Patient-Reported Outcomes and Medical Provider Satisfaction Among Adult and Pediatric Ataxia-Telangiectasia Patients.

PURPOSE: Ataxia-telangiectasia (A-T) is a rare genetic condition with malfunctioning DNA repair processes resulting in significant clinical findings, including progressive neurologic decline, elevated malignancy risk, immunodeficiency, oculocutaneous telangiectasias, and severe pulmonary disease. Research has been limited into the quality of life of such patients and yet to be completed are studies quantitatively analyzing psychosocial, physical, and cognitive patient-reported outcomes (PROs) within the A-T population. METHODS: PRO evaluations of 90 international adult and pediatric A-T patients and their caregivers were completed via secure online administration of Patient-Reported Outcomes Measurement Information System (PROMIS) short forms evaluating anger, cognition, mood, social health, fatigue, pain, anxiety, and upper extremity function. The impact of age, gender, race/ethnicity, prior malignancy diagnosis, and current supportive treatment interventions on such PROs was additionally assessed. Finally, given the importance of medical providers in the care of A-T patients and the impact of patient satisfaction on healthcare outcomes, we further analyzed, via a novel survey, how patients and caregivers perceived their primary A-T healthcare provider's A-T expertise, trustworthiness, accessibility, and level of compassion. RESULTS/CONCLUSION: It was found that a diagnosis of A-T complexly impacts patient PROs, but such data offers the potential for preventative and therapeutic interventions to improve the care of such patients. While most A-T patients and their caregivers feel their primary A-T medical provider has expertise and compassion in addition to being accessible and trustworthy, a significant percentage of study subjects did not agree that their provider was an expert in A-T or overall trustworthy.

Changes over time in self-efficacy and the allocation of responsibility for health management tasks in pediatric liver transplant recipients: Targets to improve the transition process.

BACKGROUND: The process of transition to adult-based care encompasses a critical period in the life of an adolescent and young adult living with a chronic illness and one that comes with an increase in the risk of poor health outcomes. As yet, there is a dearth of empirical data to help optimize this process to ensure the best long-term outcome. METHODS: This study used a principal components analysis to determine specific constructs measured by a revised version of the transition readiness survey used in our clinic. We investigated changes in these constructs over time. We further investigated the relationship between the change in these constructs over time spent in a focused transition program with adherence. RESULTS: The primary component underlying our transition readiness survey for patients and parents represented self-efficacy. Time spent in the transition program was an independent predictor of change in self-efficacy (rho 0.299, p = .015); however, the magnitude of that change had no relationship to adherence. Change in parent-proxy self-efficacy was found to have a statistically significant relationship with tacrolimus standard deviation (rho -0.301, p = .026). There was disagreement identified between patient and parent responses on the survey. Neither change in patient nor parent reports of self-efficacy was found to have a relationship with post-transfer adherence. CONCLUSIONS: This study reaches the novel conclusion that self-efficacy and parent-proxy self-efficacy are dynamic concepts that change over time spent in a focused transition program. The patient-parent disagreement and the relationship between parent-proxy self-efficacy and adherence stress the importance of involving parents/guardians in the transition process as well.

Adolescent eating disorder treatment outcomes of an in-person partial hospital program versus a virtual intensive outpatient program.

