Ichi Chiitiko Chinowanzoita Kuti Chive chakaoma kuMuviri weKubvisa Mafuta
Abetalipoproteinemia (inozivikanwawo seBassen-Kornzweig syndrome) chirwere chinokonzerwa nehutano chinosanganisa kuti mafuta anogadzirwa sei uye anoshandiswa mumuviri. Miti yedu inoda mafuta kuitira kuchengetedza mishonga ine utano, misumbu, uye kudya. Kufanana nemafuta nemvura, mafuta haagoni kufamba-famba nemitumbi yedu pachavo. Pane kudaro, vanobatanidza mapuroteni chaiwo anonzi lipoproteins uye vanoenda kune chero kwavanoda.
Maitiro aAbetalipoproteinemia Anobata Lipoprotein
Nemhaka yehutachiona hwekuchinja, vanhu vane abetalipoproteinemia havaiti mapuroteni anodiwa kuti vaite lipoproteins. Pasina zvakakwana lipoproteins, mafuta haagoni kugadzirwa zvakakodzera kana kuenda kunzvimbo yavanoda. Izvi zvinotungamirira kune zvinetso zvakakomba zvehutano zvinogona kukanganisa mudumbu, ropa, masimba, uye dzimwe mamiriro emitumbi.
Nemhaka yematambudziko anokonzerwa neabetalipoproteinemia, zviratidzo zvemaitiro zvinowanzoonekwa mucheche. Vanhurume vanobatwa zvakanyanya - vanenge 70-muzana kupfuura - kupfuura vakadzi. Mamiriro acho ehurumende ndiyo inotora nhaka, zvichireva kuti vabereki vose vanofanira kunge vane chakakanganisika cheM MTTP geni kuti mwana wavo agare nhaka. Abetalipoproteinemia haishamisi, makumi masere chete maitiro akataurwa.
Zviratidzo nezviratidzo zveDambudziko
Vacheche vanoberekwa neabetalipoproteinemia vane dumbu matambudziko nekuda kwekushaya kukwanisa kudya mafuta zvakanaka. Kufamba kwemafupa kazhinji kunowanzoshamisa uye kunogona kuve kwakajeka uye kwakashata.
Vana vane abetalipoproteinemia vanogonawo kunatswa, kurarisa, kubvongodza, uye kuoma kukuwana uremu kana kukura (dzimwe nguva inozivikanwa sekukundikana kubudirira).
Vaya vane chirwere vanewo zvinetso zvine chokuita nemavitamini akachengetwa mumafuta - vitamini A, E, uye K. Zviratidzo zvinokonzerwa nokushaya kwemafuta uye mavhithamini anogadzikana zvinowanzobudirira mukati memakore gumi ekutanga ehupenyu.
Izvi zvinogona kusanganisira:
- Kukanganisa kunonzwisisika: Izvi zvinosanganisira matambudziko anonzwa kutonhora nekubata - kunyanya mumaoko nemaoko (hypesthesia)
- Dambudziko rekufamba (ataxia): Inenge 33 muzana yevana vachange vachinetseka kufamba nekusvika makore gumi. Ataxia inowedzera kupfuura nguva.
- Kutambuka kwemhanyi: Kuvhiringidzika, kuzununguka (chorea), kushungurudzika kuwana zvinhu (dysmetria), kunetseka kutaura (dysarthria)
- Zvinetso zvematumbo: Kushaya simba, kupfupisa (kushandiswa) kwemisumbu mushure iyo inokonzera musana kuti upinde (kyphoscoliosis)
- Dambudziko reRopa: Low iron ( anemia ), zvinetso nekuvhara, masero matsvuku eropa (acanthocytosis)
- Dambudziko reziso : Upofu hweusiku, kusaona maziso, zvinetso nekutarisa maziso (ophthalmoplegia), cataracts
Kuwana Abetalipoproteinemia Kuziva
Abetalipoproteinemia inogona kuonekwa kuburikidza nesipo samples. Kufamba kwemafupa, kana kuedzwa, kucharatidza huwandu hwemafuta sezvo mafuta ari kubviswa panzvimbo pokushandiswa nemuviri. Kuyera ropa kunogona kubatsira kubatsira chirwere chacho. Masero matsvuku eropa anowanikwa muabetalipoproteinemia anogona kuonekwa pasi pekroscope. Ikoko pachavawo nehuwandu hwakawanda hwemafuta akaita seholesterol uye triglycerides muropa.
Kana mwana wako ane abetalipoproteinemia, miedzo yekuvhara nguva nematare ematare zvichadzoka zvakare zvisina kujairika. Ziso rekuongorora rinogona kuratidza kuputika kwemashure eziso (retinitis). Kuedzwa kwemasimba emasimba uye zvinyorwa zvinogona kuva nemigumisiro yakaipa zvakare.
Kurapa Chiitiko Nokudya Zvokudya
Zvokudya zvakanakira vanhu vane abetalipoproteinemia zvakagadzirwa. Pane zvakawanda zvinodiwa mune zvekudya, kusanganisira kudzivisa kudya mamwe marudzi emafuta (refu-chain triglycerides) mukuda kudya mamwe maitiro (midzi-chain chain triglycerides). Chimwe chinodikanwa kuwedzera kuwandisa kwemavhithamusi ekuwedzera ane vitamini A, E, uye K, pamwe nesimbi.
Mune chikafu ane ruzivo rwemashure anogadzirisa zvirwere zvinogona kukubatsira iwe kuronga hurongwa hwekudya huchasangana nezvido zvekudya zvako zvemwana wako.
Sources :
Berriot-Varoqueaux, N., Aggerbeck, LP, Samson-Bouma, M., & Wetterau, JR (2000). Basa re microsomal triglyceride transfer protein mune abetalipoproteinemia. Annu Rev Nutriti, vol. 20, mapeji 663-697.
Gregg, RE, Wetterau, JR, Berriot-Varoqueaux, N., Aggerbeck, LP, & Samson-Bouma, M. (1994). Iko molecule yeabetalipoproteinemia. Curr Opin Lipidol, vol. 5, kwete. 2, mapeji 81-86.
Rader, DJ, Brewer, HB, Jr., Gregg, RE, Wetterau, JR, Berriot-Varoqueaux, N., Aggerbeck, LP, Samson-Bouma, M., & Wetterau, JR (1993). Abetalipoproteinemia: Zvitsva zvinowanikwa mune lipoprotein musangano uye vitamini E yemetabolism kubva pane zvisingawaniki zvechirwere chetachiona. JAMA, vol. 270, kwete. 7, mapeji 865-869.
Stone, NJ, Blum, CB, & Winslow, E. (1998). Pathophysiology ye hyperlipoproteinemias. MuKutevedza kweLipids muChipical Practice. Inowanikwa paIndaneti kuMedscape.
Medline Plus. Bassen-Kornzweig syndrome. (2013)