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Respiratory distress syndrome (RDS) care pathways evolve slowly as new evidence emerges. We report the sixth version of “European Guidelines for the Management of RDS” by a panel of experienced European neonatologists and an expert perinatal obstetrician based on available literature up to end of 2022. Optimising outcome for babies with RDS includes prediction of risk of preterm delivery, appropriate maternal transfer to a perinatal centre, and appropriate and timely use of antenatal steroids. Evidence-based lung-protective management includes initiation of non-invasive respiratory support from birth, judicious use of oxygen, early surfactant administration, caffeine therapy, and avoidance of intubation and mechanical ventilation where possible. Methods of ongoing non-invasive respiratory support have been further refined and may help reduce chronic lung disease. As technology for delivering mechanical ventilation improves, the risk of causing lung injury should decrease, although minimising time spent on mechanical ventilation by targeted use of postnatal corticosteroids remains essential. The general care of infants with RDS is also reviewed, including emphasis on appropriate cardiovascular support and judicious use of antibiotics as being important determinants of best outcome. We would like to dedicate this guideline to the memory of Professor Henry Halliday who died on November 12, 2022.These updated guidelines contain evidence from recent Cochrane reviews and medical literature since 2019. Strength of evidence supporting recommendations has been evaluated using the GRADE system. There are changes to some of the previous recommendations as well as some changes to the strength of evidence supporting recommendations that have not changed. This guideline has been endorsed by the European Society for Paediatric Research (ESPR) and the Union of European Neonatal and Perinatal Societies (UENPS).

Background: Pediatric Intestinal Pseudo-obstruction (PIPO) is a congenital enteric disorder characterized by severe gastrointestinal (GI) dysmotility, without mechanical obstruction. Although several genes have been described to cause this disease, most patients do not receive a genetic diagnosis. Here, we aim to identify the genetic cause of PIPO in a patient diagnosed with severe intestinal dysmotility shortly after birth.Methods: Whole exome sequencing (WES) was performed in the patient and unaffected parents, in a diagnostic setting. After identification of the potential disease-causing variant, its functional consequences were determined in vitro and in vivo. For this, expression constructs with and without the causing variant, were overexpressed in HEK293 cells. To investigate the role of the candidate gene in GI development and function, a zebrafish model was generated where its expression was disrupted using CRISPR/Cas9 editing.Results: WES analysis identified a de novo heterozygous deletion in TFAP2B (NM_003221.4:c.602-5_606delTCTAGTTCCA), classified as a variant of unknown significance. In vitro studies showed that this deletion affects RNA splicing and results in loss of exon 4, leading to the appearance of a premature stop codon and absence of TFAP2B protein. Disruption of tfap2b in zebrafish led to decreased enteric neuronal numbers and delayed transit time. However, no defects in neuronal differentiation were detected. tfap2b crispants also showed decreased levels of ednrbb mRNA, a downstream target of tfap2b.Conclusion: We showed that TFAP2B haploinsufficiency leads to reduced neuronal numbers and GI dysmotility, suggesting for the first time, that this gene is involved in PIPO pathogenesis.

AIM We examined actual fluid intake, and routes of administration, in very low birthweight (VLBW) infants during the first week of life in a neonatal intensive care unit. METHODS This retrospective cohort study comprised 953 infants born at

Challenges and special aspects related to the management and prognosis of pulmonary hypertension (PH) in middle- to low-income regions (MLIRs) range from late presentation to comorbidities, lack of resources and expertise, cost, and rare options of lung transplantation. Expert consensus recommendations addressing the specific challenges for prevention and therapy of PH in MLIRs with limited resources have been lacking. To date, 6 MLIR-PH registries containing mostly adult patients with PH exist. Importantly, the global prevalence of PH is much higher in MLIRs compared with high-income regions: group 2 PH (left heart disease), pulmonary arterial hypertension associated with unrepaired congenital heart disease, human immunodeficiency virus, or schistosomiasis are highly prevalent. This consensus statement provides selective, tailored modifications to the current PH guidelines to address the specific challenges faced in MLIRs, resulting in the first pragmatic and cost-effective consensus recommendations for PH care providers, patients, and their families. ispartof: JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY vol:75 issue:19 pages:2463-2477 ispartof: location:United States status: published

