Characterising repetitive behaviours in young boys with fragile X syndrome.

BACKGROUND: Repetitive behaviours are frequently observed in individuals with intellectual disability (ID). The present study examined the profile, inter-correlations and predictive correlates of repetitive behaviours in boys with fragile X syndrome (FXS), the leading inherited cause of ID. Specific child characteristics examined as predictors included anxiety, nonverbal cognition and autism social-affective symptomatology. METHOD: Participants were 39 boys with FXS (aged 6-10 years). Repetitive behaviours were measured using the Repetitive Behavior Scale - Revised (RBS-R) - a 43-item caregiver-report measure normed on individuals with ID. RESULTS: Restricted Interests and Sensory Motor behaviours were reported as most problematic for this sample of boys, whereas Self-injurious behaviours were less problematic. All subscales of the RBS-R were significantly inter-correlated. Nonverbal IQ was negatively related, whereas anxiety and social affective symptoms of autism spectrum disorder were positively related, to scores for Restricted Interests. Anxiety was also positively related to scores for Compulsive behaviours and Ritualistic Sameness behaviours. CONCLUSIONS: This study provides a preliminary description of repetitive behaviours in boys with FXS, which may form the groundwork for future research.

Diagnostic yield of oesophagogastroduodenoscopy in children with abdominal pain.

BACKGROUND: Abdominal pain is the most common indication for oesophagogastroduodenoscopy (OGD) in children. However, existing studies examining the diagnostic outcomes of OGD in children with abdominal pain are limited. AIM: To examine the diagnostic yield of OGD with biopsy in the evaluation of abdominal pain and to describe the endoscopic and histological findings in patients undergoing OGD for abdominal pain of unclear aetiology. METHODS: We performed a retrospective cross-sectional cohort study in children under 18 years of age who had OGD for the primary indication of abdominal pain, at Texas Children's Hospital and Children's Hospital of The King's Daughters from 1 January 2002 to 30 June 2005. RESULTS: Overall, OGD was diagnostic in 454 (38.1%) of the 1191 procedures, including reflux oesophagitis (23%, n = 271), Helicobacter pylori infections (5%, n = 55), peptic ulcers (3%, n = 32), eosinophilic oesophagitis (2%, n = 25), celiac disease (1%, n = 9) and Crohn's disease (0.5%, n = 7). Male gender, older age, elevated C-reactive protein and vomiting were associated with increased diagnostic yield. CONCLUSIONS: Our findings suggest that OGD is valuable for the evaluation of chronic abdominal pain in children, with a diagnostic yield of 38%. The majority of alarm symptoms and routine laboratory tests are not significantly associated with diagnostic yield.

Supporting communication in young children with developmental disabilities.

The behavior of parents, adult caregivers, and peers comprises the critical features of community support for the development of communication in young children with developmental disabilities. In a bio-ecological model of development, communication development is the result of the interactions of individuals with specific characteristics, in particular contexts over time. From the perspective of this model, foundational findings of intervention research to current views of communication development in children with developmental disabilities are summarized. The contributions of individual child characteristics to child-caregiver interactions that support language development are illustrated based on research with children who have autism, Williams syndrome, Down syndrome, and children who use augmentative communication systems. Parent-child interaction and the quality and quantity of parent talk are discussed as factors in children's language development. The effects of young children's delayed language on their interactions with peers, the contributions of peers to children's language learning and use, and the critical features of classroom settings that support child language development are reviewed. MRDD Research Reviews 7:143-150, 2001.

Documenting the primary nursing summary note: a leap toward professionalism.

A newly developed documentation tool greatly enhances professional nursing practice, empowerment, and interdisciplinary collaboration. The Primary Nursing Summary Note captures the essence of the nursing process, records and validates it in the interdisciplinary patient progress notes, and facilitates the provision of high quality, cost-effective health care.

Communicating the financial worth of the CNS through the use of fiscal reports.

To maintain the clinical nurse specialist's (CNS's) leading role within hospital nursing services, routine communication to hospital administration of the impact of the CNS role on the hospital's budget is imperative. The CNS group at Children's Hospital of The King's Daughters implemented a quarterly Fiscal Report to clarify the financial worth of CNS practice. The Fiscal Report presents cost savings and revenue generating activities utilizing the role components of the CNS. During fiscal year 1991, the CNS group reported a total impact of $1,600,000. This article describes in detail the use of the Fiscal Report.

Nutritional support of the critically ill child.

There is a growing awareness that the nutrition an individual receives as a child may exert significant consequences later in life. The successful treatment of critically ill children influences their potential for full recovery and optimal outcome. This requires an understanding of how the child responds to stress and starvation. Daily energy needs of the child in the intensive care unit are highly variable. Specific knowledge of the nutritional assessment of these children, whether sustaining an acute or chronic illness, is required, as is an understanding of how the disease process affects the child. Further work needs to be done to evaluate how chronic illness affects the growth, development, and maturation of the child. Assessment parameters remain somewhat controversial, and recent studies indicate that, indeed, critically ill children may be overfed if standard equations are used to calculate needs. Poor clinical outcomes can occur if the child is underfed or overfed. The long-term results of specific diets, micronutrients, glutamine, and new access routes into the infant are not yet known. Research in these areas is rapidly growing, and the new knowledge will provide a greater ability to meet the individual needs of the critically ill child. Perhaps in the future the treatment of choice in patients with organ failure will involve specific micronutrients that influence the immune status and cellular degradation. In the meantime, critically ill children deserve to have their basic nutritional needs met, and nurses can do much to individualize the nutritional support required to produce optimal patient outcomes.

Nurse self-awareness in therapeutic relationships.

Each individual comes into nursing with personal motivations, values, beliefs, and unresolved family-of-origin issues that can impact on therapeutic relationships with families that nurses care for. Possible underlying motives that may impact a nurse's ability to sustain a therapeutic relationship appropriately are described.

The role of the pediatric clinical nurse specialist in gastroenterology.

Clinical nurse specialists have been employed by hospitals for many years. However, very little published information exists specifically describing the role of the pediatric clinical nurse specialist in the field of gastroenterology. The pediatric GI staff nurse and/or GI assistant may be uncertain as how to best utilize this important nursing resource. The author discusses her own role implementation as a pediatric gastroenterology/nutrition clinical nurse specialist and highlights in particular her role as a resource to the GI nurses.