Primary immunodeficiencies. Primary immunodeficiencies: kurapwa

Immunodeficiency - kutyora runodzivirira mashandiro kwevanhu muviri, nokuda kwakaderera masoja pakupindura utachiona dzakasiyana hunhu. Science akarondedzera dzakananga ezvinhu izvi mhando. boka iri zvirwere inozivikanwa wakawedzera maitikiro uye weighting nzira zvirwere. Malfunctions kutapata kana ichi nechokuita kuchinja mune Qualitative kana quantitative maitiro ayo nomumwe zvinoriumba.

ehupfumi kutapata

The rokuzvidzivirira wakakurira yakaoma basa chakaipa nokushanda kwenhengo dzomuviri, sezvo zvichiitirwa zvikavengwa kuparadza maantigen kuti kana ingadzika kubva kwokunze kwezvakatipoteredza (chirwere) kana kuva migumisiro nebundu kukura pachavo masero (endogenous). Unodzivirira basa anonyanya inopiwa vachiberekwa zvinhu zvakadai phagocytosis uye mubatsiri hurongwa. Nokuti adaptive kwaiita nhengo Vanobvuma vakatodzidza rokuzvidzivirira: yacho humoral uye cellular. Communication kwaitika rose mamiriro chaiwo zvinhu - cytokines.

Zvichienderana chikonzero, mamiriro kutapata zvirwere zviri rakakamurwa nesekondari immunodeficiencies.

Ndechipi immunodeficiency

Primary immunodeficiencies (PID) - kutyora masoja mhinduro nokuda vaberekwe vakaremara. Kazhinji, vari nhaka uye vari vachiberekwa abnormalities. PID chinonyanya yakaratidza vachiri zera, asi dzimwe nguva havana chirwere kusvikira kuyaruka kana kunyange vanhu vakuru.

PID - boka vachiberekwa zvirwere, akasiyana-siyana yokuora kuratidzwa. International kupatsanura pakati Diseases 36 kunosanganisira kuongororwa uye zvakakwana kurondedzerwa chikuru immunodeficiency rinoti, asi vari maererano mabhuku ezvokurapa, pane anenge 80. Chokwadi chokuti haana zvirwere majini zvose zvakaiswa kuzivikanwa mhosva.

Chete majini Kuronga X chromosome kunoratidzwa dzinenge nhanhatu masoja siyana kwokusakwana, uye saka kakawanda panowanikwa zvirwere izvi munyika vanhurume kwakakwirira zvikuru kupfuura vasikana. Pane kubvuma kuti kukura vachiberekwa immunodeficiency angava aetiological kwomudzimu intrauterine chirwere, asi ichi vanoti haisati nesayenzi zvichigere nekusimba.

zvekuchipatara mufananidzo

The zvekuchipatara kuratidzwa chikuru immunodeficiencies vakaita siyana sezvo ivo pachavo mamiriro Zvisinei, pane imwe zvakafanana chinoratidza - hypertrophic infective (kwemabhakitiriya) syndrome.

Primary immunodeficiencies, uyewo yechipiri, vanoratidza katsika kakawanda relapsing varwere (dzokororo) zvirwere zvinotapuriranwa etiology ringaita kukonzerwa atypical utachiona.

chirwere ichi kakawanda vachibatwa bronchopulmonary maitiro uye pfava-nenyere. Uyewo kazhinji kutambura mucous membranes uye integuments, izvo zvinogona kuratidza sepsis uye pute. Chirwere utachiona kukonzera bronchitis uye sinusitis. Vanhu vane zvirwere kushaikwa, kazhinji akacherechedza mangwanani mhanza uye eczema, uye dzimwe nguva neutachiona. Kakawanda uye autoimmune zvinetso, uye propensity kuti malignancy. Immune Deficiency mune vana inenge nguva dzose chinokonzera kunonoka chepfungwa uye kwomuviri.

Mashandiro okukura chinonyanya immunodeficiencies

Kupatsanura zvirwere maererano mashandiro okukura kwavo ndiko idzidzise panyaya kudzidza immunodeficiency inotaura. Doctors vagovane zvose masoja hunhu chirwere mapoka makuru 4:

- Humoral kana B-sero, izvo zvinosanganisira chirwere Bruton raMwari (agammaglobulinemia kuti machira kuti X chromosome) kushaikwa IgA kana IgG, nekumwisa IgM pamwe zvachose immunoglobulin kushaikwa, nyore shanduka immunodeficiency, akatendeuka hypogammaglobulinemia achangoberekwa uye zvimwe zvirwere chokuita humoral kutapata .

- T muchitokisi chikuru immunodeficiencies, anowanzonzi zvabatanidzwa sezvo zvirwere wokutanga nguva dzose kuputswa uye humoral kutapata, semuenzaniso hypoplasia (Di George chirwere) kana dysplasia (T-lymphopenia) thymus.

