Establishing Multiple-Control Responding of Children with Autism to People and Emotions in Context by Utilizing Derived Stimulus Relations.

This study evaluated the efficacy of a set of procedures in evoking responding under multiple stimulus control (identifying emotions when provided a person and context) in a match-to-sample arrangement. Three participants with autism achieved a mastery criterion following direct training of the target relations, and two of the participants additionally demonstrated derived relations without direct training (identifying people when provided an emotion and context). Corrective procedures were effective in promoting the emergence of derived relations for the third participant. These data suggest that incorporating derived stimulus relation training and testing procedures may have utility for clinical interventions in children with autism.

Increasing autophagy and blocking Nrf2 suppress laminopathy-induced age-dependent cardiac dysfunction and shortened lifespan.

Mutations in the human LMNA gene cause a collection of diseases known as laminopathies. These include myocardial diseases that exhibit age-dependent penetrance of dysrhythmias and heart failure. The LMNA gene encodes A-type lamins, intermediate filaments that support nuclear structure and organize the genome. Mechanisms by which mutant lamins cause age-dependent heart defects are not well understood. To address this issue, we modeled human disease-causing mutations in the Drosophila melanogaster Lamin C gene and expressed mutant Lamin C exclusively in the heart. This resulted in progressive cardiac dysfunction, loss of adipose tissue homeostasis, and a shortened adult lifespan. Within cardiac cells, mutant Lamin C aggregated in the cytoplasm, the CncC(Nrf2)/Keap1 redox sensing pathway was activated, mitochondria exhibited abnormal morphology, and the autophagy cargo receptor Ref2(P)/p62 was upregulated. Genetic analyses demonstrated that simultaneous over-expression of the autophagy kinase Atg1 gene and an RNAi against CncC eliminated the cytoplasmic protein aggregates, restored cardiac function, and lengthened lifespan. These data suggest that simultaneously increasing rates of autophagy and blocking the Nrf2/Keap1 pathway are a potential therapeutic strategy for cardiac laminopathies.

Is retinopathy of prematurity increasing among infants less than 1250 g birth weight?

OBJECTIVE: Retinopathy of prematurity (ROP) is a complication seen in many very low birth weight infants. Severe ROP has been called a "marker" for severe disability. The purpose of this study was to evaluate the occurrence and severity of ROP among infants < or =1250 g birth weight treated in the Special Care Nursery at Women & Infants' Hospital over a period of 7 years from 1994 to 2000. STUDY DESIGN: This was a retrospective review of ROP data combined with neonatal follow-up data. Of the 1002 infants born with birth weights <1250 g, ophthalmologic data were available for 739 of 839 survivors. Analysis of variance and chi2 along with logistic regression were used to analyze outcomes. RESULTS: An increase in the overall occurrence of ROP was identified (40% to 54% linear trend, p=0.007). The occurrence of threshold ROP ranged from 2% to 5% (NS). Infants at greatest risk of ROP were those micropremies with birth weights <750 g (p<0.001). CONCLUSION: Severe ROP continues to be a significant morbidity among infants <750 g.