Amyloidosis - ihe ọ bụ? Amyloidosis: akpata, mgbaàmà, ọgwụgwọ, nchoputa

Amyloidosis - ihe ọ bụ? Ọ bụ a ọrịa nke protein metabolism na nke e guzobere na deposition dị iche iche anụ ahụ na akụkụ nke a kpọmkwem protein-polysaccharide umi - amyloid.

Mmepe nke ọrịa

Zụlite amyloidosis (ihe ọ bụ - anyị na-ama hụrụ) mebiri nke protein njikọ na reticuloendothelial usoro. The plasma bụ ìgwè ndiiche na-edozi. Ndị a na-edozi na-nnoo onwe abụkwara na ime ka ihe anataghi yiri guzobe autoantibodies.

Mgbe ahụ, ndị a-alụso ọrịa ọgụ na abụkwara na-eme grubodispersionnye edozi ịkpali. Ebe ọ bụ na e guzobere amyloid. Nke a ihe onwunwe na-edebe na arịa mgbidi na dị iche iche akụkụ. Nke nta nke nta dum n'ịkpa, amyloid na-eduga ná ọnwụ nke ahu.

Types of amyloidosis. ihe

E nwere ọtụtụ ụdị nke amyloidosis. Na-akpata ọrịa bụ kpọmkwem dabere na ihe ụdị amyloidosis. Gịnị ka ọ bụ? Nhazi ọkwa a rụrụ dị ka ndị bụ isi na protein, nke iso nke amyloid fibrils. N'okpuru ebe a bụ echiche nke ọrịa.

1. Primary amyloidosis (AL-amyloidosis). Mgbe ya mmepe na plasma egosi ndiiche immunoglobulin ìhè ígwè, bụ ndị nwere ike idozi dị iche iche na anụ ahụ nke ahụ. N'otu aka ahụ gbanwere n'oge plasma mkpụrụ ndụ nke multiple myeloma, Waldenström si macroglobulinemia, monoclonal hypergammaglobulinemia.
2. Secondary amyloidosis (AA-amyloidosis). Na nke a, e nwere oké ntọhapụ nke imeju protein Alfa-globulin. Nke a nnukwu-adọ protein na-synthesized na-adịghị ala ala mbufụt. Nke a kwere omume na ọrịa dị iche iche, dị ka ọrịa ogbu ogbu na nkwonkwo, ịba, bronchiectasis, osteomyelitis, ekpenta, ụkwara nta.
3. Family amyloidosis (AF-amyloidosis). Ọ bụ a butere n'aka ọrịa na autosomal recessive nketa. Ọ na-akpọ Mediterranean fever intermittent ma ọ bụ paroxysmal poliserozita ezinụlọ. Egosi na ọrịa a ọgụ nke fever, omume nke abdominal mgbu, anụ rashes, ogbu na nkwonkwo, na pleurisy.
4. The dialysis amyloidosis (AH-amyloidosis). Ọ na-ejikọrọ na eziokwu na protein beta-2 microglobulin MHC na ndị ahụ siri ike na-ewekarị nke site akụrụ, na ọ na-adịghị ẹdụk na hemodialysis, ya na ìgwè ke idem.
5. Æ-amyloidosis. Ọ na-emepe emepe ụdị ụfọdụ nke cancer, dị ka thyroid.
6. Senail amyloidosis.

mgbaàmà

Mgbe nchoputa nke "Amyloidosis" mgbaàmà adabere na orunótu nke ego na ya. Na mmeri nke eriri afọ tract pụrụ hụrụ ikpọ ire, mkpọchị-elo ọrụ, afọ ntachi ma ọ bụ afọ ọsịsa. E nwere ike akpụ-amyloid ego na eriri afọ ma ọ bụ na afo.

amyloidosis nke eriri afọ esonyere a mmetụta nke arọ na erughị ala nwere ike ịdị nwayọọ abdominal mgbu. Ọ bụrụ na gburu anyịnya, bụ chee otu mgbaàmà dị ka pancreatitis. Ọ bụrụ na imeju mmerụ hụrụ ya na-abawanye, ọgbụgbọ, belching, vomiting, ọdụdọ, jaundice.

Amyloidosis akụkụ okuku ume na-egosi dị ka ndị a:

• keolu mgbaka olu;
• mgbaàmà nke bronchitis;
• akpa ume akpụ amyloidosis.

Ndị na-esonụ mgbaàmà nwere ike ime amyloidosis nke ụjọ usoro:

• tingling ma ọ bụ na-ere sensashion na aka na ụkwụ, nkụnwụ (elu polyneuropathy);
• isi ọwụwa, dizziness;
• sphincter ọrịa (urinary incontinence, Nsị).

Amyloidosis - ihe ọ bụ, ya na-akpata na mgbaàmà anyị enyocha. Ugbu a, anyị na-ele otú ọrịa na-chọpụtara na ihe na ụzọ nke ọgwụgwọ adị.

nchọpụta nsogbu

N'ọnọdụ dị otú a ọrịa dị ka amyloidosis, nchoputa bụ mgbagwoju. Họpụtara laabu na ngwaike nnyocha.

