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Sample records for syndrome pediatrics ethics

  1. 78 FR 48438 - Pediatric Ethics Subcommittee of the Pediatric Advisory Committee; Notice of Meeting

    Science.gov (United States)

    2013-08-08

    ...] Pediatric Ethics Subcommittee of the Pediatric Advisory Committee; Notice of Meeting AGENCY: Food and Drug... of Subcommittee: Pediatric Ethics Subcommittee of the Pediatric Advisory Committee. General Function... pediatric ethical issues. Date and Time: The meeting will be held on September 9, 2013, from 8 a.m. to 5:30...

  2. Everyday ethics issues in the outpatient clinical practice of pediatric residents.

    Science.gov (United States)

    Moon, Margaret; Taylor, Holly A; McDonald, Erin L; Hughes, Mark T; Carrese, Joseph A

    2009-09-01

    To describe the ethics issues that pediatric residents encounter during routine care in an outpatient teaching clinic. Qualitative study including in-depth interviews with pediatric residents and direct observation of interactions between preceptors and residents in a pediatric teaching clinic. The Johns Hopkins Harriet Lane Pediatric Primary Care Clinic, March 20 through April 11, 2006. A convenience sample including all pediatric faculty preceptors supervising at the clinic during the 19 half-day sessions that occurred during the observation period (N = 15) and the pediatric residents seeing patients during these clinic sessions (N = 50). Main Outcome Measure Field notes of preceptor-resident discussions about patient care were made and transcribed for qualitative analysis. Qualitative analysis of the ethics content of cases presented by residents in this pediatric teaching clinic identified 5 themes for categorizing ethics challenges: (1) promoting the child's best interests in complex and resource-poor home and social settings; (2) managing the therapeutic alliance with parents and caregivers; (3) protecting patient privacy and confidentiality; (4) balancing the dual roles of learner and health care provider; and (5) using professional authority appropriately. Qualitative analysis of the ethics content of directly observed preceptor-resident case discussions yielded a set of themes describing the ethics challenges facing pediatric residents. The themes are somewhat different from the lists of residents' ethics experiences developed using recall or survey methods and may be very different from the ideas usually included in hospital-based ethics discussions. This may have implications for improving ethics education during residency training.

  3. 76 FR 21382 - Pediatric Ethics Subcommittee; Notice of Meeting

    Science.gov (United States)

    2011-04-15

    ... DEPARTMENT OF HEALTH AND HUMAN SERVICES Food and Drug Administration [Docket No. FDA-2011-N-0184] Pediatric Ethics Subcommittee; Notice of Meeting AGENCY: Food and Drug Administration, HHS. ACTION: Notice... Administration (FDA). The meeting will be open to the public. Name of Committee: Pediatric Ethics Subcommittee of...

  4. Iranian pediatric nurse's experience: The facilitators of the learning of ethical practices

    Directory of Open Access Journals (Sweden)

    Kobra Karami

    2017-01-01

    Full Text Available Background: Ethical care is a core value in nursing. Pediatric nurses are in direct and continuous contact with children and their parents. They manage their lives and health. As part of the pediatric nurses' daily work, ethical issues play an important role in making decisions, are important to make decisions, and this capability is only achieved by ethical practice. This study aimed to explore the factors facilitating the learning of ethical practice among Iranian pediatric nurses. Materials and Methods: This study is a conventional qualitative content analysis based on the Graneheim and Lundman method. It was conducted through semi-structured interviews with two focus groups, incorporating 28 nurses working in pediatric wards. Unstructured observation and field notes were other methods of data collection. Purposive sampling continued until data saturation was ensured. All interviews were tape recorded and transcribed in verbatim. Results: Three main categories and 12 subcategories emerged from this study. The facilitating factors include (1 individual competencies (knowledge, experience, emotional intelligence, and loving children, (2 ethical imprinting (responsibility, reflection, empathy, and ethical beliefs, and (3 an environment that nurtures moral values (organizational, spiritual, family, and cultural environments as facilitating factors. Conclusions: The promotion of nurses' competencies, ethical virtues, and imprinting, as well as improvement of the quality of nursing care must be the top priority of the health team. Undoubtedly, the success of the health care system is not possible without ensuring that pediatric nurses learn ethical practices.

  5. Ethical problems in pediatrics: what does the setting of care and education show us?

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    Guedert Jucélia

    2012-03-01

    Full Text Available Abstract Background Pediatrics ethics education should enhance medical students' skills to deal with ethical problems that may arise in the different settings of care. This study aimed to analyze the ethical problems experienced by physicians who have medical education and pediatric care responsibilities, and if those problems are associated to their workplace, medical specialty and area of clinical practice. Methods A self-applied semi-structured questionnaire was answered by 88 physicians with teaching and pediatric care responsibilities. Content analysis was performed to analyze the qualitative data. Poisson regression was used to explore the association of the categories of ethical problems reported with workplace and professional specialty and activity. Results 210 ethical problems were reported, grouped into five areas: physician-patient relationship, end-of-life care, health professional conducts, socioeconomic issues and health policies, and pediatric teaching. Doctors who worked in hospitals as well as general and subspecialist pediatricians reported fewer ethical problems related to socioeconomic issues and health policies than those who worked in Basic Health Units and who were family doctors. Conclusions Some ethical problems are specific to certain settings: those related to end-of-life care are more frequent in the hospital settings and those associated with socioeconomic issues and public health policies are more frequent in Basic Health Units. Other problems are present in all the setting of pediatric care and learning and include ethical problems related to physician-patient relationship, health professional conducts and the pediatric education process. These findings should be taken into consideration when planning the teaching of ethics in pediatrics. Trial registration This research article didn't reports the results of a controlled health care intervention. The study project was approved by the Institutional Ethical Review

  6. Aortic Involvement in Pediatric Marfan syndrome: A Review.

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    Ekhomu, Omonigho; Naheed, Zahra J

    2015-06-01

    Outlining specific protocols for the management of pediatric patients with Marfan syndrome has been challenging. This is mostly due to a dearth of clinical studies performed in pediatric patients. In Marfan syndrome, the major sources of morbidity and mortality relate to the cardiovascular system. In this review, we focus on aortic involvement seen in pediatric patients with Marfan syndrome, ranging from aortic dilatation to aortic rupture and heart failure. We discuss the histological, morphological, and pathogenetic basis of the cardiac manifestations seen in pediatric Marfan syndrome and use a specific case to depict our experienced range of cardiovascular manifestations. The survival for patients with Marfan syndrome may approach the expected survival for non-affected patients, with optimal management. With this potentiality in mind, we explore possible and actual management considerations for pediatric Marfan syndrome, examining both medical and surgical therapy modalities that can make the possibility of improved survival a reality.

  7. Aspectos éticos en pediatría Ethical features in Pediatrics

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    Daisy Amparo Martínez Delgado

    2011-06-01

    Full Text Available Se realizó una revisión bibliográfica sobre la ética en pediatría, con el objetivo de corroborar los criterios actuales sobre el tema. Se analizaron algunos dilemas éticos y bioéticos que se deben tener en cuenta en la relación médico-paciente. Se brinda información sobre algunas particularidades del consentimiento informado y se diferencian las formas de comunicación según las edades pediátricas.A bibliographic review was conducted on the Ethics in Pediatrics to corroborate the current criteria on this subject. Some ethical and bioethical dilemmas were analyzed took into account in the physician-patient relation. Authors offer information on some peculiarities of informed consent and the ways of communication according to children ages were distinguished.

  8. 77 FR 2556 - Ethical and Regulatory Challenges in the Development of Pediatric Medical Countermeasures; Public...

    Science.gov (United States)

    2012-01-18

    ...] Ethical and Regulatory Challenges in the Development of Pediatric Medical Countermeasures; Public Workshop... Administration (FDA), Office of Pediatric Therapeutics, is announcing a public workshop entitled ``Ethical and... provide a forum for careful consideration of scientific, ethical, and regulatory issues confronting FDA...

  9. Pediatric acute respiratory distress syndrome: definition, incidence, and epidemiology: proceedings from the Pediatric Acute Lung Injury Consensus Conference.

    Science.gov (United States)

    Khemani, Robinder G; Smith, Lincoln S; Zimmerman, Jerry J; Erickson, Simon

    2015-06-01

    Although there are similarities in the pathophysiology of acute respiratory distress syndrome in adults and children, pediatric-specific practice patterns, comorbidities, and differences in outcome necessitate a pediatric-specific definition. We sought to create such a definition. A subgroup of pediatric acute respiratory distress syndrome investigators who drafted a pediatric-specific definition of acute respiratory distress syndrome based on consensus opinion and supported by detailed literature review tested elements of the definition with patient data from previously published investigations. International PICUs. Children enrolled in published investigations of pediatric acute respiratory distress syndrome. None. Several aspects of the proposed pediatric acute respiratory distress syndrome definition align with the Berlin Definition of acute respiratory distress syndrome in adults: timing of acute respiratory distress syndrome after a known risk factor, the potential for acute respiratory distress syndrome to coexist with left ventricular dysfunction, and the importance of identifying a group of patients at risk to develop acute respiratory distress syndrome. There are insufficient data to support any specific age for "adult" acute respiratory distress syndrome compared with "pediatric" acute respiratory distress syndrome. However, children with perinatal-related respiratory failure should be excluded from the definition of pediatric acute respiratory distress syndrome. Larger departures from the Berlin Definition surround 1) simplification of chest imaging criteria to eliminate bilateral infiltrates; 2) use of pulse oximetry-based criteria when PaO2 is unavailable; 3) inclusion of oxygenation index and oxygen saturation index instead of PaO2/FIO2 ratio with a minimum positive end-expiratory pressure level for invasively ventilated patients; 4) and specific inclusion of children with preexisting chronic lung disease or cyanotic congenital heart disease. This

  10. Ethics education for pediatric residents: a review of the literature

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    Raywat Deonandan

    2015-04-01

    Full Text Available Background: Ethics education and research on medical residents is needed because, unlike medical students or experienced doctors, medical residents have to perform multiple roles simultaneously – student, teacher and clinician – thus exposing them to unique ethical stressors.  In this paper we reviewed the literature concerning ethics education in postgraduate pediatrics training programs.  Our goal was not to simply describe educational strategies and programs, but also to explore measurements and experiences of current practices to address gaps in ethics education during residency. Method: We conducted a structured literature review to explore the extent of ethics education in pediatric residency programs.  Results:  Twelve relevant studies were found.  The studies suggest that existing training regimens are insufficient to meet the real life ethical challenges experienced in actual practice, particularly with respect to palliative care and the commission of clinical errors.  Conclusions: The increasing diversity of culture and beliefs in the clinical workplace is also serving to complicate educational needs. An interdisciplinary approach, spread over the entirety of a physician’s training, is a proposed solution worthy of more attention.

  11. Subclinical nephritic syndrome in children cohabiting with pediatric patients, Presenting acute nephritic syndrome

    OpenAIRE

    Guerrero-Tinoco Gustavo Adolfo; Julio-Barrios Emil

    2012-01-01

    Introduction: subclinical nephritic syndrome is the presence of hematuria, hypocomplementemiaand/or proteinuria without the presence of signs and/or symptoms.Objective: to determine the incidence of subclinical nephritic syndrome in childrenliving with pediatric patients diagnosed with acute nephritic syndrome.Methods: family visit to identify children living together in the two previous months, with pediatric patients hospitalized with acute nephritic syndrome, at Hospital InfantilNapoleon F...

  12. Pediatric palliative care and pediatric medical ethics: opportunities and challenges.

    Science.gov (United States)

    Feudtner, Chris; Nathanson, Pamela G

    2014-02-01

    The fields of pediatric palliative care (PPC) and pediatric medical ethics (PME) overlap substantially, owing to a variety of historical, cultural, and social factors. This entwined relationship provides opportunities for leveraging the strong communication skills of both sets of providers, as well as the potential for resource sharing and research collaboration. At the same time, the personal and professional relationships between PPC and PME present challenges, including potential conflict with colleagues, perceived or actual bias toward a palliative care perspective in resolving ethical problems, potential delay or underuse of PME services, and a potential undervaluing of the medical expertise required for PPC consultation. We recommend that these challenges be managed by: (1) clearly defining and communicating clinical roles of PPC and PME staff, (2) developing questions that may prompt PPC and PME teams to request consultation from the other service, (3) developing explicit recusal criteria for PPC providers who also provide PME consultation, (4) ensuring that PPC and PME services remain organizationally distinct, and (5) developing well-defined and broad scopes of practice. Overall, the rich relationship between PPC and PME offers substantial opportunities to better serve patients and families facing difficult decisions.

  13. Reversible Cerebral Vasoconstriction Syndrome in Pediatrics: A Case Series and Review.

    Science.gov (United States)

    Coffino, Samantha W; Fryer, Robert H

    2017-06-01

    Reversible cerebral vasoconstriction syndrome is a transient vasculopathy associated with severe headaches and stroke. In most cases of reversible cerebral vasoconstriction syndrome, there is a precipitating event or trigger, such as pregnancy, serotonin agonist treatment or illicit drug use. The authors present 2 pediatric cases of reversible cerebral vasoconstriction syndrome and review the previous 11 pediatric cases in the literature. In many instances, the clinical and radiographic features are similar in both pediatric and adult cases. In the pediatric group, reported potential triggers include trauma (1/13), exercise (2/13), water to the face (3/13), hypertension (3/13), and medication or substance use (4/13). One surprising difference is that 11 out of 13 pediatric patients with reversible cerebral vasoconstriction syndrome are male while most cases in adults are female. Many of the pediatric patients with reversible cerebral vasoconstriction syndrome were treated with a calcium channel blocker and the overall outcome of pediatric reversible cerebral vasoconstriction syndrome was good, with most patients experiencing a full recovery.

  14. Pediatric Toxic Shock Syndrome

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    Jennifer Yee

    2017-09-01

    Full Text Available Audience: This scenario was developed to educate emergency medicine residents on the diagnosis and management of a pediatric patient with toxic shock syndrome. The case is also appropriate for teaching of medical students and advanced practice providers, as well as a review of the principles of crisis resource management, teamwork, and communication. Introduction: Toxic shock syndrome is a low-frequency, high-acuity scenario requiring timely identification and aggressive management. If patients suffering from this condition are managed incorrectly, they may progress into multi-organ dysfunction and potentially death. Toxic shock syndrome has been associated with Streptococcus and Staphylococcus aureus (Staph. Approximately half of Staph cases are associated with menstruation, which was first described in the 1970s-1980s and was associated with the use of absorbent tampons.1 Group A Streptococcus may cause complications such as necrotizing fasciitis and gangrenous myositis.2 Pediatric patients may present critically ill from toxic shock syndrome. Providers need to perform a thorough history and physical exam to discern the source of infection. Management requires aggressive care with antibiotics and IV fluids. Objectives: By the end of this simulation session, the learner will be able to: 1 Recognize toxic shock syndrome. 2 Review the importance of a thorough physical exam. 3 Discuss management of toxic shock syndrome, including supportive care and the difference in antibiotic choices for streptococcal and staphylococcal toxic shock syndrome. 4 Appropriately disposition a patient suffering from toxic shock syndrome. 5 Communicate effectively with team members and nursing staff during a resuscitation of a critically ill patient. Method: This session was conducted using high-fidelity simulation, followed by a debriefing session and lecture on toxic shock syndrome.

  15. Burnout Syndrome in Pediatric Practice

    OpenAIRE

    Al-Youbi, Reem A.; Jan, Mohammed M.

    2013-01-01

    Objectives: Burnout is a common work-related syndrome consisting of emotional exhaustion, depersonalization and diminished feelings of personal accomplishment. Burnout influences the performance and efficiency of the healthcare professionals and therefore the quality of the care provided. This study aims to assess the burnout rates and potential determinants in pediatrics.Methods: A cross-sectional, descriptive study involving physicians practicing pediatrics in the Jeddah area of Saudi Arabi...

  16. Ethical dilemmas in pediatric and adolescent psychogenic nonepileptic seizures.

    Science.gov (United States)

    Cole, Cristie M; Falcone, Tatiana; Caplan, Rochelle; Timmons-Mitchell, Jane; Jares, Kristine; Ford, Paul J

    2014-08-01

    To date, only a very narrow window of ethical dilemmas in psychogenic nonepileptic seizures (PNES) has been explored. Numerous distinct ethical dilemmas arise in diagnosing and treating pediatric and adolescent patients with PNESs. Important ethical values at stake include trust, transparency, confidentiality, professionalism, autonomy of all stakeholders, and justice. In order to further elucidate the ethical challenges in caring for this population, an ethical analysis of the special challenges faced in four specific domains is undertaken: (1) conducting and communicating a diagnosis of PNESs, (2) advising patients about full transparency and disclosure to community including patients' peers, (3) responding to requests to continue antiepileptic drugs, and (4) managing challenges arising from school policy and procedure. An analysis of these ethical issues is essential for the advancement of best care practices that promote the overall well-being of patients and their families. Copyright © 2014 Elsevier Inc. All rights reserved.

  17. Ethical issues in pediatric emergency mass critical care.

    Science.gov (United States)

    Antommaria, Armand H Matheny; Powell, Tia; Miller, Jennifer E; Christian, Michael D

    2011-11-01

    As a result of recent events, including natural disasters and pandemics, mass critical care planning has become a priority. In general, planning involves limiting the scope of disasters, increasing the supply of medical resources, and allocating scarce resources. Entities at varying levels have articulated ethical frameworks to inform policy development. In spite of this increased focus, children have received limited attention. Children require special attention because of their unique vulnerabilities and needs. In May 2008, the Task Force for Mass Critical Care published guidance on provision of mass critical care to adults. Acknowledging that the critical care needs of children during disasters were unaddressed by this effort, a 17-member Steering Committee, assembled by the Oak Ridge Institute for Science and Education with guidance from members of the American Academy of Pediatrics, convened in April 2009 to determine priority topic areas for pediatric emergency mass critical care recommendations.Steering Committee members established subgroups by topic area and performed literature reviews of MEDLINE and Ovid databases. Draft documents were subsequently developed and revised based on the feedback from the Task Force. The Pediatric Emergency Mass Critical Care Task Force, composed of 36 experts from diverse public health, medical, and disaster response fields, convened in Atlanta, GA, on March 29-30, 2010. This document reflects expert input from the Task Force in addition to the most current medical literature. The Ethics Subcommittee recommends that surge planning seek to provide resources for children in proportion to their percentage of the population or preferably, if data are available, the percentage of those affected by the disaster. Generally, scarce resources should be allocated on the basis of need, benefit, and the conservation of resources. Estimates of need, benefit, and resource utilization may be more subjective or objective. While the

  18. The legal ethics of pediatric research.

    Science.gov (United States)

    Coleman, Doriane Lambelet

    2007-12-01

    Since the mid- to late 1990s, the scientific and medical research community has sought to increase its access to healthy children for research protocols that involve harm or a risk of harm. This move reverses longstanding policy within that community generally to exclude healthy children from such protocols on the grounds that the research as to them is non-therapeutic, that they are particularly vulnerable to research-related abuses, and that they are unable themselves to give informed consent to their participation. The research community's new posture has been supported by prominent pediatric bioethicists who have argued that unless healthy children are included as research subjects in harmful or risky research, the pediatric population will continue to suffer relative to the adult population in the extent to which it benefits from modern advances in science and medicine. In their view, it is possible for the research community to self-administer a rule that strikes a balance between protecting healthy children from research-related abuses and allowing their inclusion in cutting-edge pediatric research. In this scheme, parental consent is central to the research community's claims about child protection. This Article explores the flaws inherent in this ethics of pediatric research. Specifically, it challenges the view from ethics that the law permits parents to consent to their children's inclusion in harmful or risky research to the extent that related invasions would meet legal maltreatment standards. More broadly, it challenges the movement to increase access to healthy children for harmful and risky research on the ground that it risks two important regressions: First, in its willingness to risk harm to individual children in the interests of the group, it threatens the progress the law has made in its development of the concept of the child as an individual worthy of respect in his or her own right, a concept that imagines parents as fiduciaries and that

  19. Exhaust All Measures: Ethical Issues in Pediatric End-of-Life Care.

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    Thieleman, Kara J; Wallace, Cara; Cimino, Andrea N; Rueda, Heidi A

    2016-01-01

    The death of a child may have a profound impact on parents, family members, and health care providers who provided care for the child. Unique challenges are faced by parents of seriously ill children as they must serve as the legal authority for health care decisions of children under age 18, although the child's wishes must also be considered. Social workers must balance core social work values, bioethical values, and psychosocial issues presented by such situations. While studies have been conducted with physicians and nurses regarding ethical issues in pediatric end-of-life care settings, little is known about how social workers experience these conflicts. This article utilizes two vignettes to illustrate potential ethical issues in this setting and applies the National Association of Social Workers Standards for Palliative and End of Life Care (NASW, 2004 ) to explore options for their resolution. These vignettes provide descriptions of possible reactions in this setting and can be used as a basis for further exploration of ethics in pediatric end-of-life care from a social work perspective.

  20. Privacy and ethics in pediatric environmental health research-part II: protecting families and communities.

    Science.gov (United States)

    Fisher, Celia B

    2006-10-01

    In pediatric environmental health research, information about family members is often directly sought or indirectly obtained in the process of identifying child risk factors and helping to tease apart and identify interactions between genetic and environmental factors. However, federal regulations governing human subjects research do not directly address ethical issues associated with protections for family members who are not identified as the primary "research participant." Ethical concerns related to family consent and privacy become paramount as pediatric environmental health research increasingly turns to questions of gene-environment interactions. In this article I identify issues arising from and potential solutions for the privacy and informed consent challenges of pediatric environmental health research intended to adequately protect the rights and welfare of children, family members, and communities. I first discuss family members as secondary research participants and then the specific ethical challenges of longitudinal research on late-onset environmental effects and gene-environment interactions. I conclude with a discussion of the confidentiality and social risks of recruitment and data collection of research conducted within small or unique communities, ethnic minority populations, and low-income families. The responsible conduct of pediatric environmental health research must be conceptualized as a goodness of fit between the specific research context and the unique characteristics of subjects and other family stakeholders.

  1. Analyzing reflective narratives to assess the ethical reasoning of pediatric residents.

    Science.gov (United States)

    Moon, Margaret; Taylor, Holly A; McDonald, Erin L; Hughes, Mark T; Beach, Mary Catherine; Carrese, Joseph A

    2013-01-01

    A limiting factor in ethics education in medical training has been difficulty in assessing competence in ethics. This study was conducted to test the concept that content analysis of pediatric residents' personal reflections about ethics experiences can identify changes in ethical sensitivity and reasoning over time. Analysis of written narratives focused on two of our ethics curriculum's goals: 1) To raise sensitivity to ethical issues in everyday clinical practice and 2) to enhance critical reflection on personal and professional values as they affect patient care. Content analysis of written reflections was guided by a tool developed to identify and assess the level of ethical reasoning in eight domains determined to be important aspects of ethical competence. Based on the assessment of narratives written at two times (12 to 16 months/apart) during their training, residents showed significant progress in two specific domains: use of professional values, and use of personal values. Residents did not show decline in ethical reasoning in any domain. This study demonstrates that content analysis of personal narratives may provide a useful method for assessment of developing ethical sensitivity and reasoning.

  2. Brown-McLean Syndrome in a Pediatric Patient

    Science.gov (United States)

    Tourkmani, Abdo Karim; Martinez, Jaime D.; Berrones, David; Juárez-Domínguez, Brenda Y.; Beltrán, Francisco; Galor, Anat

    2015-01-01

    The purpose of this manuscript is to report the case of a 12-year-old patient who presented for routine ophthalmic examination after congenital cataract surgery performed at 2 months of age. The patient was diagnosed with bilateral Brown-McLean syndrome by slit lamp examination. No treatment was required because the patient was asymptomatic and had a clear central cornea. This is the first described case of Brown-McLean syndrome in a pediatric patient, representing the importance of clinical examination in the pediatric age group after cataract surgery because of the risk for patients of developing peripheral edema. PMID:26034485

  3. Privacy and Ethics in Pediatric Environmental Health Research—Part II: Protecting Families and Communities

    Science.gov (United States)

    Fisher, Celia B.

    2006-01-01

    Background In pediatric environmental health research, information about family members is often directly sought or indirectly obtained in the process of identifying child risk factors and helping to tease apart and identify interactions between genetic and environmental factors. However, federal regulations governing human subjects research do not directly address ethical issues associated with protections for family members who are not identified as the primary “research participant.” Ethical concerns related to family consent and privacy become paramount as pediatric environmental health research increasingly turns to questions of gene–environment interactions. Objectives In this article I identify issues arising from and potential solutions for the privacy and informed consent challenges of pediatric environmental health research intended to adequately protect the rights and welfare of children, family members, and communities. Discussion I first discuss family members as secondary research participants and then the specific ethical challenges of longitudinal research on late-onset environmental effects and gene–environment interactions. I conclude with a discussion of the confidentiality and social risks of recruitment and data collection of research conducted within small or unique communities, ethnic minority populations, and low-income families. Conclusions The responsible conduct of pediatric environmental health research must be conceptualized as a goodness of fit between the specific research context and the unique characteristics of subjects and other family stakeholders. PMID:17035154

  4. Is Whole-Exome Sequencing an Ethically Disruptive Technology? Perspectives of Pediatric Oncologists and Parents of Pediatric Patients With Solid Tumors.

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    McCullough, Laurence B; Slashinski, Melody J; McGuire, Amy L; Street, Richard L; Eng, Christine M; Gibbs, Richard A; Parsons, D William; Plon, Sharon E

    2016-03-01

    It has been anticipated that physician and parents will be ill prepared or unprepared for the clinical introduction of genome sequencing, making it ethically disruptive. As a part of the Baylor Advancing Sequencing in Childhood Cancer Care study, we conducted semistructured interviews with 16 pediatric oncologists and 40 parents of pediatric patients with cancer prior to the return of sequencing results. We elicited expectations and attitudes concerning the impact of sequencing on clinical decision making, clinical utility, and treatment expectations from both groups. Using accepted methods of qualitative research to analyze interview transcripts, we completed a thematic analysis to provide inductive insights into their views of sequencing. Our major findings reveal that neither pediatric oncologists nor parents anticipate sequencing to be an ethically disruptive technology, because they expect to be prepared to integrate sequencing results into their existing approaches to learning and using new clinical information for care. Pediatric oncologists do not expect sequencing results to be more complex than other diagnostic information and plan simply to incorporate these data into their evidence-based approach to clinical practice, although they were concerned about impact on parents. For parents, there is an urgency to protect their child's health and in this context they expect genomic information to better prepare them to participate in decisions about their child's care. Our data do not support the concern that introducing genome sequencing into childhood cancer care will be ethically disruptive, that is, leave physicians or parents ill prepared or unprepared to make responsible decisions about patient care. © 2015 Wiley Periodicals, Inc.

  5. Is Whole Exome Sequencing an Ethically Disruptive Technology? Perspectives of Pediatric Oncologists and Parents of Pediatric Patients with Solid Tumors

    Science.gov (United States)

    McCullough, Laurence B.; Slashinski, Melody J.; McGuire, Amy L.; Street, Richard L.; Eng, Christine M.; Gibbs, Richard A.; Parsons, D. Williams; Plon, Sharon E.

