Di Dzhordzhi syndrome: Amachititsa, Chithandizo

Matenda a chitetezo cha m'thupi anayamba kuphunzira ndi posachedwapa, monga sayansi ndi chimodzi mwa womaliza mankhwala. Komabe, chifukwa chitukuko mofulumira lero, pali zinthu zambiri m'munda umenewu. Matenda a dongosolo thupi chitetezo otchedwa immunodeficiency, amene anawagawa zotupa za pulaimale ndi sekondale. Zitsanzo za pathologies ngati Di Dzhordzhi ndi matenda amene pali akusowa kobadwa nako kwa T-lymphocytes. Kuwonjezera chosowa njira zodzitetezera matenda akuwonetseredwa ndi kuvutika angapo ziwalo ndi machitidwe kale noticeable mu nthawi neonatal, ndi zina - mu utero.

zimagwirira chitukuko matenda

syndrome Di Dzhordzhi amakhala ndi hypoplasia wathunthu kapena tsankho la pa thymus (thymus). limba ili mu M'mimbamo thoracic ana ndi M'pofunika mapangidwe mmene mthupi ma. Pa msinkhu, ndi thymus akuyamba kuotcha kukula ndi kusintha mu minofu mafuta. Zimenezi si zachilendo ndipo osati kudwala. The syndrome Di Dzhordzhi zifika kobadwa nako pakalibe chiwalo kapena kusowa chitukuko (hypoplasia). Chifukwa maselo ili m'thupi ndi zosalongosoka ndi osachita ntchito zawo. Komanso, pamene matenda zambiri kupanda wa zopangitsa za parathyroid, ali ndi udindo kashiamu ndi phosphorous kagayidwe. Limagwirira chitukuko cha anomalies kachitidwe zosiyanasiyana kugwirizana ndi mkhutu intrauterine kukula retardation, kunachitika mimba msanga.

Di Dzhordzhi syndrome: amachititsa kudwala

Pa nthawi, palibe momveka bwino chifukwa pali aplasia wa thymus England. Nthawi zambiri, matenda awa akupezeka chilema majini pa khromozomu 22, omwe ndi imfa ya munthu malo ake. Akukhulupirira kuti kuphwanya ndi chifukwa chibadwa Komabe, chiphunzitso sikuti zatsimikiziridwa. Komanso, chilema khromozomu 22 si anati odwala onse, koma 80% ya milandu. Zifukwa syndrome Di Dzhordzhi unakambidwanso mogwirizana ndi mkhutu yoopsa mwana wosabadwayo, chifukwa pathologies kupezeka kwa mayi. Tikhoza mtsogolo pa mimba, matenda opatsirana (chikuku, chikuku, nsungu), shuga, kuvulala ubongo, etc. Zina syndrome Di Dzhordzhi - .. Kodi kumwa mankhwala osokoneza bongo (mowa, mankhwala), komanso kukhudzana ndi mankhwala utero chitukuko.

Matenda mawonetseredwe a aplasia thymic

Matendawa zimaonekera kale m'masiku oyambirira a moyo wake, chifukwa kuwonjezera pa dongosolo akusowa m'thupi akuwonetseredwa ndi malformations angapo. The aakulu ndi matenda a mtima, monga ena a iwo sali yogwirizana ndi moyo (tetralogy wa Fallot). kuvutika kakulidwe zingakhudze dongosolo lililonse limba, koma nthawi zambiri Di Dzhordzhi matenda akuwonetseredwa ndi zizindikiro zotsatirazi:

1. Mkhutu munthu: kuchepetsa mafupa chigaza ndi chibwano, lonse anapereka maso, hypoplasia makutu, "Gothic" m'kamwa, "mphanga mlomo", ndi zina zotero ..
2. Anomalies wa kapepalako kupuma ndi kum'mero.
3. Malformations ya ubongo - manja a kotekisi oziziritsa ndi cerebellum. Chifukwa cha kuyan'anila awa anati gait chisokonezo, paresis ndi ziwalo, kusintha kwenikweni. Chiwonetsero chachikulu cha CNS anomalies imatengedwa retardation maganizo, amene akukhala noticeable mu zaka zoyambirira za moyo wa mwana.
4. Malformations ya m'mimba thirakiti stenosis ndi atresia.
5. Pathological fractures, kuwonjezera chiwerengero cha zala kapena zala.
6. Anomalies wa thupi chamawonedwe: strabismus, retinal mtima matenda.
7. Malformations a dongosolo mkodzo.

