Moebius syndrome: isithombe, izimpawu, uphethwe, ukuphathwa

Kunzima ukucabanga umuntu abangakwazi ukuhleka, ukukhala futhi ukubonisa imizwa. Nokho, kukhona abantu abanjalo, babeka ukuxilongwa esabekayo - Moebius syndrome. Izithombe zabantu unganakile kungase kubonakale kuyinto eyamukeleka ngokuphelele, kodwa zokuxhumana bukhoma bayaqaphela ukuthi ubuso babo yisimo ngokuphelele.

Kuyinto anomaly azalwa naso esakheka ku isizathu esiyinhloko ngenxa isakhiwo engafanele nezinzwa cranial. ukukhubazeka kobuso - omunye okubi kunabo bonke umzimba womuntu. It libhekene ukuntuleka sebuso, ebusweni kubonakala simisiwe, nanjengensimu engenamanzi buso.

Imininingwane zomlando

Ngokokuqala ngqá e imithi syndrome achazwa 1892 by wezinzwa German kanye wezifo zengqondo uPawulu Moebius. Lokhu anomaly ivelakancane, linanikhulu lema-abayizigidi ezingu-10-20 ngayinye 1 ukubeletha.

udokotela German uPawulu uJulius Moebius wazalwa ngo 1853 e-Germany, bahlala khona ukuphila kwayo konke. Ososayensi behlola ukuthuthukiswa basemtholampilo a ukukhubazeka lesichubekako, iziyaluyalu sezinzwa futhi kwengqondo thyrotoxicosis. Incazelo wenza oftalmoplegicheskoy migraine, ukusibiza isifo Mobius.

Izimbangela okucatshangwa

Ifomu olunzima sifo ibhekwa njengeBhayibheli Moebius Syndrome, izimbangela zazo kuze kube sekupheleni imithi ayikaziwa. Ngokwabacwaningi nochwepheshe ahlukana.

• Lo mqeqeshi owake wapheka bakholelwa ukuthi isizathu siwukuthi ukuthuthukiswa olungafanele nezinzwa cranial.
• Lesi sakamuva zikholelwa ukuthi lokhu kungaba yingozi izinqubo elimazayo, ukuthuthukisa ngenxa umoya-mpilo yokulamba we ingane esibelethweni.
• Okwesithathu njengoba sezizathu esiyinhloko kumiswa isifo ayawuveza ukuthuthukiswa okungavamile ebusweni motor isikhungo.

Moebius syndrome - lesi sifo ngempela ubudala futhi ongaqondakali kakhulu. Kuyinto engavamile azalwa naso we izicubu ezithile ebusweni, ngoba lokho kuyinto izinkulumo lingekho ngokuphelele ebusweni. I ukukhubazeka kungaba single- noma kabili emaceleni. Man akasabeli ezenzeka ngaphandle, akukho ukumomotheka, akukho frowns, elingenalo ngokuphelele semizwelo.

Ezinganeni, ekhanya ithuthukiswe abancela reflex, abakwazi ukugwinya ukudla yabo namanzi ngenxa umsebenzi owenziwe buthaka ulimi. Ngokuvamile lesi sifo kwenzeka emikhayeni.

izimpawu

Moebius syndrome - ukuphazamiseka azalwa naso zezinzwa kuyinto ngempela ezingavamile. Abantu nale zokugula ayinakho ikhono ukuhambisa imisipha ubuso, amehlo ngapha nangapha. Lesi sifo kuholela nokwephula of the system zemisipha kanye ukusonteka zezinyawo.

The main ukubonakaliswa emitholampilo ye syndrome - kwezithibelo ukuhamba, kodwa kukhona abanye, evelele, eqinisekisa ukuthi lena Moebius izimpawu syndrome:

• kwezinso yokwakheka;
• izinyawo sikhubazekile;
• nenkinga yokugwinya futhi abancela;
• iziyaluyalu sezinzwa okuqonda;
• buthaka imisebenzi yolimi;
• umbono kahle.

