Pierre Robin chirwere: photo, zvinokonzera, kurapwa, zvaitika. Chinozikamwa vanhu vane chirwere pamusoro Pierre Robin

Pierre Robin chirwere - zvokurwara ari mudumbu mudumbu. Mwana chirwere mandibular hypoplasia, izvo pamwe nokuvapo akatsemuka mukanwa arcuate uye kufema mhingaidzo. The kutsaudzira kuchengetwa mwana mumwe pakati 30.000 vacheche.

Chii chinonzi chirwere ichi?

The chirwere haana dzinza kana magariro background. Achiitirwa kufanana varombo vasina zvakakwana mune zvoutano, uye mupfumi vanhu vakakurumbira. Pierre Robin chirwere chokuita akawanda zvinhu. Chekutanga vana vanotambura kwavari, dzinoratidzwa chidukuduku yakaderera shaya. Kana saizi yayo pedyo mureza, ipapo zvingava ectopic zvikuru shure: pakona yeDehenya nechigadziko riri guru zvikuru uye haasi kushanda zvakanaka. Nokudaro mukanwa ane akanyanya convex chimiro muchimiro ane chinhu chakakombama, uye rurimi iri pedyo rokumusoro seri huro.

The chirwere ainzi mune kukudzwa kukuru akakurumbira mazino chiremba Pierre Robin. Medic akaberekwa muna 1867, akafa - muna 1950. Nokuti anenge zana makore hwake akashanda somunhu mupepeti magazini "Dental Review", kunyora chinyorwa pamusoro pokumuka mazino uye kutsenga basa acho, akava munyori dzakakurumbira mabasa yesayenzi pamusoro orthodontics. Ndiye akavhura urwere, chikuru zviri izvo vakadaro anatomical zvinhu: musoro chaizvo duku panguva nembudzana, diki ikaramba ichiserera chirebvu uye marongerwo kukodzera mutauro. Mwana zvakaoma kufema, kutsenga uye kumedza. Kana uropi wakaremara, unogona kuonekwa retardation mupfungwa. Uyewo, vana ava havana zvakanaka yakatanga panyama.

Major zvinokonzera njodzi

Pierre Robin chirwere iri yoga. Mune zvimwe zviitiko, zviri chikamu majini abnormalities. Actuating mhikuriso dzehorokodzwa chirwere wokutanga kushumira compression rushaya ari mudumbu. kukura kwayo chinokurwadzai pamuviri pevana vakawanda kana chiduku mucherechedzo dzematunhu hadzi dumbu sezvo vanga, namamota, cysts, fibrosis. Uyewo, mandibular kukura retardation ari mudumbu chinokurwadzai akabatwa chirwere muna mai panguva trimester wokutanga pamuviri, kusamuda vhitamini B9 kana neurotrophic zvirwere.

Panyaya kuvabereki kutsunga Pierre Robin urwere, zviri autosomal kuzivikanwa. Kazhinji zvokurwara iri pamwe nemamwe matambudziko. Somuenzaniso, mumwe pavatatu zvose nyaya inowira Stickler urwere, sezvamakaropafadzwa dzakabudirira Kutsauka, sezvinotaurwa Pierre Robin uye akaona raidudzwa chearthritis uye myopia. Kana vabereki mwana wokutanga akaberekwa aine nyayawo pamusoro zvokurwara mhando, mukana yokuberekwa mwana wechipiri murwere 1-5%. Apo majini muchimiro ngozi kunowedzera substantially.

nezvekugadzirwa zvokurwara

Kuti tinzwisise musimboti nokutsauka, zvinokosha kuziva sei uye nei mudumbu hwapedyo Pierre Robin syndrome. Zvikonzero, vanoti vanachiremba, vane zvakananga uye pakarepo kubatana pamwe etymological chakaita embryonic chechetere. Zvinozivikanwa kuti vhiki 5 mwana ari mudumbu, hunotanga muitiro rokuumba mukanwa zvakaoma nokuda Fusion pamusoro medial maberere iyo panguva kuumba rokumusoro muromo uye wemhino. Kana panguva ino pane kukanganisa, rurimi uye muromo bifurcated. Nechekare 5 mavhiki akatanga kuumba mukanwa yechipiri pamusoro divi matavi kudenga. Parallel wakagumiswa kuwedzera munhu, izvo anowedzera nzvimbo nokuda kukura nomumukanwa hurongwa. Language inowira panzvimbo, uye matenga ndiwo namahwindo pezvakaitwa semheni bhakoro.

