Evaluation of myocardial involvement in systemic lupus erythematosus by signal-averaged electrocardiography and echocardiography.

OBJECTIVE: The myocardial involvement in systemic lupus erythematosus (SLE) patients, frequently found at autopsy or at endomyocardial biopsy, is less easily detected clinically. The myocardial lesions are characterized by an increase in interstitial connective tissue and myocardial scarring. Signal-averaged electrocardiography (ECG-SA) is currently used for recording ventricular late potentials which are the expression of slowed and disorganized conduction through zones of myocardial scarring. M-mode, two-dimensional and Doppler echocardiography (ECHO) represent relatively simple methods for evaluating the left ventricular function. This study was aimed to evaluate by ECG-SA and ECHO the myocardial involvement of SLE patients without clinical and electrocardiographic evidence of cardiac disease. METHODS AND RESULTS: Twenty outpatients with SLE were studied and compared with 18 normal controls. Late potentials were recorded in 20% of SLE patients and in 5.5% of controls. A significant increase of abnormal left ventricular diastolic filling was found in the SLE patients, characterized by reduced E/A (p = 0.018), a lower deceleration rate of early diastolic flow velocity (p = 0.048) and a prolonged isovolumic relaxation time (p = 0.001). SLE patients had diastolic dysfunction of various degrees although the depolarization abnormalities detected by ECG-SA were found only in a few subjects. CONCLUSIONS: The depolarization abnormalities, revealed by ECG-SA, probably reflect a longer extent of myocardial fibrosis in SLE patients with ECHO evidence of abnormal left ventricular filling. The simultaneous occurrence of ECHO and ECG-SA alterations could be a marker of subclinical myocardial involvement.

Diastolic function abnormalities in rheumatoid arthritis. Evaluation By echo Doppler transmitral flow and pulmonary venous flow: relation with duration of disease.

OBJECTIVE: The aim of this study was to evaluate left ventricular filling in patients with rheumatoid arthritis (RA), analysing transmitral flow and pulmonary venous flow, with special regard to age and disease duration. METHODS: 32 patients affected by RA according to ARA criteria were selected, without evidence of cardiac disease, and compared with matched control subjects. All patients and the control group were submitted to M-mode, two dimensional, Doppler and colour Doppler (continuous and pulsed wave) echocardiography. The following diastolic parameters were evaluated: transmitralic flow (E/A ratio), pulmonary venous flow (S/D ratio), a-Pw, IVRT and DT. RESULTS: In RA patients left ventricular filling abnormalities were found characterised by a reduced E/A ratio (mean (SD) 1.16 (0.31) v. controls 1.37 (0.32); p = 0.02) and an increased S/D ratio (1.43 (0.40) v. controls 1.22 (0.29); p = 0.017). In the group of patients a relation was found between E/A ratio and disease duration (r= 0.40, p = 0.01 Spearman rank correlation). CONCLUSIONS: At present, it is concluded that RA patients, in absence of clinical evidence of heart disease, show diastolic dysfunction characterised by impaired E/A and S/D ratio. The relation between transmitral flow alteration and disease duration suggests a sub-clinical myocardial involvement.

[The usefulness of plasmapheresis in a case of nephrotic syndrome in Berger's disease]. / Utilità della plasmaferesi in un caso di sindrome nefrosica in corso di malattia di Berger.

Berger's disease, or IgA mesangial nephropathy, is a frequent form of focal and/or segmental proliferative glomerulonephritis that occasionally may present as nephrotic syndrome. The authors reports a clinical case of a young woman come to their observation with a severe clinical picture characterized by asthenia, anasarca, serious no selective proteinuria, microscopic hematuria, blood hypotension from mesangial proliferative glomerulonephritis IgA. Because of the null response to traditional therapy the patient was submitted to plasmapheresis "cascade model", or double filtration, a certainly experimental treatment for this disease, and a remission of the nephrotic syndrome was obtained as confirmed by follow-up at three, six months and one year. Since at present times the therapy is supportive only and no therapeutic maneuvers have been found to be consistently effective in the Berger's disease, plasma exchange plus immunodepressive therapy seems to be useful particularly in the rare patients with rapidly progressive glomerulonephritis. Further and more extensive studies and a fair follow-up are necessary to prove our results.

[Arrhythmias in the aged: prevalence and correlation with symptoms]. / Le aritmie nell'anziano: prevalenza e correlazione con la sintomatologia.

