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Biomedical subjects

M Weber

Publications and source records attributed to M Weber.

At least 667 records · Page 37Linked to original sources

[Hyalinosis cutis and mucosae. Ultrastructural histochemical aspects indicating intracellular accumulation of glycosaminoglycans].

The exact nature of the genetic defect of hyalinosis cutis et mucosae or d'Urbach-Whiete syndrome is still matter of controversy. The present article reports on three new cases in which several different ultrastructural and biochemical investigations add more arguments to support an anomaly of the glycosaminoglycans degradation in the dermal fibroblasts. Cationic dyes as polyethyleneimine and alcian blue show an intense ultrastructural staining of the abnormal basal laminae and the intracellular lysosomal bodies in cultured fibroblasts. These are related to the accumulation of anionic charged proteoglycans. The primary defect of hyalinosis cutis et mucosae is likely due to a lysosomal defect so far not biochemically defined.

Adolescent↗

[Antibiotic sensitivity of Pasteurella multocida and related bacteria (bacterial groups M5 and EF4). Studies of minimal inhibitory concentrations by agar dilution].

We have been isolating Pasteurella multocida and similar germs increasingly during the last few years: due to the rabies coming back in Eastern France, more consultations have been held following animal bites; samples are then taken for a bacteriologic research. We have studied their sensitivity towards 30 antibiotics. The determination of the MIC was achieved through the agar dilution method on 34 Pasteurella multocida of human origin issued after bites and expectorations, 4 EF4 and 4 M5. The Pasteurella are very sensitive to: beta-lactam antibiotics (the lowest MIC were observed for ureido-penicillins, amino-benzylpenicillins and third generation cephalosporins), chloramphenicol, cyclines and quinolones. Fosfomycin colistin and aminoglycosides are also active but with higher MIC. The macrolides have got a slow or no activity at all. The M5 are susceptible to the same antibiotics as Pasteurella but with slightly higher concentrations. Regarding EF4, minor sensitivity or resistance to penicillin G, cephalothin and cefamandole can be pointed out.

Anti-Bacterial Agents↗

[Diagnosis of lesions in the area of the facial skull--indications and effectiveness of the conventional roentgen technic in comparison with computer tomography].

The substantial information conveyed by conventional radiodiagnostics of pathological alterations of the facial skull is shown in 243 patients examined by conventional radiodiagnostic means and by computerised tomography. Except in case of special demands, conventional tomography has to be preferred in routine diagnostics of the facial skull as a primary method supplying an answer to the most important questions at a lower cost and in a shorter time.

Diagnosis, Differential↗

[Congenital and acquired foot deformities in the x-ray picture].

In addition to general radiologic aspects there are special orthopedic considerations in interpretation of x-rays of the foot. This is especially important for the diagnosis of orthopedic foot diseases. In clubfoot x-rays are useful for therapeutic planning and control. Even in the first months of life radiographs can show important disturbances of growth of the foot and displacement of the bones of the tarsus. In other congenital foot deformities x-rays are important for diagnostic reasons: they prove luxations or skeletal deformities. The most important acquired foot disease is the pronating foot. X-rays do not only show the amount of joint damage and structural changes of bones but also allow to draw conclusions to be drawn about the causes of static and dynamic changes of the foot skeleton. Functional diagnostic radiological investigation is of decisive importance for evaluation of infantile pronating foot. X-rays allow the differentiation to be made between physiological and pathologic changes. Subtle radiographic investigation is essential while planning operative treatment in childhood, as in adults.

Child↗

[Respiratory emergency in the newborn infant: extreme laryngotracheo-esophageal cleft (esophagotrachea)].

Intra vitam diagnostic procedures revealed a complete laryngo-tracheo-oesophageal cleft in a premature infant with respiratory distress. Anamnesis together with clinical and roentgenological symptoms suggested the diagnosis of oesophageal atresia or oesophago-tracheal fistula. The diagnosis of "oesophago-trachea" was finally confirmed by laryngo-tracheoscopy. The morphologic defect in this case was combined with partial supradiaphragmatic dislocation of the stomach and with intestinal malrotation.

Diagnosis, Differential↗

[HLA and familial multiple sclerosis].

