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Biomedical subjects

M Fardeau

Publications and source records attributed to M Fardeau.

At least 235 records · Page 13Linked to original sources
PubMed1981

[First-line exploration: fiberoptic gastroscopy or barium meal? A pragmatic evaluation (author's transl)].

The influence of the initial upper GI tract exploration upon the accuracy and cost of diagnosis was evaluated in 103 in- and out-patients of a hepato-gastroenterology unit. The patients were divided at random into two groups of comparable number, age, sex and clinical findings. One group was first examined by fiberoptic gastroscopy and the other by barium meal. In patients whose first examination was by endoscopy a second examination was less frequently needed (3/53 versus 14/50; p less than 0.01), significant lesions (e.g. oesophagitis, oesophageal varices, gastroduodenal losses of substance or tumours) were more often diagnosed (22/53 versus 11/50; p less than 0.05) and the time required for a diagnosis to be made was shorter (mean 7 +/- 6 days versus 14 +/- 16 days; p less than 0.01), even when only one examination was performed (7 +/- 6 days versus 12 +/- 15 days; p less than 0.05), than in patients first examined by barium meal. It is concluded that fiberoptic gastroscopy should be the initial method of exploration of the upper GI tract.

Adult↗
PubMed1981

Congenital neuromuscular disorder with predominant mitochondrial changes in type II muscle fibers.

The muscle biopsy of a young boy presenting a marked hypotrophy of stature and weight, proximal muscle weakness, uncertain gait, and partial hearing loss, showed an abnormal distribution of the mitochondrial activities in type II muscle fibers by histochemical methods. Electron microscopy confirmed the presence of giant mitochondria in these muscle fibers, and at a lesser degree in some type I fibers. These findings contrast with the usual type I predominance of the mitochondrial changes in the different "mitochondrial" myopathies.

Adenosine Triphosphatases↗
PubMed1981

Inclusion body myositis.

The histochemical and ultrastructural study of muscle biopsies of two patients with a chronic muscle weakness and wasting showed particular changes in muscle fibers: (1) peripheral lined vacuoles, containing whorls of membranes and cytoplasmic debris; (2) collections of intranuclear and intrasarcoplasmic tubular filaments (16-18 nm in external diameter and 6.5 nm in inner diameter). These changes are characteristic of a rare muscle disorder termed inclusion body myositis; its individuality is favoured by the present study. The resemblance of the tubular filaments to myxovirus nucleocapsid has been suggested by various authors but attempts to isolate the virus were unsuccessful in several reported cases as well as in those here presented. This does not exclude a viral origin of the disease. The similarity of the tubular filaments to thick myofilaments has been invoked by others, but has not been demonstrated. At the present the nature of the abnormal filaments remains unknown.

Adult↗
PubMed1980

Nuclear inclusions in oculopharyngeal dystrophy.

The ultrastructural examination of skeletal muscle biopsies of three typical cases of autosomal dominant inherited oculopharyngeal muscular dystrophy showed collections of tubular filaments (8.5 nm in diameter) within muscle fibre nuclei. These filaments appear to be a characteristic morphological feature of oculopharyngeal dystrophy.

Biopsy↗
PubMed1980

[Electromyographic and histopathological study of the Lambert-Eaton myasthenia syndrome. Apropos of a case].

In this case of Eaton-Lambert myasthenic syndrome the electrophysiological study demonstrated some modifications of latency of the muscle action potentials evoked by repetitive nerve stimulation at 30 c/sec. specially after administration of edrophonium chloride. This data suggest as possible some fluctuations in the mechanism of the neuromuscular transmission defect. The muscle biopsy showed changes secondary to a peripheral motor nerve involvement; electronmicroscopic study of the motor end-plates revealed a highly developped subneural apparatus.

Action Potentials↗
PubMed1979

[Lipidic myopathy with severe cardiomyopathy caused by a generalized carnitine deficiency. Favourable course during carnitine hydrochloride treatment].

