Kuyini i-AL Amyloidosis? Ake sixoxe ngalesi sifo esingavamile ngamazwi alula

Wake wacabanga ukuthi ukuzizwa ukhathele njalo, ukuvuvukala kwemilenze, kanye nobunzima bokuphefumula kuyinto evamile? Ngezinye izikhathi, kungase kube nesimo esiyinkimbinkimbi kakhulu ngemuva kwalezi zimpawu esingakazwa kakhulu ngazo. Namuhla, sikhuluma ngesifo esisodwa esingavamile, kodwa okufanele usiqaphele. Leso yi-AL Amyloidosis. Leli gama lingase lizwakale liyesabeka kancane, kodwa ake sixoxe ngalo kalula nangokucacile.

Kalula nje, iyini i-AL amyloidosis?

Ake sithi sinezimboni ezincane emizimbeni yethu, esizibiza ngamaseli e-plasma . Lawa atholakala emnkantsheni wethu wamathambo. Umsebenzi wawo oyinhloko ukwenza amasosha abizwa ngokuthi ama-antibodies ukulwa namagciwane angena emizimbeni yethu. Lena yingxenye ebaluleke kakhulu yesimiso sethu sokuzivikela komzimba.

Kodwa esimweni esibizwa ngokuthi i-AL amyloidosis, kukhona iphutha elincane kula maseli e-plasma. Ngenxa yalesi sici, lawo maseli akhiqiza uhlobo lweprotheni olungahlelekile, oluphukile, nolungahlelekile. Lokhu kubizwa ngokuthi iphrotheni yochungechunge olukhanyayo .

Lawa maprotheni e-light chain ahlukene kahle ayahlangana, akhe ama-tangles kanye nezakhiwo ezincane, ezine-fiber. Lawa akheka ezithweni ezahlukahlukene emizimbeni yethu, njengenhliziyo, izinso, imizwa, kanye namathumbu. Njengokungcola okuvala ipayipi, lapho lawa maprotheni akheka, lawo malungu awakwazi ukusebenza kahle. Yilokhu esikubiza ngokuthi i-AL amyloidosis.

Kungani lokhu kubizwa ngokuthi i-AL?

Kunezinhlobo eziningana ze-amyloidosis. Uhlobo ngalunye luqanjwe ngeprotheni engavamile eyibangela. Uhlamvu u-A lumelela i-Amyloidosis kanti uhlamvu u -L lumelela uchungechunge olukhanyayo .

Lesi sifo sithinta kanjani umzimba?

Kunezinhlobo eziningi ezahlukene zamaseli e-plasma emizimbeni yethu. Uhlobo ngalunye lwenza uhlobo oluthile lwe-antibody yokulwa negciwane elithile.

Ku-AL amyloidosis, iseli elilodwa le-plasma liba nesici, futhi lelo seli lihlukana ngokungalawuleki, likhiqize izinkulungwane zamaseli anesici. Elinye lala maseli lenza iphrotheni yochungechunge lokukhanya olunesici esikhulume ngalo ngaphambili.

Leli nani elikhulu lamaprotheni lihamba egazini lethu bese liqala ukugcwala ezithweni zethu zomzimba. Lezi zinhlayiya ezigciniwe zibizwa ngokuthi ama-amyloid fibrils . Lokhu kubangela ukuthi izitho zomzimba ziqine futhi zingasebenzi kahle. Ngokuhamba kwesikhathi, lokhu kungabangela umonakalo omkhulu ongaba yingozi ngisho nokuphila.

Ubani onamathuba amaningi okuthola lesi sifo?

Empeleni lesi yisifo esingavamile kakhulu. Emhlabeni wonke, sithinta inani elincane labantu, phakathi kuka-5 no-12 esigidini ngasinye.

• Okuningi kwabesilisa kunabesifazaneUkuthambekela kokwakha kukhulu kakhulu.
• Lesi simo sivame kakhulu kubantu abaneminyaka engaphezu kwengu-60. Isilinganiso seminyaka yokuxilongwa siyiminyaka engaba ngu-64.

Yiziphi izimpawu ezingabonakala?

Inkinga eyodwa ngalesi sifo ukuthi izimpawu zaso zivame ukufana nezezinye izifo ezivamile. Futhi lezi zimpawu zibonakala kancane kakhulu. Ngakho-ke ungase ungaboni umehluko omkhulu ekuqaleni. Ake sibone ukuthi ziyini lezi zimpawu kuthebula elingezansi.

Udokotela usithola kanjani lesi sifo ngokunembile?

Ngemva kokulalela izimpawu zakho, uma udokotela esola lesi sifo, uzokwenza izivivinyo eziningana ukuze asiqinisekise.