Some data suggest an environmental perhaps a viral factor but also of a genetic factor in the etiology of multiple sclerosis. Among the latter is the notably increased risk for a twin when the other twin has the disease, a risk further increased if they are monozygotic. There is also a greater than chance frequency of common HLA haplotypes in 2 affected siblings. The frequency of familial forms of multiple sclerosis is estimated at approximately 6 p. 100. We have studied 14 families of which 12 included 2 members with multiple sclerosis and 2 with 3 affected members. Parental relation between patients was parent to child (7 cases), brother to sister (5 cases), sister to sister including two pairs of twins (4 cases) and cousin to cousin on the mother's side (2 cases). When compared with non-familial multiple sclerosis there were no particular features in clinical disorders or course: 4 forms were progressive, the others evolving by episodes. In 26 patients in whom HLA antigens were determined, the DR2 antigen was present 19 times, the B7 antigen 9 times and the A3 antigen 7 times. In the 8 pairs of siblings with multiple sclerosis, 2 were HLA-identical and 5 semi-identical. One pair had no common haplotype. Grouping of HLA in 22 healthy members allowed 8 genealogic trees to be established. If a gene for susceptibility to multiple sclerosis exists, it is of low penetration, of dominant transmission and of limited frequency. It probably lies close to the region D of chromosome 6, because of the disequilibrium of crossed linking with A3, B7 and DR2 antigens.

Chromosome Mapping↗

[Budd-Chiari syndrome following dacarbazine therapy of malignant melanoma--an avoidable complication?].

A 51-year-old female patient died of Budd-Chiari syndrome during treatment with adjuvant DTIC mono-chemotherapy for malignant melanoma. We report on clinical course and laboratory findings in detail. Differentiating BCS from VOD, we describe the liver damages referring to the latest findings about the effect of DTIC in fibrinolysis. In order to prevent further lethal complications, we suggest to regard pre-existing liver damage as a contra-indication for DTIC therapy; to prefer intraspinal anesthesia for malignant melanoma of the lower extremities; to avoid hepatotoxic drugs and alcohol during chemotherapy; to protect DTIC from light; to extend the interval between first and second cycle. Laboratory data, the "finger-prints" of DTIC, are unreliable. Great attention should be paid to clinical findings which may justify immediate and high dose corticosteroid therapy as well as intensive care monitoring.

Budd-Chiari Syndrome↗

[Perioperative high-caloric alimentation with the central venous catheter. Prospective study in 404 patients].

Peri-operative high-calorie nutrition was administered, through a total of 500 catheters introduced into the superior vena cava via the subclavian vein, to 404 patients admitted to hospital for gastro-intestinal cancer resection or other major abdominal operations, some of them taking a complicated course. Most of the catheters remained in situ for 6-20 days; the longest period was 44 days. Catheter insertion was successful in 97.8%. Faulty position of the catheter occurred in 4.4%; in 2.8% it was rectified under fluoroscopic control. In 92.2% there were no complications ascribable to the catheter. A pneumothorax resulted in 1.4% of patients, central thrombosis in 0.4%. Catheter-related sepsis was noted in 6.2%.

Adult↗

Antiglomerular basement membrane antibodies in human sera: detection by a modified micro-ELISA.

An enzyme-linked immunosorbent assay (ELISA) for detection of antibodies to human glomerular basement membrane has been developed. Special emphasis has been put on the choice of microtiter plates which were coated with a collagenase digest of human glomerular basement membrane. Results differed markedly between the different microtiter plates. Best results were obtained with a flexible polyvinylchloride microtiter plate with flat wells (Dynatec). This plate exhibited the highest positive/negative ratio and the lowest intraassay standard deviation. Optimal conditions for each step in the ELISA have been determined. The assay proved to be specific, sensitive, and reproducible. Circulating antibodies in each of 11 patients with active antiglomerular basement membrane disease were detected by the ELISA, while sera from patients with various renal and nonrenal diseases were negative by the test.

Autoantibodies↗

Attempted immunization of swine against acute sarcocystosis using cystozoite-derived vaccines.

Low-dose Sarcocystis miescheriana infections have recently been shown to protect pigs against acute sarcocystosis. Because this protective immunity was short-lasting, an alternative immunization strategy was examined. Four experimental vaccines were prepared from S. miescheriana cystozoites and tested in 13 pigs. Two vaccines were prepared from intact organisms (live and formalin-fixed cystozoites) and 2 from subcellular cystozoite fractions (pellicle and protoplasm extracts). The live vaccine was injected intraperitoneally and the remainder were suspended in Freund's incomplete adjuvant and injected intramuscularly. An additional 5 pigs were injected with adjuvant or saline placebos and used as controls. Serum samples were collected regularly and tested in enzyme immunoassays for specific IgM and IgG antibodies. Low levels of IgM antibodies were detected after 8 days and elevated levels of IgG antibodies were detected after 22 days. The success of vaccination was tested 40 days after vaccination by lethal homologous challenge of each pig with 3 X 10(6) sporocysts. Despite the presence of specific antibodies at the time of challenge, all pigs died from acute sarcocystosis 12 days later. The cystozoite vaccines were therefore antigenic but not immunogenic and did not induce any protective immunity.

