Minneapolis, MN, United States
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Romano A.A.,New York Medical College | Allanson J.E.,Childrens Hospital of Eastern Ontario | Dahlgren J.,Growth Science | Gelb B.D.,Mount Sinai School of Medicine | And 7 more authors.
Pediatrics | Year: 2010

Noonan syndrome (NS) is a common, clinically and genetically heterogeneous condition characterized by distinctive facial features, short stature, chest deformity, congenital heart disease, and other comorbidities. Gene mutations identified in individuals with the NS phenotype are involved in the Ras/MAPK (mitogen-activated protein kinase) signal transduction pathway and currently explain ∼61% of NS cases. Thus, NS frequently remains a clinical diagnosis. Because of the variability in presentation and the need for multidisciplinary care, it is essential that the condition be identified and managed comprehensively. The Noonan Syndrome Support Group (NSSG) is a nonprofit organization committed to providing support, current information, and understanding to those affected by NS. The NSSG convened a conference of health care providers, all involved in various aspects of NS, to develop these guidelines for use by pediatricians in the diagnosis and management of individuals with NS and to provide updated genetic findings. Copyright © 2010 by the American Academy of Pediatrics.


Lacro R.V.,Boston Childrens Hospital | Dietz H.C.,Johns Hopkins University | Sleeper L.A.,New England Research Institutes, Inc. | Yetman A.T.,University of Utah | And 30 more authors.
New England Journal of Medicine | Year: 2014

BACKGROUND Aortic-root dissection is the leading cause of death in Marfan's syndrome. Studies suggest that with regard to slowing aortic-root enlargement, losartan may be more effective than beta-blockers, the current standard therapy in most centers. METHODS We conducted a randomized trial comparing losartan with atenolol in children and young adults with Marfan's syndrome. The primary outcome was the rate of aorticroot enlargement, expressed as the change in the maximum aortic-root-diameter z score indexed to body-surface area (hereafter, aortic-root z score) over a 3-year period. Secondary outcomes included the rate of change in the absolute diameter of the aortic root; the rate of change in aortic regurgitation; the time to aortic dissection, aortic-root surgery, or death; somatic growth; and the incidence of adverse events. RESULTS From January 2007 through February 2011, a total of 21 clinical centers enrolled 608 participants, 6 months to 25 years of age (mean [±SD] age, 11.5±6.5 years in the atenolol group and 11.0±6.2 years in the losartan group), who had an aorticroot z score greater than 3.0. The baseline-adjusted rate of change (±SE) in the aortic-root z score did not differ significantly between the atenolol group and the losartan group (-0.139±0.013 and -0.107±0.013 standard-deviation units per year, respectively; P = 0.08). Both slopes were significantly less than zero, indicating a decrease in the degree of aortic-root dilatation relative to body-surface area with either treatment. The 3-year rates of aortic-root surgery, aortic dissection, death, and a composite of these events did not differ significantly between the two treatment groups. CONCLUSIONS Among children and young adults with Marfan's syndrome who were randomly assigned to losartan or atenolol, we found no significant difference in the rate of aorticroot dilatation between the two treatment groups over a 3-year period. (Funded by the National Heart, Lung, and Blood Institute and others; ClinicalTrials.gov number, NCT00429364.) Copyright © 2014 Massachusetts Medical Society.


Klick J.C.,Children's Healthcare Of Atlanta | Friebert S.,Northeast Ohio Medical University | Hutton N.,Johns Hopkins University | Osenga K.,Childrens Hospital and Clinics of Minnesota | And 5 more authors.
Pediatrics | Year: 2014

In 2006, hospice and palliative medicine (HPM) became an officially recognized subspecialty. This designation helped initiate the Accreditation Council of Graduate Medical Education Outcomes Project in HPM. As part of this process, a group of expert clinician-educators in HPM defined the initial competency-based outcomes for HPM fellows (General HPM Competencies). Concurrently, these experts recognized and acknowledged that additional expertise in pediatric HPM would ensure that the competencies for pediatric HPM were optimally represented. To fill this gap, a group of pediatric HPM experts used a product development method to define specific Pediatric HPM Competencies. This article describes the development process. With the ongoing evolution of HPM, these competencies will evolve. As part of the Next Accreditation System, the Accreditation Council of Graduate Medical Education uses milestones as a framework to better define competency-based, measurable outcomes for trainees. Currently, there are no milestones specific to HPM, although the field is designing curricular milestones with multispecialty involvement, including pediatrics. These competencies are the conceptual framework for the pediatric content in the HPM milestones. They are specific to the pediatric HPM subspecialist and should be integrated into the training of pediatric HPM subspecialists. They will serve a foundational role in HPM and should inform a wide range of emerging innovations, including the next evolution of HPM Competencies, development of HPM curricular milestones, and training of adult HPM and other pediatric subspecialists. They may also inform pediatric HPM outcome measures, as well as standards of practice and performance for pediatric HPM interdisciplinary teams. Copyright © 2014 by the American Academy of Pediatrics.


