Kodi Gorlin Syndrome ndi chiyani? Musadandaule, tiyeni tikambirane mwatsatanetsatane!

Kodi mwaonapo mawanga atsopano kapena ziphuphu zikupangika nthawi zonse pakhungu lanu? Kapena mwamvapo za ziphuphu zopanda ululu pa fupa lanu la nsagwada? Izi zitha kukhala zachilendo. Komabe, nthawi zina, zizindikirozi zitha kukhala zokhudzana ndi matenda osowa a majini. Umenewo ndi matenda omwe tikambirane lero, otchedwa Gorlin Syndrome. Musachite mantha mukamva dzinali. Tiyeni tikambirane mwachidule, m'njira yomwe mungamvetse.

Kodi Gorlin Syndrome ndi chiyani kwenikweni?

Mwachidule, Gorlin syndrome ndi matenda osowa kwambiri a majini . Amadziwikanso ndi mayina ena, monga `(Basal Cell Nevus Syndrome)`, `(Nevoid Basal Cell Carcinoma Syndrome - NBCCS)` ndi `(Gorlin-Goltz Syndrome)`. Munthu amene ali ndi vutoli amakhala pachiwopsezo chachikulu chotenga khansa komanso zotupa zomwe sizimayambitsa khansa (kutanthauza kuti sizimavulaza thupi kwambiri). Makamaka, pali chiopsezo chowonjezeka chotenga khansa ya pakhungu yotchedwa `(Basal Cell Carcinoma)` . Uwu ndiye mtundu wofala kwambiri wa khansa ya pakhungu.

Tangoganizirani, pali majini ena m'thupi lathu, omwe ntchito yawo yayikulu ndikuwongolera kugawikana kosafunikira kwa maselo ndi kupangika kwa zotupa. Timatchanso kuti "majini oletsa chotupa". Chifukwa chake, mu Gorlin syndrome, chomwe chimachitika ndichakuti pali kusintha, kutanthauza "kusintha" kwa majini awa. Ichi ndichifukwa chake chiopsezo chokhala ndi zotupa chimawonjezeka monga tafotokozera.

Chofunika kwambiri ndichakuti palibe mankhwala enieni a Gorlin syndrome pakadali pano . Koma musadandaule! Ngati mutsatira malangizo a dokotala wanu moyenera, ndipo ngati zinthu monga khansa zapezeka ndikuchiritsidwa msanga , munthu amene ali ndi vutoli akhoza kukhala moyo wabwinobwino. Siziyenera kukhudza moyo wanu wonse kapena khalidwe la moyo wanu.

Kodi vutoli ndi lofala bwanji?

Matenda a Gorlin ndi matenda osowa kwambiri . Akatswiri amati ngakhale m'dziko ngati United States, matendawa amakhudza anthu osakwana 50,000. Komabe, chiwerengerochi chikhoza kukhala chokwera kwambiri. Chifukwa chake n'chakuti anthu ena ali ndi zizindikiro zochepa kwambiri moti sadziwa kuti ali ndi vutoli. Nthawi zambiri, zizindikiro zimawonekera mwa achinyamata kapena achinyamata .

Kodi zizindikiro za Gorlin Syndrome ndi ziti?

Zizindikiro za matendawa zimatha kusiyana malinga ndi munthu, koma pali zizindikiro zina zodziwika bwino:

Zinthu zomwe zimawonekera kwambiri

• Khansa ya pakhungu ya basal cell yambiri: Khansa ya pakhungu yamtunduwu nthawi zambiri imayamba m'malo omwe amakhudzidwa ndi dzuwa monga nkhope ndi khosi. Komabe, mwa anthu omwe ali ndi matenda a Gorlin, matendawa amatha kufalikira ali aang'ono, ali kumapeto kwa zaka zaunyamata kapena kumayambiriro kwa zaka za m'ma 20 .
• Matenda a odontogenic keratocysts:Izi ndi zotupa zopanda ululu, zopanda khansa zomwe zimapangika mu nsagwada. Zimapezeka kwambiri mwa ana ndi achinyamata omwe ali ndi matenda a Gorlin.
• Mabowo ang'onoang'ono kapena malo otsetsereka m'manja ndi pansi pa mapazi: Izi nthawi zambiri zimawoneka m'zaka za m'ma 20. Izi zimakhala zokhazikika.
• Kusintha kwa mafupa: Kusintha kwina kungaonekere momwe nthiti ndi msana zimapangikira.

Zizindikiro zosazolowereka

Si aliyense amene angakumane ndi zizindikiro izi, koma anthu ena angakumane ndi izi:

• Zotupa za muubongo: Izi zitha kukhala khansa (medulloblastoma) kapena zosakhala khansa (meningioma).
• Ma fibroma a mtima kapena mazira: Izi si zotupa za khansa, nthawi zambiri zimakhala zopanda vuto lililonse.
• Mavuto a maso: zinthu monga strabismus, nystagmus, ndi cataracts.
• Kusintha kwakukulu kwa mafupa: Mwachitsanzo, kusowa kwathunthu kwa msana (spina bifida).
• Mawonekedwe osazolowereka a nkhope: Kutalikirana kwambiri pakati pa maso (hypertelorism), kubadwa ndi milomo/kamwa kosweka, ndi madontho ang'onoang'ono oyera (milia) pankhope.
• Mutu waukulu kuposa wamba (macrocephaly).
• Kulephera kwa nzeru.
• Matenda a khunyu `(Kugwidwa)`.

