【正文】 Pract Cardiovasc Med Beijing Anzhen Hospital Affiliated to Capital Medical University Cardiac Amyloidosis ? 10 per million personyears in the . population ? No single noninvasive test or abnormality is pathognomonic of cardiac amyloid, casecontrol studies indicate that echocardiographic evidence of left ventricular wall thickening, biatrial enlargement, and increased echogenicity in conjunction with reduced electrocardiographic voltages is strongly suggestive of cardiac amyloidosis ? Newer echocardiographic techniques such as strain and strain rate imaging can demonstrate impairment in longitudinal function before ejection fraction bees abnormal J Am Coll Cardiol 2022 Beijing Anzhen Hospital Affiliated to Capital Medical University Cardiac Amyloidosis ? Recent observational studies also suggest that cardiovascular magic resonance imaging yields characteristic findings in amyloidosis, offering promise for the early detection of cardiac involvement ? Presence of detectable cardiac troponin and elevated Btype natriuretic peptide in serum of affected patients portends an adverse prognosis J Am Coll Cardiol 2022 Beijing Anzhen Hospital Affiliated to Capital Medical University Cardiac Amyloidosis ? Management strategies are largely based on nonrandomized singlecenter studies ? One of the few published randomized studies shows the superiority of oral prednisolone and melphalan pared with colchicine in systemic AL amyloidosis ? Intermediatedose infusional chemotherapy regimes (such as vincristine, adriamycin, and dexamethasone) and highdose chemotherapy with peripheral stem cell rescue have been used widely, but treatmentrelated mortality remains substantial with chemotherapy J Am Coll Cardiol 2022 Beijing Anzhen Hospital Affiliated to Capital Medical University Cardiac Amyloidosis ? Recent studies also indicate promising strategies to stabilize the native structures of amyloidogenic proteins。336:267276 EndomyocardialBiopsy Specimens from Patients with Cardiac Amyloidosis Kushwaha S et al. N Engl J Med 1997。阻滯劑、硝酸酯 ？ 4. 利尿劑減量 ？ Beijing Anzhen Hospital Affiliated to Capital Medical University ?病 史 ?初步診斷 ?鑒別診斷 ?體格檢查 ?輔助檢查 ?病情演變 ?補(bǔ)充檢查 ?初步治療 ?治療調(diào)整 ?確定診斷 ?討 論 ?修正診斷 Beijing Anzhen Hospital Affiliated to Capital Medical University 齒齦活檢 ? 鱗狀上皮粘膜慢性炎癥 ? 剛果紅染色（ ） Beijing Anzhen Hospital Affiliated to Capital Medical University 腹壁皮膚活檢 ? 剛果紅染色（ ） Beijing Anzhen Hospital Affiliated to Capital Medical University 能否排除淀粉樣變？ Beijing Anzhen Hospital Affiliated to Capital Medical University 心肌活檢 ? 右側(cè)室間隔 7塊心肌送檢 ? 間質(zhì)明顯增多，心肌細(xì)胞退行性變，心內(nèi)膜下及小血管周?chē)?jiàn)均質(zhì)淡染物沉積 ? 剛果紅偏光顯微鏡下可見(jiàn)折光物呈藍(lán)綠色，Masson（ +）， PATH（ +） Beijing Anzhen Hospital Affiliated to Capital Medical University 胃鏡及胃粘膜活檢 ? 慢性淺表性胃炎伴多發(fā)糜爛結(jié)節(jié)，十二指腸球炎，HP（ +） ? 剛果紅染色（ ） Beijing Anzhen Hospital Affiliated to Capital Medical University 腎功能受損原因？ ? 有效循環(huán)血容量不足？ ? 淀粉樣變？ Beijing Anzhen Hospital Affiliated to Capital Medical University ? 腎活檢（未做） ? 223。Beijing Anzhen Hospital Affiliated to Capital Medical University Case Presentation 首都醫(yī)科大學(xué)北京安貞醫(yī)院 楊士偉 周玉杰 Beijing Anzhen Hospital Affiliated to Capital Medical University ?病 史 ?初步診斷 ?鑒別診斷 ?體格檢查 ?調(diào)整治療 ?輔助檢查 ?病情演變 ?修正診斷 ?初步治療 ?補(bǔ)充檢查 ?確定診斷 ?討 論 Beijing Anzhen Hospital Affiliated to Capital Medical University 基本情況 ?男性， 49歲，工人 ?主訴：間斷胸悶 1年余，加重 2月 ?入院日期： Beijing Anzhen Hospital Affiliated to Capital Medical University 現(xiàn)病史 ? 1年前散步時(shí)無(wú)明顯誘因出現(xiàn) 胸悶、氣短 ，休息 半小時(shí) 后自行緩解，無(wú)明確胸痛、肩背痛，無(wú)發(fā)熱、咳嗽、咳痰、咯血，無(wú)頭暈、黑蒙、暈厥 ? 患者 活動(dòng)耐量進(jìn)行性下降 ，步行數(shù)十米即感胸悶、憋氣，偶有不能平臥和夜間陣發(fā)性呼吸困難，同時(shí)伴有 雙下肢浮腫和尿量減少 Beijing Anzhen Hospital A