Kodi Marfan syndrome, matenda ndi chithandizo

Zamkati

• Zizindikiro zazikulu
• Zomwe zimayambitsa matenda a Marfan
• Momwe matendawa amapangidwira
• Njira zothandizira

Marfan Syndrome ndi matenda amtundu omwe amakhudza minofu yolumikizana, yomwe imathandizira kuthandizira ndikukhazikika kwa ziwalo zosiyanasiyana m'thupi. Anthu omwe ali ndi vutoli amakhala ataliatali kwambiri, owonda komanso amakhala ndi zala zazitali kwambiri komanso zala zazitali komanso atha kusintha pamtima, m'maso, m'mafupa ndi m'mapapu.

Matendawa amabwera chifukwa chololera kubadwa nawo mumtundu wa fibrillin-1, womwe ndi gawo lalikulu la mitsempha, makoma a mitsempha ndi mafupa, zomwe zimapangitsa ziwalo zina ndi ziwalo za thupi kukhala zosalimba. Matendawa amapangidwa ndi dokotala kapena dokotala wa ana kudzera m'mbiri yaumoyo wa munthu, kuyesa magazi ndi kulingalira ndipo chithandizocho chimakhala ndi kuthandizira sequelae yomwe imayambitsa matendawa.

Zizindikiro zazikulu

Matenda a Marfan ndi matenda amtundu womwe amachititsa kusintha kosiyanasiyana m'thupi, kumabweretsa zizindikilo zomwe zimatha kubadwa kapena moyo wonse, kuuma kwake kumasiyana pakati pa munthu ndi mnzake. Zizindikirozi zitha kupezeka m'malo awa:

• Mtima: zoyipa zazikulu za matenda a Marfan ndikusintha kwa mtima, komwe kumabweretsa kuchepa kwa chithandizo kukhoma lamitsempha, komwe kumatha kuyambitsa aortic aneurysm, kutulutsa kwamitsempha yamagetsi ndi kuphulika kwa mitral valve;
• Mafupa: Matendawa amachititsa kuti mafupa akule mopitilira muyeso ndipo amatha kuwonekera chifukwa cha kukokomeza kwakutali kwa munthu ndi mikono, zala ndi zala zazitali kwambiri. Chifuwa chotchinga, chotchedwansopectus excavatum, ndipamene kukhumudwa kumachitika pakatikati pa chifuwa;
• Maso: ndizofala kwa anthu omwe ali ndi vutoli kusuntha kwa diso, glaucoma, cataract, myopia ndipo amatha kukhala ndi mbali yoyera kwambiri ya diso mopepuka;
• Mphepete: mawonetseredwe a matendawa atha kuwoneka pamavuto amsana monga scoliosis, komwe ndiko kupatuka kwa msana kumanja kapena kumanzere. Ndikothekanso kuwona kuwonjezeka kwa thumba lanyumba mdera lumbar, lomwe ndi nembanemba yomwe imakuta dera la msana.

Zizindikiro zina zomwe zimatha kuchitika chifukwa cha matendawa ndizosunthika kwa mitsempha, kufooka kwa milomo, komwe kumatchedwa denga la pakamwa, ndi mapazi athyathyathya, omwe amadziwika ndi mapazi ataliatali, osapindika. Onani zambiri za flatfoot ndi momwe amathandizira.

Zomwe zimayambitsa matenda a Marfan

Matenda a Marfan amayamba chifukwa cha vuto mu jini yotchedwa fibrillin-1 kapena FBN1, yomwe imagwira ntchito yotsimikizira kuthandizira ndikupanga ulusi wolimba wa ziwalo zosiyanasiyana m'thupi, monga mafupa, mtima, maso ndi msana.

Nthawi zambiri, chilema ichi chimachokera, izi zikutanthauza kuti chimafalikira kuchokera kwa abambo kapena amayi kupita kwa mwana ndipo zimatha kuchitika mwa amayi ndi abambo. Komabe, nthawi zina, vuto ili mu jini limatha kuchitika mwamwayi popanda chifukwa chodziwika.

Momwe matendawa amapangidwira

Matenda a Marfan syndrome amapangidwa ndi dokotala kapena dokotala wa ana kutengera mbiri ya banja lake ndikusintha kwakuthupi, ndipo mayeso oyerekeza, monga echocardiography ndi electrocardiogram, atha kulamulidwa kuti athe kuzindikira mavuto omwe angakhalepo mumtima, monga aortic dissection. Phunzirani zambiri za kung'ambika kwa aortic ndi momwe mungazindikire.

Ma X-rays, computed tomography kapena imaginous resonance imaging amawonetsedwanso kuti awone ngati kusintha kwa ziwalo zina ndi kuyesa magazi, monga mayeso amtundu, omwe amatha kuzindikira kusintha kwa jini komwe kumayambitsa matendawa. Zotsatira zakayeso zikatuluka, adotolo adzapereka upangiri wa majini, momwe upangiri pazokhudza banja lanu.

Njira zothandizira

Chithandizo cha matenda a Marfan sikuti chichiritse matendawa, koma chimathandiza kuchepetsa zizindikilo kuti atukule moyo wa anthu omwe ali ndi vutoli ndipo cholinga chake ndikuthandizira kuchepetsa kufooka kwa msana, kusintha magwiridwe antchito ndi kuchepetsa kuthekera kwa kusokonezeka.

Chifukwa chake, odwala omwe ali ndi matenda a Marfan ayenera kuyezetsa mtima komanso mitsempha yamagazi, ndikumwa mankhwala monga beta-blockers, kuti ateteze kuwonongeka kwa mtima. Kuphatikiza apo, chithandizo chamankhwala chitha kukhala chofunikira kukonza zotupa mu mtsempha wamagazi aortic, mwachitsanzo.