The Influence of Blind Tennis on Subjective Inclusion Experiences-An Ableism-Critical Analysis.

In this paper, we analyze the subjective inclusion experiences of visually impaired (VI) adult tennis players from an ableism-critical perspective. The primary focus of this research is the inclusive potential of blind tennis from the perspective of VI individuals. Episodic interviews were conducted to capture subjective perspectives. A qualitative text analysis revealed that the interviewees were confronted with multiple ability assumptions by sighted people in their everyday lives. Deficit notions on the performance of VI people included sports, work, and general activities. Participation in blind tennis helped the interviewees build a "competent identity" and acquire various skills useful for their everyday lives as participation in blind tennis was a pathway for competence in sports. Further research is needed to identify exclusion experiences from the perspective of disabled people to recognize the potential of different sports in reducing barriers to participation.

Universal Design for Learning and Differentiated Instruction in Physical Education.

The aim of this case study was to describe the distinct approaches used by physical education (PE) teachers to accommodate students with disabilities in New York elementary school PE classes. The participants included 1 adapted PE specialist, 5 PE teachers, and 5 elementary school students with various impairments. Through thematic analysis, observations and interviews revealed 3 main approaches: (a) normalized instruction-traditional curriculum with no differentiation in the program; (b) differentiated instruction-adaptations tailored specifically to the needs of each student with disability; and (c) universally designed instruction based on the principles of Universal Design for Learning (UDL) and accessibility to all students. Differentiated instruction, entailing modifications in the program and pedagogical accommodations, was the most prevalent approach at the research site, but lessons based on UDL principles were also observed. In association, the 2 approaches (differentiated instruction and UDL) represented significant resources to accommodate students with disabilities in PE.

Utility of Echocardiography in the Assessment of Left Ventricular Diastolic Function and Restrictive Physiology in Children and Young Adults with Restrictive Cardiomyopathy: A Comparative Echocardiography-Catheterization Study.

The aim of the study is to determine the utility of echocardiography in the assessment of diastolic function in children and young adults with restrictive cardiomyopathy (RCM). RCM is a rare disease with high mortality requiring frequent surveillance. Accurate, noninvasive echocardiographic measures of diastolic function may reduce the need for invasive catheterization. Single-center, prospective, observational study of pediatric and young adult RCM patients undergoing assessment of diastolic parameters by simultaneous transthoracic echocardiogram (TTE) and invasive catheterization. Twenty-one studies in 15 subjects [median (IQR) = 13.8 years (7.0-19.2), 60% female] were acquired with median left ventricular end-diastolic pressure (LVEDP) 21 (IQR 18-25) mmHg. TTE parameters of diastolic function, including pulmonary vein A wave duration (r s  = 0.79) and indexed left atrial volume (r s  = 0.49), demonstrated significant positive correlation, while mitral valve A (r s  = -0.44), lateral e' (r s  = -0.61) and lateral a' (r s  = -0.61) velocities showed significant negative correlation with LVEDP. Lateral a' velocity (≤0.042 m/s) and pulmonary vein A wave duration (≥156 m/s) both had sensitivity and specificity ≥80% for LVEDP ≥ 20 mmHg. In pediatric and young adult patients with RCM, lateral a' velocity and pulmonary vein A wave duration predicted elevated LVEDP with high sensitivity and specificity; however, due to technical limitations the latter was reliably measured in 12/21 patients. These noninvasive parameters may have utility in identifying patients that require further assessment with invasive testing. These findings require validation in a multicenter prospective cohort prior to widespread clinical implementation.

Doing things my way: teaching physical education with a disability.

