Syndrome Kartagener i roto i ngā tamariki: tātaritanga, photo, maimoatanga

syndrome o Kartagener - he mate congenital onge meinga e te takahi o ngä makawe motility, kei runga i te mata o maha pūtau epithelial. Ko te puka tonu o tenei koha e hauora whakakitea e te topu- o tohu:

• bronchiectasis;
• hypoplasia (hypoplasia), sinuses paranasal, haere tahi nei i pakohu kakā recurrent;
• te whakaritenga whakamuri o nga whekau (Pae inversus).

ingoa te syndrome te i muri i Kartagener tākuta Swiss, e hoatu i te taipitopito whakamārama o te kāwanatanga i roto i te 1933. Na, kia aparau i te i roto i taipitopito rahi te mate.

Okú Kartagener syndrome

Na kua whakamatauria reira e te syndrome Kartagener he āhua ira ki te autosomal aratau ngoikore o te tupu. Ko te pūtake o te mate, he mea kohakore, i roto i te maha o ngā ira e whakawaehere etahi pūmua, hangaia ki te whakarite i te mahi o te makawe iti.

Ka rite ki heke te he hua o to ratou nekeneke ngaro i te katoa ranei - i reira ko te huru i huaina "dyskinesia ciliary tuatahi." Ako neke atu i te rua tekau ira te kawenga mō te whanaketanga o Kartagener syndrome. Tūkinotanga o te mahi o tetahi o ratou i roto i te utero kia arahi ki māuiuitanga.

Kia pehea te noa ko te mate?

Ka rite ki whakahuatia i runga ake, he onge anake i roto i te kotahi anake hou i roto i o 16,000 Kartagener syndrome i roto i ngā tamariki.

Me kï reira e māuiuitanga ai tamariki e kore e whakaatu ano, a ka hanga te tātaritanga te i muri i anake i te marama torutoru ranei ara tau, ka whakawhanake i te pikitia kanapa haumanu.

Tohu o Kartagener syndrome

Kartagener tohu syndrome rerekē i kore tohu ki te pikitia haumanu motuhake.

I roto i te take o te tautuhinga asymptomatic tātaritanga tika he tino uaua, me e tupu tino maha kitea e tupono, ka kōaro nga whekau i roto i te rangahau e pā ana ki etahi atu mate.

bronchiectasis

Tikanga, te rakau arahau (te anga marama matua) e te ahua o te pekapeka tubules ki whakaiti ōrite o te diameter o te lumen i roto i te ahunga i te overlying ki te wāhanga waiwai e rite te karauna kōaro-iho.

Bronchiectasis (ranei bronchiectasis) - he roha rohe o te lumen o te bronchi momo peke, na auri e ipurau ranei. I roto i taua bronchi haka riro taea secretion kaupapa noa. stagnates ia, e te taniwha, ka arahi ki te mate, me te whanaketanga o te whakautu inflammatory.

Hauora, bronchiectasis ki Kartagener syndrome whakakitea mare ki expectoration purulent puwhero sputum, kirika ki runga ki te 38 nekehanga me te teitei, ngoikore, ānini, i ētahi wā ka taea e uru whakapairuaki, me te ruaki. Mano therapy paturopi taea mo te ora waho, engari e kore e taea enei taero faaore i te take matua o te mumura he bronchiectasis rohe. Na reira, ko te pikitia haumanu whakaahuatia toutou toutou, tētahi ki te wā asymptomatic "marama". riro te reira i te akoranga roa, me te tautohetia. akoranga paipa ma'i o te huru i huaina bronchiectasis.

pakohu kakā

Ko tētahi atu faaiteraa o Kartagener syndrome he pakohu kakā, ranei mumura o te sinuses paranasal. I roto i te epithelium ciliary noa o te mucosa nasal me pakohu tika ki ona rerekē whakarato noho kaupapa secretion runga taua mea ki matūriki puehu me te huakita. Kartagener syndrome, no te te waimaero ngä makawe mahi, me te tirotiro stagnates i te sinuses, meinga mumura.

Nuinga o ngā wā whakawhanake pakohu kakā, ranei mumura o nga sinuses maxillary, i te iti rawa - mua (pakohu frontal), ethmoiditis (pūtau o te poroiwi trellised) me sphenoiditis (pakohu rahirahinga). E whakakitea enei āhua katoa i te kirika, ānini me te ihu mātihetihe ki pirau. Kei te hoki āhuatanga Sinusitis i te mamae i runga i nga taha o nga parirau o te ihu, me nga paparinga.

Ko te whakaritenga whakamuri o whekau

Ko te whakaritenga whakamuri o nga whekau, Pae viscerusim versus ranei - ko te faaiteraa tino āhuatanga o Kartagener syndrome, e, Heoi, puta i roto i iti iho i te hawhe o te tūroro tenei. Haere kaupapa o whekau rite ki te momo o te Hangarite. Te wehe oti a oti whakaritenga whakamuri o whekau.

