Neurofibromatosis kuvana: zviratidzo, nechirwere, kurapwa

Neurofibromatosis chirwere nhaka, iyo inozivikanwa nokuva chitarisiko anomera. Kazhinji, vakawanda vavo dzinoratidzwa benign hunhu. Namamota vari vemunyika paganda, muuropi kana musana, tsinga uye dzimwe nhengo. Treatment zvinoreva neboka kuvhiya nokubviswa neoplasm kwaizotevera vachiongorora matambudziko. Kazhinji chirwere neurofibromatosis vana. Zviratidzo neimwe nyaya dzingasiyana zvishoma. Zvisinei, vanachiremba kusiyanisa rose boka kunotoda kurapwa zviratidzo yo chirwere. About pavo huru nzira yokurapa zvirwere ichi muvarwere vaduku isu tsanangura munyaya ino.

Description of chirwere

Neurofibromatosis kunoonekwa sechinhu majini chirwere iri nhaka. It kunoratidzwa kukura uropi / musana kuremara, ganda, vamwe zvomukati uye tsinga.

neurofibromatosis chirwere kunoratidza bundu inotungamirwa. Kana mapundu dzichiwedzera kukura, vanogona kanganisa rinokosha zvomukati, nokudaro Nokumukira kukura nezvinetso kufanana utano.

Neurofibromas (mapundu) kazhinji kuzviratidza muuduku, kana panzvimbo pakuyaruka. Zviratidzo kunotoda yokutanga rima mavara. Havana achiperekedzwa kurwadziwa kana kuvaviwa. Brown makwapa zvinogona vemunyika zvachose chero mumuviri, kusanganisira muhapwa uye muhudyu nharaunda.

Maererano nenyanzvi, kazhinji benign neurofibromas siyana hunhu, 5 chete% wavo kupesvedzera nedanho neoplastic kusanduka, kureva, akaipa degeneration. Zvisinei, kunyange benign growths kazhinji kutakura kukanganisa muviri. The ndechokuti pamusoro nguva kuwedzera kukura, kukanganisa chakaipa nokushanda kwenhengo imwe nhengo dzomukati.

Neurofibromatosis muvana vanogona chokuita abnormalities pfupa tishu. The kwokuomerwa hosha anoda kubva nyoro kusvika kwazvo, pamwe yokupedzisira rinobatanidza urema.

zvikonzero

Chii chinokonzera neurofibromatosis? The zvinokonzera chirwere ichi, maererano nenyanzvi, akafukidzwa muna 50% kuti zviitiko enhaka kubatwa. Kuti kuva chaiwo, ndicho majini chinhu chinobata zvimwe tsinga masero (lemmotsity, melanocytes, fibroblasts endoneurial). Hosha kunooneka pacharo muchimiro anomera dzakawanda kuti wana nezvimiro zera makwapa, subcutaneous pasikweya. Mune zvimwe zviitiko, pangava nezvinetso marangwanda. kuti mhirizhonga iri muviri mhando iyi uye zvinokanganisa musana tsinga.

Kana masero chirwere hwapedyo chikonzero ndechokunyanya zvichienderana norudzi nhaka chakaremara Gene. Somuenzaniso, kana mumwe mubereki ane hunenge Gene, hafu vana vanogona nhaka chirwere ichi. Zvakakosha kuziva kuti ukuru nokuratidzwa chirwere ndiyo akarondedzera okuti pamwero kwemajini pachavo zvakananga.

Ukuwo, chinokonzera chirwere uye kazhinji anoita anongoitika chinhu, kuti chinhu kwatangwa mutation. Masayendisiti mberi zvino kushingaira kudzidza nzira zvirwere zvakadai neurofibromatosis. Zvinokonzera hosha tichitarisa vaingoerekana mutation zvino zvinenge zvisingaiti kutsanangura.

