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Biomedical subjects

L Mauri

Publications and source records attributed to L Mauri.

At least 19 recordsLinked to original sources

Valvular Heart Disease in the Pregnant Patient.

Anticoagulation for the pregnant patient with valve disease is problematic: both the underlying thrombotic disorder and the pharmacologic agents available for its treatment pose significant risks to the mother and fetus. There are no randomized controlled trial data available to guide decision-making for this patient population. Clinical treatment algorithms usually are derived from patient registries or case series with the obvious limitations of retrospective review, selection bias, historical controls, and small patient numbers. Prospective trials clearly are needed, but clinical research in the pregnant patient presents a myriad of ethical and legal challenges. Warfarin and unfractionated heparin, the mainstays of anticoagulant therapy, fall quite short in any analysis of efficacy and safety. There is an increasing use of low molecular weight heparins (LMWHs) in clinical practice but without evidence-based validation. Anticoagulant management of the pregnant patient must begin with full disclosure of the hazards and limitations of all forms of available treatments, preferably prior to conception. Treatment should be predicated on an assessment of the relative risks of thrombosis and hemorrhage. Careful monitoring and dosage adjustment are required throughout gestation, labor, delivery, and the puerperium.

Journal Article↗

Preparation of deacetyl-, lyso-, and deacetyl-lyso-GM(3) by selective alkaline hydrolysis of GM3 ganglioside.

Three methods (using GM3 quantities ranging from a few milligrams to grams) have been developed to prepare, in high yield, the three derivatives of ganglioside GM3 [alpha-Neu5Ac-(2-3)-beta-Gal-(1-4)-beta-Glc-(1-1)-ceramide]: deacetyl-GM3 [alpha-Neu-(2-3)-beta-Gal-(1-4)-beta-Glc-(1-1)-ceramide], lyso-GM3 [alpha-Neu5Ac-(2-3)-beta-Gal-(1-4)-beta-Glc-(1-1)-sphingosine], and deacetyl-lyso-GM3 [alpha-Neu-(2-3)-beta-Gal-(1-4)-beta-Glc-(1-1)-sphingosine]. This is the first report of the preparation of lyso-GM3 by a one-pot reaction. We can now define the optimal conditions for the different preparations. Preparation of deacetyl-GM3: alkaline reagent, 2 M KOH in water; GM3 concentration, 33 mg/ml; reaction temperature, 90 degrees C; reaction time, 3.5 h; nitrogen atmosphere. Preparation of deacetyl-lyso-GM3: alkaline reagent, 8 M KOH in water; GM3 concentration, 10 mg/ml; reaction temperature, 90 degrees C; reaction time, 18 h; nitrogen atmosphere. Preparation of lyso-GM(3): alkaline reagent, 1 M sodium tert-butoxide in methanol; GM3 concentration, 10 mg/ml; reaction temperature, 80 degrees C; reaction time, 18 h; anhydrous conditions. The percentage yield of deacetyl-GM3 was 70;-75%, that of deacetyl-lyso-GM3 100%, and of lyso-GM3 36;-40%.Deacetyl-GM3, deacetyl-lyso-GM3, and lyso-GM3 were purified by column chromatography, and chemical structures were confirmed by electron spray-mass spectrometry.

Chromatography, High Pressure Liquid↗

Clinical and electrophysiologic evolution of the Wolff-Parkinson-White syndrome in children: impact on approaches to management.

There is less certainty about the recommendations for radio-frequency ablation as a therapeutic option for Wolff-Parkinson-White syndrome in children as opposed to adults because of the different natural history and the age-related risks of ablation. To help decision-making, we evaluated the long term clinical evolution and electrophysiologic characteristics of pre-excitation in our series of children and young adults. We reviewed the clinical course of 109 patients below the age of 18 years who had been followed up over a period of 9+4 years, with a range from one to 27 years. This corresponded to 986 patient-years. We examined also the electrophysiologic data from 98 of those patients who underwent a transesophageal study. At the discovery of pre-excitation, 59% of patients were asymptomatic, while 29% developed supraventricular tachycardia during follow-up. The peak incidence of the onset of supraventricular tachycardia occurred during infancy. These patients had the highest incidence of subsequent spontaneous disappearance of the tachycardia (53%), such a favourable evolution being encountered in only 12% of patients in whom the abnormal rhythm first appeared after 12 years of age. Chronic medical treatment was required in 47% of patients, and proved completely effective in 45% of cases. The potential to induce atrial fibrillation, and the incidence pre-excitation considered to be high risk, with the shortest pre-excited RR interval equal to or less than 220 msec, was lowest in the group of patients aged less than 6 years of age, and highest in those older than 12 years of age (p <0.001). Pathways producing arrhythmia with high risk were more common in symptomatic (29%) than in asymptomatic patients (7%) (p<0.001). No mortality occurred. On the basis of our findings, we suggest that ablation should be avoided before the age of 5 or 6 years. Thereafter, the procedure should become the first line of treatment for symptomatic patients older than 12 years of age.

