Ènya nu tso dɔléle sia si gblẽa nu le wò lãkusi kple ayiku siaa ŋu le ɣeyiɣi ɖeka me ŋua? Mina míaƒo nu tso Goodpasture Syndrome ŋu!

Ðe kɔdzidɔ si nu metsina o nɔa ŋuwò ɣeaɖewoɣia? Ðe kɔdzi ma hea ʋu vi aɖe vɛ ɣeaɖewoɣia? Alo ɖe nèsena le ɖokuiwò me be gbɔgbɔ le sesẽm na ye, alo ɖeko ɖeɖi te ŋuwò eye aɖuɖɔ si dona le lãme na wò dzi ɖe kpɔtɔa? Esiawo ate ŋu anye nu vevi aɖe ƒe dzesiwo. Egbea míele nu ƒom tso nɔnɔme ɖeka si mebɔ o alea ŋu, gake esi wòle be nànya.

Nukae nye Goodpasture Syndrome?

Kpuie ko la, Goodpasture Syndrome nye dɔléle si mebɔ o gake ate ŋu ade ame ƒe agbe afɔku me si dɔlélenutsiŋutete wɔna . "Autoimmune" fia be míaƒe ŋutilã ƒe ametakpɔnu, dɔlélenutsiŋutete, dzea mía ŋutɔwo ƒe lãmenugbagbevi kple lãkusi siwo le lãmesẽ me dzi dzedze gɔme vodadatɔe.

Le dɔléle sia me la, míaƒe dɔlélenutsiŋutete dzea protein aɖe si woyɔna be collagen dzi. Collagen le abe xɔtunu si le míaƒe ŋutilã me ene. Collagen kpena ɖe míaƒe ŋutigbalẽ, lãmekawo, lãkusiwo, lãkusiwo, kple ƒuwo ŋu be ŋusẽ nɔa mía ŋu.

Eyata, ne Goodpasture syndrome, protein siwo wò ŋutilã wɔna dometɔ aɖewo, míeyɔna wo be antibodies, yi ɖaléna ɖe collagen si le wò lãkusi kple ayiku me ŋu. Ne edzɔ alea la, dɔdzẽ dzea egɔme le teƒe mawo, eye lãkusi ma tea gbagbã vivivi. Ne womewɔ atike na esia o la, ate ŋu ana ayikuwo naxɔ abi vevie (glomerulonephritis), si ate ŋu ana ayiku nawɔ dɔ nyuie o. Àte ŋu adze ʋu geɖe dodo gɔme tso lãkusiwo me hã (ʋu si dona le aklã me). Le go geɖe me la, nusi koŋ nana dɔléle sia kunae nye ʋu si dona le lãmekawo me.

Ŋkɔ bubu si wotsɔ na esiae nye:

• Dɔléle si tsia tre ɖe glomerular basement membrane ŋu (anti-GBM) dɔléle
• Goodpasture dɔléle

Aleke gbegbee esia bɔe?

Esia nye dɔléle si mebɔ kura o . Le ɖɔktawo ƒe nya nu la, ame 100,000 ɖesiaɖe ƒe sia ƒe la, ame yeye ɖeka va ɖo eve koe woxɔa dɔléle sia le ƒe sia ƒe. Eyata àte ŋu akpɔ alesi gbegbe wòbɔe la le susu me.

Nukawoe nye dzesiawo?

Goodpasture syndrome nye `(aklã-ayiku)` nɔnɔme. Esia fia be dɔlélea gblẽa nu le lãkusi kple ayiku siaa ŋu le ɣeyiɣi ɖeka me. Zi geɖe la, dzesi gbãtɔ siwo dzena la do ƒome kple lãkusiwo. Esiawoe nye:

• Yagbɔgbɔ sesẽ (gbɔgbɔtsixe): Menye ne èle atrakpui liam ko o, ke ne èle ɖiɖim ɖe eme gɔ̃ hã.
• Akɔtave : Akɔtave ne wole gbɔgbɔm alo le kɔdzim.
• Kɔdzi : Kɔdzidɔ si nɔa anyi ɖaa, eye wòvea ame ɣeaɖewoɣi.
• Toɣliɖeɖe aɖe tsoa akɔta ne wole gbɔgbɔm : Ate ŋu anye gbeɖiɖi si le ʋuʋum, abe ɖe nane tsi akɔta me ene.
• Ðeɖi te ŋunye ŋutɔ.`(ɖeɖiteameŋu)`: Seselelãme be yegbɔdzɔ ale gbegbe be màte ŋu awɔ naneke o.
• Ʋu si dona le ŋɔti me (epistaxis).
• Ʋu ƒe kɔdzi : Esia nye dzesi si doa vɔvɔ̃ na ame geɖe eye wòhiã be woakpɔ egbɔ enumake.
• Pallor : Ŋutilãa trɔna zua ɣie le ʋu ƒe anyimanɔmanɔ ta.

