[Incidence of thromboembolism depending on the type of surgery].
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Biomedical subjects
Publications and source records attributed to C Marchal.
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The PulO protein required for extracellular secretion of pullulanase by Klebsiella oxytoca is known to be highly homologous to two type IV prepilin peptidases, namely XcpA(PilD) (Pseudomonas aeruginosa) and TcpJ (Vibrio cholerae). The predicted prepilin peptidase activity of PulO was confirmed by showing that it could correctly process the product of the cloned pilE.1 type IV pilin structural gene from Neisseria gonorrhoeae in Escherichia coli. The P. aeruginosa prepilin peptidase and another putative prepilin peptidase, ComC from Bacillus subtilis, also processed prePilE. Subcellular fractionation showed that the pilE gene product that had been processed by PulO remained associated with the cytoplasmic membrane, as did the unprocessed precursor. PulO was also shown to process three of the four prePilE-PhoA hybrids tested. Southern hybridization experiments suggest that a pulO homologue is present in the N. gonorrhoeae chromosome.
Sequence analysis has shown that PilA, a transcriptional regulator of pilin gene expression in Neisseria gonorrhoeae, has extensive homology with the 54-kDa protein of the signal recognition particle of eukaryotes and its receptor, as well as with two proteins of Escherichia coli, FtsY and Ffh, which have been proposed to be a part of a signal recognition particle-like apparatus. We tested the putative role of PilA in protein export in N. gonorrhoeae and did not find any effect. However, we did observe induction of a heat shock response and a previously described slow-growth phenotype when PilA function was impaired. We also examined the interference of pilA expression in E. coli with the function of the products of ftsY and ffh and observed an accumulation of pre-beta-lactamase. We argue against a direct role for PilA in protein export in gonococci and propose instead that PilA is involved in the modulation of cell growth rate in response to different environmental conditions.
Massive occurrence of polyglucosan bodies (PBs) was found within the surgically removed temporal lobe of a 34 year old woman with complex partial seizures. This peculiar feature is very unusual in neuropathological examinations of epileptogenic foci. This patient could not be included in any of the classic diseases in which PBs are found. She exhibited a localised form of glycogen storage disease.
A favourable outcome of a severe Cambylobacter fetus sepsis in a neonate is reported. The transmission was probably vertical. His mother experienced diarrhoea 15 days before birth. No infecting organism was isolated from maternal stools, but maternal antibody response was significant. Such a perinatal infection with meningitis and septicemia is uncommon. Bacteriological diagnosis requires an enriched medium and a long incubation. The diagnosis should be evoked in view of an anamnestic context, a negative culture or a resistance to antimicrobial drugs.
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Incessant, rapid, supraventricular tachycardia may be complicated by cardiac failure with ventricular dilatation and hypokinetic wall motion on echocardiography: so-called tachycardia-induced cardiomyopathy. The diagnosis is simple when the cardiac rhythm is not sinus rhythm. The authors report the cases of 4 children aged 7 months to 12 years, referred for diagnosis and treatment of apparently primary cardiomyopathy. The findings of spontaneous or vagally-induced atrioventricular conduction defects, a permanently rapid atrial rhythm though influenced by 24 hour variations, or periodic abnormal rate increases, suggested myocardial dysfunction due to an ectopic atrial tachycardia. This was an essential step in management as the control of the tachycardia by amiodarone or betablocker therapy resulted in regression of symptoms and normalisation of left ventricular function. However, some atrial tachycardias are very resistant to medical treatment and, in such cases, there should be no hesitation in using more radical approaches, surgery or ablation, even and especially in patients with severe cardiac failure. In conclusion, apparently primary dilated cardiomyopathy in children may be due to chronic atrial arrhythmia and it is essential to perform at least Holter monitoring in order not to miss this diagnosis.
