No abstract

This small, prospective, open-label study suggests that memantine may be useful in the treatment of memory functioning and some behavioral symptoms in PDDs. The investigators did not see the same degree of change as endorsed by caretakers. Controlled studies are needed to substantiate and clarify these preliminary findings.

Medical advances have signifi cantly improved the longevity of patients with cystic fi brosis (CF). The median survival age is approximately 37 years, and many patients live well into middle age. 1 Newborn screening (NBS) is an innovation that provides early intervention for affected infants. Although neo natal diagnosis offers nutritional advantages, over time, patients experience complications that require increasingly complex treatments 2-4 for which they and their families typically shoulder most of the burden. The progressive nature of CF and treatment side effects can adversely affect every aspect of life. Thus, there has been a growing interest in understanding how this chronic illness and related life-long care affect health-related quality of life (HRQOL) for persons with CF.This study examined relationships between pulmonary health and HRQOL in patients with CF who participated in the Wisconsin CF Neonatal Screening randomized clinical trial (RCT). 2 Although HRQOL Background: The objective of this study was to examine relationships between pulmonary health and health-related quality of life (HRQOL) in patients with cystic fi brosis (CF) evaluated longitudinally in the Wisconsin Newborn Screening Project. Methods: Patients aged 8 to 18 years (mean Ϯ SD, 13.5 Ϯ 2.8) in early diagnosis (n 5 45) and control (n 5 50) groups completed Cystic Fibrosis Questionnaires (CFQs) to measure HRQOL at three data points over a 2-year period. Pulmonary health was evaluated concurrently by the Wisconsin chest x-ray scoring system (WCXR) and pulmonary function tests (PFTs). Results: WCXR showed signifi cant group differences ( P Յ .023), with the early diagnosis group showing more-severe lung disease. When adjusted for group differences in mucoid Pseudomonas aeruginosa status and pancreatic status, however, WCXR differences and PFT data were not signifi cant. Most patients (74%) had FEV 1 values Ն 80% predicted (within normal range). For patients aged , 14 years, as WCXR scores worsened CFQ respiratory and physical domain scores decreased (both P Յ .007). FEV 1 /FVC showed a positive relationship with the respiratory and physical domains (both P Յ .006). WCXR scores for patients aged Ն 14 years were associated with CFQ weight, respiratory, and health domains (all P Յ .011). FEV 1 was associated with CFQ weight, respiratory, health, and physical domains (all P Յ .003). Changes in pulmonary health were not associated with changes in CFQ over time. Signifi cant group differences on the CFQ-Child social functioning domain favored the control group. Conclusions: To our knowledge, this study is the fi rst to compare pulmonary outcomes with HRQOL indicators assessed by serial, standardized, patient-reported outcome measures for patients with CF identifi ed either through newborn screening or diagnosed by use of traditional methods. This study found no benefi ts of newborn screening for pulmonary health or HRQOL after controlling for risk factors. Using WCXR and PFT data collectively helped to identify associations between pulmo...

Adolescents with CF require supports in school that foster their sense of self-efficacy and accommodations that address the learning time lost from extended health-related absences.

Stress and associated alterations in hypothalamic-pituitary-adrenal (HPA) function have deleterious impacts on the development of multiple mental and physical health problems. Prior research has aimed to identify individuals most at risk for the development of these stress-related maladies by examining factors that may contribute to inter-individual differences in HPA responses to acute stress. The objectives of this study were to investigate, in adolescents, 1) whether differences in neurocognitive abilities influenced cortisol reactivity to an acute stressor, 2) whether internalizing psychiatric disorders influenced this relationship, and 3) whether acute cognitive stress-appraisal mechanisms mediated an association between neurocognitive function and cortisol reactivity. Subjects were 70 adolescents from a community sample who underwent standardized neurocognitive assessments of IQ, achievement, and declarative memory measures at mean age 14 and whose physiological and behavioral responses to a standardized psychosocial stress paradigm (Trier Social Stress Test, TSST) were assessed at mean age 18. Results showed that, among all adolescents, lower nonverbal memory performance predicted lower cortisol reactivity. In addition, internalizing disorders interacted with verbal memory such that the association with cortisol reactivity was strongest for adolescents with internalizing disorders. Finally, lower secondary cognitive appraisal of coping in anticipation of the TSST independently predicted lower cortisol reactivity but did not mediate the neurocognitive–cortisol relationship. Findings suggest that declarative memory may contribute to inter-individual differences in acute cortisol reactivity in adolescents, internalizing disorders may influence this relationship, and cognitive stress appraisal also predicts cortisol reactivity. Developmental, research, and clinical implications are discussed.