We present here a very illustrative case of a rare pathology of recessive congenital methemoglobinemia. The patient, a newborn female, has the variant NM_000398.7:c.535G>A, p.(Ala179Thr) of the CYB5R3 gene in homozygosis, a variant classified as pathogenic. The reported population frequency of the allele is 0.0128%, reason why it is remarkable to find both parents are heterozygous carriers without consanguinity. A brief review of previously published cases are presented.

Introduction: Respiratory distress syndrome (RDS) is the most frequent cause of respiratory distress in preterm neonates. In the management of RDS, surfactant plays a pivotal role, but there are no evidence-based recommendations for moderate/late preterm neonates (32-36 weeks). Methods: A scientific committee developed a questionnaire with 53 questions addressing diagnosis, treatment, potential complications and future trends in RDS specifically focused on moderate and late preterm neonates. This was followed by the performance of a Delphi survey of expert neonatologists. Results: Consensus was reached on 98 of the 109 items. The recommendations for the diagnosis of RDS included performing a lung ultrasound and including mild respiratory distress, transient tachypnoea of the newborn, congenital pneumonia and primary pulmonary hypertension in the differential diagnosis. Most panellists agreed on the need for studies that determine the benefit/harm balance, clinical profile and methods of surfactant administration in moderate/late preterm neonates. All respondents would use the MIST approach with devices specifically designed for surfactant administration. Regarding sedation measures during MIST, most participants agreed on the use of nonpharmacological interventions and, if these proved ineffective, an opioid. All respondents agreed that moderate/late preterm neonates are at increased risk of neonatal morbidity and mortality, particularly respiratory problems, and considered the need for more specialised monitoring in hospital follow-up visits in neonates with associated risk factors or a history of complications in the neonatal period. Finally, all respondents agreed that there is a lack of studies identifying risk factors and medium-term adverse outcomes in moderate/late preterm neonates. Conclusion: This expert consensus will help with the diagnosis and management of RDS and guide decision-making about surfactant administration in moderate/late preterm neonates. (c) 2024 Asociacion Espanola de Pediatr& imath;a. Published by Elsevier Espana, S.L.U. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/ 4.0/).

Introducción El síndrome de distrés respiratorio (SDR) es la causa más frecuente de distrés respiratorio en los recién nacidos prematuros. El surfactante desempeña un papel fundamental en el tratamiento del SDR, pero no existen recomendaciones basadas en la evidencia en neonatos prematuros moderados/tardíos (32-36 semanas). Métodos Un Comité Científico diseñó un cuestionario con 53 preguntas que abordaban el diagnóstico, el tratamiento, las posibles complicaciones y las tendencias futuras del SDR, específicamente en los recién nacidos prematuros moderados y tardíos. Posteriormente, se llevó a cabo una encuesta Delphi entre neonatólogos con experiencia en el campo. Resultados Se alcanzó consenso en 98 de los 109 ítems incluidos. Se recomendó la realización de una ecografía pulmonar y la consideración de la dificultad respiratoria leve, la taquipnea transitoria del recién nacido, la neumonía congénita y la hipertensión pulmonar primaria durante el diagnóstico diferencial. La mayoría de los panelistas coincidieron en la necesidad de realizar estudios para determinar el riesgo/beneficio, el perfil clínico y los métodos de administración de surfactante en neonatos prematuros moderados/tardíos. Se recomendó la técnica MIST con dispositivos específicamente diseñados para la administración de surfactante. La mayoría de los participantes coincidieron en recomendar el uso de procedimientos no farmacológicos de sedación durante el MIST y, en caso de ineficacia, un opiáceo. Todos los encuestados coincidieron en que los prematuros moderados/tardíos presentan mayor riesgo de morbimortalidad neonatal, en particular de problemas respiratorios, y consideraron necesario más monitorización especializada en el seguimiento hospitalario de neonatos con mayor riesgo y/o complicaciones. Por último, todos los encuestados coincidieron en la falta de estudios para identificar factores de riesgo y resultados adversos a medio plazo en neonatos prematuros moderados/tardíos. Conclusiones Este consenso de expertos será de ayuda en el diagnóstico y manejo del SDR y en la decisión de administrar surfactante en neonatos prematuros moderados/tardíos.

