Ingabe amaprotheni emzimbeni aphazamisekile? Sikhuluma ngale amyloidosis!

Ingabe nawe uzizwa ukhathele, ukhathele, futhi mhlawumbe ulahlekelwa isisindo ngendlela engenakucatshangwa kulezi zinsuku? Ngezinye izikhathi, izinguquko ezincane emizimbeni yethu zingaholela ezintweni ezinkulu. Namuhla sizokhuluma ngesifo esenzeka lapho uhlobo oluthile lweprotheni emizimbeni yethu luba lubi, luphenduke kabi, futhi luhlala ezithweni zethu ezibalulekile. Yilokhu esikubiza ngokuthi i-amyloidosis . Ungakhathazeki, sizokhuluma ngalokhu kalula nangendlela ongayiqonda.

Iyini ngempela i-amyloidosis?

Kalula nje, i-amyloidosis yisimo lapho amaprotheni e-amyloid (empeleni angamaphrotheni angajwayelekile) emizimbeni yethu ehlangana, ahlangane, futhi akhe amaqoqo amancane, njengemicu ebizwa ngokuthi ama-fibrils, ahlala ezithweni ezibalulekile njengenhliziyo yethu, izinso, nesibindi. Uma enza kanjalo, futhi uma engelashwa, la ma-fibrils angajwayelekile angabangela umonakalo omkhulu ezithweni zethu.

Ngezinye izikhathi la maprotheni angajwayelekile angaqongelela endaweni encane nje yesitho. Lokhu kubizwa ngokuthi i-localized amyloidosis . Lokhu kungathinta isikhumba sethu, ingxenye encane yesinyi, noma indlela yokuphefumula. Kodwa-ke, kaningi lawa ma-fibril afakwa esithweni sonke noma ezithweni ezahlukahlukene emzimbeni wonke. Lokhu kubizwa ngokuthi i-systemic amyloidosis .

Kunezindaba ezinhle. Odokotela bangamisa ukukhiqizwa kwamaprotheni angajwayelekile abangela i-amyloidosis. Kwezinye izinhlobo ze-amyloidosis, amasosha ethu omzimba angasiza ekususeni la maprotheni. Lokhu kunganciphisa izimpawu zakho futhi kuthuthukise impilo yezitho ezithintekile.

Ingabe zikhona izinhlobo ze-amyloidosis?

Yebo, odokotela bahlukanisa lesi sifo ngokusekelwe ohlotsheni lweprotheni ethintekile. Izinhlobo eziyinhloko ezibonwayo yilezi:

• I-AL amyloidosis : Lokhu kwenzeka lapho umzimba wakho wenza inani elingavamile lamaprotheni ochungechunge lokukhanya (okuyingxenye enkulu yama-antibodies). Lawa ma-fibril avame ukwakheka enhliziyweni (i-cardiac amyloidosis) kanye nezinso. I-AL amyloidosis ingathinta nezinzwa zakho, isikhumba, kanye nezitho zokugaya ukudla. Lolu uhlobo oluvame kakhulu lwe-amyloidosis.
• I-AA amyloidosis : Kulokhu, izingcezu zeprotheyini ye-serum A ziyanqwabelana ezithweni zakho zomzimba. Lokhu kungenzeka uma unesifo sokuvuvukala isikhathi eside, okusho isifo esibangela ukuvuvukala emzimbeni (isibonelo, isifo samathambo, isifo samathumbu esivuvukalayo, noma izifo zesikhathi eside). Lokhu kungathinta izinso zakho, izitho zokugaya ukudla, noma inhliziyo.
• I-ATTR amyloidosis: Kulesi simo, isibindi sakho senza inani elingavamile leprotheyini ye-transthyretin . Le phrotheyini ye-transthyretin engajwayelekile ifakwa enhliziyweni futhi ngezinye izikhathi emithanjeni. Ezinye izinhlobo zizuzwa njengefa (i-familial amyloidosis), kanti ezinye zikhula kamuva ekuphileni njengoba umuntu ekhula (i-wild-type ATTR).

Sivame kangakanani lesi sifo?

I-Amyloidosis empeleni iyisifo esingavamile . Isibonelo, ngisho nase-United States, odokotela balinganisela ukuthi amacala amasha angu-1,275 kuya ku-3,200 kuphela e-AL amyloidosis atholakala minyaka yonke. Ngakho-ke, akusona isifo esivamile.

