One-Year Outcome for Congenital Diaphragmatic Hernia: Results From the French National Register.

OBJECTIVE: To evaluate the status of congenital diaphragmatic hernia (CDH) management in France and to assess predictors of adverse outcomes. STUDY DESIGN: We reviewed the first-year outcome of all cases of CDH reported to the French National Register in 2011. RESULTS: A total of 158 cases were included. Of these, 83% (131) were prenatally diagnosed, with a mortality rate of 39% (44 of 112) for live born infants with a known outcome at hospital discharge. Mortality increased to 47% (60 of 128) including those with termination of pregnancy and fetal loss. This contrasts with the 7% (2 of 27) mortality rate of the patients diagnosed postnatally (P = .002). Mortality worsened with 1 prenatal marker of CDH severity (OR 3.38 [1.30-8.83] P = .013) and worsened further with 2 markers (OR 20.64 [5.29-80.62] P < .001). Classic postnatal risk factors of mortality such as side of hernia (nonleft P = .001), prematurity (P < .001), low birth weight (P = .002), and size of the defect (P < .001) were confirmed. Of the 141 live births (114 prenatal and 27 postnatal diagnosis) with known outcomes, 93 (67%) survived to hospital discharge, 68 (60%) with a prenatal diagnosis and 25 (93%) with a postnatal diagnosis. The median time to hospital discharge was 34 days (IQR, 19.25-62). Of these survivors, 71 (76%) were followed up for 1 year. CONCLUSIONS: Despite advances in management of CDH, mortality was high and associated with prenatal risk factors. Postnatally, severe persistent pulmonary hypertension was difficult to predict and presented persistent challenges in management.

Thoracic computed tomography in absent pulmonary valve syndrome management.

A neonate with absent pulmonary valve syndrome (APVS) and features of tetralogy of Fallot presented respiratory distress. The prolonged need for mechanical ventilation was an indication for surgical repair. Preoperative thoracic CT imaging showed nearly complete closure of the distal trachea, compressed by the aneurysmal dilatation of the pulmonary arteries, and its reopening after ventilator pressure was increased. Management of APVS may require high-pressure ventilation to prevent bronchial collapse due to compression by the pulmonary arteries. In this context, thoracic CT imaging is very useful for adjusting ventilation support so that surgical repair can be performed in optimal conditions.

Rapid exome sequencing in critically ill infants: implementation in routine care from French regional hospital's perspective.

This monocentric study included fifteen children under a year old in intensive care with suspected monogenic conditions for rapid trio exome sequencing (rES) between April 2019 and April 2021. The primary outcome was the time from blood sampling to rapid exome sequencing report to parents. All results were available within 16 days and were reported to parents in or under 16 days in 13 of the 15 individuals (86%). Six individuals (40%) received a diagnosis with rES, two had a genetic condition not diagnosed by rES. Eight individuals had their care impacted by their rES results, four were discharged or died before the results. This small-scale study shows that rES can be implemented in a regional University hospital with rapid impactful diagnosis to improve care in critically ill infants.

Betamethasone impairs cerebral blood flow velocities in very premature infants with severe chronic lung disease.

OBJECTIVE: To assess betamethasone (BM) effects on the cerebral hemodynamics of neonates with severe chronic lung disease (CLD). STUDY DESIGN: Intravenous BM was given once daily for 6 consecutive days to 12 infants (birth weight: 698 g [range, 650-884 g], gestational age: 25.3 weeks [range, 25-26.4 weeks]) at a postnatal age of 34 days (range, 28-36 days). Cerebral blood flow velocities (CBFVs) were recorded prospectively in the anterior cerebral artery (ACA) and the lenticulostriate artery (LSA) before, during, and after treatment, using Doppler flowmetry. RESULTS: The decrease in systolic and diastolic velocities was maximum on the 5th day, reaching 32% (95% confidence interval [CI], 23%-42%) and 58% (95% CI, 39%-64%) from baseline in the ACA, and 44% (95% CI, 29%-50%) and 57% (95% CI, 33%-66%) in the LSA, respectively. The resistance index (RI) increased significantly in both arteries during treatment. Return to baseline values was observed after BM was stopped. The change in velocities and RI was independent of arterial blood gas and blood pressure variations. CONCLUSIONS: BM decreased the CBFVs of premature infants, suggesting a vasoconstrictor effect in both superficial and deep arterial vessels. Caution is recommended when BM is used to treat preterm infants with severe CLD.

Growth and bone mineralization in preterm infants fed preterm formula or standard term formula after discharge.