Seven patients with mobile right heart thrombi, 4 floating and 3 pediculated, were recensed between 1985 and 1990. Two patients were admitted for congestive cardiac failure (Group I) and 5 patients for pulmonary embolism (Group II). Both patients in Group I were treated with heparin without complications. In one case, the size of the thrombus decreased in 10 days whereas, in the second case, it disappeared within 8 days. In Group II, the first patient underwent successful thrombectomy. The other four patients were given thrombolytic therapy (UK = 2, rt-PA = 2) associated with appropriate doses of heparin. In the two patients given UK (3M units the first day followed by 1.2 M units per day for 4 days) the thrombus disappeared in the first 48 hours of treatment. One patient had a recurrent pulmonary embolism after 2 hours' treatment; both patients had a fall in haemoglobin of 3 cg/ml at the second day. The second patient died at the 5th day. In the two patients treated by rt-PA (100 mg/7 hours) the thrombus disappeared within 4 hours of starting therapy. One patient had a probable recurrent pulmonary embolism. Both patients had a fall in haemoglobin of 3 cg/ml at the 2nd day of treatment. Right heart thrombi are rare (168 cases in the literature of which 111 were mobile). The prognosis seems to be related to echocardiographic appearances: mortality of mural thrombi is about 4% compared with 50% in mobile thrombi. Very mobile "worm-like" masses are therapeutic emergencies because of the risk of embolism (about 68%).(ABSTRACT TRUNCATED AT 250 WORDS)
Neuronal migration anomalies are caused by insults occurring during the third to fifth gestational months when neuroblasts migrate from the germinal zone to the cortical plate. They lead to several cerebral malformations such as macrogyria, identified nowadays by MRI. We describe a case of bilateral parieto-rolandic macrogyria responsible for a bi-opercular syndrome resulting in a facio-linguo-masticatory diplegia associated with mental retardation and severe epilepsy.
The currently available surgical procedures for the treatment of epilepsy, from fundamental data to therapeutic results, including various means of investigation are reported. The work is based on a review of the literature and on the cases studied by the two teams from the Universities of Montreal and Bordeaux who share the same concept of epilepsy surgery. The patient groups of the two teams include 316 S.E.E.G., 214 cortectomies, 39 callosotomies and 2 multiple sub-pial transsections. In the first part, the authors attempt to demonstrate that the epileptic focus corresponds to the region where the seizures arise, that this focus is not directly comparable to the region where inter-ictal spikes are recorded and sometimes becomes autonomous from the causal lesion. The epileptic phenomenon has a definite harmful effect on cerebral functions and a probable self-aggravating potential. The second chapter summarizes the clinical data on which the indications and contraindications are based. These obviously depend on whether the intervention is intended to be curative or palliative. Various non-invasive and invasive investigations are then reviewed, according to their relative importance and the experience of each team. The main points developed are: the electroclinical correlations during seizures, the symptomatological data for differentiating between temporal and frontal lobe seizures, the contribution of M.R.I. in demonstrating the epileptogenic and epileptic lesions, the electrophysiological information suggesting that S.E.E.G. remains the most informative mean of investigation. The various methods of investigation of assessing electrical, functional (cerebral blood flow, metabolism) and morphological aspects of epilepsy, supply non-redondant findings about the localisation of the epileptic focus. The chapter on surgical techniques mainly discusses the various modes of implantation of subdural and intracerebral electrodes and reports the same rate of morbidity in both cases. Orthogonal teleradiography is still perfectly suited to the implantation of intracerebral electrodes. S.E.E.G. is still the most anatomically precise technique. However, in certain conditions, extraoperative E.Co.G. is more adequate. New surgical modalities have recently appeared such as the multiple subpial transsections which allow treatment of epileptic foci unapproachable by cortectomy and such as modified techniques of hemispherectomy, which by decreasing morbidity, renew interest in them. In the chapter on surgical results, the authors emphasize the methodological problems of evaluation that partly account for their wide variability. The results obtained with the various surgical modalities are reviewed. The outcome in cortectomies is discussed at length in terms of the data from the literature as well as the results reported by both teams.(ABSTRACT TRUNCATED AT 400 WORDS)
