Springfield, MA, United States
Springfield, MA, United States

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Botkin J.R.,University of Utah | Kaye C.I.,University of Colorado at Denver | Haddow J.E.,Brown University | Bradley L.A.,Brown University | And 3 more authors.
Genetics in Medicine | Year: 2010

Genetic tests are increasingly available for use in traditional clinical practice settings and through direct-to-consumer marketing. The need for evidence-based information and guidance on their appropriate use has never been more apparent. The independent Working Group of the Evaluation of Genomic Applications in Practice and Prevention Initiative commissions evidence-based reviews and develops recommendations to inform decision making surrounding the implementation of genetic tests and other applications of genomic technologies into clinical practice. A critical component of this analysis involves the identification and appropriate weighting of relevant health outcomes from genetic testing. Impacts of testing on morbidity and mortality are central considerations although research to document such outcomes can be challenging to conduct. In considering the broader impacts of genetic tests on the individual, familial and societal levels, psychosocial outcomes often take on increasing importance, and their systematic evaluation is a challenge for traditional methods of evidence-based review. Incorporating these types of outcomes in evidence-based processes is possible, however, and necessary to extract balanced and complete (or as complete as available data will allow) information on potential benefits and on potential harms. The framework used by the Evaluation of Genomic Applications in Practice and Prevention Working Group in considering, categorizing, and weighting health-related outcomes as applied to genomic technologies is presented here. © 2010 The American College of Medical Genetics.


Surran B.,Baystate Childrens Hospital | Surran B.,Boston Medical Center | Visintainer P.,Baystate Medical Center | Visintainer P.,Tufts University | And 4 more authors.
Journal of Perinatology | Year: 2013

Objective:To compare the efficacy of clonidine versus phenobarbital in reducing morphine sulfate treatment days for neonatal abstinence syndrome (NAS).Study Design:Prospective, non-blinded, block randomized trial at a single level III NICU (Neonatal Intensive Care Unit). Eligible infants were treated with a combination of medications as per protocol. Primary outcome was treatment days with morphine sulfate. Secondary outcomes were the mean total morphine sulfate dose, outpatient phenobarbital days, adverse events and treatment failures.Results:A total of 82 infants were eligible, of which 68 were randomized with 34 infants in each study group. Adjusting for covariates phenobarbital as compared with clonidine had shorter morphine sulfate treatment days (-4.6, 95% confidence interval (CI): -0.3, -8.9; P=0.037) with no difference in average morphine sulfate total dose (1.1 mg kg -1, 95% CI: -0.1, 2.4; P=0.069). Post-discharge phenobarbital was continued for an average of 3.8 months (range 1 to 8 months). No other significant differences were noted.Conclusion:Phenobarbital as adjunct had clinically nonsignificant shorter inpatient but significant overall longer therapy time as compared with clonidine. © 2013 Nature America, Inc.


Wretzel S.R.,Baystate Childrens Hospital | Visintainer P.F.,Baystate Medical Center | Pinkston Koenigs L.M.,Baystate Childrens Hospital
Journal of Adolescent Health | Year: 2011

Purpose: Sexual activity and sexually transmitted infection (STI) rates are high in adolescents. In this study, we sought to determine whether the initiation of a school-based condom availability program was associated with a decrease in STI rates. Methods: We compared the rates of STIs in 1519-yr-olds reported to the Massachusetts Department of Public Health for the 3 years before and after a condom availability program was introduced in Holyoke, MA, as compared with a similar city, Springfield, MA, which did not have such a program. Results: Holyoke males, aged 1519 years, showed a 47% decrease in the rates of gonorrhea and chlamydia infection combined over the 3 years after the implementation of the condom availability program, whereas similar aged males in Springfield had a 23% increase in the rates of gonorrhea and chlamydia infection. The difference in regression slopes in this period was significant (p <.01). Females, aged 1519 years, from either Holyoke or Springfield, showed moderate, variable changes in rates of STIs after 2005; there was no significant difference in the regression slopes of STIs between Holyoke and Springfield. Conclusions: Initiating a condom availability program in a city's high school was associated with a decrease in STI rates for 1519-yr-old males but not females. © 2011 Society for Adolescent Health and Medicine. All rights reserved.


