Pierre Robin syndrome: ifoto, izinto, unyango, imiphumo. Abantu abadumileyo kunye syndrome ka uPierre Robin

Pierre Robin syndrome - ngezifo yosana olungekazalwa esibelekweni. Umntwana bene hypoplasia mandibular, nto leyo sikhatshwa bubukho lwacandeka inkalakahla arcuate kunye ngokuma kokuphazanyiswa. Ukuphambuka waphawula umntwana omnye phakathi iintsana 30,000.

Yintoni na kwesi sifo ndinaso?

Le Isifo akukho imvelaphi ngokobuhlanga okanye ngokwentlalo. Ephathwa ngathi ihlwempu abangenazo ukhathalelo lwempilo olwaneleyo, kunye nabantu abazizityebi odumileyo. Pierre Robin syndrome ezinxulumene iinkalo eziliqela. Okokuqala bonke abantwana abaphethwe kubo, ebonakala ngu nomhlathi encinane kakhulu asezantsi. Ukuba ubukhulu wayo likufutshane umgamgatho, ngoko kusenokuba ethunjini kakhulu umva: i-engile isiseko ukakayi mkhulu kakhulu kwaye yayingasebenzi ngokuqhelekileyo. Ngaloo inkalakahla na imilo khaxa kakhulu ngohlobo i arc, kwaye ulwimi ifumaneka kufutshane emva engentla yomqala.

Esi sifo igama wokuwonga elaa ugqirha enkulu odumileyo zamazinyo uPierre Robin. Nabezempilo wazalwa ngo-1867, wafa - ngo-1950 Kuba phantse ikhulu iminyaka yobomi bakhe uye wasebenza ngumhleli phephancwadi "Review Dental", ukubhala ithisisi kuphuhliso kwamazinyo kunye nendima ukuhlafuna kule nkqubo, kuba nombhali imisebenzi ezithandwayo lwenzululwazi orthodontics. Yaba nguye lo, wawavulayo syndrome, iimpawu eziphambili kuzo ezinjalo iimpawu yokwakheka: intloko encinane kakhulu xa itakane, isilevu liyancipha amancinane isakhiwo ngokungafanelekanga zolwimi. Umntwana nzima ukuphefumla, ukuhlafuna liginye. Ukuba ingqondo yonakele, oko singabonakala yokudodobala kwengqondo. Kwakhona, aba bantwana ukuba zange ziphuhliswe kakuhle emzimbeni.

izinto umngcipheko

Pierre Robin syndrome ayinabantu. Ngamanye amaxesha, yinxalenye ezingaqhelekanga yemfuza. Ashukumisa lever yezi ntlobo yokuqala sifo ikhonza ucinezelo imihlathi kwi mbungu. uphuhliso kwayo kudale ukhulelwe okanye itywina elincinane weengingqi isibeleko abaziintombi ibala, nangezilonda, cysts, abanesigulo. Ukongeza, le abazizidalwa ngokukhula mandibular kwi umbungu evusa igciwane lohlobo kwi unina ngexesha ekhulelwe yokukhulelwa, ukungabikho lwalo ivithamin B9 okanye ukuphazamiseka neurotrophic.

Ngokubhekiselele izakhi uzimisele uPierre Robin syndrome, oko autosomal lubalaseleyo. Amaxesha amaninzi le ngezifo lukhatshwa nezinye izifo. Ngokomzekelo, omnye esithathwini zonke iimeko siya ngeyo syndrome, apho aphambuke iphuhlisiwe, njengoko kuchaziwe yi-Pierre Robin waza waphawula ukuba samathambo ngokukhawuleza kunye ukubona. Ukuba abazali umntwana wokuqala wazalwa kunye zohlobo ekwanti kwezimilo, amathuba yokuzalwa komntwana yesibini isigulana 1-5%. Xa uhlobo yemfuza lomngcipheko kwandisa kakhulu.

ukubunjwa leengxaki

Ukuze siyiqonde kakuhle eli kuphambuka, kuyimfuneko ukwazi ukuba kutheni umbungu iphuhlisa Pierre Robin syndrome. Izizathu, bathi oogqirha, wu ri karhi ngqo yaye ngoko nangoko kunye into etymological kwinqanaba osandul. Yinto eyaziwayo ukuba kwiveki 5 usana esizalweni, kuqala inkqubo yokutshata inkalakahla nzima ngenxa ezo ezo Intelekelelo medial leyo ngaxeshanye zenze iindevu kunye womsindo. Ukuba ngeli nqanaba kukho ulwaphulo-mthetho, inkalakahla kunye lip bifurcated. Sele iiveki 5 waqala ukwakha inkalakahla yesibini ngamasebe icala ezulwini. ukwandiswa Parallel zingama na umntu, nto leyo eyandisa isithuba ukuphuhliswa kwezakhiwo ngomlomo. Ulwimi iwela ndawo, yaye amazulu iinkqubo ezivaliweyo njengombane bopha.

