Ola kinoMaʻi a me nā kūlana

Neurofibromatosis i na keiki: symptoms,ʻike, lapaʻau

Neurofibromatosis mea he hereditary maʻi, a ua wehewehe aku ma keʻano o ka hikoko. E like me ka rula, ka hapanui o ia wehewehe aku ma ka benign külana. Hikoko hiki ke IIeEeAIEUIeXAIIUE ma luna o kaʻili, ma o ka lolo a me spinal kaula, nä olonä a me nā nā loko. Lapaʻau 'o ia hoʻi ma kaʻoki lāʻau lapaʻau ana o ka neoplasm, a mahope ka mālamaʻana complications. Muli o kona kulana pinepine diagnosed neurofibromatosis i loko o nā keiki. Symptoms ma kēlā me kēia hihia e ĸokoĸa ana nä pane i kekahi mau mea. Eia naʻe, kauka maopopo i ka pau pūʻulu o lapaʻau hoailona ano o ka maʻi. About ia, a me ka papa kuhikuhiE ano o ka hana maikaʻiʻole o kēia maʻi i loko o'ōpio hoʻomanawanui iki mai ke wehewehe mai i loko o kēia 'atikala.

Description o ka maʻi

Neurofibromatosis ua noʻonoʻo i ka aaiaoe maʻi i ua ili mai iā ia. Ua Ua wehewehe aku ma ka hooulu ana o ka lolo / spinal kaula mā,ʻili, kekahi o ka mea na nā loko, a me ka ua'emi nenoaia.

neurofibromatosis maʻi hoike mai ai iaʻeho ho okumu. I ka hikoko hoomahuahua i ka nui, ka mea i infringe ma ka nui nā loko, i laila e hoʻonāukiuki i ka hooulu ana o nā ola pilikia.

Neurofibromas (hikoko) IeAUPIIe, IAa IO ikea ma ka noho 'ana kamaliʻi, akā, e aho i puberty. Ka mua lapaʻau hoailona i pouli mau kiko. Ua i pu ma ka eha a me ka maneo. Brown mau kiko hiki ke IIeEeAIEUIeXAIIUE loa anywhere i loko o ke kino, me na armpits a me groin wahi.

E like me ka poe akamai, ka hapanui pinepine benign neurofibromas kekahi ano, wale 5% o ia i paku ole ia i ke kaʻina hana o ka neoplastic hou, 'o ia hoʻi, malignant degeneration. Eia naʻe, i benign ka ulu o ke pinepine lawe i ka hoʻoweliweli 'ana i ke kino. Ka mea o ia ma luna o manawa e mahuahua ma ka nui, hōʻoni i ka maʻamau functioning o kekahi kūloko ma nā loko.

Neurofibromatosis i loko o nā keiki i ke pili me abnormalities o ka iwi'aʻaʻa. Ke oolea o ka maʻi papa koa, mai mehana e, ua hanahana loa, a me ka la mahope entails kīnā o ke kino.

kumu

He aha ke kumu neurofibromatosis? e like me ka poe akamai, ua uhi nā kumu o kēia maʻi, ma ka 50% o ka hihia o hereditary predisposition. Ia e kūlike loa, ka mea, o ka aaiaoe ololi e loli ai kekahi ko mākou hopohopo'ōnaehana aeee (lemmotsity, melanocytes, fibroblasts endoneurial). Ka maʻi hoike mai ai ia ia iho i loko o ka palapala o ka hikoko lehulehu e kuai ano makahiki wahi lilelile, subcutaneous pele. Ma kekahi mau hihia, ma laila i ia pilikia a me ka iwi. Keia ano o ka haunaele ma ka kino, a he aha ka mea i ka spinal nā aʻalolo.

Ma ka hihia o aaiaoe maʻi? Acaeoey? Ololi e ke kaumaha ma muli o ka 'ano o ka ili hemahema'ōewe. No ka laʻana, ina kekahi makua i ka pōʻino, ua make'ōewe, ka hapalua o na keiki ke lilo mai ia ia i kēia maʻi. He nui e hoailona oukou i ka degere o ka hoike ana mai o ka maʻi mea pololei nā i ka hōʻike kiʻekiʻe o nā nā'ōewe lakou iho.

Ma ka 'ē aʻe lima, i ke kumu o ka maʻi a me ka pinepine hanaʻia he kaulele ololi, i ka mea he noho'na makaainana i ulu mutation. ʻepekema, e hoʻomau e like me neurofibromatosis 'Imi këia i ka maomeka o ka maʻi. Nā kumu o ka maʻi i loko o hua'ōlelo o ka noho'na makaainana i ulu mutation mea manawa aneane hiki ole ke hoomaopopo.

