Immunodeficiencies Hoose. immunodeficiencies Primary: daaweynta

Immunodeficiency - xadgudub ku ah hawlaha difaaca ee u shaqeeyo aadanaha, ay sabab u daciifaan jawaabta difaaca jirka si wareer ah ee dabiiciga ah oo kala duwan. Science tilmaamay tiro ka mid ah xaaladaha gaarka ah ee noocan oo kale ah. Kooxdaan xanuunada lagu gartaa kaadida oo soo badata iyo dabcan miisaan cudurrada faafa. Ayna difaac kiiskan la xiriira isbeddel ku sifooyinka tayada ama tirada qaybaha shaqsi ay.

guryaha difaac

habka difaaca jirka kaalin muhiim ah hawlaha caadiga ah ee jirka, sida waxaa loogu talagalay in lagu ogaado oo ay baabbi'iyaan cudur waxaa laga yaabaa in ama dhex gasho laga yaabaa in deegaanka dibadda (faafa) ama noqon natiijo ah oo u gaar ah unugyada koritaanka buro (qoomiyadda). Shaqada difaaca ugu horayn waxa ay bixiyaan arrimo lagu dhasho sida phagocytosis iyo nidaamka complement. Waayo, jawaabta qabsiga ee u shaqeeyo jawaab ka helay habka difaaca, oo humoral iyo gacanta. Isgaarsiinta qaadataa meel marayo nidaamka oo dhan walxaha gaar ah - cytokines.

Iyadoo ku xiran sababta, cudurada difaaca gobolka qaybsan yihiin immunodeficiencies hoose iyo kuwa sare.

Waa maxay difaaca hoose

immunodeficiencies Primary (PID) - xadgudub ku ah jawaabta difaaca jirka sababo la xiriira cilladaha hiddo. Xaaladaha intooda badan, ay ka dhaxashay iyo waa aan caadi ahayn lagu dhasho. PID waxaa ugu shaaca ka qaaday da'da hore, laakiin mararka qaarkood ay lama ogaado illaa ay qaan ama xataa qaangaarnimada.

PID - Koox ka mid ah cudurada lagu dhasho, noocyo kala duwan oo daliilka kiliinikada. Qoondaynta Caalamiga ah ee Cudurada 36 ka mid ah baaritaan iyo ku filan ku tilmaamay dalalka hoos, laakiin waxay yihiin sida ay suugaanta caafimaad, waxaa jira ah oo ku saabsan 80. Xaqiiqada ah in aan dhan hiddo cudurka loo aqoonsaday masuul ka.

Kaliya Halabuurka hiddo-ee koromosoom X ah waxa lagu gartaa ugu yaraan lix dhiman difaaca kala duwan, sidaas darteed inta jeer ee dhacdo oo ka mid ah cudurada ragga waa ka sii badan in ka mid ah gabdhaha. Waxaa jira malo ah in horumarinta difaaca cudurada wadnaha ee saamayn kara aetiological cudurka intrauterine, laakiin labo geesood ah oo aan weli la cilmi ahaan la xaqiijiyey.

sawir clinical

The daliilka caafimaad ee immunodeficiencies hoose waa sida kala duwan sida ay isu gobolka, si kastaba ha ahaatee, waxaa jira hal feature caadi ah - infective hypertrophic (bakteeriya) syndrome.

immunodeficiencies Primary, iyo sidoo kale sare, mariyo u janjeera in ay bukaannada soo noqnoqda noqoshada (bogsashada) cudurada faafa etiology oo laga yaabo inuu keenay wareer atypical.