Having a disability and being a teacher can be a critical site for examining practices associated with ability, competence, and pedagogy. While there is a growing literature base that examines the experiences of students with disabilities in physical education, there is virtually no research that examines the experiences of physical education teachers with disabilities. Using the capability approach, this article explores the experiences of a physical education teaching intern with a physical disability, significant school members, and the students he interacted with through interviews and documents. The results yielded 3 primary themes. The first, "the fluid nature of the disability discourse," demonstrated the complexity of disability and explored the contrast between static tendencies that stereotype disability and the disability experience. The second theme, "doing things my way," reflected the intern's need to distinguish himself as a teacher by defining contexts for experiencing competence. The third and final theme, "agent of change," explored how the intern's experiences as a teacher with a disability informed his educational narrative.

Perceptions of a disability sport unit in general physical education.

The purpose of this qualitative study was to assess the effectiveness of a disability sport unit in shaping perceptions of disability. Data from interviews, observations, and documents were collected on 87 elementary-aged students, one physical education teacher, and one teaching intern. Comparisons were drawn between fifth graders engaged in a five-week disability sport unit to fourth graders participating in their standard physical education curriculum. Findings revealed differences in the way fourth and fifth graders came to view individuals with disabilities. The results support an analysis of curriculum development that underscores the significance of the social model in positively impacting constructions of disability. Recommendations include the use of disability sports in physical education as an effective strategy for educating students in game play, knowledge of the Paralympics, and the inclusion of individuals with disabilities in a variety of sporting venues.

Abnormal circumferential strain is present in young Duchenne muscular dystrophy patients.

Advances in management of non-cardiac issues in Duchenne muscular dystrophy (DMD) have improved such that DMD-associated cardiac disease has become the leading cause of death for such patients. Cardiac dysfunction measured by standard transthoracic echocardiographic methods, e.g., fractional shortening (FS) and ejection fraction (EF), is rarely present during the first decade of life. The current study used transthoracic echocardiogram (TTE) to assess strain (ε), an indicator of regional ventricular function, in young DMD patients. A retrospective review of the TTE database was performed. TTE results from DMD patients <8 years (n = 63) performed during 2009 to 2010 were compared with TTE results from an unaffected control group (n = 61). Feature tracking analysis software was used to measure total circumferential strain (ε cc) as well as segmental ε cc based on the American Society of Echocardiography 16-segment model. Although there were no differences in FS, the absolute value for left-ventricular (LV) ε cc at the mid-chamber level was decreased in DMD (-21.7 % ± 3.8 % vs. -19.8 % ± 4.2 %, p < 0.01; unaffected vs. DMD). Segmental ε(cc) was similarly affected in the anteroseptal segment (-23.0 % ± 6.1 % vs. -18.9 % ± 7.0 %, p = 0.001; controls vs. DMD), the inferior segment (-20.7 % ± 5.16 % vs. -17.7 % ± 6.1 %, p = 0.003; controls vs. DMD), and the inferolateral segment (-18.3 % ± 6.2 % vs. -15.9 % ± 6.7 %, p = 0.04; controls vs. DMD). In the present study we demonstrate both total and segmental LV ε cc (anteroseptal, inferior, and inferolateral segments) abnormalities at the mid-chamber level in a large group of young DMD patients with normal FS. These novel findings substantiate that the disease process is present and results in abnormal myocardial function before standard measures detect global dysfunction.

Fetal Heart Rate < 3rd Percentile for Gestational Age Can Be a Marker of Inherited Arrhythmia Syndromes.

