Klippel - Feil ciwo: photo, magani, abin da sakamakon zai yi? Sprengel ciwo - kasa - Klippel: darussan a waha

Biyu Faransa neurologist Andre Feil da Moris Klippel a 1912, aka bayyana a cikin daki-daki, nakasar nakasar a cikin mahaifa-thoracic kashin baya a yara. Dukan su aka haifa a cikin iyaye na daya jini aboki. A tsakiyar zamanai, doka maza iya zama ko da dangi. Saboda haka, Yaduwar wannan anomaly ne ba abin mamaki ba. Daga baya Pathology aka mai suna bayan da masana kimiyya. Klippel - Feil ciwo ne ba wata cuta da cikakken. Yana da wani nakasar da cutar, wanda sau da yawa take kaiwa zuwa sauran kashin baya pathologies.

Features da cutar

Karkashin ciwo Klippel - Feil tana nufin nakasar cuta na mahaifa kashin baya, kunsha a synostosis da kuma rage yawan vertebrae. An halin a high mita na bayyanuwar - daya hali ta 120 000 kowacce haihuwa. A mafi hankula siffa daga cikin cutar da aka dauke su wani alama raguwa a tsawon na wuyansa. A mafi yawan lokuta, tare da sauran munanan na musculoskeletal tsarin da nakasar munanan na ciki gabobin. A ganewar asali na cutar da ake samu halartar da dama da kwararru :. ilimin tsarin jijiyoyi, orthopedics, halittar jini, da dai sauransu Conservative magani hada da jiki far, physiotherapy kuma tausa. A musamman mai tsanani lokuta bukatar tiyata - tiyata tservikalizatsii.

Babban Sanadin malformations

Klippel - Feil ciwo tana nufin wani genetically m cututtuka. Pathological canje-canje a cikin jiki fara faruwa ne a farkon makonni bayan ganewa. Daga cikin manyan dalilan da likitoci ciwo lura da wani na cin gaba cuta daga cikin kashin baya da kuma rabawa, musamman a verhnesheykovom matakin. Kafa synostosis thoracic da mahaifa vertebrae, Munã rage adadin su, nonunion jikinsu da kuma arches ƙyale mu mu sanin ko na asibiti hoto da cutar. A hadarin kungiyar hada da yara tare da ya fi karfinsu gadar hali, inda a can ne:

1. A kayyade aibi a cikin chromosomes. A wani rashin lafiya yaro ne mai take hakkin da samuwar bambantawa na girma wajibi ne ga cikakken ci gaban da kwarangwal. Wannan ne babu makawa nuna a cikin ci gaban thoracic da mahaifa vertebrae sassan.
2. Autosomal rinjaye juna na gādo. Idan iyaye daya tana da cuta, yiwuwar samun ciwon baby da munanan ne 50-100%.
3. Autosomal recessive gādo. A wannan yanayin, yiwuwar samun ciwon yaro da munanan na 0-50%.

Don kauce wa ya fi karfinsu wasiyya iyaye ya kamata shawarci mai jini a mataki na shiryawa da yaro.

Hankula cututtuka na ciwo

Wannan cuta ne halin da classic triad na bayyanar cututtuka: wuce kima short wuyansa, wani canji a hairline, iyaka motsi na shugaban. The rashin lafiya ne yawanci a hade tare da sauran pathologies. A 30% da marasa lafiya saukar scoliosis, torticollis m form, high tsaye ruwan wukake - Sprengel cuta. A wasu lokuta da lura munanan daga cikin manya wata gabar jiki, feet nakasawa, hakora, fuska jeri na bangaren, hyperopia. A 25% da marasa lafiya bincikar lafiya tare da nakasar wani nauyi.

