Proteus syndrome: chii icho, maitiro ekuchiona nekuchibata

Zvemukati

• Main maficha
• Chii chinokonzera chirwere
• Kurapwa kunoitwa sei
• Basa rezvepfungwa muProteus syndrome

Proteus syndrome chirwere chinowanikwa chisingawanzozivikanwa nekukura kwemapfupa, ganda uye mamwe maturusi, zvichikonzera gigantism yemitezo yakati wandei nenhengo, kunyanya maoko, makumbo, dehenya uye musana.

Zviratidzo zveProteus Syndrome zvinowanzoonekwa pakati pemwedzi mitanhatu nemakumi masere ekuberekwa uye kukura kwakanyanya uye kusingaenzanisike kunowanzo kumira mukuyaruka. Izvo zvakakosha kuti iyo syndrome ionekwe nekukurumidza kuitira kuti matanho epakarepo atorwe kugadzirisa kusagadzikana uye kugadzirisa chimiro chemuviri chevarwere vechirwere, kudzivirira matambudziko epfungwa, senge kusarudzika munharaunda uye kushushikana, semuenzaniso.

Main maficha

Proteus syndrome inowanzo kukonzera kuoneka kwehumwe hunhu, senge:

• Kukanganisika mumawoko, makumbo, dehenya uye musana;
• Mutumbi asymmetry;
• Ganda rakawandisa rinopeta;
• Spine matambudziko;
• Yakareba kumeso;
• Zvinetso zvemwoyo;
• Warts uye mavara akajeka pamuviri;
• Yakawedzera spleen;
• Wedzera muhupamhi hweminwe, inonzi digital hypertrophy;
• Kudzora pfungwa.

Kunyangwe paine shanduko dzinoverengeka mumuviri, kazhinji, varwere vane chirwere ichi vanovandudza hunyanzvi hwavo, uye vanogona kunge vaine hupenyu hwakajairika.

Izvo zvakakosha kuti iyo syndrome ionekwe nekukurumidza sezvazvinogona, nekuti kana kuongorora kuchiitwa kubva pakuonekwa kwekutanga shanduko, zvinogona kubatsira kwete chete kudzivirira kusagadzikana kwepfungwa, asi zvakare kudzivirira zvimwe zvinowanzoitika zvechirwere ichi, zvakadai sekuonekwa kwemamota asingawanzo kana kuitika kweakadzika venous thrombosis.

Chii chinokonzera chirwere

Chikonzero cheProteus syndrome hachisati chanyatsosimbiswa, zvisinei zvinofungidzirwa kuti chingave chirwere chemajini chinokonzerwa nekushanduka kweshanduko mujeni reATK1 rinoitika panguva yekukura kwe "fetus".

Kunyangwe iri genetiki, Proteus syndrome haifungidzirwe senhaka, zvinoreva kuti hapana njodzi yekufambisa shanduko kubva kuvabereki kuenda kuvana. Nekudaro, kana paine zviitiko zveProteus syndrome mumhuri, zvinokurudzirwa kuti kurairidzwa kwemajini kuitwe, nekuti panogona kunge paine mukana wakakura wekuitika kweshanduko iyi.

Kurapwa kunoitwa sei

Iko hakuna chaiyo kurapwa kweProteus syndrome, uye inowanzo kurudzirwa nachiremba kuti ashandise chaiwo marapirwo ekudzora zvimwe zviratidzo, kuwedzera pakuvhiya kugadzirisa matishu, kubvisa matumhu uye kugadzirisa muviri aesthetics.

Kana ichionekwa mumatanho ekutanga, chirwere chacho chinogona kudzorwa kuburikidza nekushandisa mushonga unonzi Rapamycin, unova mushonga unodzivirira muviri unoratidzwa nechinangwa chekudzivirira kukura kwechigunwe uye kudzivirira kuumbwa kwemamota.

Uye zvakare, zvakakosha kwazvo kuti kurapwa kunoitwa neboka revazhinji vezvehutano, rinofanira kusanganisira vana chiremba, vanachiremba vemapfupa, vanachiremba vepurasitiki, dermatologists, vanachiremba vemazino, maurosurgeon uye psychologists, semuenzaniso. Nenzira iyoyo, munhu wacho achave nerutsigiro rwese rwunodiwa kuti uve nehupenyu hwakanaka.

Basa rezvepfungwa muProteus syndrome

Kuongorora kwepfungwa kwakakosha kwete kwete chete kumurwere ane chirwere asiwo kune avo vemhuri, sezvo nenzira iyi zvinokwanisika kunzwisisa chirwere uye kutora matanho anovandudza hupenyu hwemunhu uye kuzvitarisira. Uye zvakare, wezvepfungwa akakosha kuvandudza matambudziko ekudzidza, kurapa zviitiko zvekushushikana, kudzikisira kusagadzikana kwemunhu uye kubvumidza kudyidzana.