Wiskott-Aldrich syndrome: malongosoledwe a matenda

Wiskott-Aldrich syndrome ndi matenda otengera imagwira anyamata okha. Umaonekanso ana aang'ono ndi ambiri limadzetsa imfa mwa zaka zisanu khumi.

Kwa nthawi yoyamba matendawa limafotokonzera 1937 ndi German dokotala Wiscott. Iye akhayang'ana izo kwa abale atatu amene anavutika osaleka wamagazi m'mimba, chikanga ndi kutupa zonse khutu, ngakhale azichemwali anayi anali wathanzi kwathunthu. Kale mu 1954, American dokotala Aldrich maina anapeza kuti nthendayo tinatengera ndi X-zogwirizana recessive khalidwe.

matenda imafalikira anyamata ndi chonyamulira mutating DNA ndi akazi. Chinthu Hrs alibe mphamvu pa kuchuluka kwa vuto.

Zizindikiro za matendawa

syndrome Wiskott-Aldrich amagwirizana ndi kupatsidwa zinthu za m'mwazi achiyesa wotsikirapo. Mu zaka zoyambirira za moyo wa wodwalayo akusonyeza zizindikiro zotsatirazi: osauka magazi clotting, kulimbikira chikanga, wamagazi m'mimba. Kenako, pali immunodeficiency chachikulu. Chifukwa cha kusowa T ndi B lymphocytes munthu umamvera osiyanasiyana tizilombo ndi matenda bakiteriya. Immunodeficiency ana zoyambitsa matenda okhazikika khutu pakati, chibayo, sinusitis, wambulu ndi zina zambiri matenda aakulu. Ife amadwala matenda ndi chiopsezo apamwamba a khansa. Tikumbukenso kuti chotupa chija zambiri poyera akulu.

Ife amadwala matendawa syndrome Viskota-Aldrich kawirikawiri kokha hemorrhagic matenda, imbaenda ku matenda cholakwika. Zikatero, pokhapokha kusanthula DNA, imene n'zotheka kudziwa jini udindo matendawa mankhwala mankhwalawa.

diagnostics

• A magazi wathunthu kudziwa kuchepetsa chiwerengero cha othandiza magazi kuundana.
• Kuphunzira kuipitsa mbiri ya magazi pa akusowa structural cha othandiza magazi kuundana.
• kusanthula majini (anachitira kupeza masinthidwe jini udindo kapangidwe ka mapuloteni mu magazi).
• Kuona mlingo wa immunoglobulin.
• Kuchititsa matenda akalandire chithandizo, kudziwa syndrome Wiskott-Aldrich mimba msanga.
• Khama la mapuloteni mu milingo mwazi wa syndrome Wiskott-Aldrich.

chithandizo

Mwatsoka, sayansi yamakono sanapezebe mankhwala a matenda opuwalitsa. Amadziwika kuti maselo othandiza magazi kuundana adzaonongedwa mu ndulu, kotero odwala amene anapezeka ndi "Wiskott-Aldrich Syndrome", adachotsedwa ndi thupi. Ndipo odwala anayamba kumva bwino. kuikidwa zonse immunoglobulin ndi kuika mankhwala oyenerera komanso bwino chikhalidwe ambiri wodwala. Panopa mchitidwe kuyambitsa odwala kwa mafupa maselo tsinde wathanzi. Koma pamene njira imeneyi ikuchitika yekha ndi kuyesera. Komanso mabanja omwe adakumana syndrome izi m'banja, izo tikulimbikitsidwa kuti mayeso zonse zofunika patsogolo akukonzekera kukhala ndi pakati.