He aha ka Gorlin Syndrome? Mai hopohopo, e kamaʻilio kākou e pili ana i nā kikoʻī!

Ua ʻike anei ʻoe i nā kiko hou a i ʻole nā ​​puʻupuʻu e hana mau ana ma kou ʻili? A i ʻole ua lohe paha ʻoe i nā puʻupuʻu ʻeha ʻole ma kou iwi ʻauwae? He mea maʻamau paha kēia. Eia nō naʻe, i nā hihia kakaikahi, pili paha kēia mau hōʻailona i kahi maʻi genetic kakaikahi. ʻO ia ke ʻano a mākou e kamaʻilio ai i kēia lā, i kapa ʻia ʻo Gorlin Syndrome. Mai makaʻu i kou lohe ʻana i kēia inoa. E kamaʻilio maʻalahi kākou e pili ana iā ia, ma ke ʻano hiki iā ʻoe ke hoʻomaopopo.

He aha maoli ka Gorlin Syndrome?

I ka ʻōlelo maʻalahi, he maʻi genetic kakaʻikahi loa ka maʻi Gorlin. Ua ʻike ʻia hoʻi ia e nā inoa ʻē aʻe, e like me `(Basal Cell Nevus Syndrome)`, `(Nevoid Basal Cell Carcinoma Syndrome - NBCCS)` a me `(Gorlin-Goltz Syndrome)`. ʻO ke kanaka me kēia maʻi, ʻoi aku ka nui o ka pilikia o ka loaʻa ʻana o nā puʻupuʻu kanesa a me nā puʻupuʻu ʻaʻole kanesa (ʻo ia hoʻi, ʻaʻole ia e hōʻeha nui i ke kino). Ma ke ʻano kūikawā, aia ka nui o ka pilikia o ka loaʻa ʻana o kahi ʻano maʻi ʻili i kapa ʻia ʻo `(Basal Cell Carcinoma)` . ʻO kēia ke ʻano maʻamau o ka maʻi ʻili.

E noʻonoʻo, aia kekahi mau genes i loko o ko kākou kino, nona ka hana nui e kāohi i ka mahele pono ʻole o nā cell a me ke kūkulu ʻia ʻana o nā tumors. Kāhea pū mākou i kēia mau "genes tumor suppressor". No laila, i loko o ka maʻi Gorlin, ʻo ka mea e hana ʻia ana, aia kahi loli, ʻo ia hoʻi, kahi "mutation", i loko o kekahi o kēia mau genes. ʻO ia ke kumu e piʻi ai ka pilikia o ka ulu ʻana o nā tumors e like me ka mea i ʻōlelo ʻia.

ʻO ka mea nui loa, ʻaʻohe lāʻau lapaʻau kūikawā no ka maʻi Gorlin . Akā, mai hopohopo! Inā ʻoe e hahai pono i nā kuhikuhi a kāu kauka, a inā ʻike ʻia a mālama ʻia nā mea e like me ke kanesa i ka wā mua , hiki i kekahi me kēia maʻi ke ola i kahi ola maʻamau. ʻAʻole pono ia e hoʻopilikia i kou ola a i ʻole ke ʻano o ke ola.

Pehea ka maʻamau o kēia ʻano?

He maʻi laha ʻole ka maʻi Gorlin. Wahi a nā loea, ʻoiai ma kahi ʻāina e like me ʻAmelika Hui Pū ʻIa, pili kēia maʻi i ka emi ma mua o 50,000 mau kānaka. Eia nō naʻe, ʻoi aku paha ke kiʻekiʻe o kēia helu. ʻO ke kumu, he ʻoluʻolu loa nā hōʻailona o kekahi poʻe a ʻaʻole lākou i ʻike he maʻi ko lākou. ʻO ka hapa pinepine, ʻike ʻia nā hōʻailona i nā ʻōpio a i ʻole nā ​​​​mākua ʻōpio .

He aha nā hōʻailona o ka Gorlin Syndrome?

