Matenda a Ehlers-Danlos

Matenda a Ehlers-Danlos (EDS) ndi gulu la zovuta zobadwa nazo zomwe zimadziwika ndi ziwalo zotayirira kwambiri, khungu lotambasula (hyperelastic) lomwe limaphwanya mosavuta, komanso kuwonongeka mosavuta mitsempha yamagazi.

Pali mitundu isanu ndi umodzi yayikulu komanso mitundu isanu yaying'ono ya EDS.

Kusintha kosiyanasiyana kwa majini (masinthidwe) kumayambitsa mavuto a collagen. Izi ndizomwe zimapereka mphamvu ndi kapangidwe ka:

• Khungu
• Fupa
• Mitsempha yamagazi
• Ziwalo zamkati

Collagen yachilendo imabweretsa zisonyezo zomwe zimakhudzana ndi EDS. Mwa mitundu ina ya matendawa, kuphulika kwa ziwalo zamkati kapena mavavu amtima wosazolowereka kumatha kuchitika.

Mbiri ya banja imakhala pachiwopsezo nthawi zina.

Zizindikiro za EDS ndizo:

• Ululu wammbuyo
• Kuphatikizika kawiri
• Khungu lowonongeka, lotupa, ndi lotambasula
• Zipsera zosavuta komanso machiritso osauka
• Mapazi apansi
• Kuchulukitsa kwa ziwalo, mafupa akutuluka, nyamakazi yoyambirira
• Kusokonezeka pamodzi
• Ululu wophatikizana
• Kutuluka msanga kwa nembanemba panthawi yapakati
• Khungu lofewa kwambiri komanso losalala
• Mavuto masomphenya

Kuyesedwa ndi wothandizira zaumoyo kumatha kuwonetsa:

• Pamaso pa diso (cornea)
• Kuchulukitsa kophatikizana kophatikizana
• Mitral valve mu mtima samatseka mwamphamvu (mitral valve prolapse)
• Matenda a chingamu (periodontitis)
• Kutuluka kwa matumbo, chiberekero, kapena diso (kumangowoneka m'mitsempha ya EDS, yomwe ndi yosowa)
• Khungu lofewa, lowonda, kapena lotambasula kwambiri

Kuyesa kwa kupeza EDS kumaphatikizapo:

• Kulemba kwa Collagen (kochitidwa pachitsanzo cha khungu)
• Kuyesa kusintha kwa majini a Collagen
• Echocardiogram (mtima ultrasound)
• Lysyl hydroxylase kapena oxidase zochita (kuti muwone mapangidwe a collagen)

Palibe mankhwala enieni a EDS. Mavuto ndi zizindikilo zaumwini zimayesedwa ndikusamalidwa moyenera. Kuchita masewera olimbitsa thupi kapena kuwunika kwa dokotala wodziwa bwino za mankhwala ochiritsira nthawi zambiri amafunikira.

Izi zitha kupereka zambiri pa EDS:

• National Organisation of Rare Disways - rarediseases.org/rare-diseases/ehlers-danlos-syndrome
• US National Library of Medicine, Buku Lofotokozera za Genetics - ghr.nlm.nih.gov/condition/ehlers-danlos-syndrome

Anthu omwe ali ndi EDS amakhala ndi moyo wathanzi. Luntha ndi lachibadwa.

Omwe ali ndi mitsempha yosawerengeka ya EDS ali pachiwopsezo chachikulu chotuluka chiwalo chachikulu kapena chotengera magazi. Anthu awa ali pachiwopsezo chachikulu chofa mwadzidzidzi.

Mavuto omwe angakhalepo a EDS ndi awa:

• Zowawa zophatikizana
• Matenda a nyamakazi oyambirira
• Kulephera kwa mabala a opaleshoni kutseka (kapena kumangirira)
• Kutuluka msanga kwa nembanemba panthawi yapakati
• Kung'ambika kwa ziwiya zazikulu, kuphatikizapo kuphulika kwa aortic aneurysm (kokha mu EDS)
• Kutuluka kwa chiwalo chobowoka monga chiberekero kapena matumbo (m'mitsempha ya EDS yokha)
• Kung'ambika kwa diso la diso

Funsani nthawi yokumana ndi omwe amakupatsani ngati muli ndi banja la EDS ndipo mukudandaula za chiwopsezo chanu kapena mukukonzekera kuyambitsa banja.

Itanani kuti mudzakumane ndi omwe amakupatsani ngati inu kapena mwana wanu muli ndi matenda a EDS.

Upangiri wa chibadwa umalimbikitsidwa kwa oyembekezera omwe ali ndi mbiri yabanja ya EDS. Omwe akukonzekera kuyambitsa banja ayenera kudziwa mtundu wa EDS omwe ali nawo komanso momwe amawupatsira ana. Izi zitha kutsimikiziridwa kudzera pakuyesa ndikuwunika komwe woperekayo kapena mlangizi wamtundu wanu amapereka.

Kuzindikira zovuta zilizonse zathanzi kumatha kuthandizira kupewa zovuta zazikulu mwa kuwunika mosamala ndikusintha moyo.

Krakow D. Matenda obwera chifukwa cha minofu yolumikizana. Mu: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, olemba. Kelly ndi Firestein's Bookbook of Rheumatology. 10th ed. Philadelphia, PA: Elsevier; 2017: mutu 105.

Pyeritz RE. Matenda obadwa nawo a minofu yolumikizana. Mu: Goldman L, Schafer AI, olemba. Mankhwala a Goldman-Cecil. Wolemba 25. Philadelphia, PA: Elsevier Saunders; 2016: chap 260.