Transpyloric Tube Placement Shortens Time to Full Feeding in Left Congenital Diaphragmatic Hernia.

BACKGROUND: Nutritional complications have an impact in both short- and long-term morbidity of patients with congenital diaphragmatic hernia (CDH). We aimed to compare time to full enteral tube feeding depending on route -gastric (GT) or transpyloric (TPT)- in newborns with left CDH (L-CDH). METHODS: Retrospective cohort study of L-CDH patients admitted to a referral tertiary care NICU between January 2007 and December 2014. Lethal chromosomal abnormalities and death before initiation of enteral nutrition were exclusion criteria. RESULTS: 37 patients were fed through GT, 46 by TPT. TPT children took 11.0 (6.8) days to reach full enteral tube feeding and spent 16.6 (8.1) days on parenteral nutrition vs 16.8 (14.7) days (p = 0.041) and 22.7 (13.5) days (p = 0.020) of GT patients. TPT children had 3.9 (2.4) days of fasting due to GI issues and 20% had episodes of decreased rates of enteral nutrition for extra-GI complications vs 11.4 (11.1) days (p = 0.028) and 49% (p = 0.006). According to the best fitting model (R2 0.383, p < 0.001), the TPT-group achieved full enteral feeding 8.4 days earlier than the GT-group (95% CI -14.76 to - 2.02 days), after adjustment by severity of illness during the first days, o/e LHR_liver and class of diaphragmatic defect. There were no differences in growth outcomes and length of stay between survivors of GT and TPT groups. CONCLUSION: TPT shortens time to full enteral nutrition, especially in the most severe L-CDH patients. We propose that placement of a TPT at the end of the surgical repair procedure should be considered, especially in higher-risk patients. LEVEL OF EVIDENCE: Treatment study, Level III. Retrospective comparative, case-control study.

Summary of Recommendations and Key Points of the Consensus of Spanish Scientific Societies (SEPAR, SEMICYUC, SEMES; SECIP, SENEO, SEDAR, SENP) on the Use of Non-Invasive Ventilation and High-Flow Oxygen Therapy with Nasal Cannulas in Adult, Pediatric, and Neonatal Patients With Severe Acute Respiratory Failure.

Non-invasive respiratory support (NIRS) in adult, pediatric, and neonatal patients with acute respiratory failure (ARF) comprises two treatment modalities, non-invasive mechanical ventilation (NIMV) and high-flow nasal cannula (HFNC) therapy. However, experts from different specialties disagree on the benefit of these techniques in different clinical settings. The objective of this consensus was to develop a series of good clinical practice recommendations for the application of non-invasive support in patients with ARF, endorsed by all scientific societies involved in the management of adult and pediatric/neonatal patients with ARF. To this end, the different societies involved were contacted, and they in turn appointed a group of 26 professionals with sufficient experience in the use of these techniques. Three face-to-face meetings were held to agree on recommendations (up to a total of 71) based on a literature review and the latest evidence associated with 3 categories: indications, monitoring and follow-up of NIRS. Finally, the experts from each scientific society involved voted telematically on each of the recommendations. To classify the degree of agreement, an analog classification system was chosen that was easy and intuitive to use and that clearly stated whether the each NIRS intervention should be applied, could be applied, or should not be applied.

Summary of Recommendations and Key Points of the Consensus of Spanish Scientific Societies (SEPAR, SEMICYUC, SEMES; SECIP, SENEO, SEDAR, SENP) on the Use of Non-Invasive Ventilation and High-Flow Oxygen Therapy with Nasal Cannulas in Adult, Pediatric, and Neonatal Patients With Severe Acute Respiratory Failure / Sumario de recomendaciones y puntos clave del Consenso de las Sociedades Científicas Españolas (SEPAR, SEMICYUC, SEMES; SECIP, SENeo, SEDAR, SENP) para la utilización de la ventilación no invasiva y terapia de alto flujo con cánulas nasales en el paciente adulto, pediátrico y neonatal con insuficiencia respiratoria aguda grave

