Neurofibromatosis i roto i ngā tamariki: tohumate, te tātaritanga, maimoatanga

Neurofibromatosis Ko te mate tuku, āhuatanga nei i te te ahua o te pukupuku. Ka rite ki te tikanga, te nuinga o ratou āhuatanga e te pūāhua Koló. Ka taea te paetata puku i runga i te kiri, i roto i te roro ua ranei, raru, me te tahi atu whēkau. Treatment tikanga e te tango hāparapara o te neoplasm me pōauautanga aroturuki muri. Tino maha pāngia neurofibromatosis i roto i ngā tamariki. Kia rerekē tohu i roto i ia take ahua. Heoi, tākuta te wehewehe i te rōpū katoa o tohu haumanu āhuatanga o te mate. Mō ratou ko nga tikanga matua o te maimoatanga o tenei mate i roto i tūroro taitamariki whakaahua tatou i roto i tenei tuhinga.

Whakaahuatanga o te mate

whakaaro neurofibromatosis te he mate iranga riro e te. Kei te āhuatanga i te reira e te whanaketanga o te roro / melé ua, kiri, etahi o nga whekau, me te pūnaha io.

mate neurofibromatosis faaite puku hanganga. A, no te whakanui i te pukupuku i roto i te rahi, kia takahia ratou i runga i nga whekau faufaa, reira whakapataritari i te whanaketanga o ngā raruraru hauora hāngai ana.

Neurofibromas (pukupuku) te tikanga puta i roto i te tamarikitanga, engari i roto i te tino. Ko te tohu haumanu tuatahi he wahi pouri. e kore e ratou tahi e te mamae minamina ranei. parauri taea te paetata tino ki hea i roto i te tinana, tae atu i te peke, me te rohe tapatapa.

E ai ki te tohunga, tino maha neurofibromas Koló āhua rerekē, e whai wāhi% 5 anake o ratou ki te tukanga o te hurihanga neoplastic, arā, e hōloa mārere. Heoi, growths ara Koló maha kawe te riri ki te tinana. Ko te mea, ko te e runga i te wā ake i ratou i roto i te rahi, whakararuraru i te mahi noa o etahi whekau.

Neurofibromatosis i roto i ngā tamariki kia pā ana ki abnormalities o wheua kiko. Ko te pakeke o te mate pae i ngawari ki nui atu, me te whakamutunga tura'ii te hauātanga.

take

He aha ai neurofibromatosis? Ko te take o tenei mate, kia rite ki ngā tohunga, e hipoki i roto i te 50% o ngā take o te predisposition tuku. Hei kia pau, ko reira pānga te take ira etahi pūtau io (lemmotsity, melanocytes, fibroblasts endoneurial). faaite te mate ano i roto i te puka o te maha pukupuku e whiwhi puka wahi tau, te mahuetanga subcutaneous. I roto i ētahi wā, i reira kia kia raruraru ki te tuahiwi. Tenei ahua o raruraru i roto i te tinana, me te pā i te raru tuaiwi.

I roto i te take o te mate ira whakawhanake te kei take i runga i te momo o riro ira. Hei tauira, ki te he te ira kua tūkinotia kotahi matua, ka taea e te hawhe te tamariki riro tenei mate. He mea nui ki te mōhio e te tohu o whakakitenga o te mate, ko te pā ana tika ki te taumata faaiteraa o te ira ratou.

I te tahi atu te ringa, te take o te mate, me te maha mahi he take tupurangi, i te mea he irakētanga māhorahora. tonu Scientists inaianei kaha ako i te huarahi o mate pērā i neurofibromatosis. Ko te take o te mate i roto i ngā o te irakētanga noa he tata e taea ki te whakamārama i teie nei.

He mea nui ki te mōhio e pāngia te mate te rite i roto i nga tangata, me te i roto i te takoto ataahua. Ko te aha he uaua ki te ahua ki te takoto.

Neurofibromatosis i roto i ngā tamariki puta anake no te mea o te āhua ira, rite te irakētanga noa - e riro anake reira māuiuitanga i roto i te tūroro pakeke.

whakarōpū

I tēnei wā, kei te tikanga conventionally wehea te mate ki ono puka:

• pāngia mate Recklinghausen (momo neurofibromatosis 1) i roto i 90% o te wā. Hoki te āhuatanga tenei puka e te whanaketanga o te pukupuku mārire, wahi pigmented i runga i te kiri, abnormalities wheua me huringa i roto i te iris.
• momo neurofibromatosis 2 Ko te tino rite ki faaiteraa haumanu i roto i te momo tuatahi, engari e kore e pera kitea nga hē kiri. I roto i tenei take, whakakitea te mate i roto i te puka o te maha roro neuromas me ua, me te te io rongo. Te pukupuku o te pūnaha io pokapū, rite ki te tikanga, i hanga i waenganui i te 20 me te 30 tau o te ora.
• āhuatanga toru o nga momo e te maha nui o neurofibromas, i roto i ētahi wā meinga te glioma o te karere whatu me kakā.
• te wha o nga momo te kore tuku ki rite te wāhanga, pera i roto i tenei take, pānga te māuiuitanga te kiri rohe.
• rima o te momo i te ngaro o pāpaka nodular, engari te aroaro o kotingotingo pigmented o te tae kawhe.
• pāngia ono momo o te mate i roto i ngā tūroro i runga i te tau o te 20 tau, engari i roto i tenei take, e kore e te take ira te tākaro i te tūranga motuhake.

