OBJECTIVE: To identify changes in mortality and neonatal morbidities for infants with birth weight 501 to 1500 g born from 2000 to 2009. METHODS: There were 355 806 infants weighing 501 to 1500 g who were born in 2000–2009. Mortality during initial hospitalization and major neonatal morbidity in survivors (early and late infection, chronic lung disease, necrotizing enterocolitis, severe retinopathy of prematurity, severe intraventricular hemorrhage, and periventricular leukomalacia) were assessed by using data from 669 North American hospitals in the Vermont Oxford Network. RESULTS: From 2000 to 2009, mortality for infants weighing 501 to 1500 g decreased from 14.3% to 12.4% (difference, −1.9%; 95% confidence interval, −2.3% to −1.5%). Major morbidity in survivors decreased from 46.4% to 41.4% (difference, −4.9%; 95% confidence interval, −5.6% to −4.2%). In 2009, mortality ranged from 36.6% for infants 501 to 750 g to 3.5% for infants 1251 to 1500 g, whereas major morbidity in survivors ranged from 82.7% to 18.7%. In 2009, 49.2% of all very low birth weight infants and 89.2% of infants 501 to 750 g either died or survived with a major neonatal morbidity. CONCLUSIONS: Mortality and major neonatal morbidity in survivors decreased for infants with birth weight 501 to 1500 g between 2000 and 2009. However, at the end of the decade, a high proportion of these infants still either died or survived after experiencing ≥1 major neonatal morbidity known to be associated with both short- and long-term adverse consequences.

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Importance There are substantial shortfalls in nurse staffing in US neonatal intensive care units (NICUs) relative to national guidelines. These are associated with higher rates of nosocomial infections among infants with very low birth weights. Objective To study the adequacy of NICU nurse staffing in the United States using national guidelines and analyze its association with infant outcomes. Design Retrospective cohort study. Data for 2008 were collected by web survey of staff nurses. Data for 2009 were collected for 4 shifts in 4 calendar quarters (3 in 2009 and 1 in 2010). Setting Sixty-seven US NICUs from the Vermont Oxford Network, a national voluntary network of hospital NICUs. Participants All inborn very low-birth-weight (VLBW) infants, with a NICU stay of at least 3 days, discharged from the NICUs in 2008 (n = 5771) and 2009 (n = 5630). All staff-registered nurses with infant assignments. Exposures We measured nurse understaffing relative to acuity-based guidelines using 2008 survey data (4046 nurses and 10 394 infant assignments) and data for 4 complete shifts (3645 nurses and 8804 infant assignments) in 2009–2010. Main Outcomes and Measures An infection in blood or cerebrospinal fluid culture occurring more than 3 days after birth among VLBW inborn infants. The hypothesis was formulated prior to data collection. Results Hospitals understaffed 31% of their NICU infants and 68% of high-acuity infants relative to guidelines. To meet minimum staffing guidelines on average would require an additional 0.11 of a nurse per infant overall and 0.34 of a nurse per high-acuity infant. Very low-birth-weight infant infection rates were 16.4% in 2008 and 13.9% in 2009. A 1 standard deviation–higher understaffing level (SD, 0.11 in 2008 and 0.08 in 2009) was associated with adjusted odds ratios of 1.39 (95% CI, 1.19–1.62; P < .001) in 2008 and 1.40 (95% CI, 1.19–1.65; P < .001) in 2009. Conclusions and Relevance Substantial NICU nurse understaffing relative to national guidelines is widespread. Understaffing is associated with an increased risk for VLBW nosocomial infection. Hospital administrators and NICU managers should assess their staffing decisions to devote needed nursing care to critically ill infants.

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Fragile X-associated tremor/ataxia syndrome (FXTAS) is a neurodegenerative disorder associated with a premutation trinucleotide repeat expansion in the fragile X mental retardation 1 gene (FMR1). Symptoms include gait ataxia, action tremor, and cognitive impairment. The objectives of the study were to clarify the nature of the dysexecutive syndrome observed in FXTAS and to assess the contribution of executive impairment to deficits in nonexecutive cognitive functions. Compared to controls, men with FXTAS demonstrated significant executive impairment, which was found to mediate group differences in most other cognitive abilities. Asymptomatic premutation carriers performed similarly to controls on all but two measures of executive functioning. These findings suggest that the impairment of non-executive cognitive skills in FXTAS is in large part secondary to executive dysfunction.