Chizoloŵezichi Chimachititsa Kuti Chikhale Chovuta Kuti Thupi Lidye Mafuta
Abetalipoproteinemia (yemwenso imatchedwa Bassen-Kornzweig syndrome) ndi matenda omwe anabadwa nawo omwe amakhudza momwe mafuta amapangidwira ndi kugwiritsidwa ntchito m'thupi. Matupi athu amafunikira mafuta kuti asunge mitsempha yathanzi, minofu, ndi chimbudzi. Mofanana ndi mafuta ndi madzi, mafuta sangathe kuyenda mozungulira matupi athu okha. Mmalo mwake, amagwiritsa ntchito mapuloteni apadera otchedwa lipoproteins ndikupita kulikonse komwe amafunikira.
Mmene Abetalipoproteinemia Amakhudzira Lipoproteins
Chifukwa cha kusintha kwa chibadwa, anthu okhala ndi abetalipoproteinemia samapanga mapuloteni ofunikira kuti apange lipoproteins. Popanda lipoproteins zokwanira, mafuta sangathe kudyedwa bwino kapena kupita kumalo kumene akufunikira. Izi zimayambitsa matenda aakulu omwe angakhudze mimba, magazi, minofu, ndi machitidwe ena a thupi.
Chifukwa cha mavuto omwe amabwera chifukwa cha abetalipoproteinemia, zizindikiro za mikhalidwe nthawi zambiri zimawoneka akhanda. Amuna amakhudzidwa kwambiri - pafupifupi 70% peresenti - kuposa akazi. Matendawa ndi chikhalidwe cholandira choloŵa, chomwe chikutanthauza kuti makolo onse ayenera kukhala ndi MTTP jala la mwana wawo kuti alitenge. Abetalipoproteinemia ndi yosavomerezeka, koma milandu yokwana 100 yakhala ikudziwika.
Zizindikiro ndi Zizindikiro za Matendawa
Ana obadwa ndi abetalipoproteinemia ali ndi vuto la m'mimba chifukwa cholephera kuyaka mafuta. Kusinthasintha kwa thupi kumakhala kosazolowereka ndipo kungakhale kofiira komanso kobisika.
Ana omwe ali ndi abetalipoproteinemia angathenso kusanza, kutsegula m'mimba, kuphulika, komanso kuvutika kulemera kapena kukula (nthawi zina kumatchedwa kuti kulephera kukula).
Anthu omwe ali ndi vutoli amakhalanso ndi mavuto okhudzana ndi mavitamini omwe amasungidwa m'mafuta - mavitamini A, E, ndi K. Zizindikiro zotere chifukwa cha kusowa kwa mafuta ndi mavitamini osungunula mafuta nthawi zambiri zimakula mkati mwa zaka khumi zoyambirira za moyo.
Izi ziphatikizapo:
- Kusokonezeka kolakwika: Izi zikuphatikizapo mavuto ndi kutentha kutentha ndi kukhudza - makamaka m'manja ndi m'mapazi (hypesthesia)
- Kuyenda movutikira (ataxia): Pafupifupi 33 peresenti ya ana adzakhala ndi zovuta kuyenda pakapita zaka 10. Ataxia imakula kwambiri pakapita nthawi.
- Kusokonezeka kwa magulu: Kutupa, kugwedezeka (chorea), vuto lofikira zinthu (dysmetria), vuto loyankhula (dysarthria)
- Matenda a mitsempha: Kufooka, kuchepetseratu (kupangika) kwa minofu kumbuyo komwe kumayambitsa msana (kyphoscoliosis)
- Matenda a m'magazi : Kutsika kwachitsulo ( kuchepa kwa magazi ), vuto la kutsekemera, maselo ofiira amagazi (acanthocytosis)
- Mavuto a m'maso : Ukhungu wa usiku, maso osawona bwino, mavuto oyang'anitsitsa maso (ophthalmoplegia), nthendayi
Kupeza Matenda a Abetalipoproteinemia
Abetalipoproteinemia amatha kudziwika kudzera m'mabedi. Mavitamini, akayesedwa, amasonyeza mafuta ambiri chifukwa mafuta akuchotsedwa m'malo mogwiritsidwa ntchito ndi thupi. Mayeso a magazi angathandizenso kupeza matendawa. Maselo ofiira amagazi omwe sapezeka pamabetalipoproteinemia amatha kuwona pansi pa microscope. Padzakhalanso mafuta otsika kwambiri monga cholesterol ndi triglycerides m'magazi.
Ngati mwana wanu ali ndi abetalipoproteinemia, mayesero owonongera nthawi ndi zitsulo adzabwerenso. Kuyesedwa kwa maso kungasonyeze kutupa kumbuyo kwa diso (retinitis). Kuyesedwa kwa mphamvu ya minofu ndi zopangika zingakhale ndi zotsatira zosawonongeka.
Kuchiza Mkhalidwe Kudzera Chakudya
Chakudya chapadera kwa anthu okhala ndi abetalipoproteinemia chapangidwa. Pali zofunikira zambiri mu zakudya, kuphatikizapo kupewa kudya mitundu yambiri ya mafuta (mchere wambiri wa triglycerides) pofuna kudya zina (mitundu yambiri yamakina triglycerides). Chofunika china ndikuwonjezera mavitamini othandizira mavitamini A, E, ndi K, komanso chitsulo.
Wopatsa thanzi ndi mbiri yochizira matenda angakuthandizeni kupanga ndondomeko ya chakudya yomwe idzakwaniritse zosowa zapadera za mwana wanu.
Zotsatira :
Berriot-Varoqueaux, N., Aggerbeck, LP, Samson-Bouma, M., & Wetterau, JR (2000). Udindo wa puloteni yotumizira microsomal triglyceride mu abetalipoproteinemia. Annu Rev Nutriti, vol. 20, tsamba 663-697.
Gregg, RE, Wetterau, JR, Berriot-Varoqueaux, N., Aggerbeck, LP, & Samson-Bouma, M. (1994). Ma maselo a abetalipoproteinemia. Curr Opin Lipidol, vol. 5, ayi. 2, pp 81-86.
Rader, DJ, Brewer, HB, Jr., Gregg, RE, Wetterau, JR, Berriot-Varoqueaux, N., Aggerbeck, LP, Samson-Bouma, M., & Wetterau, JR (1993). Abetalipoproteinemia: Kumvetsetsa kwatsopano ku lipoprotein msonkhano ndi kupatsa mphamvu kwa vitamini E kuchokera ku matenda osadziwika achibadwa. JAMA, vol. 270, ayi. 7, tsamba 865-869.
Stone, NJ, Blum, CB, & Winslow, E. (1998). Pathophysiology ya hyperlipoproteinemias. Mu Utsogoleri wa Lipids mu Kuchita Zipatala. Ipezeka pa intaneti pa Medscape.
Medline Plus. Matenda a Bassen-Kornzweig. (2013)