Ii-immunodefaces are primary. Iimpawu zonyango ezingundoqo: unyango

I-immunodeficiency iphulaphula imisebenzi ekhuselekileyo yomzimba womntu, ngenxa yokunyanzeliswa kwempendulo yokuzivikela komzimba kwiimpawu zendalo. Uluhlu olubanzi lwezinto zolu hlobo luchazwe yenzululwazi. Eli qela lezifo libonakaliswe ukwanda kunye nokulinganisa kwekhosi yezifo ezithathelwanayo. Ukungaphumeleli kumsebenzi wokukhuseleka kule meko kudibene nokutshintshwa kwiimpawu zobungakanani okanye zobunjani bomntu ngamnye.

Iimpawu zokhuseleko

Amasosha omzimba athatha indima ebalulekileyo ekusebenzeni ngokusemthethweni komzimba, njengoko yenzelwe ukufumanisa nokutshabalalisa ama-antigen angena phakathi kokusingqongileyo (okusuleleka) kwaye abe ngumphumo wokukhula kwezondo zamaseli abo (ekugqibeleni). Umsebenzi wokukhusela ngokuyinhloko unikezwa ngemibandela yokuzalwa, njenge-phagocytosis kunye nenkqubo yokuxhasa. Ukuphendula okuguquguqukayo kwendalo, umzimba wokufumana umzimba unoxanduva : ukuhlaziywa kunye nezixhobo zamaselula. Ukuqhagamsheliswa kwenkqubo yonke kwenzeka ngezinto ezizodwa - ii-cytokines.

Ngokuxhomekeka kweso sizathu, ukuphazamiseka kwe-immunodeficiency isahlukaniswe ibe yi-immunodeficiency primary and secondary.

Yintoni eyona nto i-immunodeficiency

Iimpawu zokuzikhusela eziphambili (FID) ziphulwa ngokuphendula komzimba ngokubangelwa yimpembelelo yemfuza. Kwiimeko ezininzi, bazuze ilifa kwaye banesifo sokubeleka. I-PID isoloko ifunyenwe ngokusesikweni, kodwa ngamanye amaxesha ayifumaneki phambi kokukhula okanye nokuba ngumdala.

I-PID - iqela leentsholongwane ezizalwe ngumzimba, ezahlukileyo kwizibonakaliso zeklinikhi. Ulwahlulo lwezizwe ngezizwe luquka u-36 oluchazwe kwaye lufunyenwe ngokufanelekileyo ukufundiswa kwe-immunodeficiency primary, kodwa ngokutsho kweencwadi zonyango kukho malunga nama-80. Inyaniso kukuba akuzona zonke izifo eziye zachongwa njengezifo zentsholongwane.

Ukuphela kohlobo lwemizimba ye-X ye-chromosome lubonakala ubuncinane ubuncinane be-immunodeographical, kwaye ngoko iziganeko zezi zifo kumakhwenkwe ngumyalelo wokuphakama ophezulu kunezo amantombazana. Kukho isiphakamiso sokuba ukusuleleka kwe-intrauterine kunokuba nempembelelo ye-etiological ekuphuhlisweni kwe-immunodeficiency of birth, kodwa le nkcazo ayifumanekanga isiqinisekiso senzululwazi.

Umfanekiso wekliniki

Ukubonakaliswa kweklinikhi yezixhobo zokuzikhusela eziphambili zifana neemeko ngokwazo, kodwa kukho uphawu olufanayo - i-hypertrophic infectious (bacterial) syndrome.

Iimpawu zokuzikhusela eziphambili, kunye nezesekondari, zibonakaliswa zizigulane zezigulane eziphindaphindiweyo (eziphindaphindiweyo) zezifo ezithathelwanayo, ezinokubangelwa zizifo ezinama-atypical.

Ezi zifo zisoloko zichaphazeleka yenkqubo ye-bronchopulmonary kunye namaziko e-ENT yabantu. Iimbumba ezinamaqanda kunye nesikhumba zihlala zichaphazelekayo, ezingabonakaliswa ngama-abscesses and sepsis. Ama-bacterium pathogens abangela i-bronchitis ne-sinusitis. Abantu abane-immunodeficiency bavame ukufumana ukukhawuleza kwangaphambili kunye ne-eczema, kwaye ngezinye izihlandlo. Ngokuqhelekileyo izifo ezizimelayo kunye nokuthambekela kwezidalwa ezinobungozi obubi. Ukunyuka kwama-immunodeficiency kubantwana becala kubangela ukulibaziseka ekuphuculweni kwengqondo nangokwenyama.

