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Biomedical subjects

M Weber

Publications and source records attributed to M Weber.

At least 343 records · Page 19Linked to original sources

Principles of treatment of the upper extremity in arthrogryposis multiplex congenita type I.

The involvement of the upper limb in arthrogryposis multiplex congenita for many patients means a far-reaching dependency on outside help. The extension contracture of the elbow joint especially makes it impossible to reach the mouth or to perform hygienic necessities. Therefore, the rehabilitation program includes an improvement of passive elbow flexion by capsulotomy or of active flexion by triceps transfer if possible, or both. In bilateral involvement, the optimal solution is to have one arm in flexion for reaching the head and mouth passively or even actively and one arm in extension for hygienic necessities. From 1973 to 1993 we performed 22 releases of the elbow contracture in 16 children. An additional triceps transfer was performed in five elbows. The overall results showed a marked increase of the range of motion and a functional improvement concerning the daily activities (in 17 children). In 5 children there was no gain but also no loss of functional capacities. In 3 of 5 children with an additional triceps transfer, an improvement of active flexion was attained. Pre- and postoperative physiotherapy is at least as important as the operative procedure itself.

Adolescent↗

A recombinant Bacillus anthracis strain producing the Clostridium perfringens Ib component induces protection against iota toxins.

The Bacillus anthracis toxinogenic Sterne strain is currently used as a live veterinary vaccine against anthrax. The capacity of a toxin-deficient derivative strain to produce a heterologous antigen by using the strong inducible promoter of the B. anthracis pag gene was investigated. The expression of the foreign gene ibp, encoding the Ib component of iota toxin from Clostridium perfringens, was analyzed. A pag-ibp fusion was introduced by allelic exchange into a toxin-deficient Sterne strain, thereby replacing the wild-type pag gene. This recombinant strain, called BAIB, was stable and secreted large quantities of Ib protein in induced culture conditions. Mice given injections of live BAIB spores developed an antibody response specific to the Ib protein. The pag-ibp fusion was therefore functional both in vitro and in vivo. Moreover, the immunized animals were protected against a challenge with C. perfringens iota toxin or with the homologous Clostridium spiroforme toxin. The protective immunity was mediated by neutralizing antibodies. In conclusion, B. anthracis is promising for the development of live veterinary vaccines.

ADP Ribose Transferases↗

Occupational contact dermatitis to propacetamol. Allergological and chemical investigations in two new cases.

BACKGROUND AND OBJECTIVE: In 2 new cases of occupational contact dermatitis due to a recently described allergen (propacetamol), a prodrug which is a soluble diethylglycidyl ester of paracetamol, an allergological investigation was performed to elucidate the nature of the allergen involved in the propacetamol contact sensitivity. OBSERVATIONS: Two nurses with eczema of the hands and face had positive patch tests to Pro-Dafalgan. Every day the nurses prepared injections of Pro-Dafalgan (propacetamol dissolved in sodium citrate). The sensitization was due to propacetamol and not to the solvent. To elucidate which part of propacetamol was responsible for the sensitization, the 2 nurses were patch-tested with diethylamine, paracetamol (diluted in different vehicles) and some of their chemical analogues and potential impurities which were all negative. CONCLUSIONS: Propacetamol induces airborne contact dermatitis with no evidence of sensitization to paracetamol or diethylglycine, possibly because of either the presence of unknown impurities and/or an antigenic structure related to the covalent bond of the prodrug.

Acetaminophen↗

Intravitreal injection of ganglioside GM1 after ischemia reduces retinal damage in rats.