BACKGROUND: Repeated fetal heart rates (FHR) < 3rd percentile for gestational age (GA) with 1:1 atrioventricular conduction (sinus bradycardia) can be a marker for long QT syndrome. We hypothesized that other inherited arrhythmia syndromes might present with fetal sinus bradycardia. METHODS: We reviewed pregnancies referred with sinus bradycardia to the Colorado Fetal Care Center between 2013 and 2023. FHR/GA data, family history, medication exposure, normalized isovolumic contraction times (n-IVRT), postnatal genetic testing, and ECGs at 4-6 weeks after birth were reviewed. RESULTS: Twenty-nine bradycardic subjects were evaluated by fetal echocardiography. Five were lost to follow-up, one refused genetic testing, and one had negative genetic testing for any inherited arrhythmia. Six had non-genetic causes of fetal bradycardia with normal prenatal n-IVRT and postnatal QTc. Thirteen carried pathogenic variants in RYR2 (n = 2), HCN4 (n = 2), KCNQ1 (6), and other LQTS genes (n = 4). The postnatal QTc was <470 ms in subjects with RYR2, HCN4, and two of those with KCNQ1 mutations, and >470 ms in subjects with CALM 2, KCNH2, SCN5A, and four of those with KCNQ1 mutations. LQTS and RYR2 mutations were associated with prolonged n-IVRT, but HCN4 was not. Two fetuses died in utero with variants of uncertain significance (CACNA1 and KCNE1). Cascade testing uncovered six affected but undiagnosed parents and confirmed familial inheritance in five. CONCLUSION: In addition to heralding LQTS, repeated FHR < 3rd percentile for GA is a risk factor for other inherited arrhythmia syndromes. These findings suggest that genetic testing should be offered to infants with a history of FHR < 3rd percentile for GA even if the postnatal ECG demonstrates a normal QTc interval.

Cross-Cultural Translation and Application of the Lieberman-Brian Inclusion Rating Scale for PE in German-Speaking Countries.

Overcoming participation barriers of students with disabilities in physical education is of great importance and an internationally recognized goal. Research highlights that students with disabilities have mixed feelings about their inclusion experiences in physical education. Physical education teachers often do not feel prepared to appropriately support all students. In German-speaking countries in particular, there is a strong tradition of segregation, with varying interpretations of inclusion. In this light, an instrument to reliably assess the inclusive potential of physical education is needed, thereby providing data on the efficacy of teachers' practices. Such an assessment scale would be important to identify barriers to inclusive physical education while providing teachers with data that could potentially enhance the learning environment. The purpose of this study was to outline initial insights into the cross-cultural translation process of the Lieberman/Brian Inclusion Rating Scale for PE in German-speaking countries. The translation process followed suggestions for transcultural validation. Expert review was provided to check content and face validity. Major item challenges centered around paraeducators, gym management, and conceptual differences regarding physical education.

Coteaching in physical education: a strategy for inclusive practice.

Qualitative research methods were used to explore the factors that informed general and adapted physical education teachers' coteaching practices within an inclusive high school physical education program. Two physical education teachers and one adapted physical education teacher were observed over a 16-week period. Interviews, field notes, and documents were collected and a constant comparative approach was used in the analysis that adopted a social model framework. Primary themes included community as the cornerstone for student learning, core values of trust and respect, and creating a natural support structure. Coteaching practices existed because of the shared values of teaching, learning, and the belief that all students should be included. Recommendations include shifting orientations within professional preparation programs to account for the social model of disability.

"Undiagnosed aortic coarctation with 2 simultaneous acute aortic syndromes: Intramural hematoma and mycotic aneurysm".

Acute aortic syndrome can be a fatal pathology if not diagnosed and managed early. Although acute aortic syndrome is more often a diagnosis of adulthood, it may occasionally afflict the pediatric patients. We herein present a case of a 5-year-old female that was discovered to have multiple acute and congenital aortic abnormalities after presenting to the emergency department with infectious symptoms and lower extremity pain. Acute aortic syndrome may not be a top differential consideration in children with acute chest pain; however, it is important to consider because delayed diagnosis and management can have fatal implications.

An Exploratory Study of Physical Education Teacher Education Graduates' Job Histories.

