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Biomedical subjects

J B Sherman

Publications and source records attributed to J B Sherman.

At least 19 recordsLinked to original sources

Treatment of refractory myositis: a randomized crossover study of two new cytotoxic regimens.

OBJECTIVE: To assess the clinical usefulness of 2 novel therapies for treatment-resistant myositis. METHODS: Thirty patients with refractory myositis, of whom 25 had an inadequate or no response to previous cytotoxic therapy, were randomized to begin either a combination of weekly oral methotrexate and daily azathioprine (MTX/AZA) or intravenous methotrexate with leucovorin rescue (I.V. MTX) every 2 weeks for 6 months. Crossover to the alternate therapy occurred according to defined rules; evaluations of muscle strength and functional abilities were performed at the beginning, and after 3 and 6 months, of each treatment. RESULTS: Of the 15 patients initially randomized to oral MTX/AZA, 8 improved with oral therapy and 1 improved with I.V. MTX during the crossover period. Of the 15 patients initially randomized to I.V. MTX therapy, 3 improved with the I.V. therapy and 4 with the oral combination during the crossover period. Although the study lacked the power to directly compare both treatments, intention-to-treat analysis showed a trend in favor of those patients who first received oral combination therapy (P = 0.025). There were 0.09 adverse events per patient-month with oral combination therapy and 0.16 per patient-month with I.V. therapy (P = 0.09). CONCLUSION: Combination oral MTX/AZA may benefit patients with treatment-resistant myositis, including those who previously had inadequate responses to either MTX or AZA alone. I.V. MTX with leucovorin rescue may also benefit some patients with refractory myositis.

Administration, Oral↗

Mutations in muscle phosphofructokinase gene.

Mutations in the muscle phosphofructokinase gene (PFK-M) result in a metabolic myopathy characterized by exercise intolerance and compensated hemolysis. PFK deficiency, glycogenosis type VII (Tarui disease) is a rare, autosomal, recessively inherited disorder. Multiple mutations, including splicing defects, frameshifts, and missense mutations, have recently been identified in patients from six different ethnic backgrounds establishing genetic heterogeneity of the disease. There is no obvious correlation between the genotype and phenotypic expression of the disease. PFK-M deficiency appears to be prevalent among people of Ashkenazi Jewish descent. Molecular diagnosis is now feasible for Ashkenazi patients who share two common mutations in the gene; the more frequent is an exon 5 splicing defect, which accounts for approximately 68% of mutant alleles in this population.

Chromosome Mapping↗

Various classes of mutations in patients with phosphofructokinase deficiency (Tarui's disease).

Muscle phosphofructokinase (PFK-M) deficiency (glycogenosis type VII, Tarui's disease) is characterized by intolerance to vigorous exercise, often accompanied by myoglobinuria. The disease is inherited as an autosomal recessive trait. The clinical manifestations are similar to those in myophosphorylase deficiency (McArdle's disease), and the diagnosis required demonstration of the enzyme defect in muscle biopsy. In the Western hemisphere PFK deficiency appears to be prevalent among people of Ashkenazi Jewish descent. To define the molecular basis of this myopathy, we have studied 11 Ashkenazi and 2 non-Ashkenazi families with the disease. Ashkenazi patients share two common pathogenic mutations, a splicing defect and a nucleotide deletion, which account for approximately 95% of mutant alleles. The molecular diagnosis is now possible in this population by using simple PCR-based tests to screen for these mutations.

Adult↗

Obesity in Mexican-American and Anglo children.

The purpose of this study was to determine the degree to which certain factors are related to obesity in Mexican-American and Anglo preschool children. The following questions were addressed: To what extent is obesity in these children related to maternal: 1) nutrition knowledge, 2) feeding practices, 3) values, 4) socioeconomic status, 5) acculturation and 6) demographic variables. A descriptive correlational design was used with 189 Mexican-American and 188 Anglo mother-child pairs recruited from WIC Clinics. Children's triceps and subscapular skinfold thicknesses, as well as weight for height Zscores were assessed. Obesity was defined as at or above the 85th percentile. Questionnaires were administered to the mothers of each child. Data analysis included logistic and multiple linear regressions, and descriptive statistics. Findings suggest risks associated with childhood obesity involving biologic, family and feeding practice factors. These risk factors provide a basis for assessment and program development for the prevention of obesity, especially in Mexican-American children.

