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A study related with molecular pathways that modulate neurotoxicity of the [beta]-amyloid peptide, which is controlled, by another pathway that modulates aging is presented. The association between the life span and the cellular stress response is suggested by the insulin-signaling pathway's requirement for heat-shock factor 1 (HSF-1), the activator of the heat-shock response that induces the expression of molecular chaperones during stress.

A vast amount of data has been generated over the last 30 years regarding neurodegenerative mechanisms. The basic question that has still not been answered is, how can selective cell [...]

OBJECTIVE. Two strategies for surgical management are used for infants with hypoplastic left heart syndrome (HLHS), primary heart transplantation and the Norwood procedure. We sought to determine how these 2 surgical approaches influence neurodevelopmental outcomes at school age. METHODS. A multicenter, cross-sectional study of neurodevelopmental outcomes among school-aged children (>8 years of age) with HLHS was undertaken between July 2003 and September 2004. Four centers enrolled 48 subjects, of whom 47 completed neuropsychologic testing. Twenty-six subjects (55%) had undergone the Norwood procedure and 2i (45%) had undergone transplantation, with an intention-to-treat analysis. The mean age at testing was 12.4 [+ or -] 2.5 years. Evaluations included the Wechsler Abbreviated Scale of Intelligence, Clinical Evaluation of Language Fundamentals, Wechsler Individual Achievement Test, and Beery-Buktenica Developmental Test of Visual-Motor Integration. RESULTS. The mean neurocognitive test results were significantly below population normative values. The mean full-scale IQ for the entire cohort was 86 [+ or -] 14. In a multivariate model, there was no association of surgical strategy with any measure of developmental outcome. A longer hospital stay, however, was associated significantly with lower verbal, performance, and full-scale IQ scores. Aortic valve atresia was associated with lower math achievement test scores. CONCLUSIONS. Neurodevelopmental deficits are prevalent among school-aged children with HLHS, regardless of surgical approach. Complications that result in prolonged hospitalization at the time of the initial operation are associated with neurodevelopmental status at school age. KEY WORDS. hypoplastic left heart syndrome, neurodevelopmental outcome, transplantation, Norwood procedure. URL: www.pediatrics.org/cgi/doi/10.1542/peds.2005-0575

OBJECTIVE. Human cytomegalovirus (CMV) is a ubiquitous human-specific DNA virus and is the main cause of congenital virus infection in developed countries leading to psychomotor impairment and deafness. Diagnostic techniques for CMV detection have greatly improved during recent years with the advent of sophisticated serological and virological methods. The aim of the present study was to assess the diagnostic and prognostic value of detection and quantification of virus in neonatal blood samples of symptomatic and asymptomatic newborns with CMV congenital infection. METHODS. Between January 1997 and December 2003, we studied 99 newborns who were born to women with primary, recurrent, and undefined CMV infection during pregnancy. CMV congenital infection was identified by isolation of the virus in urine within the second week of life. Fifty-eight of 99 infants were infected and were assessed clinically for disease in the newborn period and classified as having symptomatic or asymptomatic infection on the basis of physical, instrumental, and laboratory findings. The infants were followed up from birth according to a protocol of the tertiary NICU at the University of Bologna in a prospective study of long-term sequelae of congenital infection. Forty-seven blood samples were obtained from 47 infants in the neonatal period: 34 were examined for pp65 antigenemia test and 44 for qualitative and quantitative polymerase chain reaction (PCR and qPCR). Sequelae at 12 months were evaluated in a group of 50 infants. RESULTS. Antigenemia was positive in only 10 of 34 samples of infected newborns (29.4.% sensitivity). PCR was performed in 44 samples of infected newborns and was positive in all (100% sensitivity), qPCR showed a finding of [greater than or equal to] 100 copies per 105 of polymorphonuclear leukocytes (PMNLs) in 39 of 44 samples; in the other 5 cases, the number of copies per 105 PMNLs was 10 000 copies did develop sequelae. CONCLUSIONS. Different viremia value ranges are correlated to a different risk of sequelae: ~70% sequelae were found in newborns with a qPCR higher than 10 000 copies per [10.sup.5] PMNLs. Low neonatal viral blood load detected by pp65 antigenemia test and qPCR was highly predictive of absence of sequelae: DNAemia < 1000 copies per [10.sup.5] PMNLs has a negative predictive value of 95%. As an independent predictive factor of outcome, neonatal viremia is another useful element for neonatal counseling and therapeutic choices in symptomatic and asymptomatic newborns. KEY WORDS. cytomegalovirus, DNAemia, pp65 antigenemia, sequelae, sensorineural hearing loss, neurodevelopmental defects, prognostic value. URL: www.pediatrics.org/cgi/doi/10.1542/peds.2005-0629