Purpose: The purpose of this study was to examine the job histories of physical education teacher education (PETE) graduates over a 15-year span. Method: A total of 118 graduates/participants completed a Qualtrics, open-ended survey. Face validity of the survey questions was established via a panel of experts and the survey was pilot-tested by PE teachers. Data were analyzed inductively via constant comparison. Trustworthiness of the data was addressed via peer debriefing and the use of member checks. Results: During the first year after graduation, of the 118 participants in this study, 82% were employed in schools, with 48% securing full-time teaching positions in health and/or PE, and adapted physical education (APE), and the other 34% in roles such as part-time teaching. Ultimately, 75% of graduates taught full-time in health, PE and/or APE. A total of 7% of those who taught opted to leave teaching. Almost all of the 80 participants still teaching planned to teach till retirement, or a very long time, with none stating that they planned to leave the profession soon. A few (3%) stated that they were undecided, however. Conclusion: There is a lack of research in the area of job histories of PETE graduates in the United States, and much of the overall research on organizational socialization is dated. The authors hope to spur further inquiry into this important area. The results of this study are encouraging but follow up studies are envisioned to further examine issues such as job satisfaction and teacher/coach orientations from acculturation through the life span of teachers.

B-type natriuretic peptide predicts adverse cardiovascular events in pediatric outpatients with chronic left ventricular systolic dysfunction.

BACKGROUND: Plasma B-type natriuretic peptide (BNP) levels are elevated in adults with heart failure and correlate with functional classification and prognosis. The range and predictive power of BNP concentrations in children with chronic heart failure, however, are not known. METHODS AND RESULTS: Whole blood BNP concentrations were measured in 53 consecutive patients with chronic left ventricular (LV) systolic dysfunction (biventricular hearts, ejection fraction < 50%, > 3 months since diagnosis). Children who had been hospitalized within 3 months before potential enrollment and those < 2 months or > 21 years of age were excluded. BNP concentrations were measured with the Triage assay (Biosite Diagnostics, Inc, San Diego, Calif). Echocardiographers and clinicians were blinded to BNP levels. An adverse cardiovascular event was defined as cardiac death, cardiac-related hospitalization, or listing for cardiac transplantation. The median age of patients with LV dysfunction was 9.3 years (interquartile range [IQR], 2.7 to 15.1 years). BNP levels were elevated in children with LV dysfunction compared with healthy controls (median, 78 pg/mL [IQR, 22 to 551 pg/mL] versus median, 7 pg/mL [IQR, 5 to 11 pg/mL]; P < 0.0001). Whole blood BNP concentrations were increased in patients who had a 90-day adverse cardiovascular event compared with those who did not (median, 735 pg/mL [IQR, 685 to 1510 pg/mL] versus median, 37 pg/mL [IQR, 14 to 92 pg/mL]; P < 0.001). Patients with a BNP concentration > or = 300 pg/mL were at increased risk of death, hospitalization, or listing for cardiac transplantation (adjusted hazard ratio, 63.6; P < 0.0001). CONCLUSIONS: BNP concentrations are elevated in children with chronic LV systolic dysfunction and predict the 90-day composite end point of death, hospitalization, or listing for cardiac transplantation.

Safety and efficacy of nesiritide in pediatric heart failure.

BACKGROUND: We hypothesized that recombinant B-type natriuretic peptide (BNP) (nesiritide) could improve urine output and neurohormonal markers of heart failure without worsening renal function in pediatric patients. METHODS AND RESULTS: We analyzed our experience involving 140 nesiritide infusions in 63 consecutive children. Serum levels of BNP and electrolytes were measured before and after therapy. Dosing was begun at 0.01 mcg.kg.min without a bolus and titrated to a maximum of 0.03 mcg.kg.min, in 0.005-mcg.kg.min increments. Blood pressure, heart rate, and heart rhythm were monitored. In a substudy, 20 patients with decompensated cardiomyopathy-related heart failure received 72 hours of nesiritide with prospective assessment of aldosterone, norepinephrine, plasma renin, and endothelin-1 levels before and after therapy. The heart rate decreased significantly (P = .001). Urine output increased significantly on Days 1 and 3 (P < or = .001 and .004, respectively). The mean serum creatinine level decreased from 1.135 to 1.007 mg/dL (P < or = .001). In the substudy, aldosterone levels decreased from 37.5 +/- 57.1 to 20.5 +/- 41.9 ng/dL (P = .005). Plasma renin, norepinephrine, and endothelin-1 levels decreased nonsignificantly. Two infusions were discontinued because of hypotension. CONCLUSIONS: Nesiritide safely treated decompensated heart failure in children. Increased urine output reflected improving renal function. Improved neurohormonal markers were seen after 72 hours of therapy, and complications were uncommon.