Adolescent↗

Stent implantation without predilation in patients with a single, noncalcified coronary artery lesion.

The purpose of this study was to assess the feasibility of stent implantation without predilation in patients with a single, noncalcified coronary artery lesion. A total of 122 patients were randomized to receive a stent with or without predilation; direct stent placement was possible in 59 of the 61 patients (97%) with an immediate and long-term clinical follow-up similar to that observed in the group of patients treated conventionally.

Aged↗

Changes in poly(A) tail length of maternal transcripts during in vitro maturation of bovine oocytes and their relation with developmental competence.

Molecules of mRNA are stored in the oocyte cytoplasm in order to be used during the initial phases of embryonic development. The storage takes place during oocyte growth and the extent of poly(A) tail at the 3' end of the transcripts has emerged as an important regulatory element for determining their stability. The objective of the present study was to analyse changes in polyadenylation levels of mRNA transcripts, stored in bovine oocytes, during in vitro maturation and their possible relation with developmental competence. Oocyte developmental competence was predicted on the basis of the morphological appearance of their originating ovary as previously established (Gandolfi et al. 1997a. Theriogenology 48:1153-1160) and were divided into groups H (high competence) and L (low competence). The length of the poly(A) tail of the following genes, beta-actin (beta-Act), connexin 43, glucose transporter type 1, heat shock protein 70, oct-4, plakophilin, pyruvate dehydrogenase phosphatase (PDP), and RNA poly(A) polymerase, was determined at the germinal vesicle (GV) and metaphase II (MII) stage. The results indicated that the poly(A) tail of all genes except for beta-Act and PDP, is shorter after in vitro maturation (IVM) in both groups. Moreover, group L oocytes showed a shorter poly(A) tail than group H oocytes in all genes except for beta-Act and PDP, both at GV and MII stage. We conclude that most of the examined transcripts follow the default deadenylation pattern described during oocyte maturation in other species and that a shorter poly(A) tail is correlated with low developmental competence.

Animals↗

A procedure for the preparation of GM3 ganglioside from GM1-lactone.

A simple procedure is described for preparing GM3 ganglioside, from a few milligrams to grams, from GM1-lactone (Sonnino et al., (1985) Glycoconjugate J 2: 343-54) [1]. The synthesis was carried out under the following optimal conditions: 30 mM GM1-lactone in 0.25 M H2SO4 in DMSO, 30 min, 70 degrees C, nitrogen atmosphere, strong stirring. The yield of GM3 was 55%. The procedure applied to milligram amounts of GD1b-dilactone gave GD3 ganglioside.

Animals↗

[Clinical course of pre- and post-natal isolated congenital atrioventricular block diagnosed in utero].

We evaluated the pre- and postnatal outcome of isolated atrioventricular (AV) block detected during fetal life in order to identify factors that may affect the natural history of this lesion and to assess prenatal therapy. Over the past eight years, we consecutively evaluated 10 fetuses with complete AV block. The mean gestational age at diagnosis was 25.3 weeks and the mean heart rate was 57 bpm; two fetuses were hydropic. During pregnancy, one fetus suddenly died, while 6 out of 9 fetuses had a mean reduction in heart rate of 17.8 bpm; 4 patients had heart rate < 50 bpm. Five fetuses developed heart failure, which was severe in 2 cases and mild in 3. The mean gestational age at delivery was 31 weeks. Dexamethasone was administered to the mothers during pregnancy in 4 cases without modification of AV block and/or of heart rate, but in 3 out of 4 fetuses the general condition remained stable in spite of the reduction in heart rate in two of them. Sympathomimetic drugs were employed in 3 cases with an increase in fetal heart rate, but maternal discomfort appeared in two cases. Three newborns died during the first week of life, two of hydrops and one of persistent pulmonary hypertension. Cardiac pacing was performed in 6/9 patients within the first 8 months of life and in 3 within the first 2 days. In conclusion, morbidity and mortality are high when AV block is detected during fetal life. Negative prognostic factors are hydrops and a heart rate < 50 bpm. Pre-term delivery to enable cardiac pacing is probably the therapy of choice if gestational age is > 27-28 weeks. Sympathomimetic drugs are effective but are poorly tolerated by the mothers. Dexamethasone has no effect on AV block and/or heart rate, but may improve clinical tolerance of conduction disturbance.

