Immunodeficiencies Primary. immunodeficiencies Primary: ukwelashwa

Immunodeficiency - ukuhlukumeza imisebenzi okuvikela umzimba womuntu, ngenxa buthakathaka amasosha omzimba ukuba Amagciwane bemvelo ezahlukene. Isayensi echazwe lezimo eziningana ezithile lolu hlobo. Leli qembu kuphazamiseka libhekene imvamisa anda futhi Yiqiniso Sisindvo izifo ezithathelwanayo. Eziyimbangela yezinkinga ezizuzwa amasosha omzimba kuleli cala elihlobene ne ushintsho izici qualitative noma ambalwa of izingxenye zayo ngazinye.

izakhiwo amasosha omzimba

Amasosha omzimba udlala indima ebalulekile ukusebenza evamile umzimba, njengoba kuhloswe ukuthola futhi abhubhise antigen ezingase noma kungenzeka ukungena kusukela imvelo yangaphandle (esithathelanayo) noma kube umphumela isimila ukukhula amaseli siqu (endogenous). Umsebenzi zokuzivikela ngokuyinhloko olunikezwa izici azalwa naso ezifana phagocytosis futhi uhlelo umphelelisi. Ukuze impendulo oluhambelana umzimba aphendvule atholwe izivikeli mzimba: i humoral kanye neselula. Ukuxhumana senzeka kulo lonke uhlelo izidakamizwa ezikhethekile - cytokines.

Kuye imbangela, amasosha omzimba isimo iziyaluyalu zihlukaniswe immunodeficiencies zamabanga aphansi naphezulu.

Uyini immunodeficiency eyinhloko

immunodeficiencies eyisisekelo (PID) - ukuhlukumeza okuzivikela ngenxa ukukhubazeka kwezakhi zofuzo. Ezimweni eziningi, zizuzwe futhi kwezinso azalwa nako. PID is iyona eyembula uhlobo lomuntu esemncane, kodwa ngezinye izikhathi kungukuthi kutholakala ukuthi uphethwe yini kuze kube yilapho yobusha noma eminyakeni yokungena ngisho umuntu omdala.

PID - leqembu lezifo azalwa naso, ezihlukahlukene ukubonakaliswa emtholampilo. International Ukwahlukanisa Diseases 36 kuhlanganisa uphenyo futhi ngokwanele echazwe immunodeficiency eyinhloko uthi, kodwa ngokuvumelana izincwadi zezokwelapha ezisekelwe ekwazisweni, kunezinguqulo ezingaba ngu-80 Iqiniso lokuthi akuyona yonke izakhi zofuzo isifo sezihlonziwe wemfanelo.

Kuphela ukwakheka kwezakhi ezithile zofuzo we-X chromosome libhekene kwamaphutha mzimba ezahlukene okungenani eziyisithupha, futhi ngenxa yalokho ukuvama okuvela kuyo lezi zifo kwabesilisa lingaphezulu kakhulu kwalokho amantombazane. Kukhona kucatshangwa ukuthi ukuthuthukiswa immunodeficiency azalwa naso Ungase ube nethonya elikhulu aetiological ukutheleleka intrauterine, kodwa lesi simangalo ingakamiswa ngokwesayensi kungase kutholakale.

emitholampilo isithombe

I ukubonakaliswa basemtholampilo immunodeficiencies eyinhloko Nokho zihlukahlukene njengezinkolelo bona ngokwabo isimo, kukhona esinye isici esivamile - infective hypertrophic (webhaktheriya) syndrome.

immunodeficiencies Primary, kanye yesibili, sibonisa ukuthambekela iziguli njalo relapsing (Ukuphinda) yezifo omsakazo ezithathelwanayo okungase kubangelwe Amagciwane atypical.

