[Accuracy of prostate biopsies to evaluate tumor location in prostate cancer]. / Fiabilité des biopsies prostatiques pour l'étude de la topographie tumorale dans le cancer de prostate.

INTRODUCTION: The therapeutic approach of prostate cancer depends mainly on pathological criteria obtained through prostate biopsy. The low accuracy of prostate biopsy for Gleason grade determination is well known but its accuracy for bilateral or multifocal tumor has not been evaluated. The goal of this study was to assess the concordance between prostate biopsy and whole prostate specimen obtained after radical prostatectomy especially for bilateral and/or multifocal tumor. METHODS: We retrospectively compared the pathological results of prostate biopsy cores to the prostate specimen in patients who underwent radical prostatectomy in our department between the 01/01/1999 and the 31/12/2008. The criteria analyzed were the Gleason score, tumor bilaterality or multifocality. The impact of the number of prostate biopsy cores was also analyzed. RESULTS: Two hundred and five complete histological records were studied. Regarding the Gleason score overall concordance was 55%. In 38%, prostate biopsies downgraded the Gleason score. This concordance decreased with tumor differentiation (90.6% for Gleason 6 vs. 31% for Gleason greater than 7). For the tumor bilaterality, 78% of cancers affected both lobes at the definitive specimen analysis while only 49% were bilateral at prostate biopsies, achieving a concordance of 61%. Multifocal disease was observed in 36% at definitive pathology analysis with low concordance with prostate biopsies (36%). The number of biopsies increased the concordance for the Gleason score (60 to 81% for Gleason 7 and from 28 to 50% for Gleason greater than 7) and tumor location (44 to 70%). CONCLUSION: Pathological criteria and tumor mapping obtained from prostate biopsies were not very reliable especially when the tumor was poorly differentiated. An increased number of prostate biopsy core improved the sensitivity and specificity for the Gleason score diagnostic and of the tumor mapping.

[Muscularis mucosae invasion: prognostic factor for intravesical BCG immunotherapy failure for T1 bladder carcinoma]. / Atteinte de la Muscularis Mucosae dans les tumeurs urothéliales T1 de vessie : facteur pronostique de progression après immunothérapie par BCG.

OBJECTIVES: To study the prognostic impact of muscularis mucosae (MM) invasion for pT1 bladder cancer treated by transurethral resection (TUR) and adjuvant Bacille Calmette-Guerin (BCG) intravesical immunotherapy. METHODS: Sixty-six patients treated by BCG intravesical instillations were substaged into pT1a and pT1b, regarding Muscularis Mucosae invasion. Tumor grade, associated carcinoma in situ (CIS), multifocality, tumoral size up to 3cm, BCG maintenance were noted. With a mean follow-up of 50.5±38 months, we studied recurrence, progression, overall and specific survival. Cox's model method was used for multivariate analysis. RESULTS: Tumor recurrence was observed in 30±7% and 43±10% (P=0.29) and tumor progression in 16.3±5% and 39±10% (P=0.04) for pT1a and pT1b. The rate of progression was higher (P=0.04) and progression free survival was decreased (P=0.04) for pT1b. Specific death rates were 11±5% and 21±9% (P=0.28), median overall survival was 80.9 [1.5-92] and 48.2 [12-93] months for pT1a and pT1b. Overall and specific survival weren't different between the two populations (P=0.38; P=0.3). Cystectomy rates were 2.3±2% and 30±9% for pT1a and PT1b (P=0.0006). For pT1a patients, recurrence (P=0.8) or progression rates (P=0.64) were no different regarding BCG maintenance immunotherapy but pT1b population had a better progression free survival with BCG maintenance than without (P=0.0051). Only CIS had prognostic value in multivariate analysis. CONCLUSIONS: Tumors with Muscularis Mucosae invasion have a higher risk of progression and BCG failure. Maintenance immunotherapy should be given to improve results with these patients.

[Urinary complications of one-stitch ureteroneocystostomy in renal transplantation]. / Complications urinaires après réimplantation urétérovésicale selon la technique du "one-stitch" en transplantation rénale.

