Kino kika ki ekyewuunyisa eky’okukendeeza ku musaayi n’obuzibu mu kibumba? (Obulwadde bwa Atypical Hemolytic Uremic Syndrome - aHUS) Ka twogere!

Oyinza okulowooza ku kyandibaddewo singa enkola yaffe ey’okwekuuma, abaserikale b’omubiri, mu kifo ky’okutukuuma obutakwatibwa ndwadde, batandika okulumba emibiri gyaffe? Eyo y’emu ku mbeera ey’omukisa omubi, naye etali ya bulijjo, gye tugenda okwogerako leero ye aHUS, oba Atypical Hemolytic Uremic Syndrome . Wano emisuwa gyaffe bwe gyayonooneka, obutundutundu obutono obw’omusaayi ne bukola, n’okutambula kw’omusaayi mu bitundu ebikulu ng’ensigo ne kukendeera. Embeera eno etera okuva ku nkyukakyuka mu buzaale .

Abasawo batera okukuzuula nti olina aHUS ng’olina embeera zino zonsatule awamu:

• Microangiopathic hemolytic anemia : Kino kitegeeza nti obutoffaali bwo obumyufu busaanawo mangu nnyo, kale omubiri gwo tegusobola kukola butoffaali bupya bugenda mu kifo kyabwo.
• Thrombocytopenia : Kino kikendeeza ku muwendo gw’obutoffaali obuyitibwa platelets, obuyamba omusaayi okuzimba.
• Acute kidney injury : Kino kika kya kibumba okulemererwa, naye oluusi kiyinza okudda emabega.

Embeera zino zonsatule zisobola okubaawo omulundi gumu, oba ziyinza okusajjuka olw’endwadde endala.

Njawulo ki eri wakati wa HUS eya bulijjo ne aHUS eno?

Edda embeera eno eyitibwa HUS (Hemolytic Uremic Syndrome) yagabanyizibwamu ebika bibiri ebikulu.

• Typical HUS (diarrhea-associated HUS): Kino kitera okuwerekerwako ekiddukano. Kiva ku kika kya bakitiriya ekiyitibwa E. coli . Abantu abamu era bakiyita STEC-HUS .
• aHUS gye twogerako leero (Atypical HUS): Kino si kye kika ky’ekiddukano ekirabibwa. Ebiseera ebisinga, nga kitundu ku bantu abafuna aHUS, kiva ku nkyukakyuka mu buzaale, enkyukakyuka mu buzaale . Abasawo oluusi embeera eno bagiyita obulwadde bwa complement-mediated thrombotic microangiopathy (CM-TMA) .

Biki ebitongole ebivaako aHUS?

Mu ngeri ennyangu, si buli muntu alina enkyukakyuka mu buzaale nti ajja kufuna aHUS. Ebiseera ebisinga, kiva ku mbeera endala ez’obulamu oba okusajjuka . Lowooza ku bintu bino:

• Ekiseera ky’olubuto
• Kookolo
• Yinfekisoni ez’enjawulo
• Eddagala erimu naddala erijjanjaba eddagala , eddagala eriziyiza obusimu bw’omubiri , erikendeeza omusaayi , eriziyiza okuzaala mu kamwa , n’eddagala erimu eriziyiza okuzimba .

Bubonero ki obwa aHUS?

Kati katutunuulire obubonero omuntu alina aHUS bw’alaga. Oyinza okuba n’obubonero buno obuwerako, oba oyinza okuba n’obubonero obukula mpolampola.

• Okuwulira nga okooye buli kiseera (Obukoowu) .
• Olususu olumyufu (Pallor) .
• Okuziyira oba okusiiyibwa
• Okukendeera kw’omusulo ogufuluma
• Omusaayi mu musulo
• Puleesa (Peesa) .
• Obuzibu mu kussa (Dyspnea) .
• Obulwadde bw’okuzimba
• Okutabulwa , okubulwa okujjukira
• Omusujja

Abantu abasinga tebalina bubonero buno bwonna omulundi gumu. Oluusi zijja mpolampola, mpolampola. Oyinza okuwulira ng’obadde obonaabona n’ekintu okumala akaseera, naye nga tosobola kutegeera kiki. Obubonero buno obw’obusimu , obwo bwe butabulwa bwe nnayogeddeko emabegako, tebutuuka ku buli muntu, butono katono.

Kiki ddala ekivaako aHUS?

Ekisinga okuvaako aHUS y’enkyukakyuka mu DNA yaffe (genetic mutation) , oba okutondebwa kw’obutoffaali obuziyiza endwadde (autoantibodies ) obulumba obutoffaali bwaffe ku ‘complement proteins’ mu baserikale baffe abaserikale b’omubiri . Enkyukakyuka zino mu buzaale zisobola okusikira bazadde baffe, oba ziyinza okubaawo mu ngeri ey’ekifuulannenge. Enkyukakyuka zino mu buzaale zikwata ku nkola ya puloteyina eziyitibwa ‘complement factors’ .