Zvakakosha kuziva kuti hosha iri panoonekwa zvakafanana pakati pavanhu uye pakati akanaka pabonde. Ndokusaka zvakaoma anotaurwa zvepabonde.

Neurofibromatosis muvana kunoitika nokungoti majini hunhu, sezvo kwakanga kwatangwa mutation - iri chete kuwana zvokurwara muvarwere vakwegura.

kupatsanura

Pari maperembudzi kazhinji conventionally akakamurwa matanhatu siyana:

• Recklinghausen chirwere (neurofibromatosis mhando 1) kunokwanisa kuonekwa muna 90% nyaya. Nokuti chimiro ichi kunoratidzwa pave benign namamota, pigmented makwapa paganda, mapfupa abnormalities uye kuchinja Iris.
• Neurofibromatosis mhando 2 zvikuru zvakafanana kurapwa zvinoratidza rokutanga mhando, asi ganda zvikanganiso havasi kudaro kuoneka. Panyaya iyi, hosha kunoratidzwa muchimiro vakawanda neuromas uropi uye musana, uyewo auditory etsinga. Namamota ari nechepakati tsinga, sezvinoita mutemo, yakaumbwa pakati 20 uye 30 makore oupenyu.
• Wechitatu mhando kunoratidzwa vakawanda neurofibromas, izvo dzimwe nguva kuti glioma pamusoro optic etsinga uye yomumuzongoza.
• Mhando wechina zvimwe kunzi chidimbu, sezvo iri zvazvingava, zvokurwara kunokanganisa ganda munharaunda.
• Mhando wechishanu zvichikonzerwa nokusavapo nodular maronda, asi kuvapo pigmented makwapa pakati kofi ruvara.
• wechitanhatu mhando maperembudzi chirwere Varwere pamusoro makore 20, asi muchiitiko chino, majini chinhu hakurevi vane basa rinokosha.

Zvakakosha kuziva kuti muvarwere vaduku vaviri chete siyana chirwere: neurofibromatosis mhando 1 neurofibromatosis mhando 2.

zviratidzo

Maererano nenyanzvi, vanenge chimwe muzvitatu varwere kwenguva refu uye haagoni chifungidziro kuvapo kwezvirwere. Kakawanda, kurwara iri chirwere unotevera dzose kuongorora. Doctors vanoti kuvapo nodules duku pasi ganda.

The chetatu varwere chirwere neurofibromatosis wechipiri ticherechedze chitarisiko chero chetsinga zvirwere hunhu.

Vakawanda vanodzidza nezvezvinhu chirwere chavo chete pashure kushanyira imwe beautician. Brown makwapa aonekwa kumeso, musana uye maoko nemakumbo. Ndicho ichi chiratidzo kurapwa, sezvo ushe, uye chikonzero zvivabatsire kune nyanzvi.

Zvakakosha kuziva kuti zvishoma zvakasiyana akaratidza neurofibromatosis vana. Zviratidzo kazhinji vari kubva pamazuva oupenyu okutanga. Pano tiri achinyanya kutaura vakawanda pigmented makwapa paganda. Somuyaruki, akawana mapundu duku zvakasiyana hukuru uye zvitarisiko. mhando iyi neurofibromas kungava mashomanana zana kana kupfuura. Mune vamwe varwere, pane kuchinja marangwanda (scoliosis, hudyu wakarebesa yeDehenya mapfupa kuremara muziso).

Zvakakosha kuziva kuti bundu anogona kupararira kune tsinga pamuviri, asi vakawanda muka kubva mumasero etsinga midzi. Muchiitiko chino, havaiti kukonzera chero zvakakomba kutyorwa. Kana bundu anokanganisa zvakananga musana, pane nengozi kuti utano murwere.