Physical Activity, Screen-Time Behavior, and Obesity Among 13-Year Olds in Ireland with and without Autism Spectrum Disorder.

The primary purposes of this study were to compare (a) physical activity participation, screen-time habits, obesity, and (b) reported reasons for lack of participation in sport, between a nationally representative sample of Irish children with and without autism spectrum disorder (ASD). Participation in moderate to vigorous activity, light activity, and sports was significantly lower among the group with ASD. On examination of screen time variables, no significant differences were seen between groups. However, time spent watching TV was higher among children with ASD. Overweight or obese status was more prevalent among the group with ASD (34.4 vs. 24.7 %). The findings are discussed in relation to international statistics on youth physical activity, screen-time, and weight status, and recommendations are provided for future research.

Danon disease as an underrecognized cause of hypertrophic cardiomyopathy in children.

BACKGROUND: Some patients with hypertrophic cardiomyopathy (HCM) or left ventricular hypertrophy also present with skeletal myopathy and Wolff-Parkinson-White (WPW) syndrome; mutations in the gene encoding the lysosome-associated protein-2 (LAMP-2) have been identified in these patients, suggesting that some of these patients have Danon disease. In this study we investigated the frequency of LAMP2 mutations in an unselected pediatric HCM population. METHODS AND RESULTS: LAMP2 was amplified from genomic DNA isolated from peripheral lymphocytes of 50 patients diagnosed with HCM and analyzed by direct DNA sequencing. In 2 of the 50 probands (4%), nonsense mutations were identified. In 1 family the proband initially presented with HCM as a teenager, which progressed to dilated cardiomyopathy (DCM) and heart failure. Skeletal myopathy and WPW were also noted. The teenage sister of the proband is a carrier of the same LAMP2 mutation and has HCM without skeletal myopathy or WPW. The other proband presented with HCM, WPW, and skeletal myopathy as a teenager, whereas his carrier mother developed DCM during her 40s. Skeletal and cardiac muscle sections revealed the absence of LAMP-2 on immunohistochemical staining. CONCLUSIONS: LAMP2 mutations may account for a significant proportion of cases of HCM in children, especially when skeletal myopathy and/or WPW is present, suggesting that Danon disease is an underrecognized entity in the pediatric cardiology community.

The Management of Athletes with Congenital Heart Disease.

Although rare, sudden cardiac death (SCD) is devastating to families and communities. Screening of athletes prior to sports participation by trained professionals is useful in identifying individuals who carry known risk factors for SCD. Inclusive in this population are those athletes with congenital heart disease. Sports medicine specialists should be able to identify those at risk for adverse events surrounding vigorous activity and direct appropriate evaluation by the specialist (ie, cardiologist) as deemed appropriate. Equally importantly, they should be able to coach individuals in order to improve performance and quality of life with exercise in a safe environment.

Usefulness of combined history, physical examination, electrocardiogram, and limited echocardiogram in screening adolescent athletes for risk for sudden cardiac death.

