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Objective: To evaluate the morbidity and mortality of neonates with left-sided isolated congenital diaphragmatic hernia (CDH) according to gestational age at delivery., Methods: This was a retrospective study of fetuses diagnosed prenatally with isolated left-sided CDH that were delivered in the University Hospitals of Antoine Béclère-Bicêtre and Leuven between 1 January 2010 and 31 December 2018. The Kaplan-Meier method was used to calculate cumulative survival at 28 days after birth according to gestational age at delivery. The association between gestational age at delivery, as a continuous variable, and survival at 28 days was modeled using a fractional polynomial. Adjustment for position of the liver, management center and mode of delivery was performed. The association was also evaluated according to the severity of CDH, as defined by the observed-to-expected lung-to-head ratio (o/e-LHR), which was classified as severe (o/e-LHR < 25%), moderate (o/e-LHR between 25% and 45%) or mild (o/e-LHR > 45%)., Results: We included 213 fetuses with isolated left-sided CDH, with a median gestational age at delivery of 38 + 2 weeks (interquartile range, 37 + 0 to 39 + 1 weeks). The survival rates at 28 days and at 6 months were 66.7% (142/213) and 64.3% (137/213), respectively. Kaplan-Meier analysis showed a higher survival rate at 28 days for babies delivered between 37 + 0 and 38 + 6 weeks than for those delivered at or after 39 + 0 weeks (log-rank test, P < 0.001). In the subgroup of moderate CDH, the 28-day survival rate was significantly higher in newborns delivered between 37 + 0 and 38 + 6 weeks than in those delivered at or after 39 + 0 weeks (81.5% vs 61.5%; P = 0.03), and this was also the case for survival rate at 6 months. In the subgroup with moderate CDH, 28-day survival significantly increased with advancing gestational age at birth up to about 38-39 weeks (P = 0.005), and significantly decreased from 39 weeks onwards., Conclusion: Delivery between 37 + 0 and 38 + 6 weeks' gestation is associated with a higher survival rate at 28 days in neonates with isolated left-sided CDH and moderate lung hypoplasia, independently of intrathoracic liver, management center and mode of delivery. © 2020 International Society of Ultrasound in Obstetrics and Gynecology., (© 2020 International Society of Ultrasound in Obstetrics and Gynecology.)

Objective: To evaluate the safety and efficacy of rituximab (RTX) in juvenile dermatomyositis (JDM) in off-trial patients., Methods: We conducted a multicenter prospective study of patients with JDM included in the French Autoimmunity and Rituximab (AIR) registry., Results: Nine patients with severe JDM were studied. The main indication for RTX treatment was severe and/or refractory muscle involvement (7 patients), severe calcinosis (1 patient), or severe chronic abdominal pain associated with abdominal lipomatosis (1 patient). RTX was associated with corticosteroids, immunosuppressive drugs, and plasma exchange therapy in 9/9, 5/9, and 2/9 patients, respectively. Mild infections of the calcinosis sites occurred in 2 patients and an infusion-related event in 1. Complete clinical response was achieved in 3/6 patients treated with RTX for muscle involvement. In these responders steroid therapy was stopped or tapered to < 15% of the baseline dosage, with no relapse, with a followup ranging from 1.3 to 3 years. Calcinosis did not improve in the 6 affected patients., Conclusion: This small series suggests that rituximab may be effective for treating muscle and skin involvement in a small subset of children with severe JDM, and that its safety profile was satisfactory. Further studies are needed to identify predictive factors of response to RTX in patients with severe JDM.