Parental Preferences Regarding Outpatient Management of Children with Congenital Heart Disease.

The objective of this study was to identify patterns of health care usage among children with congenital heart disease (CHD) and determine predictors for contacting cardiologists for routine care. Parents of children with CHD completed surveys, indicating which provider they would contact first for 12 concerns. Predictors for preference for cardiologist evaluation were identified by multivariable logistic regression. Surveys were completed by 307 of 925 (33.2%) parents. Median patient age was 9.4 years [interquartile range (IQR) 5.1-14.4 years] with a median of 1 cardiac surgery (IQR 1-3). Most parents agreed primary care physicians (PCPs) could identify problems related to CHD (70.0%) and when to refer to cardiologists (89.6%). More felt PCPs best understood their values (63.2 vs. 29.6%, P < 0.001) and were more accessible (63.5 vs. 33.6%, P < 0.001) than cardiologists. Parents preferred first evaluation by PCPs for 9 of 12 concerns. Preference for cardiology evaluation was independently associated with the number of cardiac catheterizations [adjusted odds ratio (AOR) for ≥2 catheterizations 2.4, 95% confidence interval (CI) 1.1-4.9], belief the cardiologist better knew the child's medical history (AOR 2.4, 95% CI 1.3-4.6), and provider accessibility (AOR 3.6, 95% CI 1.8-7.4). Parents of CHD patients reported close alignment with PCPs and would contact PCPs first for most routine care. However, some populations continue to contact cardiologists for routine care. Further study is needed to clarify best practices for clinician and parent education.

Cys palmitoylation of the beta subunit modulates gating of the epithelial sodium channel.

The epithelial Na(+) channel (ENaC) is comprised of three homologous subunits (α, ß, and γ) that have a similar topology with two transmembrane domains, a large extracellular region, and cytoplasmic N and C termini. Although ENaC activity is regulated by a number of factors, palmitoylation of its cytoplasmic Cys residues has not been previously described. Fatty acid-exchange chemistry was used to determine whether channel subunits were Cys-palmitoylated. We observed that only the ß and γ subunits were modified by Cys palmitoylation. Analyses of ENaCs with mutant ß subunits revealed that Cys-43 and Cys-557 were palmitoylated. Xenopus oocytes expressing ENaC with a ß C43A,C557A mutant had significantly reduced amiloride-sensitive whole cell currents, enhanced Na(+) self-inhibition, and reduced single channel P(o) when compared with wild-type ENaC, while membrane trafficking and levels of surface expression were unchanged. Computer modeling of cytoplasmic domains indicated that ß Cys-43 is in proximity to the first transmembrane α helix, whereas ß Cys-557 is within an amphipathic α-helix contiguous with the second transmembrane domain. We propose that ß subunit palmitoylation modulates channel gating by facilitating interactions between cytoplasmic domains and the plasma membrane.

First Case Report of a Near Lethal Envenomation by a Salomonelaps par (Solomons Coral Snake) in the Solomon Islands.

Venomous snake bites in the Solomon Islands can be very dangerous due to lack of access to health care. There are no documented case reports of envenomation by snake bites in the Solomon Islands. This case report highlights the management of a patient with potentially lethal neurotoxicity secondary to a Solomonelaps par (Solomons coral snake) in a low resource setting. This case identifies the potential benefit of further research to determine the incidence of lethal envenomation as well as analysing the venom to determine if any commercially available antivenom would be useful in the treatment of envenomation by Salomonelaps par and other venomous snakes. There should be consideration given to providing education on first aid for people living in remote areas as well as education for health workers.

Characteristics of Children Hospitalized for Psychogenic Nonepileptic Seizures Due to Conversion Disorder Versus Epilepsy.

OBJECTIVES: Psychogenic nonepileptic seizures (PNES) are a manifestation of conversion disorder among children but can be difficult to distinguish from epilepsy. We sought to identify characteristics that differentiate children with PNES from those with epilepsy. METHODS: We conducted a retrospective cohort study of children admitted with epilepsy or PNES to 45 children's hospitals from 2004 to 2014. Children with PNES (n = 399) versus those with epilepsy (n = 13 241) were compared on demographic and clinical characteristics, testing, treatment, and health care use. Hierarchical multivariable logistic regression was used to identify characteristics associated with PNES diagnosis. RESULTS: Children with PNES were more likely to be female (adjusted odds ratio [aOR] 2.3; 95% confidence interval [CI] 1.8-3.0), older (aOR 3.8; 95% CI 2.7-5.3 for 14-16 years old), African American (aOR 2.0; 95% CI 1.5-2.7), and have diagnosis codes for psychiatric disorders (aOR 7.1; 95% CI 5.6-9.1) and pain (aOR 2.6; 95% CI 1.9-3.4). They were also more likely to be admitted in the fall (aOR 2.0; 95% CI 1.4-2.8) or spring (aOR 1.9; 95% CI 1.4-2.6) versus summer. Total adjusted hospitalization costs were greater for children with epilepsy ($4724, 95% CI $4413-$5057 vs $5326, 95% CI $5259-$5393; P = .001); length of stay was similar. CONCLUSIONS: Demographic and clinical characteristics differed among children with PNES versus those with epilepsy, including significantly increased odds of psychiatric and pain diagnoses among children with PNES. To better inform treatment and prognostication for children with PNES, research is needed to understand reasons for these differences, seasonal admission patterns, and the relationship between PNES and other psychiatric disorders.