matenda The ungadziwike singly kapena pamodzi ndi mzake. Nthawi zina, palibe kuvutika kakulidwe, ndi matenda Di Dzhordzhi ndi akusowa mu zimagwirira m'thupi. Chakuchepa kwa T-lymphocytes akuwonekera chizolowezi cha matenda bakiteriya ndi tizilombo, njira chotupa. Mu matenda, akuwuka kuchokera immunodeficiency, ndi ovuta antibacterial ndi odana ndi yotupa mankhwala. zopangitsa Aplasia parathyroid anaonetsa limati convulsive.

akugwirizana ndi matenda matendawa

matenda wopangidwa ndi osakaniza zizindikiro zotsatirazi: mkulu chiwopsezo kwa matenda opatsirana, kuvutika kakulidwe ndi khunyu. Mu m'galasi kafukufuku mungaone kuti kuchepa kwa chiwerengero cha lymphocytes ndi kuchepa mu mlingo calcium. Pofuna kutsimikizira matenda, ndikofunikira kugwira maphunziro a udindo m'thupi. Pankhaniyi, pali kusintha zotsatirazi: kupanda poyankha katemera ndi mayesero zoipa chifukwa hypersensitivity ndi lymphoblastic kusintha. Chiwerengero cha B-lymphocytes si adasandulika. Mu kuphunzira nyumba chitetezo chokwanira ma anati kuchepetsa awo, kuchepa ntchito. Pa US thorax anawulula pakalibe kapena kuchepa kwa parathyroid ndi zopangitsa thymus. Kapangidwe ka kafukufuku DNA amalola kuphunzira za kusintha kwa khromozomu 22.

matenda Syndrome Di George chithandizo

Kudwala Chithandizo zimadalira mlingo wa kuphwanya. Mu anasiya thymus wasonyeza kupatsidwa zina chiwalo. Opaleshoni chithandizo chofunika malformation choopsa cha ziwalo ndi kachitidwe. Mu wopepuka Inde cha matenda ndi kusowa kwa anomalies kwambiri zotchulidwa yokonza mankhwala. mankhwala zazikulu ndi immunostimulants. Pa moyo m'pofunika kuligonjetsa akusowa kashiamu mu thupi. matenda opatsirana ntchito mankhwala symptomatic: mankhwala mankhwala odana ndi tizilombo. Kuwonjezera mankhwala, kupewa hypothermia, nkhawa, ndi zina chokhwima.

Zotsatira za aplasia wa thymus ndi

Syndrome Di Dzhordzhi ndi oopsa chifukwa kumatanthauza kwambiri matendawo. Odwala matenda atengeke njira chotupa matenda aakulu. Syndrome ndi chifukwa kawirikawiri chitukuko cha matenda autoimmune. Ichi ndi chifukwa chakuti thupi amaona odwala eni maselo kwa wothandizila achilendo, ndipo ayamba kumenyana nawo. Izi zimachititsa matenda monga zokhudza zonse lupus erythematosus, dermatomyositis, nyamakazi. Mavuto otsatirawa angayambe anomalies choopsa cha chitukuko: retardation maganizo ziwalo za miyendo, khungu. Pamene matenda convulsive angathe kulephera kupuma chifukwa asphyxia.

Kuti madokotala ananena zawo za matenda Di Dzhordzhi

Zimagwira akusowa m'thupi ndi zilema owoneka a chigaza nkhope poyera matenda: Di Dzhordzhi syndrome. Photos wa anthu odwala matenda Tingaone mu mabuku wapadera mankhwala. Ndi kuphatikiza zizindikiro madokotala ananena zawo moyo zambiri akumane monga kuvutika kakulidwe amati mawonekedwe choopsa cha matenda. Childs, ana ndi matenda Di Dzhordzhi, sakhala kwa zaka 10 chifukwa matenda aakulu zikuchitika matenda kapena khansa. Pankhani ya otaya m'mapapo, palibe malformations ndi Mapa chokwanira m'malo mankhwala kungakhale kothandiza.

Kuteteza matenda Di Dzhordzhi

Specific kupewa matenda kulibe. Pofuna kupewa kuyan'anila za chitukuko embryonic wa mwana wosabadwayo, mayi oyembekezera ayenera kuchepetsa zinthu nkhawa, kusiya zizolowezi zoipa, komanso matenda a matenda bakiteriya ndi tizilombo. kuvutika kakulidwe angathe kuonedwa pa ultrasound yokonzedweratu, yomwe ikuchitika aliyense trimester mimba.