Adult↗

Tissue plasminogen activator binding to the annexin II tail domain. Direct modulation by homocysteine.

Tissue plasminogen activator binds to endothelial cells via the calcium-regulated phospholipid-binding protein annexin II, an interaction that is inhibited by the prothrombotic amino acid homocysteine. We sought to identify the tissue plasminogen activator binding domain of annexin II and to determine the mechanism of its modulation by homocysteine. Tissue plasminogen activator binding to immobilized annexin II was inhibited by intact fluid phase annexin II but not by its "core" fragment (residues 25-339). Two overlapping "tail" peptides specifically blocked 65-75% of binding. Localization of the tissue plasminogen activator binding domain was confirmed upon specific inhibition by the hexapeptide LCKLSL (residues 7-12). Expressed C9G annexin II protein failed to support tissue plasminogen activator binding, while binding to C133G, C262G, and C335G was equivalent to that of wild type annexin II. Upon exposure to homocysteine, annexin II underwent a 135 +/- 4-Da increase in mass localizing specifically to Cys9 and a 60-66% loss in tissue plasminogen activator-binding capacity (I50 = 11 microM). Upon treatment of cultured endothelial cells with [35S]homocysteine, the dithiothreitol-sensitive label was recovered by immunoprecipitation with anti-annexin II IgG. These data provide a potential mechanism for the prothrombotic effect of homocysteine by demonstrating direct blockade of the tissue plasminogen activator binding domain of annexin II.

Amino Acid Sequence↗

Which variable of stenosis severity best describes the significance of an isolated left anterior descending coronary artery lesion? Correlation between quantitative coronary angiography, intracoronary Doppler measurements and high dose dipyridamole echocardiography.

OBJECTIVES: This study sought to investigate the angiographic or intracoronary Doppler variables of stenosis severity that best correlate with the results of dipyridamole echocardiography. BACKGROUND: Quantitative coronary angiography and intracoronary Doppler flow velocity assessments are the commonly used techniques for the objective identification of significant coronary artery stenosis. METHODS: Thirty patients with an isolated lesion of the left anterior descending coronary artery (LAD) were studied by means of on-line quantitative coronary arteriography, intracoronary Doppler flow velocity measurements and dipyridamole echocardiography 6 months after percutaneous transluminal coronary angioplasty. The quantitative arteriographic analyses were performed on-line; post-stenotic Doppler flow velocities were measured at baseline and after adenosine infusion. Angiographic and Doppler measurements were compared with the corresponding dipyridamole echocardiographic data and analyzed by discriminant analysis. RESULTS: The dipyridamole echocardiographic response was positive in 11 patients (37%). The best cutoff values for predicting an abnormal echocardiographic response were 1) stenotic flow reserve of 2.8 (p = 0.0001); 2) 59% diameter stenosis (p = 0.0001); 3) minimal lumen diameter of 1.35 mm (p = 0.001); 4) coronary flow reserve of 2.0 (p = 0.0002); and 5) maximal peak velocity of 60 cm/s during hyperemia (p = 0.04). Multivariate analysis identified stenotic flow reserve as the only independent predictor of ischemia during dipyridamole echocardiography. CONCLUSIONS: Stenotic flow reserve is the variable that best describes the functional significance of an isolated LAD lesion, and a value of 2.8 is the best predictor of a positive dipyridamole echocardiographic response. Furthermore, angiographic variables of stenosis severity relate to echocardiographic test results better than intracoronary Doppler variables.

Adult↗

[Mid-term clinical condition and prognosis in adrenergic ventricular tachycardia in children with apparently normal hearts].

