We report the largest series of HPeV-3 infection in infants, and the first outbreak in Australia. Infants presented with a severe sepsis-like syndrome with a high rate of ICU admissions, but all recovered from the acute infection without complications. Long-term sequelae are unknown.

Cantú syndrome (CS), characterized by hypertrichosis, distinctive facial features, and complex cardiovascular abnormalities, is caused by pathogenic variants in ABCC9 and KCNJ8 genes. These genes encode gain‐of‐function mutations in the regulatory (SUR2) and pore‐forming (Kir6.1) subunits of KATP channels, respectively, suggesting that channel‐blocking sulfonylureas could be a viable therapy. Here we report a neonate with CS, carrying a heterozygous ABCC9 variant (c.3347G>A, p.Arg1116His), born prematurely at 32 weeks gestation. Initial echocardiogram revealed a large patent ductus arteriosus (PDA), and high pulmonary pressures with enlarged right ventricle. He initially received surfactant and continuous positive airway pressure ventilation and was invasively ventilated for 4 weeks, until PDA ligation. After surgery, he still had ongoing bilevel positive airway pressure (BiPAP) requirement, but was subsequently weaned to nocturnal BiPAP. He was treated for pulmonary hypertension with Sildenafil, but failed to make further clinical improvement. A therapeutic glibenclamide trial was commenced in week 11 (initial dose of 0.05 mg–1 kg–1 day–1 in two divided doses). After 1 week of treatment, he began to tolerate time off BiPAP when awake, and edema improved. Glibenclamide was well tolerated, and the dose was slowly increased to 0.15 mg−1 kg−1day−1 over the next 12 weeks. Mild transient hypoglycemia was observed, but there was no cardiovascular dysfunction. Confirmation of therapeutic benefit will require studies of more CS patients but, based on this limited experience, consideration should be given to glibenclamide as CS therapy, although problems associated with prematurity, and complications of hypoglycemia, might limit outcome in critically ill neonates with CS.

No abstract

The authors aimed to evaluate preoperative amplitude-integrated electroencephalography (aEEG) patterns for predicting neurodevelopmental outcome among infants undergoing major surgery in the neonatal period. They retrospectively reviewed the preoperative aEEG data of 58 neonates who had undergone major neonatal surgery between 2006 and 2008. The authors classified aEEGs using a weighted background score. Neurodevelopmental outcome was assessed at 3 years of age using the Bayley Scales of Toddler and Infant Development III. Over a third of infants (36%) showed an abnormal aEEG background. Seizure activity was identified in 11 (19%) infants. The majority (68%) of infants had developmental delay, with no significant differences between cardiac and other surgery groups. Logistic regression found no statistically significant but some clinically important associations between aEEG background and neurodevelopmental outcome. Comorbidity was associated with worse outcomes. While the predictive utility of aEEG in this population remains unclear, the findings suggest that further research is warranted.