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Biomedical subjects

J Gilbert

Publications and source records attributed to J Gilbert.

At least 55 records · Page 3Linked to original sources
PubMed2000

Phospholemman is a substrate for myotonic dystrophy protein kinase.

The genetic abnormality in myotonic muscular dystrophy, multiple CTG repeats lie upstream of a gene that encodes a novel protein kinase, myotonic dystrophy protein kinase (DMPK). Phospholemman (PLM), a major membrane substrate for phosphorylation by protein kinases A and C, induces Cl currents (I(Cl(PLM))) when expressed in Xenopus oocytes. To test the idea that PLM is a substrate for DMPK, we measured in vitro phosphorylation of purified PLM by DMPK. To assess the functional effects of PLM phosphorylation we compared I(Cl(PLM)) in Xenopus oocytes expressing PLM alone to currents in oocytes co-expressing DMPK, and examined the effect of DMPK on oocyte membrane PLM expression. We found that PLM is indeed a good substrate for DMPK in vitro. Co-expression of DMPK with PLM in oocytes resulted in a reduction in I(Cl(PLM)). This was most likely a specific effect of phosphorylation of PLM by DMPK, as the effect was not present in oocytes expressing a phos(-) PLM mutant in which all potential phosphorylation had been disabled by Ser --> Ala substitution. The biophysical characteristics of I(Cl(PLM)) were not changed by DMPK or by the phos(-) mutation. Co-expression of DMPK reduced the expression of PLM in oocyte membranes, suggesting a possible mechanism for the observed reduction in I(Cl(PLM)) amplitude. These data show that PLM is a substrate for phosphorylation by DMPK and provide functional evidence for modulation of PLM function by phosphorylation.

Animals↗
PubMed2000

Successful management of coronary artery perforation with helical platinum microcoil embolization.

A case of small coronary artery perforation during coronary intervention is presented. Continued leakage occurred despite prolonged intracoronary balloon inflation, in part probably related to the use of glycoprotein (GP) IIB/IIIA inhibitors. It was successfully managed by microcoil embolization without any sequel, helping avoid surgery in a high-risk patient. Cathet. Cardiovasc. Intervent. 51:320-322, 2000.

Aged↗
PubMed2000

Transient myeloproliferative disease of the newborn: case report with placental, cytogenetic, and flow cytometric findings.

Transient myeloproliferative disease (TMD) of the newborn is a rare hematologic abnormality associated with trisomy 21. It is frequently difficult to distinguish the disorder from true congenital leukemia (TCL). Unlike leukemia, which has a clinically aggressive course, TMD generally resolves within weeks to months. We present a case of TMD of the newborn diagnosed on the basis of peripheral blood studies and describe the pertinent pathological findings within the placenta. Flow cytometric analysis of the blasts in the peripheral blood showed phenotypic heterogeneity with features consistent with megakaryocytic differentiation. Cytogenetic studies showed trisomy 21 within the blastic cells. The placenta showed villous dysmaturity with associated chorangiosis and prominent intravascular aggregates of primitive-appearing cells with focal, early vascular wall invasion. The neonate recovered fully and shows no evidence of disease at 2 years of age.

Adult↗
PubMed2000

Fire deaths in children in South Australia from 1989 to 1998.

A study was undertaken of childhood deaths caused by fires in South Australia over a 10-year period from 1989 to 1998. A total of 23 deaths of children occurred with an age range of 2 months to 16 years (mean 5 years 10 months; M:F = 13:10). Fourteen deaths were associated with house fires, four with fires in cars and four were miscellaneous or unspecified. While house fire deaths remained the major cause of childhood fire deaths (65%), deaths in car fires accounted for a significant proportion of cases (17%). Although the numbers are small, cars represented a specific danger because of their confined space with highly flammable interiors, lockable doors, and built in non-childproof lighters. Cars should not be regarded as suitable places to leave young children, or for children to play in unsupervised.

Adolescent↗
PubMed2000

European priorities for research to support legislation in the area of food contact materials and articles.

A strong science base is required to underpin the planning and decision-making process involved in determining future European community legislation on materials and articles in contact with food. Significant progress has been made in the past 5 years in European funded work in this area, with many developments contributing to a much better understanding of the migration process, and better and simpler approaches to food control. In this paper this progress is reviewed against previously identified work-areas (identified in 1994) and conclusions are reached about future requirements for R&D to support legislation on food contact materials and articles over the next 5 or so years.

Decision Making↗
PubMed2000

An Internet compendium of analytical methods and spectroscopic information for monomers and additives used in food packaging plastics.