Objective: To determine the association between potential neonatal risk factors and the quality of general movements (GMs) in preterm infants.Study design: Prospective cohort study in 67 preterm infants. From video recordings made on Days 8 and 15, we scored the GMs as normal/abnormal and detailed aspects using the general movement optimality score (GMOS). Risk factors included respiratory insufficiency requiring mechanical ventilation, patent ductus arteriosus (PDA), and abnormal blood glucose levels. We used multiple regression analyses.Result: On Day 8 after birth, the presence of a PDA remained in the multivariable model, explaining 17.1% of the variance in GMOS (beta, -0.414). On Day 15, duration of mechanical ventilation and frequency of hypoglycemic episodes explained 38.8% of the variance (betas, -0.382 and -0.466, respectively).Conclusion: In preterm infants, PDA, duration of mechanical ventilation, and frequency of hypoglycemic episodes were associated with poorer neurological functioning during the first 2 weeks after birth.

As management of respiratory distress syndrome (RDS) advances, clinicians must continually revise their current practice. We report the fourth update of "European Guidelines for the Management of RDS" by a European panel of experienced neonatologists and an expert perinatal obstetrician based on available literature up to the end of 2018. Optimising outcome for babies with RDS includes prediction of risk of preterm delivery, need for appropriate maternal transfer to a perinatal centre and timely use of antenatal steroids. Delivery room management has become more evidence-based, and protocols for lung protection including initiation of CPAP and titration of oxygen should be implemented immediately after birth. Surfactant replacement therapy is a crucial part of management of RDS, and newer protocols for its use recommend early administration and avoidance of mechanical ventilation. Methods of maintaining babies on non-invasive respiratory support have been further developed and may cause less distress and reduce chronic lung disease. As technology for delivering mechanical ventilation improves, the risk of causing lung injury should decrease, although minimising time spent on mechanical ventilation using caffeine and, if necessary, postnatal steroids are also important considerations. Protocols for optimising general care of infants with RDS are also essential with good temperature control, careful fluid and nutritional management, maintenance of perfusion and judicious use of antibiotics all being important determinants of best outcome. (c) 2019 S. Karger AG, Basel

Near-infrared spectroscopy (NIRS) allows for continuous, non-invasive monitoring of end-organ tissue oxygenation. The use of NIRS, cerebral NIRS (cNIRS) in particular, in neonatal care has increased significantly over the last few years. This dynamic monitoring technique provides real-time information on the cerebral and haemodynamic status of the neonate and has the potential to serve as an important adjunct to patient care with some centres routinely utilising cNIRS to aid decision-making at the bedside. cNIRS values may be influenced by many variables, including cardiac, respiratory and metabolic parameters, and therefore it is essential to understand the pathophysiology behind alterations in cNIRS values. Correct interpretation is required to direct appropriate patient-specific interventions. This article aims to assist clinicians in deciphering cNIRS values by providing an overview of potential causes of fluctuations in cNIRS values, illustrated by common clinical scenarios, with particular emphasis on the preterm infant.

Objectives: To assess risk factors of retinopathy of prematurity leading to delayed retinal vascularization in the first stage of ROP. Methods: A retrospective study (2000 - 2016) was performed in 275 infants who were born at a gestational age of ≤ 32 weeks. Delayed vascular development was calculated as the difference in avascular area measured in terms of disc diameters between patients with risk factor and those with no risk factor. Results: Intrauterine fetal demise of one twin, apnea, exchange tranfusion and intubation days (≥ 10 days) lead to delayed retinal vascularization of 1.74, 1.58, 1.17, and 1.11 disc diameters and of 3.35, 3.04, 2.25 and 2.13 weeks respectively. Delayed retinal vascular development was less than 1 disc diameter in other factors such as HS-PDA, sepsis, degree of hyaline membrane ≥ III, cerebral hemorrhage, birth weight (every 100 g less), gestational age (every week less), postnatal weight gain (every 1 g less/day) and cerebral hemorrhage. However, since these factors are quantitative (birth weight, gestational age and postnatal weight gain), the greater the adverse effect, the greater the delay will be. Conclusions: Intrauterine fetal demise of one twin, apnea, exchange tranfusion and intubation days (≥ 10 days) lead to delayed retinal vascular development more than 1 disc diameter at 4 - 6 weeks of postnatal life.

