Evaluating dysphagia in infants with congenital heart disease using Fiberoptic Endoscopic Evaluation of Swallowing.

OBJECTIVE: Dysphagia is common in infants with congenital heart disease (CHD). However, there is minimal published data regarding its management outside of the perioperative period. The objective of this study is to describe the role of Fiberoptic Endoscopic Evaluation of Swallowing (FEES) in the diagnosis and management of dysphagia in infants with CHD. METHODS: Infants with CHD who underwent FEES exam for evaluation of swallowing dysfunction between February 2015 and February 2021 at a university-based, tertiary care urban pediatric hospital were studied. Demographic data, presenting symptoms, medical history, and dysphagia history were examined. The validated Dysphagia Outcome and Severity Scale (DOSS) was used to standardize and compare dysphagia severity. RESULTS: 62 FEES exams were performed on 48 patients. All 48 patients were diagnosed with dysphagia and had a mean dysphagia severity score (DOSS) of 2.68. Patients with wet laryngeal congestion on presentation had worse mean DOSS score and were more likely to demonstrate aspiration on FEES exam (p < 0.01). There was no significant difference in mean DOSS or presence of aspiration when comparing infants who had cardiothoracic surgery, vocal cord paralysis, or lower respiratory tract illness with those who had not (p > 0.05). CONCLUSION: A FEES exam is an effective and well-tolerated procedure for evaluating swallowing dysfunction in pediatric patients with CHD and its use reduces radiation exposure for this vulnerable population. Wet laryngeal congestion was found to be predictive of more severe dysphagia and aspiration. There is no significant association between severity of dysphagia or aspiration on FEES exam and history of cardiac surgery, vocal cord paralysis, or lower respiratory tract illness.

Influence of Age of Onset on Huntington's Disease Phenotype.

Background: Older patients with Huntington's disease (HD) are often thought to have a slower progressing disease course with less behavioral symptoms than younger patients. However, phenotypic differences based on age of onset have not been well characterized in a large HD population. This study will determine the difference in manifestations and disease progression between patients with young, typical, and late onset adult HD at different stages of disease. Methods: Data obtained from Enroll-HD. Adults with manifest HD were included. Age groups were defined as young onset (YO: 20-29 years), typical onset (TO: 30-59 years), and late onset (LO: 60+ years). Subjects were categorized by TFC score, from Stage I (least severe) to Stage V (most severe). Motor, cognitive, and behavioral symptoms were analyzed. Descriptive statistics and Bonferroni p-value correction for pairwise comparison were calculated. Results: 7,311 manifest HD participants were included (612 YO, 5,776 TO, and 923 LO). The average decline in TFC score from baseline to second visit (1.5-2.5 years) was significantly faster for YO (-1.75 points) compared to TO (-1.23 points, p = 0.0105) or LO (-0.97 points, p = 0.0017). Motor deficits were worse for LO participants at early stages of HD, and worse for YO participants at advanced stages. YO and TO participants had greater burden of behavioral symptoms at early stages of disease compared to LO. Discussion: YO is predictive of a faster functional decline for adults with HD when compared to those with TO and LO. Motor and behavioral manifestations differ based on age of onset. Highlights: This study compares HD manifestations while controlling for disease severity, detailing robust phenotypic differences by age of onset alone. These findings have implications for the clinical management of HD symptoms and have the possibility to improve prognostic and treatment precision.

Financial burden of emergency department visits for atopic dermatitis in the United States.

