Kartagener syndrome ezinganeni: ukuxilongwa, isithombe, ukwelashwa

Kartagener Syndrome - isifo ezingavamile azalwa naso kubangelwa kuphula umthetho we cilia motility, esisogwini ebusweni amaseli amaningi EPITHELIAL. Ifomu egcwele le nkinga is isifo owabonakaliswa a triad izimpawu:

• bronchiectasis;
• hypoplasia (hypoplasia), sinuses paranasal, okuyinto kuhambisana sinusitis yansukuzonke;
• ilungiselelo reverse kwezitho zangaphakathi (isayithi yeSithuba inversus).

I sifo siqanjwe ngo-Kartagener udokotela Swiss owavuma enemininingwane incazelo isimo ngo-1933. Manje, ake uxoxe ngokuningiliziwe lesi sifo.

Ubangela Kartagener syndrome

Manje it is wafakazela ukuthi Kartagener syndrome has a imvelo zofuzo nge autosomal imodi esikhubazayo yefa. Ngesisekelo lesi sifo yilabo yokulimala eziningana zofuzo encode amaprotheni ezithile, eziklanyelwe ukuqinisekisa umsebenzi cilia.

Ngenxa yalokho ukuhamba kwabo kuyehla noma ilahleke nhlobo - kukhona isimo ngokuthi "eyinhloko ciliary dyskinesia." Ngatadisha izakhi zofuzo engaphezu kwamashumi amabili obhekene nokuthuthukiswa Kartagener syndrome. Ukwephula umsebenzi yimuphi kubo utero kungaholela egunjini lokuhlolwa kwezifo.

Uvame kangakanani lesi sifo?

Njengoba kushiwo ngenhla, Kartagener syndrome ezinganeni ezingavamile -only e olusanda kuzalwa eyodwa kuphela kuzo 16.000.

Kufanele kuqashelwe ukuthi izingane zingase Pathology akusho ukuzibonakalisa, futhi inkinga itholakala kuphela ngemva kwezinyanga ezimbalwa noma iminyaka, uma ethuthukisa isithombe esikhanyayo emtholampilo.

Izimpawu Kartagener syndrome

Kartagener izimpawu syndrome ziyahlukahluka kusuka khona izimpawu ukuze isithombe abahlukene emtholampilo.

Endabeni kulungiselelwa asymptomatic okuyikona kuba nzima kakhulu, futhi kwenzeka kaningi kakhulu ukutholwa ithuba lapho elungisa kwezitho zangaphakathi phakathi ucwaningo mayelana ezinye nezinye izifo.

bronchiectasis

Ngokuvamile, isihlahla bronchial (main ukukhanya uzimele) ine uhlobo ekhelini tubules nge ukunciphisa umfaniswano ububanzi we Isikhala sayo esingaphakathi e isiqondiso esivela overlying ukuze izigaba ezibangela ufana umqhele sibheke phansi.

Bronchiectasis (noma bronchiectasis) - ukwanda endawo mafutha anciphisa isikhala esingaphakathi yohlobo bronchi izikhwama, nokwakha uthi lokuphotha noma amasilinda. In esinjalo bronchi sikhubazekile, kusuka kube nzima evamile ukunyakaza uketshezi. Yena stagnates, okuyinto nakanjani kuyoholela ukutheleleka kanye nokuthuthukiswa impendulo ukuvuvukala.

Emtholampilo, bronchiectasis nge Kartagener syndrome wabonakaliswa ukukhwehlela ne expectoration ubovu ogqamile isikhwehlela, imfiva kuze kube degrees 38 nangaphezulu, ubuthakathaka, ikhanda elibuhlungu, ngezinye izikhathi angajoyina isicanucanu nokuhlanza. Inamandla yokwelapha ngemithi elwa namagciwane ivumela yokutakula zangaphandle, kodwa le mithi ngeke ayiqede inkinga yokuntuleka imbangela eyinhloko ukuvuvukala kukhona bronchiectasis wendawo. Ngakho-ke, isithombe emtholampilo lochazwe ngokuphindaphindiwe ngokuphindaphindiwe, kushintshana ne "ukukhanya" ngezikhawu asymptomatic. Kuba Yiqiniso eside futhi eyayizothatha isikhathi eside. Okungalapheki relapsing inkambo isimo ngokuthi bronchiectasis.

sinusitis

Enye yezindlela uJehova alubonisa ngayo Kartagener syndrome sinusitis, noma ukuvuvukala sinuses paranasal. Ngo evamile ciliary epithelium we mucosa acinene futhi itheku ngenxa iyehla iyenyuka yayo ukuhlinzeka ukunyakaza uketshezi kuxazululwe on ngayo uthuli namagciwane. Kartagener syndrome Lapho cilia umsebenzi kahle, futhi okuqukethwe stagnates ku sinuses, okubangela ukuvuvukala.

Ngokuvamile ukuthuthukisa sinusitis, noma ukuvuvukala sinuses maxillary, okungenani - phambi (itheku frontal), ethmoiditis (amaseli uxhaxha trellised) kanye sphenoiditis (itheku sphenoid). Zonke lezi uthi kuboniswa imfiva, ikhanda kanye amakhala yisisu kuvuze ubomvu. Sinusitis nakho libhekene ubuhlungu ezinhlangothini kwamaphiko ekhaleni futhi izihlathi.