PURPOSE: To assess urinary complications related to the "one-stitch" technique extravesical ureteroneocystostomy in renal transplantation, and evaluate the impact of such complications on kidney graft and patient survival. PATIENTS AND METHODS: A single-institution, retrospective study was performed on 202 renal transplant recipients, from January 2004 to December 2008. Two combined kidney and liver transplantations were excluded. The "one-stitch" extravesical ureteroneocystostomy technique, fast and easy to perform, was systematically used. The evaluated urinary complications were urinary fistula, ureteral stenosis, symptomatic ureteral reflux, stone formation and complicated hematuria. We tried to point out factors impacting urinary complications occurrence and studied grafts and patients survival according to the existence of urinary complications. RESULTS: Fifty-five patients presented urinary complications (27.5%). The most frequent urinary complications were complicated hematuria (36 over 200, 18%), ureteral stenosis (15 over 200, 7.5%). Few cases of stone disease (one over 200, 0.5%), urinary fistula (two over 200, 1%) and symptomatic ureteral reflux (one over 200, 0.5%) were noted. Male gender (100 vs 34, P=0.95), age (46.78 ± 14.17 vs 48.06 ± 14.19 years, P=0.58), Body mass index (24.14 ± 5.04 vs 24.28 ± 4.83, P=0.86) and past history of renal transplantations (16 ± 3% vs 10 ± 3%, P=0.27) as well as cold ischemia time (17.08 ± 7.07 vs 16.9 ± 8.95 hours, P=0.71) were not significantly different in the urinary complications group and the non-urinary complications group. Median hospitalization time was similar in both groups (14 vs 12 days, P=0.37). The existence of urinary complications didn't affect the 5 years kidney graft survival (91.9% vs 89.9%, HR 1.21, CI 95% [0.37-3.3], P=0.83) neither the 5 years patient survival (94.8% vs 92.15%, HR 0.52 CI 95% [0.13-2.07], P=0.85). CONCLUSION: If benign urinary complications in "one-stitch" ureteroneocystostomy were frequent in our study (17% grade II Clavien Dindo), kidney graft and patients survivals were not affected.

High prevalence of ventricular repolarization abnormalities in people carrying TGFßR2 mutations.

Mutations in the TGFßR2 gene have been associated with a life threatening risk of aortic dissection but no arrhythmic death has been previously reported. Two young females carrying a TGFßR2 mutation, initially diagnosed as Marfan syndrome or Loeys Dietz syndrome, presented sudden death with autopsy ruling out dissection. The ECGs of the 2 Sudden Cardiac Deaths revealed profound ventricular repolarization abnormalities with a sinusoidal T-U morphology associated with normal left ventricular ejection fraction. These data strongly suggest sudden cardiac arrhythmic deaths and prompted us to systematically study the repolarization pattern in the patients with TGFßR2 mutations. ECG findings from 58 mutation carriers patients (TGFßR2 group) were compared with those of 46 non-affected first degree relatives (control group). TGFßR2 mutation was associated with ventricular repolarization abnormalities in 47% of patients (p < 0.001 vs. controls), including a 19.6 ms (95%CI 8.7; 30.5) QTc interval prolongation compared to the non-affected first degree relatives (p < 0.001), higher prevalence of abnormal U waves (16% vs. 2%), and sinusoidal T-U morphology (10% vs. 0%). TGFßR2 mutations can be associated with abnormal ventricular repolarization pattern, longer QT interval than non-carrier relatives and an increased risk for sudden death.

Decreased glycolytic metabolism contributes to but is not the inducer of apoptosis following IL-3-starvation.

