Crystal chirwere: zvinokonzera, photo, upenyu anotarisirwa

Crystal chirwere haiwanzowaniki nemagene pathologies umo mune mhirizhonga munyika nemamiriro pave zvetsandanyama nemapfupa ezvinhu, iyo inotungamirira yakakwirira fragility mapfupa. In mushonga, chirwere ichi kunzi osteogenesis imperfecta. Chii chinonzi chirwere ichi? Ndezvipi zviratidzo uye zviratidzo nehosha, chii kurapwa dziripo? Ngatiedzei kupindura mibvunzo yose iyi.

Chii osteogenesis imperfecta?

Crystal mapfupa chirwere - nhaka chirwere. In chirwere ichi, mapfupa munyama kuva kusimba zvikuru, nokuda uyo murwere dzevanhu mitswe zvinowanzoitika, izvo inomuka kuchangotanga kwechirwere, kunyange pasina chinhu mupfungwa. Varwere chirwere ichi kazhinji ngationgororei dzimwe kuratidzwa pathological dziri:

• tsandanyama atrophy;
• mazino abnormalities;
• hypermobility of mapfundo;
• ari muchimiro mberi kusanzwa;
• mapfupa deformation.

Siyana mabhuku osteogenesis imperfecta chirwere ane mazita akawanda:

• fetus kusimba;
• osteomalacia chokuziva;
• Lobstein nehosha;
• periosteal dystrophy;
• mapfupa pfava, kana sekristaro kurwara.

VaBennett chirwere ichi vari midzi muna majini kuchinja kutungamirira nokuvhiringidzirwa mapfupa inotungamirwa. Muvanhu, ipapo Generalized osteoporosis, uye mapfupa ave haungatani kuputsika.

The chirwere chaizvo hazviwanzoitiki. Maererano nenhamba, inowanikwa muna 1 mwana kubva 10-20 zviuru vana vakaberekwa. Maperembudzi hachirapiki, kunyange zvazvo mushonga ano, pane nzira dzakawanda kuvandudza murwere mamiriro uye kuti upenyu nyore vana vane majini chirwere.

Nei chirwere

Doctors vanoti vaviri zvinokonzera chikuru chirwere:

1. Mapfupa Gene kunotouraya kuitike, ndokusaka pane kushayikwa puroteni collagen, inova connective tishu. Muna 5% nyaya mhando nhaka chirwere chacho autosomal kuzivikanwa uye autosomal recessive.
2. Chikonzero chirwere muri kwatangwa kunotouraya. Uwandu collagen hazvisi zvechokwadi, asi puroteni usage pane shanduko ayo mamiriro. Mapfupa anogona kukura anowanzoita, asi pane mhirizhonga pamusoro Ossificação em rinenge akaderedza zvemaziso ehupfumi, pane fragility yavo.

Chirwere "Crystal mapfupa" ari nemagene zvokurwara, saka kakawanda richiwanika nevana, vane hama dzine chirwere chete. Asi pane dzimwe nguva vari apo mwana zviratidzwa osteogenesis imperfecta, ukuwo vabereki vari zvachose utano. Chimiro zvokurwara iri chokuita panowanikwa kwatangwa kunotouraya.

Kune mhando dzakawanda nezvirwere izvo kuburikidza zviratidzo siyana uye kukura mapfupa mamiriro inozivikanwa. A uchida Kutarira padanho rimwe nerimwe chirwere.

simba chimiro

About 50% varwere osteogenesis imperfecta vane mhando chirwere ichi chokutanga. chirwere Crystal muna vana (mufananidzo munyaya) achabatsirwa kakawanda mitswe, asi kusvika makore 10 pangozi akakuvadza pfupa rinenge ipfupi. Vanhu makore 40 okuberekwa vanofanira kungwarira, sezvo dambudziko mapfupa fragility kunowedzera zvakare.

Aine simba chimiro chirwere kazhinji vakachengeta ropa pamusoro nasal mhango, ichi imhaka vamwe kuchinja yeropa.

Perinatal-inouraya chimiro

Kazhinji, mhando osteogenesis imperfecta wechipiri inotungamirira kurufu mwana ari mudumbu. The chirwere chinogona kukonzera vakurumidze kuberekwa ari tanga gestation.

