Ni ubuhe bwoko budasanzwe bw'indwara yo kubura amaraso n'impyiko? (Indwara ya Hemolytic Uremic Syndrome - aHUS) Reka tuganire!

Ese ushobora kwiyumvisha uko byagenda iyo ubwirinzi bwacu, ubudahangarwa bw'umubiri, aho kuturinda indwara, butangira kwibasira imibiri yacu? Iyo ni indwara ibabaje, ariko idasanzwe, tugiye kuvugaho uyu munsi ni aHUS, cyangwa Atypical Hemolytic Uremic Syndrome . Iki ni cyo gihe imiyoboro y'amaraso yacu yangiritse, amaraso mato agatangira kuzura, kandi amaraso agatera mu ngingo z'ingenzi nk'impyiko akagabanuka. Iyi ndwara ikunze guterwa n'impinduka mu miterere y'uturemangingo .

Abaganga bakunze kugusuzuma iyo ufite ibi bibazo bitatu:

• Microangiopathic hemolytic anemia : Ibi bivuze ko uturemangingo tw'amaraso dutukura twangirika vuba cyane, bityo umubiri wawe ntushobora gukora uturemangingo dushya two kudusimbura.
• Thrombocytopenia : Iyi ni igabanuka ry'umubare wa platelets, zifasha amaraso kubumba.
• Gukomereka kw'impyiko bikabije : Ubu ni ubwoko bw'impyiko zinanirwa gukora neza, ariko rimwe na rimwe bishobora guhinduka.

Izi ndwara uko ari eshatu zishobora kubaho icyarimwe, cyangwa zigakongerwa n'izindi ndwara.

Ni irihe tandukaniro riri hagati ya HUS isanzwe n'iyi aHUS?

Mu bihe byashize, iyi ndwara yitwaga HUS (Hemolytic Uremic Syndrome) yagabanyijwemo ubwoko bubiri bw'ingenzi.

• HUS isanzwe (HUS ifitanye isano no gucibwamo): Ibi bikunze kujyana no gucibwamo. Biterwa n'ubwoko bwa bagiteri yitwa E. coli . Hari n'abandi bayita STEC-HUS .
• Indwara ya aHUS tuvuga uyu munsi (HUS idasanzwe): Iyi si yo ndwara yo gucibwamo igaragara. Akenshi, hafi kimwe cya kabiri cy'abantu barwara aHUS, iterwa n'impinduka mu miterere y'uturemangingo, mutation y'uturemangingo . Abaganga rimwe na rimwe bayita iyi ndwara complement-mediated thrombotic microangiopathy (CM-TMA) .

Ni ibihe bintu byihariye bitera aHUS?

Mu magambo make, si buri wese ufite ihinduka ry’uturemangingo azarwara HUS. Akenshi, iterwa, cyangwa ikongera imbaraga, n’izindi ndwara. Tekereza kuri ibi bintu:

• Igihe cyo gutwita
• Kanseri
• Indwara zitandukanye
• Imiti imwe n'imwe, cyane cyane imiti igabanya ubukana bw'umubiri, imiti igabanya amaraso , imiti ikoreshwa mu kuboneza urubyaro , hamwe n'imiti imwe n'imwe igabanya ubukana bw'indwara .

Ni ibihe bimenyetso bya aHUS?

Noneho reka turebe ibimenyetso umuntu urwaye aHUS agaragaza. Ushobora kuba ufite byinshi muri ibi, cyangwa ushobora kuba ufite ibimenyetso bigenda bikura buhoro buhoro.

• Kumva unaniwe igihe cyose (Umunaniro)
• Uruhu rwerurutse (Umuhondo)
• Isesemi cyangwa kuruka
• Kugabanuka k'umusaruro w'inkari
• Amaraso mu nkari
• Umuvuduko ukabije w'amaraso (Hypertension)
• Kugorwa no guhumeka (Dyspnea)
• Edema
• Urujijo , gutakaza kwibuka
• Umuriro

Abantu benshi ntibagira ibi bimenyetso byose icyarimwe. Hari igihe biza buhoro buhoro, buhoro buhoro. Ushobora kumva nk'aho umaze igihe urwaye ikintu runaka, ariko ntumenye icyo ari cyo. Ibi bimenyetso by'imitsi , ni urujijo navuze mbere, ntibibaho kuri buri wese, ni gake cyane.

Ni iki gitera aHUS mu by'ukuri?

