Klippel - Feil maʻi pale: Hawaii photo, lapaau, mea i ka hopena paha ia? Sprengel maʻi pale - pau - Klippel: kino i loko o ka 'auʻau wai

Elua Palani neurologist Andre Feil a me Moris Klippel i ka 1912, hōʻike ma ka au mamuli congenital deformities i loko o ka cervical-thoracic spine i loko o nā keiki. A pau o ia i hanau i na makua o kekahi koko ana. Ma ka Middle Ages, loio i noho male ke lilo i hoahanau. No laila, i ka prevalence o keia anomaly mea e kahaha ko oukou naau. Hope pathology Ua kapaʻia ma hope o nā kānakaʻepekema. Klippel - Feil maʻi pale mea,ʻaʻole he maʻi loa. He He congenital maʻi, a pinepine hiki aku ai i nā spinal pathologies.

Hiʻona o ka maʻi

Ma lalo maʻi pale Klippel - Feil pili i congenital kāna hana o ka cervical spine, iloko olaila ma synostosis a me ka hoemi i ka helu o vertebrae. Ua Ua wehewehe aku ma ke alapine (frequency) kiʻekiʻe o ka hoike ana - kekahi hihia no 120 000 hanau. Ka loa typical hiʻona o ka maʻi ua manaoia e ia i na hiohiona ka ho'ēmiʻana i loko o ka lōʻihi o ka'ā'ī. Ma ka hapanui o hoopii, pu ma nā abnormalities o ka musculoskeletal'ōnaehana a me congenital abnormalities o ka na nā loko. Ma kaʻike, o ka mea maʻi ua hele mau hoʻomalu :. Neurology, orthopedics, aaiaoe?, Etc. makeʻe kulana lapaʻau kikowaena physical Inc, physiotherapy a me ka lomilomi. I oi loa hoopii e noi mai anaʻoki kino -ʻoki kino tservikalizatsii.

I ka papa kuhikuhiE kumu o malformations

Klippel - Feil maʻi pale pili i ka genetically manaʻo maʻi. Pathological loli i loko o ke kino hoʻomaka e ana i loko o ka pule mua ma hope o hapai ana. Mawaena o ke kumu nui kauka maʻi pale kaulana i ka developmental kāna hana o ka spine a me ka segmentation, o ka oi aku ma ka verhnesheykovomʻilikai. Hana synostosis thoracic a me cervical vertebrae, hoemi i ko lakou helu, nonunion kino a me kona mau hoaka ae mai, e hooholo i ka lapaʻau kiʻi o ka maʻi. Ke kamepiula pūʻulu nā keiki a me nā kūpuna mai noho lakou malaila, ma laila mea:

1. Ke aaiaoe hewa i loko o ka chromosomes. Ma kekahi maʻi keiki o ka 'aʻe' ana i ke kahua o ka differentiation o ka ulu pono no ka ulu kūpono 'ana o ka iwi. Keia ua inevitably lewa i loko o ka ulu ana o ka vertebrae thoracic a me ka cervical oihana.
2. Autosomal Hoʻohaku kumu o ka'āina hoʻoili. Inā kekahi makua i ka maʻi, i ka pahiki o ka ke keiki me abnormalities mea 50-100%.
3. Autosomal recessive hooilina. Ma keia hihia, no ka pahiki o ka ke keiki me abnormalities o 0-50%.

E pale i ka noho lakou malaila hoʻoilina mau makua, e nīnau aku i ka geneticist ma ke kahua o ka hoʻoneʻe o ke keiki.

Typical symptoms o ka maʻi pale

Kēia maʻi ua wehewehe aku ma ka Classic triad o symptoms: excessive pōkole'ā'ī, he loli ma ka hairline, i kaupalena 'ia ka hoʻoneʻe o ke poo. I ka maʻi ua ana hui me nā pathologies. I ka 30% o nā mea maʻi hoikeia mai scoliosis, torticollisʻoʻoleʻa palapala, kiʻekiʻe kū kōmi '- Sprengel maʻi. Ma kekahi hihia i ka malama abnormalities o ke keena lala, na wawae deformation, niho, lehalehu t asymmetry, hyperopia. I ka 25% o nā mea maʻi diagnosed me congenital pepeiao kuli.

Klippel maʻi pale - pau - Sprengel - ua ole i olelo ia wale mea hoʻonani kina. Ua hiki no hoi loaʻa neurological symptoms. Loli ma ka CNS hoike mai ai ia noʻonoʻo retardation, hydrocephalus, epilepsy. Mai kekahi kakahiaka nui elemakule i ka hoʻomanawanui i Muscle nawaliwali ma kaʻaoʻao, e like me ka pono me synkinesis. Ma mua makahiki, i ka pono lapaʻau kiʻi ua inā e kiʻekiʻe loli o ka spine.

