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Biomedical subjects

S J Bird

Publications and source records attributed to S J Bird.

At least 19 recordsLinked to original sources
PubMed2001

Acute axonal neuropathy in maple syrup urine disease.

A 25-year-old woman with maple syrup urine disease (MSUD) developed generalized weakness over 1 week. She had severe leg and moderate arm weakness, areflexia, and distal sensory loss. Plasma branched-chain amino acid concentrations were elevated, reflecting an acute exacerbation of the disease. Electrodiagnostic studies indicated an acute axonal polyneuropathy and sural nerve biopsy revealed acute wallerian degeneration without inflammation. Peripheral neuropathy, although not identified previously as a clinical feature of MSUD, may become more common as chronic dietary restrictions and improved management of the disease allow survival into adulthood.

Acute Disease↗
PubMed2001

Mentors, advisors and supervisors: their role in teaching responsible research conduct.

Although the terms mentor and thesis advisor (or research supervisor) are often used interchangeably, the responsibilities associated with these roles are distinct, even when they overlap. Neither are role models necessarily mentors, though mentors are role models: good examples are necessary but not sufficient. Mentorship is both a personal and a professional relationship. It has the potential for raising a number of ethical concerns, including issues of accuracy and reliability of the information conveyed, access, stereotyping and tracking of advises, and the abuse of power. Nevertheless, mentors can be critically important for professional success and are one of a number of elements that affect the responsible conduct of research. In addition, the community as a whole has a responsibility to mentor junior members.

Ethics, Professional↗
PubMed2001

Scientific certainty: research versus forensic perspectives.

The scientific community and the judicial system are different components of society with different structures and functions. Nevertheless, science can contribute relevant and useful information to judicial deliberations if the inherent limitations of that information are understood. These limitations stem from the way the information is presented and perceived both by those who are providing it and those who are providing the context in which it is presented.

Conflict of Interest↗
PubMed2000

Ross syndrome plus: beyond horner, Holmes-Adie, and harlequin.

BACKGROUND: Ross syndrome is an uncommon disorder characterized by the triad of segmental anhidrosis, hyporeflexia, and tonic pupils. METHODS: The authors describe the clinical findings of five patients with Ross syndrome and detail the results of their pharmacologic and autonomic testing. RESULTS: In four patients, the classic findings of Ross syndrome were accompanied by Horner's syndrome. Other symptoms of dysautonomia were also common. CONCLUSIONS: These findings suggest that Ross syndrome is a dysautonomic condition of varying expression resulting from a generalized injury to ganglion cells or their projections.

Adolescent↗
PubMed2000

Chronic multiple paraneoplastic syndromes.

A patient presented with symptoms of limbic and brainstem encephalitis, motor and sensory neuronopathy, cerebellar dysfunction, and highly positive anti-Hu antibodies. He also harbored P/Q-type calcium channel antibodies and manifested the Lambert-Eaton myasthenic syndrome (LEMS). Small-cell lung cancer was found, and he received both antineoplastic therapy and intravenous immunoglobulin (IVIg). Remission of the malignancy was achieved. Although the anti-Hu-related manifestations improved after therapy, LEMS has persisted, leading to IVIg dependency.

Autoantibodies↗
PubMed2000

Ethical issues in communicating science.

Most of the publicized work on scientific ethics concentrates on establishing professional norms and avoiding misconduct. The successful communication of science is the responsibility of all involved in the process. In one study, the increased incidence of autism and other social developmental disorders in males was investigated by examining individuals with Turner's syndrome (XO females). In the national newspaper this became "Genetic X-factor explains why boys will always be boys". The steps by which a study on developmental disorders, published in a highly prestigious journal, was transformed into an article in the science section which 'explained' the socially expected gender-based behavior of genetically normal children are fascinating and, unfortunately far too typical. The scientists wrote an excellent article that has just one sentence at the end that hesitantly suggests that the findings might, with further study, have some relevance to understanding normal behavior. The general interest article in the front of the journal gave a good account of the research, but suggested more strongly that there could be an in-built biological dimorphism in social cognition. This was misrepresented in the press as proof of gender differences that "undermines the trend towards sexual equality", and both illustrates cultural bias and provides fodder for feminist critiques of science. The study has been made to appear to be biased in favor of justifying the social structure of society, and yet it was the translation from the scientific study to national news that produced this transformation to biased genetic determinism. It is poor communication of the actual science, coupled with a lack of skepticism on the part of the public, that contributes to such a misapplication of science. Scientists should resist the urge to generalize their results to make them more compelling. The science community should not allow misconstructions of scientific facts to go unchallenged. Journalists, for both the scientific publication and the newspaper, should resist the inclination to embellish the finding with social significance that is not present. For their part, readers must be doubly skeptical of any finding that appears to underwrite any current social hierarchy. We are all responsible for a communication and interpretation of science that is as accurate and socially responsible as possible.