Ukuhlolwa okubaluleke kakhulu kwalokhu yi-biopsy . Lokhu kuhilela ukuthatha ingxenye encane kakhulu yezicubu noma isitho okucatshangwa ukuthi sithintekile bese sihlolwa ngaphansi kwe-microscope. Lokhu kusenza sibone kahle ukuthi iphrotheni ye-amyloid ikhona yini. Ukuhlolwa kwe-biopsy kuvame ukwenziwa ukuze:

• Ukuhlolwa komnkantsha wethambo: Kuthathwa isampula encane emnkantsha wethambo ngaphakathi kwethambo.
• Ukuhlolwa kwezinso: Kuthathwa izingcezu eziningana ezincane kakhulu zezicubu ezinso.
• I-biopsy yenhliziyo: Kuthathwa izingcezu eziningana ezincane zemisipha yenhliziyo.
• Ukuhlolwa kwe-Abdominal Fat Pad Biopsy: Kuthathwa ingxenye encane yezicubu ongqimbeni olunamafutha ngaphansi kwesikhumba sesisu.

Ukuhlolwa okwengeziwe

Ngaphezu kwalokhu, kwenziwa izivivinyo ezengeziwe ukuthola ukuthi umonakalo ongakanani owenzekile ezithweni zakho zomzimba.

• Ukuhlolwa kwegazi: Lokhu kusiza ukubona ukuthi izinso, inhliziyo nesibindi zisebenza kanjani, kanye nokukala inani leprotheyini yochungechunge olukhanyayo egazini.
• Ukuhlolwa komchamo: Isampula yomchamo, evame ukuqoqwa esikhathini samahora angama-24, iyahlolwa ukuze kubonakale ukuthi kukhona yini umonakalo ezinso.
• I-ECG (i-Electrocardiogram): Ilinganisa umsebenzi kagesi wenhliziyo.
• I-Echocardiogram: Ukuskena kwenhliziyo nge-ultrasound. Lokhu kubheka izinto ezifana nokunyakaza kwenhliziyo kanye nosayizi wayo.
• I-MRI Yenhliziyo: Lokhu kuhlolwa kwenziwa ukuze kutholakale isithombe esicacile nesiningiliziwe senhliziyo.

Yiziphi izindlela zokwelapha lokhu?

Kunezinhloso eziningana eziyinhloko zokwelapha i-AL amyloidosis. Eyokuqala ukulawula izimpawu. Eyokuqala ukuphatha ukulimala kwezitho zomzimba. Okubaluleke kakhulu, ukuvimba ukukhiqizwa kweprotheni engajwayelekile .

Odokotela bavame ukusebenzisa imithi efana ne -chemotherapy , i-immunotherapy , noma ama-steroids ukubhubhisa amaseli e-plasma anephutha, abese emisa ukukhiqizwa kwamaprotheni amasha ochungechunge lokukhanya.

Kwezinye izimo, kuye ngesimo sesiguli , kunganconywa ukufakelwa umnkantsha noma i-stem cell . Konke lokhu kunqunywa udokotela okwelaphayo.

Ungaphila isikhathi esingakanani nalesi sifo?

Lokhu kuyinkinga kubantu abaningi. Eqinisweni, izindlela ezintsha zokwelapha manje sezikhulise kakhulu isikhathi sokuphila kwabantu abane-AL amyloidosis. Kwabanye abantu, sekuyinkinga engapheli engalawulwa ngemithi.

Kodwa akufanele sikhohlwe ukuthi lesi yisifo esibi kakhulu. Isimo sesikhathi esizayo sesiguli sinqunywa yizici eziningi, njengobukhulu bomonakalo ezithweni zomzimba lapho kutholakala lesi sifo, kanye nokusabela komzimba ekwelashweni.

Ingabe lesi sifo singavinjelwa? Ngingazinakekela kanjani?

Ngeshwa, i-AL amyloidosis akuyona isifo esingasivimbela. Ibangelwa ukuguquka okungahleliwe kweseli le-plasma. Kodwa uma lesi sifo sesitholakele, kunezinto ezimbalwa ongazenza ukuze uzinakekele.

• Ukudla okunempilo: Ezinye izindlela zokwelapha zingabangela ukulahlekelwa yisifiso sokudla. Kodwa ukudla okunempilo kubalulekile ukuze umzimba wakho uhlale uqinile. Khuluma nodokotela wakho mayelana nokudla okufanele wena.
• Phumula ngokwanele: Nikeza umzimba wakho isikhathi sokululama. Lala futhi uphumule ngokwanele.
• Ukuzivocavoca okufanele:Ukuzivocavoca kuhle engqondweni kanye nasemzimbeni. Kodwa buza udokotela wakho ukuthi yikuphi ukuzivocavoca okuphephile futhi okufanele umzimba wakho.
• Ukusekelwa kwengqondo: Uma unesifo esingavamile njengalesi, ungase uzizwe unesizungu futhi unesizungu ngoba abanye abaqondi. Lokho kuvamile. Buza udokotela wakho ngamaqembu okusekela abantu abanalesi simo. Ukwabelana ngolwazi lwakho nabanye kungamandla amakhulu.

Nakuba i-AL amyloidosis iyisifo esibi kakhulu, kungenzeka ukuphatha nokuphila nalesi simo ngokutholakala kusenesikhathi, ukuthola ukwelashwa okufanele, nokuzinakekela kahle.