BACKGROUND AND PURPOSE: Gangliosides are normal components of cell membranes and contribute to structural rigidity and membrane function. They have been shown to protect against various insults in the brain. We have shown previously that GM1 administered intraperitoneally before the induction of retinal ischemia provides a protective effect. This study evaluates the protective effect of GM1 administered intravitreally after ischemia on retinal lesions. METHODS: We induced retinal ischemia unilaterally in Long-Evans rats by increasing intraocular pressure to 160 mm Hg for 60 minutes. GM1 (20 microL x 10(-5) mol/L) or saline (20 microL) was injected into the vitreous 15 minutes after ischemia, and the postischemic survival time was either 8 or 15 days. The degree of retinal damage was assessed by histopathological study. RESULTS: Retinal ischemia led to reductions in thickness and cell number, principally in the inner retinal layers (39% to 80%) and to a lesser extent in the outer retinal layers (26% to 45%). Postischemic treatment with intravitreally injected GM1 conferred significant protection against retinal ischemic damage after both 8 and 15 days of survival time. After 8 days of reperfusion, the ischemia-induced loss in overall retinal thickness was reduced by 15% and those of the inner nuclear and plexiform layers by 44% and 17%, respectively. Ischemic-induced ganglion cell and inner nuclear cell density losses were reduced by 37% and 27%, respectively. After 15 days of reperfusion, approximately the same statistically significant differences could be observed in comparison with the 15-day saline-injected group. CONCLUSIONS: GM1 protects the rat retina from pressure-induced ischemic injury when given intravitreally after the insult. The protection provided by GM1 after initiation of retinal damage could be of therapeutic interest.

Animals↗

Fine needle tissue aspiration for accurate localization of histological specimens from complex structures.

A procedure is presented for exact, detailed comparison of light and electron microscopic analyses of tissues with complex architecture. Earlier techniques require one to make drawings of tissue pieces to be analyzed by electron microscopy to permit rough localization of the origin of the tissue pieces. Specifically, exact analysis of fetal cartilage and bone is hampered by the complicated arrangement of both tissue components, severely limiting the assessment of electron microscopic analyses. The advantage of the technique described here is that it allows precise localization of the tissue sample in the original tissue area. Punches 1 min in diameter were obtained from femora and coxae with a syringe and embedded for light and electron microscopy. The remaining tissue with its exactly defined punctures is prepared for standard histology. Human fetal cartilage and bone tissue were used to demonstrate this technique, but this procedure may be used for other kinds of tissues.

Biopsy, Needle↗

[Not Available].

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Academies and Institutes↗

[Does the dosage of mitomycin-C applied during filtering surgery of glaucoma vary with the individual surgeon?].

PURPOSE: The purpose of this study was to estimate the quantity of mitomycin-C contained in sponges prepared by different surgeons as compared to that in applicators of the type used in Schirmer's test graduation (5 x 5 mm). MATERIALS AND METHODS: Four surgeons each cut and prepared 10 sponges for intraoperative use according to their usual method. The same procedure was performed with 10 Schirmer's test graduations. Each sponge and each graduation was immersed in a solution of mitomycin-C 0.2 mg/ml, and the quantity of antibiotic (microgram) in each was calculated as the difference between wet and dry weight. RESULTS: The mean quantity (+/-SEM) of mitomycin-C contained in cut sponges was 9.6 +/- 4.4 micrograms (range 1.9-17.3), and differences between surgeons were statistically significant (p < 0.0001). The mean quantity of antibiotic in Schirmer's test graduation was 1.7 +/- 0.3 micrograms (range 1.1-2.5), and the differences between surgeons were not statistically significant (p = 0.79). CONCLUSION: The quantities of mitomycin-C contained in sponges prepared for glaucoma surgery differed for a given surgeon and between surgeons. Thus, variations in the doses applied to the sclera could account for certain complications due to mitomycin-C. The use of a Schirmer's test graduation improves predictability for the quantity of mitomycin-C applied to the sclera.

Antimetabolites↗

Bullous pemphigoid in association with cutaneous lesions specific to a myelodysplastic syndrome.

