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Reichman D.E.,Brigham and Womens Hospital | Laufer M.R.,Childrens Hospital of Boston | Laufer M.R.,Brigham and Womens Hospital
Best Practice and Research: Clinical Obstetrics and Gynaecology | Year: 2010

The following review seeks to summarise the current data regarding reproductive outcomes associated with congenital uterine anomalies. Such malformations originate from adverse embryologic events ranging from agenesis to lateral and vertical fusion defects. Associated renal anomalies are common both for the symmetric and asymmetric malformations. While fertility is minimally impacted upon by müllerian anomalies in most cases, such malformations have historically been associated with poor obstetric outcomes such as recurrent miscarriage, second trimester loss, preterm delivery, malpresentation and intrauterine foetal demise (IUFD). The following review delineates the existing literature regarding such outcomes and indicates therapies, where applicable, to optimise the care of such patients. © 2009 Elsevier Ltd. All rights reserved. Source


Tikkanen A.U.,Childrens Hospital of Boston
Cardiology in the young | Year: 2012

Advances in medical and surgical care have contributed to an important increase in the survival rates of children with congenital heart disease. However, survivors often have decreased exercise capacity and health-related issues that affect their quality of life. Cardiac Rehabilitation Programmes have been extensively studied in adults with acquired heart disease. In contrast, studies of children with congenital heart disease have been few and of limited scope. We therefore undertook a systematic review of the literature on cardiac rehabilitation in children with congenital heart disease to systematically assess the current evidence regarding the use, efficacy, benefits, and risks associated with this therapy and to identify the components of a successful programme. We included studies that incorporated a cardiac rehabilitation programme with an exercise training component published between January, 1981 and November, 2010 in patients under 18 years of age. A total of 16 clinical studies were found and were the focus of this review. Heterogeneous methodology and variable quality was observed. Aerobic and resistance training was the core component of most studies. Diverse variables were used to quantify outcomes. No adverse events were reported. Cardiac Rehabilitation Programmes in the paediatric population are greatly underutilised, and clinical research on this promising form of therapy has been limited. Questions remain regarding the optimal structure and efficacy of the programmes. The complex needs of this unique population also mandate that additional outcome measures, beyond serial cardiopulmonary exercise testing, be identified and studied. Source


Easter J.S.,Childrens Hospital of Boston | Josephson S.A.,University of California at San Francisco | Vinton D.T.,Denver Health Medical Center | Saint S.,University of Michigan | Edlow J.A.,Beth Israel Deaconess Medical Center
New England Journal of Medicine | Year: 2010

A 34-year-old woman presented to a community hospital with aphasia. Her husband reported that her condition had been normal until 2 hours earlier, when her arms and legs suddenly shook for several seconds. Immediately afterward, she was unable to speak or to move her limbs on the right side. Copyright © 2010 Massachusetts Medical Society. Source


Wood J.C.,Childrens Hospital | Zhang P.,Shire Pharmaceuticals | Rienhoff H.,Ferrokin Biosciences, Inc. | Abi-Saab W.,Shire Pharmaceuticals | Neufeld E.,Childrens Hospital of Boston
American Journal of Hematology | Year: 2014

MRI relaxometry (R2, R2*) has generally replaced liver biopsy for estimation of liver iron stores in response to iron chelation, but there have been no longitudinal studies comparing R2 and R2* techniques. We use R2 and R2* liver iron concentration (LIC) estimates, transfusional iron burdens, and drug compliance data to calculate iron chelation efficiency (ICE) in patients undergoing a Phase II trial of SPD602. Fifty-one patients underwent a baseline examination, 39 patients completed 1 year, and 26 patients completed 2 years. Baseline LICR2 and LICR2* estimates were unbiased, but had limits of agreement exceeding 50%, suggesting that these techniques cannot be interchanged with one another in the same patient. However, ICE estimates across the two techniques compared more favorably, with r2 values reaching 0.89 at 2 years. 95 confidence intervals for efficiency estimates were 0.0±4.1%. These data indicate that clinical trial and clinical effectiveness data calculated using LICR2 and LICR2* estimates can be compared to one another, even though LIC estimates may be disparate on cross-sectional analysis. While the choice of MRI assessment technique for clinical trials and for clinical management depends on many logistical considerations, one can have confidence comparing conclusions on clinical effectiveness. © 2014 Wiley Periodicals, Inc. Source


Wood J.R.,Childrens Hospital Los Angeles | Miller K.M.,Jaeb Center for Health Research | Maahs D.M.,Barbara Davis Center for Childhood Diabetes | Beck R.W.,Childrens Hospital Los Angeles | And 5 more authors.
Diabetes Care | Year: 2013

Objective-To assess the proportion of youth with type 1 diabetes under the care of pediatric endocrinologists in the United States meeting targets for HbA1c, blood pressure (BP), BMI, and lipids. Research design and methods-Data were evaluated for 13,316 participants in the T1D Exchange clinic registry younger than 20 years old with type 1 diabetes for ≥1 year. ResultsAmerican Diabetes Association HbA1c targets of ,8.5% for those younger than 6 years, ,8.0% for those 6 to younger than 13 years old, and ,7.5% for those 13 to younger than 20 years old were met by 64, 43, and 21% of participants, respectively. The majority met targets for BP and lipids, and two-thirds met the BMI goal of ,85th percentile. Conclusions-Most children with type 1 diabetes have HbA1c values above target levels. Achieving American Diabetes Association goals remains a significant challenge for the majority of youth in the T1D Exchange registry. © 2013 by the American Diabetes Association. Source

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