An internet website (http:¿cpf.jrc.it/smt/) has been produced as a means of dissemination of methods of analysis and supporting spectroscopic information on monomers and additives used for food contact materials (principally packaging). The site which is aimed primarily at assisting food control laboratories in the European Union contains analytical information on monomers, starting substances and additives used in the manufacture of plastics materials. A searchable index is provided giving PM and CAS numbers for each of 255 substances. For each substance a data sheet gives regulatory information, chemical structures, physico-chemical information and background information on the use of the substance in particular plastics, and the food packaging applications. For monomers and starting substances (155 compounds) the infra-red and mass spectra are provided, and for additives (100 compounds); additionally proton NMR are available for about 50% of the entries. Where analytical methods have been developed for determining these substances as residual amounts in plastics or as trace amounts in food simulants these methods are also on the website. All information is provided in portable document file (PDF) format which means that high quality copies can be readily printed, using freely available Adobe Acrobat Reader software. The website will in future be maintained and up-dated by the European Commission's Joint Research Centre (JRC) as new substances are authorized for use by the European Commission (DG-ENTR formerly DGIII). Where analytical laboratories (food control or other) require reference substances these can be obtained free-of-charge from a reference collection housed at the JRC and maintained in conjunction with this website compendium.

Data Display↗
PubMed1999

Altered expression of the MYCN oncogene modulates MRP gene expression and response to cytotoxic drugs in neuroblastoma cells.

We have recently shown a close correlation between expression of the Multidrug Resistance-associated Protein (MRP) gene and the MYCN oncogene and provided evidence that high MRP expression is a powerful independent predictor of poor outcome in neuroblastoma (Norris et al., New Engl. J. Med., 334, 231-238, 1996). The effect of MYCN down-regulation on MRP expression and response to cytotoxic drugs was investigated in NBL-S neuroblastoma cells transfected with MYCN antisense RNA constructs. Concomitant with MYCN down-regulation, the level of MRP expression was decreased in the NBAS-4 and NBAS-5 antisense transfectants. These cells demonstrated significantly increased sensitivity to the high affinity MRP substrates vincristine, doxorubicin, sodium arsenate and potassium antimony tartrate, but not to the poor MRP substrates, taxol or cisplatin. Similarly, transfection of full-length MYCN cDNA into SH-EP neuroblastoma cells resulted in increased MRP expression and significantly increased resistance specifically to MRP substrates. The results provide evidence for the MYCN oncogene influencing cytotoxic drug response via regulation of MRP gene expression. Our data also provide a link between the malignant and chemoresistant phenotypes of this childhood malignancy.

ATP-Binding Cassette Transporters↗
PubMed1999

Overview of mycotoxin methods, present status and future needs.

This article reviews current requirements for the analysis for mycotoxins in foods and identifies legislative as well as other factors that are driving development and validation of new methods. New regulatory limits for mycotoxins and analytical quality assurance requirements for laboratories to only use validated methods are seen as major factors driving developments. Three major classes of methods are identified which serve different purposes and can be categorized as screening, official and research. In each case the present status and future needs are assessed. In addition to an overview of trends in analytical methods, some other areas of analytical quality assurance such as participation in proficiency testing and reference materials are identified.

Chemistry, Analytic↗
PubMed1999

A randomized trial of ecadotril versus placebo in patients with mild to moderate heart failure: the U.S. ecadotril pilot safety study.

OBJECTIVE: To determine the short-term safety and tolerability of the addition of ecadotril to conventional therapy in patients with mild to moderate heart failure. METHODS: Fifty ambulatory patients, 18 to 75 years of age, with mild to moderate heart failure, left ventricular ejection fraction </=35%, taking stable doses of angiotensin-converting enzyme inhibitor, diuretics, and optionally digoxin were enrolled in a randomized, double-blind, placebo-controlled dose-escalation study of ecadotril 50 to 400 mg twice daily versus conventional therapy alone. RESULTS: No increases in deaths, serious adverse events, or dropouts from adverse events were observed for the ecadotril group compared with placebo. The serum measures of neurohormonal activation were highly variable. Changes in signs and symptoms of heart failure, New York Heart Association class, and patient self-assessment of symptoms were not observed with ecadotril therapy; however, the study was not designed to detect differences in these parameters. CONCLUSION: In this small pilot study, ecadotril in doses of 50 to 400 mg twice daily was generally well-tolerated and without severe short-term adverse effects in patients with mild to moderate heart failure. Evaluation of the clinical efficacy and long-term safety of ecadotril and other neutral endopeptidase inhibitors in patients with heart failure requires further study.

Adolescent↗
PubMed1999

Use of tumor-specific gene expression for the differential diagnosis of neuroblastoma from other pediatric small round-cell malignancies.

