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.

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

INTRODUCCIÓN: La escala de sedación para procedimientos del Hospital Niño Jesús (ESPHNJ) no ha sido validada. PACIENTES Y MÉTODOS: Estudio analítico prospectivo bihospitalario. Se reclutaron prospectivamente pacientes ≥ 6 meses sometidos a procedimientos invasivos bajo sedoanalgesia con propofol o midazolam y fentanilo. Todos fueron monitorizados con el índice biespectral (IB). Se realizaron videos de cada procedimiento, que fueron editados y aleatorizados. Ciento cincuenta videos fueron puntuados por cuatro observadores con la ESPHNJ, la escala de sedación de la Universidad de Michigan (ESUM) y la Escala de Ramsay (ER). Dichos observadores estaban cegados al IB y al momento de administración de fármacos. Para evaluar la fiabilidad test-retest, 50 videos de los 150 iniciales seleccionados aleatorizadamente fueron reevaluados. RESULTADOS: Fueron incluidos 65 pacientes. La concordancia intraobservadores fue alta (ρ = 0,793). La ESPHNJ presentó una fiabilidad interobservador buena al comparar con la ESUM (CCI = 0,88) y con la ER (CCI = 0,86), y no hubo con el IB. La consistencia interna fue moderada (α = 0,68). La validez de constructo fue demostrada por los cambios en las puntuaciones tras administrar sedantes (p < 0,0001). La ESPHNJ tuvo muy baja correlación con el IB (r = -0,166), y correlación moderada con la ESUM (r = 0,497) y la ER (r = 0,405). En relación a la aplicabilidad, nuestro trabajo se ha empleado en dos hospitales en cinco ámbitos distintos por cuatro profesionales de diferentes categorías. CONCLUSIONES: La ESPHNJ es válida, fiable y aplicable para la monitorización de la sedación en procedimientos invasivos bajo sedoanalgesia profunda en pediatría. La ESPHNJ presenta peores propiedades que la ESUM y que la ER

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

[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.

Meningitis víricas neonatales. Importancia de la reacción en cadena de la polimerasa en su diagnóstico / Neonatal viral meningitis. The importance of the polymerase chain reaction in their diagnosis

Introducción. Las meningitis víricas representan una entidad relativamente frecuente en los recién nacidos, aunque en muchos casos infradiagnosticadas, ante la ausencia de pleocitosis en el líquido cefalorraquídeo (LCR). Objetivos. Describir las características clínicas y los hallazgos de laboratorio de neonatos con meningitis víricas y destacar la importancia de la reacción en cadena de la polimerasa (PCR) en el LCR para diagnosticar esta patología. Pacientes y métodos. Revisión retrospectiva de historias clínicas de neonatos ingresados en la sección de neonatología diagnosticados de meningitis vírica entre mayo de 2014 y mayo de 2017. Resultados. Se registraron 17 casos de meningitis vírica (15 causadas por enterovirus y dos por parecho virus), que constituyenel 14,8% de los neonatos ingresados por síndrome febril. Todos manifestaron fiebre (100%), y otros síntomas destacados fueron irritabilidad (76%) y rechazo de la ingesta (65%). El 88% cursó con celularidad normal en el LCR y sin hiperproteinorraquia, y el 100%, sin hipoglucorraquia, por lo que previamente muchos de estos niños quedaban con el diagnóstico de síndrome febril sin foco. Estos datos resaltan la necesidad de realizar la PCR en el LCR a neonatos con fiebre sin foco, debido a la normalidad de las pruebas complementarias en la mayoría de los casos. El 64,7% de los niños recibió seguimiento neurológico posterior en consulta de neurología, sin objetivarse secuelas neurológicas, salvo en uno de ellos. Conclusiones. La PCR múltiple en el LCR se ha convertido en una técnica diagnóstica imprescindible en el recién nacido con sospecha de infección, y sustituye al cultivo viral como prueba de referencia por su mayor rapidez y sensibilidad

Introduction. The different types of viral meningitis constitute a condition that is relatively frequent in newborn infants, although in many cases they are underdiagnosed due to the absence of pleocytosis in the cerebrospinal fluid (CSF). Aims. To describe the clinical features and laboratory findings of newborn infants with viral meningitis and to highlight the importance of the polymerase chain reaction (PCR) in the CSF to diagnose this condition. Patients and methods. A retrospective review of the medical records of newborn infants hospitalised in the neonatology section who had been diagnosed with viral meningitis between May 2014 and May 2017. Results. Altogether 17 cases of viral meningitis were registered (15 caused by enterovirus and two due to parechovirus), which accounts for 14.8% of all newborns hospitalised owing to febrile symptoms. All of them had fever (100%), and other notable symptoms were irritability (76%) and rejection of feeding (65%). Normal cellularity was found in the CSF without high protein levels in 88% of them, and without hypoglycorrhachia in all of them (100%), which meant that many of these children had previously been left with a diagnosis of a febrile syndrome with no focus. These data stress the need to perform the PCR in the CSF of newborn infants who have a fever without a focus, due to the normal status of the results of the complementary tests in most cases. Subsequent neurological follow-up was performed in 64.7% of the children in the neurology service, without any neurological sequelae being found, except in one case. Conclusions. Multiple PCR in the CSF has become an essential diagnostic technique in cases of newborn infants with a suspected infection, and replaces viral culture as the reference test due its being quicker and more sensitive

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.

Trombosis venosa profunda secundaria a canalización de vena femoral en niños con cetoacidosis diabética / Deep venous thrombosis secondary to femoral vein catheterization in children with diabetic ketoacidosis

Los niños ingresados por cetoacidosis diabética (CAD) suelen manejarse con vías periféricas; sin embargo, algunos pacientes necesitan un catéter central para el manejo inicial. Es sabido que la CAD implica un estado de hipercoagulabilidad que supone un aumento del riesgo de trombosis asociada a catéter. Se presentan 2 casos de trombosis femoral asociada a canalización de vena femoral coincidiendo con cetoacidosis diabética en lactantes de 18 y 27 meses. En ambos la trombosis fue precoz (antes de 72 h tras la inserción) y se desarrolló a pesar de la rápida retirada de la vía central (menor de 48 h). Debido al alto riesgo de trombosis asociada a catéter en los pacientes diabéticos más pequeños (menores de 3 años), debe considerarse individualmente la necesidad de catéter central y evitarlo siempre que sea posible. En caso de canalización, debe valorarse la profilaxis con heparina de bajo peso molecular (AU)

Children admitted to hospital for diabetic ketoacidosis are frequently managed with peripheral venous lines. However, due to the severity of their illness, some patients need central lines for initial treatment. Diabetic ketoacidosis is known to produce hypercoagulability, increasing the risk of catheter-related deep venous thrombosis. We present two patients with diabetic ketoacidosis, aged 18 and 27 months, who developed deep venous thrombosis after placement of femoral central venous catheters. In both patients, the thrombosis occurred within 72 hours of catheter insertion, despite rapid removal of the central lines (less than 48 hours). Due to the high risk of catheter-related thrombosis in patients with diabetic ketoacidosis (especially in children aged less than 3 years old), the need for central venous lines should be evaluated in each patient and avoided as far as possible. Low molecular weight heparin prophylaxis should be considered if a venous central catheter is required (AU)