Late-onset and long-lasting neutropenias in the young: A new entity anticipating immune-dysregulation disorders.

This study identifies a new chronic form of immune neutropenia in the young with or without detectable indirect anti-neutrophil antibodies, characterized by mild/moderate neutropenia low risk of severe infection (14%), tendency to develop autoimmune phenomena over the course of the disease (cumulative incidence of 58.6% after 20 years of disease duration), leukopenia, progressive reduction of absolute lymphocyte count and a T- and B-cell profile similar to autoimmune disorders like Sjogren syndrome, rheumatoid arthritis, and systemic lupus erythematosus (increased HLADR+ and CD3 + TCRγδ cells, reduced T regulatory cells, increased double-negative B and a tendency to reduced B memory cells). In a minority of patients, P/LP variants related to primary immuno-regulatory disorders were found. This new form may fit the group of "Likely acquired neutropenia," a provisional category included in the recent International Guidelines on Diagnosis and Management of Neutropenia of EHA and EUNET INNOCHRON ACTION 18233. The early recognition of this form of neutropenia would help clinicians to delineate better specific monitoring plans, genetic counseling, and potentially targeted therapies.

Short-term cardiovascular effects of salmeterol.

The occurrence of cardiovascular side effects is sometimes associated with the utilization of beta-adrenoceptor agonists. The most important causes of these undesirable pharmacologic actions are as follows: (1) direct stimulation of cardiac beta-adrenoceptors; (2) reflex activation of adrenergic mechanisms due to peripheral vasodilation; (3) hypokalemia; and (4) hypoxemia. The aim of this study was to evaluate the potential short-term, cardiovascular side effects of salmeterol, a long-acting and highly selective beta2-adrenoceptor agonist. Eight volunteer healthy subjects and eight patients with reversible airway obstruction and without cardiovascular alterations were treated with 50 microg of salmeterol twice a day for 3 days and then with 100 microg of salmeterol twice a day for a further 3-day period. The 24-h ECG (Holter) monitoring and measurement of arterial BP, performed on the admission day and on the third and the sixth day of pharmacologic treatment, showed that salmeterol did not produce any significant change in mean heart rate, number of supraventricular and ventricular premature complexes, and BP. Furthermore, no ECG abnormality related to myocardial ischemia was recorded during 24-h Holter monitoring. These data suggest that salmeterol, administered in regular and high doses for a short period, does not cause significant cardiovascular effects in both normal subjects and patients with reversible airway obstruction.

[Quality of life after an episode of myocardial infarct]. / La qualità della vita nel paziente reduce da un episodio infartuale del miocardio.

We studied the quality of life of two groups of myocardial infarction patients (AMI) and a control group of normal subjects: 1) 100 post-AMI patients following a cardiac rehabilitation programme of 12 weeks (CRG); 2) 33 post-AMI patients following a conventional care by cardiologists (CCG); 3) 40 healthy subjects as control (CG). To determine the loss of quality of life we administered the questionnaire of De Velasco-Del Barrio. The use of the questionnaire was possible by authors permission. MI patients had significantly poorer quality of life than the healthy subjects (p < 0.0001). Cardiac rehabilitation improved quality of life (p < 0.001) more than the conventional care. Women reported poorer quality of life that men. Before and after rehabilitation programme the quality of life of the subjects age 50 years or more was worse than their younger counterparts. Invasive therapies such percutaneous transluminal coronary angioplasty and coronary artery bypass grafting are associated with an improvement of post-operative quality of life. In order to study the influence of social class on quality of life we divided our subjects into three groupings: high, medium and low according to code representing jobs and professional: a significant difference was found between high and low social class (p < 0.03). These findings suggest that cardiac rehabilitation may have positive effects on the quality of life in the MI patients. Aged patients, women, and with low social position reported poorer quality of life that younger, men and high social position patients. It seems that these patients should be assessed with psycho-social and treated with psychological and social support for improvement their quality of life.

Combined mitral valve prolapse and atrial septal aneurysm. A cause for embolic events.

The "association" atrial septal aneurysm and mitral valve prolapse is often asymptomatic with an incidence of about 1% in autopsied adults. Occasionally, however, embolic phenomena can occur. The two-dimensional echocardiography allows an accurate diagnosis of these two cardiac lesions when present. In this paper the Authors report on three patients affected by mitral valve prolapse associated with atrial septal aneurysm, one of whom complicated by a cerebral embolism. Owing to the significant incidence of peripheral or cerebral vascular embolic events in this group of patients, the Authors conclude that long-term anticoagulation therapy is mandatory in all cases, whereas a surgical repair could be advisable when a previous embolism or interatrial shunting is demonstrated.