Kartagener syndrome ee carruurta: ogaanshaha, sawir, daaweynta

Kartagener ee syndrome - cudur dhif ah ee lagu dhasho ay sababeen xadgudub ku ah motility cilia, yaal dusha sare ee unugyada badan epithelial. Foomka waxaa ka buuxa iin this waxaa caafimaad ahaan loo muujiyey by triad ka mid ah calaamadahan:

• bronchiectasis;
• hypoplasia (hypoplasia), hogaga paranasal, kaas oo la Ulasocto hogaga soo noqnoqonaysa,
• heshiis dambe oo ka mid ah xubnaha gudaha (inversus situs).

syndrome ayaa la magacaabay ka dib markii dhakhtarka Swiss Kartagener, kuwaas oo siiyey faahfaahsan sharaxaad ka mid ah gobolka ee 1933. Haddaba, bal aynu wada hadlaan si faahfaahsan weyn cudurka.

Sababaha Kartagener syndrome

Hadda waxaa la cadeeyay in syndrome Kartagener ayaa dabiiciga ah hidde la autosomal hab ah recessive dhaxalka. The salaysan cudurka yihiin qaldaad ku jira tiro ka mid ah hiddo in encode borotiinada gaar ah, oo loogu talagalay si loo hubiyo in shaqo ee cilia.

Sidaas darteed ka mid ah dhaqdhaqaaqa waa la dhimay ama lumay oo dhan - waxaa jira xaalad loo yaqaan "Tardive ciliary aasaasiga ah." Bartay in ka badan labaatan hiddo masuul ka ah horumarinta Kartagener syndrome. Ku xadgudubka shaqada wax kasta oo iyaga ka mid ah in utero keeni kara cudurada.

Sidee badan waa cudurka?

Sida kor ku xusan, Kartagener syndrome ee carruurta waa dhif aqrinaayo ee dhashay mid ka mid ah oo kaliya ka mid ah 16,000.

Waa in la ogaadaa in carruurta Pathology laga yaabaa in aysan muuqan, iyo ogaanshaha la sameeyo oo keliya dhowr bilood ama xataa sanado ka dib, markay soo koraya sawir dhalaalaya kiliinikada.

Calaamadaha Kartagener syndrome

calaamadaha syndrome Kartagener kala duwan yihiin wax calaamado ah in a sawir caafimaad oo kala duwan.

In the case of goob astaamo cudurka saxda ah waa mid aad u adag, oo ay dhacaan inta badan la ogaado by fursad marka beddelaaya ah xubnaha gudaha lagu jiro baadhitaan ku saabsan qaar ka mid ah cudurada kale.

bronchiectasis

Sida caadiga ah, ee geedka bronki (loox weyn ee iftiinka) waxa uu leeyahay qaab branching tubules la hoos uniform of dhexroor ee Lumen ah jihada ka overlying in ay qaybaha ku lammaan in ekaan taajkii ka yeellay-hoos ah.

Bronchiectasis (ama bronchiectasis) - ballaarinta a hoose ee Lumen ee hawomareenno nooca bacaha, spindles ama dhululubada. In hawomareenno ay maroorsamaan sida uu noqdo wax aan macquul aheyn dheecaan dhaqdhaqaaqa caadiga ah. Wuxuu stagnates, taas oo shaki la'aan waxay keeni doontaa in ay cudurka iyo horumarinta jawaabta bararka.

Caafimaad ahaan, bronchiectasis la Kartagener syndrome muujiyey qufac leh xaako expectoration purulent cagaaran, qandho ilaa 38 darajo iyo sare, tabar, madax-xanuun, mararka qaarkood ku biiri kartaa lallabo iyo matag. therapy antibiotic awood badan u oggolaanaysa in dib u soo kabashada dibadda, laakiin daawooyinka, kuwaas oo aan la baabi'iyo karaan sababta ugu weyn ee caabuq waa bronchiectasis maxaliga ah. Sidaa darteed, sawirka caafimaad oo qeexay si joogta ah soo noqnoqda, talantaalli ah la dura astaamo "iftiin". Waxaa uu noqonayaa Dabcan dheer iyo muddada dheer. Dabcan noqoshada Chronic xaaladda loo yaqaan bronchiectasis.

hogaga

muujinta kale oo Kartagener syndrome waa hogaga, ama caabuq ku JQSHM paranasal. In epithelium ciliary caadiga ah ee xuubka sanka iyo sanka ay sabab u tahay isbedbedelka ay bixiyaan dheecaan dhaqdhaqaaqa degto waxaa la Qurub ciidda iyo bakteeriyada. Kartagener syndrome Marka function cilia waa daciif ah, iyo waxyaabaha ku stagnates u furani, taasoo keenta caabuq.

Inta badan waxay yeeshaan hogaga, ama caabuq ku JQSHM maxillary, ugu yaraan - hore (sanka aad kaliga), ethmoiditis (unugyada Boogani trellised) iyo sphenoiditis (sanka sphenoid). Dhammaan dowlad-goboleedyada waxyaalahan waxaa loo muujiyey by qandho, madax-xanuun iyo sanka oo dareera malax. Hogaga waxaa sidoo kale lagu gartaa xanuun dhinacyadiisa baalashii sanka iyo dhabannada.

