Munyori: Tamara Smith
Zuva Rekusika: 19 Ndira 2021
Gadziridza Zuva: 1 Kubvumbi 2025
Anonim
Maroteaux-Lamy chirwere - Utano
Maroteaux-Lamy chirwere - Utano

Maroteaux-Lamy Syndrome kana Mucopolysaccharidosis VI chirwere chisingawanzo nhaka, umo varwere vane hunhu hunotevera:

  • Pfupi,
  • kumeso kukanganisika,
  • mutsipa mupfupi,
  • kudzokorora otitis,
  • mweya wekufema,
  • maronda kukanganisika uye
  • kuomarara kwemhasuru.

Chirwere ichi chinokonzerwa neshanduko yeiyo enzyme Arylsulfatase B, inoitadzisa kuita basa rayo, kunova kudzikisira polysaccharides, iyo inozounganidzwa mumasero, ichivandudza zviratidzo zvechirwere.

Vanhu vane chirwere vane njere dzakasarudzika, saka vana havadi chikoro chakakosha, chete zvigadzirwa zvakagadziriswa zvinofambisa kusangana nevadzidzisi uye vavanodzidza navo.

Iko kuongororwa kunoitwa neye geneticist kunoenderana nekiriniki kuongororwa uye kuongorora kweorabhorochemical. Kuongororwa mumakore ekutanga ehupenyu kwakakosha pakujekesa chirongwa chekutanga chekupindira, icho chinobatsira mukukudziridzwa kwemwana uye mukutumirwa kwevabereki kuenda kumazano ezvechibereko, nekuti vari panjodzi yekupfuudza chirwere ichi kuenda vana vavo vekupedzisira.


Iko hakuna mushonga weMaroteaux-Lamy Syndrome, asi kumwe kurapa kwakadai sekuiswazve kwemongo wemapfupa uye enzyme kutsiva kurapa kunoshanda mukuderedza zviratidzo. Physiotherapy inoshandiswa kudzikamisa kuomarara kwemhasuru uye kuwedzera mafambiro emuviri wemunhu. Havasi vese vanotakura vane zvese zviratidzo zvechirwere, kuomarara kunosiyana kubva pamunhu kusvika kune mumwe, vamwe vanokwanisa kurarama hupenyu hwakajairika.

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