Kodi mumamva ziphuphu ngati izi pakhungu lanu? Tiyeni tiphunzire za Brooke-Spiegler Syndrome.

Kodi mudawonapo ziphuphu zazing'ono pakhungu lanu, makamaka pankhope panu, pakhosi, kapena pamutu panu, zomwe zikukuvutitsani? Nthawi zina simungadziwe chomwe chili kapena chifukwa chake zikuchitika. Lero tikambirana za vuto la khungu lomwe lingayambitse ziphuphu zotere, koma ndi losowa pang'ono, zomwe zikutanthauza kuti sizichitika kwa aliyense. Izi zimatchedwa Brooke-Spiegler Syndrome. Musadandaule, tiyeni tikambirane za izi mosavuta.

Kodi Brooke-Spiegler Syndrome ndi chiyani?

Mwachidule, Brooke-Spiegler Syndrome ndi vuto la pakhungu losowa kwambiri . Limayambitsa ziphuphu (zomwe zimatchedwanso zotupa) pakhungu lanu, makamaka pankhope panu, pakhosi, ndi pamutu panu. Nthawi zina ziphuphuzi zimathanso kupangika m'mbali zina za thupi lanu. Nthawi zambiri, zimayamba kuonekera muli aang'ono, pafupifupi zaka khumi ndi zisanu ndi zitatu kapena makumi awiri .

Ponena za ziphuphu zimenezi, nthawi zambiri sizikhala ndi khansa (ndiko kuti, ndi 'zoipa'), ndiko kuti, ndi zabwino. Komabe, tiyeneranso kukumbukira kuti, nthawi zina, pakapita nthawi, zimatha kukhala khansa (ndiko kuti, ndi 'zoipa'). Nthawi zina, ziphuphu zimenezi zikakula, zimatha kuvulala, kukhala 'mabala otseguka', komanso zimatha kuyambitsa matenda.

Matendawa nthawi zambiri amatengera kwa makolo anu, zomwe zikutanthauza kuti ndi matenda a majini . Komabe, nthawi zambiri munthu amatha kudwala matenda a Brooks-Spiegel ngakhale palibe m'banjamo amene ali ndi vutoli.

Chabwino kwambiri n'chakuti, pali chithandizo cha izi. Nthawi zambiri, opaleshoni ndiyo njira yokhayo. Ndi chithandizo choyenera, anthu ambiri omwe ali ndi vutoli amatha kukhala ndi moyo wabwinobwino, wosangalala, komanso wotanganidwa.

Kodi pali mayina ena a izi?

Inde, madokotala amagwiritsa ntchito mayina ena angapo pa matenda a Brooke-Spiegler. Muthanso kumva mayina awa:

• Ma cylindroma a Ancell-Spiegler
• Matenda a khungu a CYLD (CCS)
• Matenda a m'mimba otchedwa Familyal cylindromatosis (FC)
• Matenda a trichoepitheliomas ambiri a m'banja (MFT)

Ngakhale mayina awa angawoneke ovuta pang'ono, onse ndi mayina a vuto lomwelo.

Kodi vutoli ndi lofala bwanji?

Izi ndi matenda osowa kwambiri . Ofufuza amati pafupifupi munthu m'modzi pa anthu miliyoni amakhala ndi matenda a Brooks-Spiegel. Choncho, si chinthu chofala kwambiri.

Kodi zizindikiro zake ndi ziti? Onani ngati muli nazonso...

Chizindikiro chachikulu cha vutoli ndi kupangika kwa ziphuphu pakhungu . Izi nthawi zambiri zimayambira pankhope, pakhosi, ndi pamutu. Ziphuphu izi nthawi zambiri zimakhala zozungulira ndipo zimatha kukula kuyambira 0.5 mpaka 3 cm.

Pakapita nthawi, ziphuphuzi zimatha kuchuluka ndi kukula. Nthawi zina zimatha kuwononga mawonekedwe. Nthawi zambiri, ziphuphuzi zimakhala zopweteka . Komanso, ngati zipangika pakhungu la malo oberekera, zimatha kuyambitsa chisokonezo chogonana. Chinanso n'chakuti nthawi zina ziphuphuzi zimatha kufalikira pamutu wonse ndikupangitsa tsitsi kutayika . Zinthuzi zimathanso kuyambitsa kuvutika maganizo.

Kuwonjezera pa ziphuphuzi, nthawi zina mungaone zinthu zazing'ono, zoyera, zonga matuza (milia) pakhungu lanu. 'Milia' izi sizowopsa, koma zimatha kuvutitsa anthu ena.