Body Mass Index↗

Functional expression of human mutant phosphofructokinase in yeast: genetic defects in French Canadian and Swiss patients with phosphofructokinase deficiency.

Human phosphofructokinase (PFK) is a tetrameric enzyme, encoded by muscle, liver, and platelet genes. Deficiency of muscle PFK (PFK-M), glycogenosis type VII (Tarui disease), is an autosomal recessive disorder characterized by an exertional myopathy and hemolytic syndrome. Several disease-causing mutations have been identified in the PFK-M gene in Japanese, Ashkenazi Jewish, and Italian patients. We describe the genetic defects in French Canadian and Swiss patients with the disease, and we use a genetically well-defined yeast system devoid of endogenous PFK for structure-function studies of the mutant PFKs. A G-to-A transition at codon 209-in exon 8 of the PFK-M gene, changing an encoded Gly to Asp, is responsible for the disease in a homozygous French Canadian patient. Gly-209-mutated protein is completely inactive in the yeast system. The Swiss patient is a genetic compound, carrying a G-to-A transition at codon 100 in exon 6 (Arg to Gln) and a G-to-A transition at codon 696 in exon 22 (Arg to His). The mutants expressed in yeast generate functional enzyme with modest changes in thermal stability. The advantages and limitations of the yeast system for expression of human mutant PFKs are discussed.

Adult↗

Common mutations in the phosphofructokinase-M gene in Ashkenazi Jewish patients with glycogenesis VII--and their population frequency.

Phosphofructokinase (PFK) catalyzes the rate-limiting step of glycolysis. Deficiency of the muscle enzyme is manifested by exercise intolerance and a compensated hemolytic anemia. Case reports of this autosomal recessive disease suggest a predominance in Ashkenazi Jews in the United States. We have explored the genetic basis for this illness in nine affected families and surveyed the normal Ashkenazi population for the mutations we have found. Genomic DNA was amplified using PCR, and denaturing gradient-gel electrophoresis was used to localize exons with possible mutations. The polymorphic exons were sequenced or digested with restriction enzymes. A previously described splicing mutation, delta 5, accounted for 11 (61%) of 18 abnormal alleles in the nine families. A single base deletion leading to a frameshift mutation in exon 22 (delta C-22) was found in six of seven alleles. A third mutation, resulting in a nonconservative amino acid substitution in exon 4, accounted for the remaining allele. Thus, three mutations could account for all illness in this group, and two mutations could account for 17 of 18 alleles. In screening 250 normal Ashkenazi individuals for all three mutations, we found only one delta 5 allele. Clinical data revealed no correlation between the particular mutations and symptoms, but male patients were more symptomatic than females, and only males had frank hemolysis and hyperuricemia. Because PFK deficiency in Ashkenazi Jews is caused by a limited number of mutations, screening genomic DNA from peripheral blood for the described mutations in this population should enable rapid diagnosis without muscle biopsy.

Adult↗

Intervention program for obese school children.

The purpose of this study was to implement and evaluate a program for obese school children. A pretest-posttest design was utilized. Data was collected related to weight status, skinfold measurements, self-esteem, and nutritional knowledge. A convenience sample of 26 children, in the fourth to sixth grades, completed this 9-week program. The results indicate that self-esteem increased significantly (p less than .001) between the pretest and posttest interval. Weight status and nutritional knowledge showed no improvement. Exercise was difficult to assess on self-report, therefore no conclusions were were drawn in relation to this variable. Future research will be directed toward refining this intervention program.

Child↗

Care needs of home-based cancer patients and their caregivers. Quantitative findings.

The purpose of this study was to identify care needs of home-based patients receiving treatment for cancer and their caregivers. The sample consisted of 30 patients receiving radiation therapy and/or immunologically based treatments for a variety of cancers and 29 caregivers. Patient and caregiver needs scales were used to elicit the needs of patients and caregivers. Patients and caregivers were able to cite their unique needs. Patients had more needs for themselves in the areas of personal care, activity management, and interpersonal interaction than did the caregivers. The necessity for individualization of care was noted by both patients and caregivers, an area that nursing is in a unique position to address.