OBJECTIVE: Though virtual outpatient psychotherapy for eating disorders is likely effective, less is known about virtual higher levels of care. The current study examined the clinical outcomes of a family-based virtual intensive outpatient program (vIOP) for youth with eating disorders which was developed in response to the COVID-19 pandemic, compared to the same institution's in-person partial hospital program (PHP). METHODS: Treatment outcomes were assessed via chart review in 102 patients between the ages of 9-23 (M = 15.2, SD = 2.5) who were predominantly cisgender female (84.3%) and primarily diagnosed with anorexia nervosa (64.7%) or atypical anorexia (23.5%). Participants were either treated in the in-person PHP before the pandemic (n = 49) or the vIOP during the pandemic (n = 53). Percent expected body weight (%EBW) was examined at baseline, end of treatment, 3-months post-treatment, and 6-months post-treatment, as well as the frequency of medical, psychiatric, and residential admissions before, during, and after vIOP or PHP participation. RESULTS: Linear mixed models demonstrated no effect of treatment modality (in-person versus virtual) on %EBW over time. The duration of the vIOP was, on average, 12 calendar days longer, though the amount billed for the vIOP was lower. Survival analyses and Cox regression models did not suggest differences in the frequency of hospital and residential treatment admissions during treatment (vIOP: 9.4%, PHP: 10.0%) or post-treatment (vIOP: 15.0%, PHP: 10.2%). DISCUSSION: Findings support virtual family-based programs as suitable alternatives to in-person treatment and underscore the potential cost-effectiveness of a family-based IOP versus PHP. PUBLIC SIGNIFICANCE: This study demonstrates that a virtual, family-based, intensive outpatient program for youth with eating disorders had similar treatment outcomes to an in-person partial hospitalization program. Specifically, the virtual and in-person programs had similar weight restoration outcomes and rates of medical, psychiatric, or residential treatment admissions during or after treatment initiation. Findings support the use of virtual treatment, even for youth requiring a high level of intervention.

Characterization of a Vigorous sucking style in early infancy and its predictive value for weight gain and eating behaviors at 12 months.

This study sought to identify sucking profiles among healthy, full-term infants and assess their predictive value for future weight gain and eating behaviors. Pressure waves of infant sucking were captured during a typical feeding at age 4 months and quantified via 14 metrics. Anthropometry was measured at 4 and 12 months, and eating behaviors were measured by parent report via the Children's Eating Behavior Questionnaire-Toddler (CEBQ-T) at 12 months. Sucking profiles were created using a clustering approach on the pressure wave metrics, and utility of these profiles was assessed for predicting which infants will have weight-for-age (WFA) percentile changes from ages 4-12 months that exceed thresholds of 5, 10, and 15 percentiles, and for estimating each CEBQ-T subscale score. Among 114 infants, three sucking profiles were identified: Vigorous (51%), Capable (28%), and Leisurely (21%). Sucking profiles were found to improve estimation of change in WFA from 4 to 12 months and 12-month maternal-reported eating behaviors above infant sex, race/ethnicity, birthweight, gestational age, and pre-pregnancy body mass index alone. Infants with a Vigorous sucking profile gained significantly more weight during the study period than infants with a Leisurely profile. Infant sucking characteristics may aid in predicting which infants may be at greater risk of obesity, and therefore sucking profiles deserve more investigation.

Self-Regulation as a Protective Factor for Diabetes Distress and Adherence in Youth with Type 1 Diabetes During the COVID-19 Pandemic.

OBJECTIVE: The COVID-19 pandemic increased economic, social, and health stressors for families, yet its impacts on families of youth with chronic conditions, such as type 1 diabetes (T1D), are not well understood. Self-regulation (SR)-or the capacities to control emotions, cognition, and behavior in response to challenge-is known to support T1D management and coping in the face of stress. Strong SR may have protected youth with T1D from the impacts of pandemic-related stressors. This study compared youth and parent emotional functioning and T1D management before and after the pandemic's onset in relation to family pandemic-related stress and youth SR. METHODS: Parents of youth with T1D (N = 88) and a subset of these youth (N = 43; Mean age 15.3 years [SD 2.2]) completed surveys regarding SR, stress, emotional functioning, and T1D-related functioning prior to and after March 2020. Outcomes were compared using mixed effects models adjusting for covariates. Family pandemic-related stress experiences and youth SR were tested as moderators of change. RESULTS: Parents' responsibility for T1D management increased across pandemic onset and their diabetes-related distress decreased. Family pandemic-related stress was associated with decreased emotional functioning over time. Youth SR, particularly emotional and behavioral aspects, predicted better emotional and T1D-related functioning. DISCUSSION: While youth with T1D whose families experienced higher pandemic-related stress had poorer adjustment, strong emotional and behavioral SR appeared to protect against worsening youth mood and adherence across pandemic onset. Both social-contextual and individual factors are important to consider when working with families managing T1D.