Inkqubo yokuphuhliswa kwee-immunodeographical primary

Ukwahlulelwa kwezifo ngendlela yokuphuhliswa kwazo yinkcazelo eninzi kunokwimeko yokufunda i-immunodeficiency states. Oogqirha bahlula zonke izifo zentsholongwane yomzimba kumagumbi amane aphezulu:

- I-Humoral okanye i-B-cell, equka i-Bruton's syndrome (i-agammaglobulinemia ekuxhaseni kwi-X chromosome), ukulahleka kwe-IgA okanye i-IgG, ubuninzi be-IgM ubuninzi be-immunoglobulin, i-immunodeficiency elula, i-hypogammaglobulinemia yeintsana kunye nezinye izifo ezichaphazelekayo .

- I-cell-immunodeographical T-cell, ezibizwa ngokuba zi-immunodeographical units, ngenxa yokuzikhusela komzimba, njenge-hypoplasia (i-Di George's syndrome) okanye i-dysplasia (T-lymphopenia) ye-thymus, ihlala iphazamiseka kwiintlungu zokuqala.

Iimpawu ezingabonakaliyo ezibangelwa ziziphoso kwi-phagocytosis.

- Iimpawu zokungabikho kwemisebenzi ngenxa yokusebenza kakubi kwenkqubo yokucedisa.

Ukuboniswa kwezifo

Ekubeni imbangela ye-immunodeficiency ingaba ukuphulwa kweekhonkco ezahlukeneyo
I-immune system, ngoko ukukhutshwa kwamagciwane okusuleleka kwiintsholongwane akuyi kufanayo kwiimeko ezithile. Ngoko, umzekelo, ngezifo zesigulane isigulane sithintekile kwiintsholongwane, ezibangelwa yi-streptococci, staphylococci, intonga ye-hemophilic. Ngexesha elifanayo, ezi zixhobo ezincinci zihlala zibonisa ukuchasana neziyobisi ezinxamnye ne-antibacterial. Kwiifom ezidibeneyo zokuzikhusela komzimba, iintsholongwane ezifana ne-herpes okanye i-fungi, ezixhomekeke kakhulu kwi-candidiasis, zinokuqhotyoshelwe kwiibhaktheriya. Ifom ye-phagocytic ibonakala ngokukodwa ngokufanayo kunye ne-staphylococci kunye ne-gram-negative bacteria.

Ubuninzi beempawu zokuzikhusela ezingundoqo

Izifo ezingabonakaliyo, njengefa, zikhona izifo zabantu ezingaqhelekanga. Ubunzima bokuvela kweengxaki zokuzikhusela kolu hlobo kufuneka zihlolwe ngokuphathelele kwisifo ngasinye, ekubeni ukusabalalisa kwabo akufani.

Ngokomyinge, kuphela umntwana osanda kuzalwa kumawaka angamashumi amahlanu uya kuba neengxaki zokufumana i-immunodeficiency. Isifo esiqhelekileyo kweli qela kukunyulwa kwe-IgA. Ukukhulelwa komzimba okwenziwe ngunobungozi kulolu hlobo lwenzeka ngokuqhelekileyo kwenye yewaka lamawaka. Ngaphezu koko, i-70% yazo zonke iziganeko zokulahleka kwe-IgA zihambelana nokungafezeki ngokupheleleyo kweli candelo. Ngelo xesha, ezinye zezifo ezingabonakaliyo zezifo zomzimba, ezizuzwe njengefa, zingahanjiswa kwi-1: 1,000,000.