Low-dose involved-field radiation in the treatment of non-hodgkin lymphoma: predictors of response and treatment failure.

PURPOSE: To investigate clinical and pathologic factors significant in predicting local response and time to further treatment after low-dose involved-field radiation therapy (LD-IFRT) for non-Hodgkin lymphoma (NHL). METHODS AND MATERIALS: Records of NHL patients treated at a single institution between April 2004 and September 2011 were retrospectively reviewed. Low-dose involved-field radiation therapy was given as 4 Gy in 2 fractions over 2 consecutive days. Treatment response and disease control were determined by radiographic studies and/or physical examination. A generalized estimating equation model was used to assess the effect of tumor and patient characteristics on disease response. A Cox proportional hazards regression model was used to assess time to further treatment. RESULTS: We treated a total of 187 sites in 127 patients with LD-IFRT. Histologies included 66% follicular, 9% chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma, 10% marginal zone, 6% mantle cell lymphoma (MCL), and 8% other. Median follow-up time was 23.4 months (range, 0.03-92.2 months). The complete response, partial response, and overall response rates were 57%, 25%, and 82%, respectively. A CLL histology was associated with a lower response rate (odds ratio 0.2, 95% confidence interval 0.1-0.5, P=.02). Tumor size, site, age at diagnosis, and prior systemic therapy were not associated with response. The median time to first recurrence was 13.6 months. Those with CLL and age ≤ 50 years at diagnosis had a shorter time to further treatment for local failures (hazard ratio [HR] 3.63, P=.01 and HR 5.50, P=.02, respectively). Those with CLL and MCL had a shorter time to further treatment for distant failures (HR 11.1 and 16.3, respectively, P<.0001). CONCLUSIONS: High local response rates were achieved with LD-IFRT across most histologies. Chronic lymphocytic leukemia and MCL histologies and age ≤ 50 years at diagnosis had a shorter time to further treatment after LD-IFRT.

Resonant frequency does not predict high-frequency chest compression settings that maximize airflow or volume.

High-frequency chest compression (HFCC) is a therapy for cystic fibrosis (CF). We hypothesized that the resonant frequency (f(res)), as measured by impulse oscillometry, could be used to determine what HFCC vest settings produce maximal airflow or volume in pediatric CF patients. In 45 subjects, we studied: f(res), HFCC vest frequencies that subjects used (f(used)), and the HFCC vest frequencies that generated the greatest volume (f(vol)) and airflow (f(flow)) changes as measured by pneumotachometer. Median f(used) for 32 subjects was 14 Hz (range, 6-30). The rank order of the three most common f(used) was 15 Hz (28%) and 12 Hz (21%); three frequencies tied for third: 10, 11, and 14 Hz (5% each). Median f(res) for 43 subjects was 20.30 Hz (range, 7.85-33.65). Nineteen subjects underwent vest-tuning to determine f(vol) and f(flow). Median f(vol) was 8 Hz (range, 6-30). The rank order of the three most common f(vol) was: 8 Hz (42%), 6 Hz (32%), and 10 Hz (21%). Median f(flow) was 26 Hz (range, 8-30). The rank order of the three most common f(flow) was: 30 Hz (26%) and 28 Hz (21%); three frequencies tied for third: 8, 14, and 18 Hz (11% each). There was no correlation between f(used) and f(flow) (r(2) = -0.12) or f(vol) (r(2) = 0.031). There was no correlation between f(res) and f(flow) (r(2) = 0.19) or f(vol) (r(2) = 0.023). Multivariable analysis showed no independent variables were predictive of f(flow) or f(vol). Vest-tuning may be required to optimize clinical utility of HFCC. Multiple HFCC frequencies may need to be used to incorporate f(flow) and f(vol).