Ichi chimiro chirwere inokamukana muzvikwata zvitatu, chimwe nechimwe kunoratidzwa mamwe maitiro:

• Boka rokutanga (A). The dehenya Mapfupa akakuvadzwa kunyange mudumbu, iyo inogona tsunga nokuisa mukadzi anepamuviri Ultrasound. Mwana aizozvarwa pamwe zvokurwara iri kukura kwete kupfuura 20-30 cm. The uropi basa uye kufema mugadziriro runovhuniwa. With chirwere ichi vana vanozvarwa vakafa kana akararama mazuva mashomanana. zviitiko zvakanyorwa apo "sekristaro" mwana akafa mwedzi pashure pokuberekwa. Chinokonzera rufu vari vakawanda mitswe.
• Boka rechipiri (B). Zviratidzo chirwere akafanana kuratidzirwa mukirasi (A), asi kufema gadziriro iri kushanda zvakanaka kana ane zvishoma kutsauka. Varwere vose mapfupa tubular vari pfupi, saka zvinoita sokuti sekristaro kurwara. Upenyu hunotarisirwa - makore mashomanana pashure pokuberekwa.
• The boka rechitatu (B) - unowanikwa zvisingawanzoitiki zviitiko. Fetus kana kufa mudumbu kana sezvo vachangoberekwa kufa mumazuva mashomanana pashure pokuberekwa. Dehenya haina dzakabudirira uye asina Ossificação em, tubular mapfupa rive dete.

Kukura mapfupa kuputswa

Wechitatu mhando ine maitiro chepfungwa - kukura mapfupa akavhuniwa. Izvi Chirwere kushoma chaizvo. Pasinei chokwadi kuti achangoberekwa miduku, ayo uremu kunogona dzinoenderana vasingambooni chakaipa nazvo. In varwere osteogenesis imperfecta ichi mhando vanowanzotambura rokuongorora nezvinetso kweropa maitiro. dambudziko iri anova chinokonzera rufu. In vacheche pamwe zvemamiriro kukura repfupa mitswe zvinowanzoitika pakusununguka.

skeletal kukura wakaputsika

Kana wechina mhando chirwere pane zvakakomba kuvhiringidzika kukura uye nemamiriro marangwanda. Kwemakore anoverengeka, murwere pamusoro Mapfupa kuumbwa callus, uye ngozi kutsemuka ipfupi. By zera 30 murwere vanogona kunzwa kurasikirwa.

Kuziva mhando chirwere uye boka vanofanira kuenda kuburikidza chose zvakawanda chirwere matanho. Shandisa ose nzira panongovira mwana akaberekwa.

nzira nechirwere

Crystal chirwere (mapikicha dzinoturwa munyaya) inogona chirwere ari mudumbu mudumbu. Dzidza pamusoro huripo zvokurwara chingava kwemutemo Ultrasound. Kana paine fungidziro kuti hadzina kufambiswa zvakanaka vari kwetunhu collagen, inoda okuwedzera kudzidza. Kuti aite izvi, vakadzi vane pamuviri vari pamusoro kuongorora Líquido mvura uye epithelial tishu.

Panongotanga mwana akaberekwa, zvakakodzera kuitisa misoro kuongororwa, izvo zvinosanganisira:

• pfupa biopsy;
• X-ray kuziva mitswe;
• densitometry (Kudzidza zvicherwa Zvinoumba pfupa mhango);
• kuongororwa ropa kuti rione zvachinja DNA;
• collagen kuongorora chirwere;
• mumwe biopsy pakati Epiderme e.

The vakawana zvabuda kubvumira vanachiremba kusarudza zvinobudirira kuchirapa zvaiitwa.

Ko chirwere "Crystal mapfupa"?

Osteogenesis imperfecta anoonekwa kuti hosha isingarapiki, asi achibatsirwa kunobudirira kurapa kunogona kuderedza murwere mamiriro uye kusimbisa ayo skeletal hurongwa.

In yawapiwa inofanira kusanganisirwa:

• magnesium uye potassium munyu;
• karusiyamu;
• vhitamini D nezvimwe zvinovaka muviri.

Zvinokurudzirwa kushandisa muviri kurapa uye physiotherapy.

Vabereki mwana abnormalities yakadaro inofanira kumbotambudzika psychotherapy, apo anotsanangura sei kuti zvakakodzera kudzidzisa mwana uye dzienderane munzanga muvanhu. Izvi zvichaita kuti mwana wako kuti adzidze mune ramangwana kudzivisa ngozi apo mukuru mukana munhu kutsemuka.

Kukura chirwere yakadaro usingagoni kudziviswa. Asi nhasi pane mazhinji nzira kurapwa, kutevera izvo unogona kuvandudza utano mwana wako. Vana vane chirwere dzakaonekwa rekristaro, havana abnormalities ari mupfungwa uye mupfungwa kukura, saka unogona akaziva muupenyu, zvinokosha chete kuti vabatsire.