Kodi mitundu iyi ya zotupa ndi iti? (Mitundu ya zotupa za pakhungu mu BSS)

Pali mitundu itatu ikuluikulu ya ziphuphu zomwe zimapezeka mu Brooke-Spiegler syndrome:

1. Ma cylindroma: Izi nthawi zambiri zimakhala zotupa zosalala, zapinki. Nthawi zambiri zimamera pamutu, pomwe pali ma follicle a tsitsi. Zingathenso kukula pankhope, m'makutu, komanso m'mapapo nthawi zambiri. Zitha kukula kuyambira mamilimita angapo mpaka masentimita angapo. Zikamera pamutu, zimatha kusonkhana pamodzi ndikuwoneka ngati zidutswa za jigsaw puzzle.

2. Spiradenomas: Izi ndi ziphuphu zolimba komanso zotupa. Zimapezeka kwambiri pakhungu, pakhosi, ndi pamwamba pa thupi. Zitha kukhala zazikulu kuyambira zosakwana sentimita imodzi mpaka masentimita angapo. Nthawi zambiri zimakhala zabuluu kapena zamtundu wa khungu. Koma zimathanso kukhala imvi, pinki, zofiirira, zofiira, kapena zachikasu. Spiradenomas nthawi zina zimakhala zopweteka.

3. Trichoepitheliomas: Awa ndi matuza olimba komanso owala. Amapezeka nthawi zambiri pankhope, m'malo okhala ndi tsitsi. Nthawi zambiri amakhala ang'onoang'ono kuposa sentimita imodzi. Akhoza kukhala abuluu, abulauni, akhungu, pinki, kapena achikasu.

Chofunika kwambiri n’chakuti nthawi zina mu Brook-Spiegler syndrome iyi, makhalidwe a mitundu iwiri kapena itatu ya zotupazi amatha kuwoneka mu chotupa chomwecho.

Kodi vutoli limakhudza ziwalo zina za thupi?

Inde, nthawi zina ziphuphu zimenezi zimatha kupangika m'zigawo zina za thupi. Zikatero, kugwira ntchito kwa ziwalo zimenezo kungasokonezeke. Mwachitsanzo:

• Ngati ipangika m'njira zopumira , ingayambitse mavuto opuma.
• Ngati ikukula m'makutu , kumva kungakhudzidwe.
• Ngati izi zimachitika m'maso , masomphenya angasinthe.
• Ngati zipangika pa ziwalo zoberekera , ntchito yogonana ingasokonezeke.
• Ngati imayamba mkamwa ndi m'malovu , kutafuna ndi kumeza kungakhudzidwe.
• MphunoNgati zitachitika, zimatha kukhudza fungo.

Nchifukwa chiyani matenda a Brooke-Spiegler amachitika?

Chifukwa chachikulu cha izi ndi kusintha kwa majini (`mutations`) mu jini ya `CYLD` m'thupi lathu. Kuti mukhale ndi vutoli, m'modzi mwa makolo anu ayenera kukhala wonyamula kusintha kwa majini kumeneku. Izi zikutanthauza kuti kumapatsiridwa kuchokera ku mibadwomibadwo kupita ku mibadwomibadwo .

Komabe, nthawi zambiri munthu amene alibe mbiri ya banja lake ya matendawa amatha kukhala ndi kusintha kwa majini (`de novo mutation`) m'thupi mwake. Zikatero, kusintha kwa majini kungakhalepo m'madera ena a thupi. Kenako, zotupa zimatha kufalikira m'madera amenewo okha.

Ndani amene ali ndi mwayi waukulu wopeza izi?

Ndipotu, aliyense wa msinkhu uliwonse, fuko, kapena jenda akhoza kudwala matenda a Brooks-Spiegel. Komabe, ngati m'modzi mwa makolo anu ali ndi kusintha kwa majini komwe kumakhudzana ndi vutoli, muli pachiwopsezo chachikulu chotenga matendawa.

Matenda a Brooke-Spiegler ndi matenda ofala kwambiri omwe amakhudza autosomal dominant. Mwachidule, ngati m'modzi mwa makolo anu ali ndi matendawa (monga ngati ali ndi zizindikiro), muli ndi mwayi wa 50% woti mudwale matendawa .

Anthu ambiri amayamba kuona zizindikiro ali aang'ono, ali ndi zaka pafupifupi 18 kapena 20. Chiwerengero cha ziphuphu zimenezi chimayamba kukwera ali ndi zaka za m'ma 30 ndi 40. Zawonedwanso kuti akazi amakhala ndi ziphuphu zambiri kuposa amuna.

Kodi madokotala amazindikira bwanji izi?

Ngati muli ndi zizindikiro izi, dokotala adzakufunsani kaye za zizindikiro zanu, mbiri yanu yachipatala, komanso mbiri yachipatala ya banja lanu. Kenako adzakuyezani thupi.

