Current opinion in pediatrics
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The essential role of the lymphatic system in fluid homeostasis, nutrient transport, and immune trafficking is well recognized; however, there is limited understanding of the mechanisms that regulate lymphatic function, particularly in the setting of critical illness. The lymphatics likely affect disease severity and progression in every condition, from severe systemic inflammatory states to respiratory failure. Here, we review structural and functional disorders of the lymphatic system, both congenital and acquired, as they relate to care of the pediatric patient in the intensive care setting, including novel areas of research into medical and procedural therapeutic interventions. ⋯ As our understanding of normal lymphatic biology continues to advance, we will be able to develop novel strategies to support and augment lymphatic function during critical illness and through convalescence.
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Curr. Opin. Pediatr. · Jun 2018
ReviewCystic fibrosis transmembrane conductance regulator modulators: precision medicine in cystic fibrosis.
The aim of this study was to describe the newest development in cystic fibrosis (CF) care, CF transmembrane conductance regulator (CFTR) modulator therapies. ⋯ CFTR modulator therapy targets the protein defective in CF and boosts its function, but the drug must match mutation pathobiology. Ivacaftor, a CFTR potentiator, was the first modulator approved in 2012, with impressive improvement in lung function and other measures of disease in patients with gating and other residual function mutations (∼10% of CF patients). In 2015, the combination of lumacaftor, a CFTR corrector, and ivacaftor was approved for patients homozygous for the F508del mutation (∼40-50% of the CF population) with positive but less impressive clinical response and 10-20% incidence of intolerance. A next-generation CFTR corrector, tezacaftor, with ivacaftor equally effective and better tolerated than lumacaftor, has also received US Food and Drug Administration approval. Novel CFTR correctors, entering Phase 3 trials in triple modulator combination with tezacaftor-ivacaftor, appear substantially more effective for patients who are homozygous for the F508del mutation and can provide benefit for patients with a single F508del mutation. This offers promise of effective CFTR modulator therapy for nearly 90% of CF patients.
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Pediatric inguinal hernias are a commonly performed surgical procedure. Currently, they can be approached via open or laparoscopic surgery. We summarize the current evidence for laparoscopic inguinal hernia repairs in children. ⋯ Laparoscopic surgery for pediatric inguinal hernias offers some advantages over open repair with most outcomes being equal. It should be considered a safe alternative to open repair to children and their caregivers.
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Curr. Opin. Pediatr. · Jun 2018
Review Case ReportsDamage control resuscitation in pediatric trauma.
Damage control resuscitation is an overall management strategy used in trauma patients to rapidly restore physiologic stability, while mitigating hypothermia, coagulopathy and acidosis. We review the evidence and current practice of damage control resuscitation in pediatric trauma patients with a specific focus on fluid management. ⋯ Attention to establishing rapid vascular access and correcting hypothermia and acidosis is essential. A judicious approach to crystalloid resuscitation in the bleeding pediatric trauma patient with early use of blood products in keeping with an organized approach to massive hemorrhage is recommended. The ideal crystalloid volumes and/or blood product ratios in pediatric trauma patients have yet to be determined.