Ivacaftor improves appearance of sinus disease on computerised tomography in cystic fibrosis patients with G551D mutation.

BACKGROUND: Most patients with Cystic fibrosis (CF) have chronic sinus disease which may require multiple sinus surgeries and antibiotic courses. Ivacaftor can improve lung function, lower sweat chloride levels and improve weight by targeting the primary defect, a faulty gene and its protein product, cystic fibrosis transmembrane conductance regulator (CFTR) in patients with the G551D mutation. Its role in improving sinus disease has not been evaluated. OBJECTIVE: The objective of this study was to evaluate efficacy of ivacaftor in improving CF related sinus disease. DESIGN: Observational study. PARTICIPANTS: Twelve patients with cystic fibrosis and a G551D-CFTR mutation. METHODS: Twelve patients with a G551D-CFTR mutation were monitored for at least one year before and after starting ivacaftor. OUTCOME MEASURES: Sinus disease progression was monitored by comparing computed tomography (CT) of sinuses before and at one year on therapy. Hospital admissions, pulmonary exacerbations, weight, BMI and lung function were also compared. RESULTS: Median age was 17 years (range 10-44). Weight, BMI, FEV1 significantly increased and sweat chloride significantly decreased by six months on ivacaftor therapy. CT of the sinuses in all patients improved. Seven patients had severe sinus disease, improved to moderate in three and mild in remaining four. Four patients had moderate disease which improved to mild in all. One patient had normal sinus CT before and after the therapy. CONCLUSIONS: Patients with CF and G551D mutation, within 6 months of starting ivacaftor had significant improvements in weight, BMI and mean % FEV1. Significant lessening of underlying sinus disease measured by CT scan was noted, suggesting a disease modifying effect.

Biobehavioral characteristics of infants with failure to thrive.

Failure to thrive (FTT) is a syndrome of growth failure that results in an infant who is behaviorally difficult. The current thinking is that FTT results from a problematic infant-mother interaction, with the infant making a significant contribution to the interactional process. It is possible that the behavioral characteristics of the infant with FTT may be related to underlying physiologic response patterns, specifically, activity of the autonomic nervous system. The purpose of this study is to examine the relationships among behavioral responsiveness, heart rate variability as a marker of autonomic nervous system activity, and nutritional status in infants with FTT. Infants with FTT were matched with healthy growing infants (n = 14 pairs). Results from the study indicated that infants with FTT exhibited considerably more negative behaviors and exhibited low heart rate variability. It appears that there may be a physiologic basis to the behaviors that are exhibited by infants with FTT. Prospective research is needed to further clarify this relationship.

Impact of the threat of war on children in military families.

The potential for war is a pervasive threat to the security and family structure of children in military families. This study compared children of active-duty, reserve, and civilian families with respect to their perceptions of war, origin of fears related to war, levels of manifest anxiety, coping strategies, and projection of emotional problems in human figure drawings. Findings regarding the adaptation of children in military families are discussed, and the need for further research from children's perspectives is highlighted.

Military women's perspectives on health care during deployment.

Approximately 367,000 women serve in the U.S. military and regularly deploy with their units to austere military environments. A survey was used to describe 841 military women's perceptions about the health care provider that is available them during deployment and their perceptions of barriers to adequate diagnosis and treatment of gynecologic infections in these austere environments. The study findings have implications for military provider training, sick call policy, predeployment training, and development of self-care alternatives for military women during deployment.

A clinical test of women's self-diagnosis of genitourinary infections.

Vaginitis and cystitis result in 8 million ambulatory health care visits annually. For military women deployed to austere environments, gynecological problems are more likely to occur yet unlikely to be adequately diagnosed and managed. The Institute of Medicine has recommended that self-care kits be made available to military women under these circumstances. In this clinical comparative study, women's (N = 86) self-diagnoses of vaginitis and cystitis using an investigator-designed, self-diagnostic decision-making guide and kit were compared with clinical diagnoses by advanced practice nurses (APN). The vaginitis guide had a sensitivity of 84% for candida vaginitis (CV) and 91.3% for bacterial vaginosis (BV) and a specificity of 81.5% for CV and 82.8% for BV. The women consistently agreed with the APN's diagnosis of presence or absence of cystitis. These findings suggest that women are capable of making reliable self-diagnostic decisions given appropriate cues. Continued development of a self-diagnosis and treatment kit for military women deployed to austere environments is warranted.

