Amyloidosis - ntoni na? Amyloidosis: izinto, iimpawu, unyango, ukuxilongwa

Amyloidosis - ntoni na? It is a sisifo protein yokucolwa apho ukusekwa nokufumba zomzimba ezahlukeneyo kunye namaziko into protein-polysaccharide ethile - amyloid.

Uphuhliso sifo

Ukuphuhlisa amyloidosis (yintoni na - sesibonile) nokunyhashwa protein kudibaniso kwinkqubo reticuloendothelial. I- plasma yi zemali iiprotheni ezingaqhelekanga. Ezi proteni eyinxalenye awakwazi ukuzithemba kwaye lenze mngundo ofanayo ukuyilwa autoantibodies.

Emva koko, le antibodies awakwazi yaye basabela neeproteni grubodispersionnye kwenze. Okoko kwasekwa amyloid. Le nto lifakwa kwi emithanjeni kunye namaziko ahlukeneyo. Ngcembe lokwandisa, amyloid kukhokelela kokufa komzimba.

Types of amyloidosis. izizathu

Kukho iintlobo ezininzi amyloidosis. Izinto ezibangela isifo luxhomekeke ngqo kwi hlobo luni amyloidosis. Yintoni na? Classification yenziwa ngokwe kwiproteni ezingundoqo, enamakhulu fibrils amyloid. Ngezantsi kukho izimvo zesi sifo.

1. amyloidosis Primary (AL-amyloidosis). Xa uphuhliso yayo kwi-plasma zivela engaqhelekanga xo immunoglobulin kukhanya, ezinako ukukulumkisela azinze kwizihlunu ezahlukeneyo zomzimba. Ngokufanayo zatshintsha ngexesha iiseli plasma of ezigula ezininzi, macroglobulinemia Waldenström kaThixo, hypergammaglobulinemia monoclonal.
2. amyloidosis Sekondari (AA-amyloidosis). Kule meko kukho ukukhululwa ngokugqithiseleyo kwesibindi Iprothini alpha-globulin. Esi sigaba protein etsolo ukuba kuhlelwe kwi ukudumba ezigulo ezondeleyo. Oku kunokwenzeka izifo ezahlukeneyo, ezifana nesifo samathambo, imalariya, bronchiectasis, osteomyelitis, iqhenqa, isifo sephepha.
3. amyloidosis Family (AF-amyloidosis). Sisifo ilifa kunye nelifa autosomal sofuzo. Libizwa ngokuba fever Mediterranean ngakanye okanye paroxysmal poliserozita usapho. Wasinika lesi sifo ukuhlaselwa ifiva, ukwenzeka iintlungu esiswini, irhashalala, samathambo, kunye unepleurisy.
4. I-dialysis amyloidosis (AH-amyloidosis). Elidityaniswe nesibakala sokuba kwiproteni beta-2 microglobulin MHC kubantu abasempilweni zilahlwa yi nezintso, kwaye akukho kuhluzeka kwi wehemodialysis, kwaye ngokunjalo nokwanda wayo emzimbeni.
5. AE-amyloidosis. Iphuhlisa ezinye iintlobo zomhlaza, ezifana lengqula.
6. amyloidosis saka.

iimpawu

Xa lokufunyaniswa "Amyloidosis" iimpawu zixhomekeke kwasendaweni yesibambiso. Emva kokoyiswa iphecana zesisu inokubonakalisa ulwimi ezandisiweyo, umsebenzi ligwinya kakuhle, ukuqhina okanye ukurhuda. Kusenokubakho ithumba-amyloid iidipozithi ethunjini okanye isisu.

amyloidosis yethumbu kunye elikhulu ubunzima kuhlolwe kusenokuba buhlungu onomoya esiswini. Ukuba usindwa injeke, ukuba bammise phambi iimpawu efanayo naleyo echazwe pancreatitis. Ukuba isibindi ukuzilimaza waphawula yayo ukwanda, isicaphucaphu, belching, ukugabha, ukuxhuzula, senyongo.

Amyloidosis zemiphunga livela ngolu hlobo lulandelayo:

• ngelizwi elitshothozayo;
• iimpawu yomoya;
• ithumba amyloidosis miphungeni.

Ezi mpawu zilandelayo kungenzeka amyloidosis e-nervous system:

• ukuntlontlozela okanye evuthayo imvakalelo e omzimba, antsi (polyneuropathy peripheral);
• intloko ebuhlungu, isiyezi;
• ukuphazamiseka sphincter (ngumchamo wokuchama, indle).

Amyloidosis - oko kukuthi, izizathu zayo kwaye iimpawu esiye sakuphonononga. Ngoku sijonga indlela isifo eso yaye ziziphi iindlela zonyango ezikhoyo.

ekuxilongweni

Xa isifo ezifana amyloidosis, uphethwe ezintsonkothileyo. Abamiselweyo luphando elebhu kunye hardware le.