He nui e hoailona oukou i ka maʻi ua diagnosed like i waena o nā kānaka, a ma waena o ka maikaʻi ke keka. Ia mea ke kumu ia mea paakiki ke kaila i ka wahine.

Neurofibromatosis i na keiki ia lŘlŘ wale nō ke no ka mea, o ka mea aaiaoe ano, e like me kekahi noho'na makaainana i ulu mutation - ka mea, ua loaʻa i loko o pathology mua nā mea maʻi wale.

Mea Maluna

Currently, ka maʻi ua ano laulā, conventionally puunaueia i eono 'ano apau loa.

  • Recklinghausen maʻi (neurofibromatosis type 1) ua diagnosed i loko o 90% o ka hihia. No keia palapala ua wehewehe aku ma ka hooulu ana o benign hikoko, pigmented pūkoʻa ia ma kaʻili, iwi abnormalities a me nā loli i nā iris.
  • Neurofibromatosisʻano 2 ka mea i like loa i ka lapaʻau hoike ana ma ke 'ano mua, akā, i kaʻili mā i ole pela i nanaia. Ma keia hihia, i ka maʻi ua hoikeia i loko o ka palapala ana i mau neuromas lolo a me ka spinal kaula, e like me ka auditory nā aʻalolo. Hikoko o ka Central ko mākou hopohopo'ōnaehana, e like me ka rula, i hanaʻia ma waena o 20 a me 30 makahiki o ke ola.
  • Ke kolu oʻano Ua wehewehe aku ma ka helu nui o neurofibromas, a ma kekahi manawa i ka glioma o ka optic nā aʻalolo a me meningitis.
  • I ka ha oʻano ole ia haawiia keia i like me ka Hoʻohana, e like me ia ma keia hihia, ke pathology e loli ai i ka ili Tuamotu.
  • O ka luna alimaʻano ma ka wa e kaawale aku o nodular lesions, akā, i ke alo pigmented mau kiko o ke kope waihoʻoluʻu o.
  • ono o kaʻano o ka maʻi ua diagnosed i loko o nā mea maʻi ma luna o ka makahiki o ka 20 makahiki, aka, ma keia hihia, no ka aaiaoe ololi aʻole i pāʻani i ke kūlana kūikawā.

He nui e hoailona oukou ia ia ma ka opiopio hoʻomanawanui iki mai, elua wale ano o ka maʻi. Neurofibromatosis type 1 neurofibromatosis type 2.

symptoms

E like me ka poe akamai, e pili ana i kekahi hapakolu o ka hoʻomanawanui iki mai i ka lōʻihi manawa, a hiki ole manao i ke alo o nā maʻi. Loa pinepine, i ka maʻi ua diagnosed ma ka ia oaeouee? Nana. Kauka e olelo aku i ke alo o ka poe liilii, nodules ma lalo o kaʻili.

Ke lua o ka hapakolu o na nā mea maʻi maʻi neurofibromatosis huai ae i ke ano o kekahi neurological kāna hana ano.

He nui nā poʻe aʻo e pili ana i kā lākouʻike, ma hope o ke kipa i ka beautician wale. Brown mau kiko ikeia ma luna o ka maka, a me ka hoʻi lala. He kēia lapaʻau hōʻailona, e like me ka rula, a me ka mea i kōkua i ka hoʻomalu.

He nui ka e hoailona oukou i ka uuku okoa hoike neurofibromatosis i loko o nā keiki. Nā symptoms loa pinepine mea, mai ka lā mua o ke ola. Eia mākou i kuhikuhi kamailio e pili ana i mau pigmented mau kiko ma ka ili. E like me ka teenager, loaʻa hikoko liʻiliʻi o nāʻano nui like 'ole a me nā kinona. Kēiaʻano o nā neurofibromas paha e wale he mau haneri a oi. Ma kekahi nā mea maʻi, ma laila nō nā loli i loko o ka iwi (scoliosis, Hip deformity o ka puniu wale iwi mā i loko o ka maka).

He nui e hoailona oukou i kaʻeho ke hohola i kekahi aʻalolo i loko o ke kino, akā, puleʻAmelika, e ala aʻeʻoe, mai keena o ke aʻalolo aa. Ma keia hihia, ka mea e mai i kekahi aʻe 'nui. Inā kaʻeho ma kahi e pili i ka spinalʻula, aia no he nui pilikia i ke ola o ka hoʻomanawanui.

