Hemodialysis patients suffer from premature atherosclerosis and various thrombotic and thromboembolic phenomena. Despite enhanced in vivo platelet activity, it is unclear whether in vitro platelet aggregation is increased or reduced in this population. We studied platelet aggregation in response to adenosine diphosphate and to epinephrine in 20 chronic hemodialysis patients and 21 controls. In the patient group the in vitro platelet aggregation was significantly enhanced in response to both aggregating agents. Our study demonstrated that in vitro platelet aggregation is enhanced in patients with chronic renal failure undergoing hemodialysis.

Fechtner syndrome, a variant of Alport syndrome, was first reported by Peterson et al. [1985]. It is characterized by nephritis, hearing loss, eye abnormalities, macrothrombocytopenia, and leucocyte inclusions, present in varying combinations in several members of the same family. This is the second family reported; 16 relatives are affected. The clinical manifestations of the syndrome are delineated. The pattern of inheritance is autosomal dominant. The hematologic abnormalities are similar to those detected in May Hegglin anomaly. They are present in every affected relative and may be present at birth. The feasibility of prenatal diagnosis is discussed.

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Increased platelet activation has been shown to be a feature of patients with familial hypercholesterolaemia. Plasma lipoprotein concentration and composition were studied in eleven male patients with familial hypercholesterolaemia and in ten age-matched healthy controls. Increased levels of cholesterol were found in very-low- and low-density lipoproteins (53 and 275%, respectively), whereas in high-density lipoprotein, both cholesterol and apolipoprotein A-I were decreased by 21 and 26%, respectively. On incubation of gel-filtered platelets derived from normolipidaemic controls with identical concentrations of lipoproteins derived from either nomolipidaemic controls or hypercholesterolaemic patients very-low- and low-density lipoproteins from the patients caused significantly greater thrombin-induced platelet aggregation (P less than 0.01). High-density lipoprotein from normal subjects reduced platelet release by 22%, whereas the patients' high-density lipoprotein had no significant effect on platelet release. Lipoprotein-deficient plasma from both groups augmented platelet function to a similar extent. Lipoprotein composition has an important effect on platelet function in vitro. The abnormal lipid and protein composition of the lipoproteins derived from hypercholesterolaemic patients appears to be the cause of platelet hyperactivity observed in these patients.

SUMMARY High-density lipoprotein (HDL) levels were studied in 10 male patients with severe coronary atherosclerosis and in 10 well-matched controls. All subjects were normolipidemic, and the presence of a disease or other factor influencing HDL levels was excluded. Very low density lipoprotein and low-density lipoprotein levels were similar in both groups, but HDL concentration was significantly lower in the patient group. Analysis of HDL subfractions revealed that both HDL2 and HDL3 concentrations were significantly lower in the patient group. The composition of both HDL subfractions was also altered in the patient group: an increased cholesterol-to-protein ratio was found. These data strengthen the evidence in support of an important and independent role for HDL in the pathogenesis of coronary atherosclerosis. It appears that both HDL2 and HDL3 are implicated and that both the concentration and composition of HDL are important.CORONARY heart disease (CHD) is the main cause of death in the Western world.' The pathogenesis of CHD is not clear, and the interrelationships of many factors appear to have a role. Hyperlipidemia increases the risk for CHD, but about half of the patients with CHD have normal plasma lipid levels. An inverse relationship between CHD and high-density lipoprotein (HDL) levels has been established for both the cholesterol and apoprotein components of the lipoprotein.2 3 HDL, however, is not a homogeneous moiety, and the concentration and composition of the HDL subclasses in CHD have hardly been studied. Gofman et al.4 determined the HDL2 and HDL3 concentrations of the lipoproteins by analytic ultracentrifugation. Concentrations of both subfractions were depressed in CHD patients, but because most of the patients were hyperlipidemic, interpretation of the results proved difficult.A positive cholesterol balance in the cells of the primary atherosclerotic lesion may be responsible for the evolution of this lesion into an advanced plaque,5 and HDL may remove cholesterol from extrahepatic tissues6 and thus prevent its accumulation. We therefore decided to study HDL concentration and composition in normolipidemic CHD patients. Patients with definite angiographic evidence of the disease were selected. Both the lipid and protein components of HDL and its subfractions were determined. Methods PatientsTen men, ages 38-64 years (mean 55 years), who had undergone coronary angiography for symptomatic CHD and were shown to have an occlusion of more than 70% of the diameter of at least one major coronary artery and who also fufilled the following criteria were selected for study. None gave a history of hyperlipide-

Concomitant occurrence of meningeal involvement and thoracic plasmacytoma was observed in a patient with IgA lambda myeloma. Cerebrospinal fluid analysis revealed IgA‐λ paraprotein and pathological plasma cells. CT scan of the chest and lumbar myelogram excluded spinal cord compression. The patient partially responded to craniospinal irradiation but succumbed to rapidly progressive myeloma 20 weeks following diagnosis of meningeal involvement.

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