Hiki ke ʻokoʻa nā hōʻailona o kēia maʻi mai kēlā me kēia kanaka, akā aia kekahi mau hōʻailona maʻamau:

Nā hiʻohiʻona i ʻike nui ʻia

• Nā carcinomas cell basal he nui: ʻO kēia ʻano maʻi ʻili e ulu pinepine ma nā wahi i hōʻike ʻia e ka lā e like me ka maka a me ka ʻāʻī. Eia nō naʻe, i ka poʻe me ka maʻi Gorlin, hiki i kēia ke ulu i ka wā ʻōpio, i ko lākou mau makahiki ʻōpio a i ʻole nā ​​​​makahiki iwakālua .
• Nā keratocysts Odontogenic:He mau ulu ʻeha ʻole kēia, ʻaʻole he maʻi kanesa e hana ana ma ka iwi ʻauwae. ʻOi aku ka maʻamau o lākou i nā keiki a me nā ʻōpio me ka maʻi Gorlin.
• Nā lua liʻiliʻi a i ʻole nā ​​lua ma nā poho lima a me nā kapuwaʻi o nā wāwae: ʻIke pinepine ʻia kēia hiʻohiʻona i nā makahiki 20. He mau loa kēia.
• Nā loli iwi: Hiki ke ʻike ʻia kekahi mau loli ma ke ʻano o ka hana ʻia ʻana o nā iwi ʻaoʻao a me ka iwi kuamoʻo.

Nā hōʻailona maʻamau ʻole

ʻAʻole nā ​​​​kānaka a pau e ʻike i kēia mau hōʻailona, ​​​​akā hiki i kekahi poʻe ke ʻike:

• Nā puʻupuʻu lolo: Hiki i kēia ke maʻi kanesa (medulloblastoma) a i ʻole ʻaʻole maʻi kanesa (meningioma).
• ʻO nā fibromas o ka puʻuwai a i ʻole nā ​​​​ovaries: He mau maʻi kanesa ʻole kēia, ʻo ka maʻamau he mau ʻōpū ʻino ʻole.
• Nā pilikia maka: nā mea e like me ka strabismus, nystagmus, a me nā cataracts.
• Nā loli koʻikoʻi i nā iwi: No ka laʻana, ka nele loa o ka iwi kuamoʻo (spina bifida).
• Nā hiʻohiʻona helehelena ʻē: Hoʻonui ʻia ka mamao ma waena o nā maka (hypertelorism), hānau ʻia me ka lehelehe māwae/palate, a me nā kiko keʻokeʻo liʻiliʻi (milia) ma ka maka.
• ʻO ke poʻo i ʻoi aku ka nui ma mua o ka maʻamau (macrocephaly).
• Nā kīnā noʻonoʻo.
• Nā kūlana epilepsy `(Nā hopu)`.

He aha ke kumu o ka Gorlin Syndrome?

E like me kā mākou i kūkākūkā mua ai, ua hoʻokumu ʻia kēia ʻano ma muli o ka loli o kā mākou mau genes. I ka pololei, aia ʻekolu mau genes i loko o ko mākou kino e hoʻōki i ka hoʻokumu ʻia ʻana o nā tumors (nā genes tumor suppressor). ʻO lākou nā genes i kapa ʻia ʻo ``PTCH1`` (ʻo ia ka mea i pili pinepine ʻia), ``PTCH2`` a me ``SUFU``. Ke hana pono ʻole kēia mau genes, hoʻomaka kekahi mau cell e ulu ma ke ʻano ʻē. ʻO ia ke kumu o nā hōʻailona e like me nā tumors.

Hoʻoilina ka hapa nui o ka poʻe i kēia hoʻololi ʻana o ka gene mai ko lākou mau mākua mai. Eia nō naʻe, i kekahi mau hihia, hiki ke hana maʻamau ʻia kēia hoʻololi ʻana o ka gene ma mua o ka hānau ʻana, me ka ʻole o ka mōʻaukala ʻohana.

Ua hoʻoilina ʻia kēia hoʻololi ʻana o ka genetics ma ke ʻano "autosomal dominant" . I ka ʻōlelo maʻalahi, loaʻa iā ʻoe kahi gene maʻamau mai kekahi makua a me kahi gene mutated mai kekahi. Pono ʻoe e loaʻa ka hoʻololi ʻana o ka gene PTCH a i ʻole SUFU e hoʻomohala ai i ka maʻi Gorlin.

Wahi a nā kānaka ʻepekema, loaʻa i ke kanaka me ka maʻi Gorlin ka maʻi kanesa inā hoʻololi ʻia ke kope maʻamau o ka gene suppressor tumor ma kekahi ʻano. No ka laʻana, hiki i ka pōʻino mai ka lā ke hoʻololi i ka gene maikaʻi. A laila, ʻaʻole hana pono nā gene ʻelua, a ʻaʻole hiki ke hoʻōki ʻia nā tumors. ʻO kēia ke kumu o ka maʻi kanesa ʻili. A inā hōʻino ʻia nā cell ʻē aʻe i loko o ke kino ma kēia ʻano, hiki ke ulu nā ʻano maʻi kanesa ʻē aʻe.