Ziyini izimpawu zalesi sifo?

Izimpawu ze-amyloidosis zingahluka kumuntu nomuntu, kuye ngohlobo lweprotheni engajwayelekile kanye nokuthi ama-fibrils abekwe kuphi.

Cabanga nje, uma la maprotheni efakwa enhliziyweni (`(cardiac amyloidosis)`), khona-ke izimpawu ezenzekayo ubuthakathaka, ubunzima bokuphefumula, ukuquleka (lokhu kungaba uphawu lwezigqi zenhliziyo ezingavamile) noma ukwehluleka kwenhliziyo okunokuvuvukala emilenzeni.

Futhi, uma la maprotheni efakwa ezinso (i-renal amyloidosis), kungase kube nezimpawu ezifana nokuvuvukala kwezinyawo nemilenze kanye nokubona amabhamuza emchameni .

Kunezimpawu eziningana ezivamile ezingabonakala ku-amyloidosis:

• Ukuzizwa ukhathele ngendlela engakholeki.
• Ukwehlisa isisindo ngaphandle kwesizathu.
• Ubuthakathaka obujwayelekile noma ukungazweli emilenzeni, ukungakwazi ukubamba into ngendlela efanele.
• Ukuzwa sengathi izandla azinamizwa.
• Ukushintsha kwesikhumba: Lokhu kusho izinto ezifana nesikhumba esiphuka kalula kanye namabala ansomi (purpura) azungeze amehlo.

Kungani kwenzeka le amyloidosis?

I-Amyloidosis yenzeka lapho amaprotheni emizimbeni yethu egoqeka futhi enamathela. Ahlangana ndawonye, ​​​​akha ama-clumps, noma ama-fibrils, bese ehlala ezithweni nasezicutshini. Odokotela bakubiza lokhu ngokuthi "isifo sokugoqeka kwamaprotheni." Amaprotheni okufanele ahleleke, ngamaketanga amade, ayabhidlika futhi angakwazi ukuhlukaniswa kahle ngumzimba, okubangela izinkinga.

Izinto ezingabangela lokhu yilezi:

• Ushintsho lwezakhi zofuzo (ukuguqulwa kwezakhi zofuzo) : Ungazuza ushintsho lwezakhi zofuzo (ukuguqulwa kwezakhi zofuzo) olwenza amaprotheni e-amyloid angajwayelekile. Noma, lokhu kuguqulwa kwezakhi zofuzo kungenzeka ngesikhathi sokuphila komuntu, ngezizathu ezingaziwa.
• Ezinye izimo zezokwelapha eziyisisekelo: Ngezinye izikhathi i-amyloidosis ingabangelwa yisimo sezokwelapha esihlukile. Yilokhu okwenzeka ku-AA amyloidosis. Lokhu kuvame kakhulu kubantu abanezifo zesikhathi eside noma izimo zokuvuvukala ezingamahlalakhona njenge-rheumatoid arthritis.

Ubani onamathuba amaningi okuthuthukisa lokhu? (Izici Eziyingozi)

Kunezici eziningana eziyingozi ezingathinta ukuthuthukiswa kwe-amyloidosis:

• Ubudala : Lesi sifo sivame ukutholakala kubantu abaneminyaka engama-60 noma ngaphezulu .
• Ubulili : Amadoda anamathuba amaningi okuthola lesi sifo.
• Uhlanga : Kutholakale ukuthi abantu abamnyama e-United States banamathuba amaningi okuthola ifa lokuguqulwa kwezakhi zofuzo okubangela uhlobo lwe-'ATTR amyloidosis' oluzuzwe njengefa. (Kunemininingwane embalwa eqondile ngalokhu ezweni lakithi, kodwa ngokuvamile kuthiwa ezinye izinhlanga zithambekele kakhulu ezinhlotsheni ezithile.)
• Ezinye izimo zezokwelapha : Ukuba nesimo sokuvuvukala esingamahlalakhona kwandisa ingozi yokuthola i-AA amyloidosis. Futhi, phakathi kuka-12% no-15% wabantu abane- multiple myeloma, uhlobo lomdlavuza wegazi, bathola i-AL amyloidosis.
• I-dialysis yesikhathi eside : Abantu abanesifo sezinso esingamahlalakhona asebesebenzisa i-dialysis isikhathi eside bangaba nezinhlobo ezithile ze-amyloidosis.
• I-Amyloidosis emndenini : Ezinye izinhlobo ze-amyloidosis zingangena emindenini. Lokhu kusho ukuthi zingazuzwa njengefa.