Mittal M.K.,Children's Hospital of Philadelphia | Dayan P.S.,Columbia University | Macias C.G.,Baylor College of Medicine | Bachur R.G.,Harvard University | And 7 more authors.
Academic Emergency Medicine | Year: 2013

Objectives The objectives were to assess the test characteristics of ultrasound (US) in diagnosing appendicitis in children and to evaluate site-related variations based on the frequency of its use. Additionally, the authors assessed the test characteristics of US when the appendix was clearly visualized. Methods This was a secondary analysis of a prospective, 10-center observational study. Children aged 3 to 18 years with acute abdominal pain concerning for appendicitis were enrolled. US was performed at the discretion of the treating physician. Results Of 2,625 patients enrolled, 965 (36.8%) underwent abdominal US. US had an overall sensitivity of 72.5% (95% confidence interval [CI] = 58.8% to 86.3%) and specificity 97.0% (95% CI = 96.2% to 97.9%) in diagnosing appendicitis. US sensitivity was 77.7% at the three sites (combined) that used it in 90% of cases, 51.6% at a site that used it in 50% of cases, and 35% at the four remaining sites (combined) that used it in 9% of cases. US retained a high specificity of 96% to 99% at all sites. Of the 469 (48.6%) cases across sites where the appendix was clearly visualized on US, its sensitivity was 97.9% (95% CI = 95.2% to 99.9%), with a specificity of 91.7% (95% CI = 86.7% to 96.7%). Conclusions Ultrasound sensitivity and the rate of visualization of the appendix on US varied across sites and appeared to improve with more frequent use. US had universally high sensitivity and specificity when the appendix was clearly identified. Other diagnostic modalities should be considered when the appendix is not definitively visualized by US. © 2013 by the Society for Academic Emergency Medicine.


Edinburgh L.,Childrens Hospital and Clinics of Minnesota | Pape-Blabolil J.,Childrens Hospital and Clinics of Minnesota | Harpin S.B.,Colorado College | Saewyc E.,University of British Columbia
Child Abuse and Neglect | Year: 2014

The aim of this study was to describe contextual events, abuse experiences, and disclosure processes of adolescents who presented to a hospital-based Child Advocacy Center for medical evaluation and evidentiary collection as indicated after experiencing multiple perpetrator rape during a single event (. n=. 32) and to compare these findings to a group of single perpetrator sexual assaults (. n=. 534). This study used a retrospective mixed-methods design with in-depth, forensic interviews and complete physical examinations of gang-raped adolescents. Patients ranged from 12 to 17 years (. M=. 14 years). Girls who experienced multiple perpetrator rape during a single event were more likely to have run away, to have drunk alcohol in the past month, and to have participated in binge drinking in the past 2 weeks. Acute presentation of these victims were rare but 30% had hymenal transections and 38% had sexually transmitted infections (STIs). Forensic interviews revealed alcohol was a common weapon used by offenders, and its use resulted in victims experiencing difficulty in remembering and reporting details for police investigation or physical and mental health care. Most victims were raped at parties they attended with people they thought they could trust, and they felt let down by witnesses who could have helped but did not intervene. Although relatively rare, multiple perpetrator rape during a single event is a type of severe sexual assault experience and has significant risks for deleterious health outcomes. These victims require health care by trained providers to diagnose physical findings, treat STIs, screen for trauma, and support victims. © 2014 The Authors.


Schipper L.M.,Childrens Hospital and Clinics of Minnesota
Journal for Nurses in Staff Development | Year: 2011

This article is a review and synthesis of the literature related to the socialization of newly graduated nurses. Personal experience and standards frame the clinical nurse educator role in facilitating learner development and socialization. Successful strategies to improve the socialization process of newly graduated nurses from the literature and recommendations for the future are presented. Copyright © 2011 Lippincott Williams & Wilkins.


Payne N.R.,Childrens Hospital and Clinics of Minnesota | Barry J.,Childrens Hospital and Clinics of Minnesota | Berg W.,Childrens Hospital and Clinics of Minnesota | Brasel D.E.,Childrens Hospital and Clinics of Minnesota | And 5 more authors.
Pediatrics | Year: 2012

BACKGROUND: Although reports of reduced nosocomial infections (NI) in very low birth weight infants have been published, the durability of these gains and changes in secondary outcomes, and clinical practices have less often been published. METHODS: This was a retrospective, observational study of NI reduction in very low birth weight infants at two hospital campuses. The intervention began in 2005 with our renewed quality improvement efforts to reduce NI. We compared outcomes before (2000-2005) and after (2006-2009) the intervention by using univariate and multiple regression analyses. RESULTS: We reduced NI by 50% comparing 2000-2005 to 2006-2009 (23.6% vs 11.6%, P < .001). Adjusting for covariates, the odds ratio for NI was 0.33 (confidence interval, 0.26-0.42, P < .001) in the more recent era. NI were lower even in infants with birth weight 501-1000 grams (odds ratio = 0.38, confidence interval, 0.29-0.51, P < .001). We also reduced bronchopulmonary dysplasia (30.2% vs 25.5%, P = .001), median days to regain birth weight (9 vs 8, P = .04), percutaneously placed central venous catheter use (54.8% vs 43.9%, P = .002), median antibiotic days (8 vs 6, P = .003), median total central line days (16 vs 15, P = .01), and median ventilator days (7 vs 5, P = .01). We sustained improvements for three years. CONCLUSIONS: Quality improvement efforts were associated with sustained reductions in NI, bronchopulmonary dysplasia, antibiotic use, central line use, and ventilator days. Copyright © 2012 by the American Academy of Pediatrics.


Barthell J.E.,Childrens Hospital and Clinics of Minnesota
Minnesota medicine | Year: 2013

In recent years, the number of babies exposed to both illegal and prescribed drugs during pregnancy has increased. This has led to an increase in the number of babies born with addiction. This article describes the signs of neonatal drug withdrawal and suggests a comprehensive approach to preventing and treating it.


Patent
Washington University in St. Louis and Childrens Hospital And Clinics Of Minnesota | Date: 2011-07-14

In one aspect, the disclosure provides isolated nucleic acids, polypeptides, primers, and probes for the detection of mutations in a nucleic acid sequence for a DICER1 polypeptide.


Patent
Childrens Hospital And Clinics Of Minnesota and Washington University in St. Louis | Date: 2014-04-30

In one aspect, the disclosure provides isolated nucleic acids, polypeptides, primers, and probes for the detection of mutations in a nucleic acid sequence for a DICER1 polypeptide.

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