    2016-01-01

    Background Some anticipate that physician and parents will be ill-prepared or unprepared for the clinical introduction of genome sequencing, making it ethically disruptive. Procedure As part of the Baylor Advancing Sequencing in Childhood Cancer Care (BASIC3) study, we conducted semi-structured interviews with 16 pediatric oncologists and 40 parents of pediatric patients with cancer prior to the return of sequencing results. We elicited expectations and attitudes concerning the impact of sequencing on clinical decision-making, clinical utility, and treatment expectations from both groups. Using accepted methods of qualitative research to analyze interview transcripts, we completed a thematic analysis to provide inductive insights into their views of sequencing. Results Our major findings reveal that neither pediatric oncologists nor parents anticipate sequencing to be an ethically disruptive technology, because they expect to be prepared to integrate sequencing results into their existing approaches to learning and using new clinical information for care. Pediatric oncologists do not expect sequencing results to be more complex than other diagnostic information and plan simply to incorporate these data into their evidence-based approach to clinical practice although they were concerned about impact on parents. For parents, there is an urgency to protect their chil's health and in this context they expect genomic information to better prepare them to participate in decisions about their chil's care. Conclusion Our data do not support concern that introducing genome sequencing into childhood cancer care will be ethically disruptive, i.e., leave physicians or parents ill-prepared or unprepared to make responsible decisions about patient care. PMID:26505993

  6. Burnout syndrome in pediatric practice.

    Science.gov (United States)

    Al-Youbi, Reem A; Jan, Mohammed M

    2013-07-01

    Burnout is a common work-related syndrome consisting of emotional exhaustion, depersonalization and diminished feelings of personal accomplishment. Burnout influences the performance and efficiency of the healthcare professionals and therefore the quality of the care provided. This study aims to assess the burnout rates and potential determinants in pediatrics. A cross-sectional, descriptive study involving physicians practicing pediatrics in the Jeddah area of Saudi Arabia was conducted utilizing the Maslach Burnout Inventory in addition to questions regarding work-related and lifestyle-related factors. One hundred and thirty pediatricians (55% females) were included with age ranging between 25 and 45 years (mean: 30). Most (46%) were consultants and 54% practiced in a university based setting. Burnout scores were abnormal in 107 (82%) and in 45 (34%) the syndrome was severe. Males were more likely to reach a severe burnout category compared to females (40% vs. 31%; p=0.012). Academic pediatricians working in a university setting were much more likely to experience severe burnout compared to their counterparts working in other hospitals (50% vs. 19%; p=0.0005). Consultants were also more likely to experience severe burnout compared to residents and assistants (46% vs. 27%; p=0.03). At least one third of practicing pediatricians suffer from burnout syndrome. Specific strategies should be developed and implemented to limit and prevent professional burnout.

  7. Burnout Syndrome in Pediatric Practice

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    Reem A. Al-Youbi

    2013-07-01

    Full Text Available Objectives: Burnout is a common work-related syndrome consisting of emotional exhaustion, depersonalization and diminished feelings of personal accomplishment. Burnout influences the performance and efficiency of the healthcare professionals and therefore the quality of the care provided. This study aims to assess the burnout rates and potential determinants in pediatrics.Methods: A cross-sectional, descriptive study involving physicians practicing pediatrics in the Jeddah area of Saudi Arabia was conducted utilizing the Maslach Burnout Inventory in addition to questions regarding work-related and lifestyle-related factors.Results: One hundred and thirty pediatricians (55% females were included with age ranging between 25 and 45 years (mean: 30. Most (46% were consultants and 54% practiced in a university based setting. Burnout scores were abnormal in 107 (82% and in 45 (34% the syndrome was severe. Males were more likely to reach a severe burnout category compared to females (40% vs. 31%; p=0.012. Academic pediatricians working in a university setting were much more likely to experience severe burnout compared to their counterparts working in other hospitals (50% vs. 19%; p=0.0005. Consultants were also more likely to experience severe burnout compared to residents and assistants (46% vs. 27%; p=0.03.Conclusion: At least one third of practicing pediatricians suffer from burnout syndrome. Specific strategies should be developed and implemented to limit and prevent professional burnout.

  8. MUNCHAUSEN SYNDROME BY PROXY IN PEDIATRIC DENTISTRY: MYTH OR REALITY?

    Directory of Open Access Journals (Sweden)

    Veronica PINTILICIUC-ŞERBAN

    2017-06-01

    Full Text Available Background and aims: Munchausen syndrome by proxy is a condition traditionally comprising physical and mental abuse and medical neglect as a form of psychogenic maltreatment of the child, secondary to fabrication of a pediatric illness by the parent or guardian. The aim of our paper is to assess whether such condition occurs in current pediatric dental practice and to evidence certain situations in which the pediatric dentist should suspect this form of child abuse. Problem statement: Munchausen syndrome by proxy in pediatric dentistry may lead to serious chronic disabilities of the abused or neglected child, being one of the causes of treatment failure. Discussion: Prompt detection of such condition should be regarded as one of the duties of the practitioner who should be trained to report the suspected cases to the governmental child protective agencies. This should be regarded as a form of child abuse and neglect, and the responsible caregiver could be held liable when such wrongful actions cause harm or endanger child’s welfare. Conclusion: Munchausen syndrome by proxy should be regarded as a reality in current pediatric dental practice and dental teams should be trained to properly recognize, assess and manage such complex situations.

  9. Ethical dimensions of genetics in pediatric neurology: a look into the future.

    Science.gov (United States)

    Avard, Denise M; Knoppers, Bartha M

    2002-03-01

    Health care providers and families with children who participate in genetic research or who need specialized genetic services, including genetic testing, will encounter not only medical but difficult social, ethical, and legal questions surrounding pediatric genetic neurology. Children are often at the center of much of the genetic revolution and their unique needs raise special concerns about the risks and benefits associated with genetic research, particularly the issues of consent, the use of genetic databases, and gene therapy. Moreover, genetic research and testing raise important psychosocial risks. In this article we discuss some of the benefits and consequences of genetic technologies for children in relation to national and international guidelines. In particular, physicians, policy-makers, and families should be knowledgeable about the guidelines and have a good understanding of the psychosocial and ethical issues associated with genetics in pediatric neurology.

  10. Reflections on Ethics and Humanity in Pediatric Neurology: the Value of Recognizing Ethical Issues in Common Clinical Practice.

    Science.gov (United States)

    Ronen, Gabriel M; Rosenbaum, Peter L

    2017-05-01

    Our goals in this reflection are to (i) identify the ethical dimensions inherent in any clinical encounter and (ii) bring to the forefront of our pediatric neurology practice the myriad of opportunities to explore and learn from these ethical questions. We highlight specifically Beauchamp and Childress's principles of biomedical ethics. We use the terms ethics in common clinical practice and an ethical lens to remind people of the ubiquity of ethical situations and the usefulness of using existing ethical principles to analyze and resolve difficult situations in clinical practice. We start with a few common situations with which many of us tend to struggle. We describe what we understand as ethics and how and why developments in technology, novel potential interventions, policies, and societal perspectives challenge us to think about and debate ethical issues. Individual patients are not a singular population; each patient has their own unique life situations, culture, goals, and expectations that need to be considered with a good dose of humanity and humility. We believe that using an ethical lens-by which we mean making an explicit effort to identify and consider these issues openly-will help us to achieve this goal in practice, education, and research.

  11. The Basic Nature of Ethical Problems Experienced by Persons with Acquired Immunodeficiency Syndrome: Implications for Nursing Ethics Education and Practice.

    Science.gov (United States)

    Cameron, Miriam E.; And Others

    1993-01-01

    Twenty-five persons with acquired immunodeficiency syndrome (AIDS) described and validated 100 ethical problems that are experienced by people with AIDS from 3 levels of ethical inquiry: descriptive ethics, normative ethics, and metaethics. Findings suggest strategies for improving nursing ethics education. (JOW)

  12. Comparison between a pediatric health promotion center and a pediatric obesity clinic in detecting metabolic syndrome and non-alcoholic fatty liver disease in children.

    Science.gov (United States)

    Yang, Hye Ran; Yi, Dae Yong; Choi, Hyoung Soo

    2014-12-01

    This study was done to evaluate the efficacy of health check-ups in children in detecting metabolic syndrome and non-alcoholic fatty liver disease (NAFLD) by comparing the pediatric health promotion center with the pediatric obesity clinic. Children who visited a pediatric health promotion center (n=218) or a pediatric obesity clinic (n=178) were included. Anthropometric data, blood pressure, laboratory tests, and abdominal ultrasonography were evaluated. Two different criteria were applied to diagnose metabolic syndrome. The prevalence of metabolic syndrome in the 2 units was 3.2%-3.7% in a pediatric health promotion center and 23%-33.2% in a pediatric obesity clinic. Significant differences were observed in the prevalence of each component of metabolic syndrome between the 2 units including abdominal adiposity, blood pressure, serum triglycerides, and fasting blood glucose (Pobesity clinic targeting obese children than that among patients visiting the health promotion center offering routine check-ups. An obesity-oriented approach is required to prevent obesity-related health problems in children.

  13. On the use of a continuous metabolic syndrome score in pediatric research

    Directory of Open Access Journals (Sweden)

    Eisenmann Joey C

    2008-06-01

    Full Text Available Abstract Background The constellation of elevated levels of abdominal adiposity, blood pressure, glucose, and triglycerides and lowered high-density lipoprotein-cholesterol has been termed the metabolic syndrome. Given the current pediatric obesity epidemic, it is perhaps not surprising that recent reports suggest the emergence of the metabolic syndrome during childhood and adolescence. The aim of this paper is to provide an overview of the derivation and utility of the continuous metabolic syndrome score in pediatric epidemiologic research. Methods/Design Data were generated from published papers related to the topic. Conclusion Although there is no universal definition in children or adolescence, recent estimates indicate that approximately 2–10% of youth possess the metabolic syndrome phenotype. Since there is no clear definition and the prevalence rate is relatively low, several authors have derived a continuous score representing a composite risk factor index (i.e., the metabolic syndrome score. This paper provides an overview of the derivation and utility of the continuous metabolic syndrome score in pediatric epidemiological research.

  14. Pediatric acute respiratory distress syndrome: Host factors in Down syndrome and the general population

    NARCIS (Netherlands)

    Bruijn, M.

    2013-01-01

    We find that Down syndrome is an important risk factor for developing acute respiratory distress syndrome (ARDS) in children, but the reason why remains to be elucidated. In addition, we find several differences between adult and pediatric ARDS. The association between C-reactive protein (CRP)

  15. A Study of Hypoalbuminemia and Pleural Effusionin Pediatric Nephrotic Syndrome

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    Tovan Perinandika

    2017-06-01

    Full Text Available Background: Nephrotic syndrome (NS is a kidney disease that is most often found in children. Hypoalbuminemia in NS can cause a decrease in oncotic pressure causing extravasation of fluid into the interstitial space. In conditions of severe hypoalbuminemia, fluid extravasation may cause occurrence of pleural effusion. The objectives of this study was to analyze the correlation between hypoalbuminemia and pleural effusion in children with NS. Methods: An analytical study was conducted on 69 medical records of pediatric nephrotic syndrome from 1 January 2008–31 December 2013 in dr. Hasan Sadikin General Hospital. Inclusion criteria were pediatric patients between 1-14 years old with NS. Exclusion criteria were patients who already had albumin transfusion, malnutrition, patients with chronic disease, and incomplete medical record information. Contingency coefficient test was carried out to discover the correlation between variables. Results: Out of 89 samples, 69 samples were included. Characteristics of the included patients are male (n=48, female (n=21, age 1–5 (n=24, 6–10 (n=22, 11–14 (n=23, mild hypoalbuminemia (n=3, moderate hypoalbuminemia (n=27, severe hypoalbuminemia (n=39, patients with pleural effusion (n=23, and non-pleural effusion (n=46. There was a significant correlation between  hypoalbuminemia and pleural effusion with p=0.000 (p<0.05 and moderate correlation (r=0.437. Conclusions: Hypoalbuminemia has correlation with pleural effusion in pediatric nephrotic syndrome. Keywords: Hypoalbuminemia, pediatric nephrotic syndrome, pleural effusion DOI: 10.15850/amj.v4n2.1075

  16. Pediatric irritable bowel syndrome patient and parental characteristics differ by care management type

    Science.gov (United States)

    This study evaluates whether certain patient or parental characteristics are associated with gastroenterology (GI) referral versus primary pediatrics care for pediatric irritable bowel syndrome (IBS). A retrospective clinical trial sample of patients meeting pediatric Rome III IBS criteria was assem...

  17. Vitamin D status in pediatric irritable bowel syndrome.

    Directory of Open Access Journals (Sweden)

    Benjamin Udoka Nwosu

    Full Text Available Irritable bowel syndrome (IBS is associated with significant morbidity in children and adolescents, and the therapeutic efficacy of available treatment options is limited. The role of vitamin D supplementation in pediatric IBS is unclear as the vitamin D status of pediatric patients with IBS is unknown. Equally, the relationship of vitamin D status with psychosomatic symptoms in children and adolescents is unclear.To characterize the vitamin D status of pediatric patients with IBS using a case-control study design.Serum 25-hydroxyvitamin D [25(OHD] concentration will be similar between patients with IBS and controls.A retrospective case-controlled study of 116 controls (age 14.6 ± 4.3 y, female (n = 67; 58% and 55 subjects with IBS (age 16.5 ± 3.1y, female (n = 44; 80%. Overweight was defined as BMI of ≥85th but 90% of IBS subjects had vitamin D deficiency at a cut-off point of 50% of the subjects with IBS had vitamin D deficiency. This is a much higher prevalence of vitamin D deficiency compared to IBD and other malabsorption syndromes. Monitoring for vitamin D deficiency should be part of the routine care for patients with IBS. Randomized control trials are warranted to determine the role of adjunctive vitamin D therapy in pediatric IBS.

  18. Neuroimaging experience in pediatric Horner syndrome

    International Nuclear Information System (INIS)

    Kadom, Nadja; Rosman, N.P.; Jubouri, Shams; Trofimova, Anna; Egloff, Alexia M.; Zein, Wadih M.

    2015-01-01

    Horner syndrome in children is rare. The frequency and spectrum of malignancy as the cause of Horner syndrome in children remains unclear. Also unclear is whether the imaging work-up should include the entire oculo-sympathetic pathway or should be more targeted. In addition, the value of cross-sectional angiographic imaging in Horner syndrome is uncertain. To review imaging pathology in a cohort of children with Horner syndrome at a major academic pediatric medical center. We reviewed a 22-year period of CT and MR imaging studies in children with a clinical diagnosis of Horner syndrome referred for imaging. We found 38 patients who fulfilled study criteria of Horner syndrome and 6/38 had relevant imaging findings: 2/6 etiologies were neoplastic (congenital neuroblastoma and central astrocytoma), 1/6 had a vascular abnormality (hypoplastic carotid artery), 1/6 had maldevelopment (Chiari I malformation), and 2/6 had inflammatory/traumatic etiology (viral cervical lymphadenopathy, post jugular vein cannulation). There was a similar number of congenital and acquired pathologies. The malignancies were found at any level of the oculosympathetic pathway. There are treatable causes, including malignancies, in children presenting with Horner syndrome, which justify imaging work-up of the entire oculosympathetic pathway, unless the lesion level can be determined clinically. (orig.)

  19. Neuroimaging experience in pediatric Horner syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Kadom, Nadja [Boston University School of Medicine, Department of Radiology, Boston University Medical Center, Boston, MA (United States); Rosman, N.P. [Boston Medical Center, Division of Pediatric Neurology, Departments of Pediatrics and Neurology, Boston University School of Medicine, Boston, MA (United States); Jubouri, Shams; Trofimova, Anna; Egloff, Alexia M. [Children' s National Medical Center, Department of Radiology and Diagnostic Imaging, Washington, DC (United States); Zein, Wadih M. [National Eye Institute (NEI), Bethesda, MD (United States)

    2015-09-15

    Horner syndrome in children is rare. The frequency and spectrum of malignancy as the cause of Horner syndrome in children remains unclear. Also unclear is whether the imaging work-up should include the entire oculo-sympathetic pathway or should be more targeted. In addition, the value of cross-sectional angiographic imaging in Horner syndrome is uncertain. To review imaging pathology in a cohort of children with Horner syndrome at a major academic pediatric medical center. We reviewed a 22-year period of CT and MR imaging studies in children with a clinical diagnosis of Horner syndrome referred for imaging. We found 38 patients who fulfilled study criteria of Horner syndrome and 6/38 had relevant imaging findings: 2/6 etiologies were neoplastic (congenital neuroblastoma and central astrocytoma), 1/6 had a vascular abnormality (hypoplastic carotid artery), 1/6 had maldevelopment (Chiari I malformation), and 2/6 had inflammatory/traumatic etiology (viral cervical lymphadenopathy, post jugular vein cannulation). There was a similar number of congenital and acquired pathologies. The malignancies were found at any level of the oculosympathetic pathway. There are treatable causes, including malignancies, in children presenting with Horner syndrome, which justify imaging work-up of the entire oculosympathetic pathway, unless the lesion level can be determined clinically. (orig.)

  20. Poor Adherence to Lung-Protective Mechanical Ventilation in Pediatric Acute Respiratory Distress Syndrome.

    Science.gov (United States)

    Ward, Shan L; Quinn, Carson M; Valentine, Stacey L; Sapru, Anil; Curley, Martha A Q; Willson, Douglas F; Liu, Kathleen D; Matthay, Michael A; Flori, Heidi R

    2016-10-01

    To determine the frequency of low-tidal volume ventilation in pediatric acute respiratory distress syndrome and assess if any demographic or clinical factors improve low-tidal volume ventilation adherence. Descriptive post hoc analysis of four multicenter pediatric acute respiratory distress syndrome studies. Twenty-six academic PICU. Three hundred fifteen pediatric acute respiratory distress syndrome patients. All patients who received conventional mechanical ventilation at hours 0 and 24 of pediatric acute respiratory distress syndrome who had data to calculate ideal body weight were included. Two cutoff points for low-tidal volume ventilation were assessed: less than or equal to 6.5 mL/kg of ideal body weight and less than or equal to 8 mL/kg of ideal body weight. Of 555 patients, we excluded 240 for other respiratory support modes or missing data. The remaining 315 patients had a median PaO2-to-FIO2 ratio of 140 (interquartile range, 90-201), and there were no differences in demographics between those who did and did not receive low-tidal volume ventilation. With tidal volume cutoff of less than or equal to 6.5 mL/kg of ideal body weight, the adherence rate was 32% at hour 0 and 33% at hour 24. A low-tidal volume ventilation cutoff of tidal volume less than or equal to 8 mL/kg of ideal body weight resulted in an adherence rate of 58% at hour 0 and 60% at hour 24. Low-tidal volume ventilation use was no different by severity of pediatric acute respiratory distress syndrome nor did adherence improve over time. At hour 0, overweight children were less likely to receive low-tidal volume ventilation less than or equal to 6.5 mL/kg ideal body weight (11% overweight vs 38% nonoverweight; p = 0.02); no difference was noted by hour 24. Furthermore, in the overweight group, using admission weight instead of ideal body weight resulted in misclassification of up to 14% of patients as receiving low-tidal volume ventilation when they actually were not. Low

  1. Shrinking lung syndrome complicating pediatric systemic lupus erythematosus

    Energy Technology Data Exchange (ETDEWEB)

    Burns, Natalie S. [University of Washington Medical Center, Department of Radiology, Seattle, WA (United States); Stevens, Anne M. [Seattle Children' s Hospital, Division of Rheumatology, Department of Pediatrics, Seattle, WA (United States); Iyer, Ramesh S. [University of Washington School of Medicine, Seattle Children' s Hospital, Department of Radiology, Seattle, WA (United States)

    2014-10-15

    Systemic lupus erythematosis (SLE) can affect the lungs and pleura, usually manifesting with pleural effusions or diffuse parenchymal disease. A rare manifestation of SLE is shrinking lung syndrome, a severe restrictive respiratory disorder. While pleuropulmonary complications of pediatric SLE are common, shrinking lung syndrome is exceedingly rare in children. We present a case of a 13-year-old girl previously diagnosed with lupus, who developed severe dyspnea on exertion and restrictive pulmonary physiology. Her chest radiographs on presentation demonstrated low lung volumes, and CT showed neither pleural nor parenchymal disease. Fluoroscopy demonstrated poor diaphragmatic excursion. While shrinking lung syndrome is described and studied in adults, there is only sparse reference to shrinking lung syndrome in children. (orig.)

  2. Shrinking lung syndrome complicating pediatric systemic lupus erythematosus

    International Nuclear Information System (INIS)

    Burns, Natalie S.; Stevens, Anne M.; Iyer, Ramesh S.

    2014-01-01

    Systemic lupus erythematosis (SLE) can affect the lungs and pleura, usually manifesting with pleural effusions or diffuse parenchymal disease. A rare manifestation of SLE is shrinking lung syndrome, a severe restrictive respiratory disorder. While pleuropulmonary complications of pediatric SLE are common, shrinking lung syndrome is exceedingly rare in children. We present a case of a 13-year-old girl previously diagnosed with lupus, who developed severe dyspnea on exertion and restrictive pulmonary physiology. Her chest radiographs on presentation demonstrated low lung volumes, and CT showed neither pleural nor parenchymal disease. Fluoroscopy demonstrated poor diaphragmatic excursion. While shrinking lung syndrome is described and studied in adults, there is only sparse reference to shrinking lung syndrome in children. (orig.)

  3. [Foetal therapy for Down syndrome: a pro-active ethical reflection].

    Science.gov (United States)

    de Wert, G M W R; Dondorp, W J

    2016-01-01

    Prenatal screening for Down syndrome has to date focused on facilitating the informed choice to continue or not with a pregnancy. The non-invasive prenatal test (NIPT) for Down syndrome does potentially offer the option to apply foetal neurocognitive therapy for Down syndrome (FTDS). Current research in animal models looks promising and therefore a proactive ethical reflection in relation to clinical trials is urgently needed. This discussion includes an exploration of the ethical aspects of FTDS. There seem to be no convincing a priori objections on the basis of the social model of disability. Arguments in terms of (respect for) autonomy, wellbeing and justice seem to in principle support such therapy. Still, both the conditions for sound clinical trials and the implications of possible effective therapy for current prenatal screening need further scrutiny.

  4. A New Ethical Challenge for Institutional Review Boards (IRBs/Ethics Committees (ECs in the Assessment of Pediatric Clinical Trials

    Directory of Open Access Journals (Sweden)

    Klaus Rose

    2015-05-01

    Full Text Available Both the US and EU have introduced pediatric pharmaceutical legislation to facilitate clinical trials in children and development of better medicines for children. The first concerns were published in 2014 that the European Medicines Agency (EMA’s Pediatric Committee (PDCO may be over-enthusiastic and has compelled questionable pediatric clinical trials from pharmaceutical companies. Numerous clinical trials are mandated in rare conditions for which not enough patients exist for even one trial. Furthermore, where these trials are mandated in adolescent patients, the legal age limit of the 18th birthday is confused with a medical age limit and can result in separate clinical trials in adolescent patients that neither make medical nor scientific sense nor will ever recruit enough patients for a meaningful outcome. To confirm our concerns we searched the registry clinicaltrials.gov and found examples for PDCO-triggered unethical trials. We conclude that such trials should not be accepted by institutional review boards (IRBs/ethics committees (ECs and that clinical trials resulting from negotiations with EMA’s PDCO need extra careful scrutiny by IRBs/ECs in order to prevent unethical studies and damage to pediatric research and unnecessary risks to pediatric patients.

  5. Unpasteurized Shared Human Milk Use in Pediatric Inpatients: Health and Ethical Implications.

    Science.gov (United States)

    Barbas, Kimberly H; Sussman-Karten, Karen; Kamin, Daniel; Huh, Susanna Y

    2017-06-01

    Growing evidence supporting the health benefits of human milk, particularly in the preterm population, has led to rising demand for donor human milk in NICUs and pediatric hospitals. There are no previous reports describing the use of unpasteurized shared human milk (USHM) in the hospital setting, but the use of USHM solicited from community donors through social networks appears to be common. Many pediatric hospitals permit inpatients to receive breast milk that has been screened and pasteurized by a human milk banking organization and will provide pasteurized donor human milk (PDHM) only to infants who are preterm or have specific medical conditions. These policies are designed to minimize potential adverse effects from improperly handled or screened donor milk and to target patients who would experience the greatest benefit in health outcomes with donor milk use. We explore the ethical and health implications of 2 cases of medically complex infants who did not meet criteria in our tertiary care hospital for the use of PDHM from a regulated human milk bank and were incidentally found to be using USHM. These cases raise questions about how best to balance the ethical principles of beneficence, nonmaleficence, justice, and patient autonomy in the provision of PDHM, a limited resource. Health care staff should ask about USHM use to provide adequate counseling about the risks and benefits of various feeding options in the context of an infant's medical condition. Copyright © 2017 by the American Academy of Pediatrics.

  6. Assessing and addressing moral distress and ethical climate Part II: neonatal and pediatric perspectives.

    Science.gov (United States)

    Sauerland, Jeanie; Marotta, Kathleen; Peinemann, Mary Anne; Berndt, Andrea; Robichaux, Catherine

    2015-01-01

    Moral distress remains a pervasive and, at times, contested concept in nursing and other health care disciplines. Ethical climate, the conditions and practices in which ethical situations are identified, discussed, and decided, has been shown to exacerbate or ameliorate perceptions of moral distress. The purpose of this mixed-methods study was to explore perceptions of moral distress, moral residue, and ethical climate among registered nurses working in an academic medical center. Two versions of the Moral Distress Scale in addition to the Hospital Ethical Climate Survey were used, and participants were invited to respond to 2 open-ended questions. Part I reported the findings among nurses working in adult acute and critical care units. Part II presents the results from nurses working in pediatric/neonatal units. Significant differences in findings between the 2 groups are discussed. Subsequent interventions developed are also presented.

  7. Conn syndrome and Crohn disease in a pediatric case: an interesting parallel.

    Science.gov (United States)

    Rodriguez, Manuel A; Alkhoury, Fuad; Malvezzi, Leopoldo; Diaz, Alejandro

    2013-01-01

    Conn syndrome is characterized by surreptitious secretion of aldosterone in which patients are found to have hypertension, hypokalemia, and metabolic alkalosis. Although rare, the most common presentation in the pediatric population is bilateral hyperplasia of the adrenal glands as opposed to an adenoma. Crohn disease is part of the spectrum of inflammatory bowel disease, which manifests in children as flare-ups of bloody diarrhea and abdominal pain. The association of concurrent Conn syndrome and Crohn disease has been previously presented in two cases in adults. This is the first pediatric case to be reported in the literature.

  8. Treating Lennox–Gastaut syndrome in epileptic pediatric patients with third-generation rufinamide

    Directory of Open Access Journals (Sweden)

    Jessica Gresham

    2010-09-01

    Full Text Available Jessica Gresham1, Lea S Eiland2,3, Allison M Chung2,41Auburn University, Harrison School of Pharmacy (AUHSOP, 2Department of Pharmacy Practice, AUHSOP, 3University of Alabama, School of Medicine, Huntsville Regional Medical Campus, 4University of South Alabama School of Medicine, Department of Pediatrics, Mobile, Alabama, USAAbstract: Lennox–Gastaut syndrome (LGS is a rare but debilitating pediatric epileptic encephalopathy characterized by multiple intractable seizure types. Treatment of LGS is challenging because of the small number of antiepileptic drugs (AEDs which are effective for this syndrome, as well as the need for polytherapy in the majority of patients. This review focuses on the treatment of LGS with rufinamide, a recently approved third-generation AED with reported efficacy as adjunctive therapy for LGS. All relevant papers identified through a PubMed search on the treatment of LGS with rufinamide were reviewed. To date, the literature suggests improvements in seizure frequency for pediatric patients with LGS on rufinamide. Rufinamide appears to be especially effective for atonic or drop attack seizures. Rufinamide also displays a favorable adverse event profile compared with the older anticonvulsants, as well as a minimal number of drug interactions, making it a promising option for the adjunctive treatment of seizures associated with LGS.Keywords: epilepsy, Lennox-Gastaut syndrome, pediatrics, seizure, rufinamide

  9. Metabolic syndrome in the pediatric population: a short overview

    Directory of Open Access Journals (Sweden)

    Natasa Marcun Varda

    2009-06-01

    Full Text Available The metabolic syndrome (MS in adults is defined as a concurrence of obesity, disturbed glucose and insulin metabolism, hypertension and dyslipidemia, and is associated with increased morbidity and mortality from cardiovascular diseases and type 2 diabetes. Studies now indicate that many of its components are also present in children and adolescents. Moreover, the clustering of these risk factors has been documented in some children, who are at increased cardiovascular risk in adulthood. The MS is highly prevalent among overweight children and adolescents. Identifying these children is important for early prevention and treatment of different components of the syndrome. The first-line treatment comprises lifestyle modification consisting of diet and exercise. The most effective tool for prevention of the MS is to stop the development of childhood obesity. The first attempt of consensus-based pediatric diagnostic criteria was published in 2007 by the International Diabetes Federation. Nevertheless, national prevalence data, based on uniform pediatric definition, protocols for prevention, early recognition and effective treatment of pediatric MS are still needed. The aim of this article is to provide a short overview of the diagnosis and treatment options of childhood MS, as well as to present the relationships between MS and its individual components.

