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The case of a child with beta-thalassaemia major who developed a massive haemorrhagic pericardial effusion is reported and in whom the clinical picture completely resolved after pericardiocentesis. Possible causes are discussed and the role of echocardiography in the follow-up of thalassaemic patients is emphasized. [ABSTRACT FROM PUBLISHER]

We report a 2-month-old child with infantile myoclonic seizures, who developed congestive heart failure secondary to hypertrophic cardiomyopathy while receiving adrenocorticotropic hormone (ACTH) therapy. Treatment with propranolol and withdrawal of ACTH led to the resolution of cardiac hypertrophy as determined by two-dimensional echocardiography. Possible links between ACTH therapy and the development of hypertrophic cardiomyopathy are examined. Our report confirms that a careful monitoring is required to detect cardiac abnormalities during ACTH administration.

Angiodysplasia of the gastrointestinal tract is a disorder consisting of ectasia of submucosal and mucosal vessels, which causes acute and chronic bleeding. We describe the case of a 58-year-old man with a history of recurrent lower intestinal bleeding and severe anemia. Endoscopy and X-ray examination of the gastrointestinal tract failed to show the source of bleeding. Selective angiography of the superior and inferior mesenteric arteries revealed a vascular ectasia. The patient underwent ileo-cecal resection with remarkable improvement after surgery.

Chronic lymphoproliferative disorders are extremely heterogeneous from a clinical, morphological and immunological point of view. The paper reports the case of a woman with chronic prolymphocytic leukemia. The neoplastic clone revealed the typical immunological features of pre-B lymphocytes, which are at an earlier stage of differentiation in respect to prolymphocytes. After polychemotherapy (CEOP) the phenotype of the neoplastic cells shifted to an immunological pattern typical of B lymphocytes.

The influence of loading conditions on mitral flow velocity profile was studies by pulsed wave Doppler echocardiography in 10 normal subjects during diving test (5 min face exposure to iced water). The cold stimulus increased blood pressure (p less than 0.001), peripheral resistances (p less than 0.001), peak-systolic and end-systolic left ventricular (LV) meridional wall stresses (p less than 0.005). No significant change was found in heart rate. LV end-diastolic volume was unchanged, while LV end-systolic volume increased (p less than 0.005). Consequently, LV fractional shortening decreased (p less than 0.001). Thus, in spite of enhanced LV contractility (assessed by a significant increase in LV end-systolic stress to volume ratio, p less than 0.02) LV pump performance fell, because of prevailing afterload as well as lack of acute recruitment of LV preload reserve (afterload-contractility mismatch). Such an increase in LV afterload was associated with remarkable changes in LV diastolic dynamics: isovolumic relaxation time (IRT) was prolonged (p less than 0.001) and ventricular filling was relatively displaced towards late diastole (peak early to peak atrial velocities ratio, pE/pA: 1.5 +/- 0.3 vs 1.9 +/- 0.3 p less than 0.001; peak atrial filling rate: 326 +/- 47 vs 242 +/- 38 ml/s; p less than 0.001; normalized late diastolic velocity: 0.87 +/- 0.10 vs 0.73 +/- 0.06; p less than 0.01). Also, IRT values significantly correlated with pE/pA values (r = -0.75; p less than 0.001). In conclusion, diving test produced important changes in LV hemodynamics which, in turn, influenced LV relaxation and filling pattern. Our results indicate that operative LV loading conditions should be considered when assessing diastolic function by the analysis of transmitral Doppler flow profile.

A case of POEMS syndrome in a 43-year-old male with polyneuropathy, osteolytic lesion of the basin due to solitary IgA-lambda plasmacytoma, cutaneous scleroderma-like changes, diffuse lymphadenopathy and hepatosplenomegaly is described. Liver biopsy showed a regenerative process of the parenchyma without laboratory and histologic evidence of necrosis. A peculiar finding was the onset of a right hemiparesis in the absence of signs of vascular disease or other predisposing factors. The possible links between the peculiar elements of the POEMS syndrome are briefly analyzed and discussed.

