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To evaluate cerebral tissue oxygenation (cTOI) and cerebral perfusion in preterm infants in supine vs prone positions.Sixty preterm infants, born before 32 weeks of gestation, were enrolled; 30 had bronchopulmonary dysplasia (BPD, defined as the need for respiratory support and/or supplemental oxygen at 36 weeks of postmenstrual age). Cerebral perfusion, cTOI, and polysomnography were measured in both the supine and prone position with the initial position being randomized. Infants with a major intraventricular hemorrhage or major congenital abnormality were excluded.Cerebral perfusion was unaffected by position or BPD status. In the BPD group, the mean cTOI was higher in the prone position compared with the supine position by a difference of 3.27% (P = .03; 95% CI 6.28-0.25) with no difference seen in the no-BPD group. For the BPD group, the burden of cerebral hypoxemia (cumulative time spent with cTOI55%) was significantly lower in the prone position (23%) compared with the supine position (29%) (P .001). In those without BPD, position had no effect on cTOI.In preterm infants with BPD, the prone position improved cerebral oxygenation and reduced cerebral hypoxemia. These findings may have implications for positioning practices. Further research will establish the impact of position on short- and long-term developmental outcomes. more...

To compare neurodevelopmental outcomes of extremely preterm infants diagnosed with patent ductus arteriosus (PDA) who were treated medically or surgically and those who were not diagnosed with PDA or who did not undergo treatment for PDA.This retrospective population-based cohort study used data from a geographically defined area in New South Wales and the Australian Capital Territory served by a network of 10 neonatal intensive care units. Patients included all preterm infants born at29 completed weeks of gestation between 1998 and 2004. Moderate/severe functional disability at 2-3 years corrected age was defined as developmental delay, cerebral palsy requiring aids, sensorineural or conductive deafness (requiring bilateral hearing aids or cochlear implant), or bilateral blindness (best visual acuity of6/60).Follow-up information at age 2-3 years was available for 1473 infants (74.8%). Compared with infants not diagnosed with a PDA or who did not receive PDA treatment for PDA, those with medically treated PDA (aOR, 1.622; 95% CI, 1.199-2.196) and those with surgically treated PDA (aOR, 2.001; 95% CI, 1.126-3.556) were at significantly greater risk for adverse neurodevelopmental outcomes at age 2-3 years.Our results demonstrate that treatment for PDA may be associated with a greater risk of adverse neurodevelopmental outcome at age 2-3 years. This was particularly so among infants born at25 weeks gestation. These results may support permissive tolerance of PDAs; however, reasons for this association remain to be elucidated through carefully designed prospective trials. more...

Hypoparathyroidism-retardation-dysmorphism (HRD) syndrome (OMIM 241410), also known as SanjadSakati syndrome, is a rare autosomal recessive disorder characterized by hypoparathyroidism, growth failure, developmental delay, and characteristic facies. We describe the effective short-term use (tapered over 12 days) of recombinant parathyroid hormone (PTH) (teriparatide) in an unusual genetic condition characterized by hypoparathyroidism. Standard treatment for hypoparathyroidism involves oral vitamin D analogues and calcium, often in large doses to achieve sufficient intestinal calcium transport to overcome the hypercalciuria and concomitant malabsorption. 1 Despite its known physiological benefits, 2 recombinant PTH is not usually considered in the treatment algorithm for hypoparathyroidism in the pediatric population. Case Report Approval for this case report was obtained from the Human Research Ethics Committee of The Children’s Hospital at Westmead. Informed consent was obtained from the family. A female infant was born to first-cousin Iraqi parents at 31 weeks gestation, after an antenatal course that included oligohydramnios and fetal growth restriction. The parents had previously experienced 2 first-trimester miscarriages and fetal death at 30 weeks, and the current pregnancy was conceived after in vitro fertilization. She had a birth weight of 1190 g (SDS, 1.02), length of 36.5 cm (SDS, 1.77), and head circumference of 25.4 cm (SDS, 1.61). Dysmorphic features included deep-set eyes, large ears with hypoplastic posterior helices, and a thin nose (Figure 1). The newborn period was complicated by respiratory distress syndrome, treated with surfactant, mechanical ventilation, and continuous positive airway pressure. Significant feeding intolerance and vomiting were present from the first week of life. Upper gastrointestinal studies showed gastric hypomotility and delayed passage into the duodenum with no mechanical obstruction. Transpyloric tube feeding was started at 4 weeks after birth. Fat malabsorption was confirmed by fecal fat analysis and low serum vitamin A and E levels. Results of a sweat test were normal. Pancreatic enzyme replacement and fat-soluble more...

To ascertain developmental differences between term infants after major noncardiac surgery and cardiac surgery compared with healthy control infants in New South Wales, Australia.This prospective population-based cohort study enrolled infants between August 1, 2006, and December 31, 2008, who required major noncardiac surgery within the first 90 days of life. Developmental outcomes were compared in these children, cohorts of term infants requiring cardiac surgery, and healthy controls. Infants were assessed at 1 year of age using the Bayley Scales of Infant and Toddler Development, Third Edition (BSID-III).Of the 784 infants enrolled, 688 (90.2%) of infants alive at 1 year were assessed. Of these, 539 infants were term and were included in the present analysis. Compared with controls, the infants who underwent cardiac surgery had significantly lower (P.001) mean scores in all 5 BSID-III subscales, and the infants who underwent noncardiac surgery had significantly lower (P.05) mean scores in 4 of the 5 BSID-III subscales. The greatest difference was in the incidence of gross motor delay in both the cardiac surgery group (OR, 0.25; 95% CI, 0.16-0.41) and the noncardiac surgery group (OR, 0.41; 95% CI, 0.26-0.63).This unique population-based prospective study compared the developmental outcomes of infants who underwent major noncardiac surgery and cardiac surgery. Major surgery in infants was found to be significantly associated with developmental delay at 1 year of age compared with control infants. These data have important implications for interventions and clinical review in the first year of life. more...