Objective of our study was to evaluate the prevalence of arrhythmias as well as their correlation with the reported anamnestic symptomatology in the elderly population. With 24 hour ambulatory electrocardiography (Holter ECG) 913 patients, (440 males and 473 females, range 60-89 years, mean age 71) were consecutively studied and subdivided according to the following criteria: (1) age (3 classes: 60-69 [I], 70-79 [II], > or = 80 [III]; (2) presence/absence of "guide" symptoms (syncope/faintness, chest pain, palpitation), and (3) Holter electrocardiography results. We have demonstrated a high prevalence of arrhythmias: 72% (657), which was significantly higher in age classes II (80.5%) and III (79.1%) in comparison to class I (60.6%). A notably higher prevalence of tachyarrhythmias is documented compared to bradyarrhythmias (5:1). With a higher age the prevalence of supraventricular tachyarrhythmias increases significantly, while bradyarrhythmias do not have the same trend. We have a similar prevalence of arrhythmias between symptomatic (77.4%) and asymptomatic patients (63.6%) and no significant correlation between anamnestic symptoms and presence of arrhythmias are observed. Considering the high prevalence of arrhythmias during Holter ECG we think that the clinical importance and prognosis should be evaluated with caution.

Signal-averaged electrocardiography and echocardiography in the evaluation of myocardial involvement in progressive systemic sclerosis.

To assess the myocardial involvement in progressive systemic sclerosis we evaluated the presence of late potentials by signal-averaged electrocardiography (signal-averaged ECG) and the left ventricular function by M-mode, two dimensional and Doppler echocardiography. Fifteen outpatients, 7 with diffuse progressive systemic sclerosis and 8 with CREST syndrome variant, without clinical or electrocardiographic evidence of cardiac disease were studied and compared with 18 normal subjects. Late potentials occurred in 5 out of 15 progressive systemic sclerosis patients (33%) with a significant difference versus controls (P < 0.05) and were present only in the patients with diffuse progressive systemic sclerosis (P < or = 0.001 vs. controls). All progressive systemic sclerosis patients showed a normal left ventricular systolic function. Abnormal left ventricular filling was found in 9 progressive systemic sclerosis patients (5 with diffuse progressive systemic sclerosis and 4 with CREST). A more severe impairment of the mean values of diastolic function indexes was found in diffuse progressive systemic sclerosis than in CREST. In all diffuse progressive systemic sclerosis patients at least one method showed altered results, whereas half the CREST patients showed no pathological findings with both techniques. These results confirm a lower myocardial involvement in the CREST syndrome than in diffuse progressive systemic sclerosis and consequently this is probably related to a better prognosis.

[A case of non-Hodgkin T-cell lymphoma with predominantly cutaneous manifestations]. / Su di un caso di linfoma non-Hodgkin a cellule T con manifestazioni prevalentemente cutanee.

There is a wealth of experiences concerning cancer and leukemia induced in human populations by radiation. The contribution of the nuclear industry to the radiation exposure of the general population is small, but there is the risk of catastrophic accidents causing a large number of deaths. The authors describe the case of a 48 year old black man accidentally exposed to the effects of radiations during the nuclear disaster of Chernobyl in 1986. The patient showed, many years later, a predominantly cutaneous high-grade T cell lymphoma, which was refractory to traditional treatments but sensitive to high doses of a recombinant interferon. Unluckily the patient died, six months later, because of autoinfection overwhelming. The goal of the authors is again to ask to reflect on the risk of the use of nuclear power and to debate the possible choice of the still experimental treatments.

[Calcium antagonists in the therapy of arterial hypertension in the aged]. / Trattamento con calcio-antagonisti dell'ipertensione arteriosa nell'anziano.

A large number of epidemiologic and interventional studies go to show that acute cerebral and cardiac events and chronic organ injury are significantly less frequent in treated compared to untreated elderly hypertensives. These observations clearly show that hypertension in the elderly must be treated. As to water-electrolyte metabolism, activity of the renin-angiotensin-aldosterone axis as well as volemia are reduced in the elderly while extravascular liquid compartment and activity of the natriuretic atrial factor are both increased. In addition, there is reduction of the baroceptor reflexes accompanied by vasoconstriction and hypertrophy of the smooth muscles associated with increased intracellular calcium ion content. These pathophysiologic conditions appear to supply the rationale for the preference accorded to calcium channel blockers compared to other drugs usually employed in the management of younger hypertensives. Moreover, second generation dihydropyridines, thanks to their liposolubility and ties with calcium channel subunits, show rapid onset and long action that allows for a single daily dose and lowers the incidence of side effects. A large number of interventional trials employing calcium channel blockers have demonstrated the efficacy of these drugs for reducing arterial hypertension. However, these trials concern young hypertensives whereas there is a lack of similar studies in elderly subjects, were clinical trials are few and involve limited numbers of subjects. Results of multicentre studies, at present in progress, will permit to assess the efficacy and incidence of side effects of second generation calcium channel blockers in hypertension of the elderly.

[The prevalence of arrhythmias in subjects over 80 years old. The prognostic role of anamnestic symptomatology and the therapeutic directions]. / Prevalenza dei disturbi del ritmo in soggetti di oltre 80 anni. Ruolo predittivo della sintomatologia anamnestica e orientamenti terapeutici.

