Ngaba izidlele zomntwana wakho zibonakala zikhukhumele kwaye umhlathi wakhe ubonakala mkhulu? Isenokuba yiCherubism. Makhe sithethe ngayo!

Usenokuba nexhala kancinci ngokuba ubuso bomntwana wakho butshintshile kancinci, izidlele zakhe zibonakala zikhukhumele. Okanye ngaba ugqirha ukuxelele ngotshintsho olusesilevini sakhe? Le meko siyibiza ngokuba yi-Cherubism . Igama lisenokuvakala lingaqhelekanga, kodwa masiyigcine ilula. Le yimeko engaqhelekanga kakhulu, kodwa kubalulekile ukuba uyiqaphele.

Yintoni iCherubism?

Ngamafutshane, iCherubism yimeko engaqhelekanga yemfuza. Yenzeka xa izicubu ezingaqhelekanga zikhula kumhlathi womntwana wakho endaweni yezicubu zethambo eziphilileyo. Le zicubu ezingaqhelekanga azinamhlaza kwaye zihlala zingenabuhlungu. Nangona kunjalo, inokubangela ukuba umhlathi womntwana ube banzi kumacala omabini kwaye izidlele zakhe zijongeke zijikeleze kwaye zidumbile . Abanye abantwana banokulahlekelwa ngamazinyo okanye babe namazinyo akhula ngokungacwangciswanga. ICherubism ayichaphazeli amanye amathambo emzimbeni, kuphela umhlathi .

Ezi mpawu aziqali ukubonakala kwangoko emva kokuzalwa. Zihlala zibonakala phakathi kweminyaka emi-2 ne-7 ubudala. Ubunzima bemeko bunokwahluka kumntu nomntu. Abanye abantwana basenokungabi nazo iimpawu konke konke, okanye iimpawu zisenokungabonakali lula kwaye zingabonakali kangako. Nangona kunjalo, kwiimeko ezinzima ngakumbi, umntwana unokuba nobunzima bokuthetha, ukuginya, okanye nokuphefumla.

Eyona nto ingcono kukuba i-cherubism idla ngokuziphelela xa umntu emdala ngaphandle konyango. Oogqirha bahlala beyikhangela le meko kwaye bacebisa unyango kuphela xa iimpawu zinzima okanye ukuba zichaphazela kakhulu umgangatho wobomi bomntwana.

Ixhaphake kangakanani iCherubism?

I-Cherubism ayisiyonto ixhaphakileyo. Abaphandi abazi kakuhle ukuba bangaphi abantu abanale meko, kodwa benze uqikelelo oluthile ngokusekelwe kumanqaku ezonyango ashicilelweyo. Uphononongo luka-2019 lufumanise ukuba kukho iimeko ezingama-513 ze-Cherubism ezixeliweyo kwiincwadi zonyango. Ngoko ke ungabona ukuba ayiqhelekanga kangakanani.

Zithini iimpawu zeCherubism?

Ezi ziimpawu eziqhelekileyo zeCherubism:

• Ithambo lomhlathi ongezantsi (i-mandible) libanzi kunokuba bekulindelwe.
• Ukukhula okufana ne-cyst kwindawo yomhlathi womntwana.
• Izidlele ezingqukuva nezivuvukileyo.
• Utshintsho kwimilo yamazinyo, amazinyo angekhoyo, okanye amazinyo achaphazelekileyo.

Abanye abantwana banokuba nezi mpawu:

• Amehlo abonakala ngathi avele phezulu. Inxalenye emhlophe yeliso (i-sclera) ibonakala phantsi kwe-iris emnyama.
• Izicubu ezingaphezulu kumhlathi ongaphezulu zichaphazela iliso kunye nemithambo-luvo enxulumene nalo, nto leyo ebangela ukulahleka kokubona okanye ukulahleka kokubona kancinci kancinci.
• Ukuphefumla ngomlomo.
• Ukurhona.
• Ukuphazamiseka kokulala.
• Ubunzima bokuthetha okanye ukuginya.

Zithini izizathu zeCherubism?

Uninzi lweemeko zeCherubism (ngaphezulu kwama-90%) zibangelwa kukuguquka kwezakhi zofuzo ezibizwa ngokuba yi-`SH3BP2`.

Le gene ye-`SH3BP2` iyalela umzimba wethu indlela yokwenza iproteni ye-`SH3BP2`. Le proteni ibandakanyeka kwinkqubo yokususa izicubu ezindala zethambo kunye nokwakha izicubu ezintsha zethambo (ukulungiswa kwamathambo). Utshintsho lwezakhi zofuzo lubangela ukuba umzimba uvelise iproteni eninzi kakhulu ye-`SH3BP2`.

