Kartagener syndrome ana: matenda, chithunzi, chithandizo

Kartagener ndi matenda - ndi chosowa kobadwa nako matenda chifukwa cha kuphwanya cilia motility, yomwe ili pamwamba pa maselo ambiri zaminyewa. Mawonekedwe zonse vuto ili kukomoka akuwonetseredwa ndi wa ku zizindikiro:

• bronchiectasis;
• hypoplasia (hypoplasia), paranasal sinuses, amene anatsagana ndi sinusitis kobwereza;
• n'zosiyana makonzedwe a ziwalo mkati (situs inversus).

Matendawa chikutchedwa Kartagener Swiss dokotala, amene anapereka zambiri ponena za boma mu 1933. Tsopano, tiyeni tikambirane mwatsatanetsatane matenda.

Chimayambitsa matenda Kartagener

Tsopano izo imatsimikizirika kuti syndrome Kartagener ali ndi chikhalidwe majini ndi autosomal recessive akafuna cholowa. Chifukwa cha matenda ndi zopindika nambala imene imachititsa kuti encode mapuloteni zina, cholinga kuonetsetsa ntchito ya cilia.

Chifukwa cha kuyenda awo yafupika kapena anataya konse - pali chikhalidwe wotchedwa "chachikulu ciliary dyskinesia." Anaphunzitsidwa majini zoposa makumi awiri udindo chitukuko cha matenda Kartagener. Violation ntchito ya aliyense wa iwo mu utero kungayambitse kudwala.

Kodi wamba matenda?

Monga tanena Kartagener syndrome ana ali osowa -only mu wakhanda mmodzi yekha pa 16,000.

Tikumbukenso kuti ana akhoza kudwala alibe kuonekera, ndi matenda anapangidwa pambuyo miyezi ingapo kapena zaka, pamene kukhala ndi yowala chithunzi matenda.

Zizindikiro za matenda Kartagener

Kartagener syndrome zizindikiro zimasiyana popanda zizindikiro ndi osiyana chithunzi matenda.

Pankhani ya asymptomatic kolowera matenda lolondola ndi kovuta kwambiri, ndipo zimachitika nthawi zambiri wapezeka mwangozi pamene kuchotseratu ndi ziwalo pa kafukufuku wa nkhani za matenda ena.

bronchiectasis

Mwachibadwa, ndi mtengo bronchial (waukulu kuwala chimango) ali mawonekedwe a nthambi tubules ndi yunifolomu kuchepetsa m'mimba mwake wa lumen mu malangizo ochokera kwa overlying ku chigawo amabweretsa akufanana chadodolido korona.

Bronchiectasis (kapena bronchiectasis) - ka kufutukuka m'dera wa lumen wa bronchi mtundu matumba, spindles kapena zonenepa. Mu bronchi ngati opunduka kumakhala kovuta yachibadwa kayendedwe katulutsidwe. Iye stagnates, amene n'kogwiritsa matenda ndi chitukuko mmene yotupa.

Kukomoka, bronchiectasis ndi matenda Kartagener akuwonetseredwa chifuwa ndi expectoration Kamaoneka purulent sputum, malungo mpaka madigiri 38 ndipo apamwamba, kufooka, mutu, nthawi zina agwirizane kunyansidwa ndi kusanza. Wamphamvu mankhwala mankhwala amalola kuti apeze kunja, koma mankhwala sangakwanitse kuthetsa chifukwa chachikulu cha kutupa ali m'dera bronchiectasis. Choncho, chithunzi matenda anatchulidwa mobwerezabwereza mobwerezabwereza, alternating ndi "kuwala" intervals asymptomatic. Kumakhaladi yaitali ndi protracted Inde. Matenda relapsing Inde chikhalidwe wotchedwa bronchiectasis.

sinusitis

mawonetseredwe wina wa matenda Kartagener ndi sinusitis, kapena kutupa m'njira za m'mphuno paranasal. Mu yachibadwa ciliary epithelium wa amphuno mucosa ndi nkusani chifukwa kusinthasintha ake kupereka kayendedwe katulutsidwe kukhazikika pa izo ndi fumbi ndi mabakiteriya. Kartagener syndrome Pamene cilia ntchito ndi opuwala, ndipo zili stagnates mu sinuses, ndi kuchititsa kutupa.

Nthawi zambiri kukhala sinusitis, kapena kutupa m'njira za m'mphuno maxillary, osachepera - kutsogolo (mazunzo nkusani), ethmoiditis (maselo a kukhotakhota trellised) ndi sphenoiditis (sphenoid nkusani). limati zonsezi anaonetsa malungo, kupweteka chimfine ndi mafinya. Sinusitis amadziwikanso yodziwika ndi ululu m'mbali mwa mapiko a mphuno ndi masaya.

N'zosiyana makonzedwe a ziwalo

Kakonzedwe n'zosiyana ziwalo mkati, kapena situs viscerusim Versus - ichi kwambiri khalidwe chiwonetsero cha Kartagener syndrome, amene Komabe, amapezeka osachepera theka la odwala. Kupita kayendedwe ka ziwalo molingana ndi mtundu wa mirroring. Kusiyanitsa wathunthu ndi chosakwanira n'zosiyana makonzedwe a ziwalo.

