Techniques to Communicate Better With Parents During End-of-Life Scenarios in Neonatology.

Background And Objectives: Clinicians are urged to optimize communication with families, generally without empirical practical recommendations. The objective of this study was to identify core behaviors associated with good communication during and after an unsuccessful resuscitation, including parental perspectives.

Methods: Clinicians from different backgrounds participated in a standardized, videotaped, simulated neonatal resuscitation in the presence of parent actors.

Fluid restriction for treatment of preterm infants with chronic lung disease.

Background: Fluid restriction is often recommended as part of the management of infants with early or established bronchopulmonary dysplasia (BPD).

Objectives: To determine whether fluid restriction as part of the therapeutic intervention for early or established BPD improves clinical outcomes.

Search Methods: We used the standard search strategy of Cochrane Neonatal to search the Cochrane Central Register of Controlled Trials (CENTRAL; 2016, Issue 1) in the Cochrane Library (searched 16 February 2016), MEDLINE via PubMed (1966 to 16 February 2016), Embase (1980 to 16 February 2016), and CINAHL (1982 to 16 February 2016).

Nitric oxide for respiratory failure in infants born at or near term.

Background: Nitric oxide (NO) is a major endogenous regulator of vascular tone. Inhaled nitric oxide (iNO) gas has been investigated as treatment for persistent pulmonary hypertension of the newborn.

Objectives: To determine whether treatment of hypoxaemic term and near-term newborn infants with iNO improves oxygenation and reduces rate of death and use of extracorporeal membrane oxygenation (ECMO), or affects long-term neurodevelopmental outcomes.

Inhaled nitric oxide for respiratory failure in preterm infants.

Background: Inhaled nitric oxide (iNO) is effective in term infants with hypoxic respiratory failure. The pathophysiology of respiratory failure and the potential risks of iNO differ substantially in preterm infants, necessitating specific study in this population.

Objectives: To determine effects of treatment with inhaled nitric oxide (iNO) on death, bronchopulmonary dysplasia (BPD), intraventricular haemorrhage (IVH) or other serious brain injury and on adverse long-term neurodevelopmental outcomes in preterm newborn infants with hypoxic respiratory failure.

Exploring a New Simulation Approach to Improve Clinical Reasoning Teaching and Assessment: Randomized Trial Protocol.

Background: Helping trainees develop appropriate clinical reasoning abilities is a challenging goal in an environment where clinical situations are marked by high levels of complexity and unpredictability. The benefit of simulation-based education to assess clinical reasoning skills has rarely been reported. More specifically, it is unclear if clinical reasoning is better acquired if the instructor's input occurs entirely after or is integrated during the scenario.

Pathogenesis of congenital diaphragmatic hernia: additional clues regarding the involvement of the endothelin system.

Congenital diaphragmatic hernia (CDH) has an incidence of around 1/3,000 births. The pathogenesis of this developmental anomaly remains largely unknown and the description of small chromosomal imbalances in cases of CDH is of major interest for the identification of candidate genes. We report on a tandem 4q31.

Pathophysiology of persistent pulmonary hypertension of the newborn: impact of the perinatal environment.

The main cause of pulmonary hypertension in newborn babies results from the failure of the pulmonary circulation to dilate at birth, termed 'persistent pulmonary hypertension of the newborn' (PPHN). This syndrome is characterized by sustained elevation of pulmonary vascular resistance, causing extrapulmonary right-to-left shunting of blood across the ductus arteriosus and foramen ovale and severe hypoxaemia. It can also lead to life-threatening circulatory failure.

Positioning effects on lung function and breathing pattern in premature newborns.

Objective: To compare breathing patterns and lung function in the supine, lateral, and prone positions in oxygen-dependent preterm infants.

Study Design: Respiratory function in preterm infants receiving nasal continous positive airway pressure therapy for mild respiratory failure was evaluated by respiratory inductive plethysmography. Infants were randomized to supine, left lateral, and prone positions for 3 hours.

Hemodynamic effects of fluid restriction in preterm infants with significant patent ductus arteriosus.

Objective: To determine the hemodynamic impact of fluid restriction in preterm newborns with significant patent ductus arteriosus.

Study Design: Newborns ≥24 and <32 weeks' gestational age with significant patent ductus arteriosus were eligible for this prospective multicenter observational study. We recorded hemodynamic and Doppler echocardiographic variables before and 24 hours after fluid restriction.

Xq12q13.1 microduplication encompassing the EFNB1 gene in a boy with congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) has an incidence of around 1/3000 births. Chromosomal anomalies constitute an important etiology for non-isolated CDH, and may participate to the identification of candidate genes for diaphragm development. We report on a microduplication identified by array-CGH (comparative genomic hybridization) including five contiguous genes (OPHN1, YIPF6, STARD8, EFNB1 and PJA1) and arising de novo in a male presenting a congenital diaphragmatic hernia (CDH).

Heterogeneity in the processing of CLCN5 mutants related to Dent disease.

Mutations in the electrogenic Cl(-)/H(+) exchanger ClC-5 gene CLCN5 are frequently associated with Dent disease, an X-linked recessive disorder affecting the proximal tubules. Here, we investigate the consequences in Xenopus laevis oocytes and in HEK293 cells of nine previously reported, pathogenic, missense mutations of ClC-5, most of them which are located in regions forming the subunit interface. Two mutants trafficked normally to the cell surface and to early endosomes, and displayed complex glycosylation at the cell surface like wild-type ClC-5, but exhibited reduced currents.

Novel CLCN5 mutations in patients with Dent's disease result in altered ion currents or impaired exchanger processing.

Dent's disease is an X-linked recessive disorder affecting the proximal tubules and is frequently associated with mutations in CLCN5, which encodes the electrogenic chloride-proton exchanger ClC-5. To better understand the functional consequences of CLCN5 mutations in this disease, we screened four newly identified missense mutations (G179D, S203L, G212A, L469P), one new nonsense mutation (R718X), and three known mutations (L200R, C219R, and C221R), in Xenopus laevis oocytes and HEK293 cells expressing either wild-type or mutant exchanger. A type-I mutant (G212A) trafficked normally to the cell surface and to early endosomes, underwent complex glycosylation at the cell surface like wild-type ClC-5, but exhibited significant reductions in outwardly rectifying ion currents.