Xa le protein iveliswa ngokugqithisileyo, ukudumba kwenzeka kwithambo lomhlathi. Kwakhona kwandisa ukuveliswa kweeseli ezibizwa ngokuba zii-osteoclasts . Ii-Osteoclasts luhlobo lweeseli ezidlala indima ebalulekileyo ekuqhekezeni izicubu zamathambo ezingasafunekiyo ngumzimba. Nangona kunjalo, xa ezi seli ziba zininzi kakhulu, ziqala ukuqhekeza izicubu zamathambo kwithambo lomhlathi xa zingafuneki. Oku kulahleka kwamathambo okungaqhelekanga, kunye nokudumba okubangelwa kukwanda kwe-SH3BP2, kukhokelela ekwakhiweni kokukhula okufana ne-cyst kwithambo lomhlathi. Oku kukhula kukhula kancinci kancinci, nto leyo ebangela iimpawu eziphawulekayo zesifo (iCherubism).

Nangona kunjalo, abanye abantwana abaneCherubism abanayo i-SH3BP2 gene mutation. Kwimeko ezinjalo, ezinye ii-genetic mutations zinokuba yimbangela. Kodwa abaphandi abakafumani ukuba zeziphi kanye kanye ezo nguqu.

Ngaba (iCherubism) yinto evela kwizizukulwana ngezizukulwana?

Ewe, abantwana abaninzi bazuza i-genetic mutation ebangela i-Cherubism. Le meko idluliselwa kwi-autosomal dominant pattern yelifa. Ngamafutshane, ukuba omnye umzali une-genetic mutation, umntwana unethuba lokuyifumana.

Nangona kunjalo, abanye abantwana banokuba nale meko nokuba akukho mntu kusapho lwakhe wakha waba nayo ngaphambili. Abaphandi bayibiza le nto ngokuthi 'yinguqu entsha' . Oko kukuthi, utshintsho olutsha lwemfuza olwenzeka emntwini, ngaphandle kwembali yosapho.

Ngoko ke, nangona iCherubism idla ngokuba yimfuza, ayisoloko injalo. Nokuba akukho mntu kusapho onale meko, oogqirha bacinga ukuba iCherubism yinto enokwenzeka ukuba ifumaneke.

Ngaba kukho ezinye iimeko ezinxulumene neCherubism?

Kwabanye abantwana, iCherubism inokwenzeka njengenxalenye yesinye isifo semfuza. Ezinye zezi meko zinxulumene nazo ziquka:

• `(Isifo seFragile X)`
• `(Isifo sikaJaffe-Campanacci)`
• `(Uhlobo lwe-Neurofibromatosis 1)`
• `(Isifo sikaNoonan)`
• `(Isifo sikaRamon)`

Kwimeko ezinjalo, unobangela weCherubism asikokuguquka kwezakhi zofuzo zeSH3BP2. Endaweni yoko, ezinye iinguqu zezakhi zofuzo ezinxulumene ne-syndrome zibangela oku.

Oogqirha bayixilonga njani iCherubism?

Oogqirha balandela la manyathelo ukuze baxilonge iCherubism:

• Uvavanyo lomzimba: Oku kuquka ukujonga umntwana iimpawu ezifana nomhlathi obanzi ngokungaqhelekanga, amazinyo angekhoyo, okanye iingxaki zamazinyo (iCherubism).
• Uku-odola iimvavanyo zomfanekiso: Ezi ziquka iimvavanyo ezifana ne -X-ray okanye i-CT scans (i-computed tomography scans) . Ezi zinokunceda ekuvavanyeni ngcono imeko yomhlathi.
• Uku-odola uvavanyo lwemfuza: Olu vavanyo lwenziwa ukujonga utshintsho lwemfuza.

Oogqirha kufuneka bakhuphe ezinye izifo ezineempawu ezifanayo, ezifana ne -fibrous dysplasia okanye i-aneurysmal bone cysts , baze bafumanise ukuba i-Cherubism iyinxalenye ye-genetic syndrome.

Ziziphi iindlela zonyango lweCherubism?

Uninzi lwabantwana abane-cherubism abadingi naluphi na unyango olukhethekileyo. Iingcali zabantwana zihlala zithatha indlela ethi "linda ubone". Oko kukuthi, bajonga imeko baze babone ukuba ikhula njani. Kwiimeko ezininzi, ezi zicubu ezingaqhelekanga ziyakhula de umntwana afikelele ekufikiseni, zihlale zinjalo iminyaka embalwa, zize ziqale ukuncipha kancinci kancinci. Iimpawu ze-cherubism zihlala ziphela xa umntwana eselula.

Nangona kunjalo, ukuba umntwana wakho uneengxaki zokutya okanye zokuphefumla, ugqirha unokucebisa utyando. Nangona kunjalo, oogqirha badla ngokucebisa utyando emva kokuba ukukhula okungaqhelekanga kwezicubu kuyekile. Ukuba umntu useneempawu kwaye ufuna ukutshintsha inkangeleko yobuso bakhe, utyando lunokucingelwa. Utyando lokwakha kwakhona lunceda ekubumbeni ubuso bomntu ngendlela ayithandayo.

