Kuyini i-AL Amyloidosis? Ake sixoxe ngalesi sifo esiyingozi!

Wake wezwa ngalesi sifo esibizwa ngokuthi 'i-AL Amyloidosis'? Mhlawumbe akunjalo, akunjalo? Empeleni lokhu kuyinto engavamile, okusho ukuthi akusona isifo esithinta wonke umuntu. Kodwa-ke, uma kwenzeka, kungaba yinto engathí sina. Kalula nje, kungushintsho ohlotsheni lweseli olubizwa ngokuthi 'amaseli e-Plasma' esinawo emizimbeni yethu, futhi amanye amaprotheni akhiqizwa yiwo ayaphuma ekulawuleni futhi agcine ezithweni ezahlukahlukene zomzimba wethu, azilimaze. Kufana nomshini owawusebenza kahle futhi ngokuzumayo waqala ukwenza izingxenye ezingalungile. Ake sixoxe ngalokhu ngemininingwane ethe xaxa, ngoba kubaluleke kakhulu ukuqaphela lokhu.

Iyini ngempela i-'AL Amyloidosis'?

Le `AL amyloidosis` uhlobo olulodwa lweqembu elikhulu lezifo ezibizwa ngokuthi i-`Amyloidosis`. I-`Amyloidosis` isifo esingavamile esenzeka lapho kukhona ushintsho, noma 'ukuguqulwa', 'kumaseli e-plasma' emnkantsheni wethu wamathambo. Lawa maseli e-plasma ashintshiwe akhiqiza uhlobo olungavamile lweprotheni. Lawa maphrotheni, njengamabhola entambo ahlanganisiwe, ayahlangana, futhi ahlala ezithweni nasezicutshini zethu ezibalulekile.

Ku-AL amyloidosis, uhlobo lweprotheyini oluphazamiseka kanje yiprotheyini yochungechunge lokukhanya. Lawa maprotheni ochungechunge lokukhanya ayizingxenye zama-antibodies asetshenziswa yimizimba yethu ukulwa nezifo. Lawa ma-antibodies enziwa ngamaseli e-plasma afanayo engikhulume ngawo ekuqaleni.

Ngokuvamile, i-AL amyloidosis ithinta kakhulu inhliziyo kanye/noma izinso zethu. Kodwa-ke, ngezinye izikhathi ingathinta isisu, amathumbu, imizwa kanye nesikhumba.

Into engcono kakhulu ukuthi uma lesi sifo siqashelwa kusenesikhathi futhi selashwa ngokushesha , ngezinye izikhathi singaphathwa njengesifo esingamahlalakhona. Kodwa-ke, uma singelashwa, singaholela ezimweni ezisongela ukuphila ngisho nokufa. Ngakho-ke lokhu akuyona into okufanele ithathwe kalula.

Zisho ukuthini izinhlamvu ezimbili ezithi `AL` kuleli gama?

Kunezinhlobo ezahlukene ze-amyloidosis. Uhlobo ngalunye luqanjwe ngeprotheni engavamile ebangela lesi sifo. Ku-AL amyloidosis, uhlamvu u-A lumelela i-amyloidosis kanti uhlamvu u-L lumelela iphrotheni ebizwa ngokuthi uchungechunge lokukhanya olubangela lesi sifo. Ezinye izinhlobo ezaziwayo ze-amyloidosis yi-AA amyloidosis (ebangelwa yiprotheni ye-amyloid A esegazini) kanye ne-ATTR amyloidosis (ebangelwa yi-transthyretin).

I-'AL amyloidosis' iwuthinta kanjani umzimba wami?

Kalula nje, i-AL amyloidosis yisifo samaseli e-plasma. Amaseli e-plasma ayingxenye yesimiso sethu somzimba sokuzivikela. Umsebenzi wawo oyinhloko ukwenza ama-antibodies okulwa nokutheleleka. Cabanga ukuthi emizimbeni yethu, kunezinkulungwane zama-clone amaseli e-plasma enza izinhlobo ezahlukene zama-antibodies okulwa nezifo ezahlukene. Iseli elilodwa le-plasma liyahlukana futhi lenze amaseli amaningi.

Ezifweni ezihilela 'amaseli e-plasma', iqembu lala 'maseli e-plasma' lihlukana ngokungalawuleki, likhiqize inani elikhulu lohlobo olufanayo 'lwama-antibodies'.

Ku-AL amyloidosis, lapho kwenziwa ama-antibodies, kwenziwa amaketanga amabili amaprotheni abizwa ngokuthi amaketanga asindayo kanye namaketanga alula. Okwenzekayo lapha ukuthi amaseli e-plasma enza amaprotheni amaningi kakhulu eketanga alula. La maketanga alula ayahlukana futhi ahlangane. Lawa ma-protein ahlanganisiwe abizwa ngokuthi ama-amyloid fibrils. Lawa ma-fibrils afakwa ezithweni zethu zomzimba. Yilapho-ke umonakalo omkhulu uqala khona ukwenzeka kulezo zitho zomzimba, ngezinye izikhathi kuze kube sezingeni lokuba yingozi empilweni.

