Amyloidosis - kuyini? Amyloidosis: izimbangela, izimpawu, ukwelashwa, uphethwe

Amyloidosis - kuyini? Kuyinto isifo amaprotheni umzimba lapho ukwakheka kanye sebethule ezicutshini ahlukahlukene kanye nezinhlaka ethile amaprotheni-polysaccharide izidakamizwa - amyloid.

Ukuthuthukiswa lesi sifo

Thuthukisa amyloidosis (ukuthi iyini - sesibonile) ayephuli amaprotheni zamagama ohlelweni reticuloendothelial. I -plasma kuyinto ekuqongeleleni amaprotheni ongaphilile. Lezi amaprotheni empeleni self antigen futhi abangele yokungezwani komzimba efana ukwakheka autoantibodies.

Khona-ke, la masosha omzimba kuya antigen futhi sisabele kahle lapho amaprotheni grubodispersionnye kwenze. Kusukela kwasungulwa amyloid. Le nto imali kwi ezindongeni umkhumbi izitho ezahlukene. Kancane kancane olwenzeka amyloid kuholela ekufeni komzimba.

Izinhlobo amyloidosis. izizathu

Kunezinhlobo eziningana amyloidosis. Izimbangela lesi sifo encike ngqo ngokuthi uhlobo olunjani amyloidosis. Kuyini lokhu? Ukwahlukanisa Kwenziwa ngokuvumelana amaprotheni esiyisisekelo, aqukethe amyloid fibrils. Ngezansi ukubukwa lesi sifo.

1. amyloidosis eyisisekelo (AL-amyloidosis). Lapho ukuthuthukiswa yayo plasma avele normal immunoglobulin ukukhanya ngamaketanga, ekwazi ukuba bazohlala kwezicubu ezihlukahlukene zomzimba. Ngokufanayo yashintsha ngesikhathi plasma amaseli we myeloma amaningi, macroglobulinemia Waldenström sika, hypergammaglobulinemia monoclonal.
2. amyloidosis Okwesibili (AA-amyloidosis). Kulokhu kukhona ukukhululwa ngokweqile wamaprotheni isibindi-alpha-globulin. Lesi sigaba amaprotheni acute ukuthi kuhlelwe ukuvuvukala ezingamahlalakhona. Lokhu kungenzeka yezifo ezihlukahlukene, njenge-rheumatoid arthritis, umalaleveva, bronchiectasis, osteomyelitis, uchoko, isifo sofuba.
3. amyloidosis Family (AF-amyloidosis). Kuyinto kuyisifo esiwufuzo nge autosomal ifa esikhubazayo. Ibizwa nangokuthi Mediterranean imfiva ngakanye namkha paroxysmal poliserozita umndeni. Wazwakalisa lesi ukuhlaselwa isifo fever, okuvela kuyo ubuhlungu besisu, ukuqubuka, sokuqaqamba kwamalunga, futhi pleurisy.
4. I amyloidosis zelashwe (AH-amyloidosis). Ixhumeke nge yokuthi amaprotheni beta-2 microglobulin MHC kubantu onempilo okungadingeki izinso, futhi akuhlungangwa e hemodialysis, sinjalo ekuqongeleleni yayo emzimbeni.
5. AE-amyloidosis. It is asathuthuka izinhlobo ezithile kuphela zomdlavuza, ezifana yegilo.
6. amyloidosis Senile.

izimpawu

Lapho sitholakele "Amyloidosis" izimpawu zincike kwasendaweni imali. Lapho kunqotshwa pheshana emathunjini kungabonakala nasemkhakheni ulimi lakhulisiwe kahle ukugwinya umsebenzi, ukuqunjelwa noma isifo sohudo. Kungase kube ezifakwa isimila-amyloid emathunjini noma isisu.

amyloidosis emathunjini ephelezelwa umuzwa wobukhulu nokuphatheka kabi kungase kube omnene ubuhlungu besisu. Uma ehlaselwa kwamanyikwe, kuyinto ukwethula izimpawu ezifanayo njengoba e pancreatitis. Uma ukulimala kwesibindi waphawula landa, isicanucanu, ukubhodla, ukuhlanza, ukuquleka, jaundice.

Amyloidosis zokuphefumula livela ngale ndlela:

• izwi hoshozela;
• izimpawu Ukucinana kwemigudu yomoya emaphashini;
• yamaphaphu isimila amyloidosis.

Izimpawu ezilandelayo kungenzeka amyloidosis isimiso sezinzwa:

• esibuhlungu noma evutha ukuzwa e nemilenze, ukuba ndikindiki (polyneuropathy neuropathy);
• ikhanda, isiyezi;
• sphincter iziyaluyalu (incontinence urinary, fecal).

Amyloidosis - kuyini, nezimbangela zayo futhi izimpawu siye sibuyekezwe. Manje sibheka ukuthi lesi sifo sitholakale umuntu futhi yiziphi izindlela zokwelashwa khona.

ukuhlola

Ngo isifo esinjengo amyloidosis, uphethwe eziyinkimbinkimbi. Aqokwe zocwaningo laboratory kanye hardware.

