Reka twige ku ndwara ya Tangier, igaragara ku bantu bafite cholesterol nziza nkeya (HDL) mu mubiri.

Ese wigeze wumva ubwoko bwa cholesterol yitwa cholesterol nziza (HDL / lipoprotein ifite ubucucike bwinshi) ? Yego, yego... Uyu munsi tugiye kuvuga ku ndwara idakunze kugaragara cyane ibaho iyo urugero rw'iyi cholesterol nziza mu mubiri wacu ruri hasi cyane. Iyi yitwa Indwara ya Tangier . Ushobora kuba utazi iri zina, ariko ni ingenzi cyane kurimenya.

Indwara ya Tangier ni iki? Reka tubisobanukirwe mu buryo bworoshye!

Reka dutangire duhereye ku ntangiriro. Indwara ya Tangier ni indwara idakunze kugaragara cyane dukura ku babyeyi bacu, ni ukuvuga, twarazwe mu buryo bw'uturemangingo . Abantu bafite iyi ndwara bafite urugero ruto cyane rwa cholesterol nziza (HDL) mu mibiri yabo. Wari uzi ko iyi cholesterol ya HDL idakunze kwitwa "cholesterol nziza" ku busa. Ni bo bafasha kugira imibiri yacu isukuye bakuraho cholesterol mbi (LDL / lipoprotein nkeya) ikunda kwirundanya mu mitsi yacu, ni ukuvuga mu mitsi yacu .

Noneho tekereza uko bigenda iyo iyi cholesterol nziza (HDL) igabanutse. Iyo cholesterol mbi (LDL) n'ubundi bwoko bw'ibinure (lipide) bitangira kwinjirira mu mitsi yacu. Nk'uko umwanda ufata umuyoboro w'amazi. Ibi bishobora guteza ibibazo ku mutima wacu, indwara z'umutima , cyangwa se bigatera indwara nko gucika imitsi . Si ibyo gusa, iyi ndwara ishobora kugira ingaruka ku zindi ngingo nyinshi mu mubiri wacu.

Kuki byitwaga "Tangier"?

Iyi ni inkuru ishimishije cyane. Iyi ndwara yitwa "Tangier" bitewe n'ikirwa cya Tangier , giherereye hakurya y'inyanja uvuye muri leta ya Virijiniya muri Amerika. Iyi ndwara yavumbuwe bwa mbere mu bantu bari batuye kuri icyo kirwa. Ariko, ibyo ntibivuze ko iki ari ikintu gihari gusa kuri icyo kirwa. Hari n'abantu bafite iyi ndwara mu bindi bihugu by'isi.

Indwara ya Tangier ikunze kugaragara gute?

Mu by’ukuri, indwara ya Tangier ni indwara idakunze kugaragara cyane . Abantu bake cyane, bagera ku 100, ni bo bamaze kumenyekana ku isi yose. Niyo mpamvu itavugwaho byinshi. Ariko nubwo idakunze kugaragara, ni ngombwa kumenya iyi ndwara, si byo se?

Ni gute indwara ya Tangier igira ingaruka ku mubiri wacu?

Dore uko bigenda. Iyo turwaye diyabete, urugero rwa cholesterol nziza (HDL) mu mubiri wacu ruba ruri hasi, bityo ibinure (lipide) ntibishobora kwimurwa mu mwijima wacu neza. Umwijima ni wo ukuraho ibinure bidakenewe mu mubiri. Bityo, ibi binure bitashobora gukurwaho bitangira kwirundanya mu bice bitandukanye by'umubiri wacu. Iyo ibi binure byirundanyije, urwo rugingo narwo rushobora kuba runini gato.

Ibinure bikunze gushyirwa muri ubu buryo:

• Umwijima: Umwijima ushobora kwiyongera.
• Udusimba:Uduheri na two dushobora kubyimba no kwiyongera.
• Tonsils: Tonsils zo mu muhogo wacu. Zishobora guhinduka umuhondo n'icunga mu ibara iyo zuzuyemo ibinure.
• Uduce tw’umurabyo: Utu duce ni bimwe mu bigize ubudahangarwa bw’umubiri wacu. Dushobora no kubyimba.
• Umutima: Ibi bishobora kandi kugira ingaruka ku mikorere y'umutima.
• Ubwonko: Hari igihe ubwonko nabwo bushobora kugira ingaruka.

Uretse ibi, hari n'ibindi bibazo bishobora kuvuka:

• Ibibazo by'imitsi: Ibibazo by'imitsi, nko kubura ibinure mu maguru, bishobora kubaho.
• Urugero rwa triglyceride ruri hejuru: Uru ni ubwoko bw'ibinure biboneka mu maraso, kimwe na cholesterol.
• Ibibazo byo kureba: Hashobora kubaho ibicu bidasanzwe mu maso, kandi rimwe na rimwe kureba bishobora kugabanuka.

