Ke nānā aku nei anei nā papālina o kāu pēpē e pehu ana a me he mea lā he nui kona ʻauwae? He Cherubism paha ia. E kamaʻilio kākou e pili ana i ia mea!

Ke hopohopo iki nei paha ʻoe ua loli iki ke alo o kāu keiki liʻiliʻi, me ko lākou mau papālina e pehu ana. A i ʻole ua haʻi aku ke kauka iā ʻoe e pili ana i kahi loli ma ko lākou ʻauwae? Kāhea mākou i kēia ʻano (Cherubism) . He mea ʻē paha ke kani ʻana o ka inoa, akā e maʻalahi kākou. He ʻano kakaikahi loa kēia, akā he mea nui e makaʻala i ia mea.

He aha ka Kerubisma?

I ka ʻōlelo maʻalahi, he maʻi hoʻoilina laha ʻole ka Cherubism. Hana ʻia ia i ka wā e ulu ai nā ʻiʻo ʻē aʻe i loko o ka iwi ʻauwae o kāu keiki ma kahi o ka iwi olakino. ʻAʻole maʻi kanesa kēia ʻiʻo ʻē a ʻaʻohe ʻeha. Eia nō naʻe, hiki iā ia ke hana i ka ʻauwae o ke keiki e ākea ma nā ʻaoʻao ʻelua a me ko lākou mau papālina e nānā poepoe a pehu . Hiki i kekahi mau keiki ke nalowale nā ​​niho a i ʻole he mau niho e ulu ana i ke ʻano ʻē. ʻAʻole pili ka Cherubism i nā iwi ʻē aʻe o ke kino, ʻo ka iwi ʻauwae wale nō .

ʻAʻole hoʻomaka koke kēia mau hōʻailona e ʻike ʻia ma hope o ka hānau ʻana. ʻIke pinepine ʻia lākou ma waena o nā makahiki 2 a me 7. Hiki ke ʻokoʻa ke koʻikoʻi o ke ʻano mai kēlā kanaka a kēia kanaka. ʻAʻohe hōʻailona iki o kekahi mau keiki, a he palupalu loa paha nā hōʻailona a ʻike iki ʻia. Eia nō naʻe, i nā hihia koʻikoʻi, hiki i ke keiki ke paʻakikī i ka ʻōlelo ʻana, ka moni ʻana, a i ʻole ka hanu ʻana.

ʻO ka mea maikaʻi loa, ʻo ia ka hoʻopau pinepine ʻana o ke cherubism iā ia iho i ka wā makua me ka ʻole o ka lāʻau lapaʻau. Ke nānā mau nei nā kauka i kēia kūlana a ke paipai wale nei i ka lāʻau lapaʻau inā koʻikoʻi nā hōʻailona a inā paha e hoʻopilikia nui ana i ke ʻano o ke ola o ke keiki.

Pehea ka maʻamau o ke Kerubisma?

ʻAʻole maʻamau ka Cherubism. ʻAʻole maopopo i nā kānaka noiʻi i ka nui o ka poʻe i loaʻa i kēia maʻi, akā ua hana lākou i kekahi mau kuhi ma muli o nā ʻatikala lapaʻau i paʻi ʻia. Ua ʻike ʻia kahi noiʻi 2019 he 513 mau hihia o Cherubism i hōʻike ʻia ma nā palapala lapaʻau. No laila hiki iā ʻoe ke ʻike i kona kakaikahi.

He aha nā hōʻailona o ke Kerubimana?

Eia nā hōʻailona maʻamau o ke Cherubism:

• ʻOi aku ka laulā o ka iwi ʻāwae lalo (mandible) ma mua o ka mea i manaʻo ʻia.
• Nā ulu ʻana e like me ka cyst ma ka ʻauwae o ke keiki.
• Nā papālina poepoe a pupuhi.
• Nā loli i ke ʻano o nā niho, nā niho nalowale, a i ʻole nā ​​​​niho i pā.

Hiki i kekahi mau keiki ke loaʻa i kēia mau hōʻailona:

• Me he mea lā e puka mai ana nā maka a e nānā ana i luna. ʻIke ʻia ka ʻāpana keʻokeʻo o ka maka (sclera) ma lalo o ka iris ʻeleʻele.
• Hoʻopilikia ka ʻiʻo keu ma ka ʻāwae luna i ka maka a me nā aʻalolo pili, e hoʻoulu ai i ka nalowale o ka ʻike a i ʻole ka nalowale mālie o ka ʻike.
• Ka hanu ʻana o ka waha.
• Ka hānō ʻana.
• ʻO ka apnea hiamoe obstructive.
• Ka paʻakikī i ka ʻōlelo ʻana a i ʻole ke ale ʻana.