Aberrant genital practices: an unrecognized form of child sexual abuse.

The sexual abuse of children in the United States is a problem of epidemic proportions. Sexual abuse is defined as any activity with a child that is conducted for the sexual gratification of the perpetrator. Common examples of sexual abuse include vaginal intercourse; oral-genital contact; anal-genital contact; fondling; finger manipulation; and stimulation of the offender. Sexual abuse may also occur in less typical patterns in which children are subjected to aberrant genital care practices, typically by their parents. Three case studies illustrate examples of aberrant genital practices. This type of abuse can be identified in primary care settings by asking specific questions of parents and children. Examples of such questions are provided.

School-age children's fears, anxiety, and human figure drawings.

INTRODUCTION: The purpose of this study was to identify the fears of school-age children and determine the relationship between fear and anxiety. METHOD: A descriptive, correlational, secondary analysis study was conducted using a convenience sample of 90 children between the ages of 8 and 12 years. Each child was instructed to complete the Revised Children's Anxiety Scale and then answer questions from a structured interview. On completion, each child was instructed to draw a human figure drawing. Frequency charts and correlational statistics were used to analyze the data. RESULTS: Findings indicated that the most significant fears of the boys were in the categories of animals, safety, school, and supernatural phenomena, whereas girls were more fearful of natural phenomena. High correlations existed between anxiety scores and the number of fears and emotional indicators on human figure drawings. DISCUSSION: Because human figure drawings are reliable tools for assessing anxiety and fears in children, practitioners should incorporate these drawings as part of their routine assessments of fearful children.

The health and physical readiness of Army reservists: a current review of the literature and significant research questions.

Individual soldier combat readiness through enhanced physical fitness is a major emphasis in the United States military today. Unfortunately, the prevalence of cigarette smoking, alcohol abuse, and other poor health practices continues to be reported at higher rates for active duty military personnel than for civilian populations. There is a dearth of research about health and fitness in the Army Reserve. Changing trends point toward more rapid mobilization of reservists for peacekeeping and combat missions throughout the world; however, it is important to remember that the Army Reserve is unique in its blending of both military and civilian occupational environments. The purpose of this paper is to review the current literature regarding Army Reserve health behaviors and physical readiness. Examples of significant future research questions are provided. More studies are needed to assess both individual motivational activities and organizational values that enhance health-promoting efforts. Interventions can then be designed to eliminate impediments to health and endorse specific organizational values and interpersonal skills that increase healthy behaviors.

The clinical reasoning case study: a powerful teaching tool.

This article describes the limitations of typical published case studies with respect to their congruence with actual clinical practice and their utility in teaching clinical decision making to novice or student health care providers. The authors propose a Clinical Reasoning Case Study that closely resembles an actual patient encounter, yet is also a rigorous academic exercise. In which health care providers must think aloud as the encounter unfolds. The Clinical Reasoning Case Study explicates and substantiates health care providers' thought processes underlying each decision to collect objective and subjective data. Other unique characteristics of this case study include a discussion of the working diagnosis and the provider's relative certainty about that decision; selection of the single most important objective and subjective finding that led to the diagnosis; a chronological list of diagnostic hypotheses that were generated throughout the patient encounter; and an analysis of costs, including the office visit, diagnostic tests, medications, and treatments. The Clinical Reasoning Case Study is a powerful tool for teaching and evaluating the clinical reasoning process. Two sample case studies are provided: "A Child with a Heart Murmur" and "An infant with Diaper Rash."

Children, coping, and the stress of illness: a synthesis of the research.

PURPOSE: To synthesize and critique the current research on coping strategies used by children who are ill, to identify gaps in the research base, and to provide direction for practice, future research, and theory development. POPULATION: 32 research articles on coping strategies used by children with chronic or acute illness. CONCLUSIONS: Children's repertoire of coping strategies is the same during health and illness, although the frequency or effectiveness of some of the strategies is often different. PRACTICE IMPLICATIONS: Much more in-depth descriptive and longitudinal research is needed, particularly how, when, and why coping strategies are learned, retained, and later discarded. Research is needed to examine the relationship between coping and health outcomes, and on the effectiveness of nursing interventions designed to influence children's coping behavior.