Dzesi siwo do ƒome kple ayiku hã ate ŋu adze emegbe. Wo dometɔ aɖewoe nye:

• Ʋumenugbagbevi dzĩ siwo le ʋua me dzi ɖeɖe kpɔtɔ, si nye ʋusɔgbɔdɔ .
• Ʋu si le aɖuɖɔ me (hematuria): Aɖuɖɔ ate ŋu azu dzĩ alo aŋutiɖiɖi.
• Aɖuɖɔ si dona le lãme mesɔ gbɔ o wu alesi wònɔna ɖaa.
• Ʋusɔgbɔdɔ .
• Aɖuɖɔtoe kple ʋuʋudedi : Esia ate ŋu adzɔ ne nuɖuɖu megadzroa ame hã o.

Nukae hea esia vɛ?

Goodpasture syndrome dzɔna ne dɔlékuiwutike siwo míaƒe dɔlélenutsiŋutete wɔna dze collagen si le glomerular basement membrane (GBM) si le ayikuwo me dzi. GBM sia nye glomeruli, si nye ʋu ƒe akpa suesuesue siwo le míaƒe ayiku me la ƒe akpa aɖe. Dɔlékuiwutike siawo dzea kolagen si le yakotoku siwo le lãkusi me hã dzi. Esia wɔnɛ be lãkusia gblẽna, si wɔnɛ be ʋu dona le lãme na ame eye wònana gbɔgbɔ sesẽna.

Womenya nusita tututu dɔlélenutsiŋutete dzea eya ŋutɔ ƒe kolagen dzi haɖe o. Ate ŋu adzɔ be nutomenuwo kple míaƒe domenyiŋusẽfianuwo nakpɔ gome le nɔnɔmea me. Numekukuwo ɖee fia be kadodo sẽŋu aɖe le amesiwo ŋu dɔlélea le kple domenyiŋusẽfianu ƒe dzesi aɖe si woyɔna be Human Leukocyte Antigen (HLA) DR15 dome. Esia nye protein si kpena ɖe dɔlélenutsiŋutete ŋu wòdea vovototo mía ŋutɔwo ƒe lãkusiwo kple dutanuwo dome.

Ɣeaɖewoɣi la, dɔléle sia ate ŋu adze le dɔlékui aɖe, abe dzamezã alo dzamezã ene, megbe. Azɔ hã, wò:

• Ne èno atama la .
• Ne ègbɔ atike vɔ̃ɖiwo abe cocaine ene .
• Afɔku si le dɔléle sia xɔxɔ me lolo wu ne èdo go ga ƒe ke alo atike siwo nye hydrocarbon abe methane kple propane ene.

Ameka dzie esia le ŋusẽ kpɔm ɖo wu?

Goodpasture syndrome ate ŋu adzɔ le ƒe ɖesiaɖe me, gake ebɔ ɖe sɔhɛwo me wu, siwo xɔ tso ƒe ewo va ɖo blaetɔ̃, eye emegbe wògabɔ ɖe amesiwo le woƒe ƒe 60 kple 70 me.

Aleke wowɔa dɔlélea ƒe dzesi?

Ne èyi ɖɔkta gbɔ la, woabia wò dzesiwo eye woado wò ŋutilã kpɔ. Tsɔ kpe ɖe eŋu la, woate ŋu awɔ dodokpɔ siwo gbɔna hã atsɔ aɖo kpe Goodpasture ƒe dɔlélea dzi:

• Ʋudodokpɔ : Esiawo léa ŋku ɖe wò ayiku ƒe ʋuʋu ƒe agbɔsɔsɔme si wobu (eGFR) ŋu, si dzidzea alesi wò ayikuwo le dɔ wɔm nyuie.
• Aɖuɖɔtoe dodokpɔwoAɖuɖɔtoe me dzodzro: Esia léa ŋku ɖe ʋu si le wò aɖuɖɔ me alo protein ƒe agbɔsɔsɔ si sɔ gbɔ (proteinuria) ŋu hã.
• Nɔnɔmetatawo dodokpɔ : Wowɔa akɔta ƒe X-ray alo CT scan be woatsɔ akpɔe ɖa be lãkusi me gblẽ hã.
• Bronchoscopy : Le dɔ sia wɔwɔ me la, ɖɔkta dea mɔ̃ tsɛ aɖe si didi (bronchoscope) si me fotoɖemɔ̃ kple akaɖi le to wò ŋɔti alo nu me be wòakpɔe ɖa be nu gblẽ le wò lãkusi me hã.
• Ayiku me lãmenugbagbeviwo ɖeɖe : Le esia me la, woɖea lãkusi sue aɖe tso ayiku me eye wodoa wo kpɔna le nusuekpɔmɔ̃ me be woakpɔe ɖa be ayikudɔ le eme hã.

