Immunodeficiencies Paraimere. immunodeficiencies Paraimere: maimoatanga

Ārai - he takahi o nga mahi tiaki o te rauropi tangata, e tika ana ki te vaivai o te whakautu ārai ki tukumate o te natura rerekē. fakamatala'i Pūtaiao he maha o ngā āhuatanga motuhake o tenei ahua. Tenei rōpū o disorders āhuatanga e nui haere auau, me te akoranga Nuiōrau o ngā mate hopuhopu. Malfunctions wātea i te mate i roto i tenei take e pā ana ki te huringa i roto i te āhuatanga kounga ine ranei o ona wae takitahi.

āhuatanga wātea i te mate

Ko te pūnaha ārai whai wāhi tino i roto i te mahi noa o te tinana, kia rite ki te tikanga ki te kitea me te whakangaro i nga antigens e kia kia tomo ranei i te taiao o waho (hopuhopu) kia te putanga o te tupu puku ake ngā pūtau (endogenous) ranei. Kei te matua i te mahi tiaki e whakaratohia ana e te āhuatanga congenital pērā i phagocytosis me pūnaha ngä. Hoki te whakautu urutau o te kaiao riro pahono pūnaha ārai: te humoral me pūtau. Communication e wahi i roto i te pūnaha katoa o matū motuhake - cytokines.

Tei runga i te take, kua wehea disorders wātea i te mate kāwanatanga ki immunodeficiencies tuatahi, me te tuarua.

He aha te mea he ārai tuatahi

immunodeficiencies Tuatahi (PID) - he takahi o te whakautu ārai tika ki hē ira. I roto i te nuinga o te wā, kei te riro ratou me he abnormalities congenital. Kei te tino whakakitea PID i te wawe tau, engari i ētahi wā e kore ratou e pāngia noa te taiohitanga noa pakeketanga ranei.

PID - he rōpū o mate congenital, he momo o faaiteraa haumanu. International Whakarōpū o Diseases 36 kau uiui, a makona whakaahuatia āhua ārai tuatahi, engari kei te rite ratou ki te tuhinga hauora, i reira e pā ana ki 80. Ko te take i kua tāutuhia e kore ira mate katoa haepapa.

āhuatanga anake te hanganga ira o te pūira X e te iti rawa e ono rerekē hē ārai, a reira ko nui ki runga ake i taua o kotiro te auau o te takanga o enei mate i roto i nga tane. He he he whakaaro e kia whai i te whanaketanga o te ārai congenital mana aetiological o mate intrauterine, engari kua kore e ano i pūtaiao kï tenei kerēme.

pikitia haumanu

Ko nga mau faaiteraa haumanu o immunodeficiencies tuatahi rite rere rite ratou ratou kāwanatanga, Heoi, i reira he kotahi āhuatanga noa - infective hypertrophic (kitakita) syndrome.

immunodeficiencies Paraimere, me te tuarua, i whakaatu i te haafifi i ki tūroro auau paipa (hohokitanga) mate o etiology hopuhopu e kia meinga e tukumate mohiotia.

Kei te tino maha kitea tenei mate ki te pūnaha bronchopulmonary me Ent-okana. Hoki maha mamae kiriuhi mucous me integuments, e kia whakaatu sepsis me te whēwhē. meinga tukumate kitakita Bronchitis me te pakohu kakā. Iwi ki te hē ārai, e maha kitea moremore wawe me te katoatia, me ngā tauhohe ētahi wā pāwera. disorders toutou me autoimmune, me keno ki repara. hē ārai i roto i ngā tamariki te ai tata tonu te roa o te whanaketanga hinengaro, me te tinana.

Tikanga o te whanaketanga o immunodeficiencies tuatahi

Whakarōpū o mate rite ki te huarahi o to ratou whanaketanga ko te kōrero tino i roto i te ako take o āhua ārai. Doctors wehe te āhua ārai katoa o te mate ki 4 ngā rōpū matua:

- Humoral B-pūtau ranei, e ngā syndrome o Bruton (agammaglobulinemia ki hono ki te pūira X) iga hē IgG, i te taikaha IgM ranei ki te katoa hē immunoglobulin, ārai te ana'iraa ohie, tahuri hypogammaglobulinemia hou me te maha o ētahi atu mate e pā ana ki te mate humoral .

- T pūtau immunodeficiencies tuatahi, maha huaina ngā, mai pakaru tonu nga disorders tuatahi, me te wātea i te mate humoral, hei tauira hypoplasia (Di George syndrome) ranei dysplasia (T-lymphopenia) thymus.