IL-3 regulates the glycolytic pathway. In Baf-3 cells IL-3 starvation leads to a decrease in glucose uptake and in lactate production. To determine if there is a link between the decreased metabolism induced by growth factor-starvation and the induction of cell death, we have compared the cell death characteristics and the metabolic modifications induced by IL-3-deprivation or glucose-deprivation in Baf-3 cells. We show that in both conditions cells die by an apoptotic process which involves the activation of similar Caspases. Different metabolic parameters (i.e. intracellular ATP levels and lactate accumulation in the culture medium) were measured. We show that IL-3 deprivation leads to a partial decrease in lactate production in contrast to glucose deprivation that completely inhibits lactate production. Similarly following IL-3-starvation a significant drop in the intracellular ATP levels in live cells is observed only after 16 h when a large fraction, more than 50 per cent of cells, is already apoptotic. On the contrary, glucose deprivation is followed by an abrupt decrease in ATP levels in the first 2 h of treatment. However, in the presence of IL-3, cells are able to survive for an extended time in these conditions since 70% of cells survived with low ATP levels for up to 16 h. This was not due to partial inhibition of the apoptotic process by the low level of ATP as glucose-deprivation in the absence of IL-3 led to faster death kinetics of Baf-3 cells compared with IL-3 starvation only. These results indicate that the drop in ATP levels and the triggering of apoptosis can be dissociated in time and that when the glycolytic pathway is strongly inhibited, cells are able to survive with relatively low ATP levels if IL-3 is present. Finally we show that induction of bcl-x by IL-3 protects cells from glucose-deprivation induced cell death.

Quantitative two-dimensional echocardiography in massive pulmonary embolism: emphasis on ventricular interdependence and leftward septal displacement.

In 14 patients requiring aggressive therapy for circulatory failure resulting from massive pulmonary embolism, hemodynamic and two-dimensional echocardiographic data were obtained at bedside (acute phase) and again after circulatory improvement (intermediate phase) and during recovery. The acute stage was characterized by a low cardiac output state despite inotropic support (cardiac index 1.9 +/- 0.6 liters/min per m2) associated with increased right atrial pressure (12.4 +/- 4.2 mm Hg), increased right ventricular end-systolic and end-diastolic area (12.4 +/- 3.4 and 15.4 +/- 4.1 cm2/m2, respectively) and reduced right ventricular fractional area contraction (20.1 +/- 8.6%). Two-dimensional echocardiography also revealed interventricular septal flattening at both end-systole and end-diastole and markedly decreased left ventricular end-diastolic dimensions. Left ventricular fractional area contraction remained normal. Hemodynamic improvement occurred during the intermediate phase as shown by restoration of cardiac index (3.3 +/- 0.6 liters/min per m2), decrease in right atrial pressure (8.3 +/- 4.8 mm Hg), reduction in right ventricular end-systolic area (9.0 +/- 3.6 cm2/m2 at the intermediate stage and 6.1 +/- 1.8 cm2/m2 at recovery) and end-diastolic area (10.5 +/- 3.6 cm2/m2 at the intermediate stage and 8.9 +/- 2.9 cm2/m2 at recovery) and improvement in right ventricular fractional area contraction (31.5 +/- 16.4%). The interventricular septum progressively returned to a more normal configuration at both end-systole and end-diastole, and left ventricular diastolic dimension steadily increased. It is concluded that circulatory failure secondary to massive pulmonary embolism was mediated through a profound decrease in left ventricular preload, resulting from both pulmonary outflow obstruction and reduced left ventricular diastolic compliance.(ABSTRACT TRUNCATED AT 250 WORDS)

A dose-response study of intravenous enoximone in congestive heart failure.

Previous clinical studies with intravenous enoximone have used cumulative dosing to quantify enoximone's hemodynamic effects. The magnitude and duration of the hemodynamic effects of single intravenous doses of enoximone were evaluated in patients with congestive heart failure. Sixty patients, who were in New York Heart Association functional classes III and IV, received single intravenous doses of enoximone, either 0.25 (12 patients), 0.5 (13 patients), 1 (14 patients), 1.5 (10 patients) or 2 mg/kg (11 patients). Cardiac index was increased by 20% with the 0.25 mg/kg dose and by 48% and 42% with the 1.5 and 2 mg/kg doses, respectively. These increases were statistically significant (Student's paired t test with Bonferroni's correction, p less than 0.007) for 1 hour after 0.25 and 0.5 mg/kg, for 2 hours after 1 mg/kg and for 4 hours after 1.5 and 2 mg/kg. Enoximone also reduced pulmonary artery diastolic pressure by 19% with 0.25 mg/kg and by 29% with 2 mg/kg. The duration of effect varied from 1 hour with 0.25 mg/kg to 4 hours with 2 mg/kg. Enoximone produced no consistent or dose-related effects on heart rate or blood pressure. Eighteen adverse reactions were reported by 15 patients, of which 11 were minor and transient (vein pain, flushes, nausea). In 5 patients ventricular or supraventricular arrhythmias were observed, including nonsustained ventricular tachycardia and extrasystoles; 3 of these patients had evidence of arrhythmias before enoximone. Laboratory studies before and after treatment showed no drug-related effects. Dose-related effects on the magnitude and duration of hemodynamic responses to intravenous enoximone were evident within the dose range of 0.25 to 2 mg/kg.(ABSTRACT TRUNCATED AT 250 WORDS)