Wu P.S.,Baystate Medical Center | Beres A.,McGill University | Tashjian D.B.,Baystate Childrens Hospital | Moriarty K.P.,Baystate Childrens Hospital
Pediatric Emergency Care | Year: 2011

Objectives: The management of dog bite wounds is controversial, and current data on risk of infection are variable and inconsistent. Furthermore, the use of prophylactic or empiric antibiotics for the treatment of these wounds is debatable. We investigate the rate of wound infections and other complications after primary repair of pediatric facial dog bite injuries. Methods: We reviewed 87 consecutive patients aged 18 years or younger who had facial dog bite injuries from January 2003 to December 2008. Variables examined were age, sex, setting of repair, number of sutures used for repair, whether surgical drains were used, and antibiotic administration. End points measured were incidence of wound infection, need for scar revision, and any wound complications. Results: The mean age of patients was 6.8 years, and the majority were women (53%). All facial injuries were primarily repaired at the time of presentation either in the emergency department (ED; 46%), operating room (OR; 51%), or an outpatient setting (3%). All patients received an antibiotic course, none of the patients developed wound infection, and no subsequent scar revisions were performed. Three patients repaired in the OR underwent placement of a total of 4 closed-suction drains. The mean (SD) age of patients repaired in the OR was significantly younger than those repaired in the ED (5.7 [3.9] vs 8.0 [4.5] years, respectively; P < 0.01). The number of sutures used were greater for patients repaired in the OR than in the ED (66.4 [39.6] vs 21.7 [12.5], respectively; P < 0.01). Conclusions: Intuitively, younger patients and patients with greater severity injuries are more likely to undergo repair in the OR, and this was supported by our data. Overall, we found that primary repair of pediatric facial dog bite injuries, including complex soft-tissue injuries, is safe when performed in conjunction with antibiotic administration; however, further cross-specialty studies are needed to fully characterize these end points in a larger population. Copyright © 2011 by Lippincott Williams & Wilkins.


Ross J.,Thomas Jefferson University | Czernichow P.,Necker Enfants Malades Hopital | Biller B.M.K.,Massachusetts General Hospital | Colao A.,University of Naples Federico II | And 2 more authors.
Pediatrics | Year: 2010

The therapeutic benefit of growth hormone (GH) therapy in improving height in short children is widely recognized; however, GH therapy is associated with other metabolic actions that may be of benefit in these children. Beneficial effects of GH on body composition have been documented in several different patient populations as well as improvements in lipid profile. Marked augmentation of bone mineral density also seems evident in many pediatric populations. Some of these benefits may require continued therapy past the acquisition of adult height. With long-term therapy of any kind, the adverse consequences of treatment should also be considered. Fortunately, long-term GH treatment seems to be safe and well-tolerated. This review describes the long-term metabolic effects of GH treatment in the pediatric population and considers how these may benefit children who are treated with GH.


Snyder J.,Tufts University | Snyder J.,Baystate Childrens Hospital | Fisher D.,Tufts University | Fisher D.,Baystate Childrens Hospital
Pediatrics in Review | Year: 2012

• Pertussis is a serious and potentially fatal disease caused by the bacterium Bordetella pertussis. In infants under age 6 months, who are too young to be adequately protected by the vaccine, pertussis is associated with a hospitalization rate of almost 80% and a mortality rate of nearly 1%. • Complications of pertussis include encephalopathy, pneumonia, apnea, seizures, and death. The course of the illness is more severe in young children, with infants under age 6 months most at risk for hospitalization and severe complications. • A high degree of suspicion is important. Treatment usually is initiated too late in the illness to alter the course, but can prevent transmission of the disease to others. • An effective vaccine is available and recommended for all children. Because of waning vaccine immunity over time, an additional dose of vaccine is recommended for older children and adults. • Women whose pregnancy has passed 20 weeks or who are in the postpartum period who were not vaccinated previously or whose vaccination status is unknown, and other individuals who may come in contact with a newborn, should be vaccinated as part of a strategy to "cocoon" the newborn from infection. • Enlarging pockets of underimmunization may be a contributing factor to the current upswing in pertussis cases, reminding us of the importance of maintaining high vaccination rates for the prevention of disease outbreaks.


Rosen B.A.,Tufts University | Rosen B.A.,Baystate Childrens Hospital
Pediatrics in Review | Year: 2012

• Based on strong research evidence, in countries where poliomyelitis has been eliminated, GBS is the most common cause of acquired paralysis in children. (9) • Based on strong research evidence, GBS describes a spectrum of disorders caused by an autoimmune reaction against peripheral nerve components, including the myelin sheath and the axon. (10)(11) • Based on strong research evidence, GBS usually is preceded by a bacterial or viral infection, less likely by vaccination in the 1 to 4 weeks before onset. The strongest relationship is with infection by C jejuni. (12)(13) • Based on strong research evidence, GBS in children most often presents with symmetrical ascending paralysis, diminished or absent reflexes, and often severe pain. Pain may lead to a delay in diagnosis. (2) (3)(9)(14) • Based on strong research evidence, the progressive phase peaks in 7 to 14 days and can lead to various levels of weakness, from abnormal gait to total paralysis, cranial nerve weakness, pain, respiratory compromise, and autonomic instability. (2) • Based on some research evidence and consensus, children require hospitalization and often intensive care until their condition stabilizes because of significant risk of respiratory compromise and autonomic instability. (15) • Based on strong research evidence in adults and some research evidence in children, IVIG and plasma exchange hasten recovery from GBS in patients with impaired ability to ambulate. (4)(6) • Based primarily on consensus, IVIG is the treatment of choice in children with GBS. (6)(9)(15) • Based on strong research evidence, the prognosis for full functional recovery in childhood GBS is excellent. (2)(8)(9).


