Chilichonse Chimene Muyenera Kudziwa Zokhudza Proteus Syndrome

Zamkati

• Kodi mumadziwa?
• Zizindikiro za matenda a Proteus
• Zomwe zimayambitsa Proteus syndrome
• Kuzindikira matenda a Proteus
• Chithandizo cha matenda a Proteus
• Zovuta za matendawa
• Chiwonetsero

Chidule

Matenda a Proteus ndi osowa kwambiri koma osakhalitsa, kapena okhalitsa. Zimayambitsa khungu, mafupa, mitsempha, ndi mafuta komanso othandizira. Kukula kwakukulu kumeneku nthawi zambiri sikukhala khansa.

Kukula kwakukulu kumatha kukhala kofatsa kapena kovuta, ndipo kumatha kukhudza gawo lililonse la thupi. Ziwalo, msana, ndi chigaza zimakhudzidwa kwambiri. Nthawi zambiri samawonekera pobadwa, koma amawonekera kwambiri pofika miyezi 6 mpaka 18. Ngati sanalandire chithandizo, kuchuluka kwake kumatha kudzetsa mavuto azaumoyo komanso kuyenda.

Akuti anthu ochepera 500 padziko lonse lapansi ali ndi matenda a Proteus.

Kodi mumadziwa?

Matenda a Proteus adatchedwa ndi mulungu wachi Greek Proteus, yemwe amasintha mawonekedwe ake kuti asatengeke. Amaganiziranso kuti a Joseph Merrick, otchedwa Elephant Man, anali ndi vuto la Proteus.

Zizindikiro za matenda a Proteus

Zizindikiro zimasiyanasiyana malinga ndi munthu wina ndipo zimaphatikizapo:

• kupitirira kwa asymmetric, monga mbali imodzi ya thupi yokhala ndi miyendo yayitali kuposa inayo
• zotupa, zotupa pakhungu zomwe zimatha kukhala zopindika, zowoneka bwino
• msana wopindika, wotchedwanso scoliosis
• kuchuluka kwamafuta, nthawi zambiri pamimba, mikono, ndi miyendo
• zotupa zopanda khansa, zomwe zimapezeka nthawi zambiri m'mimba mwake, ndi nembanemba zomwe zimaphimba ubongo ndi msana
• mitsempha yamagazi yopunduka, yomwe imawonjezera chiopsezo cha magazi omwe amaika moyo pangozi
• kusokonezeka kwa dongosolo lamanjenje, lomwe limatha kuyambitsa kufooka kwamaganizidwe, komanso mawonekedwe monga nkhope yayitali ndi mutu wopapatiza, zikope zothothoka, ndi mphuno zazikulu
• matumba achikopa onenepa pamapazi

Zomwe zimayambitsa Proteus syndrome

Proteus syndrome imachitika panthawi yomwe mwana amakula. Zimayambitsidwa ndi zomwe akatswiri amatcha kusintha, kapena kusintha kwamuyaya, kwa jini AKT1. Pulogalamu ya AKT1 jini imathandizira kuwongolera kukula.

Palibe amene akudziwa chifukwa chake kusinthaku kumachitika, koma madotolo amaganiza kuti ndizosintha komanso sanatengere. Pachifukwa ichi, Proteus syndrome si matenda omwe amapatsirana kuchokera m'badwo wina kupita ku wina. Proteus Syndrome Foundation ikugogomezera kuti vutoli silimayambitsidwa ndi zomwe kholo lidachita kapena silinachite.

Asayansi apezanso kuti kusintha kwa majini ndikosiyanasiyana. Izi zikutanthauza kuti zimakhudza maselo ena mthupi koma osati ena. Izi zimathandiza kufotokoza chifukwa chomwe mbali ina ya thupi imakhudzidwa osati ina, komanso chifukwa chake kuopsa kwa zizindikilo kumatha kusiyanasiyana pakati pa munthu ndi mnzake.

Kuzindikira matenda a Proteus

Kuzindikira kumakhala kovuta. Matendawa ndi osowa, ndipo madokotala ambiri sawadziwa. Gawo loyamba lomwe dokotala angatenge ndikutulutsa chotupa kapena minofu, ndikuyesa chitsanzocho ngati pali kusintha AKT1 jini. Ngati wina atapezeka, kuyesa zowunika, monga X-rays, ultrasound, ndi CT scan, kungagwiritsidwe ntchito kuyang'ana anthu amkati.

Chithandizo cha matenda a Proteus

Palibe mankhwala a Proteus syndrome. Chithandizo chimayang'ana pakuchepetsa ndikuwongolera zizindikilo.

Vutoli limakhudza mbali zambiri za thupi, chifukwa chake mwana wanu angafunikire chithandizo kuchokera kwa madotolo angapo, kuphatikiza izi:

• katswiri wamtima
• dermatologist
• pulmonologist (m'mapapo katswiri)
• mafupa (dokotala wa mafupa)
• wothandizira thupi
• katswiri wazamisala

Kuchita opaleshoni kuti muchepetse kuchuluka kwa khungu ndi minofu yochulukirapo kungalimbikitsidwe. Madokotala amathanso kunena kuti achite opaleshoni kuchotsa mbale zokulirapo m'fupa kuti zisawonongeke kwambiri.

Zovuta za matendawa

Proteus syndrome imatha kubweretsa zovuta zambiri. Zina zitha kupha moyo.

Mwana wanu akhoza kukhala ndi unyinji waukulu. Izi zitha kukhala zowononga ndikuwongolera zovuta zoyenda. Zotupa zimatha kupondereza ziwalo ndi mitsempha, zomwe zimayambitsa zinthu monga mapapu omwe agwa ndikumva kumva mwendo. Kukula kwa fupa kumathandizanso kuti munthu asayende bwino.

Kukula kumathanso kuyambitsa mavuto amitsempha omwe angakhudze kukula kwamisala, ndikupangitsa kuti masomphenya ndi khunyu ziwonongeke.

Anthu omwe ali ndi vuto la Proteus amakhala ndi vuto la mitsempha yozama chifukwa imatha kukhudza mitsempha yamagazi. Mitsempha yakuya ndi magazi omwe amapezeka m'mitsempha yakuya ya thupi, nthawi zambiri mwendo. Cholemacho chimatha kumasuka ndikuyenda mthupi lonse.

Ngati khungu limakwatirana mumitsempha yam'mapapu, yotchedwa pulmonary embolism, imatha kuletsa kuyenda kwa magazi ndikufa. Embolism embolism ndi yomwe imayambitsa imfa mwa anthu omwe ali ndi matenda a Proteus. Mwana wanu amayang'aniridwa pafupipafupi chifukwa chamagazi. Zizindikiro zodziwika za m'mapapo mwanga ndi:

• kupuma movutikira
• kupweteka pachifuwa
• chifuwa chomwe nthawi zina chimatha kubweretsa ntchofu zamagazi

Chiwonetsero

Matenda a Proteus ndichinthu chachilendo kwambiri chomwe chimatha kusiyanasiyana. Popanda chithandizo, vutoli limaipiraipira pakapita nthawi. Chithandizochi chingaphatikizepo kuchitidwa opaleshoni komanso kuchiritsa. Mwana wanu adzawunikidwanso magazi.

Vutoli lingakhudze moyo wabwino, koma anthu omwe ali ndi vuto la Proteus amatha msinkhu mwachizolowezi pothandizidwa ndi kuwunika ndi kuwunika.