Placebo-controlled trial of oral enoximone in end-stage congestive heart failure refractory to optimal treatment.

A double-blind, randomized, concurrent trial of enoximone vs placebo was undertaken to assess the efficacy and safety of enoximone, 100 mg t.d.s. added to optimal therapy in 30 patients (mean age, 66.4 +/- 14 years) with severe congestive heart failure. Before inclusion, all patients remained markedly symptomatic despite treatment with diuretics, digitalis, vasodilators and angiotensin converting enzyme inhibitors. Symptoms and quality of life were evaluated at inclusion, and at days 4 and 31; 24-hour electrocardiography and Doppler echocardiography were performed at inclusion and at day 31. Clinical and echocardiographic baseline characteristics were similar in the two groups. During the study, 10 patients dropped out: 3 in the enoximone group (1 death) and 7 in the placebo group (3 deaths). At day 4, symptoms were improved in 13 enoximone-treated patients and in 8 patients on placebo (P less than 0.05). At day 31, symptoms were still improving in 10 of 12 patients on enoximone and in 6 of 8 patients on placebo (NS). No serious clinical side-effects were reported, and no statistically significant difference in the frequency of premature ventricular contractions between the two groups was apparent on Holter monitoring. Peak acceleration of ascending aortic blood flow at entry was 17 +/- 6 m/second2 in the enoximone group and 18 +/- 5 m/second2 in the placebo group (NS). At day 31, the change in peak acceleration was +20% in the enoximone group vs -6% in the placebo group (P less than 0.05). Cardiac index increased by 18% in the enoximone group (from 2.17 +/- 0.7 litres/minute/m2 to 2.4 +/- 1.0 litres/minute/m2 (NS).(ABSTRACT TRUNCATED AT 250 WORDS)

The contribution of bidimensional echocardiography in the diagnosis of cardiac tumours. Based on 25 observed cases.

This paper reviews 25 intracardiac tumours (12 myxomas and 13 thrombi) studied by T.M. echography, bidimensional (2-D) echography and cineangiography during a two year period. In addition, five cardiac tumours (two myxomas, three thrombi) were studied by echography alone, and correctly diagnosed. T.M. echography gives false negative results, whereas both 2-D echo and cineangiography give reliable diagnosis. However, 2-D echo should be the procedure of choice as it is non-invasive, and may be used on patients with equivocal symptoms. Since bidimensional echography has become available during the past two years, 12 myxomas have been correctly diagnosed, compared to 14 found during the previous 13 years.

[The accuracy of angiography in the evaluation of benign renal tumours (author's transl)]. / Wertigkeit der Angiographie in der Beurteilung der Gutartigkeit von Nierentumoren. Eine Literaturübersicht.

The authors review the literature regarding the angiography of three benign renal tumours: adenoma, angiomyolipoma and angioma. Renal adenomas area rare. Oncocytomas can be classified with papillary adenomas; they are fairly characteristic and can be diagnosed pre-operatively. The tubular, and particularly alveolar form cannot be distinguished angiographically from a carcinoma. Angiomyolipomas (hamartomas) occur in tuberous sclerosis or may be found alone. Angiography can first of all be an aid to topographic classification. Its isolated occurrence may cause difficulties in differentiation from a carcinoma. Certain angiographic signs may enable pre-operative diagnosis: the demonstration of aneurysmal arterial changes, linear course of tumour veins and the frequency of a peri-renal haematoma. Angiolipoma is the renal tumour which is most frequently complicated by pre-renal bleeding. Capillary, plexiform or cavernous angiomas are rare. If the first two are large enough (2 cm), their homogenous hypervascularisation may permit preoperative diagnosis. The diagnosis of cavernous angiomas is more difficult.