Non-invasive respiratory support (NIRS) in adult, pediatric, and neonatal patients with acute respiratory failure (ARF) comprises two treatment modalities, non-invasive mechanical ventilation (NIMV) and high-flow nasal cannula (HFNC) therapy. However, experts from different specialties disagree on the benefit of these techniques in different clinical settings. The objective of this consensus was to develop a series of good clinical practice recommendations for the application of non-invasive support in patients with ARF, endorsed by all scientific societies involved in the management of adult and pediatric/neonatal patients with ARF.To this end, the different societies involved were contacted, and they in turn appointed a group of 26 professionals with sufficient experience in the use of these techniques. Three face-to-face meetings were held to agree on recommendations (up to a total of 71) based on a literature review and the latest evidence associated with 3 categories: indications, monitoring and follow-up of NIRS. Finally, the experts from each scientific society involved voted telematically on each of the recommendations. To classify the degree of agreement, an analog classification system was chosen that was easy and intuitive to use and that clearly stated whether the each NIRS intervention should be applied, could be applied, or should not be applied. (AU)

El soporte respiratorio no invasivo (SRNI) comprende 2 modalidades de tratamiento, la ventilación mecánica no invasiva (VMNI) y la terapia de alto flujo con cánulas nasales (TAFCN) que se aplican en pacientes adultos, pediátricos y neonatales con insuficiencia respiratoria aguda (IRA). Sin embargo, el grado de acuerdo entre las distintas especialidades sobre el beneficio de estas técnicas en diferentes escenarios clínicos es controvertido. El objetivo del presente consenso fue elaborar una serie de recomendaciones de buena práctica clínica para la aplicación de soporte no invasivo en pacientes con IRA, avaladas por todas las sociedades científicas involucradas en el manejo del paciente adulto y pediátrico/neonatal con IRA.Para ello se contactó con las diferentes sociedades implicadas, quienes designaron a su vez a un grupo de 26 profesionales con suficiente experiencia en su aplicación. Se realizaron 3 reuniones presenciales para consensuar las recomendaciones (hasta un total de 71) fundamentadas en la revisión de la literatura y en la actualización de la evidencia disponible en relación con 3 categorías: indicaciones, monitorización y seguimiento del SRNI. Finalmente, se procedió a votación telemática de cada una de las recomendaciones, por parte de los expertos de cada sociedad científica implicada. Para la clasificación del grado de acuerdo se optó por un sistema analógico de clasificación fácil e intuitivo de usar, y que expresara con claridad si el procedimiento relacionado con el SRNI debía hacerse, podía hacerse o no debía hacerse. (AU)

Ecografía pulmonar para el shock cardiogénico en ECMO-VA / Lung ultrasound for cardiogenic shock in VA-ECMO

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Recomendaciones para la asistencia respiratoria en el recién nacido (IV). Ventilación de alta frecuencia, ex-utero intrapartum treatment (EXIT), oxigenador de membrana extracorpórea (ECMO) / Recommendations for respiratory support in the newborn (IV). High frequency ventilation, ex-utero intrapartum treatment (EXIT), extracorporeal membrane oxygenation (ECMO)

Las recomendaciones incluidas en este documento forman parte de una revisión actualizada de la asistencia respiratoria en el recién nacido. Están estructuradas en 12 módulos, y en este trabajo se presenta el módulo 8. El contenido de cada módulo es el resultado del consenso de los miembros del Grupo Respiratorio y Surfactante de la Sociedad Española de Neonatología. Representan una síntesis de los trabajos publicados y de la experiencia clínica de cada uno de los miembros del grupo (AU)

The recommendations included in this document will be part a series of updated reviews of the literature on respiratory support in the newborn infant. These recommendations are structured into 12 modules, and in this work module 8 is presented. Each module is the result of a consensus process amongst all members of the Surfactant and Respiratory Group of the Spanish Society of Neonatology. They represent a summary of the published papers on each specific topic, as well as the clinical experience of each one of the members of the group (AU)

[Recommendations for respiratory support in the newborn (IV). High frequency ventilation, ex-utero intrapartum treatment (EXIT), extracorporeal membrane oxygenation (ECMO)]. / Recomendaciones para la asistencia respiratoria en el recién nacido (IV). Ventilación de alta frecuencia, ex-utero intrapartum treatment (EXIT), oxigenador de membrana extracorpórea (ECMO).