Lapaʻau o keia hookolokolo ana

Ka hōʻoia 'ana i ka maʻi hiki ke nānā' ana i ae nānā mau triad o symptoms, physical hookolokoloʻikepili a me vocal examinations. A kūikawā kūlana i loko o kaʻike, i haawiia mai ai i ke ao o ka ohana mōʻaukala o ka hoʻomanawanui. E hoʻopaʻa i Klippel - Feil maʻi pale me ka piha ano o ka hele pū ana anomalies hiki i ka hopena o ka wahi hana o ka hoʻomalu. E like me ka rula, ka mea neurologists, geneticists, orthopedists, cardiologists a me pulmonologists.

No ka Loiloi i ka ano o ka loli ia vertebral radiography. Ke kuka nei lakou i mālama 'ia i loko o nā wānana, a i ka loa informative nō kāu mea kīloi paʻewa. No ka mea, o ka mea uncharacteristic kulana o ke aka o ka puniu wale no o ua kūponoʻia me ke kiʻi o ka spinal kolamu i ke poo, e ka mea paakiki e kuhikuhi 'ikamu. Eia kekahi, ka mea, ua? Aeiiaiaoaony e lawe nā kiʻi ma kā mākou flexion / palena manawa no ka ai. Ua apono 'e hooholo i ka hiki instability ununited vertebrae, a hōʻoia maʻi pale Klippel - Feil. X-kukuna o cervical-thoracic spine kōkua i ka hōʻike:

• ino vertebrae;
• emi iho i loko o lākou ka helu;
• kuʻi nukeliu o ka vertebral mau kino;
• 'āno'ē e ku ana kōmi 'ana;
• curvature o ka spine.

Ma hope e hookupaa aku ana i kaʻike, a me ka hoʻomanawanui iki mai aiiieieoaeuii kohoia ultrasound o na nā loko, e huai ae anomalies i loko o kā lākou 'ole. Ma ke alo o neurological abnormalities i koi na moku Doppler ultrasound, angiography, EEG a me MRI o ka cervical spine. He muli o ke nīnau aku i kekahi kauka-geneticist. E like me kona hualoaʻa i ke akamai, e e hiki ke hooholo i keʻano o ka hoʻoilina, a me ka Loiloi i ka ka pilikia a hiki i na hanauna e hiki.

Ka walaʻauʻana ano o ka maʻi

Klippel maʻi pale - Feil mea he nui loa pohihihi keia maʻi. Wahi a ka 'ikepili helu, e diagnosed ka typical symptoms i loko o kekahi keiki mai o 120 tausani. Ke anomaly ikeia ma ka hoomanawanui i loko o kekahi o ekolu 'ano apau loa.

• Hoemi i ka helu ana o Hoʻohana i loko o ka cervical spine, a i nā kaula hō'ā a visually hoʻopōkole 'i ka'ā'ī. Keia ano o ka pathology ke kumu pilikia ka wā e neʻe poo.
• Synostosis cervical spine kuʻi nukeliu i loko o ke kāʻei kua me ka occipital iwi. Ke hoomanawanui hiki ole huli i kona poo me ka eha. Cervical vertebrae a me ka'ā'ī i kekahi.
• Ke kolu o ka palapala o ka maʻi hoike ana nā ka mua elua.

A mau Lolina o ka maʻi pale hiki ke nānā 'wale kauka ma ke kumu o ke anamanaʻo i nā hualoaʻa.

Walaʻauʻana me nā loina o ka lapaau

Eia naʻe, kēia lapaʻau hiki ole kaumaha kūpono ano o ana i ka loa hoʻopau i ka maʻi pale o Klippel - Feil. Lapaau komo ai hikiʻole i ka hooulu ana o nā haumāna deformities. Ia me he kumu makeʻe kulana ano, i nā physical Inc a me ka lomilomi. ua haawiia mai Uluwehi O lapaau i loko o ka hanana i ka maʻi kaʻina ua pu ma kaʻeha a me ka discomfort mīkini'opihia o nā aʻalolo aa. E like me ka rula, hoʻohana analgesics, Anati-hoʻoʻino a me ka 'ole-steroidal ai'. Hooloihi ae i mīkini'opihia o ke aʻalolo aʻaʻimi maiʻoki kino. I ka papa kuhikuhiE manao o ka hana - e hoʻopau ka eha hapa 'ia' pololei mawaho mā.

