Di Dzhordzhi syndrome: Sababaha, Daaweynta

Cudurada of habka difaaca jirka bilaabay in la derso xad dhawaan, sida sayniska tani waa mid ka mid ah ugu yaraa ee daawo. Si kastaba ha ahaatee, ay sabab u tahay horumarka deg deg ah oo maanta, waxaa jira daahfurtay in badan duurka this. Cudurada of nidaamka difaaca jirka ee loo yaqaan difaaca, kuwaas oo u kala qaybsan yihiin lesions hoose iyo kuwa sare. Tusaalooyinka heley sida Di Dzhordzhi waa syndrome ah taas oo ay jirto Nusqaan lagu dhasho ee T-limfosaytis. Waxa intaa dheer in la'aanta ah ee hababka difaaca, cudurka lagu muujiyey by aan caadi ahayn kala duwan ee xubnaha iyo nidaamyada hore u dareemi in muddada dhalaanka, iyo mararka qaarkood - in utero.

hababka horumarinta cudurka

Di Dzhordzhi syndrome waxa lagu gartaa hypoplasia dhammaystiran ama qayb ka mid ah thymus ah (thymus). xubin Tani waxa uu ku yaalaa saabka thoracic in carruurta iyo waa lagama maarmaan u ah formation of jawaabta difaaca ee gacanta. At qaangaadhnimada, thymus bilaabmin si loo yareeyo in xajmiga iyo u beddesho galay unugyada baruurta. nidaamka Tani waa caadi oo ma cudurada a. syndrome The Di Dzhordzhi ay timaado in maqnaanshaha lagu dhasho ee xubinta ama la'aanta dhinaca horumarinta (hypoplasia). Natiijada unugyada difaaca jirka this yihiin haleysan yahay oo ma u qabtaan hawlo ka. Intaas waxaa sii dheer, marka cudurka inta badan waa la'aanta ah oo ka mid ah qanjidhada parathyroid, ayaa ka mas'uul ah calcium iyo Dheef fosfooraska. farsamo ee horumarka caadi ahaanshaha hababka kala duwan ee la xidhiidha dhimaal koritaanka intrauterine oo yaraada, dhaca hore ee uurka.

Di Dzhordzhi syndrome: sababa cudurada

Xilligan la joogo, ma jirto sharaxaad cad u ah sababta ay jirto aplasia qanjirka thymus ah. Xaaladaha intooda badan, cudurkan ku dhacaa iin hidaha on koromosoom 22, taas oo khasaaraha ka mid ah mid ka mid ah goobaha ay. Waxaa la rumeysan yahay in xadgudub noocaas ah waxaa sabab u ah hiddo, si kastaba ha ahaatee, aragtida this aan la tijaabiyey. Intaa waxaa dheer, iin ee koromosoom 22 aan arkay bukaanka oo dhan, laakiin kaliya in 80% ee kiisaska. Sababaha Di Dzhordzhi syndrome waxa sidoo kale la uurjiifka uurjiifka oo yaraada, taasoo keentay in laga heley heli karo hooyada. Waxaa ka mid ah dib loogu dhigay inta ay uurka leedahay, cudurrada faafa (rubella, cudurka jadeecada, herpes), sonkorowga, dhaawaca soo gaara maskaxda, iwm sababaha kale ee syndrome Di Dzhordzhi - .. Ma isticmaalka mukhaadaraadka (khamriga, daroogada), iyo gaadhista kiimikada ee utero horumarinta.

daliilka Clinical of aplasia thymic

Cudurku wuxuu ku caddaato horay maalmood ee ugu horreeya nolosha ilmaha, maxaa yeelay, marka lagu daro deficiency habka difaaca la muujiyey by curyaanimo badan. Waxaa ugu daran yihiin cudurka wadnaha, sida qaar ka mid ah ma aha kuwo la jaan qaada nolosha (tetralogy ah Fallot). aan caadi ahayn Developmental saamayn ku yeelan kara nidaamka xubin kasta, laakiin marar badan Di Dzhordzhi syndrome la muujiyey by calaamadaha soo socda:

1. qof oo yaraada: hoos lafaha dhaladii iyo daanka, indhaha kala dhigay, hypoplasia dhegood, "Gothic" dhanxanegga, "dibinta dillaacii", iyo wixii la mid ah ..
2. Cillado of mareenka-mareenka iyo hunguriga.
3. Cilladaha habka dareemayaasha - atrophy of kiliyaha cerebral iyo cerebellum. Sidaas darteed xadgudubyada, kuwaas oo arkay qas wiiqmin, paresis oo curyaannimo, isbedel dareenka. muuqaalka ugu weyn ee cillado habdhiska dhimirka waxaa loo arkaa dhimaal maskaxeed, kaasoo noqda dareemi in sannadaha hore ee nolosha ilmaha.
4. Cilladaha stenosis ka shiidka iyo atresia.
5. jab bahal ah, sii kordhaya tirada faraha ama suulasha.
6. Cillado ee jirka optic: strabismus, xanuunada vascular isha.
7. Cilladaha nidaamka kaadida.

disorder waxaa lagu muujiyey kartaa jeer ama in lagu daro kasta oo kale. Xaaladaha qaarkood, waxaa jira ma aan caadi ahayn koritaanka, iyo Di Dzhordzhi ee syndrome waa deficiency ah hababka difaaca. Deficiency of T-limfosaytis lagu muujiyey janjeera waayo, jeermiska iyo Fayraska, geedi socodka buro. In cudurkan, ka timi difaaca, waa adag tahay in bakteeriyada iyo therapy anti-bararka. qanjidhada parathyroid Aplasia muujiyey by dalalka convulsive.