Chosakwanira n'zosiyana dongosolo ndi swapped mapapo. Izi zikhoza kukhala limodzi, ngati galasi chinyezimiro cha mtima ndi olakwika mbali pamwamba lamanja la pachifuwa M'mimbamo (dextrocardia).

Ndi makonzedwe zonse n'zosiyana akugwirizira galasi kupita ziwalo zonse za mkati. Pankhaniyi, chiwindi ndi kulamanzere, ndulu - lamanja. Mwangozi kudziwika kudwala izi ultrasound pa kudzifufuza nthawi ndi limalola kuganiza syndrome Kartagener pamene asymptomatic.

Kakonzedwe n'zosiyana ziwalo mkati ndi chifukwa kuphwanya kusamuka maselo embryonic ndipo zimakhala pa embryogenesis. mabungwe ambiri Mwachibadwa bbwino poti ali pambuyo kubadwa. Choncho, impso anaika mu dera m'chiuno ndipo pang'onopang'ono mbakwira mlingo XI-XII nthiti.

Kupita (kapena kusamuka) wa matupi m'nthawi akalandire chithandizo ikuchitika ndi cilia, Kartagener syndrome omwe pamene kugwira ntchito zomwe zimabweretsa dongosolo n'zosiyana ziwalo mkati. Mwamwayi, ngati kuopseza chikhalidwe ichi sankanyoza, ndi nthawi zambiri sichichititsa kuyan'anila yokulirapo ya moyo wa chamoyo.

zinthu zina syndrome Kartagener

Mwa zizindikiro zina za matendawa ndi zofunika kwambiri mwamuna osabereka. Iwo amayamba ndi kulephera kuyenda la umuna chifukwa chakuti cilia awo silingayende.

Odwala komanso kusokoneza matenda kobwereza khutu ndi imfa kumva. Izi ndi chifukwa stagnation wa secretions khutu pakati, chimenenso chimafotokoza ayenera kuchotsedwa ciliated zaminyewa maselo mucosal.

Kartagener syndrome matenda ana

Ngati mwana, kuyambira padziko m'badwo wa mwezi umodzi, nthawi zonse amadwala chibayo, rhinitis ndi sinusitis ayenera amaganiziridwa Kartagener matenda, matenda a omwe si yovuta kwambiri. Pamafunikanso maphunziro ambiri a malangizo ndi zasayansi:

• Kupenda thupi, kapena zachipatala Kupenda wamba, ati awulule vuto kupuma m'mphuno, mndandanda auscultatory chithunzi mu Mapapo ndi mtima komanso kusintha phalanges osachiritsika a "drumsticks" mtundu mu njira yaitali kwa ndondomekoyi.
• X-ray Kupenda adzafotokoza zotupa pathological mu mapapo ndi makonzedwe n'zosiyana kwa mtima (dextrocardia). Njira yosavuta otetezedwa mokwanira Choncho, limachititsa kuti agwire matenda a syndrome Kartagener kuchipatala.
• Bronchoscopy - njira yolondola kwambiri kufufuza kudziwa bronchiectasis. Komanso, mwa bronchoscopy akhoza kutenga biopsy wa mucosa bronchial.
• biopsies mucosal kusonyeza kuopsa kwa ndondomeko yotupa adziwe cilia dongosolo.

Tonga zipatala ndi mayunivesite padziko lonse mukukambirana ndithu nthawi yayitali kale Kartagener syndrome, chithunzi cha zimene zili m'nkhani ino. Nazo akatswiri otchuka izo angaphunzitse achinyamata madokotala kuzindikira matenda amenewa kawirikawiri.

Muzilemekeza syndrome Kartagener

Ndingatani kuchotsa chikhalidwe ichi? Panopa, chithandizo cha matenda Kartagener kumadalira pa zizindikiro. Palibe mankhwala kuti adzabwezeretsa ntchito ya cilia maselo zaminyewa, koma mankhwala amakono ali ndi zida wachuma wa zipangizo kuti magawowa Inde matendawa. Ndi thandizo lawo, wodwala akhoza kuyiwala za matenda osowa.

The njira waukulu wa mankhwala:

• Mankhwala. mankhwala awa analamula kuti kutukusira kwa mapapo chifukwa bronchiectasis, ndipo sinusitis. Ikani chakale mankhwala penicillin, macrolides ndi mankhwala ochokera ku gulu la "kupuma" fluoroquinolones.
• Njira kukonza ngalande ntchito ya bronchi ndi - postural ngalande, kutikitirako ntchito mucokinetic ndi mucolytic mankhwala, etc ...
• Physiotherapy.

Pamene pamaso pa kutchulidwa bronchiectasis ndi matenda pafupipafupi kobwereza ndi chibayo mankhwala ntchito - kuchotsa (resection) Malo ambiri akhudzidwa ya phapo. Pambuyo opareshoni chikhalidwe cha odwala bwino zayamba.