Gasier H.G.,Duke University | Hughes L.M.,United Medical Systems | Young C.R.,United Medical Systems | Richardson A.M.,Baystate Childrens Hospital
Osteoporosis International | Year: 2014

Summary: The submarine environment is unique in that there is limited space and no sunlight, which may negatively affect skeletal health and lead to accelerated bone loss, osteoporosis, and fractures. Introduction: The primary purpose of this study was to determine whether there was an association with submarine service, specifically time spent at sea, and bone mineral content (BMC) and bone mineral density (BMD) of the lumbar spine and dual proximal femur (total hip and femoral neck) measured by DXA. Methods: This is a cross-sectional study of 462 submariners 20-91 years old. Variables included in the analysis were age, height, race, alcohol intake, tobacco use, fracture history, conditions, and medications known to cause bone loss and osteoporosis and submarine service. Results: Of the submarine service predictors, only serving onboard a diesel submarine was determined to be independently associated with a reduction in BMD of the total hip and femur neck, while no submarine service predictor increased the odds of having low BMD. In submariners 50+ years old, the age-adjusted prevalence of osteopenia was 15.7 % (lumbar spine) and 40.4 % (femur neck), while the prevalence of osteoporosis was 4.8 % (lumbar spine) and 4.2 % (femur neck), rates that did not differ from NHANES 2005-2008. In submariners <50 years old, 3.1 % was below the expected range for age. The proportion of submariners 50+ years old that met the FRAX criteria for pharmacological treatment was 12 %. Conclusions: Intermittent periods of submergence that can range from a few days to 3+ months do not appear to compromise skeletal health differently than the general population. © 2014 International Osteoporosis Foundation and National Osteoporosis Foundation.


Tam P.-Y.I.,Baystate Childrens Hospital | Angelides A.,Baystate Childrens Hospital
American Journal of Perinatology | Year: 2010

Infant and pediatric cholelithiases are well-recognized conditions, thought to be predisposed to by several factors. Fetal cholelithiasis by comparison is a rare finding, and little is known about the natural history and clinical significance of echogenic foci in the fetal gallbladder. The occurrence of fetal gallstones has not been reported among siblings. We report on the first known case of perinatal detection of gallstones in siblings. © 2010 by Thieme Medical Publishers, Inc.


Lauer S.R.,Emory University | Edgar M.A.,Emory University | Gardner J.M.,University of Arkansas for Medical Sciences | Sebastian A.,Baystate Childrens Hospital | Weiss S.W.,Emory University
American Journal of Surgical Pathology | Year: 2013

Soft tissue chordomas (STCs) have never been systematically studied because of their rarity and the difficulty in separating them from similar-appearing lesions. Using brachyury to confirm the diagnosis, we have analyzed our experience with 11 cases. Cases coded as "chordoma" or "parachordoma" were retrieved from institutional and consultation files (1989 to 2011) and were excluded from further analysis if they arose from the bone or in a patient with previous axial chordoma. Eleven of 27 cases met inclusion criteria. Patients (8 male; 3 female) ranged in age from 13 to 71 years (mean 44 y). Tumors were located on the buttock (n=2), wrist (n=2), leg (n=2), toe (n=1), thumb (n=1), ankle (n=1), shoulder (n=1), and chest wall (n=1), ranged in size from 0.5 to 10.9 cm (mean 5.3 cm), and consisted of cords and syncytia of spindled/epithelioid cells with vacuolated eosinophilic cytoplasm and a partially myxoid background. Tumors expressed brachyury (10/10), 1 or more cytokeratins (11/11), and S100 protein (10/11). Follow-up information was available for 10 patients (69 mo; range, 2 to 212 mo). Most (n=6) were alive without disease, 2 developed local recurrence and lung metastases, and 1 developed lung metastasis only. One died with unknown disease status. STCs are histologically identical to osseous ones, but differ in their greater tendency to occur in distal locations where small size and surgical resectability result in better disease control. The existence of STC implies that notochordal remnants are not a prerequisite for chordoma development. Copyright © 2013 by Lippincott Williams &Wilkins.

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