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Purpose: The purpose of this article is to demonstrate the superiority of gevokizumab as compared to placebo, on top of current standard of care, in reducing the risk of Behçet's disease uveitis (BDU) exacerbations.Methods: Randomized, double-masked, placebo-controlled, parallel group, event-driven trial in BDU patients having recently experienced an ocular exacerbation, subsequently undergoing a tapering procedure from high-dose corticosteroids and receiving 60 mg gevokizumab or placebo every 4 weeks subcutaneously (EYEGUARD B-ClinicalTrials.gov NCT 01965145).Results: A total of 83 patients (40 gevokizumab, 43 placebo) were included. Gevokizumab did not significantly affect the risk of occurrence of ocular exacerbations. However, data suggested that gevokizumab could preserve visual acuity, reduce the uveitis severity, decrease the emergence of macular edema, and have a corticosteroid sparing effect. Gevokizumab was well tolerated.Conclusions: While the primary efficacy endpoint was not met with gevokizumab, the control of IL-1β pathway in patients with BDU may still be a relevant target. [ABSTRACT FROM AUTHOR]

Objective To provide outcome data concerning pregnancies exposed to the Interleukin-1 (IL-1) inhibitors prior to conception in both men and women, during pregnancy and breast feeding. Methods Retrospective data were collected from members of the International Society for Systemic Autoinflammatory diseases and collated in a single centre. A uniform data collection sheet was used to obtain standardized data including maternal age and diagnosis, type, duration of and response to IL-1 blockade, pregnancy duration, delivery, mode of feeding and neonatal development. Results There were 31 maternal-exposed pregnancies from seven countries and we report the first data on paternal exposure: six to anakinra and five to canakinumab, with no negative outcomes. We also report the first data on canakinumab-exposed pregnancies: eight pregnancies that resulted in the delivery of seven healthy infants of normal gestational age and birthweight. There were 23 anakinra-exposed pregnancies resulting in the birth of 21 healthy infants, and one baby with unilateral renal agenesis and ectopic neurohypophysis. There were two first trimester miscarriages affecting a mother with active disease. There were no serious neonatal infections. Fourteen infants were breast fed with no complications. There were no reports of developmental delay, with follow-up of up to 10 years (median 18 months). Conclusion This series substantially increases the published experience of IL-1 blockade and reproduction including the first data on canakinumab and on paternal exposure to these agents. Data are generally reassuring, although the case of renal agenesis is the second reported in an anakinra-exposed pregnancy. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the natural history of isolated muscular ventricular septal (m-VSD) defects during gestation and up to 1 year postnatally, as well as the association with chromosomal anomalies. Material and Methods: Between August 2007 and July 2012, 76 fetuses with isolated m-VSDs represented the study population. The following variables were evaluated: site and size of the m-VSDs, presence of chromosomal anomalies, pregnancy outcome, and spontaneous closure rate from diagnosis up to 1 year postnatally. Results: Of the 76 cases with m-VSD, 1 fetus died after birth and 31 cases were lost to follow-up after birth. Thus, a total of 44 fetuses reached their first year of postnatal life, and these cases were available for analysis. Three (6.8%) of 44 defects closed spontaneously in utero, 33 (75%) closed within 1 year, and 8 (18.2%) remained patent. Overall, spontaneous closure occurred more frequently in the apical defects, but no significant difference was found for spontaneous closure between the mid-muscular and apical defects (p > 0.05). Also, 83.8% (36 of 44) of defects ≤3 mm closed during gestation or the first year of life. Conclusion: We infer that m-VSDs have a high spontaneous closure rate during the first year of life. Also, small m-VSDs frequently close spontaneously. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the prenatal findings, associated anomalies, and prognosis of right aortic arch (RAA) anomalies. Material and Methods: All cases referred for detailed ultrasonography and fetal echocardiography between October 2006 and July 2009 were systematically examined for aortic arch anomalies and associated cardiac and extracardiac anomalies. Prenatal findings of all cases with aortic arch anomalies and intracardiac and extracardiac findings were prospectively registered in an electronic database that included fetal echocardiography. Outcomes of the cases were collected postnatally from the patients' obstetricians, neonatal unit archieves, and pediatric cardiologists. Results: We detected 12 cases of RAA (0.37%; n=12/3200). Mean gestational age at diagnosis was 24 weeks (range, 21-33 weeks). Of the 12 cases of RAA, five (41.7%) had a major cardiac defect, including tetralogy of Fallot (n= 3), atrioventricular septal defect (n=1), and ventricular septal defect (n=1). An extracardiac anomaly was observed in three cases (25%). The fetal karyotype was trisomy 21 in one case with increased nuchal translucency (6.6 mm). Microdeletion 22q11 analyses performed in three cases were normal. The postnatal courses of the cases with isolated RAA were uneventful. Two cases associated with major cardiac and extracardiac anomalies were lost in the early neonatal period. The case of trisomy 21 was terminated. The other four cases of RAA with an associated cardiac anomaly are currently in follow up. Conclusion: Aortic arch anomalies, particularly RAA, can be diagnosed by fetal echocardiography. The prognosis for isolated RAA is relatively good compared with that for RAA with associated anomalies. [ABSTRACT FROM AUTHOR]