Cudurkaan waxaa inta badan soo if-baxay nidaamka bronchopulmonary iyo ENT-xubnaha. Sidoo kale inta badan ku xanuunsado xub-axaleedka iyo integuments, taas oo muujin laga yaabaa sepsis iyo malax. wareer ah bakteeriyo keeni boronkiitada iyo hogaga. Dadka qaba yaraanta difaaca, waxaa inta badan lagu arkay goynina, hore iyo canbaar, iyo jawaab mararka qaar xasaasiyad. cudurada soo noqnoqda iyo shalalka, ka dhaxleen in kansar. deficiency Immune in caruurta waxaa had iyo jeer keena dib u dhac ah horumarka maskaxda iyo jirka.

Hab of horumarinta immunodeficiencies hoose

Qoondaynta oo ah cudurada sida ay farsamo ee ay horumarka waa ugu wargelin ah in daraasad dhacdo dalalka difaaca ah. Dhakhaatiirta kala qaybiyaan oo dhan nooca difaaca cudurka galay 4 kooxood oo waaweyn:

- Humoral ama B-cell, kaas oo ka mid ah Bruton ayaa syndrome (agammaglobulinemia in la xidhay si koromosoom X) IGA deficiency ama IgG, xad-dhaaf ah IgM la total deficiency immunoglobulin, difaaca variable fudud, jeestay dhashay hypogammaglobulinemia iyo tiro ka mid ah cudurada kale ee la xiriira difaaca humoral .

- unugyada T immunodeficiencies aasaasiga ah, inta badan lagu magacaabo marka la isku daro, tan iyo cudurada ugu horeysay had iyo jeer jebiyey iyo difaac humoral, tus hypoplasia (Di George syndrome) ama Kobac la'aanta (T-lymphopenia) thymus.

- Immunodeficiencies sababa cilladaha ee phagocytosis.

- Immunodeficiencies sababa cudurada nidaamka complement ah.

nuglaato infakshan

Tan iyo sababta difaaca waxaa laga yaabaa in xadgudub ku ah qaybaha kala duwan
habka difaaca, iyo gaadhista waxii jeermis ah ma aha isku mid kiis kasta. Tusaale ahaan, marka cudurada humoral bukaanka waa mid u nugul cudurada in waxa sababa streptococci, ✰Malax mid, Haemophilus influenzae. Sayidka sheyga, kuwaas oo inta badan muujiyaan caabiga antibiotics. Marka foomamka difaaca marka la isku daro lagu lifaaqi karaa in fayrusyada bakteeriyada, sida herpes ama fangaska kuwaas oo inta badan wakiil candidiasis. foomka Phagocytic waxa lagu gartaa inta badan by ✰Malax mid la mid ah oo bakteeriyada Gram-negative.

Badnaanta immunodeficiencies hoose

Immunodeficiencies, dhaxlay, - ilaa xad cudur dhif ah aadanaha. Inta jeer ee cudurada difaaca jirka sida waa in la qiimeeyaa iyada oo la tixgelinayo cudur kasta gaar ah maxaa yeelay, baahsanaanta kala duwan.

Celcelis ahaan, mid ka mid kaliya oo ka mid ah konton kun dhashay oggolaan doono difaaca dhaxlo ee lagu dhasho. cudurka ugu badan ee kooxdan waa la'aanta ah ee IGA la xushay. difaaca Congenital noocan ah waxaa laga helaa dhexe ee mid ka mid ah kumanaan ka mid ah dhallaanka. Iyo 70% dhammaan xaalado kasta oo deficiency IGA yihiin failure guud ee ka kooban this. Isla mar ahaantaana, qaar ka mid ah cudurka aadanaha wax dhif ah ee dabiiciga ah difaaca jirka, dhaxlay, waxaa loo qaybin karaa in saamiga 1: 1,000,000.

Haddii aan ka fiirsan dhacdooyinka cudurrada PID waxay ku xiran tahay hab ay, waxa ka kooban yahay xaalad aad u xiiso badan. B-cell immunodeficiencies hoose, ama sida inta badan loogu yeedho, xadgudub ku ah antibody ah, ka caadi kuwa kale oo ka samaysan 50-60% ee kiisaska oo dhan. Isla mar ahaantaana, T-cell iyo foomka phagocytic ogaadaa in 10-30% bukaannada kasta. Waxaa loo tixgeliyaa cudurada ugu dhif ah nidaamka difaaca jirka ay keento cillado ee dhammaystir ah - 1-6%.

