Moebius syndrome: ifoto, iimpawu, isifo, unyango

Kunzima ukucinga umntu ukuhleka, ukulila phofula mintlhaveko. Nangona kunjalo, kukho abantu abanjalo, baza babeka unxilongo eyoyikekayo - Moebius syndrome. Iifoto zabantu uqala kusenokubonakala eqhelekileyo ngokupheleleyo, kodwa unxibelelwano live bayaqonda ukuba ubuso babo ekumiseni ngokupheleleyo.

It is a anomaly naso ukuvela esona sizathu ngenxa isakhiwo ngokungafanelekanga le luvo cranial. ekukhubazekeni yobuso - enye ububi obukhulu omzimba womntu. Lubonakala yi kukungabikho yobuso, ubuso kubonakala ukuba emkhenkceni, yaye iikoma.

ulwazi zembali

Ngokuba lixesha lokuqala amayeza syndrome kuchazwa ngo-1892 yi-luvo waseJamani kunye wezifo Paul Moebius. Le anomaly inqabile kakhulu, ubuninzi 10-20 nganye-1 million ezizalwayo.

ugqirha German Paul Julius Moebius wazalwa ngo-1853 eJamani wahlala apho bonke ubomi bakhe. Izazinzulu ziye zafunda uphuhliso bezonyango a ekukhubazekeni okuqhubekayo, ukuphazamiseka Wayesoyika engqondo thyrotoxicosis. Inkcazelo wenza oftalmoplegicheskoy migraine, embiza ngokuba isifo Mobius.

Kubanga okuthiwa

Eyona ndlela kakhulu zesi sifo ithathwa ukuba Moebius Syndrome, oonobangela apho de kube sekupheleni amayeza akwaziwa. Ngokutsho kwabaphandi kunye neengcali ahlukana.

• I yangaphambili bakholelwa ukuba isizathu uphuhliso engalunganga ze-luvo cranial.
• Le yokugqibela bakholelwa ukuba oku zomonakalo iinkqubo etshabalalisayo, ukuphuhlisa ngenxa oksijini yindlala komntwana esibelekweni.
• Okwesithathu njengoko izizathu eziphambili yokwakhiwa wesifo abonisa indlela yophuhliso okungaqhelekanga kweziko motor yobuso.

Moebius syndrome - isifo sele emdala kakhulu yaye kungakholeleki kakhulu. It is a ezingaqhelekanga, naso ye imisipha yobuso, ngenxa yokuba yintoni na amabinzana yobuso engekho ngokupheleleyo. Zizihlunu ingaba single- okanye kabini kabini. Umntu modem zizinto zangaphandle, akukho unkabi, akukho frowns, azisekelwanga ngokupheleleyo iimvakalelo.

Xa abantwana, ephuhliswe kakubi na usana lwakhe reflex, abakwazi ukuginya ukutya kwabo kunye namanzi ngenxa umsebenzi buthathaka kolwimi. Ngokufuthi sifo kwenzeka kwiintsapho.

iimpawu

Moebius syndrome - a luvo disorder naso kakhulu inqabile. Abantu nale ngezifo akanalo ikhono ukususa izihlunu ubuso, amehlo ngapha nangapha. Esi sifo kukhokelela ekubeni yanyhashwa kwenkqubo musculoskeletal kunye deformation beenyawo.

Ezi mpawu ziphambili kwezonyango syndrome - iLand ukuhamba, kodwa kukho abanye, ngokukhawuleza, eqinisekisa ukuba oku iimpawu syndrome Moebius:

• ukungaphili yokwakheka;
• iinyawo likhubazeke;
• ubunzima yokuginya ngokumungunya;
• izifo sensory okuqonda;
• buthathaka imisebenzi zolwimi;
• ayengaboni kahle.

