Ingabe izihlathi zengane yakho zibukeka zikhukhumele futhi umhlathi wayo ubonakala mkhulu? Kungaba yiCherubism. Ake sixoxe ngakho!

Ungase ukhathazeke kancane ngokuthi ubuso bomntwana wakho bushintshile kancane, izihlathi zakhe zibukeka zikhukhumele. Noma ingabe udokotela ukutshelile ngoshintsho esilevini sakhe? Lesi simo sisibiza ngokuthi (i-Cherubism) . Igama lingase lizwakale lingavamile, kodwa masiligcine lilula. Lesi yisimo esingavamile kakhulu, kodwa kubalulekile ukuqaphela.

Kuyini iCherubism?

Kalula nje, i-Cherubism iyisimo esingavamile sofuzo. Kwenzeka lapho izicubu ezingavamile zikhula ethanjeni lomhlathi wengane yakho esikhundleni sezicubu zethambo ezinempilo. Lesi zicubu ezingavamile azinawo umdlavuza futhi ngokuvamile azibuhlungu. Kodwa-ke, singabangela umhlathi wengane ukuba ubanzi kuzo zombili izinhlangothi futhi izihlathi zazo zibukeke zizungezile futhi zivuvukele . Ezinye izingane zingase zilahlekelwe amazinyo noma zibe namazinyo akhula ngokungajwayelekile. I-Cherubism ayithinti amanye amathambo emzimbeni, umhlathi kuphela .

Lezi zimpawu aziqali ukuvela ngokushesha ngemva kokuzalwa. Ngokuvamile zivela phakathi kweminyaka engu-2 no-7. Ubunzima besimo bungahluka kumuntu nomuntu. Ezinye izingane zingase zingabi nazimpawu nhlobo, noma izimpawu zingase zibe zicashile kakhulu futhi zingabonakali kalula. Kodwa-ke, ezimweni ezimbi kakhulu, ingane ingaba nobunzima bokukhuluma, ukugwinya, noma ngisho nokuphefumula.

Ingxenye engcono kakhulu ukuthi i-cherubism ivame ukuxazululeka yodwa lapho umuntu esekhulile ngaphandle kokwelashwa. Odokotela bahlala beqaphile ngalesi simo futhi batusa ukwelashwa kuphela uma izimpawu zinzima noma uma zithinta kakhulu izinga lempilo yengane.

I-Cherubism ivame kangakanani?

I-Cherubism ayisona isimo esivamile kakhulu. Abacwaningi abazi kahle ukuthi bangaki abantu abanalesi simo, kodwa benze izilinganiso ezithile ngokusekelwe ezihlokweni zezokwelapha ezishicilelwe. Ucwaningo lwango-2019 luthole ukuthi kunezimo ezingu-513 ze-Cherubism ezibikiwe ezincwadini zezokwelapha. Ngakho ungabona ukuthi kuyivelakancane kangakanani.

Ziyini izimpawu zeCherubism?

Lezi yizimpawu ezivamile zeCherubism:

• Ithambo lomhlathi ongezansi (i-mandible) libanzi kunalokho obekulindelwe.
• Ukukhula okufana ne-cyst endaweni yomhlathi wengane.
• Izihlathi eziyindilinga, ezivuvukele.
• Izinguquko esimweni samazinyo, amazinyo angekho, noma amazinyo athintekile.

Ezinye izingane zingase zibe nalezi zimpawu:

• Amehlo abonakala ephuma futhi ebheke phezulu. Ingxenye emhlophe yeso (i-sclera) ibonakala ngaphansi kwe-iris emnyama.
• Izicubu ezengeziwe emhlathini ongaphezulu zithinta iso kanye nemizwa ehambisana nalo, okubangela ukulahlekelwa umbono noma ukulahlekelwa umbono kancane kancane.
• Ukuphefumula komlomo.
• Ukuhona.
• Ukuphefumula okuvimbelayo kokulala.
• Ubunzima bokukhuluma noma ukugwinya.

Ziyini izimbangela zeCherubism?

Iningi lamacala e-Cherubism (angaphezu kuka-90%) abangelwa izinguquko zezakhi zofuzo ezibizwa ngokuthi `SH3BP2`.

Lesi sakhi sofuzo esithi `SH3BP2` siyala umzimba wethu ukuthi singawenza kanjani iphrotheni ethi `SH3BP2`. Le phrotheni ihilelekile enqubweni yokususa izicubu ezindala zamathambo nokwakha izicubu ezintsha zamathambo (ukulungiswa kwamathambo). Ukuguqulwa kwezakhi zofuzo kubangela ukuthi umzimba ukhiqize kakhulu le phrotheni ethi `SH3BP2`.

