Ukuphazamiseka Kwegazi
I-Hemophilia - ukuthambekela njengelifa leendoda ukuphuma kwigazi - isifo sasendulo senziwa phantsi kolawulo kwiminyaka engama-50 okanye ezayo. Iimibhalo zamaYuda zephondo yesibini ye-AD zibhekisela kubafana ababethelwa ngokubulala emva kokusoka, kwaye udokotela waseArabhu u-Albucasis (1013-1106) naye uchaza amadoda kwintsapho enye emva kokumala kancinci.
Kwimbali yakutshanje, u-Queen Victoria waseBrithani unyana kaLebold u-hemophilia, kunye neentombi zakhe ezimbini, u-Alice noBeatrice, babephethe i-gene.
Ngabo, i-hemophilia yayidluliselwa kwiintsapho zasebukhosini eSpeyin naseRashiya, ekhokelela kwelinye lamadoda ahloniphekileyo anesi sifo, unyana kaTsar Nicholas II kuphela, u-Alexei.
Malunga ne-1 abantu abayi-10 000 bazalwa nge-hemophilia A. malunga ne-1 kwi-50,000 abantu bazalwa nge-hemophilia B.
Izizathu kunye neNdidi
Nangona eso sifo saziwa kwaye sabhalwa malunga, kwindoda esele idlulayo yafa yile nto ngenxa yokuba oogqirha abazange bazi ukuba kubangelwa yintoni okanye indlela yokuyiphatha ngayo. Kwiminyaka ye-1800, oogqirha bacinga ukuba ukuphuma kwegazi kwenzeka ngenxa yokuba imithwalo yegazi yayibuthakathaka. Ngowe-1937, into efunyenwe kwigazi eliqhelekileyo elenza igazi le-hemophilic clot, elibizwa ngokuba yi-"anti-hemophilic globulin."
Ngomnyaka we-1944, abaphandi bafumana kwimeko enye ukuba xa igazi livela kwimimandla emibili ehlukeneyo ye-hemophiliacs, badibanisa. Akukho mntu unokuchazela oku kude kube ngowe-1952, xa abaphandi baseNgilani baqaphela ukuba kukho iindidi ezimbini ze-hemophilia.
Baye bafunda inkwenkwe eneminyaka eyi-10 ubudala ne-hemophilia egama linguStephen uKrisimesi owayengabonakali " Bambiza i-hemophilia B, okanye "isifo sikaKrismesi," kunye ne-hemophilia A ephakamileyo, okanye "i-hemophilia yeklasi." Isifo seKrismesi sichaphazela kuphela abantu abangama-15-20% abane-hemophilia.
Izinto zeCoagulation
Ukufumanisa iintlobo ze-A ne-B zeza kuqonda ukuba kukho iintlobo ezahlukeneyo ze-globulin "ezichasene ne-hemophilic" ezibandakanyeka kwinkqubo yokucima. Amagama abelwe kule "miba ye-coagulation" eyahlukeneyo yikomiti yamazwe ngamazwe ngowe-1962. I-Hemophilia A yintsilelo ye-Factor VIII, kwaye i-hemophilia B yintsilelo ye-Factor IX.
Unyango
Emva kokuba kwacaca ukuba i-hemophilia yabangelwa ukungabikho kwe-coagulation factor, ukutshintshwa kwezinto ezingekhoyo kwaba yindlela yokonyango. Kwiminyaka yokuqala yee-1950, i-plasma yezilwanyana yayisetyenziswa. Ngama-1970, i-coagulation factor concentrates made from plasma yabantu yayifumaneka. Ngelishwa, izazinzulu ngoku ziyazi ukuba abo bagxila baphatha ii-virus ezifana ne-hepatitis ne-HIV, kwaye abantu abaninzi abane-hemophilia bahlaselwe zizifo.
Namhlanje, iinguqu ze-coagulation zenziwe ngokutsha (ezenziwe ngezofuzo), eziphelisa ingozi yeentsholongwane. Abantwana abane-hemophilia banikezwa i-coagulation factor njengonyango lokuthintela ukunciphisa ukuphaphazeka okungapheliyo kunye nokubanceda baphile ubomi obude, obunempilo kunye obusebenzayo.
Imithombo:
"Imbali yokuphazamiseka kwemizimba." Ziyintoni ukuphazamiseka kokungcoliswa? 2006. ISiseko seHemophilia yesizwe.
17 Disemba 2008
I-Shord, i-SS, kunye no-CM Lindley. "Iimveliso zoqhagamshelwano kunye nokusetyenziswa kwazo." Ngaba i-J Health-Syst Pharm 57 (2000): 1403-1417.