Neurofibromatosis Recklinghausen ( 'ano 1) IeAUPIIe, IAa IO hoike mai ai ia ia iho ma ka koke elemakule. No keia maʻi wehewehe aku ma ke kahua o ka makahiki kiko kuni i mea eleele hue. Interestingly, ka mea hiki ke loaʻa pū ma ka pono ola mau keiki. Inā oi ma mua o elima na kiko ma ka kino anawaena o 5 mm o a oi ka pono e hoʻomaka PalekanaEND_LINK ikea ai ka mea i hookaawale ia na maʻi like Recklinghausen neurofibromatosis.

I ka lua o kaʻano o ka maʻi ua loa pinepine diagnosed like me ka teenager. Ua Ua wehewehe aku ma ke kahua o ka hikoko ka heluna i ka auditory nā aʻalolo. Eia hou, nā mea maʻi i ke kāʻili 'ana, ringing i loko o nā pepeiao, a me ka headaches vestibular kāna hana.

lapaʻau '

Ma ka wahi mua e hōʻoia i kēia maʻi he pono i ka 'inikua ninaninau, e like me ka maopopo lapaʻau hoailona ae he pololeiʻike. Eia naʻe, ma kekahi hihia, e pono hou ninaninau. Ke kahuna lapaau e pono e lawe i ka mooolelo o ka luna-i hoike ia ke ano o ka neurofibromatosis no kaʻike, hope loa.

Recklinghausen ka maʻi ua hoʻopaʻaʻia ke alo i loko o ka hoʻomanawanui o ma ka liʻiliʻiʻelua o nā kēia mau hiʻona:

  • Lisch nodules.
  • Brown mau kiko o ka mea eleele waihoʻoluʻu a me ka kekahi nui o ka nui.
  • I Kekahi Mau abnormalities o ka iwi.
  • Elua hou ole neurofibromas.
  • Hyperpigmentation i loko o ka armpit / groin wahi.
  • Ke alo aku o ka maʻi i loko o pili hoahanau.

No ka nui piha study o na hikoko kauka ana e koho i ka MRI, X-kukuna a me HI scan NineManga.com. Radiograph mea pono, e huai ae hiki aʻe 'o kanaka iwi mīkini.

Loaa i ka lua o kaʻano o ka maʻi hoʻohuʻu ka audiogram. Kēia kūikawā like ana i oaeuiinoe ka oolea o ke hoʻomanawanui i ka hālāwai hoʻolohe manaʻo.

Nānā 'ole o ka' ano o ka maʻi ua waiho ai i nā mea a pau, me ka koe nae, ka ai-i kapaia Ka Ikepili o ka neurofibromatosis. He o ia hoʻi, e iho i ka loa, he pono ole aaiaoe ikea ai, me prenatal. Na hope koho - ka mea, o ka Ikepili o amniotic loli ole chorionic villi.

I ka wa a me ka mea e kapaia e ke kauka

Mua o nā mea a pau ia e hoʻomaopopo i ka iapaau ana o keia maʻi loa pinepine 'ole kekahi mau hoʻomalu. Neurologist kiai opiopio hoomanawanui me ka kamalii, a me ka hope hui ia e nā kauka. Me ua i haiia ae maluna, o ka neurofibromatosis mea i ili mai i ke pili me ka mutation o kekahi chromosomes. Nolaila,'ano'ē lapaʻau a ianoiyuaa a kaumaha aku ai i na kumu e hiki ole. Ma ka hihia o ka hoʻokēʻai-e kahe ana o ka maʻi he mea he pono i loko o kaʻoki lāʻau lapaʻau lapaʻau ina e IIeEeAIEUIeXAIIUE neurofibromas ma ka na nā loko. Neurosurgeons ma ka poʻohiwi, e like me ka rula, i ua kuleana no kaʻeho nodules, i mea no hoi wehe.

He aha e e hana

Hoikehonua, lapaau hiki ole kaumaha'ano'ē ano o ka hana maikaʻiʻole o kēia maʻi. A no ke aha kauka kamaʻilioʻana, e koi 'ia symptomatic Inc. Ua hoʻohuʻu He? Eaia o kekahi pūʻulu o ai '( "Ketotifen", "Fenkarol", "Tigazon") e hoihoi metabolic keʻano o ka hanaʻana i loko o ke kino a me ka hoemi i ka lapaʻau hoailona o ka maʻi e like me neurofibromatosis.

