Efficacy and Safety of Eculizumab in Pediatric Patients Affected by Shiga Toxin-Related Hemolytic and Uremic Syndrome: A Randomized, Placebo-Controlled Trial.

Significance Statement: Shiga toxin-related hemolytic uremic syndrome (STEC-HUS) is a serious condition, characterized by multiorgan thrombotic microangiopathy, mainly affecting children. Renal involvement is severe, with approximately half of patients requiring dialysis. So far, no specific treatment has been proven efficient in STEC-HUS.

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 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).

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.

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.

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.

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).