Neurofibromatosis a yara: cututtuka, ganewar asali, magani

Neurofibromatosis ne a hereditary cuta, wanda aka halin bayyanar siffofin maruran. Matsayin mai mulkin, mafi yawansu ba su halin da kirki, manya. Marurai za a iya sarrafa a kan fata, a cikin kwakwalwa ko laka, da jijiyoyi da kuma sauran gabobin. Jiyya nufin da wani m kau da neoplasm kuma m sa idanu rikitarwa. Popular sau da yawa kamu neurofibromatosis a yara. Alamun a kowane hali iya bambanta da ɗan. Duk da haka, likitoci rarrabe a dukan kungiyar na asibiti ãyõyi halayyar da cutar. Game da su da kuma babban hanyoyin magani da wannan cuta a cikin matasa marasa lafiya da muka bayyana, a wannan labarin.

Description da cutar

Neurofibromatosis an dauki wani kwayoyin cutar da aka gada. An halin da ci gaban da kwakwalwa / laka lahani, fata, da wasu daga cikin kayan ciki da juyayi tsarin.

neurofibromatosis cuta bayyana ƙari samuwar. Lokacin da marurai kara a size, sun iya ƙeta a kan muhimmanci gabobin, game da shi, har suka sa cin gaban related kiwon lafiya matsaloli.

Neurofibromas (marurai) yawanci bayyana a cikin shimfiɗar jariri, amma a lokacin balaga. A farko na asibiti alamun akwai duhu aibobi. Suna ba tare da ciwo ko itching. Brown spots za a iya sarrafa cikakken ko'ina a jiki, ciki har da armpits da kuma makwancin gwaiwa yankin.

A cewar masana, mafi sau da yawa benign neurofibromas daban-daban yanayi, kawai 5% na su anã gitta su a kan aiwatar da neoplastic canji, watau, m degeneration. Duk da haka, ko da benign growths sau da yawa kawo barazana ga jiki. The abu ne cewa, a kan lokaci da suka kara a size, cike da damuwa da al'ada aiki na wasu kayan ciki.

Neurofibromatosis a yara iya dangantawa da munanan na kashi nama. The mai tsanani da cuta jeri daga m ga mai tsanani, tare da karshen entails tawaya.

dalilai

Abin da ya haddasa neurofibromatosis? A Sanadin wannan cuta, a cewar masana, an rufe a 50% na lokuta da hereditary predisposition. Don zama daidai, shi ne kayyade factor rinjayar wasu juyayi tsarin sel (lemmotsity, melanocytes, fibroblasts endoneurial). Haka kuma cutar bayyana kanta a cikin nau'i na da yawa marurai cewa saya siffofin shekaru spots, subcutaneous Cones. A wasu lokuta, ƙila a sami matsaloli da kwarangwal. Wannan irin disturbances a cikin jiki da kuma shafi na kashin baya jijiyoyi.

A cikin hali na kwayoyin cuta tasowa factor ne dangane da irin gaji m gene. Alal misali, idan iyaye daya tana da lalace gene, rabin yara na iya gada da wannan cuta. Yana da muhimmanci a lura da cewa mataki na bayyanuwar da cuta ne kai tsaye related to da magana matakin da kwayoyin halittu da kansu.

A daya hannun, a cikin hanyar cutar da kuma sau da yawa ya aikata wani bazuwar factor, cewa shi ne wani yunkri maye gurbi. Masana kimiyya ci gaba yanzu na rayayye karatu da hanyoyin da cututtuka kamar neurofibromatosis. A Sanadin cutar cikin sharuddan maras wata-wata maye gurbi ne yanzu kusan ba zai yiwu a bayyana.

Yana da muhimmanci a lura da cewa cutar da ake kamu daidai daga mutãne kuma daga cikin adalci jima'i. Wannan shi ne dalilin da ya sa yana da wuya a sanyã wa jima'i.

Neurofibromatosis a yara auku kawai saboda kwayoyin halitta, kamar yadda wani yunkri maye gurbi - shi ne kawai samu Pathology a mazan marasa lafiya.

rarrabuwa

A halin yanzu, cutar da ake kullum conventionally kasu kashi shida siffofin:

• Recklinghausen cuta (neurofibromatosis irin 1) ne suka kamu da a 90% na lokuta. Domin wannan nau'i ne halin da ci gaban kirki marurai, pigmented aibobi a kan fata, kashi munanan da canje-canje a cikin Iris.
• Neurofibromatosis irin 2 ne sosai kama da na asibiti manifestations a farko type, amma fata lahani ba haka ba ne a bayyane. A wannan yanayin, da cutar da aka bayyana a cikin nau'i na mahara neuromas kwakwalwa da kuma laka, kazalika da auditory jijiya. Marurai na tsakiya m tsarin, kamar yadda mai mulkin, kafa tsakanin 20 da 30 shekara na rayuwa.
• The uku irin halin da manyan yawan neurofibromas, wanda a wasu lokuta sa a glioma na na gani jijiya da kuma meningitis.
• A karo na hudu irin in ba haka ba kira a matsayin wani kashi, kamar yadda a cikin wannan hali, da Pathology rinjayar da fata gida.
• A karo na biyar da irin da babu nodular raunuka, amma gaban pigmented aibobi da kofi launi.
• shida da irin cutar da ake bincikar lafiya a marasa lafiya a kan shekaru 20 da shekaru, amma a cikin wannan hali, da kayyade factor ba ya taka musamman rawa.

