I-amyloidosis eyinhloko

I-amyloidosis eyinhloko yisifo esingajwayelekile lapho amaprotheni angajwayelekile akha khona ezicutshini nasezithweni. Ukuqubuka kwamaprotheni angajwayelekile kubizwa ngokuthi ama-amyloid deposits.

Isizathu se-amyloidosis eyinhloko asiqondakali kahle. Izakhi zofuzo zingadlala indima.

Lesi simo sihlobene nokukhiqizwa okungajwayelekile nokwedlulele kwamaprotheni. Ukuqubuka kwamaprotheni angajwayelekile kuyakheka ezithweni ezithile. Lokhu kwenza kube nzima ukuthi izitho zisebenze kahle.

I-amyloidosis eyinhloko ingaholela ezimweni ezibandakanya:

• I-Carpal tunnel syndrome
• Ukulimala kwemisipha yenhliziyo (i-cardiomyopathy) okuholela ekuhlulekeni kwenhliziyo
• I-malabsorption yamathumbu
• Ukuvuvukala kwesibindi nokungasebenzi kahle
• Ukuhluleka kwezinso
• I-Nephrotic syndrome (iqembu lezimpawu ezibandakanya amaprotheni emchameni, amazinga aphansi wamaprotheni egazini, amazinga aphezulu e-cholesterol, amazinga aphezulu e-triglyceride, nokuvuvukala emzimbeni wonke)
• Izinkinga zemizwa (i-neuropathy)
• I-Orthostatic hypotension (yehla umfutho wegazi lapho usukuma)

Izimpawu zincike ezithweni ezithintekile. Lesi sifo singathinta izitho nezicubu eziningi, kufaka phakathi ulimi, amathumbu, imisipha yamathambo nebushelelezi, izinzwa, isikhumba, imisipha, inhliziyo, isibindi, ubende nezinso.

Izimpawu zingafaka noma yikuphi okulandelayo:

• Isigqi senhliziyo esingajwayelekile
• Ukukhathala
• Ubuntofontofo bezandla noma izinyawo
• Ukuphelelwa umoya
• Izinguquko zesikhumba
• Izinkinga zokugwinya
• Ukuvuvukala ezingalweni nasemilenzeni
• Kuvuvukele ulimi
• Ukubamba isandla okuntekenteke
• Ukwehla kwesisindo noma ukuzuza kwesisindo

Ezinye izimpawu ezingase zivele ngalesi sifo:

• Ukwehla kokukhishwa komchamo
• Uhudo
• Ukubola noma ukushintsha kwezwi
• Ubuhlungu obuhlangene
• Ubuthakathaka

Umhlinzeki wezokunakekelwa kwempilo uzokuhlola. Uzobuzwa ngomlando nezimpawu zakho zezokwelapha. Ukuhlolwa komzimba kungakhombisa ukuthi unesibindi sokuvuvukala noma ubende, noma izimpawu zokulimala kwemizwa.

Isinyathelo sokuqala sokuthola i-amyloidosis kufanele kube ukuhlolwa kwegazi nomchamo ukubheka amaprotheni angajwayelekile.

Olunye uvivinyo luxhomeke kwizimpawu zakho kanye nohlaka olungathinteka. Ezinye izivivinyo zifaka:

• I-ultrasound yesisu ukuhlola isibindi nobende
• Ukuhlolwa kwenhliziyo, njenge-ECG, noma i-echocardiogram, noma i-MRI
• Ukuhlolwa kokusebenza kwezinso ukuhlola izimpawu zokuhluleka kwezinso (nephrotic syndrome)

Ukuhlolwa okungasiza ukuqinisekisa ukuxilongwa kufaka:

• Ukulangazelela kwesisu samafutha esiswini
• I-bone marrow biopsy
• I-biopsy yemisipha yenhliziyo
• I-Rectal mucosa biopsy

Ukwelashwa kungafaka:

• Ukwelashwa ngamakhemikhali
• Ukufakelwa kwe-stem cell
• Ukufakelwa komzimba

Uma lesi simo sidalwa esinye isifo, leso sifo kufanele siphathwe ngolaka. Lokhu kungathuthukisa izimpawu noma kwehlise lesi sifo ekubeni sibi kakhulu. Izinkinga ezinjengokwehluleka kwenhliziyo, ukwehluleka kwezinso, nezinye izinkinga kwesinye isikhathi zingelashwa, lapho zidingeka.

Ukwenza kahle kwakho kuncike ekutheni yiziphi izitho ezithintekayo. Ukubandakanyeka kwenhliziyo nezinso kungaholela ekuhlulekeni kwesitho nasekufeni. I-amyloidosis yomzimba wonke (ehlelekile) ingaholela ekufeni kungakapheli iminyaka emibili.

Shayela umhlinzeki wakho uma unezimpawu zalesi sifo. Fonela futhi uma utholwe unalesi sifo futhi une:

• Ukwehla komchamo
• Kunzima ukuphefumula
• Ukuvuvukala kwamaqakala noma ezinye izitho zomzimba ezingapheli

Akukho ukuvimbela okwaziwayo kwe-amyloidosis eyinhloko.

I-Amyloidosis - eyinhloko; I-immunoglobulin light chain amyloidosis; Amyloidosis wesistimu oyinhloko

• I-Amyloidosis yeminwe
• Amyloidosis ebusweni

UGertz MA, uBuadi FK, uLacy MQ, uHayman SR. I-immunoglobulin light-chain amyloidosis (i-primary amyloidosis). Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ama-eds. I-Hematology: Izimiso Eziyisisekelo Nokuzijwayeza. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2018: isahluko 88.

I-Hawkins PN. I-Amyloidosis.Ku: Hochberg MC, Gravallese EM, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, abahleli. I-Rheumatology. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2019: isahluko 177.