  10. An update on Cushing syndrome in pediatrics.

    Science.gov (United States)

    Stratakis, Constantine A

    2018-04-09

    Cushing syndrome (CS) in childhood results mostly from the exogenous administration of glucocorticoids; endogenous CS is a rare disease. The latter is the main reason pediatric patients with CS escape diagnosis for too long. Other barriers to optimal care of a pediatric patient with CS include improper following of the proper sequence of testing for diagnosing CS, which stems from lack of understanding of pathophysiology of the hypothalamic-pituitary-adrenal axis; lack of access to proper (i.e., experienced, state-of-the-art) surgical treatment; and unavailability of well-tolerated and effective medications to control hypercortisolemia. This report reviews the state-of-the-art in diagnosing CS and provides an update on the most recent discoveries in its genetics and treatment. Copyright © 2018. Published by Elsevier Masson SAS.

  11. Ethical issues at the interface of clinical care and research practice in pediatric oncology: a narrative review of parents' and physicians' experiences.

    NARCIS (Netherlands)

    Vries, M.C. de; Houtlosser, M.; Wit, J.M.; Engberts, D.P.; Bresters, D.; Kaspers, G.J.L.; Leeuwen, E. van

    2011-01-01

    BACKGROUND: Pediatric oncology has a strong research culture. Most pediatric oncologists are investigators, involved in clinical care as well as research. As a result, a remarkable proportion of children with cancer enrolls in a trial during treatment. This paper discusses the ethical consequences

  12. Privacy and ethics in pediatric environmental health research-part I: genetic and prenatal testing.

    Science.gov (United States)

    Fisher, Celia B

    2006-10-01

    The pressing need for empirically informed public policies aimed at understanding and promoting children's health has challenged environmental scientists to modify traditional research paradigms and reevaluate their roles and obligations toward research participants. Methodologic approaches to children's environmental health research raise ethical challenges for which federal regulations may provide insufficient guidance. In this article I begin with a general discussion of privacy concerns and informed consent within pediatric environmental health research contexts. I then turn to specific ethical challenges associated with research on genetic determinants of environmental risk, prenatal studies and maternal privacy, and data causing inflicted insight or affecting the informational rights of third parties.

  13. Ethical issues at the interface of clinical care and research practice in pediatric oncology: a narrative review of parents' and physicians' experiences

    Directory of Open Access Journals (Sweden)

    de Vries Martine C

    2011-09-01

    Full Text Available Abstract Background Pediatric oncology has a strong research culture. Most pediatric oncologists are investigators, involved in clinical care as well as research. As a result, a remarkable proportion of children with cancer enrolls in a trial during treatment. This paper discusses the ethical consequences of the unprecedented integration of research and care in pediatric oncology from the perspective of parents and physicians. Methodology An empirical ethical approach, combining (1 a narrative review of (primarily qualitative studies on parents' and physicians' experiences of the pediatric oncology research practice, and (2 comparison of these experiences with existing theoretical ethical concepts about (pediatric research. The use of empirical evidence enriches these concepts by taking into account the peculiarities that ethical challenges pose in practice. Results Analysis of the 22 studies reviewed revealed that the integration of research and care has consequences for the informed consent process, the promotion of the child's best interests, and the role of the physician (doctor vs. scientist. True consent to research is difficult to achieve due to the complexity of research protocols, emotional stress and parents' dependency on their child's physician. Parents' role is to promote their child's best interests, also when they are asked to consider enrolling their child in a trial. Parents are almost never in equipoise on trial participation, which leaves them with the agonizing situation of wanting to do what is best for their child, while being fearful of making the wrong decision. Furthermore, a therapeutic misconception endangers correct assessment of participation, making parents inaccurately attribute therapeutic intent to research procedures. Physicians prefer the perspective of a therapist over a researcher. Consequently they may truly believe that in the research setting they promote the child's best interests, which maintains the

  14. Promoting an ethic of engagement in pediatric palliative care research.

    Science.gov (United States)

    Rahimzadeh, Vasiliki; Bartlett, Gillian; Longo, Cristina; Crimi, Laura; Macdonald, Mary Ellen; Jabado, Nada; Ells, Carolyn

    2015-10-16

    This paper defends the ethical and empirical significance of direct engagement with terminally ill children and adolescents in PPC research on health-related quality of life. Clinical trials and other forms of health research have resulted in tremendous progress for improving clinical outcomes among children and adolescents diagnosed with a life-threatening illness. Less attention has been paid, however, to engaging this patient population directly in studies aimed at optimizing health-related quality of life in PPC. Though not restricted to care at the end of life, PPC--and by extension PPC research--is in part dependent on recognizing the social complexities of death and dying and where health-related quality of life is a fundamental element. To explore these complexities in depth requires partnership with terminally ill children and adolescents, and acknowledgement of their active social and moral agency in research. Principles of pediatric research ethics, theoretical tenets of the "new sociology of the child(hood)," and human rights codified in the United Nations Convention on the Rights of the Child (UNCRC) underpin the position that a more engagement-centered approach is needed in PPC research. The ethics, sociologies and human rights of engagement will each be discussed as they relate to research with terminally ill children and adolescents in PPC. Qualitative method(ologies) presented in this paper, such as deliberative stakeholder consultations and phenomenology of practice can serve as meaningful vehicles for achieving i) participation among terminally ill children and adolescents; ii) evidence-bases for PPC best practices; and iii) fulfillment of research ethics principles. PPC research based on direct engagement with PPC patients better reflects their unique expertise and social epistemologies of terminal illness. Such an approach to research would strengthen both the ethical and methodological soundness of HRQoL inquiry in PPC.

  15. [Aortic root dilatation rate in pediatric patients with Marfan syndrome treated with losartan].

    Science.gov (United States)

    Mariucci, Elisabetta; Guidarini, Marta; Donti, Andrea; Lovato, Luigi; Wischmeijer, Anita; Angeli, Emanuela; Gargiulo, Gaetano D; Picchio, Fernando M; Bonvicini, Marco

    2015-12-01

    Medical therapy with angiotensin II receptor blockers/angiotensin-converting enzyme inhibitors and/or beta-blockers was reported to reduce aortic root dilatation rates in pediatric patients with Marfan syndrome. No data are available in the literature on losartan effects after 3 years of therapy. The aim of our study was to establish whether losartan reduces aortic root dilatation rates in pediatric patients with Marfan syndrome in the mid and long term. This is a retrospective analysis of 38 pediatric patients with Marfan syndrome followed at the Marfan Clinic of S. Orsola-Malpighi Hospital of the University of Bologna (Italy). Aortic diameters were measured at sinuses of Valsalva and proximal ascending aorta with transthoracic echocardiography. After a mean follow-up of 4.5 ± 2.5 years (range 2-9 years), aortic root z score at sinuses of Valsalva and proximal ascending aorta remained stable. The average annual rate of change in aortic root z score was -0.1 ± 0.4 and 0 ± 0.3 at sinuses of Valsalva and proximal ascending aorta, respectively. The mean dose of losartan was 0.7 ± 0.3 mg/kg/day. Three patients were non-responders, probably because of late beginning or low dose of therapy. Eight patients underwent cardiac surgery (aortic root surgery in 5 and mitral valve repair in 3), all of them started losartan later in life. Despite the retrospective design of the study and the small sample size, a beneficial effect of losartan therapy was observed in pediatric patients with Marfan syndrome in the mid and long term. Late beginning or low doses of losartan can turn off the effects of therapy.

  16. Metabolic Syndrome Components After Pediatric Liver Transplantation: Prevalence and the Impact of Obesity and Immunosuppression.

    Science.gov (United States)

    Perito, E R; Lustig, R H; Rosenthal, P

    2016-06-01

    Metabolic syndrome is associated with long-term morbidity and mortality after adult liver transplantation (LT). Whether pediatric LT recipients have a higher prevalence of metabolic syndrome remains controversial. In a cross-sectional study, we evaluated pediatric LT recipients aged 8-30 years using National Health and Nutrition Examination Survey (NHANES) protocols. LT recipients were matched by gender, race/ethnicity, and age with controls from NHANES. Pediatric LT recipients (n = 83), after adjusting for overweight/obesity and glucocorticoid use, had increased prevalence of prehypertension and hypertension, impaired glucose tolerance (IGT; 2-h glucose after oral glucose tolerance test ≥140 mg/dL), and low high-density lipoprotein compared to matched NHANES controls (n = 235) despite a lower prevalence of overweight/obesity. Among LT recipients, the adjusted odds of IGT doubled for every 7.5 years taking calcineurin inhibitors (odds ratio = 2.10, 95% confidence interval 1.06-4.17 per 7.5 years taking calcineurin inhibitors, p = 0.03). Among all subjects with IGT, LT recipients had a lower prevalence of overweight/obesity and less insulin resistance (homeostatic model assessment of insulin resistance) than did controls with IGT. Among normal weight subjects, LT recipients were significantly more likely than controls to have prehypertension/hypertension, IGT, low high-density lipoprotein, and metabolic syndrome. Pediatric LT recipients have unique metabolic syndrome profiles and risk factors and will require tailored screening and management protocols. © Copyright 2016 The American Society of Transplantation and the American Society of Transplant Surgeons.

  17. Aspectos éticos en Pediatría, las inmunodeficiencias primarias Ethical aspects in Pediatrics, the primary immunodeficiencies

    Directory of Open Access Journals (Sweden)

    Martha Leonor Paradoa Pérez

    2012-12-01

    Full Text Available Las inmunodeficiencias primarias son enfermedades genéticas, caracterizadas por infecciones crónicas devastadoras que conllevan a la muerte, al desarrollo de tumores y a enfermedades autoinmunes, y la mayoría de estas enfermedades cursan en la edad pediátrica. Desde la última década del pasado siglo, el desarrollo de la tecnología, el surgimiento del Proyecto del Genoma Humano y la profundización en los aspectos bioéticos, han dado lugar a especificidades en la atención médica e investigativa del paciente pediátrico y del que desarrolla una enfermedad inmunológica. Este trabajo recoge los aspectos bioéticos más importantes y los dilemas éticos con los que se enfrenta el inmunólogo pediátrico en la atención al paciente con inmunodeficiencia primaria, el cual demanda la atención de, prácticamente, todas las especialidades pediátricas.Primary immunodeficiencies are genetic diseases characterized by devastating chronic infections leading to death, tumor development and autoimmune diseases, and most of diseases occur at pediatric ages. Since the last decade of the past century, the development of technology, the emergence of the Human Genome Project and the in-depth consideration of bioethical aspects have given rise to specificities in the medical and research care for the pediatric patients and for those suffering immunological diseases. This paper covered the most important bioethical aspects and the ethical dilemmas that the pediatric immunologist faces when taking care of a primary immunodeficiency patient, who practically requires the attention of all the pediatric specialties.

  18. Neonatal and pediatric organ donation: ethical perspectives and implications for policy

    Directory of Open Access Journals (Sweden)

    Ajit Ashok Sarnaik

    2015-11-01

    Full Text Available The lifesaving processes of organ donation and transplantation in neonatology and pediatrics carry important ethical considerations. The medical community must balance the principles of autonomy, nonmaleficence, beneficence, and justice to ensure the best interest of the potential donor and to provide equitable benefit to society. Accordingly, the US Organ Procurement and Transplantation Network (OPTN has established procedures for the ethical allocation of organs depending on several donor-specific and recipient-specific factors. To maximize the availability of transplantable organs and opportunities for dying patients and families to donate, the US government has mandated that hospitals refer potential donors in a timely manner. Expedient investigation and diagnosis of brain death where applicable are also crucial, especially in neonates. Empowering trained individuals from organ procurement organizations to discuss organ donation with families has also increased rates of consent. Other efforts to increase organ supply include recovery from donors who die by circulatory criteria (DCDD in addition to donation after brain death (DBD, and from neonates born with immediately lethal conditions such as anencephaly. Ethical considerations in DCDD compared to DBD include a potential conflict of interest between the dying patient and others who may benefit from the organs, and the precision of the declaration of death of the donor. Most clinicians and ethicists believe in the appropriateness of the Dead Donor Rule, which states that vital organs should only be recovered from people who have died. The medical community can maximize the interests of organ donors and recipients by observing the Dead Donor Rule and acknowledging the ethical considerations in organ donation.

  19. Neonatal and Pediatric Organ Donation: Ethical Perspectives and Implications for Policy.

    Science.gov (United States)

    Sarnaik, Ajit A

    2015-01-01

    The lifesaving processes of organ donation and transplantation in neonatology and pediatrics carry important ethical considerations. The medical community must balance the principles of autonomy, non-maleficence, beneficence, and justice to ensure the best interest of the potential donor and to provide equitable benefit to society. Accordingly, the US Organ Procurement and Transplantation Network (OPTN) has established procedures for the ethical allocation of organs depending on several donor-specific and recipient-specific factors. To maximize the availability of transplantable organs and opportunities for dying patients and families to donate, the US government has mandated that hospitals refer potential donors in a timely manner. Expedient investigation and diagnosis of brain death where applicable are also crucial, especially in neonates. Empowering trained individuals from organ procurement organizations to discuss organ donation with families has also increased rates of consent. Other efforts to increase organ supply include recovery from donors who die by circulatory criteria (DCDD) in addition to donation after brain death (DBD), and from neonates born with immediately lethal conditions such as anencephaly. Ethical considerations in DCDD compared to DBD include a potential conflict of interest between the dying patient and others who may benefit from the organs, and the precision of the declaration of death of the donor. Most clinicians and ethicists believe in the appropriateness of the Dead Donor Rule, which states that vital organs should only be recovered from people who have died. The medical community can maximize the interests of organ donors and recipients by observing the Dead Donor Rule and acknowledging the ethical considerations in organ donation.

  20. Suspected Urine Leak in a Pediatric Renal Transplant Patient With Prune Belly Syndrome.

    Science.gov (United States)

    Liu, Bin; Kaplan, Summer L; Zhuang, Hongming

    2016-03-01

    Patients with prune belly syndrome usually have tortuous ureters, which can cause difficulty in the interpretation of renal scan used to evaluate possible urine leak after renal transplant. We reported a renal scan finding in a pediatric renal transplant patient with prune belly syndrome. The radioactivity in the dilated ureter, which was lateral to the renal transplant, appears to be urine leak.

  1. Pediatric sleep apnea

    Science.gov (United States)

    Sleep apnea - pediatric; Apnea - pediatric sleep apnea syndrome; Sleep-disordered breathing - pediatric ... Untreated pediatric sleep apnea may lead to: High blood pressure Heart or lung problems Slow growth and development

  2. [Case report : usefulness of the airwayscope for difficult intubations in a pediatric patients with Coffin-Siris syndrome].

    Science.gov (United States)

    Sakugawa, Yoko; Kamizato, Kota; Miyata, Yuji; Kakinohana, Manabu; Sugahara, Kazuhiro

    2013-05-01

    We experienced management of general anesthesia in a patients with Coffin-Siris syndrome (CS syndrome) which is an autosomal dominant disorder characterized by mental retardation, growth failure, hypoplasia of the fifth finger's distal phalanx and limb, and syndrome-specific facial appearance. Anesthesia was induced by sevoflurane by mask. After obtaining muscle relaxation by rocuronium, laryngoscopy by Machintosh #2 failed to reveal the vocal cord. However, the vocal cord was revealed by AirwayScope (AWS) for the pediatrics and then tracheal intubation was successful. Surgical procedures and anes-thetic management were performed uneventfully. This case demonstrates usefulness of AWS in pediatric patients with difficult intubation.

  3. Turner syndrome: transition from pediatrics to adulthood.

    Science.gov (United States)

    Rubin, Karen R

    2008-09-01

    To highlight the importance of an improved, seamless, and effective transition from pediatric to adult care, especially for medically complex conditions such as Turner syndrome (TS). The morbidities in adult patients with TS are reviewed, including features of the metabolic syndrome, congenital and acquired cardiovascular conditions, osteopenia and osteoporosis, autoimmune thyroid disease, and obesity, and psychobehavioral issues are addressed, in terms of promoting the development of independent self-care and autonomy in adolescent patients. An essential component of high-quality health care, transition for adolescents with TS needs to be reengineered as a staged process initiated during early-stage adolescence (about age 12 years), when exogenous estrogen therapy is begun in coordination with the final phase of growth hormone therapy. At this time, the focus of care shifts from the parent to the adolescent and from maximizing final adult height to inducing puberty with gradually increasing doses of estrogen. During this transition, the development of healthful and independent healthcare behaviors should be promoted to prepare patients with TS for the adult responsibility of self-care. During the final phase of transition, an adult care plan should be formulated in collaboration with the adolescent with TS and her providers of adult care to improve the likelihood that she will continue to be carefully monitored in a way that optimizes her adult health and longevity. The transitional period from pediatrics to adulthood is the ideal time for patients with TS to be made aware of their health history and health needs and of the evolving impact of TS into adulthood.

  4. Case report: Guillain-Barre syndrome with pneumococcus - A new association in pediatrics.

    Science.gov (United States)

    Khatib, Hassan El; Naous, Amal; Ghanem, Soha; Dbaibo, Ghassan; Rajab, Mariam

    2018-01-01

    Guillain-Barre Syndrome, an acute flaccid paralysis known to be caused by recent Gastro-intestinal infections mainly campylobacter, and Respiratory infections mainly mycoplasma pneumoniae and influenza. One reported case of severe invasive pneumococcal disease in a 68 year old female, that presented with Austrian's triad of meningitis, pneumonia and endocarditis, and progressed to develop Guillain Barre syndrome, an association never been documented before. We present a case of 13 year old male, presented with hypoactivity and inability to bare his own weight, developed septic shock due to pneumococcus with Acute Respiratory Distress Syndrome, and was found to have neurological findings of Guillain-Barre Syndrome. A new association in pediatric age group, never been reported before.

  5. Costello Syndrome and Umbilical Ligament Rhabdomyosarcoma in Two Pediatric Patients: Case Reports and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Carlos Sánchez-Montenegro

    2017-01-01

    Full Text Available Costello syndrome is caused by heterozygous de novo missense mutations in the protooncogene HRAS with tumor predisposition, especially rhabdomyosarcoma. We here report two pediatric patients with Costello syndrome and umbilical ligament rhabdomyosarcoma. A review of the literature published in English in MEDLINE from January 1971 to June 2016 using the search terms “Costello syndrome” and “rhabdomyosarcoma” was performed, including two new cases that we describe. Twenty-six patients with Costello syndrome and rhabdomyosarcoma were recorded with mean age of diagnosis of 2 years and 8 months. The most common tumor location was the abdomen/pelvis, including four out of ten of those in the umbilical ligament. The most common histological subtype was embryonal rhabdomyosarcoma. Overall survival was 43%. A total of 17 rhabdomyosarcomas in pediatric patients arising in the umbilical ligament were recorded with mean age of diagnosis of 3 years and 4 months. Overall survival was 69%. Costello syndrome is a poorly known disorder in pediatric oncology but their predisposition to malignancies implies the need for a new perspective on early diagnosis and aggressive medical and surgical treatment.

  6. Parry-Romberg syndrome in a pediatric patient. A case report.

    Directory of Open Access Journals (Sweden)

    Edgar Reyes

    2015-07-01

    Full Text Available The Parry-Romberg syndrome is a rare degenerative disease of unknown etiology that has dental implications. It is characterized by a progressive hemifacial atrophy that appears in the early stages of life. It causes aesthetic, functional and psychological alterations, and has social implications for the patient. There is no definitive treatment for the Parry-Romberg syndrome. Systemic and immune alterations produce oral and maxillofacial manifestations, which need to be managed by specialized dental professionals. The aim of this paper is to do a literature review of the Parry-Romberg syndrome and describe the oral and clinical characteristics of this condition in a 12-year-old male pediatric patient, who had a history of neurological disorders and facial asymmetry on the left side. Dentists require an adequate knowledge of the clinical and dental characteristics of this syndrome. With early diagnosis and appropriate surgical and orthodontic treatment at an early age, they can improve the quality of life of patients and minimize invasive long-term effects.

  7. Aggressive Fluid Resuscitation in Severe Pediatric Hyperglycemic Hyperosmolar Syndrome: A Case Report

    Directory of Open Access Journals (Sweden)

    Sharara-Chami Rana

    2010-03-01

    Full Text Available Objective. This report describes a severe case of hyperglycemic hyperosmolar syndrome complicated by rhabdomyolysis, acute kidney injury, hyperthermia, and hypovolemic shock, with management centred upon fluid administration. Design. Case report. Setting. Pediatric intensive care unit in university teaching hospital. Patients. 12 years old adolescent female presenting with hyperglycemic hyperosmolar syndrome with a new diagnosis of type 2 diabetes mellitus. Intervention. Aggressive fluid resuscitation and insulin. Main results. The patient had a good outcome, discharged home on hospital day 6. Conclusions. Hyperglycemic hyperosmolar syndrome is associated with a number of complications. Management strategies are undefined, given the rarity of its presentation, and further studies are warranted.

  8. Mortality in Pediatric Acute Respiratory Distress Syndrome: A Systematic Review and Meta-Analysis.

    Science.gov (United States)

    Wong, Judith Ju-Ming; Jit, Mark; Sultana, Rehena; Mok, Yee Hui; Yeo, Joo Guan; Koh, Jia Wen Janine Cynthia; Loh, Tsee Foong; Lee, Jan Hau

    2017-01-01

    Sparse and conflicting evidence exists regarding mortality risk from pediatric acute respiratory distress syndrome (ARDS). We aimed to determine the pooled mortality in pediatric ARDS and to describe its trend over time. MEDLINE, EMBASE, and Web of Science were searched from 1960 to August 2015. Keywords or medical subject headings (MESH) terms used included "respiratory distress syndrome, adult," "acute lung injury," "acute respiratory insufficiency," "acute hypoxemic respiratory failure," "pediatrics," and "child." Study inclusion criteria were (1) pediatric patients aged 0 days to 18 years, (2) sufficient baseline data described in the pediatric ARDS group, and (3) mortality data. Randomized controlled trials (RCTs) and prospective observational studies were eligible. Data on study characteristics, patient demographics, measures of oxygenation, and mortality were extracted using a standard data extraction form. Independent authors conducted the search, applied the selection criteria, and extracted the data. Methodological quality of studies was assessed. Meta-analysis using a random-effects model was performed to obtain pooled estimates of mortality. Meta-regression was performed to analyze variables contributing to change in mortality over time. Eight RCTs and 21 observational studies (n = 2274 patients) were included. Pooled mortality rate was 24% (95% confidence interval [CI]: 19-31). There was a decrease in mortality rates over 3 epochs (≤2000, 2001-2009, and ≥2010: 40% [95% CI: 24-59], 35% [95% CI: 21-51], and 18% [95% CI: 12-26], respectively, P < .001). Observational studies reported a higher mortality rate than RCTs (27% [95% CI: 24-29] versus 16% [95% CI: 12-20], P < .001). Earlier year of publication was an independent factor associated with mortality. Overall mortality rate in pediatric ARDS is approximately 24%. Studies conducted and published later were associated with better survival.

  9. Pediatric Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Halted by Etanercept.

    Science.gov (United States)

    Gavigan, Geneviève M; Kanigsberg, Nordau D; Ramien, Michele L

    2018-02-01

    We report a case of an 11-year-old female with Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) overlap, most likely triggered by sulfamethoxazole-trimethoprim, who was treated with the combination of methylprednisolone, cyclosporine, and etanercept. Her condition stabilized and her skin involvement did not progress after the addition of etanercept. To our knowledge, this is the first report of etanercept for pediatric SJS/TEN.

  10. Gastric leiomyoma in a child with Gorlin-Goltz syndrome: First pediatric case.

    Science.gov (United States)

    Virgone, Calogero; Decker, Emily; Mitton, Sally G; Mansour, Sahar; Giuliani, Stefano

    2016-04-01

    Gorlin-Goltz syndrome (GGS), also known as nevoid basal cell carcinoma syndrome (MIM 109 400), is a rare genetic condition with a prevalence between 1/56 000 and 1/256 000. Clinical presentation is usually characterized by multiple basal cell carcinomas, odontogenic jaw keratocysts, palmar or plantar pitting and skeletal anomalies. It is furthermore associated with the development of various tumors beside basal cell carcinoma, among which medulloblastoma is the most frequent. Increased incidence of other mesenchymal neoplasms, however, is also well known: recently the first adult case of gastric leiomyoma in GGS was reported, and the inclusion of "fibromas and leiomyomas of other organs" in the minor criteria for the diagnosis was suggested. We report the first case of a pediatric patient with GGS who also developed a gastric leiomyoma: the present case illustrates the need for this change to the diagnostic criteria to encompass the highly variable presentations and phenotype in GGS. © 2015 Japan Pediatric Society.

  11. Burnout syndrome during residency in internal medicine and pediatrics in a country without working time directive.

    Science.gov (United States)

    Aksoy, Duygu Yazgan; Durusu Tanriover, Mine; Unal, Sule; Dizdar, Omer; Kalyoncu, Umut; Karakaya, Jale; Unal, Serhat; Kale, Gulsev

    2014-01-01

    The purpose of this paper is to demonstrate burnout syndrome among internal medicine and pediatrics residents in a country that does not have the working time directive (WTD) and also to determine the risk factors and consequent impact on efficient functioning in clinical areas. A 57-item questionnaire was given to internal medicine and pediatrics residents. Responses from 22 pediatrics and 33 internal medicine residents were evaluated. Demographic findings, burnout scores, having hobbies, social activities and reading books unrelated to medicine were similar between the two groups. Six pediatrics residents (27.3 per cent) and 11 (33.3 per cent) internal medicine residents met the criteria for clinically significant burnout. Personal accomplishment scores and reading books unrelated to medicine were found to be related to burnout. Burnout is a syndrome characterized by depersonalization, emotional exhaustion and a low sense of personal accomplishment. It is important to document burnout in countries where WTDs are not implemented. Further studies might demonstrate burnout's effect on patient safety, service quality and physician's performance.

