Pyruvate carboxylase deficiency type C; variable presentation and beneficial effect of triheptanoin.

Pyruvate carboxylase is a mitochondrial enzyme essential for the tricarboxylic acid cycle (TCA), gluconeogenesis and fatty-acid synthesis. Pyruvate carboxylase deficiency (PCD) mostly presents with life-limiting encephalopathy (types A/B). A milder type C presentation is rare, with a comparatively favourable prognosis. Therapies remain essentially supportive. Triheptanoin is an odd-chain triglyceride, with the potential to replenish TCA intermediates (anaplerosis), and its metabolites cross the blood-brain-barrier. Outcomes of triheptanoin treatment in PCD types A/B have been disappointing, but have not been reported in type C. Here, we present two new patients with PCD type C, and report the response to treatment with triheptanoin in one. Patient 1 (P1) presented with neonatal-onset lactic acidosis and recurrent symptomatic lactic acidosis following exercise and during illnesses, with frequent hospitalisations. Speech development was delayed. MRI-brain showed delayed cerebral myelination. Patient 2 (P2) presented with episodic ketoacidosis, hyperlactataemia and hypoglycaemia at 2 years of age, with gross motor delay and mild global volume loss on MRI brain. Treatment with triheptanoin was commenced in P1 at 3 years of age with up-titration to 35 mL/day (25% of daily energy intake) over 6 months, due to transient diarrhoea. Dietary long-chain triglycerides were restricted, with fat-soluble vitamin supplementation. Subsequently, hospitalisations during intercurrent illnesses decreased, post-exertional hyperlactataemia resolved and exercise tolerance improved. Continued developmental progress was observed, and repeat MRI 18 months after initiation showed improved myelination. Triheptanoin was well-tolerated and appeared efficacious during 2 years' follow-up, and has potential to restore energy homeostasis and myelin synthesis in PCD type C.

A minimal physiologically based pharmacokinetic model that predicts anti-PEG IgG-mediated clearance of PEGylated drugs in human and mouse.

Circulating antibodies that specifically bind polyethylene glycol (PEG), a polymer routinely used in protein and nanoparticle therapeutics, have been associated with reduced efficacy and increased adverse reactions to some PEGylated therapeutics. In addition to acute induction of anti-PEG antibodies (APA) by PEGylated drugs, typically low but detectable levels of APA are also found in up to 70% of the general population. Despite the broad implications of APA, the dynamics of APA-mediated clearance of PEGylated drugs, and why many patients continue to respond to PEGylated drugs despite the presence of pre-existing APA, remains not well understood. Here, we developed a minimal physiologically based pharmacokinetic (mPBPK) model that incorporates various properties of APA and PEGylated drugs. Our mPBPK model reproduced clinical PK data of APA-mediated accelerated blood clearance of pegloticase, as well as APA-dependent elimination of PEGyated liposomes in mice. Our model predicts that the prolonged circulation of PEGylated drugs will be compromised only at APA concentrations greater than ~500 ng/mL, providing a quantitative explanation to why the effects of APA on PEGylated treatments appear to be limited in most patients. This mPBPK model is readily adaptable to other PEGylated drugs and particles to predict the precise levels of APA that could render them ineffective, providing a powerful tool to support the development and interpretation of preclinical and clinical studies of various PEGylated therapeutics.

State of polyphenols in the drying process of fruits and vegetables.

This review presents an overview of drying technologies and its impact on the polyphenol content of vegetables and fruits. Polyphenols contribute to many health benefits and can act as antioxidants. Specifically an increased intake of polyphenols has been shown to decrease the incidence of cardiovascular disease; furthermore, it has been shown to help reduce the risk of neurodegenerative diseases in humans. Many researchers have reported on the effect of different drying techniques on the polyphenol content in fruits and vegetables. Polyphenol degradation mechanisms proposed in literature and pretreatments that potentially lead to higher retention of polyphenols during drying are also discussed.

Measurement of fatigue in people with cancer.

PURPOSE/OBJECTIVES: To evaluate the quality of existing instruments measuring cancer-related fatigue (CRF). DATA SOURCES: Nursing and medical literature. DATA SYNTHESIS: Although fatigue is highly prevalent among patients with cancer and adversely affects their quality of life, CRF often is unrecognized and untreated. The instruments available to measure CRF have numerous limitations. Many have been generated from investigators' observations, not actual experiences described by patients. Others operationalize different definitions of fatigue or differ in dimensionality, which leads to limited reliability and validity testing. CONCLUSIONS: All of the instruments in this review need further study of their psychometric properties. Qualitative studies of CRF from the patients' perspective are needed to develop better instruments. IMPLICATIONS FOR NURSING PRACTICE: Nurses need to increase their knowledge of assessing CRF to intervene and improve the quality of life for patients with cancer.

Finding and evaluating clinical practice guidelines.

Clinical practice guidelines are valuable, clinical decision-making aids for preventive, diagnostic, therapeutic, and palliative patient care. This article identifies ways to access clinical practice guidelines and discusses evaluative criteria for identifying gold-standard evidence-based practice guidelines. Finally, clinicians are reminded of crucial questions to ask before applying any guidelines to patient care.