Klippel ciwo - kasa - Sprengel - ba kawai ya nuna kwaskwarima lahani. Yana iya kuma sun hada da neurological bayyanar cututtuka. Canje-canje a cikin CNS bayyana shafi tunanin mutum retardation, hydrocephalus, epilepsy. Daga wani wuri shekaru da mãsu haƙuri yana da tsoka rauni a cikin extremities, kazalika da synkinesis. A mazan shekaru daban-daban, da sauran asibiti hoto ne suna goyon bayan da sakandare canje-canje na kashin baya.

Medical jarrabawa

Biciken da cutar za a iya dogara ne a kan ya lura triad na cututtuka, jiki da jarrabawa data da kuma instrumental Nazarin. A musamman rawa a cikin ganewar asali da aka ba wa nazarin iyali tarihi na haƙuri. Saita Klippel - Feil ciwo tare da wani cikakken bayanin rakiyar anomalies yiwu a sakamakon hadewa aikin kwararru. Kamar yadda mai mulkin, shi neurologists, jinsi, orthopedists, cardiologists da pulmonologists.

Don tantance yanayin da canje-canje sanya vertebral radiography. A binciken da aka gudanar a biyu tsinkaya, na abin da ya fi m yawanci ne a kaikaice. Saboda uncharacteristic matsayi na da inuwa daga cikin kwanyar da shugaban masu hada kai tare da image na kashin baya shafi, sa shi wuya a gano abubuwa. Bugu da ƙari, shi ne shawarar ka dauki hotuna a iyakar lankwashewa / tsawo na wuyansa. Wannan damar domin sanin yiwu rashin zaman lafiya ununited vertebrae da kuma tabbatar da ciwo Klippel - Feil. X-haskoki na mahaifa-thoracic kashin baya taimaka wajen gano:

• maras kyau vertebrae.
• rage a cikin adadin su.
• Fusion na vertebral jikinsu.
• mahaukaci tsaye ruwan wukake.
• curvature daga cikin kashin baya.

Bayan gaskatãwa ga ganewar asali na marasa lafiya bugu da žari nada duban dan tayi na ciki gabobin don gane anomalies a cikin tsarin. A gaban neurological munanan iya bukatar tasoshin Doppler duban dan tayi, angiography, EEG da MRI na mahaifa kashin baya. Shi ne wajibi a yi shawarci likitan-jini. A cewar ta sakamakon da gwani da zai iya sanin ko yanayin na gado, da kuma tantance da kasada to nan gaba.

Ainihin nau'i na cutar

Klippel ciwo - Feil ne mai matukar rare cuta. Bisa kididdigar da, da hankula bayyanar cututtuka suna kamu a daya yaro daga 120 dubu. A anomaly bayyana a cikin wani haƙuri a daya daga uku siffofin:

• Rage yawan segments a cikin mahaifa kashin baya, wanda a hankali ya fis da kuma gani rage wuyansa. Wannan nau'i na Pathology Yanã matsaloli a lokacin da motsi kai.
• Synostosis mahaifa kashin baya Fusion a bango tare da occipital kashi. A haƙuri ba zai iya juya kansa ba tare da jin zafi. Mahaifa vertebrae kuma wuyansa ne daya.
• The uku nau'i na cutar bayyanuwar hada da na farko guda biyu.

A musamman bambance-bambancen da ciwo za a iya ƙaddara kawai likita a kan tushen da binciken sakamakon.

Ka'idodi na magani

Abin baƙin ciki, zamani magani ba zai iya bayar da isassun hanyoyin da yadda za a gaba daya kawar da ciwo na Klippel - Feil. Jiyya ya shafi hana ci gaban sakandare musakai. Dauka a matsayin tushen ra'ayin mazan jiya hanyoyin, wanda sun hada da jiki far da kuma tausa. Drug aka ba da jiyya a cikin taron cewa cutar tsari ne tare da zafi da rashin jin daɗi matsawa na jijiya asalinsu. Kamar yadda mai mulkin, amfani analgesics, anti-kumburi da kuma wadanda ba steroidal kwayoyi. Tsawo matsawa na jijiya Tushen bukatar tiyata. Babban manufar da aiki - domin kawar da zafi partially daidai waje lahani.