Ukuba sicinga ukuphindaphindiweyo kwezifo ze-PID ngokuxhomekeka kwindlela, ke umfanekiso othakazelisayo ukhiwa. I-cell-immunodeographical B-cell, okanye, njengoko zibizwa ngokuba zikhona, ukungaqhelekanga kokuveliswa kwe-antibody, zenzeke ngokuphindaphindiweyo kunabanye kwaye i-akhawunti ibe yi-50-60% yazo zonke iimeko. Ngexesha elifanayo, iifom zeT-cell kunye neepgogocytic ziyafumaneka kwi-10-30% yezigulane nganye. Eyona nto inqabileyo izifo zamasosha omzimba obangelwa ziziphoso zokuxhasa - 1-6%.

Kufuneka kwakhona kuqaphele ukuba idatha kwixesha eliqhelekileyo le-PID lihluke kakhulu kumazwe ahlukeneyo, anokuthi ihambelane ne-genetic predisposition yeqela elithile lesizwe kwiinguqu ezithile ze-DNA.

Ukuxilongwa kwe-immunodeographical

I-immunodeficiency primary school in abantwana idlalwa ngokungaqhelekanga, ngokunxulumene nayo
Ngoko ukuba ukuxilongwa okunjalo kwinqanaba lezithili zesithili sempilo kunzima.

Oku, njengomthetho, kukhokelela ekuqalekeni kwonyango kunye nokugqithiswa kakubi kweyeza. Ukuba, ngenxa yesithombeni seklinikhi yesifo kunye neziphumo zohlalutyo jikelele, ugqirha uye wafumana isimo somzimba wokuzibulala, into yokuqala ayifanele ukuyenza kukuba athumele umntwana ekubonisaneni nomzimba we-immunologist.
EYurophu, kukho uMbutho we-Immunologists, ojongene nophando kunye nophuhliso lweendlela zokunyanga kwezi zifo, ezibizwa ngokuthi i-ESI (i-European Society ye-Immunodeficiency). Bakha kwaye bahlaziywa rhoqo kwiziko lezifo ze-PID kwaye bavumile i-algorithm yokuxilonga ngokuxilongwa ngokukhawuleza.

Baqala ukuxilonga kwiqoqo le-anamnesis yesifo. Ingqwalasela ekhethekileyo kufuneka ihlawulwe kwi-genealogical aspect, kuba iinzi-immunitalficience zezenzo zizalwa zifa. Emva kokuvavanywa ngokomzimba kunye nokufumana iinkcukacha eziqhelekileyo zeklinikhi, ukuxilongwa kwangaphambili kwenziwa. Kwixesha elizayo, ukwenzela ukuqinisekisa okanye ukuphikisa ukugqithwa kwegqirha, isigulane kufuneka sifunde uvavanyo olunzulu kwiingcali ezifana ne-geneticist kunye ne-immunologist. Emva kokuba senze zonke ezi zikhankanywe ngasentla, sinokuthetha malunga nokuqulunqwa kwesifo sokugqibela.

Uphando lweLebhu

Ukuba ngethuba lokuxilongwa kukho ukukrokrelwa kwe-syndrome ye-immunodeficiency primary, kuyimfuneko ukuqhuba ezi zilandelayo mvavanyo:

- Ukusekwa kwe-formula egazini yegazi (ingqalelo ekhethekileyo ihlawulwa kwinani le-lymphocytes);

- ukuzimisela komxholo we-immunoglobulins kwi-serum yegazi;

Ukubalwa kwamanani e-B- ne-T-lymphocytes.

Uphando olongezelelweyo

Ukongeza kwiimvavanyo zelabhu-zonyango, ezikhankanywe ngasentla, iimvavanyo ngabanye ziya kunikwa kwiimeko ezithile. Kukho amaqela omngcipheko afuna ukufumana uvavanyo lokusuleleka kwe-HIV okanye ukungaqhelekanga kwemfuyo. Kwakhona, ugqirha unikezela ukuba kukho ukuhlaselwa komzimba kumntu we-3 okanye we-4, oza kugxininisa ekufundweni ngokubanzi kwe-phagocytosis yesigulane ngokubeka uvavanyo kunye ne-tetrazolin eluhlaza okwesibhakabhaka kwaye ukhangele indlela yokwakheka kwenkqubo yenkxaso.

