A Bruton mate: etiology, ai, tohu me ngā āhuatanga maimoatanga

mate ira - ko reira he mate onge congenital e he uaua ki te matapae i roto i te mua. puta ratou ara i te wa ka i reira te mea he hanganga o te ira. Nuinga o ngā wā, kei te haere ratou i matua, engari e kore e tupu tonu reira. I roto i ētahi wā, maumaui puta i roto i te ira ratou. Ko tētahi o ēnei pathologies whakaaro te agammaglobulinemia X-hono. E pā ana te reira ki te ārai tuatahi. Kua tata whakatuwheratia te mate i roto i te rau tau pokapū o-20. Na reira, e kore e tino mohio e taote. puta reira rawa uaua, anake i roto i te mau tamaroa.

mate a Bruton: te hītori o

Tenei māuiuitanga e pā ana ki ngā kōhikohiko pūira X-hono tuku i te taumata ira. āhuatanga mate o Bruton e abnormalities i roto i te wātea i te mate humoral. Ko te tohu matua ko tona susceptibility ki tukanga hopuhopu. Ko te whakahua tuatahi o kaute mō te 1952 o tenei māuiuitanga. I taua wa, ako te pūtaiao American Bruton hītori o te tamaiti he kino neke atu i te 10 ngā wā i roto i te 4 tau o tau. I roto i kua tutaki tukanga hopuhopu tenei tamaiti te septicaemia, kakā, meningitis, i runga mumura wahi romahā. A, no te ui ki tamariki, i kitea ai e paturopi ki enei mate he mea motu. I roto i te mau parau te tahi atu, i muri i te whakautu ārai kore i kitea mate.

I muri mai, i roto i te mutunga o te rau tau 20, X-hono agammaglobulinemia tākuta te uiui ano. I roto i te 1993, i taea e ki te tāutu i te ira e ai wātea i te mate waimaero tākuta.

Take o te agammaglobulinemia X-hono

Agammaglobulinemia (mate o Bruton) maha he tuku. whakaaro te koha te ki te waiho i te huru ngoikore, na te tūponotanga o ia i te tamaiti ki te māuiuitanga, ko te 25%. Carriers o te ira mutant he wahine. Ko e tika ana ki te meka i paetata te koha kei i runga i te pūira X tenei. Otiia, tuku te mate e ki te tane anake. Ko te take matua agammaglobulinemia he pūmua hapa, wahi o te waehere ira mō te tyrosine kinase. I tua atu, ka taea e te mate o te Bruton kia idiopathic. Tenei te tikanga e te take o tona tupu tonu mārama. I roto i te āhuatanga mōrearea e pā waehere iranga o te tamaiti, tohatoha:

1. Abuse o waipiro me taero i roto i te hapū.
2. riaka psycho-aronganui.
3. Mārakerake ki ionizing radiation.
4. nanamu matū (production whanoke, taiao kino).

He aha te mea te pathogenesis o te mate?

Tikanga o te mate e pā ana ki te pūmua hapa. Tikanga, te ira kawenga mō te whakawaehere tyrosine kinase, whai wāhi i roto i te hanganga o te B-lymphocytes. Ko ratou pūtau ārai e he kawenga mō te korero humoral. Nā ki te kaihautanga o lymphocytes tyrosine kinase B kore e pakari tino. Ka rite ki te hua, e taea ki te whakaputa i immunoglobulins ratou - paturopi. Ko te pathogenesis o te mate Bruton ko te ārai oti o te tiaki humoral. Ka rite ki te hua, i roto i te whakapā ki te āpiha hopuhopu ki te paturopi tinana kore e taua hua. He āhuatanga motuhake o tenei mate ko e taea ki te whawhai i te huaketo, ahakoa te ngaro o te B-lymphocytes te pūnaha ārai mate he. Nature o te ofatiraa o te tiaki humoral tei runga i te pakeke o te koha.

Mate a Bruton: tohu mate

Māuiuitanga mo te wa tuatahi hanga ite iho i roto i te aruaruraa. Nuinga o ngā wā, faaite te mate ano i roto i 3-4-th o te ora marama. Ko e tika ana ki te meka e i tenei tau mutu te tinana o te tamaiti ki te tiaki i paturopi o te whaea tenei. Ko te tohu tuatahi o te mate e taea te waiho i te tauhohenga mauiui e whai ake nei werohanga, rashes kiri, mate o te wahi romahā o runga o raro ranei. Heoi, whāngai ū tiaki te tamaiti i ngā tukanga inflammatory, mai te waiu, uma he i roto i tona hanganga o immunoglobulins.

mate Bruton faaite e pā ana ki 4 tau. I tenei wa mai i te tamaiti ki te whakapā ki ētahi atu tamariki i roto i te kindergarten. I roto i pāpaka hopuhopu te nuinga meningo, streptococci me microflora staphylococcal. Ka rite ki te hua, kia kia rongo ngā tamariki ki te mumura purulent. Ko te mate tino auau ngā kakā, pakohu kakā, pāpāho otitis, pakohu kakā, kiriuhi, pīkaru. I te maimoatanga mea mate o te katoa o enei tukanga taea haere i roto i te sepsis. Ko hoki te reira i te faaiteraa o te mate kia Bruton māuiuitanga Dermatological. Nā ki tini tere te microorganisms whakautu ārai iti i te pae o te patunga me rapirapi.