Specific cutaneous lesions are a rare occurrence in myelodysplastic syndromes (MDS). The concurrent association of blistering skin lesions similar to those in bullous pemphigoid (BP), even though a rare event, suggests that BP may be a paraneoplastic syndrome. We report an 86-year-old man who had a refractory anaemia with excess bone marrow blasts in transformation, who developed a generalized pruritic blistering eruption. Immunohistopathological tests showed subepidermal blisters with linear deposits of IgG and C3 along the basement membrane zone of the epidermis surrounding a tumoral dermal infiltrate of CD13+ and CD15+ cells. Immunoblotting studies using epidermal extracts revealed circulating IgG antibodies against three protein bands: a 210-215 kDa band, a 180 kDa band which co-migrated with the BP 180 antigen, and a 190 kDa band. The tumour infiltrate may have revealed antigenic determinants which led to the onset of BP. The concept of paraneoplastic pemphigoid remains to be either confirmed or invalidated by further epidemiological studies.

Aged↗

Metabolic factors clustering, lipoprotein cholesterol, apolipoprotein B, lipoprotein (a) and apolipoprotein E phenotypes in premature coronary artery disease in French Canadians.

Plasma lipoprotein cholesterol abnormalities, diabetes, hypertension and smoking have all been identified as independent predictors of cardiovascular events. Clustering of multiple risk factors suggests a common metabolic link among high blood pressure, insulin resistance, plasma lipoprotein abnormalities and obesity. New guidelines for the management of dyslipidemias target patients with established coronary artery disease (CAD), and high risk patients with multiple risk factors and severe genetic lipoprotein disorders, such as familial hypercholesterolemia. To determine the prevalence of lipoprotein, apolipoprotein and metabolic disorders in premature CAD, 243 men and 61 women with premature CAD (occurring before age 60 years) and 203 age- and sex-matched controls (152 men, 61 women) were studied. After correcting for beta-blocker use (40% of men and 54% of women), hypertension and diabetes were seen more frequently in CAD patients than in controls. In men and women, cholesterol, triglycerides, low density lipoprotein (LDL) cholesterol, apolipoprotein B and lipoprotein (a) were significantly higher, and high density lipoprotein (HDL) cholesterol was lower, in CAD patients than in controls. By stratifying patients according to LDL cholesterol: HDL cholesterol ratio (5 or less, or greater than 5) and by triglyceride levels (less than 2.3 mmol/L, or 2.3 mmol/L or greater), significantly more men and women with CAD were found to have an elevated LDL cholesterol:HDL cholesterol ratio and elevated triglycerides (13.8% versus 1.9%, men and women combined, CAD versus controls, P < 0.0001). A metabolic factor index was devised, assigning a score of 1 each for presence of hypertension, lipoprotein abnormalities, diabetes or fasting blood glucose above 7.0 mmol/L, and a body mass index of 27 or greater. The prevalence of a metabolic factor index of 3 or more was 29.2% in CAD men versus 6.7% in controls (P < 0.0001) and 38.3% in CAD women versus 11.7% in controls (P < 0.01). Familial hypercholesterolemia was seen in fewer than 5% of patients with premature CAD and type III dyslipoproteinemia in one of 343 CAD patients. The distribution of apolipoprotein E phenotypes was the same in CAD patients and controls. Multivariate analysis revealed that in men, HDL cholesterol, lipoprotein (a) levels and smoking were the best predictors of risk. In men, plasma levels of LDL cholesterol, triglycerides or body mass index did not enter the model at the P < 0.05 level. In women, low HDL cholesterol, lipoprotein (a), the presence of diabetes, smoking and apolipoprotein B levels were all predictors of risk (P < 0.05). However, the clustering of risk factors may be the best predictor of risk. In this selected population, HDL and lipoprotein (a) are the best metabolic markers of premature CAD; metabolic factor clustering is common in patients with premature CAD.

Adult↗

Role of delayed cellular hypersensitivity and adhesion molecules in amoxicillin-induced morbilliform rashes.