Ketanserin, an antagonist of 5-HT2-serotonergic and alpha 1-adrenergic receptors, has come into use for the therapy of mild to moderate arterial hypertension. Quite recent observations have shown changes in transmembrane sodium (Na) transport after the acute administration of high doses of this drug to normal subjects. It is well known that some of these transport systems have an altered activity in essential hypertension. We evaluated the effects of long-term (3 months) administration of ketanserin (40-80 mg/day) on Na and potassium (K) intracellular concentrations and transmembrane fluxes in red blood cells (RBCs) from 12 essential hypertensive patients. In addition the present study describes the in vitro effects of two different concentrations of the drug (5 x 10(-8) and 5 x 10(-7) M) on erythrocytes in normal subjects. In the first study, both systolic and diastolic blood pressure were significantly lowered by the treatment with ketanserin (from 165/103 to 143/89; p less than 0.001). Na and K intraerythrocyte concentrations fell markedly during ketanserin administration (both p less than 0.001). A marked decrease in Na,K-pump activity (p less than 0.001) and an increase in Na,lithium(Li)-countertransport function (p less than 0.001) were observed. Na outward cotransport, Na leak, and K leak were not modified by the therapy. Direct correlation was found between the fall in mean blood pressure and in Na and K intraerythrocyte concentration (respectively, p less than 0.01 and p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Changes in transmembrane sodium fluxes have been reported in normotensive and in hypertensive subjects after ketanserin administration. In this study, the effects of the serotonergic system on transmembrane sodium transport mechanisms have been investigated in vitro. In erythrocytes drawn from ten healthy subjects, we studied the effects of serotonin (5HT) on the Na/K pump, Na/K cotransport, Na/Li countertransport, and passive permeability of Na. No significant changes were found. A direct, non-receptor-mediated action of ketanserin was then suspected, and the effects of two concentrations of ketanserin (5 x 10(-8) and 5 x 10(-7) M) were evaluated in erythrocytes from 12 normal volunteers. Both concentrations of ketanserin significantly decreased the activity of the Na/K pump and increased the activity of Na/Li countertransport. Na/K cotransport and passive permeability were not affected. Indirect evidence of the action of ketanserin on sodium transmembrane fluxes came from other experiments. In the red blood cells taken from five normal subjects and incubated for 2 hours in a plasma pool, we evaluated the changes in intracellular sodium concentration induced by the presence of 5HT or ketanserin. A significant decrease in intracellular sodium concentration occurred only with ketanserin. This study indicates that ketanserin has a direct influence on transmembrane sodium fluxes. If this action were also present in other cells, it might contribute to the actions of the drug at vascular, nervous, and renal tubular levels.

The mean circumferential contraction rate of the myocardial fiber cell (Mvcf) and the ejection fraction (EF) are echocardiographic parameters commonly employed in the assessment of left ventricular performance. In cases where septal asynchonism due to fibrosis or bundle-branch block make their determination impossible, however, one is forced to use parameters obtained from movement of the posterior wall. Investigation of normal subjects and patients with idiopathic hypertrophic subaortic stenosis showed that the "normalized" systolic velocity of the posterior wall (Vpws) and the non-normalized mean velocity (PWV) closely fitted Mvcf and that Vpws offered a good fit with EF. It is suggested that a non-invasive way of obtaining sufficiently reliable data for the evaluation of cardiac performance is offered by these two parameters.

The standardized mean protodiastolic speed of the posterior wall (Vpwd), non standardized speed (DEV) and maximum speed (DEVM) obtained with ultrasounds, are capable of distinguishing subjects with hypertrophic subaortic stenosis (IHSS), in whom there is a diminution in compliance, from normal subjects. As this compliance diminution is liable to limit fibrocell lengthening to the extent of its distension speed, the Vpwd has been used in the plotting of ventricular function curves in replacement of telediastolic volume (as an expression of fibre lengthening). An excellent correlation was found between Vpwd and mean speed of circumferential shortening of the fibre (Mvcf) and the ejection fraction (EF) used as indices of cardiac work. The comparison shows that the two groups lie on the same curve of ventricular function and that subjects with IHSS operate low down owing to the brevity of their sarcomeres. The Vpwd can thus be utilized as an indirect index of fibre length and in the plotting of left ventricular function curves.