Lesi sifo ngokuvamile kunazo wadalula ohlelweni bronchopulmonary futhi ENT-izitho. Futhi bevame nolwelwesi lwamafinyila kanye integuments, okuyinto bangase balwisane sepsis futhi ithumba. Amagciwane Amagcikwane kubangele ukucinana kwemigudu yokuphefumula kanye sinusitis. Abantu ne ukuntula mzimba, bavame waphawula impandla ekuseni futhi eczema, futhi ukusabela ngezinye izikhathi nomzimba. iziyaluyalu njalo futhi sokuhlasela kwamasosha omzimba, futhi propensity kuya umdlavuza. ukuntula Immune ezinganeni cishe njalo kubambezela yentuthuko ngokwengqondo nangokomzimba.

Mechanism yentuthuko immunodeficiencies eyinhloko

Ukwahlukanisa izifo ngokuvumelana indlela yentuthuko yabo iyona ulwazi kakhulu kulolu cwaningo uma immunodeficiency uthi. Odokotela uhlukanise yonke imvelo mzimba lesi sifo ku 4 amaqembu ayinhloko:

- Humoral noma B-cell, okuhlanganisa Bruton Syndrome (agammaglobulinemia ukuba kuhambisane X chromosome) ukuntula Iga noma IgG, Igm ngokweqile nge Imininingwane immunoglobulin ukuntuleka kwamavithamini, ukungezwani immunodeficiency elula variable, ukuvula hypogammaglobulinemia obelusanda kuzalwa eziningi ezinye izifo ezihambisana ne amasosha omzimba humoral .

- T seli immunodeficiencies eyinhloko, ngokuvamile abizwa ngokuthi kuhlanganiswe, kusukela iziyaluyalu lokuqala ephukile njalo nokuvikeleka humoral, isb hypoplasia (Di George syndrome) noma dysplasia (T-lymphopenia) thymus.

- Immunodeficiencies kubangelwa yokulimala phagocytosis.

- Immunodeficiencies kubangelwa iziyaluyalu uhlelo umphelelisi.

ezifweni ekungenweni

Njengoba imbangela immunodeficiency kungaba ukwephulwa amayunithi ahlukahlukene
izivikeli mzimba nokuchayeka ezithwala izifo akuyona esimweni ngasinye okufanayo. Ngokwesibonelo, lapho izifo humoral isiguli okwakumenza angenwe izifo kalula ezibangwa streptococci, staphylococci, haemophilus influenzae. Ngakho alawa magciwane ngokuvamile abonise ukumelana nemithi. Lapho immunodeficiency kuhlanganiswe izinhlobo kungase kufakwe i-bacterium namagciwane, ezifana herpes noma isikhunta okuyinto ngokuyinhloko emelelwa candidiasis. ifomu Phagocytic libhekene ikakhulukazi yi staphylococci efanayo namagciwane Gram-HIV.

Ukwanda immunodeficiencies eyinhloko

Immunodeficiencies, ngofuzo, - ngempela isifo womuntu ezingavamile. Imvamisa ezifana izinkinga zesimiso sokuzivikela somzimba kumele lihlolwe mayelana isifo ngasinye ngoba nokudlanga yabo kuyahlukahluka.

Ngokwesilinganiso, eyodwa kuphela kuzo eziyizinkulungwane ezingamashumi ayisihlanu osanda kuzalwa kuzoba ahlupheke azalwa naso lezinceku immunodeficiency. Lesi sifo ezivamile kuleli qembu ukungabi Iga zikhethe. immunodeficiency azalwa naso yalolu hlobo kutholakala phakathi ungomunye wezinkulungwane kwezinsana. Futhi u-70% wabo bonke amacala Iga ukuntula kukhona ukwehluleka okuphelele kwazo le ngxenye. Ngesikhathi esifanayo, abanye isifo ngaphezulu ezingavamile womuntu yesimo somzimba sokuzivikela ezifweni, ngofuzo, kungenzeka kwasakazwa isilinganiso 1: 1,000,000.