BACKGROUND: Morbilliform rashes induced by amoxicillin are though to be caused by a delayed cell-mediated immune reaction. The importance of amoxicillin skin tests is not well defined. A better understanding of the mechanisms of amoxicillin-induced morbilliform rashes can be obtained by performing cutaneous immunohistological studies on specimens from amoxicillin-induced morbilliform rashes and positive amoxicillin skin test results. OBSERVATIONS: Skin biopsy specimens were obtained from 5 patients who had developed an amoxicillin-induced morbilliform rash. All patients underwent amoxicillin prick, patch, and intradermal tests. Similar immunohistological investigations were performed on amoxicillin-induced morbilliform rashes and positive skin test biopsy specimens, with a special focus on the expression of adhesion molecules. Three of the 5 patients developed delayed positive results to intradermal and patch tests and 2 patients developed delayed positive results to prick tests. Amoxicillin-induced morbilliform rashes were well reproduced by skin tests, with similar immunohistological results in amoxicillin-induced morbilliform rashes and skin test biopsy specimens. Keratinocytes were activated and expressed CD54 (intercellular adhesion molecule 1); perivascular lymphocytes were mostly CD2+, CD3+, and CD4+ and exhibited CD11a through CD18 (leukocyte function-associated antigen 1) and often HLA-DR and/or CD62L (leukocyte endothelial cell adhesion molecule 1); and endothelial cells were activated with a strong expression of CD54 (intercellular adhesion molecule 1), CD62E (endothelial leukocyte adhesion molecule 1), and CD31 (platelet endothelial cell adhesion molecule 1) in lesser amounts. CONCLUSIONS: Findings of this clinical and immunohistochemical study support the theory of a T-cell-mediated immune reaction in patients with amoxicillin-induced morbilliform rashes, with a strong involvement of adhesion molecules both on endothelial and infiltrating cells. Our findings emphasize the importance of delayed readings of amoxicillin prick, intradermal, and patch tests.

Amoxicillin↗

[Radiology information systems: improved performance evaluation, economics and quality assurance?].

By means of complete service control and standardized accounting processes, radiological information systems clearly contribute to improved results. They provide the prerequisites for the establishment of expanded networks and allow comparisons with comparable institutions. The quality of patient care can be improved since, for example, the production time from referral to finished result becomes shorter. Direct access to patient and findings data from several positions is possible. Preliminary results can be viewed immediately. The patient's history is accessible to authorized users at all times. The exact reproducibility and assignment of services leads to more clarity. By means of the information available form RIS, rapid adaptive processes can be undertaken. The system assists the to fulfill the requirements of health regulations. The above-mentioned relationships demonstrate that the EDP systems are well accepted by physicians, medical assistants, and administrators and represent an indispensable aid for solving problems.

Cost-Benefit Analysis↗

Presumptive red maple (Acer rubrum) toxicosis in Grevy's zebra (Equus grevyi).

Two female Grevy's zebras (Equus grevyi), one juvenile and one adult, were treated for hemolytic anemia. The juvenile survived, but the adult animal, which also had methemoglobinemia, was euthanized after it failed to recover from anesthesia. Significant pathologic findings in the adult zebra included generalized icterus, hemoglobinuric nephrosis, and paracentral hepatic necrosis. Serum titers for known infectious causes of anemia were negative. Examination of the zebra holding areas revealed two hybrid red maple (Acer sp.) trees. There was no known exposure to other hemolytic agents. This is the first report of probable red maple-induced hemolysis in zebra.

Anemia, Hemolytic↗

[Etiology and pathogenesis of spondylolytic spondylolisthesis].

The commonly accepted hypothesis set up by Brocher in 1950 that the dysplasia of the pars interarticularis vertebrae is an essential requirement for the occurrence of spondylolysis should be thought over in light of recent findings. Newly published investigations show that disturbed spinal growth leads to dysplasia of the vertebral arch and to spondylolysis. Disturbances that play an important role are biomechanical stress factors that result from the evolution from a quadripedal to a bipedal human locomotion as well as stress fractures of the vertebral pars inter-articularis and defects in vertebral maturation in small infants.

Adult↗

[MNGIE syndrome in 2 siblings].

