Osteogenesis imperfecta: ndi chiyani, mitundu ndi chithandizo

Zamkati

• Mitundu yayikulu
• Zomwe zimayambitsa matenda a osteogenesis
• Zizindikiro zotheka
• Momwe mungatsimikizire matendawa
• Kodi njira zamankhwala ndi ziti?
• Momwe mungasamalire ana omwe ali ndi vuto la osteogenesis

Osteogenesis imperfecta, yemwenso amadziwika kuti matenda am'mafupa a magalasi, ndi matenda osowa kwambiri omwe amachititsa kuti munthu akhale wopunduka, wamfupi komanso wosalimba mafupa, omwe amatha kugwidwa nthawi zonse.

Kufooka uku kumawoneka kuti kumachitika chifukwa cha vuto la chibadwa lomwe limakhudza kupanga mtundu wa collagen 1, womwe mwachilengedwe umapangidwa ndi ma osteoblast ndikuthandizira kulimbitsa mafupa ndi ziwalo. Chifukwa chake, munthu yemwe ali ndi matenda a osteogenesis imperfecta amabadwa kale ndi vutoli, ndipo atha kuphulika pafupipafupi ali mwana.

Ngakhale osteogenesis imperfecta sinachiritsidwebe, pali mankhwala ena omwe amathandizira kukonza moyo wamunthuyo, kuchepetsa ngozi komanso kusokonekera kwa mafupa.

Mitundu yayikulu

Malinga ndi mtundu wa Sillence, pali mitundu 4 ya osteogenesis imperfecta, yomwe ndi:

• Lembani I: ndi matenda ofala kwambiri komanso opepuka kwambiri, omwe amachititsa kuti mafupa asasinthe. Komabe, mafupawo ndi osalimba ndipo amatha kuthyoka mosavuta;
• Mtundu Wachiwiri: ndi mtundu woopsa kwambiri wa matenda womwe umapangitsa mwana wosabadwa kusweka mkati mwa chiberekero cha mayi, zomwe zimabweretsa kutaya mimba nthawi zambiri;
• Mtundu Wachitatu: anthu omwe ali ndi mtundu uwu, nthawi zambiri, samakula mokwanira, opunduka pakali pano pamsana ndipo azungu amaso amatha kupereka imvi;
• Mtundu wachinayi: ndi mtundu wowerengeka wa matendawa, momwe mumakhala mafupa pang'ono m'mafupa, koma palibe kusintha kwamitundu yoyera m'maso.

Nthawi zambiri, matenda a osteogenesis imperfecta amapitilira kwa ana, koma zizindikilo ndi kuuma kwa matendawa zimatha kukhala zosiyana, chifukwa mtundu wa matendawa umatha kusintha kuchokera kwa makolo kupita kwa ana.

Zomwe zimayambitsa matenda a osteogenesis

Matenda a magalasi amayamba chifukwa cha kusintha kwa majini omwe amapezeka mu jini lomwe limatulutsa mtundu woyamba wa collagen, womwe ndi puloteni yayikulu yomwe imagwiritsidwa ntchito popanga mafupa olimba.

Popeza ndikusintha kwa majini, osteogenesis imperfecta imatha kuchoka kwa makolo kupita kwa ana, koma imathanso kuonekera popanda zochitika zina m'banjamo, chifukwa cha kusintha kwa nthawi yapakati.

Zizindikiro zotheka

Kuphatikiza pakupangitsa kusintha kwa mafupa, anthu omwe ali ndi osteogenesis imperfecta amathanso kukhala ndi zisonyezo zina monga:

• Mafupa omasuka;
• Mano ofooka;
• Mtundu wabuluu wamaso oyera;
• Kupindika kwachilendo kwa msana (scoliosis);
• Kutaya kwakumva;
• Pafupipafupi mavuto kupuma;
• Mfupi;
• Inguinal ndi umbilical hernias;
• Kusintha kwa ma valve amtima.

Kuphatikiza apo, mwa ana omwe ali ndi matenda a osteogenesis imperfecta, matenda a mtima amathanso kupezeka, omwe amatha kukhala owopsa.

Momwe mungatsimikizire matendawa

Kuzindikira kwa osteogenesis imperfecta, nthawi zina, kumatha kupangidwa ngakhale ali ndi pakati, bola ngati pangakhale chiwopsezo chachikulu kuti mwana abadwe ndi vutoli. Pazochitikazi, chitsanzo chimatengedwa kuchokera ku umbilical chingwe komwe collagen yopangidwa ndi maselo a fetal pakati pa masabata 10 mpaka 12 a bere amafufuzidwa. Njira ina yocheperako ndiyo kupanga ultrasound kuti izindikire mafupa.

Pambuyo pobadwa, matendawa amatha kupangidwa ndi dokotala wa ana kapena wamankhwala a ana, kudzera pakuwona zizindikilo, mbiri ya banja kapena mayeso monga X-rays, kuyesa kwa majini komanso kuyesa magazi m'magazi.

Kodi njira zamankhwala ndi ziti?

Palibe chithandizo chenicheni cha matenda a osteogenesis imperfecta, chifukwa chake, ndikofunikira kupeza chitsogozo kuchokera kwa sing'anga. Kawirikawiri mankhwala a bisphosphonate amagwiritsidwa ntchito kuthandiza kupangitsa mafupa kukhala olimba ndikuchepetsa chiopsezo cha mafupa. Komabe, ndikofunikira kwambiri kuti mtundu uwu wamankhwala uyesedwe nthawi zonse ndi adotolo, chifukwa kungakhale kofunikira kusintha mankhwalawa pakapita nthawi.

Pakaphulika, adotolo amatha kulepheretsa fupalo ndi choponya kapena kusankha opaleshoni, makamaka pakagwa zophulika zingapo kapena zomwe zimatenga nthawi yayitali kuchira. Chithandizo cha zophulika chimafanana ndi cha anthu omwe alibe vutoli, koma nthawi yoperewera nthawi zambiri imakhala yayifupi.

Physiotherapy ya osteogenesis imperfecta itha kugwiritsidwanso ntchito nthawi zina kuthandiza kulimbitsa mafupa ndi minofu yomwe imawathandiza, kuchepetsa chiopsezo chovulala.

Momwe mungasamalire ana omwe ali ndi vuto la osteogenesis

Njira zina zofunika kusamalirira ana omwe ali ndi vuto loperewera kwa mafinya ndi:

• Pewani kukweza mwana kukhwapa, kuthandizira kulemera ndi dzanja limodzi pansi pa bumbu ndi linalo kumbuyo kwa khosi ndi mapewa;
• Osakoka mwanayo ndi dzanja kapena mwendo;
• Sankhani mpando wachitetezo wokhala ndi zokutira zofewa zomwe zimalola kuti mwanayo achotsedwe ndikuyika khama pang'ono.

Ana ena omwe ali ndi vuto la osteogenesis opanda ungwiro amatha kuchita masewera olimbitsa thupi, monga kusambira, chifukwa amathandizira kuchepetsa ngozi yophulika. Komabe, ayenera kuchita izi atalangizidwa ndi adotolo ndikuyang'aniridwa ndi aphunzitsi azamisala kapena othandizira.