Ngaba wakha waziva ngathi uza kuba negazi elijiyileyo? Okanye wakha waziva ngathi umlenze wakho udumbile kwaye ubuhlungu? Isizathu sezi zinto sisenokuba sahlukile kunendlela ocinga ngayo. Namhlanje sithetha ngemeko engaqhelekanga kodwa ebaluleke kakhulu ekufuneka uyiqaphele. Oko kukunqongophala kweProteni C. Nangona igama lisenokuvakala lingaqhelekanga, linxulumene nenkqubo yokujiyileka kwegazi emzimbeni wethu. Makhe sibone ukuba yintoni, kutheni isenzeka, kwaye ungenza ntoni ngayo.
Yintoni kanye kanye iProtein C Deficiency?
Ngamafutshane, ukunqongophala kweProteni C yimeko apho igazi lethu liqina ngaphezu kokuba lifuna, okanye ngendlela engaqhelekanga. Nantsi ingcaciso emfutshane.
Khawuthelekelele ukuba unenxeba emzimbeni wakho. Emva koko, ukuze umise ukopha, kufuneka igazi elithile lijiye. Yinto eqhelekileyo leyo. Yindlela yokuzikhusela yomzimba wethu. Kodwa kwenzeka ntoni ukuba oku kujiya kwegazi kuba yinto engalawulekiyo negqithisileyo? Kulapho ingxaki iqala khona.
Kukho izinto ezininzi zendalo egazini lethu ezilawula le nkqubo yokujiya kwegazi, njengenkqubo yokubhuleka kwemoto. Iproteni C yenye yezo zinto, iproteni ebaluleke kakhulu elawula ukujiya kwegazi. Oku kubizwa ngokuba yi-'anti-clotting agent' yendalo okanye i- anticoagulant . Oko kukuthi, into ethintela ukujiya kwegazi.
Ngoku uyaqonda, akunjalo? Ukunqongophala kweProteni C kuthetha ukuba awunayo eyaneleyo le protein egazini lakho. Xa oko kusenzeka, ibhuleki elawula ukujiya kwegazi ayisebenzi kakuhle. Isiphumo kukuba amahlwili egazi aqala ukwakheka kwimithambo yegazi. Amahlwili egazi ayenzeka ngale ndlela anokubangela iimeko eziyingozi kakhulu, kwanezisongela ubomi. Umzekelo, amahlwili egazi ayenzeka kwimithambo enzulu yemilenze (Deep Vein Thrombosis) kwaye iimeko ezifana nelo hlwili legazi liyaqhekeka lize libambeke emiphungeni (Pulmonary Embolism) .
Ixhaphake kangakanani le meko?
Oku akuqhelekanga ukubonwa, kodwa akunqabile njengoko sicinga.
- Uhlobo olungephi: Kuqikelelwa ukuba oku kunokuchaphazela umntu omnye kuphela kubantu abangama-200 ukuya kuma-500 kuluntu ngokubanzi.
- Uhlobo oluqatha: Olu lolona hlobo lunqabileyo. Luchaphazela malunga nomntu omnye kubantu abangama-500,000 ukuya kuma-750,000. Nangona kunjalo, inani lokwenyani linokuba phezulu kakhulu kunolu qikelelo.
Le meko ichaphazela amadoda nabafazi ngokulinganayo.
Zithini iimpawu zoku?
Iimpawu zesi sifo ziyahluka ngokuxhomekeke ekubeni sincinci okanye sinzima. Makhe sijonge le theyibhile ingezantsi ukuze sikuqonde kakuhle oku.
| Uhlobo lwesifo | Iimpawu ezinokubonwa |
|---|---|
| Imo ethambileyo |
|
| Ifomu enzima |
Zithini izizathu zokunqongophala kweProteni C?
Isizathu esiphambili sale meko yimpembelelo yemfuza, oko kuthetha ukuba yinto edluliselwa kwizizukulwana ngezizukulwana.
Izizathu Ezizuzwe Njengelifa
Kukho ijini emzimbeni wethu esiyalela ukuba senze iproteni ebizwa ngokuba yiProtein C. Ibizwa ngokuba yi-PROC gene . Ukuba kukho utshintsho kule jini, iProtein C isenokungaveliswa kakuhle, okanye nokuba iveliswa, isenokungasebenzi kakuhle.
- Ukunqongophala kohlobo I: Oku kubangelwa kukwehla kwamanqanaba eProtheni C.