Health-seeking behavior as an outcome of a homeless population.

Health care is an ever-present concern for homeless individuals. Health-Seeking Behaviors within this population are examined from a nursing perspective. Complex Relationship Building, considered essential in addressing Health-seeking Behaviors for the homeless, is examined in regard to access, trust, and follow-up. It is believed that access and follow-up are related to convenience of site and the matter of trust. A pilot study of how to measure the concept of trust was conducted. Factor analysis for the Gibson Trust Instrument shows two factors: (1) interpersonal attributes and (2) behavior attributes. The Gibson Trust Instrument can be used to measure trust, which is crucial for Complex Relationship Building.

Effective conflict management begins with knowing your style.

The well being of patients depends on the ability of nurses to manage conflict creatively. The Bartol/McSweeney Conflict Management Scale, an assessment tool that helps nurses identify their predisposition toward conflict, is described. The tool is simple to use and suitable for nurses working in different settings.

Assessing elderly patients with congestive heart failure via in-home interactive telecommunication.

The efficacy of telemedicine technology was tested for equivalence of nursing assessment with chronic congestive heart failure (CHF) home care patients (N = 28). The equivalence of nurses' physical assessment findings was estimated using an innovative two-way, telemedicine audiovisual system. Nurses were randomly assigned to a method of client assessment: on-site (real time) or telemedicine (monitor time). Each assessment was performed within 10 minutes of each other. Assessment variables compared were auscultation of lung sounds, heart sounds, rate and rhythm, blood pressure, weight, edema, respiratory effort, and client's face, lip, and nail color. Eighteen physiological parameters were analyzed, using either the Wilcoxon signed ranks test or the McNemar test. Results indicate few significant differences between the assessments of the real time and monitor time nurses. The monitor nurse was more likely to claim abnormality than the real nurse was when assessing the color of nails (p = .048). The real nurse picked up ankle edema (p = .024), pedal edema (p = .099), and inspiratory wheeze (p = .01) more frequently than did the monitor nurse. Kappa coefficients to determine the extent of agreement between nurse's assessments were significant. Nurses' comments were favorable, but they recommended altering the interview to elicit symptoms not easily observed by the monitor nurse such as diaphoresis. Exit interviews of the elderly patients revealed a favorable reaction to using the telemedicine monitor, citing a quick connection to a nurse and response to their concerns and questions. Both nurses and patients reported the need to have real nurse home visits along with telemedicine visits.

An analysis of nurses' communications in a shelter setting.

The purpose of this study was to analyze written communications among nurses providing care to battered women and their children in a community shelter setting over a 1-year period of time. Interviews were conducted with the nurses to study the perceived effectiveness of the manual used to update daily shelter health operations, clients of concern, follow-up recommendations, or other ongoing issues. The unit of analysis was each entry recorded in the manual. The Communications Process Model guided the study (Budd, Thorp, & Donohew, 1967). Communications were transcribed and analyzed for categories of meaning from the data utilizing the Ethnograph software (Seidel & Clark, 1983). When content analysis was applied, 10 distinct categories emerged: (a) injury assessment, (b) acute condition assessment, (c) chronic condition assessment, (d) pregnancy assessment, (e) emergency assessment, (f) nursing care and procedures, (g) teaching and counseling, (h) community referrals and resources, (i) housekeeping, and (j) health program issues. From the categories, 3 primary themes were identified: nursing assessments, nursing interventions, and health program operations. The nurses were positive about using the manual for various communications. Nurses who consider working in shelter settings may find descriptions of the realities of practice and application of a working tool for communications useful. Shelter administrators and board members need to recognize the importance of having nurses on staff to address inevitable injuries and health concerns of battered women and their children. Researchers are encouraged to develop models that relate to care of clients and caregivers in this challenging practice setting.

Characteristics of patient visits to nurse practitioners and physician assistants in hospital outpatient departments.

Many authors have described differences between nurse practitioners and physician assistants. Most studies have compared physician with nonphysician providers' practice. Few studies have compared nurse practitioners and physician assistants, and none has used a national data base. This exploratory, atheoretical research examined which of the following characteristics predicted patients being seen by nurse practitioners and physician assistants: patient and hospital demographics, diagnosis, diagnostic/screening services, therapeutic services, and disposition of the visit. The data set used for analysis was the 1992 National Hospital Ambulatory Medical Care Survey. Based on a multistage probability design yielding national estimates for patient visits in hospital outpatient settings, the National Hospital Ambulatory Medical Care Survey showed that there were 2,847 weighted patient visits to either nurse practitioners or physician assistants (4.6 million patient visits using national estimates). Results of multivariate logistic regression suggest that nurse practitioners were the most likely nonphysician provider for outpatients receiving more health promotion and counseling (therapeutic) services and for those needing women's and children's services. Outpatients in rural areas predicted visits to physician assistants. As more nonphysician providers enter the work force, the results of this research may assist with understanding the utilization of nurse practitioners and physician assistants in primary care.

Conceptualization and measurement of insight.