Uma le phrotheni ikhiqizwa ngokweqile, ukuvuvukala kwenzeka ethanjeni lomhlathi. Kuphinde kwandise ukukhiqizwa kwamaseli abizwa ngokuthi ama-osteoclasts . Ama-osteoclasts awuhlobo lweseli oludlala indima ebalulekile ekuqhekekeni kwezicubu zamathambo umzimba ongasawadingi. Kodwa-ke, lapho la maseli eba maningi kakhulu, aqala ukuqhekeka izicubu zamathambo ethanjeni lomhlathi uma engadingeki. Lokhu kulahlekelwa kwamathambo okungavamile, kuhlangene nokuvuvukala okubangelwa ukwanda kwe-SH3BP2, kuholela ekwakhekeni kokukhula okufana ne-cyst ethanjeni lomhlathi. Lokhu kukhula kukhula kancane kancane, okuyikho okubangela izimpawu eziphawulekayo zalesi simo (i-Cherubism).

Kodwa-ke, ezinye izingane ezine-Cherubism azinawo uguquko lwezakhi zofuzo lwe-SH3BP2. Ezimweni ezinjalo, ezinye izinguquko zezakhi zofuzo zingase zibe yimbangela. Kodwa abacwaningi abakakatholi ukuthi yiziphi ngempela lezo zinguquko.

Ingabe (ubuCherubism) buyinto efika izizukulwane ngezizukulwane?

Yebo, izingane eziningi zizuza i-genetic mutation ebangela i-Cherubism. Lesi simo sidluliselwa ngendlela elawulwa yi-autosomal yefa. Kalula nje, uma umzali oyedwa ene-genetic mutation, ingane inethuba lokuyithola nayo.

Kodwa-ke, ezinye izingane zingaba nalesi simo ngisho noma kungekho muntu emndenini oke waba naso ngaphambili. Abacwaningi bakubiza lokhu ngokuthi 'ukuguqulwa kwezakhi zofuzo' . Okusho ukuthi, ushintsho olusha lwezakhi zofuzo olwenzeka kumuntu, ngaphandle komlando womndeni.

Ngakho-ke, nakuba i-Cherubism ivame ufuzo, akunjalo ngaso sonke isikhathi. Ngisho noma kungekho muntu emndenini onalesi simo, odokotela babheka i-Cherubism njengokuxilongwa okungenzeka.

Ingabe zikhona ezinye izimo ezihlobene neCherubism?

Kwabanye abantwana, i-Cherubism ingenzeka futhi njengengxenye yesinye isimo sofuzo. Ezinye zalezi zimo ezihlobene zifaka:

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

Ezimweni ezinjalo, imbangela yeCherubism akuyona i-SH3BP2 gene mutation. Kunalokho, ezinye izinguquko zezakhi zofuzo ezihlobene ne-syndrome yizo ezibangela lokho.

Odokotela bahlonza kanjani i-Cherubism?

Odokotela balandela lezi zinyathelo ukuze baxilonge i-Cherubism:

• Ukuhlolwa komzimba: Lokhu kuhilela ukuhlola ingane izimpawu ezifana nomhlathi obanzi ngokungavamile, amazinyo angekho, noma izinkinga zamazinyo (i-Cherubism).
• Uku-oda izivivinyo zesithombe: Lokhu kufaka phakathi izivivinyo ezifana ne -X-ray noma i-CT scans (i-computed tomography scans) . Lokhu kungasiza ekuhloleni kangcono isimo sethambo lomhlathi.
• Uku-oda ukuhlolwa kwezakhi zofuzo: Lokhu kuhlolwa kwenziwa ukuze kuhlolwe izinguquko zezakhi zofuzo.

Odokotela badinga futhi ukuqeda ezinye izimo ezinezimpawu ezifanayo, njenge -fibrous dysplasia noma i-aneurysmal bone cysts , kanye nokunquma ukuthi i-Cherubism iyingxenye ye-genetic syndrome.

Yiziphi izindlela zokwelapha i-Cherubism?

Iningi lezingane ezine-cherubism azidingi ukwelashwa okukhethekile. Odokotela bezingane bavame ukuthatha indlela ethi "linda ubone". Okusho ukuthi, babheka lesi simo futhi babone ukuthi sikhula kanjani. Ezimweni eziningi, lezi zicubu ezingavamile ziyakhula kuze kube yilapho ingane ifinyelela ekuthombeni, zihlala zinjalo iminyaka embalwa, bese kancane kancane ziqala ukuncipha. Izimpawu ze-cherubism zivame ukuphela lapho umuntu esemdala.

Kodwa-ke, uma ingane yakho inezinkinga zokudla noma ukuphefumula, udokotela angase ancome ukuhlinzwa. Kodwa-ke, odokotela bavame ukuncoma ukuhlinzwa ngemva kokuba ukukhula kwezicubu okungavamile sekuphelile. Uma umuntu esenezimpawu futhi efuna ukushintsha ukubukeka kobuso bakhe, ukuhlinzwa kungacatshangelwa. Ukuhlinzwa kabusha kusiza ekwakheni kabusha ubuso bomuntu ngendlela ayithandayo.