The arguments in favour of mitochondrial pathology of certain childhood cardiomyopathies (multi tissue involvement, lactic acidosis, histochemical abnormalities of skeletal muscle) are indirect and may be absent in isolated cardiomyopathy. The authors therefore set up a prospective study of enzyme activity of the mitochondrial respiratory chain directly by endomyocardial biopsy. Fifty children aged 2 months to 16 years were included. Thirty four had cardiomyopathy which was dilated and hypokinetic with thin walls in 21 cases, restrictive in 2 cases, and hypertrophic in 11 cases; the remaining 16 children had either normal hearts (13 catheterised for other reasons) or myocardial hypertrophy due to pulmonary or aortic stenosis (3 cases). Both ventricles were evaluated in 3 cases; macro-surgical biopsies were obtained in 6 cases and skeletal muscle biopsy was obtained in 9 cases. The results indicate the method to be reliable with no difference between the micro and macro biopsies. The absolute values of enzyme activity were too variable to serve as quantitative parameters but some ratios of activity were remarkably stable and allowed a qualitative assessment which was all the more accurate when identical values were obtained in the myocardium, skeletal muscle and liver. The mitochondrial respiration was independent of ventricular pressures and of the type (right or left) of ventricle. Enzyme activity was nearly always normal in dilated cardiomyopathy (20/21) which suggests that it was unaffected by dilatation of the heart and by abnormal myocardial contractility. The results could be normal in myocardial hypertrophy and in valvular stenosis and in over half the cases of hypertrophic cardiomyopathy.(ABSTRACT TRUNCATED AT 250 WORDS)
We report an unusual case of congenital rubella. The infant was suffering from a serious encephalopathy, and both prenatal echography and neonatal CT scan showed passive ventriculomegaly with a calcified periventricular border. Usually, such lesions are strongly suggestive of cytomegalovirus (CMV) infection and have never previously been reported in congenital rubella. Classic cerebral lesions in rubella are related to a prominent obstructive vasculopathy. Conversely, encephaloclastic lesions in CMV infection are likely related to a necrosis of brain parenchyma following upon an initial ventriculitis, and perhaps also to a disturbance of neuronal proliferation. Recently, Carey described a neonate with proven congenital rubella and cranial ultrasound findings typical of ventriculitis. However, in spite of the close similarity between our patient's lesions and the typical CMV lesions, we think it's impossible to assert similar pathogenic mechanisms. Actually, it's quite conceivable that only a severe or extensive vasculopathy can lead to brain atrophy with periventricular calcification in congenital rubella.
Early and late prognosis after percutaneous balloon aortic valvuloplasty (PBAV) was assessed in 38 consecutive elderly patients (mean age, 78.5 +/- 6.1 years). Significant valve opening was achieved in 35 patients. The hospital mortality was 8% (three patients); two other patients died within the first month and three underwent aortic valve replacement. At 2 years follow-up, there were 10 additional deaths (seven cardiac deaths) and five patients had symptom recurrence managed by aortic valve replacement (3) or repeat PBAV (2). Overall, six patients underwent surgery without untoward events and six had repeat PBAV. Only two out of six patients with repeat PBAV had sustained improvement; one was referred to surgery and the remaining three died soon after the second PBAV. One- and 2-year survival were respectively 72 and 62% and percentage of survivors with persistent improvement 68 and 41%. Although aortic valve area after PBAV was associated with outcome, predictors of poor long-term prognosis were primarily related to the pre-operative haemodynamic status. Patients with pulmonary resistances greater than 400 dynes cm-1 s-5 had the poorest outcome (chi 2 = 18.4-P less than 0.0001). Overall, signs of heart failure were predictors of poor long term follow-up. These data indicate that long-term success of PBAV is mainly related to the left ventricular dysfunction noted prior to intervention.
The aim of this in-vitro study was to evaluate the combination of busulfan with radiotherapy on TE-671 human medulloblastoma cells since unexpected clinical toxicity of busulfan was reported during the treatment of brain tumors, suggesting a possible radiopotentiation. The cytotoxicity of busulfan was determined by using colony forming assays, and doses inducing growth inhibitions of 10, 20 and 50% were selected to be tested in association: 6, 12 and 32 mumol/l for busulfan and 0.5, 1 and 3 Gy for irradiation. All possible combinations were considered within this frame and the results showed that the combination of busulfan with radiotherapy exerted an additive effect without potentiation.
