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Epilepsy is one of the most common childhood neurologic conditions affecting roughly 1% of children younger than the age of 15 years. Epileptic events that occur early in a child’s life can range in severity from ‘benign’ cases, where a child ultimately outgrows their seizures, to more severe cases with children suffering from drug-resistant seizures. In fact, there are over 50 forms of early infantile epileptic encephalopathies (EIEE) caused by different genes, several of which are within voltage-gated sodium channels (VGSCs). The focus of this dissertation was to create a deeper understanding of the clinical and biological risk factors of developmental outcome in children affected by mutations within VGSCs. Closer examination of the similarities among VGSCs can reveal channel-specific sensitivities to mutations as well as common sensitivities that lead to childhood epilepsy. By analyzing patient and healthy variant distribution of three central nervous system VGSCs (NaV1.1, NaV1.2, and NaV1.6) I found that all three channels have a heightened sensitivity to variation from the voltage-sensing domain to the pore-forming domain. Channel-specific sensitivities were also found within the inactivation gate, responsible for fast inactivation of the channel, as well as the linker containing the selectivity filter, which is responsible for sodium ion permeation. These sensitivities are hypothesized to reflect the exact neuronal location of the channel and highlight key regions of interest for future studies. Once the pattern of variant distribution for SCN8A variants was established, I explored the phenotypic spectrum of patients. Like other childhood epilepsy disorders, the age at seizure onset, current seizure freedom, and febrile seizures were all found to be positively correlated with development in the SCN8A-cohort. Interestingly a clear distinction between children with a single SCN8A variant and those with an additional reported variant was found, suggesting a possi

Purpose: To conduct an educational intervention to improve knowledge about compassion fatigue among pediatric oncology nurses at Phoenix Children’s Hospital (PCH). Background: Burnout, secondary traumatic stress, and decreased compassion satisfaction contribute to compassion fatigue. Pediatric oncology nurses face stressful situations based on the unique care demands of children battling cancer such as complex treatment regimens, managing professional boundaries, providing emotional support. grief, and bereavement. Compassion fatigue is a progressive process that can impact a nurse’s quality of life and contributes to decreased job satisfaction, performance, patient satisfaction, and turnover. Methods: A 20-minute educational PowerPoint presentation was presented on compassion fatigue to highlight what compassion fatigue is, signs and symptoms, and common triggers. There was also a handout provided with available resources at PCH that could be utilized to help with compassion fatigue. The Professional Quality of Life (ProQOL) scale was used to measure compassion fatigue and compassion satisfaction levels and a pre- and post-test was completed to measure if an educational intervention improves nurse’s knowledge about compassion fatigue. Findings: The results showed that participants (n=30) knowledge significantly improved after the 20-minute educational intervention (p=0.001) with a mean score of 76.2% on the pre-test and a mean score of 95.4% on the post-test. In addition, nurses had a significant increase (p=0.0001) in the utilization of resources; prior to the intervention only seven participants (23.3%) utilized some of the available resources offered by PCH compared to 24 participants (80%) one-month post-intervention. However, there were no statistically significant changes in raw scores for the pre- and one-month post ProQOL survey (p > 0.05). Overall the participants viewed the class worthwhile and had positive things to say about the class. Implications: The

Background: A critical component of sexual health is healthy sexual functioning; being able to experience sexual enjoyment and satisfaction when desired. Sexual difficulties are not unusual and can have either physical or psychological causes. An estimated 100 million males worldwide suffer from erectile dysfunction (ED), with the expectation that this sexual problem will increase to 322 million cases by the year 2025 (Bacon, Mittleman, Kawachi, Giovannicci, Glasser, & Rimm, 2003). The prevalence of ED greatly escalates in older males and those with Cardiovascular Disease, Hyperlipidemia, and Diabetes due to related endothelial dysfunction (Bivalacqua, Usta, Champion, Kadowitz, & Hellstrom, 2003). Sexual dysfunction is a concern for all males, including those over the age of 65. Because sexual function or sexuality is a critical factor contributing to the quality of life among older adults (Gelfand, 2000; Robinson & Molzahn, 2007), attention to sexual dysfunction is needed to ensure a high quality of life among older adult males (Vincent & Velkoff, 2010). Furthermore, psychological and emotional stress have been associated with sexual dysfunction among males; both as a cause and a result of this condition. Purpose: The purpose of this study was to determine the level of distress associated with perceived stress and sexual function among community-dwelling older adult males in stable relationships. Study Aims: The study aims are to 1) describe perceived stress and sexual function in a community-dwelling older adult male population, 2) describe the relationship between perceived stress and sexual function, and 3) describe the relationship between perceived stress and sexual function while controlling for individual characteristics of the sample. Methods: A descriptive, cross-sectional research design was used. The sampling technique was a convenience sample of males aged 65 and older enrolled in a university affiliated lifelong learning institute in the southwestern U