Aged↗

Obesity in Mexican-American preschool children--a population group at risk.

This research built on our previous work focusing on the Mexican-American population as one group at risk for the development of obesity. The purpose of this study was to explore the relationship among knowledge of nutrition, feeding practices, values, and select demographic variables of Mexican-American mothers and obesity in their preschool children. One hundred forty-three mothers of children were recruited to participate. Each mother was interviewed and asked to complete questionnaires regarding maternal nutrition knowledge, maternal feeding practices, weight locus of control, and ideal infant body habitus. Compared to thin infants and their mothers, obese children had a significantly greater mean birth weight and their mothers had a significantly greater body mass index. Analysis of responses to the ideal infant body habitus scale revealed that mothers of obese children selected a chubby baby as ideal significantly more often than mothers of nonobese children. The data collected from this study can alert public health nurses to children at risk for obesity.

Chi-Square Distribution↗

Evaluation of public health science in the graduate community health nursing curriculum.

The purpose of this article is to describe and evaluate a unique course in Public Health Science that has recently been implemented in our College of Nursing. This course seeks to provide a synthesis of public health and nursing. The course description as well as the course objectives are outlined. A 22-item questionnaire was developed to obtain demographic information regarding the students enrolled in the course, their community health experience prior to entering the graduate program, their clinical area of specialization, reasons for enrollment in the course, strengths and weaknesses of the course, the degree to which the course met the objectives, and recommendations for future courses in Public Health Science. The findings suggest that the content of Public Health Science has been integrated into the graduate community health nursing program with much success.

Adult↗

Factors associated with obesity in school children.

Factors associated with obesity in school children, based on current research in the field, are described. Both family environment and individual characteristics have been shown to relate to children's weight status. Research to date supports the complex interrelationships between nutritional knowledge, feeding practices, values, and select demographic variables and the development of obesity in school children. An example of an ethnic group at risk for the development of obesity is provided. The present study suggests implications for school nursing practice in relation to the problem of obesity in children.

Child↗

Obesity in children: a research update.

Factors associated with obesity in children are described based on current research in the field. Consideration is given to the family environment as well as to individual characteristics that have been shown to relate to children's weight status. Research to date supports the complex interrelationships between nutritional knowledge, feeding practices, values, and select demographic variables and the development of obesity in children. This article suggests areas for further research to advance understanding of obesity in children.

Child↗

Evaluation of a worksite wellness program: impact on exercise, weight, smoking, and stress.

This study evaluated the effects of a wellness program at the workplace in relation to its impact on exercise, weight, smoking, and stress of the employee participants. A quasi-experimental design was selected in which data were collected at three specific intervals: before and immediately after program completion, and three months after program completion. The sample consisted of experimental and control groups. Persons (n = 59) who did participate in the wellness program made up the experimental group, and those (n = 49) who did not participate in the wellness program served as controls. Descriptive statistics were used to summarize the data. No significant differences were noted between participants and nonparticipants in the areas of exercise, weight, smoking, and stress between the initial evaluation and after completing the wellness program. The areas of stress reduction and increased exercise were emphasized more in the participants than the nonparticipants, however.

Adult↗

Caring: commitment to excellence or condemnation to conformity?

The art of caring to alleviate illness and to promote health is nursing's unique commitment to society and the health-care industry. How this practice is implemented dictates the success or failure of prescribed strategies. In addition, the way in which caring is implemented defines emotional maturity on both a personal and professional level. Several goals to rehabilitate the psychologically codependent person include: Developing an awareness of the origins of codependency and how early family experiences affect subsequent behavior and beliefs about oneself; Identifying the personal price and payoffs for continuing codependent behaviors; The ability to openly express personal needs, wishes, feelings, and opinions while respecting the rights of others; Learning to discriminate between loving/caring and the destructive control of codependency; and The ability to take responsibility for another rather than being responsible to another. Codependent behaviors are prevalent within professional nursing practice, as evidenced by examples provided from three specialty areas in nursing and the fact that women (who are traditionally assigned the cultural role of caring) constitute the majority of professional nurses. Therefore, it is a professional challenge to each nurse to ascertain whether practices are functional or dysfunctional: is the professional interpretation of caring a commitment to excellence or a condemnation to conformity in the unique delivery of health-care practice?

Dependency, Psychological↗