Unyango oluxhasayo

I-Cherubism inokuchaphazela iinkalo ezininzi zobomi bomntwana, ingakumbi ukuba imeko imbi kakhulu. Ke ngoko, umntwana unokufuna unyango oluhambelana neemfuno zakhe. Iindlela zonyango zingabandakanya:

• Unyango lwamazinyo: Abantwana abane-cherubism banokufuna unyango lwamazinyo, njengokukhutshwa kwamazinyo okanye ukufakelwa kwamazinyo . Xa ukukhula okufana ne-cyst emhlathini kuqala ukuncipha, umntwana usenokufuna nee-braces (orthodontics) .
• Inkxaso yokubona: Kwiimeko ezinzima ze-cherubism, umbono womntwana unokuchaphazeleka. Ingcali yamehlo inokuhlola umbono womntwana ize ifumane isisombululo esifanelekileyo kwiimfuno zakhe.
• Nceda ekuthetheni nasekuginyeni: Ukuba umntwana unengxaki yokuthetha okanye yokuginya,Ingcali yezifo zolwimi lokuthetha (SLP) inokunceda.
• Inkxaso yengqondo: I-Cherubism inokuchaphazela ukuzithemba komntwana. Kubalulekile ukuthetha nomntwana wakho malunga nendlela aziva ngayo kunye nendlela le meko echaphazela ngayo indlela azijonga ngayo. Umntwana wakho angazuza ngokuthetha nogqirha wengqondo yabantwana malunga neemvakalelo zakhe.

Iyintoni imbono yabo baneCherubism?

Uninzi lwabantu aluyi kuba nazimpawu zeCherubism xa beneminyaka engama-30 ubudala. Ngelo xesha, izicubu ezingaqhelekanga zithathelwe indawo yizicubu zethambo eziqhelekileyo.

Amaxesha amaninzi, iimpawu ze-cherubism zinokuhlala zize zibe zikhulile. Ukuba oku kuyenzeka, ugqirha uya kunika isikhokelo nenkxaso efunekayo.

Ngaba iCherubism ingathintelwa?

Oogqirha abazi ukuba bangasithintela njani esi sifo. Ukuba kukho umntu kusapho lwakho oneCherubism, kungaba licebo elihle ukufumana ingcebiso ngemfuza ngaphambi kokuba ukhulelwe.

Ndingamnyamekela njani umntwana wam?

Ukuba umntwana wakho uneCherubism, qaphela ezi zinto zilandelayo:

• Qhubeka nokuthatha umntwana wakho aye kugqirha wabantwana. Ugqirha uza kukuxelela ukuba ubuye kangaphi.
• Hlola amehlo omntwana wakho ngokweshedyuli ecetyiswe ngugqirha.
• Mxhase umntwana ngokweemvakalelo. Mkhuthaze, thetha naye.

Ndingambiza nini ugqirha?

Fowunela ugqirha wezingane ukuba umntwana wakho unenye yezi zinto zilandelayo:

• Ubunzima bokuphefumla, njengokurhona okanye i-sleep apnea .
• Ubunzima bokuginya.
• Ubunzima bokuthetha.
• Iingxaki zokubona.

Lavela njani igama elithi (iCherubism)?

Esi sifo sathiywa ngo-1933 nguGqr. William A. Jones . Wakhetha igama elithi "Cherubism" kuba iimpawu zaso ziquka ubuso obufana nobomntwana, obuvuvukileyo kunye namehlo ajonge phezulu ngamanye amaxesha. Wayekholelwa ukuba ezi mpawu zifana neze "kherub," umfanekiso omhle, oyingelosi obonwa kubugcisa nakwiinkcubeko ezahlukeneyo zonqulo. Eli gama belisetyenziswa ukususela ngoko.

Kungamangalisa ukwazi ukuba umntwana wakho unesimo esingaqhelekanga. Kwimeko yeCherubism, iimpawu azibonakali xa ezalwa, ngoko ke unokukhathazeka xa ubona utshintsho ebusweni bomntwana wakho okanye xa ugqirha ekhankanya olo tshintsho. Kodwa khumbula, uninzi lweemeko zeCherubism ziyaphela zodwa xa umntwana efikelela ebudaleni.Kwiimeko ezinzima, oogqirha banokunyanga iziphumo zeso sifo (iCherubism). Ukunika umntwana wakho uthando oluninzi kunye nenkxaso yeemvakalelo kuya kumnceda afumane lonke unyango aludingayo ukuze asebenzise kakuhle ubuntwana bakhe kunye nokufikisa kwakhe.

Umyalezo Wokuya Ekhaya

I-Cherubism yimeko engaqhelekanga yemfuza echaphazela umhlathi. Oku kunokubangela ukuba izidlele zomntwana zivuvukale kwaye umhlathi ubonakale ubanzi. Oku kudla ngokuba ngcono njengoko umntwana ekhula. Nangona kunjalo, ukuba unengxaki yokuphefumla okanye yokuginya, bona ugqirha. Eyona nto ibalulekileyo kuqhubeka nokunika umntwana wakho uthando lwakho kunye nenkxaso. Eyona nto ibalulekileyo kukunceda umntwana wakho ahlale eqinile ngelixa ulandela iingcebiso zonyango. Ungakhathazeki, awuwedwa kolu hambo.

I- Cherubism, izifo zemfuza, umhlathi, izidlele ezivuvukileyo, izifo zabantwana, iingxaki zamazinyo, i-SH3BP2 gene