Pierre Robin chirwere apo rezasi rushaya iduku kwazvo. Nokuda mutauro uyu havana nzvimbo pokugara, uye vachambokanganwa kune veshure madziro. Zvayaiva pamusoro chakaipa chechetere, izvozvo zvinogumbura kubatana zvokudenga kwendangariro. Izviwo, zvinotungamirira pakuti nyoro mukanwa anovhara kwete zvachose, pokubatana yokufeva chimiro.

pathogenesis

Kuti kuva wakakwana anodzidzisa mharidzo pamusoro chirwere, unoda kuona mifananidzo vana Pierre Robin syndrome. Photos of vana izvi zvakasiyana zvatinosiita mifananidzo yevana. Kutsauka kazhinji kukuvadzisa chitarisiko mwana ane muromo diki, underdeveloped yakaderera chiso uye retrognatiya - rwemberi nzvimbo rokumusoro kana rezasi rushaya kuti dehenya. Language haachatauri saka zvakasimba: ipapo glossoptoz.

Mwana kuramba vakachengeta yemweya mhingaidzo - vanonzi mhingaidzo. Baby kufema zvikuru. ezvinhu ake ari kuvandudzika chete panguva kurovedza muviri kana ndichichema pashure mhingaidzo yavo kunoitika zvakare. Various kufema Zvirwere omesa zvokudya kukambaira, saka chinowanzoonekwa havachazotamburi reflexively kuchema uye kuregedza kufema turakiti. Somugumisiro, mwana haana kuwana uremu, ndiye simba uye ane usimbe. Kana pasina zvinoitwa, iye chakaremara kutenderera nokukurumidza kuita kuti Kuparadzwa muviri duku, munhu kumira kushanda anoverengeka hurongwa, uye kunyange kufa kwemwana zvakakwana.

kukura chirwere

Pierre Robin Syndrome ane dzakawanda kuti zvisingapindirani ukuru chirwere nekuomarara:

• Easy chimiro. Mwana kusangana nematambudziko kuduku uye nechinouya panguva dzichifura. kufema kwake utrudneno. Panyaya iyi, chiremba prescribes kuchinja kurapa, ndoupi positional uye ichiitwa imwe outpatient marongero.
• Avhareji mwero zvakaoma. Munguva kudya mbudzana anonzwa mwero zvakaoma dög uye kumedza. Akariwana zvakaoma kufema, asi mamiriro ezvinhu hakukoshi. Kazhinji, mwana akadaro vakabvuma kuti kuchipatara, uko positional aisirwe kurapwa uye vanopedza glossopeksiyu - mutauro tightening uye Kuda kutamba kunzvimbo dzakaderera muromo.
• Zvakakomba chimiro. Ispăşire zvakaoma chaizvo kana kunyange kurwadza kudya zvokudya, uye kufema. In izvi zvinoda kukurumidza kuita - kuvhiyiwa. Vamwe vanhu vanokurudzira zvokudya, kupinda womukati vachiongorora uye vaite rimwe tracheostomy - munhu kwetsinga of trachea kuuyisa mariri ari chubhu.

With chero mwero kwokuomerwa chirwere vanofanira kubvunza ane ruzivo nyanzvi. Self-mishonga inogona kukuvadza mwana, kukonzera zvakakomba kusingarapiki yakaipa.

yematambudziko

Rokuongorora murwere mwana haasi kuoma. Asi chaiyo mashoko vanofanira kubvunza ane ruzivo orthodontist uye genetics. Pamwe chete nevamwe vanachiremba vakati vanokurudzira munhu marapiro kuitira kudzivisa zvakakomba. Panyaya iyi, kuti matambudziko zvinogona akasiyana chaizvo: kutadza kufema panguva hope, pave laryngeal kuremara, nezve ruzha, raucous kufema. Pazvinotanga uye kufambira mberi chemwoyo uyewo chinokurwadzai Pierre Robin chirwere: migumisiro zvakasikwa ichi vari kuchengetwa 14% vana.

