Amyloidosis yamabanga aphantsi

I-amyloidosis yaseprayimari sisifo esinqabileyo apho iiproteni ezingaqhelekanga zakha izicwili kunye namalungu. Ukuvutha kweeproteni ezingaqhelekanga kuthiwa ziidipozithi zeamyloid.

Isizathu se-amyloidosis ephambili asiqondwa kakuhle. Imfuza inokudlala indima.

Imeko inxulumene nemveliso engaqhelekanga kunye nokugqithisa kweeproteni. Ukuvutha kweeproteni ezingaqhelekanga zakha kumalungu athile. Oku kwenza ukuba kube nzima kumalungu ukuba asebenze ngokuchanekileyo.

I-amyloidosis yaseprayimari ingakhokelela kwiimeko ezibandakanya:

• Isifo seCarpal tunnel
• Umonakalo wentliziyo (cardiomyopathy) ekhokelela ekusileleni kwentliziyo
• I-malabsorption yamathumbu emathunjini
• Ukudumba kwesibindi kunye nokungasebenzi
• Ukungaphumeleli kwezintso
• I-Nephrotic syndrome (iqela leempawu ezibandakanya iprotheni kumchamo, amanqanaba aphantsi eproteni egazini, amanqanaba aphezulu e-cholesterol, amanqanaba aphezulu e-triglyceride, kunye nokudumba emzimbeni wonke)
• Iingxaki zemithambo-luvo
• I-Orthostatic hypotension (yehla kuxinzelelo lwegazi xa usukuma)

Iimpawu zixhomekeke kumalungu achaphazelekayo. Esi sifo sinokuchaphazela amalungu amaninzi kunye nezicubu, kubandakanya ulwimi, amathumbu, amathambo kunye nezihlunu ezigudileyo, imithambo-luvo, ulusu, imisipha, intliziyo, isibindi, udakada kunye nezintso.

Iimpawu zinokubandakanya nayiphi na kwezi zinto zilandelayo:

• Isingqisho sentliziyo esingaqhelekanga
• Ukudinwa
• Ubungqongqo bezandla okanye iinyawo
• Ukuqhawukelwa ngumphefumlo
• Ulusu luyatshintsha
• Ukuginya iingxaki
• Ukudumba ezingalweni nasemilenzeni
• Ukudumba ulwimi
• Ukubambelela kwesandla esibuthathaka
• Ukwehla kobunzima okanye ukutyeba

Ezinye iimpawu ezinokuthi zenzeke kwesi sifo:

• Ukuncipha kwemveliso yomchamo
• Urhudo
• Ukugquma okanye ukutshintsha kwelizwi
• Intlungu edibeneyo
• Ubuthathaka

Umboneleli wezempilo uyakukuvavanya. Uya kubuzwa malunga nembali yakho yonyango kunye neempawu. Uvavanyo lomzimba lunokubonisa ukuba unesibindi esidumbileyo okanye udakada, okanye iimpawu zomonakalo wemithambo-luvo.

Isinyathelo sokuqala ekuchongeni i-amyloidosis kufuneka ibe luvavanyo lwegazi kunye nomchamo ukukhangela iiproteni ezingaqhelekanga.

Olunye uvavanyo luxhomekeke kwiimpawu zakho nakwilungu elinokuchaphazeleka. Ezinye iimvavanyo zibandakanya:

• I-ultrasound yesisu ukujonga isibindi kunye ne-spleen
• Uvavanyo lwentliziyo, njenge-ECG, okanye i-echocardiogram, okanye iMRI
• Uvavanyo lomsebenzi wezintso ukukhangela iimpawu zokungaphumeleli kwezintso (nephrotic syndrome)

Uvavanyo olunokukunceda ukuqinisekisa ukuxilongwa kubandakanya:

• Isisu somnqweno wokudibana kwamanqatha
• Umongo wethambo
• I-biopsy yentliziyo
• Inqaku le-mucosa biopsy

Unyango lunokubandakanya:

• Unyango ngamayeza
• Ukutshintshwa kweseli
• Ukufakelwa komzimba

Ukuba imeko ibangelwa sesinye isifo, eso sifo kufuneka siphathwe gadalala. Oku kunokuziphucula iimpawu okanye kusinciphise isifo ekubeni mandundu. Iingxaki ezinje ngokusilela kwentliziyo, ukusilela kwezintso, kunye nezinye iingxaki ngamanye amaxesha zinokunyangwa, xa zifuneka.

Uqhuba kakuhle kangakanani kuxhomekeke kumalungu achaphazelekayo. Ukubandakanyeka kwentliziyo kunye nezintso kunokukhokelela ekusileleni komzimba kunye nokufa. Amyloidosis umzimba wonke (inkqubo) angakhokelela ekufeni kwisithuba seminyaka emibini.

Tsalela umnikezeli wakho ukuba unempawu zesi sifo. Biza kwakhona ukuba ufumene ukuba unesi sifo kwaye unayo:

• Ukuncipha komchamo
• Kunzima ukuphefumla
• Ukudumba kwamaqatha okanye amanye amalungu omzimba angatshiyo

Akukho sithintelo saziwayo se-amyloidosis yokuqala.

IAmyloidosis - iprayimari; Ikhonkco lokukhanya kwe-Immunoglobulin amyloidosis; Inkqubo yeprayimari ye-amyloidosis

• I-Amyloidosis yeminwe
• Amyloidosis ebusweni

IGertz MA, iBuadi FK, iLacy MQ, iHayman SR. I-Immunoglobulin light-chain amyloidosis (iphambili i-amyloidosis). Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ii-eds. I-Hematology: Imigaqo esisiseko kunye nokuziqhelanisa. Umhla wesi-7. IPhiladelphia, PA: Elsevier; I-2018: isahluko 88.

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