He mea nui ki te mōhio e i roto i ngā tūroro taitamariki, e rua anake ngā puka o te mate: momo neurofibromatosis momo 1 neurofibromatosis 2.

tohu

E ai ki te tohunga, e pā ana ki tetahi toru o nga tūroro mo te wa roa, me te kore e taea e whakapae i te aroaro o mate. Nuinga o ngā wā, pāngia te ma'i e i te tirohanga i muri ngā. mea kaiwhakaako te aroaro o nodules iti i raro i te kiri.

Ko te tuatoru tuarua o nga tūroro mate neurofibromatosis kitea te ahua o tetahi disorders āhua neurological.

ako tokomaha te iwi e pā ana ki to ratou tātaritanga muri anake i te haerenga ki te faanehenehe. puta parauri ki runga ki te mata, hoki me wahi. Ko tenei tohu haumanu, rite ki te tikanga, me te take mo te tirohia ki te mātanga.

He mea nui ki te mōhio e rerekē whakakitea he iti neurofibromatosis i roto i ngā tamariki. Ko te tohu tino maha e i nga ra tuatahi o te ora. Tenei e matua korero tatou e pā ana ki wahi pigmented maha i runga i te kiri. Ka rite ki te taiohi, kitea pukupuku iti o rerekē te rahi o, me ngā āhua. Tenei ahua o neurofibromas kia he torutoru noa rau atu ranei. I roto i te tahi mau tūroro, i reira he huringa i roto i te kōhiwi (scoliosis, hip hira o te kohakore angaanga wheua i roto i te kanohi).

He mea nui ki te mōhio e taea te puku horapa ki tetahi karere i roto i te tinana, engari te nuinga ara ake i ngā pūtau o te pakiaka karere. I roto i tenei take, e kore e meinga e ratou tetahi maumaui nui. Ki te tika pā i te puku te ua, i reira te mea he fakatu'utāmaki nui ki te hauora o te manawanui.

te tikanga faaite neurofibromatosis Recklinghausen (momo 1) ano i te wawe tau. Hoki tenei mate āhuatanga e te hanganga o kotingotingo tau te marama hue parauri. Te mea faahiahia, ano e taea te kitea ratou i roto i ngā tamariki tino hauora. Ki te taua wahi neke atu i te rima i runga i te diameter 5 mm atu ranei o te tinana, ko te tika ki te whakahaere whakamātautau tātaritanga i roto i te tikanga ki te aukati i taua mate rite Recklinghausen neurofibromatosis.

Kei te tino maha pāngia te momo tuarua o te mate rite te taiohi. Kei te āhuatanga i te reira e te hanganga o te pukupuku e pa te io rongo. I tua atu, tūroro i hukihuki, waea i roto i te taringa, pāhoahoa me disorders vestibular.

tātaringa

I roto i te wahi tuatahi ki te whakaū i tenei mate rapu i te whakamātautau hauora, tukua rite kitea tohu haumanu he tātaritanga tika. Heoi, i roto i ētahi wā, e titau te reira whakamātautau atu. kia tango i te rata ki te pūkete te momo i runga ake-whakahuatia o neurofibromatosis mo te tātaritanga whakamutunga.

whakapumautia a Recklinghausen mate e te aroaro i roto i te manawanui o te iti rawa e rua o nga āhuatanga e whai ake nei:

• nodules Lisch.
• parauri o te tae parauri me te nui etahi o te rahi.
• abnormalities motuhake o te wheua.
• E rua, neke atu rānei neurofibromas.
• Hyperpigmentation i roto i te kēkē rohe / tapatapa.
• Ko te aroaro o te mate i roto i whanaunga tata.

Hoki te ako atu taipitopito o ngā tākuta pukupuku te tikanga e whakaturia ana he MRI, X-hihi, me scans CT. he tika Radiograph ki te kitea maumaui taea o te mīhini wheua tangata.

Te kimi i te momo tuarua o te mate fakahu'unga te audiogram. Tenei ako motuhake e i tata te pakeke o te rongo o te manawanui.

Ahakoa o te momo o te mate e tūtohu ki te katoa, kaore he aweretanga, te pera-ka karanga tātaritanga o neurofibromatosis. tikanga ia i tahaki te whakamātau ira tino noa, tae atu prenatal. Ko te kōwhiringa whakamutunga - ko reira te tātari o te waikahu Villi chorionic ranei.

