Pierre Robin syndrome: chithunzi, zimayambitsa, chithandizo, zotsatira zake. anthu chidwi ndi matenda a Pierre Robin

Pierre Robin syndrome - kudwala wa mwana wosabadwayo m'mimba. The mwana matenda mandibular hypoplasia, amene anatsagana ndi pamaso pa abadwe arcuate ndi airway kutsekeka. kupatuka anaona mwana mmodzi Wa makanda 30,000.

Kodi matenda?

matenda alibe mtundu kapena chikhalidwe maziko. Alikumchitira ngati osauka amene alibe zaumoyo okwanira, olemera anthu otchuka. Pierre Robin syndrome kugwirizana mbali zingapo. Choyamba ana amene amadwala iwo, yodziwika ndi ochepa nsagwada m'munsi. Ngati kukula kwake ndi pafupi muyezo, ndiye kungakhale ectopic kwambiri kumbuyo: mbali ya Chibade m'munsi ndi lalikulu kwambiri ndipo si zikugwira ntchito bwinobwino. Choncho m'kamwa ali kwambiri otukukira kunja mawonekedwe mu mawonekedwe a Arc, ndi lilime lili pafupi kumbuyo kumtunda kwa mmero.

Matendawa dzina lake polemekeza wotchuka mano dokotala Pierre Robin. Medic anabadwa mu 1867, anafa - mu 1950. Kwa zaka pafupifupi zana zaka za moyo wake wachita monga mkonzi wa magazini "Owona Zamano Review", kulemba nkhani yolembedwa pa chitukuko cha mano ndi kumatafuna ntchito pochita kukhala woyambitsa wa ntchito otchuka sayansi pa orthodontics. Ndipo Iye anatsegula syndrome, zochitika zazikulu za amene ali ngati mbali anatomical: mutu waung'ono kwambiri pa mwana wa mbuzi, ang'ono zikuzimiririka pang'onopang'ono chibwano ndi dongosolo zosayenera chinenero. Mwanayo yopumula, kutafuna ndi kumeza. Ngati ubongo chawonongeka, tingathe anati retardation maganizo. Komanso, ana awa sali bwino anayamba thupi.

zinthu zikuluzikulu chiopsezo

Pierre Robin syndrome ndi lakutali. Nthawi zina, ndi mbali ya kuvutika chibadwa. Yoyendetsa ndalezo mitundu woyamba wa matenda akutumikira psinjika nsagwada mu Ndisanaumbidwe lapansi. chitukuko amakwiya mimba angapo kapena yaing'ono chisindikizo m chiberekero wamkazi ngati chilonda, zotupa, cysts, fibrosis. Komanso, mandibular kukula retardation mu mwana wosabadwayo amakwiya ndi tizilombo mayi pa trimester mimba, wopanda vitamini B9 kapena matenda neurotrophic.

Pankhani chibadwa anatsimikiza syndrome Pierre Robin, ndi autosomal lalikulu. Nthawi zambiri kudwala imeneyi limodzi ndi matenda ena. Mwachitsanzo, gawo limodzi la milandu onse imagwera pa Stickler syndrome, mmene anayamba kuchimwa, wonenedwa ndi Pierre Robin ndipo anaona kutchulidwa nyamakazi myopia. Ngati makolo mwana woyamba anabadwa ndi mtundu lakutali wa kudwala, Mwina kubadwa kwa mwana wachiwiri wa wodwalayo 1-5%. Pamene mtundu majini a chiopsezo kumawonjezera zayamba.

mapangidwe kudwala

Kuti timvetse akamanena za kupatuka pa, m'pofunika amadziwa chifukwa wosabadwa akufotokozera Pierre Robin syndrome. zifukwa, kunena madokotala, ndi kugwirizana mwachindunji mwamsanga ndi chinthu etymological mu msinkhu embryonic. Amadziwika kuti sabata 5 mwana chiberekero, imayamba kupanga m'kamwa zovuta chifukwa maphatikizidwe wa zolosera medial amene imodzi kupanga mlomo kumtunda ndi m'mphuno. Ngati pa siteji kuli siudzalandilidwa ngathi lamulo, m'kamwa ndi mlomo bifurcated. Kale masabata 5 anayamba kupanga m'kamwa yachiwiri ya mphukira mbali ya kumwamba. Kufanana kutsazikana kutambasuka kwa munthu, amene kumawonjezera malo chitukuko cha nyumba m'kamwa. Language igwera mu malo, ndipo miyamba ili njira chatsekedwa monga chomangira lamba mphezi.

