the case of randy johnson a near death from exotic ：兰迪约翰逊从异国情调的临近死亡的案例

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1、L.V.KutsClinicalImmunology:PrimaryImmunodeficiencyStudyGuideMinistryofEducationandScienceofUkraineMinistryofHealthofUkraineSumyStateUniversityL. V.KutsClinicalImmunology:PrimaryImmunodeficiencyStudyGuideRecommendedbytheAcademicCouncilofSumyStateUniversitySumySumyStateUniversity2014

2、УДК616-092:612.017.1(075.8)ББК52.523.25К95Reviewers:V. A. Bocharov–DoctorofMedicine,ProfessorofOdessaMedicineandPharmacologyInstitute;A. D. Diudiun–DoctorofMedicine,ProfessorofDnipropetrovskMedicalAcademy,MinistryofHealthofUkraineRecommendedforpublicationbytheAcademicCouncilofSumyS

3、tateUniversity(theMinutes№4of21.11.2013)К95KutsL.V.ClinicalImmunology:PrimaryImmunodeficiency:studyguide /L.V.Kuts.–Sumy:SumyStateUniversity,2014.–165 p.ISBN978-966-657-511-4Inthestudyguidetheauthorexplainsthemainfeaturesofpathogenesisandclinicalpictureofthemostcommonimmunodeficien

4、cydisorders,givesclinicalexamples.Унавчальномупосібникуподаніголовніланкипатогенезутаклінікинайчастішихімунодефіцитівінаведеніклінічніприклади.УДК616-092:612.017.1(075.8)ББК52.523.25ISBN978-966-657-511-4ÓKutsL.V.,2014ÓSumyStateUniversity,2014Primaryimmunodeficiency.X-linkedagammagl

5、obulinemiaBackgroundX-linkedagammaglobulinemia(XLA),orBrutonagammaglobulinemia,isaninheritedimmunodeficiencydiseasecausedbymutationsinthegenecodingforBrutontyrosinekinase(BTK).ThediseasewasfirstelucidatedbyBrutonin1952,forwhomthegeneisnamed.TheBTKiscriticaltothematurationofpre–Bcel

6、lstodifferentiatingmatureBcells.ThegenedefecthasbeenmappedtothelongarmoftheXchromosomeatbandXq21.3toXq22,spanning37.5kbwith19exonsforming659aminoacidstocompletetheBTKcytosolictyrosinekinase.AdatabaseofBTKmutationslists544mutationentriesfrom471unrelatedfamiliesshowing341uniquemolecu

7、larevents.Noneofthemutationaccountsformorethan3%ofmutationsinpatients.Inadditiontomutations,anumberofvariantsorpolymorphismshavebeenfound.Seetheimagebelow.EarlystagesofB-celldifferentiationcanbeidentifiedbythestatusoftheimmunoglobulingenesandbythecellsurfacemarkersCD34,CD19,andsurf

8、aceimmunoglobulin(sIg).Fro