Supporting family meal frequency: Screening Phase results from the Simply Dinner Study.

We aimed to test main, additive, interactive effects, and feasibility of all possible combinations of six intervention components implemented for 8 weeks (Cooking/Serving Resources; Meal Delivery; Ingredient Delivery; Community Kitchen; Nutrition Education; Cooking Demonstrations). Primary outcomes were family meal frequency and preschoolers' dietary quality; secondary outcomes included family meal preparation type, meal preparation barriers, family functioning, and kitchen inventory adequacy. All possible intervention combinations were tested using a randomized factorial trial design in the first phase of a Multiphase Optimization Strategy (MOST). Feasibility was assessed via attendance, delivery logs, and satisfaction. Parent-reported data collection included: socio-demographics, frequency and type of family meals; preschooler dietary intake; perceived barriers to meal planning and preparation; assessment of family functioning; and a kitchen inventory of materials generally needed for meal preparation. Participants (n = 499) were recruited at two Head Start agencies in mid-Michigan with data collection and delivery of some intervention components in participants' homes. Promising intervention bundles were identified by evaluating pre-to post-intervention effect sizes. The combination of Cooking/Serving Resources and Meal Delivery increased family meal frequency (Cohen's d = 0.17), cooking dinner from scratch (d = 0.21), prioritization of family meals (d = 0.23), and kitchen inventory (d = 0.46) and decreased use/consumption of ready-made (d = -0.18) and fast foods (d = -0.23). Effects on diet quality were in the expected direction but effect sizes were negligible. Community Kitchen, Nutrition Education, and Cooking Demonstration showed poor feasibility due to low attendance while Ingredient Delivery was infeasible due to staffing challenges related to its labor intensity. Additionally, although not one of our pre-specified outcomes, Cooking/Serving Resources (RR = 0.74) and Meal Delivery (RR = 0.73) each decreased food insecurity. Cooking/Serving Resources combined with Meal Delivery showed promise as a strategy for increasing family meal frequency.

Early-life epilepsy after acute symptomatic neonatal seizures: A prospective multicenter study.

OBJECTIVE: We aimed to evaluate early-life epilepsy incidence, seizure types, severity, risk factors, and treatments among survivors of acute neonatal seizures. METHODS: Neonates with acute symptomatic seizures born 7/2015-3/2018 were prospectively enrolled at nine Neonatal Seizure Registry sites. One-hour EEG was recorded at age three months. Post-neonatal epilepsy and functional development (Warner Initial Developmental Evaluation of Adaptive and Functional Skills - WIDEA-FS) were assessed. Cox regression was used to assess epilepsy-free survival. RESULTS: Among 282 infants, 37 (13%) had post-neonatal epilepsy by 24-months [median age of onset 7-months (IQR 3-14)]. Among those with post-neonatal epilepsy, 13/37 (35%) had infantile spasms and 12/37 (32%) had drug-resistant epilepsy. Most children with post-neonatal epilepsy had abnormal neurodevelopment at 24-months (WIDEA-FS >2SD below normal population mean for 81% of children with epilepsy vs 27% without epilepsy, RR 7.9, 95% CI 3.6-17.3). Infants with severely abnormal neonatal EEG background patterns were more likely to develop epilepsy than those with mild/moderate abnormalities (HR 3.7, 95% CI 1.9-5.9). Neonatal EEG with ≥3 days of seizures also predicted hazard of epilepsy (HR 2.9, 95% CI 1.4-5.9). In an adjusted model, days of neonatal EEG-confirmed seizures (HR 1.4 per day, 95% CI 1.2-1.6) and abnormal discharge examination (HR 3.9, 95% CI 1.9-7.8) were independently associated with time to epilepsy onset. Abnormal (vs. normal) three-month EEG was not associated with epilepsy. SIGNIFICANCE: In this multicenter study, only 13% of infants with acute symptomatic neonatal seizures developed post-neonatal epilepsy by age 24-months. However, there was a high risk of severe neurodevelopmental impairment and drug-resistant seizures among children with post-neonatal epilepsy. Days of EEG-confirmed neonatal seizures was a potentially modifiable epilepsy risk factor. An EEG at three months was not clinically useful for predicting epilepsy. These practice changing findings have implications for family counseling, clinical follow-up planning, and future research to prevent post-neonatal epilepsy.