Kwakhona, kukho kukwaphula excitability vestibular kunye nokungabikho secretion iinyembezi. Ulusu umntu ogulayo phezu kobuso iqine, ukushwabana-free naxa behleka okanye ukukhala. Ngokumalunga inyama ongama ayithethi ukuhamba, iimbombo emlonyeni zisoloko lishiyiwe.

Siphene yesiqephu ihamba ukungaqheleki ezifana syndactyly, kokukhubazeka, brachydactyly neminwe ezongezelelweyo. Xa ekukhubazekeni kwenxenye ye-nkqubo ibandakanya ikakhulu izihlunu nakumphezulu ubuso ngamalaphu kuba akukho Alenga njengoko izifo ezifunyenweyo.

uxilongo zokugula

anomaly kakhulu ezinqabileyo ezifana Moebius syndrome. Uxilongo, ke ngoko, ingakumbi kubantwana abaselula, kunzima kwaye kukhokelela ekuxilongweni, kuyingozi kakhulu kwaye kufuna unyango ngotyando, ezifana:

• Cerebral Palsy ;
• ukungaboni;
• yokungeva ngeendlebe.

Examination kunye nokunyangwa kwenziwa ngale ndlela.

amayeza Modern abanakukwazi ukuhlola ngokupheleleyo unobangela wale sifo, ngoko, nokuphilisa ngokupheleleyo, kakhulu, hayi kunokwenzeka. Ndiqhuba uphando kunye ukufunda genetic material kwi zofuzo ezigulane kunye inqanaba ekhangela cell kwezo.

Namhlanje, oogqirha baye bazitshisa umsebenzi ukunceda abazali babantwana nokubandezeleka kulo sifo, ekugcinweni ubomi obuqhelekileyo kuzo zonke inqanaba lobomi. Oku kungenzeka ngexesha ulungiso intetho, strabismus, umlenze ahambe.

uphethwe ngexesha elifanelekileyo lubalulekile nje kuphela ekufunyanisweni kwe-iintlobo ezahlukeneyo ezinxaxhileyo kuphuhliso, kodwa kwakhona ukuhlola uphuhliso imisebenzi yengqondo nokuchonga iindlela ngokwasengqondweni nokuphazamiseka clinical.

Lokufunyaniswa Uphuhliso engaqhelekanga iquka uphando ngokwasengqondweni, imfundo, unyango kunye yemfuza. Iye ukuze kubonwe inqanaba kokulibaziseka kuphuhliso zengqondo nezokuziphatha, ngokwasengqondweni nolwimi. Kwakhona kuchaphazela ukusekwa unxibelelwano kunye nesifo engundoqo ukubonakaliswa lwezidumbu, ubudala nemeko luvo yesigulana.

Moebius Syndrome Treatment

Lahla lo sifo singanyangekiyo ezifana Moebius syndrome, unyango kunokunceda kuhlinzwa kuphela. Akwenzeki ukuba kunyanga ngokupheleleyo esi sifo, ukongeza sengozini izithambiso, ukuphazamiseka kukuginya umsebenzi kunye nezinye iingxaki lomzimba ibandakanya enesiphene, izigulane baziva ukukhathazeka engqondweni ubomi olomeleleyo. Kuyiphelisa le meko azikwazi intsumpa kuphela.

Ngoncedo kotyando zeplastiki oko kuyenzeka ukuba wenze i zobuso ngumntu, isigulane ke ngoko uya kuba nako ukuphakamisa iimbombo emlonyeni, uziva nemvo uvuyo. ubuso enye into kwaye ihlala ukugogeka expressionless. Kukho amatyala emva iinkqubo ezinjalo, izigulane nemband 'esikhova. Ngoko ke, ngaphambi kokuba lo msebenzi lwenziwa kwazisa ngokupheleleyo isigulana yeziphumo kwakunye nezigulo kunokwenzeka.

Psychology yonxulumano loluntu

Olu tyando kunokuba nzima evela inkalo yobugcisa, ubude balo olunqabileyo ngaphantsi kwama iiyure ezisi-8. Emva kokuba kwimeko yesiqhelo izigulane labagula iintsuku ezimbini. Ngethamsanqa, abantu abaninzi kule isiphene, usenokuphila ubomi obuqhelekileyo, umsebenzi, ukuqala intsapho, babe nabantwana.

