Neurofibromatosis ana: zizindikiro, matenda, mankhwala

Neurofibromatosis ndi matenda otengera kwa makolo omwe amakhala ndi maonekedwe a zotupa. Monga ulamuliro, ambiri a iwo yodziwika ndi khalidwe chosaopsa. Zotupa akhoza isanafalikire pa khungu, mu ubongo kapena msana, mitsempha ndi ziwalo zina. Kuchiza ndi kuchotsa opaleshoni ya neoplasm ndi wotsatira mavuto polojekiti. Kaŵirikaŵiri matenda neurofibromatosis ana. Zizindikiro pa nkhani iliyonse zimachitika mosiyana. Komabe, madokotala kusiyanitsa gulu lonse la zizindikiro matenda khalidwe la matenda. Za iwo ndi njira yaikulu ya chithandizo cha matenda odwala wamng'ono ife kufotokoza m'nkhani ino.

Kufotokozera za matenda

Neurofibromatosis imatengedwa matenda majini amene tinatengera. Iwo amakhala ndi chitukuko cha ubongo / zopindika msana, khungu, ena ziwalo mkati ndi wamanjenje dongosolo.

matenda neurofibromatosis kuwawonetsera chotupa mapangidwe. Pamene zotupa kuwonjezera kukula, iwo akhoza kungapondereze ziwalo zofunika, potero n'zothandiza chitukuko cha mavuto ena thanzi.

Neurofibromas (zotupa) zimakhala ana, koma akamakula. Woyamba zizindikiro matenda ndi mdima mawanga. Iwo limodzi ndi ululu kapena kuyabwa. mawanga Brown akhoza isanafalikire mwamtheradi paliponse mu thupi, kuphatikizapo m'khwapa ndi groin m'deralo.

Akatswiri amanena zambiri chosaopsa neurofibromas chikhalidwe chosiyana,% 5 okha a iwo timakumana ndi ndondomeko ya kusintha neoplastic, ie, zilonda alibe. Komabe, zophuka ngakhale chosaopsa nthawi zambiri zimanyamula kuopseza thupi. Chinthu ndi pa nthawi kuwonjezera kukula, akumusokoneza uziyenda yachibadwa ziwalo zina mkati.

Neurofibromatosis ana akhoza kugwirizana ndi kuvutika minofu fupa. The kukula kwa matenda ranges ku ofatsa kwambiri, ndi yamasika kumatanthauza chilema.

zifukwa

Kodi chimayambitsa neurofibromatosis? Zimayambitsa matenda, malinga ndi akatswiri, anaphimba 50% ya milandu kutengera za kumtundu. Kukhala enieni, ndi chinthu majini zimakhudza ena mantha dongosolo maselo (lemmotsity, melanocytes, fibroblasts endoneurial). Matendawa kumaonekera mwa mawonekedwe a zotupa zambiri kuti tikhale mitundu zaka mawanga, cones subcutaneous. Nthawi zina, pangakhale mavuto ndi mafupa a. Mtundu uwu wa chisokonezo thupi bwanji misempha msana.

Pankhani ya matenda majini akufotokozera Chinthu ndi malingana ndi mtundu wa tinatengera zosalongosoka mbewu. Mwachitsanzo, ngati kholo limodzi ali ndi jini zowonongeka, theka anawo amatengera nthenda ili. Nkofunika kudziwa kuti digiri ya chiwonetsero cha matenda mwachindunji ndi msinkhu mawu a majini okha.

Komano, chifukwa cha matenda ndipo nthawi zambiri amachita chinthu chodabwitsa kuti ndi apitirize kusintha. Asayansi kupitiriza tsopano mwakhama kuphunzira njira za matenda monga neurofibromatosis. Zimayambitsa matenda mawu a kusintha mowiriza tsopano kovuta kufotokoza.

Nkofunika kukumbukira kuti matenda amawapeza mofanana pakati pa amuna ndi pakati pa kugonana chilungamo. N'chifukwa chake ndi zovuta kunena kuti kugonana.

Neurofibromatosis ana kumachitika makamaka chifukwa chosintha chikhalidwe chibadwa, monga kusintha mowiriza - izo anapeza yekha kudwala odwala achikulire.

Gawo

Panopa matenda ambiri conventionally ogaŵikana mitundu isanu:

• Recklinghausen matenda (neurofibromatosis mtundu 1) amawapeza 90% ya milandu. Pakuti fomu iyi amakhala ndi chitukuko cha zotupa mtima wabwino mawanga inki pa khungu, kuvutika fupa ndi kusintha Iris lapansi.
• Neurofibromatosis mtundu 2 ndi zofanana kwambiri ndi mawonetseredwe matenda mu mtundu woyamba, koma zopindika khungu sali choncho kuwoneka. Pankhaniyi, nthendayo anawonetseredwa mu mawonekedwe a angapo neuromas ubongo ndi msana, komanso mitsempha Makutu. Zotupa za chapakati mantha dongosolo, monga ulamuliro, unali pakati pa 20 ndi 30 zaka za moyo.
• Mtundu wachitatu amakhala ndi ambiri neurofibromas, zomwe zina chifukwa glioma ya mitsempha chamawonedwe ndi meninjaitisi.
• Yachinayi mwinamwake amatchedwa gawo, monga mu nkhani iyi, kudwala zimakhudza khungu kwanuko.
• mtundu wachisanu ndi kusapezeka kwa zotupa nodular, koma pamaso pa mawanga inki ya mtundu khofi.
• mtundu chimodzi cha matenda amawapeza odwala pa zaka 20, koma mu nkhani iyi, mfundo majini alibe udindo wapadera.