Ukwelashwa okusekelayo

I-Cherubism ingathinta izici eziningi zokuphila kwengane, ikakhulukazi uma isimo sibi kakhulu. Ngakho-ke, ingane ingadinga ukwelashwa okufanela izidingo zayo. Izinketho zokwelapha zingafaka:

• Ukwelashwa kwamazinyo: Izingane ezine-cherubism zingadinga ukwelashwa kwamazinyo, njengokukhishwa kwamazinyo noma ukufakelwa . Uma ukukhula okufana ne-cyst emhlathini kuqala ukuncipha, ingane ingadinga ne-braces (orthodontics) .
• Ukusekelwa okubonakalayo: Ezimweni ezimbi kakhulu ze-cherubism, umbono wengane ungathinteka. Udokotela wamehlo angahlola umbono wengane futhi athole ikhambi elingcono kakhulu lezidingo zayo.
• Usizo ngokukhuluma nokugwinya: Uma ingane inenkinga yokukhuluma noma yokugwinya,Udokotela wezifo zolimi lokukhuluma (SLP) angasiza.
• Ukusekelwa kwengqondo: I-Cherubism ingathinta ukuzethemba kwengane. Kubalulekile ukukhuluma nengane yakho ngendlela ezizwa ngayo nokuthi lesi simo sithinta kanjani indlela ezibona ngayo. Ingane yakho ingazuza ngokukhuluma nodokotela wengqondo wengane ngemizwa yayo.

Iyini imbono yalabo abaneCherubism?

Iningi labantu ngeke libe nezimpawu ze-Cherubism lapho lineminyaka engu-30. Ngaleso sikhathi, izicubu ezingavamile sezithathelwe indawo izicubu ezivamile zamathambo.

Akuvamile kakhulu ukuthi izimpawu ze-cherubism ziqhubeke kuze kube yilapho umuntu emdala. Uma lokhu kwenzeka, udokotela uzonikeza isiqondiso nokusekelwa okudingekayo.

Ingabe ubuCherubism bungavinjelwa?

Odokotela abazi ukuthi bangakuvimbela kanjani lesi simo. Uma othile emndenini wakho ene-Cherubism, kungaba ngumqondo omuhle ukuthola ukwelulekwa ngofuzo ngaphambi kokukhulelwa.

Ngingayinakekela kanjani ingane yami?

Uma ingane yakho ineCherubism, qaphela lezi zinto:

• Qhubeka nokuyisa ingane yakho kudokotela wezingane. Udokotela uzokutshela ukuthi kufanele ubuye kangaki.
• Hlola amehlo engane yakho ngokwesimiso esinconywe udokotela.
• Sekela ingane ngokomzwelo. Mkhuthaze, khuluma naye.

Kufanele ngishayele nini udokotela ucingo?

Shayela udokotela wezingane uma ingane yakho inanoma yikuphi kwalokhu okulandelayo:

• Ubunzima bokuphefumula, njengokuhona noma i-sleep apnea .
• Ubunzima bokugwinya.
• Ubunzima bokukhuluma.
• Izinkinga zokubona.

Lavela kanjani igama elithi (iCherubism)?

Lesi simo saqanjwa ngo-1933 nguDkt. William A. Jones . Wakhetha igama elithi "Cherubism" ngoba izimpawu zalesi simo zifaka phakathi ubuso obufana nobomntwana, obukhukhumele kanye namehlo abheke phezulu ngezinye izikhathi. Wayekholelwa ukuthi lezi zici zifana neze "kherubi," isithombe esihle, esiyingelosi esibonakala kwezobuciko kanye namasiko ahlukahlukene enkolo. Leli gama belilokhu lisetshenziswa kusukela ngaleso sikhathi.

Kungaba yinto eshaqisayo ukuzwa ukuthi ingane yakho inesifo esingavamile. Endabeni ye-Cherubism, izimpawu azibonakali lapho izalwa, ngakho-ke ungase ukhathazeke lapho ubona izinguquko ebusweni bengane yakho noma lapho udokotela ekhuluma ngazo. Kodwa khumbula, izimo eziningi ze-Cherubism ziphela zodwa ngesikhathi ingane ifika ebudaleni.Ezimweni ezimbi kakhulu, odokotela bangelapha imiphumela yalesi simo (i-Cherubism). Ukunikeza ingane yakho uthando oluningi kanye nokusekelwa ngokomzwelo kuzoyisiza ukuthi ithole konke ukunakekelwa ekudingayo ukuze isebenzise kahle ubuntwana bayo kanye nobusha bayo.

Umlayezo Wokuya Nawe Ekhaya

I-Cherubism yisimo esingavamile sofuzo esithinta umhlathi. Lokhu kungabangela ukuba izihlathi zengane zivuvuke futhi umhlathi ubonakale ubanzi. Lokhu kuvame ukuba ngcono njengoba ingane ikhula. Kodwa-ke, uma inenkinga yokuphefumula noma yokugwinya, bona udokotela. Into ebaluleke kakhulu ukuqhubeka nokunikeza ingane yakho uthando lwakho nokusekela. Into ebaluleke kakhulu ukusiza ingane yakho ihlale iqinile ngenkathi ilandela iseluleko sezokwelapha. Ungakhathazeki, awuwedwa kulolu hambo.

I- Cherubism, izifo zofuzo, umhlathi, izihlathi ezivuvukele, izifo zezingane, izinkinga zamazinyo, i-SH3BP2 gene