Pehea e ʻike ai nā kauka i kēia maʻi?

No ka hōʻoia ʻana i ka maʻi Gorlin, e hana kāu kauka i kēia mau mea:

• Hoʻokolokolo kino: E nānā inā he maʻi ʻili.
• Nā hoʻāʻo kiʻi: E nānā no nā puʻupuʻu, fibromas, a i ʻole nā ​​​​pilikia iwi i loko o ka iwi ʻauwae (e.g., X-ray, CT scan, MRI scan).
• Biopsy: He kaʻina hana e lawe ʻia ai kahi ʻāpana liʻiliʻi o ka ʻiʻo mai ka ʻili a i ʻole kahi puʻupuʻu a nānā ʻia ma lalo o kahi microscope e ʻike inā he mau hunaola kanesa.
• Hoʻāʻo hoʻoilina: Lawe ʻia kahi hāpana koko e nānā inā he loli hoʻoilina kou e pili ana me ka maʻi Gorlin.

He aha nā lāʻau lapaʻau no ka Gorlin Syndrome?

ʻOiai ʻaʻohe lāʻau lapaʻau kūikawā no ka maʻi Gorlin ponoʻī, inā loaʻa iā ʻoe nā puʻupuʻu i loaʻa i ka maʻi kanesa a i ʻole ke kumu o nā pilikia ʻē aʻe, pono paha ʻoe e mālama iā lākou. Eia kekahi laʻana:

• Hiki ke wehe ʻia kahi maʻi ʻili a i ʻole kahi puʻupuʻu iwi ʻauwae me ke ʻoki ʻana .
• Hiki ke hoʻohana ʻia nā lāʻau lapaʻau ʻē aʻe no ke kanesa, e like me nā kirīmi topical a i ʻole ka photodynamic therapy, e hoʻopau i nā hunaola kanesa.

No ka mea, ua piʻi aʻe kou pilikia o ka loaʻa ʻana o ke kanesa, hiki i kāu kauka ke ʻōlelo aku iā ʻoe e hoʻohana i kahi pale lā maikaʻi, e ʻaʻahu i nā lole lima lōʻihi, a e ʻaʻahu i ka pāpale ke ʻike ʻoe i ka lā. Eia kekahi, hana ʻia nā hoʻokolohua e like me nā kukuna X ke pono loa. Ma ke ʻano laulā, pale nā ​​kauka oncology i ka hāʻawi ʻana i ka radiation therapy ma ke ʻano he lāʻau lapaʻau no ka poʻe me ka maʻi Gorlin. ʻO kēia no ka mea hiki i ka radiation ke hoʻonui i ka pilikia o ka maʻi kanesa.

Hiki ke pale ʻia ka Gorlin Syndrome?

ʻAʻole hiki iā mākou ke kāohi i nā genes i hānau ʻia me mākou. No laila, ʻaʻohe ala e pale aku ai i ka maʻi Gorlin. Eia nō naʻe, hiki iā ʻoe ke hōʻemi i kou pilikia o ka loaʻa ʻana o ka maʻi ʻili ma ka hana ʻana i nā hana e pale aku ai . Eia kekahi, ma ka loaʻa ʻana o nā nānā lapaʻau maʻamau, hiki iā ʻoe ke ʻike i ka maʻi ʻaʻai i ka wā mua. A laila e maʻalahi ka ho'ōla.

Inā loaʻa iā ʻoe ka maʻi Gorlin (a inā loaʻa i kekahi o kou ʻohana) a ke hoʻolālā nei ʻoe e loaʻa nā keiki a hopohopo paha e hoʻoilina paha kāu keiki iā ia, e ʻike i kahi kākāʻōlelo genetic . E wehewehe lākou i nā pilikia iā ʻoe a me kāu hoa.

Pehea lā ka wā e hiki mai ana no kekahi e noho nei me kēia maʻi? (Outlook)

Hoʻokele ka nui o ka poʻe i ka maʻi Gorlin ma o ka lawe ʻana i nā hana e pale aku ai i ka maʻi ʻili a me ka loaʻa ʻana o nā nānā lapaʻau maʻamau. Ma ka loaʻa ʻana o nā nānā maʻamau e like me ka ʻōlelo a kāu kauka, hiki ke ʻike mua ʻia kekahi pilikia. Hiki i ka poʻe me ka maʻi Gorlin ke ola lōʻihi a like me ka poʻe me ka ʻole o ke ʻano e like me ka poʻe me ka ʻole o ke ʻano.