The recommendations included in this document will be part a series of updated reviews of the literature on respiratory support in the newborn infant. These recommendations are structured into 12 modules, and in this work module 8 is presented. Each module is the result of a consensus process amongst all members of the Surfactant and Respiratory Group of the Spanish Society of Neonatology. They represent a summary of the published papers on each specific topic, as well as the clinical experience of each one of the members of the group.

Maternal mutations of FOXF1 cause alveolar capillary dysplasia despite not being imprinted.

Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) is a rare cause of pulmonary hypertension in newborns. Maternally inherited point mutations in Forkhead Box F1 gene (FOXF1), deletions of the gene, or its long-range enhancers on the maternal allele are responsible for this neonatal lethal disorder. Here, we describe monozygotic twins and one full-term newborn with ACD and gastrointestinal malformations caused by de novo mutations of FOXF1 on the maternal-inherited alleles. Since this parental transmission is consistent with genomic imprinting, the parent-of-origin specific monoallelic expression of genes, we have undertaken a detailed analysis of both allelic expression and DNA methylation. FOXF1 and its neighboring gene FENDRR were both biallelically expressed in a wide range of fetal tissues, including lung and intestine. Furthermore, detailed methylation screening within the 16q24.1 regions failed to identify regions of allelic methylation, suggesting that disrupted imprinting is not responsible for ACDMPV.

The Burden of Respiratory Disease in Very-Low-Birth-Weight Infants: Changes in Perinatal Care and Outcomes in a Decade in Spain.

BACKGROUND: Advances in perinatal care have led to a significant reduction in morbidity and mortality among very-low-birth-weight (VLBW) infants. Much of this progress is related to the prevention and management of respiratory disease. OBJECTIVES: To evaluate changes in perinatal care and its influence on respiratory morbidity and mortality among VLBW infants in Spain in 2 consecutive periods (2002-2006 and 2007-2011). METHODS: This is a retrospective analysis of data prospectively collected of all VLBW infants included in the Spanish SEN1500 network. Patients with major congenital anomalies, those who died in the delivery room (DR) and infants <230 or >346 weeks of gestational age (GA) were excluded. RESULTS: During the study period, out of 27,205 eligible VLBW infants, 24,598 (90.4%) met inclusion criteria. The most striking and statistically significant results found in the second period were: (i) reduction in the proportion of "outborn" patients; (ii) an increase in prenatal steroid administration; (iii) enhanced non-invasive respiratory support in the DR and NICU; (iv) reduction in invasive mechanical ventilation, surfactant administration, and steroids for bronchopulmonary dysplasia (BPD). Moreover, survival to hospital discharge increased (83.5 vs. 84.7%; p = 0.015); however, survival without BPD increased only among the most immature (230 to 266 weeks' GA) from 26.6 to 31.6% (p < 0.001). CONCLUSIONS: Enhanced adherence to international recommendations in perinatal care and a significant reduction in mortality were found during the second period. Survival without BPD increased only among the most immature. Further investigation is needed to optimize the strategies to prevent and manage respiratory disease in this group of patients.

Patent Ductus Arteriosus Banding for Circular Shunting After Pulmonary Valvuloplasty.

We report two cases of newborns with critical pulmonary stenosis having intact ventricular septum, who underwent pulmonary valve balloon valvuloplasty followed by banding of a patent ductus arteriosus. Transcatheter pulmonary valvuloplasty was performed one week after delivery. Following the procedure, both developed "circular shunting" as a consequence of left-to-right ductal flow and pulmonary regurgitation. This in turn caused increased blood flow into a dysfunctional right ventricle and low systemic cardiac output syndrome. The PDA banding was performed urgently as a rescue measure in order to restore systemic flow while still maintaining some duct-dependent pulmonary blood flow. This approach resolved the circular shunting. Outcome was favorable in both the patients.

Aortic thrombosis successfully treated with local recombinant tissue plasminogen activator in a newborn.

Arterial thrombosis in newborns varies depending on the location of the thrombus and can be a life-threatening emergency. We present a case of extensive aorta thrombosis with a left kidney thrombosis in a newborn successful treated with local recombinant tissue-type plasminogen activator.

Characteristics and outcomes of transposition of great arteries in the neonatal period.

