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Biomedical subjects

Heather Long

Publications and source records attributed to Heather Long.

8 recordsLinked to original sources

Alexander-disease mutation of GFAP causes filament disorganization and decreased solubility of GFAP.

Alexander disease is a fatal neurological illness characterized by white-matter degeneration and the formation of astrocytic cytoplasmic inclusions called Rosenthal fibers, which contain the intermediate filament glial fibrillary acidic protein (GFAP), the small heat-shock proteins HSP27 and alphaB-crystallin, and ubiquitin. Many Alexander-disease patients are heterozygous for one of a set of point mutations in the GFAP gene, all of which result in amino acid substitutions. The biological effects of the most common alteration, R239C, were tested by expressing the mutated protein in cultured cells by transient transfection. In primary rat astrocytes and Cos-7 cells, the mutant GFAP was incorporated into filament networks along with the endogenous GFAP and vimentin, respectively. In SW13Vim(-) cells, which have no endogenous cytoplasmic intermediate filaments, wild-type human GFAP frequently formed filamentous bundles, whereas the R239C GFAP formed 'diffuse' and irregular patterns. Filamentous bundles of R239C GFAP were sometimes formed in SW13Vim(-) cells when wild-type GFAP was co-transfected. Although the presence of a suitable coassembly partner (vimentin or GFAP) reduced the potential negative effects of the R239C mutation on GFAP network formation, the mutation affected the stability of GFAP in cells in a dominant fashion. Extraction of transfected SW13Vim(-) cells with Triton-X-100-containing buffers showed that the mutant GFAP was more resistant to solubilization at elevated KCl concentrations. Both wild-type and R239C GFAP assembled into 10 nm filaments with similar morphology in vitro. Thus, although the R239C mutation does not appear to affect filament formation per se, the mutation alters the normal solubility and organization of GFAP networks.

Actin Cytoskeleton↗

Medicinal use of cocaine: a shifting paradigm over 25 years.

OBJECTIVES/HYPOTHESIS: Human cocaine research is predicated on data from the clinical practice of otolaryngology that are more than 25 years old and predate both the cocaine epidemic and the first reported association between cocaine use and myocardial infarction. The authors' objective was to reassess the epidemiology and toxicity of medicinal cocaine use among otolaryngologists and to compare current trends in usage and safety data with previously reported data. STUDY DESIGN: An anonymous closed-question survey replicating the methodology of a previous study was used. METHODS: The survey was mailed to active members of the American Academy of Otolaryngology-Head and Neck Surgery. The survey used a closed-question format asking about the use of cocaine, safety measures taken, and adverse outcomes and included information about practice type and location. Results were compared with previously published data using a chi test with P < .05 considered significant. RESULTS: In all, 7815 surveys were mailed. Four thousand seventeen otolaryngologists returned the survey, representing a 54% response rate. Of the respondents, only 50% had used cocaine in their practice during the previous year. Physicians who had been in practice for less than 10 years were less likely to have used cocaine than those who had been in practice for more than 10 years (78% vs. 93% [P < .001]). Compared with the data reported in 1977, fewer physicians reported ever using cocaine in their practice (88% vs. 92% [P < .001]), fewer physicians had used cocaine in their practice at any time in the previous 10 years (68% vs. 92% [P < .001]), and a greater number of adverse reactions were reported by current respondents (26% vs. 22% [P < .001]). Tachycardia and hypertension were the most commonly reported adverse effects. Other important adverse events included 14 deaths, survivable cardiac arrest, ventricular tachycardia, and seizures. CONCLUSION: The clinical use of cocaine in otolaryngology has decreased significantly in the past 25 years as a result of discontinuation of use by physicians who had previously used cocaine and an increasing number of otolaryngologists who have never used it. This decline may reflect a better understanding of its potential toxicities, problems associated with storing and dispensing of a tightly controlled substance, increased availability of safer alternative medications, or a combination of these.

Administration, Inhalation↗

A fatal case of spongiform leukoencephalopathy linked to "chasing the dragon".

BACKGROUND: "Chasing the dragon" involves placing heroin on aluminum foil, heating it from below with a flame, and inhaling the pyrolysate through a straw. It has rarely been associated with the development of a progressive spongiform leukoencephalopathy. CASE REPORT: A 43-year-old woman presented with 2 weeks of bizarre behavior, forgetfulness, and slowed speech and movements. Serum, cerebrospinal fluid, and head computed tomography (CT) scan were normal. The patient progressed to coma and expired during week 4 of hospital admission. The family confirmed that she "chased the dragon." Cause of death at post mortem examination was spongiform leukoencephalopathy. CONCLUSION: The diagnosis of heroin pyrolysate-induced spongiform leukoencephalopathy should be considered in a patient with a history of "chasing the dragon" and neurobehavioral changes, including confusion, apathy, cerebellar signs, and motor restlessness.

Adult↗

Alpha-methyltryptamine revisited via easy Internet access.

Alpha-methyltryptamine (AMT) is a synthetic hallucinogenic indolealkylamine, that was initially studied as a monoamine oxidase inhibitor and used as an antidepressant. Although a popular hallucinogen in the 1960's, use of this drug is currently uncommon. We report a patient with sympathomimetic features who was found to have ingested AMT.

Adolescent↗

Evaluating patient acceptance of a teledermatology link of an urban urgent-care dermatology clinic run by residents with board certified dermatologists.

BACKGROUND: A teledermatology system was established in a dermatology urgent-care clinic staffed by dermatology residents in the emergency department. These residents had previously lacked attending dermatology supervision. METHODS AND PATIENTS: Resident physicians took digital pictures of patients' lesions and downloaded the images onto a network personal computer (PC). The images were stored on the hospital's server and then viewed within 5 minutes by an attending dermatologist, in a different location, using a network PC with monitor. Resident and attending physicians discussed the cases over the telephone. Patient acceptance was assessed via separate written surveys. Fifty-one patients, approximately 10 resident physicians, and two attending dermatologists participated in the study. RESULTS: Patient acceptance of the teledermatology system was high (93%). Teledermatology may prove a viable means of evaluating dermatologic complaints in the emergency department.

Adult↗