Iyalan lipoprotein lipase

Rashin lipoprotein lipase na iyali wani rukuni ne na rikice-rikicen ƙwayoyin cuta wanda mutum ba shi da furotin da ake buƙata don lalata ƙwayoyin mai. Rashin lafiyar na haifar da mai mai yawa don taruwa a cikin jini.

Arancin lipoprotein lipase na iyali yana faruwa ne ta hanyar kwayar halitta mai tawaya wanda aka yada ta cikin iyalai.

Mutanen da ke da wannan matsalar ba su da enzyme da ake kira lipoprotein lipase. Idan ba tare da wannan sinadarin enzyme ba, jiki baya iya fasa kitse daga abinci mai narkewa. Particleswayoyin kitse waɗanda ake kira chylomicrons suna haɗuwa a cikin jini.

Abubuwan haɗarin sun haɗa da tarihin iyali na ƙarancin lipoprotein lipase.

Yanayin yawanci ana fara ganin sa yayin yarinta ko yarinta.

Kwayar cutar na iya haɗawa da ɗayan masu zuwa:

• Ciwon ciki (na iya bayyana kamar maƙarƙashiya a cikin jarirai)
• Rashin ci
• Tashin zuciya, amai
• Jin zafi a cikin tsokoki da kasusuwa
• Liverara hanta da baƙin ciki
• Rashin yin nasara a jarirai
• Adadin mai a cikin fata (xanthomas)
• Matakan triglyceride masu girma a cikin jini
• Ruwan idanuwa masu launuka da launuka masu launin fari a cikin kwayar ido
• Ciwon kumburi na kullum
• Raunin idanu da fata (jaundice)

Mai ba da sabis na kiwon lafiya zai yi gwajin jiki kuma ya yi tambaya game da alamun.

Za ayi gwajin jini don duba matakan cholesterol da triglyceride. Wani lokaci, ana yin gwajin jini na musamman bayan an ba ku magungunan kashe jini ta wata jijiya. Wannan gwajin yana neman aikin lipoprotein lipase a cikin jininka.

Ana iya yin gwajin kwayar halitta.

Jiyya yana nufin sarrafa alamun da matakan triglyceride na jini tare da abinci mai ƙarancin mai. Mai ba ku sabis zai iya ba da shawarar cewa ku ci fiye da gram 20 na mai a kowace rana don hana alamun bayyanar daga dawowa.

Giram ashirin na mai daidai yake da ɗayan masu zuwa:

• Gilashi biyu na awo 8 (mililita 240) na madarar madara
• Cokali 4 (gram 9.5) na margarine
• Oza 4 (gram 113) na nama

Matsakaicin abincin Amurka yana da mai mai ƙanshi har zuwa 45% na yawan adadin kuzari. Ana ba da shawarar bitamin A, D, E, da K da kuma na ma'adinai don mutanen da suke cin abinci mai ƙarancin mai. Kuna so ku tattauna bukatun abincinku tare da mai ba da sabis da kuma likitan abinci mai rijista.

Pancreatitis wanda ke da alaƙa da rashi na lipoprotein lipase yana amsar magunguna don wannan cuta.

Waɗannan albarkatun na iya samar da ƙarin bayani game da rashi lipoprotein lipase na iyali:

• Nationalungiyar forasa don Rare Rashin Lafiya - rarediseases.org/rare-diseases/familial-lipoprotein-lipase-deficiency
• NIH Tsarin Gida na Gida - ghr.nlm.nih.gov/condition/familial-lipoprotein-lipase-deficiency

Mutanen da ke da wannan yanayin waɗanda ke bin abinci mai ƙarancin mai na iya rayuwa cikin girma.

Pancreatitis da lokuta masu maimaitarwa na ciwon ciki na iya haɓaka.

Xanthomas ba yawanci mai zafi ba sai dai idan an shafa su da yawa.

Kira mai ba ku sabis don yin gwaji idan wani a cikin danginku yana da ƙarancin lipoprotein lipase. Ana ba da shawara kan kwayoyin halitta don duk wanda ke da tarihin iyali na wannan cuta.

Babu wata sananniyar rigakafin wannan rashin lafiyar, gado. Sanin haɗari na iya ba da damar ganowa da wuri. Biyan abinci mai mai mai yawa na iya inganta alamun wannan cuta.

Rubuta I hyperlipoproteinemia; Chylomicronemia na dangi; Iyalan LPL na rashi

• Ciwon jijiyoyin jini

Genest J, Libby P. Rashin lafiyar Lipoprotein da cututtukan zuciya. A cikin: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, eds. Braunwald na Ciwon Zuciya: Littafin rubutu na Magungunan zuciya da jijiyoyin jini. 11th ed. Philadelphia, PA: Elsevier; 2019: babi na 48.

Semenkovich CF, Goldberg AC, Goldberg IJ. Rashin lafiya na maganin ƙwayar cuta. A cikin: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. Littafin Williams na Endocrinology. 13th ed. Philadelphia, PA: Elsevier; 2016: babi na 37.