- Determinants of Exercise Performance in Children and Adolescents with Repaired Tetralogy of Fallot Using Stress Echocardiography. [Journal Article]
- PCPediatr Cardiol 2018 Aug 18
- Exercise performance is variable and often impaired in patients with repaired tetralogy of Fallot (rTOF). We sought to identify factors associated with exercise performance by comparing high to low p...
Exercise performance is variable and often impaired in patients with repaired tetralogy of Fallot (rTOF). We sought to identify factors associated with exercise performance by comparing high to low performers on cardiopulmonary exercise testing (CPET) in patients with rTOF. We conducted a cross-sectional study of subjects presenting for CPET who underwent echocardiograms at rest and peak exercise. Patients with pacemakers and arrhythmias were excluded. Right ventricular (RV) global longitudinal strain was used as a measure of systolic function. Pulmonary insufficiency (PI) was assessed with the diastolic systolic ratio and the diastolic systolic time-velocity integral ratio by Doppler interrogation of the pulmonary artery. CPET measures included percent-predicted maximum [Formula: see text] [Formula: see text], percent-predicted maximum work and oxygen pulse. High versus low performers were identified as those achieving [Formula: see text] of at least 80% or falling below, respectively. Differences in echocardiographic parameters from rest to peak exercise were examined using mixed-effects regression models. Compared to the low performers (n = 17), high performers (n = 12) were younger (12.8 ± 3.3 years vs. 18.3 ± 4.8 years), had normal chronotropic response (peak heart rate > 185 bpm) with greater heart rate reserve and superior physical working capacity. High performers also had a greater reduction in PI at peak exercise, despite greater PI severity at rest. Oxygen pulse was comparable between groups. For both groups, there was no association of PI severity and RV systolic function at rest with exercise parameters. There was no group difference in the magnitude of change in RV strain and diastolic parameters from rest to peak exercise. Chronotropic response to exercise appears to be an important parameter with which to assess exercise performance in rTOF. Chronotropic health should be taken into consideration in this population, particularly given that RV function and PI severity at rest were not associated with exercise performance.
- Pulmonary Vein Flow Impedance: An Early Predictor of Cardiac Dysfunction in Intrauterine Growth Restriction. [Journal Article]
- FDFetal Diagn Ther 2018 Aug 17; :1-7
- CONCLUSIONS: Higher PVPI in IUGR reflects decreased LV compliance and altered LA dynamics. As LV dysfunction precedes right ventricle, our results suggest that PVPI could be an early echocardiographic parameter of fetal diastolic function in IUGR.
- Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome in two young children: the importance of an early diagnosis. [Journal Article]
- IJItal J Pediatr 2018 Aug 15; 44(1):93
- CONCLUSIONS: Our cases highlight that paediatricians should be aware of the clinical presentations of and therapeutic approaches for DRESS syndrome, especially in children receiving long-term antibiotic treatment. The removal of the offending drug is crucial and may be the only life-saving measure. In more aggressive cases, corticosteroid or other immunosuppressive drugs should be considered to achieve the best outcome.
- Pleiotropic effect of vitamin D in cystic fibrosis. [Journal Article]
- ARAdv Respir Med 2018 Aug 15
- Cystic fibrosis - CF - is the most common recessively autosomally and inherited disorder in the Caucasian population. It is incurable, multi-systemic disease with progressive course. CF is caused by ...
Cystic fibrosis - CF - is the most common recessively autosomally and inherited disorder in the Caucasian population. It is incurable, multi-systemic disease with progressive course. CF is caused by CFTR gene mutation, the product of which is Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). CF patients are exposed to fat-soluble vitamins deficiency, including vitamin D. It is due to the fat malabsorption (caused by exacerbation exocrine pancreatic insufficiency), decreased sun exposure (caused by receiving antibiotics photophobia), reduction of adipose tissue and insufficient supply with food. The discovery of vitamin D receptor (VDR) presence outside the skeletal system allowed to conclude, that vitamin D is responsible not only for mineral economy, but also for immunological processes, respiratory status, intestial microflora and cystic fibrosis - related diabetes (CFRD) course. Based on literature data, it is suggested that vitamin D plays an important role in the prevention of diseases coexisting with CF. The right dosage of vitamin D allows to maintain a better lung function and prevent chronic pulmonary infections. It has also been shown that normal levels of vitamin D may be important in increasing the chances of successful lung transplant surgery. Taking the wide spectrum of vitamin D effect into account, it is recommended to maintain serum concentrations above the minimum in patients with CF. In summary, maintaining the proper vitamin D levels in patients with CF is important because of its pleiotropic effect. It can be achieved through regular monitoring of vitamin D levels and individual supplementary dose for each patients.
