Kufooketsa Mavuto a Kugona Chifukwa cha Cartilage Kulephereka M'ndege
Zikuwoneka zovuta kukhulupilira, koma kugwiritsidwa ntchito kawiri kumakhala chizindikiro chakuti mutha kukhala pachiopsezo cha kuperewera kwa kugona kwa kugona ? Ehlers-Danlos syndrome (EDS) ndi chikhalidwe chomwe chimakhudza mtembo m'thupi lonse, kuphatikizapo pamphepete mwa msewu, ndipo izi zingachititse kuti anthu azivutika kupuma, kugona tulo, ndi usana.
Phunzirani za zizindikiro, mawonekedwe, kufalikira, ndi chiyanjano pakati pa Ehlers-Danlos ndi kugona tulo, komanso ngati chithandizo chingathandize.
Kodi Ehlers-Danlos Syndrome (EDS) ndi chiyani?
Ehlers-Danlos syndrome (EDS), kapena Ehlers-Danlos matenda, ndi matenda omwe amakhudza ziphuphu zomwe zimathandiza khungu, mafupa, mitsempha ya magazi, ndi ziwalo zina ndi ziwalo zina. EDS ndi chibadwa chomwe chimakhudza chitukuko cha collagen ndi mapuloteni omwe amagwiritsidwa ntchito monga matabwa a tizilombo. Zizindikiro zake zimakhala zovuta zambiri, zomwe zimayambitsa ziwalo zochepa zomwe zimawopsyeza moyo.
Kusinthika kwa mazira oposa khumi ndi awiri kumalumikizidwa ku chitukuko cha EDS. Zovuta zachibadwa zimakhudza malangizo opanga zidutswa zingapo za collageni, chinthu chomwe chimapanga dongosolo ndi mphamvu kuti zikhale zomangira thupi lonse. Colgogen ndi mapuloteni okhudzana sangagwirizane bwino.
Ziphuphu zimenezi zimayambitsa zofooka m'matenda, pakhungu, mafupa, ndi ziwalo zina.
Pali ma autosomal akuluakulu (AD) ndi ma autosomal (AR) mawonekedwe a cholowa, malingana ndi subtype ya EDS. Mu cholowa cha AD, buku limodzi la jini yosinthidwa ndilokwanira kuchititsa matendawa. Mu cholowa cha AR, maginito onse awiri a jini ayenera kusinthidwa kuti chikhalidwe chichitike ndipo makolo nthawi zambiri amanyamula jini koma amakhala oyenerera.
Kuphatikiza mitundu yosiyanasiyana, EDS ikuyenera kukhudza pafupifupi mmodzi mwa anthu 5,000.
Zizindikiro za Ehlers-Danlos Syndrome
Zizindikiro zosiyana ndi matenda a Ehlers-Danlos zimasiyana malinga ndi chifukwa chomwe chimayambitsa matendawa. Zina mwa zizindikiro zowonjezera ndizo:
- Kuphatikizana kophatikizana: Pakhoza kukhala gulu lalikulu lophatikizana (nthawi zina limatchedwa "ophatikizana"). Zolumikizana izi zingakhale zosasunthika ndipo zimakhala zovuta kuzimitsa (kapena kugonjetsa) ndipo zimapweteka kwambiri.
- Kusintha kwa khungu: Khungu likhoza kukhala lofewa komanso labwino. Ndi yotambasula kwambiri, yotanuka, ndi yofooka. Izi zingachititse kuti kukhale kovuta kuphwanya komanso kusala pang'ono.
- Kulankhula kochepa kwa minofu: Makanda angakhale ndi minofu yofooka ndi kuchedwa kwa chitukuko cha motor (chokhudza kukhala, kuima, ndi kuyenda).
