Di Dzhordzhi syndrome: Okú, Treatment

timata Diseases o te pūnaha ārai mate ki te kia ako ngā tata, rite tenei pūtaiao ko tetahi o te whakaotinga i rongoa. Heoi, e tika ana ki te whanaketanga tere o tenei ra, i reira e maha ngā āhuatanga i roto i tenei mara. Diseases o te pūnaha o te tinana korero i huaina ārai, e kua wehea ki pāpaka tuatahi, me te tuarua. Tauira o taua pathologies Di Dzhordzhi ko te syndrome i nei i reira ko te hē congenital o T-lymphocytes. I tua atu ki te kore o ngā tikanga tiaki, whakakitea te mate e abnormalities maha o whēkau me he kitea i roto i kua pūnaha te wā fā'ele'i, a i roto i ētahi wā - i roto i te utero.

tikanga o te whanaketanga mate

āhuatanga Di Dzhordzhi syndrome e hypoplasia oti te kanohi ranei o te thymus (thymus). Kei tēnei okana te i roto i te kōhao rei i roto i ngā tamariki, me te he e tika ana mō te hanganga o te whakautu ārai pūtau. I te tino, tīmata te thymus ki holomui i te rahi me te whakaahua ke ki kiko ngako. He noa a kihai te māuiuitanga tenei tukanga. Ko te syndrome Di Dzhordzhi mai te reira ki te ngaro congenital o te okana kore ranei o tona whanaketanga (hypoplasia). Ka rite ki te hua o tenei pūtau ārai e hapa, me te kore e mahi ratou mahi. I tua atu, ka ko maha te mate he kore o te repe parathyroid, he kawenga mō te konupūmā me te ūkuikui pākia. Ko te tikanga o te whanaketanga o kōhikohiko o ngā pūnaha e pā ana ki waimaero retardation tupu intrauterine, tupu i roto i te hapū wawe.

Di Dzhordzhi syndrome: ai māuiuitanga

I te kau, i reira he kahore whakamārama mārama o te aha i reira he aplasia o te repe thymus. I roto i te nuinga o te wā, puta tenei mate i roto i te koha ira i runga i te pūira 22, i te mea te mate o tetahi o ona pae. Kei te whakapono te reira e te takahi taua he e tika ana ki te whakapapa, Heoi, tenei ariā kua kore i mohiotia. I tua atu, e kore e he koha i roto i te pūira 22 kitea i roto i ngā tūroro katoa, engari anake i roto i te 80% o te wā. Kei te whai pānga hoki Ngā take Di Dzhordzhi syndrome ki kukune fetal waimaero, hua i pathologies e wātea ana ki te whaea. ngā taringa roa ēnei i roto i hapūtanga, mate hopuhopu (kōpukupuku, karawaka, herpes), mate huka, roro whara, me ētahi atu Ētahi atu take syndrome Di Dzhordzhi - .. He taonga tūkino (waipiro, taero), me te rongo ki ngā matū i roto i te utero whanaketanga.

faaiteraa haumanu o aplasia thymic

riro kitea te mate kē i roto i nga ra tuatahi o te ora o te tamaiti, no te mea i roto i te tua ki te hē pūnaha ārai mate whakakitea e malformations maha. He mate ngakau te kino tino, rite kahore e etahi o ratou hototahi ki te ora (tetralogy o Fallot). Ka taea e abnormalities whanaketanga pā tetahi pūnaha okana, engari atu maha whakakitea Di Dzhordzhi syndrome e te tohu e whai ake nei:

1. tangata waimaero: whakaiti o nga wheua angaanga me kauae, whānui-whakaturia kanohi, hypoplasia o taringa, "Gothic" mangai, "ngutu ngatata", a na runga i ..
2. Kōhikohiko o te wahi romahā me te pū kai.
3. Malformations o te pūnaha io - atrophy o te uho kaka, me te roro tuarongo. Ka rite ki te hua o enei maumaui kitea kiwi e ngangau, paresis me te pāpāngia, huringa tairongo. whakaaro te faaiteraa matua o kōhikohiko CNS te retardation hinengaro, e riro te kite atu i roto i nga tau wawe o te ora o te tamaiti.
4. Malformations o te stenosis wahi kūnatu me te atresia.
5. piere pathological, te whakanui ake i te maha o maihao waewae ranei.
6. Kōhikohiko o te tinana whatu: strabismus, tuaroa disorders iaia.
7. Malformations o te pūnaha mimi.