Burnout, professional fulfillment, and post-traumatic stress among pediatric solid organ transplant teams.

BACKGROUND: Adverse effects of clinician burnout have been studied across multiple specialties; however, there have been no studies examining rates of burnout among pediatric solid organ transplant teams. This study aimed to measure burnout, work exhaustion, professional fulfillment, and post-traumatic stress symptoms among clinicians and administrators practicing in this high-stress field. METHODS: This cross-sectional study utilized a 50 item web-based survey that included the Personal Fulfillment Index and the IES-R. This survey was distributed across four pediatric solid organ transplant centers in North America. Basic demographics, clinician characteristics, and information regarding wellness and self-care activities were collected. Descriptive and correlational analyses were performed. RESULTS: One hundred and thirty five participants completed the survey, 76% were female and 78% were Caucasian. One-third (34%) of participants endorsed burnout, while 43% reported professional fulfillment. Approximately 15% of respondents endorsed clinically significant levels of post-traumatic stress symptoms related to patient deaths, with female clinicians more likely to endorse symptoms (p = .01). Nearly 80% of participants reported engaging in self-care activities outside of work and only 10% of participants reported participation in hospital-sponsored wellness programs. CONCLUSIONS: Pediatric solid organ transplant team members exhibited moderate levels of burnout, professional fulfillment, and post-traumatic stress. Female clinicians were the most likely to experience both work exhaustion and post-traumatic stress symptoms. Transplant centers are encouraged to consider interventions and programming to improve clinician wellness.

Rural-urban differences in body mass index and obesity-related behaviors among low-income preschoolers.

BACKGROUND: With one in eight preschoolers classified as obese in the USA, childhood obesity remains a significant public health issue. This study examined rural-urban differences in low-income preschoolers' body mass index z-scores (BMIz), eating behaviors, dietary quality, physical activity (PA) and screen time. METHODS: Pre-intervention data from 572 preschooler-parent dyads participating in a randomized, controlled obesity prevention trial in the Midwest USA were analyzed. We examined the associations among living in rural versus urban areas, child BMIz and child obesity-related behaviors, including eating behaviors, dietary quality, PA and screen time. RESULTS: Rural children had higher BMIz, more emotional overeating behaviors and more time spent playing outdoors compared with urban children. We found no associations between children living in rural versus urban areas and dietary quality and screen time. CONCLUSIONS: The study found that rural-urban differences in BMIz may start as early as 3-4 years of age, if not earlier. To reverse the weight-related health disparities between rural and urban low-income preschoolers, structural changes in rural locations and family supports around coping skills may be needed.

Targeted self-regulation interventions in low-income children: Clinical trial results and implications for health behavior change.

