Clinical Practice Guidelines From the Cystic Fibrosis Foundation for Preschoolers With Cystic Fibrosis.

Cystic fibrosis (CF) clinical care guidelines exist for the care of infants up to age 2 years and for individuals ≥6 years of age. An important gap exists for preschool children between the ages of 2 and 5 years. This period marks a time of growth and development that is critical to achieve optimal nutritional status and maintain lung health. Given that disease often progresses in a clinically silent manner, objective and sensitive tools that detect and track early disease are important in this age group. Several challenges exist that may impede the delivery of care for these children, including adherence to therapies. A multidisciplinary committee was convened by the CF Foundation to develop comprehensive evidence-based and consensus recommendations for the care of preschool children, ages 2 to 5 years, with CF. This document includes recommendations in the following areas: routine surveillance for pulmonary disease, therapeutics, and nutritional and gastrointestinal care.

Attention and working memory abilities in children treated for acute lymphoblastic leukemia.

BACKGROUND: To extend investigation beyond global cognitive measures prevalent in the literature, this study examined attention and working memory (WM) abilities of survivors of childhood acute lymphoblastic leukemia (ALL), the separate contributions of attention and WM to intelligence quotient (IQ), and their association with neuroimaging changes. METHODS: Ninety-seven children with ALL received risk-directed therapy based on presenting clinical and biological factors. During consolidation therapy, low-risk patients received half the dose of intravenous methotrexate that standard-risk/high-risk patients received, and fewer doses of triple intrathecal therapy. Patients were classified according to end of consolidation magnetic resonance imaging scans (normal or leukoencephalopathy), and continuous measures of white matter structure were computed. As part of the protocol study, children completed cognitive assessment 2 years later (completion of therapy), using Digit Span Forward (DSF) for attention and Digit Span Backward (DSB) for WM. RESULTS: For the total sample and the standard-/high-risk group, Total Digit Span (TDS), DSF, and DSB were impaired relative to norms (P<.05). In the low-risk group, only DSB was impaired (P<.0001). Across groups, a higher percentage of patients performed below the average range (scale score<7) on DSB (66%) compared with the DSF (14%) or TDS (18%). Regression analysis indicated that DSB predicted estimated IQ (P<.05), after accounting for DSF. Leukoencephalopathy was predictive of lower TDS (P<.05). CONCLUSIONS: WM appears to be especially sensitive to treatment-related changes in ALL survivors, detecting difficulties potentially missed by global intelligence measures. These findings may facilitate the identification of vulnerable neural pathways and the development of targeted cognitive interventions.