Loaʻa iā ʻoe nā ʻōhū e like me kēia ma kou ʻili? E aʻo kākou e pili ana i ka Brooke-Spiegler Syndrome.

Ua ʻike anei ʻoe i nā puʻupuʻu liʻiliʻi ma kou ʻili, ʻoi aku hoʻi ma kou maka, ʻāʻī, a i ʻole ke poʻo, e hoʻopilikia nei iā ʻoe? I kekahi manawa ʻaʻole paha ʻoe i ʻike i ke ʻano o ia mau mea a i ʻole ke kumu o ko lākou hana ʻana. I kēia lā e kamaʻilio mākou e pili ana i kahi maʻi ʻili e hiki ai ke hana i ia mau puʻupuʻu, akā he mea kakaikahi iki ia, ʻo ia hoʻi, ʻaʻole ia e hana i nā mea āpau. Ua kapa ʻia kēia ʻo Brooke-Spiegler Syndrome. Mai hopohopo, e kamaʻilio maʻalahi kākou e pili ana i kēia.

He aha ka Brooke-Spiegler Syndrome?

I ka ʻōlelo maʻalahi, he maʻi ʻili laha ʻole ka Brooke-Spiegler Syndrome. Hoʻoulu ia i nā puʻupuʻu (i kapa ʻia hoʻi he tumors) e hana ma kou ʻili, ʻoi aku hoʻi ma kou maka, ʻāʻī, a me ke poʻo. I kekahi manawa hiki i kēia mau puʻupuʻu ke hana ma nā ʻāpana ʻē aʻe o kou kino. ʻO ka hapa pinepine, hoʻomaka lākou e ʻike ʻia i kou wā ʻōpiopio, ma kahi o nā makahiki he ʻumikumamāwalu a iwakālua paha .

I ka wā e pili ana i kēia mau puʻupuʻu, ʻo ka hapa nui o ka manawa ʻaʻole lākou he maʻi kanesa (ʻo ia hoʻi, he 'benign' lākou), ʻo ia hoʻi, he benign lākou. Eia nō naʻe, pono kākou e hoʻomanaʻo hoʻi, i ka manawa pinepine, hiki iā lākou ke lilo i maʻi kanesa (ʻo ia hoʻi, he 'malignant' lākou). I kekahi manawa, ke ulu kēia mau puʻupuʻu benign, hiki iā lākou ke ʻeha, lilo i 'ʻeha hāmama', a hiki ke hoʻoulu i ka maʻi.

ʻO ka maʻamau, ua hoʻoilina ʻia kēia maʻi mai kou mau mākua mai, ʻo ia hoʻi he maʻi hoʻoilina ia . Eia nō naʻe, he mea kakaikahi loa, hiki i kekahi ke loaʻa i ka maʻi Brooks-Spiegel ʻoiai inā ʻaʻohe mea o ka ʻohana i loaʻa i ke ʻano.

ʻO ka mea maikaʻi loa, aia kahi lāʻau lapaʻau no kēia. ʻO ka hapa nui, ʻo ke ʻoki kino wale nō ke koho. Me ka lāʻau lapaʻau kūpono, hiki i ka hapa nui o ka poʻe me kēia maʻi ke ola maʻamau, hauʻoli, a me ke ola ikaika.

Aia kekahi mau inoa ʻē aʻe no kēia?

ʻAe, hoʻohana nā kauka i kekahi mau inoa ʻē aʻe no kēia maʻi Brooke-Spiegler. Hiki paha iā ʻoe ke lohe i kēia mau inoa:

• Nā cylindromas Ancell-Spiegler
• ʻO ka maʻi ʻili CYLD (CCS)
• ʻO ka cylindromatosis ʻohana (FC)
• Nā trichoepitheliomas familial lehulehu (MFT)

ʻOiai paha he mea paʻakikī iki kēia mau inoa, he mau inoa lākou a pau no ke kūlana hoʻokahi.

Pehea ka maʻamau o kēia kūlana?