He aha nā kumu o ke Kerubi?

ʻO ka hapa nui o nā hihia o Cherubism (ʻoi aku ma mua o 90%) ke kumu o nā mutations i loko o ka gene i kapa ʻia ʻo SH3BP2.

Ke kuhikuhi nei kēia gene SH3BP2 i ko mākou kino i ke ʻano o ka hana ʻana i ka protein SH3BP2. Pili kēia protein i ke kaʻina hana o ka wehe ʻana i nā ʻiʻo iwi kahiko a me ke kūkulu ʻana i nā ʻiʻo iwi hou (bone remodeling). ʻO nā hoʻololi genetic ke kumu o ke kino e hana nui i kēia protein SH3BP2.

Ke hana nui ʻia kēia protein, loaʻa ka mumū ma ka iwi ʻauwae. Hoʻonui pū ia i ka hana ʻana o nā cell i kapa ʻia ʻo osteoclasts . ʻO nā Osteoclasts kahi ʻano cell e pāʻani ana i kahi kuleana koʻikoʻi i ka wāwahi ʻana i nā ʻiʻo iwi i pono ʻole ai ke kino. Eia nō naʻe, i ka wā e nui loa ai kēia mau cell, hoʻomaka lākou e wāwahi i nā ʻiʻo iwi ma ka iwi ʻauwae i ka wā ʻaʻole pono. ʻO kēia nalowale iwi maʻamau, i hui pū ʻia me ka mumū i hoʻokumu ʻia e ka hoʻonui ʻana o SH3BP2, e alakaʻi i ka hoʻokumu ʻia ʻana o nā ulu ʻana e like me ka cyst ma ka iwi ʻauwae. Hoʻonui mālie kēia mau ulu ʻana, ʻo ia ke kumu o nā hōʻailona maʻamau o ke ʻano (Cherubism).

Eia nō naʻe, ʻaʻohe o kekahi mau keiki me Cherubism i ka mutation gene SH3BP2. I ia mau hihia, hiki i nā mutation genetic ʻē aʻe ke kuleana. Akā, ʻaʻole i ʻike pono nā kānaka noiʻi i kēlā mau mutation.

He mea anei (Kerubi) e hele mai ana mai kēlā hanauna a i kēia hanauna?

ʻAe, hoʻoilina ka nui o nā keiki i ka hoʻololi ʻana o ka genetic e hoʻoulu ai i ka Cherubism. Hoʻoili ʻia ke ʻano ma ke ʻano hoʻoilina autosomal dominant. I ka ʻōlelo maʻalahi, inā loaʻa i kekahi makua ka hoʻololi ʻana o ka genetic, loaʻa i ke keiki ka manawa e loaʻa ai iā ia.

Eia nō naʻe, hiki i kekahi mau keiki ke loaʻa i ke kūlana ʻoiai inā ʻaʻohe mea o ka ʻohana i loaʻa iā ia ma mua. Ua kapa ʻia kēia e nā kānaka noiʻi he 'de novo mutation' . ʻO ia hoʻi, he loli genetic hou e hana ʻia i loko o ke kanaka, me ka ʻole o ka mōʻaukala ʻohana.

No laila, ʻoiai he mea hoʻoilina ka Cherubism, ʻaʻole ia ka hihia mau. ʻOiai inā ʻaʻohe mea o ka ʻohana i loaʻa i ke ʻano, manaʻo nā kauka he hōʻailona paha ka Cherubism.

Aia kekahi mau kūlana ʻē aʻe e pili ana me ke Kerubi?

I kekahi mau keiki, hiki ke hana ʻia ke Cherubism ma ke ʻano he ʻāpana o kahi maʻi genetic ʻē aʻe. ʻO kekahi o kēia mau kūlana pili:

• (Fragile X syndrome)
• ʻO ka maʻi Jaffe-Campanacci
• (Neurofibromatosis ʻano 1)
• (Noonan syndrome)
• (ʻO ka maʻi Ramon)

I ia mau hihia, ʻaʻole ke kumu o Cherubism ka mutation gene SH3BP2. Akā, ʻo nā loli genetic ʻē aʻe e pili ana i ka syndrome ke kuleana.

Pehea e ʻike ai nā kauka i ka Cherubism?