Na laabu ọmụmụ, a n'ozuzu nyochaa nke ọbara hụrụ abawanye na ESR, leukocytes na-ebelata platelet ọnụ. The n'ozuzu analysis of mmamịrị protein bụ ugbu ke sedimenti - cylinders, leukocytes na erythrocytes. Na coprogram chee buru ibu nke starch, abụba na muscle emep uta eri. Na ọbara n'ihi mmiri ọgwụ na ọnya nke imeju ekpughewo ubara cholesterol, bilirubin, alkaline phosphatase.

Na isi amyloidosis na mmamịrị na ọbara plasma gosiri a elu ọdịnaya nke amyloid. Mgbe nke abụọ n'oge laabu ule na-egosi ihe ịrịba ama nke-adịghị ala ala mbufụt.

Ọzọkwa na-eduzi ndị ọzọ na-achọpụta ọrịa jikoro:

• X-ray ule;
• echocardiography (na ikpe nke a-enyo enyo na obi agha);
• ọtọ assays na e ji esiji ákwà;
• biopsy ozu.

ọgwụgwọ

Mgbe ọrịa a rụrụ-ndidi ọgwụgwọ. Amyloidosis, nke e nwere na oké njọ ọnọdụ, dị ka ala ala gbasara akụrụ odida ma ọ bụ oké obi odida emeso a ọgwụ.

Na isi amyloidosis ibido họpụtara ọgwụ ọjọọ ndị dị ka "Chloroquine" "Melphalan" "prednisolone", "Colchicine".

Sekọndrị amyloidosis emeso ndị kpatara ọrịa, e.g., osteomyelitis, ụkwara nta, pleural empyema et al., All mgbaàmà nke amyloidosis mgbe kpamkpam mgbe ọgwụgwọ.

Ọ bụrụ na ọrịa na-eto ka a n'ihi nke akụrụ hemodialysis, mgbe ahụ, ndị dị otú ahụ a-enwe ndidi na-agafere peritoneal dialysis.

N'ihe banyere nke afọ ọsịsa binders eji ọgwụ, ndị dị ka "bismuth subnitrate," ma ọ bụ adsorbing n'ụlọnga.

Ha pụkwara symptomatic ọgwụgwọ:

• preparations mbenata nsogbu;
• vitamin, diuretics;
• plasma mmịnye, wdg

Ọzọkwa, ọ nwere ike na-etinyere na-awa ahụ na ọgwụgwọ. amyloidosis nke splin nwere ike agbahapụ mgbe mwepụ nke ngwa. N'ọnọdụ ka ukwuu, a na-eduga onye na mma na ọnọdụ nke ndị ọrịa na belata guzobere amyloid.

nri

Amyloidosis achọ mgbe nile na-eri. Na mmepe nke-adịghị ala ala ọrịa akụrụ ga-amachi nnu oriri, na protein oriri ndị dị ka anụ, azụ, àkwá. Ọ bụrụ na-emepe emepe adịghị ala ala obi ọdịda, a ga-ekwe n'aka nri nke nnu, na-ese siga na pickled oriri.

gbasara obi amyloidosis

Nke a na ọrịa a na-akpọ amyloid cardiomyopathy. Na ya mmepe nke amyloid deposition nwere ike ime na myocarditis, pericarditis, endocarditis, ma ọ bụ na mgbidi nke aorta na akwara akwara. Ihe kpatara nke a ọrịa obi na ọrịa nwere ike isi amyloidosis, nke abụọ ma ọ bụ ezinụlọ. Ọtụtụ mgbe amyloidosis nke obi - ọ bụghị ihe ndị dịpụrụ adịpụ ọrịa, na ọ na-emepe emepe ke ukem na amyloidosis nke akpa ume, akụrụ, eriri afọ ma ọ bụ splin.

Mgbaàmà nke gbasara obi amyloidosis

Ọtụtụ mgbe ihe mgbaàmà nke ọrịa a yiri hypertrophic cardiomyopathy ma ọ bụ ịrịa ọrịa obi. Mgbe mbụ ogbo, mgbaàmà ndị na-edochaghị anya. Nwere ike ogwe na ike ọgwụgwụ, ụfọdụ arọ ọnwụ, ọzịza nke anụ ahụ na dizziness.

The nkọ arịa ọrịa na-emekarị emee mgbe ọ bụla nsogbu ma ọ bụ akụkụ okuku ume na ọrịa. Mgbe na-ejikarị na-egosi na obi mgbu ụdị rịaworo pectoris, arrhythmia, akpọ edema, dyspnea, imeju nditat. Ọbara mgbali na-emekarị, zie.

The ọrịa na-aga n'ihu ngwa ngwa, na ya pụrụ iche mma bụ ndị na-eguzogide (eguzogide) na ọgwụ. Na oké ikpe, ọrịa nwere ike na-enwe ascites (ìgwè ọmụmụ na ndị abdominal oghere) ma ọ bụ pericardial effusion. N'ihi na amyloid infiltrates ịzụlite imi bradycardia. Nke a nwere ike ime ka ọnwụ mberede.

ele ihe anya

Na obi amyloidosis ogbenye prognosis. Congestive obi ọdịda ọrịa a na-aga n'ihu iji nwayọọ nwayọọ na na ọnwụ dị nso. Na Russia ọ dịghị pụrụ iche na emmepe-emeso a nke.