Helu Inc a me ka hoʻokō 'ana i loko o ka wai ÿauÿau

Makeʻe kulana lapaau o ka maʻi pale nā physical Inc papa, pākōlea lomilomi o ka cervical spine, a me ka ai-kapaia'ā'ī wahi. Kino i wae pākahi, ma muli o ka lapaʻau kiʻi o ka hoʻomanawanui. Ka manawa mua, ka mea e halihaliia oia iwaho ma lalo o ka hooponopono ana o ka hoʻomalu, e hiki laila, neʻe ma ia wahi lulu hauna e. Hoʻomanawanui iki mai i kaʻike, o "Sprengel maʻi pale - pau - Klippel" wai kino kōkua i loaʻa i ka huikau papa o ka maʻi. E like me ka rula, ui nā mea maʻi, kauka waiho aku 'auʻau. More eiiieaena kino i wae pākahi. Universal koi e pili ana i ka 'auʻau' gymnastics hakuʻole.

ʻoki lāʻau lapaʻau lapaʻau

Paakiki ka eha, kena ae la ia e nā aʻalolo kumu mīkini'opihia o ka iniiaiie PAaIEN no kaʻoki kino. Hoʻomanawanui iki mai i kaʻike, o ka "maʻi pale Klippel - Feil" prognosis worsens me ka elemakule. No laila, i ka hana ua lanakila koke ma hope o ka hōʻoiaʻiʻo o pathology. E hoomahuahua i ka ka hoʻoneʻe o ka ai ua hoʻohana 'ia no ka tservikalizatsiya Bonola. Iloko o ke kaʻina hana, ke kauka e lawe aku oe i ka luna eha iwi aoao a me ka periosteum i mea e emi ka puʻe ma luna o ka mea na nā loko. Ke hana ua hana ia ma kekahi mau kahua o ka hoʻonaʻauao. Initially wehe iwi aoao ma kekahiʻaoʻao, a, ma hope o ke ola ana o ke kino - i kekahi.

Hoʻopulapula manawa i pono i ka puu o ka manawa a me ka hana. No kekahi mau mahina, i ka hoʻomanawanui e ia i maha. Eia naʻe, e haawi aku i ka hoohana ia mea,ʻaʻole pono. Currently ka mea, o ka mea wale nō ka hoʻokō ala e hoʻomaʻa i ka mawaho mā, ka hanana a i triggers ka maʻi pale Klippel - Feil. He aha ka hopena no ke ola, ina o ka iapaau ana mākou haalele, ho'ākāka 'ia ma lalo nei.

Complications a me prognosis no ka hoʻomanawanui

Ma hope oʻoki kino a me ka hoʻopulapula au i ka hoʻomanawanui ke alakai i ka piha ola. I ka'ā'ī o kona wahi iloko o keia manawa māhuahua ka lōʻihi, a, mai ka aesthetic wahi o ke hoomaopopo, ua noʻonoʻo maikaʻi kēia lākiō. Inā ka hoʻomanawanui ignores ke kauka i ka'ōlelo aʻo no ke ola, i ke kino hoʻomaka e "pane" complications. Kuhikuhi ulu pathology o na nā loko, eia ikaika eha ma ka'ā'ī. Hala aie i ka mea a eia iʻaihue o ke aʻalolo aa, a hiki ke alakai i ke hoʻokō 'obezdvizhevaniyu lala. Visceral kamepiula o irreversible pathological keʻano o ka hanaʻana, a ua hiki ia i ke kumu o ka wanaʻao make.

kāohi ana

Ua mea hiki ke pale aku i ka maʻi pale o Klippel - Feil? Photo o nā mea maʻi i kēiaʻike, puʻe wale akula kēlā i ka manaʻo, no keia manao. I Kekahi Mau Kāohiʻana o kēia maʻi hakuʻole, no ka mea, mea hereditary. Ma nāʻohana kahi hihia i like anomalies i ua palapala ia, ia mea e pono ai aaiaoe aʻoaʻo 'ia. Kona o Mānoa, maʻi hēhē ia e ana i ka hui i ka hoomakaukau ana no ke kūlana o ka makua, e Loiloi i ka pilikia o ka maʻi.

e hōʻuluʻulu

Klippel - Feil maʻi pale mea he congenital anomaly o ke keena spine. Ua Ua wehewehe aku ma ka triad o mawaho na hoailona, a me ka helu o radiological hiʻona. I ka maʻi i ka 'oluʻolu nui prognosis, akā, e ho'ōla ia pau ole. 'Āwīwīʻoki lāʻau lapaʻau' uao e paʻipaʻi wale e leie aku mawaho flaws i loaʻa. Inā 'oe i mua, ua hookupaa iho la ia e ka hoahanau pili no ia i kaʻike,, ia mea kupono e undergo i ka piha aaiaoe loiloi mua i kō ai ke keiki. E hoʻokaʻaʻike ola!