Sengoli: Gregory Harris
Letsatsi La Creation: 15 April 2021
Ntlafatsa Letsatsi: 3 November 2024
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Ma scolarité avec le Syndrome d’Ehlers Danlos
Video: Ma scolarité avec le Syndrome d’Ehlers Danlos

Ehlers-Danlos syndrome (EDS) ke sehlopha sa mathata a futsitsoeng a tšoauoang ka manonyeletso a hlephileng haholo, letlalo le otlolohileng haholo (hyperelastic) le lutlang habonolo le methapo ea mali e senyehang habonolo.

Hona le mefuta e meholo e tšeletseng le bonyane mefuta e mehlano e nyane ea EDS.

Liphetoho tse fapaneng tsa liphatsa tsa lefutso li baka mathata ka collagen. Ena ke sesebelisoa se fanang ka matla le sebopeho ho:

  • Letlalo
  • Lesapo
  • Methapo ea mali
  • Litho tsa ka hare

Collagen e sa tloaelehang e lebisa matšoao a amanang le EDS. Mefuteng e meng ea lefu lena, ho ka phatloha ha litho tsa ka hare kapa li-valve tse sa tloaelehang tsa pelo.

Nalane ea lelapa e baka kotsi maemong a mang.

Matšoao a EDS a kenyelletsa:

  • Bohloko ba morao
  • Ho kopanya habeli
  • Letlalo le senyehileng habonolo, le robehileng le le otlolohileng
  • Ho senyeha habonolo le pholiso e mpe ea leqeba
  • Maoto a bataletseng
  • Keketseho ea motsamao o kopanetsoeng, manonyeletso a hlaha, ramatiki ea pele
  • Dislocation e kopanetsoeng
  • Bohloko bo kopaneng
  • Ho phatloha ha lera la pele ho nako nakong ea bokhachane
  • Letlalo le bonolo haholo ebile le boreleli
  • Mathata a pono

Ho hlahlojoa ke mofani oa tlhokomelo ea bophelo ho ka bontša:


  • Sebaka se holofetseng sa leihlo (cornea)
  • Ho se kopane ho feteletseng ha lenonyello le ho se kopane ho kopaneng
  • Mitral valve ka pelong ha e koalehe ka thata (mitral valve prolapse)
  • Tšoaetso ea marenene (periodontitis)
  • Ho phatloha ha mala, popelo kapa leihlo (ho bonoa feela ka methapo ea methapo, e leng seoelo)
  • Letlalo le bonolo, le lesesane kapa le otlolohileng haholo

Liteko tsa ho fumana EDS li kenyelletsa:

  • Thaepa ea Collagen (e etsoang sampoleng ea biopsy ea letlalo)
  • Ho hlahlojoa ha liphatsa tsa lefutso tsa Collagen
  • Echocardiogram (pelo ea ultrasound)
  • Lysyl hydroxylase kapa oxidase (ho lekola sebopeho sa collagen)

Ha ho na pheko e khethehileng bakeng sa EDS. Mathata le matšoao a motho ka mong a hlahlojoa le ho hlokomeloa ka nepo. Kalafo ea 'mele kapa tlhahlobo ea ngaka e sebetsanang le kalafo ea tlhabollo hangata ea hlokahala.

Lisebelisoa tsena li ka fana ka tlhaiso-leseling e batsi ho EDS:

  • Mokhatlo oa Naha oa Mathata a Rare - rarediseases.org/rare-diseases/ehlers-danlos-syndrome
  • Laeborari ea Naha ea Bongaka ea US, Referense ea Lehae la Liphatsa tsa lefutso - ghr.nlm.nih.gov/condition/ehlers-danlos-syndrome

Batho ba nang le EDS ka kakaretso ba na le nako e tloaelehileng ea bophelo. Bohlale bo tloaelehile.


Ba nang le mofuta o sa tloaelehang oa methapo ea EDS ba kotsing e kholo ea ho phatloha ha setho se seholo kapa sejana sa mali. Batho bana ba na le kotsi e kholo ea lefu la tšohanyetso.

Mathata a ka bang teng a EDS a kenyelletsa:

  • Bohloko bo sa foleng ba manonyeletso
  • Ramatiki ea pele ho nako
  • Ho hloleha ha maqeba a ho buoa ho koala (kapa ho rokoa hoa ntša)
  • Ho phatloha ha lera la pele ho nako nakong ea bokhachane
  • Ho phatloha ha likepe tse kholo, ho kenyelletsa le aortic aneurysm (feela ka methapo ea methapo)
  • Ho phatloha ha setho se sekoti se kang popelo kapa mala (feela ka methapo ea methapo)
  • Ho phatloha ha leihlo

Bitsa tumellano ea kopano le mofani oa hau haeba u na le nalane ea lelapa ea EDS mme u tšoenyehile ka kotsi ea hau kapa u rera ho qala lelapa.

Bitsa kopano le mofani oa hau haeba uena kapa ngoana oa hau a e-na le matšoao a EDS.

Ho khothaletsoa tlhabollo ea lefutso bakeng sa bao e tla ba batsoali ba nang le nalane ea lelapa ea EDS. Ba rerileng ho qala lelapa ba lokela ho hlokomela mofuta oa EDS eo ba nang le eona le mokhoa oa eona oa ho fetisetsa bana. Sena se ka etsoa qeto ka liteko le litlhahlobo tse hlahisitsoeng ke mofani oa hau kapa moeletsi oa lefutso.


Ho khetholla likotsi tse bohlokoa tsa bophelo bo botle ho ka thusa ho thibela mathata a maholo ka ho hlahloba ka hloko le ka liphetoho tsa bophelo.

Krakow D. Maloetse a tšoaetsanoang a lisele tse sebetsang. Ka: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, bahlophisi. Kelly le Firestein's Textbook of Rheumatology. 10th ed. Philadelphia, PA: Elsevier; 2017: khaolo ea 105.

Pyeritz RE. Lefu le futsitsoeng la lisele tse sebetsang. Ka: Goldman L, Schafer AI, li-eds. Phekolo ea Goldman-Cecil. La 25th ed. Philadelphia, PA: Elsevier Saunders; 2016: khaolo ea 260.

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