Lowooza ku ‘complement factors’ zino ng’abaserikale abatono abayamba obutoffaali bwaffe obw’abaserikale b’omubiri. Okufaananako n’okusiiga emmere, obutoffaali buno bufuna obuwuka obw’obulabe (nga bakitiriya ne akawuka) ne bubussaako akabonero obutoffaali obulala obuziyiza endwadde busobole ‘okubulya’. Ebisinga okukosebwa mu aHUS ze nsonga ezijjuliza H, I, 3, ne B.

Okugeza, omulimu gwa complement factor H kwe kukuuma obutoffaali obulamu mu mubiri gwaffe n’okukuuma complement factors endala obutakola nga tezeetaagisa. Kati teebereza ekibaawo singa ensonga eno ey’okujjuliza H tekola bulungi (kwe kugamba, singa wabaawo enkyukakyuka mu ggiini ya CFH) ? Obutoffaali bwaffe tebufuna bukuumi bwe bwetaaga. Olwo bino bye bintu ebibaawo:

• Obutoffaali bwaffe obw’abaserikale b’omubiri bulumba mu bukyamu ne bwonoona obutoffaali obubeera mu layini munda mu misuwa gyaffe (obutoffaali bw’omubiri) .
• Platelets zikuŋŋaana okwetooloola okwonooneka kuno ne zikola ekizimba ky’omusaayi. Kino kiziba emisuwa.
• Kino kikendeeza ku kutambula kw’omusaayi mu bitundu byaffe, ekibiviirako okwonooneka n’emirimu gyabyo okukendeera. Ensigo ze zisinga okukosebwa.
• Ebizimba bino era byonoona obutoffaali obulala obubuyitamu, ne kireetawo embeera eyitibwa hemolytic anemia .

Omanya otya oba olina aHUS?

Omusawo ajja kutera okuzuula aHUS okusinziira ku bubonero bwo n’ebyava mu kukeberebwa okukebera omuwendo gw’omusaayi gwo n’engeri ebitundu byo gye bikolamu. Era bajja kukola okukebera okukakasa nti tolina ndwadde ndala ezireeta obubonero obufaananako bwe butyo (nga STEC-HUS eyogeddwako waggulu).

Bikebere ki ebikozesebwa okuzuula aHUS?

Oyinza okwetaaga okukola ebigezo nga bino:

• Okukebera omusaayi: Complete Blood Count (CBC) , okukebera enkola y’ekibumba, n’ebirala.
• Okukebera obuzaale : Kino kikebera enkyukakyuka mu buzaale.
• Okukebera omusulo : Kebera oba temuli yinfekisoni nga E. coli.
• Kidney biopsy : Okutwala akatundu akatono ku kibumba okwekebejjebwa.
• Okukebera ebifaananyi : gamba nga ultrasound oba CT scan .

aHUS ejjanjabwa etya?

Obujjanjabi bwa aHUS businziira ku kivaako n’obuzibu bw’embeera eno. Bw’oba ​​olina obulwadde bwa aHUS obutono, omusawo ayinza okukulondoola n’okukuwa obujjanjabi obuyamba , gamba ng’okuteekebwamu omusaayi oba eddagala lya puleesa, bwe kiba kyetaagisa.

Wabula bw’oba ​​olina aHUS ey’amaanyi, oyinza okwetaaga obujjanjabi nga buno:

• Therapeutic plasma exchange : Mu kino, ekitundu kya plasma eky’omusaayi kiggyibwamu ne kiweebwa plasma empya.
• Rituximab oba eddagala eddala erikozesebwa okujjanjaba endwadde z’abaserikale b’omubiri.
• Okulongoosa omusaayi : Enkola y’okuyonja omusaayi ng’ensigo tezikola bulungi.
• Okukyusa ekibumba : Kino kikolebwa mu mbeera enzibu ennyo zokka.

Ku abo abalina enkyukakyuka z’obuzaale eza ‘complement’ gene mutations ezoogeddwako waggulu , abasawo batera okukozesa eddagala eriyitibwa eculizumab okujjanjaba aHUS. Eculizumab ekola nga eziyiza obutoffaali obumu obuyamba, okukomya abaserikale b’omubiri gwo okwonoona emisuwa gyo.

Waliwo ebizibu byonna ebiva mu bujjanjabi buno?

Bw’oba ​​okozesa Eculizumab , oyinza okuba nga tolina bukuumi butono okuva ku ddagala erigema obulwadde bwa meningococcal ne pneumococcal . N’olwekyo, bwe kiba kisoboka, omusawo ayinza okukuwa amagezi okugema eddagala lino nga tonnatandika kugema Eculizumab .