A, no te me aha kia huaina te tākuta

Tuatahi o te katoa kia tuhia te reira e tino maha whai wāhi te maimoatanga o tenei mate e rave rahi tohunga. Toketā neave matakitaki ana manawanui taitamariki ki tamarikitanga, a muri piri e te tahi atu mau taote. Ka rite ki kua whakahuatia i runga ake, te neurofibromatosis ko te mate riro e pā e te ki te irakētanga o etahi pūira. Na reira, whakahere tēnei wā maimoatanga tuwhena kore e taea e ngā tākuta. I roto i te take o nohopuku-e rerengia ana o te mate i reira ko te hiahia i roto i te maimoatanga hāparapara ki te e paetata neurofibromas i runga i nga whekau. Neurosurgeons i nga pokohiwi, kia rite ki te tikanga, kua kawenga mō nodules puku, e kua nekehia atu ano hoki.

He aha kia tukinotia

e kore e taea e rongoā Modern whakahere tikanga tuwhena o te maimoatanga o tenei mate. Ko te aha tākuta ahu ki te whakahau therapy symptomatic. fakahu'unga te reira i te fariiraa o te tahi mau rōpū o taero ( "Ketotifen", "Fenkarol", "Tigazon") ki te whakahoki tukanga pūkoro i roto i te tinana, me te whakaiti i nga tohu haumanu o mate pērā i neurofibromatosis.

whakaritea Treatment te me te tākuta anake i runga i te pūtake takitahi, tango ki pūkete te faaiteraa motuhake o te mate. I roto i te whakarite o te therapy kua riro ano hoki ki te pūkete nga hua o te tātaritanga ira, te mutunga o orthopedic, Ophthalmology me te matepukupuku.

A maha rapu wawaotanga hāparapara arä. He mea nui ki te mōhio e ki te tango taea pae puku katoa kia tino onge tika ki te nuinga tiketike o te tukanga. I roto i ētahi wā, ato'a hiahiatia grafts kiri. Ko te tohu matua mō te pokanga ko te whai ake: mamae nui i roto i te wāhi kua pā, pitting kōpuku, repara, tauwhatinga o whekau faufaa. I roto i te pokanga Paku ki kia whakaritea he tino onge, rite he pea atu ki te whakahōhā neurofibromatosis. iwi rongoā Treatment Kāore.

I teie mahana, e matau ana tatou he maha nui o ngā take o tenei mate, e puta rite ki te hua o tahi atu mate. Ko Ko te aha e mea faufaa e kore ki te waiho i te raruraru tautahi.

pōauautanga taea

Ka rite ki kua tuhia i runga ake, e kore e e mahue tautahi tamariki neurofibromatosis. kia wā, me te whai tohu Treatment. Te kore, te tūponotanga tiketike rawa o ngā raruraru nui e pa ana i te hauora hinengaro, me te tinana o te manawanui taitamariki, tona kounga o te ora. tautuhi Tohunga nga pōauautanga taea e whai ake nei:

• Takawhita.
• Haurangi (kitea i roto i te 40% o ngā take i roto i te tamariki ki te momo tuatahi o te mate).
• Turi.
• disorders Kōrero.
• Scoliosis.
• kite waimaero.
• Roa te whanaketanga fakasekisualé.

Neurofibromatosis i roto i ngā tamariki, te tohu o e kua whakaahuatia i runga, titau te te tirotiro whakahauanga tūturu hauora, te ārai o pōauautanga. kia tūtohu koutou tākuta mehua whakanohonoho ki te tika.

whakaaro ngaronga o rongo i roto i neurofibromatosis momo tuarua te ki hei hei tohu mō te whakanoho i te rawatia ētehi rongo motuhake. Ko te pūrere tonu whakawhiti tohu oro ki taea te roro te tamaiti ki te kite oro, me te mohio ki nga korero.

ārai

Kia aroha mai, e kore e taea e ngā tākuta te whakarato tēnei wā ārai whai hua o te mate. Ko te mea, ko te e te mutunga kihai i i ako ona take. Tohunga anake tūtohu ki te aroturuki tonu te hauora o te tamaiti, wā arahina ia ki whakamātautau hauora.

A, no te e mōhio neurofibromatosis

I roto i te tamariki, tohu tuatahi puta i roto i te puka o parauri marama i runga i te kiri. Ki te mea i enei tohu tō tamaiti, he mea nui ki te kōrero ki te mātanga ki te whakahaere tikanga i roto i te tātaritanga reira tenei.

Ki te mea kua oti te whakau neurofibromatosis, me te aroturuki tonu koe i te huru o te manawanui iti, puku hanganga. A, no te e tūtohu hoki te hua whakamutunga ki te ani i te tauturu tohu.

Ko te mea tino nui i roto i tenei mea - ki te kitea te mate rite wawe rite taea. E ai ki ngā tatauranga, e pā ana ki 60% o ngā tamariki ki tenei tātaritanga i tohu āhua ngawari o te mate. Ano, e kore e tokomaha hiahia maimoatanga motuhake.

Ko te kupu, riro te reira mārama e kore e mua o te wa e wehi o taua mate rite neurofibromatosis i roto i ngā tamariki. Kua āta faaite Photos o tūroro taitamariki e taea tenei māuiuitanga ora. O te akoranga, he maha tamariki ki ngā puka atu nui o te mate e ara pōauautanga. Heoi, ki te awhina tamau me te tautoko ka taea e ratou arahi rawa hua, me tino nui, he ora oaoa.