Di Dzhordzhi chirwere: Kunokonzerwa, Treatment

Zvirwere zvirwere akatanga kudzidzwa tichishandisa yapfuura, sezvo sainzi uyu ndomumwe gotwe mukurapa. Zvisinei, nokuda kukurumidza kukura nhasi, pane zvinhu zvakawanda zviri mumunda uyu. Zvirwere muviri paaizvidzivirira ezvinhu inonzi immunodeficiency, izvo rakakamurwa nesekondari maronda. Mienzaniso pathologies dzakadaro Di Dzhordzhi ari chirwere iyo pane vachiberekwa kushayiwa T-lymphocytes. Kuwedzera kushaya anodzivirira zvenhengo chirwere kunoratidzwa vakawanda abnormalities zvomukati uye nehurongwa vatova kuoneka mune iri neonatal nguva, uye mune zvimwe zviitiko - mu utero.

zvigadziriswe zvirwere kukura

Di Dzhordzhi chirwere kunoratidzwa kwakakwana kana rushoma hypoplasia kuti ari thymus (thymus). Nhengo iyi iri muna thoracal mhango muvana uye zvakakodzera kuti paumbwe cellular masoja mhinduro. Panguva yokuyaruka, kuti thymus anotanga ndizvipete kukura uye zvinochinja kuva mafuta tishu. Izvi zvinoitika kwete zvokurwara. The chirwere Di Dzhordzhi totaura vachiberekwa pasina nhengo kana kushaya kukura kwayo (hypoplasia). Somugumisiro ichi masoja masero vari chakaremara uye havaiti mabasa avo. Uyezve, kana chirwere kazhinji kushayikwa pamusoro parathyroid omumuviri, vane basa calcium phosphorus muviri. The mashandiro pamusoro pokumuka anomalies-siyana gadziriro chokuita havachanyatsogoni intrauterine kukura retardation, zvichiitika mangwanani pamuviri.

Di Dzhordzhi chirwere: kunokonzera zvokurwara

Panguva iyoyo, hapana tsananguro yakajeka nei paine aplasia yacho thymus shwanu. Kazhinji, chirwere ichi kumbowanikwa majini urema pamusoro chromosome 22, inova kurasikirwa nomumwe nzvimbo dzayo. Zvinofungidzirwa kuti vakadaro kutyorwa kuri kukonzerwa yedzinza Zvisinei, dzidziso iyi haina zvakaratidza. Mukuwedzera, akaremara chromosome 22 haina kuchengetwa Varwere zvose, asi chete muna 80% nyaya. Zvikonzero Di Dzhordzhi chirwere kunewo chokuita havachanyatsogoni fetus mudumbu, zvichibva pathologies riwanikwe mai. Izvi zvinosanganisira ichinonoka panguva pamuviri, zvirwere (rubella, gwirikwiti, herpes), chirwere cheshuga, kukuvara uropi uye zvichingodaro D. Other anoita ari chirwere von Giorgio -. Imwe kushandisa zvinodhaka (doro, zvinodhaka), uye kuona kuti mishonga iri utero budiriro.

Clinical kuratidzwa thymic aplasia

The chirwere anova pachena kare mumazuva upenyu mwana wokutanga, nekuti kuwedzera rokuzvidzivirira kushaikwa kunoratidzwa vakawanda malformations. Wakakura zvikuru vari chirwere mwoyo, sevamwe vavo hahuenderani upenyu (tetralogy pamusoro Fallot). Zvokukura abnormalities kunogona chero nhengo hurongwa, asi kakawanda Di Dzhordzhi chirwere kunoratidzwa anotevera zviratidzo:

1. Havachanyatsogoni munhu: rutapudzo yeDehenya mapfupa uye shaya, paupamhi-nameso, hypoplasia nenzeve, "Gothic" mukanwa, "mumukaha muromo", uye zvichingodaro ..
2. Anomalies ari kufema turakiti uye yegurokuro.
3. Malformations pakati tsinga - atrophy of chepfungwa cortex uye cerebellum. Somugumisiro kutyorwa izvi akacherechedza gait chinovanetsa, paresis uye muviri, kutsinga dzinouya kuchinja. The chikuru kuratidza CNS anomalies anonzi retardation mupfungwa, iyo inova kuoneka mumakore oupenyu mwana vepakutanga.
4. Malformations of zvomudumbu turakiti stenosis uye Surgery.
5. Pathological mitswe, kuwedzera minwe kana zvigunwe.
6. Anomalies of optic muviri: strabismus, retinal vascular zvirwere.
7. Malformations nezvezvimwe zvinetso zvedundira.