Communication↗
PubMed2000

Of mice and men (and women and children): scientific and ethical implications of animal models.

1. Animal models of human behavior and disease are commonly used and have contributed significantly to progress in understanding the physiological mechanisms of both normal function and disease, and in the development of effective therapies. 2. Little attention has been given, however, to the scientific and ethical implications of choosing a particular animal model. 3. This paper discusses the rationale for the selection of particular animal models that have been chosen to study certain human diseases or behaviors, and provides examples to illustrate how underlying assumptions about methods and about physiological mechanisms and other relevant features of the disease or behavior of interest are embedded in the choice of an animal model. 4. Although these assumptions influence the direction of research, they are rarely analyzed explicitly, or evaluated empirically. The authors recommend that assumptions should be clearly stated and that, whenever possible, they be specifically and thoroughly evaluated empirically.

Animal Testing Alternatives↗
PubMed1999

Serial evoked potential studies and MRI imaging in chronic progressive multiple sclerosis.

Measurements of serial evoked potential latencies and plaque burden on MRI scans are often obtained during clinical studies of multiple sclerosis patients to provide additional information to the disability-based primary endpoints. The ideal laboratory-based marker of progression would be expected to significantly change over the time period of study. Serial visual (VEP) and brainstem auditory evoked potentials (BAEP) and MRI scans of 11 chronic progressive MS patients were obtained over a 1.5 year period in a clinical study. Over this period, there was no significant change in disability as measured by the Kurtzke EDSS, Ambulation Index or Neurological Rating Score. The VEP P100 significantly progressed over the period of study. However, the MRI T(2) plaque burden and BAEP I-V intrapeak latency did not significantly progress over the 1.5 years. We conclude that, in chronic progressive MS, serial visual evoked potential tests may complement standard disability-based endpoints to assess disease progression.

Disability Evaluation↗
PubMed1999

A double-blind, placebo-controlled trial of extracorporeal photopheresis in chronic progressive multiple sclerosis.

Extracorporeal photopheresis is a safe therapy for cutaneous T-cell lymphoma and may have efficacy in certain autoimmune disorders. We performed a randomized, double-blinded, placebo-controlled trial of monthly photopheresis therapy in 16 patients with clinically definite multiple sclerosis (MS). All patients had progressed during the preceding year with entry Expanded Disability Status Scale (EDSS) scores between 3.0 and 7.0. Patients received photopheresis or sham therapy for 1 year and were followed for an additional 6 to 12 months. Patients were clinically evaluated by three disability scales: (1) EDSS; (2) Ambulation index and (3) Scripp's quantitative neurologic assessment. No serious side effects occurred in either group. There were no differences between the photopheresis and sham therapy groups by the disability measures. Additionally, there were no differences in progression of MRI plaque burden or evoked potential latencies. In this limited study, photopheresis was found to be safe but did not significantly alter the course of chronic progressive MS.

Adult↗
PubMed1998

Bulbocavernosus reflex studies and autonomic testing in the diagnosis of erectile dysfunction.

The electrophysiologic evaluation of patients with erectile dysfunction presents an important diagnostic challenge. The bulbocavernosus reflex (BCR) latency has been commonly used to evaluate these disorders. However, it is a measure of somatic penile innervation, whereas erection is primarily dependent on autonomic function. We evaluated 195 men with erectile dysfunction over a 3 year period. Each had electrophysiologic studies, nerve conduction studies and a BCR. BCR studies were abnormal in only 7%, of which most had diabetes or pelvic trauma. The BCR was the sole electrophysiologic abnormality in only 2%. Autonomic testing (AT) was additionally performed in 19 diabetic and 23 non-diabetic patients. This included sympathetic skin responses and measurement of the Valsalva ratio and heart rate variability with 6/min breathing. In the diabetic group, AT was positive in 63%, and most often was the sole abnormality. The bulbocavernosus reflex is relatively insensitive in the diagnosis of erectile dysfunction. Brief autonomic testing may provide valuable additional data, particularly in diabetics.

Adult↗