The case of a girl who presented with gastrointestinal upsets with nausea, vomiting and occasional hypoglycaemic attacks during childhood is reported. At about 5 years of age generalised muscular weakness with severe amyotrophy, cardiomegaly with a cardiothoracic ratio of 0,63, left ventricular hypertrophy on electrocardiography and left ventricular dilatation with hypokinesis on echocardiography were observed. A few weeks later she developed severe cardiac failure. Muscle biopsy showed muscular dystrophy with lipid infiltration due to carnitine deficiency )serum carnitine 9 nmoles/ml, normal values: 46 +/- 6,9 nmoles/ml; muscle carnitine 0,27 nmoles/mg, normal values: 3,0 +/- 0,79 nmoles/mg fresh frozen weight). She improved rapidly with carnitine chlorhydrate and a diet low in lipids and high in medium chain triglycerides. Regression of muscular symptoms and cardiac failure was observed. After 13 months follow-up with no tonicardiac therapy she is much improved; the signs of heart failure have disappeared, the cardiothoracic ratio is now 0,55 and the electrocardiogramme and echocardiogramme are normal.

Biopsy↗
PubMed1978

[Modifications in the type distribution of muscle fibers induced by repeated local freezings the rat sciatic nerve].

In the Rat, after a localized freezing of the sciatic nerve inducing a complete denervation of the medial head of the gastrocnemius muscle, the reinnervation took place within 16-18 days under our experimental conditions. After only one freezing, a limited and stable "type-grouping" of muscle fibres is observed from 30 to 360 days. After 2 to 5 freezing repeated every three weeks, the muscular changes observed one month after the last freezing are much more pronounced than when only one freezing is performed. These changes consist of a progressive increase in the number of type I and type II C fibres. This transformation is not stable: 3 months after the 3rd freezing, the muscle pattern does not differ from that which is noted after a single freezing.

Animals↗
PubMed1978

[Histochemical characterization of the tonic fibers in the flexor carpi radialis muscle of frogs (Rana temporaria, L.)].

In the flexor carpi radialis muscle of the Frog, extra-fusal muscular fibres with histochemical characteristics of tonic fibres were identified by reactions for enzyme activities, especially for myofibrillar adenosine triphosphatase. This evidence suggests no difference between the sexes in the organization of this muscle, in spite of a strongly marked sexual dimorphism.

Adenosine Triphosphatases↗
PubMed1978

[Recent findings on the genealogy, clinical aspects and histology of a family with periodic hyperkalemic paralysis].

A family presenting a hyperkalemic form of periodic paralysis was studied through six generations; 31 individuals were affected, 14 could be examined. Periods of diffuse or localized muscle weakness, lasting one to four days, were associated with the classic adynamic attacks. Myotonic symptoms were discrete. Some patients, in particular the hard manual workers, exhibited a permanent proximal deficiency. Serum potassium level was in the lower normal range but raised during the attacks. EMG showed a polymorphic pattern, suggesting a functional neuro-muscular block. The histological and ultrastructural changes were pleiomorphic: vacuoles, tubular aggregates and fibers were found in a patient with permanent weakness; a pure type II fibre atrophy was noticed in another patient, without any weakness between the attakcs. Acetazolamide treatment gave good results in four cases out of seven.

Adolescent↗
PubMed1977

Ultrastructure of muscle and sensory nerve in Fabry's disease.

A study of a muscle biopsy has provided evidence of storage involvement of the skeletal muscle fibres in Fabry's disease. In the endothelial cells of the capillaries, the inclusions were more abundant and pleomorphic. Muscle satellite cells were spared. In the sensory nerve biopsy, the perineurial and endothelial cells contained lamillar bodies, but not the Schwann cells. There was a slight reduction of the number of the small myelinated fibres and of the unmyelinated fibres. An electron microscopic study of a muscle biopsy can be helpful in the diagnosis of the disease. The physiopathology of the pain attacks is still a matter for discussion.

Adult↗
PubMed1977

[Eaton-Lambert myasthenic syndrome: clinical, electrophysiologic, histological and ultrastructural study].

In this case, showing the classical features of the Eaton-Lambert myasthenic syndrome, the neuromuscular symptoms appeared 21 months before the bronchial carcinoma was diagnosed. The electrophysiological study demonstrated, beside the marked potentiation under repetitive nerve stimulation at 30 c/sec., some modifications of distal latency, specially after administration of Edrophonium chloride. The muscle biopsy showed changes secondary to a peripheral motor nerve involvement; electronmicroscopic study of the motor end-plates revealed a highly developped subneural apparatus. Long-run treatment by guanidine hydrochloride determined haematologic and renal toxic manifestations. Prednisolone was for a time active on the myasthenic syndrome.

Action Potentials↗