I tua atu ki te mau faaiteraa o te mate ngā bronchiectasis - huringa pathological i roto i te pūkahukahu. Tohu ngā hāhā o te manawa, mamae i roto i te pouaka, i ētahi wā hemoptysis. Ko taea ano hoki te putanga o pāpaka inflammatory i roto i nga whekau kūnatu, mimi, kiriuhi mucous. Wā kitea ngawhatanga me te here i roto i te hononga.

Ko te paearu tātaritanga mo te mate

kia whakauru i te paearu tātaritanga tuatahi te pā auau. Tamariki e mate ana i te māuiuitanga o Bruton, kawe neke atu i te 10 mate ia tau, me te rave rahi taime i roto i te marama. kia toutou mate ai kē (otitis pāpāho, tonsillitis, kakā) ranei. I te whakamātautau, i reira te mea he hypertrophy kore pharyngeal puku. Ko te taua pā ki palpation o taikiri piringa. kia utu ano koe whakarongo ki te tauhohenga o te tamaiti i muri i te werohanga. kitea tino huringa i roto i assays taiwhanga. Ko te tohu KLA o mumura (nui haere te maha o leukocytes whakatere ESR). iti te maha o ngā pūtau ārai te. Ka kitea tēnei i roto i te tātai leukocyte: he maha iti o lymphocytes me neutrophils nui haere ihirangi. rangahau nui he immunogram. whakaata te reira i te whakaiti ngaro ranei o paturopi. Tēnei āhuatanga e āhei ki a koe te hanga i te tātaritanga. Ki te he feaa te tākuta, he reira taea ki te kawe i roto i whakamātautau ira.

Rerekētanga mate Bruton i pathologies rite

rerekëtanga tēnei māuiuitanga e i te tahi atu tuatahi me immunodeficiencies tuarua. I roto ia ratou - te momo agammaglobulinemia Swiss, syndrome Di Dzhordzhi o, HIV. I roto i te rerekē ki enei pathologies mō te mate o Bruton āhuatanga e te ofatiraa anake o te mate humoral. whakakitea ana tēnei e te meka e taea ki te whawhai āpiha viral te tinana ko. Ko rerekē i te momo agammaglobulinemia Swiss, pakaru nei te ka rua whakautu ārai humoral me pūtau tenei take. Hei hanga i te tātaritanga pārōnaki ki te syndrome Di Dzhordzhi, e hiahia ana koe ki te mahi i pouaka X-ray (thymic aplasia) me ki te whakatau i te ihirangi konupūmā. Hei aukati HIV palpation whakahaere o taikiri, Elisa.

Methods o te maimoatanga o te agammaglobulinemia

Kia aroha mai, tino hinga mate o Bruton e taea. Ko te tikanga o te maimoatanga ngā whakakapinga agammaglobulinemia me therapy symptomatic. whakaaro te whakaaro matua o te paetae te ki hei taumata noa o immunoglobulins i te toto. kia te nui o paturopi kia approximated ki te 3 g / l. Hoki tenei whakaaro, gamma globulin te auau o 400 mg / taimaha tinana kg. kia nui haere antibody kukū i roto i whakapeka mate hopuhopu, rite kore e taea e te tinana akakoromaki i ratou takitahi.

I tua atu i whakamana i maimoatanga symptomatic. Ko te tino pinepine whakaritea paturopi "Ceftriaxone", "rongoā paturopi", "Ciprofloxacin". A, no te Me faaiteraa kiri ki te maimoatanga wā. tūtohutia hoki mucosal horoi rongoā patuero (korokoro me te irrigation nasal).

Ko te waitohunga mō agammaglobulinemia o Bruton

Ahakoa te therapy whakakapinga mo'uí, te waitohunga mo te agammaglobulinemia pai. maimoatanga tonu me te prophylaxis o tukanga hopuhopu whakaiti atu tahumaero whakaiti. Tikanga, E taea e-marohirohi, me te kaha tūroro. I te huarahi he ki te maimoatanga e taea te whakawhanake pōauautanga tae noa sepsis. I roto i te take o te rere mate waitohunga rawakore.

Prevention o agammaglobulinemia X-hono

I roto i te aroaro o te mate i roto i whanaunga whakapae ranei he mea e tika ana ki te whakahaere whakamātau ira noa i roto i te huatū tuatahi o te hapūtanga. Hoki kia whakauru ki āraihia atu te rongo ki te rangi, te ngaro o te mate ma'i me ngā pānga kino. I roto i te hapū, ko te ahotea contraindicated te whaea. Hoki te ārai tuarua he huaora therapy, whakahaere o te gamma globulin, he āhua hauora. He mea nui hoki ki te karo whakapā ki te iwi huaketo.