Sidoo kale waa in la ogaadaa in inta jeer ee PID xogta dhacdo waa mid aad u kala duwan oo dalal kala duwan, taas oo la xidhiidha laga yaabaa in cabista hidaha ee group qaran oo Isbedelo DNA gaar ah.

ogaanshaha of difaaca

difaaca aasaasiga ah ee carruurta waxaa inta badan lagu go'aamiyaa dhicis ah, maxaa yeelay,
xaqiiqda ah sida ogaanshaha heerka dhakhtarka degmada in ay adag tahay.

Tani waxay badanaa keentaa dib u dhac ah ee bilowga ah ee daaweynta iyo saadaasha saboolka ah ee daaweynta. Haddii dhakhtarka ku saleysan sawirka caafimaad ee cudurka iyo natiijada imtixaanka guud jeediyay gobolka difaaca, waxa ugu horeeya ee waa inuu u sameeyaa - ilmaha u dhakhtar-immunologist diro.
In Europe waxaa jira Ururka Immunologists, kaas oo ka hadlaya waxbarashada iyo horumarinta hababka daaweynta cudurada sida, kaasoo magaca EDI ah (Society Yurub ee immunodeficiencies). Waxaa loo abuuray oo si joogto ah updated cudurada PID database oo ansixiyay geynta ogaanayo baaritaanka ku filan si degdeg ah.

Ku bilow cudurka by ururinta taariikhda caafimaad. fiiro gaar ah in la bixiyo, in ay dhinaca abtirsiinta, sida ugu immunodeficiencies lagu dhasho waa la iska dhaxlo. Next, ka dib markii baaritaan jireed iyo aad u hesho xog guud ee cilmi-baarista caafimaad oo ku riday cudurka hordhaca ah. In mustaqbalka, si loo xaqiijiyo ama ku doodeen dhakhtar la xiqiijiyay, bukaanka waa in baaritaan buuxa ku takhasusay sida geneticist iyo immunologist. Kaliya ka dib markii dhamaan manipulations kor ku xusan waxaan ka hadli karnaa wax soo saarka ee ogaanshaha kama dambaysta ah.

cilmi baaris shaybaar

Haddii inta lagu guda jiro cudurka looga shakisan yahay hoos-syndrome, imtixaanada soo socda shaybaarka lagama maarmaan u yihiin:

- abuuritaanka dhamaystiran count dhiigga (fahanka ku saabsan tirada limfosaytis);

- go'aanka immunoglobulins serum;

- xisaabinta taranta of B- iyo T-limfosaytis.

waxbarashada dheeraadka ah

Waxa intaa dheer in shaybaarka iyo ogaanshaha imtixaanada, kuwaas oo kor ku xusan ayaa durba la soo sheegnay, imtixaanada oo dheeraad ah shaqsi lagu qori doonaa xaalad kasta. Waxaa jira koox khatar ah, kuwaas oo u baahan in laga baaro cudurka HIV, ama aan caadi ahayn hidaha. Sidoo kale dhakhtarka bixisaa suurtagalnimada in la joogo difaaca 3 ama 4 noocyada, kaas oo ku adkeyso doonaa baadhitaan faahfaahsan bukaanka by qaab tijaabo phagocytosis tetrazolinovym tilmaame ka kooban qayb buluug iyo xaqiijinta nidaamka complement ah.

