Interventions for Pulmonary Vein Stenosis.

Pulmonary vein stenosis (PVS) is a rare and unique disease of infants and young children. PVS is attended by high morbidity and mortality, and for many decades, effective therapy eluded the practitioner. However, in the most recent era, interventional techniques when employed in combination with systemic (primary) therapy have had a remarkable impact on outcomes in these at-risk children. Despite apparent complete relief of PVS in a discrete region of a pulmonary vein, stenosis reliably recurs and progresses. In this review, we discuss the current state-of-the-art interventional techniques, through the lens of our collective experiences and practices.

Hemostatic efficacy of pathogen-reduced platelets in children undergoing cardiopulmonary bypass.

BACKGROUND: Pediatric patients undergoing cardiopulmonary bypass (CPB) often require blood component transfusions. Pathogen-reduction (PR) of platelets reduces the risk of microbial contamination; however, its effect on hemostatic efficacy in this population is unclear. This study sought to characterize the hemostatic efficacy of PR platelets in children undergoing CPB. STUDY DESIGN AND METHODS: We performed a retrospective chart review of patients admitted to a pediatric intensive care unit following CPB surgery from 2015 to 2019. Demographic data, validated scoring of repair complexity, products received, and outcomes were compared. The primary outcome was postoperative chest tube bleeding. RESULTS: A total of 140 patients were enrolled. The majority of surgeries (124/140) were Risk Adjustment for Congenital Heart Surgery (RACHS) 1-3 repairs. Seventy-four percent of patients (104/140) received only standard platelets whereas 26% (36/140) received PR platelets. There were no differences between the groups in the age (p = .90), sex (p = .20) or RACHS score (p = .06). Postoperatively, there was no difference in the median chest tube output for 1 h (p = .27), 2 h (p = .26), 4 h (p = .09), 8 h (p = .16), or for the first 24 h following surgery (p = .23) in patients who received standard versus PR platelets. There was also no difference in receipt of platelets (p = .18), cell saver (p = .79), or cryoprecipitate (p = .28). CONCLUSION: Patients receiving PR platelets did not have more blood loss or require more transfusions than those who received standard platelets. This suggests that PR platelets may provide acceptable hemostasis with the additional benefits of reduced risk of microbial contamination in pediatric patients undergoing CPB.

Microbiota modulate behavioral and physiological abnormalities associated with neurodevelopmental disorders.

Neurodevelopmental disorders, including autism spectrum disorder (ASD), are defined by core behavioral impairments; however, subsets of individuals display a spectrum of gastrointestinal (GI) abnormalities. We demonstrate GI barrier defects and microbiota alterations in the maternal immune activation (MIA) mouse model that is known to display features of ASD. Oral treatment of MIA offspring with the human commensal Bacteroides fragilis corrects gut permeability, alters microbial composition, and ameliorates defects in communicative, stereotypic, anxiety-like and sensorimotor behaviors. MIA offspring display an altered serum metabolomic profile, and B. fragilis modulates levels of several metabolites. Treating naive mice with a metabolite that is increased by MIA and restored by B. fragilis causes certain behavioral abnormalities, suggesting that gut bacterial effects on the host metabolome impact behavior. Taken together, these findings support a gut-microbiome-brain connection in a mouse model of ASD and identify a potential probiotic therapy for GI and particular behavioral symptoms in human neurodevelopmental disorders.