Okuphambene ilungiselelo yezitho zangaphakathi

Leli lungiselelo reverse izibilini, noma isayithi yeSithuba viscerusim Ukuqhathanisa - lena ukubonakaliswa isici iningi Kartagener syndrome, Nokho, okuyinto, kwenzeka ngesikhathi esingaphansi kwesigamu iziguli. Ukuya ukunyakaza kwezitho zangaphakathi ngokuvumelana uhlobo isibuko. Hlukanisa okuphelele futhi ayiphelele reverse ilungiselelo yezitho zangaphakathi.

Akuphelele reverse ilungiselelo kuthiwa ushintshanisiwe emaphashini. Lokhu kungase kuhambisane kunjengokungathi reflection esibukweni inhliziyo nge shift ehlangothini lwawo lesidla le ingaphakathi esifubeni (dextrocardia).

Nge ilungiselelo umnyuziki reverse ophethe esibukweni ukuhambisa zonke izitho zangaphakathi. Kulokhu, isibindi ingakwesokunxele, ubende - ngakwesokudla. Ukuthola kwengozi lokhu zokugula by ultrasound ngesikhathi luhlolo isimiso evumela ukuba usola Kartagener syndrome lapho asymptomatic.

Leli lungiselelo reverse izibilini kungenxa yokwephulwa yokufuduka ngamangqamuzana ombungu nezicubu ngesikhathi embryogenesis. ejensi abaningi ngokuvamile musa zikhula zize bakuphi esezele. Ngakho, izinso sibekwa esifundeni okhalo futhi kancane kancane bavuke ezingeni XI-XII izimbambo.

Ukuhambisa (noma kwenye indawo kwe) yemizimba ngesikhathi besakhulelwe Kwenziwa cilia, Kartagener syndrome okuyinto lapho kodwa angasebenzi, elibangela ilungiselelo reverse of the izitho zangaphakathi. Ngenhlanhla, njengoba uma esongela kulesi simo akazange ababukele, kuba ezimweni eziningi aliholeli yokuphula eningi zokuphila umzimba.

Okunye kuwukubonakaliswa Kartagener syndrome

Phakathi kwezinye izimpawu zesifo iyona ukungazali besilisa ezibaluleke kakhulu. Sibangelwa baxhashazwe kwesidoda ngenxa yokuthi cilia yabo musa ukusebenza.

Iziguli ingase futhi ukuphazamisa yansukuzonke indlebe izifo kanye ubuthulu. Lokhu kungenxa yokujama tsi of uketshezi maphakathi nendlebe, ngokuvamile kumelwe kususwe cells ciliated EPITHELIAL mucosal.

Kartagener syndrome uphethwe ngo izingane

Uma ingane, ukuqala bezungeza engu-ngenyanga eyodwa, njalo ohlushwa i-pneumonia, rhinitis futhi sinusitis kufanele esolwa Kartagener syndrome, sitholakele engesiyo oyinkimbinkimbi kakhulu. Kuhlanganisa eziningi izifundo wezinsimbi futhi laboratory:

• behlolwa, noma luhlolo abavamile yezokwelapha, kuyokwembula ukuthi kunzima acinene ukuphefumula, ethize isithombe auscultatory emaphashini futhi inhliziyo, kanye ushintsho phalanges esibulalayo "drumsticks" uhlobo ngokuhamba eside yenqubo.
• -X-ray luhlolo kuyokwembula izilonda sokugembula emaphashini nelungiselelo reverse inhliziyo (dextrocardia). Le ndlela ilula futhi ephephile ngokwanele, ngakho-ke, wenza sikwazi ukufeza sitholakele Kartagener syndrome esibhedlela.
• Bronchoscopy - indlela inembe kakhulu kunazo zonke uphenyo ukuthola bronchiectasis. Ngaphezu kwalokho, kuphela esebenzisa bronchoscopy kungathatha biopsy we mucosa bronchial.
• biopsies mucosal ukukhombisa kobukhulu ukuvuvukala futhi zihlole cilia isakhiwo.

izibhedlela eziholayo amanyuvesi emhlabeni jikelele baxoxisana Adalberto isikhathi eside kakade Kartagener syndrome, isithombe zazo okwethulwa kulesi sihloko. Batfola ochwepheshe edume kakhulu ke ungakwazi ukuqeqesha esizukulwaneni esisha of odokotela ukuqaphela isifo ezingavamile ezifana.

Ukwelapha Kartagener syndrome

Ngingaba ukuqeda lesi simo? Okwamanje, ekwelapheni Kartagener Syndrome incike izimpawu. Akukho imithi ukuthi uzobuyisela umsebenzi we cilia lamaseli EPITHELIAL, kodwa kwezokwelapha zanamuhla has a arsenal ocebile amathuluzi lula Yiqiniso lesi sifo. Ngosizo lwabo, isiguli singase sikhohlwe ngesifo sakhe ezingavamile.

Izindlela eziyinhloko ukwelashwa:

• Imithi elwa namagciwane. Le mithi kuyabekwa ukuvuvukala emaphashini okubangelwa bronchiectasis, futhi sinusitis. Sebenzisa classical imithi elwa namagciwane penicillin akuzange kusize, macrolides nezidakamizwa kusukela iqembu "zokuphefumula" fluoroquinolones.
• Izindlela zokwenza ngcono umsebenzi amanzi ze- bronchi - amanzi postural, massage, isicelo izidakamizwa mucokinetic futhi mucolytic, njll ...
• Physiotherapy.

Lapho khona bronchiectasis liphinyiselwe nge ukucinana kwemigudu yokuphefumula njalo yansukuzonke kanye nenyumoniya ukwelashwa op - ukususwa (resection) Le ndawo ethintekile kakhulu wamaphaphu. Ngemva isimo esinjalo ukusebenza iziguli ngcono kakhulu.