Ukusilela kwefactor X

Ukusilela kweFactor X (ishumi) sisiphazamiso esibangelwe kukusilela kweprotein ebizwa ngokuba yic factor X egazini. Kukhokelela kwiingxaki ze-clotting yegazi (i-coagulation).

Xa usopha, uthotho lweempendulo zenzeka emzimbeni onceda amahlwili egazi. Le nkqubo ibizwa ngokuba yi-coagulation cascade. Ibandakanya iiproteni ezikhethekileyo ezibizwa ngokuba yi-coagulation, okanye i-clotting, factor. Unokuba namathuba aphezulu okuphuma egazini ngokugqithisileyo ukuba enye okanye ezingakumbi zezi zinto zilahlekile okanye azisebenzi ngendlela ebekufanele ukuba zisebenza ngayo.

Inqaku X yenye yezinto ezinje ngokuxinana. Ukusilela kwefactor X kuhlala kubangelwa sisiphene esizuzwe njengelifa kwi-X factor gene. Oku kubizwa njengelifa le-X lokusilela. Amanqanaba okopha ukusuka kubumnene ukuya kubunzima ngokuxhomekeke ekubeni ukusilela kakhulu kangakanani.

Ukusilela kwefactor X kusenokubangelwa yenye imeko okanye ukusetyenziswa kwamayeza athile. Oku kubizwa ngokuba kukusilela kwe-factor X. Ukufumaneka kwe-factor X kuqhelekile. Ingabangelwa ngu:

• Ukungabikho kwe-vitamin K (ezinye iintsana zizalwa zine-vitamin K)
• Ukwakhiwa kweeproteni ezingaqhelekanga kwizicubu nakwizitho (amyloidosis)
• Isifo esibi sesibindi
• Ukusetyenziswa kwamayeza athintela i-clotting (ii-anticoagulants ezifana ne-warfarin)

Abasetyhini abanesiphene se-X bangaqala ukufumanisa ukuba banegazi elinzima kakhulu lokuya exesheni kunye nokopha emva kokubeleka. Imeko inokuqatshelwa okokuqala kumakhwenkwe asandul 'ukuzalwa ukuba aphuma igazi elihlala ixesha elide kunesiqhelo emva kokwaluka.

Iimpawu zinokubandakanya nayiphi na kwezi zinto zilandelayo:

• Ukopha kumalungu
• Ukopha kwimisipha
• Ukukrala ngokulula
• Ukopha okuninzi kokuya exesheni
• Inwebu yencindi iyopha
• Ukuphuma kweempumlo okungayeki ngokulula
• Umtya we-umbilical ukopha emva kokuzalwa

Uvavanyo olunokwenziwa lunokubandakanya:

• Inqaku X uvavanyo
• Ixesha lecandelo le-thromboplastin (PTT)
• Ixesha leprothrombin (PT)

Ukopha kunokulawulwa ngokufumana infravenous (IV) infusions zeplasma okanye imixube yezinto ezijiyisayo. Ukuba awunayo i-vitamin K, ugqirha wakho uya kukunika i-vitamin K ukuba uyithathe ngomlomo, nge-injection phantsi kwesikhumba, okanye nge-vein (intravenously).

Ukuba unale ngxaki yokuphuma kwegazi, qiniseka ukuba:

• Xelela ababoneleli bakho bezempilo ngaphambi kokuba ube naluphi na uhlobo lwenkqubo, kubandakanya utyando kunye nomsebenzi wamazinyo.
• Xelela amalungu osapho lwakho kuba banokuba nengxaki efanayo kodwa abayazi okwangoku.

Ezi zixhobo zinokubonelela ngolwazi ngakumbi malunga nokusilela kwe-X:

• Isiseko seSizwe seHemophilia Foundation: Okunye ukusilela kweFactor- www.hemophilia.org/Bleeding-Disorder/Iindidi-ukukhupha igazi-Iingxaki/Okunye-Ifactor-Ukusilela
• Umbutho weSizwe weengxaki zoRare -rarediseases.org/rare-diseases/factor-x-deficiency
• Isalathiso seKhaya le-NIH yemfuzo- ghr.nlm.nih.gov/condition/factor-x-defence

Iziphumo zilungile ukuba imeko ithambile okanye ufumana unyango.

Ukusilela kwilifa le-X yimeko yobomi bonke.

Imbonakalo yokunqongophala kwe-factor X ixhomekeke kwisizathu. Ukuba sibangelwa sisifo sesibindi, iziphumo zixhomekeke ekubeni isifo sakho sesibindi singanyangwa njani. Ukuthatha izongezo ze vithamin K kuya kunyanga ukusilela kwe vithamin K Ukuba ukuphazamiseka kubangelwa yi-amyloidosis, kukho iindlela ezininzi zonyango. Ugqirha wakho unokukuxelela ngakumbi.

Ukopha okumandla okanye ukulahleka kwegazi ngesiquphe (ukopha) kunokwenzeka. Amalungu anokukhubazeka kwisifo esibuhlungu esivela kwigazi elininzi.

Fumana uncedo lonyango olungxamisekileyo ukuba unegazi elingachazwanga okanye elibi.

Akukho sithintelo saziwayo sokusilela kwilifa X. Xa ukunqongophala kukavithamini K kungunobangela, ukusebenzisa ivithamin K kunokunceda.

Ukunqongophala kweStuart-Prower

• Ukubumba kwegazi
• Amahlwili egazi

UGailani D, Wheeler AP, Neff AT. Ukunqongophala kwezinto ezinqabileyo. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ii-eds. I-Hematology: Imigaqo esisiseko kunye nokuziqhelanisa. Umhla wesi-7. IPhiladelphia, PA: Elsevier; I-2018: isahluko 137.

Iholo JE. I-Hemostasis kunye nokuqina kwegazi. Ku: Iholo JE, ed. Incwadi kaGuyton kunye neHolo yePhysical Physiology. Umhla we-13. IPhiladelphia, PA: Elsevier; 2016: isahluko 37.

Ragni MV. Ukuphazamiseka kwento eyophayo: ukungabikho kwento ekunqandeni. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Umhla wama-25. IPhiladelphia, PA: Elsevier Saunders; Ngo-2016: isahluko 174.