  12. Pediatric palliative care for youth with HIV/AIDS: systematic review of the literature

    Directory of Open Access Journals (Sweden)

    Wilkins ML

    2013-07-01

    Full Text Available Megan L Wilkins,1 Ronald H Dallas,1 Kathleen E Fanone,2 Maureen E Lyon3,4 1St Jude Children's Research Hospital, Department of Infectious Diseases, Memphis, TN, USA; 2Johns Hopkins Medical Center, Department of Pediatric Medicine, Baltimore, MD, USA; 3Children's National Medical Center, 4George Washington University School of Medicine and Health Sciences, Washington, DC, USA Abstract: Improvement in treatment has led to decreased death in youth with human immunodeficiency virus (HIV in developed countries. Despite this, youth with HIV are still at risk for increased mortality and morbidity compared with their uninfected counterparts. In developing countries, high numbers of youth die from acquired immune deficiency syndrome (AIDS-related illnesses due to lack of access to consistent antiretroviral treatment. As a result, pediatric palliative care is a relevant topic for those providing care to youth with HIV. A systematic review was conducted to gather information regarding the status of the literature related to pediatric palliative care and medical decision-making for youth with HIV. The relevant literature published between January 2002 and June 2012 was identified through searches conducted using PubMed, CINAHL, Scopus, and PSYCInfo databases and a series of key words. Articles were reviewed by thematic analysis using the pillars of palliative care set out by the National Consensus Project. Twenty-one articles were retained after review and are summarized by theme. In general, few empirically based studies evaluating palliative care and medical decision-making in youth with HIV were identified. Articles identified focused primarily on physical aspects of care, with less attention paid to psychological, social, ethical, and cultural aspects of care. We recommend that future research focuses on broadening the evaluation of pediatric palliative care among youth with HIV by directly evaluating the psychological, social, ethical, and cultural

  13. Pediatric imaging in DICER1 syndrome

    International Nuclear Information System (INIS)

    Bueno, Marta Tijerin; Martinez-Rios, Claudia; Ahyad, Rayan A.; Greer, Mary-Louise C.; Puente Gregorio, Alejandro de la; Villani, Anita; Malkin, David; Druker, Harriet; Van Engelen, Kalene; Gallinger, Bailey; Aronoff, Laura; Grant, Ronald

    2017-01-01

    DICER1 syndrome, arising from a mutation in the DICER1 gene mapped to chromosome 14q32, is associated with an increased risk of a range of benign and malignant neoplasms. To determine the spectrum of abnormalities and imaging characteristics in patients with DICER1 syndrome at a tertiary pediatric hospital. This retrospective analysis evaluated imaging in patients ≤18 years with DICER1 germline variants between January 2004 and July 2016. An imaging database search including keywords pleuropulmonary blastoma, cystic nephroma, pineoblastoma, embryonal rhabdomyosarcoma, ovarian sex cord-stromal tumor, ovarian Sertoli-Leydig cell tumor and DICER1 syndrome, was cross-referenced against the institutional Cancer Genetics Program database, excluding patients with negative/unknown DICER1 gene testing. Sixteen patients were included (12 females; mean age at presentation: 4.2 years, range: 14 days to 17 years), with surveillance imaging encompassing the following modalities: chest X-ray and CT; abdominal, pelvic and neck US; and brain and whole-body MRI. Malignant lesions (68.8% of patients) included pleuropulmonary blastoma (5), pineoblastoma (3), ovarian Sertoli-Leydig cell tumor (1), embryonal rhabdomyosarcoma (1) and renal sarcoma (1); benign lesions (37.5% of patients) included thyroid cysts (2), thyroid nodules (2), cystic nephroma (2), renal cysts (1) and pineal cyst (1). A common lesional appearance observed across modalities and organs was defined as the ''cracked windshield'' sign. The spectrum of DICER1-related tumors and the young age at presentation suggest early surveillance of at-risk patients is critical, while minimizing exposure to ionizing radiation. (orig.)

  14. Pediatric imaging in DICER1 syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Bueno, Marta Tijerin; Martinez-Rios, Claudia; Ahyad, Rayan A.; Greer, Mary-Louise C. [The Hospital for Sick Children, Department of Diagnostic Imaging, Toronto, Ontario (Canada); University of Toronto, Department of Medical Imaging, Toronto, Ontario (Canada); Puente Gregorio, Alejandro de la [Hospital Son Espases, Radiotherapy Department, Palma de Mallorca (Spain); Villani, Anita; Malkin, David [University of Toronto, Department of Pediatrics, Toronto, Ontario (Canada); The Hospital for Sick Children, Division of Hematology/Oncology, Toronto, Ontario (Canada); The Hospital for Sick Children, Genetics and Genomic Biology Program, Toronto, Ontario (Canada); Druker, Harriet [The Hospital for Sick Children, Division of Hematology/Oncology, Toronto, Ontario (Canada); The Hospital for Sick Children, Department of Genetic Counselling, Toronto, Ontario (Canada); The Hospital for Sick Children, Department of Molecular Genetics, Toronto, Ontario (Canada); Van Engelen, Kalene [The Hospital for Sick Children, Genetics and Genomic Biology Program, Toronto, Ontario (Canada); Gallinger, Bailey [The Hospital for Sick Children, Genetics and Genomic Biology Program, Toronto, Ontario (Canada); The Hospital for Sick Children, Department of Genetic Counselling, Toronto, Ontario (Canada); The Hospital for Sick Children, Department of Molecular Genetics, Toronto, Ontario (Canada); Aronoff, Laura [The Hospital for Sick Children, Division of Hematology/Oncology, Toronto, Ontario (Canada); Grant, Ronald [University of Toronto, Department of Pediatrics, Toronto, Ontario (Canada); The Hospital for Sick Children, Division of Hematology/Oncology, Toronto, Ontario (Canada)

    2017-09-15

    DICER1 syndrome, arising from a mutation in the DICER1 gene mapped to chromosome 14q32, is associated with an increased risk of a range of benign and malignant neoplasms. To determine the spectrum of abnormalities and imaging characteristics in patients with DICER1 syndrome at a tertiary pediatric hospital. This retrospective analysis evaluated imaging in patients ≤18 years with DICER1 germline variants between January 2004 and July 2016. An imaging database search including keywords pleuropulmonary blastoma, cystic nephroma, pineoblastoma, embryonal rhabdomyosarcoma, ovarian sex cord-stromal tumor, ovarian Sertoli-Leydig cell tumor and DICER1 syndrome, was cross-referenced against the institutional Cancer Genetics Program database, excluding patients with negative/unknown DICER1 gene testing. Sixteen patients were included (12 females; mean age at presentation: 4.2 years, range: 14 days to 17 years), with surveillance imaging encompassing the following modalities: chest X-ray and CT; abdominal, pelvic and neck US; and brain and whole-body MRI. Malignant lesions (68.8% of patients) included pleuropulmonary blastoma (5), pineoblastoma (3), ovarian Sertoli-Leydig cell tumor (1), embryonal rhabdomyosarcoma (1) and renal sarcoma (1); benign lesions (37.5% of patients) included thyroid cysts (2), thyroid nodules (2), cystic nephroma (2), renal cysts (1) and pineal cyst (1). A common lesional appearance observed across modalities and organs was defined as the ''cracked windshield'' sign. The spectrum of DICER1-related tumors and the young age at presentation suggest early surveillance of at-risk patients is critical, while minimizing exposure to ionizing radiation. (orig.)

  15. Gender eugenics? The ethics of PGD for intersex conditions.

    Science.gov (United States)

    Sparrow, Robert

    2013-01-01

    This article discusses the ethics of the use of preimplantation genetic diagnosis (PGD) to prevent the birth of children with intersex conditions/disorders of sex development (DSDs), such as congenital adrenal hyperplasia (CAH) and androgen insensitivity syndrome (AIS). While pediatric surgeries performed on children with ambiguous genitalia have been the topic of intense bioethical controversy, there has been almost no discussion to date of the ethics of the use of PGD to reduce the prevalence of these conditions. I suggest that PGD for those conditions that involve serious medical risks for those born with them is morally permissible and that PGD for other "cosmetic" variations in sexual anatomy is more defensible than might first appear. However, importantly, the arguments that establish the latter claim have radical and disturbing implications for our attitude toward diversity more generally.

  16. Care of Pediatric Neurosurgical Patients in Iraq in 2007: Clinical and Ethical Experience of a Field Hospital

    Science.gov (United States)

    2010-09-01

    penetrating spine injury      •      bioethics Abbreviations used in this paper: EMDG = Expeditionary Medical Group; GCS = Glasgow Coma Scale; GOS...select group of high-acuity patients capable of consuming significant medical resources in a deployed environment. This information has the potential to...impact medical planning, logistics, and policy. 25 256 Care of pediatric neurosurgical patients in Iraq in 2007: clinical and ethical experience of

  17. Pediatric Electrocardiographic Imaging (ECGI) Applications

    Science.gov (United States)

    Silva, Jennifer N. A.

    2014-01-01

    Summary Noninvasive electrocardiographic imaging (ECGI) has been used in pediatric and congenital heart patients to better understand their electrophysiologic substrates. In this article we focus on the 4 subjects related to pediatric ECGI: 1) ECGI in patients with congenital heart disease and Wolff-Parkinson-White syndrome, 2) ECGI in patients with hypertrophic cardiomyopathy and pre-excitation, 3) ECGI in pediatric patients with Wolff-Parkinson-White syndrome, and 4) ECGI for pediatric cardiac resynchronization therapy. PMID:25722754

  18. Cellulitis as complication of nephrotic syndrome in a pediatric patient

    Science.gov (United States)

    Siregar, R. S.; Daulay, K. R.; Siregar, B.; Ramayani, O. R.; Eyanoer, P. C.

    2018-03-01

    Nephrotic syndrome is a chronic disease that may act as a risk for other major infection in skin, respiratory and urinary tract, while also increasingthe chance for other diseases, like peritonitis, meningitis, and cellulitis. Cellulitis is often caused by Streptococcus β-hemolytic, Staphylococcus aureus, and Escherichia coli. The clinical features of cellulitis marked with redness rash and well-defined borders, pain pressure and swelling. Hypoalbuminemia which occurs due to proteinuria occurred in this patient acts as a risk factor for cellulitis. It has been reported the case of cellulitis as one of the complications of the nephrotic syndrome in the pediatric patient. The treatment has been given to the patient such as antibiotics and supportive therapy and also planned albumin substitution.

  19. [Maxillofacial and dental abnormalities in some multiple abnormality syndromes. "Cri du chat" syndrome, Wilms' tumor-aniridia syndrome; Sotos syndrome; Goldenhar syndrome].

    Science.gov (United States)

    Berio, A; Trucchi, R; Meliota, M

    1992-05-01

    The paper describes the maxillo-facial and dental anomalies observed in some chromosome and non-chromosome poly-malformative syndromes ("Cri du chat" syndrome; Wilms' tumour; Sotos' syndrome; Goldenhar's syndrome). The Authors emphasise the possibility of diagnosing these multiple deformity syndromes from maxillo-facial alterations in early infancy; anomalous tooth position and structure cal also be successfully treated immediately after the first appearance of teeth. This is a particularly promising field of pediatrics and preventive pediatric medicine.

  20. Evaluation of Retinal Changes Using Optical Coherence Tomography in a Pediatric Case of Susac Syndrome

    Directory of Open Access Journals (Sweden)

    Mehmet Kola

    2017-01-01

    Full Text Available Susac syndrome is a rare occlusive vasculopathy affecting the retina, inner ear and brain. The cause is unknown, although it generally affects young women. This syndrome can be difficult to diagnose because its signs can only be revealed by detailed examination. These signs are not always concomitant, but may appear at different times. This report describes a pediatric case who was diagnosed with Susac syndrome when retinal lesions were identified in the inactive period with the help of optical coherence tomography (OCT. The purpose of this case report is to emphasize the importance of OCT in clarifying undefined retinal changes in Susac syndrome.

  1. The Ethics of Vaccination Nudges in Pediatric Practice.

    Science.gov (United States)

    Navin, Mark C

    2017-03-01

    Techniques from behavioral economics-nudges-may help physicians increase pediatric vaccine compliance, but critics have objected that nudges can undermine autonomy. Since autonomy is a centrally important value in healthcare decision-making contexts, it counts against pediatric vaccination nudges if they undermine parental autonomy. Advocates for healthcare nudges have resisted the charge that nudges undermine autonomy, and the recent bioethics literature illustrates the current intractability of this debate. This article rejects a principle to which parties on both sides of this debate sometimes seem committed: that nudges are morally permissible only if they are consistent with autonomy. Instead, I argue that, at least in the case of pediatric vaccination, some autonomy-undermining nudges may be morally justified. This is because parental autonomy in pediatric decision-making is not as morally valuable as the autonomy of adult patients, and because the interests of both the vaccinated child and other members of the community can sometimes be weighty enough to justify autonomy-infringing pediatric vaccination nudges. This article concludes with a set of worries about the effect of pediatric vaccination nudges on parent-physician relationships, and it calls on the American Academy of Pediatrics to draw on scientific and bioethics research to develop guidelines for the use of nudges in pediatric practice and, in particular, for the use of pediatric vaccination nudges.

  2. Use of Hemadsorption in a Case of Pediatric Toxic Shock Syndrome

    Directory of Open Access Journals (Sweden)

    Andrea Berkes

    2017-01-01

    Full Text Available Background. Toxic shock syndrome is a potentially fatal toxin-mediated disease. The role of toxins in this clinical entity made us hypothesize that extracorporeal blood purification with CytoSorb® could play a beneficial role in the clinical management of toxic shock syndrome. This case report describes the successful treatment of toxic shock syndrome using a combination of renal replacement therapy and hemadsorption in a pediatric patient. Case Presentation. A 5-year-old girl with Down’s syndrome presented with an inflamed area surrounding an insect bite, signs of systemic inflammation, and multiple organ failure. As previous attempts of immune modulation therapy were unsuccessful, renal replacement therapy was supplemented by the cytokine absorber CytoSorb. Treatment using this combination was associated with a rapid and significant stabilization in the hemodynamic situation and a decrease in inflammatory mediators within hours after the initiation of therapy. The application of CytoSorb therapy was simple and safe. Conclusion. The use of extracorporeal blood purification with CytoSorb proved potentially beneficial by removing toxins and inflammatory mediators in this case and could therefore play a role in the clinical management of toxic shock syndrome. Whether CytoSorb has the potential to even positively influence mortality in patients with toxic shock syndrome still needs to be confirmed.

  3. Pediatric biobanking

    DEFF Research Database (Denmark)

    Salvaterra, Elena; Giorda, Roberto; Bassi, Maria T

    2012-01-01

    Ethical, legal, and social issues related to the collection, storage, and use of biospecimens and data derived from children raise critical concerns in the international debate. So far, a number of studies have considered a variety of the individual issues crucial to pediatric biobanking such as ......Ethical, legal, and social issues related to the collection, storage, and use of biospecimens and data derived from children raise critical concerns in the international debate. So far, a number of studies have considered a variety of the individual issues crucial to pediatric biobanking...... such as decision making, privacy protection, minor recontact, and research withdrawal by focusing on theoretical or empirical perspectives. Our research attempted to analyze such issues in a comprehensive manner by exploring practices, rules, and researcher opinions regarding proxy consent, minor assent, specimens...

  4. Pediatric Chronic Abdominal Pain and Median Arcuate Ligament Syndrome: A Review and Psychosocial Comparison.

    Science.gov (United States)

    Mak, Grace Zee; Lucchetti, Amanda R; Drossos, Tina; Fitzsimmons-Craft, Ellen E; Accurso, Erin C; Stiles-Shields, Colleen; Newman, Erika A; Skelly, Christopher L

    2016-07-01

    Chronic abdominal pain (CAP) occurs in children and adolescents with a reported prevalence of 4% to 41% with significant direct and indirect costs to the child, family, and society. Median arcuate ligament syndrome (MALS) is a vascular compression syndrome of the celiac artery that may cause symptoms of epigastric pain and weight loss and is a frequently overlooked cause of CAP in the pediatric population. We have observed that the psychosocial presentation of patients with MALS is notable for various psychiatric comorbidities. In this article, we review MALS as well as our study results of the psychosocial profile of 30 MALS patients. Our data suggest that children and adolescents with MALS have similar psychosocial profiles to children with other gastrointestinal disorders resulting in CAP. The overlap of physical and psychosocial symptoms of patients who have MALS with other CAP disorders leads us to recommend that patients with CAP should be evaluated for MALS. [Pediatr Ann. 2016;45(7):e257-e264.]. Copyright 2016, SLACK Incorporated.

  5. Pediatric Ramsay Hunt Syndrome: Analysis of Three Cases

    Directory of Open Access Journals (Sweden)

    İmran Aydoğdu

    2015-01-01

    Full Text Available Ramsay Hunt syndrome (RHS is a disorder characterized by herpetic eruptions on the auricle, facial paralysis, and vestibulocochlear dysfunction and is attributed to varicella zoster virus (VZV infection in the geniculate ganglion. Although it is a common cause of acute peripheral facial paralysis, children are not usually affected. The diagnosis is based on history and physical findings. Treatment of RHS uses a combination of high-dose corticosteroids and acyclovir. This paper presents three cases diagnosed as RHS in the pediatric age group in association with the literature review. The aim of this paper is to emphasize the importance of careful examination and early initiation of therapy in suspected cases of RHS.

  6. Pediatric Ramsay Hunt Syndrome: Analysis of Three Cases

    Science.gov (United States)

    Aydoğdu, İmran; Ataç, Enes; Saltürk, Ziya; Atar, Yavuz; Özdemir, Erdi; Arslanoğlu, Ahmet; Berkiten, Güler

    2015-01-01

    Ramsay Hunt syndrome (RHS) is a disorder characterized by herpetic eruptions on the auricle, facial paralysis, and vestibulocochlear dysfunction and is attributed to varicella zoster virus (VZV) infection in the geniculate ganglion. Although it is a common cause of acute peripheral facial paralysis, children are not usually affected. The diagnosis is based on history and physical findings. Treatment of RHS uses a combination of high-dose corticosteroids and acyclovir. This paper presents three cases diagnosed as RHS in the pediatric age group in association with the literature review. The aim of this paper is to emphasize the importance of careful examination and early initiation of therapy in suspected cases of RHS. PMID:26435868

  7. Pediatric Nonfracture Acute Compartment Syndrome: A Review of 39 Cases.

    Science.gov (United States)

    Livingston, Kristin; Glotzbecker, Michael; Miller, Patricia E; Hresko, Michael T; Hedequist, Daniel; Shore, Benjamin J

    2016-01-01

    Compartment syndrome in the absence of fracture is rare and poorly described within the pediatric literature. The purpose of this study was to report the varying etiologies, risk factors, and treatment outcomes associated with pediatric nonfracture acute compartment syndrome (NFACS). We conducted a retrospective chart review on 37 children who suffered a NFACS and were treated at a single pediatric trauma center between 1997 and 2013. Demographic, diagnostic, treatment, and outcome characteristics were reviewed. Five causal groups were generated: trauma, exercise related (acute presentation after exercise without trauma), infectious, vascular, and postoperative (in the absence of osteotomy). Univariate and multivariate analyses were performed to identify risk factors of NFACS. P-values 39 cases of NFRCS in 37 children [6 females, 31 males, mean age of 11.7 y (SD+7.2 y)]. The leg was the most commonly involved limb (29 cases, 74%). Diagnosis of NFRCS was made either by compartment pressure monitoring [59%, 23/39 cases, mean pressure 66 mm Hg (SD+28)] or by clinical examination. According to etiology, vascular was most common (11/39, 28%), followed by trauma (10/39, 26%) and postoperative (8/39, 21%), with exertion and infection representing a small proportion (6/39, 15% and 4/39, 10%, respectively). Pain was present in 33 cases (85%), swelling in 28 cases (72%), paresthesias in 13 cases (33%), motor deficit in 12 cases (31%), and poor perfusion in 11 cases (28%). Average time from symptom onset to diagnosis was 48 hours (IQR, 9 to 96 h). At surgery, 21 patients (54%) had evidence of myonecrosis. Children required an average of 3 surgeries for wound closure. The median time to follow-up was 232 days (IQR, 73 to 608 d). A total of 54% made a full recovery, whereas 31% suffered a persistent neurological or functional deficit. NFACS in children is associated with a delay in diagnosis and a high rate of myonecrosis. Timely assessment with high clinical suspicion is

  8. Science and ethics of human immunodeficiency virus/acquired immunodeficiency syndrome controversies in Africa.

    Science.gov (United States)

    Brewster, David

    2011-09-01

    The human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) epidemic in Africa has raised important ethical issues for both researchers and clinicians. The most notorious controversy has been related to the zidovudine (AZT) trials in Africa in the late 1990s, in which the control groups were given a placebo rather than an effective drug to prevent vertical transmission. This raised concerns in the sponsoring country about exploitation of subjects, injustice and an ethical double standard between donor countries and resource-poor settings. However, the real double standard is between clinical practice standards in Western versus African countries, which must be addressed as part of the increasing global inequity of wealth both between countries and also within countries. There are important limitations to ethical declarations, principles and guidelines on their own without contextual ethical reasoning. The focus on research ethics with the HIV epidemic has led to a relative neglect of ethical issues in clinical practice. Although the scientific advances in HIV/AIDS have changed the ethical issues since the 1990s, there has also been progress in the bioethics of HIV/AIDS in terms of ethical review capability by local committees as well as in exposure to ethical issues by clinicians and researchers in Africa. However, serious concerns remain about the overregulation of research by bureaucratic agencies which could discourage African research on specifically African health issues. There is also a need for African academic institutions and researchers to progressively improve their research capacity with the assistance of research funders and donor agencies. © 2011 The Author. Journal of Paediatrics and Child Health © 2011 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

  9. Toward a Child Rights Theory in Pediatric Bioethics.

    Science.gov (United States)

    Goldhagen, Jeffrey; Mercer, Raul; Webb, Elspeth; Nathawad, Rita; Shenoda, Sherry; Lansdown, Gerison

    2016-01-01

    This article offers a child rights theory in pediatric bioethics, applying the principles, standards, and norms of child rights, health equity, and social justice to medical and ethical decision-making. We argue that a child rights theory in pediatric bioethics will help pediatricians and pediatric bioethicists analyze and address the complex interplay of biomedical and social determinants of child health. These core principles, standards and norms, grounded in the U.N. Convention on the Rights of the Child (CRC), provide the foundational elements for the theory and a means for better understanding the complex determinants of children's health and well-being. Rights-based approaches to medical and ethical decision-making provide strategies for applying and translating these elements into the practice of pediatrics and pediatric bioethics by establishing a coherent, consistent, and contextual theory that is relevant to contemporary practice. The proposed child rights theory extends evolving perspectives on the relationship between human rights and bioethics to both child rights and pediatric bioethics.

  10. Infectious component of the pediatric acute-onset neuropsychiatric syndrome (PANS in terms of evidence-based medicine principles (review of literature

    Directory of Open Access Journals (Sweden)

    L.O. Bezrukov

    2017-04-01

    Full Text Available The first clinical cases of obsessive-compulsive di­sorder and/or tic disorder in children with acute sudden onset associated with infectious diseases have been named pediatric infection-triggered autoimmune neuropsychiatric disorders (PITANDS. The relationship of such neuropsychiatric manifestations with preceding infectious diseases caused by group A beta-hemolytic Streptococcus was the most important, and it has been called paediatric autoimmune neuropsychiatric disorder associated with streptococcal infection (PANDAS. Due to the low level of evidence of the research on the relationship of infectious agents with neurological and behavioral symptoms with an acute onset, since 2014 another syndrome is diagnosed in children — pediatric acute-onset neuropsychiatric syndrome (PANS. Currently, the question about infectious etiology, pathogenesis and autoimmune mechanisms of these paediatric neuropsychiatric syndromes are still debatable.

  11. Home-Based Hypnotherapy Self-exercises vs Individual Hypnotherapy With a Therapist for Treatment of Pediatric Irritable Bowel Syndrome, Functional Abdominal Pain, or Functional Abdominal Pain Syndrome: A Randomized Clinical Trial

    NARCIS (Netherlands)

    Rutten, Juliette M. T. M.; Vlieger, Arine M.; Frankenhuis, Carla; George, Elvira K.; Groeneweg, Michael; Norbruis, Obbe F.; Tjon A ten, Walther; van Wering, Herbert M.; Dijkgraaf, Marcel G. W.; Merkus, Maruschka P.; Benninga, Marc A.

    2017-01-01

    Individual gut-directed hypnotherapy (HT) is effective in pediatric irritable bowel syndrome (IBS) and functional abdominal pain or functional abdominal pain syndrome (FAP[S]). It is, however, unavailable to many children. To compare the effectiveness of HT by means of home-based self-exercises

  12. Withdrawal syndrome associated with cessation of fentanyl and midazolam in pediatrics

    OpenAIRE

    Bicudo, J.n. [UNIFESP; Souza, N. de [UNIFESP; Mângia, C.m.f. [UNIFESP; Carvalho, Werther Brunow de [UNIFESP

    1999-01-01

    PURPOSE: To determine the incidence of abstinence syndrome in children interned in the Pediatric Intensive Care Unit (PICU) in fentanyl use and midazolam METHODS: Evaluation of 36 children interned in PICU of the Hospital São Paulo - Federal University of São Paulo, in the period from March to September 1997, with age varying from 5 days to 22 months (22 masc: 14 fem) who used fentanyl use and midazolam for more than 24 hours. Used the Escore Neonatal of Abstinence adapted by Finnegan determi...

  13. Pediatric Acute Respiratory Distress Syndrome: Fluid Management in the PICU.

    Science.gov (United States)

    Ingelse, Sarah A; Wösten-van Asperen, Roelie M; Lemson, Joris; Daams, Joost G; Bem, Reinout A; van Woensel, Job B

    2016-01-01

    The administration of an appropriate volume of intravenous fluids, while avoiding fluid overload, is a major challenge in the pediatric intensive care unit. Despite our efforts, fluid overload is a very common clinical observation in critically ill children, in particular in those with pediatric acute respiratory distress syndrome (PARDS). Patients with ARDS have widespread damage of the alveolar-capillary barrier, potentially making them vulnerable to fluid overload with the development of pulmonary edema leading to prolonged course of disease. Indeed, studies in adults with ARDS have shown that an increased cumulative fluid balance is associated with adverse outcome. However, age-related differences in the development and consequences of fluid overload in ARDS may exist due to disparities in immunologic response and body water distribution. This systematic review summarizes the current literature on fluid imbalance and management in PARDS, with special emphasis on potential differences with adult patients. It discusses the adverse effects associated with fluid overload and the corresponding possible pathophysiological mechanisms of its development. Our intent is to provide an incentive to develop age-specific fluid management protocols to improve PARDS outcomes.

  14. Nutrition: A Primary Therapy in Pediatric Acute Respiratory Distress Syndrome

    Directory of Open Access Journals (Sweden)

    Bryan Wilson

    2016-10-01

    Full Text Available Appropriate nutrition is an essential component of intensive care management of children with Acute Respiratory Distress Syndrome (ARDS and is linked to patient outcomes. One out of every two children in the PICU will develop malnutrition or have worsening of baseline malnutrition, and present with specific micronutrient deficiencies. Early and adequate enteral nutrition (EN is associated with improved 60-day survival after pediatric critical illness and yet, despite early EN guidelines, critically ill children receive on average only 55% of goal calories by PICU day 10. Inadequate delivery of EN is due to perceived feeding intolerance, reluctance to enterally feed children with hemodynamic instability, and fluid restriction. Underlying each of these factors is large practice variation between providers and across institutions for initiation, advancement and maintenance of EN. Strategies to improve early initiation, advancement, and to maintain delivery of EN are needed to improve morbidity and mortality from pediatric ARDS. Both over and underfeeding prolongs duration of mechanical ventilation in children and worsens other organ function such that precise calorie goals are needed. The gut is thought to act as a ‘motor’ of organ dysfunction and emerging data regarding the role of intestinal barrier functions and the intestinal microbiome on organ dysfunction and outcomes of critical illness present exciting opportunities to improve patient outcomes. Nutrition should be considered a primary rather than supportive therapy for pediatric ARDS. Precise nutritional therapies, which are titrated and targeted to preservation of intestinal barrier function, prevention of intestinal dysbiosis, preservation of lean body mass, and blunting of the systemic inflammatory response, offer great potential for improving outcomes of pediatric ARDS. In this review we examine the current evidence regarding dose, route, and timing of nutrition, current

  15. Frequency of Usher syndrome in two pediatric populations: Implications for genetic screening of deaf and hard of hearing children.