Indications for ultrasonography in parotid pathologies.

In connection with 141 cases of parotid tumours, 21 adenopathies of the parotid region and 22 cases of parotitis, the authors define the role of ultrasonography in the exploration of the salivary glands. Use of simple criteria permits differentiation of benign from malignant tumours with a good degree of sensitivity (79.8% in our series). This score allows ultrasonography to be offered as the first complementary examination when dealing with a tumefaction of the parotid region. When ultrasonographic findings evoke a malignant lesion, a CT scan seems necessary to evaluate any extension in depth. In contrast, ultrasonography does not appear justified in cases of parotitis or lithiasic pathologies.

[Serum sickness after fibrinolysis using intravenous streptokinase in myocardial infarction]. / Maladie sérique après fibrinolyse par streptokinase intraveineuse au cours d'un infarctus du myocarde.

A case of serum sickness was observed 7 days after administration of intravenous streptokinase in the acute phase of myocardial infarction. The clinical presentation was the sudden development of fever, a papuloerythematous skin rash, myalgia and polyarthritis accompanied by a severe biological inflammatory syndrome without any signs of bacterial or viral infection. Spontaneous regression was observed within 72 hours. Although this complication of streptokinase fibrinolysis seems to be rare, clinicians should be aware of it and not confuse the allergic reaction with that of another drug, the withdrawal of which could be prejudicial for the patient.

[Hypertrophic cardiomyopathy. Data from Doppler echocardiography]. / La cardiomyopathie hypertrophique. Données de l'échographie Doppler.

Hypertrophic cardiomyopathy (HCM) often involves the interventricular septum in the absence of any other cardiac or systemic abnormality capable of explaining this hypertrophy. M mode echocardiography shows septal hypertrophy and a posterior left ventricular wall of normal thickness. Two-dimensional echocardiography gives a better idea of the spatial distribution of the hypertrophy, especially in the short axis parasternal views. Obstruction to left ventricular ejection is another feature of this condition but presence of the obstruction remains a subject of controversy. Doppler studies give easy access to the changes in intraventricular hemodynamics and thereby contribute to fueling the controversy between supporters of intraventricular obstruction and those who prone the theory of hypercontractility. Color coded Doppler enables visualisation of the acceleration of blood flow in the left ventricular outflow tract and the demonstration of mitral regurgitation, which is always present in HCM with obstruction. The echocardiographic evaluation has been facilitated by the recent introduction of transesophageal probes. Doppler studies of mitral blood flow also enable evaluation of left ventricular diastolic function. The evaluation of left ventricular relaxation is often difficult and inaccurate in patients with supraventricular arrhythmias, intraventricular conduction defects or mitral regurgitation. Nevertheless, Doppler echocardiography remains the most reliable and useful method of evaluating HCM.

[Role of scintigraphy in the localization of pheochromocytoma]. / Apport de la scintigraphie à la localisation des phéochromocytomes.

Of the metabolic precursors of the catecholamines fixed by the adrenal medulla, 131I marked thyrosine was chosen as a radio-tracer. 6 positive results out of 11 pheochromocytomas confirmed at surgery were obtained in a series of 23 patients. This result is comparable with the results of arteriography but there are more false positives, reflecting the insecurity of this technique which is otherwise quite innocuous.

[Two-dimensional echocardiography in a cardiac intensive care unit]. / L'échocardiographie bidimensionnelle dans une unité de soins intensifs cardiologiques.

