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Calmodulin-Dependent Regulation of Overexpressed however, not Endogenous TMEM16A Indicated inside Air passage Epithelial Cellular material.
BACKGROUND The use of radiation therapy to treat metastases in patients with metastatic Ewing sarcoma (MES) has been controversial and variable. The authors report outcomes and patterns of failure after metastatic site irradiation (MSI). PROCEDURE A total of 27 pediatric patients with MES were treated with chemotherapy and received radiation therapy to their primary site. Ten patients additionally received MSI, which consisted of whole-lung irradiation (WLI) in patients with lung metastases. Metastatic sites were followed from diagnosis to the first relapse. RESULTS Median follow-up was 29 months. Seventy-eight percent of patients relapsed. Two-year progression-free survival (PFS) and overall survival with and without MSI were 30 versus 29% (log rank P=0.38) and 60 versus 70% (log rank P=0.11), respectively. The median time to relapse among patients who relapsed was 19.5 versus 12.3 months for those receiving MSI versus those who did not (P=0.04).Seven of 20 (35%) patients with lung metastases received WLI±other MSI. Two-year PFS with and without MSI was 43% versus 23% (log rank P=0.02). Among patients with a complete response to computed tomography, 5 of 14 (36%) patients received MSI. Two-year PFS with and without MSI was 60% versus 33% (log rank P=0.04).In the cohort of patients who relapsed, among all metastatic sites at diagnosis, the disease recurred at 15% of irradiated sites and 31% of unirradiated sites. On logistic regression, no factors were statistically associated with increased risk of recurrence at initial sites of metastases. CONCLUSIONS Relapses frequently occur at sites of prior unirradiated disease in patients with MES. WLI may improve 2-year PFS, regardless of chemotherapy response. Further investigation of the role of MSI is warranted.Hereditary spherocytosis arises from alterations in the genes encoding red blood cell membrane proteins. Although its diagnosis is mostly clinical, recent advances in next-generation sequencing (NGS) technologies have allowed for a faster cost-effective gene-based diagnosis. We report the case of a boy with spherocytic anemia and development delay in whom a de novo 2.84-Mb deletion at chromosome 14 including SPTB (ß-spectrin gene) was identified by array-based comparative genomic hybridization. This alteration, consistent with de novo spherocytosis, was missed by a NGS gene panel. When associated with other symptoms, especially neurological, NGS may not be appropriate to genetically diagnose spherocytic anemia.The incidence of pediatric venous thromboembolism (VTE) has been increasing in the past few decades and can be associated with significant mortality and morbidity. There are known risk factors associated with VTE, including estrogen therapy. However, the relationship between testosterone and VTE remains unclear. Here, we present a 17-year-old female-to-male transgender patient without a history of inherited thrombophilia, who developed pulmonary embolism while receiving testosterone injections for gender dysphoria. Despite the limited data on testosterone and the risk of VTE, health care providers should counsel patients and family about the possible increased risk of VTE when starting testosterone.Inflammatory Bowel Disease (IBD) is a chronic disease causing inflammation of the gastrointestinal tract. Some patients require ostomy surgery to optimize their health. This study assessed perceived medical and psychosocial educational needs related to ostomy surgery in pediatric patients with IBD. This mixed-methods study included qualitative interviews of pediatric patients and caregivers with demographic/medical variables obtained from medical records. Participants (n = 8) had an average age of 15.62 years (SD = 2.97). Mean length of diagnosis was 4.5 years (SD = 3.6 years). Interviews were transcribed and coded. Qualitative coding of narratives identified main codes of Ostomy Surgery, Pre-Operative Concerns, Post-Operative Concerns, Education Preferences, and Social Concerns, with various sub-codes. Codes captured unfamiliarity with the ostomies, preferences for education from a medical provider, and psychosocial concerns. Results suggest pediatric patients with IBD have limited understanding of ostomies and limited insight into educational preferences. These findings highlight the importance of developmentally-appropriate information for accessible ostomy education.OBJECTIVES The accuracy of different bioelectrical impedance analysis (BIA) devices for assessing body composition in children with obesity is unclear. We determined the relative accuracy of two BIA devices compared to dual x-ray absorptiometry (DXA) in obese and severely obese children. METHODS We measured body composition in a cross-sectional study of 78 obese children by a handheld single frequency tetrapolar BIA device (Omron), a stationary multifrequency octopolar BIA device (InBody 370) and DXA. Inter-method agreement was assessed by intraclass correlations, paired t-tests, and Bland-Altman analyses. RESULTS Participants (37% female, age 14.8 ± 2.7 years) had mean (±SD) BMI of 36.7 ± 7.5 kg/m, body fat percentage of 46.4 ± 5.2% and appendicular lean mass of 22.5 ± 6.0 kg by DXA. Mubritinib clinical trial Intraclass correlations with DXA for body fat percentage were 0.39 and 0.87 for single frequency tetrapolar and multifrequency octopolar BIA devices, respectively. The single frequency tetrapolar BIA underestimated body fat percentage by 5.5 ± 2.9% (p  less then  0.0001). Differences between the multifrequency octopolar BIA and DXA for body fat percentage (-1.1 ± 2.8%) and appendicular lean mass (-0.3 ± 1.4 kg) were small, and 95% limits of agreement were approximately ± 5%. CONCLUSIONS BIA machines vary in relative accuracy in measuring body composition in children who are obese and severely obese. The multifrequency octopolar BIA device accurately estimated body fat percentage and appendicular lean mass relative to DXA and has the advantage of point of care performance.OBJECTIVE To determine whether clinical characteristics and management of pediatric acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP) differ across INSPPIRE (INternational Study Group of Pediatric Pancreatitis In Search for a CuRE) sites. STUDY DESIGN Data were collected from INSPPIRE and analyzed per US regions and "non-US" sites. Between-group differences were compared by Pearson Chi-Square test. Differences in disease burden were compared by Kruskal-Wallis test. RESULTS Of 479 subjects, 121 (25%) were enrolled in West, 151 (32%) Midwest, 45 Northeast (9%), 78 (16%) South and 84 (18%) at non-US sites. Hispanic ethnicity was more common in South (p  less then  0.0001); white race in Northeast (p = 0.009). CP was less common and time from diagnosis of first acute pancreatitis to CP was longer in children at non-US sites (p = 0.0002 and p = 0.011 respectively). Genetic mutations were most common among all groups; PRSS1 variants predominated in Midwest (p = 0.002). Gallstones were more frequent in South (p = 0.
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