In Spain, the most frequent clinical presentation of CD is the classical form, mainly diagnosed during the first 2 years of life. The observed incidence of CD in Spanish children is much higher than the present CD incidence rates observed in other European countries.

BackgroundMinocycline is an old tetracycline antibiotic that has shown antiinflammatory and antiapoptotic properties in different neurological disease mouse models. Previous single arm study in humans demonstrated benefits in individuals with Angelman Syndrome (AS); however, its efficacy in patients with Angelman Syndrome has not been assessed in a controlled trial.This was a randomized, double-blind, placebo-controlled, crossover trial in individuals with AS, aged 6 years to 30 years (n = 32, mean age 12 [SD 6·29] years). Participants were randomized to minocycline or placebo for 8 weeks and then switched to the other treatment (a subset of 22 patients) or to receive minocycline for up to 16 weeks (10 patients). After week 16, all patients entered a wash-out 8-week follow-up period.ResultsThirty-six subjects were screened and 34 were randomized. Thirty two subjects (94·1%) completed at least the first period and all of them completed the full trial. Intention-to-treat analysis demonstrated the lack of significantly greater improvements in the primary outcome, mean changes in age equivalent of the development index of the Merrill-Palmer Revised Scale after minocycline compared with placebo (1·90 ± 3·16 and 2·00 ± 3·28, respectively, p = 0·937). Longer treatment duration up to 16 weeks did not result in better treatment outcomes (1·86 ± 3·35 for 8 weeks treatment vs 1·20 ± 5·53 for 16 weeks treatment, p = 0·667). Side effects were not significantly different during minocycline and placebo treatments. No serious adverse events occurred on minocycline.ConclusionsMinocycline treatment for up to 16 weeks in children and young adults with AS resulted in lack of significant improvements in development indexes compared to placebo treatment. Treatment with minocycline appears safe and well tolerated; even if it cannot be completely ruled out that longer trials might be required for a potential minocycline effect to be expressed, available results and lack of knowledge on the actual mechanism of action do not support this hypothesis.Trial registrationEuropean Clinical Trial database (EudraCT 2013-002154-67), registered 16th September 2013; US Clinical trials database (NCT02056665), registered 6th February 2014.Electronic supplementary materialThe online version of this article (10.1186/s13023-018-0891-6) contains supplementary material, which is available to authorized users.

Objective This study aimed to use real-time safety audits to establish whether preparation of the equipment required for the stabilization and resuscitation of newborns in the delivery room areas is adequate. Study Design This was a descriptive, multicenter study performed at five-level III-A neonatal units in Madrid, Spain. For 1 year, one researcher from each center performed random real-time safety audits (RRTSAs), on different days and during different shifts, of at least three neonatal stabilization areas, either in the delivery room or in the operating room used for caesarean sections. Three factors in each area were reviewed: the set-up of the radiant warmer, the materials, and medication available. The global audit was considered without defect when no errors were detected in any of the audited factors. Possible differences in the results were analyzed as a function of the study month, day of the week, or shift during which the audit had been performed. Results A total of 852 audits were performed. No defects were detected in any of the three factors analyzed in the 534 (62.7%, 95% confidence interval [CI]: 59.3–65.9) cases. Slight defects were detected in 98 (11.5%, 95% CI: 9.4–13.8) cases and serious defects capable of producing adverse events in the newborn during resuscitation were found in 220 (25.8%, 95% CI: 22.9–28.9) cases. No statistically significant differences in the results were found according to the day of the week or time during which the audits were performed. However, the percentage of RRTSAs without defect increased as the study period progressed (first quarter 38.1% vs. the last quarter 84.2%; p < 0.001). Conclusion The percentage of adequately prepared resuscitation areas was low. RRTSAs made it possible to detect errors in the correct availability of the neonatal stabilization areas and improved their preparation by preventing errors from being perpetuated over time. Key Points

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