Ukushoda kwe-lipoprotein lipase ejwayelekile

Ukushoda kwe-lipoprotein lipase okujwayelekile yiqembu lezifo ezingavamile zofuzo lapho umuntu engenayo iprotheni edingekayo ukuze adilize ama-molecule amafutha. Lesi sifo senza inani elikhulu lamafutha ukuthi lakhe egazini.

Ukushoda kwe-lipoprotein lipase ejwayelekile kudalwa yisakhi sofuzo esidlulayo esidluliselwa emindenini.

Abantu abanalesi simo abanayo i-enzyme ebizwa nge-lipoprotein lipase. Ngaphandle kwale enzyme, umzimba awukwazi ukwehlisa amanoni ekudleni okugayiwe. Izinhlayiya zamafutha ezibizwa ngama-chylomicrons zakha egazini.

Izici zobungozi zifaka umlando womndeni wokushoda kwe-lipoprotein lipase.

Isimo ngokuvamile siqala ukubonwa ngesikhathi sobuntwana noma ebuntwaneni.

Izimpawu zingafaka noma yikuphi okulandelayo:

• Ubuhlungu besisu (bungabonakala njenge-colic ezinganeni)
• Ukuphelelwa isifiso sokudla
• Isicanucanu, ukuhlanza
• Ubuhlungu emisipheni nasemathanjeni
• Isibindi esikhulisiwe nobende
• Ukwehluleka ukuchuma ezinganeni
• Amafutha afakwa esikhumbeni (xanthomas)
• Amazinga aphezulu e-triglyceride egazini
• I-retina ephaphathekile nemithambo yegazi enemibala emhlophe kuma-retina
• Ukuvuvukala okungapheli kwamanyikwe
• Ukuphuzi kwamehlo nesikhumba (i-jaundice)

Umhlinzeki wezokunakekelwa kwempilo uzokwenza ukuhlolwa komzimba abuze ngezimpawu.

Ukuhlolwa kwegazi kuzokwenziwa ukuhlola amazinga e-cholesterol ne-triglyceride. Kwesinye isikhathi, ukuhlolwa kwegazi okukhethekile kwenziwa ngemuva kokuthi unikezwe izinciphisi zegazi ngomthambo. Lokhu kuhlolwa kubheka umsebenzi we-lipoprotein lipase egazini lakho.

Ukuhlolwa kofuzo kungenziwa.

Ukwelashwa kuhlose ukulawula izimpawu namazinga we-triglyceride egazi ngokudla okunamafutha aphansi kakhulu. Umhlinzeki wakho uzoncoma ukuthi ungadli ngaphezu kwama-gramu angama-20 amafutha ngosuku ukuvimbela izimpawu ukuthi zingabuyi.

Amagremu angamashumi amabili amafutha alingana nokukodwa kokulandelayo:

• Izingilazi ezimbili ezingama-8-ounce (240 milliliters) obisi lonke
• 4 amathisipuni (9.5 amagremu) imajarini
• Ama-ounces ama-4 (113 amagremu) wenyama

Ukudla okujwayelekile kwaseMelika kunamafutha afinyelela kuma-45% wama-calories aphelele. Amavithamini ancibilikiswa ngamafutha u-A, D, E, no-K kanye nezengezo zamaminerali kunconywa abantu abadla ukudla okunamafutha aphansi kakhulu. Ungahle ufune ukuxoxa ngezidingo zakho zokudla nomhlinzeki wakho kanye nodokotela wezokudla obhalisiwe.

I-Pancreatitis ehlobene nokushoda kwe-lipoprotein lipase iphendula ekwelashweni kwalesi sifo.

Lezi zinsizakusebenza zinganikeza imininingwane eminingi ngokushoda komndeni we-lipoprotein lipase:

• Inhlangano Kazwelonke Yezinkinga Ezingajwayelekile - rarediseases.org/rare-diseases/familial-lipoprotein-lipase-deficiency
• Isethenjwa Sasekhaya se-NIH - ghr.nlm.nih.gov/condition/familial-lipoprotein-lipase-deficiency

Abantu abanalesi simo abalandela ukudla okunamafutha aphansi kakhulu bangaphila babe ngabantu abadala.

I-Pancreatitis kanye neziqephu eziphindaphindwayo zobuhlungu besisu zingakhula.

AmaXanthomas awavame ukuba buhlungu ngaphandle kokuthi agcotshwe kakhulu.

Shayela umhlinzeki wakho ukuze ahlolwe uma othile emndenini wakho enesifo se-lipoprotein lipase. Ukwelulekwa ngofuzo kunconyelwa noma ngubani onomlando womndeni walesi sifo.

Akukho ukuvimbela okwaziwayo kwalesi sifo esingajwayelekile, esizuzwe njengefa. Ukuqwashiswa kwezingozi kungavumela ukutholwa kusenesikhathi. Ukulandela ukudla okunamafutha aphansi kakhulu kungathuthukisa izimpawu zalesi sifo.

Thayipha i-hyperlipoproteinemia; I-chylomicronemia ejwayelekile; Ukushoda komndeni kwe-LPL

• Isifo semithambo yegazi

UGenest J, ukuphazamiseka kweLibby P. Lipoprotein nesifo senhliziyo. Ku: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, ama-eds. Isifo Senhliziyo SikaBraunwald: Incwadi Yemithi Yezinhliziyo Nemithambo. Umhla ka-11. IPhiladelphia, PA: Elsevier; 2019: isahluko 48.

Semenkovich CF, Goldberg AC, Goldberg IJ. Ukuphazamiseka kwe-lipid metabolism. Ku: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, abahleli. Incwadi kaWilliams ye-Endocrinology. Umhlaka 13. IPhiladelphia, PA: Elsevier; 2016: isahluko 37.