Self-regulation, known as the ability to harness cognitive, emotional, and motivational resources to achieve goals, is hypothesized to contribute to health behaviors across the lifespan. Enhancing self-regulation early in life may increase positive health outcomes. During pre-adolescence, children assume increased autonomy in health behaviors (e.g., eating; physical activity), many of which involve self-regulation. This article presents results from a clinical trial (NCT03060863) that used a factorial design to test behavioral interventions designed to enhance self-regulation, specifically targeting executive functioning, emotion regulation, future-oriented thinking, and approach biases. Participants were 118 children (9-12 years of age, M = 10.2 years) who had a history of living in poverty. They were randomized to receive up to four interventions that were delivered via home visits. Self-regulation was assayed using behavioral tasks, observations, interviews, and parent- and child-report surveys. Results were that self-regulation targets were reliably assessed and that interventions were delivered with high fidelity. Intervention effect sizes were very small to moderate (d range = .02-.65, median = .14), and most were not statistically significant. Intercorrelation analyses indicated that associations between measures within each target varied based on the self-regulation target evaluated. Results are discussed with regard to the role of self-regulation-focused interventions in child health promotion. Implications of findings are reviewed for informing next steps in behavioral self-regulation interventions among children from low-income backgrounds.

Observed behavioral indicators of child satiation at mealtime: Associations with child characteristics and parent-reported child eating behaviors.

The notion of promoting parents' recognition of child satiation to reduce overfeeding and overeating in children is prevalent. To do so, it is important to identify common behaviors that may indicate satiation and can be easily recognized by parents. Relatively little work has focused on identifying behaviors that may indicate child satiation as they occur during naturalistic mealtimes, which is an important context for parents to observe their children's eating behavior. Hence, the goal of the current study is to examine whether observed behavioral indicators of child satiation at mealtimes are associated with child characteristics (i.e., sex, age, and BMIz) and parent-reported child appetitive traits. We coded observed behaviors thought to indicate satiation, specifically mealtime disengagement and mealtime negativity, in a cohort of 240 families with children between 4- and 8-years old (53% boys). First, we documented the occurrence of child disengagement and negativity during naturalistic mealtimes. Second, we found that lower child BMIz and being a boy were associated with greater mealtime disengagement, but child age was not associated with mealtime disengagement. No associations were found between child characteristics and mealtime negativity. Third, we found that mealtime disengagement and mealtime negativity were associated with mother-reported satiety responsiveness on the Child Eating Behavior Questionnaire. Taken together, our findings suggest observed mealtime disengagement and mealtime negativity behaviors could be helpful indicators of child satiation in a naturalistic mealtime context.

Cortisol in early childhood moderates the association between family routines and observed affective balance in children from low-income backgrounds.

The study of emotion regulation often addresses control of negative emotion. Researchers have proposed that affective balance is an indicator of emotion regulation that incorporates the role of positive emotion in the context of negative emotional experiences. Environmental and individual factors, such as family processes and biological stress regulation, are known to shape emotion regulation. The present study investigated whether child diurnal cortisol, an indicator of biological stress regulation, moderated the association between family routines and observed affective balance. Children (N = 222; M age = 4.70 years, SD = 0.60) from low-income households provided saliva samples to measure diurnal cortisol and completed a behavioral task designed to elicit negative emotions. Affective balance was defined as the difference score between the proportion of positive and negative emotional expressions displayed during the task. A higher affective balance score indicated greater positive compared with negative emotional displays. Simple slope analyses indicated that for children with a low morning cortisol intercept, more frequent family routines were associated with more affective balance. This pattern was not observed in children with average or high morning cortisol. Positive family routines may play an important role in shaping affective balance among children with disrupted cortisol levels from low-income backgrounds.

Clinical Features and Biomarkers Suggestive of Bloodstream Infection in Pediatric Patients With Intestinal Failure and Central Venous Lines for Parenteral Nutrition: A Retrospective Pilot Study.

