Isifo seChylomicronemia

I-Chylomicronemia syndrome sisifo apho umzimba ungaphuli mafutha (lipids) ngokuchanekileyo. Oku kubangela amasuntswana amanqatha abizwa ngokuba zii-chylomicrons ukuba akhe egazini. Esi sifo sidluliselwa kwiintsapho.

I-Chylomicronemia syndrome inokwenzeka ngenxa yesifo esingaqhelekanga apho iprotein (enzyme) ebizwa ngokuba lipoprotein lipase (LpL) yaphukile okanye ilahlekile. Inokubangelwa kukungabikho kwento yesibini ebizwa ngokuba yi-apo C-II, eyenza i-LpL isebenze. I-LpL ihlala ifumaneka kumanqatha nakwizihlunu. Inceda ukwaphula iipids ezithile. Xa i-LpL ilahlekile okanye yaphukile, amasuntswana amanqatha abizwa ngokuba ziichylomicrons akha egazini. Olu lwakhiwo lubizwa ngokuba yi-chylomicronemia.

Ukusilela kwi-apolipoprotein CII kunye ne-apolipoprotein AV kunokubangela isifo. Kunokwenzeka ukuba kwenzeke xa abantu abachongiweyo ukuba babe ne-triglycerides ephezulu (njengaleyo ine-hyperlipidemia yosapho okanye usapho lwe-hypertriglyceridemia) iba nesifo seswekile, ukutyeba kakhulu okanye ukuvezwa amayeza athile.

Iimpawu zingaqala ebusaneni kwaye zibandakanya:

• Iintlungu zesisu ngenxa yokuphazamiseka (ukudumba kwepancreas).
• Iimpawu zomonakalo wemithambo-luvo, njengokulahleka kwemvakalelo ezinyaweni okanye emilenzeni, kunye nokulahleka kwememori.
• Iidipozithi ezimthubi zempahla enamafutha kulusu olubizwa ngokuba yiXanthomas. Oku kukhula kunokuvela ngasemva, iimpundu, iinyawo, okanye amadolo neengqiniba.

Uvavanyo lomzimba kunye neemvavanyo zingabonisa:

• Ukwandiswa kwesibindi kunye ne-spleen
• Ukudumba kwepancreas
• Amafutha afakwa phantsi kolusu
• Amanqatha anokubakho kwi-retina yeliso

Uluhlu olukhrim luya kubonakala xa igazi lijikeleza kumatshini waselebhu. Olu luhlu lubangelwa zii-chylomicrons egazini.

Inqanaba le-triglyceride liphezulu kakhulu.

Ukutya okungenamafutha, ngaphandle kotywala kuyafuneka. Kuya kufuneka uyeke ukuthatha amayeza athile anokwenza iimpawu zibe mandundu. Sukuyeka ukuthatha naliphi na iyeza ungakhange uqale uthethe nomboneleli wakho wezempilo. Iimeko ezinjengokuphela kwamanzi emzimbeni kunye nesifo sikashukela kunokwenza iimpawu zibe zibi nakakhulu. Ukuba ifunyenwe, le miqathango kufuneka inyangwe kwaye ilawulwe.

Ukutya okungenamafutha kunokunciphisa iimpawu.

Xa unganyangwa, ii-chylomicrons ezigqithileyo zinokukhokelela ekuphumeni kwepancreatitis. Le meko inokuba buhlungu kakhulu kwaye isongele nobomi.

Funa unyango ngokukhawuleza ukuba unentlungu esiswini okanye ezinye izilumkiso zepancreatitis.

Fowunela umnikezeli wakho ukuba unembali yobuqu okanye yosapho yamanqanaba aphezulu e-triglyceride.

Akukho ndlela yakuthintela umntu ekufumaneni le syndrome.

Ulwaphulo lweepoprotein lipase oluqhelekileyo; Isifo sehyperchylomicronemia esaziwayo, Uhlobo I hyperlipidemia

• I-hepatomegaly
• Xanthoma edolweni

UGenest J, uLibby P.Lipoprotein ukuphazamiseka kunye nesifo sentliziyo. Ku: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, ii-eds. Isifo sentliziyo seBraunwald: Incwadi yesifundo seMpilo yeNtliziyo. Ngomhla we-11. IPhiladelphia, PA: Elsevier; I-2019: isahluko 48.

URobinson JG. Ukuphazamiseka kwemetabolism yelipid. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahl. 195.