- Kuchuluka kwa Scoliosis pa kubadwa
- Kupweteka kosautsa (kawirikawiri kumagwiritsa ntchito minofu ndi kukhudza ziwalo)
- Kumayambiriro koyambitsa nyamakazi
- Machiritso osavulaza ovulaza
- Mitral valve prolapse
- Matenda a chingamu
- Kugonana kwanthaŵi yamadzulo
- Kutopa
- Mkhalidwe wopanda umoyo wa moyo
Zingakhale zothandiza kubwereza magawo asanu ndi limodzi a EDS kuti mumvetse bwino zizindikiro zomwe zimagwirizanitsidwa ndi zoopsa zomwe zingakhalepo.
Kumvetsetsa magawo 6 a Ehlers-Danlos Syndrome
Mu 1997, kudasinthidwanso mu magawo osiyanasiyana a Ehlers-Danlos syndrome.
Zotsatira zake, mitundu ikuluikulu isanu ndi umodzi yosiyana ndi zizindikiro, zizindikiro, zomwe zimayambitsa zamoyo, ndi njira za cholowa zinadziwika. Ma subtypes awa ndi awa:
Mtundu wamakono: Wodziwika ndi mabala omwe amagawanika ndi kutuluka pang'ono, kusiya zipsera zomwe zimatambasuka pakapita nthawi kupanga "pepala la ndudu". Mtundu uwu umakhala ndi chiopsezo chochepa chotsegula zitsulo zamagazi. Lili ndi cholowa chodziwika kwambiri chokhudza autosomal, chokhudza mmodzi mwa anthu 20,000 mpaka 40,000.
Mtundu wosasinthasintha: Wodziwika bwino subtype wa EDS, umawoneka ndi zizindikiro zogwirizana kwambiri. Ndiyotchuka kwambiri ndipo ingakhudze munthu mmodzi mwa anthu 10,000 mpaka 15,000.
Mtundu wa mitsempha: Imodzi mwa mitundu yoopsa kwambiri, ingayambitse moyo, kuwombera (kapena kupasuka) kosadziŵika kwa mitsempha ya magazi. Izi zingayambitse magazi, kupweteka, ndi mantha. Palinso chiopsezo chowonjezeka cha kuphulika kwa thupi (kumakhudza matumbo ndi chiberekero pa nthawi ya mimba). Ndi autosomal yaikulu, koma imakhudza munthu mmodzi mwa anthu 250,000.
Mtundu wa Kyphoscoliosis: Kawirikawiri umakhala ndi kupindika kwapang'onopang'ono kwa msana umene ungasokoneze kupuma. Amakhala ndi chiopsezo chochepa chotseketsa mitsuko ya magazi. Ndi autosomal recessive ndi yosavuta, ndi 60 milandu okha padziko lonse.
Mtundu wa Arthrochalasia: EDS subtype ikhoza kupezeka pa kubadwa, ndipo chiuno chomwe chimayambitsa kusokonezeka kwa mbali zonse ziwiri chimaonekera pakubereka. Ndi autosomal yaikulu ndi pafupifupi 30 milandu yotchulidwa padziko lonse.
Mtundu wa Dermatosparaxis: Chiwonekedwe chosaoneka bwino kwambiri, chimakhala ndi khungu lomwe limatuluka ndi makwinya, ndipo limapanga mapepala owonjezera omwe angakhale otchuka pamene ana akula. Ndi autosomal recessive ndi milandu yokwana khumi ndi awiri padziko lapansi.
Kugonana Kumapweteka EDS ndi Link ku OSA
Kodi pali mgwirizano wotani pakati pa matenda a Ehlers-Danlos ndi matenda osokoneza bongo? Monga taonera, chitukuko chosawonongeka cha karotika chimakhudza minofu mthupi lonse, kuphatikizapo zomwe zimayendera panjira. Matendawa angakhudze kukula ndi chitukuko cha mphuno komanso maxilla (nsagwada kumtunda) komanso pamwamba pamtunda. Ndi kukula kosazolowereka, misewu ingakhale yopapatiza, yofooka, ndipo imatha kugwa.
