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Biomedical subjects

Michael Beck

Publications and source records attributed to Michael Beck.

42 records · Page 3Linked to original sources
PubMed2002

The heart in Anderson Fabry disease.

Anderson Fabry disease is a life threatening, X-linked inborn metabolic defect of the lysosomal enzyme áalpha-galactosidase A. The deficiency of alpha-galactosidase A leads to a progressive accumulation of globotriaosylceramide (Gb(3)), the major glycosphingolipid substrate of the enzyme, within vulnerable cells, tissues, and organs, including the cardiovascular system. Cardiac involvement is frequent and patients with cardiac affection develop progressive hypertrophic infiltrative cardiomyopathy, valvular abnormalities, arrhythmias, and conduction abnormalities and may develop coronary heart disease. Hemizygous male patients have no detectable alpha-galactosidase A activity, while affected heterozygous females may have normal level of alpha-galactosidase A activity. Death occurs in male patients at 45 to 50 years, about 15 to 20 years earlier than in female patients due to a vicious circle from chronic renal insufficiency, arterial hypertension, atherosclerotic lesions and cerebrovascular hemorrhage or insults, and cardiomyopathy. Cardiac involvement in hetero- and hemizygotes will be discussed as well as the influence of enzyme replacement of alpha-galactosidase A.

Adult↗
PubMed2002

Agalsidase alfa--a preparation for enzyme replacement therapy in Anderson-Fabry disease.

Anderson-Fabry disease is an X-linked multisystemic disorder caused by a genetic deficiency of the lysosomal enzyme a-galactosidase A. The enzyme is responsible for degradation of glycolipids inside the lysosomes. Lack of catalytic activity leads to progressive depositions of undegraded glycolipids in a large number of organs. Crises of severe pain in the extremities (acroparesthesias), hypohidrosis, corneal opacities and dysfunction of several organs (kidney, brain, heart) are the leading symptoms in patients with Anderson-Fabry disease. Females may have the same symptoms as males but to a more variable degree. The variable manifestations seen in heterozygotes can be explained by the Lyon hypothesis. This hypothesis predicts that in X-linked diseases, the carriers are a mosaic of normal and mutant cells in varying proportions and hence have variable expression. As in Gaucher's disease, enzyme replacement therapy recently became available for Anderson-Fabry disease. Two drugs have gained approval in the EU by the European Agency for the Evaluation of Medicinal Products. These are agalsidase beta (Fabrazyme, Genzyme Corporation) and agalsidase alfa (Replagal, Transkaryotic Therapies, Inc.). This review will describe clinical efficacy, safety and tolerability of agalsidase alfa.

Animals↗
PubMedDate not supplied

Alcoholism: low standing with the public? Attitudes towards spending financial resources on medical care and research on alcoholism.

AIMS: To assess the extent to which the German public supports the allocation of financial resources to the care of people with alcoholism and to research on alcoholism as compared with other conditions. METHODS: 5025 interviews were conducted in the scope of a representative survey in Germany during May and June of 2001, using a personal, fully structured interview. RESULTS: Respondents most frequently selected alcoholism as the disease for which medical care expenditures could be spared and cut down and on which research funds should neither be spent in the first place nor should be spent at all. CONCLUSION: Our study shows that despite the spread of the concept 'alcoholism is an illness', this disease is still treated 'unfavourably' compared to other conditions. Health campaigns that increase the public's awareness that alcoholism is not a personal failure but an illness with severe medical and social consequences may help reduce the public acceptance of structural discrimination.

Alcoholism↗
PubMedDate not supplied

Board certification status and pediatric dentists' practice characteristics.

PURPOSE: Board certification is often used as a surrogate indicator of provider competence and quality of care, although few studies have demonstrated its validity. The aim of this study was to assess the relationship between board certification status and a set of quality characteristics of pediatric dental practice. METHODS: A 30-item questionnaire was developed that collected information regarding practice characteristics in the areas of: (1) professional growth/practice management; (2) emergency readiness; (3) treatment guidelines utilization; (4) patient pool selection; (5) safety; and (6) behavior management. The questionnaire was mailed to 250 board-certified and 250 noncertified pediatric dentists paired by year and program of graduation. RESULTS: Overall, respondents-irrespective of pairing by program and year of graduation-tended to answer affirmatively or largely positively to most questions. Maintaining hospital privileges and having routinely CPR-certified staff were significantly related to the board certification status. When year of graduation and residency program attended was considered, however, this significance disappeared. In categories of treatment guidelines utilization, patient pool selection, safety protocols and behavior management, there was no significant difference between board certified and nonboard certified pediatric dentists (P > .05). CONCLUSIONS: Generally, pediatric dentists independent of certification status, practice at a high level of quality, as measured in this study.

Certification↗
PubMedDate not supplied

Attitudes of contemporary parents toward behavior management techniques used in pediatric dentistry.

PURPOSE: The objective of this study was to examine parental attitudes toward behavior management techniques currently used in pediatric dentistry. METHODS: Fifty-five parents viewed videotaped scenes of 8 behavior management techniques being used during actual pediatric dental treatment. The 8 techniques shown were: (1) tell-show-do; (2) nitrous oxide sedation; (3) passive restraint; (4) voice control; (5) hand-over-mouth; (6) oral premedication (sedation); (7) active restraint; and (8) general anesthesia. Parents rated their acceptance of each technique using a visual analogue scale (VAS). RESULTS: Forty-six parents completed survey forms for analysis. Tell-show-do was rated as the most acceptable technique, followed (in order of decreasing acceptance) by: (1) nitrous oxide sedation; (2) general anesthesia; (3) active restraint; (4) oral premedication; (5) voice control; (6) passive restraint; and (7) hand-over-mouth. The following groups emerged with statistically similar means: (1) tell-show-do and nitrous oxide sedation; (2) nitrous oxide sedation, general anesthesia, and active restraint; and (3) general anesthesia, active restraint, oral premedication, and voice control. Hand-over-mouth and passive restraint were rated as the least acceptable techniques, and the ratings for both techniques were significantly different from all other techniques and from each other. Overall, hand-over-mouth was the least acceptable technique. Acceptance of each behavior management technique was not related to parental age, gender, education level, or social status. CONCLUSIONS: The mean parental acceptance rating was in the acceptable range for all behavior management techniques examined in this study except for hand-over-mouth. General anesthesia was ranked as the third most acceptable technique. This high level of acceptance of general anesthesia compared to earlier studies may suggest that parental acceptance of this technique is increasing.

Adult↗