Ukuphazamiseka Kwegazi
I-Hemophilia - ukuthambekela okuzuzwa ngabesilisa ukuphuma igazi - isifo sasendulo senziwa ngaphansi kokulawulwa eminyakeni engu-50 edlule. Imibhalo yamaJuda yekhulu lesibili AD idlulisela kubafana ababethelwe ukufa ngemva kokusoka, kanti udokotela wase-Arab Albucasis (1013-1106) naye uchaze abesilisa emndenini owodwa befa ngemuva kokulimala okuncane.
Emlandweni wamuva nje, iNdlovukazi uVictoria, indodana kaBrithani uLopold une-hemophilia, kanti amadodakazi akhe amabili, u-Alice noBeatrice, ayengabathwali begesi.
Ngabo, i-hemophilia yadluliselwa emindenini yasebukhosini eSpain naseRussia, eholele kwenye yezinsizwa ezidumile kakhulu lesi sifo, indodana kaTsar Nicholas II kuphela, u-Alexei.
Abantu abangaba ngu-1 ku-10 000 bazalwa nge-hemophilia A. Mayelana no-1 abantu abayi-50 000 bazalwa nge-hemophilia B.
Izimbangela Nezinhlobo
Nakuba lesi sifo saziwa futhi sasilotshwe ngaso, ezinsizwa ezedlule zafa nje zafa ngenxa yokuthi odokotela babengazi ukuthi yini eyabangela noma indlela yokuphatha ngayo. Ngama-1800, odokotela bacabanga ukuthi ukuphuma kwegazi kwenzeka ngenxa yokuthi imithwalo yegazi yayibuthakathaka. Ngo-1937, into eyayitholakala egazini elivamile elingenza i-hemophilic blood clot, ebizwa ngokuthi "i-globulin anti-hemophilic."
Ngo-1944, abacwaningi bathola kwesinye isimo ukuthi uma igazi livela emzimbeni ohlukene we-hemophiliacs ahlukene, bobabili bakwazi ukuvala. Akekho owayengachaza lokhu kuze kufike ngo-1952, lapho abacwaningi baseNgilandi beqaphela ukuthi kunezinhlobo ezimbili ze-hemophilia.
Bebefundela umfana oneminyaka engu-10 ene-hemophilia ogama lakhe linguStephen Christmas owayengabonakali ukuthi unesifo "esivamile". Bayibiza ngokuthi inguqulo yakhe i-hemophilia B, noma "isifo sikaKhisimusi," kanye ne-hemophilia A ejwayelekile kakhulu, noma "i-hemophilia yakudala." Isifo sikaKhisimusi sithinta kuphela abantu abangu-15-20% abane-hemophilia.
Izinto zokuzihlanganisa
Ngokutholakala kwezinhlobo ze-A no-B kwaqaphela ukuthi kumele kube nezinhlobo ezahlukene ze-globulin "anti-hemophilic" ehilelekile ekugubheni. Amagama abelwe kulezi zici ezihlukahlukene "ze-coagulation" yi-komiti yamazwe ngamazwe ngo-1962. I-Hemophilia A yikuntuleka kwe Factor VIII, kanti i-hemophilia B yikuntuleka kwe Factor IX.
Ukwelapha
Uma sekucacile ukuthi i-hemophilia ibangelwa ukungabi nalutho kwe-coagulation factor, ukushintshwa kwesici esingekho kwaba indlela yokwelapha. Ekuqaleni kuka-1950, isilwane se-plasma sasetshenziswa. Ngama-1970, i-coagulation factor concentrates eyenziwa ngamaplasma abantu ayatholakala. Ngeshwa, ososayensi manje bayazi ukuthi lezo zigxila zinezifo ezinjenge-hepatitis ne-HIV, futhi abantu abaningi abane-hemophilia bahlaselwe yizifo.
Namuhla, kunezici ze-coagulation eziphindaphindiwe (ezakhiwe ngokobuchwepheshe), eziqeda ingozi yegciwane. Izingane ezine-hemophilia zinikezwa i-coagulation factor njengendlela yokuvimbela ukuvimbela ukuphuma kwegazi okungapheli futhi ubasize baphile impilo ende, enempilo futhi esebenzayo.
Imithombo:
"Umlando Wokuphazamiseka Kwegazi." Kuyini ukuphazamiseka kokuphuza ?. 2006. I-National Hemophilia Foundation.
17 Disemba 2008
Ukugubha, i-SS, no-CM Lindley. "Imikhiqizo yokuhlanganisa kanye nokusetshenziswa kwayo." Am J Health-Syst Pharm 57 (2000): 1403-1417.