Matenda Obadwira Amayambitsa Kuopsa Kwambiri kwa dzuwa
Xeroderma pigmentosum (XP) ndi matenda obadwa nawo omwe amachititsa kuwala kwambiri kwa ultraviolet (UV) kuwala. Kuwala kwa UV kumapweteka ma genetic ( DNA ) m'maselo ndipo kumasokoneza maselo ambiri. Kaŵirikaŵiri, DNA yowonongeka imakonzedwa ndi thupi, koma mawonekedwe okonza DNA a anthu omwe ali ndi XP sagwira ntchito bwino. Mu XP, DNA yowonongeka imamangirira ndipo imavulaza maselo a thupi, makamaka pakhungu ndi maso.
Xeroderma pigmentosum imachokera muyeso yodabwitsa ya autosomal . Zimakhudza amuna ndi akazi amitundu yonse. XP ikuyembekezeka kuti ichitike m'modzi mwa anthu 1,000,000 ku United States. M'mayiko ena, monga North Africa (Tunisia, Algeria, Morocco, Libya, Egypt), Middle East (Turkey, Israel, Syria), ndi Japan, XP imachitika kawirikawiri.
Zizindikiro za Khungu
Anthu omwe ali ndi xeroderma pigmentosum amawona zizindikiro za khungu komanso kusintha kosakhala dzuwa. Izi ziphatikizapo:
- kutentha kwa dzuwa kwakukulu komwe kungaphatikizepo kuthamanga patangopita kanthawi kochepa chabe dzuwa
- Kutentha kwa dzuwa kumatenga nthawi yaitali kuposa momwe amayembekezera, nthawi zina kwa milungu ingapo
- Kukula kosavuta kwambiri ali wamng'ono
- khungu loopsya (xerosis)
- Kukula kwa khungu kofiira (dzuwa keratoses)
- malo osadetsedwa
- Kukula kwa mitundu itatu ya khansa yapakhungu yofanana: basal cell carcinoma, squamous cell carcinoma, kapena melanoma
Dzina lakuti "xeroderma pigmentosum" limatanthauza "khungu louma." Kuwonetsera kwa dzuwa pa nthawi kumapangitsa khungu kukhala mdima, wouma, ndi zikopa. Ngakhale kwa ana, khungu limawoneka ngati khungu la alimi ndi oyendetsa sitima omwe akhala padzuwa kwa zaka zambiri.
Anthu omwe ali ndi xeroderma pigmentosum omwe ali ndi zaka zoposa 20 amakhala ndi chiopsezo choposa khansa ya khungu koposa nthawi 1,000 kuposa anthu omwe alibe matendawa.
Khansara yoyamba ya khungu ingakhalepo mwana asanakhale ndi XP ali ndi zaka 10, ndipo khansa zambiri za khungu zingakonzekere mtsogolomu. Mu XP, khansara ya khungu imafala nthawi zambiri pamaso, milomo, maso, ndi pamwamba pa lilime.
Zizindikiro za Maso
Anthu omwe ali ndi xeroderma pigmentosum amakhalanso ndi zizindikiro za maso ndi kusintha kosakhala dzuwa. Izi ziphatikizapo:
- Maso amavutika kwambiri ndi dzuwa
- Maso akukwiya mosavuta ndikukhala magazi
- ntchentche, yotembenukira m'mphepete mwa chikopa
- Kutupa (keratitis) ya cornea, mbali yoyera ya kutsogolo kwa diso lomwe limatulutsa kuwala
- kuthamanga kwa cornea
- mdima wa khungu la maso; Nthawi zina ma eyelashes amatha
Zizindikiro Zamadzimadzi
Pafupifupi 20 mpaka 30 peresenti ya anthu omwe ali ndi xeroderma pigmentosum amakhalanso ndi zizindikiro zamanjenje monga:
- Kutaya kwakumva kukuipiraipira pa nthawi
- kusagwirizana bwino
- minofu yapastiki
- mutu wamtundu wochuluka kuposa wamba (wotchedwa microcephaly)
- Kuwonongeka kwa nzeru komwe kumaipiraipira pa nthawi
- kugwidwa
Matenda a mitsempha amatha kukhalapo kuyambira ali wakhanda, kapena sangathe kuwonekera kuyambira ali mwana kapena unyamata. Anthu ena omwe ali ndi XP amangokhala ndi zizindikiro zochepa za matenda amtunduwu poyamba, koma zizindikiro zimakhala zovuta kwambiri pakapita nthawi.
Kudziwa
Matendawa a xeroderma pigmentosum amachokera pakhungu, maso, ndi zizindikiro zamatenda (ngati alipo). Mayeso apadera opangidwa pamagazi kapena chithunzi cha khungu angayang'ane kachilombo ka DNA kukonza mu XP. Mayesero angachitidwe kuti athetse mavuto ena omwe angayambitse zizindikiro zofanana, monga Cockayne syndrome, trichothiodystrophy, matenda a Rothmund-Thomson, kapena matenda a Hartnup.
Chithandizo
Palibe chithandizo cha xeroderma pigmentosum, kotero mankhwala akuyang'ana pa mavuto aliwonse omwe alipo ndi kupeŵa mavuto amtsogolo kuti asapitike. Mankhwala amtundu uliwonse kapena zilonda zokayikitsa ayenera kuchiritsidwa kapena kuchotsedwa ndi katswiri wa khungu ( dermatologist ).
Katswiri wa maso ( ophthalmologist ) akhoza kuthana ndi vuto lililonse la maso lomwe likuchitika.
Chifukwa chakuti kuwala kwa UV komwe kumawononga, mbali yaikulu yothetsera mavuto imateteza khungu ndi maso ku dzuwa. Ngati wina yemwe ali ndi XP ayenera kutuluka panja masana, ayenera kuvala manja aatali, mathalauza, magolovesi, chipewa, magalasi okhala ndi zishango, ndi sunscreen. Pakhomo kapena m'galimoto, mawindo ayenera kutsekedwa kuti alephere kuwala kwa dzuwa kuchokera ku dzuwa (ngakhale kuti kuwala kwa UVA kungathe kudutsa, kotero munthu ayenera kuvala kwathunthu). Ana omwe ali ndi XP sayenera kusewera panja masana.
Mitundu ina ya kuwala kwa mkati (monga halogen nyali) ikhoza kuchotsanso kuwala kwa UV. Mafakitale apansi a kuwala kwa dzuwa m'nyumba, sukulu, kapena malo a ntchito ayenera kudziwika ndi kuthetsedwa, ngati n'kotheka. Anthu omwe ali ndi XP amatha kuvala zowonjezera za dzuwa m'nyumba kuti ateteze motsutsana ndi magwero osadziwika a kuwala kwa UV.
Zina zofunika pakupewa mavuto ndi kafukufuku wa khungu kawirikawiri, kuyezetsa maso, ndi kuyesa koyambirira ndi mankhwala kwa mavuto a dongosolo la mitsempha monga kumva kutaya.
Zotsatira:
"Kumvetsa Xeroderma Pigmentosum." Information Patient Information. 2006. Clinical Center, National Institutes of Health.
Kraemer, Kenneth. "Xeroderma Pigmentosum." GeneReviews. 22 Apr 2008. GeneTests.