Futhi, kukhona kokunikezwa kwe-excitability vestibular kanye nokuntuleka uketshezi izinyembezi. Skin umuntu ogulayo ebusweni iqine, umbimbi-free ngisho nalapho elihlekayo noma ukukhala. Mayelana izicubu ongama awusebenzi ukuhambisa, amakhona umlomo njalo ivinjelwe.

Isici emikhawulweni kuhambisana angahlangani ezifana syndactyly, kokukhubazeka, brachydactyly neminwe ezengeziwe. Ngo ukukhubazeka olulinganiselwe kuhilela ikakhulu imisipha engxenyeni engenhla indwangu ubuso ngoba akukho droop njengoba e isifo kuluthola.

zokugula ukuhlola

anomaly Abaningana impela ezingavamile ezifana Moebius syndrome. Sifo, Ngakho-ke, ikakhulukazi izingane ezincane, kunzima futhi ngokuvamile uholela ekubeni ekuxilongweni, kuyingozi kakhulu futhi zidinga ukwelashwa kuhlinzwa, njenge:

• cerebral palsy ;
• ubumpumputhe;
• ubuthulu.

Examination futhi okunye ukwelashwa abazenzi kulesi siqondiso.

imithi yesimanje abasuke bengakakwazi ukusebenzisa ukuhlola ngokugcwele imbangela yalesi sifo, ngakho-ke, ukwelapha ngokuphelele, futhi, ngeke kwenzeke. Ukuqhuba ucwaningo nokutadisha izinhlayiya eziyisisekelo zofuzo kuma-chromosome kweziguli ezingeni beyofuna cell ukuguquka kwezakhi zofuzo.

Namuhla, odokotela baye bazishisa umsebenzi ukusiza abazali bezingane abaphethwe yilesi sifo, ekugcinweni ukuphila okuvamile zonke izigaba zokuphila. Lokhu kungenzeka ngesikhathi inkulumo ukulungiswa, strabismus, Gait.

Imiphumela yaloku kuhlola okungenhla Esifike Ngesikhathi kubalulekile hhayi nje ngobe kutawutfutfukisa ukuthola ezihlukahlukene ngeziphambeko ukuthuthukiswa, kodwa futhi ukuhlola ukuthuthukiswa imisebenzi yengqondo kanye ukuhlonza izindlela ngokwengqondo neziyaluyalu emtholampilo.

Imiphumela yaloku kuhlola okungenhla yentuthuko normal kuhlanganisa ucwaningo ngokwengqondo, ezemfundo, emitholampilo kanye zofuzo. Kuye kwenziwa ukuba kutholakale izinga ukubambezeleka ukuthuthukiswa ngokomzwelo futhi zokuziphatha, bokusungula nolimi. Futhi kuthinta ukusungulwa ukuxhumana lesi sifo ecashile kwezinye izimpawu, ukubona sokugembula, yobudala kanye isimo zezinzwa isiguli.

Moebius Syndrome Ukwelashwa

Lahla lesi sifo esikhohlisayo like Moebius syndrome, ukwelashwa kungakusiza kuhlinzwa kuphela. Akunakwenzeka ukwelapha ngokuphelele lesi sifo, ngaphezu sengozini yezimonyo, iziyaluyalu kokugwinya umsebenzi nezinye izinkinga emzimbeni lokho kusho ukuthi kunenkinga, iziguli zizizwe ekucindezelayo ngokwengqondo eqinile kukho konke ukuphila. Ukudambisa kulesi simo can kuphela surgically.

Ngosizo bokuhlinzwa kokulungisa kungenzeka ukudala i izinkulumo yokufakelwa ebusweni ke isiguli bazokwazi ukuthi siphakamise amakhona emlonyeni, uzizwa umuzwa wenjabulo. Yonke enye into ubuso uhlala ongasanyakazi futhi ngaphandle komumo wobuso. Kukhona amacala ukuthi ngemva lezo zinqubo, iziguli ukudumala ngemiphumela. Ngakho-ke, ngaphambi kokuba ukusebenza Kwenziwa wazise ngokugcwele ogulayo imiphumela kanye nezinkinga kungenzeka.