He ʻano maʻi laha ʻole kēia. Wahi a nā kānaka noiʻi ma kahi o hoʻokahi i loko o hoʻokahi miliona mau kānaka e loaʻa i ka maʻi Brooks-Spiegel. No laila, ʻaʻole ia he mea maʻamau loa.

He aha nā hōʻailona? E nānā inā loaʻa iā ʻoe kēia mau mea pū kekahi...

ʻO ka hōʻailona nui o kēia maʻi ka hoʻokumu ʻia ʻana o nā puʻupuʻu ʻili . Hoʻomaka pinepine kēia ma kou maka, ʻāʻī, a me ke poʻo. He poepoe ke ʻano o kēia mau puʻupuʻu a hiki ke ʻokoʻa ka nui mai 0.5 a 3 kenimika (cm).

I ka hala ʻana o ka manawa, hiki i kēia mau puʻupuʻu ke hoʻonui i ka nui a me ka nui. I kekahi manawa hiki iā lākou ke hoʻopilikia i ke ʻano. I ka manawa pinepine, ʻeha kēia mau puʻupuʻu . Eia kekahi, inā hana lākou ma ka ʻili o ka ʻāpana genital, hiki iā lākou ke hana i ka hana moekolohe. ʻO kekahi mea ʻē aʻe, i kekahi manawa hiki i kēia mau puʻupuʻu ke pālahalaha ma luna o ke poʻo a hoʻoulu i ka hāʻule ʻana o ka lauoho . Hiki i kēia mau mea ke hoʻoulu i ke kaumaha.

Ma waho aʻe o kēia mau puʻupuʻu, ʻike paha ʻoe i kekahi manawa i nā mea liʻiliʻi, keʻokeʻo, e like me ka pehu (milia) ma kou ʻili. ʻAʻole ʻino kēia mau 'milia', akā hiki iā lākou ke hoʻopilikia i kekahi poʻe.

He aha kēia mau ʻano puʻupuʻu? (Nā ʻano puʻupuʻu ʻili ma BSS)

ʻEkolu mau ʻano nui o nā puʻupuʻu i ʻike ʻia ma ka maʻi Brooke-Spiegler:

1. Nā Cylindromas: He mau puʻupuʻu laumania a poni kēia. Hoʻomohala pinepine lākou ma ke poʻo, kahi i loaʻa ai nā follicle lauoho. Hiki iā lākou ke ulu ma ka maka, nā pepeiao, a kākaʻikahi hoʻi i loko o nā māmā. Hiki iā lākou ke ʻokoʻa ka nui mai kekahi mau milimita a i kekahi mau kenimika. Ke ulu lākou ma ke poʻo, hiki iā lākou ke hui pū a like me nā ʻāpana o kahi puzzle jigsaw.

2. Spiradenomas: He mau puʻupuʻu paʻa a puʻupuʻu kēia. Loaʻa pinepine ʻia lākou ma ke poʻo, ka ʻāʻī, a me ke kino o luna. Hiki iā lākou ke ʻokoʻa ka nui mai ka liʻiliʻi ma mua o hoʻokahi kenimika a i kekahi mau kenimika. He uliuli pinepine a ʻili paha ke kala. Akā hiki nō hoʻi iā lākou ke hina, poni, poni, ʻulaʻula, a melemele paha. Hiki i nā Spiradenomas ke ʻeha i kekahi manawa.

3. Trichoepitheliomas: He mau puʻupuʻu paʻakikī a ʻālohilohi hoʻi kēia. Loaʻa pinepine lākou ma ka maka, ma nā wahi me ka lauoho. ʻOi aku ka liʻiliʻi ma mua o hoʻokahi kenimika. Hiki iā lākou ke uliuli, ʻeleʻele, ʻili, poni, a melemele paha.

ʻO ka mea nui, i kekahi manawa ma kēia maʻi Brook-Spiegler, hiki ke ʻike ʻia nā ʻano o ʻelua a ʻekolu paha o kēia ʻano puʻupuʻu i loko o ka puʻupuʻu hoʻokahi.

