A multi-disciplinary, comprehensive approach to management of children with heterotaxy.

Heterotaxy (HTX) is a rare condition of abnormal thoraco-abdominal organ arrangement across the left-right axis of the body. The pathogenesis of HTX includes a derangement of the complex signaling at the left-right organizer early in embryogenesis involving motile and non-motile cilia. It can be inherited as a single-gene disorder, a phenotypic feature of a known genetic syndrome or without any clear genetic etiology. Most patients with HTX have complex cardiovascular malformations requiring surgical intervention. Surgical risks are relatively high due to several serious comorbidities often seen in patients with HTX. Asplenia or functional hyposplenism significantly increase the risk for sepsis and therefore require antimicrobial prophylaxis and immediate medical attention with fever. Intestinal rotation abnormalities are common among patients with HTX, although volvulus is rare and surgical correction carries substantial risk. While routine screening for intestinal malrotation is not recommended, providers and families should promptly address symptoms concerning for volvulus and biliary atresia, another serious morbidity more common among patients with HTX. Many patients with HTX have chronic lung disease and should be screened for primary ciliary dyskinesia, a condition of respiratory cilia impairment leading to bronchiectasis. Mental health and neurodevelopmental conditions need to be carefully considered among this population of patients living with a substantial medical burden. Optimal care of children with HTX requires a cohesive team of primary care providers and experienced subspecialists collaborating to provide compassionate, standardized and evidence-based care. In this statement, subspecialty experts experienced in HTX care and research collaborated to provide expert- and evidence-based suggestions addressing the numerous medical issues affecting children living with HTX.

Feasibility of Assessing Adolescent and Young Adult Heart Transplant Recipient Mental Health and Resilience Using Patient-Reported Outcome Measures.

BACKGROUND: Although adolescents and young adults may be particularly prone to mental health symptoms after heart transplant, screening practices are variable. OBJECTIVE: To assess the feasibility of using patient-reported outcome (PRO) measures to assess mental health, functional status, and resiliency in posttransplant adolescents and young adult patients. METHODS: Patients transplanted between ages 15 and 25 years at 3 centers completed 6 PRO instruments via web-based platforms: PROMIS instruments for anxiety, depression, satisfaction with social roles, and physical functioning; the Posttraumatic Stress Diagnostic Scale for Diagnostic and Statistical Manual of Mental Disorders, version 5; and the Connor-Davidson Resilience Scale-10. Feasibility (completion, time to completion, and measure missingness) and PRO results were described and compared between patients with congenital heart disease and cardiomyopathy. RESULTS: Nineteen patients (median age at transplant 17.7 y [interquartile range 16.3, 19.2 y], 84% male) were enrolled at an average of 3 ± 1.8 years after transplant. Enrollment was 90% among eligible patients. Measure missingness was zero. The average completion time was 12 ± 15 minutes for all instruments. Timely PRO completion was facilitated by in-clinic application. The PRO results indicated that 9 patients (47%) had at least mild posttraumatic stress disorder symptoms (≥11 points on Posttraumatic Stress Diagnostic Scale for Diagnostic and Statistical Manual of Mental Disorders, version 5). Among them, 4 patients had scores >28 suggestive of probable posttraumatic stress disorder. Two (11%) and 6 (32%) patients had anxious and depressive symptoms, respectively. The cardiomyopathy cohort had a higher median Posttraumatic Stress Diagnostic Scale for Diagnostic and Statistical Manual of Mental Disorders, version 5 score than that of the congenital heart disease subgroup (11.0 vs 6.0; P = 0.015). Twelve (63%) had resiliency scores that were lower than the population average. No significant differences were found in PRO results between patients with cardiomyopathy and congenital heart disease apart from the posttraumatic stress disorder assessment. CONCLUSIONS: This novel PRO-based approach to psychiatric screening of adolescents and young adult patients after transplant appears feasible for assessing mental health, functional status, and resiliency, with excellent enrollment and completion rates. These instruments characterized the burden of mental health symptoms within this adolescents and young adult heart transplant cohort, with a high prevalence of posttraumatic stress disorder symptoms. Resiliency scores were lower than in a comparison population. Electronically-administered PRO administration could facilitate more consistent mental health screening in this at-risk group.