Xa izifundo laboratory, uphononongo jikelele yegazi waphawula yokwanda ESR, leukocytes nokwehla zegazi count. Uhlalutyo jikelele weeproteni umchamo ekhoyo kwi ezike - iisilinda, leukocytes kunye erythrocytes. Xa coprogram ukubonisa ulwazi oluninzi kwimicu starch, amafutha kunye kwezihlunu. Egazini ngamachiza kwi izitshanguba kwesibindi watyhila enye okwandileyo cholesterol, yibilirubin, phosphatase obrak.

Xa amyloidosis zamabanga aphantsi nomchamo kunye negazi iplasma wabonisa isiqulatho eliphezulu amyloid. Xa secondary ngexesha iimvavanyo elebhu ukubonisa iimpawu ukudumba ezigulo ezondeleyo.

Kwakhona ukuqhuba namanye amanyathelo diagnostic:

• iimviwo X-ray;
• echocardiography (kwimeko yesifo sentliziyo okukrokrelwayo);
• zePCR esebenza kunye nedayi;
• imizimba biopsy.

unyango

Xa isifo yenziwa unyango ngaphandle-yezigulana. Amyloidosis, apho kukho iimeko ezinzima, ezifana ukusilela esinganyangekiyo kwezintso okanye ukusilela intliziyo kakhulu ngayo esibhedlele.

Xa amyloidosis zaseprayimari amisiweyo ekuqaleni iziyobisi ezifana "Chloroquine" "Melphalan" "prednisolone", "Colchicine".

Xa amyloidosis secondary ukunyanga esi sifo engundoqo, umz, osteomyelitis, isifo sephepha, pleural empyema et al., Zonke iimpawu amyloidosis kudla liyalahleka emva gula.

Ukuba isifo kuphuhliswa ngenxa kidney wehemodialysis, ngoko isigulana eso wasiwa dialysis peritoneal.

Kwimeko yokuhlanganisa urhudo kusetyenziswa amayeza, ezifana "bismuth subnitrate," okanye adsorbing arhente.

Kwakhona unyango lingaze:

• amalungiselelo ekunciphiseni uxinzelelo;
• iivithamini, womchamo;
• plasma, njl

Ngapha koko, inokuba ufakwe unyango ngotyando. amyloidosis le udakada lisenokungafuni emva ngokususwa kwelungu. Kwiimeko ezininzi, oku kukhokelela kukuphucuka imeko izigulane nokunciphisa ukuyilwa amyloid.

ukudla

Amyloidosis kufuna ukutya rhoqo. Ngophuhliso isifo sezintso esingapheliyo kufuneka umda ukusebenzisa ityuwa, kunye nokutya iiproteins njengenyama, intlanzi, amaqanda. Ukuba ekuphuhliseni ezingapheliyo sisifo sentliziyo, kufuneka ngaphandle ezityiwa netyuwa, watshaya kunye nokutya zifakwa.

amyloidosis intliziyo

Esi sifo kwakhona ngokuba amyloid cardiomyopathy. Nophuhliso kwakhe amyloid nokufumba ezingakhona myocarditis, pericarditis, endocarditis, okanye iindonga aorta kunye nemithambo yenhliziyo. Isizathu soku sifo intliziyo ibe amyloidosis lokuqala, lesibini okanye usapho. Ngokufuthi amyloidosis kwentliziyo - akukho sisifo ekwanti, kwaye iyakhula ngaxeshanye kunye amyloidosis kwemiphunga, zezintso, emathunjini okanye udakada.

Iimpawu amyloidosis intliziyo

Ngokufuthi iimpawu zesi sifo ezifana cardiomyopathy hypertrophic okanye isifo sentliziyo. Ngelo isanda, iimpawu ezi mbaxa. Okungakanani ucaphuka nokudinwa, ezinye ukwehla kobunzima, ukudumba izicwili kunye nesiyezi.

Ngokuguquguquka ezibukhali ngokuqhelekileyo kwenzeka emva kokuba nayiphi na uxinzelelo okanye usuleleko sokuphefumla. Emva koko ziqhele kuvela uhlobo buhlungu intliziyo angina sifuba, arrhythmia, nokudumba ngokukhawuleza, dyspnea, isibindi ukukhula. uxinzelelo lwegazi kukholisa kwehlisa.

Esi sifo sanda ngokukhawuleza, kwaye feature yayo lisahlulayo luvukelo (ukuxhathisa) kuthomalaliso. Kwiingozi ezimasikizi, izigulane zinokuba ascites (ukuqokelelana ulwelo kwi kwisisu) okanye effusion pericardial. Ngenxa lingene amyloid ukuphuhlisa sinus bradycardia. Oku kunokukhokelela ekufeni ngesiquphe.

Outlook

Xa intliziyo amyloidosis abane amahlwempu. ukusilela intliziyo Congestive kule isifo siqhubela phambili ngokuthe ngcembe yaye ukufa igalelekile. In Russia akukho kumaziko ezizodwa ukujongana nalo mba.