Impamvu nyamukuru ya aHUS ni impinduka muri ADN yacu (mutation genetique) , cyangwa ikorwa rya autoantibodies yibasira uturemangingo twacu ku 'poroteyine zuzuzanya' mu budahangarwa bwacu bw'umubiri . Izi mpinduka mu ngirabuzimafatizo zishobora kuragwa n'ababyeyi bacu, cyangwa zishobora kubaho ku buryo butunguranye. Izi mpinduka mu ngirabuzimafatizo zigira ingaruka ku mikorere ya poroteyine zitwa 'ibintu byuzuzanya' .

Tekereza kuri ibi 'bintu byuzuzanya' nk'abasirikare bato bafasha uturemangingo tw'ubudahangarwa bw'umubiri wacu. Kimwe n'ibirungo by'ibiribwa, izi poroteyine zibona mikorobe zangiza (nk'udukoko twa bagiteri na virusi) zikabishyira ku rutonde kugira ngo utundi turemangingo tw'ubudahangarwa tubashe 'kuzirya'. Ibikunze kwibasirwa na aHUS ni ibintu byuzuzanya H, I, 3, na B.

Urugero, inshingano ya complement factor H ni ukurinda uturemangingo tuzima mu mubiri wacu no kurinda izindi complement factors gukora igihe zidakenewe. Noneho tekereza uko bigenda iyo iyi complement factor H idakora neza (ni ukuvuga niba hari impinduka muri gene ya CFH) ? Uturemangingo twacu ntitwongere kubona uburinzi dukeneye. Dore ibintu bibaho:

• Uturemangingo tw’umubiri wacu twibasira kandi tukangiza uturemangingo turi imbere mu miyoboro y’amaraso (uturemangingo tw’amaraso) .
• Uduce duto tw’amaraso duterana dukikije iki cyangiritse maze tugakora ikibyimba cy’amaraso. Ibi biziba imiyoboro y’amaraso.
• Ibi bigabanya amaraso atembera mu ngingo zacu, bigatuma zangirika ndetse n'imikorere yazo ikagabanuka. Impyiko nizo zikunze kwibasirwa cyane.
• Izi ngingo z'amaraso zangiza izindi selile z'amaraso zinyura muri zo, bigatera indwara yitwa hemolytic anemia .

Wamenya ute niba ufite aHUS?

Ubusanzwe umuganga azasuzuma aHUS akurikije ibimenyetso byawe n'ibisubizo by'ibizamini bisuzuma umubare w'amaraso yawe n'uko ingingo zawe zakora. Azakora kandi ibizamini kugira ngo arebe neza ko nta zindi ndwara zitera ibimenyetso bisa (nk'ibyavuzwe haruguru).

Ni ibihe bipimo bikoreshwa mu gusuzuma aHUS?

Ushobora gukenera gukora ibizamini nk'ibi:

• Ibizamini by'amaraso: Umubare wuzuye w'amaraso (CBC) , ibizamini by'imikorere y'impyiko, n'ibindi.
• Gupima imiterere y'uturemangingo : Ibi bireba niba hari impinduka mu miterere y'uturemangingo.
• Ibizamini by'umwanda : Reba ubwandu nka E. coli.
• Gupima impyiko : Gufata agace gato k'impyiko kugira ngo kasuzumwe.
• Ibizamini byo gupima : nka ultrasound cyangwa CT scan .

Ivurwa rite na aHUS?

Uburyo bwo kuvura aHUS buterwa n'impamvu nyamukuru n'uburemere bw'indwara. Niba ufite ubwoko bw'aHUS budakabije, muganga wawe ashobora kugukurikirana no kugufasha , nko guterwa amaraso cyangwa imiti y'umuvuduko ukabije w'amaraso, nibiba ngombwa.

Ariko, niba ufite aHUS ikomeye, ushobora gukenera ubuvuzi nk'ubu:

• Guhinduranya plasma mu buryo bwo kuvura : Muri ibi, igice cy'amaraso gikurwamo maze hagatangwa plasma nshya.
• Rituximab cyangwa indi miti ikoreshwa mu kuvura indwara ziterwa n’ubwiyongere bw’umubiri.
• Dialysis : Uburyo bwo gusukura amaraso iyo impyiko zidakora neza.
• Gutera impyiko : Ibi bikorwa gusa mu bihe bikomeye cyane.

Ku bafite "impinduka" zavuzwe haruguru, abaganga bakunze gukoresha umuti witwa eculizumab mu kuvura aHUS. Eculizumab ikora mu kubuza poroteyine zimwe na zimwe, ikabuza ubudahangarwa bw'umubiri kwangiza imiyoboro y'amaraso yawe.