Near-infrared spectroscopy (NIRS) allows for continuous, non-invasive monitoring of end-organ tissue oxygenation. The use of NIRS, cerebral NIRS (cNIRS) in particular, in neonatal care has increased significantly over the last few years. This dynamic monitoring technique provides real-time information on the cerebral and haemodynamic status of the neonate and has the potential to serve as an important adjunct to patient care with some centres routinely utilising cNIRS to aid decision-making at the bedside. cNIRS values may be influenced by many variables, including cardiac, respiratory and metabolic parameters, and therefore it is essential to understand the pathophysiology behind alterations in cNIRS values. Correct interpretation is required to direct appropriate patient-specific interventions. This article aims to assist clinicians in deciphering cNIRS values by providing an overview of potential causes of fluctuations in cNIRS values, illustrated by common clinical scenarios, with particular emphasis on the preterm infant.

This review focuses on the clinical use of near infrared spectroscopy (NIRS) to assess brain oxygenation by the tissue oxygenation index (TOI), and monitoring regional cerebral oxygen saturation (rScO2), cerebral fractional tissue oxygen extraction (cFTOE), which is derived from rScO2, and systemic oxygen saturation. Its precision and pitfalls are discussed. At this stage, it is clear that NIRS-monitored oxygenation of the brain by rScO2 or TOI lacks the precision required to be used as a robust quantitative variable to monitor cerebral oxygenation. Intra- and especially interpatient variability are too large for this aim. On the other hand, when used merely as a trend monitor in the individual patient, substantial changes in rScO2 or TOI and consequently of cFTOE, larger than the limits of agreement, can yield important clinical information that suggest an intervention. Since neonatal intensive care is for a substantial part ‘brain orientated’ this approach seems conceivable. This gives rise to the conclusion that NIRS-monitored TOI, rScO2 and cFTOE increasingly will have a role in clinical practice as semiquantitative indicators of changes in cerebral oxygenation and oxygen extraction. Combination with other (cerebral) parameters such as amplitude-integrated EEG and blood pressure seems promising for further optimization of monitoring the immature brain.

This review focuses on the clinical use of near infrared spectroscopy (NIRS) to assess brain oxygenation by the tissue oxygenation index (TOI), and monitoring regional cerebral oxygen saturation (rScO(2)), cerebral fractional tissue oxygen extraction (cFTOE), which is derived from rScO(2), and systemic oxygen saturation. Its precision and pitfalls are discussed. At this stage, it is clear that NIRS-monitored oxygenation of the brain by rScO(2) or TOI lacks the precision required to be used as a robust quantitative variable to monitor cerebral oxygenation. Intra- and especially interpatient variability are too large for this aim. On the other hand, when used merely as a trend monitor in the individual patient, substantial changes in rScO2 or TOI and consequently of cFTOE, larger than the limits of agreement, can yield important clinical information that suggest an intervention. Since neonatal intensive care is for a substantial part 'brain orientated' this approach seems conceivable. This gives rise to the conclusion that NIRS-monitored TOI, rScO(2) and cFTOE increasingly will have a role in clinical practice as semiquantitative indicators of changes in cerebral oxygenation and oxygen extraction. Combination with other (cerebral) parameters such as amplitude-integrated EEG and blood pressure seems promising for further optimization of monitoring the immature brain. Copyright (C) 2008 S. Karger AG, Basel