Insight is a complex phenomenon which has been conceptualized variously as a symptom of schizophrenia and as awareness of illness, making comparisons of studies measuring insight difficult. Furthermore, attempts to establish a correlation between insight and successful treatment outcomes are fraught with methodological problems. Specifically, categorical judgments fail to capture important information about the quality of insight; inappropriate inferential statistics have been used; and validity of instruments to measure insight has not been established. In future research, the concept of insight needs to be clarified. Nonparametric statistical analysis should be considered for small, nonnormally distributed samples. Qualitative exploratory research is needed.

Stress and morale in general practice: a comparison of two health care systems.

BACKGROUND: Poor morale and high levels of stress among general practitioners (GPs) are causing concern. Little research has previously been carried out to study possible differences in morale and stress between GPs working in two different but geographically similar health care systems. AIM: To compare perceived levels of stress and morale between GPs working in two different health care systems--one having a state monopoly (Northern Ireland) and the other having mixed private and state funding (Republic of Ireland)--and to look for factors that might help explain any differences in stress levels and morale between the two systems. METHOD: Anonymous and confidential questionnaires were sent to all 986 National Health Service (NHS) GPs in Northern Ireland (NI) and a random sample of 900 GPs in the Republic of Ireland (ROI). A common set of core questions on demographic details, partners and work patterns, perceived levels of stress and morale, safety, violence, and complaints were asked. RESULTS: Response rates were high in both areas: 91% in NI and 78% in the ROI. GPs in NI had significantly higher stress levels and significantly lower levels of morale than GPs in the ROI. The NI sample expect matters to get worse over the following year. Doctors in the ROI were more likely to be single handed and to work from two sites. Also, more GPs in ROI had fears for their safety and had been the subject of physical violence, but fewer had received complaints and medico-legal actions than in NI. CONCLUSIONS: A significant proportion of both groups of doctors report feeling highly stressed but GPs in NI appear more unhappy and have a poorer outlook for the future. It is suggested that the structure, management, and expectations of the NHS have taken a severe toll on its GPs, whereas a system in which doctors have less practice support but more control is good for morale.

Preschool children of battered women identified in a community setting.

This study describes and compares health, developmental, and behavioral manifestations of witnessing domestic violence in a health department population of preschool children (n = 15) of women detected to be battered (Conflict Tactics Scale [CTS], Straus, 1979). Comparisons included a community sample of the preschool children of nonbattered women (n = 62) to study the effect of mothers being battered, and a shelter sample of the preschool children of battered women (n = 53) to study the effect of "sheltering" on children's characteristics. Instrumentation included behavioral subscales, physical assessment, Denver II Developmental Screening, hemoglobin and lead levels, immunization, and nonmotor vehicle accident histories. Results showed more similarities between children of community (nonsheltered), battered and nonbattered women, but showed more differences between the children of community (nonsheltered) battered women and the children of sheltered, battered women. Battering intensity of the women was more severe in the shelter sample according to the CTS (p < .001), and more abnormal findings in their children were detected than in the community sample. When children of community (nonsheltered), battered women (n = 15) and children of sheltered, battered women (n = 53) were included in discriminant analysis of demographic characteristics, health status, and psychosocial variables, 87% of each group was classified correctly.

Defining vascular nursing: a survey of vascular nurses.

For the purpose of defining the specialty of vascular nursing, a survey was sent to the nursing membership of the Society for Vascular Nursing. Nurses in all geographic locations were included, and the survey was accompanied by a self-addressed, stamped return envelope. A total of 873 nurses were surveyed. Respondents were encouraged to give the survey to other vascular nurses who were not members of the Society for Vascular Nursing. The initial mailing resulted in 276 responses. A second mailing provided an additional 224 responses for a total response of 500. Fifty-six of the 500 respondents (11.2%) were nonmembers. Response rate of members was 50.9% (444 of 873). Demographic information was obtained regarding age, years in nursing and vascular nursing, sex, employment type, and location. In addition to demographic information, the survey asked the respondent to identify a definition or definitions of vascular nursing. There was no restriction on space or content. In a small pilot study, "defining the specialty" was the only question asked. However, on the basis of responses to the pilot, the form was refined to request a description of the skills necessary to be a vascular nurse. A total of 73 definitions were identified on the basis of coded responses. The range in number of definitions given on any survey was 0 to 13. Essential skills listed by any one respondent ranged in number from 0 to 14. A total of 90 skills were identified. The purpose of this study is to identify the definitions of vascular nursing given by vascular nurses. In addition, the skills identified as necessary will be elaborated on.(ABSTRACT TRUNCATED AT 250 WORDS)

Partner abuse against female nursing students.

Although nurses may have the necessary skills to plan care of clients in a variety of settings, experience and research demonstrate that nursing interventions with women victims of violence have been consistently inadequate. Of the 243 nursing students included in this study, 8% reported experiencing physical abuse, and 18.9% reported experiencing nonphysical abuse. Difficulties with depression for 26.1% of the students and some level of clinical stress were reported in over half of the group. Specific needs to combat violence and abuse against nurses and nursing students include providing better information on such behavior by incorporating family-violence education into nursing-school curricula, and using this proactively in confronting domestic violence in home and work settings.