Pierre syndrome Robin pamene m'munsi nsagwada ndi laling'ono kwambiri. Chifukwa cha chinenero ichi alibe malo akomere, ndipo akugwiritsa khoma kumbuyo. Inali pamwamba pa munthu wamba, izo midadada kugwirizana njira zakuthambo. Izi, mu zingathandize chakuti m'kamwa zofewa atseka osati kwathunthu, musayambe mawonekedwe arched.

pathogenesis

Pofuna kukhala wathunthu nkhani kufotokozera za matenda, mukufuna kuona zithunzi za ana ndi matenda Pierre Robin. Photos wa ana awa ndi osiyana ndi zithunzi watsiku ndi ana. Kupatuka zambiri zake maonekedwe a mwanayo: ali mkamwa chochepa, underdeveloped m'munsi nkhope ndi retrognatiya - kumbuyo malo a nsagwada chapamwamba kapena m'munsi chigaza lapansi. Language Choncho kwambiri ankatembenukira: kumeneko glossoptoz.

The mwana nthawi zonse anati airway kutsekeka - ndi kutsekeka otchedwa. Baby kupuma kwambiri. chikhalidwe chake chikusintha lokha pa zolimbitsa thupi kapena kulira pambuyo kutsekeka awo amapezeka kachiwiri. Various matenda kupuma kusokoneza kudyetsa kuyenda, moti anthu amangonena alibenso reflexively kulira ndi kumasula mundawo kupuma. Chifukwa cha zimenezi, mwana si kupeza kulemera, ali ofooka ndi waulesi. Ngati palibe kanthu akutengedwa, vuto zosalongosoka msanga kuyambitsa zikutha m'mayiko ang'onoang'ono, kutha thupi lathunthu la chamoyo kachitidwe angapo, ndipo ngakhale imfa ya mwana.

mmene matenda

Pierre Robin Syndrome ali m'njira zosiyanasiyana kuti amasiyana mlingo wa kusalekerera matenda:

• Easy mawonekedwe. A mwana m'mavuto zazing'ono ndi zovuta pa kudyetsa. kupuma wake utrudneno. Pankhaniyi, dokotala n'kukulemberani mankhwala ndiwofatsa, ndilo Pamalo ndi kumulondolera pokhazikitsa ndi outpatient.
• Mlingo avareji mwamphamvu. Akamadya mwana amamvera movutikira zolimbitsa ndi kutafuna ndi kumeza. Iye amavutika kupuma, koma sikuti yovuta. Childs, monga mwana anavomereza kuchipatala, komwe Pamalo mankhwala mankhwala ndi kuthera glossopeksiyu - kumangika chinenero ndi fixation kuti mlomo wa mmunsi.
• mawonekedwe kwambiri. Tot zovuta kapena ngakhale chowawa kudya chakudya, ndi kupuma. Imeneyi akufunika mwamsanga - opaleshoni. Anthu ena amalangiza kudyetsa, kulowa kafukufuku mkati n'kukagwira tracheostomy - ndi kung'ambika wa trachea ndi kuyambitsa mu izo a chubu.

Ndi digiri iliyonse ya kukula kwa matenda kufunsa katswiri wodziwa. Self-mankhwala akhoza kuwononga mwana, chifukwa mavuto aakulu sizingasinthe.

zosokonezeka

Oterewa mwana wodwala sikovuta. Koma mawu enieni ayenera kufunsa munthu orthodontist odziwa ndiponso chibadwa. Pamodzi ndi ena zachipatala iwo amalangiza mankhwala makamaka pofuna kupewa mavuto aakulu. Pankhaniyi, zosokonezeka angakhale osiyana kwambiri: suffocation akagona, chitukuko vuto laryngeal, maonekedwe a phokoso, raucous kupuma. Isanayambike ndi kupitirira matenda a mtima komanso amakwiya Pierre Robin syndrome: zotsatira za chikhalidwe ichi ndi anamuona pa 14% ya ana.

