Risk factors for neurocognitive impairment, emotional distress, and poor quality of life in survivors of pediatric rhabdomyosarcoma: A report from the Childhood Cancer Survivor Study.

BACKGROUND: Prevalence and risk of poor psychological outcomes following rhabdomyosarcoma (RMS) are not well-established. METHODS: Participants in this cross-sectional, case-control study (n = 713 survivors, 42.5% female; mean [SD] age, 30.5 [6.6] years; n = 706 siblings, 57.2% female; mean age, 32.8,[7.9] years) completed measures of neurocognition, emotional distress, and health-related quality of life (HRQOL). Multivariable logistic regression models identified treatments, health behaviors, and chronic conditions associated with impairment. RESULTS: Relative to siblings, more survivors reported neurocognitive impairment (task efficiency: 21.1% vs. 13.7%, emotional regulation: 16.7% vs. 11.0%, memory: 19.3% vs. 15.1%), elevated emotional distress (somatic distress: 12.9% vs. 4.7%, anxiety: 11.7% vs. 5.9%, depression: 22.8% vs. 16.9%) and poorer HRQOL (physical functioning: 11.1% vs. 2.8%, role functioning due to physical problems: 16.8% vs. 8.2%, pain: 17.5% vs. 10.0%, vitality: 22.3% vs. 13.8%, social functioning: 14.4% vs. 6.8%, emotional functioning: 17.1% vs. 10.6%). Cranial radiation increased risk for impaired task efficiency (odds ratio [OR], 2.30; 95% confidence interval [CI], 1.14-4.63), whereas chest and pelvic radiation predicted increased risk of physical functioning (OR, 2.68; 95% CI, 1.16-6.21 and OR, 3.44; 95% CI, 1.70-6.95, respectively). Smoking was associated with impaired task efficiency (OR, 2.06; 95% CI, 1.14-3.70), memory (OR, 2.23; 95% CI, 1.26-3.95), anxiety (OR, 2.71; 95% CI, 1.36-5.41) and depression (OR, 1.77; 95% CI, 1.01-3.11). Neurologic conditions increased risk of anxiety (OR, 2.30; 95% CI, 1.04-5.10), and hearing conditions increased risk of depression (OR, 1.79; 95% CI, 1.05-3.03). Neurologic and hearing conditions, respectively, were associated with impaired memory (OR, 2.44; 95% CI, 1.20-4.95 and OR, 1.87; 95% CI, 1.05-3.35) and poor health perception (OR, 2.62; 95% CI, 1.62-1.28 and OR, 2.33; 95% CI, 1.34-4.06). CONCLUSIONS: RMS survivors are at significant risk for poor psychological outcomes. Advancing therapies for local control, smoking cessation, and managing chronic medical conditions may mitigate poor outcomes following RMS.

Pediatric aleukemic leukemia cutis: report of 3 cases and review of the literature.

Leukemia cutis (LC) denotes a cutaneous infiltration of neoplastic myeloid cells or lymphoid blasts, which can present in the setting of acute myeloid leukemia, particularly in those cases with monocytic or myelomonocytic differentiation. Rarely, cutaneous involvement by a leukemic infiltrate can occur in the absence of bone marrow or peripheral blood involvement by acute leukemia; this then is referred to as aleukemic LC (ALC). Recognition of LC is important for further classification and early diagnosis of the disease, but the diagnosis is difficult in the absence of a systemic presentation of acute leukemia. Although the molecular and cytogenetic features of ALC are poorly characterized, some have shown specific molecular alterations in common with classic forms of acute leukemias. We present 3 cases of ALC in pediatric patients.

A double-blind placebo-controlled pilot study of olanzapine in childhood/adolescent pervasive developmental disorder.

Atypical antipsychotics have been shown to improve disruptive and repetitive behaviors in pervasive developmental disorders (PDDs), but they require assessment of potential side effects. This is the first placebo-controlled trial of olanzapine in the treatment of children and adolescents with PDD. Eleven patients with a diagnosis of either autism, Asperger's syndrome, or PDD not otherwise specified (PDD-NOS) and aged 6-14 years were randomized into an 8-week double-blind, placebo-controlled, parallel treatment study with olanzapine. There was a significant linear trend x group interaction on the Clinical Global Impressions- Improvement (CGI-I) and 50% on olanzapine versus 20% on placebo were responders. Olanzapine was associated with significant weight gain (7.5 +/- 4.8 lbs vs. 1.5 +/- 1.5 lbs on placebo). Olanzapine may be a promising treatment for improving global functioning of PDDs, but the risk of significant weight gain remains a concern. Additional studies are needed to determine the efficacy and safety of olanzapine in the treatment of children with PDD.

Pediatrician attitudes, clinical activities, and knowledge of environmental health in Wisconsin.

Pediatricians can reduce exposures to environmental hazards but most have little training in environmental health. To assess whether Wisconsin pediatricians perceive a relative lack of self-efficacy for common environmental exposures and diseases of environmental origin, we assessed their attitudes and beliefs about the role of the environment in children's health. A 4-page survey was sent to the membership of the Wisconsin Chapter of the American Academy of Pediatrics. We obtained a 35.4% response rate after 1 follow-up mailing. Respondents agreed that the role of the environment in children's health is significant (mean 4.28 +/- .78 on 1-5 Likert scale). They expressed high confidence in dealing with lead exposure (means 4.22-4.27 +/- 1.01-1.09), but confidence in their skills for pesticide, mercury, and mold was much lower (means 2.49-3.09 +/- 1.06-1.26; P<.001). Of those surveyed, 88.6% would refer patients to a clinic "where pediatricians could refer patients for clinical evaluation and treatment of their environmental health concerns." These findings indicate that Wisconsin pediatricians agree that children are suffering preventable illnesses of environmental origin, but feel ill equipped to educate families about many common exposures. Significant demand exists for centers that can evaluate environmental health concerns, as well as for educational opportunities.

Orthographic distinctiveness and semantic elaboration provide separate contributions to memory.

Orthographic distinctiveness and semantic elaboration both enhance memory. The present behavioral and functional magnetic resonance imaging (fMRI) studies examined the relationship between the influences of orthographic distinctiveness and semantic elaboration on memory, and explored whether they make independent contributions. As is typical for manipulations of processing levels, words studied during semantic encoding were better remembered than words studied during nonsemantic encoding. Notably, orthographically distinct words were better recalled and received more remember responses on recognition memory tests than orthographically common words regardless of encoding task, suggesting that orthographic distinctiveness has an additive effect to that of semantic elaboration on memory. In the fMRI study, orthographic distinctiveness and semantic elaboration engaged separate networks of brain regions. Semantic elaboration modulated activity in left inferior prefrontal and lateral temporal regions. In contrast, orthographic distinctiveness modulated activity in distinct bilateral inferior prefrontal, extrastriate, and parietal regions. Orthographic distinctiveness and semantic elaboration appear to have separate behavioral and functional-anatomic contributions to memory.