Ngo izifundo laboratory, ukubuyekeza jikelele igazi waphawula ukwanda ESR, leukocyte futhi kwehle platelet count. Uhlaziyo jikelele amaprotheni umchamo elikhona inzika - amasilinda, leukocyte futhi erythrocyte. Ngo coprogram ukwethula esiningi isitashi, fat and imisipha zesivikelo sesifuba. Ngo-biochemistry igazi e izilonda kwesibindi lwembula okhushuliwe cholesterol, bilirubin, phosphatase ane-alikhali.

Ngo amyloidosis oyinhloko umchamo kanye plasma wabonisa wokuqukethwe lelisetulu amyloid. Lapho yesibili phakathi ukuhlolwa laboratory ukubonisa izimpawu ukuvuvukala ezingamahlalakhona.

Futhi ukuqhuba nezinye izinyathelo zokuxilonga:

• -X-ray luhlolo;
• echocardiography (ku ka a okusolwa senhliziyo);
• assays ukusebenza nge udayi;
• emizimbeni biopsy.

ukwelashwa

Lapho lesi sifo wenziwa ukwelashwa ngaphandle nesineke. Amyloidosis, lapho kukhona izimo ezinkulu, njengalezo ezingamahlalakhona ukwehluleka kwezinso noma okunzima inhliziyo ukwehluleka ziphathwe esibhedlela.

Ngo amyloidosis eyinhloko ekuqaleni esimisiwe izidakamizwa ezifana "Chloroquine" "Melphalan" "prednisolone", "Colchicine".

Ngo amyloidosis yesibili ilapha lezo zifo ecashile, isib, osteomyelitis, isifo sofuba, pleural empyema et al., Zonke izimpawu of amyloidosis ngokuvamile ziyanyamalala ngemva ikhambi.

Uma lesi sifo eba ngenxa izinso hemodialysis ke isiguli enjalo udluliselwa zelashwe peritoneal.

Endabeni sohudo binders imithi, njenge "bismuth subnitrate," noma adsorbing ejenti.

Zingase ukwelashwa okunezimpawu:

• amalungiselelo ekunciphiseni ingcindezi;
• amavithamini, isisu;
• ukumpontshelwa plasma, njll

Ngaphezu kwalokho, kungase kusetshenziswe futhi ukwelashwa kuhlinzwa. amyloidosis we ubende kungase ashona ngemva kokususwa isitho. Ezimweni eziningi, lokhu kuholela ngcono isimo iziguli kanye ukunciphisa ukwakheka amyloid.

ukudla

Amyloidosis kudinga yokudla njalo. With ukuthuthukiswa isifo sezinso kumele inciphise kusawoti, futhi ukudla amaprotheni ezifana inyama, inhlanzi, amaqanda. Uma uthuthukisa ezingamahlalakhona ukumelwa inhliziyo, kufanele ngaphandle kusukela ekudleni kasawoti, ngibhema futhi ukudla zicwiliswe.

amyloidosis inhliziyo

Lesi sifo ubizwa nangokuthi amyloid zenhliziyo. Nge ukusungula kwakhe amyloid sebethule kungavuka myocarditis, pericarditis, endocarditis, noma ezindongeni le-aorta ne nemithambo yegazi yenhliziyo. Isizathu salokhu isifo senhliziyo kungase kube amyloidosis lokuqala, lesibili noma umndeni. Ngokuvamile amyloidosis wenhliziyo - akuyona isifo elingalodwa, futhi ethuthuka parallel amyloidosis yamaphaphu, izinso, amathumbu noma ubende.

Izimpawu amyloidosis inhliziyo

Ngokuvamile izimpawu zalesi sifo iyafana zenhliziyo hypertrophic noma isifo senhliziyo. Ngesikhathi besanda kuqala, izimpawu azicacile. Kwangathi ukuzwa ukucasuka kanye ukukhathala, ulahlekelwe isisindo, ukuvuvukala kwezicubu nesiyezi.

Ukuwohloka abukhali ngokuvamile kwenzeka ngemva kwanoma iyiphi ukucindezeleka noma izinkinga zokuphefumula. Ngemva kwalokho ngokuvamile bavele inhliziyo ubuhlungu uhlobo angina pectoris, arrhythmia, edema evelele, dyspnea, isibindi ukwandisa. Umfutho wegazi ngokuvamile eyehlisiwe.

Isifo siqhubeka ngokushesha, futhi sici yaso eyingqayizivele yilona ukumelana (ukumelana) yokwelapha. Ezimweni ezimbi kakhulu, iziguli zingase zibhekane ascites (ekuqongeleleni uketshezi ngaphakathi komgodi ongaphakathi kwamathumbu) noma effusion pericardial. Ngenxa lingene amyloid ukuthuthukisa itheku bradycardia. Lokhu kungaholela ngokufa okuzumayo.

ukuba nombono ongafani ngeke

Ngesikhathi inhliziyo amyloidosis Izibikezelo abampofu. Congestive inhliziyo ukwehluleka kule isifo siqhubeka kancane kancane futhi ukufa kuseduze. ERussia akukho izikhungo ezikhethekile Lapho esingatha le ndaba.