Protein S deficiency. Description of a case associated with chronic inflammatory bowel disease.

Thrombotic disease is one of the most relevant clinical problems for morbility and mortality. We can differentiate congenital and acquired forms. In this short communication we describe 1 case observed by us that seems interesting for the association of a congenital and acquired form [Protein S deficiency and inflammatory bowel disease (IBD)] and for the dramatic events suffered before receiving a complete diagnosis and therapy, indicating the importance of recollection of information from the patients, starting from anamnestic data.

Value of pharmacologic stress echocardiography in risk stratification of patients with single-vessel disease: a report from the Echo-Persantine and Echo-Dobutamine International Cooperative Studies.

OBJECTIVES: This study sought to verify the effectiveness of pharmacologic stress echocardiography in risk stratification of patients with single-vessel disease. BACKGROUND: Noninvasive prognostic assessment of single-vessel disease is an unresolved issue to date. METHODS: The study evaluated prospectively collected data from 754 patients with angiographic single-vessel disease who underwent either dipyridamole (n = 576) or dobutamine (n = 178) stress echocardiography. Invasive treatment (coronary revascularization within 3 months of stress testing) was performed in 260 patients and medical treatment in 494. RESULTS: Echocardiographic positivity was observed in 421 patients (56%). Patients treated invasively had a higher incidence of stress test positivity (69% vs. 49%, p < 0.001) and left anterior descending coronary artery involvement (60% vs. 46%, p < 0.001) than patients maintained with medical therapy. During a mean follow-up of 37 months, 54 hard cardiac events occurred (14 deaths, 40 nonfatal infarctions): 37 in medically and 17 in invasively treated patients (7.5% vs. 6.5%, p = NS). On Cox analysis, a positive result on stress testing was the only independent prognostic predictor in medically treated patients (relative risk 2.92, 95% confidence interval 1.29 to 6.59). The 4-year infarction-free survival rate was higher for a negative than a positive stress test result in medically (93.9% vs. 87.3%, p = 0.009) but not invasively treated patients (92.7% vs. 97.1%, p = 0.545). Moreover, a significantly higher 4-year infarction-free survival rate was found in invasively versus medically treated patients with a positive (p = 0.012), but not in those with a negative, stress test result (p = 0.853). CONCLUSIONS: Pharmacologic stress echocardiography is effective in risk stratification of single-vessel disease and can accurately discriminate patients in whom coronary revascularization can have the maximal beneficial effect. These findings have a potential favorable impact on the cost-effectiveness of invasive procedures.

Methotrexate, mitoxantrone, 5-fluorouracil and leucovorin in metastatic breast cancer patients.

We have evaluated the efficacy and toxicity of a chemotherapy consisting of methotrexate, mitoxantrone, 5-Fluorouracil and leucovorin in 21 advanced breast cancer patients. Among 20 evaluable patients, objective response was obtained in 6 patients (30%) with two complete responses, stable disease in 4 patients (20%), while 10 patients (50%) progressed. Median progression-free survival and survival were 10 and 15 months, respectively. The most frequently observed side-effects were myelosuppression and emesis; one patient, who had previously received doxorubicin at the maximum dose-limiting cardiac toxicity, died of congestive heart failure after the third cycle. This treatment is moderately effective for advanced breast cancer patients.

Quantitative ultrasonic analysis of myocardium in patients with thalassemia major and iron overload.