BACKGROUND: Little is known about the usage and financial burden of emergency care visits for atopic dermatitis (AD) or eczema (AD-E) in the United States. OBJECTIVE: To determine the prevalence, risk factors, and cost of emergency care for AD-E in the United States. METHODS: Cross-sectional study of the 2006-2012 National Emergency Department Sample, including a 20% sample of emergency department (ED) visits throughout the United States (n = 198,102,435). RESULTS: The mean annual incidence of ED visits with a primary diagnosis of AD-E was 3368.4-3553.0 cases/1 million persons. The prevalence of ED visits for AD-E increased significantly during 2006-2012 (survey logistic regression, P < .05). ED visits with a primary diagnosis of AD-E versus ED visits without were associated with younger patient age, Medicaid or no insurance, and lower household income quartile and more likely to occur during weekends and summer months. The geometric mean and total costs of ED visits for AD-E significantly increased from $369.07 and $127,275,080, respectively, in 2006 to $642.10 and $265,541,084, respectively, in 2012. LIMITATIONS: The National Emergency Department Sample did not include data on AD severity, recurrent ED visits, race/ethnicity, or treatments provided. CONCLUSION: There is a substantial and increasing financial burden of ED visits for AD-E in the United States. Interventions are needed to decrease ED visits for AD.

Cardiovascular and cerebrovascular comorbidities of juvenile dermatomyositis in US children: an analysis of the National Inpatient Sample.

Objective: JDM is associated with multiple potential risk factors for cardiovascular disease, including reduced heart rate variability, systolic/diastolic cardiac dysfunction, abnormal brachial artery reactivity and metabolic syndrome. However, little is known about cardiovascular risk in JDM. We sought to examine the association between JDM and cardiovascular risk factors and disease in US children. Methods: Data from the 2002-12 National Inpatient Sample was analysed, including ∼20% of all US hospitalizations (n = 14 535 620 paediatric hospitalizations). Results: JDM was significantly associated with 12 of 13 comorbidities, including hypertension [survey logistic regression; crude odds ratio (95% CI): 22.25 (15.51, 31.92)], obesity [5.87 (3.44, 10.02)], uncomplicated diabetes [7.95 (4.21, 15.00)], lipid abnormalities [5.84 (2.77, 12.31)], particularly lipodystrophy [151.08 (38.24, 596.86)], peripheral and visceral atherosclerosis [10.09 (3.70, 27.56)], late effects of cerebrovascular disease [15.49 (2.37, 101.43)], personal history of transient ischaemic attack and cerebral infarction [10.82 (2.46, 47.65)], pulmonary circulatory disorder [12.23 (2.59, 57.73)], arrhythmia [3.93 (2.80, 5.52)], bradycardia [4.22 (2.65, 6.74)] and hypotension [2.62 (1.27, 5.39)]. Conclusions: There are significantly higher odds of cardiovascular and cerebrovascular comorbidities among inpatients with JDM, with adolescents, girls and racial/ethnic minorities being at highest risk.

Cardiovascular comorbidities of pediatric psoriasis among hospitalized children in the United States.

BACKGROUND: Psoriasis has been shown to be associated with cardiovascular disease in adults. Little is known about cardiovascular risk in pediatric psoriasis. OBJECTIVE: To determine if there is an association between pediatric psoriasis and cardiovascular comorbidities. METHODS: Data were analyzed from the 2002-2012 Nationwide Inpatient Sample, which included 4,884,448 hospitalized children aged 0-17 years. Bivariate and multivariate survey logistic regression models were created to calculate the odds of psoriasis on cardiovascular comorbidities. RESULTS: In multivariate survey logistic regression models adjusting for age, sex, and race/ethnicity, pediatric psoriasis was significantly associated with 5 of 10 cardiovascular comorbidities (adjusted odds ratio [95% confidence interval]), including obesity (3.15 [2.46-4.05]), hypertension (2.63 [1.93-3.59]), diabetes (2.90 [1.90-4.42]), arrhythmia (1.39 [1.02-1.88]), and valvular heart disease (1.90 [1.07-3.37]). The highest odds of cardiovascular risk factors occurred in blacks and Hispanics and children ages 0-9 years, but there were no sex differences. LIMITATIONS: The study was limited to hospitalized children. We were unable to assess the impact of psoriasis treatment or family history on cardiovascular risk. CONCLUSION: Pediatric psoriasis is associated with higher odds of multiple cardiovascular comorbidities among hospitalized patients. Strategies for mitigating excess cardiovascular risk in pediatric psoriasis need to be determined.