OBJECTIVE: To evaluate growth and bone mineralization in very low birth weight (VLBW) infants fed preterm formula (PF) or term formula (TF). STUDY DESIGN: In a double-blind prospective study, 49 preterm infants of gestational age 33 weeks or less were randomly fed PF or TF for 2 months after discharge, then all of the infants were fed TF for the next 2 months. Anthropometric and dual-energy x-ray absorptiometry data were collected at discharge and at 2 months and 4 months after discharge. Anthropometric data also were collected at 12 months postterm. RESULTS: Four months after discharge, both body weight (6139 +/- 1254 g vs 5540 +/- 863 g; P = .03) and bone mineral content (104.4 +/- 29.2 g vs 87.5 +/- 17.1 g; P = .01) were significantly higher in the PF group compared with the TF group. At 12 months postterm, mean body weight, length, and head circumference remained higher in the PF group than in the TF group, and body mass index was similar and within the normal range in the 2 groups. CONCLUSIONS: At 4 months after discharge, growth and mineralization were better in the VLBW infants who were fed PF during the first 2 months after discharge compared with those who were fed TF, suggesting that PF may be particularly valuable at this early stage of development.

Probiotics and growth in preterm infants: A randomized controlled trial, PREMAPRO study.

BACKGROUND & AIMS: Recent studies have suggested that the gut microflora has metabolic effects. We aimed to evaluate postnatal growth in preterm infants who received different probiotic supplements, and to assess the safety of probiotic administration. METHODS: This prospective, randomized, double-blind, controlled trial was performed at three tertiary care neonatal units. Preterm infants were randomly assigned to receive daily supplementation over 4-6 weeks with placebo (group C) or probiotics (group P). Group P comprised three subgroups: P1 received Bifidobacterium lactis, P2 received Bifidobacterium longum, and P3 received B. lactis and B. longum. We assessed postnatal growth during the supplementation period and up to a corrected gestational age (GA) of 41 weeks when body composition was assessed using whole-body dual-energy X-ray absorptiometry. Aerobic and anaerobic blood cultures were performed on suspicion of late-onset sepsis. RESULTS: The study comprised 199 preterm infants with a mean GA of 29.1 ± 1.4 weeks and a mean birth weight of 1173 ± 210 g, who received a placebo (group C, n = 52) or probiotics (group P, n = 147) from the first week of life. At the end of the supplementation period, no statistically significant differences were seen between the groups in relation to the mean body weight (group C = 1906 ± 23 g, group P = 1875 ± 14 g, p = 0.25), length, or head circumference. The incidence rates of necrotizing enterocolitis and late-onset sepsis were similar in the two groups. At the corrected GA of 41 weeks, there were no differences between the groups with respect to anthropometric measurements or body composition analysis. CONCLUSIONS: Preterm infants receiving Bifidobacterium supplements did not exhibit better postnatal growth compared with those who received placebo treatment. No adverse effects were associated with probiotic administration. Registered under ClinicalTrials.gov Identifier no. NCT01379417.

6 cmH2O continuous positive airway pressure versus conventional oxygen therapy in severe viral bronchiolitis: a randomized trial.

OBJECTIVE: To compare the effects of nasal continuous positive airway pressure (nCPAP) and conventional oxygen therapy on the clinical signs of respiratory distress and the respiratory muscle workload in acute viral bronchiolitis. DESIGN: Prospective, randomized, monocentric study carried out in the pediatric intensive care unit (PICU) of a university hospital. PATIENTS: Infants <6 months old, admitted to the PICU with severe respiratory syncytial virus bronchiolitis. INTERVENTION: The patients were randomized into two groups for 6 hr. The nCPAP group (n = 10) received 6 cmH(2)O pressure support delivered by a jet flow generator and the control group (n = 9) received an air/oxygen mixture from a heated humidifier. Respiratory distress was assessed by the modified Wood's clinical asthma score (m-WCAS), and inspiratory muscle work was evaluated by calculating the pressure-time product per breath (PTP(insp) /breath) and per minute (PTP(insp) /min) from the esophageal pressure (Pes) recordings. MEASUREMENTS AND MAIN RESULTS: Compared with control condition, nCPAP decreased m-WCAS [-2.4 (1.05) vs. -0.5 (1.3), P = 0.03], PTPes(insp)/breath [-9.7 (5.7) vs. -1.4 (8.2), P = 0.04], PTPes(insp) /min [-666 (402) vs. -116 (352), P = 0.015], and FiO(2) [-7 (10) vs. +5 (15), P = 0.05]. Significant worsening of m-WCAS was only observed in the control group (4/9 vs. 0/10, P = 0.03). CONCLUSIONS: nCPAP rapidly decreased inspiratory work in young infants with acute bronchiolitis. Improvement in the respiratory distress score at 6 hr was proportional to the initial clinical severity, suggesting the importance of rapid nCPAP initiation in the more severe forms of the disease.

Organ volume measurements: comparison between MRI and autopsy findings in infants following sudden unexpected death.