Eia kekahi, ʻo ka mea nui e hoʻomanaʻo ai, ʻaʻole e loaʻa i nā kānaka a pau me ka maʻi Gorlin syndrome ka maʻi kanesa a i ʻole nā ​​pilikia like. He ʻokoʻa ia mai kēlā kanaka a kēia kanaka. E aʻo aku kāu kauka iā ʻoe i kou kūlana ma muli o kāu mau hōʻailona.

Pehea e mālama ai iaʻu iho? (Mālama ponoʻī)

Inā loaʻa iā ʻoe ka maʻi Gorlin, pono ʻoe e hana i kēia mau hana kūikawā e pale aku ai i ka maʻi ʻili:

• E hōʻalo i ka hoʻohana ʻana i nā moena tanning.
• E noho i loko o ka hale i ka hiki i nā hola kiʻekiʻe o ka lā (10 a.m. a i 4 p.m.).
• E hoʻohana i ka sunscreen ākea me ka liʻiliʻi he SPF 30 i kēlā me kēia lā. E hoʻopili hou.
• I ke hele ʻana i waho, e ʻaʻahu i nā lole pale UV a me ka pāpale ākea.

Pono nō hoʻi ʻoe e ʻike i nā kauka like ʻole e nānā i ka maʻi ʻaʻai a me nā kūlana ʻē aʻe. ʻO ka pinepine o kou hele ʻana e pili ana i ka loaʻa ʻana o nā puʻupuʻu a i ʻole nā ​​​​​​mea ʻē aʻe. Hiki i kēia mau kipa kauka ke komo pū me:

• E nānā i kou ʻili e ke kauka ʻili.
• Nā hoʻokolohua niho a me nā hoʻokolohua kiʻi e nānā i nā ʻōpū i loko o ka iwi ʻā.
• Nā hoʻokolohua kiʻi e nānā i nā fibromas, nā ʻōpū lolo, a i ʻole nā ​​​​pilikia ʻike.

Āhea wau e ʻike ai i ke kauka?

Inā kānalua ʻoe he basal cell carcinoma kou a i ʻole kāu keiki, e ʻike koke i ke kauka . ʻO kēia ka hōʻailona maʻamau o ka maʻi Gorlin. E like me nā ʻano maʻi ʻili ʻē aʻe, e makaʻala i nā puʻupuʻu a i ʻole nā ​​kiko hou, mau, a ulu paha.

ʻAʻole pili pinepine ka maʻi ʻili me ka maʻi Gorlin, akā me ka nānā ʻole i ke kumu, he mea nui e hoʻāʻo ʻia.

He aha nā nīnau āu e nīnau ai i ke kauka?

Inā ua ʻike ʻia ʻoe me ka maʻi Gorlin, makemake paha ʻoe e nīnau i kāu kauka i kēia mau nīnau:

• He aha nā hōʻailona o ke kanesa e pono iaʻu e ʻike?
• ʻEhia pinepine e pono ai iaʻu e hana i nā hoʻokolohua kanesa?
• He aha kaʻu e hana ai e hōʻemi i koʻu pilikia o ka maʻi kanesa?
• E pono anei iaʻu ka lāʻau no nā puʻupuʻu ʻaʻole maʻi kanesa?
• He aha nā manawa kūpono e loaʻa iaʻu kahi keiki me ka maʻi Gorlin?

Hele mai ka maʻi Gorlin me kekahi mau pilikia e hiki ʻole i ka poʻe me ke ʻano ʻole ke ʻike. Inā loaʻa iā ʻoe ka NBCCS, pono ʻoe a me kāu hui lapaʻau e makaʻala loa i ka ʻike ʻana a me ka mālama ʻana i kekahi basal cell carcinoma e ulu ana.

Leka Hoʻihoʻi Hope Loa

ʻO ka nūhou maikaʻi, hiki ke ho'ōla loa ʻia nā carcinoma cell basal inā mālama ʻia i ka wā mua. Eia kekahi, ʻaʻole pilikia nā puʻupuʻu ʻaʻole cancerous. Inā hana lākou, hiki i ke kauka ke wehe iā lākou a ʻōlelo paha i nā lāʻau lapaʻau ʻē aʻe. Hiki iā ʻoe ke ola i kahi ola lōʻihi a piha me ka maʻi Gorlin. No laila, mai hopohopo, akā e makaʻala. E hahai i nā ʻōlelo aʻoaʻo lapaʻau kūpono, a e maikaʻi nā mea āpau!

ʻO ka maʻi Gorlin, NBCCS, basal cell carcinoma, nā maʻi genetic, ka maʻi ʻili, dermatology