Ngati dokotala akukayikira kuti Brooke-Spiegler syndrome ndi yotani, angakulimbikitseni kuti mutenge kachidutswa kakang'ono ka maselo kuchokera pa chotupacho ndikutumiza ku labu kuti akawunikidwe pogwiritsa ntchito maikulosikopu.

Dokotala wanu angakulimbikitseninso kuti muyesedwe majini , zomwe zingayang'ane kusintha kwa majini komwe kumayambitsa matenda a Brooks-Spiegel.

Kodi pali mankhwala athunthu a izi?

Mwatsoka, pakadali pano palibe mankhwala a Brooke-Spiegel syndrome. Koma musadandaule. Pali mankhwala othandiza omwe angathandize kuchotsa ziphuphuzi ndikuletsa kupitirira kwa matendawa.

Ndiye kodi mankhwala a BSS ndi otani?

Dokotala wanu angakulangizeni kuti muchotse ziphuphuzi pa opaleshoni . Izi zimaphatikizapo kudula ziphuphuzo ndi chida chakuthwa chotchedwa scalpel. Pochita izi, madokotala amasamala kuti asawononge khungu labwino lozungulira momwe angathere.

Nthawi zina, ngati khungu lalikulu la mutu likufunika kuchotsedwa, dokotala wanu akhozaKuika khungu lopyapyala kungalimbikitsidwe. Izi zimaphatikizapo kutenga khungu kuchokera mbali ina ya thupi lanu, nthawi zambiri kuchokera m'matako kapena m'ntchafu, kuti mutseke bala ndikulithandiza kuchira.

Kuwonjezera pa izi, pali njira zina zingapo zochizira matenda a Bruck-Spiegler:

• Kukonzanso khungu pogwiritsa ntchito laser ya carbon dioxide: Izi zimagwiritsa ntchito laser kuti zichotse zigawo zoonda za khungu popanda kuwononga minofu yozungulira.
• Kuchiritsa ndi kuzizira kwambiri: Izi zimaphatikizapo kuyika chimfine choopsa kuti chizizire ndikuwononga minofu yomwe ili mu chotupacho. Zili ngati kuiwotcha ndi ayezi.
• Dermabrasion: Izi zimaphatikizapo kugwiritsa ntchito chipangizo chozungulira mofulumira kuti muchotse khungu lapamwamba.
• Kutsukira kwa Electro: Mphamvu yamagetsi imagwiritsidwa ntchito kuumitsa minofu yomwe ili mkati mwa chotupacho.
• Kufukula: Kugwiritsa ntchito mphamvu yamagetsi kuti awononge chotupacho.
• Kutuluka kwa madzi m'thupi: Kugunda kwa magetsi kumatumizidwa kudzera mu singano yaying'ono, yomwe imawotcha ndikuchotsa ziphuphu.
• Opaleshoni ya Mohs: Mu njira iyi, dokotalayo amachotsa chotupacho pamodzi ndi minofu yathanzi yozungulira ndipo amachitumiza kuti akachione.
• Chithandizo cha Photodynamic: Mu izi, mankhwala apadera (photosensitizers) amaperekedwa, kenako kuwala kumalunjika ku chotupacho, kuyambitsa mankhwalawo ndikuwononga zotupazo.

Kuwonjezera pa mankhwala amenewa, madokotala angakupatseninso mankhwala omwe amathandiza kuletsa kukula kwa zotupa.

Kodi mungayembekezere chiyani mukakhala ndi vutoli?

Matenda a Brooks-Spiegel ndi matenda omwe munthu amakhala nawo kwa moyo wake wonse . Komabe, monga tanenera kale, anthu omwe ali ndi vutoli amatha kukhala ndi moyo wabwinobwino komanso wokwanira.

Komabe, pali chiopsezo chochepa. Pakati pa 5% ndi 10% ya anthu omwe ali ndi matenda a Bruck-Spiegel amakhala ndi zotupa zoopsa . Anthu omwe ali ndi vutoli alinso ndi chiopsezo chowonjezeka pang'ono chotenga mitundu ina ya khansa. Mwachitsanzo:

• Khansa ya Adenocarcinoma
• Khansa ya pakhungu (yomwenso ndi khansa ya pakhungu)
• Khansa ya m'matumbo a m'malovu, mwachitsanzo adenoid cystic carcinoma
• Khansa ya khungu la squamous (komanso khansa ya pakhungu)

Kodi tsogolo lidzakhala lotani? (Outlook)

Kawirikawiri, ziphuphuzi zimatha kubweranso . Ngati izi zitachitika, opaleshoni yambiri kapena mankhwala ena angafunike kuti achotse ziphuphu zatsopanozo.

Kodi pali njira yochepetsera izi?

Mwatsoka, palibe njira yeniyeni yopewera kapena kuchepetsa chiopsezo chanu chokhala ndi matenda a Brook-Spiegel. Komabe, monga tanenera kale, chithandizo chingachepetse kuchuluka kwa ziphuphu zomwe muli nazo komanso kukula kwake.