    Science.gov (United States)

    Kimberling, William J; Hildebrand, Michael S; Shearer, A Eliot; Jensen, Maren L; Halder, Jennifer A; Trzupek, Karmen; Cohn, Edward S; Weleber, Richard G; Stone, Edwin M; Smith, Richard J H

    2010-08-01

    Usher syndrome is a major cause of genetic deafness and blindness. The hearing loss is usually congenital and the retinitis pigmentosa is progressive and first noticed in early childhood to the middle teenage years. Its frequency may be underestimated. Newly developed molecular technologies can detect the underlying gene mutation of this disorder early in life providing estimation of its prevalence in at risk pediatric populations and laying a foundation for its incorporation as an adjunct to newborn hearing screening programs. A total of 133 children from two deaf and hard of hearing pediatric populations were genotyped first for GJB2/6 and, if negative, then for Usher syndrome. Children were scored as positive if the test revealed > or =1 pathogenic mutations in any Usher gene. Fifteen children carried pathogenic mutations in one of the Usher genes; the number of deaf and hard of hearing children carrying Usher syndrome mutations was 15/133 (11.3%). The population prevalence was estimated to be 1/6000. Usher syndrome is more prevalent than has been reported before the genome project era. Early diagnosis of Usher syndrome has important positive implications for childhood safety, educational planning, genetic counseling, and treatment. The results demonstrate that DNA testing for Usher syndrome is feasible and may be a useful addition to newborn hearing screening programs.

  16. A nationwide survey of pediatric acquired demyelinating syndromes in Japan

    Science.gov (United States)

    Yamaguchi, Y.; Kira, R.; Ishizaki, Y.; Sakai, Y.; Sanefuji, M.; Ichiyama, T.; Oka, A.; Kishi, T.; Kimura, S.; Kubota, M.; Takanashi, J.; Takahashi, Y.; Tamai, H.; Natsume, J.; Hamano, S.; Hirabayashi, S.; Maegaki, Y.; Mizuguchi, M.; Minagawa, K.; Yoshikawa, H.; Kira, J.; Kusunoki, S.; Hara, T.

    2016-01-01

    Objective: To investigate the clinical and epidemiologic features of pediatric acquired demyelinating syndromes (ADS) of the CNS in Japan. Methods: We conducted a nationwide survey and collected clinical data on children with ADS aged 15 years or younger, who visited hospitals between 2005 and 2007. Results: Among 977 hospitals enrolled, 723 (74.0%) responded to our inquiries and reported a total of 439 patients as follows: 244 with acute disseminated encephalomyelitis (ADEM), 117 with multiple sclerosis (MS), 14 with neuromyelitis optica (NMO), and 64 with other ADS. We collected and analyzed detailed data from 204 cases, including those with ADEM (66), MS (58), and NMO (10). We observed the following: (1) the estimated annual incidence rate of pediatric ADEM in Japan was 0.40 per 100,000 children (95% confidence interval [CI], 0.34–0.46), with the lowest prevalence in the north; (2) the estimated prevalence rate of MS was 0.69 per 100,000 children (95% CI, 0.58–0.80), with the lowest prevalence in the south; (3) NMO in Japan was rare, with an estimated prevalence of 0.06 per 100,000 children (95% CI, 0.04–0.08); and (4) the sex ratio and mean age at onset varied by ADS type, and (5) male/female ratios correlated with ages at onset in each ADS group. Conclusions: Our results clarify the characteristic clinical features of pediatric ADS in the Japanese population. PMID:27742816

  17. Scalp Lesions in a Pediatric Patient with Hyper IgM Syndrome: Clinical and Histologic Mimicry of Cryptococcus neoformans Infection.

    Science.gov (United States)

    Acker, Karen P; Fetch, Audrey; Schnell, Stephanie A; Hammond, Jennifer; Herrera, Christina; Niedt, George; Ratner, Adam J; Lauren, Christine T

    2018-01-01

    We report a case of cutaneous cryptococcosis due to Cryptococcus neoformans in a pediatric patient with hyper IgM syndrome with scalp lesions that resembled tinea capitis on gross examination and mimicked juvenile xanthogranuloma on histologic examination. This case highlights the importance of considering cutaneous cryptococcosis in patients with hyper IgM syndrome. Copyright © 2017 Elsevier Inc. All rights reserved.

  18. Causes of Pediatric Cardiomyopathy

    Science.gov (United States)

    ... inherited metabolic or congenital muscle disorder such as Noonan syndrome, Pompe disease, fatty acid oxidation defect or Barth ... where a specific chromosome is deleted or duplicated. Noonan syndrome is the most common form associated with pediatric ...

  19. Pediatric acute respiratory distress syndrome: fluid management in the PICU

    Directory of Open Access Journals (Sweden)

    Sarah A Ingelse

    2016-03-01

    Full Text Available The administration of an appropriate volume of intravenous fluids, while avoiding fluid overload, is a major challenge in the pediatric intensive care unit. Despite our efforts, fluid overload is a very common clinical observation in critically ill children, in particular in those with pediatric acute respiratory distress syndrome (PARDS. Patients with ARDS have widespread damage of the alveolar capillary barrier, potentially making them vulnerable to fluid overload with the development of pulmonary edema leading to prolonged course of disease. Indeed, studies in adults with ARDS have shown that an increased cumulative fluid balance is associated with adverse outcome. However, age-related differences in the development and consequences of fluid overload in ARDS may exist due to disparities in immunologic response and body water distribution. This systematic review summarizes the current literature on fluid imbalance and management in PARDS, with special emphasis on potential differences with adult patients. It discusses the adverse effects associated with fluid overload and the corresponding possible pathophysiological mechanisms of its development. Our intent is to provide an incentive to develop age-specific fluid management protocols to improve PARDS outcomes.

  20. The impact of pediatric nephrotic syndrome on families.

    Science.gov (United States)

    Mitra, Sulagna; Banerjee, Sushmita

    2011-08-01

    The objective of our study was to assess the psychologic and economic effects of pediatric nephrotic syndrome (NS) on caregivers. Caregivers of 50 children with NS were compared with a control group of 50 families of children with minor illnesses attending the same outpatient facility. Beck's Depression Inventory (BDI) IA was used to assess the mental status of the primary caregiver. The socioeconomic status of the family was assessed using the modified Kuppuswamy scale. Expenditure for the illness was calculated during parent interviews. The difference between groups was analyzed using analysis of variance (ANOVA) and Duncan's multiple range test. BDI scores signified moderate to severe depression in 48% of NS caregivers compared with 12% controls. The mean BDI score was significantly higher in NS caregivers, correlating positively with disease severity and negatively with socioeconomic status. Expenditure for disease also was significantly higher in families with NS patients, varying between 30% and 60% of monthly income depending on disease severity compared with 6.9% in controls. In 10% of NS families, it was more than total income, forcing families to break into savings or go into debt. Although pediatric NS most commonly has an excellent long-term outcome, it causes significant mental and economic stress on families. Severe forms should be categorized as a chronic illness and be eligible for disability benefits and subsidized travel and medical care. Establishing support groups and supportive care at local levels would help reduce the burden on families of patients wtih NS.

  1. The impact of metabolic syndrome on child weight outcomes in pediatric obesity program for Mexican Americans

    Science.gov (United States)

    Adults with metabolic syndrome (MetS) are three to five times more likely to develop type 2 diabetes and cardiovascular disease. Given the long term consequences of MetS, the growing number of children meeting criteria for MetS is concerning. In order to determine the impact of MetS on pediatric wei...

  2. Variability in Usual Care Mechanical Ventilation for Pediatric Acute Respiratory Distress Syndrome: Time for a Decision Support Protocol?

    Science.gov (United States)

    Newth, Christopher J L; Sward, Katherine A; Khemani, Robinder G; Page, Kent; Meert, Kathleen L; Carcillo, Joseph A; Shanley, Thomas P; Moler, Frank W; Pollack, Murray M; Dalton, Heidi J; Wessel, David L; Berger, John T; Berg, Robert A; Harrison, Rick E; Holubkov, Richard; Doctor, Allan; Dean, J Michael; Jenkins, Tammara L; Nicholson, Carol E

    2017-11-01

    Although pediatric intensivists philosophically embrace lung protective ventilation for acute lung injury and acute respiratory distress syndrome, we hypothesized that ventilator management varies. We assessed ventilator management by evaluating changes to ventilator settings in response to blood gases, pulse oximetry, or end-tidal CO2. We also assessed the potential impact that a pediatric mechanical ventilation protocol adapted from National Heart Lung and Blood Institute acute respiratory distress syndrome network protocols could have on reducing variability by comparing actual changes in ventilator settings to those recommended by the protocol. Prospective observational study. Eight tertiary care U.S. PICUs, October 2011 to April 2012. One hundred twenty patients (age range 17 d to 18 yr) with acute lung injury/acute respiratory distress syndrome. Two thousand hundred arterial and capillary blood gases, 3,964 oxygen saturation by pulse oximetry, and 2,757 end-tidal CO2 values were associated with 3,983 ventilator settings. Ventilation mode at study onset was pressure control 60%, volume control 19%, pressure-regulated volume control 18%, and high-frequency oscillatory ventilation 3%. Clinicians changed FIO2 by ±5 or ±10% increments every 8 hours. Positive end-expiratory pressure was limited at ~10 cm H2O as oxygenation worsened, lower than would have been recommended by the protocol. In the first 72 hours of mechanical ventilation, maximum tidal volume/kg using predicted versus actual body weight was 10.3 (8.5-12.9) (median [interquartile range]) versus 9.2 mL/kg (7.6-12.0) (p Ventilator management varies substantially in children with acute respiratory distress syndrome. Opportunities exist to minimize variability and potentially injurious ventilator settings by using a pediatric mechanical ventilation protocol offering adequately explicit instructions for given clinical situations. An accepted protocol could also reduce confounding by mechanical

  3. Pediatric Guillain-Barré syndrome: Indicators for a severe course

    Directory of Open Access Journals (Sweden)

    Muhammet Ali Varkal

    2015-01-01

    Full Text Available Objectives: This study aims to retrospectively evaluate pediatric Guillain-Barré syndrome cases in a tertiary center in Istanbul, Turkey. Materials and Methods: The data of 40 patients with Guillain-Barré syndrome who had been admitted to the Department of Pediatrics at the Istanbul University Medical Faculty between 2005 and 2011 were collected. Mann-Whitney U, Kruskal-Wallis, chi-square, and Fisher′s exact tests were used for statistical analysis. Results: Mean patient age was 5.4 ± 3.0 years; 20 out of 40 patients (50% were female and 20 (50% were male. Preceding infection was detected in 32 cases (80%. Six patients had speech impairment. Out of eight patients with respiratory distress (20%, five required respiratory support (12.5% of which three of them had speech impairment as well. According to nerve conduction studies, 21 patients (52.5% had acute inflammatory demyelinating polyradiculoneuropathy, 14 (35% had acute motor axonal neuropathy, and five (12.5% had acute motor-sensory axonal neuropathy. Thirty-three patients (82.5% received intravenous immunglobulin, 3 (7.5% underwent plasmapheresis and 4 (10% received both. Time until recovery (P = 0.022 and time until aided (P = 0.036 and unaided (P = 0.027 walking were longer in patients with acute gastrointestinal infection than in those with upper respiratory tract infection (P < 0.05. Time until response to treatment (P = 0.001, time until aided (P = 0.001 and unaided (P = 0.002 walking, and time until complete recovery (P = 0.002 were longer in acute motor axonal neuropathy cases as compared to acute inflammatory demyelinating polyradiculoneuropathy cases. Conclusion: Recovery was longer with acute gastrointestinal infection and acute motor axonal neuropathy. Speech impairment could be a clinical clue for the need of mechanical ventilation.

  4. Taking Assent to New Heights: A Case Report on Do-Not-Resuscitate Status in Pediatric Palliative Care Procedures.

    Science.gov (United States)

    Ballard, Heather A; Suresh, Santhanam

    2018-03-01

    Dying adolescents presenting for palliative procedures have complicated developmental and ethical issues, especially when reconsidering do-not-resuscitate orders. Though the American Academy of Pediatrics has guidelines, there is limited information in the literature on how to take care of these patients. We describe the case of a 14-year-old patient presenting to the interventional radiology suite for management of superior vena cava syndrome. The patient's goals of treatment were elucidated through a comprehensive care team consisting of the procedural and oncology teams. Effective communication with the patient and family was paramount for success.

  5. Pediatric catastrophic antiphospholipid syndrome: descriptive analysis of 45 patients from the "CAPS Registry".

    Science.gov (United States)

    Berman, Horacio; Rodríguez-Pintó, Ignasi; Cervera, Ricard; Gregory, Simone; de Meis, Ernesto; Rodrigues, Carlos Ewerton Maia; Aikawa, Nádia Emi; de Carvalho, Jozélio Freire; Springer, Janusz; Niedzwiecki, Maciej; Espinosa, Gerard

    2014-02-01

    Given the lack of information about catastrophic antiphospholipid syndrome (APS) in pediatric patients, the objective of the current study was to describe the clinical characteristics, laboratory features, treatment, and outcome of pediatric patients with catastrophic APS and compare them with the adult patients with catastrophic APS. We identified patients who were under 18years of age at time of catastrophic APS diagnosis included in the international registry of patients with catastrophic APS (CAPS Registry). Their main demographic and clinical characteristics, laboratory features, treatment, and outcome were described and compared with those of adult patients with catastrophic APS. From the 446 patients included in the CAPS Registry as of May 2013, 45 (10.3%) patients developed 46 catastrophic events before 18years of age (one patient presented two episodes). Overall, 32 (71.1%) patients were female and the mean age was 11.5±4.6years (range, 3months-18years). A total of 31 (68.9%) patients suffered from primary APS and 13 (28.9%) from systemic lupus erythematosus (SLE). The main differences between the two groups of patients were the higher prevalence of infections as precipitating factor for catastrophic event in the pediatric population (60.9% versus 26.8% in the adult population, p<0.001) and of peripheral vessel thrombosis (52.2% versus 34.3%, p=0.017). In addition, catastrophic APS was the first manifestation of APS more frequently in pediatric patients (86.6% versus 45.2%, p<0.001). Interestingly, pediatric patients showed a trend of lower mortality, although the difference was not statistically significant (26.1% versus 40.2%; odds ratio, 1.9; 95% confidence interval, 0.96-3.79; p=0.063). No differences were found neither in the laboratory features nor in the isolated or combination treatments between groups. Catastrophic APS in pediatric patients is a rare disease. There are minimal differences in the clinical and laboratory features, treatment, and

  6. Coordinated Pediatric and Periodontal Dental Care of a Child with Down syndrome.

    Science.gov (United States)

    Byrd, Gentry; Quinonez, Rocio B; Offenbacher, Steven; Keels, Martha Ann; Guthmiller, Janet M

    2015-01-01

    The purpose of this report was to describe the management of an eight-year-old Bulgarian male with Down syndrome presenting with periodontitis as a manifestation of systemic disease in the early mixed dentition. Treatment involved full-mouth mechanical debridement and extraction of hopeless teeth under general anesthesia followed by systemic antibiotics and chemical adjunctive therapy. Microbial culture and sensitivity testing aided in diagnosis and guided treatment decisions. This case report demonstrates a multidisciplinary approach in the management of aggressive periodontal disease in an internationally adopted pediatric patient with special health care needs.

  7. The pragmatics of feeding the pediatric patient with acute respiratory distress syndrome.

    Science.gov (United States)

    Verger, Judy T; Bradshaw, Darla J; Henry, Elizabeth; Roberts, Kathryn E

    2004-09-01

    Acute respiratory distress syndrome (ARDS) represents the ultimate pulmonary response to a wide range of injuries, from septicemia to trauma. Optimal nutrition is vital to enhancing oxygen delivery, supporting adequate cardiac contractility and respiratory musculature, eliminating fluid and electrolyte imbalances, and supporting the proinflammatory response. Research is providing a better understanding of nutrients that specifically address the complex physiologic changes in ARDS. This article highlights the pathophysiology of ARDS as it relates to nutrition, relevant nutritional assessment, and important enteral and parenteral considerations for the pediatric patient who has ARDS.

  8. Inhibitory actions of the gamma-aminobutyric acid in pediatric Sturge-Weber syndrome.

    Science.gov (United States)

    Tyzio, Roman; Khalilov, Ilgam; Represa, Alfonso; Crepel, Valerie; Zilberter, Yuri; Rheims, Sylvain; Aniksztejn, Laurent; Cossart, Rosa; Nardou, Romain; Mukhtarov, Marat; Minlebaev, Marat; Epsztein, Jérôme; Milh, Mathieu; Becq, Helene; Jorquera, Isabel; Bulteau, Christine; Fohlen, Martine; Oliver, Viviana; Dulac, Olivier; Dorfmüller, Georg; Delalande, Olivier; Ben-Ari, Yehezkel; Khazipov, Roustem

    2009-08-01

    The mechanisms of epileptogenesis in Sturge-Weber syndrome (SWS) are unknown. We explored the properties of neurons from human pediatric SWS cortex in vitro and tested in particular whether gamma-aminobutyric acid (GABA) excites neurons in SWS cortex, as has been suggested for various types of epilepsies. Patch-clamp and field potential recordings and dynamic biphoton imaging were used to analyze cortical tissue samples obtained from four 6- to 14-month-old pediatric SWS patients during surgery. Neurons in SWS cortex were characterized by a relatively depolarized resting membrane potential, as was estimated from cell-attached recordings of N-methyl-D-aspartate channels. Many cells spontaneously fired action potentials at a rate proportional to the level of neuronal depolarization. The reversal potential for GABA-activated currents, assessed by cell-attached single channel recordings, was close to the resting membrane potential. All spontaneously firing neurons recorded in cell-attached mode or imaged with biphoton microscopy were inhibited by GABA. Spontaneous epileptiform activity in the form of recurrent population bursts was suppressed by glutamate receptor antagonists, the GABA(A) receptor agonist isoguvacine, and the positive allosteric GABA(A) modulator diazepam. Blockade of GABA(A) receptors aggravated spontaneous epileptiform activity. The NKCC1 antagonist bumetanide had little effect on epileptiform activity. SWS cortical neurons have a relatively depolarized resting membrane potential and spontaneously fire action potentials that may contribute to increased network excitability. In contrast to previous data depicting excitatory and proconvulsive actions of GABA in certain pediatric and adult epilepsies, GABA plays mainly an inhibitory and anticonvulsive role in SWS pediatric cortex.

  9. Ethics Review of Pediatric Multi-Center Drug Trials

    NARCIS (Netherlands)

    Needham, Allison C.; Kapadia, Mufiza Z.; Offringa, Martin

    2015-01-01

    The assessment of safety and efficacy of therapeutics for children and adolescents requires the use of multi-centered designs. However, the need to obtain ethical approval from multiple independent research ethics boards (REBs) presents as a challenge to investigators and sponsors who must consider

  10. Trends in standard workup performed by pediatric subspecialists for the diagnosis of adolescent polycystic ovary syndrome.

    Science.gov (United States)

    Powers, Sarah E; Uliassi, Nicole W; Sullivan, Shannon D; Tuchman, Lisa K; Mehra, Rinku; Gomez-Lobo, Veronica

    2015-02-01

    The purpose of this study is to identify trends in the clinical workup, diagnosis, and treatment of polycystic ovary syndrome by pediatric endocrinologists, pediatric gynecologists, and adolescent medicine specialists. Retrospective chart review. Tertiary care medical center. Females aged 11-18 y who were evaluated for PCOS from June 2009 to October 2011 were included. Any patients with coexisting diagnoses of other primary etiology for amenorrhea were excluded. Patients were identified by ICD-9 codes for PCOS, hypersecretion of ovarian androgens, irregular menses, hirsutism, oligomenorrhea, or amenorrhea. 261 patients were included: 144 from endocrinology, 9 from gynecology, and 108 from adolescent pediatric practices. There were no significant differences in the androgen labs ordered by the subspecialties. Gynecologists ordered pelvic ultrasonography for 89% (n = 8) of patients, compared to 9% (n = 10) by adolescent medicine specialists and 24% (n = 34) by endocrinologists (P PCOS with metformin (58%, n = 66), compared to gynecologists (14%, n = 1) and adolescent medicine specialists (5%, n = 3) (P PCOS are evident among pediatric subspecialties, reflecting lack of standardized care for adolescents. Quantifying outcomes based on diagnostic and therapeutic approaches are important next steps. Copyright © 2015 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  11. Comparison of Prevalence and Outcomes of Pediatric Acute Respiratory Distress Syndrome Using Pediatric Acute Lung Injury Consensus Conference Criteria and Berlin Definition.

    Science.gov (United States)

    Gupta, Samriti; Sankar, Jhuma; Lodha, Rakesh; Kabra, Sushil K

    2018-01-01

    Our objective was to compare the prevalence and outcomes of pediatric acute respiratory distress syndrome using the Pediatric Acute Lung Injury Consensus Conference (PALICC) criteria and Berlin definitions. We screened case records of all children aged 1 month to 17 years of age admitted to the Pediatric Intensive Care Unit (PICU) over a 3-year period (2015-2017) for presence of any respiratory difficulty at admission or during PICU stay. We applied both PALICC and Berlin criteria to these patients. Data collection included definition and outcome related variables. Data were compared between the "PALICC only group" and the "Berlin with or without PALICC" group using Stata 11. Of a total of 615 admissions, 246 were identified as having respiratory difficulty at admission or during PICU stay. A total of 61 children (prevalence 9.9%; 95% CI: 7.8-12.4) fulfilled the definition of acute respiratory distress syndrome (ARDS) with either of the two criteria. While 60 children (98%) fulfilled PALICC criteria, only 26 children (43%) fulfilled Berlin definition. There was moderate agreement between the two definitions (Kappa: 0.51; 95% CI: 0.40-0.62; observed agreement 85%). Greater proportion of patients had severe ARDS in the "Berlin with or without PALICC group" as compared to the "PALICC only" group (50 vs. 19%). There was no difference between the groups with regard to key clinical outcomes such as duration of ventilation (7 vs. 8 days) or mortality [51.4 vs. 57.7%: RR (95% CI): 0.99 (0.64-1.5)]. In comparison to Berlin definition, the PALICC criteria identified more number of patients with ARDS. Proportion with severe ARDS and complications was greater in the "Berlin with or without PALICC" group as compared to the "PALICC only" group. There were no differences in clinical outcomes between the groups.

  12. Pediatric Donation After Circulatory Determination of Death: A Scoping Review.

    Science.gov (United States)

    Weiss, Matthew J; Hornby, Laura; Witteman, William; Shemie, Sam D

    2016-03-01

    Although pediatric donation after circulatory determination of death is increasing in frequency, there are no national or international donation after circulatory determination of death guidelines specific to pediatrics. This scoping review was performed to map the pediatric donation after circulatory determination of death literature, identify pediatric donation after circulatory determination of death knowledge gaps, and inform the development of national or regional pediatric donation after circulatory determination of death guidelines. Terms related to pediatric donation after circulatory determination of death were searched in Embase and MEDLINE, as well as the non-MEDLINE sources in PubMed from 1980 to May 2014. Seven thousand five hundred ninety-seven references were discovered and 85 retained for analysis. All references addressing pediatric donation after circulatory determination of death were considered. Exclusion criteria were articles that did not address pediatric patients, animal or laboratory studies, surgical techniques, and local pediatric donation after circulatory determination of death protocols. Narrative reviews and opinion articles were the most frequently discovered reference (25/85) and the few discovered studies were observational or qualitative and almost exclusively retrospective. Retained references were divided into themes and analyzed using qualitative methodology. The main discovered themes were 1) studies estimating the number of potential pediatric donation after circulatory determination of death donors and their impact on donation; 2) ethical issues in pediatric donation after circulatory determination of death; 3) physiology of the dying process after withdrawal of life-sustaining therapy; 4) cardiac pediatric donation after circulatory determination of death; and 5) neonatal pediatric donation after circulatory determination of death. Donor estimates suggest that pediatric donation after circulatory determination of death will

  13. A histopathological outlook on nephrotic syndrome: A pediatric perspective

    Directory of Open Access Journals (Sweden)

    M K Arif

    2016-01-01

    Full Text Available The developing world is observing changing histopathological patterns of idiopathic nephrotic syndrome (INS. However, the true burden of non-minimal change disease (non-MCD presenting as INS remains unestimated owing to a paucity of data on renal biopsies. Data were collected from January 2006 to June 2014 on 75 children up to 16 years of age who underwent renal biopsies for INS. Mean age at biopsy was 11.2 ± 3.7 years. The male to female ratio was 1.5:1. A total of 25 (33.3% children were steroid sensitive, 36 (48% were steroid resistant, 10 (13.3% were steroid dependent and 4 (5.3% came with relapse of nephrotic syndrome (NS. Focal segmental glomerulosclerosis (FSGS was the most common histopathological subtype observed in 35 (46.8% children followed by membranous glomerulonephritis (MGN in 11 (14.7%, membranoproliferative glomerulonephritis (MPGN and mesangioproliferative glomerulonephritis (MSGN in 4 (5.3% each and IgA nephropathy in one (1.3%. MCD was the histological lesion in 19 (25.3% children. The histopathology established FSGS as the main underlying cause of steroid resistant NS. The study highlights the emergence of non-MCD as the common cause of INS in the pediatric population and signifies the importance of renal biopsies in children with INS.

  14. Genetic features of myelodysplastic syndrome and aplastic anemia in pediatric and young adult patients

    Science.gov (United States)

    Keel, Siobán B.; Scott, Angela; Sanchez-Bonilla, Marilyn; Ho, Phoenix A.; Gulsuner, Suleyman; Pritchard, Colin C.; Abkowitz, Janis L.; King, Mary-Claire; Walsh, Tom; Shimamura, Akiko

    2016-01-01

    The clinical and histopathological distinctions between inherited versus acquired bone marrow failure and myelodysplastic syndromes are challenging. The identification of inherited bone marrow failure/myelodysplastic syndromes is critical to inform appropriate clinical management. To investigate whether a subset of pediatric and young adults undergoing transplant for aplastic anemia or myelodysplastic syndrome have germline mutations in bone marrow failure/myelodysplastic syndrome genes, we performed a targeted genetic screen of samples obtained between 1990–2012 from children and young adults with aplastic anemia or myelodysplastic syndrome transplanted at the Fred Hutchinson Cancer Research Center. Mutations in inherited bone marrow failure/myelodysplastic syndrome genes were found in 5.1% (5/98) of aplastic anemia patients and 13.6% (15/110) of myelodysplastic syndrome patients. While the majority of mutations were constitutional, a RUNX1 mutation present in the peripheral blood at a 51% variant allele fraction was confirmed to be somatically acquired in one myelodysplastic syndrome patient. This highlights the importance of distinguishing germline versus somatic mutations by sequencing DNA from a second tissue or from parents. Pathological mutations were present in DKC1, MPL, and TP53 among the aplastic anemia cohort, and in FANCA, GATA2, MPL, RTEL1, RUNX1, SBDS, TERT, TINF2, and TP53 among the myelodysplastic syndrome cohort. Family history or physical examination failed to reliably predict the presence of germline mutations. This study shows that while any single specific bone marrow failure/myelodysplastic syndrome genetic disorder is rare, screening for these disorders in aggregate identifies a significant subset of patients with inherited bone marrow failure/myelodysplastic syndrome. PMID:27418648

  15. Development of a tissue engineered heart valve for pediatrics: a case study in bioengineering ethics.

    Science.gov (United States)

    Merryman, W David

    2008-03-01

    The following hypothetical case study was developed for bioengineering students and is concerned with choosing between two devices used for development of a pediatric tissue engineered heart valve (TEHV). This case is intended to elicit assessment of the devices, possible future outcomes, and ramifications of the decision making. It is framed in light of two predominant ethical theories: utilitarianism and rights of persons. After the case was presented to bioengineering graduate students, they voted on which device should be released. The results revealed that these bioengineering students preferred the more reliable (and substantially more expensive) design, though this choice precludes the majority of the world from having access to this technology. This case is intended to examine and explore where the balance lies between design, cost, and adequate distribution of biomedical devices.

  16. [Prevalence of burnout syndrome in health professionals of an onco-hematological pediatric hospital].