Ubani onamathuba amaningi okuthola lesi sifo?

I-Amyloidosis iyisifo esingavamile. Kulinganiselwa ukuthi ithinta abantu abaphakathi kuka-9 no-14 ngesigidi e-United States kanye nabantu abaphakathi kuka-5 no-12 ngesigidi kwezinye izingxenye zomhlaba. I-AL amyloidosis ivame kakhulu kwabesilisa kunabesifazane. Ivame futhi ukuthinta abantu abaneminyaka engaphezu kuka-60. Isilinganiso seminyaka yokuxilongwa sicishe sibe yiminyaka engu-64.

Ziyini izimpawu ze-'AL amyloidosis'?

I-'AL amyloidosis' ingathinta hhayi nje izitho zomzimba wethu, kusukela ekhanda kuya ezinyaweni, kodwa nasezithweni zomzimba. Ngokuvamile, izimpawu zalesi sifo zingafana nezimpawu zezinye izifo ezingatheni kangako. Futhi, izimpawu zivela kancane kakhulu . Ngakho-ke ungase ungaluboni ngokushesha ushintsho emzimbeni wakho.

Izimpawu ezihlobene nekhanda nentamo:

• Ingabe uzizwa unesiyezi noma unekhanda elibuhlungu (`ikhanda elibuhlungu`) uma uvuka?
• Ingabe une- rash enombala onsomi eduze kwamehlo akho noma ezinkopheni zakho?
• Ingabe ulimi lwakho luzwakala lukhulu kunolujwayelekile ?

Izimpawu ezihlobene nezandla nezinyawo:

Izandla zingase zibe nalezi zici:

• Ingabe uzwa ukuqaqamba, ukusha, noma ukuhlaba ezandleni zakho? Lokhu kungaba izimpawu zesimo esibizwa ngokuthi "i-Peripheral Neuropathy".
• Ingabe uzwa ukuqaqamba kanye nokungabi namandla eminweni yakho? Lokhu kungaba izimpawu ze-"Carpal Tunnel Syndrome".

Izimpawu emilenzeni nasezinyaweni zingafaka:

• Ingabe imilenze noma izinyawo zakho ziyavuvukala ?
• Ingabe imilenze yakho izizwa ingenampilo ?

Ngaphezu kwalokho, ungase uqaphele ukuthi uphuka kalula , wopha kalula, noma unombala onsomi wesikhumba lapho kunemibimbi khona.

Izimpawu ezibonisa izinkinga zenhliziyo namaphaphu:

Izimpawu ezingezansi zingaba yizimpawu zezinye izifo, kodwa qaphela:

• Ukushaya kwenhliziyo: Umuzwa sengathi inhliziyo iyashaya futhi igijima.
• Ukuphelelwa umoya (ukuphelelwa umoya): Umuzwa wokuqina esifubeni, njengokungathi awukwazi ukuphefumula ngokujulile.
• Ubuhlungu besifuba : Ubuhlungu noma kuphi esifubeni. Lobuhlungu bungaba bukhali, bube buthuntu, noma budlule. Ubuhlungu besifuba bungaba uphawu lokuhlaselwa yinhliziyo, ngakho-ke uma ubuhlungu besifuba buhlala imizuzu engaphezu kwemihlanu futhi bungapheli ngokuphumula noma ngemithi, shayela u-911 ngokushesha noma ucele othile akuyise esibhedlela.
• Ukukhathala : Ukuzizwa ukhathele kangangokuthi awukwazi ngisho nokwenza imisebenzi yakho yansuku zonke, njengokungathi umzimba wakho awusenawo amandla okuphila.

Izimpawu ezibonisa izinkinga zesisu noma zamathumbu:

I-AL amyloidosis ingathinta nemikhuba yakho yokudla. Ungase ube nezimpawu ezifana nalezi:

• Ukungathandi ukudla : Ukudla kwawo wonke umuntu kuyashintsha ngezikhathi ezithile. Kodwa uma ulahlekelwa ukudla izinsuku eziningana ngaphandle kwesizathu, kungaba uphawu lwenkinga yamathumbu ehlobene ne-AL amyloidosis.
• Ukuqunjelwa noma igesi eningi : Ukuqunjelwa kanye negesi kuyizimpawu ezivamile, kodwa ngezinye izikhathi ezibangela amahloni. Kodwa-ke, uma unegesi eqhubekayo, kungaba uphawu lwenkinga yesisu ehlobene ne-AL amyloidosis.
• Ukuqunjelwa : Ukuqunjelwa kusho ukuhamba kwamathumbu okungaphansi kwezintathu ngesonto. Uma uqunjelwa isikhathi esingaphezu kwamasonto amathathu, kuyinkinga okufanele ukhulume ngayo nodokotela wakho.
• Uhudo : Indle enamanzi. Uma uhudo luqhubeka, kufanele utshele nodokotela wakho.