Ese hari ingaruka mbi zikomoka ku buvuzi?

Mu gihe ukoresha Eculizumab , ushobora kuba udakingiwe cyane n'inkingo za meningococcal na pneumococcal . Kubwibyo, niba bishoboka, muganga wawe ashobora kukugira inama yo kubona izi nkingo mbere yo gutangira Eculizumab .

Ejo hazaza hafite iki ku muntu ufite aHUS?

Iyo iyi ndwara imenyekanye kandi ikavurwa hakiri kare, indwara nk'indwara y'impyiko iterwa na aHUS zishobora gukemurwa neza. Ushobora kugira ibihe byo koroherwa nta bimenyetso. Ariko, niba hari ikindi kintu cyateye aHUS, gishobora kongera gushyira ubuzima mu kaga.

Mu bihe byashize, hafi kimwe cya kabiri, cyangwa 50%, by'abantu barwaye aHUS barwaye indwara y'impyiko yo mu cyiciro cya nyuma (ESKD) , bivuze ko impyiko zabo zacitse intege burundu.

Ariko hari inkuru nziza! Ubushakashatsi bushya bwerekana neza ko ibyago byo kurwara ESKD byagabanutse cyane ku bantu bafite aHUS bitewe n'ubuvuzi bwa Eculizumab .

Ni izihe ngaruka zo gupfa ziturutse kuri aHUS?

Indwara zikomeye za aHUS, nko kunanirwa kw'impyiko cyangwa kwangirika kw'izindi ngingo, ni mbi cyane. Mu bihe nk'ibi bikomeye, hari ibyago byo gupfa bigera kuri 15%.

Ese aHUS ishobora kwirindwa?

Kubera ko akenshi biterwa n'impinduka mu miterere y'uturemangingo, aHUS ntabwo ishobora kwirindwa. Ariko, niba ufite ibyago byo kurwara indwara zikomeye, ushobora kwirinda bimwe mu bintu biyitera .

Nitwara nte?

Niba waranduye aHUS, ganira na muganga wawe ku buryo bwo kwirinda ibintu bishobora gutuma iyi ndwara irushaho kuba mbi. Ni ngombwa kandi kumenya ibimenyetso by'indwara ikomeye.

Ni ryari ngomba kujya kwa muganga?

Niba umaze igihe kinini wumva unaniwe cyangwa unaniwe - nk'aho ufite indwara idakira itazashira - vugana na muganga wawe. Nanone, vugana n'ibindi bimenyetso byose wagize vuba aha, nko guhindura ubwiherero bwawe.

Ni ryari nkwiye kujya mu ishami rishinzwe ubuvuzi bwihutirwa (ETU) ?

Niba ufite ibi bimenyetso bikomeye, jya mu cyumba cy’ubutabazi bwihuse:

• Umusaruro w'inkari ugabanuka cyane.
• Urujijo cyangwa guhindura ubwenge.
• Kubyimba gukabije.
• Guhumeka bigoranye cyane.

Ni ibihe bibazo ngomba kubaza muganga?

Bishobora kuba byiza kubaza muganga wawe ibibazo nk'ibi:

• Indwara yanjye ikomeye gute?
• Ni iki cyatumye ibi byiyongera?
• Ese nshobora gukumira ibintu nk'ibi mu gihe kizaza?
• Ni ibihe bimenyetso ngomba kwitondera?
• Ni ryari nkwiye kuzaza kureba?

Iyi ndwara yitwa aHUS ikunze kugaragara gute?

aHUS ni gake cyane.Indwara. Ifata umuntu umwe ku bantu ibihumbi magana atanu. Ikunze kugaragara ku bantu bakuru kurusha abana.

Amaherezo, ibintu byo kwibuka

Kumenya ko wowe cyangwa umwana wawe mufite ikibazo cya genetiki bishobora guhindura ubuzima. aHUS ni indwara ikomeye ishobora gutera amaraso kuzura no kwangiza ingingo. Ariko, ivugururwa riherutse ry'umuti witwa eculizumab ryazamuye cyane umubare w'abantu barwaye aHUS. Niba waranduye aHUS, ganira na muganga wawe ku buryo bwo kwirinda icyagutera indwara no kugabanya ibyago byo kurwara indwara zikomeye. Ashobora kugufasha gusobanukirwa icyo ugomba gukora kugira ngo ukomeze kugira ubuzima bwiza.

aHUS , indwara z'impyiko, kubyimba kw'amaraso, ubudahangarwa bw'umubiri, impinduka mu ngirabuzimafatizo, kubura amaraso, Eculizumab