Ke hoʻopilikia nei kēia maʻi i nā ʻāpana ʻē aʻe o ke kino?

ʻAe, i kekahi manawa hiki i kēia mau puʻupuʻu ke ulu aʻe ma nā wahi ʻē aʻe o ke kino. I ia mau hihia, hiki ke hoʻopilikia ʻia ka hana o kēlā mau ʻōkana. Eia kekahi laʻana:

• Inā hana ia i loko o nā alaea , hiki iā ia ke hoʻopilikia i ka hanu ʻana.
• Inā ulu ia i loko o nā pepeiao , hiki ke hoʻopilikia ʻia ka lohe.
• Inā loaʻa ia i nā maka , hiki ke hoʻopilikia ʻia ka ʻike.
• Inā hana lākou ma nā ʻōpū , hiki ke hoʻopilikia ʻia ka hana moekolohe.
• Inā ulu ia i loko o ka waha a me nā ʻōpū salivary , hiki ke hoʻopilikia ʻia ka nau ʻana a me ke moni ʻana.
• IhuInā hana ia, hiki iā ia ke hoʻopilikia i ke ʻano o ka honi.

No ke aha e kū mai ai ka Brooke-Spiegler Syndrome?

ʻO ke kumu nui o kēia, ʻo ia nā loli genetic (`mutations`) i loko o ka gene `CYLD` i loko o ko kākou kino. I mea e loaʻa ai kēia maʻi, pono i kekahi o kou mau mākua ke lawe i kēia mutation genetic. ʻO ia hoʻi, ua hoʻoili ʻia mai ia hanauna a i ia hanauna .

Eia nō naʻe, he mea kākaʻikahi loa, hiki i kahi kanaka me ka ʻohana ʻole o ka maʻi ke hoʻomohala i kēia hoʻololi ʻana o ka gene ('de novo mutation`) i loko o ko lākou kino. I kēlā mau hihia, hiki ke loaʻa wale ka hoʻololi ʻana o ka gene ma kekahi mau ʻāpana o ke kino. A laila, hiki ke ulu wale nā ​​​​pūpū ma kēlā mau ʻāpana.

ʻO wai ka mea i ʻoi aku ka paʻakikī ke hoʻomohala i kēia?

ʻO ka ʻoiaʻiʻo, hiki i kekahi o kēlā me kēia makahiki, lāhui, a kāne paha ke loaʻa i ka maʻi Brooks-Spiegel. Eia nō naʻe, inā loaʻa i kekahi o kou mau mākua ka hoʻololi ʻana o ka gene e pili ana i ke kūlana, ʻoi aku ka nui o kou pilikia o ka loaʻa ʻana.

He maʻi autosomal dominant ka maʻi Brooke-Spiegler. I ka ʻōlelo maʻalahi, inā he mea lawe kekahi o kou mau mākua (ʻo ia hoʻi, he mau hōʻailona kona), he 50% kou manawa e loaʻa ai ke ʻano .

ʻIke mua ka hapa nui o ka poʻe i nā hōʻailona i ko lākou wā ʻōpiopio, ma kahi o nā makahiki he ʻumikumamāwalu a iwakālua paha . Hoʻomaka ka nui o kēia mau puʻupuʻu e piʻi i ko lākou mau makahiki 30 a me 40. Ua ʻike ʻia hoʻi he nui aku nā puʻupuʻu o nā wahine ma mua o nā kāne.

Pehea e ʻike ai nā kauka i kēia?

Inā loaʻa iā ʻoe kēia mau hōʻailona, ​​e nīnau mua ke kauka iā ʻoe e pili ana i kāu mau hōʻailona, ​​​​kou mōʻaukala olakino, a me ka mōʻaukala olakino o kou ʻohana. A laila e hana lākou i kahi hoʻokolokolo kino.

