IHemophilia ezinjeni kukusilela kokunqumama okubangela ukopha kakhulu. Amanye amanyathelo okhuseleko kufuneka athathwe ukuthintela nakuphi na ukwenzakala kwinja, kunye notofelo-gazi luyimfuneko kwimeko yokopha okuphindaphindayo okanye ngexesha lotyando ukuba kukho imfuneko.
IHemophilia sisifo esizuzwe njengelifa ichaphazela uninzi lwezinja ezingamadoda, ukuba ngabasetyhini abathwali besi siphene semfuza kwaye ke ngenxa yoko ngabasasazi sesi sifo.
Iindidi
Ezinye iintlobo zithambekele ngakumbi kunabanye ukusifumana esi sifo, kwaye izinja ezichaphazelekayo kufuneka zisuswe kwinzala yazo kubukho bokuqala okanye ulwazi lwaso. Kufuneka siyazi loo nto nathi Kubekho iimeko kwiikati, nangona uninzi lwezi zixilongo lususwe.
Zimbini iintlobo zehemophilia ezinjeni, uhlobo A kunye noH B, konke kuxhomekeke kwimfuza echaphazelekayo. Ukungasebenzi gazi kwegazi ngendlela efanelekileyo kubangela ukopha, ukuba nzima ukuyinqanda. Kwenzeka into efanayo nakubantu abaphethwe yiyo, kule meko amadoda achaphazeleka kakhulu.
Yeyiphi imiqondiso ekhoyo kwi-hemophilia?
Iimpawu zeklinikhi ziyahluka kwaye azidingi nyango lwesiqhelo, ngaphandle kokonzakala okulandelwa kukuphuma kwegazi. Ngamanye amaxesha iimpawu zisenokungabikho kwaphela. Ezona fom ziqatha zenzeka besebancinci kakhulu, kwaye ezona ndlela zibukhali ze-hemophilia ebudaleni zihlala zinesifo esingalunganga.
Iimpawu zehemophilia ezinjeni zahlukene, ukuphindaphinda ukopha, ukopha phantsi kolusu, ukugruzuka Ngexesha lokutofa, ukopha ngexesha lokukhula kwamazinyo, ukopha empumlweni, ubukho begazi esitulweni okanye kumchamo, njl. Iimpawu ezinganakwa, ngakumbi xa zibandakanya amalungu angaphakathi, umzekelo indlela yokuphefumla, zibeka ubomi besilwanyana emngciphekweni.
Ukuba kurhanelwa ihemophilia, ugqirha wezilwanyana uza kusebenzisa iimvavanyo ezithile ukumisela kunye nokucacisa isifo. Kukwakho nesifo, la Isifo seVille Willebrand, eyenzeka ikakhulu kuhlobo lwe-Doberman kwaye iyafana ne-hemophilia.
Loluphi unyango olona lufanelekileyo kwi-hemophilia ezinjeni? Akukho lunyango lwe-hemophilia. Kwezinye iimeko ugqirha wezilwanyana uya kuncoma ukulawulwa kwee-coagulants okanye unyango olusekwe ku-vitamin K ukuzama ukulwa nefuthe lesi sifo.
Ngaphambi kwayo nayiphi na impawu yokugula kwinja yethu, kufuneka siye kugqirha wezilwanyana ngokukhawuleza. Oku kuya kunika uxilongo kunye nokuchonga olona nyango lubonisiweyo isilwanyana. Ngenja ye-hemophiliac, kuya kufuneka uthathe amanyathelo okhuseleko kubomi bemihla ngemihla, enyanisweni, baya kuba ngononophelo ukuze ingazenzakalisi. Ingqwalaselo kufuneka inikwe nezinye izinja okanye izilwanyana ukuze kungabikho thuba lokuba semngciphekweni wokulunywa okanye ukukrwitshwa.
Yintoni uhlobo lwe-hemophilia ezinjeni?
Uhlobo lwe-hemophilia lolona hlobo lweklasi kwizinja. Kungenxa yokusilela kwento ekunqandayo (factor VIII) kunye neziphumo zokopha okungaphezulu okanye okungaphantsi kokubalulekileyo kwisilwanyana esincinci. IHemophilia sisifo esosulelwa ngokunxulumene nesondo ngakumbi ngakumbi nge-chromosome yesini (X). Ke ngoko, ikakhulu ngamadoda aphethwe sesi sifo.