Uma sicabanga tigameko izifo PID kuncike indlela, yakhiwa isimo ethakazelisa kakhulu. B-cell immunodeficiencies eyinhloko, noma njengoba bebizwa ngokuvamile ngokuthi, ukunikezwa kwe-omzimba, ezivame kakhulu kunezinye futhi bakha 50-60% azo zonke izimo. Ngesikhathi esifanayo, T-cell kanye ifomu phagocytic kutholakala ayi-10-30% eziguli ngamunye. Kubhekwe izifo ezingavamile kakhulu izivikeli mzimba kubangelwa yokulimala umphelelisi - 1-6%.

Kufanele kuphawulwe ukuthi imvamisa nesenzakalo idatha PID zihluke kakhulu emazweni ahlukahlukene, okuyinto kungenzeka ukuthi umataniswe nge yofuzo leqembu kazwelonke ukuze ukuguquka kwezakhi zofuzo ethize DNA.

ukuxilongwa immunodeficiency

immunodeficiency Primary ezinganeni uvame kunqunywa kungazelelwe, ngoba
yokuthi kokutshelwa ngokukhubazeka kwengane ezingeni wezingane wesifunda kunzima.

ngokuvamile Lokhu kuphumela ukubambezeleka ekuqaleni ukwelashwa kanye Izibikezelo abampofu yokwelapha. Uma udokotela esekelwe esithombeni emitholampilo ye isifo futhi imiphumela yokuhlolwa jikelele aphakanyisiwe isimo immunodeficiency, into yokuqala okumelwe ikwenze - ukuthumela ingane kudokotela-immunologist.
EYurophu lehla ngokushesha inani kukhona Association of Immunologists, elibhekene nomse- cwaningo kanye nokuthuthukisa izindlela ekwelapheni izifo ezifana, okuyinto uthela igama ESI (European Society for immunodeficiencies). Kudaliwe futhi avuselelwe izifo semininingwane PID futhi ivunyelwe algorithm inkinga ukuxilongwa ngokwanele okusheshayo.

Qala uphethwe ngokubuthana nemlandvo wetekwelashwa kwakhe. Ngokukhethekile kufanele kukhokhelwe isici zozalo, njengoba immunodeficiencies kakhulu azalwa naso kukhona sitholwa njengefa. Ngokulandelayo, ngemva behlolwa futhi bathole jikelele idatha ucwaningo lwezempilo wafaka le uphethwe ukuthethwa kwecala. Esikhathini esizayo, ukuze uqinisekise noma zingamanga udokotela hypothesis, isiguli kufanele ahlolwe obuphelele ongoti ezifana zofuzo immunologist. Kwaze kwaba yilapho sebeshiye yonke manipulations ngenhla singaxoxa mayelana ukukhiqizwa uphethwe wokugcina.

zocwaningo laboratory

Uma ngesikhathi uphethwe esolwa eyinhloko immunodeficiency syndrome, tivivinyo elandelayo laboratory ayadingeka:

- ukusungulwa ephelele segazi (Ukugcizelelwa inani lymphocyte);

- ukuzimisela serum immunoglobulin, ayisiza;

- ukubala ambalwa of B- ne-T-lymphocyte.

izifundo ezengeziwe

Ngaphezu ukuhlolwa laboratory kanye kusebenta kwahulumende, osekuvele kushiwo ngenhla, umuntu ukuhlolwa eyengeziwe bayonikezwa esimweni ngasinye. Kukhona iqembu ingozi, abadinga ukuthi zihlolelwe igciwane le-HIV, noma kwezinso zofuzo. Futhi udokotela inikeza kungenzeka ukuthi okwamanje human immunodeficiency 3 noma 4 zilwane, okwakuyothi ngaphansi kwawo kutawugcizelela isifundo eningiliziwe ngesineke phagocytosis yokuhlola ukusebenza inkomba tetrazolinovym okwesibhakabhaka futhi yokuqinisekisa ingxenye Ukwakheka kohlelo umphelelisi.

