Amyloidosis - chii ichocho? Amyloidosis: zvinokonzera, zviratidzo, kurapa, kuongororwa

Amyloidosis - chii ichocho? Ichi chirwere, chinokonzerwa nekuputsa kweprotein metabolism, umo kuumbwa uye kuiswa muzvimedu zvakasiyana-siyana uye nhengo dzepurotini-polysaccharide chinhu-amyloid.

Kukura kwechirwere

Amyloidosis inotanga (chii ichocho - isu chatatoziva kare) mukuputsa mapuroteni mukati re reticulo-endothelial system. Mune plasma yeropa, kuunganidza kweprotini isina kukwana kunoitika. Aya mapuroteni anonyanya kurwisa autoantigens uye anokonzera, nekuenzanirana nehutachiona, kuumbwa kwe autoantibodies.

Izvi zvinopesana nemasikeni zvino zvinoita uye mapuroteni akapararira akaoma. Izvi ndizvo zvakagadzirirwa amyloid. Izvi zvinogadziriswa pamadziro edzimba uye nhengo dzakasiyana-siyana. Zvishoma nezvishoma kuunganidza, amyloid inotungamirira kurufu rwechirongwa.

Mhando dzeamyloidosis. Zvinokonzera

Kune marudzi akawanda emamyloidosis. Zvinokonzera kukwidziridzwa kwechirwere zvakananga zvichienderana nemhando yei amyloidosis. Chii ichocho? Chirongwa chinoitwa zvichienderana neprotein yakakura, inosanganisira amyloid fibrils. Pasi pane marudzi ehosha iyi.

1. Primary amyloidosis (AL-amyloidosis). Apo inokura, kusvibiswa kwemaketani emaketani emu immunoglobulin anoonekwa muropa remazi, iro rinokwanisa kugadzikana mumitumbi yakasiyana-siyana yemuviri. Saizvozvowo, masero eplasma anoshandura mu myeloma, Waldenstrom's macroglobulinemia, monoclonal hypergammaglobulinemia.
2. Secondary amyloidosis (AA-amyloidosis). Muchiitiko ichi, kunyanya kusunungurwa kweproteni alpha-globulin kunoitika. Ichi chikamu chakaoma cheprotheni chinoshandiswa mune chirwere chisingaperi chine kupisa. Izvi zvinogona kuitika nezvirwere zvakasiyana-siyana, somuenzaniso, nerheumatoid arthritis, malaria, bronchiectasis, osteomyelitis, maperembudzi, tuberculosis.
3. Mhuri amyloidosis (AF-amyloidosis). Iyi ndiyo maitiro ehutano hwechirwere ichi ne autosomal recessive nhaka nhaka. Inonziwo Mhepo yeMediterranean inopinda mukati mefivha kana mhuri yepodoxysmal polyserositis. Ichi chirwere chinoratidzirwa nekurwiswa kwefivha, kuitika kwemarwadzo mukati mudumbu, mashizha paganda, arthritis uye pleurisy.
4. Dialysis amyloidosis (AH-amyloidosis). Inobatanidza nechokwadi chokuti puroteni beta-2-microglobulin MHC muvanhu vane utano inoshandiswa neitsvo, uye pasi pehemodialysis haisati yakasvibiswa, uye naizvozvo kuunganidza kwayo kunoitika mumuviri.
5. AE-amyloidosis. Inokura mune dzimwe nzira dzekenza, somuenzaniso, chirwere chetachiona.
6. Kukwegura amyloidosis.

Zviratidzo

Kana uchitaridzika se "amyloidosis," zviratidzo zvinoenderana nenzvimbo yemadhora. Kana chirwere chepamuviri chakakanganiswa, ruzivo rwakawedzerwa, kushaya simba kumedza basa, kuvhiringidzika kana chirwere chinogona kuitika. Dzimwe nguva amyloid tumoral inopinda mumuviri kana mudumbu inogoneka.

Amyloidosis yemukumbo inofambidzana nemanzwiro ekuoma uye asinganzwisisiki, pangava nekurwadziwa mupfava mudumbu. Kana pancreas ichibatwa, ipapo pane zviratidzo zvakafanana sezvinoitwa pancreatitis. Kana kukanganisa kwechiropa kunoonekwa, kunowedzera, kusuruvara, kukanganisa, kurutsa, jaundice.

Amyloidosis yomuviri wekufuridzira inoratidzirwa seizvi:

• Inzwi rinoputika;
• Symptomatology ye bronchitis;
• Chirwere chepulmonary-like amyloidosis.

Ne amyloidosis yenheyo yenheyo, zviratidzo zvinotevera zvinogona kuitika:

• Kufungidzira kwekurira kana kupisa mumagumo, manzwiro ekushaya simba (peripheral polyneuropathy);
• Musoro wemusoro, chizoro;
• Sphincter matenda (incontinence, feces).

Amyloidosis - chii ichocho, zvikonzero zvekuitika kwaro uye zviratidzo zvatakurukura. Iye zvino tichazonzwisisa kuti chirwere ichi chinowanikwa sei uye ndeapi nzira dzokurapa nadzo dziripo.

Diagnostics

Nechirwere chakadai seamyloidosis, kuongororwa kwakaoma. Zvidzidzo zveraibhurari uye zvigadzirwa zvakagadzirwa.

