Amyloidosis - yog dab tsi? Qhov no yog ib tug kab mob tshwm sim los ntawm kev ua txhaum ntawm cov protein metabolism, uas tsim thiab deposition nyob rau hauv ntau yam ntaub so ntswg thiab kabmob ntawm ib tug tshwj xeeb protein-polysaccharide tshuaj - amyloid.
Kev txhim kho kab mob
Amyloidosis tsim (nws yog dab tsi - peb twb pom lawm) ua txhaum ntawm cov protein synthesis hauv reticuloendothelial system. Cov protein ntau txawv txav hauv cov ntshav plasma. Cov proteins no yog qhov tseem ceeb ntawm tus kheej-antigens thiab ua rau tsim cov autoantibodies los ntawm kev sib piv nrog kev ua xua.
Tom qab ntawd, cov tshuaj tiv thaiv no ua rau cov tshuaj tiv thaiv kab mob thiab cov proteins sib cais sib cais ua ntej. Qhov no yog li cas amyloid tsim. Cov tshuaj no nyob rau ntawm phab ntsa vascular thiab ntau yam kabmob. Maj mam sib sau ua ke, amyloid ua rau lub cev tuag.
Hom amyloidosis. Yog vim li cas
Muaj ntau hom amyloidosis. Qhov ua rau ntawm kev loj hlob ntawm tus kab mob ncaj qha nyob ntawm seb hom amyloidosis. Nws yog dab tsi? Kev faib tawm yog ua raws li cov protein tseem ceeb uas ua rau amyloid fibrils. Hauv qab no yog cov homkab mob.
- Primary amyloidosis (AL-amyloidosis). Nrog nws txoj kev loj hlob, lub teeb tsis txawv txav ntawm immunoglobulins tshwm sim hauv cov ntshav plasma, uas tuaj yeem nyob hauv ntau cov ntaub so ntswg ntawm lub cev. Ib yam li ntawd, cov plasma hlwb hloov ntau yam myeloma, Waldenström's macroglobulinemia, monoclonal hypergammaglobulinemia.
- Secondary amyloidosis (AA-amyloidosis). Hauv qhov no, muaj ntau qhov tso tawm ntawm alpha-globulin protein los ntawm lub siab. Nws yog ib tug mob theem protein uas yog synthesized thaum lub sij hawm inflammatory txheej txheem. Qhov no ua tau rau ntau yam kab mob, xws li mob caj dab rheumatoid, malaria, bronchiectasis, osteomyelitis, mob ruas, tuberculosis.
- Tsev neeg amyloidosis (AF-amyloidosis). Qhov no yog ib daim ntawv keeb kwm ntawm tus kab mob nrog autosomal recessive inheritance mechanism. Nws tseem yog hu ua Mediterranean tsis sib haum xeeb ua npaws lossis tsev neeg paroxysmal polyserositis. Tus kab mob no tshwm sim los ntawm kev tawm tsam ntawm kub taub hau, tshwm sim ntawm qhov mob plab, daim tawv nqaij ua pob, mob caj dab thiab pleurisy.
- YDialysis amyloidosis (AH-amyloidosis). Nws muaj feem xyuam rau qhov tseeb tias cov protein beta-2-microglobulin MHC hauv cov neeg noj qab haus huv tau siv los ntawm ob lub raum, thiab thaum lub sij hawm hemodialysis nws tsis lim, thiab yog li ntawd nws accumulates nyob rau hauv lub cev.
- AE-amyloidosis. Tsim muaj qee yam mob qog noj ntshav, xws li mob qog noj ntshav.
- senile amyloidosis.
Symptoms
Thaum kuaj pom tias muaj amyloidosis, cov tsos mob nyob ntawm qhov chaw ntawm qhov tso nyiaj. Thaum swb lawmntawm txoj hnyuv, tus nplaig loj, nqos nyuaj, cem quav lossis raws plab yuav raug pom. Amyloid qog zoo li tso rau hauv cov hnyuv lossis plab yog qee zaum ua tau.
plab hnyuv amyloidosis yog nrog los ntawm kev xav hnyav thiab tsis xis nyob, tej zaum yuav muaj mob me me hauv plab. Yog tias tus kab mob pancreatic cuam tshuam, ces tib cov tsos mob tshwm sim nrog tus mob pancreatitis. Thaum daim siab puas lawm, nws pom tias nws nce, xeev siab, belching, bouts ntawm ntuav, jaundice tshwm.
Ua pa amyloidosis tshwm sim raws li hauv qab no:
- suab nrov;
- mob bronchitis;
- pulmonary amyloidosis qog.
Cov kab mob plawv amyloidosis tuaj yeem tshwm sim nrog cov tsos mob hauv qab no:
- tingling lossis kub hnyiab hauv nqua, loog (peripheral polyneuropathy);
- mob taub hau, kiv taub hau;
- sphincter mob (urinary incontinence, quav).
Amyloidosis - nws yog dab tsi, peb tau txiav txim siab nws qhov ua rau thiab cov tsos mob. Tam sim no cia peb txheeb xyuas seb tus kab mob no kuaj tau li cas thiab nws txoj kev kho li cas.
Diagnosis
Nyob hauv tus kab mob xws li amyloidosis, kev kuaj mob yog nyuaj. Lub chaw soj ntsuam thiab kev tshawb fawb kho vajtse.