- Ukunqongophala kohlobo lwesibini: Oku kwenzeka xa amanqanaba eProteni C eqhelekile, kodwa iproteni ingasebenzi kakuhle.
Khawucinge ukuba omnye wabazali une-gene ye-PROC eguquliweyo. Emva koko kukho ithuba elingama-50% lokuba umntwana abanaye uza kuzuza elo gene liguquliweyo. Ukuba oko kuyenzeka, umntwana uza kuba nohlobo oluncinci lwesi sifo.
Ngoku khawucinge ukuba bobabini abazali banale jini iguqukileyo. Kukho amathuba angama-25% okuba umntwana aza kuzuza zombini ezo jini ziguqukileyo. Loo mntwana uza kuba nemeko enzima ebonisa iimpawu ekuzalweni.
Izizathu Ezifunyenweyo
Oku akusoloko kubangelwa lufuzo. Ezinye iimeko zonyango okanye ezinye izizathu zinokubangela amanqanaba aphantsi eProteni C.
- Ukunqongophala kweVitamin K
- Ukusebenzisa iWarfarin enciphisa igazi (siza kuthetha ngale nto kamva)
- Isifo sesibindi esibi kakhulu
- Imeko ebizwa ngokuba yiDisseminated Intravascular Coagulation (DIC)
- Usulelo olukhulu lwebhaktheriya (iSepsis)
Indlela yokuxilonga esi sifo?
Ukuba ugqirha wakho ukrokrela ukuba unesi sifo, uza kuvavanya izinto ezininzi ukuqinisekisa ukuba sikhona.
- Imbali yakho yezonyango: Nokuba ukhe waba neengxaki zokujiya kwegazi ngaphambili.
- Imbali yezonyango yosapho lwakho: Ngaba kukho umntu kusapho lwakho oneengxaki zokujiya kwegazi ezinje?
- Uvavanyo lwegazi: Olu lolona lubalulekileyo. Lulinganisa umsebenzi wakho weProtein C kunye nenqanaba leProtein C egazini lakho.
- Uvavanyo lwemfuza: Uvavanyo lwemfuza lunokwenziwa ukujonga utshintsho kwimfuza ye-PROC. Nangona kunjalo, oku akuyomfuneko ukuqinisekisa isifo.
Iphathwa njani?
Unyango luxhomekeke kuhlobo lokungabikho kweProteni C olunayo kunye nobunzima beempawu zakho.
Unyango lwefomu elithambileyo
- Uninzi lwabantu aludingi nyango: Uninzi lwabantu aludingi nyango. Nangona kunjalo, ingqwalasela ekhethekileyo mhlawumbi nonyango lunokufuneka ngexesha lotyando, ngexesha lokukhulelwa, ukuba benze ingozi enkulu (umz., ingozi yemoto), okanye ngamaxesha okungasebenzi komzimba (umz., ukuba balele ebhedini).
- Ii-Anticoagulants: Ukuba sele ukhe waba ne-thrombosis, ugqirha wakho uza kukunika ii-anticoagulants.
Kubaluleke kakhulu: Ukuba usela iyeza elifana neWarfarin, kufuneka ulinike inaliti ebizwa ngokuba yiHeparin ngaphambi kokuba ulisebenzise. Ukuba awusebenzisi, ngamanye amaxesha linokubangela iingxaki ezinkulu ezifana nokuqhekeka kwegazi eluswini nakwezinye iindawo. Kodwa kukho amayeza amatsha angawadingiyo. Nokuba yeyiphi na iyeza olisebenzisayo, ugqirha wakho uya kuhlala elijonga. Khumbula, ungayeki ukuthatha naliphi na iyeza ugqirha wakho akunike lona. Ukuba uneengxaki, thetha nogqirha wakho. Ukuba uneengxaki, ezifana nokopha kakhulu, fowunela ugqirha wakho ngoko nangoko.Yiya kwiYunithi yoNyango oluNgxamisekileyo (i-ETU).
Unyango lwefomu elinzima
Kule meko inzima kwiintsana ezisandul’ ukuzalwa, unyango olukhethekileyo olubizwa ngokuba yiProtein C concentrate (Ceprotin®) okanye iplasma eqandisiweyo entsha iyanikwa.
Zeziphi iingxaki ezinokubangela oku?
Kukho iingxaki ezinkulu ezininzi ezinokuvela ngenxa yale meko.