Pierre Robin syndrome xa umhlathi esezantsi lincinane kakhulu. Ngenxa yale ulwimi akukho ndawo ukuze kubonelelwe, yaye ijolise eludongeni ngasemva. Ofumaneka ngaphezu kwe-kwinqanaba eziqhelekileyo, iibhloko kuyo udibaniso iinkqubo izikhakhamela. Oku kukhokelela ekubeni inkalakahla ethambileyo yokuqala hayi ngokupheleleyo, ukwakha imilo enhle.

Pathogenesis

Ukuze ube ntetho epheleleyo ifundise malunga nesifo, ufuna ukubona imifanekiso babantwana Pierre Robin syndrome. Iifoto kwezi kids ezahlukeneyo ukusuka imifanekiso eqhelekileyo abantwana. Umahluko rhoqo ungcolisa ukubonakala umntwana; ke umlomo encinane kakhulu, ubuso esezantsi ngokwaneleyo kunye retrognatiya - Indawo ngasemva kwemihlathi oluphezulu okanye olusezansti ukuya ukakayi. Ulwimi wayeka ngoko ngamandla; akukho glossoptoz.

Umntwana usoloko wabona ngokuma isithintelo - ekuthiwa-kokuphazanyiswa. Baby uphefumla kakhulu. imeko yakhe ekuphuculeni kuphela ngexesha umsebenzi ngokwasemzimbeni okanye ekhala emva kwenzeka kwakhona kokuphazanyiswa zabo. zezigulo ezahlukeneyo nokuphefumla nzima ukondla nosana, ngoko oko kudla akuseyomfuneko ukulila kusincede kwaye khupha phecana sokuphefumla. Ngenxa yoko, usana hayi lokufumana ubunzima, yena amandla nelidangeleyo. Ukuba akukho manyathelo athathiweyo, umjikelo enesiphako ngokukhawuleza kukhokelela kwamanani, ibe lwe umzimba mncinane ngokupheleleyo ukusebenza iinkqubo eziliqela, nkqu ukufa kwenkwenkwe.

ubukhulu sifo

Pierre Robin Syndrome uye iindlela eziliqela nezahlukileyo kwi iqondo ubuzaza izifo:

• ifomu Easy. Umntwana abaneengxaki ezingephi kunye bajonga iingxaki ngethuba lokutyisa. ukuphefumla lakhe utrudneno. Kulo mzekelo, ugqirha umisela unyango endala, oko kukuthi ngokokuma kwaye kwenziwa kwimeko kwesibhedlele.
• I-avareji degree of ubungnqongqo. Ngexesha lokudla itakane uziva ubunzima oluphakathi kunye ehlafuna kunye ligwinya. Yena ufumana kube nzima ukuphefumla, kodwa imeko akuyonto ibalulekileyo. Ngokuqhelekileyo, umntwana onjalo kusamkelwa esibhedlele, unyango kwenkcazo ngokokuma apho kwaye bachitha glossopeksiyu - ukuqinisa ulwimi nitrogen kumlebe asezantsi.
• ifomu Severe. Enokwenzeka nzima kakhulu okanye kube buhlungu ukuba badle ukudla, uphefumlele. Kule meko ufuna amanyathelo akhawulezileyo - uqhaqho. Abanye abantu bacebisa ukondla, faka uphenyo lwangaphakathi a endla ntirho tracheostomy - a asike ze uqhoqhoqho kunye nokuqaliswa kuyo tube.

Nayo nayiphi na iqondo nobungozi besi sifo kufuneka ubuze ugqirha onamava. Self-iyeza banokubenzakalisa usana, ukuba nemiphumela emibi kakhulu vuhlayiseki.

ukungaphili

Ukuhlola usana nomonde ayikho nzima. Kodwa amazwi ngqo kufuneka bajonge wamazinyo onamava imizila yemfuza. Kunye nezinye zonyango bacebisa unyango ethile ukuze baphephe imiphumo emibi. Kulo mzekelo, iingxaki yahluke kakhulu: ukutsarhwa xa ulele, uphuhliso isiphako laryngeal, ukubonakala unomsindo, ukuphefumla kwaziwe. Xa eqala and progression of nesifo sentliziyo kwakhona Uxhokonxwa Pierre Robin syndrome: imiphumo kunje kuvandlakanywa-14% abantwana.

