Matenda a Batten, Zizindikiro, ndi Chithandizo

Matenda Opatsirana Okhudza Ana

Matenda a Batten ndi matenda ovuta kwambiri komanso oopsa omwe amakhudza dongosolo la manjenje. Ana ambiri amayamba kusonyeza zizindikiro pakati pa zaka zisanu ndi zisanu, pamene mwana wangwiro wathanzi angayambe kusonyeza zizindikiro za kugwidwa kapena masomphenya. Nthaŵi zambiri, zizindikiro zoyambirira ndizobisika, monga kukhumudwa, kuvutika kwa kuphunzira, ndi kuwonongeka kwa masomphenya.

Anthu ambiri omwe ali ndi matenda a Batten amafa ali ndi zaka khumi kapena makumi awiri.

Matenda a Batten ndi amodzi omwe amapezeka ndi karoid lipofuscinoses (NCLs). Poyamba, matenda a Batten ankaonedwa kuti ndi a NCL achinyamata, koma zaka zaposachedwapa, ana adokotala agwiritsa ntchito matenda a Batten kufotokoza zambiri za NCL.

Matenda a Batten ndi osowa kwambiri. Zikuoneka kuti zimakhudza 1 pa obadwa 50,000 ku United States. Ngakhale kuti zochitika zimachitika padziko lonse lapansi, matenda a Batten amapezeka m'madera ena kumpoto kwa Ulaya, monga Sweden kapena Finland.

Matenda a Batten ndi matenda a autosomal, omwe amatanthauza kuti amapezeka mwa mwana ngati makolo onse ali ndi jini la matendawa . Ngati mwana ali ndi kholo limodzi lobadwa ndi jini, mwanayo amawoneka ngati wothandizira ndipo amatha kupatsira mwana wakeyo jini, kuchititsa matenda a Batten ngati mnzakeyo ali ndi jini.

Zizindikiro

Pamene matendawa akupita, kuwonongeka kwa minofu, matenda akuluakulu a ubongo, kupweteka kwa ubongo , kuperewera kwa maso, ndi kugwidwa mtima kwaumtima .

Kudziwa

Chifukwa chakuti zizindikiro zoyambirira za matenda a Batten zimakhudza masomphenya, matenda a Batten nthawi zambiri amakayikiridwa nthawi yoyesedwa. Komabe, sizingatheke kupyolera mwa kuyesedwa kwa diso ndekha.

Matenda a Batten amapezeka chifukwa cha zizindikiro zomwe mwanayo akukumana nazo. Makolo kapena adokotala a mwana angazindikire kuti mwanayo ayamba kukulitsa mavuto a masomphenya kapena kugwidwa .

Maphunziro apadera a magetsi a maso, mawonekedwe oterewa owonetsedwa kapena electroretinogram (ERG), akhoza kuchitidwa. Kuwonjezera pamenepo, mayesero a matenda monga electroencephalogram (EEG, kuyang'ana ntchito yokopa) ndi kujambulira maginito (MRI, kufunafuna kusintha mu ubongo) zikhoza kuchitika. Chitsanzo cha khungu kapena minofu (yotchedwa biopsy ) ikhoza kuyang'aniridwa pang'onopang'ono kuti ayang'ane lipofuscins.

Chithandizo

Palibe chithandizo chomwe chilipo kuti chichiritsidwe kapena kuchepetsa kukula kwa Matenda a Batten, koma amachiritsidwa kuti athetse zizindikirozo ndikupangitsa mwana kukhala womasuka. Kugonjetsedwa kumatha kulamulidwa ndi mankhwala osokoneza bongo , ndipo mavuto ena azachipatala angathe kuchiritsidwa. Thandizo la thupi ndi ntchito lingathandize wodwalayo kugwiritsitsa kugwira ntchito mwakuthupi nthawi yaitali musanayambe atrophy.

Kafukufuku wina wasonyeza deta yam'mbuyomu kuti mavitamini C ndi E akhoza kuchepetsa matendawa, ngakhale kuti palibe mankhwala omwe amaletsa kupha.

Magulu othandizira monga Batten Matenda ndi Support Research Association amapereka chithandizo komanso mfundo zokhudzana ndi chithandizo ndi kafukufuku. Kukumana ndi mabanja ena omwe adutsa chinthu chomwecho kapena akukumana ndi magawo omwewo angakhale chithandizo chachikulu pamene akulimbana ndi matenda a Batten.

Kafukufuku wamankhwala akupitirizabe kuphunzira vutoli kuti akuyembekeza kupeza mankhwala abwino m'tsogolomu.

Zotsatira:

"Matenda a Batten". National Institute of Neurological Disorders ndi Stroke, 2015.

"Kodi matenda a Batten ndi otani?" Matenda a Batten Research and Support Association, 2015.