Yiziphi izinkinga ezingabangelwa yilokhu?

Lezi zinhlayiya ze-amyloid zingavimbela izitho ezithintekile ekusebenzeni kahle. Izinkinga ezingaba khona zifaka:

• Ukungasebenzi kahle kwenhliziyo futhi ekugcineni ukwehluleka kwenhliziyo.
• Isifo sezinso kanye nokwehluleka kwezinso ekugcineni .
• I-neuropathy (ebangela ukungazweli kanye nobuthakathaka).

Kodwa khumbula, uma ufuna ukwelashwa nethimba lezokwelapha elifanele, unganciphisa amathuba alezi zinkinga.

Lesi sifo sihlolwa kanjani ngokunembile? (Ukuxilongwa)

Odokotela benza i-biopsy ukuze bathole iphrotheni ebangela i-amyloidosis. Lokhu kuhilela ukuthatha ingxenye encane yezicubu esithweni esithintekile noma endaweni lapho la ma-fibril evame ukuqongelela khona (isibonelo, emafutheni angaphansi kwesikhumba sesisu sakho noma emnkantsheni wakho wamathambo).

Ngaphezu kwalokho, kunezinye izivivinyo ongazihlola:

• Ukuhlolwa kwegazi nomchamo : Lokhu kuhlolwa kungahlola amazinga angavamile amaprotheni egazini noma emchameni wakho. Kungasetshenziswa futhi ukubona ukuthi izitho zomzimba ezithintekile zisebenza kanjani.
• Ukuhlolwa kwezithombe: Lokhu kuhlolwa kungasiza odokotela ukuthi babone umonakalo ezithweni ezithintekile. Kuye ngokuthi ama-fibril akuphi, ungadinga i-echocardiogram , ebheka inhliziyo, i- MRI , ekhombisa izithombe ezinemininingwane zezitho nezicubu, noma okunye ukuhlolwa.
• Ukuhlolwa kofuzo : Uma udokotela wakho esola ukuthi une-amyloidosis ngenxa yokuguquka kofuzo, lokhu kuhlolwa kungenziwa.

Ingabe zikhona izigaba zesifo?

Yebo, odokotela basebenzisa i-amyloidosis staging ukuze bathole ukuthi lesi sifo sisakazeke kangakanani nokuthi sibi kangakanani. Kunezinhlelo ezahlukene ze-staging zezinhlobo ezahlukene zesifo. Ukuze ahlele lesi sifo, udokotela wakho angase acabangele okulandelayo:

• Izinga lomonakalo ezithweni zomzimba.
• Ukuhlolwa kwegazi kubonisa inani leprotheyini engajwayelekile uma kuqhathaniswa neprotheyini evamile ye-amyloid.
• Izimpawu zakho kanye nobunzima bazo.

Kubaluleke kakhulu ukukhuluma nodokotela wakho ngesigaba se-amyloidosis yakho nokuthi sizothinta kanjani ukubikezela kwakho.

Yiziphi izindlela zokwelapha ezitholakalayo?

Odokotela baphatha i-amyloidosis ngokwelapha isimo esiyisisekelo. Ukwelashwa kunganciphisa ukuqhubeka kwe-amyloidosis futhi kuvimbele ukwakheka kwama-fibrils amasha. Imithi ingasiza futhi ekunciphiseni izimpawu zakho. Ku-AL amyloidosis, lapho ukwelashwa kuvimbela ukwakheka kwama-deposit amasha e-amyloid, amasosha omzimba wakho angasusa lawo asevele ekhona. Ucwaningo lusaqhubeka ukuthola izindlela zokuthuthukisa inqubo yokususa ama-amyloid ezithweni zakho zomzimba.

