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Abstract Background The first consensus standardised neonatal parenteral nutrition formulations were implemented in many neonatal units in Australia in 2012. The current update involving 49 units from Australia, New Zealand, Singapore, Malaysia and India was conducted between September 2015 and December 2017 with the aim to review and update the 2012 formulations and guidelines. Methods A systematic review of available evidence for each parenteral nutrient was undertaken and new standardised formulations and guidelines were developed. Results Five existing preterm Amino acid-Dextrose formulations have been modified and two new concentrated Amino acid-Dextrose formulations added to optimise amino acid and nutrient intake according to gestation. Organic phosphate has replaced inorganic phosphate allowing for an increase in calcium and phosphate content, and acetate reduced. Lipid emulsions are unchanged, with both SMOFlipid (Fresenius Kabi, Australia) and ClinOleic (Baxter Healthcare, Australia) preparations included. The physicochemical compatibility and stability of all formulations have been tested and confirmed. Guidelines to standardise the parenteral nutrition clinical practice across facilities have also been developed. Conclusions The 2017 PN formulations and guidelines developed by the 2017 Neonatal Parenteral Nutrition Consensus Group offer concise and practical instructions to clinicians on how to implement current and up-to-date evidence based PN to the NICU population.

ObjectiveTo determine the risk factors for mortality associated with late onset sepsis (LOS) among preterm very-low-birthweight (VLBW) infants.Study DesignWe performed a retrospective cohort study of infants born 0.3 [AOR 3.6 (1.3, 9.7)] were independently associated with LOS mortality.ConclusionsIn a setting with predominantly Gram-negative bacterial infections, the need for inotropic support, leukopenia, and elevated I: T ratio were significantly associated with LOS mortality among preterm VLBW infants.

Background: Patent ductus arteriosus (PDA) causing significant left to right shunt can increase key morbidities in preterm infants. Yet, treatment does not improve outcomes and spontaneous closure is the natural course of PDA. The Impact of PDA on 23–26-week gestation infants is uncertain. Selective treatment of such infants would likely balance outcomes.Objective: To test the hypothesis that treatment of PDA in high-risk VLBW infants [birth weight ≤800 g or gestation

The pathogenesis of massive lower gastrointestinal bleed (LGIB) due to angio-dysplasia and ulceration is rare and not well understood. We discuss an unusual presentation of a child with painless massive LGIB arising from chronic intestinal adhesions causing subacute small bowel obstruction and angio-dysplastic ulceration but without any clinical or radiological evidence of bowel obstruction.

Maturity-onset diabetes of the young (MODY) classically describes dominantly inherited forms of monogenic diabetes diagnosed before 25 years of age due to pancreatic β-cell dysfunction. In contrast, mutations in certain MODY genes can also present with transient or persistent hyperinsulinemic hypoglycemia in newborn infants, reflecting instead β-cell dysregulation. Of the MODY genes described to date, only hepatocyte nuclear factor-4-alpha (HNF4A; MODY1) and hepatocyte nuclear factor-1-alpha (HNF1A; MODY3) mutations may result in a biphasic phenotype of hypoglycemia in early life and hyperglycemia in later life. We report a family with a novel HNF4A mutation with diverse phenotypic presentations of glucose dysregulation. The proband was a term, appropriate-for-gestational age male infant with symptomatic hypoglycemia on day 3 of life needing high glucose infusion rate to maintain normoglycemia. He was born to a non-obese and non-diabetic mother. Glucose regulation was optimized using diazoxide upon confirmation of hyperinsulinism. Cascade genetic screening identified the same mutation in his father and elder sister, but mother was negative. Father was diagnosed with Type 1 diabetes at 15 years of age that required insulin therapy. Proband's elder sister, born at term appropriate for gestational age, presented with transient neonatal hypoglycemia needing parenteral glucose infusion for a week followed by spontaneous resolution. The paternal grandparents were negative for this mutation, confirming a paternal de novo mutation and autosomal dominant inheritance in this family. This pedigree suggests that the presence of early-onset paternal diabetes should prompt molecular testing in infants presenting in the newborn period with diazoxide-responsive hyperinsulinemic hypoglycemia, even in the absence of maternal diabetes and macrosomia.