Ni ibihe bimenyetso by'indwara ya Tangier?

Ibi ni ingenzi cyane. Ibimenyetso bya Tangier Disease bishobora gutandukana bitewe n'umuntu ku giti cye. Biterwa n'aho ibinure biri mu mubiri, nk'uko nabivuze mbere. Hari abantu bashobora kugaragaza ibimenyetso igihe bavuka, mu gihe abandi bashobora kutagaragaza ibimenyetso bikomeye kugeza bagejeje ku myaka 60-65.

Dore ibimenyetso bimwe na bimwe bikunze kugaragara:

• Kubabara mu gifu cyangwa isesemi.
• Ibinure byinjira mu mitsi (atherosclerosis): Ibi byongera ibyago byo kurwara indwara z'umutima.
• Uruhu rwumye.
• Gutembera imbere kw'ijisho ry'amaso (Ectropion): Iki ni ikimenyetso kidasanzwe.
• Anemia: Ibi bivuze kubura amaraso.
• Intege nke z'imitsi mu maboko, amaguru, amaboko, cyangwa ibirenge.
• Utunyangingo tw’imitsi twabyimbye igihe kirekire nta bwandu.

Wibuke ko hari igihe ibi bimenyetso bishobora kwitiranywa n'izindi ndwara, ariyo mpamvu ari ngombwa kumenya neza indwara.

Ni iki gitera indwara ya Tangier? Reka tuvuge ku ngirabuzima fatizo!

Indwara ya Tangier iterwa n'inenge iri muri gene yitwa ABCA1 gene dukomoka ku babyeyi bacu. Muri make, gene ya ABCA1 irimo amabwiriza yo gukura cholesterol mu turemangingo twacu no kuyishyira muri cholesterol nziza (HDL). HDL ijyana iyi cholesterol mu mwijima, aho isohoka.

Noneho, niba hari inenge cyangwa impinduka muri iyi gene ya ABCA1, ayo mabwiriza ntabwo akora neza. Icyo gihe gukuraho cholesterol mu turemangingo no kwirema kwa HDL ntibibaho neza. Ingaruka ni uko urugero rwa HDL rugabanuka cyane.

Uku ni ko byarazwe:

• Iyo ababyeyi bombi bazunguye gene ya ABCA1 ifite inenge, umwana azarwara indwara ya Tangier.
• Iyo umubyeyi umwe gusa azunguye gene ifite inenge, umwana azaba ari we uyimutwaza. Ashobora kutagaragaza ibimenyetso, ariko urugero rwa HDL rwe rushobora kuba ruri hasi ugereranije n’ibisanzwe.

Noneho tekereza ko ababyeyi bombi ari bo batwara iyi gene ifite inenge, bivuze ko bashobora kuba badafite ibimenyetso, ariko bombi bafite iyo gene. Iyo ababyeyi nk'abo bafite umwana:

• Hari amahirwe angana na 25% (1/4) y'uko umwana azarwara indwara ya Tangier.
• Hari amahirwe angana na 50% (1/2) y'uko umwana ashobora kuba ariwe ufata iyi ndwara.
• Hari amahirwe angana na 25% (1/4) y'uko umwana atazavukana uturemangingo dufite inenge kandi azaba afite ubuzima bwiza.

Ibi ni nko kuzunguza igiceri ugafata imitwe cyangwa imirizo. Ayo mahitamo ni amwe kuri buri gutwita.

Ni gute abaganga bapima indwara ya Tangier?

Kugira ngo umenye neza niba ufite indwara ya Tangier, ugomba kujya kwa muganga. Azabanza agukorere isuzuma ry’umubiri akakubaza ibimenyetso byawe. Hanyuma, bazagutegeka gupima amaraso menshi. Ibi bipimo by’amaraso bireba cyane urugero rwa cholesterol ya HDL yawe n’urugero rwa poroteyine yitwa apolipoprotein A1 (ApoA1) iri hasi. Ibi byombi ni bike cyane ku bantu barwaye indwara ya Tangier.

Isuzuma ry’uturemangingo rishobora kandi gukorwa kugira ngo hemezwe ko indwara yagaragaye. Rishobora gusuzuma impinduka runaka muri gene ya ABCA1. Ariko, isuzuma ry’uturemangingo rishobora kutaboneka ahantu hose. Muri ibyo bihe, muganga wawe ashobora gufata agace gato k’umubiri wawe (biopsy) nko mu ruhu, mu nda, cyangwa mu mwijima. Ubwoko bw’ibinure biri muri izi ngingo bushobora gukoreshwa kugira ngo hamenyekane indwara.

Ni poroteyine iyihe ibura mu ndwara ya Tangier?