Ebiseera by’omu maaso biba bitya eri omuntu alina aHUS?

Singa embeera eno ezuulibwa era n’ejjanjabwa nga bukyali, embeera ng’obuvune obw’amaanyi mu kibumba obuva ku aHUS zisobola okuddukanyizibwa obulungi. Oyinza okuba n’ebiseera by’okusonyiyibwa nga tolina bubonero. Naye singa ekintu ekirala kiddamu okuleeta aHUS, kiyinza okuddamu okufuuka eky’obulabe eri obulamu.

Edda, nga kitundu, oba 50%, ku bantu abalina aHUS baafuna obulwadde bw’ekibumba obw’omutendera ogw’enkomerero (ESKD) , ekitegeeza nti ensigo zaabwe zaafuuka ezitakola bulungi.

Naye waliwo amawulire amalungi! Okunoonyereza okupya kulaga bulungi nti obulabe bwa ESKD bukendedde nnyo mu bantu abalina aHUS olw'obujjanjabi bwa Eculizumab .

Obulabe ki obw’okufa olw’obulwadde bwa aHUS?

Emisango egy’amaanyi egya aHUS, gamba ng’ekibumba okulemererwa oba okwonooneka kw’ebitundu ebirala, ddala bya maanyi. Mu mbeera ez’amaanyi bwe zityo, waliwo akabi k’okufa nga 15%.

aHUS esobola okuziyizibwa?

Olw’okuba kitera okuva ku nkyukakyuka mu buzaale, aHUS ebiseera ebisinga tesobola kuziyizibwa. Kyokka bw’oba ​​oli mu bulabe bw’okufuna obulwadde obw’amaanyi, oyinza okwewala ebimu ku bintu ebibuleetera .

Nze nneerabirira ntya?

Bw’oba ​​ozuuliddwa n’obulwadde bwa aHUS, yogerako n’omusawo wo ku ngeri gy’oyinza okwewala ebintu ebiyinza okwonoona embeera eno. Era kikulu okumanya obubonero bw’obulwadde obw’amaanyi.

Ddi lwe nnina okulaba omusawo?

Bw’oba ​​omaze ebbanga ng’owulira ng’okooye oba ng’okooye – ng’olinga alina obulwadde obutawona obutagenda kuggwaawo – yogerako n’omusawo wo. Ate era yogera ku bubonero obulala bwonna bw’obadde nabwo gye buvuddeko, gamba ng’okukyusa mu kaabuyonjo.

Ddi lwe nnina okugenda mu kitongole ekijjanjaba abalwadde ab’amangu (ETU) ?

Bw’oba ​​olina obubonero buno obw’amaanyi, genda mangu mu kisenge ky’abalwadde ab’amangu:

• Omusulo ogufuluma gukendeera nnyo.
• Okutabulwa oba okukyusa okutegeera.
• Okuzimba okw’amaanyi.
• Okukaluubirirwa ennyo mu kussa.

Bibuuzo ki bye nnina okubuuza omusawo?

Kiyinza okuba eky’omugaso okubuuza omusawo wo ebibuuzo nga bino:

• Embeera yange ya maanyi etya?
• Kiki ekyaviirako kino okweyongera?
• Nsobola okuziyiza embeera nga zino mu biseera eby’omu maaso?
• Bubonero ki bwe nnina okwegendereza?
• Ddi lwe nnandizzeemu okukulaba?

Embeera eno eyitibwa aHUS ya bulijjo etya?

aHUS si nnyangu nnyo.Embeera y’obujjanjabi. Bukwata abantu nga omu ku buli mitwalo bitaano. Busingako katono mu bantu abakulu okusinga mu baana.

N’ekisembayo, ebintu by’olina okujjukira

Okukizuula nti ggwe oba omwana wo olina embeera y’obuzaale kiyinza okukyusa obulamu. aHUS mbeera ya maanyi eyinza okuvaako omusaayi okuzimba n’okwonoona ebitundu by’omubiri. Wabula okukola eddagala eriyitibwa eculizumab gye buvuddeko kirongoosezza nnyo omuwendo gw’abantu abalina aHUS okuwangaala. Bw’oba ​​ozuuliddwa n’obulwadde bwa aHUS, yogerako n’omusawo wo ku ngeri gy’oyinza okwewala ebikuviirako n’okukendeeza ku bulabe bw’okulwala endwadde ez’amaanyi. Asobola okukuyamba okutegeera ky’olina okukola okusobola okubeera omulamu obulungi.

` aHUS, obulwadde bw’ekibumba, okuzimba omusaayi, abaserikale b’omubiri, enkyukakyuka mu buzaale, okukendeera kw’omusaayi, Eculizumab