Nkofunika kudziwa kuti odwala wamng'ono, mitundu iwiri yokha ya matenda: neurofibromatosis mtundu 1 neurofibromatosis mtundu 2.

zizindikiro

Akatswiri amanena za chachitatu cha odwala kwa nthawi yaitali ndipo sangathe kuganiza pamaso pa matenda. Nthawi zambiri, matenda amawapeza pa chizolowezi anayendera lotsatira. Madokotala amanena pamaso pa tinatake tozungulira ochepera khungu.

Wachitatu chachiwiri cha odwala matenda neurofibromatosis kudziwa maonekedwe a aliyense ubongo matenda chikhalidwe.

anthu ambiri kuphunzira za matenda awo okha mutapita kwa wokongoletsa a. Brown mawanga kuonekera pa nkhope, mmbuyo ndi miyendo. Ndi chizindikiro matenda, monga ulamuliro, ndi chifukwa kungapezeke kuti katswiri.

Nkofunika kudziwa kuti pang'ono mosiyana akuwonetseredwa neurofibromatosis ana. zizindikiro zambiri zimachokera kwa masiku oyamba a moyo. Apa ife makamaka akulankhula za mawanga angapo inki pa khungu. Monga wachinyamata, anapeza zotupa ka zazikulu ndi zooneka mosiyanasiyana. Mtundu uwu wa neurofibromas akhale ochepa zana kapena kupitirirapo. Odwala ena, pali kusintha mu mafupa a (kupindika msana, ntchafu chilema ya zopindika chigaza fupa diso).

Nkofunika kudziwa kuti chotupa ingafalire kwa mitsempha aliyense mu thupi, koma ambiri uka kwa maselo a mizu mitsempha. Pankhaniyi, iwo alibe chifukwa zakusokonenezedwa kwambiri. Ngati chotupa chimakhudza chingwe msana, pali ngozi moyo wa wodwalayo.

Neurofibromatosis Recklinghausen (mtundu 1) kawirikawiri kumaonekera ali aang'ono. Pakuti matenda yodziwika ndi mapangidwe mawanga zaka kuyatsa bulauni hue. N'zochititsa chidwi kuti iwo akhoza kupezeka ana mwangwiro wathanzi. Ngati oposa asanu mawanga zinthu pa thupi awiri a 5 mm kapena zambiri n'kofunika kuchita kuyezetsa matenda kuti kukusalani matenda monga Recklinghausen neurofibromatosis.

Mtundu wachiwiri wa matenda nthawi zambiri anapeza kuti wachinyamata. Iwo amakhala ndi mapangidwe zotupa okhudza mitsempha Makutu. Komanso, odwala ndi khunyu, kulira m'makutu, mutu ndi matenda vestibular.

diagnostics

Mu malo oyamba kutsimikiza matenda amafuna kufufuza mankhwala, monga zoonekeratu zizindikiro matenda kulola ndi matenda zolondola. Komabe, nthawi zina, pamafunika Kupenda zina. dokotala ayenera kuganizira tatchulawa mitundu ya neurofibromatosis kwa matenda komaliza.

Disease Recklinghausen a zikutsimikiziridwa ndi kukhalapo mu mtima wa osachepera awiri pa mbali zotsatirazi:

• Lisch tinatake tozungulira.
• Brown mawanga a bulauni mtundu ndi kuchuluka kwa kukula.
• kuvutika zosonyeza fupa.
• Awiri kapena kuposa neurofibromas.
• Hyperpigmentation mu armpit / groin m'deralo.
• Pamaso pa matenda mu achibale.

Pakuti kuphunzira zambiri za zotupa alipo dokotala zambiri amawaika m'mutu ndipo anamupeza, X-ray ndi CT mapanga sikani. Radiograph n'kofunika kudziwa kuyan'anila zotheka kwa makina anthu fupa.

Kupeza mtundu wachiwiri wa matenda zikutanthauza kuti audiogram. phunziro ili yapadera imene akuti zovuta kumva wodwalayo.

Kaya mtundu wa matenda tikulimbikitsidwa kuti zonse, popanda yekha, otchedwa kusanthula neurofibromatosis. Iye akutanthauza palokha chiyesedwe ambiri chibadwa, kuphatikizapo akalandire chithandizo. Njira lomaliza - ndi kusanthula madzimadzi amniotic kapena chorionic villi.