Shuruudaha lagu ogaanayo cudurka

Waxaa cudurka lagu sameeyey isku dhafan oo ka mid ah calaamadaha soo socda: nuglaato sare si ay cudurrada faafa, aan caadi ahayn koritaanka iyo qalal. Giisa assays arki kartaa hoos u dhac tirada limfosaytis iyo hoos u heer calcium. Si loo xaqiijiyo cudurka, waxaa lagama maarmaan ah si ay u fuliyaan waxbarashada xaaladda difaaca. Xaaladdan oo kale, waxaa jira isbedelka soo socda: la'aanta ah ee laga jawaabayo tallaalka iyo imtixaanada negative for diidmo iyo Isbadal lymphoblastic. Number of B-limfosaytis aan la beddeli karin. In daraasadda ee dhismayaasha difaac gacanta lagu arkay ay hoos, hoos u dhaqdhaqaaqa. On US thorax shaaca ka maqan yahay ama uu hoos u dhac ah parathyroid iyo qanjiro thymus. Dhismaha cilmi DNA u ogolaanaya in ay bartaan oo ku saabsan iskujoog oo koromosoom 22aad.

daaweynta cudurka Syndrome Di George ee

Pathology Daaweyntu waxay ku xidhan heerka uu xadgudub. Maqnaanshaha dhamaystiran oo thymus muujiyay badalida xubin. daaweynta Qalliinka waxaa lagama maarmaan ah in khalad daran ee xubnaha iyo nidaamka. In koorsada badnayn cudurka iyo la'aanta ah ee cillado daran qoray therapy dayactirka. Daawooyinka aasaasiga ah waa immunostimulants. Inta nolosha waxaa lagama maarmaan ah inay dib u buuxiyaan deficiency calcium ee jirka. cudurrada faafa codsan therapy astaamaha: antibiotics, daawooyinka anti-viral. Waxa intaa dheer in daawada, si looga fogaado in hypothermia, stress, iyo arrimo kale oo loo kasin.

Cawaaqibta aplasia of thymus ah

Syndrome Di Dzhordzhi waa khatar, sababtoo ah waxaa weeyaan badan oo dhibaatooyin. Bukaanka qaba cudurka this nugul geedi socodka buro, caabuq daran. Syndrome waa natiijo ah oo soo noqnoqda ee horumarinta xanuunada, shalalka. Tan waxa u sabab xaqiiqada ah in jidhka arkaa bukaanka leedahay unugyada wakiilada ajnabiga ah, oo uu bilaabo inuu la dagaallamo. Tani waxa ay keentey in cudurada sida lupus erythematosus oo nidaamsan, dermatomyositis, rheumatoid arthritis. Dhibaatooyinka soo socda uu dhici karaa in cillado daran ee horumarinta: dhimaal maskaxeed, curyaannimo of adimada, indho la'aan. Marka xanuunada convulsive laga yaabaa in neefta ay sabab u tahay qabatin.

saadaasha ee syndrome ee Di Dzhordzhi

Marka lagu daro deficiency difaaca iyo cilladaha muuqda qalfoofku wajiga loo geysto ogaanshaha: Di Dzhordzhi syndrome. Photos of qof oo qaba cudurkan waxaa lagu arki karaa in suugaanta ee caafimaadka ee gaarka ah. Iyada oo isku darka this ka mid ah calaamadaha saadaasha nolosha guud ahaan allifey oo sida aan caadi ahayn koritaan soo jeedin qaab daran ee cudurka. Caadi ahaan, caruurta la syndrome Di Dzhordzhi ah, ma ku nool yihiin da'da 10 ay sabab u dhaca cudurrada faafa daran ama kansarka. In the case of socodka sambabada, ma curyaanimo iyo daaweynta bedelka saadaasha ku filan waxaa laga yaabaa in ay faa'iido.

Ka Hortagga syndrome Di Dzhordzhi

ka hortagga gaar ah oo cudurkan kajirin. Si looga hortago xadgudubyada horumarinta SNMna of uurjiifka, hooyada la filayo u baahan tahay in aad xaddiddo arrimaha walwalka, la siiyo ilaa caadooyinka xun, laakiin sidoo kale si looga hortago infakshanka cudurada bakteeriyada iyo fayraska. aan caadi ahayn Developmental lagu ogaan karaa at ultrasound ah loo qorsheeyey, taas oo waa la fuliyay in saddex bilood kasta ee uurka.