The increasing number of physicians competent to carry out emergency echocardiography, and the availability of high performance equipment, facilitated the development of this technique in the Coronary Care Unit (CCU). This paper reports our experience of 610 2D echocardiographic recordings performed on 319 patients admitted to the CCU. The quality of the recording was excellent or satisfactory in 67% of cases and mediocre in 30% of cases. In 11 patients (3%) the quality of the recording was too poor to obtain reliable data. In this group of patients, the apical 4-chamber and subcostal views seemed better than the apical 2-chamber and parasternal views. An echocardiographic diagnosis was made in 94% of cases. It contributed to the diagnostic process in 70% of cases. Of the patients studied, 54% were admitted for a recent myocardial infarction. Echocardiography was particularly useful in atypical forms or when the diagnosis was difficult. It was also helpful in detecting complications of recent myocardial infarction, the frequency of which was determined. With respect to other cardiovascular emergencies, echocardiography was determined. With respect to other cardiovascular emergencies, echocardiography was very useful in the diagnosis of dissection of the aorta, pericarditis and for assessing left ventricular function and the causal mechanism in cases of decompensated cardiac failure. The non invasive nature of the investigation allows repeated examination of the patient at the bedside and makes it a particularly valuable technique to monitor the evolution of acute cardiac conditions requiring admission to the Coronary Care Unit.

[Anti-angina effect of amiodarone versus delayed-action propranolol. A double-blind randomized study]. / Effet anti-angineux de l'amiodarone. Etude randomisée en double aveugle contre le propranolol retard.

Long-acting Propranolol (160 mg/day) and Amiodarone (200 mg/day after impregnation) were compared in chronic stable angina pectoris. Forty-three patients with stable angina of effort were included in a randomised double blind trial (19 in the amiodarone and 24 in the propranolol group). The duration of the study was 8 weeks; the placebo phase (2 weeks) was followed by 6 weeks of active treatment. An exercise stress test was performed before and after the treatment period. The number of episodes of angina and the consumption of glyceryl trinitrate decreased significantly (p less than 0.001) in the same proportion with both drugs with respect to the placebo period. The time to the appearance of criteria of positivity of the exercise stress test increased from 6.82 +/- 0.50 mn to 8.35 +/- 0.50 mn with amiodarone, and from 7.15 +/- 0.47 mn to 9.50 +/- 0.52 with the propranolol preparation. This improvement was very significant compared with the placebo phase (p less than 0.001) but the difference between the two drugs was not statistically significant (p = 0.39). The other parameters which were studied (time to onset of angina, total duration of exercise, maximum heart rate, double product, maximum ST depression) changed in a parallel fashion significantly versus placebo. There were no differences between the two treatment groups with the exception of the resting heart rate which decreased more in patients on propranolol (80.94 +/- 3.92 to 62.47 +/- 1.97) than in patients on amiodarone (84.87 +/- 2.63 to 73.41 +/- 2.01; p less than 0.0005).(ABSTRACT TRUNCATED AT 250 WORDS)

[Diagnosis of acute aortic dissection by echocardiography]. / Diagnostic des dissections aortiques aiguës par l'échocardiographie.

Until recently, the diagnosis of aortic dissection rested on aortography. The purpose of this study was to evaluate the diagnostic value of echocardiography in that disease and its ability to inform on the extent of the dissection and on the presence of associated lesions. Twenty-six patients (mean age 64 +/- 10 years) admitted for suspected aortic dissection were explored by echocardiography and the results were compared with those of angiography and/or anatomical findings. Echocardiography provided the diagnosis in 14 of the 16 patients with aortic dissection and excluded it in the remaining 10 patients. The sensitivity and specificity of the method were 87.5 p. 100 and 100 p. 100 respectively. The type of dissection was correctly determined in 90 p. 100 of the patients whose aorta had been totally explored by echocardiography. Aortic regurgitation and pericardial effusion were detected in 81 p. 100 and 50 p. 100 respectively of patients with aortic dissection. These results confirm the diagnostic value of echocardiography in dissection of the aorta. The extent of the lesion can only be evaluated when the whole of the aorta is visualized. The echocardiographic diagnosis is easier when the ascending aorta is involved (type I), while in type III aortic dissection there is a risk of missing a retrograde lesion of the aorta and confusing this type with type I. In this study two kinds of intimal flap motion were observed: in the first one the motion was independent of that of the aorta, while the second one resembled a division of the aortic, wall the motion of which is parallel to that of the aorta.

[Dose-response relation of intravenous enoximone in congestive cardiac insufficiency]. / Etude de la relation dose-réponse de l'énoximone intraveineux dans l'insuffisance cardiaque congestive.

