Preliminary Research On Detection Of The Relevant Antibodies Of The Hemophilia A Ⅷ Gene Injection In Zhuhai City

Hemophilia A, also called congenital or genetic VIII genedefection, is a kind of recessive genetic hemorrhagic disease related toX-chromosome due to the shortage or the functional disorder of FVIII.Its genetic feature is as follows: it is recessive genetic disease onX-chromosome, females are carriers and it comes on males with theoccurrence rate of 1/5000 to 1/10000. It has no racial difference. Thespecific occurrence rate of it is five to ten in 0.1 million people inwestern countries and 2.73 in China. Under effective substitutetreatment, the disablement rate and death rate of it is immenselyreduced. The life span of the sufferer is increased from ten years in the50s to an almost normal level. Since the 80s, with the continuousdevelopment of virus-killing techniques, the safety of substitutetreatment has been guaranteed and the ratio of the sufferers'chance ofdeveloping virosis hepatitis or AIDS has been greatly played down.During the 90s, the wide application of reformed FVIII has not onlyimproved the treatment effect, but also avoided the side effects ofappearance of antibodies and tendency of developing virosis with theuse of traditional FVIII derived from human bodies. Genetic treatmenthas achieved success in animal experiments and the preliminaryachievement in clinic experiments announces the dawn of completeeradication.In clinic, hemophilia is featured by the hemorrhage of skin,mucous membranes, parenchyma, arthrosis, muscles and visceraorgans. The degree and frequency of the hemorrhage is related to theactiveness of FVIII. At present, the main task is to further reduce thedeath rate, disablement rate and birth rate of hemophilia and improvethe life quality of the sufferers. The main treatment method at presentis to inject FVIII preparation to prevent and cure spontaneous andwound-induced hemorrhage but constant injection can result in theproduction of antibodies in sufferers. With the development of thetechnology of eliminating virus, the infection ratio caused by bloodtransfusion is considerably reduced but the antibodies resulted fromthe injection of FVIII gene is now one of the worst syndromes in thetreatment of hemophilia. According to the report of foreignresearchers, the occurrence rate of the antibodies is 2%-5%. Thesufferers with antibodies are more likely to die compared with thosewithout them and the treatment of the sufferer with antibodies is nowone of the medical conundrums and is receiving more and moreattention. Efforts are made to perform some preliminary research onproblem concerning the treatment effect diminished by the appearanceof stayers due to the constant injection of FVIII injection into HAsufferers. Because of the electropolar union of the FVIII antibodies withFVIII genes, the activeness of FVIII genes are neutralized, thuschoking back the process of condensation of blood and making itdifficult to staunch blood in clinic practice. Therefore, for thosesufferers who constantly inject FVIII preparations, if the clinic effectdoes not accord to the doses injected, the possibility of the productionof antibodies should be taken into consideration. Thus, to check thedynamic levels of FVIII antibodies and the concentration ratio FVIII:C and adjust treatment method accordingly is of great importance inclinic practice. In Shanghai, Beijing, Tianjin, the researches on HA is verysystematic and comprehensive, but the focus of these researches is thegenetic mutation and clinic research. The researches in Zhuhai Cityare restricted to cursory statistics and treatment according to thedisease. The research on the possibility of the production of antibodiesin the course of constant injection of FVIII preparations and its cliniceffect is far from enough. This research falls into the category of clinic experimentdiagnostics and the purpose of it is preliminary research on detectionof the relevant antibodies of the Hemophilia A VIII gene injection inZhuhai City, thus to provide reliable theory for the clinic observationand adjustment of therapy. The subjects of the research are 23 diagnosed hemophiliasufferers in People's Hospital of Zhuhai City, The Medical CareHospital for Women and Children in Zhuhai City, The HerbalistHospital of Guangdong Province (in Zhuhai City), The 5th HospitalAttached to Zhongshan University, The Red Flag Hospital of ZhuhaiCity, The Gongbei Hospital of Zhuhai City and the Central Station forBlood Supply in Zhuhai City and another 11 family members of them.Among the 23 sufferers, 13 of them have familial records and theother 10 are causal sufferers. In the diagnosis, the following methods are used for preliminaryexamination: the counting of blood platelets, Duke Method, QuickMethod, the duration of part of the active enzyme for bloodcondensation and the Ckp 77. The Biggs Method is used to fix thecase. The rate of VIIIR: Ag (Laurell Method), the RCF related toFVIII and the activeness of the FVIII are checked for some of thesufferers. Among the 23 sufferers, 17 are in-patients, 6 of them areoutpatients; 3 of them are under 4 years old, 2 of them are 4, one is 12,13 of them are between 20 and 30, 4 are between 30 and 40. Amongthem, the youngest is 2.6 and the oldest is 40. Among the 23 HA sufferers, 15 (65.2%) of them suffer fromhemorrhage before they were 2 and 2 of them showed unnaturalbleeding from outside wounds within 6 months, one suffered fromnosebleed because of outside wound, 3 of them bleed controllablyfrom operation or tooth-lifting, and another one suffered fromhematuria in adulthood. Most of the unnatural bleeding occurred fromoutside wounds and they are 16 (69.9%) in total and hypodermicbleeding is a close second. The 23 sufferers are treated with the method of condensation ofblood plasma, the examination of FVIII: C and the examination of...