[The clinical significance of signal-averaged electrocardiography in hypertrophic cardiomyopathy]. / Significato clinico dell'elettrocardiogramma mediato di superficie nella cardiomiopatia ipertrofica.

We examined 44 patients (pts) with hypertrophic cardiomyopathy to evaluate the prognostic value of signal-averaged electrocardiography and its possible correlations with clinical and instrumental data. All pts (31 male, 13 female, mean age 47 +/- 15) underwent clinical examination, standard electrocardiography, M-mode and two-dimensional echocardiography, 24-72 hour dynamic electrocardiography and signal-averaged electrocardiography. The mean follow-up was 14 +/- 4 months. Signal-averaged electrocardiography was performed using a 40-250 Hz bidirectional filter. An abnormal signal-averaged electrocardiography with late potentials (filtered QRS duration greater than or equal to 120 msec and root mean square voltage in terminal 40 msec less than or equal to 20 microvolts) was detected in 5 pts (group A, 11%) while 39 pts (group B, 89%) had a normal signal-averaged electrocardiography. Ventricular tachycardia runs at dynamic ECG were present in 2 pts in group A (40%), and in 8 in group B (21%, p = NS). No statistical differences were found between the two groups for any clinical or instrumental data. During our study, one group A patient died suddenly. In detecting subjects with ventricular tachycardia runs, signal-averaged electrocardiography sensitivity was 20%, and specificity was 91%. High specificity suggests that signal-averaged electrocardiography might be used to detect pts at a lower risk for ventricular tachycardia. Further investigations are required to evaluate the predictive value of signal-averaged electrocardiography for sudden death in hypertrophic cardiomyopathy.

[Myocardial perfusion in coronary disease: thallium-201 planar scintigraphy]. / La perfusione miocardica nella cardiopatia coronarica: scintigrafia planare con tallio-201.

This research evaluated the diagnostic and analytic effectiveness of 201-thallium imaging in a group of patients with coronary artery disease, each one of them with different localization, extent and number of coronary vessel obstructions. The thallium-201 imaging was performed immediately after ergometric test and repeated 4 hours later. In a large percentage of patients thallium-201 imaging of reversible and irreversible perfusion defects have been found; thallium-201 scintigraphy showed a greater sensitivity than exercise ECG. With particular evidence 201-Tl imaging showed perfusion defects in a high percentage of patients with obstruction of one and two coronary vessels; in these patients, in the former especially, the results of exercise ECGs showed a lower sensitivity. Both thallium-201 and exercise ECG had a similar sensitivity in patients with obstruction of three coronary vessels. In patients with obstruction of one or two vessels, thallium-201 imaging clearly showed a regional hypoperfusion corresponding to the specific perfusion coronary vessels, while exercise ECG gave aspecific topographic results. Irreversible perfusion defects resulted corresponding to the myocardial segments that were infarcted, but were also observed in patients without previous myocardial infarction.

Aspergillosis and atopy in cystic fibrosis.

Forty-six patients with cystic fibrosis (CF) were studied for colonization and sensitization by Aspergillus organisms. The fungus was cultured from 21 of 37 (57%) patients who produced sputum. The non-CF sputum isolation rate was 6.5%. Sputum hyphae were seen in 95% of patients with positive cultures. Sputum eosinophilia accompanied colonization by Aspergillus. Aspergillus precipitins were found in 37% of CF patients; positive immediate skin tests were found in 39%. "Atopy" defined by skin test criteria was found in 46%. All of these findings were more common in patients with more severe disease (p less than 0.05). CF patients had predominant mold sensitivity in contrast to predominant pollen sensitivity in a control group of asthmatic subjects. Serum IgE concentrations were increased in 22% of CF patients. CF patients who had increased serum concentrations of IgE were more likely to have a family history of atopy. Wheezing was more common in those who were atopic, especially the group with predominant mold sensitivity who had more severe disease. Four patients fulfilled the diagnostic criteria for allergic bronchopulmonary aspergillosis (ABPA) during the time of study and follow-up. A fifth patient had ABPA diagnosed before the study. The incidence of ABPA during a 2-yr period in our CF population was 11%.

Perivascular hemosiderin deposition in muscle in Waldenström's macroglobulinemia.

A 63-year old woman with Waldenström's macroglobulinemina of 5 years' duration developed disabling pain in her extremities. The patient had required frequent plasmapheresis and transfusion for treatment of hypervisosity. Muscle biopsy from 2 separate sites showed striking deposition of hemosiderin in macrophages surrounding small vessels and features of slight denervation. Immunofluorescence demonstrated IgM deposition in vessel walls. Perivascular hemosiderin deposition in muscle has not previously been desicribed and may reflect traansfusion-hemosiderosis in a patient with a dysglobulinemia.

Systemic lupus erythematosus in a 1-year-old child.

An 11-month-old girl had systemic lupus erythematosus with a widespread skin rash. She manifested five of the criteria necessary for the diagnosis: oral ulcerations, alopecia, positive lupus erythematosus preparations, convulsions, and thrombocytopenia with leukopenia. Direct immunofluorescence showed deposits of IgG and C3 at the dermoepidermal junction of both abnormal and clinically uninvolved skin, and in subepithelial deposits along the glomerular basement membrane.