- Iingxaki zesikhumba ezinxulumene neWarfarin: Emva kokuqala iWarfarin, abanye abantu banokuba nezilonda ezibuhlungu, ezibomvu okanye ezimfusa. Ezi zixhaphake kakhulu emzimbeni ongaphezulu, ezingalweni okanye emilenzeni. Ukuba azinyangwa kakuhle, oku kunokukhokelela ekufeni kolusu kunye nezicubu ezingaphantsi.
- I-Pulmonary Embolism: Ihlwili legazi elenzeka emilenzeni (i-deep vein thrombus) linokuphuma, lihambe ngegazi, lize lihlale emthanjeni osemaphashini. Le yimeko esongela ubomi.
- I-Purpura Fulminans: Ingxaki yokujiya kwegazi emzimbeni wosana olusandula ukuzalwa. Ukuba ayinyangwa, ingabulala.
- Iingxaki zonyango olutsha lwe-plasma: Xa abantwana benikwa olu nyango rhoqo, iingxaki zinokubakho ngenxa yokwanda kwamanqanaba olwelo emzimbeni (ubuninzi bolwelo).
Yintoni onokuyilindela emva kokuba ufunyaniswe ukuba unesifo?
Isiphumo sale nto siyahluka kakhulu ngokuxhomekeke kubukhulu besifo.
Ingqikelelo yeentsana ezineProteni C eyoyikekayo ayilunganga. Zisenokufa kungekudala emva kokuzalwa. Akukho lwazi lwaneleyo okwangoku lokuchaza ukuba yintoni ukusinda ixesha elide kwabo banesi sifo sinzima.
Ukuba unesimo esincinci, usengozini yokuphinda-phinda i-venous thromboembolism. Kukwakho nomngcipheko wokuba amahlwili aya kuhamba aye emiphungeni. Ukuze ufumane iziphumo ezilungileyo, kubalulekile ukugcina amadinga okulandelana nogqirha wakho. Oku kuya kumvumela ukuba ajonge imeko yakho ngokusondeleyo aze alungise unyango lwakho njengoko kufuneka.
Ngaba oku kungathintelwa?
Ekubeni ukunqongophala kweProteni C kubangelwa lufuzo, akunakuthintelwa. Nangona kunjalo, abazali nabantwana banokuqhagamshelana nengcali ye -hematologist ukuze bafunde ngakumbi ngayo baze benze uvavanyo olufunekayo.
Kwakhona, ukunqongophala kweProteni C okubangelwa zezinye iimeko zonyango ngamanye amaxesha kunokuthintelwa. Umzekelo,
- I-hormone ye-estrogen kwezinye iipilisi zokulawula ukuzala ezisetyenziswa ngabasetyhini inokunyusa umngcipheko wokuqhekeka kwegazi.
- Le ngozi iyanda ngakumbi ngenxa yezinto ezinje ngokutshaya, ukutyeba kakhulu, ukukhulelwa, kunye nokungazilolongi.
Kwezinye iimeko, ugqirha wakho unokukunika iidosi zokuthintela ukugabha kwegazi ukunciphisa lo mngcipheko. Ke ngoko, kubaluleke kakhulu ukuthetha ngokukhululekileyo nogqirha wakho ngezinto ezikubangela umngcipheko.
Umyalezo Wokuya Ekhaya
- Ukunqongophala kweProteni C yimeko ebangela ukujiya kwegazi okungaqhelekanga, okudla ngokuzuzwa njengelifa.
- Kukho iintlobo ezimbini zale meko, ethambileyo neqatha. Abantu abaninzi abaneengxaki ezincinci abazibonisi zimpawu.
- Ukuba uneempawu ezifana nokudumba kwemilenze kunye nentlungu, isenokuba yi-deep vein thrombosis (DVT). Musa ukuyityeshela loo nto.
- Ukuba unyango luyimfuneko, landela imiyalelo kagqirha wakho ngokuchanekileyo. Musa ukuyeka okanye ukutshintsha amayeza akho wedwa.
- Ukuba unale meko, kubaluleke kakhulu ukuba ungabandakanyeki kwizinto ezinobungozi ezifana nokutshaya nokutyeba kakhulu.
- Ukuba kukho umntu kusapho lwakho onesi sifo, bubulumko ukuba ubone ugqirha ukuze akunike ingcebiso, kwaye ukuba kuyimfuneko, wenze uvavanyo.











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