Neurofibromatosis Recklinghausen (mhando 1) kazhinji hworatidza pacharo pazera duku. Nokuti chirwere ichi dzinoratidzwa paumbwe zera mavara Light brown Hue. Sezvineiwo, vanogona kuwanikwawo vana gwindiri. Kana mavara anopfuura mashanu akadaro muviri dhayamita 5 mm kana kupfuura zvakafanira kuti kuitisa nekuongororwa chirwere kuti asava zvirwere zvakadai Recklinghausen neurofibromatosis.

The mhando chirwere chechipiri kakawanda akanzi achiri kuyaruka. It kunoratidzwa nokuumbwa namamota kukanganisa auditory etsinga. Uyezve, varwere vane pfari, achirira munzeve, nomusoro uye vestibular zvirwere.

Diagnostics

Panzvimbo yokutanga kusimbisa chirwere ichi vanoda kuongororwa yokurapa, sezvo pachena kurapwa zviratidzo kubvumira kuongororwa zvakarurama. Zvisinei, mune zvimwe zviitiko, zvinoda okuwedzera kuzviongorora. Chiremba anofanira kufunga mhando dzataurwa pamusoro apa kubva neurofibromatosis chokupedzisira zvawanikwa.

Disease Recklinghausen raMwari kunosimbiswazve nokuvapo murwere vanenge maviri anotevera zvinhu:

• Lisch nodules.
• Brown makwapa dzebhurauni ruvara uye mumwe yakawanda saizi.
• Dzinenge abnormalities pfupa.
• Maviri kana kupfuura neurofibromas.
• Hyperpigmentation ari hapwa / groin nharaunda.
• Pamberi chirwere pedyo nehama.

Kuti uwane kudzidza zviripo anomera chiremba kazhinji anogadza ane MRI, maekisirei uye CT Scans. Radiograph zvakafanira kuona zvinobvira kutyorwa kwevanhu mapfupa muchina.

Kuwana rudzi chirwere chechipiri chinoreva chinhu audiogram. Izvi chidzidzo chinokosha naro zvinofungidzirwa kwokuomerwa murwere kunzwa.

Pasinei norudzi hosha inokurudzirwa vose, pasina chaakasiya anonzi kuongorora neurofibromatosis. Iye zvinoreva roga rakajairika nemagene yekuedzwa, kusanganisira pamuviri. The wokupedzisira nzira - ndiwo kuongorora Líquido mvura kana chorionic villi.

Kana uye chii Achanzi chiremba

Chekutanga zvinofanira kucherechedzwa kuti kurapa chirwere ichi unowanzosangana zvinobatanidzwa unyanzvi akawanda. Wetsinga vakatarira wechidiki murefu pauduku, uye gare gare vamwe vanachiremba. Sezvambotaurwa pamusoro apa, neurofibromatosis chiri nhaka chirwere kuti kusonganirana mutation vamwe okutodza. Naizvozvo, yakakura kurapwa Pari kupa vanachiremba havagoni. Kana kutsanya-inoyerera chirwere pane kudiwa ari kuvhiya kurapwa kana neurofibromas vari mupasi riri zvomukati. Neurosurgeons panguva mapfudzi, sezvinoita mutemo, zvave kukonzera bundu nodules, iyo vari kubviswa.

Chii chinofanira kubatwa

mushonga Modern havagoni kupa okunyanyisa nzira yokurapa chirwere ichi. Ndicho chikonzero vanachiremba vanowanzotevedzera taurira symptomatic kurapa. Rinoreva panosvikira dzimwe mapoka zvinodhaka ( "Ketotifen", "Fenkarol", "Tigazon") kudzorera metabolic pezvakaitwa mumuviri uye kuderedza zvekuchipatara zviratidzo chirwere akadai neurofibromatosis.

Treatment ari ushandiswe uye chete chiremba nomunhu, kufungisisa chaizvo zvinoratidza chirwere. In kugadzwa ARV uyewo kufungwa migumisiro majini pachisara kuguma vachibatsira, ophthalmology uye oncology.