Hauv kev tshawb fawb hauv chav kuaj ntshav dav dav, pom qhov nce hauv ESR, leukocytes thiab txo qis hauv platelets. Hauv kev tsom xam dav dav ntawm cov zis muaj protein, hauv cov sediment muaj cov thooj voos kheej kheej, leukocytes thiab erythrocytes. Hauv coprogrammuaj ntau cov hmoov txhuv nplej siab, rog thiab nqaij fibers. Hauv biochemistry ntawm cov ntshav nrog lub siab puas tsuaj, cov ntsiab lus ntawm cov roj cholesterol, bilirubin, alkaline phosphatase tau pom.
Hauv thawj amyloidosis, cov ntsiab lus siab ntawm amyloid muaj nyob hauv cov zis thiab ntshav ntshav. Hauv qhov thib ob thaum lub sijhawm kuaj kuaj, pom pom muaj cov tsos mob ntawm cov txheej txheem mob ntev.
Tseem ua lwm yam kev kuaj mob:
- kuaj hluav taws xob;
- echocardiography (nyob rau hauv cov ntaub ntawv xav tias mob plawv);
- kev ntsuas kev ua haujlwm nrog dyes;
- organ biopsy.
Kev kho mob
Tus kab mob no raug kho raws li tus neeg mob sab nraud. Amyloidosis uas muaj mob hnyav, xws li lub raum tsis ua haujlwm lossis lub plawv tsis ua haujlwm hnyav, raug kho hauv tsev kho mob.
Nyob rau hauv thawj amyloidosis, nyob rau hauv thawj theem, tshuaj xws li Chloroquine, Melphalan, Prednisolone, Colchicine yog sau.
Nyob rau hauv amyloidosis theem nrab, cov kab mob hauv qab yog kho, piv txwv li, osteomyelitis, tuberculosis, pleural empyema, thiab lwm yam. Feem ntau, tom qab nws kho, tag nrho cov tsos mob ntawm amyloidosis ploj.
Yog tus kab mob no tshwm sim los ntawm lub raum hemodialysis, ces tus neeg mob no raug xa mus rau peritoneal dialysis.
Astringents xws li Bismuth Subnitrate lossis adsorbents yog siv yog tias raws plab tshwm sim.
kev kho mob kuj tseem siv:
- tshuaj txo cov ntshav siab;
- Vitamins, tshuaj diuretics;
- ntshav ntshav ntshav, thiab lwm yam.
Ntxiv rau, kev kho phais tuaj yeem siv tau. Amyloidosis ntawm tus po tuaj yeem thim rov qab tom qab tshem tawm ntawm lub cev. Feem ntau, qhov no ua rau muaj kev txhim kho ntawm cov neeg mob thiab txo qis ntawm kev tsim amyloid.
Khoom noj
Amyloidosis xav tau kev noj zaub mov tas li. Nrog rau kev loj hlob ntawm lub raum tsis ua haujlwm, kev noj cov ntsev thiab cov khoom noj protein xws li nqaij, ntses, thiab qe yuav tsum raug txwv. Yog tias mob plawv tsis ua haujlwm, ntsev, haus luam yeeb thiab cov khoom noj qab zib yuav tsum raug cais tawm ntawm kev noj haus.
Tus kab mob no tseem hu ua amyloid cardiopathy. Nrog nws txoj kev loj hlob, amyloid deposition tuaj yeem tshwm sim hauv myocardium, pericardium, endocardium, lossis ntawm phab ntsa ntawm lub aorta thiab cov hlab ntsha. Qhov ua rau muaj kev puas tsuaj rau lub plawv yuav yog thawj amyloidosis, theem nrab lossis tsev neeg. Feem ntau, amyloidosis ntawm lub plawv tsis yog ib qho kab mob sib cais, thiab nws tshwm sim nyob rau tib lub sijhawm nrog amyloidosis ntawm lub ntsws, ob lub raum, txoj hnyuv, lossis tus po.
Symptoms of cardiac amyloidosis
Feem ntau cov tsos mob ntawm tus kab mob no zoo ib yam li hypertrophic cardiopathy lossis kab mob plawv. Thaum pib, cov tsos mob tsis tau qhia meej meej. Kev npau taws thiab qaug zog, qee qhov poob phaus, cov ntaub so ntswg o thiab kiv taub hau tuaj yeem tshwm sim.
Ib qho kev puas tsuaj loj feem ntau tshwm sim tom qab muaj kev ntxhov siab lossis kab mob ua pa. Tom qab ntawd, qhov mob hauv lub plawv feem ntau tshwm sim raws li hom angina pectoris, arrhythmias, pronounced edema, ua tsis taus pa, o ntawm daim siab. Ntshav siab feem ntau yog qis.
Tus kab mob no sai heev thiab nws qhov txawv yog qhov tsis kam (tiv taus) rau kev kho tsis tu ncua. Hauv cov xwm txheej hnyav, cov neeg mob yuav muaj ascites (kua kua hauv plab) lossis pericardial effusion. Vim yog amyloid infiltrates, tsis muaj zog ntawm sinus node thiab bradycardia tsim. Qhov no tuaj yeem ua rau tuag sai sai.
Huab cua
Nrog amyloidosis ntawm lub plawv, qhov kev tshwm sim tsis zoo. Lub plawv tsis ua hauj lwm nyob rau hauv tus kab mob no tsis tu ncua, thiab kev tuag yog inevitable. Tsis muaj cov chaw tshwj xeeb hauv tebchaws Russia daws qhov teeb meem no.