Pamene ndi zimene titchedwe dokotala

Choyamba tisaiwale kuti mankhwala a matenda zambiri nawo madokotala angapo. Katswiriyu kuonera wamng'ono wodwalayo ndi mwana, ndipo kenako anagwirizana ndi madokotala ena. Monga tanenera kale pamwambapa, neurofibromatosis ndi matenda tinatengera amagwirizana ndi kusintha kwa DNA ena. Choncho, chithandizo mopitiriza panopa kupereka madokotala sindingakhoze. Pankhani ya mofulumira loyenda matendawa pali kufunika mankhwalawa opaleshoni ngati neurofibromas ali isanafalikire ziwalo mkati. Neurosurgeons pa mapewa, monga ulamuliro, akhala udindo tinatake tozungulira chotupa, amene achotsedwa.

Kodi kuchitiridwa

mankhwala amakono sangathe kupereka njira kwakukulu ya chithandizo cha matenda. Ndicho chifukwa madokotala amakonda mankhwala mankhwala symptomatic. Amatanthauza phwando la magulu ena a mankhwala ( "Ketotifen", "Fenkarol", "Tigazon") kubwezeretsa njira kagayidwe kachakudya mu thupi ndi kuchepetsa zizindikiro matenda a matenda monga neurofibromatosis.

Chithandizo Kwalamulidwa ndi chabe dokotala anthu paokhapaokha, kutenga nkhani maonekedwe enieni matenda. Poika mankhwala amadziwikanso n'komwe zotsatira za kuwunika chibadwa, mapeto zamafupa, maso ndi oncologist.

Ndipo nthawi zambiri amafuna mwamsanga alowererepo pa opaleshoni. Nkofunika kudziwa kuti kuchotsa malo onse chotupa akhoza kukhala osowa kwambiri chifukwa cha kuwanda mkulu wa ndondomeko. Nthawi zina, Komanso chofunika grafts khungu. The zikuonetsa chachikulu opaleshoni ndi zotsatirazi: ululu mu zinkakhala m'dera, pitting mfundo, chotupa chija, kusokonezeka kwa ziwalo zofunika kwambiri. Opaleshoni zodzikongoletsera kuikidwa ali osowa kwambiri, monga zambiri kukulitsa neurofibromatosis. wowerengeka azitsamba Chithandizo zosavomerezeka.

Lero tikudziwa ambiri milandu matenda, kumene kumachitika chifukwa cha matenda ena. Ndicho chifukwa chake ndi chofunika kuti asachoke vuto sizikugwiridwa.

zosokonezeka zotheka

Monga tanenera kale pamwambapa, sayenera sizikugwiridwa ana neurofibromatosis. Chithandizo akhale yake ndi oyenerera. Apo ayi, mwina wapatali wa mavuto aakulu okhudza thanzi ozindikira ndipo thupi la wodwalayo wamng'ono, khalidwe lake la moyo. Akatswiri kudziwa zotsatirazi mavuto zotheka:

• Oopsa.
• Khunyu (anamuona pa 40% ya milandu ya ana ndi mtundu woyamba wa matenda).
• Ugonthi.
• Kulankhula matenda.
• Kupindika msana.
• Mkhutu masomphenya.
• Anachedwa chitukuko kugonana.

Neurofibromatosis ana, zizindikiro amene akhala tafotokozazi, amafuna kuvomerezedwa okhazikika achipatala, kupewa mavuto. dokotala wanu angakulangizeni miyeso konzanso ngati n'koyenera.

Loss wa kumva mu neurofibromatosis mtundu wachiwiri amati chizindikiro kuwongola wapadera kumva analili. chipangizo mosalekeza transmits chizindikiro zomvetsera ku ubongo walola mwana kuzindikira phokoso ndi kuzindikira mawu.

kupewa

Mwatsoka, madokotala sangakhoze panopa kupereka ogwira kupewa matenda. Chinthu ndi kuti potsiriza zimayambitsa sizinachitike kuphunzira. Akatswiri kokha analimbikitsa zonse kuyan'ana umoyo wa mwana, nthawi zonse anamutengera mayeso zachipatala.

Kodi kuchenjeza neurofibromatosis

Ana, zizindikiro adzawonekera mu mawonekedwe a bulauni mawanga kuwala pa khungu. Ngati mwana wanu ali ndi zizindikiro izi, ndikofunika kufunsa katswiri kulamulira ichi matenda izo.

Ngati neurofibromatosis kale anatsimikizira, muyenera zonse kuyan'ana mkhalidwe wa mtima pang'ono, chotupa mapangidwe. Pamene kuwonjezeka otsiriza analimbikitsanso kupempha thandizo oyenerera.

Chinthu chofunika kwambiri pa nkhani imeneyi - kudziwa matenda mwamsanga. Malinga ndi kafukufuku, 60% ya ana ndi matenda awa zizindikiro ndi wofatsa matenda. Komanso, ambiri safuna chithandizo chapadera.

Choncho, zimaonekeratu kuti pasadakhale usaope cha matenda monga neurofibromatosis ana. Photos odwala achinyamata poyera kuti kudwala uyu akhoza moyo. Inde, ana ndi mitundu kwambiri za matenda nthawi zambiri mavuto uka. Komabe, mothandizidwa zonse ndi kuthandizana kungachititse bwino kwambiri, ndipo ambiri Chofunika, losangalala.