Osteogenesis imperfecta: chii icho, mhando uye kurapwa

Zvemukati

• Mhando huru
• Chii chinokonzera osteogenesis imperfecta
• Zviratidzo zvinogona kuitika
• Maitiro ekusimbisa kuongororwa
• Ndedzipi nzira dzekurapa
• Nzira yekuchengeta vana vane osteogenesis imperfecta

Osteogenesis imperfecta, inozivikanwawo sechirwere chemapfupa egirazi, chirwere chisingawanzoitika chinokonzera kuti munhu ave akaremara, mapfupi uye mapfupa asina kusimba, achinetseka nekugara achitsemuka.

Uku kusimba kunoratidzika kunge kuri nekuda kwehurema hwechibereko hunokanganisa kugadzirwa kwerudzi rwekutanga collagen, iyo inogadzirwa nemaosteoblast uye inobatsira kusimbisa mapfupa nemajoini. Nekudaro, munhu ane osteogenesis imperfecta atozvarwa aine mamiriro, uye anogona kupa zviitiko zvekutsemuka kazhinji muhudiki, semuenzaniso.

Kunyangwe osteogenesis imperfecta ichigere kurapwa, kune mamwe marapiro ayo anobatsira kuvandudza hupenyu hweiye munhu, zvichideredza njodzi uye kuwanda kwekutsemuka.

Mhando huru

Zvinoenderana nesaruro yaSillence, pane mana marudzi eosteogenesis imperfecta, ayo anosanganisira:

• Tora I: ndiyo yakajairika uye yakapusa chimiro chechirwere, ichikonzera kushomeka kana kusavapo kwemapfupa. Zvisinei, mapfupa acho haana kusimba uye anogona kutsemuka zviri nyore;
• Rudzi rwechipiri: ndicho chirwere chakakomba kwazvo chinokonzera kuti "fetus" itsemuke mukati mechibereko chaamai, zvichitungamira pakubvisa nhumbu kazhinji.
• Rudzi rwechitatu: vanhu vane mhando iyi, kazhinji, havakure zvakakwana, zviripo zvino musana uye vachena vemeso vanogona kupa grey ruvara;
• Rudzi IV: iyo iri mhando ine mwero yechirwere, umo mune kushomeka kushoma mumapfupa, asi hapana shanduko yemavara mune chena chikamu chemeso.

Muzviitiko zvakawanda, osteogenesis imperfecta inopfuurira kuvana, asi zviratidzo nekuomarara kwechirwere zvinogona kunge zvakasiyana, nekuti mhando yechirwere ichi inogona kuchinja kubva kuvabereki kuenda kuvana.

Chii chinokonzera osteogenesis imperfecta

Girazi bone bone chirwere chinomuka nekuda kwekuchinja kwemajini mugene inoitisa kuburitsa mhando 1 collagen, puroteni huru inoshandiswa kugadzira mapfupa akasimba.

Sezvo iko kushanduka kwemajini, osteogenesis imperfecta inogona kupfuura kubva kuvabereki ichienda kuvana, asi inogona zvakare kuoneka isina zvimwe zviitiko mumhuri, nekuda kwekuchinja panguva yepamuviri, semuenzaniso.

Zviratidzo zvinogona kuitika

Mukuwedzera pakukonzera shanduko mukuumbwa kwemapfupa, vanhu vane osteogenesis imperfecta vanogona zvakare kuve nezvimwe zviratidzo zvakaita se:

• Mahara akasununguka;
• Mazino asina kusimba;
• Bluish coloration yeye chena yemeso;
• Kukanganisika kusingaite kwemuzongoza (scoliosis);
• Kunzwa kurasikirwa;
• Kakawanda kunetseka kufema;
• Pfupi;
• Inguinal uye umbilical hernias;
• Shanduko yemagetsi mavharuvhu.

Uye zvakare, muvana vane osteogenesis imperfecta, hurema hwemwoyo hunogona zvakare kuongororwa, izvo zvinogona kupedzisira zvave kutyisidzira hupenyu.

Maitiro ekusimbisa kuongororwa

Kuongororwa kweosteogenesis imperfecta, mune dzimwe nguva, kunogona kuitwa kunyangwe panguva yekuzvitakura, chero bedzi paine njodzi huru yekuti mwana azvarwe aine chirwere. Muzviitiko izvi, sampuro inotorwa kubva kuumbilical tambo uko collagen inogadzirwa nemaseru e "fetal" pakati pemasvondo gumi negumi nemaviri ekubata. Imwe nzira isinganetsi ndeyekuita ultrasound kuti ione kutsemuka kwemapfupa.

Mushure mekuzvarwa, kuongororwa kwacho kunogona kuitwa nachiremba wevana kana chiremba wemazino, kuburikidza nekucherechedza zviratidzo, nhoroondo yemhuri kana kuburikidza nemiedzo yakadai seX-ray, kuongororwa kwemajini uye kuongororwa kweropa.

Ndedzipi nzira dzekurapa

Iko hakuna chaiwo marapirwo eosteogenesis imperfecta uye, nekudaro, zvakakosha kuve nehungwaru kubva kuna chiremba wemapfupa. Kazhinji bisphosphonate zvinodhaka zvinoshandiswa kubatsira kuita mapfupa akasimba uye kudzora njodzi yekutyoka. Nekudaro, zvakakosha zvakanyanya kuti mhando iyi yekurapa irambe ichiongororwa nachiremba, sezvo zvingave zvichidikanwa kugadzirisa madosi ekurapa nekufamba kwenguva.

Kana kutsemuka kukamuka, chiremba anogona kudzikamisa pfupa nechisimbi kana kusarudza kuvhiya, kunyanya mune zvakaputsika kana kuti zvinotora nguva yakareba kuti zvipore. Kurapa kwekutsemuka kwakafanana neyavanhu vasina mamiriro, asi iyo nguva yekudzikisira inowanzo ipfupi.

Physiotherapy yeosteogenesis imperfecta inogona zvakare kushandiswa mune dzimwe nguva kubatsira kusimbisa mapfupa uye mhasuru dzinozvitsigira, kudzikisa njodzi yekutyoka.

Nzira yekuchengeta vana vane osteogenesis imperfecta

Dzimwe nzira dzekuchengetedza vana vane osteogenesis isina kukwana ndeiyi:

• Dzivisa kusimudza mwana nemahapwa, kutsigira huremu neruoko rumwe pasi pebute uye rwumwe kuseri kwemutsipa nemapendekete;
• Usadhonze mwana neruoko kana gumbo;
• Sarudza chigaro chechengetedzo chine padding rakapfava iro rinotendera kuti mwana abviswe uye aiswe nekuedza kushoma.

Vamwe vana vane osteogenesis isina kukwana vanogona kuita zvishoma kurovedza muviri, sekushambira, sezvo ivo vachibatsira kudzikisa njodzi yekutyoka. Nekudaro, ivo vanofanirwa chete kuzviita mushure mekurairwa nachiremba uye pasi pekutarisirwa kwemudzidzisi wezvepanyama kana chiremba wepanyama.