The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement

We reviewed clinical presentation, investigations, therapy, prognosis and outcome of 232 patients with primary (AL) cardiac amyloidosis. There were 142 men and 90 women. Median age at presentation was 59 years (range 29-85). AL heart disease was unusual both in patients under the age of 40 (3.0%) and in non-Caucasians (6.5%). Fatigue and weakness were the commonest presenting symptoms. Hallmark features of periorbital ecchymoses and macroglossia were present in 12.5% and 27.2%, respectively. AL cardiac amyloidosis was unusual in isolation (3.9%), and most frequently patients had features of multiorgan dysfunction; heavy proteinuria and features of malabsorption predominating in this respect. Heart involvement represents the worst prognostic indicator, with a median survival from diagnosis of 1.08 years, falling to 0.75 years with the onset of heart failure. Current therapeutic procedures appear to prolong survival, with left ventricular wall thickness, mass and ejection fraction on echocardiography and late potentials on signal averaged electrocardiography of use in prognostic stratification. Cardiac involvement from AL amyloidosis is rapidly fatal. It should be suspected in all patients with heart failure who have wall thickening on echo, normal chamber sizes, low EKG voltages and evidence suggesting a multisystem disease.      

双氢埃托啡的燐光测定法及其应用

用药物—染料生成复合物的原理,建立了双氢埃托啡的燐光分析法?椒槊?可检测2ng。适用于动物血药、脑药分析。兔im0.1,0.2,1mg/kg双氢埃托啡后1与5min。血药,脑药与剂量成比例关系,其回归方程为:血药:Y=100.6X+17.73(1min), Y=367.8X+4.34(5min); 脑药:Y=5363 X+275.4(1 min),Y=6084 X+1022(5 min) 式中Y=药物浓度(ng/ml血或ng/g脑),X=双氢埃托啡剂量(mg/kg)。