Kartagener ciwo a yara: ganewar asali, photo, magani

Kartagener ta ciwo - a rare nakasar cuta lalacewa ta hanyar wani take hakkin cilia motility, located a farfajiya na da yawa epithelial Kwayoyin. Cikakken tsari na wannan aibi ne asibiti bayyana ta a triad na bayyanar cututtuka:

• bronchiectasis.
• hypoplasia (hypoplasia), paranasal sinuses, wanda yana tare da maimaita sinusitis.
• da baya da tsari na kayan ciki (situs inversus).

A ciwo ne mai suna bayan Kartagener Swiss likita, wanda ya ba da cikakken bayanin irin jihar a 1933. Yanzu, bari mu tattauna a zurfafe cutar.

Sa Kartagener ciwo

Yanzu an tabbatar da cewa Kartagener ciwo yana da wani kwayoyin halitta da wani autosomal recessive yanayin na gādo. Dalili na cuta ne lahani a yawan kwayoyin halittu da cewa encode wasu sunadarai, tsara don tabbatar da aiki na cilia.

A sakamakon su motsi an rage ko rasa a duk - akwai wani yanayin da ake kira "primary ciliary dyskinesia." Karatu fiye da ashirin genes alhakin ci gaban Kartagener ciwo. Take hakkin da aikin wani daga cikinsu a utero iya haifar da Pathology.

Yadda kowa ne cuta?

Kamar yadda aka ambata a sama, Kartagener ciwo a yara ne rare -only a daya kawai jariri daga 16,000.

Ya kamata a lura da cewa yara na iya Pathology ba bayyana kanta, da kuma bincike da aka yi ne kawai, bayan 'yan watanni ko ma shekaru, lokacin da yake bunkasa a haske na asibiti hoto.

Alamun Kartagener ciwo

Kartagener ciwo cututtuka bambanta daga wani cututtuka ga wani jinsin na asibiti hoto.

A cikin hali na asymptomatic saitin daidai ganewar asali ne musamman wuya, kuma ya auku mafi sau da yawa gano kwatsam a lokacin da reversing da kayan ciki a lokacin da wani binciken game da wasu sauran cututtuka.

bronchiectasis

Yadda aka saba, da Bronchial itacen (babban haske frame) yana da nau'i na sashe tubules tare da uniform rage daga cikin diamita na lumen a cikin shugabanci daga overlying zuwa muhimmi sassan da yayi kama da wani kifar-saukar da kambi.

Bronchiectasis (ko bronchiectasis) - a gida fadada daga cikin lumen na bronchi irin bags, spindles ko cylinders. A cikin irin wannan maras kyau bronchi zama ba zai yiwu ba al'ada motsi mugunya. Ya stagnates, wanda zai babu makawa kai ga kamuwa da cuta da kuma ci gaban da kumburi martani.

Asibiti, bronchiectasis da Kartagener ciwo bayyana tari da expectoration greenish surkin jini sputum, zazzabi up to 38 darajõji, kuma mafi girma, wani rauni, ciwon kai, wani lokacin za su iya shiga tashin zuciya da kuma amai. Iko kwayoyin far damar domin na waje dawo, amma wadannan kwayoyi ba zai iya kawar da babban dalilin da kumburi ne na gida bronchiectasis. Saboda haka, na asibiti hoto ya bayyana akai-akai maimaita, alternating tare da "hasken" asymptomatic jinkiri. Yana zama mai tsawo da kuma protracted shakka. Kullum relapsing shakka daga cikin yanayin da ake kira bronchiectasis.

sinusitis

Wani bayyanuwar Kartagener ciwo ne sinusitis, ko kumburi da paranasal sinuses. A al'ada ciliary epithelium na hanci mucosa da sinus saboda da hawa da sauka samar da motsi mugunya shirya a kan shi tare da wãtsarwa da kwayoyin. Kartagener ciwo Lokacin cilia aiki ne sosai, da kuma abinda ke ciki stagnates a cikin sinuses, haddasa kumburi.

Mafi sau da yawa ci gaba sinusitis, ko kumburi da maxillary sinuses, akalla - gaban (frontal sinus), ethmoiditis (sel a rumfuna labyrinth) da kuma sphenoiditis (sphenoid sinus). Duk wadannan jihohi suna bayyana da zazzabi, ciwon kai da kuma zubar hanci da ruɓaɓɓen jini. Sinusitis ne ma halin da zafi a tarnaƙi na fikafikan hanci da kumatunta.

A baya tsari na kayan ciki

A baya tsari na kayan ciki, ko situs viscerusim gaban - wannan shi ne mafi halayyar bayyanuwar Kartagener ciwo, wanda, duk da haka, na faruwa a kasa da rabin da marasa lafiya. Za motsi na ciki gabobin bisa ga irin mirroring. Rarrabe cikakken da kuma bai cika ba kuma baya da tsari na kayan ciki.

