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Biomedical subjects

C Marchal

Publications and source records attributed to C Marchal.

At least 73 records · Page 4Linked to original sources

[Neonatal thrombosis of the aortic arch].

The authors report a case of aortic arch thrombosis in a neonate probably related to perinatal placental emboli. However the finding of a fibroblastic endarteritis raised the possibility of a primitive lesion of the aortic wall. The evolution was complicated by cerebral ischemia. A surgical thrombectomy was performed. The outcome was favourable with persistence of a moderate right motor deficiency at 9 months. The authors advise the addition of an enlargement patch and the use of anticoagulation therapy in case of intimal lesion or very adherent thrombus.

Aorta, Thoracic↗

[Role of ultrasonographic tests in the diagnosis of moderate to severe pulmonary embolism].

Between November 1991 and June 1992, 50 patients suspected of pulmonary embolism (SP > 20%) underwent Doppler echocardiography, venous Doppler ultrasonography and pulmonary angiography. Pulmonary embolism was confirmed by pulmonary angiography in all patients but 3 (2 pts: mean pulmonary pressure > 50 mmHg and 1 pt: mobile thrombus between the infundibulum and the main pulmonary artery). Two groups were identified on the basis of Miller's index: Group 1: "non-massive" pulmonary embolism, Miller < 60% (n = 18); Group 2: "massive" pulmonary embolism, Miller > or = 60% (n = 29). The patient with thrombus in the main pulmonary artery and the two with high pulmonary pressures were included in Group 2. Venous Doppler ultrasonography was performed in 96% (n = 48) of patients, including 90% within the first 24 hours. No distinction could be drawn between the two groups on the basis of venous Doppler ultrasonography findings. A majority of patients had thrombosis of main collecting vessels (Group 1 = 75%, Group 2 = 78%) and 10% of patients had no venous thrombosis of the lower limbs. Doppler echocardiography was performed in all patients, including 94% (n = 47) within the first 24 hours. Dilatation of the left ventricle as well as analysis of septal contraction was evaluable in all patients except one of Group 2, because of poor technical quality and of artificial pacing. A RV/LV ratio > 0.60 was found in 97% (30/31) of patients of Group 2 as compared with 39% (7/18) in Group 1.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

[Diffuse neonatal hemangiomatosis. A case with tetralogy of Fallot].

In neonatal disseminated hemangiomatosis, multiple immature capillary hemangiomas are widely distributed in the skin, mucous membranes, and internal organs including the liver, lungs, and larynx. In patients with life-threatening visceral involvement, corticosteroids or interferon may be required. A case in a female infant with tetralogy of Fallot is reported.

Adrenal Cortex Hormones↗

[Polysaccharide bodies: an unusual finding in a case of temporal epilepsy. Review of the literature].

Massive occurrence of polyglucosan bodies (PB) was found in the surgically removed temporal lobe of a 34 year-old woman presenting with complex partial seizures. The term of PB was proposed in order to group Lafora bodies (LB), corpora amylacea (CA) and Bielschowsky bodies (BB) on the basis of their biochemical similitude. A rigorous histochemical differentiation between these anomalies appears to be impossible at present. LB, BB and CA are mainly made up of glucose polymers i.e. polyglocusans. The pathway(s) leading from glycogen accumulation to PB formation is still unknown. PB are a hallmark of two diseases: Lafora disease and adult polyglucosan body disease. PB have occasionally been reported in rare cases of a variety of other neurological diseases. In all cases they were located within the neurons. This site characterizes Lafora bodies. BB are intraneuronal inclusions but restricted to neurons of the external pallidum. CA occur predominantly in the astroglia during the course of ageing. The significance of these structures depends on their regional distribution. The resemblance does not imply a common etiology for all conditions in which such bodies occur; it is probably due to the sharing of the final path in their causative pathway. Our case does not correspond to any of the classical diseases in which PB have been found. In our opinion, the patient exhibited a localized form of glycogen storage disease.

Adult↗

[Selective bronchial occlusion in the treatment of unilateral interstitial emphysema in respiratory distress in premature infants].

A case of right unilateral pulmonary interstitial emphysema in a compromised premature infant was managed by right selective bronchial occlusion with a favorable outcome. Occlusion with a Rashkind catheter occurred on day 8 and was well-tolerated. The balloon was deflated 10 hours later and no recurrence was observed. Right occlusion is easier than left selective intubation and seems to be the best method for treatment of unilateral interstitial emphysema in infants in extremely poor conditions.

Bronchi↗

PulO, a component of the pullulanase secretion pathway of Klebsiella oxytoca, correctly and efficiently processes gonococcal type IV prepilin in Escherichia coli.

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.

Bacterial Outer Membrane Proteins↗

Role of pilA, an essential regulatory gene of Neisseria gonorrhoeae, in the stress response.

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.

Bacterial Outer Membrane Proteins↗

Occurrence of polyglucosan bodies in temporal lobe epilepsy.

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.

Adult↗

[Neonatal Campylobacter fetus septicemia: a case secondary to maternal-fetal contamination].

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.

Bacteremia↗

[Rhythmogenic cardiomyopathies of atrial origin in children. Myth or reality?].

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.

Adrenergic beta-Antagonists↗

[Thrombi of the right heart. Value of thrombolytic therapy in mobile thrombi].

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)

Adult↗

[Facio-linguo-masticatory diplegia and epilepsy. Cortical dysplasia].

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.

Adult↗

[Research and surgical treatment of 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)

Adolescent↗

[Enzymatic activities of the mitochondrial respiratory chain in child cardiomyopathies. 34 cases prospectively studied by endomyocardial biopsy].

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)

Adolescent↗

Unusual encephaloclastic lesions with paraventricular calcification in congenital rubella.

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.

Brain Diseases↗