Background Bronchopulmonary dysplasia (BPD) is still a major concern in preterm infants and adequate screening methods for secondary right ventricle (RV) failure are needed. Early detection could be aided by taking measurements of RV deformation using speckle tracking echocardiography. A prospective longitudinal study was carried out over 28 months at a tertiary care pediatric cardiology center. Preterm infants < 32 weeks gestational age (GA) were eligible for the study. Enrolled patients were separated in two groups: NO-BPD or BPD. At three timepoints over the first year of life, echocardiogram measurements were performed. Right ventricle strain was studied using speckle tracking analysis and compared to conventional function parameters. Results Fifty patients were enrolled in the study, 22 in the NO-BPD group and 28 in the BPD group. RV strain showed no statistical differences between groups. However, the BPD group showed worse RV function than the NO-BPD group, using speckle tracking analysis and other conventional parameters. During the study follow-up, an improvement trend is shown in RV strain. Conclusions RV longitudinal strain and strain rate derived by speckle tracking is feasible in preterm infants. Although there seems to be a good correlation between RV strain and BPD severity, the results of this study were not conclusive. More studies should be carried out to investigate the optimum echocardiographic screening model of RV dysfunction in BPD patients.

INTRODUCTION AND OBJECTIVE:Neonatal hypoglycemia offers multiple controversies. The study aims to assess the main factors involved in the development of early hypoglycemia in term and late preterm infants, and the implication of different environmental circumstances.METHODS:A prospective cohort study, in infants born between 34 0/7 weeks and 36 6/7 weeks of gestation. Three capillary blood glucose determinations were performed during the eight first hours after birth.SAMPLE SIZE:207; 59 neonates developed hypoglycemia.RESULTS:Prenatal risk factors include gestational diabetes with poor glycemic control, twin pregnancy and gestational age. The presence of meconium amniotic fluid and planned cesarean delivery are associated with a higher probability of hypoglycemia. After birth, skin to skin contact, breastfeeding, soft lightening, and normothermia are described as protective factors. The predictive model that combines the type of lightening, body temperature and the excess of bases level, correctly classifies 98% of the severe hypoglycemia cases, with a high Nagelkerke R2 value (0.645) and specificity of 99.5%.CONCLUSIONS:Postnatal environmental factors seem to be directly related to early hypoglycemia development, so it is essential to support the maternal-child union and breastfeeding. Our results allow better identification of neonates who are not subsidiary to performing blood glucose determinations because they have little risk of developing it.

Detailed below is a very illustrative case of a rare pathology of recessive congenital methemoglobinemia. The patient, a newborn female, was homozygous for c.535G > A, p.(Ala179Thr) a pathogenic variant in the CYB5R3 gene. The reported population frequency of the allele is 0.853%, demonstrating why it is remarkable to find both parents are heterozygous carriers without consanguinity. A brief review of previously published cases is also presented.

Introduction and objective: neonatal hypoglycemia persistently offers multiple diagnostic controversies. This study aims to present the current situation regarding neonatal hypoglycaemia detection, and to gain insight into the most widely used portable glucometers in neonatal units today. Methods: an online questionnaire was prepared and sent to the members of the Spanish Society of Neonatology; a total of 75 hospitals participated. Results: portable glucometers continue to be widely used in the neonatal population. More than 75 % of units perform neonatal hypoglycemia screening in specific clinical circumstances, and 13 % of units continue to perform protocolized screening on all newborns at neonatal units. The higher the level of care, the higher the percentage of hypoglycaemia detection by other tests (such as blood gas analysis): chi(2), p = 0.019. Multiple models of portable glucometers are currently used, with differences according to level of care (chi(2), p = 0.01). Nova Biomedical, Abbott, and Roche Diagnostics models are most commonly used. Conclusions: differences in the performance of neonatal hypoglycaemia screening are observed, so standardised procedures and limiting the neonatal population at risk are important to reduce variability in clinical practice, and to improve the quality of neonatal care.