Inā kānalua ke kauka i ka maʻi Brooke-Spiegler, hiki iā ia ke kuhikuhi i kahi biopsy ʻili , ʻo ia hoʻi ka lawe ʻana i kahi hāpana liʻiliʻi o nā ʻāpana mai ka puʻupuʻu a hoʻouna aku i kahi lab no ka hoʻokolokolo ʻana ma lalo o kahi microscope.

Hiki paha i kāu kauka ke kuhikuhi i ka hoʻāʻo genetic , hiki ke nānā i nā loli i nā genes e hoʻoulu ai i ka maʻi Brooks-Spiegel.

Aia kekahi lāʻau lapaʻau piha no kēia?

ʻO ka mea pōʻino, ʻaʻohe lāʻau lapaʻau no ka maʻi Brooke-Spiegel i kēia manawa . Akā, mai hopohopo. Aia nā lāʻau lapaʻau kūpono e kōkua i ka hoʻopau ʻana i kēia mau puʻupuʻu a kāohi i ka holomua o ka maʻi.

No laila he aha nā lāʻau lapaʻau no BSS?

E paipai paha kāu kauka i ka ʻoki ʻoki ʻana e wehe i kēia mau puʻupuʻu. ʻO kēia ke ʻoki ʻana i nā puʻupuʻu me kahi mea ʻoi i kapa ʻia he scalpel. I ka hana ʻana pēlā, makaʻala nā kauka e pale aku i ka hōʻino ʻana i ka ʻili olakino a puni e like me ka hiki.

I kekahi manawa, inā pono e wehe ʻia kahi ʻāpana nui o ka ʻili poʻo, hiki i kāu kauka keHiki ke ʻōlelo ʻia kahi kāpili ʻili mānoanoa māhele. ʻO kēia ka lawe ʻana i kahi papa o ka ʻili mai kahi ʻāpana ʻē aʻe o kou kino, pinepine mai ka puʻupuʻu a i ʻole ka ʻūhā luna, e pani i ka ʻeha a kōkua iā ia e ho'ōla.

Ma waho aʻe o kēia, aia kekahi mau ala ʻē aʻe e mālama ai i ka maʻi Bruck-Spiegler:

• ʻO ka hoʻopili hou ʻana o ka laser carbon dioxide: Hoʻohana kēia i nā kukuna laser e wehe i nā papa lahilahi o ka ʻili me ka ʻole o ka hōʻino ʻana i nā ʻiʻo a puni.
• Cryotherapy: Pili kēia i ka hoʻopili ʻana i ke anu nui e hoʻopaʻahau a luku i nā ʻiʻo i loko o ka puʻupuʻu. Ua like ia me ke puhi ʻana me ka hau.
• Dermabrasion: Pili kēia i ka hoʻohana ʻana i kahi mea e wili wikiwiki ana e wehe i ka papa luna o ka ʻili.
• Electrodessication: Hoʻohana ʻia ke au uila e hoʻomaloʻo i ke kino i loko o ka puʻupuʻu.
• Fulguration: Ka hoʻohana ʻana i kahi haʻalulu uila e hoʻopau ai i ka puʻupuʻu.
• Hyfrecation: Hoʻouna ʻia kahi pulse uila ma o kahi nila liʻiliʻi, kahi e puhi ai a wehe i ka wart.
• ʻO ke ʻoki kino Mohs: Ma kēia kaʻina hana, wehe ke kauka ʻoki i ka puʻupuʻu me kahi liʻiliʻi o nā ʻiʻo olakino a puni ia a hoʻouna aku no ka hoʻokolokolo ʻana.
• ʻO ka lāʻau lapaʻau Photodynamic: Ma kēia, hāʻawi ʻia nā lāʻau lapaʻau kūikawā (photosensitizers), a laila kuhikuhi ʻia ke kukui i ka puʻupuʻu, e hoʻoulu ana i nā lāʻau lapaʻau a luku i nā puʻupuʻu.