We have previously reported the identification of two genes, pilA and pilB, which act in trans to regulate pilus expression in Neisseria gonorrhoeae. Here we show that PilA and PilB have amino acid sequence similarities with members of the two component 'sensor-regulator' family of proteins. PilB has homology with histidine kinase sensors. Alkaline phosphatase fusions to the predicted sensor and transmitter domains are described. Their PhoA activity and cellular location suggest that PilB is inserted in the cytoplasmic membrane and predict periplasmic and cytoplasmic locations for the sensor and the transmitter domains, respectively. PilA has homology with response regulators in its N-terminal part, and with components of the eukaryotic protein secretory apparatus (SRP 54 and SRP receptor) as well as two Escherichia coli gene products in its C-terminal part. In particular, it contains a putative GTP-binding site. Mini-transposon insertions into different regions of pilA were obtained. The phenotypes and genotypes of these mutants and preliminary biochemical studies of the gene products of two of these mutants lend further support to the hypothesis that PilA is a DNA-binding response regulator and confirm that it participates in an essential function in the bacterium.
The pilE gene of Neisseria gonorrhoeae MS11 and a series of pilE-phoA gene fusions were expressed in Escherichia coli. The PhoA hybrid proteins were shown to be located in the membrane fraction of the cells, and the prepilin product of the pilE gene was shown to be located exclusively in the cytoplasmic membrane. Analysis of the prepilin-PhoA hybrids showed that the first 20 residues of prepilin can function as an efficient export (signal) sequence. This segment of prepilin includes an unbroken sequence of 8 hydrophobic or neutral residues that form the N-terminal half of a 16-residue hydrophobic region of prepilin. Neither prepilin nor the prepilin-PhoA hybrids were processed by E. coli leader peptidase despite the presence of two consensus cleavage sites for this enzyme just after this hydrophobic region. Comparisons of the specific molecular activities of the four prepilin-PhoA hybrids and analysis of their susceptibility to proteolysis by trypsin and proteinase K in spheroplasts allow us to propose two models for the topology of prepilin in the E. coli cytoplasmic membrane. The bulk of the evidence supports the simplest of the two models, in which prepilin is anchored in the membrane solely by the N-terminal hydrophobic domain, with the extreme N terminus facing the cytoplasm and the longer C terminus facing the periplasm.
Twenty-five cases of the congenital, idiopathic long QT syndrome, occasionally diagnosed at birth and persisting after the 4th day of life, were analysed retrospectively to determine the medium-term prognosis and to identify risk factors. The age of the patients varied: 13 neonates including 10 of less than 5 days of age, and 12 children 1 to 12 years old. Eleven patients belonged to families with long QT syndromes. The recruitment also varied: syncope or near syncope (9 cases), bradycardias or tachyarrhythmias (9 cases) or by systematic familial enquiry (7 cases). The corrected QT interval (QTc) was greater than 0.44 s in all patients, and 17 patients had conduction (atrioventricular or intraventricular) defects and/or ventricular arrhythmias (tachycardia, torsades de pointe or fibrillation). All except two older children were prescribed betablocker therapy and in 6 cases of resistant arrhythmias a pacemaker was implanted. There were 6 deaths: 4 deaths occurred in neonates who had the longest QT intervals (greater than 0.65 s) complicated by conduction defects and tachyarrhythmias. The other 2 fatalities were in older children who both died during syncope. The 19 survivors were followed up for 3 months to 16 years: 4 children with the shortest QTc values (0.44 to 0.48 s) have completely recovered; 2 neonates treated for 9 months and then weaned off therapy without any complications and 2 older children who were not treated. The other 15 cases are all under treatment but do well and have had no syncopal episodes during follow-up.(ABSTRACT TRUNCATED AT 250 WORDS)