daaweynta PID

Waa wax iska cad in therapy loo baahan yahay waxay ku xirnaan doontaa ugu horrayn ka cudur difaaca, laakiin, nasiib darro, qaab ee lagu dhasho oo aan la si buuxda u suulin karin, taas oo aan la yiri karo oo ku saabsan difaaca ah helay. Iyada oo ku saleysan horumarka caafimaad ee hadda jira, saynisyahano ayaa isku dayaya in la helo hab si loo soo saaro sababta keentay heerka hidaha. Ilaa hadda, dadaalkooda aan ku guulaysan, waxaan dhihi karaa in deficiency difaaca jirka - xaalad aan la bogsiin karin. Tixgeli mabaadi'da therapy codsatay.

daaweynta bedelaada

Daaweynta deficiency difaaca caadi ahaan hoos u Daaweyn laguugu beddelo. Sida hore loo sheegay, jirka ka mid ah bukaanka oo aan awood in ay soo saaraan qaybaha qaar ka mid ah habka difaaca jirka, ama aad u hooseeyo tayada looga baahan yahay. Therapy at this noqon lahaa a antibodies dhaweynta daawo ama immunoglobulins, wax soo saarka dabiiciga ah waa jajabaan. Inta badan, daroogada waxaa intravenously maamusho, laakiin mararka qaarkood waxaa suurtagal ah, iyo wadada subcutaneous, si loo fududeeyo nolosha bukaanka, taas oo haddii ay taasi ma aha inuu mar kale soo booqdo xarunta caafimaadka.

Mabda 'bedelay inta badan u ogolaanaya in ay bukaanada nolol caadi ku dhow, si ay u bartaan, shaqada iyo ciyaarta. Dabcan, a wiiqay cudurka habka difaaca, arrimaha humoral iyo gacanta iyo baahida loo qabo in si joogto ah, waayo, soo saarida daawooyin qaali ah uma oggolaan doono in bukaan-socodka ay si buuxda u nasato, laakiin waxa ay weli ka fiican tahay ku nool qolkii cadaadis ah waa.

Calaamadaha iyo ka hortagga

Marka la eego xaqiiqada ah in wax yar infekshanka caafimaad bakteeriyo ama fayras aadanaha ee kooxda bukaanka cudurka hoos loo dhiman karo, waxaa lagama maarmaan ah si ay u fuliyaan hortagga hawlkarnimo. Halkan soo galay bakteeriyada play, antifungal iyo daawooyinka fayruska. heerka Key in la sameeyo waa on tallaabooyin ka hortag ah, sababtoo ah nidaamka difaaca aan awoodin in ay bixiyaan daaweyn tayo leh.

Intaa waxaa dheer, waa in la xusuusnaado in bukaanka, kuwaas oo u nugul xasaasiyad, shalalka, iyo, xitaa ka sii daran, in dalalka buro. All this iyada oo aan kormeer caafimaad buuxda ma ogolaan karo qofka inuu nolol buuxda.

badalida

Marka khubaro go'aan in uusan qofka bukaanka ah sii aan la doortay ma jiro, laakiin si qaliin, waxa lagu samayn karaa lafta dhuuxa. Habkani waxa uu la xidhiidhaa khatarta badan ee nolosha iyo caafimaadka ee bukaan-socodka iyo ku dhaqanka, xataa haddii ay dhacdo natiijo lagu guuleysto mar walba ma xallin karo dhammaan dhibaatooyinka ka cabanaya disorder ah difaaca jirka. In fuliyaan howlgal noocaas ah, nidaamka oo dhan waxa loo bedelay hematopoiesis qaataha sida ay bixiyaan deeq.

immunodeficiencies Primary waa dhibaatada ugu adag ee daawada casriga ah, taas oo, nasiib daro, ma aysan si buuxda u xaliyaan. saadaal Poor cudurrada noocan weli ahaaday, waana laab nasiib darro, siin xaqiiqada ah in ay inta badan ku xanuunsado carruurta. Si kastaba ha ahaatee, noocyo badan oo deficiency difaaca jaanqaadi nolol buuxda haddii ay yihiin ogaanshaha hore iyo isticmaalka daaweynta ku filan.