Mu ndwara ya Tangier, poroteyine yitwa apolipoprotein A1 (ApoA1) iragabanuka cyane. Iyi ApoA1 ni igice cy'ingenzi cya molekile ya cholesterol ya HDL. Rero iyo ApoA1 igabanutse, urugero rwa HDL narwo ruragabanuka.

Ni ibihe bipimo bikoreshwa mu gusuzuma no gukurikirana indwara?

Abaganga bashobora kandi gukora ibindi bipimo byinshi kugira ngo bamenye indwara no gukurikirana ingaruka z'indwara ku mubiri.

• Ibipimo by'imikorere y'imitsi n'imitsi (Electromyograms / EMGs): Reba niba imitsi yangiritse cyangwa idafite imbaraga.
• Isuzuma ry'amaso: Reba niba nta kibazo cy'amaso cyangwa ikibazo cyo kutabona neza.
• Ultrasound ku nda yawe / mu nda: Reba imiterere y'ingingo nk'umwijima n'umwobo.
• Ultrasound ku mitsi yawe ya carotid: Reba niba hari ibinure byishyize mu mitsi minini iri mu ijosi ryawe.
• Igipimo cya CT angiogramu cy'umutima wawe:Reba niba hari imitsi ifunganye itanga amaraso mu mutima.
• Echocardiogram: Isuzuma ry'umutima rigamije kureba imikorere n'imiterere y'umutima.
• Ikizamini cy'imihangayiko mu myitozo ngororamubiri: Reba uko umutima witabira imyitozo ngororamubiri.

Indwara ya Tangier ivurwa ite?

Mu by’ukuri, nta buryo bwihariye bwo kuvura indwara ya Tangier bushobora gukiza burundu indwara. Kubera ko ari indwara iterwa n’uturemangingo, hari byinshi ushobora gukora kugira ngo ugenzure ibimenyetso no kugabanya ingorane.

Hari igihe urugingo rwangiritse bitewe n’ibinure, urugero nk’amagufwa cyangwa urwagashya, rushobora gukenera kubagwa .

Ese ibiryo n'ibinyobwa bishobora gufasha?

Yego, birashoboka rwose. Hari ibiryo bimwe na bimwe bishobora gufasha kongera gato urugero rwa cholesterol nziza (HDL), ndetse bikanafasha kugabanya urugero rwa cholesterol mbi (LDL).

• Avoka
• Amavuta ya elayo
• Ibinyamisogwe nk'ibishyimbo n'ibishyimbo (Ibishyimbo)
• Ibinyampeke byuzuye (urugero: ingano y'ifu, umuceri w'ifu y'ifu)
• Amafi afite ibinure ( urugero: salmon, mackerel, herring)
• Imbuto ( urugero: ubunyobwa, amande, cashew - ariko nta munyu)
• Imbuto zifite fibre nyinshi (urugero: guava, mango, imineke)
• Imbuto za Chia n'imbuto za flax

Kugerageza gushyira ibi biribwa mu mirire yawe buri gihe bizagufasha kugumana urugero rwiza rwa cholesterol.

Ni izihe mpinduka mu miti n'imibereho?

Muganga wawe ashobora kukwandikira imiti igabanya cholesterol , nka statins.

Irasaba kandi guhindura imibereho myinshi bishobora gufasha kongera urugero rwa HDL:

• Gukora imyitozo ngororamubiri kenshi: Ibi birimo kugenda n'amaguru cyangwa kwiruka nibura iminota 30 ku munsi.
• Kudakoresha ibikomoka ku itabi: Ugomba kureka kunywa itabi burundu.
• Gukomeza kugira ibiro byiza.
• Kurya ibinure bitagira ibinure byinshi aho kurya ibinure byinshi: Ibinure byinshi biboneka mu bintu nk'amavuta ya kokonati, amavuta ya palme, n'amavuta y'inyamaswa. Ibinure bitagira ibinure byinshi biboneka mu mavuta ya elayo na avoka.

Ingaruka mbi z'ubuvuzi ni izihe?

Imiti igabanya cholesterol ishobora rimwe na rimwe gutera ingaruka mbi nto. Si buri wese uyibona, ariko ni byiza kuyimenya.

• Kuribwa umutwe
• Kubabara imitsi
• Impatwe
• Inda ibabaye

Niba ikintu nk'iki kibaye, ugomba kubibwira muganga wawe. Hanyuma ashobora guhindura imiti cyangwa akaguha izindi nama.

Ese indwara ya Tangier ishobora kwirindwa?

Kubera ko iyi ari indwara y’uturemangingo, ntabwo dushobora kuyibuza kuyikuramo. Ibi bivuze ko niba ufite iyi nenge mu turemangingo twawe, idashobora guhinduka. Ariko, niba ufite iyi ndwara, cyangwa niba uri umuntu uyirwaye, ushobora kugisha inama umujyanama mu by’uturemangingo kugira ngo amenye ibyago byo kuba abana bawe bayirwara. Hanyuma, ukurikije uko undi mubyeyi ameze, ushobora kubona neza amahirwe abana bawe bashobora kuyirwara.

