Matenda a mitsempha ya mitsempha (MSUD) ndi matenda a chibadwa omwe amachititsa kuti ubongo uwonongeke komanso kuwonongeka kwa dongosolo la mantha. Matenda a chibadwa omwe amachititsa MSUD amalephera kukhala ndi vuto la enzyme yotchedwa alpha-keto acid dehydrogenase, (BCKD), yomwe ndi yofunika kuti amino acids leucine, isoleucine, ndi valine iwonongeke. Popanda tizilombo toyambitsa matenda a BCKD, timadzi timeneti timene timayambitsa amino timapanga tizilombo toyambitsa matenda m'thupi.
MSUD imatchedwa dzina lakuti, nthawi zina pamene magazi amino acid ali pamwamba, mkodzo umatenga fungo lokoma.
Matenda a mitsempha a mitsempha amafika pafupifupi 1,000 pa 180,000 kubadwa kwa moyo ndipo amakhudza onse amuna ndi akazi. MSUD imakhudza anthu a mafuko onse, koma chiwerengero chapamwamba cha matendawa chikuchitika m'madera omwe muli maukwati ambiri, monga a Mennonite ku Pennsylvania (US).
Zizindikiro
Pali mitundu yambiri ya matenda a mitsempha ya maluwa. Kawiri kawiri kawiri kawiri kawiri ndi kawiri (7). Zizindikiro izi zingaphatikizepo izi:
- Kudyetsa kosauka
- Kuthamanga
- Kulemera kochepa
- Kuwonjezereka kwa khunyu (zovuta kuwuka)
- Makhalidwe amatentha fungo la shuga ku mkodzo
- Kusintha kwa minofu ya minofu, kuthamanga kwa minofu, ndi kugwidwa
Ngati sanatengedwe, ana awa adzafa ndi miyezi yoyamba ya moyo.
Anthu omwe ali ndi MSUD mwachangu, kachiwiri kawiri kawiri ka matendawa, amakula bwino koma pamene akuwonetsa zizindikiro za MSUD yachikale.
MSUD wapakati ndi mawonekedwe ochepa kwambiri. Anthu omwe ali ndi mtundu uwu ali ndi 3-30% pa mlingo wokwanira wa ma PC enzyme ya BCK, kotero zizindikiro zingayambe pa msinkhu uliwonse.
Mu MSUD yokhudzidwa ndi thiamine, anthu amasonyeza kusintha komwe atapatsidwa mankhwala owonjezera a thiamine .
Mtundu wosadziwika kwambiri wa matendawa ndi E3-yoperewera MSUD, momwe anthu ali ndi mavitamini owonjezera opangidwira.
Kudziwa
Ngati matenda a mitsempha yamapu amatsutsidwa chifukwa cha zizindikiro zakuthupi, makamaka mnofu wa mkodzo, kuyesa kwa magazi kwa amino acid kungatheke. Ngati alloisoleucine ikupezeka, matendawa amatsimikiziridwa. Kuwonetseratu kawirikawiri kwa ana obadwa kwa MSUD kumachitika m'mayiko angapo m'mayiko a US
Chithandizo
Chithandizo chachikulu cha matenda a mitsempha ya mitsempha amaletsa zakudya zamagulu atatu a amino acid leucine, isoleucine, ndi valine. Zakudya zimenezi zimayenera kukhala moyo wonse. Pali njira zambiri zamalonda ndi zakudya kwa anthu omwe ali ndi MSUD.
Chinthu chimodzi chokhudzidwa ndi chithandizo cha MSUD ndi chakuti pamene munthu wodwalayo akudwala, akuvulala, kapena akuchitidwa opaleshoni, matendawa akuwonjezereka. Anthu ambiri adzafuna chipatala panthawiyi kuti asamalire matenda aakulu.
Mwamwayi, kumatsatira zoletsa zakudya ndi kafukufuku wamachiritso nthawi zonse anthu omwe ali ndi matenda a mitsempha ya mitsempha akhoza kukhala ndi moyo wautali komanso wathanzi.
Zotsatira:
Bodamer, OA (2003). Matenda a mitsempha ya maluwa. eMedicine.
Morton DH, Strauss KA, Robinson DL, et al. Kuzindikira ndi kuchiza matenda a maple: kuphunzira kwa odwala 36. Matenda a TB; 109: 999.