    Science.gov (United States)

    Zanatta, Aline Bedin; Lucca, Sergio Roberto de

    2015-04-01

    To identify the prevalence of Burnout Syndrome in medical professionals, nurses and nursing technicians working in an Onco-Hematological Pediatric Hospital in São Paulo. An exploratory, descriptive study with cross-sectional design and quantitative approach, with a sample of 188 health professionals. Data were collected using two self-report instruments: the Maslach Burnout Inventory (MBI-HSS) which is a biosocial data form, and a non-participant observation guide. High depersonalization for nurses (29.8%), low job performance for physicians (27.8%), and of nursing technicians (25.5%). High scores were identified in at least two domains of Burnout in 19.2% of nurses, 16.8% of nursing technicians, and 16.6% of doctors. Health professionals are highly vulnerable to each of the dimensions of Burnout syndrome - namely emotional exhaustion, alienation, and low job performance/satisfaction- in the hospital work.

  17. Pediatric fibromyalgia.

    Science.gov (United States)

    Buskila, Dan

    2009-05-01

    Fibromyalgia is an idiopathic chronic pain syndrome defined by widespread nonarticular musculoskeletal pain and generalized tender points. The syndrome is associated with a constellation of symptoms, including fatigue, nonrefreshing sleep, irritable bowel, and more. Central nervous system sensitization is a major pathophysiologic aspect of fibromyalgia; in addition, various external stimuli such as trauma and stress may contribute to development of the syndrome. Fibromyalgia is most common in midlife, but may be seen at any age. This article reviews the epidemiology, clinical characteristics, etiology, management, and outcome of pediatric fibromyalgia.

  18. Experiences of ethical issues when caring for children with cancer.

    Science.gov (United States)

    Bartholdson, Cecilia; Lützén, Kim; Blomgren, Klas; Pergert, Pernilla

    2015-01-01

    The treatment for pediatric cancer is often physically, socially, and psychologically demanding and often gives rise to ethical issues. The purpose of this study was to describe healthcare professionals' experiences of ethical issues and ways to deal with these when caring for children with cancer. A study-specific questionnaire was given to healthcare professionals at a pediatric hospital in Sweden. Qualitative content analysis was used to analyze answers to open-ended questions. The data were sorted into 2 domains based on the objective of the study. In the next step, the data in each domain were inductively coded, generating categories and subcategories. The main ethical issues included concerns of (1) infringing on autonomy, (2) deciding on treatment levels, and (3) conflicting perspectives that constituted a challenge to collaboration. Professionals desired teamwork and reflection to deal with ethical concerns, and they needed resources for dealing with ethics. Interprofessional consideration needs to be improved. Forums and time for ethics reflections need to be offered to deal with ethical concerns in childhood cancer care. Experiences of ethical concerns and dealing with these in caring for children with cancer evoked strong feelings and moral perplexity among nursing staff. The study raises a challenging question: How can conflicting perspectives, lack of interprofessional consideration, and obstacles related to parents' involvement be "turned around," that is, contribute to a holistic perspective of ethics in cancer care of children?

  19. Pediatric-onset multiple sclerosis and other acquired demyelinating syndromes of the central nervous system in Denmark during 1977-2015

    DEFF Research Database (Denmark)

    Boesen, Magnus Spangsberg; Magyari, Melinda; Koch-Henriksen, Nils

    2018-01-01

    BACKGROUND: The incidence of acquired demyelinating syndromes (ADS) including multiple sclerosis (MS) has never been investigated in a Danish pediatric population. OBJECTIVES: We estimated the nationwide age- and sex-specific incidence of pediatric ADS including MS. METHODS: Data were sourced from...... the Danish Multiple Sclerosis Registry, providing cases of pediatric MS for 1977-2015, and the National Patient Register, providing cases of ADS during 2008-2015. All medical records were reviewed to validate the register-based diagnoses. RESULTS: We identified 364 cases of pediatric MS occurring during 1977......-2015 (incidence rate = 0.79 per 100,000 person-years). MS was exceptionally rare before puberty, but the incidence rose considerably from 9 years in girls and 11 years in boys. The female-to-male ratio was 2.5; the median age at onset was 16 years (range = 7-17 years). The MS incidence rate was relatively stable...

  20. Prevalence of Burnout syndrome in health professionals of an onco-hematological pediatric hospital

    Directory of Open Access Journals (Sweden)

    Aline Bedin Zanatta

    2015-04-01

    Full Text Available OBJECTIVE To identify the prevalence of Burnout Syndrome in medical professionals, nurses and nursing technicians working in an Onco-Hematological Pediatric Hospital in São Paulo. METHOD An exploratory, descriptive study with cross-sectional design and quantitative approach, with a sample of 188 health professionals. Data were collected using two self-report instruments: the Maslach Burnout Inventory (MBI-HSS which is a biosocial data form, and a non-participant observation guide. RESULTS High depersonalization for nurses (29.8%, low job performance for physicians (27.8%, and of nursing technicians (25.5%. High scores were identified in at least two domains of Burnout in 19.2% of nurses, 16.8% of nursing technicians, and 16.6% of doctors. CONCLUSION Health professionals are highly vulnerable to each of the dimensions of Burnout syndrome - namely emotional exhaustion, alienation, and low job performance/satisfaction- in the hospital work.

  1. The Ethics of Health Care Delivery in a Pediatric Malaria Vaccine Trial: The Perspectives of Stakeholders From Ghana and Tanzania.

    Science.gov (United States)

    Ward, Claire Leonie; Shaw, David; Anane-Sarpong, Evelyn; Sankoh, Osman; Tanner, Marcel; Elger, Bernice

    2018-02-01

    This study explores ethical issues raised in providing medical care to participants and communities of low-resource settings involved in a Phase II/III pediatric malaria vaccine trial (PMVT). We conducted 52 key informant interviews with major stakeholders of an international multi-center PMVT (GSK/PATH-MVI RTS,S) (NCT00866619) in Ghana and Tanzania. Based on their stakeholder experiences, the responses fell into three main themes: (a) undue inducement, (b) community disparities, and (c) broad therapeutic misconceptions. The study identified the critical ethical aspects, from the perspectives of stakeholders, of delivering health care during a PMVT. The study showed that integrating research into health care services needs to be addressed in a manner that upholds the favorable risk-benefit ratio of research and attends to the health needs of local populations. The implementation of research should aim to improve local standards of care through building a collaborative agenda with local institutions and systems of health.

  2. Cytogenetic studies of Brazilian pediatric myelodysplastic syndrome cases: challenges and difficulties in a large and emerging country.

    Science.gov (United States)

    Velloso, E D R P; Chauffaille, M L; Peliçario, L M; Tanizawa, R S S; Toledo, S R C; Gaiolla, R D; Lopes, L F

    2013-01-01

    Myelodysplastic syndromes (MDS) and juvenile myelomonocytic leukemia (JMML) are rare hematopoietic stem cell diseases affecting children. Cytogenetics plays an important role in the diagnosis of these diseases. We report here the experience of the Cytogenetic Subcommittee of the Brazilian Cooperative Group on Pediatric Myelodysplastic Syndromes (BCG-MDS-PED). We analyzed 168 cytogenetic studies performed in 23 different cytogenetic centers; 84 of these studies were performed in patients with confirmed MDS (primary MDS, secondary MDS, JMML, and acute myeloid leukemia/MDS+Down syndrome). Clonal abnormalities were found in 36.9% of the MDS cases and cytogenetic studies were important for the detection of constitutional diseases and for differential diagnosis with other myeloid neoplasms. These data show the importance of the Cooperative Group for continuing education in order to avoid a late or wrong diagnosis.

  3. Comparison of Prevalence and Outcomes of Pediatric Acute Respiratory Distress Syndrome Using Pediatric Acute Lung Injury Consensus Conference Criteria and Berlin Definition

    Directory of Open Access Journals (Sweden)

    Samriti Gupta

    2018-04-01

    Full Text Available ObjectivesOur objective was to compare the prevalence and outcomes of pediatric acute respiratory distress syndrome using the Pediatric Acute Lung Injury Consensus Conference (PALICC criteria and Berlin definitions.MethodsWe screened case records of all children aged 1 month to 17 years of age admitted to the Pediatric Intensive Care Unit (PICU over a 3-year period (2015–2017 for presence of any respiratory difficulty at admission or during PICU stay. We applied both PALICC and Berlin criteria to these patients. Data collection included definition and outcome related variables. Data were compared between the “PALICC only group” and the “Berlin with or without PALICC” group using Stata 11.ResultsOf a total of 615 admissions, 246 were identified as having respiratory difficulty at admission or during PICU stay. A total of 61 children (prevalence 9.9%; 95% CI: 7.8–12.4 fulfilled the definition of acute respiratory distress syndrome (ARDS with either of the two criteria. While 60 children (98% fulfilled PALICC criteria, only 26 children (43% fulfilled Berlin definition. There was moderate agreement between the two definitions (Kappa: 0.51; 95% CI: 0.40–0.62; observed agreement 85%. Greater proportion of patients had severe ARDS in the “Berlin with or without PALICC group” as compared to the “PALICC only” group (50 vs. 19%. There was no difference between the groups with regard to key clinical outcomes such as duration of ventilation (7 vs. 8 days or mortality [51.4 vs. 57.7%: RR (95% CI: 0.99 (0.64–1.5].ConclusionIn comparison to Berlin definition, the PALICC criteria identified more number of patients with ARDS. Proportion with severe ARDS and complications was greater in the “Berlin with or without PALICC” group as compared to the “PALICC only” group. There were no differences in clinical outcomes between the groups.

  4. The moral landscape of pediatric oncology : an empirical study on best interests, parental authority and child participation in decision making

    NARCIS (Netherlands)

    Vries, Martine Charlotte de

    2012-01-01

    Few medical specialties encounter so many ethical challenges as pediatrics does. It is a specialty that inherently has features that are morally charged. Pediatric ethics examines the broad issues of (1) the concept of the child’s best interest; (2) parental responsibility and authority in

  5. Anterior Cutaneous Nerve Entrapment Syndrome in a Pediatric Patient Previously Diagnosed With Functional Abdominal Pain: A Case Report.

    Science.gov (United States)

    DiGiusto, Matthew; Suleman, M-Irfan

    2018-03-23

    Chronic abdominal pain is common in children and adolescents but challenging to diagnose, because practitioners may be concerned about missing serious occult disease. Abdominal wall pain is an often ignored etiology for chronic abdominal pain. Anterior cutaneous nerve entrapment syndrome causes abdominal wall pain but is frequently overlooked. Correctly diagnosing patients with anterior cutaneous nerve entrapment syndrome is important because nerve block interventions are highly successful in the remittance of pain. Here, we present the case of a pediatric patient who received a diagnosis of functional abdominal pain but experienced pain remittance after receiving a trigger-point injection and transverse abdominis plane block.

  6. Diagnosis and clinical genetics of Cushing syndrome in pediatrics

    Science.gov (United States)

    Stratakis, Constantine A.

    2016-01-01

    SYNOPSIS Endogenous Cushing syndrome (CS) in pediatrics is rare; it may be caused by tumors that produce corticotropin (ACTH) in the pituitary gland (this form of CS is called Cushing disease) or elsewhere (ectopic CS), tumors that produce corticotropin-releasing hormone (CRH) anywhere (mostly neuroendocrine tissues), and finally adrenocortical masses that produce cortisol, such as adrenocortical cancer (ACC) or adenomas, and bilateral adrenocortical hypeprlasia (BAHs). ACC is a very rare cause of CS in children but should be excluded first, especially among younger patients. CS in children is often caused by germline or somatic mutations in an expanding list of genes with implications for the prognosis of the patients and for their families. CS should be early recognized in children; otherwise, it can lead to significant morbidity and mortality. All patients with suspected CS should be referred to specialized clinical centers for work-up; these centers should have access to experienced endocrine and neurological surgeons. PMID:27241967

  7. A Pediatric Case of Cowden Syndrome with Graves’ Disease

    Directory of Open Access Journals (Sweden)

    Cláudia Patraquim

    2017-01-01

    Full Text Available Cowden syndrome (CS is a rare dominantly inherited multisystem disorder, characterized by an extraordinary malignant potential. In 80% of cases, the human tumor suppressor gene phosphatase and tensin homolog (PTEN is mutated. We present a case of a 17-year-old boy with genetically confirmed CS and Graves’ disease (GD. At the age of 15, he presented with intention tremor, palpitations, and marked anxiety. On examination, he had macrocephaly, coarse facies, slight prognathism, facial trichilemmomas, abdominal keratoses, leg hemangioma, and a diffusely enlarged thyroid gland. He started antithyroid drug (ATD therapy with methimazole and, after a 2-year treatment period without achieving a remission status, a total thyroidectomy was performed. Diagnosis and management of CS should be multidisciplinary. Thyroid disease is frequent, but its management has yet to be fully defined. The authors present a case report of a pediatric patient with CS and GD and discuss treatment options.

  8. Pediatric Tourette Syndrome: A Tic Disorder with a Tricky Presentation.

    Science.gov (United States)

    Warsi, Qurratul; Kirby, Caroline; Beg, Mirza

    2017-01-01

    Dysphagia is a condition in which disruption of the swallowing process interferes with a patient's ability to eat. This may result in coughing or choking while swallowing, food sticking in the throat, or globus sensation. Eosinophilic esophagitis (EoE) is a chronic immune-mediated disease with a varied clinical spectrum of symptoms including dysphagia. Tourette syndrome (TS) is an inherited neurological disorder that manifests itself as a series of motor and vocal tics and may include oropharyngeal dysphagia. Dysphagia as a result of TS generally affects female, elderly patients and is not reported in children. While the pathophysiology is relatively unknown, experts believe TS is closely linked to damage or abnormalities in the basal ganglia of the brain. We present this interesting pediatric case of dysphagia due to EoE, which had been previously thought to be related to the patient's TS.

  9. Should Pediatric Euthanasia be Legalized?

    Science.gov (United States)

    Brouwer, Marije; Kaczor, Christopher; Battin, Margaret P; Maeckelberghe, Els; Lantos, John D; Verhagen, Eduard

    2018-02-01

    Voluntary active euthanasia for adults at their explicit request has been legal in Belgium and the Netherlands since 2002. In those countries, acceptance of the practice for adults has been followed by acceptance of the practice for children. Opponents of euthanasia see this as a dangerous slippery slope. Proponents argue that euthanasia is sometimes ethically appropriate for minors and that, with proper safeguards, it should be legally available in appropriate circumstances for patients at any age. In this Ethics Rounds, we asked philosophers from the United States and the Netherlands, and a Dutch pediatrician, to discuss the ethics of legalizing euthanasia for children. Copyright © 2018 by the American Academy of Pediatrics.

  10. [The development of the ethical thinking in children and the teaching of ethics in pediatrics].

    Science.gov (United States)

    Lejarraga, Horacio

    2008-10-01

    The child's ethical thinking is not installed in his mind as a single act, but as a consequence of an evolving process. Kohlberg, based on Piaget's studies, described three main developmental stages: preconventional, conventional and post conventional. However, Vigostky and others emphasized the importance of the environment for the moral sculpture of children. Three models can be recognised for teaching ethics in children: the deontological way, the descriptive way, and the only one morally acceptable: the one used by Socrates, by which ethics becomes not merely an adjective, but an institutionalised social practice built on axiological basis.

  11. Cytogenetic studies of Brazilian pediatric myelodysplastic syndrome cases: challenges and difficulties in a large and emerging country

    Directory of Open Access Journals (Sweden)

    E.D.R.P. Velloso

    2013-01-01

    Full Text Available Myelodysplastic syndromes (MDS and juvenile myelomonocytic leukemia (JMML are rare hematopoietic stem cell diseases affecting children. Cytogenetics plays an important role in the diagnosis of these diseases. We report here the experience of the Cytogenetic Subcommittee of the Brazilian Cooperative Group on Pediatric Myelodysplastic Syndromes (BCG-MDS-PED. We analyzed 168 cytogenetic studies performed in 23 different cytogenetic centers; 84 of these studies were performed in patients with confirmed MDS (primary MDS, secondary MDS, JMML, and acute myeloid leukemia/MDS+Down syndrome. Clonal abnormalities were found in 36.9% of the MDS cases and cytogenetic studies were important for the detection of constitutional diseases and for differential diagnosis with other myeloid neoplasms. These data show the importance of the Cooperative Group for continuing education in order to avoid a late or wrong diagnosis.

  12. Arthritis dermatitis syndrome in children

    International Nuclear Information System (INIS)

    Velasquez Mendez, Monica Patricia; Ramirez Gomez, Luis Alberto

    2004-01-01

    The pediatric rheumatology is a medical specialization with many areas under developed. The prevalence, pathophysiology and form of presentation of the pediatric rheumatic disease are different of adults. The skin compromise in many pediatric rheumatic diseases is a helping sing for diagnosis. The arthritis-dermatitis syndrome can be the first manifestation of many diseases as infections, tumors and endocrine diseases, but in pediatric age the immunologic and infections diseases are really important. Among infections diseases, virus (parvovirus, rubella, HIV) and bacteria (gonococcus, meningoccus) are the most Important. Within the group of autoimmune diseases the vasculitis such as Henoch-Schonlein purpura and Kawasaki disease are among the more prevalent autoimmune disease. This is a general review about arthritis-dermatitis syndrome in pediatric age

  13. Síndromes autoinflamatórias hereditárias na faixa etária pediátrica Pediatric hereditary autoinflammatory syndromes

    Directory of Open Access Journals (Sweden)

    Adriana Almeida Jesus

    2010-10-01

    Full Text Available OBJETIVO: Descrever as principais síndromes autoinflamatórias hereditárias na faixa etária pediátrica. FONTES DOS DADOS: Foi realizada uma revisão da literatura nas bases de dados PubMed e SciELO, utilizando as palavras-chave "síndromes autoinflamatórias” e "criança”, e incluindo referências bibliográficas relevantes. SÍNTESE DOS DADOS: As principais síndromes autoinflamatórias são causadas por defeitos monogênicos em proteínas da imunidade inata, sendo consideradas imunodeficiências primárias. Elas são caracterizadas clinicamente por sintomas inflamatórios sistêmicos recorrentes ou contínuos e devem ser diferenciadas das doenças infecciosas, autoimunes e outras imunodeficiências primárias. Nesta revisão, foram enfatizadas características epidemiológicas, manifestações clínicas, alterações laboratoriais, prognóstico e terapia das principais síndromes autoinflamatórias: febre familiar do Mediterrâneo; síndrome periódica associada ao receptor de fator de necrose tumoral; criopirinopatias; deficiência de mevalonato-quinase; artrite granulomatosa pediátrica; síndrome de pioderma gangrenoso, artrite piogênica e acne; síndrome de Majeed; e deficiência do antagonista do receptor de interleucina-1. As criopirinopatias discutidas foram: doença inflamatória multissistêmica de início neonatal ou síndrome neurológica, cutânea e articular crônica infantil, síndrome de Muckle-Wells e síndrome autoinflamatória familiar associada ao frio. CONCLUSÕES: É importante que o pediatra reconheça as síndromes autoinflamatórias hereditárias mais prevalentes, pois o encaminhamento ao reumatologista pediátrico pode permitir um diagnóstico precoce e uma instituição de tratamento adequado, possibilitando uma melhora da qualidade de vida dos pacientes.OBJECTIVE: To describe the most prevalent pediatric hereditary autoinflammatory syndromes. SOURCES: A review of the literature including relevant references

  14. Children, ethics, and modern medicine

    National Research Council Canada - National Science Library

    Miller, Richard B

    2003-01-01

    ... 220 11. Ethical Issues in Pediatric Research 238 :     ⁄ 275  ⁄ 301 268 AcknowledgmentsACKNOWLEDGMENTS The remotest origin...

  15. Pediatric Melanoma and Drug Development

    Directory of Open Access Journals (Sweden)

    Klaus Rose

    2018-03-01

    Full Text Available Importance—Pediatric melanoma occurs, albeit rarely. Should patients be treated by today’s medical standards, or be subjected to medically unnecessary clinical studies? Observations—We identified international, industry-sponsored pediatric melanoma studies triggered by regulatory demands in www.clinicaltrials.gov and further pediatric melanoma studies demanded by European Union pediatric investigation plans. We retrieved related regulatory documents from the internet. We analyzed these studies for rationale and medical beneficence on the basis of physiology, pediatric clinical pharmacology and rationale. Regulatory authorities define children by chronological age, not physiologically. Newborns’ organs are immature but they develop and mature rapidly. Separate proof of efficacy in underage patients is justified formally/regulatorily but lacks medical sense. Children—especially post-puberty—and adults vis-a-vis medications are physiologically very similar. Two adolescent melanoma studies were terminated in 2016 because of waning recruitment, while five studies in pediatric melanoma and other solid tumors, triggered by European Union pediatric investigation plans, continue recruiting worldwide. Conclusions and Relevance—Regulatory-demanded pediatric melanoma studies are medically superfluous. Melanoma patients of all ages should be treated with effective combination treatment. Babies need special attention. Children need dose-finding and pharmacokinetic studies but adolescents metabolize and respond to drugs similarly to adults. Institutional Review Boards/ethics committees should suspend ongoing questionable pediatric melanoma studies and reject newly submitted questionable studies.

  16. Pediatric Tourette Syndrome: A Tic Disorder with a Tricky Presentation

    Directory of Open Access Journals (Sweden)

    Qurratul Warsi

    2017-03-01

    Full Text Available Dysphagia is a condition in which disruption of the swallowing process interferes with a patient’s ability to eat. This may result in coughing or choking while swallowing, food sticking in the throat, or globus sensation. Eosinophilic esophagitis (EoE is a chronic immune-mediated disease with a varied clinical spectrum of symptoms including dysphagia. Tourette syndrome (TS is an inherited neurological disorder that manifests itself as a series of motor and vocal tics and may include oropharyngeal dysphagia. Dysphagia as a result of TS generally affects female, elderly patients and is not reported in children. While the pathophysiology is relatively unknown, experts believe TS is closely linked to damage or abnormalities in the basal ganglia of the brain. We present this interesting pediatric case of dysphagia due to EoE, which had been previously thought to be related to the patient’s TS.

  17. Calcium and Vitamin D Metabolism in Pediatric Nephrotic Syndrome; An Update on the Existing Literature

    Directory of Open Access Journals (Sweden)

    Mohammad Esmaeeili

    2015-03-01

    Full Text Available  Minimal Change Disease (MCD is the leading cause of childhood Nephrotic Syndrome (NS. Therefore in pediatrics nephrotic syndrome, most children beyond the first year of life will be treated with corticosteroids without an initial biopsy. Children with NS often display a number of calcium homeostasis disturbances causing abnormal bone histology, including hypocalcemia, reduced serum vitamin D metabolites, impaired intestinal absorption of calcium, and elevated levels of immunoreactive parathyroid hormone (iPTH. These are mainly attributed to the loss of a variety of plasma proteins and minerals in the urine as well as steroid therapy. Early diagnosis and management of these abnormalities, could prevent the growth retardation and renal osteodystrophy that affects children with nephrotic syndrome. Here we reviewed the literature for changes of calcium and vitamin D metabolism in nephrotic syndrome and its consequences on bones, also the effect of corticosteroid and possible preventive strategies that could be done to avoid long term outcomes in children. Although the exact biochemical basis for Changes in levels of calcium and vitamin D metabolites in patients with NS remains speculative; Because of the potential adverse effects of these changes among growing children, widespread screening for vitamin D deficiency or routine vitamin D supplementation should be considered.

  18. Pediatric Opsoclonus-Myoclonus-Ataxia Syndrome Associated With Anti-N-methyl-D-aspartate Receptor Encephalitis.

    Science.gov (United States)

    Player, Brittany; Harmelink, Matthew; Bordini, Brett; Weisgerber, Michael; Girolami, Michael; Croix, Michael

    2015-11-01

    The full clinical spectrum of anti-N-methyl-D-aspartate receptor encephalitis is unknown in the pediatric population. We describe a previously healthy 4-year-old girl presenting with opsoclonus-myoclonus together with ataxia who had NR1-specific, anti-N-methyl-D-aspartate receptor antibodies in the cerebral spinal fluid. The presence of NR1-specific, anti-N-methyl-D-aspartate receptor antibodies in the setting of opsoclonus-myoclonus and ataxia syndrome may represent an expansion of the clinical presentations of anti-N-methyl-D-aspartate receptor encephalitis. Copyright © 2015 Elsevier Inc. All rights reserved.

  19. The Correlation of Regulatory T (TReg and Vitamin D3 in Pediatric Nephrotic Syndrome

    Directory of Open Access Journals (Sweden)

    Yunika Nurtyas

    2018-01-01

    Full Text Available Nephrotic syndrome (NS is an autoimmune disease that correlates to the imbalance of regulatory T cells (TReg. This study was aimed to investigate the effect of vitamin D as adjuvant therapy of TReg population in pediatric nephrotic syndrome. This study was designed randomized clinical trial, double blind, with pre- and post-test control groups involving 15 subjects newly diagnosed with NS. Subjects were divided into 2 groups, namely K1 for group treated with prednisone+vitamin D and K2 group for prednisone treatment only. The population of TReg in peripheral blood mononuclear cells (PBMC was analyzed using flowcytometry. Vitamin D serum level was measured through ELISA method. Results showed that there was a significant elevation of TReg (independent t-test, p = 0.010 in K1 group, which was higher than in K2 group. The Pearson test in the K1 group showed that vitamin D level was positively correlated with TReg (p = 0.039, r = 0.779.

  20. Should general surgery residents be taught laparoscopic pyloromyotomies? An ethical perspective.

    Science.gov (United States)

    Escobar, Mauricio A; Hartin, Charles W; McCullough, Laurence B

    2014-01-01

    The authors examine the ethical implications of teaching general surgery residents laparoscopic pyloromyotomy. Using the authors' previously presented ethical framework, and examining survey data of pediatric surgeons in the United States and Canada, a rigorous ethical argument is constructed to examine the question: should general surgery residents be taught laparoscopic pyloromyotomies? A survey was constructed that contained 24 multiple-choice questions. The survey included questions pertaining to surgeon demographics, if pyloromyotomy was taught to general surgery and pediatric surgery residents, and management of complications encountered during pyloromyotomy. A total of 889 members of the American Pediatric Surgical Association and Canadian Association of Paediatric Surgeons were asked to participate. The response rate was 45% (401/889). The data were analyzed within the ethical model to address the question of whether general surgery residents should be taught laparoscopic pyloromyotomies. From an ethical perspective, appealing to the ethical model of a physician as a fiduciary, the answer is no. We previously proposed an ethical model based on 2 fundamental ethical principles: the ethical concept of the physician as a fiduciary and the contractarian model of ethics. The fiduciary physician practices medicine competently with the patient’s best interests in mind. The role of a fiduciary professional imposes ethical standards on all physicians, at the core of which is the virtue of integrity, which requires the physician to practice medicine to standards of intellectual and moral excellence. The American College of Surgeons recognizes the need for current and future surgeons to understand professionalism, which is one of the 6 core competencies specified by the Accreditation Council for Graduate Medical Education. Contracts are models of negotiation and ethically permissible compromise. Negotiated assent or consent is the core concept of contractarian

  1. Fertility Preservation for Pediatric Patients: Current State and Future Possibilities.

    Science.gov (United States)

    Johnson, Emilie K; Finlayson, Courtney; Rowell, Erin E; Gosiengfiao, Yasmin; Pavone, Mary Ellen; Lockart, Barbara; Orwig, Kyle E; Brannigan, Robert E; Woodruff, Teresa K

    2017-07-01

    This review provides an overview of pediatric fertility preservation. Topics covered include the patient populations who could benefit, the current state of fertility preservation options and research, and considerations related to ethics and program development. A broad Embase® and PubMed® search was performed to identify publications discussing investigational, clinical, ethical and health care delivery issues related to pediatric fertility preservation. Relevant publications were reviewed and summarized. Populations who could benefit from fertility preservation in childhood/adolescence include oncology patients, patients with nononcologic conditions requiring gonadotoxic chemotherapy, patients with differences/disorders of sex development and transgender individuals. Peripubertal and postpubertal fertility preservation options are well established and include cryopreservation of oocytes, embryos or sperm. Prepubertal fertility preservation is experimental. Multiple lines of active research aim to develop technologies that will enable immature eggs and sperm to be matured and used to produce a biological child in the future. Ethical challenges include the need for parental proxy decision making and the fact that fertility preservation procedures can be considered not medically necessary. Successful multidisciplinary fertility preservation care teams emphasize partnerships with adult colleagues, prioritize timely consultations and use standardized referral processes. Some aspects of fertility preservation are not covered by insurance and out-of-pocket costs can be prohibitive. Pediatric fertility preservation is an emerging, evolving field. Fertility preservation options for prepubertal patients with fertility altering conditions such as cancer and differences/disorders of sex development are currently limited. However, multiple lines of active research hold promise for the future. Key considerations include establishing a multidisciplinary team to provide

  2. Peregrination in the problem pediatric patient. The pediatric Münchhausen syndrome?

    Science.gov (United States)

    Fialkov, M J

    1984-10-01

    Peregrinating pediatric patients are those who go from physician to physician either within hospitals or from clinic to clinic within a community. They are often the symptom bearers of dysfunctional multiproblem families requiring an interdisciplinary approach to diagnosis and management. Because of the obscure nature of the child's pediatric problems, such children are often shunned by the medical profession and other social agencies, setting in motion a sequence of events that can prove detrimental to the child, his family, the community, and the state. This article illustrates the need for an anticipatory, coordinated approach in the management of this complex psychosocial condition.

