Zvese zvaunofanirwa Kuziva Nezve Proteus Syndrome

Zvemukati

• Waizviziva here?
• Zviratidzo zveProteus syndrome
• Zvinokonzera Proteus syndrome
• Kuongorora Proteus syndrome
• Kurapa kweProteus syndrome
• Matambudziko eichi chirwere
• Outlook

Mhedziso

Proteus syndrome inonyanya kushomeka asi isingaperi, kana yakareba-nguva, mamiriro. Inokonzera kuwanda kweganda, mapfupa, tsinga dzeropa, uye mafuta uye anobatana materu. Izvi zvinowedzera kuwanda kazhinji hazvina cancer.

Izvo zvinowedzera zvinogona kuva zvinyoro kana zvakanyanya, uye zvinogona kukanganisa chero chikamu chemuviri. Mitezo, musana, uye dehenya zvinowanzobatwa. Ivo kazhinji havaratidzike pakuzvarwa, asi vanozoonekwa zvakanyanya nezera remwedzi mitanhatu kusvika gumi nesere. Yakasiiwa isina kurapwa, kuwanda kwacho kunogona kutungamira kune zvakakomba hutano uye kufamba nyaya.

Inofungidzirwa kuti pasi pevanhu mazana mashanu pasi rese vane Proteus syndrome.

Waizviziva here?

Proteus syndrome yakawana zita rayo kubva kuna mwari wechiGiriki Proteus, uyo aizochinja chimiro chake kunzvenga kubatwa. Zvinofungidzirwawo kuti Joseph Merrick, uyo anonzi anonzi Nzou Munhu, aive nechirwere cheProteus.

Zviratidzo zveProteus syndrome

Zviratidzo zvinowanzosiyana zvakanyanya kubva kune mumwe munhu kuenda kune mumwe uye zvinogona kusanganisira:

• asymmetric overgrowths, senge rimwe divi remuviri rine mitezo mirefu kupfuura iyo imwe
• yakakwidziridzwa, isina kunaka maronda eganda anogona kunge aine ane bumpy, ane grooved chitarisiko
• musana wakakombama, unonziwo scoliosis
• mafuta anowedzera kuwanda, kazhinji padumbu, maoko, uye makumbo
• tumota tusina kenza, hunowanzo kuwanikwa pamazai, uye ma membuamu anofukidza uropi uye musana
• isina kukanganiswa tsinga dzeropa, izvo zvinowedzera mukana wekutyisidzira hupenyu kutyisa ropa
• kusarongeka kwenzvimbo yepakati yetsinga, iyo inogona kukonzera kuremara kwepfungwa, uye maficha akaita sekumeso kumeso uye musoro wakamanikana, maziso anodonha, nemhino dzakafara
• yakakora ganda mapads pane tsoka dzetsoka

Zvinokonzera Proteus syndrome

Proteus syndrome inoitika panguva yekukura kwechi fetus. Izvo zvinokonzerwa neinonzi nenyanzvi shanduko, kana shanduko yekusingaperi, yegene AKT1. Iyo AKT1 geni inobatsira kudzora kukura.

Hapana anonyatsoziva kuti nei shanduko iyi ichiitika, asi vanachiremba vanofungidzira kuti zvakangoitika uye hazvina kugarwa nhaka. Neichi chikonzero, Proteus syndrome hachisi chirwere chinopfuudzwa kubva kune chimwe chizvarwa kuenda kune chinotevera. Proteus Syndrome Foundation inosimbisa kuti mamiriro aya haasi kukonzerwa nechinhu chakaitwa kana kusaitwa nemubereki.

Masayendisiti akawana zvakare kuti iyo geni mutation ndeye mosaic. Izvi zvinoreva kuti zvinokanganisa mamwe maseru mumuviri asi kwete mamwe. Izvi zvinobatsira kutsanangura kuti nei rumwe rutivi rwemuviri rungakanganisike uye kwete rumwe, uye nei kuomarara kwezviratidzo kuchikasiyana zvakanyanya kubva kune mumwe munhu kuenda kune mumwe.

