Osteogenesis Imperfecta (OI), yomwe nthawi zambiri imatchedwa brittle bone matenda, ndi matenda omwe amachititsa chilema kupanga collagen mapuloteni. Collagen ndi puloteni yofunikira yomwe imathandiza kuthandizira thupi; ganizirani za mthunzi umene thupi limamangidwa. Pali mitundu yambiri ya collagen, yomwe yambiri imakhala mtundu wa collagen.
Ikani mtundu wa collageni umapezeka mu fupa, phokoso la diso, mitsempha, ndi mano.
Osteogenesis imperfecta imapezeka pamene pali vuto la mtundu wa collagen kupanga.
Kodi mumalandira bwanji Osteogenesis Imperfecta?
Osteogenesis imperfecta amachokera ku jini yomwe imayambitsa thupi kupanga collagen. Osteogenesis imperfecta ikhoza kuperekedwa kuchokera kwa kholo lomwe liri ndi matendawa, kapena ilo lingakhoze kuchitika monga kusintha kwadzidzidzi kwa mwana. Ambiri mwa osteogenesis imperfecta omwe amachokera kwa makolo amayamba kusintha kwambiri , omwe amachokera kwa kholo limodzi amene ali ndi osteogenesis imperfecta, ngakhale kuti angapo amapezeka chifukwa cha kusintha kwa thupi komanso amachokera ku jini kuchokera kwa makolo onse, komanso alibe matenda.
Zizindikiro ndi Zizindikiro
Mavuto omwe amapezeka kwa odwala omwe ali ndi osteogenesis imperfecta amaphatikizapo fupa, fupa, scoliosis, kuchepa kwa dzino, kumva kuchepa, bluish sclera, ndi mitsempha yosayera . Osteogenesis imperfecta imakhala ndi dzina lofala kwambiri, matenda otupa mafupa chifukwa ana awa amapezeka kuti atakhala ndi mafupa angapo osweka .
Zizindikiro za osteogenesis imperfecta zimasiyana kwambiri pakati pa anthu. Ana ena amapezeka kuti ali atangoyamba kumene, pomwe ena angakhale ovuta kuti azindikire momwe zilili. Mafractures ambiri chifukwa cha kuvulala kwakukulu ayenera kukhumudwitsidwa ndi vuto lalikulu monga osteogenesis imperfecta.
Mwachizoloŵezi, osteogenesis imperfecta inagawidwa kukhala imodzi mwa magulu anai akuluakulu:
- Lembani I: mawonekedwe ofatsa; kuwonedwa kwa ana omwe ali ndi sukulu
- Mtundu Wachiwiri: Wobadwa pa kubadwa
- Mtundu Wachitatu: kuwonedwa pa kubadwa; zambiri zophulika; nthawi zambiri samatha kuyenda
- Mtundu IV: kuuma pakati pa mtundu Woyamba ndikulemba III
Kwa zaka 10 zapitazi, mitundu yambiri ya osteogenesis inanenedwa. Panopa, pali mitundu 8 ya osteogenesis imperfecta.
Chithandizo
Pakalipano, palibe mankhwala ochiritsira osteogenesis imperfecta. Cholinga chachikulu cha mankhwala ndikuteteza kuvulala ndikukhala ndi mafupa abwino. Kuonetsetsa kuti ana omwe ali ndi osteogenesis imperfecta amadya chakudya chopatsa thanzi, azichita masewera olimbitsa thupi nthawi zonse, komanso amakhala ndi kulemera kwabwino. Kawirikawiri ana omwe ali ndi osteogenesis imperfecta amafunika thandizo lachidziwitso, ma-brace, kapena ma wheelchairs.
Kuchiza opaleshoni ya osteogenesis imperfecta kumadalira kukula kwa chikhalidwe ndi msinkhu wa wodwalayo. Mu ana aang'ono kwambiri, zophulika zambiri zimachitidwa chimodzimodzi, ngati kuti mwanayo analibe vuto lalikulu. Komabe, kugwiritsidwa ntchito pochita opaleshoni kumakhala kofala, ngakhale ali wamng'ono.
Kugwidwa mafupa kwa ana omwe ali ndi osteogenesis imperfecta kawirikawiri amachizidwa ndi opaleshoni kuti athetse mafupa ndi kuteteza kufooka.
Intramedullary rodding , pomwe ndodo yachitsulo imayikidwa pansi pamtunda wa fupa, nthawi zambiri imagwiritsidwa ntchito kuthandiza pang'onopang'ono mafupa ndi kuteteza kufooka kwa fupa. Ma Scoliosis nthawi zambiri amachitira nkhanza pamene vuto limakhala likupita patsogolo kwa ana omwe ali ndi osteogenesis imperfecta.
Udindo wa mankhwala pa chithandizo cha osteogenesis imperfecta ukutuluka, koma kafukufuku waposachedwapa amasonyeza phindu la mankhwala ndi bisphosphonates ndi calcium supplements . Chofunikira ndi chakuti mankhwalawa amathandizira fupa ndi kuchepetsa kuchuluka kwa ziphuphu. Kafukufuku akuchitiranso ntchito pogwiritsa ntchito mahomoni otchuka komanso mankhwala achibadwa.
Zotsatira:
Burnei G, et al. "Osteogenesis Imperfecta: Kuzindikira ndi Chithandizo" J. Am. Acad. Ortho. Kukonza., June 2008; 16: 356 - 366.