OBJECTIVES: This study aimed to describe demographic, clinical, and laboratory values in febrile pediatric intestinal failure patients with indwelling central venous catheters presenting to the emergency department to determine if there are low-risk features predictive of negative blood cultures that could help identify a subset that do not need hospitalization and/or broad-spectrum antibiotics. METHODS: We conducted a retrospective cohort study of febrile patients younger than 18 years with diagnosis of intestinal failure requiring a central venous catheter for home parenteral nutrition to identify factors associated with normal blood cultures to identify a low-risk subset. RESULTS: Of 119 encounters, 46% had a bloodstream infection. Children with a bloodstream infection were older than those without. A normal lactate and heart rate were associated with negative blood cultures. CONCLUSION: Pediatric intestinal failure patients with a central venous catheter for home parenteral nutrition presenting to the emergency department with fever and found to have a normal lactate and heart rates had lower rates of bloodstream infection. Although we were unable to create a highly sensitive clinical decision-making rule to identify a low-risk cohort because of the low number of patients meeting both criteria, the promising candidate variables identified merit for future multicenter studies.

Clinician Accuracy in Identifying and Predicting Organ Dysfunction in Critically Ill Children.

OBJECTIVES: To determine clinician accuracy in the identification and prediction of multiple organ dysfunction syndrome. DESIGN: Prospective cohort study. SETTING: University of Michigan's C.S. Mott Children's Hospital PICU. PATIENTS: Patients admitted to the PICU with an anticipated PICU length of stay greater than 48 hours. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: For each patient, the clinical team (attending, fellow, resident/nurse practitioner) was surveyed regarding existing and anticipated organ dysfunction. The primary outcomes were clinicians' accuracy at identifying multiple organ dysfunction syndrome and predicting new or progressive multiple organ dysfunction syndrome, compared to the objective assessment of multiple organ dysfunction syndrome using Proulx criteria. We also measured sensitivity, specificity, negative and positive predictive values, and negative and positive likelihood ratios of clinician assessments. We tested for differences in accuracy by clinician type using chi-square tests. Clinicians rated their confidence in prediction on a 5-point Likert scale. There were 476 eligible PICU admissions, for whom 1,218 surveys were completed. Multiple organ dysfunction syndrome was present in 89 patients (18.7%) at enrollment, and new or progressive multiple organ dysfunction syndrome occurred in 39 (8.2%). Clinicians correctly identified multiple organ dysfunction syndrome with 79.9% accuracy and predicted additional organ dysfunction with 82.6% accuracy. However, the positive and negative likelihood ratios for new or progressive multiple organ dysfunction syndrome prediction were 3.0 and 0.7, respectively, indicating a weak relationship between the clinician prediction and development of new or progressive multiple organ dysfunction syndrome. The positive predictive value of new or progressive multiple organ dysfunction syndrome prediction was just 22.1%. We found no differences in accuracy by clinician type for either identification of multiple organ dysfunction syndrome (80.2% vs 78.2% vs 81.0%; p = 0.57) or prediction of new or progressive multiple organ dysfunction syndrome (84.8% vs 82.8% vs 80.3%; p = 0.26) for attendings, fellows, and residents/nurse practitioners, respectively. There was a weak correlation between the confidence and accuracy of prediction (pairwise correlation coefficient, 0.26; p < 0.001). CONCLUSIONS: PICU clinicians correctly identified multiple organ dysfunction syndrome and predicted new or progressive multiple organ dysfunction syndrome with 80% accuracy. However, only 8% of patients developed new or progressive multiple organ dysfunction syndrome, so accuracy was largely due to true negative predictions. The positive predictive value for new or progressive multiple organ dysfunction syndrome prediction was just 22%. Accuracy did not differ by clinician type, but was correlated with self-rated confidence and was higher for negative predictions.

Longitudinal associations between overweight/obesity and stress biology in low-income children.