Yana da muhimmanci a lura da cewa a cikin matasa marasa lafiya, kawai biyu siffofin da cutar: neurofibromatosis irin 1 neurofibromatosis irin 2.

cututtuka

A cewar masana, game da daya bisa uku na marasa lafiya na dogon lokaci da zai iya ba zargin gaban cututtuka. Mafi sau da yawa, da rashin lafiya da aka yi fama a gaba na yau da kullum dubawa. Likitoci suka ce gaban kananan nodules karkashin fata.

Na biyu bisa uku na marasa lafiya cuta neurofibromatosis gane bayyanar wani neurological cuta yanayi.

Mutane da yawa koyi game da ganewar asali ne kawai bayan ziyarar da wani beautician. Brown spots bayyana a kan fuskarsa, baya da kuma wata gabar jiki. Yana da wannan asibiti ãyã, kamar yadda mai mulkin, da kuma dalilin game da wani gwani.

Yana da muhimmanci a lura da cewa kadan daban bayyana neurofibromatosis a yara. A bayyanar cututtuka mafi sau da yawa ne daga farkon zamanin rayuwa. A nan muna da farko magana game da mahara pigmented aibobi a kan fata. Kamar yadda wani matashi, sami kananan marurai na daban-daban masu girma dabam da kuma siffofi. Wannan irin neurofibromas iya zama kawai 'yan dari ko fiye. A wasu marasa lafiya, akwai canje-canje a cikin kwarangwal (scoliosis, hip nakasar da kwanyar kashi lahani a ido).

Yana da muhimmanci a lura da cewa ciwon daji iya yada wa wani jijiya a cikin jiki, amma yawanci tashi daga sel da jijiya asalinsu. A wannan yanayin, ba su haifar da wani tsanani take hakki. Idan ƙari kai tsaye shafi cikin laka, akwai wani tsanani hatsari ga lafiya na haƙuri.

Neurofibromatosis Recklinghausen (irin 1) yawanci bayyana kanta a wani wuri shekaru. Domin wannan cuta halin da samuwar shekaru spots da haske launin ruwan dafa. Abin sha'awa, suka kuma za a iya samu a daidai da lafiya yara. Idan fiye da biyar wannan spots a jiki diamita na 5 mm ko fiye wajibi ne a gudanar da bincike gwaji domin ware irin wannan cututtuka kamar Recklinghausen neurofibromatosis.

Nau'i na biyu na cutar da ake mafi sau da yawa bincikar lafiya a matsayin saurayi. An halin da samuwar marurai da shafi cikin auditory jijiya. Bugu da kari, marasa lafiya da seizures, ringing a cikin kunnuwansu, ciwon kai da kuma vestibular cuta.

bincikowa da

A farko wuri tabbatar da wannan cuta bukatar wani likita jarrabawa, kamar yadda na fili da na asibiti da ãyõyinMu da damar da wani cikakken ganewar asali. Duk da haka, a wasu lokuta, shi na bukatar ƙarin jarrabawa. A likita ya kamata ya yi la'akari da muka ambata a sama iri neurofibromatosis ga karshe ganewar asali.

Recklinghausen ta cuta da aka tabbatar da kasancewarsu a cikin haƙuri na akalla biyu daga cikin wadannan siffofin:

• Lisch nodules.
• Brown spots na ruwan kasa launi da kuma wani adadin size.
• Specific munanan daga kashi.
• Biyu ko fiye neurofibromas.
• Hyperpigmentation a cikin hamata / makwancin gwaiwa yankin.
• A gaban da cutar a kusa da dangi.

Domin ƙarin bayani nazarin data kasance marurai likita yawanci nada wani MRI, X-haskoki da CT scans. Radiograph wajibi ne don gane yiwu take hakki na mutum kashi na'ura.

Gano wani biyu irin cuta yakan haifar da wani audiogram. Wannan musamman binciken da wanda kiyasta tsanani daga cikin haƙuri na ji.

Ko da kuwa da irin cutar da ake da shawarar a duk, ba tare da togiya, da ake kira bincike na neurofibromatosis. Ya yi nufi da kanta ya fi na kowa kayyade gwaji, ciki har da prenatal. A karshe wani zaɓi - shi ne bincike na ruwar ko chorionic villi.