Ma waho aʻe o kēia mau lāʻau lapaʻau, hiki i nā kauka ke kuhikuhi i nā lāʻau lapaʻau e kōkua i ka hoʻōki ʻana i ka ulu ʻana o nā tumors.

He aha kāu e manaʻo ai i ka wā e noho ai me kēia maʻi?

He maʻi olakino ke ola holoʻokoʻa ka maʻi Brooks-Spiegel. Eia nō naʻe, e like me ka mea i ʻōlelo ʻia ma mua, hiki i ka poʻe me kēia maʻi ke ola maʻamau a piha.

Eia nō naʻe, aia kahi pilikia liʻiliʻi. Ma waena o 5% a me 10% o ka poʻe me ka maʻi Bruck-Spiegel e hoʻomohala i nā ʻōpū ʻino . Loaʻa i ka poʻe me kēia maʻi kahi piʻi iki o ka pilikia o ka hoʻomohala ʻana i nā ʻano maʻi ʻē aʻe. Eia kekahi laʻana:

• Nā maʻi ʻaʻai adenocarcinoma
• ʻO ka maʻi kanesa cell basal (he maʻi ʻili nō hoʻi)
• ʻO ke kanesa ʻōpū salivary, no ka laʻana adenoid cystic carcinoma
• ʻO ka maʻi kanesa cell squamous (he maʻi ʻili nō hoʻi)

He aha ka mea e hiki mai ana? (Outlook)

I ka manawa pinepine, hiki i kēia mau puʻupuʻu ke hana hou . Inā hana ʻia kēia, pono paha ke ʻoki kino hou a i ʻole nā ​​​​lāʻau ʻē aʻe e wehe ai i nā puʻupuʻu hou.

Aia kekahi ala e hōʻemi ai i kēia hanana?

ʻO ka mea pōʻino, ʻaʻohe ala kikoʻī e pale ai a hōʻemi paha i kou pilikia o ka loaʻa ʻana o ka maʻi Brook-Spiegel. Eia nō naʻe, e like me ka mea i ʻōlelo ʻia ma mua, hiki i ka lāʻau lapaʻau ke hōʻemi i ka helu o nā puʻupuʻu i loaʻa iā ʻoe a me ko lākou nui.

Pehea ʻoe e mālama ai iā ʻoe iho ma ke ʻano he kanaka me Brooke-Spiegler Syndrome?

I ke ola ʻana me kēia maʻi, aia kekahi mau mea nui e hiki iā ʻoe ke hana e mālama iā ʻoe iho:

• E ʻike pinepine i kahi kauka ʻili no ka nānā ʻana i ka ʻili. Pono e hana ʻia kēia ma ka liʻiliʻi hoʻokahi manawa i ka makahiki. Pono paha kekahi poʻe e ʻike i ke kauka i kēlā me kēia ʻekolu a ʻehā mahina inā he mau ʻeha pinepine ko lākou.
• E hana i kahi hoʻokolokolo ʻili ponoʻī ma ka liʻiliʻi i hoʻokahi manawa i ka mahina. E nānā i nā puʻupuʻu hou a i ʻole nā ​​​​loli i nā mea e kū nei.
• E hōʻoia e hoʻohana i ka sunscreen i ka wā e hele ai i waho i ka lā. E kōkua kēia i ka hōʻemi ʻana i ka pōʻino o ka lā i kou ʻili a me ka hōʻemi ʻana i ka pilikia o ka maʻi ʻaʻai ʻili.

He aha nā manawa kūpono loa e ʻike ai i ke kauka?

Inā ʻike ʻoe i kekahi o kēia mau loli i kou mau ʻōpū ʻili, pono ʻoe e ʻike koke i ke kauka :

• Inā ʻeha nā puʻupuʻu .
• Inā e kahe ana ke koko o nā puʻupuʻu.
• Inā loli ke kala o nā puʻupuʻu .
• Inā e ulu koke ana nā puʻupuʻu.
• Inā like ke ʻano o ka puʻupuʻu me kahi ʻōpū ('ulcerated') .