Bai cika kuma baya da tsari ake swapped huhu. Wannan na iya zama tare, kamar yadda suka madubi tunani na zuciya da motsi a saman dama gefen daga cikin kirji rami (dextrocardia).

Tare da cikakken baya tsari riqe da madubi don matsar da duk kayan ciki. A wannan yanayin, da hanta ne a kan hagu, da baƙin ciki - dama. Mai haɗari ganewa na wannan Pathology da duban dan tayi a lokacin wani zaman yau da kullum jarrabawa da kuma damar zuwa zargin Kartagener ciwo a lokacin da asymptomatic.

A baya tsari na kayan ciki ne saboda take hakkin da hijirarsa daga amfrayo Kwayoyin kuma tsokoki a lokacin embryogenesis. Mutane da yawa hukumomin kullum ba ci gaba ga zance inda su ne bayan da haihuwa. Saboda haka, koda aza a pelvic yankin da kuma sannu a hankali tashi kan matakin XI-XII hakarkarinsa.

Motsi (ko hijirarsa) na gawawwakin a lokacin prenatal lokacin da aka za'ayi da cilia, Kartagener ciwo wanda idan ba su aiki, wanda ya haddasa wani kuma baya da tsari na kayan ciki. Abin farin, kamar yadda idan barazanar wannan yanayin bai duba, shi ne a mafi yawan lokuta ba ya haifar da gwaji take hakki na rayuwa da kwayoyin.

Sauran manifestations Kartagener ciwo

Daga cikin sauran cututtuka da cuta ne mafi muhimmanci namiji rashin haihuwa. Yana ne ya sa ta immobility maniyyi saboda gaskiyar cewa su cilia ba aiki.

Marasa lafiya na iya ma ta da maimaita kunne cututtuka da kuma ji hasara. Wannan shi ne saboda da stagnation na secretions a tsakiyar kunne, wanda kullum dole ne a cire ciliated epithelial mucosal Kwayoyin.

Kartagener ciwo ganewar asali a cikin yara

Idan yaro, da suka fara a kusa da shekaru daya watan, a kai a kai shan wahala daga ciwon huhu, rhinitis da kuma sinusitis ya kamata a da ake zargi Kartagener ciwo, da ganewar asali daga wanda ba sosai rikitarwa. Yana hada da wani yawan instrumental da dakin gwaje-gwaje karatu:

• Jiki jarrabawa, ko talakawa likita jarrabawa, za ta bayyana da wahala daga hanci numfashi, da takamaiman auscultatory hoto a cikin huhu da zuciya, kazalika da canji a cikin m phalanges na "drumsticks" type a cikin dogon shakka daga cikin tsari.
• X-ray jarrabawa za ta bayyana da pathological raunuka a cikin huhu da baya da tsari na zuciya (dextrocardia). Wannan hanya mai sauki ne, kuma mai lafiya isa, saboda haka, ya sa ya yiwu a gudanar da ganewar asali Kartagener ciwo a asibiti.
• Bronchoscopy - mafi m Hanyar bincike a gane bronchiectasis. Haka kuma, kawai ta wajen bronchoscopy iya daukar wani biopsy na Bronchial mucosa.
• mucosal biopsies nuna tsanani daga cikin kumburi tsari da kuma tantance cilia tsarin.

Jagoranci asibitoci da kuma jami'o'i a duniya an tattauna quite lokaci mai tsawo riga Kartagener ciwo, wani photo na wanda aka gabatar a wannan labarin. Experience da shahararrun masana da shi zai iya horar da ƙaramin tsara likitoci don gane da irin wannan rare cuta.

Zalunta Kartagener ciwo

Zan iya rabu da wannan yanayin? A halin yanzu, magani na Kartagener ta ciwo ya dogara da cututtuka. Babu magani da cewa zai mayar da aikin na cilia na epithelial Kwayoyin, amma zamani magani yana da wani arziki arsenal na kayan aikin zuwa sauƙaƙe mana da cutar. Tare da su taimako, da mãsu haƙuri iya manta game da m rashin lafiya.

Babban hanyoyin da magani:

• Maganin rigakafi. Wadannan kwayoyi suna wajabta kumburi daga cikin huhu ya sa bronchiectasis, da kuma sinusitis. Aiwatar gargajiya maganin rigakafi penicillin, macrolides da kwayoyi daga cikin rukuni na "numfashi" fluoroquinolones.
• Hanyar na inganta da magudanun aiki na bronchi - postural magudanun ruwa, tausa, aikace-aikace mucokinetic da mucolytic kwayoyi, da dai sauransu ...
• Physiotherapy.

Lokacin gaban furta bronchiectasis da akai-akai maimaita mashako da kuma ciwon huhu gudanar da magani - kau (resection) The mafi shafa yankin na huhu. Bayan irin wannan aiki yanayin marasa lafiya inganta ma.