Enoximone (MDL 17043) is a new generation inotropic drug which acts by inhibiting phosphodiesterase and is endowed with both inotropic and vasodilator properties. The purpose of this study, which involved 23 patients aged from 18 to 75 years in NYHA class III or IV and with evidence of severe haemodynamic disturbances (cardiac index below 2.5 1/mn/m2, pulmonary wedge pressure above 15 mmHg), was to evaluate the acute haemodynamic responses to doses of enoximone that ranged from 0.25 to 2.50 mg/kg administered by bolus intravenous injection. Heart failure was either of ischaemic origin (6 cases) or idiopathic (10 cases) or due to various causes (7 cases). Group A patients (n = 11) received the drug in low doses (less than or equal to 1 mg/kg) as opposed to group B patients (n = 12) who were given high doses (greater than 1 mg/kg). Results were evaluated from the amplitude and duration of the haemodynamic response at maximum effect time (30 min). The following parameters were measured: cardiac index, pulmonary wedge pressure, systemic vascular resistance, mean arterial pressure and heart rate. Cardiac index and pulmonary wedge pressure were significantly improved in both groups (P less than 0.005): cardiac index +39 p. 100 in group A, +55 p. 100 in group B; pulmonary wedge pressure -36 p. 100 in group A, -48 p. 100 in group B; systemic vascular resistance -46 p. 100 in group B. Heart rate and arterial pressure were not significantly altered. The duration of response was 1 to 3 hours in group A patients and 4 to 8 hours in group B patients.(ABSTRACT TRUNCATED AT 250 WORDS)

[Marfan syndrome]. / Syndrome de Marfan.

Marfan's syndrome is a monogenetic disease with an autosomal dominant transmission generally accompanied by type I fibrillin abnormality. This widely-distributed molecule participates in the structure of connective tissues so that any aberration may result in disease of many systems: skeletal morphology, dislocation of the lens, neurological or cutaneous signs and dilatation of the aorta predisposing to dissection, mitral valve prolapse being a common association. The diagnosis, clinical because of the size of the culprit gene and the multiplicity of the possible mutations, is sometimes difficult, and diagnostic criteria have been proposed. It is important to make the diagnosis because treatment is based on the restriction of violent exercise, betablocker therapy and regular echocardiographic monitoring of the ascending aorta, the region at highest risk of dilatation and dissection. A family enquiry is essential to make the diagnosis before the onset of complications in pauci-symptomatic patients (great intra-familial variability). Pregnancy poses special problems in these patients.

[Double-blind clinical and echocardiographic study of oral enoximone versus placebo in severe cardiac insufficiency]. / Etude clinique et échocardiographique en double insu de l'enoximone oral contre placebo dans l'insuffisance cardiaque sévère.

The effect of enoximone was assessed by a randomised double blind trial versus placebo. The clinical status of the patients was evaluated by the NYHA classification and quality of life score. Inotropic state was estimated from the maximum acceleration of aortic and pulmonary blood flow recorded by Doppler echocardiography. Thirty patients with severe cardiac failure, aged 66.4 +/- 14 years, symptomatic despite maximal therapy associating diuretics, digitalis, nitrate derivatives and angiotensin converting enzyme inhibitors, were included. Fifteen patients were given enoximone 100 mg three times a day orally (Group E) and the other 15 were given a placebo (Group P). The NYHA class and quality of life scores were assessed at D0, D4 and D31. Doppler echocardiography and Holter recordings were performed on D0 and D31. The two groups were comparable at D0. Ten patients abandoned the trial, 3 from Group E (including 1 death) and 7 from Group P (including 3 deaths). At D4, 13 patients from Group E and 8 from Group P were clinically improved (p < 0.05). At D31, the clinical state was stable or improved in 10 of the 12 patients in Group E and 6 of the 8 patients in Group P (NS). No secondary effects were severe enough to warrant the withdrawal of treatment: the frequency of ventricular extrasystoles was comparable in the two groups at D0 and D31. At D31 the maximal aortic acceleration had increased by 20% compared with D0 (p < 0.05) and the maximal pulmonary acceleration by 31% (p < 0.05) in Group E. The same parameters showed no significant change in Group P (-6% and +5% respectively).(ABSTRACT TRUNCATED AT 250 WORDS)