Wannan Ƙananan Yanayi Yana Da wuya ga Jiki don Yarda Fat
Abetalipoproteinemia (wanda aka sani da ciwo na Bassen-Kornzweig) wani cuta ne wanda yake da nasaba da yadda ake yin ƙwayoyi da kuma amfani dasu cikin jiki. Jikunanmu na bukatar ƙwayoyi don kula da jijiyoyin lafiya, tsokoki, da narkewa. Kamar man fetur da ruwa, ƙwayoyi ba zasu iya tafiya a jikin jikinmu kadai ba. Maimakon haka, sun haɗa da sunadarai na musamman da ake kira lipoproteins kuma suna tafiya zuwa duk inda ake bukata.
Yadda Abetalipoproteinemia ke shafan lipoproteins
Saboda maye gurbin kwayoyin halitta, mutane da abetalipoproteinemia ba su samar da sunadaran da ake buƙatar yin lipoproteins. Ba tare da isasshen lipoproteins ba, ba za a iya yin amfani da ƙwayoyi ba daidai ba ko tafiya zuwa inda ake bukata. Wannan zai haifar da matsalolin lafiya mai kyau wanda zai iya rinjayar ciki, jini, tsokoki, da sauran tsarin jiki.
Saboda matsalolin da ake haifar da abetalipoproteinemia, alamun yanayi ana ganin su a lokacin jariri. Mace sukan kara yawanci - game da kashi 70 cikin 100 - fiye da mata. Yanayin shine yanayin haɗin gwiwa, wanda ke nufin dukkan iyayensu dole ne suna da mummunan nau'in MTTP don yaron su gaji shi. Abetalipoproteinemia yana da matukar wuya, kawai 100 ne aka ruwaito.
Alamai da cututtukan cututtuka
Baban da aka haife su tare da abetalipoproteinemia suna da matsalolin ciki saboda rashin iyawar da za su yi amfani da su yadda ya dace. Ƙungiyoyin bakuna suna da mawuyaci kuma suna iya zama launin fatar launin fata da ƙanshi.
Yara da ciwon abetalipoproteinemia kuma zasu iya shawo kan cutar, zazzabin ciki, shafewa, da wahalar samun karfin ko girma (wani lokaci ma ana sani da rashin cin nasara).
Wadanda ke da yanayin suna da matsalolin da suka shafi bitamin da aka adana a cikin bitamin A, E, da K. Kwayoyin cututtuka da lalacewar ƙwayoyi da kuma bitamin sunadaran sunada karuwa a cikin shekaru goma na rayuwa.
Wadannan sun haɗa da:
- Rashin haɗari: Wannan ya hada da matsalolin da zazzabi da zafin jiki da tabawa - musamman ma a cikin hannaye da ƙafa (hypesthesia)
- Balarin tafiya (ataxia): kimanin kashi 33 cikin 100 na yara zasu fuskanci wahalar tafiya ta hanyar lokacin da suka kai shekaru 10. Ataxia yana ci gaba da muni fiye da lokaci.
- Maganin motsa jiki: Tremors, girgiza (chorea), damuwa da damuwa ga abubuwa (dysmetria), maganganun magana (dysarthria)
- Matsarar matsalolin: Rashin rauni, ragewa (ƙanƙancewa) na tsokoki a bayan da ke haifar da spine to curve (kyphoscoliosis)
- Matsalar jini: Ƙananan ƙarfe ( anemia ), matsaloli tare da clotting, mahaukaciyar jan jini (acanthocytosis)
- Matsalar idanu: Hasken duhu, hangen nesa, matsaloli tare da kula ido (ophthalmoplegia), cataracts
Samun Abetalipoproteinemia ganewar asali
Abetalipoproteinemia za a iya gano ta hanyar samfurori. Ayyukan baka, idan an gwada su, za su nuna babban kitsen kitsen tun lokacin da aka kawar da kiba maimakon amfani da jiki. Yin gwajin jini zai iya taimakawa wajen gano asalin yanayin. Ana iya ganin mahaukacin jini na jan jini a abetalipoproteinemia a ƙarƙashin kwayar microscope. Har ila yau akwai matakan ƙananan matakan kamar cholesterol da triglycerides cikin jini.
Idan yaronka yana da abetalipoproteinemia, gwaje-gwaje don yin katsewar lokaci da matakan ƙarfe zai dawo da mawuyacin hali. Nazarin ido zai iya nuna kumburi na bayan ido (retinitis). Yin gwaji akan ƙarfin tsoka da sabani na iya samun sakamako mawuyacin.
Yin maganganun ta hanyar cin abinci
An shirya wani abinci na musamman ga mutanen dake da abetalipoproteinemia. Akwai buƙatu da yawa a cikin abincin abincin, ciki har da kaucewa cin wasu nau'in ƙwayoyin cuta (dogayen triglycerides mai tsawo) don son cin sauran nau'ikan (matsanancin sarkar triglycerides). Wani abin da ake buƙata yana ƙara ƙwayoyin karin bitamin da ke dauke da bitamin A, E, da K, da baƙin ƙarfe.
Wani mai cin abinci mai gina jiki tare da maganin yanayi na iya taimaka maka tsara tsarin abinci wanda zai dace da bukatun yaranka na musamman.
Sources :
Berriot-Varoqueaux, N., Aggerbeck, LP, Samson-Bouma, M., & Wetterau, JR (2000). Matsayin da kwayar cutar canza kwayar triglyceride ta samu a abetalipoproteinemia. Annu Rev Nutr, vol. 20, shafi na 663-697.
Gregg, RE, Wetterau, JR, Berriot-Varoqueaux, N., Aggerbeck, LP, & Samson-Bouma, M. (1994). Tushen kwayoyin abetalipoproteinemia. Curr Opin Lipidol, kundi. 5, a'a. 2, shafi na 81-86.
Rader, DJ, Brewer, HB, Jr., Gregg, RE, Wetterau, JR, Berriot-Varoqueaux, N., Aggerbeck, LP, Samson-Bouma, M., & Wetterau, JR (1993). Abetalipoproteinemia: Sabbin abubuwan da ake ganin su a cikin taron lipoprotein da kuma bitamin E metabolism daga cututtukan kwayoyin cututtuka. JAMA, vol. 270, babu. 7, shafi na 865-869.
Stone, NJ, Blum, CB, & Winslow, E. (1998). Pathophysiology na hyperlipoproteinemias. A Gudanarwa na Lipids a Practical Practice. Akwai shi a kan layi a Medscape.
Medline Plus. Bassen-Kornzweig ciwo. (2013)