A mwana akudwala matenda, ndi zoipa kwambiri kudya. The makhalidwe oipa zofunika pa yoyamwitsa, palatal mphanga limakupatsani kulenga ayi. Komanso, mwana uyu ndi laling'ono kwambiri m'munsi nsagwada, zomwe si kumupatsa zabwino odulidwa minofu zozungulira mkamwa ndi kukuchititsani akathyole m'mawere a mayi. Pamene kudyetsa kuyenda zingalepheretse monga palatal mphanga kwambiri alekanitsa m'mphuno ndi danga m'kamwa, kuwonjezera chiopsezo suffocation. Mwa mavuto ndi madokotala amatchedwanso mapindikidwe mano, malocclusion, aakulu kutupa kwa khutu, tingayambe kugontha, ndi ngakhale kungokhala pamodzimodzi kukula thupi ndi m'maganizo.

chithandizo ndiwofatsa

Nthawi kulipira chithandizo oyenerera, makolo ali ndi mwayi kuthetsa vuto Pierre Robin. Mankhwala Choncho kokhazikika. Choyamba, mwana anaziika zonse chibadwa, otolaryngologist, maso, ndi orthodontist, mano ndi mawu dokotala. Chachiwiri, mwana zonse ndondomeko ya kudya ndi kuyesa kuthetsa kutsekeka kwa airway chapamwamba. Ngati inu kutsatira malangizo onse, ndiye kuti zaka 6, ana ambiri kukula ndi mawonekedwe a nsagwada m'munsi pang'onopang'ono dekhetsa.

A njira kwambiri mankhwala ndiwofatsa ndi Pamalo mankhwala. Kumafuna kuwayika mwanayo m'mimba mwake bola padzakhala kukula kofunika nsagwada m'munsi. Kutangoyamba, mwana ayenera kukhala mu udindo uwu. Pamene mwana akukula, akulu ayenera kumuphunzitsa kugona okha kaimidwe chomwecho. Ngati kuyenda atagona mimba yake, Analenga kuthamanga mutu ndi kuwasintha yokoka ndi chinenero akukhala malo. mankhwala Izi zimathandiza 80% ya milandu.

alowererepo opaleshoni

M'pofunika mitundu zolemera ndi sing'anga matendawa. Pierre Robin Syndrome akhoza pang'ono inathetsedwa ntchito ntchito chinenero kumangika ndi kukonza mu malo m'munsi mlomo. Ndondomeko, wotchedwa glossopeksiya, yosavuta si owopsa. Komanso, ana angapyoze ndi trachea ndi chubu anaikapo mdzenje: chifukwa cha ichi adzatha kupuma mosavuta ndi wopanda mavuto. Tsekani dzenje zimenezi mu 3 zaka. njira sikawirikawiri ntchito, pakufunika za 1% ya ana ndi aakulu Kuvuta matenda.

Madokotala mwina perekani psinjika-chododometsa osteosynthesis. Ndondomeko kumafuna breaching clippings mu mafupa a nsagwada m'munsi. Iwo anaikapo mu zikhomo kuti angagwirizanitse kumutu, ndi njira yapadera chifukwa kuwonjezera ndi kukula pachibwano. Tsiku lililonse utithandize zotsatira za anatengera, dokotala amakwaniritsa pang'onopang'ono koma ogwira kutambasuka kwa mmunsi kutsogolo. Chotero ukugwirizana ndi ikuchitika kwa mwezi, ndiyeno kupanga yopuma kwa masabata 8. M'pofunika yachibadwa fupa machiritso ndi fixation ake odalirika mapangidwe latsopano.

Malangizo

Ana ndi matenda Pierre Robin amafuna kusintha ndondomeko kudya. Choyamba, ayenera kuwonjezera zili caloric mkaka: ichi chifuwa mapeto decanted ndi wothira munthu yokumba. Chachiwiri, pa kudya zambiri amafuna kuti azipumira kuti mwana wanu mwayi kukoka mpweya wanu, ndi thandizo la kulira ndi kusanza kuti muchotse airway. Komanso, ngati mayi stroking chibwano wake kuyenda, zimathandiza kwambiri kuti kuyamwa mkaka.

Chachitatu, ana awa kugula botolo wapadera, kulengedwa ndi akatswiri pa kudyetsa ana ndi matendawa. Dzenje ndi onse kuposa mu nipple ochiritsira, kuwonjezera, pali vavu wapadera kuti amalola kholo kulamulira otaya mkaka. Makanda ndi cholinga pacifiers wapadera. Kachinayi, madokotala angakulangizeni kukhazikitsa mu m'kamwa zibowo zam'mano tot obturator kuti atseka ndi mpita. Tisaiwale kuti palibe njira pamwambapa si njira ya chithandizo. Izi ndi monga chipangizo kuti imathandizira ndi atsogolere mphamvu ya chikhalidwe mankhwala a kuyenda wanu.