BACKGROUND: Patients with beta-thalassemia major present with severe anemia and need continuous transfusion therapy. The consequent iron overload leads to hemochromatosis. Initial cardiac dysfunction has been documented even in thalassemics without clinical manifestations of heart failure as well as by conventional echocardiographic-Doppler techniques. The purpose of this study was to assess the acoustic quantitative properties of myocardium in patients with iron overload. METHODS AND RESULTS: Thirty-eight patients with beta-thalassemia major, without clinical signs of cardiac failure, and 20 age- and sex-matched young controls were studied by echocardiography. An on-line analysis of the ultrasonic radiofrequency signal was performed to obtain quantitative operator-independent measurements of the integrated backscatter (IB) signal of the ventricular septum and the posterior wall. The integrated values of the radiofrequency signal were normalized for the pericardial interface and expressed in percent (IB%). Thalassemic patients had been receiving transfusion therapy for 16 +/- 5 years and had received 313 +/- 138 transfusion units; they all had received chelation treatment (desferroxiamine) for 9 +/- 2 years. Patients and controls showed comparable values of echocardiographically assessed percent fractional shortening (32 +/- 3% versus 36 +/- 4%, p = NS), whereas thalassemics showed higher values of left ventricular mass index (118 +/- 30 versus 98 +/- 15 g/m2, p < 0.05). The IB% values were higher in patients with thalassemia major than in controls for both septum (35 +/- 14% versus 21 +/- 6%, p < 0.001) and posterior wall (16 +/- 6% versus 11 +/- 3%, p < 0.001). In thalassemic patients, no significant correlation was found between the septum IB% value and hematological parameters, such as the total number of transfusions (r = 0.2, p = NS) or the mean ferritin value (r = 0.1, p = NS). No significant correlation was also found between the septum IB% value and the echocardiographically assessed left ventricular mass index (r = 0.2, p = NS). CONCLUSIONS: These data demonstrate that myocardial reflectivity is abnormally increased in patients with thalassemia major under transfusion treatment, probably due to myocardial iron deposits and/or secondary structural changes. These quantitatively assessed abnormalities in regional reflectivity can be detected when conventional echocardiographic parameters of systolic left ventricular function are undistinguishable from normal controls.

Two-dimensional and Doppler echocardiographic diagnosis of left ventricular pseudoaneurysm.

In an oligosymptomatic patient with recent acute anterior myocardial infarction, two-dimensional and Doppler echocardiography allowed us to detect, unmistakably, the presence of an apical pseudoaneurysm. Prompt and successful repair was made without performing left ventriculography. To our knowledge, this is the first case of pseudoaneurysm referred for surgery on the basis of echocardiographic findings.

[Clinical applicability of myocardial scintigraphy with gallium-67 in the study of dilated cardiomyopathy]. / Applicabilità clinica della scintigrafia miocardica con 67gallio nello studio delle cardiomiopatie dilatative.

Gallium-67-citrate imaging has been recently proposed, in addition to endocardial biopsy, to detect myocardial inflammation in idiopathic dilated cardiomyopathy (IDCM). In order to evaluate the clinical usefulness of this method, 33 patients (pts) suffering from IDCM, 24 pts with various other cardiac diseases (inflammatory etiology in 7), and 11 controls underwent Gallium-67 scintigraphy (anterior and 30 degrees left anterior oblique projections; acquisition at 48 and 72 hrs). In 31 pts repeated scintigraphic examinations (at least two) were obtained. Scans were interpreted by two independent observers. Positivity of scintigrams was based on three different criteria: 1) myocardial activity greater than that of the sternum; 2) presence of focal myocardial activity; 3) semiquantitative index (index = activity of sternum/myocardial activity). Significant differences, either in the percentage of positive scans or in the values of the semiquantitative index, were found between controls and all pts with cardiac diseases. However, no difference was observed when comparing pts with IDCM to pts with other cardiac diseases. Finally, among the pts with cardiac disorders other than IDCM, the qualitative and semiquantitative results of the 7 pts with inflammatory etiology of the disease were similar to those obtained in the remaining 17. Of those pts who underwent longitudinal study, about a third showed modified scintigraphic results later on. No significant difference in behaviour was observed between IDCM and other cardiac disorders. Therefore, we conclude that Gallium 67-citrate imaging does not appear to be a suitable method for the identification of pts. with IDCM. The usefulness of this technique should be tested in samples of pts should previously selected with other more specific methods.