INTRODUCTION: Ventricular tachycardia is the most common diagnosis among children with exercise or emotion-related syncope. The aim of the study was to assess the prognosis and the clinical course of children, without long QT syndrome, with exercise-related ventricular tachycardia and apparently normal heart. METHODS: Since January 1984, we studied 14 children (mean age 9.7 +/- 3.8 years) with exercise-related ventricular tachycardia, and, at the initial detection, without demonstrable heart disease, as assessed by clinical evaluation, electrocardiogram and mono-bidimensional echocardiogram. Six patients had exercise related syncope, 1 exercise intolerance and 7 were asymptomatic. All patients underwent exercise testing on treadmill and 24-hour ECG monitoring. All patients were treated with antiarrhythmic drugs. All patients were tested by exercise testing and 24 hour ECG monitoring, alternatively, every six months, during therapy or after complete wash-out of the drug used. RESULTS: Ventricular tachycardia, detected by exercise testing and Holter monitoring, was polymorphic in 4 symptomatic patients, monomorphic with left bundle branch block morphology and inferior axis in the others. The mean rate of monomorphic ventricular tachycardia was 224 +/- 35 bpm in the symptomatic children and 180 +/- 33 bpm in the others (p = 0.03). Successful chronic treatment was achieved with beta-blockers in 5 cases, propafenone in 6, sotalol in 2 and flecainide plus metoprolol in 1. During follow-up (6 +/- 2.7 years), 1 patient with polymorphic ventricular tachycardia, on therapy, suddenly died during exercise, the other symptomatic patients had no variation of their arrhythmia on routine tests, except 1 who, after propafenone was stopped, had ventricular fibrillation during exercise testing. Among the 7 asymptomatic patients, 5 had no evidence of ventricular tachycardia on routine test after 2 +/- 3 years; the others had no variation of their treatment. CONCLUSION: Exercise related ventricular tachycardia in children seems: 1) to have more severe prognosis if symptomatic or polymorphic; 2) mostly to arise from right ventricle; 3) to be successfully treated by antiarrhythmic drugs with beta-blocking activity.

Adolescent↗

The Kleinian Psychoanalytic Diagnostic Scale (revised version): presentation and study of reliability.

The Kleinian Psychoanalytic Diagnostic Scale (KPDS) is a psychodiagnostic instrument of assessment based on the psychoanalytical theory of object relations. It was created in order to introduce an intrapsychic and relational dimension into the clinical research on and epidemiology of psychiatric diagnosis. It consists of 15 subscales grouped into the following four dimensions: "Ego Abilities'; "Projective Identification'; "Paranoid-Schizoid'; and "Depressive'. These dimensions constitute different, relatively stable aspects of the mental life of the subject, their demarcation and description permitting the obtention of a chart and profile of the intrapsychic and relational structure. Following several free, unstructured interviews the scale is assessed on the basis of what the subject says and what the rater may detect in the relationship. The inter-rater reliability of the scale, its stability over a time lapse, as well as its internal consistency have all been shown to be high.

Adolescent↗

[Factors limiting adaptation to exercise after the Fontan operation: role of altered pulmonary function].

BACKGROUNDS: The abnormal adaptation to exercise after Fontan operation, is primarily due to a suboptimal increase in cardiac output. We postulated that abnormal pulmonary function could cooperate in reducing effort tolerance of these patients (pts). METHODS: We have evaluated 10 pts, mean age 15 +/- 8 yrs, with a mean postoperative follow-up of 6 +/- 2 yrs. Seven pts were asymptomatic while 3 had mildly decreased effort tolerance. These pts were evaluated with echocardiogram, cycle ergometer stress test, basal and effort spirometry and perfusion lungs scanning. RESULTS: Basal ejection fraction was normal in 9 pts and mildly depressed in 1 pt. Basal cardiac index was 1.8 + 2.2 l/min/m2 and increased to 4.8 + 5 l/min/m2 during exercise. Effort tolerance was 73% +/- 21% of predicted values, with maximal O2 consumption of 60% +/- 13% of predicted. Basal spirometry showed a mild restrictive pattern in 7 pts with a decrease in total pulmonary capacity and in forced vital capacity (mean values respectively 79% +/- 14% and 75% +/- 18% of predicted), otherwise respiratory reserve resulted normal in all pts. Carbon monoxide diffusion was impaired in all pts with a mean value of 49% +/- 10% of predicted. O2 saturation near normal in basal condition decrease during exercise (mean value 96% +/- 2% and 91% +/- 7%). The VE/VO2 and Vd/Vt ratio were both abnormally increased (respectively 111% +/- 28% and 186% +/- 39% of predicted). Lung scanning was abnormal in 6 pts with defects of perfusion localized prevalently in left lung (5/6) especially in upper lobe (4/6). CONCLUSIONS: After Fontan operation exercise tolerance is reduced as consequence of 1) a suboptimal increase in cardiac output and 2) abnormal gas diffusion probably due to a damage of alveolo-capillary membrane and/or to a ventilation/perfusion mismatch as showed by abnormalities in lungs perfusion and by increase Vd/Vt ratio.

