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Background:The Ramsay scale is the most widely used scale during pediatric procedures although it has not been formally validated.Objective: To validate the Ramsay scale during invasive procedures under sedation in pediatrics.Methods: A prospective analytic study was conducted in two hospitals. All patients ≥6 months that were undergoing invasive procedures under sedation were enrolled.All were recorded, and these videos were edited and randomized. 150 videos were scored by four observers (a pediatrician, a pediatric intensive care unit nurse, an anesthetist, and an operating room nurse). All videos were scored with the Ramsay scale and University of Michigan Sedation Scale. Observers were blinded to drug administration. Construct validity was measured through Wilcoxon test paired samples after administration of sedatives. Criterion validity, intra-observer reliability, and interobserver correlation were evaluated by comparing the scores of the scales using Spearman's correlation coefficient. Interobserver agreement was measured using the intraclass correlation coefficient. To assess test-retest reliability, 50 videos were randomly selected and reevaluated.Results: Sixty-five patients were included. Construct validity was demonstrated through changes in the Ramsay scale scoring after administration of sedatives (p < .0001). Regarding criterion validity, the Ramsay scale had a high correlation with the UMSS (ρ = 0.621). Intra-observer agreement was ρ = 0.884. The Ramsay scale showed interobserver reliability with an intraclass correlation coefficient = 0.94 when comparing it with the University of Michigan Sedation Scale. Internal consistency was α = 0.91. Regarding applicability, in our study, it was applied in two hospitals in different areas by four professionals from distinct categories. Conclusions:The Ramsay scale is valid, reliable, and applicable to monitoring sedation for invasive procedures under deep sedation in pediatrics.

Microcephaly is defined by a head circumference that is at least two standard deviations below the mean for age and sex of the general population in a specific race. Primary microcephaly may occur as an isolated inborn error, which may damage to the central nervous system or as part of the congenital abnormalities associated with genetic syndrome, affecting multiple organ systems. One of the syndromic forms consists of microcephaly, seizures, and developmental delay caused by biallelic mutations in the gene that encode polynucleotide kinase 3′ − phosphatase protein (PNKP). We report here a newborn male who presented with microcephaly, severe developmental delay, and early-onset refractories seizures, caused by a novel homozygous mutation of the PNKP gene.

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