Background Cerebral arteriopathies, including an idiopathic focal cerebral arteriopathy of childhood (FCA), are common in children with arterial ischemic stroke (AIS) and strongly predictive of recurrence. To better understand these lesions, we measured predictors of arteriopathy within a large international series of children with AIS. Methods and Results Between 1/2003 and 7/2007, 30 centers within the International Pediatric Stroke Study (IPSS) enrolled 667 children (29 days-19 years of age) with AIS and abstracted clinical and radiographic data. Cerebral arteriopathy and its subtypes were defined using published definitions; FCA was defined as cerebral arterial stenosis not attributed to specific diagnoses such as moyamoya, arterial dissection, vasculitis, or post-varicella angiopathy. We used multivariate logistic regression techniques to determine predictors of arteriopathy and FCA among those subjects who received vascular imaging. The authors had full access to and take full responsibility for the integrity of the data. All authors have read and agree to the manuscript as written. Of 667 subjects, 525 had known vascular imaging results, and 53% of those (n=277) had an arteriopathy. The most common arteriopathies were FCA (n=69, 25%), moyamoya (n=61, 22%), and arterial dissection (n=56; 20%). Predictors of arteriopathy include early school age (5-9 years), recent upper respiratory infections (URI), and sickle cell disease, while prior cardiac disease and sepsis reduced the risk of arteriopathy. The only predictor of FCA was recent URI. Conclusions Arteriopathy is prevalent among children with AIS, particularly those presenting in early school age, and those with a history of sickle cell disease. Recent URI predicted cerebral arteriopathy, and FCA in particular, suggesting a possible role for infection in the pathogenesis of these lesions.

Objective Children undergoing cardiac surgery with cardiopulmonary bypass (CPB) are susceptible to additional inflammatory and immunogenic insults from blood transfusions. We hypothesize that washing red blood cells (RBC) and platelets transfused to these patients will reduce post-operative transfusion-related immune modulation and inflammation. Design Prospective randomized controlled clinical trial. Setting University hospital pediatric cardiac intensive care unit. Patients Children from birth to 17 years old undergoing cardiac surgery with CPB. Interventions Children were randomized to an unwashed or washed RBC and platelet transfusion protocol for their surgery and postoperative care. All blood was leukoreduced, irradiated, and ABO identical. Plasma was obtained for laboratory analysis: pre-op, immediately, six and 12 hours after CPB. Primary outcome was the 12-hour post-CPB interleukin (IL)-6: IL-10 ratio. Secondary measures were IL levels, C-reactive protein (CRP), and clinical outcomes. Measurements and main results 162 subjects were studied, 81 per group. 34 subjects (17 per group) did not receive any blood transfusions. Storage duration of blood products was similar between groups. Among transfused subjects, the 12-hour IL ratio was significantly lower in the washed group (3.8 v. 4.8; p=0.04) secondary to lower IL-6 levels (post-CPB: 65 v.100 pg/ml; p = 0.06; 6 hour: 89 v.152 pg/ml; p = 0.02; 12-hour: 84 v.122 pg/ml; p = 0.09). Post-operative CRP was lower in subjects receiving washed blood (38 v. 43 mg/L; p = 0.03). There was a numerical, but not statistically significant decrease in total blood product transfusions (203 v. 260) and mortality (2 v. 6 deaths) in the washed group compared to the unwashed group. Conclusions Washed blood transfusions in cardiac surgery reduced inflammatory biomarkers, number of transfusions, donor exposures, and were associated with a non-significant trend towards reduced mortality. A larger study powered to test for clinical outcomes is needed to determine whether these laboratory findings are clinically significant.

Children with single-ventricle physiology do not benefit from a liberal transfusion strategy after cavopulmonary connection. A restrictive red blood cell transfusion strategy decreases the number of transfusions, donor exposures, and potential risks in these children. Larger studies with clinical outcome measures are needed to determine the transfusion threshold for children post cardiac repair or palliation for congenital heart disease.