Umntwana nokubandezeleka kulo sifo, kubi kakhulu ukuba adle. Uxinzelelo embi eziyimfuneko ngexesha lokuncancisa, zicandeke izihlambo palatal ikuvumela ukuba ukudala na. Ukongeza, le sana umhlathi encinane kakhulu asezantsi, oko akuthethi amnike izihlunu omhle cut setyhula zomlomo ngokuqinileyo ubambe lwebele sikanina. Xa ukondla nosana ano- njengokuba palatal emfanteni kakhulu esahlula ngeempumlo nesithuba zomlomo, okwandisa ingozi ukutsarhwa. Phakathi iingxaki, oogqirha ekwabizwa ngokuba deformation kwamazinyo, malocclusion, ukukrala okondeleyo indlebe, nto leyo enokukhokelela ekubeni yokungeva ngeendlebe, kwaye nkqu yabo ayilingani nophuhliso ngokwasemzimbeni nangokwasengqondweni.

unyango elondoloza

Ixesha ukuhlawulela unyango abafanelekayo, abazali babe nethuba elihle ukuphelisa uPierre Robin syndrome. Unyango ke ngoko igcinwe. Okokuqala, usana esweni rhoqo izakhi, otolaryngologist, zamehlo, wamazinyo, wamazinyo kunye owayendifundisa ukuthetha. Okwesibini, umntwana ukulawula inkqubo lokondla zizama ukuphelisa ekuphazamiseni ngokuma eliphezulu. Ukuba ubambelela zonke iingcebiso, ngoko ukuya kwiminyaka-6, abaninzi abantwana ubukhulu kunye nokumila emhlathini asezantsi ngcembe nganye ezinga malungu abantu.

A indlela esebenzayo unyango endala unyango ngokokuma. Oku kubandakanya ukubeka wosana esiswini sakhe gama kukho njengoko kuya kuba ukukhula eyimfuneko emhlathini asezantsi. Ebuntwaneni, usana kufuneka kusoloko kule ndawo. Xa umntwana ekhula phezulu, abadala kufuneka bafundise ukuba alale kuphela ngendlela efanayo. Ukuba lwakukhula olele esiswini sakhe, udala ingcinezelo iiyure womxhuzulane entloko nolwimi iba indawo. Olu nyango kunceda-80% yamatyala.

ukungenelela kuhlinzwa

Kuyimfuneko ukuba iifom enzima nakulwimi esi sifo. Pierre Robin Syndrome ukuze samiliselwa ngokungaphelelanga bungaveli usebenzisa zokusebenza ukuqinisa ulwimi ukulungisa ngayo kuloo mmandla iindevu asezantsi. Le nkqubo, ekuthiwa glossopeksiya, ilula kwaye kuyingozi. Kwakhona, abantwana sifinyelele uqhoqhoqho kunye ityhubhu kufakwa kwi emngxunyeni: ngenxa yoko uya kukwazi ukuphefumla lula kwaye ngaphandle iingxaki. Vala lo mngxuma kwiminyaka 3. Le ndlela isetyenziswa kunqabile, kukho kufuneka malunga ne-1% abantwana zintsonkothe kakhulu sifo.

Oogqirha zingabela ucinezelo-ukuphazamiseka osteosynthesis. Le nqubo ifaka yokwaphula ezisikwe emathanjeni kwemihlathi asezantsi. Bona ifakwe izikhonkwane nca entloko, kunye nendlela esikhethekileyo lwandiseni nokwandisa emhlathini. Yonke imihla, ukwandisa ifuthe adaptation, ugqirha lifumane ukwandiswa kancinane kodwa olusebenzayo kumazantsi ngaphambili. Loo ulwandiso lwenziwa kangangenyanga, uze wenze ikhefu iiveki 8. Kuyimfuneko ukuba uphilise bone eqhelekileyo kunye nenkanuko yalo ithembekile ukwakheka entsha.

amacebo aluncedo

Abantwana Pierre Robin syndrome kufuna utshintsho kwinkqubo yesondlo. Okokuqala, kufuneka ukwandisa isiqulatho caloric yobisi; kule incum ekupheleni decanted kwaye oxutywe kukho omnye ngumntu. Okwesibini, ngexesha ukondla kufuneke uthathe ikhefu ukuba umntwana wakho ithuba lokuba ukubamba umoya wakho, kwaye ngoncedo lokukhala nokugabha ukucima ngokuma. Ukongeza, ukuba umama nangesandla nosana lwakhe esilevini, nto inceda kakhulu ukuba ukufunxa ubisi.

Okwesithathu, aba bantwana kufuneka athenge ibhotile ekhethekileyo, eyenzelwe ziingcali lokondla iintsana kunye onaso. Hole kuwo kuba banzi kakhulu kwindawo ingono eqhelekileyo, ukongeza, kukho umba ekhethekileyo ovumela umzali ukulawula ukuhamba ubisi. Yentsana kunye pacifiers ekhethekile. Okwesine, oogqirha ingenza isindululo ukuseka em- ngomlomo esesi obturator ukuba ivale mqolomba. Asimele silibale ukuba akukho nanye kwezi ndlela ayikho indlela zonyango. Esi sisixhobo nje ekukhawuleziseni nokuququzelela ukusebenza imeko unyango lwe encane yakho.