INTRODUCTION AND OBJECTIVES: Transposition of the great arteries is a prevalent congenital heart defect with a high survival rate and a good long-term outcome, especially if managed with early surgical intervention during the neonatal period. In this study, our main objective was to describe patient characteristics and outcomes and to identify possible predictors of early and long-term morbidity and mortality. METHODS: Retrospective analysis through review of clinical and surgical charts of patients with transposition of the great arteries admitted to the service of neonatology during 2000-2011. RESULTS: The study included 136 patients; 119 of them had undergone corrective surgery during the neonatal period. Patients were divided into 3 groups: group I, 81 cases of isolated transposition; group II, 24 cases with ventricular septal defect; and group III, 31 with "complex" transposition of the great arteries. The overall postoperative survival was 96.7% (115 of 119 patients); no patients from group I died after surgery. Duration of surgery, intubation, inotropic treatment, and length of stay were higher in patients in groups II and III. The overall survival rate after an average of 6 years of follow-up was 90.4% 123 of 136 patients, with no deaths after discharge in group I. The most frequent residual defect during cardiac follow-up was supravalvular pulmonary stenosis, in 33 of 113 patients that had follow-up data. CONCLUSIONS: In our study, the survival rate was high in patients with transposition of great arteries and especially in those undergoing arterial switch. The number of subsequent residual heart defects was low.

Características y evolución de la transposición de grandes vasos en el periodo neonatal / Characteristics and outcomes of transposition of great arteries in the neonatal period

Introducción y objetivos. La transposición de grandes vasos es una cardiopatía congénita frecuente, con alta supervivencia y escaso número de secuelas, especialmente si se interviene precozmente, en el periodo neonatal. Nuestro objetivo es describir las características y la evolución de los pacientes afectados y determinar posibles factores pronósticos de morbimortalidad precoz y a largo plazo. Métodos. Estudio retrospectivo mediante revisión de historias clínicas y base de datos de los pacientes con transposición de grandes vasos ingresados entre los años 2000 y 2011. Resultados. Ingresaron 136 pacientes afectados de transposición de grandes vasos, de los que 119 se sometieron a cirugía correctora durante el periodo neonatal. Los pacientes se dividieron en tres grupos: grupo I , 81 transposición de grandes vasos simple; grupo II , 24 con comunicación interventricular concomitante, y grupo III , 31 transposiciones «complejas». La supervivencia postoperatoria fue de 96,7% (115 de 119 pacientes), aunque ningún paciente del grupo I falleció. La duración de la cirugía, la intubación y el uso posquirúrgico de inotrópicos y el tiempo de ingreso fueron mayores para los pacientes de los grupos II y III . Tras una media de 6 años de seguimiento, el 90,4% (123 de 136) de los pacientes estaban vivos. La lesión residual más frecuente en el seguimiento fue la estenosis supravalvular pulmonar en 33 de 113 pacientes seguidos. Conclusiones. En nuestro estudio, la supervivencia en el conjunto de pacientes con transposición de grandes vasos, y especialmente los sometidos a switch arterial, es alta. Los pacientes con formas más complejas tienen más complicaciones hospitalarias, pero no tras el seguimiento (AU)

Introduction and objectives. Transposition of the great arteries is a prevalent congenital heart defect with a high survival rate and a good long-term outcome, especially if managed with early surgical intervention during the neonatal period. In this study, our main objective was to describe patient characteristics and outcomes and to identify possible predictors of early and long-term morbidity and mortality. Methods. Retrospective analysis through review of clinical and surgical charts of patients with transposition of the great arteries admitted to the service of neonatology during 2000-2011. Results. The study included 136 patients; 119 of them had undergone corrective surgery during the neonatal period. Patients were divided into 3 groups: group I, 81 cases of isolated transposition; group II, 24 cases with ventricular septal defect; and group III, 31 with "complex" transposition of the great arteries. The overall postoperative survival was 96.7% (115 of 119 patients); no patients from group I died after surgery. Duration of surgery, intubation, inotropic treatment, and length of stay were higher in patients in groups II and III. The overall survival rate after an average of 6 years of follow-up was 90.4% 123 of 136 patients, with no deaths after discharge in group I. The most frequent residual defect during cardiac follow-up was supravalvular pulmonary stenosis, in 33 of 113 patients that had follow-up data. Conclusions. In our study, the survival rate was high in patients with transposition of great arteries and especially in those undergoing arterial switch. The number of subsequent residual heart defects was low (AU)