Background: With increasing acceptance of universal developmental screening in primary care, it is essential to evaluate the local validity and psychometric properties of commonly used questionnaires like the parent-completed Ages and Stages Questionnaires, 3rd Edition (ASQ-3) in identifying developmental delays. The aim of this study is to assess the convergent validity of the ASQ-3 with the Bayley Scales of Infant Development-3rd edition (Bayley-III) in identifying developmental delay in a low-risk term cohort in Singapore. Methods: ASQ-3 and Bayley-III data was collected prospectively with generation of ASQ-3 cut-off scores using three different criteria: 1-standard deviation (SD) (Criterion-I) or 2-SD (Criterion-II) below the mean, and using a Receiver Operator Curve (ROC) (Criterion-III). Sensitivity, specificity, positive (PPV) and negative (NPV) predictive values were calculated. Correlations between the ASQ-3 and Bayley-III domains were evaluated using Pearson coefficients. Results: With all three criteria across different domains ASQ-3 showed high specificity (72–99%) and NPV (69–98%), but lower sensitivity (19–74%) and PPV (11–59%). Criterion-I identified 11–21% of children as “at-risk of developmental delay,” and was the most promising criterion measure, with high specificity (82–91%), NPV (69–74%) and overall agreement of 64–71%. Moderate-strong correlations were seen between ASQ-3 Communication and Bayley-III Language scales (r = 0.44–0.59, p

ObjectivesTo evaluate serial tissue Doppler cardiac imaging (TDI) in the evolution of bronchopulmonary dysplasia-associated pulmonary hypertension (BPD-PH) among extremely preterm infants.DesignProspective observational study.SettingSingle-center, tertiary-level neonatal intensive care unit.PatientsInfant born

Background: Very preterm infants are at risk for neurodevelopmental impairment because of postnatal morbidities. This study aims to (1) compare the outcomes of very-low-birth-weight (VLBW) infants in Singapore during two time periods over a decade; 2) compare performances among Singaporean neonatal intensive care units (NICUs); and 3) compare a Singapore national cohort with one from the Australian and New Zealand Neonatal Network (ANZNN). Methods: Singapore national data on VLBW infants born during two periods, 2007–2008 (SG2007, n = 286) and 2015–2017 (SG2017, n = 905) were extracted from patient medical records. The care practices and clinical outcomes among three Singapore NICUs were compared using SG2017 data. Third, using data from the ANZNN2017 annual report, infants with gestational age (GA) ≤29 weeks in SG2017 were compared with their Oceania counterparts. Results: SG2017 had 9.9% higher usage of antenatal steroids (p

Objective Neonatal encephalopathy remains a major cause of infant mortality and neurodevelopmental impairment. Infection may exacerbate brain injury and mitigate the effect of therapeutic hypothermia (TH). Additionally, infants with sepsis treated with TH may be at increased risk of adverse effects. This study aimed to review the clinical characteristics and outcomes for infants with sepsis treated with TH.Design and setting Retrospective cohort study of infants treated with TH within Australia and New Zealand.Patients 1522 infants treated with TH, including 38 with culture-positive sepsis from 2014 to 2018.Intervention Anonymised retrospective review of data from Australian and New Zealand Neonatal Network. Infants with culture-positive sepsis within 48 hours were compared with those without sepsis.Main outcome measures Key outcomes include in-hospital mortality, intensive care support requirements and length of stay.Results Overall the rate of mortality was similar between the groups (13% vs 13%). Infants with sepsis received a higher rate of mechanical ventilation (89% vs 70%, p=0.01), high-frequency oscillatory ventilation (32% vs 13%, p=0.003) and inhaled nitric oxide for persistent pulmonary hypertension (38% vs 16%, p