Jiki far da kuma darussan a cikin pool

Conservative magani na ciwo hada da jiki far Hakika, warkewa tausa na mahaifa kashin baya da kuma abin da ake kira wuyansa yankin. Darussan aka zaba akayi daban-daban, dangane da asibiti hoto na haƙuri. A karo na farko, da suka kamata a da za'ayi a karkashin kulawa da wani gwani, za ka iya sa'an nan matsa zuwa gida harkarsa. Marasa lafiya tare da ganewar asali "Sprengel ciwo - kasa - Klippel" pool bada taimaka wa kauce wa mai rikitarwa hanya da cuta. Matsayin mai mulkin, matasa marasa lafiya, likitoci da shawarar ninkayar. More hadaddun darussan ma zabi akayi daban-daban. Universal shawarwari game da iyo gymnastics ba ya wanzu.

m magani

M zafi lalacewa ta hanyar jijiya tushen matsawa shi ne na farko nuni ga tiyata. Marasa lafiya tare da ganewar asali "ciwo Klippel - Feil" hangen nesa worsens da shekaru. Saboda haka, aiki da ake sa nan da nan bayan da tabbatarwa daga Pathology. Don kara da motsi na da wuyansa da ake amfani da tservikalizatsiya Bonola. A lokacin hanya, likita kawar saman hudu hakarkarinsa da periosteum domin rage matsa lamba a kan kayan ciki. The aiki aka yi a dama, saukarwa. Da farko cire hakarkarinsa a gefe daya da kuma, bayan dawo da na jiki - zuwa wani.

Fi lokaci na bukatar lokaci mai yawa, kuma kokarin. Watanni da dama, da mãsu haƙuri ya kamata a sauran. Duk da haka, ba up aiki ba lallai ba ne. A halin yanzu shi ne kawai tasiri hanyar daidaita da waje lahani, da ya faru na wanda triggers da ciwo Klippel - Feil. Mene ne sakamakon da kiwon lafiya, idan muka bari da magani, ya bayyana a kasa.

Matsalolin da kuma hangen nesa ga mãsu haƙuri

Bayan aikin tiyata da na fi lokacin da haƙuri iya kai cikakken rai. The wuyansa ne kadan a lokacin wannan lokaci karuwa a tsawon, wanda, daga wani ado ra'ayi, aka dauke m al'amari. Idan haƙuri watsi da likita ta shawara ga maida, jiki zai fara zuwa "mayar da martani" rikitarwa. Da farko ɓullo da Pathology na ciki gabobin, akwai karfi sha raɗaɗin a cikin wuyansa. Past saboda da m ƙeta na jijiya tushen da zai iya kai ga kammala obezdvizhevaniyu wata gabar jiki. Visceral hadarin komowar pathological tafiyar matakai, da kuma iya zama wani dalili na farkon mutuwa.

m matakan

Shin yana yiwuwa a hana ciwo na Klippel - Feil? Photo da marasa lafiya tare da wannan ganewar asali tilasta tunani game da wannan tambaya. Specific rigakafin wannan cuta ba ya wanzu, tun da shi ne hereditary. A cikin iyalai inda lokuta da irin wannan anomalies da aka rubuta, wajibi ne ga kwayoyin shawara. Its ainihi tafasa saukar zuwa nazarin ma'auratan shirya domin rawar da iyaye, domin tantance hadarin cutar.

don takaita

Klippel - Feil ciwo ne mai nakasar anomaly daga cikin manya kashin baya. An halin a triad na waje da ãyõyinMu, kuma da dama daga radiological fasali. A cuta yana da wani m m hangen nesa, amma in warkar da shi gaba daya ba zai yiwu ba. Dace m baki don gyara kawai damar waje flaws suna samuwa. Idan ka yi a baya, an tabbatar da wani makusancin zumunta ne daga irin wannan ganewar asali, shi ne bu mai kyau zuwa sha cikakken kayyade nunawa kafin conceiving yaro. Zauna lafiya!