Mwana vanotambura nezvirwere ichi, zvakaipa chaizvo kudya. The akaipa kumanikidzwa zvinodiwa paanenge achiyamwa, palatal mumukaha unokubvumira kusika kwete. Uyezve, mwana iyi iduku kwazvo yakaderera rushaya chisingazivi kumupa wakanaka chekawo dzeraundi tsandanyama mumuromo uye zvakasimba kubata zamu ramai. Kana achidyisa kukambaira kunogona kuvhunga kunyange sezvo palatal mumukaha zvikuru Anopatsanura nasal uye dzaiita nzvimbo, kuwedzera pangozi kutadza kufema. Pakati matambudziko, vanachiremba anonziwo deformation mazino, malocclusion, chisingaperi kuzvimba nzeve, izvo zvingaita kuti kusanzwa, uye kunyange kukakanganiswa mumuviri uye mupfungwa kukura.

kuchinja mabatiro

Nguva kubhadhara vanokwanisa kurapwa, vabereki vane mukana wakanaka kubvisa Pierre Robin syndrome. Kunokwanisa Saka conserved. Chokutanga, mwana ari pasi chenguva zvokuongorora mu genetics, otolaryngologist, ophthalmologist, imwe orthodontist, chiremba wemazino uye chiremba wechikadzi achindidzidzisa kutaura. Chechipiri, mwana masero zvokudya uye edza kurambidza mhingaidzo rokumusoro yemweya. Kana ukaramba zvose kunenge, ipapo 6 makore, vana vakawanda vari kukura uye chimiro repasi shaya zvishoma normalized.

A nzira inoshanda chaizvo kuchinja kurapwa positional kurapa. Kunosanganisira akaisa mwana dumbu rake chero bedzi pachava zvakakodzera kukura repasi shaya. In paucheche, mwana anofanira kuva panzvimbo iyi. Kana mwana paanokura, vakuru vanofanira kudzidzisa kurara chete chete mamiriro. Kana kukambaira avete dumbu rake, anosika kumanikidzwa mumusoro negiravhiti inochinja uye mutauro anova nzvimbo. mushonga uyu unobatsira muna 80% nyaya.

kuvhiya kupindira

Zvakafanira kuti rinorema uye svikiro siyana chirwere. Pierre Robin Syndrome anogona zvishoma zvachose kushandisa operative mutauro tightening uye kugadzirira nayo yepazasi muromo nzvimbo. The nzira, inonzi glossopeksiya, nyore uye kwete ngozi. Uyewo, vana vanogona kuurura kuti trachea uye kumicheto akaiswa mugomba; nekuda ichi achakwanisa kufema nyore uye pasina matambudziko. Kuvhara buri ino anenge 3 years. Nzira haawanzopiwi kushandiswa, pakava zvinodiwa anenge 1% vana zvakaoma. chirwere kunzwisisa.

Vanachiremba vanogona basa compression-kukanganisika osteosynthesis. The nzira kunosanganisira nokudziurira clippings mu mapfupa rezasi shaya. Vari rakapinzwa nembambo kuti kuisa mumusoro, uye ane anokosha nomazviri kuwedzera uye kuwedzera shaya. Zuva nezuva, kuwedzera kushanda kwadzakabva, chiremba inowana zvishoma asi anobudirira kuwedzera yepazasi chikamu mberi. Yakadaro ekisitenjeni rinoitwa kwemwedzi, uye ipapo kuregawo 8 kwemavhiki. Zvakafanira kuti kwomuzvarirwo pfupa kuporesa uye ayo akavimbika Kuda kutamba pakugadzirwa itsva.

anobatsira zvahungava hwakaita zvakavimbika

Vana vane Pierre Robin chirwere zvinoda kuchinja zvokudya kwacho. Chekutanga, vanofanira kuwedzera caloric zviri mukaka: kwaizvozvi zamu decanted uye hwakakanyiwa rimwe risiri chairo. Chechipiri, panguva dzokudya kakawanda vanofanira kutora hwatsvuka kupa mwana wako mukana kubata mweya wako, uye achibatsirwa kuchema uye kurutsa kubvisa yemweya. Uyezve, kana amai stroking vake chirebvu kukambaira, zvinobatsira kwazvo yokufungisisa mukaka.

Chechitatu, vana ava vanofanira kutenga chaiwo bhodhoro, rakagadzirirwa nenyanzvi zvokudya pamwe urwere vana. Buri zviri vakwanise zvikuru pane ari kwakajairika munyatso, mukuwedzera, pane vharafu chaiyo unobvumira mubereki kudzora aibuda mukaka. For vacheche uye vakasikwa anokosha Pacifiers. Chechina, vanachiremba vanogona akakurudzira mukudenga nemumuromo mhango ispăşire obturator kuti anovhara crevice. Hatifaniri kukanganwa kuti hapana apa nzira haisi nzira kurapwa. Uyu unongova mumwe mudziyo kuti mhanyisa uye aivabatsira kubudirira mishonga nemamiriro kukambaira yenyu.