Animals↗

[Significance of abnormally high somatosensory evoked potentials (SEV)].

Data found in the literature and our own observations prompted us to consider the possibility that abnormally enlarged Somatosensory Evoked Potentials (SEPs) may have a diagnostic and physiopathological significance, particularly in a group of diseases which include common clinical features of encephalopathy with stimuli-sensitive myoclonus and epilepsy, whatever their etiology may be (degenerative or storage disease, metabolic, toxic or post-hypoxic encephalopathy...). We discuss the amplitude, morphology, diagnostic and therapeutic contribution of these 'giant' SEPs and pathogenic assumptions with reference to 'cortical reflex myoclonus'. Studies of back-averaged encephalogram, SEPs and long-loop reflexes allow some illustration of a functional hyperreactivity of the sensori-motor cortex, but no conclusive demonstration of its origin.

Adolescent↗

[Changes in somatosensory evoked potentials during EEG activation by bemegride in man].

The use of evoked potentials for the evaluation of neuronal mechanisms by which convulsant drugs activate epilepsy and produce seizure has been reported by many authors. Electrophysiological effects of bemegride with augmented responsivity of brain structure to sensory stimulation is well known, especially in experiments performed with implanted electrodes in animals. After recording evoked activity from parietal scalp following median nerve stimulation before and during 12 human EEG activations by bemegride, the authors find an increase in amplitude of cortical somatosensory evoked potentials. Changes in amplitude after diazepam administration are analysed, as well as morphological changes preceding and following grand mal seizures induced by bemegride administration. This variation in amplitude of evoked responses is compared with those recorded in animals with several convulsant drugs and in man affected with progressive myoclonic encephalopathies. A common action of these agents, beyond their effects on specific neurotransmitters (perhaps mediated by a blockade of neurotransmitter-induced chloride conductance increases), is discussed.

Adolescent↗

[Changes in the semeiology of epileptic seizures after status epilepticus: apropos of 65 cases].

In 65 patients with status epilepticus, we compared the clinical expression of isolated seizures and of status seizures. In 22 patients there was no relationship between status and isolated seizures. In addition the number of partial status is greater than that of partial seizures. In 9 patients, the type of seizures was modified after the status. From these results, status epilepticus seems to favor the eruption of secondary epileptogenic focuses, generally transitory.

Adolescent↗

Early development of locomotion: significance of prematurity, cerebral palsy and sex.

Data on the development of locomotion during the first two years of life were collected for 128 preterm infants (21 with varying degrees of cerebral palsy) and for 111 healthy term infants. Most stages of locomotion occurred at slightly later ages among the neurologically unimpaired preterm infants than among those born at term (age corrected for prematurity). There was no difference between preterm and term infants with regard to the number and types of pathways of locomotion, or to age at onset and type of first movements through space. By 9.5 months of age, 95 per cent of term infants and 92 per cent of preterm infants showed some ability to move through space. The infants with severe cerebral palsy had considerable delay in the development of locomotion, but this occurred to only a minor extent when the degree of cerebral palsy was mild or moderate. Most stages of locomotion occurred at slightly earlier ages for boys than for girls, but these differences were not significant at any age. The number and types of pathways of locomotion, and types of first movements, were comparable in both sexes.

Cerebral Palsy↗

Ultrasonic findings in analgesic nephropathy.

34 patients with analgesic nephropathy (AN) were investigated by real-time ultrasonography. In 11 out of 14 patients on maintenance dialysis and in 16 out of 20 patients with renal insufficiency calcified renal papillae were documented surrounding the central sinus in a typical garland pattern. Moreover, by surveying a group of patients with renal insufficiency of unknown origin, AN was assumed in 10 patients and was then confirmed by a hitherto unknown history of analgesic abuse as well as by laboratory findings. In 37 patients radiologic or autopsy data were additionally available. A close correlation to the scanning pattern was found in 31 of these patients. In 30 healthy volunteers and 56 patients with renal insufficiency due to chronic glomerulonephritis (n = 24) or diabetic nephropathy (n = 32) calcified renal papillae were found only in 1 case. 5 out of 20 patients on maintenance dialysis due to other diseases than AN showed renal calcifications forming an approximate garland arrangement. In these patients sonography may therefore indicate AN only in very characteristic cases. We believe that renal papillary calcifications surrounding the central sinus in a garland pattern may indicate AN in most cases and thus may be helpful in establishing the diagnosis of AN.

Adult↗