- Immunodeficiencies anokonzerwa urema mu phagocytosis.

- Immunodeficiencies anokonzerwa zvinetso zviri mukwanisi hurongwa.

susceptibility kuti chirwere

Sezvo chinokonzera immunodeficiency angava kutyora siyana dzakabatana
dziviriro yezvirwere mumuviri, uye kuona kuti zvirwere yomuchipatara haisi saizvozvo nechimwe. Somuenzaniso, apo humoral zvirwere murwere asingatani Zvirwere zvinokonzerwa streptococci, staphylococci, Haemophilus influenzae. Saka tusingaoneki izvi kakawanda kuratidza kuramba nemishonga. Kana zvabatanidzwa immunodeficiency siyana chinogona kubatanidzwa utachiona nemavhairasi, zvakadai herpes kana notumwe izvo Anonyanyowanika kumiririrwa candidiasis. Phagocytic chimiro inozivikanwa kunyanya nokuda chete staphylococci uye giremu negative utachiona.

Kuwanda chikuru immunodeficiencies

Immunodeficiencies, nhaka, - chirwere zvisinganyanyi kuitika dzevanhu. The kakawanda masoja zvinetso zvakadaro anofanira kungoonekwa noruremekedzo mumwe chaiyo chirwere nekuti kuwanda kwavo dzinosiyana.

Paavhareji, mumwe chete kubva zviuru makumi mashanu achangoberekwa vachatambura vachiberekwa enhaka immunodeficiency. The inonyanya chirwere muboka iri kushaya IgA anyatsosarudza. Vachiberekwa immunodeficiency kwakadai kunowanikwa pakati rimwe zviuru vachangoberekwa. Uye 70% vose zviitiko IgA kushaikwa vari zvachose kukundikana chikamu ichi. Panguva iyoyowo, vamwe chirwere zvakawanda zvisingawanzoitiki dzevanhu pamusoro masoja hunhu, nhaka, angadzorwa akaparadzirwa nhamba 1: 1.000.000.

Kana tikafunga kuitika PID zvirwere zvichienderana mashandiro, izvozvo rinoumbwa mamiriro anonakidza zvikuru. B-sero chikuru immunodeficiencies, kana sezvo vari kupfuura anowanzonzi, kutyora antibody, anonyanya pane vamwe uye anoumba 50-60% vose nyaya. Panguva iyoyowo, T-sero uye phagocytic chimiro chirwere muna 10-30% wevarwere nerimwe. Vaiona kupfuura zvisingawanzoitiki zvirwere zvirwere zvinokonzerwa urema ari mukwanisi - 1-6%.

Zvinofanira kucherechedzwa kuti kakawanda panowanikwa mashoko PID zviri chaizvo dzakasiyana munyika dzakasiyana, izvo zvingazivikanwa chokuita nemagene kubatwa munhu National boka kusaruraude DNA kunotouraya.

kuongorora chirwere kuti immunodeficiency

Primary immunodeficiency muvana kakawanda pakutsvaga rwaikurumidza kuuya, nokuti
kuti vakadaro chirwere pamusoro yoruwa wevana pamwero kwakaoma.

Izvi kazhinji zvinoguma kunonoka kutanga kurapwa uye murombo fungidziro yokurapa. Kana chiremba kwakavakirwa kunotoda mufananidzo chirwere mukuru pakuongororwa kunoburitsa immunodeficiency mamiriro, chinhu chokutanga anofanira kuita - kutumira mwana kuna chiremba-Immunology.
MuEurope kune Association of Immunologists, iyo anobata ari kudzidza uye kukura nzira yokurapa zvirwere zvakadai, iro rine zita Venera (European Society kuti immunodeficiencies). Zvasikwa uye kuramba achiudza zvirwere Database PID uye mvumo chirwere algorithm kuti zvakakwana nokukurumidza kuongorora chirwere.

Tanga chirwere nokuwadzana nhoroondo zvokurapa. Cherechedzai inofanira kubhadharwa kune mazita zvinoumba, sezvo vakawanda vachiberekwa immunodeficiencies vari nhaka. Next, pashure kuongorora utano uye kuwana zvayo kurapwa tsvakurudzo Data akaisa rokutanga zvawanikwa. Mune ramangwana, kuti asimbise kana kuramba pfungwa chiremba, murwere unofanira kumbotambudzika kwazvo kuongorora kubudikidza unyanzvi hwakadai geneticist uye Immunology. pashure chete vose pamusoro manipulations tinogona kutaura pagadzirwe rokupedzisira nechirwere.

tsvakurudzo rabhoritari

Kana munguva nechirwere iri vakafunga chikuru immunodeficiency chirwere, zvinotevera rabhoritari miedzo zvaanofanira:

