Servo-controlled cooling during neonatal transport for babies with hypoxic-ischaemic encephalopathy is practical and beneficial: Experience from a large UK neonatal transport service.

AIM: Servo-controlled therapeutic hypothermia is a routine therapy for babies with hypoxic-ischaemic encephalopathy in the neonatal unit and is delivered in designated cooling centres. It is increasingly being used during neonatal transport in the UK to deliver this therapy in a timelier manner for babies not born in a cooling centre. Prior to the implementation of this treatment, passive cooling alone was used in transport. Comparison of passive and servo-controlled cooling during neonatal transfers with reference to: (i) the proportion of babies in the therapeutic range (33-34°C) at three time points during the transport mission (arrival of the team at the referring unit, departure of the team from the referring unit and at the completion of transport); (ii) the proportion of babies overcooled at any point once the transport team was present (<33°C); and (iii) duration of phases of the transfer to evaluate the impact of active cooling on how long it takes to undertake transfer. METHODS: This was a retrospective observational study comparing babies with passive and servo-controlled hypothermia (January 2015 to May 2016) following introduction of the servo-controlled cooling mattress. RESULTS: A total of 48 patients were treated with hypothermia in transit (29 passive, 19 servo-controlled). The median gestational age (GA) was 40 weeks (interquartile range: 39-41) and mean birthweight (BW) 3420 g (standard deviation 600 g); there was no differences in GA, BW and clinical characteristics between the groups. There was a statistically significant difference in the temperature at the end of the transport, where 94% (n = 18) of babies who received servo-controlled cooling were in the target temperature in comparison with only 65% (n = 19) of the passive cooling group babies (P = 0.045). In addition, none of the babies in the servo-controlled group were warm (>34°C) at the end of the transfer. Babies who underwent servo-controlled cooling are more likely to maintain a target temperature (33-34°C) (17 (89%) vs. 17 (58%), P = 0.021); in particular, there is less overcooling (<33°C) in this group (2 (10%) vs. 15 (51%), P = 0.004). Total mission time was not significantly different. CONCLUSION: The use of servo-controlled cooling devices during neonatal transport improves the ability to maintain the baby's temperature within the target range (33-34°C) with less overcooling.

SNP microarray abnormalities in a cohort of 28 infants with congenital diaphragmatic hernia.

Chromosomal abnormalities are an important factor in the pathogenesis of congenital diaphragmatic hernia (CDH), a relatively common congenital defect associated with high morbidity and mortality. The adoption of array-based platforms for chromosome analysis has resulted in the identification of numerous copy number variants (CNVs) in infants with CDH, highlighting the potential pathogenic role of many novel genes. We identified a retrospective cohort of 28 infants treated for CDH at a single institution who had microarray testing to determine the proportion of microarray abnormalities and whether these were contributory to CDH pathogenesis. Eight patients (29%) had microarray abnormality. Seven (25%) were considered likely contributory to CDH pathogenesis, including two mosaic trisomy 9s, a 9q22.31q22.32 microduplication, two atypical 22q11.21 microdeletions, a 2q35q36.1 microdeletion, and a 15q11.2 microdeletion, offering insights into the genetic mechanisms underlying CDH development.

Sildenafil weaning after discharge in infants with congenital diaphragmatic hernia.

Sildenafil is used to treat pulmonary hypertension (PAH) in infants with congenital diaphragmatic hernia (CDH). However, data to guide sildenafil dosing and weaning are limited. This is concerning in light of a recent report describing increased risk associated with high-dose sildenafil regimens in non-CDH PAH. A retrospective cohort study of sildenafil usage, dosing, and weaning in infants with CDH was conducted at the authors' institution. The findings show that 17 % (19/122) of infants were discharged receiving sildenafil at a median dose of 8 mg/kg/day (range 2.91-5.78 mg/kg/day). The weaning rate was 0.1 mg/kg/week (range 0.01-0.5 mg/kg/week). The infants ceased therapy after a median of 343 days. At the age of 1 year, 29 % were receiving sildenafil at a dose higher than 1.5 mg/kg/day. One infant died of severe PAH. Sildenafil therapy at discharge is common in severe CDH. Variation in dosing and weaning rates highlights the need for standardized assessment and treatment of PAH after discharge to optimize the benefits and minimize the adverse effects of sildenafil.