Izimpawu ezibonisa izinkinga zezinso noma zesinye:

Ungase uqaphele ushintsho ekubukekeni komchamo wakho noma ukuthi udinga ukuchama kangaki. Lezi zimpawu zingase zihlanganise:

• Ubona amabhamuza amaningi emchameni wakho kunokujwayelekile?
• Ingabe uchama kancane kunokujwayelekile, noma kufanele uvuke ebusuku ukuze uchame ?

Yini ebangela i-AL amyloidosis?

Sesikhulume ngalokhu kancane ngaphambili. I-'AL amyloidosis' ibangelwa 'amaseli e-plasma' enza 'ama-antibodies', amaprotheni abizwa ngokuthi 'amaketanga asindayo' kanye 'namaketanga alula', lapho lawo maprotheni 'amaketanga alula' enziwa ngokweqile. Lawa 'maketanga alula' ayaphambana, ahlangane ndawonye, ​​​​futhi akhe 'ama-amyloid fibrils' abese efakwa ezithweni zethu zomzimba. Yilapho izinkinga ziqala khona.

Odokotela bakuthola kanjani i-AL amyloidosis?

Odokotela basebenzisa izivivinyo eziningana ukuze bathole i-AL amyloidosis. Isivivinyo esibaluleke kakhulu ukuthatha isampula encane yezicubu noma isitho okucatshangwa ukuthi sithintekile yilesi sifo. Lokhu kubizwa ngokuthi i-biopsy. Ama-biopsy angahlukaniswa kanje:

• Ukuhlolwa komnkantsha wethambo : Lokhu kuhilela ukuthatha isampula encane yomnkantsha wethambo ngaphakathi kwethambo.
• I-biopsy yezinso : Kungase kuthathwe izingcezu ezimbalwa ezincane zezicubu zezinso.
• I-biopsy yenhliziyo : Kungase kuthathwe izingcezu ezimbalwa ezincane zemisipha yenhliziyo.
• I-biopsy ye-fat pad : Kuthathwa ingxenye encane yezicubu ezinamafutha esiswini. Lokhu kuyindlela elula yokuhlola.

Ukuhlolwa okwengeziwe

Udokotela wakho angenza ezinye izivivinyo ukuze abone ukuthi izitho zakho zomzimba zisebenza kahle kangakanani. Lokhu kungafaka:

• Ukuhlolwa kwegazi : Lokhu kwenziwa ukuhlola ukuthi izinso zakho, inhliziyo, nesibindi zisebenza kanjani, kanye nokuhlola inani lamaketanga okukhanya egazini lakho.
• Ukuhlolwa komchamo : Lokhu kuvame ukuba ukuhlolwa kokuqoqa umchamo amahora angama-24. Uqoqa umchamo wakho ekhaya bese uletha isampula kudokotela wakho. Lokhu kuhlolwa kuhlola ukuthi izinso zakho zithintekile yini yi-amyloidosis.
• I-Electrocardiogram (ECG): I-ECG ilinganisa umsebenzi kagesi wenhliziyo.
• I-Echocardiogram : Ngezinye izikhathi ibizwa ngokuthi i-ultrasound yenhliziyo, lokhu kusebenzisa amaza omsindo avame kakhulu ukukala ukunyakaza kwenhliziyo.
• I-Cardiac MRI (I-Cardiac Magnetic Resonance Imaging) : Lokhu kuhlolwa kungathatha izithombe ezicacile nezinemininingwane zenhliziyo.

Iphathwa kanjani i-AL amyloidosis?

Odokotela belapha i-AL amyloidosis ukuze banciphise izimpawu zakho, balawule umonakalo wezitho zomzimba, futhi banciphise noma bamise ukunqwabelana kwamaprotheni e-amyloid angavamile.

Odokotela bangelapha i-AL amyloidosis nge-chemotherapy, i-immunotherapy, noma ama-steroids. Iningi labantu linikezwa umuthi owodwa noma emibili ye-chemotherapy, ngokuvamile ihlanganiswa nama-steroids. Le mithi isebenza ndawonye ukubhubhisa amaseli e-plasma enza amaprotheni e-light chain.

Okubalulekile, yize le mithi ingamisa noma inciphise ukuqhubeka kwe-AL amyloidosis, ayikwazi ukususa ama-amyloid fibrils asevele akhene ezithweni zakho. Uma ukwelashwa kuqala, amasosha omzimba wakho angasusa kancane kancane lawa maprotheni angajwayelekile. Abacwaningi bafuna nama-antibodies amasha e-monoclonal angasusa lawa ma-fibrils.