- pokugadzwa rakakwana ropa count (Kumikidzo nhamba lymphocytes);

- Kutsunga serum immunoglobulins;

- quantitative kukarukureta pamusoro B- uye T-lymphocytes.

mamwe zvidzidzo

Kuwedzera kwomurabhoritari uye chirwere miedzo, ayo kare pamusoro apa, mashoko okuwedzera nemiedzo wacho ichapiwa neimwe nyaya. Pane boka panjodzi, vanoda kuongororwa HIV chirwere, kana nemagene abnormalities. Uyewo chiremba inopa mukana kuti chipo dzevanhu immunodeficiency 3 kana 4 marudzi, pasi iyo ichange kuomerera tsanangudzo kudzidza murwere nokuda phagocytosis bvunzo waivako chakarurama tetrazolinovym rwedenga uye ongororo chinoumba Kuronga mukwanisi hurongwa.

PID kurapwa

Zviri pachena kuti zvakafanira kurapa kuchaenderana zvikurukuru kubva masoja chirwere, asi zvinosuwisa kuti vachiberekwa chimiro hazvigoni kubviswa zvachose, risingagoni akati pamusoro vawana immunodeficiency. Kwakavakirwa kuitwa kuitika, masayendisiti ari kuedza kuwana nzira kubvisa chikonzero panguva majini chechetere. Saka kure, kuedza kwavo havana kubudirira, tinogona kutaura kuti Immune Deficiency - chisingarapiki ezvinhu. Funga mitemo kuiswa mishonga.

chitsividzo kurapa

Mabatirwo Immune Deficiency kazhinji wakaderedzwa kusvika yokutsiva mishonga. Sezvambotaurwa, muviri murwere haakwanisi kugadzira zvimwe zvinoumba dziviriro yezvirwere mumuviri, kana yekudyidzana yakaderera unhu hwavo kunodiwa. Zvipatara panguva iyi yaizova marapiro mabiko antibodies kana immunoglobulins, zvakasikwa zvigadzirwa akavhuniwa. Kakawanda, zvinodhaka dzakabatwa nomutsinga, asi dzimwe nguva zvinokwanisika, uye subcutaneous nzira, kuti zviite upenyu murwere, iyo iri nyaya asina kushanyira vanachiremba nzvimbo kamwe zvakare.

kuzvitsinhanisa musimboti kazhinji kunoita varwere kutungamirira zvinenge chakaipa upenyu: kudzidza, basa uye kutamba. Chokwadi, munhu simba rokuzvidzivirira chirwere, humoral uye cellular zvinhu uye kugara kudiwa pakatanga zvinodhaka anodhura Haazobvumiri murwere kuti zvakazara dzikama asi zvichiri nani pane kugara nevezera kamuri.

Symptomatic kurapwa uye kudzivirira

Akapiwa chokwadi kuti chero diki kuti utano hwevanhu kwemabhakitiriya kana chaikonzerwa noutachiona murwere boka chikuru immunodeficiency chirwere chinogona kuuraya, zvinokosha kuita kudzivirira nounyanzvi. Pano kupinda kutamba antibacterial, antifungal uye zvinodhaka antiviral. zvazvingava Key kuita zviri pamusoro matanho, nokuti simba rokuzvidzivirira havagoni kukwikwidza kupa unhu kurapwa.

Uyezve, tinofanira kuyeuka kuti varwere izvi havatani sema, autoimmune, uye, kunyange kuipa, kuti bundu inoti. Zvose izvi pasina wenguva wezvokurapa cherechedzo haagoni kubvumira munhu kurarama upenyu huzere.

transplantation

Kana nyanzvi vanosarudza kuti murwere haagari hapana sarudzo asi kuti kuvhiyiwa, kunogona kuitwa mwongo transplantation. nzira iyi Rinobatanidzwa multiple pangozi upenyu uye utano murwere uye tsika, kunyange chiitiko rakabudirira mugumisiro havangagoni zvinetso zvose arwarewo zvirwere nyonganyonga. Paaiita kuvhiyiwa yakadaro, nyika yose iri yatsiviwa mugamuchiri hematopoiesis yakadai inopiwa wacho anyatsoronga.

Primary immunodeficiencies ndivo dambudziko zvokurapa ano, iyo, zvinosuruvarisa haana kugadzirisa zvachose chakaoma zvikuru. Murombo fungidziro zvirwere izvi mhando achiri rikunde, uye zviri nemhosva mbiri varombo, akapiwa chokwadi kuti kutambura kunyanya vana. Kunyange zvakadaro, siyana Immune Deficiency inowirirana hupenyu huzere kana vari mangwanani chirwere uye kushandisa zvakakwana mishonga.