Pavatoriyano inotsvakurudza munharaunda yakawanda kana inowanzoongororwa yeropa kuwedzerwa kweERR, leukocytes uye kuora mwoyo kweprombocytes kunoonekwa. Muchidimbu kuongorora urine kune protein, mu sediment - cylinders, leukocytes uye erythrocytes. Mune purogiramu pane huwandu hwakawandisa hwemashizha, mafuta uye masumichu. Mune biochemistry yeropa ine kukuvadzwa kwechiropa, pane kuwedzerwa kwehuwandu hwehushuga yeklesterol, bilirubin, alkaline phosphatase.

Muchikamu chikuru amyloidosis mumuti uye plasma yeropa, chikamu chepamusoro cheamyloid chinowanikwa. Pachipiri paongororo yeongororo inowana zviratidzo zvekusava nechirwere chekuvhiringidza.

Uyewo ita mamwe maitiro ekuongorora:

• X-ray kuongorora;
• Echocardiography (kana zvichifungidzirwa chirwere chemwoyo);
• Maitiro anoshandiswa nemavara;
• Nyaya yezvikamu.

Kurapa

Nechirwere ichi, kunze kwekurapa kunopiwa. Amyloidosis, umo maitiro akaoma anocherechedzwa, somuenzaniso, mukusagadzikana kwemasimba kusingaperi kana kuora mwoyo kwakanyanya, kunobatwa muchipatara.

Pakutanga amyloidosis panguva yekutanga yakagadzirwa, "Chloroquine", "Melfalan", "Prednisolonum", "Kolkhitsin" inogadzwa.

Muchikamu chechipiri amyloidosis, chirwere chepachirwere chinorapwa, somuenzaniso, osteomyelitis, tuberculosis, pleuralma empyema, nezvimwewo. Kazhinji mushure mekurapa kwayo zviratidzo zvose zvemamyloidosis zvinonyangarika.

Kana chirwere chacho chikavapo nekuda kwepvo hemodialysis, zvino murwere uyu anotamirwa ku peritoneal dialysis.

Kana pane chirwere, chirwere che-astringent, somuenzaniso, "Bismuth Subnitrate" kana kuti adsorptive agents, anoshandiswa.

Shandisawo zviratidzo zvinoratidzira:

• Zvinodhaka zvinoderedza kuwedzera kweropa;
• Vitamines, diuretics;
• Kuwedzerwa kweplasma, nezvimwewo.

Mukuwedzera, kurapwa kunogona kushandiswa zvakare. Amyloidosis yepleen inogona kudzokera shure mushure mekubviswa kwechirongwa. Muzviitiko zvakawanda izvi zvinotungamirira mukuvandudzika mumamiriro ezvinhu evarwere nekuderera kwekuumbwa kweamyloid.

Power supply

Mukana we amyloidosis, kudya kunodiwa. Nekuvandudzwa kwekusagadzikana kwetsvo kusingaperi kunofanira kuderedza kushandiswa kwemunyu nemaprotheni michina, zvakadai se nyama, hove, mazai. Kana kusagadzikana kwepfungwa kusakonzera kuitika, munyu, kusvuta uye fukweti zvokudya zvinofanira kuiswa kunze kwekudya.

Amyloidosis yemwoyo

Ichi chirwere chinonziwo amyloid cardiopathy. Muchigadzirwa chayo, kuiswa kwema-amyloid kunogona kuitika mumagarikoriyumu, pericardium, endocardium kana pamasvingo e-aorta nemidziyo yemakononi. Chinokonzera kukundwa uku kwemoyo kunogona kushanda sekutanga amyloidosis, yechipiri kana mhuri. Kazhinji, amyloidosis yemwoyo haisi chirwere chisina kurega, uye inowedzera zvakafanana neamyloidosis yemapapu, itsvo, matumbo kana chirwere.

Zviratidzo zveamyloidosis yemwoyo

Kakawanda zviratidzo zvechirwere ichi zvakafanana nehutachida hwemwoyo kana chirwere chemwoyo chinonzi ischemic. Pakutanga kwekutanga, zviratidzo hazvisi pachena zvakajeka. Kukanganisika uye kuneta, kumwe kurasikirwa, kufukura kwemasumbu uye chirwere chinogona kuonekwa.

Kuora kwakanyanya kunowanzoitika mushure mokunge mamiriro ekumanikidzira mamiriro kana kupemha kutapukirwa. Mushure meizvozvo, kazhinji kune marwadzo emwoyo akaita se angina pectoris, arrhythmias, kutumidzwa kuputika, kufema kwepfufu, chiropa chakawedzerwa. Ropa rinowanzoperera.

Chirwere chacho chinokura nokukurumidza uye chakasiyana-siyana chiri kukanganisa kurapwa. Muzviitiko zvakakomba, varwere vanogona kunge vane ascites (mvura inowedzera mumakumbo emimba) kana pericardial effusion. Nokuda kweamyloid inopinda, kusava nesimba kwekodhi ye sinus uye bradycardia inobudirira. Izvi zvinogona kutungamirira kukufa kamwe kamwe.

Forecast

Ne amyloidosis, kubvuma hakushamisi. Kukundikana kwemoyo mune chirwere ichi kunoramba kuchifambira mberi, uye chiitiko chinouraya hachidziviriki. MuRussia hapasina nzvimbo dzakakosha dzinobata nedambudziko iri.