Avoidant/Restrictive Food Intake Disorder in Diet-treated Children With Eosinophilic Esophagitis.

Eosinophilic esophagitis (EoE) is an inflammatory condition of the esophagus with rising incidence in children. Owed to potential adverse effects and high costs of EoE medications, strict elimination diets are often employed as a mainstay of long-term EoE therapy in children. Currently, there are no effective tests to pinpoint food protein triggers in children with EoE. Therefore, EoE elimination diets are often broad (including milk, soy, wheat, egg, fish/shellfish, and nuts) and can greatly alter a child's baseline eating habits. Herein, we describe 2 cases of avoidant/restrictive food intake disorder (ARFID) in children with remitted EoE maintained on an elimination diet. We also present comorbidity data on ARFID and diet-treated EoE from our pediatric EoE clinic. This is the first report of disordered eating associated with EoE therapy. As EoE is becoming more common, close monitoring of intake and growth in patients treated with elimination diets will be key.

Fathers of children with cancer: involvement, coping, and adjustment.

INTRODUCTION: This study examined the role of fathers caring for children with cancer. Psychological adjustment, coping, and work patterns of mothers and fathers were described. METHOD: Twenty fathers of children with cancer were compared with 20 mothers of children with cancer and 20 control fathers of healthy children. Questionnaire data were collected regarding coping, parental adjustment, child adjustment, and family involvement. RESULTS: Fathers did not differ from mothers or control fathers in terms of psychological adjustment or coping. However, fathers of children with cancer spent more hours at work and more hours caring for children than did control fathers. Paternal adjustment was significantly related to child adjustment only when the child had cancer. Coping was related to work outside the home for fathers and adjustment for mothers. DISCUSSION: Models of family adaptation may be different for fathers and mothers. Treatment teams must attend to the unique needs of fathers.

Sensory modality, temperament, and the development of sustained attention: a vigilance study in children and adults.

Applying optimal stimulation theory, the present study explored the development of sustained attention as a dynamic process. It examined the interaction of modality and temperament over time in children and adults. Second-grade children and college-aged adults performed auditory and visual vigilance tasks. Using the Carey temperament questionnaires (S. C. McDevitt & W. B. Carey, 1995), the authors classified participants according to temperament composites of reactivity and task orientation. In a preliminary study, tasks were equated across age and modality using d' matching procedures. In the main experiment, 48 children and 48 adults performed these calibrated tasks. The auditory task proved more difficult for both children and adults. Intermodal relations changed with age: Performance across modality was significantly correlated for children but not for adults. Although temperament did not significantly predict performance in adults, it did for children. The temperament effects observed in children--specifically in those with the composite of reactivity--occurred in connection with the auditory task and in a manner consistent with theoretical predictions derived from optimal stimulation theory.

Equating tasks and sustaining attention in children and adults: the methodological and theoretical utility of d' matching.

The present study develops a method to create task equivalency for children and adults in vigilance research. Experiment 1 employed the signal detection index of perceptual sensitivity (d') to calibrate vigilance tasks for 32 children (ages 7-8) and 32 adults. The results of Experiment 1 suggest that task equivalency across groups can be attained, and a vigilance decrement was observed for children. In Experiment 2, the task parameter of event rate was manipulated across two vigils. Again, 32 children (ages 7-8) and 32 adults participated. The results suggest that a reverse event rate effect emerged for both children and adults. In addition, developmental differences in response to the event rate manipulation and with respect to the vigilance decrement emerged: Children benefited less from the manipulation and were more likely to demonstrate a vigilance decrement than were adults. No developmental differences emerged for the sensitivity decrement. The results are explained with reference to how task demands interact with participant arousal, and implications for development and for future research are discussed.