OBJECTIVE: To assess the accuracy of a semiautomated 3D volume reconstruction method for organ volume measurement by postmortem MRI. METHODS: This prospective study was approved by the institutional review board and the infants' parents gave their consent. Postmortem MRI was performed in 16 infants (1 month to 1 year of age) at 1.5 T within 48 h of their sudden death. Virtual organ volumes were estimated using the Myrian software. Real volumes were recorded at autopsy by water displacement. The agreement between virtual and real volumes was quantified following the Bland and Altman's method. RESULTS: There was a good agreement between virtual and real volumes for brain (mean difference: -0.03% (-13.6 to +7.1)), liver (+8.3% (-9.6 to +26.2)) and lungs (+5.5% (-26.6 to +37.6)). For kidneys, spleen and thymus, the MRI/autopsy volume ratio was close to 1 (kidney: 0.87±0.1; spleen: 0.99±0.17; thymus: 0.94±0.25), but with a less good agreement. For heart, the MRI/real volume ratio was 1.29±0.76, possibly due to the presence of residual blood within the heart. The virtual volumes of adrenal glands were significantly underestimated (p=0.04), possibly due to their very small size during the first year of life. The percentage of interobserver and intraobserver variation was lower or equal to 10%, but for thymus (15.9% and 12.6%, respectively) and adrenal glands (69% and 25.9%). CONCLUSIONS: Virtual volumetry may provide significant information concerning the macroscopic features of the main organs and help pathologists in sampling organs that are more likely to yield histological findings.

Validation of a neonatal pain scale adapted to the new practices in caring for preterm newborns.

UNLABELLED: BACKGROUND Neonatal pain assessment generally requires access to facial expression. Improved neonatology practices, such as greater protection against bright lights and non-invasive mask ventilation, have made facial observation more difficult. OBJECTIVE: To validate a 'faceless' acute neonatal pain scale (FANS), which does not depend on facial expression. METHODS: In a prospective, multicentre study, 24-40-week-old neonates were videotaped during a painful procedure (heel prick). Three investigators then scored the pain using FANS and a previously validated scale: DAN (Douleur aiguë du Nouveau-né). FANS is based on assessment of limb movement, cry and autonomic reaction. Reliability was assessed by inter-rater agreement and internal consistency (Cronbach's alpha). Validity was evaluated by agreement between scales (intraclass correlation coefficient (ICC)). The Wilcoxon test evaluated the FANS score differences between conditions. Results are expressed as medians (25th and 75th percentiles). Ranges are presented for outcome parameters. RESULTS: From April 2006 to September 2007, 53 preterms of 32 (30-35) gestational weeks and 1500 (1000-2200) g were observed. Cronbach's alpha was 0.72. The ICC was 0.92 (0.9-0.98) for inter-rater agreement and 0.88 (0.76-0.93) for agreement between scales. CONCLUSION: FANS, which is reliable and valid, is the first scale to score pain in preterm newborns when facial expression is not accessible.

Nitrous oxide analgesia for intubating preterm neonates: a pilot study.

AIM: To evaluate the administration of an equimolar mixture of N2O and O2 for intratracheal intubation in preterm neonates with respiratory distress syndrome (RDS). DESIGN: Prospective evaluation of N2O/O2 in premature neonates with RDS. SETTING: Tertiary neonatal unit from March to August 2003. PATIENTS: Twenty-six of 79 neonates admitted for RDS within 48 h of birth. INTERVENTION: N2O/O2 was administered until muscle tone was suppressed. Surfactant was given intratracheally. Patients were extubated as soon as possible. MAIN OUTCOME MEASURES: The time needed for N2O/O2 to suppress muscle tone, an evaluation of sedation/analgesia through movements of the limbs, and indicators of stress-related haemodynamic change, all recorded by an independent observer. RESULTS: In the 26 patients, gestational age was 30.5 (25th, 75th percentile: 30, 32) wk and median body weight was 1540 (1220, 1900) g. Postnatal age at intubation was 2 (2, 3) h. N2O/O2 administration time was 8 (6, 10) min (range 4-15 min). Sedation/analgesia was complete in 77% of patients. No significant differences between pre-procedure and post-procedure values were found for heart rate (p=0.29) or mean arterial blood pressure (p=0.13) (paired Wilcoxon test). Time needed for intubation was 30 (20, 37) s (range 10-60 s). Side effects included transient agitation (3/26) and retching (2/26). Extubation occurred 5 (5, 10) min (range 2-15 min) after surfactant instillation. Apnoeas occurred in 3/26 patients within 2 h after extubation. Two patients required reintubation to repeat surfactant administration within 24 h after extubation. CONCLUSION: N2O/O2 may be helpful for intubation in preterm neonates. Larger randomized, double-blind studies are needed for a thorough evaluation of effectiveness and safety.