Nthawi zina kupuma kungakhale kosabadwa mwa kubadwa. Mwachibadwa cha majini, omwe nthawi zambiri amatchedwa kutukwana kwa Ondine, izi zingakhale zovuta kwambiri ndipo zimakhala ndi zotsatira zofunikira. Kodi temberero la Ondine ndi chiyani? Fufuzani zina mwazifukwa zomwe zimayambitsa matenda a hypoentilation ndi momwe angathandizire.
Kodi Kutembereredwa kwa Ondine N'kutani?
Temberero la Ondine (lomwe limatchedwanso congenital central hypoventilation syndrome kapena CCHS) ndilosazolowereka kwambiri, komwe kumakhala kosalala komwe munthu wodwalayo amasiya kupuma pamene akugona.
Kawirikawiri zimakhala zosiyana, zomwe zikutanthauza kuti zimachokera ku kubadwa. Zinazindikirika mu chipatala cha pambuyo pa kubereka. Kugonana kwapakati pakati kumakhala ndi ubongo wosagwira mpweya wabwino. Izi zikuwoneka kuti zakhala chifukwa cha kuchepetsa kutaya kwa mpweya wabwino wa carbon dioxide ndi mpweya wotsika m'magazi. Izi zimakhala zoopsa kwambiri pogona.
Temberero la Ondine limatchulidwa ndi nthano yonena za nymph yamtima yamtima yomwe imatemberera mwamuna wake wosakhulupirika kuti asiye kupuma ngati atayambiranso kugona. Malinga ndi zamankhwala, pakatikati ya hypoventilation imayimira matenda obisala kwambiri.
Nchiyani Chimachititsa Central Hypoventilation?
Mankhwalawa amachititsa munthu mmodzi mwa anthu mamiliyoni makumi atatu, omwe amatanthauza anthu mazana angapo omwe ali nawo padziko lapansi. Momwemo, izo zimawoneka kuti ndizovuta kwambiri. Zikuoneka kuti kusintha kwa chibadwa ndiko chifukwa chachikulu.
Zimalingalira kuti zimachitika pamene ubongo umalepheretsa kupuma, monga momwe zingathenso kuwonetsetsa pakati pa kugonana kwapakati .
Matendawa angakhalepo kuchokera kubadwa kapena angapangitse atatha kuwonongeka kwa ubongo, womwe umayendetsa kupuma. Magulu a hypo hypotiltilation angagwirizane ndi vuto lomeza, matumbo a m'mimba otchedwa matenda a Hirschsprung , kapena zotupa zotchedwa neuroblastoma .
Ngakhale kuti kawirikawiri chikhalidwechi chikuchitika mobwerezabwereza, pakhoza kukhala chizoloŵezi cha chibadwa chomwe chimapezeka m'mabanja. Achibale akhoza kukhala ndi mawonekedwe oopsa kwambiri omwe amakhudza dongosolo lokhazikika la mantha. Vuto lalikulu likuwonekera chifukwa cha kusintha kwa PHOX2B (4q13) jini. Ambiri amaphatikizapo kufalikira kwa majeremusi kubwereza kuchokera ku mabungwe makumi awiri (otchedwa alanine) mpaka mabasi 25 mpaka 33. Pa 10% za milandu, kusintha kwasintha kwina kumalo omwewo kumawoneka kukhala ndi gawo.
Kodi Central Hypoventilation Yachitidwa Bwanji?
Anthu ambiri okhudzidwa amayamba posakhalitsa atabadwa. Zakhala zikufotokozedwa kuti ziyambe mtsogolo, komabe, ngakhale ngakhale akulu akulu akulembedwa. Zizindikiro zingatuluke m'mavuto akuluakulu pogwiritsa ntchito anesthesia kapena sedative.
Chithandizo cha vutoli chimaphatikizapo kugwiritsa ntchito mpweya wothamangitsidwa ndi tracheostomy tube patsogolo pa mmero pomwe munthu yemwe wagwidwapo atagona, ngakhale panthawi yopuma. Ngati izi sizinagwiritsidwe ntchito, munthu amene ali ndi matendawa akhoza kufa nthawi iliyonse atagona.
Chifukwa cha mtundu wa chithandizocho, mabanja a anthu ovutika nthawi zambiri amakhala odziwa bwino kuyendetsa zipangizo zofunika kuti apume bwinobwino. Zingakhale zowopsya poyamba, koma kuthandiza kuchipatala kumapangitsa kusintha kosamalidwa kuchipatala kunyumba.
Malangizo ochokera kwa opatsirana opuma, kuphatikizapo zipangizo zam'nyumba, akhoza kuthetsa kusinthaku.
Mawu Ochokera
Ngati mukufuna kudziwa zambiri za temberero la Ondine, makamaka ngati muli ndi mwana wokhudzidwa, ndibwino kuti muganizire kuti mukufunsana ndi mwana wa pulmologist kuchipatala cha zamaphunziro. Chifukwa cha vutoli, ndi kuopsa kwa zotsatira zake, chisamaliro cha katswiri chidzafunikanso poyamba. Zitha kukhala zotheka kuyanjana ndi mabanja ena omwe akudwala matendawa. Thandizo la chikhalidwe ichi lingakhale lothandiza pa zifukwa zambiri.
Yesetsani kupeza thandizo limene mukufunikira kuchokera kwa katswiri kuti mukwaniritse thanzi lanu ndi ubwino wa mwana wanu ndi banja lanu.
> Zotsatira:
> Amiel J et al . "Kuwonjezereka kwa Polyalanine ndi kusintha kwasintha kwa nyumba ya bokosi loyendetsa bwalo la PHOX2B, lomwe lili ndi mapaundi ophatikizana, omwe ali pakati pa matenda a hypoventilation." Nat Genet 2003; 33 (4): 459-461.
> Chen ML, Keens TG. "Congenital central hypoventilation syndrome: osati matenda ena osowa." Kuchiza kwapadera Rev 2004; 5 (3): 182-189.
> Faraco J ndi Mignot E. "Mafupa a Kugona ndi Matenda a Kugona mwa Anthu." Muzikhalidwe ndi Zochita za Kugona Mankhwala , Elsevier, 2011, mas. 93-94.