Eia kekahi, inā ʻoe e kali ana i kahi keiki, ʻo ia hoʻi, inā ʻoe e hoʻolālā ana e hāpai, he mea nui loa paha e kamaʻilio me ke kauka a loaʻa ka ʻōlelo aʻoaʻo genetic .

He aha ka ʻokoʻa ma waena o ka Brooke-Spiegler Syndrome a me ka Neurofibromatosis?

He mau maʻi hoʻoilina kēia mau maʻi ʻelua. ʻO nā mea ʻelua ke kumu nui o nā puʻupuʻu benign (ʻaʻole cancerous). Eia nō naʻe, aia kahi ʻokoʻa iki.

• I ka maʻi Brooke-Spiegler (BSS), ʻike nui ʻia nā puʻupuʻu ma ka ʻili .
• I loko o ka neurofibromatosis, hiki ke ulu nā puʻupuʻu ma ka ʻili, ma lalo o ka ʻili, a ma kou mau aʻalolo .

ʻEkolu mau ʻano nui o ka neurofibromatosis. E aʻo iki kākou e pili ana iā lākou:

• ʻAno Neurofibromatosis 1 (NF1): Ma kēia, ulu nā puʻupuʻu (neurofibromas) ma luna a ma lalo paha o ka ʻili.
• ʻAno Neurofibromatosis 2 (NF2): Ma kēia ʻano neurofibromatosis, ulu nā ʻōpū (schwannomas) i loko o nā aʻalolo o ka lolo a me ke kaula spinal (ʻōnaehana nerve peripheral). ʻO ka hapa pinepine, hoʻopilikia ʻia nā aʻalolo lohe.
• ʻO ke ʻano o ka Schwannomatosis:Pili pū kēia i nā puʻupuʻu i kapa ʻia ʻo schwannomas, akā aia lākou i loko o ka ʻōnaehana nerve peripheral. ʻAʻole lākou e hoʻopilikia pinepine i nā aʻalolo e pili ana i ka lohe.

No laila, ʻoiai ʻo kēia mau maʻi ʻelua he mau kūlana genetic e hoʻoulu ai i nā puʻupuʻu, aia kekahi mau ʻokoʻa iki ma nā wahi a lākou e hoʻopilikia ai a me nā ʻano o nā puʻupuʻu.

Leka Lawe-Home

ʻAe, ke lana nei koʻu manaʻo ua ʻike maikaʻi ʻoe i ka Brooke-Spiegler Syndrome a mākou i kamaʻilio ai i kēia lā.

E hoʻomanaʻo, inā ʻike ʻoe i nā puʻupuʻu hou ma kou ʻili, ʻoi aku hoʻi ma kou maka, ʻāʻī, a i ʻole ke poʻo, a inā ʻike ʻoe i kahi loli i loko o kahi mea i loaʻa, mai hoʻowahāwahā iā ia. ʻOi aku ka maikaʻi o ke kipa ʻana i ke kauka a hoʻāʻo e nānā iā ia.

ʻOiai he maʻi kakaikahi ka maʻi Brooke-Spiegel, he mea nui e makaʻala i ia mea. ʻOiai he maʻi ʻole kēia mau puʻupuʻu, ʻaʻole hiki ke lilo i maʻi kanesa, no laila he mea nui e ʻimi i ka ʻōlelo aʻoaʻo lapaʻau kūpono a me ka mālama ʻana . Inā ʻike mua ʻia a mālama pono ʻia, hiki iā ʻoe ke ola i kahi ola maʻamau a olakino. ʻAʻohe mea e hopohopo ai, hiki ke hoʻoponopono ʻia nā mea āpau ma ke kamaʻilio ʻana me kāu kauka.

ʻO ka maʻi Brooke-Spiegler, nā puʻupuʻu ʻili, nā maʻi hoʻoilina, cylindroma, spiradenom, trichoepithelioma, gene CYLD