  3. A critical review of health research ethical guidelines regarding ...

    African Journals Online (AJOL)

    Trials, 2006;[22] and Ethics in Health Research Principles, Structures and Procedures, 2015[15]) regarding caregivers' consent in research involving minors as research ..... 11. Jeff H, Ramesh R, Sanjay MB. Pediatric airway management.

  4. Cerebral imaging in pediatrics

    Energy Technology Data Exchange (ETDEWEB)

    Gordon, I [London, Great Ormond Street Hospital for Children (United Kingdom)

    1998-06-01

    Radioisotope brain imaging has focused mainly on regional cerebral blood flow (rCBF). However the use of ligand which go to specific receptor sites is being introduced in pediatrics, mainly psychiatry. rCBF is potentially available in many institutions, especially with the availability of multi-headed gamma cameras. The use of this technique in pediatrics requires special attention to detail in the manner of data acquisition and handling the child. The interpretation of the rCBF study in a child requires knowledge of normal brain maturation. The major clinical use in pediatrics is epilepsy because of the advances in surgery and the frequency of complex partial seizures. Other indications in pediatric neurology include brain death, acute neurological loss including stroke, language disorders, cerebral palsy, hypertension due to renovascular disease, traumatic brain injury and migraine. There are pediatric physiological conditions in which rCBF has been undertaken, these include anorexia nervosa, autism, Gilles de la Tourette syndrome (GTS) and attention deficit disorder-hyperactivity (ADHD). Research using different ligands to specific receptor sites will also be reviewed in pediatrics.

  5. Cerebral imaging in pediatrics

    International Nuclear Information System (INIS)

    Gordon, I.

    1998-01-01

    Radioisotope brain imaging has focused mainly on regional cerebral blood flow (rCBF). However the use of ligand which go to specific receptor sites is being introduced in pediatrics, mainly psychiatry. rCBF is potentially available in many institutions, especially with the availability of multi-headed gamma cameras. The use of this technique in pediatrics requires special attention to detail in the manner of data acquisition and handling the child. The interpretation of the rCBF study in a child requires knowledge of normal brain maturation. The major clinical use in pediatrics is epilepsy because of the advances in surgery and the frequency of complex partial seizures. Other indications in pediatric neurology include brain death, acute neurological loss including stroke, language disorders, cerebral palsy, hypertension due to renovascular disease, traumatic brain injury and migraine. There are pediatric physiological conditions in which rCBF has been undertaken, these include anorexia nervosa, autism, Gilles de la Tourette syndrome (GTS) and attention deficit disorder-hyperactivity (ADHD). Research using different ligands to specific receptor sites will also be reviewed in pediatrics

  6. Moral Hazard in Pediatrics.

    Science.gov (United States)

    Brunnquell, Donald; Michaelson, Christopher M

    2016-07-01

    "Moral hazard" is a term familiar in economics and business ethics that illuminates why rational parties sometimes choose decisions with bad moral outcomes without necessarily intending to behave selfishly or immorally. The term is not generally used in medical ethics. Decision makers such as parents and physicians generally do not use the concept or the word in evaluating ethical dilemmas. They may not even be aware of the precise nature of the moral hazard problem they are experiencing, beyond a general concern for the patient's seemingly excessive burden. This article brings the language and logic of moral hazard to pediatrics. The concept reminds us that decision makers in this context are often not the primary party affected by their decisions. It appraises the full scope of risk at issue when decision makers decide on behalf of others and leads us to separate, respect, and prioritize the interests of affected parties.

  7. Informed Consent in Pediatric Oncology: A Systematic Review of Qualitative Literature.

    Science.gov (United States)

    Alahmad, Ghiath

    2018-01-01

    Obtaining informed consent in pediatric cancer research can be subject to important ethical challenges because of the difficulty in distinguishing between care and research, which are interrelated. Pediatric oncologists also often conduct research, such as clinical trials, on their own patients, which may influence voluntary informed consent. This review aims to determine the ethical issues encountered in obtaining informed consent in pediatric oncology by identifying and summarizing the findings of existing qualitative studies on this topic. A systematic review of qualitative studies was conducted. Medline, Embase, CINAHL, and PubMed were searched using the following terms: (oncolog* or cancer or hematol* or haematol* or leuk* or malign* or neoplasm*) and (child* or adolescent* or minor* or young people or pediatr* or paediatr*) and ethic* or moral*) and (qualitative or interview). Other sources were also mined to identify all relevant studies. The data analysis method used was thematic analysis. At the end of the search process, 2361 studies were identified. Duplicates were removed and irrelevant studies were excluded. After screening the full text of the remaining studies against our inclusion and exclusion criteria, 13 studies were included in the qualitative analysis. All studies were qualitative studies using semistructured and structured interviews, qualitative analysis of open-ended questions, and observation of informed consent conferences. Four themes were identified: parental comprehension of the trial and medical terms, influence of parental distress on decision-making, no offer of an alternative treatment, and influence of the doctor-parent relationship. Many ethical challenges affect the informed consent process. These challenges may include a lack of parental understanding, the potential influence of treating doctors, and vulnerability because of psychological status. All of these result in parents being unable to give well-informed and voluntary

  8. [Malformation syndromes in the spanish literature: The first descriptions].

    Science.gov (United States)

    García Nieto, V; Zafra Anta, M; Bassat, Q; de Arana Amurrio, J I; Fernández Menéndez, J M; Fernández Teijeiro, J J; Gorrotxategi Gorrotxategi, P J; Ponte Hernando, F

    2013-12-01

    Malformation or dysmorphic syndromes are conditions that are defined by the combination of a set of major and minor malformations that generally have a genetic origin. We investigated the early Spanish descriptions of a large number of sydromes. We started the study from the definition in a classic treatise on the subject. Among the 60 selected syndromes studied, at least two articles of each syndrome among those published in Spain and for the first time were listed in the Spanish Medical Index. For years prior to 1970, it expanded The search was expanded to PubMed, for the years before 1970. We collected 64 articles that referred to 58 syndromes. Four articles were written during the first half of the twentieth century. Almost half of the works (n=30) appeared in the 1970's. The papers were published in ten Spanish pediatric, nine Spanish non-pediatric, three pediatric non-Spanish and two non-Spanish non-pediatric Journals. The Journals with the most articles published were Anales de Pediatría y Revista Española de Pediatría. The hospitals where the articles originate more often were Hospital La Paz, Madrid and Hospital Clinic, Barcelona. The rest of the works were written in 30 pediatric hospitals and two more in non-Spanish hospitals. The number of authors increased with the passage of time. Although there were potential limitations, we have identified what may be the first descriptions of 58 syndromes, among the 60 chosen initially. Copyright © 2012 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  9. 2016 CAPS ethics session/Ein debate: 1. Regionalization of pediatric surgical care 2. Ethical introduction of surgical innovation 3. Addressing stress in a surgical practice: resiliency, well-being, and burnout.

    Science.gov (United States)

    Bagwell, Charles E; Chiu, Priscilla; Fecteau, Annie; Gow, Kenneth W; Mueller, Claudia M; Price, David; Zigman, Andrew F

    2017-05-01

    The following is the conference proceeding of the Second Ein Debate from the 48th Annual Meeting of the Canadian Association of Paediatric Surgeons held in Vancouver, BC, from September 22 to 24, 2016. The three main topics for debate, as prepared by the members of the CAPS Ethics Committee, are: 1. Regionalization of care: pros and cons, 2. Innovation in clinical care: ethical considerations, and 3. Surgeon well-being: caring for the caregiver. The authors of this paper, as participants in the debate, were assigned their positions at random. Therefore, the opinions they express within this summary might not reflect their own viewpoints. In the first discussion, arguments for and against the regionalization of pediatric surgical care are discussed, primarily in the context of a case of BA. In the pro argument, the evidence and lessons learned from different European countries are explored as well as different models to provide the best BA care outside of large teaching centers. In the counterargument, the author explains how regionalization of care could be detrimental for the patient, the family, the regional center, and for the health care system in general. In the debate on surgical innovation the authors define surgical innovation. They review the pertinent ethical principles, explore a model for its implementation, and the role of the institution at which the innovation is proposed. In the third section, surgeon well-being is examined, and recent literature on surgeon resiliency and burnout both at the attending and resident level is reviewed. Copyright © 2017 Elsevier Inc. All rights reserved.

  10. Safety and efficacy of aripiprazole for the treatment of pediatric Tourette syndrome and other chronic tic disorders.

    Science.gov (United States)

    Cox, Joanna H; Seri, Stefano; Cavanna, Andrea E

    2016-01-01

    Tourette syndrome is a childhood-onset chronic tic disorder characterized by multiple motor and vocal tics and often accompanied by specific behavioral symptoms ranging from obsessionality to impulsivity. A considerable proportion of patients report significant impairment in health-related quality of life caused by the severity of their tics and behavioral symptoms and require medical intervention. The most commonly used medications are antidopaminergic agents, which have been consistently shown to be effective for tic control, but are also associated with poor tolerability because of their adverse effects. The newer antipsychotic medication aripiprazole is characterized by a unique mechanism of action (D2 partial agonism), and over the last decade has increasingly been used for the treatment of tics. We conducted a systematic literature review to assess the available evidence on the efficacy and safety of aripiprazole in pediatric patients with Tourette syndrome and other chronic tic disorders (age range: 4-18 years). Our search identified two randomized controlled trials (involving 60 and 61 participants) and ten open-label studies (involving between six and 81 participants). The majority of these studies used two validated clinician-rated instruments (Yale Global Tic Severity Scale and Clinical Global Impression scale) as primary outcome measures. The combined results from randomized controlled trials and open-label studies showed that aripiprazole is an effective, safe, and well-tolerated medication for the treatment of tics. Aripiprazole-related adverse effects (nausea, sedation, and weight gain) were less frequent compared to other antidopaminergic medications used for tic management and, when present, were mostly transient and mild. The reviewed studies were conducted on small samples and had relatively short follow-up periods, thus highlighting a need for further trials to assess the long-term use of aripiprazole in pediatric patients with Tourette syndrome

  11. The catastrophic antiphospholipid syndrome in children.

    Science.gov (United States)

    Go, Ellen J L; O'Neil, Kathleen M

    2017-09-01

    To review the difficult syndrome of catastrophic antiphospholipid syndrome, emphasizing new developments in the diagnosis, pathogenesis and treatment. Few recent publications directly address pediatric catastrophic antiphospholipid syndrome (CAPS). Most articles are case reports or are data from adult and pediatric registries. The major factors contributing to most pediatric catastrophic antiphospholipid syndrome include infection and the presence of antiphospholipid antibodies, but complement activation also is important in creating diffuse thrombosis in the microcirculation. Treatment of the acute emergency requires anticoagulation, suppression of the hyperinflammatory state and elimination of the triggering infection. Inhibition of complement activation appears to improve outcome in limited studies, and suppression of antiphospholipid antibody formation may be important in long-term management. CAPS, an antibody-mediated diffuse thrombotic disease of microvasculature, is rare in childhood but has high mortality (33-50%). It requires prompt recognition and aggressive multimodality treatment, including anticoagulation, anti-inflammatory therapy and elimination of inciting infection and pathogenic autoantibodies.

  12. COMPLIANCY AS A MEDICO-SOCIAL AND ETHIC PROBLEM OF PEDIATRICS

    Directory of Open Access Journals (Sweden)

    G. L. Mikirtichan

    2012-01-01

    Full Text Available The article represents the clinic-sociological analysis of the compliancy among pediatric patients and the estimation of various factors influence on the patients’ and their surroundings’ adherence to the treatment regimen. For the compliance assessment physicians, children with allergic dermatosis at the age of 13–18 years old (n =295 and their parents (n =270 were interrogated with the special questionnaires. Received data confirms that there is decrease in compliance among the pediatric patients and their patients. It is more significant in older girls than in boys.

  13. Genetics Home Reference: Gitelman syndrome

    Science.gov (United States)

    ... MJ, Lifton RP, Simon DB; Yale Gitelman's and Bartter's Syndrome Collaborative Study Group. Gitelman's syndrome revisited: an evaluation ... chloride channel gene, CLCNKB, leading to a mixed Bartter-Gitelman phenotype. Pediatr Res. 2000 ... NV, Levtchenko EN. Gitelman syndrome. Orphanet J Rare Dis. 2008 Jul 30;3: ...

  14. Pediatric neurological syndromes and inborn errors of purine metabolism.

    Science.gov (United States)

    Camici, Marcella; Micheli, Vanna; Ipata, Piero Luigi; Tozzi, Maria Grazia

    2010-02-01

    This review is devised to gather the presently known inborn errors of purine metabolism that manifest neurological pediatric syndromes. The aim is to draw a comprehensive picture of these rare diseases, characterized by unexpected and often devastating neurological symptoms. Although investigated for many years, most purine metabolism disorders associated to psychomotor dysfunctions still hide the molecular link between the metabolic derangement and the neurological manifestations. This basically indicates that many of the actual functions of nucleosides and nucleotides in the development and function of several organs, in particular central nervous system, are still unknown. Both superactivity and deficiency of phosphoribosylpyrophosphate synthetase cause hereditary disorders characterized, in most cases, by neurological impairments. The deficiency of adenylosuccinate lyase and 5-amino-4-imidazolecarboxamide ribotide transformylase/IMP cyclohydrolase, both belonging to the de novo purine synthesis pathway, is also associated to severe neurological manifestations. Among catabolic enzymes, hyperactivity of ectosolic 5'-nucleotidase, as well as deficiency of purine nucleoside phosphorylase and adenosine deaminase also lead to syndromes affecting the central nervous system. The most severe pathologies are associated to the deficiency of the salvage pathway enzymes hypoxanthine-guanine phosphoribosyltransferase and deoxyguanosine kinase: the former due to an unexplained adverse effect exerted on the development and/or differentiation of dopaminergic neurons, the latter due to a clear impairment of mitochondrial functions. The assessment of hypo- or hyperuricemic conditions is suggestive of purine enzyme dysfunctions, but most disorders of purine metabolism may escape the clinical investigation because they are not associated to these metabolic derangements. This review may represent a starting point stimulating both scientists and physicians involved in the study of

  15. Pediatric acute lung injury

    NARCIS (Netherlands)

    Dahlem, P.; van Aalderen, W. M. C.; Bos, A. P.

    2007-01-01

    Among ventilated children, the incidence of acute lung injury (ALI) was 9%; of that latter group 80% developed the acute respiratory distress syndrome (ARDS). The population-based prevalence of pediatric ARDS was 5.5 cases/100.000 inhabitants. Underlying diseases in children were septic shock (34%),

  16. Anomaly-Related Pathologic Atlantoaxial Displacement in Pediatric Patients.

    Science.gov (United States)

    Pavlova, Olga M; Ryabykh, Sergey O; Burcev, Alexander V; Gubin, Alexander V

    2018-06-01

    To analyze clinical and radiologic features of pathologic atlantoaxial displacement (PAAD) in pediatric patients and to compose a treatment algorithm for anomaly-related PAAD. Criteria of different types of PAAD and treatment algorithms have been widely reported in the literature but are difficult to apply to patients with odontoid abnormalities, C2-C3 block, spina bifida C1, and children. We evaluated results of treatment of 29 pediatric patients with PAAD caused by congenital anomalies of the craniovertebral junction (CVJ), treated in Ilizarov Center in 2009-2017, including 20 patients with atlantoaxial displacement (AAD) and 9 patients with atlantoaxial rotatory fixation. There were 14 males (48.3%) and 15 females (51.7%). We singled out 3 groups of patients: nonsyndromic (6 patients, 20.7%), Klippel-Feil syndrome (13 patients, 44.8%), and syndromic (10 patients, 34.5%). Odontoid abnormalities and C1 dysplasia were widely represented in the syndromic group. Local symptoms predominated in the nonsyndromic and KFS groups. In the syndromic group, all patients had AAD and myelopathy. A pronounced decrease of space available for chord C1 and increase of anterior atlantodental interval were noted compared with other groups. We present a unified treatment algorithm of pediatric anomaly-related PAAD. Syndromic AAD are often accompanied by anterior and central dislocation and myelopathy and atlantooccipital dissociation. These patients require early aggressive surgical treatment. Nonsyndromic and Klippel-Feil syndrome AAD, atlantoaxial subluxation, and atlantoaxial fixation often manifest by local symptoms and need to eliminate CVJ instability. Existing classifications of symptomatic atlantoaxial displacement are not always suitable for patients with CVJ abnormalities. Copyright © 2018 Elsevier Inc. All rights reserved.

  17. Cyclic Vomiting Syndrome (CVS: is there a difference based on onset of symptoms - pediatric versus adult?

    Directory of Open Access Journals (Sweden)

    Kumar Nilay

    2012-05-01

    Full Text Available Abstract Background Cyclic Vomiting Syndrome (CVS is a well-recognized functional gastrointestinal disorder in children but its presentation is poorly understood in adults. Genetic differences in pediatric-onset (presentation before age 18 and adult-onset CVS have been reported recently but their clinical features and possible differences in response to therapy have not been well studied. Methods This was a retrospective review of 101 CVS patients seen at the Medical College of Wisconsin between 2006 and 2008. Rome III criteria were utilized to make the diagnosis of CVS. Results Our study population comprised of 29(29% pediatric-onset and 72 (71% adult-onset CVS patients. Pediatric-onset CVS patients were more likely to be female (86% vs. 57%, p = 0.005 and had a higher prevalence of CVS plus (CVS + neurocognitive disorders as compared to adult-onset CVS patients (14% vs. 3%, p = 0.05. There was a longer delay in diagnosis (10 ± 7 years in the pediatric-onset group when compared to (5 ± 7 years adult-onset CVS group (p = 0.001. Chronic opiate use was less frequent in the pediatric-onset group compared to adult-onset patients (0% vs. 23%, p = 0.004. Aside from these differences, the two groups were similar with regards to their clinical features and the time of onset of symptoms did not predict response to standard treatment. The majority of patients (86% responded to treatment with tricyclic antidepressants, anticonvulsants (topiramate, coenzyme Q-10, and L-carnitine. Non-response to therapy was associated with coalescence of symptoms, chronic opiate use and more severe disease as characterized by longer episodes, greater number of emergency department visits in the year prior to presentation, presence of disability and non-compliance on univariate analysis. On multivariate analysis, only compliance to therapy was associated with a response. (88% vs. 38%, Odds Ratio, OR 9.6; 95% Confidence Interval [CI], 1.18-77.05. Conclusion Despite reported

  18. Cyclic Vomiting Syndrome (CVS): is there a difference based on onset of symptoms--pediatric versus adult?

    Science.gov (United States)

    Kumar, Nilay; Bashar, Qumseya; Reddy, Naveen; Sengupta, Jyotirmoy; Ananthakrishnan, Ashwin; Schroeder, Abigail; Hogan, Walter J; Venkatesan, Thangam

    2012-05-28

    Cyclic Vomiting Syndrome (CVS) is a well-recognized functional gastrointestinal disorder in children but its presentation is poorly understood in adults. Genetic differences in pediatric-onset (presentation before age 18) and adult-onset CVS have been reported recently but their clinical features and possible differences in response to therapy have not been well studied. This was a retrospective review of 101 CVS patients seen at the Medical College of Wisconsin between 2006 and 2008. Rome III criteria were utilized to make the diagnosis of CVS. Our study population comprised of 29(29%) pediatric-onset and 72 (71%) adult-onset CVS patients. Pediatric-onset CVS patients were more likely to be female (86% vs. 57%, p = 0.005) and had a higher prevalence of CVS plus (CVS + neurocognitive disorders) as compared to adult-onset CVS patients (14% vs. 3%, p = 0.05). There was a longer delay in diagnosis (10 ± 7 years) in the pediatric-onset group when compared to (5 ± 7 years) adult-onset CVS group (p = 0.001). Chronic opiate use was less frequent in the pediatric-onset group compared to adult-onset patients (0% vs. 23%, p = 0.004). Aside from these differences, the two groups were similar with regards to their clinical features and the time of onset of symptoms did not predict response to standard treatment. The majority of patients (86%) responded to treatment with tricyclic antidepressants, anticonvulsants (topiramate), coenzyme Q-10, and L-carnitine. Non-response to therapy was associated with coalescence of symptoms, chronic opiate use and more severe disease as characterized by longer episodes, greater number of emergency department visits in the year prior to presentation, presence of disability and non-compliance on univariate analysis. On multivariate analysis, only compliance to therapy was associated with a response. (88% vs. 38%, Odds Ratio, OR 9.6; 95% Confidence Interval [CI], 1.18-77.05). Despite reported genetic differences, the clinical features and

  19. Pediatric irritable bowel syndrome and other functional abdominal pain disorders: an update of non-pharmacological treatments.

    Science.gov (United States)

    Gupta, Shivani; Schaffer, Gilda; Saps, Miguel

    2018-05-01

    Functional abdominal pain disorders, including irritable bowel syndrome, are common in children and treatment can often be difficult. Pharmacological therapies and complementary treatments are widely used, despite the limited data in pediatrics. Areas covered: This review provides an overview of the available data for the use of diet, probiotics, percutaneous electrical nerve stimulation, and psychosocial interventions, including hypnotherapy, yoga, cognitive and behavioral therapy, and mind-body interventions for the treatment of functional abdominal pain disorders in children. The literature review included a PubMed search by each therapy, children, abdominal pain, and irritable bowel syndrome. Relevant articles to this review are discussed. Expert commentary: The decision on the use of pharmacological and complementary therapies should be based on clinical findings, evidence, availability, and in-depth discussion with the patient and family. The physician should provide education on the different interventions and their role on the treatment in an empathetic and warm manner providing ample time for the family to ask questions.

  20. Annual Costs of Care for Pediatric Irritable Bowel Syndrome, Functional Abdominal Pain, and Functional Abdominal Pain Syndrome.

    Science.gov (United States)

    Hoekman, Daniël R; Rutten, Juliette M T M; Vlieger, Arine M; Benninga, Marc A; Dijkgraaf, Marcel G W

    2015-11-01

    To estimate annual medical and nonmedical costs of care for children diagnosed with irritable bowel syndrome (IBS) or functional abdominal pain (syndrome; FAP/FAPS). Baseline data from children with IBS or FAP/FAPS who were included in a multicenter trial (NTR2725) in The Netherlands were analyzed. Patients' parents completed a questionnaire concerning usage of healthcare resources, travel costs, out-of-pocket expenses, productivity loss of parents, and supportive measures at school. Use of abdominal pain related prescription medication was derived from case reports forms. Total annual costs per patient were calculated as the sum of direct and indirect medical and nonmedical costs. Costs of initial diagnostic investigations were not included. A total of 258 children, mean age 13.4 years (±5.5), were included, and 183 (70.9%) were female. Total annual costs per patient were estimated to be €2512.31. Inpatient and outpatient healthcare use were major cost drivers, accounting for 22.5% and 35.2% of total annual costs, respectively. Parental productivity loss accounted for 22.2% of total annual costs. No difference was found in total costs between children with IBS or FAP/FAPS. Pediatric abdominal pain related functional gastrointestinal disorders impose a large economic burden on patients' families and healthcare systems. More than one-half of total annual costs of IBS and FAP/FAPS consist of inpatient and outpatient healthcare use. Netherlands Trial Registry: NTR2725. Copyright © 2015 Elsevier Inc. All rights reserved.

  1. Pediatric Cushing′s disease: Management Issues

    Directory of Open Access Journals (Sweden)

    Martin O Savage

    2012-01-01

    Full Text Available Cushing′s disease (CD, caused by an ACTH-secreting pituitary corticotroph adenoma, is the commonest cause of Cushing syndrome in children over 5 years of age. It is rare in the pediatric age range and presents difficult diagnostic and therapeutic challenges. Key presenting features include weight gain, growth failure and change in facial appearance. Most pediatric endocrinologists have limited experience managing children or adolescents with CD and thus benefit from close consultation with adult colleagues. We describe a diagnostic protocol which broadly follows the model for adult patients. Treatment strategies are examined and appraised. The management of pediatric CD patients after cure is also discussed.

  2. Pediatric microdose and microtracer studies using 14C in Europe.

    Science.gov (United States)

    Turner, M A; Mooij, M G; Vaes, W H J; Windhorst, A D; Hendrikse, N H; Knibbe, C A J; Kõrgvee, L T; Maruszak, W; Grynkiewicz, G; Garner, R C; Tibboel, D; Park, B K; de Wildt, S N

    2015-09-01

    Important information gaps remain on the efficacy and safety of drugs in children. Pediatric drug development encounters several ethical, practical, and scientific challenges. One barrier to the evaluation of medicines for children is a lack of innovative methodologies that have been adapted to the needs of children. This article presents our successful experience of pediatric microdose and microtracer studies using (14) C-labeled probes in Europe to illustrate the strengths and limitations of these approaches. © 2015 ASCPT.

  3. [Ethical principles of clinical trials in minors].

    Science.gov (United States)

    Koch, H J; Raschka, C

    2002-12-05

    Clinical trials in volunteers and patients are essential to ensure rational treatment of patients. As a rule, drugs are routinely developed for adults, but children are excluded. A major reason for this restriction are ethical justifications, in particular the lack of autonomy on the part of children. The principle of fairness, however, requires that everyone should benefit from progress. Industry, science and society are therefore called upon to find ways of making available safe and adequate treatment for children as quickly as possible, by defining the required conditions for pediatric clinical trials. Important principles are minimal risk, minimal invasivity, rapid decision-making, and careful documentation of trial results. Dynamic ethical principles, such as autonomy and competence in adolescents must be considered on equal footing with existing international GCP guidelines. Aspects of child psychology indicate that the autonomy of adolescents should be respected. Where economic incentives for such trials are absent, for example, in the case of non-pharmacological problems, pediatric trials must be considered a task for society as a whole.

  4. Disparities in policies, practices and rates of pediatric kidney transplantation in Europe

    DEFF Research Database (Denmark)

    Harambat, J; van Stralen, K J; Schaefer, F

    2013-01-01

    We aimed to provide an overview of kidney allocation policies related to children and pediatric kidney transplantation (KTx) practices and rates in Europe, and to study factors associated with KTx rates. A survey was distributed among renal registry representatives in 38 European countries...... pediatric KTx rate, and a lower proportion of living donor KTx. The rates of pediatric KTx, distribution of donor source and time on waiting list vary considerably between European countries. The lack of harmonization in kidney allocation to children raises medical and ethical issues. Harmonization...