The differential diagnosis of neuroblastoma from other small round-cell tumors of childhood, although clinically of great importance, is sometimes difficult due to the almost indistinguishable appearance of such tumors by conventional microscopy. Because neuroblastomas are characterized by the synthesis of catecholamines, we investigated the possibility that expression of genes involved in this pathway could serve as a molecular marker for this disease. A reverse transcriptase polymerase chain reaction assay was used to analyze expression of tyrosine hydroxylase and dopa decarboxylase in 84 pediatric malignancies including 55 neuroblastomas, 6 Ewing's sarcomas/primitive neuroectodermal tumors, 7 lymphomas, 6 leukemias, 2 rhabdomyosarcomas, 6 osteosarcomas, and 2 phaeochromocytomas. Of the 55 neuroblastoma samples analyzed, 54 expressed clearly detectable levels of both genes. The one sample that did not express either of the genes was rediagnosed both clinically and by molecular genetic analysis as a Ewing's sarcoma. Of the 29 non-neuroblastoma tumor samples examined, the only tumor samples that expressed clearly detectable levels of both tyrosine hydroxylase and dopa decarboxylase were phaeochromocytomas. Like neuroblastomas, these tumors are characterized by high levels of catecholamines. These findings suggest that expression of genes involved in catecholamine biosynthesis may be useful for differentiating neuroblastoma from other small round-cell tumors of childhood.

Child, Preschool↗
PubMed1999

Posttraumatic diaphragmatic hernia and death--etiologic factor or putrefactive artifact?

Late-presenting diaphragmatic hernias, whether congenital or acquired, may remain clinically undetected until mediastinal shift with cardiorespiratory compromise or intestinal or gastric infarction with perforation occur. A right-sided diaphragmatic hernia with herniation of small intestine into the adjacent pleural cavity is described in a 72-year-old man who was found dead at home in a putrefactive state. Subsequently, a history of a motor vehicle accident 8 years previously was obtained. Although postmortem herniation due to extensive putrefactive change may have occurred, producing gaseous distention and migration of the intestine, tight twisting of the herniated intestine several times around a pleural fibrous adhesion suggested that the herniation could have been an antemortem event. Unfortunately, marked tissue autolysis prevented assessment of possible ischemic changes in the herniated intestine. Although diaphragmatic hernia may be a contributing factor to death at all ages, it may not be possible to exclude it as an artifact of putrefaction in a predisposed individual.

Aged↗
PubMed1999

Pathologic features of farm and tractor-related fatalities in children.

A study of 15 childhood fatalities due to farm or tractor-related accidents demonstrated extensive injuries typified by crushing, evisceration, and amputation of limbs. Although these injuries are characteristic of industrial accidents in adults, such accidents do not commonly occur in children. However, the unique circumstance of the farm, which incorporates home and industrial environments, results in particularly severe patterns of injuries in accidental childhood deaths. A knowledge of the range of machines that are used and the environment of the farm facilitates assessment of the types of injuries that may be found at autopsy in cases of pediatric farm accident.

Accidents, Occupational↗
PubMed1999

Murder-suicides involving children: a 29-year study.

Review was undertaken from February 1969 to January 1998 at the State forensic science center (Forensic Science) in Adelaide, South Australia, of all cases of murder-suicide involving children <16 years of age. A total of 13 separate cases were identified involving 30 victims, all of whom were related to the perpetrators. There were 7 male and 6 female perpetrators (age range, 23-41 years; average, 31 years) consisting of 6 mothers, 6 father/husbands, and 1 uncle/son-in-law. The 30 victims consisted of 11 daughters, 11 sons, 1 niece, 1 mother-in-law, and 6 wives of the assailants. The 23 children were aged from 10 months to 15 years (average, 6.0 years). The 6 mothers murdered 9 children and no spouses, with 3 child survivors. The 6 fathers murdered 13 children and 6 wives, with 1 child survivor. This study has demonstrated a higher percentage of female perpetrators than other studies of murder-suicide. The methods of homicide and suicide used were generally less violent among the female perpetrators compared with male perpetrators. Fathers killed not only their children but also their wives, whereas mothers murdered only their children. These results suggest differences between murder-suicides that involve children and adult-only cases, and between cases in which the mother rather than the father is the perpetrator.

Adolescent↗
PubMed1999

Managing congestive heart failure with thoracic electrical bioimpedance.

Because of clinical utility, immediate feedback, absence of risk to the patient, portability, and use in virtually any practice setting, facilities using thoracic electrical bioimpedance monitoring are increasing throughout the world. Specialized centers treating patients with heart failure have shown remarkable benefits, both in health care costs and patient morbidity, attributable to the careful monitoring and management of patients with heart failure. The ability of thoracic bioimpedance monitoring to provide objective data in the management of congestive heart failure provides practitioners with the clinical reassurance to optimize pharmacologic therapy safely.

Cost-Benefit Analysis↗