Ka taea te whakakitea te whakararuraru, putawa ngā ki ia atu ranei. I roto i ētahi wā, i reira he kahore abnormalities whanaketanga, me te syndrome Di Dzhordzhi o ko te hē i roto i te tikanga ārai. whakakitea hē o te T-lymphocytes te he hehema mo mate kitakita me te viral, ngā tukanga puku. I roto i tenei mate, i puta ake i ārai, he uaua ki te antibacterial me therapy anti-inflammatory. repe Aplasia parathyroid whakakitea e āhua takiri.

Ko te paearu tātaritanga mo te mate

hanga te tātaritanga te ki te huinga o te tohu e whai ake nei: susceptibility tiketike ki ngā mate hopuhopu, abnormalities whanaketanga me hukihuki. I roto i te vitro kia kite assays hekenga i roto i te maha o lymphocytes me te whakaiti i roto i te taumata konupūmā. I roto i te tikanga ki te whakaū i te tātaritanga, he mea e tika ana ki te kawe i roto i rangahau o mana ārai. I roto i tenei take, i reira e te huringa e whai ake nei: ko te kore o te whakautu ki te werohanga me whakamātautau kino mō te hypersensitivity, me te panoni lymphoblastic. e kore te Tau o te B-lymphocytes puta ke. I roto i te ako o ngā hanganga wātea i te mate pūtau kitea ratou whakaiti, iti i roto i te mahi. I US ãpoko whakakitea te ngaro heke o te parathyroid me repe thymus ranei. Ko te hanganga o te rangahau DNA taea ki te ako e pā ana ki te huringa o te pūira 22.

maimoatanga mate Syndrome Di George o

Pathology Treatment tei runga i te tohu o te ofatiraa. I roto i te ngaro oti o thymus whakaaturia transplantation okana. he tika i roto i te malformation nui o whēkau me ngā pūnaha maimoatanga hāparapara. I roto i te akoranga ngāwari o te mate, me te kore o kōhikohiko nui whakaritea tiaki therapy. Ko te taero taketake he immunostimulants. I roto i te ora, ko te mea e tika ana ki te faaî i te hē te konupūmā i roto i te tinana. mate hopuhopu tono therapy symptomatic: paturopi, rongoā anti-viral. I tua atu ki te rongoā, ki te karo hauhauaitu, ahotea, me ētahi atu āhuatanga kino.

Ngā mutunga iho o aplasia o te thymus

He mōrearea Syndrome Di Dzhordzhi no te tura'ii te reira i te rota o pōauautanga. Tūroro ki tenei mate i haria ki tukanga puku, mate nui. Syndrome Ko te putanga auau o te whanaketanga o disorders autoimmune. Ko e tika ana ki te meka i te tinana kite nga tūroro ake ngā pūtau mo māngai ke, a ka tīmata ki te whawhai ki a ratou tenei. Nā tēnei, i roto i te mate pērā i erythematosus lupus pūnahanaha, dermatomyositis, kaikōiwi. Ka taea e te pōauautanga e whai ake nei puta i roto i kōhikohiko nui o te whanaketanga: retardation hinengaro, pāpāngia o wahi, matapo. A, no te disorders takiri kia whakawhanake kore romahā tika ki asphyxia.

Ko te waitohunga o te syndrome Di Dzhordzhi

A, no te ngā hē ārai me hē kitea o te angaanga kanohi kitea te tātaritanga: Di Dzhordzhi syndrome. Ka taea te kite Photos o iwi e mate ana i tenei mate i roto i te tuhinga motuhake hauora. Ki tenei huinga o enei tohu waitohunga mō te ora ko te tikanga kino rite abnormalities whanaketanga whakaaro puka nui o te mate. Tikanga, tamariki ki te syndrome Di Dzhordzhi, e kore e ora ki te tau o te 10 e tika ana ki kino e puta ana mate hopuhopu mate pukupuku ranei. I roto i te take o te rere pūkahukahu, kia kia painga kahore malformations me tika matapae whakakapinga therapy.

Prevention syndrome Di Dzhordzhi

e kore e ārai motuhake o tenei mate te tīariari. I roto i te tikanga ki te ārai maumaui o te whanaketanga embryonic o te kukune, Me te whaea teuteu ki te whakawhāiti i te āhuatanga ahotea, hoatu ake mau peu kino, engari ano ki te ārai i te mate o mate kitakita me te viral. Ka taea te kitea abnormalities whakawhanake i te ultrasound whakaritea, kawea nei i roto i roto i ia huatū o te hapūtanga.