BACKGROUND/OBJECTIVES: Associations between overweight and altered stress biology have been reported cross-sectionally during childhood, but it is unclear whether overweight precedes altered stress biology or if altered stress biology predicts greater likelihood of overweight over time. The current longitudinal study investigates associations between overweight/obesity, salivary alpha amylase and cortisol morning intercept, diurnal slope, and reactivity to social stress in a cohort of low-income children during preschool and middle childhood. SUBJECTS/METHODS: Children were recruited through Head Start and were observed and followed into middle childhood (N = 257; M = 8.0 years). Height and weight were measured at both time points. Saliva samples were collected across the day and in response to a social challenge at both ages for alpha amylase and cortisol determination. RESULTS: Cross-lagged panel analyses indicated that overweight/obesity at preschool predicted lower morning alpha amylase (ß = -0.18, 95% CI: -0.34, -0.03; p = 0.023), lower morning cortisol (ß = -0.22, 95% CI: -0.38, -0.06; p = 0.006), lower sAA diurnal slope (ß = -0.18, 95% CI: -0.34, -0.03; p = 0.021), and lower cortisol stress reactivity (ß = -0.19, 95% CI: -0.35, -0.02; p = 0.031) in middle childhood. Lower alpha amylase reactivity at preschool was the only biological factor that predicted higher likelihood of overweight/obesity at middle childhood (ß = -0.20, 95% CI: -0.38, -0.01; p = 0.035). CONCLUSIONS: These findings suggest that overweight/obesity may be driving changes in stress biology across early-to-middle childhood, particularly in downregulation of morning levels of stress hormones, diurnal sAA slope, and cortisol reactivity to stress, rather than stress biology driving overweight/obesity.

Associations between Infant and Parent Characteristics and Measures of Family Well-Being in Neonates with Seizures: A Cohort Study.

OBJECTIVE: To characterize and determine risk factors for key dimensions of well-being at hospital discharge in families of neonates with acute symptomatic seizures. STUDY DESIGN: This prospective, observational cohort study enrolled 144 parent-infant dyads among neonates with acute symptomatic seizures from 9 pediatric hospitals in the Neonatal Seizure Registry. One parent per family completed a discharge survey, which included measures of anxiety and depression, health-related quality of life, and impact on the family. Multivariable regression analyses adjusted for site were constructed to examine parent and infant characteristics associated with well-being. RESULTS: At discharge, 54% of parents reported symptoms of anxiety and 32% reported symptoms of depression. Parents of infants with hypoxic-ischemic encephalopathy reported more depression and worse quality of life than parents of infants with other seizure etiologies. Parental quality of life was also lower with greater infant age at discharge. A higher level of maternal education was associated with greater impact on the family. All these differences were medium to large effect sizes, ranging from 0.52 to 0.78. CONCLUSIONS: Symptoms of anxiety and depression are common in parents of infants with neonatal seizures, and several parent and infant characteristics are associated with poorer parental quality of life and family well-being. These findings are a call to action to improve mental health screening and services for parents of infants with neonatal seizures.

Thrombocytopenia in pediatric patients on concurrent cannabidiol and valproic acid.

In January 2019, a new plant-derived purified cannabidiol preparation, approved by the US Food and Drug Administration, became commercially available for patients ≥2 years old with Lennox-Gastaut syndrome or Dravet syndrome. Among our patients who were prescribed the new cannabidiol formulation, we observed several cases of thrombocytopenia and therefore embarked on this study. We conducted a single-center systematic chart review of all pediatric patients (<21 years old) who were prescribed cannabidiol from January to August 2019. We evaluated salient features of the patients' epilepsy syndrome, age, concurrent medications, and surveillance laboratory results before and after cannabidiol initiation. Among 87 patients, nine (10%) developed thrombocytopenia (platelet nadir range = 17 000-108 000) following initiation of cannabidiol. Each of these nine children was on combination therapy of cannabidiol with valproic acid. Whereas no children on cannabidiol without valproic acid (0/57) developed thrombocytopenia, nine of 23 treated with combination valproic acid and cannabidiol developed platelets < 110 000/µL (P < .0001). We report a novel and clinically important side effect of thrombocytopenia in one-third of patients treated concurrently with cannabidiol and valproic acid. If this finding is confirmed, clinicians should perform close monitoring for thrombocytopenia when adding cannabidiol to a regimen that includes valproic acid.

