[Formula: see text]Associations among parent-child relationships and cognitive and language outcomes in a clinical sample of preschool children.

OBJECTIVE: To examine associations among parent-child relationship characteristics and child cognitive and language outcomes. METHODS: Preschool children (n = 72) with early neurological insult completed assessments of cognitive and language functioning and participated in a parent-child semi-structured interaction. RESULTS: Quality of the parent-child relationship accounted for a significant amount of unique variance (12%) in predicting children's overall cognitive and language functioning. Impact of neurological insult was a significant predictor. CONCLUSIONS: Caregiver-child interactions that are harmonious and reciprocal as evidenced by affective and/or verbal exchanges support children's cognitive and language development. Observations of interactions can guide providers in facilitating child- and family-centered interventions.

Outcomes after allogeneic hematopoietic cell transplantation for childhood cerebral adrenoleukodystrophy: the largest single-institution cohort report.

Cerebral adrenoleukodystrophy (cALD) remains a devastating neurodegenerative disease; only allogeneic hematopoietic cell transplantation (HCT) has been shown to provide long-term disease stabilization and survival. Sixty boys undergoing HCT for cALD from 2000 to 2009 were analyzed. The median age at HCT was 8.7 years; conditioning regimens and allograft sources varied. At HCT, 50% demonstrated a Loes radiographic severity score ≥ 10, and 62% showed clinical evidence of neurologic dysfunction. A total of 78% (n = 47) are alive at a median 3.7 years after HCT. The estimate of 5-year survival for boys with Loes score < 10 at HCT was 89%, whereas that for boys with Loes score ≥ 10 was 60% (P = .03). The 5-year survival estimate for boys absent of clinical cerebral disease at HCT was 91%, whereas that for boys with neurologic dysfunction was 66% (P = .08). The cumulative incidence of transplantation-related mortality at day 100 was 8%. Post-transplantation progression of neurologic dysfunction depended significantly on the pre-HCT Loes score and clinical neurologic status. We describe the largest single-institution analysis of survival and neurologic function outcomes after HCT in cALD. These trials were registered at www.clinicaltrials.gov as #NCT00176904, #NCT00668564, and #NCT00383448.