Udokotela wakho uzokhuluma nawe ngokuthi ungazuza yini "ngokufakelwa umnkantsha wamathambo" noma "ngokufakelwa iseli le-stem." Nakuba lokhu kuyizindlela zokwelapha eziyinkimbinkimbi kakhulu, zingaba yimpumelelo enkulu kwabanye abantu.

Ingabe ukuthuthukiswa kwe-'AL amyloidosis' kungavinjelwa?

Ngeshwa, cha. Ayikho indlela yokuvimbela i-AL amyloidosis noma olunye uhlobo lwe-amyloidosis. I-AL amyloidosis yenzeka lapho iseli elilodwa le-plasma liba yinto engajwayelekile futhi liqala ukuhlukana ngokungalawuleki. Lokhu akuyona into esingayilawula.

Ungaphila isikhathi esingakanani ne-'AL amyloidosis'?

Odokotela manje basiza abantu abane-AL amyloidosis baphile isikhathi eside. Kwabanye, i-AL amyloidosis yisimo sesikhathi eside, noma esingamahlalakhona, esingaphathwa ngemithi. Kodwa-ke, kubalulekile ukukhumbula ukuthi lesi yisimo esibi kakhulu futhi singaholela ezimweni zezokwelapha ezisongela ukuphila.

Ngenxa yokuthi i-AL amyloidosis iyisifo esingavamile, kunzima kodokotela ukwazi ukuthi umuntu onesifo angaphila isikhathi esingakanani. Uma une-AL amyloidosis, buza udokotela wakho ngesimo sakho ngemva kokwelashwa. Udokotela wakho angakunikeza ulwazi olungcono kakhulu mayelana nokuthi yini ongayilindela.

Ngizinakekela kanjani ?

Uma une-AL amyloidosis, udokotela wakho uzogxila kakhulu ekwelapheni izimpawu zakho kanye nokumisa noma ukunciphisa ukuqhubeka kwesifo. Kungase kudingeke uqhubeke uthatha imithi, futhi leyo mithi ingaba nemiphumela emibi. Enye indlela ongazinakekela ngayo ukufunda ngemiphumela emibi yokwelashwa kwakho nokuthi ungayiphatha kanjani . Nazi ezinye iziphakamiso:

• Ezinye izindlela zokwelapha zingathinta isifiso sakho sokudla.Uma kunjalo, buza udokotela wakho ngemikhuba yokudla enempilo ezokusiza uhlale unamandla ngesikhathi sokwelashwa. Ukudla ukudla okunempilo kubaluleke kakhulu.
• Zama ukuphumula ngokwanele . Ukuphumula kubalulekile emzimbeni uma ulwa nesifo esinjengalesi.
• Ukuzivocavoca umzimba kuyindlela enhle yokugcina ingqondo yakho ijabule futhi ikusize ugcine isisindo esinempilo. Kodwa-ke, cela iseluleko kudokotela wakho mayelana nokuzivocavoca okungeke kubeke umthwalo omkhulu emzimbeni wakho.
• Ngenxa yokuthi i-AL amyloidosis iyisifo esingavamile, ungase uzizwe unesizungu futhi unesizungu . Abanye bangase bangasiqondi lesi sifo. Buza udokotela wakho ngamaqembu okusekela noma izinhlelo zabantu abane-AL amyloidosis. Ukukhuluma nabantu abanjalo kungaba umthombo omkhulu wamandla.

Okokugcina, amaphuzu ambalwa abalulekile (Umyalezo Wokuya Ekhaya)

I-AL amyloidosis (i-Amyloid light chain noma i-primary amyloidosis) yisifo esingavamile esibangelwa ukugcwala kwamaprotheni e-light chain angajwayelekile ezithweni nasezicutshini zomzimba wethu. Kungaba yisifo esibi kakhulu , ngezinye izikhathi esiphathwa njengesifo esingamahlalakhona, kodwa futhi singadala izimo zezokwelapha ezisongela impilo.

Kodwa ungakhathazeki, odokotela bathola izindlela ezintsha zokusiza abantu abane-AL amyloidosis baphile isikhathi eside futhi baphile impilo engcono. Uma une-AL amyloidosis, buza udokotela wakho ngezivivinyo ezintsha zemitholampilo kanye nocwaningo lokwelapha lesi sifo. Ukutholwa kusenesikhathi kanye nokwelashwa kubalulekile. Kubalulekile futhi ukuhlala unethemba futhi ulandele iseluleko sikadokotela wakho.

I- AL amyloidosis, i-primary amyloidosis, amaseli e-plasma, amaprotheni ochungechunge olukhanyayo, ama-amyloid fibrils, isifo sezinso, isifo senhliziyo