Ðe woate ŋu ada Goodpasture ƒe dɔlélea?

Ẽ, woate ŋu ada esia ne wokpɔ dɔlélea nyuie eye wodze edada gɔme kaba.

Aleke wowɔa atike nɛ?

Goodpasture ƒe dɔlélea dada toa vovo le alesi dɔlélea nu sẽe nu. Ne dɔlélea mesẽ akpa o la, ɖɔktawo ate ŋu aŋlɔ atikewo abe:

• Corticosteroids : Le kpɔɖeŋu me, atikewo abe prednisone ene. Esiawo kpena ɖe ame ŋu be ʋu si dona le lãkusiwo me nu tsona.
• Dɔlélenutsiŋutete ƒe atikewo : Atikewo abe cyclophosphamide ene. Esiawo xea mɔ na wò dɔlélenutsiŋutete be wòagadze eya ŋutɔ ƒe lãkusiwo dzi o.
• Atike siwo ɖea ʋu ƒe sisi dzi kpɔtɔna (atike siwo tsia ʋusɔgbɔdɔ nu): Esiawo kpena ɖe ame ŋu wòɖua ʋu ƒe sisi dzi eye wòɖea nusiwo gblẽ le ayiku ŋu dzi kpɔtɔna.

Tsɔ kpe ɖe eŋu la, ɖɔktawo zãa atike aɖe hã si woyɔna be plasmapheresis . Esia bia be nàɖe ʋu tso ʋuka aɖe si le wò alɔnu me to aŋe me, eye nàma ʋua ƒe akpa si nye tsi, si woyɔna be plasma, kple ʋumenugbagbeviwo dome. Dɔlékuiwutike siwo gblẽa nu le GBM ŋu la le ʋumenugbagbevi dzĩ sia me. Emegbe woɖea ʋumenugbagbeviɣia ɖa, eye wogatsɔa ʋumenugbagbeviɣi si tso ame si le lãmesẽ me la dea wò ʋumenugbagbeviwo me hegbugbɔna naa wò. Kpuie ko la, eɖea dɔlékuiwutike siwo gblẽa nu le ame ŋu la ɖa le wò ŋutilã me.

Ɣeyiɣi didi kae wòxɔna hafi wòhayana le atikewɔwɔ vɔ megbe?

Ele be nàno dɔlélenutsiŋutete ƒe atikewo abe ɣleti 6 va ɖo 12 ene. Le go geɖe me la, ɖewohĩ ahiã be nàwɔ plasmapheresis gbesiagbe kwasiɖa geɖe.

Nukae adzɔ emegbe?

Ne womeda Goodpasture ƒe dɔlélea o la, ate ŋu ana ʋu nado le lãkusi me si ate ŋu awu ame eye ayiku nawɔ dɔ nyuie o. Gake ne wokpɔ dɔlélea dze sii kaba eye wodze edada gɔme la, zi geɖe la, emetsonua nyona. Ɣeyiɣi kpui aɖe koe wò ŋutilã wɔa dɔlékuiwutike mawo siwo gblẽa nu le ame ŋu – tso kwasiɖa ʋee aɖewo va ɖo abe ƒe eve ene. Ƒã ŋutɔ be dɔlélea gbugbɔna alo eƒe gbugbɔgadzɔ.

Esia ƒe kuxi sesẽtɔ kekeakee nye ayiku ƒe dɔmawɔmawɔ nyuie. Ne edzɔ alea la, ehiã be woawɔ atike na ʋu si woɖe tso ʋu me be woatsɔ akɔ ʋua me.Ehiã be woado ayiku na ame.

Nya kae le asinye magblɔ tso nye agbenɔɣi ŋu?

Ame siwo ŋu Goodpasture ƒe dɔlélea le dometɔ siwo ade 80% ƒe agbetsitsi ƒe atɔ̃ nye 80%. Emae nye ne wokpɔ dɔlélea kaba eye wodae nyuie.

Ðe woate ŋu axe mɔ ɖe esia nua?

