Key Clinical MessageDystonia 24 was first reported in 2000 as an autosomal dominant cause of dystonia caused by variants in the ANO3 gene. Although many adults have been described with dystonia 24, since 2014, an increasing number of children have also been reported. Dystonia 24 should also be considered in the differential of a child with unexplained dystonia.
Objective: To determine clinical correlates of moderate to severe bronchopulmonary dysplasia (BPD) in preterm infants following surgical necrotizing enterocolitis (NEC).
Methods: A retrospective, single-center cohort study comparing patients with moderate to severe BPD to patients with non/mild BPD among surgical NEC infants. BPD was defined by NIH 2001 consensus definition
Results: Of 92 consecutive neonates with surgical NEC, 77% (71/92) had moderate/severe BPD and 22% (21/92) had non/mild BPD. The patent ductus arteriosus (PDA) was significantly higher in those developing moderate/severe BPD (67.6% [48/71]) than non/mild BPD (28.6% [6/21]; p=0.001). Postoperatively,infants with moderate/severe BPD had more severe AKI (67.6% [48/71] vs. 28.6% [6/21]; p=0.001), were intubated longer (40.5 days [IQR 12, 59] vs. 6 days [IQR 2, 13]; p<0.001), received more parenteral nutrition (109 days [IQR 77, 147] vs. 55 days [IQR 19, 70]; p<0.001), developed higher surgical morbidity (46.5% [33/71] vs. 14.3% [3/21]; p=0.008), had more intestinal failure (62.5% vs. 13.3%; p<0.001), required a longer hospital stay (161 days [IQR 112, 186] vs. 64 days [IQR 20, 91]; p<0.001), and were more likely to need home oxygen. In a multivariable analysis, lower birth weight (OR=0.3, [95% CI 0.1-0.5]; p=0.001), patent ductus arteriosus (OR=10.3, [95%CI 1.6-65.4]; p=0.014) and longer parenteral nutritional days (OR=8.8; [95%CI 2.0 -43.0]; p=0.005) were significantly and independently associated with higher odds of moderate/severe vs. non/mild BPD.
Conclusion: Development of moderate/severe BPD occurred in the majority of preterm infants with surgical NEC in this consecutive series. Preterm infants with moderate/severe BPD were more likely to have a PDA before NEC. Development of moderate/severe BPD was associated with significantly greater burden and duration of post-operative morbidity following surgical NEC. Identifying surgical NEC infants at increased risk of moderate/severe BPD and developing lung protection strategies may improve surgical NEC outcomes.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.