Ventricular assist devices (VAD) are used more in children. Safe and effective anticoagulation is required for successful management of children supported with ventricular assist devices. Developmental hemostasis, device hemocompatibility, plastic to body ratio, surgical variable techniques, lack of knowledge on pharmacokinetics of anticoagulants, and wide variability in anticoagulation protocols have all contributed to increased incidence of bleeding and thromboembolic complications. New collaborative learning networks, such as the ACTION network, provide opportunities to define best practices, optimize, and reduce anticoagulation related adverse events. ACTION was established Dec 2017. It consists of expert clinicians in heart failure, as well as researchers, parents, and patients, with goals to improve outcomes, share data, improve education and standard practice for children with heart failure (1, n.d). Changes in pediatric VAD anticoagulation strategy from using mainly heparin to DTI such as bivalirudin have helped reduce bleeding and clotting complications.
The pathogenesis of necrotizing enterocolitis (NEC) remains poorly understood but is thought to be multifactorial. There are no specific recurring chromosomal abnormalities previously associated with NEC. We report 3 cases of intestinal necrosis associated with large chromosome 6 deletions. The first patient was found to have a 7.9-Mb deletion of chromosome 6 encompassing over 40 genes, arr[GRCh37] 6q25.3q26(155699183_163554531)×1. The second patient had a 19.5-Mb deletion of chromosome 6 generated by an unbalanced translocation with chromosome 18, 46,XY,der(6)t (6;18)(q25.1;p11.23), arr[GRCh37] 6q25.1q27(151639526_ 171115067)×1, 18p11.32p11.23(131700_7694199)×3, which included the whole 7.9-Mb region deleted in the first patient. The third patient was the younger sibling of the second patient with an identical derivative chromosome 6. The shared abnormal chromosome 6 region includes multiple genes of interest, particularly EZR. Mouse models have demonstrated that Ezr is expressed in microvillar epithelium and helps regulate cell-cell adhesion in the gut. We hypothesize that deletion of this shared region of 6q leads to gastrointestinal vulnerability which may predispose patients to intestinal necrosis.
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