Heshiiskaasi dambe ee xubnaha gudaha

Heshiiskaasi dambe oo ka mid ah xubnaha gudaha, ama situs viscerusim kasoo horjeeddo - taasi waa muujinta ugu caan ku Kartagener syndrome, taas oo, si kastaba ha ahaatee, waxay dhacdaa in ka yar kala bar dadka bukaanka ah. Going dhaqdhaqaaqa xubnaha gudaha sida ay nooca mirroring. Kala heshiis dambe dhamaystiran oo aan dhamaystirnayn ee xubnaha gudaha.

heshiis dambe aan dhammeystirneyn waxaa ku bedesheen sambabada. Tani waxaa laga yaabaa in la socda, sida ay ahaayeen milicsiga muraayad qalbiga ku leh wareeg ah ee dhinaca midig ee saabka xabadka (dextrocardia) top.

Iyada oo heshiis dambe buuxda heysta muraayad ah in ay u guuraan oo dhan xubnaha gudaha. Xaaladdan oo kale, beerka waa bidixda, beeryarada - xaq. ogaanshaha shil of cudurada this by ultrasound lagu jiro baaritaanka joogtada ah oo kuu ogolaanaya in ay ka shakisan Kartagener syndrome markii astaamo.

Heshiiskaasi dambe oo ka mid ah xubnaha gudaha waa ay sabab u tahay xadgudub socdaalka ee unugyada embriyaha iyo unugyada lagu jiro embryogenesis. Hay'adaha badan oo sida caadiga ah ma yeeshaan in ay dhibic halka ay dhalmada ka dib. Sayidka, kelyaha dhigay ee gobolka miskaha iyo si tartiib ah ku kici ilaa feeraha heerka XI-XII ah.

Guuritaanka (ama socdaalka) ee meydadka muddada dhalmada ka hor waxaa lagu fuliyaa by cilia, Kartagener syndrome oo markii aan shaqeeya, kaas oo keena heshiis dambe oo ka mid ah xubnaha gudaha. Nasiib wanaag, sida haddii ay halis xaalad this ma uu eegi jirin, waa in kiisaska intooda badan ma noqonin mid horseeda in xadgudubyada la taaban karo ee nolosha ee u shaqeeyo.

daliilka kale ee Kartagener syndrome

Waxaa ka mid ah astaamaha kale ee cudurka waa dhalmo la'aan lab ugu muhiimsan. Waxaa keena muddaada dhibic ay sabab u tahay xaqiiqada ah in cilia ay ma shaqayn.

Bukaan-socodka ayaa sidoo kale rabshin laga yaabaa dhego xanuun soo noqnoqda iyo lumista maqalka. Tan waxa u sabab fadhiidka ah ee dheecaanka ku jira dhegta dhexe, kaas oo sida caadiga ah waa in la saaro unugyada xuubka epithelial ciliated.

Kartagener cudurka syndrome ee carruurta

Haddii ilmaha, laga bilaabo qiyaas ahaan da'da hal bil, si joogto ah la ildaran pneumonia, rhinitt iyo hogaga waa in la tuhunsan yahay Kartagener syndrome, cudurka of taas oo aan aad u adag. Waxaa ka mid ah tiro ka mid ah waxbarashada iyo shaybaarka,

• Baaritaanka jirka, ama baaritaan caafimaad ee caadiga ah, u muujin doonaa dhib ah ee neefsashada sanka, sawirka gaarka ah auscultatory ee sambabada iyo wadnaha, iyo sidoo kale isbeddelka phalanges terminal nooca "diggaagga," koorsada dheer ee geedi socodka.
• Baaritaanka X-ray muujin doonaa lesions bahal ah ee sambabada iyo nidaamka dambe ee wadnaha (dextrocardia) ah. Habkani waa mid fudud oo ammaan ah oo ku filan, sidaas darteed, waxay fursad si ay u fuliyaan ogaanshaha of Kartagener syndrome ee isbitaalka.
• Bronchoscopy - habka ugu saxsan ee baaritaan si loo ogaado bronchiectasis. Waxaa intaa dheer, keliya macnaha of Bronchoscopy qaadan kartaa biopsy ah xabka bronki.
• jarista xuubka tusi darnaanta geedi socodka bararka iyo qiimeeyo dhismeedka cilia.

Taasoo keentay cusbitaallada, jaamacadaha adduunka oo dhan ay yihiin wakhti dheer dheer hadlayaan horay Kartagener syndrome, sawir ka mid ah waxaa lagu soo bandhigay in this article. La kulmaan khubarada ugu caansan waxaa tababar kartaa jiilka ka yar ee dhakhaatiirta ah in la aqoonsado cudurka dhif noocan oo kale ah.

Daweynta Kartagener syndrome

Baan uga takhalusi karaa xaaladda this? Currently, daaweynta Kartagener ee syndrome waxay ku xiran tahay calaamadaha. Ma jirto daawo soo celin lahaa shaqo ee cilia ee unugyada, laakiin daawooyinka casriga ah ayaa Arsenal qani ku ah qalabka si ay u fududeeyaan koorsada cudurka. Iyadoo ay gargaar, bukaanka waxaa laga yaabaa in ilaawaan uu cudur dhif ah.

Hababka ugu weyn ee lagu daweeyo:

• Antibiyootikada. Daawooyinkan waxaa loo qoray, waayo, caabuq sambabada sababay bronchiectasis, iyo hogaga. Codso classical antibiotics penicillin, macrolides iyo daroogada ka tirsan ee fluoroquinolones "neefsashada".
• Hababka hagaajinta shaqada dheecaan of hawomareenno ah - biyo saare, massage, codsiga daroogada mucokinetic iyo mucolytic, iwm ...
• Daaweynta dabiiciga ah.

Marka joogitaanka bronchiectasis dhawaaqaa la boronkiitada inta badan soo noqnoqda iyo pneumonia daaweynta operative - saarista (resection) Meesha ugu weyn u saameysey ee sambabada. Ka dib markii xaalad noocaas ah hawlgalka bukaanka si xooga hagaagtay.