Kuva nehotera yakashata yakawanda inofungidzirwa kuti chinhu chakanaka chehutano. Asi mhuri ye hypobetalipoproteinemia inoratidzika, iyo inogarwa nhaka inokonzera zvishoma kusvika kune yakaderera zvikuru LDL cholesterol mazinga. Inogona kubudisa zviratidzo zvinogona kudiwa kuti zvibatwe nekudya kwakakosha uye kuwedzera. (Chimwe chirwere chingave chinoratidzirwa nehuwandu hweHCDL ndechekuti mhuri yeAlpha lipoprotein inoshaiwa kana chirwere cheTangier .)
Aina
Dambudziko iri rinowanzoitika nekuda kwekuchinjwa mumapuroteni, apolipoprotein b (apoB). Iyi protein inobatanidza neLDL particles uye inobatsira kutakura cholesterol kumasero mumuviri.
Kune marudzi maviri e hypobetalipoproteinemia: homozygous uye heterozygous. Vanhu vane homozygous nokuda kwechiitiko ichi vane kuchinja mumakopi maviri ejeni. Zviratidzo mumunhu uyu zvichava zvakanyanya uye zvichaitika pakutanga kwehupenyu, kazhinji mukati memakore gumi ekutanga ehupenyu.
Vanhu veHeterozygous, kune rumwe rutivi, chete vane kopi imwechete yemutambo we mutated. Zviratidzo zvavo zvinotyisa, uye dzimwe nguva, vangave vasingazivi kunyange kuti vane chirwere ichi kusvika vasati vawana cholesterol panguva yevanhu vakuru.
Kuderera kwecholesterol, kunyanya LDL cholesterol, kunogona kuderedza ngozi yako yepfungwa yemwoyo. Zvisinei, kana cholesterol yako yakanyanya kuderera, inogona kutungamirira kune zvinetso zvakasiyana-siyana zvemutano.
Zviratidzo
Kuoma kwezviratidzo kunobva pane rudzi rwemhuri ye hypobetalipoproteinemia yaunayo. Vanhu vane hypozygous family hypobetalipoproteinemia vane zviratidzo zvakanyanya kupfuura rudzi rwe heterozygous, urwo runosanganisira:
- Diarrhea
- Bloating
- Kudzora
- Kuvapo kwemafuta, marara-mavara
Kunze kwezviratidzo zvemimba, mwana mucheche kana mwana ane homozygous hypobetalipoproteinemia anoratidza zvimwe zviratidzo kubva pakuva nemadekari akawandisa emuviri muropa, zvakafanana nemamiriro ezvinhu, abetalipoproteinemia. Zviratidzo izvi zvinosanganisira:
- Zvinetso zvehutano. Izvi zvinogona kunge zvakakonzerwa nehuwandu huwandu hwemapidhi kana mavitamini anogadzikana ane mafuta ari kufamba mumuviri. Zviratidzo izvi zvinogonawo kusanganisira kukura zvishoma, kwete yakadzika tendon reflexes, utera, kuoma kufamba, uye kudengenyeka.
- Zvinetso neropa kuvhara . Izvi zvingangodaro zvichienderana nehutachiona hwema vitamini K. Masero matsvuku eropa anogonawo kuonekwa asina kujairika.
- Vitamini kukwana. Mavhithamini ane mafuta (A, K, uye E) anowanikwawo muvanhu ava. Lipidha dzinokosha mukutakura mavitamini ane svubvu kune dzimwe nzvimbo dzomuviri. Apo lipid, sezvinoita cholesterol uye triglycerides , dzakaderera, hazvikwanisi kutakura mavitamini apo inodiwa.
- Matambudziko anonzwa . Izvi zvinosanganisira nyaya nekuona uye kugunzva kunzwa.
Vanhu vane heterozygous hypobetalipoproteinemia vangave vane zviratidzo zvishoma zvematumbo, kunyange vazhinji vasina zviratidzo.
Zvose zviri zviviri izvi zvinenge zvichange zvakaderera uye LDL cholesterol mazinga.
Kuziva
Pane jira rinoputika richaitwa kuti riongorore mazinga echolesterol ari kubuda muropa.
Chirwere checholesterol chaizowanzobudisa zvinotevera:
Homozygous
- Kose cholesterol mahara <80 mg / dL
- LDL cholesterol mahara <20 mg / dL
- Zvishoma zvakanyanya triglyceride mazinga
Heterozygous
- Kose cholesterol inokwana <120 mg / dL
- LDL cholesterol mahara <80 mg / dL
- Triglyceride mazinga anowanzojairika (<150 mg / dL)
Nhamba dze apolipoprotein B dzinogona kuva dzakaderera kusvika dzisingabviri zvichienderana nerudzi rwe hypobetalipoproteinemia iripo. Chirwere chechiropa chinogona kutorwawo kuitira kuti chione kana chirwere chechiropa chinenge chichiita kuti pave nepididho yakaderera. Zvinhu izvi, kunze kwezviratidzo izvo munhu ari kutarisana nazvo, zvichabatsira kutsigira kufungidzira kwemhuri ye hypobetalipoproteinemia.
Kurapa
Kurapa kwemhuri ye hypobetalipoproteinemia kunobva pane rudzi rwechirwere. Mune vanhu vane cheyzygous type, vitamini supplementation-kunyanya mavitamini A, K, uye E-inokosha. Mumwe wezvokudya anokwanisawo kubvunzurudzwa munyaya idzi sezvo chikafu chakakosha chemafuta supplementation chingada kushandiswa.
Muvanhu vanoonekwa kuti vane heterozygous rudzi rwe hypobetalipoproteinemia, kurapwa kungave kusingadikanwi kana vasina kuwana zviratidzo zvechirwere. Zvisinei, vamwe vanhu vane heterozygous vangada kuiswa pazvokudya zvakakosha kana kugamuchira supplementation nemavhithamini ane mafuta-soro kana zviratidzo-zvakadai sedhara kana kuputika-zviripo.
Sources:
Bheri MH, Porter RS, Jones TV. Merck Manual of Diagnosis uye Therapy. 19th edition, 2011.
Fauci AC, Kasper DL, Longo DL et al. Harrison's Principles of Internal Medicine. 18th edition, 2015.