Psychology of ubudlelwano nomphakathi

Lo msebenzi esikhundleni eziyinkimbinkimbi kusuka isici lobuchwepheshe, ubude bayo kuyaqabukela esingaphansi 8 amahora. Ngemva kokuthi avuselelwa iziguli egunjini labagula kakhulu izinsuku ezimbili. Ngenhlanhla, abantu abaningi abane-le nkinga, phila evamile ekuphileni, umsebenzi, ukuqala umndeni, banezingane.

Nokho, isimo sobuso okuxhumana futhi lithathelwe yezenhlalakahle kubaluleke kakhulu. Isiguli akakwazi ukuthuthukisa ngokugcwele. Ngenxa yokuthi umuntu engalali ithuba smile futhi squint evelele, ke ngephutha waqonda njengoba umuntu ngokuhlakanipha emuva. Nakuba retardation engqondo % 10 kuphela eziguli.

Interlocutors kunzima kakhulu ukuyiqonda onjani ukuphendula ngamazwi abo kubangele nalokho futhi unomuzwa wokuthi ngalesi sikhathi lo muntu. Konke lokhu kukwenza ungakwazi ukuxhumana evamile, okwenza iziguli ezine Moebius syndrome bangaphandle.

Moebius syndrome ezinganeni

Izingane ezincane azikwazi ukuhlaziya ukuveza imizwelo ezahlukene futhi kaningi, ngakho ingane Moebius syndrome, kucace bha. Yena akasabeli ezenzeka ngaphandle, hhayi ukukhala, hhayi laughing, ngisho kunzima blink. Kuphela imisindo ngalokwanele baqonde ukuthi kuthatha ingane.

Kusukela azalwa, lezi zingane nazo namanje, kukhona asymmetry ecacile, vula umlomo, amehlo ungavali, watch out for lezinto yengane, ezihambayo ikhanda lakho kuphela. Akuvamile waphawula retardation engqondo, isiguli liyadingeka massage akuyona ubuso kuphela kodwa futhi ulimi, ukuthuthukisa amakilasi ukuhambisana.

Izimpawu e usana

Moebius syndrome - a zokugula lezinceku ifa lingenxa autosomal esikhubazayo noma ngaphansi evelele. Uma lesi sifo namanje esibhedlela umama uqaphela ukuthi ulingisa kwengane ezingezona ezemvelo, lokhu akusho ukuthi ukukhala ungavali amehlo akho ngezinye izikhathi ebusuku. Lezi zingane atholakala imikhuba emibi ongaphilile:

• ukungabi noma Fusion neminwe;
• ukusonteka auricles;
• epicanthus.

Nakuba izimbangela ezingokoqobo lesi sifo ingakamiswa esungulwe, odokotela basikisela ukuthi ekuphathweni izidakamizwa kanye izinto kwezidakamizwa ngesikhathi sokukhulelwa kwandisa ingozi yesifo. Ngokugcwele Moebius sifo ngo izingane ngeke welaphe, kodwa ngeminyaka iba inkulumo ethambile.

Ukuqeqesha ukuthuthukisa ikhono elihle lokukhulumisana nabanye

Ngenxa ukuqeqeshwa okukhethekile, okuyinto ihlela Uprofesa Danish, kubantu abanegciwane Moebius syndrome, ukuthuthukisa amakhono ezenhlalo. Inhloso ukuqeqeshwa ukuba baqinise izinhlelo izindlela paralinguistic yokuxhumana usebenzisa intonations ezahlukene futhi ukuthinta.

Ukuphumelela ukuqeqeshwa okuye kungase kutholakale ngokuphindaphindiwe ngo ucwaningo nalokho esikubonayo, ngesikhathi inkhulumomphendvulwano, iziguli ayisekho uzizwa uthuka, musa Fuss futhi ukuba sikhululeke kakhudlwana. Ngokusho imiphumela iziguli ngokwabo, eziningi ungazizwa ngcono.

Co-nombhali msebenzi Ketlin Bogar, eyathola ukuthi inesifo Moebius syndrome. Yakha i-career kanye iziqu zobudokotela niphumelele emsebenzini wakhe, kungcono umgomo oyinhloko - ukuthuthukisa uhlelo kuzosiza ukujwayela emphakathini kubantu abaphethwe Moebius syndrome.