Unyango lwePID

Kucacile ukuba, unyango olufunekayo luya kuxhomekeka kokuqala kwi-immune system ngokwayo, kodwa, ngelanga, ifom yokuzalwa ayinakupheliswa ngokupheleleyo, engenakutsholwa malunga nokufumana imithwalo-mzimba. Ngokusekelwe kwiinkqubela zonyango zanamhlanje, izazinzulu zizama ukufumana ithuba lokuphelisa imbangela yesigaba somzimba. Nangona iinzame zabo zingakhange zithweswe ngempumelelo, singatsho ukuba ukukhulelwa komzimba kungenakuphilisa. Cinga ngemigaqo yonyango.

Ulwaphulo lwendawo

Ukunyanga kwe-immunodeficiency ngokuvamile kunciphisa ukutshintshwa kweyeza. Njengoko sele kukhankanywe ngaphambili, umzimba wesigulane awukwazi ukuzimela ngokuzimeleyo izixhobo ezithile ze-immune system, okanye umgangatho wabo ungaphantsi kunyanzelekile. Unyango kulolu hlobo luya kufakwa kwi-medicinal reception ye-antibodies okanye i-immunoglobulins, ezo iimveliso zendalo ziphazamiseka. Uninzi lwamaxesha amaninzi, iziyobisi zilawulwa ngokugqithiseleyo, kodwa ngamanye amaxesha indlela enqamlekileyo inokukwazi ukuququzelela umphefumlo wesigulane, ngubani na apha akudingeki ukuba abuyele kwisibonelelo sonyango kwakhona.

Umgaqo wokufaka indawo uvame ukuvumela izigulane zikhokele ngendlela eqhelekileyo yobomi: ukufunda, ukusebenza nokuphumla. Ngokuqinisekileyo, ukukhuselwa komzimba kubuthathaka ngenxa yesifo, iimpawu zesintu kunye neziselula kunye nesidingo esiqhubekayo seziyobisi ezibizayo asiyi kuvumela isiguli ukuba siphumelele, kodwa oku kusengcono kunokuphila kwinkampu yecindezelo.

Inkqubo yokunyanga nokukhusela

Ukuqwalasela ukuba nayiphi na intsholongwane yegciwane okanye intsholongwane engabalulekanga kumntu onempilo kwisigulane esinegciwane eliphambili le-immunodeficiency group, ingaba yingozi, ukuthintela kufuneka kwenziwe ngokufanelekileyo. Amachiza e-Antibacterial, antifungal kunye ne-anti-anti-viral asebenza apha. Ukubheja okubalulekileyo kufuneka kwenziwe ngokuthe ngqo kumanyathelo okukhusela, ngenxa yokuba unomzimba obuthathaka awuvumelekanga ukuba unikeze unyango lomgangatho.

Ukongezelela, kufuneka kukhunjulwe ukuba izigulane ezinjalo zixhomekeke ekubeni zihlaselwe, zizenzeke kwaye zibi nakakhulu, zibhekiselele kwi-tumor. Konke oku ngaphandle kokulawulwa kwezonyango ngokupheleleyo kungavumelekanga umntu ukuba aphile ngendlela epheleleyo.

Ukutshintshwa kwezityalo

Xa iingcali zigqiba ukuba isiguli asikho enye indlela yokuphuma, kodwa njengongenelelo lokusebenza, ukutsalwa kwethambo lomongo kungenziwa. Le nkqubo inxulumene nemingcipheko emininzi kwimpilo kunye nempilo yesigulane kunye nokusebenza, nangona kwenzeka isigqibo esiphumeleleyo, akunakuze isombulule zonke iingxaki zesigulane esineengxaki zomzimba. Ekufezeni olo hlobo, inkqubo yohlobo lwe-hematopoiesis yomncedisi ithatyathwa yinkqubo efanayo enikezelwa ngumnikeli.

Iimpawu zokuzikhusela eziphambili ziyiyona nkathazo yinkxalabo yezolimo zanamhlanje, okhe, ngelishwa, ayilungiswanga ngokupheleleyo. Ukugqithiswa kakubi kwezifo zolu hlobo kusekhona kakhulu, kwaye oku kubuhlungu kakhulu, kunikwa ukuba baninzi abachaphazelekayo ngabantwana. Kodwa ke, ezininzi iindlela zokuzikhusela i-immunodeficiency ziyahambelana nobomi obuzeleyo xa zifumaneke ngexesha elifanelekileyo kwaye zisebenzise unyango olwaneleyo.