Ukongeza kwimvelaphi yelifa, ihemophilia A inokuba sisiphumo soguquko «ngokuzenzekelayo»Kwi-chromosome yesini. Kule meko, abazali abanayo i-chromosome "engaqhelekanga" kwaye ke abadlulisi sifo, nangona kunjalo, le meko inqabile. Ngenxa yobume besi sifo, kuyacetyiswa ukuba izilwanyana ezichaphazelekayo zingazivelisi.
Zithini iimpawu zekliniki ezinjeni?
Ziyakwazi ukwahluka kakhulu, kodwa ezona zixhaphakileyo kukusopha okanye ukugruzuka. Ezinye izinja ezichaphazelekayo azinakuba nazo iimpawu zeklinikhi nangaliphi na ixesha ebomini babo. Kumanani amakhulu amatyala, imiqondiso ayibonakali kwaye azidingi nyango lukhethekileyo, ngaphandle kokuba ukwenzakala kulandelwa kukopha.
Kwelinye icala, ezona fomu zinzima zenzeka besebancinci. Ukopha kunokubakho ngokuzenzekelayo kulo naliphi na ilungu, iimpawu zeklinikhi ziya kuxhomekeka ikakhulu kwindawo ekuyo. Ukuba ukopha kwenzeka kumalungu okanye izihlunu, kunokubakho isiqhwala. Banokubonakala ngexesha lokulahleka kwamazinyo obisi (Iinyanga ezi-4 ukuya kwezi-6). Igazi elitsha linokuhamba liphume emlonyeni wenjana.
Ukopha kunokubakho kuphela ngexesha lotyando (umzekelo, ukuthena, umzekelo) kwaye kunokujongeka njenge-hematoma enqamlezileyo okanye iscrotum. Ezona hemorrhages ziyingozi zezi zibandakanya amalungu angaphakathi kwaye iimpawu zeklinikhi zihlala zikhathazekile okanye zingabonakali, zibeka ubomi benja emngciphekweni kwaye zinokubangelwa kukuba iiplatelets ezisezantsi. Ukuxilongwa kwe-hemophilia A kusekelwe kwi- ukumiselwa kwento ejiyisayo ye-VIII egazini lenja. Ukugqitywa kovavanyo olupheleleyo lwe-coagulation kunokungazibandakanyi ezinye izizathu eziqhelekileyo zokopha ezinje nge-anticoagulant rodenticide poisoning.
Okwangoku alukho unyango oluthile lokulwa i-hemophilia A ezinjeni. Unyango ke ngoko ubukhulu becala luyalondolozwa. Ngamanye amaxesha, utofelo-gazi lunokuba yimfuneko kukopha okukhuluNgesi sizathu, kuyacetyiswa ukuba kwenziwe iqela legazi layo nayiphi na inja ene-hemophilia A.
Uphononongo olwenziwe e-United States ngu-David A. Wilcox ngo-2013 luchaze unyango lwezinja ezintathu ezine-hemophilia A enzima ngokufaka inaliti eguqula imo yeentsholongwane (ophethe uhlobo olungalunganga lweFactor VIII) kwiiseli zakho zegazi. Izinja ezimbini kwezintathu zikwazile ukuvelisa into eyoneleyo VIII ukunqanda ukopha iinyanga ezingama-30. Lo msebenzi unokukhokelela ekusetyenzisweni okusebenzayo ebantwini nasezizinjeni kungekudala.
Ngawaphi amanyathelo othintelo ekufuneka ewenzile?
Kwizinja ezine-hemophilia kunjalo kubalulekile ukuthintela ukopha. Inja kufuneka ihlale kwindawo apho umngcipheko wokwenzakala uncitshisiwe, ngaphandle kokudlala ngesiquphe nezinye izinja, iikati okanye abantwana. Phambi kothethathethwano (nditsho ngenaliti enye), ugqirha wezilwanyana kufuneka aziswe ngalo lonke ixesha.
Inja eyindoda ene-hemophilic isenofuzo olungalunganga kwi-X chromosome yayo. Akufuneki uphinde uveliseNgaphandle koko bonke abantwana babo baya kuthwala imfuza engalunganga (kodwa abayi kugula). Kwinja eyindoda, isakhi esineziphene sivela kunina (ngaphandle kwento enqabileyo yokutshintsha okuzenzekelayo). Ngoko ke, kuya kufuneka ukuba asuse unina ekuzaleni kunye nokulandelela yonke inzala yakhe. Kuya kubakho oonyana abagulayo kunye namantombazana athile.