Niba mfite indwara ya Tangier, bizagenda bite mu gihe kizaza?

Igipimo cy’indwara ya Tangier muri rusange ni cyiza, ariko biterwa n’uko ibimenyetso byawe byageze ku rugero rungana. Kimwe n’izindi ndwara zose, gusuzuma indwara hakiri kare ni byiza mbere yuko ibimenyetso bikomera.

Nubwo nta muti uhari kugeza ubu, hari icyizere cy'uko ibintu nk'ubuvuzi bw'uturemangingo bizafasha mu gihe kizaza. Ubushakashatsi buracyakomeje.

Indwara ya Tangier imara igihe kingana iki?

Kubera ko iyi ari indwara iterwa n’uturemangingo, ni indwara y’ubuzima bwose. Ariko, iyo igaragaye hakiri kare kandi ikagenzurwa buri gihe, iyi ndwara ishobora kwitabwaho neza no kubaho.

Nitwara nte?

Niba ufite urwagashya rwagutse bitewe n’indwara ya Tangier, ugomba kwirinda siporo zo gukoranaho, kuko gukoranaho bishobora gutuma urwagashya rwacika, ibyo bikaba ari akaga.

Byongeye kandi, hari n'ibindi bintu bishobora gutera indwara z'umutima bigomba kugenzurwa:

• Kugenzura umuvuduko ukabije w'amaraso .
• Niba urwaye diyabete , genzura neza.
• Kwirinda ikoreshwa ry'ibikomoka ku itabi.

Ni ryari ngomba kujya kwa muganga?

Niba urwaye indwara ya Tangier, ugomba kujya kwa muganga wawe buri gihe . Muri ubwo buryo, niba ibimenyetso byawe birushijeho kwiyongera, ushobora gufata ingamba vuba.

Muganga akunda gusuzuma ibi bintu:

• Udutsinga tw'imitsi.
• Umutima.
• Amaso.

Ni ibihe bibazo ngomba kubaza muganga wanjye?

Iyo ugiye kwa muganga wawe, ushobora kubaza ibibazo nk'ibi:

• Ese ngomba gufata imiti kugira ngo ndusheho cholesterol yanjye?
• Ese hari itsinda ry’ubufasha abantu barwaye indwara ya Tangier bashobora kwinjiramo?
• Ni kangahe nkwiye kuza kugusura?

Kubera ko indwara ya Tangier ishobora gufata ibice bitandukanye by'umubiri, ni byiza kwisuzumisha buri gice buri gihe. Ushobora gukenera kubonana n'inzobere zitandukanye. Fata imiti neza nk'uko muganga wawe yabigutegetse, kandi ukore uko ushoboye kose kugira ngo ukomeze kugira indyo nziza n'imyitozo ngororamubiri.

Ibintu by'ingenzi tugomba kwibuka (Ubutumwa bwo kujyana mu rugo)

Sawa, reka nguhe muri make bimwe mu bintu ugomba kwibuka mu byo twaganiriyeho.

• Indwara ya Tangier ni indwara idakunze kugaragara, iterwa n'uturemangingo, ituma cholesterol nziza (HDL) igabanuka cyane.
• Ibi bishobora gutuma cholesterol mbi (LDL) n'ibinure byiyongera mu mubiri kandi bikagira ingaruka ku ngingo nk'umwijima, urwagashya, tonsils, n'umutima.
• Ibimenyetso biratandukanye, kandi bamwe bashobora kugira ibibazo by'imitsi, ibibazo by'uruhu, n'ibibazo byo kutabona neza.
• Ibi biterwa n'inenge iri muri gene ABCA1 .
• Iyi ndwara ipimwa binyuze mu gupima amaraso, gupima imiterere y’uturemangingo, ndetse rimwe na rimwe no gupima ingingo (biopsy) .
• Nubwo nta muti wihariye uhari, ibimenyetso bishobora kugenzurwa hakoreshejwe imirire, guhindura imibereho, imiti, ndetse no kubagwa bimwe na bimwe.
• Kumenya hakiri kare no kwitabwaho kenshi na muganga ni ingenzi cyane.

Rero, ndizera ko aya makuru azagufasha. Niba ufite ikindi kibazo kuri ibi, ntutindiganye kuvugana na muganga. Komeza ugire ubuzima bwiza!

Indwara ya Tangier , cholesterol ya HDL, cholesterol ya LDL, indwara zikomoka ku ngirabuzimafatizo, gucunga cholesterol, ibyago by'indwara z'umutima, ApoA1