Purpose Enuresis and sleep disordered breathing are common among children with sickle cell anemia. We evaluated whether enuresis is associated with sleep disordered breathing in children with sickle cell anemia. Materials and Methods Baseline data were used from a multicenter prospective cohort study of 221 unselected children with sickle cell anemia. A questionnaire was used to evaluate, by parental report during the previous month, the presence of enuresis and its severity. Overnight polysomnography was used to determine the presence of sleep disordered breathing by the number of obstructive apneas and/or hypopneas per hour of sleep. Logistic and ordinal regression models were used to evaluate the association of sleep disordered breathing and enuresis. Results The mean age of participants was 10.1 years (median 10.0, range 4 to 19). Enuresis occurred in 38.9% of participants and was significantly associated with an obstructive apnea-hypopnea index of 2 or more per hour after adjusting for age and gender (OR 2.19; 95% CI 1.09, 4.40; p = 0.03). Enuresis severity was associated with obstructive apneas and hypopneas with 3% or more desaturation 2 or more times per hour with and without habitual snoring (OR 3.23; 95% CI 1.53, 6.81; p = 0.001 and OR 2.07; 95% CI 1.09, 3.92; p = 0.03, respectively). Conclusions In this unselected group of children with sickle cell anemia, sleep disordered breathing was associated with enuresis. Results of this study support that children with sickle cell anemia who present with enuresis should be evaluated by a pulmonologist for sleep disordered breathing.

Background: Pre-mature birth impacts left ventricular development, predisposing this population to long-term cardiovascular risk. The aims of this study were to investigate maturational changes in rotational properties from the neonatal period through 1 year of age and to discern the impact of cardiopulmonary complications of pre-maturity on these measures. Methods: Pre-term infants (<29 weeks at birth, n = 117) were prospectively enrolled and followed to 1-year corrected age. Left ventricular basal and apical rotation, twist, and torsion were measured by two-dimensional speckle-tracking echocardiography and analysed at 32 and 36 weeks post-menstrual age and 1-year corrected age. A mixed random effects model with repeated measures analysis was used to compare rotational mechanics over time. Torsion was compared in infants with and without complications of cardiopulmonary diseases of pre-maturity, specifically bronchopulmonary dysplasia, pulmonary hypertension, and patent ductus arteriosus. Results: Torsion decreased from 32 weeks post-menstrual age to 1-year corrected age in all pre-term infants (p < 0.001). The decline from 32 to 36 weeks post-menstrual age was more pronounced in infants with cardiopulmonary complications, but was similar to healthy pre-term infants from 36 weeks post-menstrual age to 1-year corrected age. The decline was due to directional and magnitude changes in apical rotation over time (p < 0.05). Conclusion: This study tracks maturational patterns of rotational mechanics in pre-term infants and reveals torsion declines from the neonatal period through 1 year. Cardiopulmonary diseases of pre-maturity may negatively impact rotational mechanics during the neonatal period, but the myocardium recovers by 1-year corrected age.

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Prior studies have demonstrated higher odds of mortality for non-white and lower socioeconomic status patients with single ventricle (SV) heart disease. However, these studies have not examined survival through staged surgical palliation or rates of morbidity in this patient population. We hypothesized that non-white race/ethnicity and lower socioeconomic status were associated with lower overall survival and increased morbidity through 1 st birthday in patients undergoing SV palliation. Patients with SV heart disease, live birth, and planned SV palliation in the National Pediatric Cardiology Quality Improvement Collaborative between January 1, 2016, and December 31, 2020, were included. Race/ethnicity, payer, and neighborhood characteristics were analyzed. Morbidity was defined as acute neurological events, unplanned procedures, renal support, cardiac arrest, extracorporeal life support, necrotizing enterocolitis, or surgical site infection. Morbidity-free survival was defined as survival through each stage of palliation without experiencing one of the above morbidities. Cox proportional hazards and multivariable regression modeling were performed to evaluate the association between socioeconomic variables, race/ethnicity, and overall morbidity-free survival to 1st birthday. There were 2,184 live births with an overall survival to 1st birthday of 76%. There was no difference in survival to 1st birthday between non-Hispanic White, non-Hispanic Black, and Hispanic patients. Patients with government insurance had decreased survival prior to their 1st birthday (HR 0.79, 95% CI 0.64, 0.98 vs commercial insurance). Infants with government insurance had lower morbidity-free survival during stage 1 (OR 0.72, 95% CI 0.59, 0.87) and stage 2 (OR 0.67, 95% CI 0.52, 0.85) hospitalizations. Patients with “Other” race/ethnicity had lower odds of morbidity-free survival to one year of age (OR 0.68 95% CI 0.47, 0.98). Household income and deprivation index were not associated with survival or morbidity. Infants with government insurance experienced decreased survival to 1st birthday, and those of “Other” race/ethnicity had lower morbidity-free survival. Addressing disparities in these patient populations is an area of ongoing need.