I-hemophilia

I-hemophilia isho iqembu lezinkinga zokopha lapho ukunqamuka kwegazi kuthatha isikhathi eside.

Kunezinhlobo ezimbili ze-hemophilia:

• I-Hemophilia A (i-hemophilia yakudala, noma i-factor VIII)
• I-Hemophilia B (isifo sikaKhisimusi, noma ukushoda kwe-factor IX)

Lapho wopha, uchungechunge lwezinto ezenzekayo emzimbeni olusiza ukwakheka kwamahlule egazi. Le nqubo ibizwa ngokuthi yi-coagulation cascade. Kubandakanya amaprotheni akhethekile abizwa ngokuthi yi-coagulation, noma ama-clotting factor. Ungaba nethuba eliphakeme lokopha ngokweqile uma eyodwa noma eziningi zalezi zinto zilahlekile noma zingasebenzi ngendlela obekufanele zenze ngayo.

I-hemophilia idalwa ukungabi khona kwe-clotting factor VIII noma i-IX egazini. Ezimweni eziningi, i-hemophilia idluliselwa emindenini (ezuzwe njengefa). Isikhathi esiningi, idluliselwa ezinganeni zesilisa.

Uphawu olukhulu lwe-hemophilia ukuphuma kwegazi. Amacala amancane awatholakali kuze kube sekugcineni empilweni, ngemuva kokuphuma ngokweqile ngemuva kokuhlinzwa noma ukulimala.

Ezimweni ezimbi kakhulu, ukuphuma kwegazi kwenzeka ngaphandle kwesizathu. Ukopha kwangaphakathi kungenzeka noma kuphi futhi ukopha emalungeni kujwayelekile.

Imvamisa, i-hemophilia itholakala ngemuva kokuba umuntu enesiqephu esopha ngokweqile. Kungatholakala futhi ngokuhlolwa kwegazi okwenziwe ukuthola inkinga, uma amanye amalungu omndeni enalesi sifo.

Ukwelashwa okuvame kakhulu ukufaka endaweni into elahlekile yegazi egazini ngomthambo (ukufakwa emithanjeni ngemithambo).

Ukunakekelwa okukhethekile ngesikhathi sokuhlinzwa kufanele kuthathwe uma unale nkinga yokopha. Ngakho-ke, qiniseka ukuthi utshela udokotela wakho ohlinzayo ukuthi unalesi sifo.

Kubuye kubaluleke kakhulu ukwaba imininingwane mayelana nokuphazamiseka kwakho nezihlobo zegazi ngoba nazo zingathinteka.

Ukujoyina iqembu lokusekelwa lapho amalungu abelana ngezinkinga ezivamile kunganciphisa ukucindezeleka kwesifo sesikhathi eside (esingapheli).

Iningi labantu abane-hemophilia bayakwazi ukwenza imisebenzi ejwayelekile. Kodwa abanye abantu bopha igazi emajoyintini, okunganciphisa ukusebenza kwabo.

Inani elincane labantu abane-hemophilia bangafa ngokopha kakhulu.

I-hemophilia A; I-hemophilia yakudala; Ukuntuleka kwe-Factor VIII; I-hemophilia B; Isifo sikaKhisimusi; Ukushoda kwe-Factor IX; Ukuphazamiseka kwegazi - i-hemophilia

• Amahlule egazi

UCarcao M, Moorehead P, uLillicrap D.Hemophilia A noB B. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, eds. I-Hematology: Izimiso Eziyisisekelo Nokuzijwayeza. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2018: isahluko 135.

IHholo JE. I-hemostasis negazi coagulation. Ku: IHholo JE, kuhleliwe. Incwadi kaGuyton neHholo yePhysical Physiology. Umhlaka 13. IPhiladelphia, PA: Elsevier; 2016: isahluko 37.

I-Ragni MV. Ukuphazamiseka kokopha: ukuswela kwe-coagulation factor. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-25. IPhiladelphia, PA: Elsevier Saunders; 2016: isahluko 174.