Pediatric restrictive cardiomyopathy associated with a mutation in beta-myosin heavy chain.

Most children do not have a known cause of cardiomyopathy which limits the potential for disease-specific therapies. Of the different phenotypic presentations of cardiomyopathy, the restrictive form carries the poorest prognosis and has the lowest rate of identification of etiology. We present the first description of a beta-myosin heavy chain gene mutation in an infant with restrictive cardiomyopathy requiring cardiac transplantation. As demonstrated by three-dimensional protein structure modeling, the missense mutation is in a highly conserved amino acid at the critical binding region for the essential light chain. This case emphasizes that mutations in sarcomeric proteins, which are known to cause hypertrophic cardiomyopathy in adults, may be associated with the development of restrictive physiology in childhood. Identification of the genetic basis of pediatric cardiomyopathy has important implications for management and genetic counseling.

Ethical and policy considerations for centenarians--the oldest old.

PURPOSE: To illustrate the incongruence of ethical standards and fiscal and policy constraints on quality care for the oldest old. As the fastest growing demographic segment in the United States, care needs of the oldest old are a special challenge to the health care system. DESIGN: Narrative analysis of interviews with centenarians who used nursing home services. The sample was three participants of the Georgia Centenarian Study who had been community dwelling and cognitively intact at the onset of participation (between 1988 and 1997). Interviews were conducted in nursing homes or after discharge. METHODS: Case histories were constructed from interviews in 1997 to improve understanding of quality of care. FINDINGS: Less-than-optimal care was provided for these elders, and little consideration was given to their input to care decisions and prospects for medical improvement. Appropriate consideration was not given to providing least-restrictive environments, appropriate restraint use, and options for community care. CONCLUSIONS: Six policy reforms are suggested for meeting the needs of the oldest-old before and after institutionalization. These include: integration of resident involvement in care decisions; development of alternate models of care; greater input from nurses concerning nursing care of special populations; more effective family and community involvement in the caring of elderly populations; increased research to promote function and independence; and increased education of personnel and nursing students to allow for more accurate assessment of cognitive and physical status.

Health characteristics of elderly personal care home residents.

The purpose of this descriptive study was to explore the health characteristics of elderly personal care home residents. Personal care homes are community-based facilities that provide living arrangements, assistance with basic needs and protective oversight. Personal care home providers were interviewed regarding the health status of 80 elderly residents. The most frequent ADL needs were bathing, personal hygiene and dressing. Thirty-seven per cent of the residents had some bladder incontinence. Eighty-two per cent of residents needed some help with medication management. Over half of the residents had some cognitive impairment. These findings support the proposition of increased prevalence of cognitive impairment in personal care home residents as compared to the first studies a decade ago. The combination of functional deficits and cognitive impairment indicates that these elderly residents may have unmet health needs. The role of the registered nurse in this environment is discussed.

Psychosocial correlates of subjective health in sexagenarians, octogenarians, and centenarians.

The purpose of this study was to determine if a psychosocial model was a significant improvement over a demographic or a physical health model in predicting subjective health in older adults. Correlates of subjective health in sexagenarians, octogenarians, and centenarians were examined with hierarchical regression analysis. Data were obtained in the first wave of the Georgia Centenarian Study from 1988 to 1992. Psychosocial variables helped explain a significant component of subjective health variance above and beyond the effects of demographic or physical health variables. For centenarians an apprehensive personality and low levels of control over health were additional correlates of poor subjective health. Centenarians were the only cohort to have a unique set of correlates, indicating a uniqueness in the oldest-old, as compared to the young-old and old-old. These findings indicate that a multidimensional perspective of health in older adults is more appropriate than medical models.

Future realities in nursing: partnerships, practice, and economics.

Health care reform, innovations in technology, and the need to make health care cost-effective have affected all aspects of health care practice and education. Critical thinking skills, interpersonal and communication skills, leadership and motivation skills, computer literacy, and cultural sensitivity are all capabilities nursing graduates must now possess if they are to practice effectively in the complex and competitive contexts that today define the health care marketplace. Partnerships with community agencies are essential if faculty are to prepare a new generation of nurses who will be proficient in the skills that 21st-century nursing practice will demand. Although academic institutions have made some changes to meet marketplace demands, nursing educators, practitioners, and researchers must thoroughly reconceptualize their philosophies and retool their curricula in response to these changes.