Noko ke, igama elithi yobuso ukuze unxibelelwano kunye nolwamkelo lwentlalo kubaluleke kakhulu. Esi sigulane asikwazi zingakhuli ngokupheleleyo. Ngenxa yokuba umntu, ndayihluthwa ithuba ndincume kwaye squint ngokukhawuleza, oko ngokuphosakeleyo waqonda njengoko umntu ayikaphuhlisisi umva. Nangona ukukhubazeka engqondweni % 10 kuphela kwizigulane.

Interlocutors kunzima kakhulu ukuqonda ukuba yintoni uhlobo abasabela amagama abo yaye uvakalelwa ukuba lo mzuzu umntu. Konke oku kukwenza zonxibelelwano eziqhelekileyo, ukwenza izigulane Moebius bangaphandle syndrome.

Moebius syndrome kubantwana

Abantwana abancinane badla ukuvakalisa iimvakalelo ezahlukeneyo yaye ngokufuthi, ngoko ke umntwana Moebius syndrome, kuba sobala. Yena modem kwizinto ngaphandle, hayi bedanduluka, hayi ehleka, kwaye de kube nzima ukuba idanyaze. Kuphela izandi ungakwazi akuphuhlanga kakuhle ukuqonda oko kuthatha oko umntwana.

Ukusuka ekuzalweni, aba ngabantwana kunjalo, kukho asymmetry ecacileyo, vula umlomo, amehlo musa ukuvala, lumkani ukuba izinto umntwana unako, ushukumise intloko kuphela. Ayifane waphawula ukukhubazeka engqondweni, isigulane kufuneka yokuthanjiswa ayikho ubuso kuphela kodwa ulwimi, nokuphuhlisa iiklasi ngqo.

Iimpawu sosana

Moebius syndrome - a ngezifo ilifa ilifa autosomal sofuzo okanye ngaphantsi lubalaseleyo. Xa sifo nangoku esibhedlele umama ephawula ukuba ufana nqwa yomntwana engaqhelekanga, akazi ukulila musa uvale amehlo akho maxa wambi ebusuku. Aba bantwana zifumaneka imikhuba ezingaqhelekanga:

• ukungabikho okanye fusion neminwe;
• auricles deformation;
• epicanthus.

Nangona ezibangela ezithile sifo okwangoku lusekiwe, oogqirha zibonisa ukuba ulawulo iziyobisi kunye iziyobisi zalo ngexesha lokukhulelwa kwandisa ubungozi sifo. Ngokupheleleyo Moebius syndrome ebantwaneni asinyangeki, kodwa Kwasephakadeni kuba amazwi othambileyo.

Noqeqesho ukuphucula izakhono zonxibelelwano

Ngenxa yoqeqesho olulodwa, nto leyo Ihlela unjingalwazi Danish, kubantu abane Moebius syndrome, ukuphucula izakhono zentlalo. Injongo yale uqeqesho ukuqinisa iindlela paralinguistic zonxibelelwano ngokusebenzisa imvakalozwi ezahlukeneyo nezijekulo.

Ukusebenza zoqeqesho singqiniwe, ngokuthi uhlolisiso nezinto esizibonayo, ngexesha incoko, izigulane akusekho koyika, musa engaka aze abe ekhululekile ngakumbi. Ngokutsho iziphumo izigulane ngokwabo, abaninzi bazive ngcono.

Co-umbhali le projekthi Ketlin Bogar, ngubani kwafunyaniswa Moebius syndrome. Yakha umsebenzi waza waba ugqirha ngempumelelo umsebenzi wakhe, ukuba injongo ephambili - ukuphuhlisa inkqubo eza kunceda ukuqhelana kuluntu abantu abanengxaki Moebius syndrome.