Asthma management in school-aged children, particularly controller medication use, is best conceptualized as parent-child shared management. Controller medication nonadherence is common, and leads to higher disease morbidity such as cough, sleep disruption, poor activity tolerance, and asthma exacerbation. The purpose of this study was to describe asthma illness representations of both school-aged children (6-11 years) with persistent asthma and their parents, and to examine their interdependence. The Common Sense Model of Self-regulation, modified to include Parent-Child Shared Regulation, provided the framework for this descriptive, cross-sectional study. Thirty-four parent-child dyads independently reported on asthma control, controller medication adherence, and asthma illness representations by completing the Childhood Asthma Control Test, Medication Adherence Report Scale for Asthma, Brief Illness Perception Questionnaire, and Beliefs about Medicines Questionnaire. Using intraclass correlations, moderate agreement was evident between the parent and child timeline (perceived duration) illness representation domain (ICC= .41), and there was a weak association between the parent and child symptoms domain (ICC = .13). The remaining controllability and consequences domains showed no agreement. Hierarchical regression analyses were used to test parent and child illness representation domain variables as predictors of parent or child estimates of medication adherence. With parent-reported medication adherence as the dependent variable, regression models used parent illness representation variables followed by the corresponding child variable. Parent beliefs about medication necessity versus concerns was a significant predictor of parent-reported treatment adherence (β = .55, p < .01). Child-reported treatment control was also predictive of parent-reported treatment adherence (β -.50, p < .01). When child-reported medication adherence was the dependent variable, the ch

Cigarette smoke contributes to many diseases. The actions of second and third hand smoke, which have implications for non-smokers and the very young, are just beginning to be appreciated. The overarching hypothesis of this project is that cigarette smoke has different injurious actions on alveolar cells based on chronological age. The purpose here was to learn more about the susceptibility of alveolar cells to cigarette smoke extract (CSE)- induced injury by performing studies on pulmonary alveolar and endothelial cells derived from neonatal, young, and old rats. The aims involved: 1. Developing cell culture models to study age-related effects of cigarette smoke on alveolar type I cells and microvascular endothelial cells from the lung, and 2. Using these models to examine the effects of CSE on markers of oxidative stress, inflammation and aging in alveolar cells harvested from neonatal, young and old rats. Descriptive and experimental studies involved using a variety of cell culture, biochemical and molecular techniques, including gene expression arrays. The most significant findings were that: 1. primary proliferating alveolar type I cells were used to develop novel cell culture model systems, including single culture, co-culture and three-dimensional cultures that were used to study the effects of CSE; 2. Hydrogen peroxide production by endothelial cells was markedly reduced by co-culturing with AT I cells; 3. Gene expression profiling of oxidative stress-specific pathways suggest that genes responsible for both stopping production of H2O2 or mopping-up H2O2 are involved; and 4. Cigarette smoke shortens telomeres of cells from neonates, but unexpectedly preserves telomere length of cells from young and old rats. Data from telomeric pathway-specific gene expression arrays suggest that there are age-related differences in response to gene expression to CSE. The significant conclusions are: 1. Contrary to prior observations, alveolar type I cells demonstrate prolong

The application of a high performance liquid chromatography method was described for determination of phospholipid levels in the cerebrospinal fluid of children suffering with acute lymphoblastic leukemia (ALL). The levels of two major membrane phospholipids (phosphatidycholine and sphingomyelin) were monitored during the course of therapy and compared among three different therapeutic protocols. The monitored levels were correlated with the results of cognitive testing and assessed as biomarkers of cellular damage associated with ALL treatment.

The application of gas chromatography/mass spectrometry (GC/MS) method was described for the generation of fatty acid profiles of medically important fungi. The composition of fatty acids was characteristic for different fungi and was used for identification and taxonomical purposes. The GC/MS methodology was shown to be suitable for diagnostic pusposes in clinical settings thanks to its sensitivity, speed and accuracy.