A requirement for the Rho-family GTP exchange factor Vav in positive and negative selection of thymocytes.

The T cell repertoire is shaped by positive and negative selection of thymocytes that express low levels of T cell receptor (TCR) and both CD4 and CD8. TCR-mediated signals that determine these selection processes are only partly understood. Vav, a GDP-GTP exchange factor for Rho-family proteins, is tyrosine phosphorylated following TCR stimulation, suggesting that it may transduce TCR signals. We now demonstrate that mice lacking Vav are viable and display a profound defect in the positive selection of both class I- and class II-restricted T cells. In contrast, Vav is not essential for negative selection, though in its absence negative selection is much less effective. Vav may influence the efficiency of TCR-induced selection events by regulating the intracellular calcium flux of thymocytes.

Factors of nutritional health-seeking behaviors. Findings from the Georgia Centenarian Study.

Health-seeking behaviors are described by Harris and Guten (1979) as any behavior of an individual that promotes, protects, or maintains one's health, regardless of actual or perceived health status. The purpose of this study was to determine if nutritional health-seeking behavior (Bausell, 1986) comprised one or more factors for older adults (N = 256). Participants were in their 60s (n = 90), 80s (n = 91), and 100+ (n = 75). Exploratory factor analysis indicated that nutritional health-seeking behavior items formed two factors: avoid (i.e., avoiding unhealthy nutritional behavior) and seek (i.e., attempting or seeking healthy nutritional behavior). Multiple regression analysis revealed that risk factors for poor nutritional health-seeking behaviors in older adults include advanced age, low economic resources, and male gender. Protective factors included in the personality factors of self-discipline, enthusiasm, sensitivity, and warmth.

Syk tyrosine kinase is required for the positive selection of immature B cells into the recirculating B cell pool.

The tyrosine kinase Syk has been implicated as a key signal transducer from the B cell antigen receptor (BCR). We show here that mutation of the Syk gene completely blocks the maturation of immature B cells into recirculating cells and stops their entry into B cell follicles. Furthermore, using radiation chimeras we demonstrate that this developmental block is due to the absence of Syk in the B cells themselves. Syk-deficient B cells are shown to have the life span of normal immature B cells. If this is extended by over-expression of Bcl-2, they accumulate in the T zone and red pulp of the spleen in increased numbers, but still fail to mature to become recirculating follicular B cells. Despite this defect in maturation, Syk-deficient B cells were seen to give rise to switched as well as nonswitched splenic plasma cells. Normally only a proportion of immature B cells is recruited into the recirculating pool. Our results suggest that Syk transduces a BCR signal that is absolutely required for the positive selection of immature B cells into the recirculating B cell pool.

Intraindividual change and interindividual differences in factors influencing older women's health-seeking behavior.

Our primary purpose in conducting this study was to examine the factor structure of older women's health-seeking behavior, which we assessed over 100 occasions. This was accomplished with a daily self-report of nutritional, exercise, and adherence behaviors. A secondary purpose was to determine differences in the stability and lability of health-seeking behavior factors. The sample consisted of 4 women in their 60s and 4 women in their 80s. By using P-technique factor analysis, in which the sample size is determined by number of occasions rather than participants, we examined individual factors of health-seeking behavior for variability over time. Both activity and dietary items made up the health-seeking behavior factors of participants who were in their 60s, whereas for participants in their 80s factors comprised only activity items. The stability of most of these health-seeking behaviors indicated that these behaviors may be more trait-like than state-like in these older women.

Differences in factors of health orientation of older women.

The purpose of this exploratory study was to examine the factor structure of health orientation. A secondary purpose was to determine differences in the stability and lability of health orientation. The sample consisted of four women in their sixties and four women in their eighties. A replicated, single-subject design was used to assess short-term intraindividual change in health orientation. Participants completed the Health Orientation Scale questionnaire for 100 days, and P-technique factor analysis was used to determine the factor structure of health orientation. These health orientation factors were graphed over time to assess stability and change. Results indicate that health orientation is more complex for participants in their eighties than for those in their sixties; this may represent age-appropriate development. Health orientation factors were found to be more labile or statelike for women in their eighties. These findings may help health care providers understand differences in interest or adherence to health-seeking behavior in the elderly and improve age-appropriate health care for this population.