Tus Sau: Gregory Harris
Hnub Kev Tsim: 15 Lub Plaub Hlis Ntuj 2021
Hloov Hnub: 3 Lub Kaum Ib Hli Ntuj 2024
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Ehlers-Danlos Syndrome- causes, symptoms, diagnosis, treatment, pathology
Daim Duab: Ehlers-Danlos Syndrome- causes, symptoms, diagnosis, treatment, pathology

Ehlers-Danlos syndrome (EDS) yog ib pab pawg neeg ntawm caj ces muaj kab mob sib kis tau los ntawm cov pob qij txha xoob, tawv nqaij tawv nqaij uas ua rau doog ntshav yooj yim, thiab cov hlab ntshav puas yooj yim.

Muaj rau ntau hom loj thiab tsawg kawg yog tsib hom me EDS.

Ntau cov noob hloov pauv (hloov zuj zus) ua rau muaj teeb meem nrog collagen. Nov yog cov ntaub ntawv uas muab lub zog thiab qauv rau:

  • Daim tawv nqaij
  • Pob txha
  • Cov hlab ntsha
  • Sab hauv nruab nrog cev

Qhov sib txawv ntawm collagen ua rau cov tsos mob cuam tshuam nrog EDS. Hauv qee cov tsos mob ntawm tus mob, qhov tawg ntawm lub plab hnyuv siab raum lossis lub plawv dhia tsis zoo tuaj yeem tshwm sim.

Tsev neeg keeb kwm yog qhov txaus ntshai nyob rau qee kis.

Cov tsos mob ntawm EDS suav nrog:

  • Mob nraub qaum
  • Muab ob leeg los sib koom ua ke
  • Yooj yim puas, nqaij doog, thiab tawv nqaij tawv
  • Txoj kev yooj yim kiav txhab thiab qhov mob zoo
  • Tiaj taw
  • Txhawb kev sib koom tes ntau ntxiv, pob qij txha tawm, mob caj dab thaum ntxov
  • Kev sib koom tes
  • Mob tej pob txha
  • Kev puas tsuaj ua ntej ntawm daim nyias nyias thaum lub cev xeeb tub
  • Cov tawv nqaij mos heev thiab mos mos
  • Cov teeb meem tsis pom kev

Kev kuaj mob los ntawm kws kho mob tej zaum yuav qhia:


  • Deformed saum npoo ntawm lub qhov muag (pom pob zeb)
  • Tshaj dhau ntawm kev sib koom tes ua ke thiab lub siab sib txuam
  • Mitral valve nyob rau hauv lub siab tsis kaw nruj nreem (mitral valve prolapse)
  • Cov kab mob ntawm cov pos hniav (mob ncig)
  • Kev txhaws ntawm cov hnyuv, lub tsev menyuam, lossis pob ntseg (pom tsuas yog hauv EDS vascular, uas yog qhov tsis tshua muaj)
  • Cov tawv nqaij mos, nyias nyias lossis ntau sab

Kev ntsuam xyuas los ntsuas EDS suav nrog:

  • Collagen typing (ua rau ntawm daim tawv nqaij kuaj me me)
  • Collagen noob hloov mus kuaj
  • Echocardiogram (mob plawv hauv lub plawv)
  • Lysyl hydroxylase lossis oxidase kev ua si (tshawb xyuas collagen tsim)

Nws tsis muaj ib txog kev kho tau rau EDS. Tus kheej cov teeb meem thiab cov tsos mob raug ntsuas thiab tu kom tsim nyog. Txoj kev kho lub cev lossis tshuaj ntsuas los ntawm tus kws kho mob tshwj xeeb hauv kev kho kom rov zoo nkauj tuaj yeem xav tau.

Cov peev txheej no tuaj yeem muab cov ntaub ntawv ntxiv ntawm EDS:

  • Koom Haum Koom Haum Tsis tshua muaj teeb meem - rarediseases.org/rare-diseases/ehlers-danlos-syndrome
  • Teb Chaws Asmeskas Chaw Qiv Ntawv Tshuaj, Tshuaj Txhim Tsev Hauv Tsev - ghr.nlm.nih.gov/condition/ehlers-danlos-syndrome

Cov neeg nrog EDS feem ntau muaj lub neej ntev. Txoj kev ntse ntse yog qhov ib txwm.


Cov uas muaj feem tsawg vascular vascular EDS muaj qhov pheej hmoo ntau dua rau kev sib tawg ntawm ib qho khoom nruab nrog lossis ntshav hlab ntaws. Cov neeg no muaj qhov pheej hmoo ua rau tuag sai.

Cov teeb meem tshwm sim ntawm EDS suav nrog:

  • Mob sib koom tes mob
  • Ntxov mob thaum ntxov
  • Ua tsis raug mob kev phais kom kaw (lossis pluaj tawv muag tawm)
  • Kev puas tsuaj ua ntej ntawm daim nyias nyias thaum lub cev xeeb tub
  • Kev tawg ntawm cov hlab ntsha loj, suav nrog lub pob txha caj qaum aortic aneurysm (tsuas yog hauv EDS vascular)
  • Kev puas tsuaj ntawm ib qho khoom siv khoob lug xws li lub tsev menyuam lossis txoj hnyuv (tsuas yog hauv EDS vascular)
  • Kev khawb qhov muag ntawm qhov muag

Hu kev teem caij nrog koj tus neeg muab kev pab yog tias koj muaj tsev neeg muaj keeb kwm ntawm EDS thiab koj muaj kev txhawj xeeb txog koj qhov kev pheej hmoo lossis npaj pib tsev neeg.

Hu teem sijhawm nrog koj tus kws khomob yog koj lossis koj tus menyuam muaj EDS.

Kev tawm tswv yim rau caj ces raug pom zoo rau cov niam txiv yav tom ntej nrog tsev neeg zaj keeb kwm ntawm EDS. Cov neeg npaj yuav pib tsev neeg yuav tsum paub txog hom EDS uas lawv muaj thiab nws hom kev yuav kis li cas rau cov menyuam li cas. Qhov no tuaj yeem txiav txim siab los ntawm kev sim thiab ntsuas uas tau qhia los ntawm koj tus kws khomob lossis tus kws sab laj tswv yim kuaj mob.


Txheeb xyuas ib qho kev pheej hmoo tseem ceeb ntawm kev noj qab haus huv tuaj yeem pab tiv thaiv kev mob nyhav los ntawm kev soj ntsuam thiab kev hloov pauv hauv lub neej.

Krakow D. Cov kab mob zoo ntawm cov nqaij mos sib txuas. Hauv: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, eds. Kelly thiab Firestein Cov Ntawv Phau Ntawv ntawm RheumatologyCov. 10 tus ed. Philadelphia, PA: Elsevier; 2017: chap 105.

Pyeritz RE. Inherited kab mob ntawm cov nqaij mos. Hauv: Goldman L, Schafer AI, eds. Goldman-Cecil TshuajCov. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016: chap 260.

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