Ezinye zezindlela zokwelapha i-amyloidosis yilezi:

• Ukwelashwa ngamakhemikhali : Lokhu kwelashwa kubhubhisa amaseli e-plasma angajwayelekile enza amaprotheni ochungechunge lokukhanya anephutha abangela i-AL amyloidosis. Abantu abaningi bathatha i -steroid kanye nemithi yamakhemikhali.
• Ukwelashwa okuqondiwe : Lokhu kwelashwa kuhlose amaprotheni athile, izakhi zofuzo, noma izicubu ezibangela i-amyloidosis. Kuhilela imithi evimbela amaprotheni ukuthi angasondeli kahle.
• Ukufakelwa kwezitho zomzimba : Uma izinso, isibindi, noma inhliziyo yonakele kwaze kwaba yilapho zingasakwazi ukusebenza kahle, odokotela bangase batuse ukufakelwa. Lokhu kungaba ukwelashwa kwezinhlobo ze-amyloidosis ezizuzwe njengefa.

Yini okufanele ikhathaze umuntu one-amyloidosis?

Udokotela wakho angelapha izimpawu zakho, alawule ukusabalala kwesifo, futhi kwezinye izimo, asize ngisho nokubuyisela emuva lesi sifo. Kodwa-ke, ezinye izinhlobo ze-amyloidosis, uma zingelashwa, zingabangela umonakalo wezitho zomzimba osongela ukuphila. Yingakho kubaluleke kakhulu ukuqaphela lesi sifo kusenesikhathi bese uqala ukwelashwa ngokushesha.

Ufuna ukuzinakekela kanjani nawe?

Ngenxa yokuthi kunezinhlobo eziningana ze-amyloidosis, ayikho indlela eyodwa yokuphatha lesi sifo. Buza udokotela wakho ukuthi yiziphi izinyathelo ezifanele wena.

Lokhu kungafaka:

• Ukunakekela impilo yakho yomzimba ngokudla ukudla okunempilo nokuvivinya umzimba njalo .
• Beka impilo yakho yengqondo kuqala . Buza udokotela wakho ngamaqembu okusekela abantu abane-amyloidosis. Kungaba yindlela enhle yokubhekana nokucindezeleka kanye nesizungu sokuphila nesifo esingavamile kangaka, nokuxhumana nabanye ababhekene nento efanayo nawe.

Kufanele ubonane nini nodokotela?

Njengoba izimpawu zihluka kakhulu, kungaba nzima ukwazi ukuthi uzobona nini udokotela. Kodwa ngokuvamile, uma unezimpawu ongaziqondi futhi ongaziqedi ngemva kwesikhathi esithile, kufanele nakanjani ubone udokotela. Kungase kungabi yi-amyloidosis, kodwa uma kunjalo, uma wazi ngokushesha, kungcono.

Yimiphi imibuzo okufanele uyibuze udokotela?

Nazi ezinye imibuzo ongayibuza udokotela wakho:

• Uhlobo luni lwe-amyloidosis enginalo?
• Kungani ngithole i-amyloidosis?
• Hlobo luni lokwelashwa engiludingayo?
• Iyini imiphumela emibi yokwelashwa?
• Iyini i-prognosis yami ngemva kokwelashwa?

Okokugcina, izinto okufanele uzikhumbule (Umyalezo Wokuya Ekhaya)

I-Amyloidosis iyisifo esiyinselele ngempela ngoba sithinta wonke umuntu ngendlela ehlukile. Kwezinye izimo, singalawuleka. Kwezinye izimo, singasongela impilo. Lokhu kungaba yinto edidayo futhi ekhungathekisayo. Futhi, ngoba yisifo esingavamile, ungase uzizwe uwedwa lapho udinga usizo kakhulu.

Into ebaluleke kakhulu ukukhumbula ukuthi awuwedwa. Udokotela wakho angakuchazela izindlela zokwelapha ezingcono kakhulu, kuye ngohlobo lwe-amyloidosis onayo. Bangakukhombisa nezinsiza kanye namaqembu okusekela angakusiza. Ithimba lakho lezokwelapha lingakuqondisa ukuthola ukwelashwa okungcono kakhulu kanye nokusekelwa okudingayo ukulwa nalesi sifo.

I- Amyloidosis, amaprotheni, amaprotheni angajwayelekile, ukulimala kwezitho zomzimba, isifo senhliziyo, isifo sezinso, izifo zezinzwa