Background: Congenital diaphragmatic hernia (CDH) is a common birth defect associated with significant mortality and morbidity. There is limited outcome data on CDH in the Southeast Asian region. Rapid accessibility to our CDH Perinatal Center, as a consequence of the small geographic size of our country and efficient land transportation system, has largely eliminated deaths of live outborn babies prior arrival at our center. We selected a study period when extracorporeal membrane oxygenation (ECMO) support was not available at our institution. The data will therefore be relevant in developing management guidelines and antenatal counselling for perinatal centers in this region managing CDH with limited resources, without ECMO facilities. Methods: A retrospective study of antenatally or postnatally diagnosed CDH infants born between January 2002 and June 2005 was performed. We selected this study period as ECMO support was not available over this period. We studied the demographics, clinical characteristics, postnatal predictors of mortality and outcomes of CDH infants in a single tertiary institution. Results: A total of 24 patients with CDH were identified. Seventy-nine percent of liveborns with CDH survived to hospital discharge. Antenatal detection rate was 83.3%. Significant postnatal predictors of mortality were preoperative pneumothorax (p = 0.035), high CRIB score (p = 0.007), low one- and five-minute Apgar score (p = 0.011, p = 0.026 respectively) and high pCO2 on initial arterial blood gas (p = 0.007). At one-year follow-up, three patients had delayed gross motor milestones which resolved subsequently. Re-admissions were required for recurrent bronchiolitis (33%) and oesophageal reflux which resolved in all cases. Two (13.3%) infants had surgical complications and needed re-admission for probable adhesive intestinal obstruction; one required adhesiolysis and the other was managed conservatively with good outcome. Conclusion: A single-center CDH outcome in Singapore, without ECMO use, was good. This is a cohort now with long-term survival outcome which will be valuable to the neonatology community.

Suresh Chandran,1–4 Victor Samuel Rajadurai,1–3 Abdul Alim Abdul Haium,1–3 Khalid Hussain5,6 1Department of Neonatology, KK Women’s and Children’s Hospital, Singapore; 2Duke-NUS Graduate School of Medicine, Singapore; 3Yong Loo Lin School of Medicine, National University of Singapore, Singapore; 4Lee Kong Chian School of Medicine, Nanyang Technological University, Singapore; 5Department of Paediatric Endocrinology, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, 6The Institute of Child Health, University College London, London, UK Abstract: Glucose is an essential substrate for mammalian cells; in particular, the brain needs glucose continuously as a primary source of energy. Hypoglycemia is the most common biochemical finding in the neonatal period. However, despite the common occurrence, there is still controversy on the definition of hypoglycemia in the newborn period. This has led to the development of guidelines designed to identify infants “at-risk” and the implementation of an “operational threshold” for physicians to consider intervention. In healthy term infants, the optimal hormonal and metabolic adaptations during the immediate neonatal period ensure an adequate energy substrate for the vital organs, whereas the abnormal glucose homeostasis observed in preterm and growth-retarded infants is multifactorial in origin. For these high-risk infants, it is important to identify, screen, and prevent significant hypoglycemia. Detailed investigations are warranted in infants with severe and persistent hypoglycemia. Neonatal hypoglycemia is a major cause of brain injury. The speculated mechanisms of cellular injury include excitatory neurotoxins active at N-methyl-D-aspartate receptors, increased mitochondrial free radical generation with initiation of apoptosis and altered cerebral energetic characteristics. This hypoglycemic brain injury predominantly affects parieto-occipital regions causing cognitive, sensory, psychomotor, and behavioral deficits in children. This state of the art review article covers the fetal–neonatal metabolic adaptation, glucose homeostasis in normal and abnormal conditions, management strategies, and late neurological sequelae of hypoglycemia. Keywords: hyperinsulinism, hypoglycemic brain injury