IHemophilia

IHemophilia ibhekisa kwiqela lokuphazamiseka kwegazi apho ukujiya kwegazi kuthatha ixesha elide.

Zimbini iintlobo zehemophilia:

• IHemophilia A (hemophilia yeklasikhi, okanye into yokusilela kwe-VIII)
• IHemophilia B (isifo seKrisimesi, okanye ukusilela kwe-IX)

Xa usopha, uthotho lweempendulo zenzeka emzimbeni onceda amahlwili egazi. Le nkqubo ibizwa ngokuba yi-coagulation cascade. Ibandakanya iiproteni ezikhethekileyo ezibizwa ngokuba yi-coagulation, okanye izinto ezijiyisayo. Unokuba nethuba eliphezulu lokopha kakhulu ukuba enye okanye ezingakumbi zezi zinto zilahlekile okanye azisebenzi ngendlela ebekufanele ukuba zisebenza ngayo.

IHemophilia ibangelwa kukunqongophala kwehlwili VIII okanye IX egazini. Kwiimeko ezininzi, i-hemophilia idluliselwa kwiintsapho (ezizuzwe njengelifa). Uninzi lwexesha, lidluliselwa kubantwana abangamadoda.

Uphawu ephambili hemophilia ukopha. Amatyala amancinci anokungafunyanwa de kube kamva ebomini, emva kokopha kakhulu emva kotyando okanye ukwenzakala.

Kwiimeko ezimbi kakhulu, ukopha kwenzeka ngaphandle kwesizathu. Ukopha kwangaphakathi kunokwenzeka naphi na kwaye ukopha kumalungu kuqhelekile.

Rhoqo, i-hemophilia ifunyaniswa emva kokuba umntu enegazi elingaqhelekanga. Inokuchongwa kwakhona kuvavanyo lwegazi olwenziwe ukukhangela ingxaki, ukuba amanye amalungu osapho anayo le meko.

Olona nyango luxhaphakileyo kukutshintsha into ekunqandayo egazini ngokusebenzisa umthambo (ukufakwa ngaphakathi ngaphakathi).

Unonophelo olukhethekileyo ngexesha lotyando kufuneka luthathwe ukuba unale ngxaki yokopha. Ke, qiniseka ukuxelela ugqirha wakho ukuba unale ngxaki.

Kukwabaluleke kakhulu ukuba wabelane ngolwazi malunga nokuphazamiseka kwakho kunye nezihlobo zegazi njengoko banokuchaphazeleka.

Ukujoyina iqela lenkxaso apho amalungu abelana ngemicimbi eqhelekileyo kunokuphelisa uxinzelelo lwesifo sexesha elide (esinganyangekiyo).

Uninzi lwabantu abane-hemophilia bayakwazi ukwenza imisebenzi yesiqhelo. Kodwa abanye abantu bayopha kumalungu, anokuwunciphisa umsebenzi wabo.

Inani elincinci labantu abane-hemophilia banokufa ngenxa yokopha kakhulu.

UHemophilia A; Hemophilia Classic; Ukusilela kweFactor VIII; UHemophilia B; Isifo seKrisimesi; Ukusilela kwefactor IX; Ukuphazamiseka kwegazi - ihemophilia

• Amahlwili egazi

UCarcao M, Moorehead P, uLillicrap D.Hemophilia A kunye no-B Ku: Hoffman R, Benz EJ, Silberstein LE, et al, eds. I-Hematology: Imigaqo esisiseko kunye nokuziqhelanisa. Umhla wesi-7. IPhiladelphia, PA: Elsevier; I-2018: isahluko 135.

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.