Kugwera kwapadera kapena kukwanira kwa mpweya wapamwamba pa nthawi ya tulo kumayambitsa matenda obwera chifukwa cha kugona. Izi zingachititse madontho m'magazi a mpweya wa magazi, kugawidwa kwa tulo, kuwuka kowonjezereka, ndi khalidwe lochepetsedwa. Chifukwa chake, kugona kwa tsiku ndi tsiku ndikutopa kumatha kuchitika. Pangakhale kuwonjezeka kwa chidziwitso, maganizo, ndi zopweteka. Zina mwa zizindikiro za matenda obisala, monga kukweta , kupukuta kapena kupukuta, kuwona mphuno, kukodza ( nocturia ), ndi kukupera mano (bruxism) kungakhalensopo.
Kafukufuku wam'mbuyo ochepa odwala EDS kuchokera mu 2001 wathandizira mavuto owonjezereka ndi kugona. Ankaganiza kuti anthu omwe ali ndi EDS, 56 peresenti anali ndi vuto logona. Kuwonjezera pamenepo, 67 peresenti amadandaula za kusuntha kwa magulu nthawi ndi nthawi . Ululu, makamaka kupweteka kwa ululu, unkawonjezeredwa ndi odwala EDS.
Kodi Apnea Amagona Bwanji Ku Ehlers-Danlos Syndrome?
Kafukufuku amasonyeza kuti kugona tulo ndi kofala pakati pa omwe ali ndi EDS. Phunziro la 2017 likusonyeza kuti 32 peresenti ya anthu omwe ali ndi matenda a Ehlers-Danlos amachititsa kuti munthu asagone tulo (poyerekezera ndi 6 peresenti ya mphamvu). Anthuwa amadziwika kuti ali ndi vuto labwino (46 peresenti), lachikale (35 peresenti), kapena ena (19%) subtypes. Iwo ankadziwika kuti ali ndi ubwino wochuluka wa usana wa tsiku monga momwe amayesedwa ndi chiwerengero cha kugona kwa Epworth. Mlingo wa apnea ogona umagwirizanitsidwa ndi msinkhu wa kugona usana komanso khalidwe lapansi la moyo.
Kuchiza kwa Apnea Kugona ndi Kuyankha kwa Thandizo mu EDS
Pamene kugona kwa apnea kumatsimikizirika, chithandizo chachipatala chikuthandiza kulandira chithandizo pakati pa odwala Ehlers-Danlos syndrome. Pamene zaka zikuwonjezereka, kupuma kosokonezeka kugona kungasinthe kuchokera ku mpweya wochepa wa mpweya ndi kumenyedwa kwa mimba kumbali zina zooneka bwino za hypopnea ndi apnea zomwe zimayambitsa kugona kwa kugona. Kupuma kwachilendo kungakhale kosadziwika. Kugona kwa masana, kutopa, kugona tulo, ndi zizindikiro zina zikhoza kunyalanyazidwa.
Mwamwayi, kugwiritsa ntchito mankhwala opatsirana opatsirana (CPAP) opitilirapo angapereke mpumulo mwamsanga ngati kugona kwa mpweya kumakhala bwino. Kafufuzidwe kafukufuku amafunikanso kuti apeze chithandizo chamankhwala odwala matenda obisala m'mimba muno.
Ngati mukumva kuti muli ndi zizindikiro zofanana ndi Ehlers-Danlos syndrome ndi matenda obongoletsa ogona, yambani kulankhula ndi dokotala wanu za kuyesa, kuyesa, ndi chithandizo.
> Zotsatira:
> Gaisl T, et al . "Kupewa kugona tulo ndi moyo wabwino ku Ehlers-Danlos syndrome: phunziro lofanana la kagulu. 2017 Jan 10.
> Guilleminault C, et al . "Kupuma mokwanira ku Ehlers-Danlos syndrome: chibadwa cha OSA." Chifuwa. 2013 Nov; 144 (5): 1503-11.
> "Ehlers-Danlos matenda". Laibulale ya National National Medicine. 2017 Feb 21.
> Verbraecken J, et al . "Kufufuza kwa kugonana kwa ogona ndi Ehlers-Danlos syndrome ndi Marfan: kufufuza mafunso." Clin Genet. 2001 Nov; 60 (5): 360-5.