Ðewohĩ manya wɔ be woaxe mɔ ɖe enu keŋkeŋ o. Gake àte ŋu aɖe wò afɔkua dzi akpɔtɔ ne èƒo asa na nu aɖewo:

• Hydrocarbons, si gɔmee nye nusiwo le abe methane, propane, mɔ̃memi, kerosene, kple aŋɔ ene.
• Ga ƒe ke.
• Cocaine si wotsɔna ƒoa ƒui.
• Nusiwo wotsɔ dea ama ɖae.

Aleke mawɔ alé be na ɖokuinye?

Ne Goodpasture syndrome le ŋuwò la, anyo be nàwɔ nusiawo:

• Dzudzɔ atamanono keŋkeŋ. Ƒo asa na teƒe siwo ame bubuwo noa atama le (i.e. ƒo asa na `dzudzɔ evelia` me nɔnɔ).
• Ƒo ɖokuiwò ɖe kamedede me aɖabaƒoƒo 30 ya teti gbesiagbe.
• Dze agbagba nàɖe nuteɖeamedzi dzi akpɔtɔ .

Ɣekaɣie wòle be makpɔ ɖɔkta?

Ne èkɔ ʋu, gbɔgbɔ sesẽ na wò, aɖuɖɔtoe dzi ɖe kpɔtɔ, alo dzesi bubu siwo míeyɔ ɖe etame dometɔ aɖe le ŋuwò la, yi ɖɔkta gbɔ enumake. Ne èkpɔe kaba la, edzia mɔnukpɔkpɔ si li be nàhaya la ɖe edzi ŋutɔ. Ne womewɔ atike nɛ o la, ate ŋu ana ayiku nagblẽ tegbee eye wòana aɖuɖɔtoe me kuxi siwo wua ame gɔ̃ hã.

Nya kawoe wòle be nàbia ɖɔktaa?

Ne èyi ɖɔkta gbɔ la, àte ŋu abia nya siawo:

• Aleke mawɔ anya kple kakaɖedzi be Goodpasture Syndrome le ŋunye?
• Aleke gbegbee nye nɔnɔmea nu sẽe?
• Atikewɔwɔ kawoe nèkafu?
• Nukae mate ŋu awɔ atsɔ alé be na nye ayiku?
• Ðe wòle be makpɔ ayikuŋutinunyala alo aklãŋutinunyala?
• Àte ŋu agblɔ kpekpeɖeŋunaha aɖe si kpena ɖe amesiwo ŋu dɔléle sia le ŋu nam?

Nukae nye Goodpasture ƒe dɔlélea ƒe nɔnɔme vevi etɔ̃ (Triad)?

Le atikewɔwɔ me la, ``triad'' fia dzesi vevi etɔ̃ siwo ɖɔa atikewɔwɔ ƒe nɔnɔme aɖe. Goodpasture Syndrome ƒe dzesi vevi etɔ̃e nye:

• Ʋu si dona le lãkusiwo me.
• Ayikuwo ƒe dɔdzẽ ƒe dzidziɖedzi kabakaba.
• Dɔlékuiwutike mawo siwo woyɔna be ``anti-GBM antibodies'' ƒe anyinɔnɔ le ŋutilã me.

Dɔléle sia ƒe amedzidzedze ate ŋu aɖe fu na ame ŋutɔŋutɔ eye wòatsi akogo, vevietɔ esi womenya nusi tututu gbɔ wòtso haɖe o ta. Ate ŋu ado kplamatse wò gbesiagbedɔwo, eye ɖewohĩ ahiã be nàtrɔ dɔ gɔ̃ hã ne ètsɔ gaku, mɔ̃memi, alo aŋɔ wɔa dɔ.

Mlɔeba la, nusi dzi wòle be woaɖo ŋkui

Eyata, Goodpasture Syndrome nye nusi wòle be nàvɔ̃ na, gake ne ède dzesi dzesiawo kaba, yi ɖɔkta gbɔ kaba, eye nèdze atikewɔwɔ gɔme la, àte ŋu akpɔ emetsonu nyuiwo ŋutɔ. Lé ŋku ɖe tɔtrɔ siwo le wò ŋutilã me ŋu. Ne èse le ɖokuiwò me be nane wɔ nuku la, ele vevie be nàdi atikewɔlawo ƒe aɖaŋuɖoɖo evɔ màgbee be enye nu sue aɖe o. Menye wò ɖeka koe o, eye ɖɔktawo ate ŋu akpe ɖe ŋuwò.

` Goodpasture syndrome, Anti-GBM dɔléle, aklã me ʋu si dona le lãme na ame, ayiku ƒe dɔléle, autoimmune dɔlélewo, gbɔgbɔ sesẽ, ʋu le aɖuɖɔ me