- A risk score model of 30-day readmission in ulcerative colitis after colectomy or proctectomy. [Journal Article]
- CTClin Transl Gastroenterol 2018 Aug 15; 9(8):175
- CONCLUSIONS: Multiple potentially preventable risk factors are associated with 30-day readmission following colectomy or proctectomy in UC patients. Higher risk scores are associated with increased risk of unplanned readmission.
- GeneReviews® [BOOK]
- BOOKUniversity of Washington, Seattle: Seattle (WA)
- FBLN5-related cutis laxa is characterized by cutis laxa, early childhood-onset pulmonary emphysema, peripheral pulmonary artery stenosis, and other evidence of a generalized connective disorder such ...
FBLN5-related cutis laxa is characterized by cutis laxa, early childhood-onset pulmonary emphysema, peripheral pulmonary artery stenosis, and other evidence of a generalized connective disorder such as inguinal hernias and hollow viscus diverticula (e.g., intestine, bladder). Occasionally, supravalvular aortic stenosis is observed. Intrafamilial variability in age of onset is observed. Cardiorespiratory failure from complications of pulmonary emphysema (respiratory or cardiac insufficiency) is the most common cause of death.
- Immune checkpoint inhibitor therapy-induced hypophysitis ∼ a case series of Taiwanese patients. [Journal Article]
- JFJ Formos Med Assoc 2018 Aug 10
- Immune checkpoint blockade-based immunotherapy is a new modality of cancer treatment with a unique mechanism that has gained increased numbers of indication and is now used in several cancer types. H...
Immune checkpoint blockade-based immunotherapy is a new modality of cancer treatment with a unique mechanism that has gained increased numbers of indication and is now used in several cancer types. However, immune-related adverse events (irAEs) emerge as a new entity of diseases involving one or multiple organ systems. irAEs could result in interruption of immunotherapy, morbidities or even death. Among various manifestations of irAEs, immune-mediated hypophysitis is rare but important, requiring prompt diagnosis and treatment to avoid life-threatening conditions. We report seven cases of immune-mediated hypophysitis in Taiwan. They suffered from various types of advanced cancer and received different regimens of immune checkpoint inhibitors. The time of onset after initiation of immunotherapy ranged from 5 to 36 weeks. All seven subjects were diagnosed of central adrenal insufficiency, while four of them had primary hypothyroidism. There was no typical finding of infiltrative hypophysitis on the pituitary MRI. There was no documented hormone recovery after diagnosis of hypophysitis, and the tumor responses to immunotherapy were variable in these seven patients. In conclusion, immune-mediated hypophysitis is often irreversible. Fortunately, it can be managed adequately with hormone replacements. Further investigations are warranted to unveil underlying mechanisms and ethnic differences to guide the solutions.
- Late results of the Ross procedure. [Journal Article]
- JTJ Thorac Cardiovasc Surg 2018 Jul 18
- CONCLUSIONS: The long-term results of the Ross procedure are excellent regardless of the implantation technique, but there is a progressive deterioration of function of both semilunar valves.
- Vitamin A and beta (β)-carotene supplementation for cystic fibrosis. [Review]
- CDCochrane Database Syst Rev 2018 Aug 09; 8:CD006751
- CONCLUSIONS: Since no randomised or quasi-randomised controlled studies on retinoid supplementation were identified, no conclusion on the supplementation of vitamin A in people with CF can be drawn. Additionally, due to methodological limitations in the included study, also reflected in the low-quality evidence judged following the specific evidence grading system (GRADE), no clear conclusions on β-carotene supplementation can be drawn. Until further data are available, country- or region-specific guidelines regarding these practices should be followed.
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- [The Old-Age Heart]. [Journal Article]
- PPraxis (Bern 1994) 2018; 107(16):894-901
- The Old-Age Heart Abstract. Knowledge of cardiovascular changes in old age and their therapeutic options is important. Old age can lead to hypertrophy of the left ventricle, diastolic dysfunction, he...
The Old-Age Heart Abstract. Knowledge of cardiovascular changes in old age and their therapeutic options is important. Old age can lead to hypertrophy of the left ventricle, diastolic dysfunction, heart valve changes and pulmonary hypertension. Patients often develop arterial hypertension. Valvular changes are common in people over 100 years of age (aortic stenosis and mitral insufficiency). The risk of coronary heart disease is 35 % for men and 24 % for women. In old age, sinus node dysfunction and atrial fibrillation are common. 25 % of all strokes are cardiac embolisms in atrial fibrillation. Cardiac interventions in the elderly are increasingly frequent and include coronary catheter revascularization or valve interventions (percutaneous aortic valve replacement or MitraClip). Optimal therapy in old age includes not only cardiovascular interventions also include drugs and a lifestyle modification and mainly serves to improve the quality of life.