Computational approaches to understanding interaction and development.

Audio-visual recording and location tracking produce enormous quantities of digital data with which researchers can document children's everyday interactions in naturalistic settings and assessment contexts. Machine learning and other computational approaches can produce replicable, automated measurements of these big behavioral data. The economies of scale afforded by repeated automated measurements offer a potent approach to investigating linkages between real-time behavior and developmental change. In our work, automated measurement of audio from child-worn recorders-which quantify the frequency of child and adult speech and index its phonemic complexity-are paired with ultrawide radio tracking of children's location and interpersonal orientation. Applications of objective measurement indicate the influence of adult behavior in both expert ratings of attachment behavior and ratings of autism severity, suggesting the role of dyadic factors in these "child" assessments. In the preschool classroom, location/orientation measures provide data-driven measures of children's social contact, fertile ground for vocal interactions. Both the velocity of children's movement toward one another and their social contact with one another evidence homophily: children with autism spectrum disorder, other developmental disabilities, and typically developing children were more likely to interact with children in the same group even in inclusive preschool classrooms designed to promote interchange between all children. In the vocal domain, the frequency of peer speech and the phonemic complexity of teacher speech predict the frequency and phonemic complexity of children's own speech over multiple timescales. Moreover, children's own speech predicts their assessed language abilities across disability groups, suggesting how everyday interactions facilitate development.

Clinical presentation of delayed hemolytic transfusion reactions and hyperhemolysis in sickle cell disease.

Red blood cell (RBC) transfusion therapy is a key component in the comprehensive management of patients with sickle cell disease (SCD). Consequently, most adult SCD patients will receive at least one, and many will receive more than a hundred RBC transfusions in their lifetime. SCD patients develop RBC alloantibodies much more frequently than non-SCD transfused patients, which often make the selection of compatible RBCs extremely difficult, in addition to placing patients at significantly higher risk of suffering from delayed hemolytic transfusion reactions (DHTRs). Similar to alloimunization, DHTRs are much more common in patients with SCD compared to other heavily transfused populations, and are particularly consequential due to their propensity to cause hyperhemolysis, a life-threatening phenomenon in which both transfused RBCs in addition to the patient's own sickle-erythrocytes are destroyed. In this review, we highlight the incidence and pathophysiology of DHTRs; illustrate common presentations, appropriate evaluations and outcomes of DHTRs in patients with SCD; and discuss strategies for preventing or reducing the likelihood of DHTRs from occurring.

Transfusion service knowledge and immunohaematological practices related to sickle cell disease and thalassemia.

OBJECTIVES: To assess current knowledge of National Heart, Lung and Blood Institutes (NHLBI) and Thalassemia International Federation (TIF) recommendations, blood banking practices and perceived challenges among transfusion services in the management of patients with haemoglobinopathies. BACKGROUND: Previous reports have demonstrated variations in transfusion practices for sickle cell disease (SCD) and thalassemia patients. Recently, NHLBI/TIF have provided transfusion recommendations for patients with haemoglobinopathies. METHODS: A cross-sectional survey was conducted of transfusion services from the state of Georgia previously identified as having SCD/thalassemia populations. The survey assessed transfusion service practices in pre-transfusion testing and blood product selection; awareness/implementation of NHLBI/TIF transfusion-based recommendations and perceived challenges in transfusing haemoglobinopathy patients. RESULTS: Responses were received from 35 of 49 (71%) institutions. Only institutions indicating transfusing SCD or thalassemia patients (32) were included in analysis. Seventy-one percent of non-sickle cell treatment centres (SCTCs) and 20% of non-thalassemia treatment centres follow NHLBI and TIF recommendations to perform a red blood cell phenotype beyond ABO/Rh(D) and provide Rh and Kell prophylactically matched units for SCD and thalassemia patients, respectively. Forty percent of institutions (33% of non-SCTCs) employ RBC genotyping to evaluate the red cell phenotype for SCD patients. Over 77% of institutions do not utilise a reliable method to identify SCD patients prior to transfusion, such as a required question/answer field on type/screen or crossmatch orders. CONCLUSION: Many healthcare systems' transfusion practices for haemoglobinopathy patients are discordant with NHLBI/TIF recommendations. Efforts are needed to increase awareness and implementation of current recommendations among all transfusion services seeing these patients.