Hana nā kauka i kēia mau ʻanuʻu e ʻike ai i ka Cherubism:

• Hoʻokolokolo kino: Pili kēia i ka nānā ʻana i ke keiki no nā hōʻailona e like me ka ʻāwae ākea loa, nā niho nalowale, a i ʻole nā ​​​​pilikia niho (Cherubism).
• Ke kauoha ʻana i nā hoʻokolohua kiʻi: ʻO kēia mau mea e komo pū me nā hoʻokolohua e like me nā X-ray a i ʻole nā ​​​​scan CT (computed tomography scans) . Hiki i kēia ke kōkua i ka loiloi maikaʻi ʻana i ke kūlana o ka iwi ʻauwae.
• Ke kauoha ʻana i ka hoʻāʻo genetic: Hana ʻia kēia mau hoʻokolohua e nānā i nā hoʻololi genetic.

Pono nō hoʻi nā kauka e kāpae i nā kūlana ʻē aʻe i loaʻa nā hōʻailona like, e like me ka fibrous dysplasia a i ʻole nā ​​​​cysts iwi aneurysmal , a e hoʻoholo inā he ʻāpana o kahi maʻi hoʻoilina ʻo Cherubism.

He aha nā lāʻau lapaʻau no ke Cherubism?

ʻAʻole pono ka hapa nui o nā keiki me ke kerubim i kekahi lāʻau lapaʻau kūikawā. Lawe pinepine nā kauka pediatric i kahi ala "kali a ʻike". ʻO ia hoʻi, nānā lākou i ke kūlana a ʻike pehea e ulu ai. I ka hapanui o nā hihia, ulu kēia mau ʻiʻo ʻē a hiki i ka wā ʻōpio o ke keiki, noho like no kekahi mau makahiki, a laila hoʻomaka mālie e emi. ʻO ka maʻamau, nalowale nā ​​​​hōʻailona o ke kerubim i ka wā makua mua.

Eia nō naʻe, inā pilikia kāu keiki i ka ʻai ʻana a i ʻole ka hanu ʻana, hiki i ke kauka ke ʻōlelo aku i ke ʻoki kino. Eia nō naʻe, ʻōlelo pinepine nā kauka i ke ʻoki kino ma hope o ka pau ʻana o ka ulu ʻana o nā ʻiʻo ʻē. Inā loaʻa mau nā hōʻailona i ke kanaka a makemake e hoʻololi i ke ʻano o ko lākou helehelena, hiki ke noʻonoʻo ʻia ke ʻoki kino. Kōkua ke ʻoki kino hou i ka hoʻoponopono hou ʻana i ka helehelena o ke kanaka i ko lākou makemake.

Nā lāʻau lapaʻau kākoʻo

Hiki i ke kerubism ke hoʻopilikia i nā ʻano he nui o ke ola o kahi keiki, ʻoiai inā he koʻikoʻi ke kūlana. No laila, pono paha i ke keiki ka lāʻau lapaʻau i hoʻopilikino ʻia i ko lākou mau pono. Hiki i nā koho lapaʻau ke komo pū me:

• Lapaʻau niho: Pono paha nā keiki me ke cherubism i ka lapaʻau niho, e like me ka unuhi ʻana i nā niho a i ʻole nā ​​implants . Ke hoʻomaka ka emi ʻana o nā ulu ʻana e like me ka cyst i loko o ka ʻāwae, pono paha i ke keiki nā braces (orthodontics) .
• Kākoʻo ʻike maka: I nā hihia koʻikoʻi o ke kerubimu, hiki ke hoʻopilikia ʻia ka ʻike o ke keiki. Hiki i ke kauka maka ke nānā i ka ʻike o ke keiki a loaʻa ka hopena maikaʻi loa no kā lākou mau pono.
• Kōkua me ka ʻōlelo ʻana a me ke ale ʻana: Inā paʻakikī ke keiki i ka ʻōlelo ʻana a i ʻole ke ale ʻana,Hiki i ke kauka ʻōlelo-ʻōlelo (SLP) ke kōkua.
• Kākoʻo noʻonoʻo: Hiki i ke Cherubism ke hoʻopilikia i ka manaʻo ponoʻī o ke keiki. He mea nui e kamaʻilio me kāu keiki e pili ana i ko lākou manaʻo a pehea e hoʻopilikia ai kēia kūlana i ko lākou manaʻo ponoʻī. Hiki i kāu keiki ke pōmaikaʻi mai ke kamaʻilio ʻana me kahi psychologist keiki e pili ana i ko lākou mau manaʻo.

He aha ka manaʻolana no ka poʻe me ke Kerubimana?

ʻAʻole loaʻa i ka hapa nui o ka poʻe nā hōʻailona o ke Cherubism i ka hiki ʻana i 30 mau makahiki. I kēlā manawa, ua pani ʻia nā ʻiʻo ʻē aʻe e nā ʻiʻo iwi maʻamau.