Uye kazhinji zvinoda chiriporipocho kuvhiya kupindira. Zvakakosha kuziva kuti kubvisa zvose bundu site kunogona apo neapo nokuda mukuru hwakapararira kwacho. Mune zvimwe zviitiko, anowedzera zvaida ganda grafts. Huru zvairatidza kuti kuvhiyiwa ndizvo zvinotevera: kurwadziwa Munzvimbo, pitting nodes, malignancy, nokuvhiringidzirwa zvinokosha nhengo. In zvizorwa Kuvhiya vanogadzwa zvikuru kushoma, sezvo kazhinji sembura neurofibromatosis. pevairwara mishonga Treatment isingagamuchiriki.

Nhasi tinoziva vazhinji Muzviitiko chirwere ichi, kunoitika somugumisiro nezvimwe zvirwere. Ndokusaka zvichikosha kuti arege kusiya dambudziko vasina akavatarisa.

zvinogoneka yematambudziko

Sezvatotaurwa apa, hazvifaniri kusiyiwa vasina akavatarisa vana neurofibromatosis. Treatment vanofanira kuva nenguva uye vanokwanisa. Zvikasadaro, refu chaizvo mukana matambudziko zvakakomba kukanganisa njere uye utano vechiduku murwere, unhu hwake. Nyanzvi ziva zvinotevera matambudziko zvichibvira:

• BP.
• Pfari (kuchengetwa 40% kuti nyaya vana rokutanga mhando chirwere).
• Kusanzwa.
• Speech zvirwere.
• Scoliosis.
• Vasanyatsoona.
• Kunonoka kurara kukura.

Neurofibromatosis nevana, zviratidzo zvange zvataurwa pamusoro apa, chinoda madikanwa zvechigarire zvokurapa cherechedzo, ekudzivirira yematambudziko. chiremba wako ringavakurudzira vapore matanho kana zvakakodzera.

Kurasikirwa kunzwa neurofibromatosis chechipiri mhando anoonekwa kuva chiratidzo nomuenzaniso rinokosha kunzwa kudyara. The mudziyo anoramba transmits maCD anonongedzera kuti uropi anobvumira mwana kunzwisisa mitinhimira uye kuziva matauriro.

dziviriro

Zvinosuruvarisa, vanachiremba havagoni pari kupa inobudirira kudzivirira chirwere. The ndechokuti pakupedzisira zvayo chinokonzera havana kudzidza. Nyanzvi chete akakurudzira kuramba kuongorora utano mwana, vaigara zvakamutungamirira zvokurapa bvunzo.

Kana vanofanira kuona neurofibromatosis

In vana, zviratidzo kutanga kuonekwa muchimiro chiedza shava makwapa paganda. Kana mwana wako aine zviratidzo izvi, zvinokosha kubvunza nyanzvi kutonga kunze ichi chirwere izvozvo.

Kana neurofibromatosis yakatotanga yasimbiswa unofanira kuramba kuongorora mamiriro duku murwere, bundu inotungamirwa. Kana wokupedzisira kuwedzera varumbidzwa uye kutsvaka rubatsiro vakakodzera.

Chinhu chinonyanya kukosha panyaya iyi - kuziva chirwere sezvo mangwanani sezvinobvira. Maererano nenhamba, vanenge 60% vana vane chirwere ichi vane pfupi munyoro zviratidzo chirwere. Uyezve, vakawanda havadi vavashandire.

Saka, zviri pachena kuti mberi nguva usatya zvirwere zvakadai neurofibromatosis muvana. Photos wevarwere vaduku vakaratidza zvakajeka kuti zvokurwara ichi vanogona kurarama. Chokwadi, vana vane zvakawanda kwazvo marudzi chirwere kazhinji matambudziko angamuka. Zvisinei, pamwe kugara rubatsiro uye rutsigiro vanogona kutungamirira zvibereko chaizvo, uye vakawanda pazvese, upenyu hunofadza.