Lapaau ua hoakaka ia a wale i ke kauka ma kekahi kanaka 'aelike, e lawe i ka mooolelo o ka hana i ho'ākāka' hoike ana mai o ka maʻi. Ma ke kauoha o ka Inc ua no hoi ia i loko, no ka hopena o ka aaiaoe Ka Ikepili, i ka hopena o orthopedic, ophthalmology a me ka oncology.

A pinepine he pono ke akeʻoki lāʻau lapaʻau 'uao. He nui e hoailona oukou ia ia, e wehe a pauʻeho ioauoa hiki e loa pohihihi no i ka prevalence kiʻekiʻe o ke kaʻina. Ma kekahi mau hihia, aiiieieoaeuii koi ili pakui. I ka papa kuhikuhiE nāʻikepili no kaʻoki kino i ka mea penei: hanahana loa kaʻeha i loko o ka maikaʻiʻole ia ma ia wahi,ʻoiai aka wahi, malignancy, hoʻohuikau o ka nui nā loko. I mea hoʻonaniʻoki kino e e kohoia ka loa pohihihi keia, e like me ka mea hou aku paha i ka aggravate neurofibromatosis. hoolaʻi Kids lapaau unacceptable.

I kēia manawa, ua ike i ka nui helu o na hihia o keia maʻi, a ia lŘlŘ like me ka hopena o nā maʻi. Ia mea ke kumu ia mea ai nui ole i ka pilikia haʻalele unattended.

hiki complications

Me ua kaulana ma luna, e ole e waiho unattended keiki neurofibromatosis. Lapaʻau e e 'āwīwī a me ka palapala hōʻoia no. I ole ia, he pahiki kiʻekiʻe loa o ka mea pilikia ka heluna o ka 'imi naʻauao a me ka physical ola o ka uʻi hoomanawanui, i kona mea e like ai o ke ola. Poe akamai i kuhikuhi i ke kēia hiki complications:

  • Hypertension.
  • Kāʻili 'ana (mālama i loko o 40% o ka hihia i loko o na keiki me kaʻano mua o ka maʻi).
  • Pepeiao kuli.
  • Olelo ana kāna hana.
  • Scoliosis.
  • Hoʻopōʻino hihio.
  • Hoolohi moe ulu.

Neurofibromatosis i na keiki, na symptoms o i i ua hōʻike luna, pono i ka OAXA paʻa 'inikua hooponopono, Kāohiʻana o complications. kou kauka i waiho aku hoʻopulapula ana ina e pono ai.

Loss o ka lohe ana ma neurofibromatosis lua oʻano ua manaoia e lilo i PAaIEN no ka holo lohe ana e kanu pūʻia kūikawā kekahi. Ka mea noke? Aaeaao nā leo lepa i ka lolo e leie aku e ike kani, a hooiaio olelo i ke keiki.

Kāohi

Eia naʻe, kauka hiki ole a ianoiyuaa a i ka hoʻokō Kāohiʻana o ka maʻi. Ka mea ka mea hope kona mea, lalau mai i ole ua? Anoee. Poe akamai i wale paipai aku i mau kanaka hoʻoponopono i ke ola o ke keiki, mau alakai aku la ia i ka 'inikua examinations.

I ka wā e Alert neurofibromatosis

Ma na keiki, symptoms hōʻike mua i loko o ka palapala mālamalama mea eleele pūkoʻa ia ma luna o kaʻili o. Inā kāu keiki i kēia mau symptoms, ia mea nui, e kukakuka me ka hoʻomalu, e alii ai maluna o mai i kēiaʻike ia.

Inā neurofibromatosis ua mua, ua hookupaa iho la ia, e pono mau kanaka hoʻoponopono i ke ano o ka mea uuku hoomanawanui,ʻeho ho okumu. A hiki aku i ka mea ulu hope ua hoi pono e ninau no ka palapala hōʻoia no kōkua.

Ka mea nui wale nō i loko o keia mea - e huai ae maʻi i kakahiaka nui me hiki. Wahi a ka 'ikepili helu, ma kahi o 60% o nā keiki i kēiaʻike, i kŘpa a mehana hoailona o ka maʻi. No hoi, he nui mai e pono ai kuikawa lapaau.

Pela, e lilo ia i ike i ole e makau ana o na maʻi e like neurofibromatosis i loko o nā keiki ma mua o ka manawa mai. Photos o'ōpiopio nā mea maʻi i maopopo nō ia i kēia pathology ke ola. O ka holo ana, na keiki me ka oi loa ano o ka maʻi i pinepine complications e ala mai oe. Naʻe, a me ka ikaika kōkua a kākoʻo ka mea, ke alakaʻi loa hoʻomāhuahua ai, a me ka hapanui importantly, hauʻoli ke ola.

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