Introduction: Neonatal cardiogenic shock is an uncommon condition whose detection and management continues to be a major challenge in the NICU. Clinical case: We present a case of neonatal cardiogenic shock in a 25 days old newborn admitted in the NICU due to renal failure, where the point-of-care ultrasound was the main tool for hemodynamic management. After progressive deterioration of renal function (creatinine 5.67mg/dl, urea 96mg/dl), she needs peritoneal dialysis during 6 days. Two weeks after the extrarrenal assistance withdrawal, and simultaneously with a blood transfusion, she suffers a clinical descompensation with maintained blood pressure >p99, tachycardia, oligoanuria and respiratory distress needing CPAP. Point-of-care ultrasound is practiced showing systolic dysfunction and left ventricle dilatation (left ventricle ejection fraction 23%), moderate mitral and tricuspid insufficiency, mild aortic insufficiency and acute pulmonary edema findings. The clinical signs supported by the echographical findings presuppose an increased systemic postcharge within nephrogenic hypertension and hypervolemia due to blood transfusion, triggering a left heart failure with postcapillary pulmonary hypertension, acute pulmonary edema and, secondarily, right heart overload. Diuretics are started (furosemide=1mg/kg/h) and inodilators (milrinone=0.75mcgr/kg/min and dobutamine=5mcgr/kg/min), monitoring the hemodynamic response with point-of-care ultrasound, adding four hours later vasodilators (nitroprusside=0, 75 mcgr/kg/min). Early heart failure improvement is observed, withdrawing all hemodynamic support after 36 hours, with recovery of myocardial contractility, normalisation of the valve insufficiencies, and resolution of pulmonary edema. Conclusions: Point-of-care ultrasound guided the physiopathology of the cardiogenic shock as well as the therapeutic decisions allowing optimizing the hemodynamic management, minimizing therapeutic delays and early reverting the life-threatening descompensation. *Correspondence: Irene Baquedano Lobera, Department of Pediatrics, Children Hospital Miguel Servet, Isabel la Católica Avenue 1-3, 50009 Zaragoza, SPAIN. Tel: 665117669. Received: 29 November 2020; Accepted: 16 December 2020 Journal of Pediatrics & Neonatology ISSN 2689-1085

Background: Bloodstream infections (BSIs) are the most frequent nosocomial infections in neonatal intensive care units (NICUs), especially in very low birth weight (VLBW) infants (birth weight <= 1500g). An epidemiologic surveillance system may contribute to the prevention of infection by continuous monitoring of its frequency and associated risk factors. The aim of this article was to describe the implementation of the NeoKissEs surveillance system for BSIs in VLBW newborns in a group of Spanish NICUs. Methods: We assessed the clinical cohort consisting of all VLBW newborns aged Less than 28 days admitted to the participating units. In the pilot phase, 2 NICUs translated and adapted materials from the original German NEO-KISS system. During implementation, 210 health care professionals attended one of 8 educational workshops. A web-based system was created that allows entering data regarding patients and BSI episodes, data monitoring, benchmarking and providing feedback to the units. At each NICU, one neonatologist was responsible for the implementation of the system and reporting the difficulties perceived throughout the process. Results: Out of the 50 units that agreed to participate, 45 successfully started using the surveillance platform during the implementation phase, recording 1108 episodes of catheter associated BSI (CABSI) in 3638 newborns, and finding an overall rate of CABSI of 18.4 (95% CI, 17.8-19.1) per 1000 catheter days. Conclusions: The NeoKissEs surveillance system constitutes a helpful source of information for the purpose of benchmarking the performance of neonatal units, assessing factors associated with BSI in VLBW infants and measuring the impact of future preventive interventions in NICUs. (C) 2018 Published by Elsevier Espana, S.L.U. on behalf of Asociacion Espanola de Pediatria. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Introduction Background and objective: hemodynamically significant patent ductus arteriosus (HS-PDA) is associated with an increased risk of necrotizing enterocolitis (NEC) and worse enteral tolerance in preterm newborns (PN). An association has been demonstrated between brain natriuretic propeptide (proBNP) and HS-PDA. Our objective was to analyze the relationship between proBNP levels and enteral tolerance, NEC risk and weight gain in PN. Material and methods: a retrospective study was performed in neonates born before 32 weeks' gestation or with birth weight below 1500 grams, in whom proBNP determination and echocardiography were performed at 48 to 72 h of life. Results: 117 patients were included. 65.8% had a HS-PDA and 9.4% had an outcome of NEC. HS-PDA was associated with longer duration of parenteral nutrition (p < 0.001), a confirmed NEC (p = 0.006) and worse weight gain during admission (p < 0.001). ProBNP levels were associated to NEC (no NEC 12189.5 pg / mL, range 654-247986; NEC 41445 pg/mL, range 15275-166172, p < 0.001). No association was found with the rest of gastrointestinal outcomes. Multivariate logistic regression analysis showed a significant association of NEC with gestational age and proBNP above 22,400 pg/mL (OR 13,386, 95% CI 1,541-116,262, p = 0.019). Conclusions: proBNP could be an early marker of severe digestive pathology in PN. Increased proBNP levels could be associated with a significant increased risk of NEC in very immature newborns.