The goal of this project was to identify the presence of microorganisms in the environment by using their gas chromatography/mass spectral (GC/MS) characteristics as biomarkers. A combination of GC/MS results and pattern recognition statistical evaluation were used to provide a mathematical model with defined parameters for different microorganisms, which allows the organisms to be classified as bacterial, fungal or viral nature. Results from fast atom bombardment mass spectral (FAB/MS) analysis showed differences in the complex lipid profiles for most of the analyzed microorganisms. A general screening method that can provide early detection of microorganisms would permit initiation of most therapeutics. Various drugs are used to treat bacterial, fungal or viral diseases and, when treatment is started early, there is a dramatic reduction in mortality. A methodology for detection and classification of microorganisms is described in this paper.

The application of the following methods was described for the purpose of exploration of different cerebrospinal fluid biomarkers related to the cognitive impairment in children suffering from acute lymphoblastic leukemia and receiving methotrexate as part of the treatment regimen: gas chromatography/mass spectrometry was employed to measure the levels of homovanillic acid and vanilmandelic acid: twodimensional electrophoresis was used to find the changes in protein profiles present in cererebrospinal fluid: and high performance liquid chromatography was utilized to measure the levels of cerebrospinal fluid ceramides. The overall goal of this project was to utilize different analytical techniques in order to monitor chemical changes in cerebrospinal fluid associated wuth the administered amount of methotrexate.

Prostate cancer is the most commonly diagnosed cancer and second leading cause of death in American men. Patients with localized prostate cancer may experience unique and multidimensional symptoms that are distressful from treatment and thereafter. This cross-sectional correlational study aimed to investigate the relationships among stress, symptoms, symptom distress, and symptom self-management and identify the effective strategies of symptom self-management in men with localized prostate cancer following prostatectomy or radiation therapy.Eight saliva samples and 3 questionnaires (Perceived Stress Scale, Symptom Indexes, and Strategy and Effectiveness of Symptom Self-Management) were obtained from each participant between 1 and 3 months following their first prostate cancer treatment. The sample consisted of 53 men with localized prostate cancer. Mean salivary cortisol concentrations for the entire sample ranged from 0.3 to 0.08 ug/dL. Cortisol was secreted in a circadian rhythm with heightened activity in the early morning and lowered activity late in the day. The circadian pattern of cortisol secretion was similar in both the prostatectomy and radiation therapy groups, although the values were slightly different. Two areas Under the Curve (AUC) of salivary cortisol were calculated. Three cortisol circadian rhythms were identified, but the majority of the sample had a typical negative consistent circadian rhythm.Patients with localized prostate cancer who underwent radical prostatectomy or radiation therapy had low perceived stress. Perceived stress was positively correlated with AUCg, noon salivary cortisol concentrations, and afternoon salivary cortisol concentrations. Subjects reported a moderate degree of symptoms and symptom distress on urinary, bowel, and sexual dysfunction 1-3 months following treatments. The most effective strategies of urinary symptom management were pad and kegel exercise; the most effective strategy of bowel symptom management was rest

When a child is diagnosed with a chronic life threatening illness there is a significant impact on the entire family. Siblings are at risk for psychological adaptation problems because of their unique relationship with the ill child and the effect of that illness on family functioning. Few studies have been reported which examine the impact of chronic life-threatening illnesses in children on healthy siblings.The purpose of this study was to investigate the predisposing risk and protective factors that affect the psychological adaptation of healthy siblings of a child with Cystic Fibrosis (CF). A descriptive study was conducted using a convenience sample from two CF centers. The relationship between several variables was explored using non- parametric correlations.In this sample, significant negative correlations were found between parental stress and their reported financial well being and emotional or behavioral problems in the well siblings prior to the diagnosis of CF was made in the sick sibling and perceived parental support.For adolescent siblings, The Behavioral Symptoms Index (BSI) was correlated to reported stress in their parents and negatively correlated to the parental perceived support. The Emotional Symptoms Index (ESI) was correlated to the BSI. A significant negative correlation was also noted between age and Internalizing Behaviors. These relationships were not significant for the child siblings in this sample.There were no significant relationships among gender, maternal education, and caretaker for the well sibling when the child with CF was hospitalized and their Internalizing and Externalizing Behaviors. The health care teams in these clinics seldom discussed CF with the well sibling. However, for the children in this study, there was a significant negative correlation with this discussion and their Externalizing Behaviors.Findings from this study suggest that the family environment, especially parental stress and perceived social support, may