24-hour Holter monitoring was performed in 129 unselected subjects older than 80 years (69 women and 60 men) in order to evaluate arrhythmia incidence and to establish significance of symptoms; 56% patients had lipothymia and/or syncope, chest pain and palpitations, 44% were asymptomatic. All patients showed high incidence of hyperkinetic and hypokinetic arrhythmias; no significant differences between symptomatic and asymptomatic patients in arrhythmia incidence were seen. Moreover, no correlation could be seen of the symptoms reported by the patient to the incidence of any particular type of arrhythmia. In conclusion, these preliminary data suggest a criticism of the opportunity of indiscriminate and long term antiarrhythmic therapy. Nevertheless, Holter monitoring is useful in detecting hypokinetic arrhythmias of asymptomatic patients in whom pacemaker implantation is essential and curative.

High density lipoprotein cholesterol low serum level as the only risk factor in male patients with coronary heart disease.

Forty-three male patients with myocardial infarction, severe angiographic coronary lesions, high or normal low density lipoprotein (LDL) cholesterol serum levels, and without other risk factors for coronary heart disease were selected. In all patients high density lipoprotein (HDL) cholesterol and HDL-cholesterol/total cholesterol ratio were significantly lower than in the control group; in particular, the ratio was below 0.240 which was the median value of normal subjects. Six patients with total cholesterol and LDL-cholesterol below 5.16 and 3.35 mmol/l respectively, had low serum levels of HDL-cholesterol; in 4 of them the value of this risk factor was below 0.9 mmol/l; 2 of 6 patients had a lipoprotein (a) serum concentration above 0.3 g/l but not a premature myocardial infarction or a clinical history of coronary heart disease. Our data confirm that HDL-cholesterol/total cholesterol ratio could be a better marker of coronary heart disease than HDL-cholesterol, total cholesterol or LDL-cholesterol and suggest the importance to check HDL-cholesterol serum levels also in subjects without risk factors for atherosclerotic disease.

[The efficacy of i.v. immunoglobulins at high doses in a case of thrombocytopenia with an immune pathogenesis in chronic lymphocytic leukemia]. / Efficacia delle immunoglobuline e.v. ad alte dosi in un caso di piastrinopenia a patogenesi immunitaria in corso di leucemia linfatica cronica.

The authors report a case of chronic lymphocytic leukemia with thrombocytopenia caused by antiplatelet antibodies. The customary treatments of this condition having proved ineffective, therapy with large I.V. doses of Ig (0.4 g/kg body weight daily for five consecutive days) was started, which is believed to increase platelet count although the mechanism of this action is not yet fully understood. Interesting changes in laboratory parameters were observed, especially those concerning electroimmunophoresis and immunofixation and these might be useful for a better understanding of the various hypotheses that have been put forward to account for the mechanism(s) of action possibly at work. Treatment proved effective wherefore the authors hope that further studies will be carried out in order to increase our knowledge of the modulation and regulation of the immune response, a primary element in the management of these disorders which is at present only experimental.

[Amebic liver abscess: clinical and therapeutic considerations]. / Ascesso amebico epatico. Considerazioni cliniche e terapeutiche.

The authors report the case of a young adult with an amebic liver abscess. The primary infection localized in the gut occurred during a vacation in Africa. This disorder is fairly uncommon in western countries which is why the authors considered it worthwhile to draw practitioners' attention to it, stressing that it should be taken into account in differential diagnosis of fever of unknown origin in young adults who have travelled to regions where amebiasis is endemic. In addition, the diagnostic procedures used and especially the treatment applied are briefly set out. Medical therapy is almost always successful but if significant improvement is not achieved within a reasonable lapse of time and if complications are suspected, all possible therapeutic measures, including surgery, must be applied.

[Considerations on a case of hemolytic microangiopathic anemia in a patient with adenocarcinoma of the gallbladder]. / Considerazioni su di un caso di anemia emolitica microangiopatica in soggetto con adenocarcinoma della colecisti.

The authors report a case of hemolytic microangiopathic anemia (HMA) in a patient with gallbladder carcinoma with metastases to the liver, lymph nodes of the hepatic hilum, omentum, iliac bone and its bone marrow. After an overview of the literature on HMA and particularly in the peculiar mechanism of the small vessel damage, the clinical and laboratory picture is described. It is also underlined that this is the first case described in which the association of gallbladder adenocarcinoma and HMA is present. The progressive and fatal course of the disease is tied to the severe erythrocytic lysis and mechanical damage by the massive microembolization of small vessel walls by neoplastic cells. This also explains the complete uselessness of blood-transfusion therapy.

[Congenital thyroid abnormality (subhyoid ectopy) associated with mitral valve prolapse]. / Anomalia congenita della tiroide (ectopia sottoioidea) associata a prolasso della mitrale.

By echocardiographic technique the Authors have studied a rare congenital anomaly: "sub-hyoid ectopy of the thyroid gland". The patient, a 35 years unmarried woman, with manifest clinic symptoms of hypothyroidism, was affected with mesotelesystolic prolapse of the posterior leaflet of the mitral valve. It has also been possible to put in evidence the findings of mixoedematous cardiopathy and its resolution after thyroid opotherapy.