The care of the umbilical cord until its detachment still remains controversial. The latest updated recommendations by the World Health Organisation advocate dry cord care in those countries with adequate obstetric care and low neonatal mortality rate. In recent years, new studies and reviews attribute some benefit to applying chlorhexidine on the umbilical stump. An analysis is presented here of the available evidence and results in the advisability of still recommending the dry cord care in the newborns in our setting.

El cuidado del cordón umbilical hasta su desprendimiento continúa siendo motivo de controversia en la actualidad. La Organización Mundial de la Salud recomienda en su última revisión realizar la cura en seco en países con cuidados obstétricos adecuados y una tasa baja de mortalidad neonatal. Por otro lado, en los últimos años han surgido nuevos estudios y revisiones que atribuyen un beneficio a aplicar clorhexidina tópica en el muñón. El presente documento analiza la evidencia disponible y concluye en la conveniencia de continuar recomendando la cura en seco en los nacimientos de nuestro entorno.

Neonates born very preterm (before 32 weeks' gestational age), are a significant public health concern because of their high-risk of mortality and life-long disability. In addition, caring for very preterm neonates can be expensive, both during their initial hospitalization and their long-term cost of permanent impairments. To address these issues, national and regional neonatal networks around the world collect and analyse data from their constituents to identify trends in outcomes, and conduct benchmarking, audit and research. Improving neonatal outcomes and reducing health care costs is a global problem that can be addressed using collaborative approaches to assess practice variation between countries, conduct research and implement evidence-based practices. The International Network for Evaluating Outcomes (iNeo) of neonates was established in 2013 with the goal of improving outcomes for very preterm neonates through international collaboration and comparisons. To date, 10 national or regional population-based neonatal networks/datasets participate in iNeo collaboration. The initiative now includes data on >200,000 very preterm neonates and has conducted important epidemiological studies evaluating outcomes, variations and trends. The collaboration has also surveyed >320 neonatal units worldwide to learn about variations in practices, healthcare service delivery, and physical, environmental and manpower related factors and support services for parents. The iNeo collaboration serves as a strong international platform for Neonatal-Perinatal health services research that facilitates international data sharing, capacity building, and global efforts to improve very preterm neonate care.

espanolAntecedentes y objetivos: El ductus arterioso persistente (DAP) es una patologia prevalente en el recien nacido pretermino (RNPT) que puede relacionarse con mayor morbimortalidad, siendo la tendencia actual el manejo conservador. El objetivo es describir nuestra expeciencia en una unidad neonatal de nivel IIIB, en relacion a la epidemiologia, manejo y morbi-mortalidad asociada al DAP. Material y metodos: Estudio retrospectivo observacional analitico, que incluyo RNPT menores de 32 semanas de gestacion o 1.500 g, con estudio ecocardiografico. Resultados: De 117 pacientes, 75,2% presento DAP, el 87,5% de los cuales fue hemodinamicamente significativo (DAP-HS). Todos, salvo uno, recibieron tratamiento medico. 17,2% de los DAP preciso cierre quirurgico. Los pacientes con DAP-HS presentaron mas displasia broncopulmonar (DBP) moderada-grave (p EnglishBackground and objectives: Patent ductus arteriosus (PDA) is a prevalent condition in preterm infants that may be related to increased morbidity and mortality. Current trend is a conservative management. The aim of the study was to describe our experience in a IIIB level neonatal unit in relation to the epidemiology, management and morbi-mortality associated with PDA. Patients and methods: A retrospective observational and analitical study was conducted on preterm infants less than 32 weeks of gestation or weight less than 1500 grams, in which echocardiogram was performed. Results: Of 117 patients enrolled, 75.2% had PDA, of which 87.5% were hemodynamically significant (HS-PDA). All of them, but one, received medical treatment. Surgical closure was performed in 17.2% of PDA. Patients with HS-PDA presented higher incidence of moderate-severe bronchopulmonary dysplasia (BPD) (p