Feeding disorders in children with oesophageal atresia: a cross-sectional study.

INTRODUCTION: With advances in surgical and neonatal care, the survival of patients with oesophageal atresia (OA) has improved over time. Whereas a number of OA-related conditions (delayed primary anastomosis, anastomotic stricture and oesophageal dysmotility) may have an impact on feeding development and although children with OA experience several oral aversive events, paediatric feeding disorders (PFD) remain poorly described in this population. The primary aim of our study was to describe PFD in children born with OA, using a standardised scale. The secondary aim was to determine conditions associated with PFD. METHODS: The Feeding Disorders in Children with Oesophageal Atresia Study is a national cohort study based on the OA registry from the French National Network. Parents of children born with OA between 2013 and 2016 in one of the 22 participating centres were asked to complete the French version of the Montreal Children's Hospital Feeding Scale. RESULTS: Of the 248 eligible children, 145 children, with a median age of 2.3 years (Q1-Q3 1.8-2.9, min-max 1.1-4.0 years), were included. Sixty-one children (42%) developed PFD; 13% were tube-fed (n=19). Almost 40% of children with PFD failed to thrive (n=23). The presence of chronic respiratory symptoms was associated with the development of PFD. Ten children with PFD (16%) had no other condition or OA-related complication. CONCLUSION: PFD are common in children with OA, and there is no typical profile of patients at risk of PFD. Therefore, all children with OA require a systematic screening for PFD that could improve the care and outcomes of patients, especially in terms of growth.

The dynamics of cardiac autonomic control in sleeping preterm neonates exposed in utero to smoking.

OBJECTIVE: We aimed to determine whether in utero exposure to smoking may influence the activity and dynamics of cardiac autonomic control in preterm infants. We hypothesized that cardiac autonomic control is altered in preterm infants exposed prenatally to smoking and that these effects may vary as a function of the sleep state. METHODS: We studied healthy, preterm neonates born to mothers who had smoked throughout pregnancy but not since birth (n=16). In utero-exposed neonates were matched with control preterm neonates born to non-smoking mothers (n=18). Cardiac autonomic control was monitored as a function of the sleep state by assessing heart rate variability with both linear and non-linear methods. RESULTS: Preterm neonates with in utero exposure to smoking displayed alterations (relative to control neonates) in short-term cardiac autonomic control in all sleep states. These alterations included low vagal activity, elevated sympathetic activity, and low complexity and adaptability in heart rate control dynamics. CONCLUSIONS: Our results constitute direct evidence that cardiac autonomic activity and control are altered in sleeping preterm infants exposed to smoking in utero. SIGNIFICANCE: These alterations may place the affected infants at a higher risk of neurological and cardiovascular complications, which could conceivably persist throughout childhood and adulthood.

A patient with Simpson-Golabi-Behmel syndrome, biliary cirrhosis and successful liver transplantation.

Simpson-Golabi-Behmel syndrome type 1 (SGBS1) -OMIM 312870- is a rare X-linked inherited overgrowth syndrome caused by a loss of function mutation in the GPC3 gene. Affected patients present a variable phenotype with pre- and post-natal macrosomia, distinctive facial dysmorphism, organomegaly, and multiple congenital anomalies. Intellectual disability is not constant. About 10% of patients have an increased risk of developing embryonic tumors in early childhood. Only one case of biliary disease has been described so far. GPC3 is localized on Xq26. It encodes for glypican 3, a heparan sulfate proteoglycan, which among its different known roles, negatively regulates liver regeneration and hepatocyte proliferation. This report concerns a male with a SGBS1, carrier of a GPC3 pathogenic mutation, and neonatal liver disease, who developed an early biliary cirrhosis. Together with the associated risk of cancer and developmental delay, liver transplantation was discussed and then successfully performed at the age of 19 months. A hypothesis on the role of GPC3 in the patient's liver disease is also proposed.

Involvement of autonomic nervous activity changes in gastroesophageal reflux in neonates during sleep and wakefulness.

BACKGROUND: It has been suggested that disturbed activity of the autonomic nervous system is one of the factors involved in gastroesophageal reflux (GER) in adults. We sought to establish whether transient ANS dysfunction (as assessed by heart rate variability) is associated with the occurrence of GER events in neonates during sleep and wakefulness. METHODS: Nineteen neonates with suspected GER underwent simultaneous, synchronized 12-hour polysomnography and esophageal multichannel impedance-pH monitoring. We compared changes in HRV parameters during three types of periods (control and prior to and during reflux) with respect to the vigilance state. RESULTS: The vigilance state influenced the distribution of GER events (P<0.001), with 53.4% observed during wakefulness, 37.6% observed during active sleep and only 9% observed during quiet sleep. A significant increase in the sympathovagal ratio (+32%, P=0.013) was observed in the period immediately prior to reflux (due to a 15% reduction in parasympathetic activity (P=0.017)), relative to the control period. This phenomenon was observed during both wakefulness and active sleep. CONCLUSION: Our results showed that GER events were preceded by a vigilance-state-independent decrease in parasympathetic tone. This suggests that a pre-reflux change in ANS activity is one of the factors contributing to the mechanism of reflux in neonates.

Effects of postnatal environmental tobacco smoke on non-nutritive swallowing-breathing coordination in newborn lambs.

While prenatal environmental tobacco smoke (ETS) exposure is a well-known risk factor for sudden infant death syndrome, the effect of postnatal ETS exposure is less clear. The objective of this study was to investigate the effect of postnatal ETS exposure on non-nutritive swallowing (NNS) and NNS-breathing coordination, which are crucial to prevent aspiration related-cardiorespiratory events. Eighteen newborn lambs (6 per group) were randomly exposed to either 10 cigarettes/day, 20 cigarettes/day or room air for 15 days. Lambs were instrumented for recording states of alertness, swallowing, electrocardiogram and breathing; recordings were performed in non-sedated lambs at the end of ETS exposure. Urinary cotinine/creatinine ratio confirmed relevant real-life exposure. Postnatal ETS exposure had no effect on NNS frequency but tended to decrease inspiratory NNS (p=0.07) during quiet sleep. No effect on respiratory or heart rate (p>0.6), apnea index (p=0.2) or sleep states (p=0.3) was observed. In conclusion, postnatal ETS exposure in lambs had only mild effects on NNS-breathing coordination.

Assessment of body composition in adolescent subjects with anorexia nervosa by bioimpedance.

Our main goal was to test various impedance methods for measuring body composition in adolescents with anorexia nervosa (AN) during refeeding therapy. A specific objective was to compare the information provided by a foot-to-foot impedancemeter (FFI) with that supplied by a medical multifrequency impedancemeter and by dual X-ray absorptiometry (DXA). We have monitored 13 young AN subjects and 17 healthy controls of a similar age group using a Xitron 4200 multifrequency impedancemeter measuring extracellular (ECW) and total body water (TBW) volumes and a Tefal Bodymaster FFI measuring weight (W), fat-free-mass (FFM) and body fat mass (FM). This Tefal device has been modified to measure in addition ECW and TBW resistances permitting to calculate ECW and TBW volumes using appropriate algorithms. In addition FFM and FM were measured by DXA on AN subjects. FFM measured by the FFI and the Xitron in AN subjects were found to be respectively 7.8% and 4.5% lower than FFM measured by DXA. TBW measured by FFI was not significantly different from that measured by Xitron in AN subjects and in controls. ECW measured by the FFI was not significantly different from that measured by Xitron in controls, but was in AN subjects. The body cell mass to (height)2 ratio was found to be significantly different between AN subjects and controls. The modified FFI was found to be simpler and quicker to use than the Xitron, while giving similar information.