Kodi n’chiyani chimayambitsa Gorlin Syndrome?

Monga tafotokozera kale, vutoli limayamba chifukwa cha kusintha kwa majini athu. Kunena zoona, pali majini atatu m'thupi lathu omwe amaletsa kupangika kwa zotupa (majini oletsa chotupa). Ndi majini otchedwa ``PTCH1`` (imeneyi ndi yomwe imakhudzidwa kwambiri), ``PTCH2`` ndi ``SUFU``. Majini amenewa akapanda kugwira ntchito bwino, maselo ena amayamba kukula modabwitsa. Ndicho chimene chimayambitsa zizindikiro monga zotupa.

Anthu ambiri amalandira kusintha kwa majini kuchokera kwa makolo awo. Komabe, nthawi zina, kusintha kwa majini kumeneku kungachitike mwachisawawa asanabadwe, popanda mbiri ya banja.

Kusintha kwa majini kumeneku kumachokera ku "autosomal dominant pattern" . Mwachidule, mumapeza jini yabwinobwino kuchokera kwa kholo limodzi ndi jini yosinthika kuchokera kwa lina. Muyenera kukhala ndi jini ya PTCH kapena SUFU kuti mupeze Gorlin syndrome.

Asayansi amati munthu amene ali ndi matenda a Gorlin syndrome amadwala khansa pokhapokha ngati mtundu wamba wa jini loletsa khansa wasinthidwa mwanjira ina. Mwachitsanzo, kuwonongeka kwa dzuwa kungayambitse kusintha kwa jini yabwino. Kenako, majini onse awiri sagwira ntchito bwino, ndipo zotupa sizingathe kuimitsidwa. Izi ndi zomwe zimayambitsa khansa ya pakhungu. Ndipo ngati maselo ena m'thupi awonongeka mwanjira imeneyi, mitundu ina ya khansa imatha kukula.

Kodi madokotala amazindikira bwanji vutoli?

Kuti adziwe matenda a Gorlin, dokotala wanu adzachita izi:

• Kuwunika thupi: Kufufuza khansa ya pakhungu.
• Mayeso ojambulira: Yang'anani zotupa, ma fibromas, kapena mavuto a mafupa m'chibwano (monga X-ray, CT scan, MRI scan).
• Kufufuza za khansa: Njira yochotsera minofu yaing'ono pakhungu kapena pa chotupa ndikuchiyang'ana pogwiritsa ntchito maikulosikopu kuti aone ngati chili ndi maselo a khansa.
• Kuyezetsa majini: Kuyezetsa magazi kumatengedwa kuti kuone ngati muli ndi kusintha kwa majini komwe kumakhudzana ndi matenda a Gorlin.

Kodi mankhwala a Gorlin Syndrome ndi ati?

Ngakhale kuti palibe mankhwala enieni a Gorlin syndrome, ngati mukupeza zotupa zomwe ndi khansa kapena zomwe zimayambitsa mavuto ena, mungafunike kuzichiza. Mwachitsanzo:

• Khansa ya pakhungu kapena chotupa cha m'chibwano chingachotsedwe ndi opaleshoni .
• Mankhwala ena a khansa, monga mafuta odzola pakhungu kapena mankhwala odzola pogwiritsa ntchito kuwala kwa dzuwa, angagwiritsidwenso ntchito kuwononga maselo a khansa.

Popeza muli pachiwopsezo chachikulu chotenga khansa, dokotala wanu angakulangizeni kuti mugwiritse ntchito mafuta abwino oteteza ku dzuwa, kuvala zovala za manja aatali, ndikuvala chipewa mukakhala padzuwa. Komanso, mayeso monga X-ray amachitidwa pokhapokha ngati pakufunika kutero. Kawirikawiri, madokotala a khansa amapewa kupereka chithandizo cha radiation ngati chithandizo cha khansa kwa anthu omwe ali ndi matenda a Gorlin. Izi zili choncho chifukwa radiation imatha kuwonjezera chiopsezo cha khansa.

Kodi Gorlin Syndrome ingapewedwe?

Sitingathe kuwongolera majini omwe timabadwa nawo. Chifukwa chake, palibe njira yopewera Gorlin syndrome. Komabe, mutha kuchepetsa chiopsezo chanu chokhala ndi khansa ya pakhungu mwa kuchitapo kanthu kuti mupewe . Komanso, mukapita kukayezetsa dokotala nthawi zonse, mutha kuzindikira khansa pachiyambi. Kenako zimakhala zosavuta kuchira.