Adaptation, Physiological↗

[Idiopathic recurrent ventricular tachycardia in children: characteristics and long-term prognosis].

BACKGROUND: The aim of this study was to assess the prognosis and the clinical course of incessant idiopathic ventricular tachycardia (VT) with left bundle branch morphology, in a group of 14 children consecutively evaluated in our institutions between 1983 and 1994. METHODS: Mean age of these patients was 8 +/- 2 years, and they were followed for 56 +/- 32 months. In all the children, VT was present for more than 80% of the day, in non sustained runs. The mean rate of VT was of 142 +/- 30 beats/min. Four children were symptomatic. The evaluation of these children was obtained by treadmill stress test (12/14), cardiac catheterization (13/14), electrophysiologic study (10/14), nuclear magnetic resonance (7/14), and serial echocardiographic and Holter examinations. RESULTS: Right ventricular dysplasia was suspected in 2 cases on the basis of nuclear magnetic resonance and angiographic data. In both these cases VT was not suppressed by exercise. Medical treatment was started in 9/14 patients (69%), it was completely effective in 4 (44%) and partially effective in 2 further cases (22%). Thirteen out of fourteen patients had a regular follow-up. No patient died. All patients remained asymptomatic and in 6 (46%) VT disappeared. In 4 of these last 6 patients therapy has been previously effective. Two children, one with right ventricle dysplasia, continue antiarrhythmic treatment. CONCLUSIONS: Incessant VT is well tolerated in pediatric age. The frequency of spontaneous remissions is relatively high (46% of cases). In few patients this arrhythmia is associated with evident cardiac abnormalities. In our opinion, medical treatment is necessary in symptomatic patients and in those with heart abnormalities.

Angiocardiography↗

Isolated congenital left ventricular diverticulum.

Two cases of isolated left ventricular diverticulum are presented. A 12-year-old boy and a 7-year-old girl, both asymptomatic, were admitted to our Department for evaluation of an abnormal electrocardiogram and an abnormal cardiac silhouette, respectively. Both patients had the diagnosis confirmed by cardiac catheterization and angiography and underwent successful surgical correction of the abnormality. We discuss the diagnosis, prognosis, and surgical treatment of this entity and we advocate early surgical treatment for all diagnosed ventricular diverticula, even if asymptomatic.

Angiocardiography↗

The use of propafenone in the treatment of tachyarrhythmias in children.

Propafenone was given to 60 children (mean age 4.5 years) with paroxysmal re-entrant supraventricular tachycardia (rSVT: 41 cases), postoperative automatic junctional tachycardia (JET: eight cases), automatic atrial tachycardia (AT:four cases), ventricular tachycardia (VT:four cases) and atrial flutter (AF: three cases). In acute cases (29) propafenone was administered intravenously (mean dose 1.3 +/- 0.5 mg.kg-1) chronic oral treatment (mean dose of either 11 +/- 3.3 mg.kg.day-1 or 265 +/- 78 mg.m-2 x day-1) was given to 48 children, in 2-3 divided doses. Overall efficacy was 76% for acute and 69% for chronic treatment, with best results in paroxysmal rSVT. It was effective in 89% acutely of those treated acutely and in 69% of those administered chronically. The efficacy of propafenone in the prevention of rSVT was strictly related to the incidence of attacks before treatment:propafenone was indeed inefficacious in 6/11, 6/14 and 0/12 of patients with daily, weekly and monthly attacks respectively. Side effects were observed in 25% of patients: 6% required suspension of therapy due to pro-arrhythmic effects in one patient on intravenous administration, peripheral neuropathy in one case and hypotension in two postoperative JET patients. In conclusion, its efficacy and limited side effects make propafenone a relatively safe and very useful drug in the treatment of various tachyarrhythmias. However, risk of unpredictable toxic levels requires careful use of the drug especially in the first days of treatment, when a daily monitoring of ECG is strongly recommended.

Administration, Oral↗