E I Whakawhiti whakaritenga whakamuri pahara pūkahukahu. Tenei kia haere tahi ai, me te mea he whakaata whakaata o te ngakau ki te neke i roto i tona runga taha matau o te kōhao pouaka (dextrocardia).

Ki te whakaritenga whakamuri tonu mau te whakaata ki te neke i te whekau katoa. I roto i tenei take, ko te ate i runga i te maui, te kōateate - te tika. te kitenga kōpeka o tenei māuiuitanga i ultrasound i roto i te whakamātautau mahi, me te taea ki te whakapae Kartagener syndrome ka asymptomatic.

Ko te whakaritenga whakamuri o nga whekau he tika ki te takahi o te hekenga o ngā pūtau embryonic me te kopa i roto i embryogenesis. He maha ngā pokapū e kore tikanga e whakawhanake ki te wāhi i reira he i muri i te whanautanga ratou. Ko te kupu, whakatakotoria tākihi i roto i te rohe papatoiake, me te āta whakatika ake ki nga rara XI-XII taumata.

Moving (hekenga ranei) o te tinana i roto i te wā prenatal kawea i roto i ngä makawe, Kartagener syndrome nei, no te kore mahi, e ai te whakaritenga whakamuri o nga whekau. Aua'e, me te mea whakawehi tenei huru kihai i titiro, he mea i roto i te nuinga o te wā e kore e arahi ki maumaui nui o te ora o te rauropi.

Ētahi atu faaiteraa o Kartagener syndrome

I roto i te tahi atu tohu o te mate, ko te te pākoko tane tino nui. Kei te meinga te reira na roto i te immobility o parāoa tika ki te meka e kore ratou makawe iti e mahi.

kia hoki whakakorikoria tūroro mate taringa recurrent, me te rongo mate. Ko e tika ana ki te stagnation o secretions i roto i te taringa waenga, e te tikanga me nekehia ciliated pūtau mucosal epithelial tenei.

Kartagener tātaritanga syndrome i roto i ngā tamariki

Ki te, tīmata a tawhio noa te tau o tetahi marama, mamae wā he tamaiti i te kakā, Rhinitis me te pakohu kakā kia whakapae Kartagener syndrome, e kore he tino uaua te tātaritanga o nei. Kei roto te reira i te maha o rangahau mauhaa me te taiwhanga:

• whakamātautau tinana, whakamātautau mau hauora ranei, ka whakakitea te fifi o nasal manawa, te pikitia auscultatory motuhake i roto i te pūkahukahu, me te ngakau, kia rite ki te pai kia rite ki te huringa i roto i phalanges kāpeka o "ngā waewae" momo i roto i te akoranga roa o te tukanga.
• X-ray whakamātautau ka whakakitea i te pāpaka pathological i roto i te pūkahukahu, me te whakaritenga whakamuri o te ngakau (dextrocardia). Ko te kuware, me te haumaru nui tenei tikanga, koia, hanga ana e ia taea ki te kawe i roto i te tātaritanga o Kartagener syndrome i te fare ma'i.
• Bronchoscopy - te tikanga tika tino o te tūhuratanga ki te kitea bronchiectasis. Ano, anake i tikanga o bronchoscopy taea te tango i te unuhanga o te mucosa arahau.
• whakaatu biopsies mucosal te pakeke o te tukanga inflammatory me te aromatawai i te hanganga ngä makawe iti.

Kei te matapaki ārahi ngā hōhipera me ngä whare wänanga huri noa te ao rawa i te wa roa kē Kartagener syndrome, he whakaahua o e e te aroaro i roto i tenei tuhinga. Wheako ki te tohunga tino rongonui taea ako'i reira te whakatupuranga o muri o ngā tākuta ki te mohio ki te taua mate onge.

Waihanga ki Kartagener syndrome

Ka taea e ahau te maka o tenei huru? I tēnei wā, maimoatanga o te syndrome o Kartagener tei runga i te tohu. Kahore he rongoā e e whakahoki te mahi o te makawe iti o ngā pūtau epithelial, engari rongoā hou he täoro taonga o taputapu ki te whakahaere i te akoranga o te mate. Ki te to ratou awhina, kia wareware te manawanui e pā ana ki tona mate onge.

Ko te tikanga matua o te maimoatanga:

• Paturopi. meinga e whakaritea mo te mumura o te pūkahukahu enei taero bronchiectasis, me pakohu kakā. Hoatu puāwaitanga paturopi rongoā paturopi, macrolides me taero i te rōpū o "romahā" fluoroquinolones.
• Methods o te whakapai ake i te mahi pana o nga bronchi - waikeri postural, mirimiri, tono taero mucokinetic me mucolytic, aha ...
• I tua.

A, no te te aroaro o te bronchiectasis korerotia ki Bronchitis pinepine recurrent me kakā maimoatanga operative - tango (resection) Ko te wāhi tino pā o te pukapuka. I muri ake te taua huru mahi o te tūroro nui.