Two siblings (one man, one woman), presenting with diarrhea, severe weight loss peripheral neuropathy, ophthalmoparesis, asymptomatic leukoencephalopathy were diagnosed as a new cases of Mitochondrial Neuro Gastro Intestinal Encephalomyopathy syndrome (MNGIE). Hirano (1994) defined four criteria for the diagnostic: peripheral neuropathy, ophthalmoparesis, gastro intestinal dysmotility, muscle biopsy with histologic features of mitochondrial myopathy (ragged-red fibers, muscle fibers with increased succinate deshydrogenase stain or ultra structurally abnormal mitochondria). In a review of the literature, we found 31 cases with MNGIE. With our two cases, we study this group of 33 patients. First symptoms begin about 13.5 years with a median of 10 years and extremes for 1 to 32 years. The first signs are gastro intestinal symptoms (recurrent nausea, vomiting or diarrhea with intestinal dysmotility) in 22 cases, an ophthalmoparesia in 4 cases, intestinal and ocular signs in 1 case, gait ataxia or peripheral neuropathy in 3 cases, hearing loss in 1 case, gait ataxia or peripheral neuropathy in 3 cases, hearing loss in 1 case. During the evolution, besides the cardinal signs, the following features have been observed with a variable frequency: hearing loss, short stature, facial palsy, dysphonia, dysarthria, sweating, orthostatic hypotension, bladder dysfunction, hepatomegalia, The laboratory features are: abnormal Nerve Condition Studies/EMG compatible with a sensory motor neuropathy, lactic acidosis, mitochondrial respiratory chain defect (essentially complex IV deficiency, complex I deficiency or multiple complex defect), MRI leukodystrophy, elevated CSF protein, heart block, ragged-red fibers or increased SDH stain. The prognosis is poor, due to a severe weight loss bordering on cachexia 13 patients died with a mean age of 28.5 years (median 24 years, extreme 3 years to 51 years). The prognosis seems to be worsened by a young age of onset. The 33 patients belong to 19 families with 7 cases of consanguinity. 25 patients had a brother, a sister or a cousin affected. The study of these families is compatible with an autosomic recessive transmission, suggesting a pathology of the nuclear genomi, probably impliying the control of the mitochondrial DNA replication. In fact, in 13 cases, a study of the mt DNA was realized: multiple deletions were founded in 6 cases, multiples mutations in one case, unique mutation in 1 case. In 5 cases ther was no evidence of abnormality. These precise etiology and pathophysiologic significance of the mt DNA deletions, and the heterogeneity of the modifications of the mt DNA remain unknown. However, the possibility of various phenotypes for a same genotype or inversely is known in mitochondriopathies.

Adult↗

[Chronic sclerodermiform syndrome disclosing subcutaneous T-cell lymphoma].

INTRODUCTION: Subcutaneous tissue is an uncommon primary localization for T-cell lymphomas. Panniculitis with recurrent papulonodules are reported in most cases. CASE REPORT: We report a case in which small-cell pleiomorphic non-Hodgkin lymphoma developed initially in subcutaneous tissue followed by secondary systemic extension. The first clinical manifestation was a sclerodermiform syndrome involving the four limbs. DISCUSSION: This type of lymphoma appears to have a polymorphic clinical presentation. Physicians should be aware of this type of lymphoma and use immunohistological techniques for early diagnosis.

Fatal Outcome↗

[Congenital skin defect and fetus papyraceus. A case].

BACKGROUND: Congenital skin defect is an uncommon condition. The term of "congenital skin aplasia" should be avoided since the origin may not necessarily be congenital malformation. The scalp is involved in 80 p. 100 of the cases. CASE REPORT: We observed a congenital skin defect located exclusively on the trunk. The infant lacked wide areas of skin in symmetrical star-like configurations. DISCUSSION: The notion of a twin expulsed after 4 months gestation suggested the diagnosis of congenital skin defect and fetus papyraceus or group V congenital skin aplasia in the Frieden classification as recalled. This diagnosis must not be overlooked as the prognosis is good. Closure with atrophic skin usually occurs within a few weeks. The etiopathogenesis remains obscure.

Diseases in Twins↗