Ngati muli ndi matenda a Gorlin (kapena ngati wina m'banja mwanu ali nawo) ndipo mukukonzekera kukhala ndi ana ndipo mukuda nkhawa kuti mwana wanu angalandire matendawa, onani mlangizi wa majini . Adzakufotokozerani zoopsa zomwe zingachitike kwa inu ndi mnzanu.

Kodi tsogolo la munthu amene ali ndi vutoli lidzakhala lotani? (Outlook)

Anthu ambiri amatha kuchiza matenda a Gorlin mwa kuchitapo kanthu kuti apewe khansa ya pakhungu komanso kupita kukayezetsa kuchipatala nthawi zonse. Mwa kuyezetsa khansa nthawi zonse monga momwe dokotala wanu akulangizira, mavuto aliwonse amatha kupezeka msanga . Anthu omwe ali ndi matenda a Gorlin amatha kukhala ndi moyo wautali komanso omwe alibe matendawa monga momwe anthu omwe alibe matendawa amakhalira.

Komanso, chofunika kwambiri kukumbukira ndichakuti si aliyense amene ali ndi matenda a Gorlin amene angadwale khansa kapena kukhala ndi mavuto ofanana. Zimasiyana malinga ndi munthu aliyense. Dokotala wanu adzakulangizani za matenda anu kutengera zizindikiro zanu.

Kodi ndimadzisamalira bwanji? (Kudzisamalira)

Ngati muli ndi matenda a Gorlin, muyenera kuchita izi kuti mupewe khansa ya pakhungu:

• Pewani kugwiritsa ntchito mipanda yopaka utoto.
• Khalani m'nyumba momwe mungathere nthawi ya dzuwa (10 koloko m'mawa mpaka 4 koloko madzulo).
• Valani mafuta oteteza ku dzuwa okhala ndi mphamvu ya SPF 30 tsiku lililonse. Pakaninso mafutawa pafupipafupi.
• Mukatuluka panja, valani zovala zoteteza ku UV ndi chipewa chachikulu.

Muyeneranso kuonana ndi madokotala osiyanasiyana kuti akuthandizeni kudziwa ngati muli ndi khansa kapena matenda ena. Nthawi yomwe muyenera kupitako imadalira ngati apeza zotupa kapena matenda ena. Maulendo a dokotala awa angaphatikizepo:

• Pitani kukayezetsa khungu lanu ndi dokotala wa khungu.
• Kuyezetsa mano ndi kujambula zithunzi kuti aone ngati pali zotupa mu fupa la nsagwada.
• Mayeso ojambulira zithunzi kuti aone ngati pali ma fibromas, zotupa muubongo, kapena mavuto a masomphenya.

Kodi ndiyenera kupita kwa dokotala liti?

Ngati mukukayikira kuti inu kapena mwana wanu ali ndi basal cell carcinoma, pitani kwa dokotala nthawi yomweyo . Ichi ndi chizindikiro chofala kwambiri cha Gorlin syndrome. Monga mitundu ina ya khansa ya pakhungu, dziwani ngati pali ziphuphu kapena mawanga atsopano, osatha, kapena omwe akukula.

Khansa ya pakhungu nthawi zambiri sigwirizana ndi Gorlin syndrome, koma mosasamala kanthu za chifukwa chake, ndikofunikira kuyezetsa.

Ndi mafunso ati omwe muyenera kufunsa dokotala?

Ngati mwapezeka ndi matenda a Gorlin, mungafunike kufunsa dokotala wanu mafunso awa:

• Ndi zizindikiro ziti za khansa zomwe ndiyenera kudziwa?
• Kodi ndiyenera kuyezetsa khansa kangati?
• Kodi ndingatani kuti ndichepetse chiopsezo cha khansa yanga?
• Kodi ndikufunika chithandizo cha zotupa zomwe sizili za khansa?
• Kodi mwayi wanga woti ndikhale ndi mwana wodwala matenda a Gorlin ndi wotani?

Matenda a Gorlin amabwera ndi mavuto angapo omwe anthu opanda matendawa sangakumane nawo. Ngati muli ndi NBCCS, inu ndi gulu lanu lachipatala muyenera kukhala maso kwambiri pozindikira ndikuchiza khansa iliyonse ya basal cell yomwe imayamba.

Uthenga Womaliza Wopita Nawo Kunyumba

Nkhani yabwino ndi yakuti khansa ya basal cell imachiritsidwa kwathunthu ngati yachiritsidwa msanga. Komanso, zotupa zomwe sizili khansa nthawi zambiri sizimayambitsa mavuto. Ngati zitatero, dokotala akhoza kuzichotsa kapena kulangiza mankhwala ena. Mutha kukhala ndi moyo wautali komanso wathunthu ndi Gorlin syndrome. Chifukwa chake, musachite mantha, koma samalani. Tsatirani upangiri woyenera wachipatala, ndipo zonse zikhala bwino!

Matenda a Gorlin , NBCCS, khansa ya basal cell, matenda a majini, khansa ya pakhungu, matenda a khungu