Lokacin da abin da ya kamata a kira wani likita

Da farko ya kamata a lura da cewa magani da wannan cuta mafi sau da yawa da hannu da dama da kwararru. Neurologist kallon matasa haƙuri da yara, da kuma daga baya ya koma da sauran likitoci. Kamar yadda riga aka ambata a sama, da neurofibromatosis ne gaji cuta da ake dangantawa da maye gurbi na wasu chromosomes. Saboda haka, m magani a halin yanzu bayar da likitoci iya ba. A cikin hali na sauri-gudãna da cutar akwai bukatar a m magani idan neurofibromas ake sarrafa a ciki gabobin. Neurosurgeons a kafaɗunsu, kamar yadda mai mulkin, sun kasance alhakin ƙari nodules, wanda ake ma cire.

Abin da ya kamata a bi

Modern magani ba zai iya bayar da m hanyoyin da magani daga wannan cuta. Shi ya sa likitoci ayan rubũta symptomatic far. Yana yakan haifar da wata liyafar da wasu kungiyoyin da kwayoyi ( "Ketotifen", "Fenkarol", "Tigazon") don mayar da tafiyar matakai na rayuwa a cikin jiki da kuma rage asibiti ãyõyin cuta kamar neurofibromatosis.

Jiyya An wajabta kuma kawai wani likita a kan wani mutum akai, shan la'akari da takamaiman manifestations da cutar. A nada far an kuma dauki la'akari da sakamakon da kwayoyin analysis, cikar orthopedic, ophthalmology da Oncology.

Kuma sau da yawa bukatar m m baki. Yana da muhimmanci a lura da cewa a cire duk ƙari shafuka iya zama musamman rare saboda da high Yaduwar tsari. A wasu lokuta, bugu da žari bukata fata grafts. Babban alamomi ga tiyata ne da wadannan: ciwo mai tsanani a cikin shafa yankin, pitting nodes, malignancy, rushewa daga muhimmanci gabobin. A kwaskwarima tiyata a nada ne musamman rare, kamar yadda shi ne mafi kusantar su tsananta neurofibromatosis. jama'a magunguna Jiyya unacceptable.

A yau mun san babban yawan lokuta wannan cuta, wanda ya auku a sakamakon wasu cututtuka. Wannan shi ne dalilin da ya sa yana da muhimmanci kada su bar matsala unattended.

zai yiwu rikitarwa

Kamar yadda muka gani a sama, ba za a bar unattended yara neurofibromatosis. Jiyya ya zama dace da m. In ba haka ba, matukar high yiwuwa na tsanani matsaloli da shafi da ilimi da kuma kiwon lafiya ta jiki da matasa haƙuri, da ingancin rayuwa. Masana sun gano wadannan yiwu rikitarwa:

• Hauhawar jini.
• Seizures (lura a 40% na lokuta a yara tare da na farko da irin rashin lafiya).
• Wani nauyi.
• Jawabin cuta.
• Scoliosis.
• Illa wahayi.
• Jinkirin jima'i ci gaba.

Neurofibromatosis a yara, da bayyanar cututtuka da wanda aka bayyana a sama, na bukatar wani m m likita dubawa, rigakafin rikitarwa. your likita iya bayar da shawarar fi matakan idan ya cancanta.

Asarar ji a neurofibromatosis biyu irin aka dauke su da wani nuni ga kafa musamman ji implant. The na'urar ci gaba da watsa audio sakonni zuwa kwakwalwa damar da yaron ya gane sauti da kuma gane magana.

rigakafin

Abin baƙin ciki, likitoci ba zai iya a halin yanzu samar da tasiri rigakafin cutar. The abu ne cewa, a karshe ta haddasawa ba a karatu. Masana sun bayar da shawarar a kullum saka idanu da kiwon lafiya na yaro, a kai a kai ya kai shi ga likita Nazarin.

Lokacin da ya kamata faɗakar da neurofibromatosis

A yara, cututtuka na farko bayyana a cikin nau'i na haske launin ruwan kasa spots a kan fata. Idan yaro yana da wadannan cututtuka, yana da muhimmanci domin ganawa da wani gwani ya yi mulkin fitar da wannan ganewar asali da shi.

Idan neurofibromatosis an riga an tabbatar da, dole ne ka kullum saka idanu da yanayin da kananan haƙuri, ƙari samuwar. Lokacin da na karshe karuwa ne kuma shawarar zuwa neman m taimako.

Abu mafi muhimmanci a cikin wannan al'amari - don gane cuta a farkon yiwu. Bisa kididdigar da, game da 60% na yara tare da wannan ganewar asali da mun gwada da m ãyõyin cuta. Haka kuma, da yawa ba su bukatar musamman da magani.

Saboda haka, ya bayyana cewa kafin lokacin kada ka ji tsoro irin wannan cututtuka kamar neurofibromatosis a yara. Photos of matasa marasa lafiya sun fili ya nuna cewa wannan Pathology iya rayuwa. Hakika, yara da mafi tsanani siffofin da cutar ne sau da yawa rikitarwa bayyana. Duk da haka, tare da m taimako da kuma goyon baya da suka iya kai sosai m, da kuma mafi muhimmanci, wani farin ciki rai.