- Immunodeficiencies meinga e mea kohakore i phagocytosis.

- Immunodeficiencies meinga e disorders o te pūnaha ngä.

susceptibility ki te mate

Mai kia te take o te ārai te ofatiraa o te ngā wae
e kore ko te taua hoki ia take te pūnaha ārai mate, me te rongo ki āpiha hopuhopu. Hei tauira, ka te mate humoral ko e pängia ana te manawanui ki mate e kua meinga e te streptococci, staphylococci, rewharewha kukuti. Ko te kupu maha whakaatu enei microorganisms ātete ki paturopi. A, no te taea te piri puka ārai ngā ki te huakita wheori, pērā i herpes ranei harore e e te nuinga kanohi candidiasis. āhuatanga puka Phagocytic te nuinga e te staphylococci taua me huakita Gram-kino.

Ko te nuinga o immunodeficiencies tuatahi

Immunodeficiencies, riro, - rawa he mate tangata onge. Me aro mātaitia te auau o taua disorders ārai ki te faatura ki ia mate ngā no rerekē ratou nuinga.

I te toharite, kotahi anake i roto i o e rima tekau mano hou ka tukua e ārai tuku iho congenital. Ko te mate tino noa i roto i tenei rōpū ko te kore o te iga whiriwhiri. kitea ārai congenital o tenei momo i roto i te waenganui o tetahi o nga mano o hou. A 70% o te wā katoa o iga hē he kore katoa o tenei wae. I te wa ano, kia tohaina etahi mate tangata nui atu te utu o te natura ārai, riro, i roto i te ōwehenga o te 1: 1,000,000.

Ki te whakaaro tatou ki te pā o mate PID i runga i te huarahi rānei, kua tito reira āhuatanga rawa ngā. B pūtau-immunodeficiencies tuatahi, ranei rite ratou e atu nuitia huaina, he takahi o te antibody, atu noa atu i ētahi atu, me te hanga ake 50-60% o te wā katoa. I te wa ano, T-pūtau me te puka phagocytic pāngia i roto i te 10-30% o tūroro ia. Whakaaro nga mate tino onge o te pūnaha ārai mate meinga e hē i roto i te ngä - 1-6%.

Me mahara hoki käore te reira e he tino rerekē i roto i nga whenua rerekē, e ai kia pā ana ki te predisposition ira o te rōpū ā-motu ki irakētanga DNA motuhake te auau o te tupu PID raraunga.

tātaritanga o te ārai

ārai Paraimere i roto i te tamariki te tino maha whakaritea e te materoto, no te mea
te meka e te tātaritanga taua i runga i te taumata tākuta tamariki rohe he uaua.

te tikanga hua tenei i roto i te roa i roto i te timatanga o te maimoatanga me te waitohunga rawakore o te therapy. Ki te te whakaaro te tākuta e hāngai ana i runga i te pikitia haumanu o te mate, me nga hua whakamātautau whānui āhua ārai, te mea tuatahi me mahi ia - ki te tukua atu te tamaiti ki te tākuta-immunologist.
I roto i Europe reira ko te Association o Immunologists, e no ni'ai te te ako me te whanaketanga o ngā tikanga o te maimoatanga o taua mate, e huaina nei i te ingoa o te EDI (Pākehā Society mō immunodeficiencies). E hanga ana ratou, a whakahoutia tonu pātengi raraunga PID mate, a whakaaetia hātepe tātaritanga mo te tātaritanga makona tere.

A haamata tātaritanga mā te kohikohi hītori hauora. kia utua ngā whakarongo ki te āhuatanga whakapapa, rite he tuku te nuinga o immunodeficiencies congenital. Next, hoatu i muri i te whakamātautau tinana, me te whiwhi whānui raraunga rangahau haumanu te tātaritanga tuatahi. I roto i te heke mai, ki te whakaū i turaki i te tākuta whakapae ranei, kia amohia e te manawanui he hi'opo'a maitai e tohunga pērā i geneticist me immunologist. Anake i muri i te katoa o nga whawhe runga ake ka taea e tatou e te kōrero e pā ana ki te hanga o te tātaritanga whakamutunga.

rangahau taiwhanga

Ki te i roto i te tātaritanga whakapae te ārai tuatahi syndrome, he tika nga whakamātautau taiwhanga e whai ake nei ki:

- te whakatūnga o te tatau toto oti (aronga i runga i te maha o lymphocytes);

- faaotiraa o immunoglobulins serum;

- te tātaitanga ine o B- me T-lymphocytes.

rangahau atu

I tua atu ki te taiwhanga, me te tātaritanga whakamātautau, e kua oti whakahuatia i runga ake, ka te whakamātautau atu takitahi kia whakaritea i roto i ia take. He he he rōpū mōrearea, e hiahia ana ki te kia whakamatauria hoki HIV mate, abnormalities ira ranei. Hoki whakarato te tākuta te pea e te hakari ārai tangata 3 4 ranei momo, i raro i nei e tohe ki runga ki te ako taipitopito o te manawanui i te phagocytosis mahi whakamātautau tohu tetrazolinovym wae puru, me te whakaū hanganga o te pūnaha ngä.

maimoatanga PID

Ko reira kitea e ka whakawhirinaki matua te therapy tika i te mate ārai, engari, kāore, e kore e taea te whakakorea te puka congenital rawa, e kore e taea te mea e pā ana ki te ārai riro. I runga i ngā whanaketanga hauora o nāianei, e tamata mau aivanaa ki te kitea te ara ki te tango i te take i te taumata ira. Heoi tawhiti, kihai i ta ratou mau tutavaraa i angitu, ka taea e tatou te mea e te hē ārai - he tu'unga mahu. A feruri i te mau parau tumu o te therapy tono.

therapy whakakapinga

Maimoatanga o te hē ārai te tikanga heke ki te whakakapinga therapy. Ka rite ki whakahuatia mua, e kore he ahei te tinana o te manawanui ki te whakaputa i etahi wāhanga o te pūnaha ārai mate, tino raro ratou kounga hiahiatia ranei. Therapy i tenei e waiho he paturopi rongoā fariiraa immunoglobulins ranei, he whati hua tūturu. Nuinga o ngā wā, kei te whakahaerea e pâmuhia taero, engari i ētahi wā ko reira taea, me ara subcutaneous, ki te whakahaere i te ora o te manawanui, i roto i tenei take e kore e whai ki te haere ki kotahi ano te whare hauora.

whakauru parau tumu maha taea tūroro ki te arahi ora tata noa: ki te ako, mahi, me te tākaro. O te akoranga, he ngoikore mate pūnaha ārai mate, ngā āhuatanga humoral me pūtau me te hiahia tamau mo te whakataki o taero utu kore e tukua te manawanui ki tino akaanga, engari he reira tonu pai atu i noho i roto i te ruma pēhanga.

maimoatanga symptomatic me te ārai

Homai te meka e tetahi iti mo te whakapokenga huakita viral ranei tangata hauora mō te rōpū manawanui o te mate ārai tuatahi e taea e mate, he mea e tika ana ki te kawe i roto i te ārai mātau. Here mai ki antibacterial tākaro, antifungal me taero patu huaketo. auau matua ki te mahi i te mea te reira i runga i āraihia, no te mea e kore e taea e te pūnaha ārai mate ngoikore utu ki te whakarato i te kounga maimoatanga.

I tua atu, kia mahara ai e he pängia ana ki mate pāwera, autoimmune, a, tae noa kino, ki āhua puku enei tūroro. tenei katoa, kahore he te tirotiro tonu hauora kore e taea e tukua te tangata ki te arahi i te ora tonu.

transplantation

A, no te whakatau tohunga e kore e te manawanui noho kore whiriwhiri engari ki te pokanga, e taea te whakamana wheua wheua transplantation. e pā ana tēnei tukanga i te tūponotanga maha mo te ora, me te hauora o te manawanui, me te i roto i te mahi, ara i roto i te hui o te putanga angitu e kore e taea e whakaoti tonu i nga raruraru katoa o te mamae i te mate ārai. I roto i te mau ana i roto i te mahi taua, whakakapia te pūnaha katoa e te hematopoiesis kaiwhiwhi pērā i whakaratohia e te kaituku.

immunodeficiencies Paraimere Ko te raruraru uaua tino o rongoā hou, e, kāore, kihai i whakatau rawa. kaha tonu waitohunga Poor mō mate o tenei ahua, a ko reira whiunga meapango, homai te meka e mamae ratou te nuinga tamariki. Otiia, he maha ngā momo o te hē ārai he hototahi ki te ora tonu ki te he ratou tātaritanga wawe me te whakamahi o te tika therapy.