Objective: The purpose of this article is to summarize the clinical, methodologic, and ethical considerations for researchers interested in designing future trials in neonatal analgesia and anesthesia, hopefully stimulating additional research in this field.Methods: The MEDLINE, PubMed, EMBASE, and Cochrane register databases were searched using subject headings related to infant, newborn, neonate, analgesia, anesthesia, ethics, and study design. Cross-references and personal files were searched manually. Studies reporting original data or review articles related to these topics were assessed and critically evaluated by experts for each topical area. Data on population demographics, study characteristics, and cognitive and behavioral outcomes were abstracted and synthesized in a systematic manner and refined by group members. Data synthesis and results were reviewed by a panel of independent experts and presented to a wider audience including clinicians, scientists, regulatory personnel, and industry representatives at the Newborn Drug Development Initiative workshop. Recommendations were revised after extensive discussions at the workshop and between committee members.Results: Designing clinical trials to investigate novel or currently available approaches for analgesia and anesthesia in neonates requires consideration of salient study designs and ethical issues. Conditions requiring treatment include pain/stress resulting from invasive procedures, surgical operations, inflammatory conditions, and routine neonatal intensive care. Study design considerations must define the inclusion and exclusion criteria, a rationale for stratification, the confounding effects of comorbid conditions, and other clinical factors. Significant ethical issues include the constraints of studying neonates, obtaining informed consent, making risk-benefit assessments, defining compensation or rewards for participation, safety considerations, the use of placebo controls, and the variability among institutional review boards in interpreting federal guidelines on human research. For optimal study design, investigators must formulate well-defined study questions, choose appropriate trial designs, estimate drug efficacy, calculate sample size, determine the duration of the studies, identify pharmacokinetic and pharmacodynamic parameters, and avoid drug-drug interactions. Specific outcome measures may include scoring on pain assessment scales, various biomarkers and their patterns of response, process outcomes (eg, length of stay, time to extubation), intermediate or long-term outcomes, and safety parameters.Conclusions: Much more research is needed in this field to formulate a scientifically sound, evidence-based, and clinically useful framework for management of anesthesia and analgesia in neonates. Newer study designs and additional ethical dilemmas may be defined with accumulating data in this field. Copyright (c) 2005 Excerpta Medica, Inc.

We present two cases of Ortner's syndrome, a syndrome with hoarseness caused by unilateral left recurrent laryngeal nerve palsy as a complication of cardiovascular diseases. In the first case, aneurysmatic dilatation of the aorta and in the second case a dilated pulmonary trunk was thought to be the cause.

We present two cases of Ortner's syndrome, a syndrome with hoarseness caused by unilateral left recurrent laryngeal nerve palsy as a complication of cardiovascular diseases. In the first case, aneurysmatic dilatation of the aorta and in the second case a dilated pulmonary trunk was thought to be the cause.

Objective: To determine in preterm infants with a patent ductus arteriosus (PDA) the effect of indomethacin treatment on spontaneous motor activity. Study Design: Motor activity was assessed from repeated videotape recordings in 32 preterm infants (≤33 weeks gestation). Sixteen infants required indomethacin therapy for treatment of PDA, 16 were control infants, matched for gestational age. Indomethacin (0.2 mg/kg i.v. in 5 min) was given thrice, with an interval of 12 h. One-hour recordings were made immediately before and immediately after the first dose of indomethacin and 24 h later before and after the third dose. The same recording schedule was used for the control infants. The effects of indomethacin on quantity and quality of spontaneous motor activity were examined. Results: A significant reduction in the quantity of several spontaneous movement patterns and an increase in the occurrence of rest periods were found following the first indomethacin administration (p < 0.01). Concerning the quality of general movements, a reduction in the speed was found (p < 0.05). Both effects were not found after the third indomethacin administration. Conclusion: In preterm infants with a PDA, treatment with indomethacin leads to a transient reduction in the quantity of spontaneous movement patterns and to a decrease in the speed of general movements. We recommend a cautious use of bolus indomethacin for the treatment of PDA.

Near-infrared spectroscopy-derived regional tissue oxygen saturation of haemoglobin (rStO2) reflects venous oxygen saturation. If cerebral metabolism is stable, rStO2 can be used as an estimate of cerebral oxygen delivery. The SafeBoosC phase II randomised clinical trial hypothesises that the burden of hypo- and hyperoxia can be reduced by the combined use of close monitoring of the cerebral rStO2 and a treatment guideline to correct deviations in rStO2 outside a predefined target range. Aims: To describe the rationale for and content of this treatment guideline. Methods: Review of the literature and assessment of the quality of evidence and the grade of recommendation for each of the interventions. Results and Conclusions: A clinical intervention algorithm based on the main determinants of cerebral perfusion-oxygenation changes during the first hours after birth was generated. The treatment guideline is presented to assist neonatologists in making decisions in relation to cerebral oximetry readings in preterm infants within the SafeBoosC phase II randomised clinical trial. The evidence grades were relatively low and the guideline cannot be recommended outside a research setting.

