A 20-year-old female with trisomy 18 is described. Survival past infancy is rare in this disorder. Little is known concerning the factors that contribute to prolonged survival with this syndrome. This case provides an opportunity to review the management of older children and young adults with trisomy 18.
Background:The incidence of late coronary artery abnormalities after arterial switch operation (ASO) for d-loop transposition of the great arteries may be underestimated. Methods and Results:We retrospectively reviewed coronary artery morphology in 40 of 97 patients who survived the first year after ASO. Seven asymptomatic patients developed significant late coronary artery abnormalities. One patient died suddenly at home with severe left coronary artery (LCA) ostial stenosis at age 3.8 years. The second patient collapsed during exercise at age 9.6 years due to ventricular fibrillation and severe LCA ostial stenosis despite prior negative exercise stress test (EST) and myocardial perfusion imaging (MPI). The third patient was found to have moderate ostial stenosis of the LCA with negative EST and MPI. The fourth patient with exercise-induced ST-T depression and myocardial perfusion defect was shown to have complete LCA occlusion with collateral vessel formation. Three other patients had complete proximal obliteration of either of the coronary arteries with collateral supply. An additional 4 asymptomatic patients had trivial-mild narrowing of the LCA on routine selective coronary angiogram. Conclusions:Incidence of late coronary stenosis or occlusion was not infrequent after ASO (11.3%) and presented usually without preceding symptoms and often after negative non-invasive screening. We advocate routine coronary imaging in all patients after ASO before they participate in competitive sports. 2373 Late Coronary Problems After ASO Laboratory). MPI was performed with technetium-99 m sestamibi injection, and perfusion imaging was obtained before and after exercise. Studies were read by a radiologist specialized in nuclear medicine. All cardiac catheterizations and angiograms were reviewed by W.R., Director of the Cardiac Catheterization Laboratory. All study results and original images were presented and carefully discussed in a weekly Multidisciplinary Case Conference at Nemours Cardiac Center for final consensus among the group, which included all attending cardiologists, intensivists, surgeons, and anesthesiologists prior to any critical decisions being made.
Patients with intraatrial baffle procedure for transposition of the great arteries (TGA) have diastolic dysfunction, decreased exercise capacity, stroke volume response and elevated systemic vascular resistance (SVR) during exercise. Angiotensin-converting enzyme (ACE) inhibitors improve exercise capacity in adults with congestive heart failure by improving diastolic function and decreasing SVR. We tested the hypothesis that ACE inhibitors decrease SVR and improve exercise capacity in patients after intraatrial baffle procedure for TGA. We studied the effects of enalapril in nine patients with TGA s/p intraatrial switch (mean age, 13.8 +/- 3 years) 7 to 21 years (mean, 12 +/- 4 years) after intraatrial baffle procedure. Enalapril (0.5 mg/kg/day, maximum dosage 20 mg bid) was administered for 12 months. Patients exercised using a cycle ergometer ramp protocol (0.25 W/kg/min) before enalapril (baseline), 1 month, 6 months, and 12 months after treatment initiation. Heart rate, blood pressure, cardiac output, respiratory rate, minute ventilation, oxygen consumption (VO2), total exercise time, work, and power were measured. SVR, cardiac index, and stroke volume index (SVI) were calculated. Two-tailed paired Student's t-test was used to compare data to those of normal control patients and the patients' baseline data. Patients had lower resting heart rate, cardiac index, maximum heart rate, cardiac index (CI), SVI, VO2, exercise time, work, and power and higher maximal SVR at baseline compared to normal control patients. There was no significant difference in total exercise time, work, power, VO2 (rest/peak), SVR, SVI, and CI after 12 months of therapy compared to patients' baseline values. We conclude that short-term (<1 year) use of enalapril does not improve exercise performance in patients with TGA in whom the intraatrial baffle procedure has been performed.
Although rugby union as a sport is well established, the strength and conditioning practices of high school level players are not well known. Therefore, the main purpose of this study was to examine the current strength and conditioning practices that coaches implement at South African high school level rugby. A secondary purpose was to compare practices between high schools of different socio-economic status in South Africa. An online survey or in person interview (depending on the school) was conducted and 43 responses were received: from 28 coaches at schools among the top 100 rugby schools in South Africa for 2016 and from 15 coaches at no-fee public schools in the Eastern Cape Province of South Africa. Most coaches (72%) performed some form of physical testing, with the most common parameter tested being ‘speed’. The most common strength and conditioning practices utilised included flexibility (stretching), speed (free sprinting), agility (cone drills), plyometric (box drills) and resistance (weight lifting) training. Unlike the no-fee schools, the top 100 rugby schools implemented conditioning practices similar to best-known international practices. Furthermore, no-fee school coaches did not have the qualifications necessary to administer the correct training techniques. Education and upskilling on the best strength and conditioning practices for school level coaches need to be improved, particularly in less privileged schools. Such improvement is crucial to the transformation goals set out by the South African Rugby Union, which would benefit from player development in lower socio-economic schools.
In their introduction to a recent special issue of Qualitative Inquiry, Taguchi and St. Pierre observed that Foucault’s notion of “fruitful disorientation” seemed to circulate throughout the included articles. Embracing this fruitfully disorienting impulse, the authors of this article provide a detailed reading of Baruch Spinoza’s ontological concept of affect as he articulated it in the Ethics. Through this return, the authors consider the implications of affect for qualitative and postqualitative inquiry and offer an example of how a return to the philosophical origins of a theory can produce new lines of thinking and inquiry.
This article examines how three African American girls, ages 10 to 18, used journaling and interviews to better understand science, technology, engineering, and mathematics (STEM) as part of their literate identities. Drawing on prior work about literate identities, the authors introduce the concept of literate intersectional identities, which describes how participants’ diverse histories, literacies, and identities traverse categories, communities, genres, and modes of meaning within the context of a STEAM workshop. The authors employed open and thematic coding to analyze the girls’ journal entries in an effort to answer a question: In what ways do African American girls’ journal writings and interviews about STEM reflect and influence their literate identities in a digital app coding workshop? Findings reveal how their writings about race, access, and the underrepresentation of women of color in STEM helped them make sense of their self-assurance, self-awareness, and agency as girls of color interested in STEM careers.
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