Telediastolic pressure and volume can readily be employed as expressions of sarcomere length when constructing ventricular performance curves for the normal heart. In pathological hearts, however, these parameters are rendered inadequate as a result of altered compliance, which means that diastolic lengthening of the muscle fibre cell must be determined from telediastolic stress and linear compliance, corrected for wall thickness (muscle fibre stretch, strain index). Ventricular performance curves were constructed for normal subjects and patients with hypertrophic subaortic stenosis, using the normalised mean rate of circumferential lengthening of the fibre as the expression of fiber strength. Comparison was made with the mean circumferential shortening rate and the ejection fraction as the expression of ventricular effort. It was found that both normal subjects and stenosis patients lay on the same curve, the latter acting low down through having short sarcomeres.

Both mitral valve closing speed (MVV) and the mean standardized circumferential lengthening of the myocardial fibrocell (MVCL), between which a good correlation exists, may be used as indirect indices of ventricular compliance. These two indices have been compared with those obtained echocardiographically from the movement of the posterior wall of the ventricle in a group of normal subjects and in a group suffering from hypertrophic subaortic stenosis (IHSS). The comparison between MVCL and mean standardized speed of protodiastolic prolapse of the posterior wall (Wpwd) was fairly significant (P=0.01); that between MVCL and mean speed of protodiastolic prolapse of the posterior wall (DEV) was only slightly significant (P=0.05) or was not significant at all; that between MVCL and the maximum speed of protodiastolic release (DEVM) was slightly significant (P=0.05). Although the correlation between MVV and the various indices obtained from the movement of the posterior wall (DEV, DEVM, Vpwd) were quite significant (P=0.01), it is considered that Vpwd is preferable as an indirect index of ventricular compliance. As it is capable of distinguishing subjects with IHSS from normal subjects inthose cases in which the movement of valvular peaks, modified or impeded by inflammatory or fibrotic processes does not a-low MVV to be measured, we think Vpwd can be usefully employed as it can in all those circumstances in which it is impossible to measure MVCL owing to alterations to normal septal synchronism (infarction and fibrosis of the septum, branch blocks, etc.).

Thirty-seven patients with severe hypertension were randomly assigned to receive 20 mg of ketanserin sublingually, 10 mg of ketanserin intravenously, or 20 mg of nifedipine sublingually. Systolic and diastolic blood pressures fell significantly after the three treatments. The maximum effects were reached 25 minutes after sublingual ketanserin (with decreases of 7.7% in systolic and 7.1% in diastolic blood pressure), six minutes after intravenous ketanserin (decreases of 9.4% and 9.6%, respectively), and 25 minutes after sublingual nifedipine (decreases of 16.9% and 15.9%, respectively). Blood pressure returned to pretreatment levels 20 minutes after intravenous ketanserin. Heart rate increased significantly in the group receiving nifedipine. No changes in plasma aldosterone, sodium, or potassium levels or in erythrocyte sodium and potassium levels were found after ketanserin. It is concluded that even intravenous ketanserin is inferior to sublingual nifedipine in the control of blood pressure.

The following parameters obtained by echocardiography have been measured in a group of normal subjects and in a group suffering from hypertrophic subaortic stenosis (IHSS): 1) speed of mitral valve protodiastolic closing (MVV); 2) circumferential lengthening speed of the fibre (MVCL) both standardized and otherwise. There is a good correlation between MVCL and MVV (P = 0.01 and 0.05). Since a low mitral valve closing rate is an indirect index for measuring ventricular compliance, the use of the MVCL can be considered valid for the same purposes. Using this it has in fact been possible to distinguish subjects with IHSS in which lower MVV is an index of reduced compliance, from normal subjects.