We present two cases of Ortner's syndrome, a syndrome with hoarseness caused by unilateral left recurrent laryngeal nerve palsy as a complication of cardiovascular diseases. In the first case, aneurysmatic dilatation of the aorta and in the second case a dilated pulmonary trunk was thought to be the cause.

We studied regional blood flows with radioactive-labeled microspheres in 12 7-wk-old lambs with an aortopulmonary left-to-right shunt [59 +/- 3% (SE) of left ventricular (LV) output] and in 11 control lambs, at rest and during exercise at 80% of predetermined peak O2 consumption. At rest, systemic blood flow was similar in the two groups. Blood flow to the heart and diaphragm was substantially higher in the shunt than in the control lambs. Blood flow to the other organs was not significantly different between the two groups. During exercise, systemic blood flow increased substantially but less in shunt (81%) than in control lambs (134%). Blood flow to the heart and diaphragm increased, that to the heart still being higher in shunt than in control lambs. Blood flow to the brain did not change, whereas that to the kidneys and splanchnic organs decreased to the same extent (25%) in shunt and control lambs. Intrahepatic and intrarenal blood flow redistribution in the shunt lambs persisted during exercise. In conclusion, myocardial blood flow is not increased at the expense of one particular organ, nor is it associated with an essential change in exercise-induced redistribution in shunt lambs.

A 62-yr-old female presented with complaints of progressive chest pain, increasing in severity with inspiration, a productive cough and fever for 2 days. Moreover, she had lost 12 kg in weight in the last 3 months and was experiencing night sweats. During this period, she had a folliculitis in her axilla. Her medical history yielded diabetes mellitus type II, hypertension and anaemia. Transthoracic echocardiography, performed 4 years ago, had shown flow abnormalities in the pulmonary artery and left ventricular volume overload. The patients’ medication included metformin, insulin, hydrochlorothiazide, simvastatin, metoprolol, ferrofumerate and vitamin B. On physical examination, a pale and cachectic patient was seen. Her body temperature was 39°C, with a heart rate of 104 beats·min−1, respiratory rate of 20 breaths·min−1 and blood pressure of 160/65 mmHg. Chest auscultation revealed normal breath sounds, a 2/6 systolic murmur in the right parasternal area and a 1/6 continuous murmur in the left parasternal area. Otherwise, physical examination was normal. An overview of investigations is presented in table 1⇓. A chest radiograph was performed upon admission (fig. 1⇓) and a contrast-enhanced computed tomography (CT) scan was performed before and after treatment (figs 2⇓ and 3⇓, respectively). A CT scan was also obtained in the parenchymal setting (fig. 4⇓). Fig. 1— Chest radiograph on admission showing a large right hilum and consolidations in both lower lobes. Fig. 2— Contrast-enhanced computed tomography images at admission. The sagittal plane is shown, which has been reconstructed from data of transverse slices. Arrow A indicates the patent ductus arteriosus connecting the aortic arch to the pulmonary artery. Arrow B indicates opacities in the pulmonary trunk suggestive of vegetations. A calcified area is visible in the aortic arch. Fig. 3— Contrast-enhanced computed tomography images 4 months after antibiotic treatment. The sagittal plane is shown, which has been reconstructed from data of transverse slices. The arrow indicates …

We studied regional blood flows with radioactive-labeled microspheres in 12 7-wk-old lambs with an aortopulmonary left-to-right shunt [59 +/- 3% (SE) of left ventricular (LV) output] and in 11 control lambs, at rest and during exercise at 80% of predetermined peak O2 consumption. At rest, systemic blood flow was similar in the two groups. Blood flow to the heart and diaphragm was substantially higher in the shunt than in the control lambs. Blood flow to the other organs was not significantly different between the two groups. During exercise, systemic blood flow increased substantially but less in shunt (81%) than in control lambs (134%). Blood flow to the heart and diaphragm increased, that to the heart still being higher in shunt than in control lambs. Blood flow to the brain did not change, whereas that to the kidneys and splanchnic organs decreased to the same extent (25%) in shunt and control lambs. Intrahepatic and intrarenal blood flow redistribution in the shunt lambs persisted during exercise. In conclusion, myocardial blood flow is not increased at the expense of one particular organ, nor is it associated with an essential change in exercise-induced redistribution in shunt lambs.