Sudden cardiac death in the young (SCDY) is the leading cause of death in young athletes during sport. Screening young athletes for high-risk cardiac defects is controversial. The purpose of this study was to assess the utility and feasibility of a comprehensive cardiac screening protocol in an adolescent population. Adolescent athletes were recruited from local schools and/or sports teams. Each subject underwent a history and/or physical examination, an electrocardiography (ECG), and a limited echocardiography (ECHO). The primary outcome measure was identification of cardiac abnormalities associated with an elevated risk for sudden death. We secondarily identified cardiac abnormalities not typically associated with a short-term risk of sudden death. A total of 659 adolescent athletes were evaluated; 64% men. Five subjects had cardiac findings associated with an elevated risk for sudden death: prolonged QTc >500 ms (n = 2) and type I Brugada pattern (n = 1), identified with ECG; dilated cardiomyopathy (n = 1) and significant aortic root dilation; and z-score = +5.5 (n = 1). History and physical examination alone identified 76 (11.5%) subjects with any cardiac findings. ECG identified 76 (11.5%) subjects in which a follow-up ECHO or cardiology visit was recommended. Left ventricular mass was normal by ECHO in all but 1 patient with LVH on ECG. ECHO identified 34 (5.1%) subjects in whom a follow-up ECHO or cardiology visit was recommended. In conclusion, physical examination alone was ineffective in identification of subjects at elevated risk for SCDY. Screening ECHO identified patients with underlying cardiac disease not associated with immediate risk for SCDY. Cost of comprehensive cardiac screening is high.

Conduction abnormalities in pediatric patients with restrictive cardiomyopathy.

BACKGROUND: Pediatric restrictive cardiomyopathy carries a poor prognosis secondary to a high risk of sudden death previously attributed to ventricular tachyarrhythmias. The extent of conduction abnormalities in this population and their relationship to life-threatening events has not been previously reported. METHODS AND RESULTS: A retrospective study of pediatric patients with restrictive cardiomyopathy diagnosed between April 1994 and May 2011 was performed. Demographic, cardiac, and ECG characteristics and the mechanisms of serious arrhythmic events (death or episode of acute hemodynamic compromise thought to be secondary to arrhythmia) were evaluated. Sixteen patients (1-17 years of age) were reviewed, with 5 sudden cardiac events noted, including 4 deaths. Two deaths were caused by development of acute heart block; another patient with syncope had intermittent heart block and survived as the result of pacing features of an implanted defibrillator system. The median PR interval (222 versus 144 ms; P<0.01) and the QRS duration (111 versus 74; P=0.01) were significantly longer in those who had an acute cardiac event. Older age at presentation was associated with sudden cardiac events (P<0.01). No other functional or echocardiographic variables were associated with a sudden cardiac event. CONCLUSIONS: Pediatric patients with restrictive cardiomyopathy are at risk for acute high-grade heart block, and, in this cohort, bradycardic events represented a significant portion of all arrhythmic events. Aggressive ECG monitoring strategies looking for conduction system disease should be ongoing in all patients with restrictive cardiomyopathy. Implantation of a defibrillator/pacemaker should be considered as prophylactic management.

Malonyl coenzyme A decarboxylase deficiency: early dietary restriction and time course of cardiomyopathy.

Malonyl coenzyme A (CoA) decarboxylase (MCD) deficiency is a rare autosomal recessive organic acidemia characterized by varying degrees of organ involvement and severity. MCD regulates fatty acid biosynthesis and converts malonyl-CoA to acetyl-CoA. Cardiomyopathy is 1 of the leading causes of morbidity and mortality in this disorder. It is unknown if diet alone prevents cardiomyopathy development based in published literature. We report a 10-month-old infant girl identified by newborn screening and confirmed MCD deficiency with a novel homozygous MLYCD mutation. She had normal echocardiogram measurements before transition to high medium-chain triglycerides and low long-chain triglycerides diet. Left ventricular noncompaction development was not prevented by dietary interventions. Further restriction of long-chain triglycerides and medium-chain triglycerides supplementation in combination with angiotensin-converting enzyme inhibitors helped to improve echocardiogram findings. Patient remained asymptomatic, with normal development and growth. Our case emphasizes the need for ongoing cardiac disease screening in patients with MCD deficiency and the benefits and limitations of current dietary interventions.