He mea kākaʻikahi loa, hiki i nā hōʻailona o ke kerubima ke hoʻomau a hiki i ka wā makua. Inā hana ʻia kēia, e hāʻawi ke kauka i ke alakaʻi a me ke kākoʻo e pono ai.

Hiki ke pale ʻia ka Cherubism?

ʻAʻole ʻike nā kauka i kekahi ala e pale aku ai i kēia maʻi. Inā loaʻa kekahi o kou ʻohana i ka Cherubism, he manaʻo maikaʻi paha e loaʻa ka ʻōlelo aʻoaʻo genetic ma mua o ka hāpai ʻana.

Pehea lā wau e mālama ai i kaʻu keiki?

Inā loaʻa i kāu keiki ka Cherubism, e makaʻala i kēia mau mea:

• E hoʻomau i ka lawe ʻana i kāu keiki e ʻike i ke kauka keiki. E haʻi aku ke kauka iā ʻoe i ka pinepine o ka hoʻi ʻana mai.
• E nānā ʻia nā maka o kāu keiki e like me ka papa hana i ʻōlelo ʻia e ke kauka.
• E kākoʻo i ke keiki ma ka naʻau. E paipai iā lākou, e kamaʻilio me lākou.

Āhea wau e kāhea aku ai i ke kauka?

E kāhea i kāu pediatrician inā loaʻa i kāu keiki kekahi o kēia mau mea:

• Nā pilikia hanu, e like me ka snoring a i ʻole ka sleep apnea .
• Paʻakikī ke ale ʻana.
• Paʻakikī i ka ʻōlelo ʻana.
• Nā pilikia ʻike.

Pehea i loaʻa ai ka inoa (Cherubism)?

Ua kapa ʻia ke ʻano maʻi i ka makahiki 1933 e Kauka William A. Jones . Ua koho ʻo ia i ka inoa ʻo "Cherubism" no ka mea, ʻo nā hōʻailona o ke ʻano maʻi e komo pū ana me kahi helehelena keiki, pehu a me nā maka i luna i kekahi manawa. Ua manaʻoʻiʻo ʻo ia ua like kēia mau hiʻohiʻona me ko kahi "cherub," he kiʻi ʻānela maikaʻi i ʻike ʻia ma nā kiʻi a me nā kuʻuna hoʻomana like ʻole. Ua hoʻohana ʻia ka inoa mai ia manawa mai.

Hiki ke lilo i mea hoʻohoka ke ʻike ʻia he maʻi laha ʻole kāu keiki. I ke ʻano o Cherubism, ʻaʻole ʻike ʻia nā hōʻailona i ka wā hānau, no laila e hopohopo paha ʻoe ke ʻike ʻoe i nā loli i ka helehelena o kāu keiki a i ʻole ke ʻōlelo ʻia e ke kauka. Akā e hoʻomanaʻo, ʻo ka hapa nui o nā hihia o Cherubism e hoʻopau iā lākou iho i ka wā e hiki ai ke keiki i ka wā makua.I nā hihia koʻikoʻi, hiki i nā kauka ke mālama i nā hopena o ke ʻano (Cherubism). ʻO ka hāʻawi ʻana i kāu keiki i ke aloha nui a me ke kākoʻo naʻau e kōkua iā lākou e loaʻa i nā mālama āpau e pono ai lākou e hoʻohana pono i ko lākou wā kamaliʻi a me ko lākou wā ʻōpio.

Leka Lawe-Home

He maʻi laha ʻole ka Cherubism e hoʻopilikia ana i ka iwi ʻauwae. Hiki i kēia ke hoʻoulu i nā papālina o ke keiki e pehu a ʻike ʻia ka ʻauwae e ākea. Hoʻomaikaʻi pinepine kēia i ka wā e ulu ana ke keiki. Eia nō naʻe, inā pilikia lākou i ka hanu ʻana a i ʻole ka moni ʻana, e ʻike i ke kauka. ʻO ka mea nui loa, ʻo ia ka hoʻomau ʻana i ke aloha a me ke kākoʻo i kāu keiki. ʻO ka mea nui loa, ʻo ia ke kōkua i kāu keiki e noho ikaika i ka wā e hahai ana i nā ʻōlelo aʻoaʻo lapaʻau. Mai hopohopo, ʻaʻole ʻoe hoʻokahi i kēia huakaʻi.

ʻO ke kerubi, nā maʻi hoʻoilina, ka iwi ʻauwae, nā papālina puʻupuʻu, nā maʻi o nā keiki, nā pilikia niho, ka gene SH3BP2