Kodi mumadzisamalira bwanji ngati munthu amene ali ndi matenda a Brooke-Spiegler?

Mukakhala ndi vutoli, pali zinthu zofunika zomwe mungachite kuti mudzisamalire:

• Pitani kwa dokotala wa khungu nthawi zonse kuti akaone khungu lanu. Izi ziyenera kuchitika kamodzi pachaka. Anthu ena angafunike kupita kwa dokotala miyezi itatu kapena inayi iliyonse ngati ali ndi ziphuphu pafupipafupi.
• Dziyezetseni khungu lanu kamodzi pamwezi. Yang'anani ziphuphu zatsopano kapena kusintha kwa zomwe zilipo kale.
• Onetsetsani kuti mukugwiritsa ntchito mafuta oteteza ku dzuwa mukapita padzuwa. Izi zithandiza kuchepetsa kuwonongeka kwa khungu lanu ndi kuchepetsa chiopsezo cha khansa ya pakhungu.

Kodi nthawi zabwino zokaonana ndi dokotala ndi ziti?

Ngati muwona kusintha kulikonse pakhungu lanu, muyenera kuwona dokotala nthawi yomweyo :

• Ngati zotupazo zayamba kupweteka .
• Ngati zotupa zikutuluka magazi .
• Ngati mtundu wa zipolopolozo wasintha .
• Ngati zipolopolo zikukulirakulira mofulumira .
• Ngati chotupacho chikuoneka ngati chilonda (`ulcerated`) .

Komanso, ngati mukuyembekezera mwana, ndiko kuti, ngati mukukonzekera kutenga pakati, kungakhale kofunikira kwambiri kulankhula ndi dokotala ndikupeza upangiri wa majini .

Kodi kusiyana pakati pa Brooke-Spiegler Syndrome ndi Neurofibromatosis ndi kotani?

Matenda onsewa ndi matenda a majini. Onsewa amayambitsa makamaka zotupa zosaopsa (zosakhala khansa). Komabe, pali kusiyana pang'ono.

• Mu matenda a Brooke-Spiegler (BSS), ziphuphu zimapezeka makamaka pakhungu .
• Mu neurofibromatosis, ziphuphu zimatha kupangika pakhungu, pansi pa khungu, komanso m'mitsempha yanu .

Pali mitundu itatu ikuluikulu ya neurofibromatosis. Tiyeni tiphunzire pang'ono za iwo:

• Mtundu wa Neurofibromatosis 1 (NF1): Pa vutoli, ziphuphu (neurofibromas) zimakula pakhungu kapena pansi pake.
• Mtundu wa Neurofibromatosis 2 (NF2): Mu mtundu uwu wa neurofibromatosis, zotupa (schwannomas) zimakula m'mitsempha ya ubongo ndi msana (m'thupi la mitsempha). Nthawi zambiri, mitsempha yomvera imakhudzidwa.
• Schwannomatosis:Izi zimakhudzanso zotupa zotchedwa schwannomas, koma zili m'thupi la mitsempha ya m'mphepete mwa thupi. Nthawi zambiri sizimakhudza mitsempha yomwe imakhudzidwa ndi kumva.

Kotero, ngakhale kuti matenda onsewa ndi majini omwe amayambitsa ziphuphu, pali kusiyana pang'ono m'madera omwe amakhudza ndi mitundu ya ziphuphu.

Uthenga Wopita Nawo Kunyumba

Chabwino, ndikukhulupirira kuti tsopano mwamvetsa bwino za matenda a Brooke-Spiegler omwe takambirana lero.

Kumbukirani, ngati muwona ziphuphu zatsopano pakhungu lanu, makamaka pankhope panu, pakhosi, kapena pamutu panu, kapena ngati muwona kusintha kwa zomwe zilipo kale, musanyalanyaze. Ndi bwino kupita kwa dokotala ndikupita naye kuchipatala.

Ngakhale kuti matenda a Brooke-Spiegel ndi matenda osowa kwambiri, ndikofunikira kudziwa za matendawa. Ngakhale kuti zotupazi nthawi zambiri zimakhala zosaopsa, sizingakhale za khansa, choncho ndikofunikira kufunafuna upangiri woyenera wachipatala ndi chithandizo . Ngati mwazindikira msanga ndikuthandizidwa bwino, inunso mutha kukhala ndi moyo wabwinobwino komanso wathanzi. Palibe chodetsa nkhawa, chilichonse chingathetsedwe mwa kulankhula ndi dokotala wanu.

Matenda a Brooke -Spiegler, ziphuphu za pakhungu, matenda a majini, cylindroma, spiradenom, trichoepithelioma, CYLD gene