |
|---|---|
| Inqanaba eliphezulu kakhulu le-cholesterol | Amanqanaba e-cholesterol iyonke anokuba yi-600 mg/dL okanye ngaphezulu (inqanaba eliphilileyo lingaphantsi kwama-200). |
| Iidipozithi zesikhumba (iiXanthomas) | Amaqhuma atyheli, anjengencindi phantsi kolusu kwiingqiniba, emadolweni, nasezimpundu. La ngamaqhuma e-cholesterol. |
| Iidiphozithi kwiinkophe (iiXanthelasmas) | Amanqatha atyheli kwiinkophe. |
| Utshintsho lwamehlo (i-Arcus cornealis) | Isangqa esingwevu okanye esimhlophe esijikeleze iliso elimnyama. |
| Iimpawu zentliziyo | Iimpawu ezifana neentlungu zesifuba (i-angina), ukuphefumla okunzima, kunye nokubetha kwentliziyo ngokukhawuleza zinokwenzeka xa umntwana esemncinci. |
Indlela yokuxilonga esi sifo?
Ukuba unale mpawu, kubalulekile ukubona ugqirha wakho. Ugqirha uza kuqala enze uvavanyo lomzimba aze akubuze imibuzo embalwa.
- Ngaba ukhe waphawula into efana namabala atyheli eluswini lwakho?
- Ngaba unentlungu esifubeni?
- Ngaba uziva uphelelwa ngumphefumlo?
- Ngaba abazali bakho nabo bane-cholesterol ephezulu?
Emva koko, ugqirha wakho uza kwenza uvavanyo lwegazi ukujonga amanqanaba akho e-cholesterol. Banokwenza uvavanyo lwe-genetic ukubona ukuba unayo na i-gene engalunganga ebangela esi sifo. Ugqirha wakho angavavanya namalungu osapho lwakho asondeleyo.
Iindlela zonyango kunye nolawulo
Injongo ephambili yonyango kukunciphisa amanqanaba akho e-cholesterol ye-LDL kunye nokunciphisa umngcipheko wesifo sentliziyo.Uhlala uthunyelwa kugqirha ogxile kwizifo ze-cholesterol ezifana nale (Lipidologist).
Utshintsho kwindlela yokuphila kunye nokutya
Inyathelo lokuqala kukwamkela ukutya okunempilo kwentliziyo okunamafutha agcweleyo aphantsi, i-cholesterol, kunye neswekile. Kubalulekile nokuyeka ukutshaya, ukunciphisa utywala, kunye nokuzilolonga yonke imihla.
Amayeza
Ngenxa yokuba amanqanaba akho e-cholesterol ephezulu ngendlela engaqhelekanga, unokuqala ukuthatha iidosi eziphezulu ze -statins , ezisebenza ngokuthintela isibindi ekuveliseni i-cholesterol.
Kunye noko, amanye amayeza anciphisa ubungakanani be-cholesterol efunxwa ekutyeni (umz. i-Ezetimibe , i-PCSK9 inhibitors , i-Lomitapide ) anokongezwa.
Unyango oluthile
Ukuba i-cholesterol ayinakulawulwa ngamayeza odwa, kuya kufuneka kuqwalaselwe ezinye iindlela zonyango.
- I-Apheresis: Le yinkqubo efana ne-dialysis kwizigulana zezintso. Uqhagamshelwe kumatshini esibhedlele, igazi lakho liyakhutshwa emzimbeni wakho, i-cholesterol ye-LDL iyahluzwa, kwaye igazi elicociweyo libuyiselwa emzimbeni wakho. Olu lunyango oluthatha iiyure ezininzi kwaye kufuneka lwenziwe rhoqo.
- Ukufakelwa kwesibindi: Ukuba akukho nanye kwezi ndlela zonyango zingasentla ephumelelayo, ukufakelwa kwesibindi kunokuba yindlela yokugqibela. Isibindi esitsha esisempilweni sinee-receptor ze-LDL, ezinokunceda ukulawula i-cholesterol. Olu lutyando olunzima kakhulu, kwaye ukuchacha kunokuthatha iinyanga ezi-6 ukuya kunyaka.
Xa usenza unyango olunzima kangaka, kubaluleke kakhulu ukufuna inkxaso yeemvakalelo kusapho nakubahlobo. Thetha ngokukhululekileyo ngale nto nogqirha wakho.
Umyalezo Wokuya Ekhaya
- I-Homozygous Familyal Hypercholesterolemia yimeko embi kakhulu, kodwa engaqhelekanga, yemfuza ebangela amanqanaba aphezulu kakhulu e-cholesterol.
- Oku kungenxa yokuba bafumana ilifa lee-genes ezimbini ezineengxaki kumama nakutata wabo.
- Ngokuchonga nokuqala unyango kwasebuntwaneni, umngcipheko wesifo sentliziyo kwasebuntwaneni unokuncipha.
- Unyango luhlala ubomi bonke kwaye lunokubandakanya amayeza, ukutya, umthambo, kwaye mhlawumbi unyango olukhethekileyo (i-apheresis).
- Ukuba kukho umntu kusapho lwakho oye waba nesifo sentliziyo okanye i-cholesterol ephezulu esemncinci, kububulumko ukuba uhlolwe i-cholesterol yakho njengoko ugqirha wakho ekucebisile.
- Awuwedwa kolu hambo. Sebenzisana nogqirha wakho, usapho lwakho, kunye namaqela enkxaso ukuze ulawule le meko ngempumelelo.
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