ukwelashwa PID

Kusobala ukuthi indlela yokwelapha edingekayo kuzoncika ngokuyinhloko kusukela isifo somzimba sokuzivikela ezifweni, kodwa, ngeshwa, ifomu azalwa naso kungenakususwa ngokuphelele, okungakwazi ishiwo immunodeficiency kuluthola. Kususelwa ngentuthuko zokwelapha zanamuhla, ososayensi bazama ukuthola indlela ukususa imbangela ezakhini zofuzo. Kuze kube manje, imizamo yabo ungazange yimpumelelo, singasho ukuthi ukuntula mzimba - isimo ezingelapheki. Cabangela izimiso yokwelapha isicelo.

replacement therapy

Ukwelashwa nokuvikelwa ukuntula enamandla abavamile sehliselwe replacement therapy. Njengoba kushiwo ngaphambili, umzimba wesiguli ayikwazi ukukhiqiza izingxenye ezithile amasosha omzimba, noma aphansi izinga zabo kuyadingeka. Ukwelashwa ngalesi kungaba amasosha omzimba imithi reception noma immunoglobulin, ayisiza, imikhiqizo yemvelo ayaphuka. Ngokuvamile, izidakamizwa iphathwa ngemithambo yegazi, kodwa ngezinye izikhathi kungenzeka, futhi umzila ongama, lula ukuphila esiguli, lokukhulunywa ngako kulendzaba akanayo waphinda uvakashele isikhungo sokwelapha.

esikhundleni isimiso ngokuvamile ivumela iziguli ukuhola ukuphila cishe evamile: ukufunda, basebenze badlale. Yiqiniso, esibuthaka sokuzivikela isifo uhlelo, izici humoral kanye neselula kanye nesidingo njalo ukwethulwa izidakamizwa ezibizayo ngeke sivumele isiguli uphumule ngokugcwele, kodwa namanje ungcono futhi ehlala egumbini ingcindezi.

ukwelashwa okunezimpawu kanye nokuvinjelwa

Sikhishwe yokuthi iyiphi encane esifweni enempilo womuntu webhaktheriya noma viral lweqembu ngesineke immunodeficiency eyinhloko isifo kungaba yingozi, kubalulekile ukuba bafeze zokuvimbela ngokuphumelelayo. Lapha ezihilelekile elwa namagciwane, antifungal nezidakamizwa namagciwane. Izinga Key ukukwenza ivuliwe izinyathelo, ngoba izivikeli mzimba buthaka abanakukwazi babanikeze ukwelashwa izinga.

Ngaphezu kwalokho, kufanele sikhumbule ukuthi kulezi ziguli zithambekele nomzimba, sokuhlasela kwamasosha omzimba, futhi, kuba kubi nakakhulu uma, ukuze isimila uthi. Konke lokhu ngaphandle kokuqondisa umnyuziki yezokwelapha abakwazi ukuvumela umuntu aphile ukuphila ngokugcwele.

ukufakelwa

Lapho ochwepheshe anqume ukuthi isiguli asihlali akukho abangakwenza ngaphandle kokuhlala ukuhlinzwa, engenziwa umnkantsha transplantation. Le nqubo lihlotshaniswa izingozi eziningi ze-ukuphila nempilo isiguli futhi umkhuba, ngisho uma kwenzeka lokhu kwakuyoba nomphumela yimpumelelo abanakwethenjwa ngaso sonke isikhathi ukuxazulula zonke izinkinga uhlushwa ukuphazamiseka kwemikhuba mzimba. Lapho efeza eyohlinzwa esinjalo, sonke isimiso Limiselelwe hematopoiesis umamukeli ezifana olunikezwa abanikelayo.

immunodeficiencies Primary kukhona inkinga enzima kakhulu kwezokwelapha zanamuhla, okuyinto, ngeshwa, akazange ukuxazulula ngokuphelele. Izibikezelo Kubi ngoba izifo yalolu hlobo namanje liyanqoba, futhi isibili ngeshwa, unikezwa iqiniso lokuthi ababuzwayo ikakhulukazi izingane. Noma kunjalo, izinhlobo eziningi ukuntula mzimba iyahambisana impilo egcwele uma ekuhlonzweni nasekwelashweni ukusetshenziswa ukwelashwa ezanele.