Neutropenia Is an Underrecognized Finding in Pediatric Primary Immunodeficiency Diseases: An Analysis of the United States Immunodeficiency Network Registry.

BACKGROUND: The frequency of neutropenia in pediatric primary immunodeficiency disorders (PIDDs) is unknown and potentially underappreciated. Our study aimed to determine the overall frequency and severity of neutropenia in children diagnosed with a PIDD entered in the United States Immunodeficiency Network (USIDNET) patient registry. PROCEDURE: Neutropenia data and demographic/clinical information from 1145 patients younger than 21 years of age was obtained from the USIDNET registry. RESULTS: Neutropenia is more common in PIDD patients entered within the USIDNET registry than previously appreciated. There was a >10% occurrence rate of neutropenia in all broad primary immunodeficiency categories as well as in nearly all individual PIDDs. Neutropenia frequency was greater in African American pediatric PIDD patients than in white or Asian patients. The degree of neutropenia did not associate with mortality in pediatric patients with a PIDD. CONCLUSION: Although our study did not assess the frequency of PIDD in patients presenting with neutropenia, the possibility of a primary immune disorder should be considered in patients with idiopathic neutropenia.

Sucking behavior in typical and challenging feedings in association with weight gain from birth to 4 Months in full-term infants.

Sucking behavior has been described as an obesity risk marker. Sucking behavior in response to challenge has not been examined as a prospective predictor of infant weight gain. Healthy, full term infants had sucking behavior assessed at ages 2 weeks and/or 2 months via a sucking pressure measurement device in two feeding conditions: during a standard feeding and during a feeding with a more challenging nipple. Weight and length were measured at 2 weeks, 2 months, and 4 months and weight-for-length z-score (WLZ) calculated. Among 45 full term infants, adjusted for age at measurement and time since last feeding, the challenging versus typical feedings differed with regard to amount consumed (54.1g vs. 65.6g, p < .05), maximum sucking pressure (121.3 mmHg vs. 99.2 mmHg, p < .05), mean burst duration (17.5s vs. 28.4s, p < .05), and feeding duration (18.51 min vs. 13.89 min, p < .01). Grams consumed in the challenging, but not typical, feeding, adjusted for age and time since last feeding, predicted rate of change in WLZ from time of measurement to age 4 months (r = 0.46, p = .013 for challenging, r = -0.07, p = .702 for typical). Nipples that are more challenging to suck from change the sucking behavior and intake among full term infants. Infants who consume more when the nipple is more challenging have greater prospective weight gain. This persistent sucking behavior in the face of challenge may reflect that a greater willingness to work for food, a known obesity risk factor, is detectable in early infancy.

Greater analgesic effects of sucrose in the neonate predict greater weight gain to age 18 months.

Intraoral sucrose has analgesic effects in the newborn period. The hedonic and analgesic effects of sucrose overlap and hedonic response to sweet food is associated with adiposity. The potential association between the analgesic effects of intraoral sucrose in the newborn period and subsequent weight gain has not been examined. Healthy, term newborns received 25% intraoral sucrose or water prior to metabolic screen heel stick. Negative affect, quiet alert behavior, and sleepiness were coded during heel stick. Weight and length were measured and z-score (WLZ) calculated at birth, 9, and 18 months. Mixed models tested associations of behavioral response to heel stick with WLZ trajectory among infants receiving sucrose (n = 154) versus water (n = 117). Among infants receiving sucrose prior to heel stick with birth WLZ ≥ the median, less negative affect and more sleepiness during heel stick were each associated with greater increases in WLZ. These associations were not present among infants receiving water only prior to heel stick. Greater analgesic effects of sucrose in the neonate were associated with greater future increases in WLZ, especially among infants with higher birth WLZ. Greater opioid-mediated newborn behavioral response to intraoral sucrose may be a marker for future obesity risk. CLINICAL TRIALS NUMBER: NCT02728141.