Validation of the Ramsay scale for invasive procedures under deep sedation in pediatrics.

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.

A Novel c.968C > T homozygous Mutation in the Polynucleotide Kinase 3' - Phosphatase Gene Related to the Syndrome of Microcephaly, Seizures, and Developmental Delay.

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). In this article, we reported 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.

[Validity and reliability of the Niño Jesús Hospital procedural sedation-analgesia scale of the under deep sedation-analgesia]. / Validez y fiabilidad de la escala de sedación para procedimientos del Hospital Niño Jesús bajo sedoanalgesia profunda.

INTRODUCTION: The procedural sedation scale of the Niño Jesús Hospital (Madrid) (SSPNJH) has not been validated. PATIENTS AND METHODS: A prospective analytical study was conducted in 2 hospitals on patients ≥ 6 months undergoing invasive procedures using sedation-analgesia with propofol or midazolam and fentanyl. All were monitored using the bispectral index (BIS). Videos were made of each procedure, which were then edited and randomised. A total of 150 videos were rated by four observers using the SSPNJH, the sedation scale of the University of Michigan (UMSS), and the Ramsay Scale (SR). These observers were blinded to the BIS, and at the time of drug administration. To assess test-retest reliability, 50 of the initial 150 randomly selected videos were re-assessed. RESULTS: The study included a total of 65 patients. The within-observer agreement was high (ρ = 0.793). The SSPNJH gave a good interobserver reliability when compared with the UMSS (ICC = 0.88) and the SR (ICC = 0.86), and there was none with the BIS. Internal consistency was moderate (α = 0.68). Construct validity was demonstrated by changes in scores after administering sedatives (p < 0.0001). The SSPNJH had a very low correlation with the BIS (r = -0.166), and a moderate correlation with the UMSS (r = 0.497) and the SR (r = 0.405). As regards the applicability, this scale has been used in two hospitals in five different areas by four professionals of different categories. CONCLUSIONS: The SSPNJH is valid, reliable and applicable for sedation monitoring in invasive procedures under deep sedation-analgesia in paediatric patients. The SSPNJH has worse properties than the UMSS and the SR.

Atypical glycine encephalopathy in an extremely low birth weight infant: description of a new mutation and clinical and electroencephalographic analysis.

We present the clinical course and EEG evolution of an extreme low birth weight preterm neonate with an uncommon type of glycine encephalopathy. The patient presented with myoclonic jerks, apnea and encephalopathy three months after birth without satisfactory therapeutic response. During the first days of clinical symptoms the patient presented a paroxystic burst-attenuation EEG pattern which progressively evolved into an established typical burst-suppression pattern within a few days. West syndrome occurred four weeks later and the patient died at seven months of extra-uterine life due to a serious respiratory infection with cardio-respiratory arrest. Genetic analysis showed a non-previously described mutation affecting a consensus splice site (IVS2-1G > C 3) in the AMT gene encoding the T protein of the glycine cleavage system.