Deakins K.M.,Rainbow Hospitals
Respiratory Care | Year: 2015
Asthma continues to be recognized as a well-known respiratory disease requiring complex management. Asthma is assessed and treated by clinicians across the continuum. The interest in evidence- based recommendations for diagnosis, treatment, and long-term management is ongoing and essential for aligning clinical practice with its changes. The purpose of this review is to provide updates from recent literature on asthma for clinicians. © 2015 Daedalus Enterprises.
Chandrasekharam V.V.S.,Rainbow Hospitals
Journal of Pediatric Urology | Year: 2013
Laparoscopic pyeloplasty is being performed successfully in children with results comparable to open pyeloplasty. Pediatric laparoscopic pyeloplasty, however, remains a technically demanding procedure. Some of the most difficult, time-consuming, and at the same time critical steps of the procedure are ureteral spatulation and intracorporeal suturing of the anastomosis [1,2,3]. It is also preferable to avoid grasping the ureter with any instrument. The difficulty of ureteral spatulation has led to the development of some technical modifications [1,2], including an ex-vivo spatulation technique . In our own practice, we tried some modifications to overcome this challenging part of the procedure. We have finally developed a technique that simplifies this surgical step. This technique can be performed with simple conventional laparoscopic instruments and is easily reproducible. © 2012 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Gurion R.,Rainbow Hospitals |
Lehman T.J.A.,Cornell University |
Moorthy L.N.,University of New Brunswick
International Journal of Inflammation | Year: 2012
Systemic juvenile idiopathic arthritis (sJIA) constitutes a small part of juvenile idiopathic arthritis (JIA), yet has a disproportionally higher rate of mortality. Despite being grouped under JIA, it is considered to be a multifactorial autoinflammatory disease. The objective of this paper is to review the epidemiology, pathogenesis, genetics, clinical manifestations, complications, therapy, prognosis, and outcome of sJIA. The presentation and clinical manifestations of sJIA have not changed much in the past several decades, but the collective understanding of the pathogenesis and the development of new targeted therapies (particularly the biologic agents) have transformed and improved the disease outcome for children with sJIA. © 2012 R. Gurion et al.
Kerr D.S.,Rainbow Hospitals
Molecular Genetics and Metabolism | Year: 2010
While many treatments for mitochondrial electron transport (respiratory) chain disorders have been suggested, relatively few have undergone controlled clinical trials. This review focuses on the recent history of clinical trials of dichloroacetate (DCA), arginine, coenzyme Q10, idebenone, and exercise in both primary (congenital) disorders and secondary (degenerative) disorders. Despite prior clinical impressions that DCA had a positive effect on mitochondrial disorders, two trials of diverse subjects failed to demonstrate a clinically significant benefit, and a trial of DCA in MELAS found a major negative effect of neuropathy. Arginine also has been used to treat MELAS with promising effects, although a controlled trial is still needed for this potentially toxic agent. The anti-oxidant coenzyme Q10 is very widely used for primary mitochondrial disorders but has not yet undergone a controlled clinical trial; such a trial is now underway, as well as trials of the co-Q analogue idebenone for MELAS and LHON. Greater experience has accumulated with multi-center trials of coenzyme Q10 treatment to prevent the progression of Parkinson disease. Although initial smaller trials indicated a benefit, this has not yet been confirmed in subsequent trials with higher doses; a larger Phase III trial is now underway. Similarly, a series of trials of idebenone for Friedreich ataxia have shown some benefit in slowing the progression of cardiomyopathy, and controlled clinical trials are now underway to determine if there is significant neurological protection. Uncontrolled trials of exercise showed an increase of exercise tolerance in patients with disorders of mitochondrial DNA, but did not selectively increase the percentage of normal mtDNA; a larger partially controlled trial is now underway to evaluate this possible benefit. In summary, none of the controlled trials so far has conclusively shown a benefit of treatment with the agents tested, but some promising therapies are currently being evaluated in a controlled manner. These experiences underscore the importance of controlled clinical trials for evaluation of benefits and risks of recommended therapies. Application of such clinical trials to future more effective therapies for mitochondrial disorders will require multi-center collaboration, organization, leadership, and financial and advocacy support. © 2009 Elsevier Inc. All rights reserved.
Dasenbrook E.C.,Rainbow Hospitals
Current Opinion in Pulmonary Medicine | Year: 2011
Purpose of Review: Respiratory infection is a major contributor to morbidity and mortality in cystic fibrosis (CF). One infection the CF community is particularly concerned about is methicillin-resistant Staphylococcus aureus (MRSA). Worldwide, the prevalence of MRSA has been rising and the impact on clinical outcomes and optimal prevention and treatment strategies are unclear. Recent Findings: Studies have demonstrated MRSA is independently associated with poor clinical outcomes, even after taking into account severity of illness. Additionally, characteristics of MRSA strains, such as small colony variants and borderline oxacillin-resistant S. aureus, may be important in predicting the subsequent clinical course. The treatment of MRSA has had variable results and emergence of resistance to commonly prescribed antibiotics is a concern. Summary: The evidence to date supports MRSA infection is independently associated with worse outcomes. The next step is to build upon the current research to expand the knowledge about the impact different strains of MRSA have on infection control strategies and MRSA treatment protocols. Interventions should balance patient safety, efficacy, and treatment burden to improve the quality and length of life in patients with CF. © 2011 Wolters Kluwer Health | Lippincott Williams & Wilkins.