Kuongorora Proteus syndrome

Kuongororwa kunogona kuve kwakaoma. Mamiriro acho haawanzoitika, uye vanachiremba vazhinji havana kujaira. Nhanho yekutanga inogona kutorwa nachiremba ndeyekuongorora bundu kana zvinyama, uye kuyedza muyenzaniso wekuvapo kweakachinjika AKT1 geni. Kana imwe ikawanikwa, kuongorora bvunzo, senge X-rays, ultrasound, uye CT scans, inogona kushandiswa kutsvaga masasi emukati.

Kurapa kweProteus syndrome

Iko hakuna mushonga weProteus syndrome. Kurapa kunowanzo tarisa pakuderedza uye kugadzirisa zviratidzo.

Mamiriro acho anokanganisa zvikamu zvakawanda zvemuviri, saka mwana wako angangoda kurapwa kubva kuna vanachiremba vanoverengeka, kusanganisira zvinotevera:

• cardiologist
• dermatologist
• pulmonologist (nyanzvi yemapapu)
• orthopedist (pfupa chiremba)
• murapi wepanyama
• chiremba wepfungwa

Kuvhiya kubvisa kukura kweganda uye zvinyama zvakawandisa zvinogona kukurudzirwa. Vanachiremba vanogona zvakare kukurudzira kuvhiya kubvisa mahwendefa ekukura mupfupa kudzivirira kukura kwakanyanya.

Matambudziko eichi chirwere

Proteus syndrome inogona kukonzera matambudziko akawanda. Zvimwe zvinogona kutyisidzira hupenyu.

Mwana wako anogona kukura masasi makuru. Izvi zvinogona kuve zvinoshatisa uye zvinotungamira kune zvakakomba zvekufamba nyaya. Mamota anogona kumanikidza nhengo uye tsinga, zvichikonzera zvinhu zvakaita semapapu akadonha uye kurasikirwa kwekunzwa munhengo. Kukura kwepfupa kunogona zvakare kukonzera kurasikirwa kwekufamba.

Kukura kwacho kunogona zvakare kukonzera kushushikana kwetsinga dzinogona kukanganisa kukura kwepfungwa, uye zvinotungamira pakurasikirwa kwemaonero nekubatwa.

Vanhu vane Proteus syndrome vanowanzo tenderera kune yakadzika vein thrombosis nekuti inogona kukanganisa mitsipa yeropa. Yakadzika vein thrombosis ndeyekuvhara ropa kunoitika mutsinga dzakadzika dzemuviri, kazhinji mugumbo. Hembe inogona kusununguka uye kufamba nemuviri wese.

Kana ganda rikasungwa mune tsinga yemapapu, inonzi pulmonary embolism, inogona kuvhara ropa kuyerera uye kutungamira kurufu. Pulmonary embolism ndiyo inokonzeresa kufa kune vanhu vane Proteus syndrome. Mwana wako anogara achitariswa nekuda kweropa. Zviratidzo zvakajairika zve pulmonary embolism ndeizvi:

• mweya wekufema
• kurwadziwa pachipfuva
• chikosoro icho dzimwe nguva chinogona kuunza marosi akazara neropa

Outlook

Proteus syndrome chinhu chisingawanzoitika chinogona kusiyana mukuomarara. Pasina kurapwa, mamiriro acho achawedzera nekufamba kwenguva. Kurapa kunogona kusanganisira kuvhiyiwa uye kurapwa kwemuviri. Mwana wako anozotariswawo neropa rakagwamba.

Mamiriro acho anogona kukanganisa hupenyu hwehupenyu, asi vanhu vane Proteus syndrome vanogona kuchembera zvakajairika nekupindira kwekurapa uye kuongorora.