The nyonganyonga kungaratidzwa singly kana pamwe nemumwe. Mune zvimwe zviitiko, hamuna zvokukura abnormalities, uye Di Dzhordzhi kuti chirwere chiri kushayikwa iri pazvirwere zvenhengo. Kushayikwa T-lymphocytes kunoratidzwa katsika kuti kwemabhakitiriya uye kutapukirwa noutachiona, bundu pezvakaitwa. In chirwere ichi, kunobva immunodeficiency, anonetsa antibacterial uye Anti-anogumbura kurapa. Aplasia parathyroid mumasero kunoratidzwa convulsive inoti.

The chirwere dzekuziva kuti chirwere

The chirwere inoitwa ibatanidzo zvinotevera zviratidzo: yakakwirira susceptibility kune zvirwere, zvokukura abnormalities uye pfari. Ezvokunambira kunze kwomuviri waamai assays ungacherechedza kuderera kwenhamba lymphocytes uye kuderera mune karusiyamu pamwero. Kuti asimbise nechirwere, zvakakodzera kuti tiite zvidzidzo kubva masoja hweHIV. Muchiitiko chino, pane zvinotevera kuchinja: kushaya achipindura kubaya uye kunaka miedzo nokuda hypersensitivity uye lymphoblastic kushanduka. Nhamba B-lymphocytes haina akachinjwa. Kudzidza cellular kutapata zvivako vakachengeta rutapudzo yavo, kutapudzwa mune basa. On US thorax akazivisa kusavapo kana kuderera pamusoro parathyroid uye thymus mumasero. Sechivako tsvakurudzo DNA kunoita kudzidza pamusoro shanduko pakati yechi22 chromosome.

kurapwa chirwere Di George chirwere

Zvokurwara Treatment kunobva mwero kutyora. Pasina thymus zvakakwana dzakaratidza nhego transplantation. Kuvhiya kurapwa kunodikanwa zvikuru malformation zvomukati uye hurongwa. In nyore weboka chirwere uye kushaya zvakakomba anomalies ushandiswe yokuzvigadzirisa mishonga. Mishonga inokosha ndivo immunostimulants. Kwoupenyu zvakafanira kuti zadzai karusiyamu kushayiwa mumuviri. Zvirwere kushandisa symptomatic kurapa: nemishonga, Anti-nemavhairasi mishonga. Kuwedzera mishonga, kunzvenga hypothermia, kushushikana, uye zvimwe nematambudziko zvinhu.

Migumisiro aplasia of thymus

Syndrome Di Dzhordzhi ndiyo ngozi nokuti kunosanganisira zvakawanda yematambudziko. Varwere nyonganyonga iri kubatwa bundu pezvakaitwa, yakanyanya noutachiona. Syndrome ari kakawanda mugumisiro kukura autoimmune zvirwere. Izvi imhaka yokuti muviri anoona varwere vane masero kuti dzimwe vamiririri, uye anotanga kuti urwe navo. Izvi zvinoguma nezvirwere zvakadai chinonzi systemic lupus erythematosus, dermatomyositis, dzinonzi rheumatoid arthritis. Zvinotevera yematambudziko munogona kwazvo anomalies okukura: retardation mupfungwa, kuoma maoko nemakumbo, upofu. Kana convulsive zvirwere zvingamuka kufema kukundikana nokuda asphyxia.

The fungidziro of urwere Di Dzhordzhi

Kana zvabatanidzwa Immune Deficiency uye kuonekwa kuremara pamusoro chiso yeDehenya iri pachena chirwere: Di Dzhordzhi syndrome. Photos of vanhu vanotambura nezvirwere izvi zvinogona kuonekwa mabhuku ezvokurapa inokosha. With pamwe ichi izvi zviratidzo fungidziro upenyu Kazhinji vanoshorwa sezvo zvokukura abnormalities zvinoratidza kwazvo chimiro zvirwere. Kazhinji, vana vane chirwere Di Dzhordzhi, regai kurarama makore 10 nokuda kwazvo yokuvamba zvirwere zvinotapuriranwa kana kenza. Kana zvemapapu aibuda, hapana malformations uye zvakakwana for yokutsiva kurapa zvingabatsira.

Kudzivirira chirwere Di Dzhordzhi

Dzakananga kudzivirira chirwere ichi haako. Kuitira kudzivirira kutyorwa embryonic kukura ari mudumbu, amai vanotarisira vanofanira kutirambidza kunetseka zvinhu, regedza tsika dzakaipa, asiwo hutachiwana kwemabhakitiriya uye nemavhairasi zvirwere. Zvokukura abnormalities dzinogona kuonekwa panguva yakarongwa Ultrasound, riri kuitwa nomumwe trimester akazvitakura.