  5. Treatment of complex regional pain syndrome type 1 in a pediatric patient using the lidocaine patch 5%: a case report

    Directory of Open Access Journals (Sweden)

    Steven G Frost, MD

    2003-09-01

    Full Text Available Background: Successful treatment of complex regional pain syndrome type 1 (CRPS-1 requires a coordinated, multidisciplinary approach. Physical rehabilitation is an important component of long-term treatment. Unfortunately, patients with significant allodynia or hyperalgesia characteristic of CRPS-1 often have difficulty progressing through a physical therapy (PT regimen. In most adults with CRPS-1, the treatment of choice is PO opioids. Objective: This article presents a case report of the use of the lidocaine patch 5%, a targeted peripheral analgesic, in a pediatric patient and its effects on reducing pain, improving the patient's overall attitude, and facilitating compliance with ongoing PT. Results: A 10-year-old girl developed CRPS-1 after arthroscopic surgery for a sprained ankle. Attempts at PT were unsuccessful due to inadequate pain relief from various treatment modalities. Therapy with the lidocaine patch 5% was initiated and resulted in significant pain relief, improvements in the patient's attitude, and progress with PT. Conclusion: This case report of a child with CRPS-1 showed that therapy with lidocaine patch 5% may be efficacious in treating children with pain resulting from CRPS-1, thereby increasing the success of PT. Keywords: complex regional pain syndrome, lidocaine patch 5%, targeted peripheral analgesic, pediatrics

  6. Congenital seminal vesicle cyst accompanying with ipsilateral renal agenesis in an adolescent patient: A pediatric radiologist approach to Zinner’s syndrome

    Directory of Open Access Journals (Sweden)

    Mehmet Burak Özkan

    2015-12-01

    Full Text Available A fifteen-year-old boy who had complaints of left sided pelvic pain with known ipsilateral left renal agenesia was referred to pediatric radiology department. Incidentally, his sonography examination revealed a dilated tubular structure located in the retro-vesicular region from cephalic to prostate. Contrast enhanced pelvic MRI showed a huge seminal vesicle cyst which is over 6 cm without a mass effect near the aspect border of the prostate and bladder. The patient was diagnosed with Zinner syndrome. The patient doesn’t have new complaint with no definite increase in the diameter of the cyst. In this case presentation we are discussing the Zinner syndrome’s imaging findings from a pediatric radiologist approach with a brief review of the literature.

  7. Factors influencing emergency nurses' ethical problems during the outbreak of MERS-CoV.

    Science.gov (United States)

    Choi, Jeong-Sil; Kim, Ji-Soo

    2018-05-01

    Whenever there has been a worldwide contagious disease outbreak, there have been reports of infection and death of healthcare workers. Particularly because emergency nurses have contact with patients on the front line, they experience ethical problems in nursing while struggling with infectious diseases in an unfavorable environment. The objective of this study was to explore emergency nurses' ethical problems and to identify factors influencing these problems during the outbreak of Middle East respiratory syndrome-coronavirus in Korea. For this cross-sectional study, a questionnaire survey was conducted with emergency nurses working in six hospitals selected through convenience sampling from the hospitals designated for Middle East respiratory syndrome-coronavirus patients in the capital area. Data were collected from 169 emergency nurses in Korea during August 2015. Ethical considerations: This research was approved by the Institutional Review Board of G University in Korea. The findings of this study suggest that during the Middle East respiratory syndrome-coronavirus outbreak, emergency nurses experienced ethical problems tied to a mind-set of avoiding patients. Three factors were found to influence emergency nurses' ethical problems (in order of influence): cognition of social stigmatization, level of agreement with infection control measures, and perceived risk. Through this study, we obtained information on emergency nurses' ethical problems during the Middle East respiratory syndrome-coronavirus outbreak and identified the factors that influence them. As found in this study, nurses' ethical problems were influenced most by cognitions of social stigmatization. Accordingly, to support nurses confidently care for people during future health disasters, it is most urgent to promote appropriate public consciousness that encourages healthcare workers.

  8. Pediatric central nervous system vascular malformations

    Energy Technology Data Exchange (ETDEWEB)

    Burch, Ezra A. [Brigham and Women' s Hospital, Department of Radiology, Boston, MA (United States); Orbach, Darren B. [Boston Children' s Hospital, Neurointerventional Radiology, Boston, MA (United States)

    2015-09-15

    Pediatric central nervous system (CNS) vascular anomalies include lesions found only in the pediatric population and also the full gamut of vascular lesions found in adults. Pediatric-specific lesions discussed here include infantile hemangioma, vein of Galen malformation and dural sinus malformation. Some CNS vascular lesions that occur in adults, such as arteriovenous malformation, have somewhat distinct manifestations in children, and those are also discussed. Additionally, children with CNS vascular malformations often have associated broader vascular conditions, e.g., PHACES (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, eye anomalies and sternal anomalies), hereditary hemorrhagic telangiectasia, and capillary malformation-arteriovenous malformation syndrome (related to the RASA1 mutation). The treatment of pediatric CNS vascular malformations has greatly benefited from advances in endovascular therapy, including technical advances in adult interventional neuroradiology. Dramatic advances in therapy are expected to stem from increased understanding of the genetics and vascular biology that underlie pediatric CNS vascular malformations. (orig.)

  9. Pediatric central nervous system vascular malformations

    International Nuclear Information System (INIS)

    Burch, Ezra A.; Orbach, Darren B.

    2015-01-01

    Pediatric central nervous system (CNS) vascular anomalies include lesions found only in the pediatric population and also the full gamut of vascular lesions found in adults. Pediatric-specific lesions discussed here include infantile hemangioma, vein of Galen malformation and dural sinus malformation. Some CNS vascular lesions that occur in adults, such as arteriovenous malformation, have somewhat distinct manifestations in children, and those are also discussed. Additionally, children with CNS vascular malformations often have associated broader vascular conditions, e.g., PHACES (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, eye anomalies and sternal anomalies), hereditary hemorrhagic telangiectasia, and capillary malformation-arteriovenous malformation syndrome (related to the RASA1 mutation). The treatment of pediatric CNS vascular malformations has greatly benefited from advances in endovascular therapy, including technical advances in adult interventional neuroradiology. Dramatic advances in therapy are expected to stem from increased understanding of the genetics and vascular biology that underlie pediatric CNS vascular malformations. (orig.)

  10. The moral landscape of pediatric oncology: an empirical study on best interests, parental authority and child participation in decision making

    OpenAIRE

    Vries, Martine Charlotte de

    2012-01-01

    Few medical specialties encounter so many ethical challenges as pediatrics does. It is a specialty that inherently has features that are morally charged. Pediatric ethics examines the broad issues of (1) the concept of the child’s best interest; (2) parental responsibility and authority in decision-making about the life and health of a child; (3) the emerging desire and capacity for self-determination of an older child, and (4) the professional obligation of a pediatrician to act in the best ...

  11. Canakinumab (ACZ885, a fully human IgG1 anti-IL-1β mAb) induces sustained remission in pediatric patients with cryopyrin-associated periodic syndrome (CAPS).

    Science.gov (United States)

    Kuemmerle-Deschner, Jasmin B; Ramos, Eduardo; Blank, Norbert; Roesler, Joachim; Felix, Sandra D; Jung, Thomas; Stricker, Kirstin; Chakraborty, Abhijit; Tannenbaum, Stacey; Wright, Andrew M; Rordorf, Christiane

    2011-02-28

    Cryopyrin-associated periodic syndrome (CAPS) represents a spectrum of three auto-inflammatory syndromes, familial cold auto-inflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease/chronic infantile neurological cutaneous and articular syndrome (NOMID/CINCA) with etiology linked to mutations in the NLRP3 gene resulting in elevated interleukin-1β (IL-1β) release. CAPS is a rare hereditary auto-inflammatory disease, which may start early in childhood and requires a life-long treatment. Canakinumab, a fully human anti-IL-1β antibody, produces sustained selective inhibition of IL-1β. This study was conducted to assess the efficacy, safety, and pharmacokinetics of canakinumab in the treatment of pediatric CAPS patients. Seven pediatric patients (five children and two adolescents) with CAPS were enrolled in a phase II, open-label study of canakinumab in patients with CAPS. Canakinumab was administered at a dose of 2 mg/kg subcutaneously (s.c.) (for patients with body weight ≤ 40 kg) or 150 mg s.c. (for patients with body weight > 40 kg) with re-dosing upon each relapse. The primary efficacy variable was time to relapse following achievement of a complete response (defined as a global assessment of no or minimal disease activity and no or minimal rash and values for serum C-reactive protein (CRP) and/or serum amyloid A (SAA) within the normal range, CAPS. ClinicalTrials.gov: NCT00487708.

  12. Oral Rehabilitation for Amniotic Band Syndrome: An Unusual Presentation.

    Science.gov (United States)

    Hotwani, Kavita; Sharma, Krishna

    2015-01-01

    Amniotic band syndrome (ABS) is a congenital disorder caused by entrapment of fetal parts in fibrous amniotic bands while in utero. The syndrome is underdiagnosed and its presentation is variable. The syndrome has been well described in the pediatric, orthopedic and obstetric literature; however, despite the discernable craniomaxillofacial involvement, ABS has not been reported in the dental literature very often. The present report describes a case of a patient with ABS and concomitant dental findings. How to cite this article: Hotwani K, Sharma K. Oral Rehabilitation for Amniotic Band Syndrome: An Unusual Presentation. Int J Clin Pediatr Dent 2015;8(1):55-57.

  13. [Ethical Dilemmas in Practice of Medicine Child].

    Science.gov (United States)

    Ruiz López, Justo; Navarro Zaragoza, Javier; Carrillo Navarro, Francisco; Luna Maldonado, Aurelio

    2017-01-01

    After reviewing the existing bibliography in the last 20 years, we concluded that there is a lack of information regarding the ethical conflicts that affect to pediatrics in their daily practice. It produces certain degree of uncertainty in these professionals at the time of solving these problems. We made a systematic search in the main data bases, finding more than 150 articles related, of which 80 were considered outstanding. After studying them, we have found 40 ethical dilemmas, related to some principle of solution and that we described in this article. Through them we can find such important dilemmas as those related to physical disability, palliative care or consent from children.

  14. The Globalization of Pediatric Research: An Analysis of Clinical Trials Completed for Pediatric Exclusivity

    Science.gov (United States)

    Pasquali, Sara K.; Burstein, Danielle S.; Benjamin, Daniel K.; Smith, P. Brian; Li, Jennifer S.

    2010-01-01

    Background Recent studies have examined the globalization of clinical research. These studies focused on adult trials, and the globalization of pediatric research has not been examined to date. We evaluated the setting of published studies conducted under the US Pediatric Exclusivity Program, which provides economic incentives to pharmaceutical companies to conduct drug studies in children. Methods Published studies containing the main results of trials conducted from 1998–2007 under the Pediatric Exclusivity Provision were included. Data were extracted from each study and described, including the therapeutic area of drug studied, number of patients enrolled, number of sites, and location where the study was conducted, if reported. Results Overall, 174 trials were included (sample size 8–27,065 patients); 9% did not report any information regarding the location or number of sites where the study was conducted. Of those that did report this information, 65% were conducted in at least one country outside the US, and 11% did not have any sites in the US. Fifty-four different countries were represented and 38% of trials enrolled patients in at least one site located in a developing/transition country, including more than one third of infectious disease, cardiovascular, and allergy/immunology trials. Conclusions The majority of published pediatric trials conducted under the Pediatric Exclusivity Provision included sites outside of the US, and over a third of trials enrolled patients in developing/transition countries. While there are many potential benefits to the globalization of pediatric research, this trend also raises certain scientific and ethical concerns which require further evaluation. PMID:20732941

  15. Clinical manifestations and management of prune-belly syndrome in a large contemporary pediatric population.

    Science.gov (United States)

    Seidel, Natan E; Arlen, Angela M; Smith, Edwin A; Kirsch, Andrew J

    2015-01-01

    To review the clinical manifestations and operative management of a large contemporary pediatric cohort of patients with prune-belly syndrome (PBS). PBS patients aged <21 years followed up in our pediatric urology clinic were identified by the International Classification of Diseases, Ninth Revision code (756.71). Demographics, concomitant diagnoses, surgical history, imaging studies, and renal or bladder function were evaluated. Data were available for 46 pediatric patients (44 boys and 2 girls). Mean age was 7.6 ± 4.7 years (range, 0.9-20 years). Average length of clinical follow-up was 6.8 ± 5 years. Forty-five children (97.8%) had hydroureteronephrosis, and 36 of them (78.3%) had vesicoureteral reflux. Five patients (10.9%) had significant pulmonary insufficiency, and 2 patients (4.3%) were oxygen dependent. Eighteen children (39.1%) had other congenital malformations, including cardiac in 4 patients (8.7%) and musculoskeletal anomalies in 10 patients (21.7%). Orchidopexy was the most common surgery, with all boys aged ≥3 years having undergone the procedure. Twenty-two patients (47.8%) had a history of ureteral surgery, 22 (47.8%) had bladder surgery, 11 (23.9%) had renal surgery, and 6 (13%) had urethral procedures. Nineteen patients (41.3%) underwent abdominoplasty. Eighteen children (39.1%) had documented chronic kidney disease, and 8 children (17.4%) underwent renal transplantation. Average age at transplantation was 5.1 ± 2.9 years. The mean nadir creatinine level for patients with end-stage renal disease was 1.4 mg/dL compared with 0.4 mg/dL for those not requiring transplantation (P <.001). Children with PBS have significant comorbidities and require frequent operative intervention, with disease heterogeneity necessitating an individualized management approach. Early end-stage renal disease is prevalent, with approximately 15% of children requiring kidney transplantation. Copyright © 2015 Elsevier Inc. All rights reserved.

  16. Pediatric Microdose and Microtracer Studies Using 14C in Europe

    NARCIS (Netherlands)

    M.A. Turner; M.G. Mooij (Miriam); W.H.J. Vaes (Wouter H. J.); A.D. Windhorst (Albert); N.H. Hendrikse (N. Harry); C.A.J. Knibbe (Catherijne); L.T. Kõrgvee; W. Maruszak; G. Grynkiewicz; R.C. Garner; D. Tibboel (Dick); B.K. Park; S.N. de Wildt (Saskia)

    2015-01-01

    textabstractImportant information gaps remain on the efficacy and safety of drugs in children. Pediatric drug development encounters several ethical, practical, and scientific challenges. One barrier to the evaluation of medicines for children is a lack of innovative methodologies that have been

  17. Current approaches for informed consent in pediatrics

    Directory of Open Access Journals (Sweden)

    Hatice Betül Gemici

    2014-09-01

    Full Text Available Today in medical practis; codes of ethic are becoming important. New aprroaches has emerged that about to participate with patient’s consent and child absent understand the own situation should be taken for treatment or process on child. Means of child informing is to given simple information about understandable language. Physicians should support to be shared with the patient’s medical decision according to the child’s age and understanding capacity. Informed consent in pediatric patients makes contribution to develepmont of future individuals; therefore that is an important ethic assignment for doctors. J Clin Exp Invest 2014; 5 (3: 496-503

  18. HRV biofeedback for pediatric irritable bowel syndrome and functional abdominal pain: a clinical replication series.

    Science.gov (United States)

    Stern, Mark J; Guiles, Robert A F; Gevirtz, Richard

    2014-12-01

    Irritable bowel syndrome (IBS) and Functional Abdominal Pain (FAP) are among the most commonly reported Functional Gastrointestinal Disorders. Both have been associated with varying autonomic dysregulation. Heart Rate Variability Biofeedback (HRVB) has recently begun to show efficacy in the treatment of both IBS and FAP. The purpose of this multiple clinical replication series was to analyze the clinical outcomes of utilizing HRVB in a clinical setting. Archival data of twenty-seven consecutive pediatric outpatients diagnosed with IBS or FAP who received HRVB were analyzed. Clinical outcomes were self-report and categorized as full or remission with patient satisfaction, or no improvement. Qualitative reports of patient experiences were also noted. Full remission was achieved by 69.2 % and partial remission was achieved by 30.8 % of IBS patients. Full remission was achieved by 63.6 % and partial remission was achieved by 36.4 % of FAP patients. No patients in either group did not improve to a level of patient satisfaction or >50 %. Patient's commonly reported feeling validated in their discomfort as a result of psychophysiological education. Results suggest that HRVB is a promising intervention for pediatric outpatients with IBS or FAP. Randomized controlled trials are necessary to accurately determine clinical efficacy of HRVB in the treatment of IBS and FAP.

  19. Researchers' perceptions of the ethical implications of pharmacogenomics research with children.

    Science.gov (United States)

    Avard, D; Silverstein, T; Sillon, G; Joly, Y

    2009-01-01

    This paper presents the results of an exploratory qualitative study that assesses Canadian pediatric researchers' perceptions of a pre-selected group of ethical issues raised by pharmacogenomics research with children. As a pilot study, we conducted semi-structured telephone interviews with Canadian pediatric pharmacogenomic researchers. The interviews were guided by the following themes: (1) benefits and risks of inclusion, (2) the consent/assent process, and (3) the return of research results. Issues about assent, consent, risks and benefits, as well as the communication of results were addressed by the respondents. Some issues, such as the unique vulnerability of children, the long term privacy concerns associated with biobanking, additional core elements that need to be discussed and included in the consent/assent forms, as well as the challenges of communicating research results in a pediatric research were not explicitly identified by the respondents. Further consideration should be given to address the ethical challenges of including children in pharmacogenomics research. This exploratory study indicates that further guidance is needed if children are to be protected and yet benefit from such research. Copyright 2009 S. Karger AG, Basel.

  20. Pediatric epilepsy: The Indian experience.

    Science.gov (United States)

    Gadgil, Pradnya; Udani, Vrajesh

    2011-10-01

    Epilepsy is a common clinical entity in neurology clinics. The understanding of the genetics of epilepsy has undergone a sea change prompting re-classification by the International league against epilepsy recently. The prevalence rates of epilepsy in India are similar to those of developed nations. However, the large treatment gap is a major challenge to our public health system. Perinatal injuries are a major causative factor in pediatric group. We have discussed a few common etiologies such as neurocysticercosis and newer genetic epilepsy syndromes. We have also briefly touched upon the Indian experience in pediatric epilepsy surgery.

  1. Delirium in Pediatric Critical Care.

    Science.gov (United States)

    Patel, Anita K; Bell, Michael J; Traube, Chani

    2017-10-01

    Delirium occurs frequently in the critically ill child. It is a syndrome characterized by an acute onset and fluctuating course, with behaviors that reflect a disturbance in awareness and cognition. Delirium represents global cerebral dysfunction due to the direct physiologic effects of an underlying medical illness or its treatment. Pediatric delirium is strongly associated with poor outcomes, including increased mortality, prolonged intensive care unit length of stay, longer time on mechanical ventilation, and increased cost of care. With heightened awareness, the pediatric intensivist can detect, treat, and prevent delirium in at-risk children. Copyright © 2017 Elsevier Inc. All rights reserved.

  2. Optic nerve pilomyxoid astrocytoma in a patient with Noonan syndrome.

    Science.gov (United States)

    Nair, Sushmita; Fort, John A; Yachnis, Anthony T; Williams, Charles A

    2015-06-01

    Noonan syndrome (NS; MIM 163950) is an autosomal dominant syndrome which is clinically diagnosed by the distinct facial features, short stature, cardiac anomalies and developmental delay. About 50% of cases are associated with gain of function mutations in PTPN11 gene which leads to activation of the RAS/mitogen-activated protein kinase signaling pathway. This is known to have a role in tumorigenesis. Despite this, only limited reports of solid tumors (Fryssira H, Leventopoulos G, Psoni S, et al. Tumor development in three patients with Noonan syndrome. Eur J Pediatr 2008;167:1025-1031; Schuettpelz LG, McDonald S, Whitesell K et al. Pilocytic astrocytoma in a child with Noonan syndrome. Pediatr Blood Cancer 2009;53:1147-1149; Sherman CB, Ali-Nazir A, Gonzales-Gomez I, et al. Primary mixed glioneuronal tumor of the central nervous system in a patient with Noonan syndrome. J Pediatr Hematol Oncol 2009;31:61-64; Sanford RA, Bowman R, Tomita T, et al. A 16 year old male with Noonan's syndrome develops progressive scoliosis and deteriorating gait. Pediatr Neurosurg 1999;30:47-52) and no prior reports of optic gliomas have been described in patients with NS. We present here a patient with NS with a PTPN11 mutation and an optic pathway pilomyxoid astrocytoma. © 2015 Wiley Periodicals, Inc.

  3. Bioethical Issues in Conducting Pediatric Dentistry Clinical Research.

    Science.gov (United States)

    Garrocho-Rangel, Arturo; Cerda-Cristerna, Bernardino; Pozos-Guillen, Amaury

    Pediatric clinical research on new drugs and biomaterials involves children in order to create valid and generalizable knowledge. Research on vulnerable populations, such as children, is necessary but only admissible when researchers strictly follow methodological and ethical standards, together with the respect to human rights; and very especially when the investigation cannot be conducted with other population or when the potential benefits are specifically for that age group. Clinical research in Pediatric Dentistry is not an exception. The aim of the present article was to provide the bioethical principles (with respect to the child/parents' autonomy, benefit/risk analysis, and distributive justice), and recommendations, including informed consent, research ethics committees, conflict of interest, and the "equipoise" concept. Current and future worldwide oral health research in children and adolescents must be conducted incorporating their perspectives in the decision-making process as completely as possible. This concept must be carefully considered when a dental clinical study research is going to be planned and conducted, especially in the case of randomized controlled trials, in which children will be recruited as participants.

  4. Pediatric health, medicine, and therapeutics

    Directory of Open Access Journals (Sweden)

    Claire E Wainwright

    2011-03-01

    Full Text Available Claire E Wainwright1,21Royal Children’s Hospital, Brisbane and Queensland, Queensland, Australia; 2Queensland Children’s Medical Research Institute, The University of Queensland, Brisbane, AustraliaThe idea of children as small adults with health care needs that can be managed by extrapolation from adult studies has now largely been abandoned. We now recognize that adult health and disease are closely linked to childhood factors and the critical and ethical importance of clinical research in pediatrics is increasingly being recognized.  While funding and output from pediatric clinical research continues to lag behind health research in adults, particularly in the area of therapeutics, the last decade has thankfully seen a dramatic increase in the number of pediatric studies and particularly randomized controlled clinical trials (RCTs. Since the 1997 Food and Drug Administration (FDA Modernization Act in the United States (US and the subsequent changes in drug registration regulatory systems in the US and Europe, there has been a huge increase in the number of pediatric studies sponsored by pharmaceutical companies. In the United Kingdom, the Medicine for Children’s Research Network was established in 2005 to address the lack of clinical studies in pediatrics. Over the first five years they reported an exciting increase in the number of high quality clinical studies and on their website they have a current portfolio of over 200 pediatric studies, half of which are RCTs and half are sponsored by pharmaceutical companies. Other countries particularly across Europe are also establishing similar programs. 

  5. Pediatric Sjogren syndrome with distal renal tubular acidosis and autoimmune hypothyroidism: an uncommon association.

    Science.gov (United States)

    Agarwal, Amit; Kumar, Pradeep; Gupta, Nomeeta

    2015-11-01

    A 14-year-old female came with the history of sudden onset weakness; during work up, she was found to have hyperchloremic metabolic acidosis with normal anion gap and normal renal function suggesting the possibility of renal tubular acidosis (RTA). On further evaluation of RTA, she had positive antinuclear antibody, anti-Ro, and anti-La antibodies. On nuclear scan of salivary glands, her left parotid gland was nonfunctional. Her parotid biopsy revealed dilated interlobular ducts engulfed by lymphoid cells. She also had autoimmune hypothyroidism as suggested by raised TSH and positive anti-TPO antibodies. At admission, her serum potassium levels were low and she was treated with intravenous potassium chloride. After she recovered from acute hypokalemic paralysis, she was started on oral potassium citrate along with phosphate supplements, hydroxychloroquine, oral prednisolone and thyroxine supplements. Over the next 6 months, she has significant reduction in the dosage of potassium, bicarbonate and phosphate and gained 3 kg of weight and 3.5 cm of height. As primary Sjogren syndrome itself is rare in pediatric population and its association with renal tubular acidosis is even rarer, we suggest considering Sjogren syndrome as a differential diagnosis during the RTA work-up is worth trying.

  6. Safety and efficacy of aripiprazole for the treatment of pediatric Tourette syndrome and other chronic tic disorders

    Directory of Open Access Journals (Sweden)

    Cox JH

    2016-06-01

    Full Text Available Joanna H Cox,1 Stefano Seri,2,3 Andrea E Cavanna,2,4,5 1Heart of England NHS Foundation Trust, 2School of Life and Health Sciences, Aston Brain Centre, Aston University, 3Children’s Epilepsy Surgery Programme, The Birmingham Children’s Hospital NHS Foundation Trust, 4Department of Neuropsychiatry, Birmingham and Solihull Mental Health NHS Foundation Trust, Birmingham, 5Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology and UCL, London, UK Abstract: Tourette syndrome is a childhood-onset chronic tic disorder characterized by multiple motor and vocal tics and often accompanied by specific behavioral symptoms ranging from obsessionality to impulsivity. A considerable proportion of patients report significant impairment in health-related quality of life caused by the severity of their tics and behavioral symptoms and require medical intervention. The most commonly used medications are antidopaminergic agents, which have been consistently shown to be effective for tic control, but are also associated with poor tolerability because of their adverse effects. The newer antipsychotic medication aripiprazole is characterized by a unique mechanism of action (D2 partial agonism, and over the last decade has increasingly been used for the treatment of tics. We conducted a systematic literature review to assess the available evidence on the efficacy and safety of aripiprazole in pediatric patients with Tourette syndrome and other chronic tic disorders (age range: 4–18 years. Our search identified two randomized controlled trials (involving 60 and 61 participants and ten open-label studies (involving between six and 81 participants. The majority of these studies used two validated clinician-rated instruments (Yale Global Tic Severity Scale and Clinical Global Impression scale as primary outcome measures. The combined results from randomized controlled trials and open-label studies showed that aripiprazole is an

  7. Pediatric clinical drug trials in low-income countries: key ethical issues.

    Science.gov (United States)

    MacLeod, S M; Knoppert, D C; Stanton-Jean, M; Avard, D

    2015-02-01

    Potential child participants in clinical research trials in low-income countries are often vulnerable because of poverty, high morbidity and mortality, inadequate education, and varied local cultural norms. However, vulnerability by itself must not be accepted as an obstacle blocking children from the health benefits that may accrue as an outcome of sound clinical research. As greater emphasis is placed on evidence-based treatment of children, it should be anticipated that there will be a growing call for agreement on principles to guide clinical investigations in low-income countries. There is now general acceptance of the view that children must be protected from non-evidence-based interventions and from substandard treatments. The questions remaining relate to how best to stimulate clinical research activity that will serve the needs of infants, children, and youth in developing countries and how best to assign priority to ethically sound research that will meet their clinical requirements. In low-income countries, 39 % of citizens are 13 years of age or younger, and consequently it is certain that clinical investigations of some new therapeutic products will be conducted there more frequently. This review offers some suggestions for approaches that will help to achieve more effective ethical consideration, including (1) improving the quality of research ethics boards; (2) fostering collaborative partnerships among important stakeholders; (3) making concerted efforts to build capacity; (4) improving the quality of the consent and waiver process; and (5) developing improved governance for harmonized ethics platforms. Continuing support by international organizations is required to sustain the establishment and maintenance of stronger research ethics boards to protect children enrolled in clinical trials. This review underscores the importance of developing a culture of solidarity and true partnership between developed and low-income country organizations, which

  8. Pediatric Acute Respiratory Distress Syndrome: Fluid Management in the PICU

    NARCIS (Netherlands)

    Ingelse, Sarah A.; Wösten-van Asperen, Roelie M.; Lemson, Joris; Daams, Joost G.; Bem, Reinout A.; van Woensel, Job B.

    2016-01-01

    The administration of an appropriate volume of intravenous fluids, while avoiding fluid overload, is a major challenge in the pediatric intensive care unit. Despite our efforts, fluid overload is a very common clinical observation in critically ill children, in particular in those with pediatric

  9. [Ethical Evaluation of Non-Therapeutic Male Circumcision].

    Science.gov (United States)

    İzgi, M Cumhur

    2015-01-01

    Elective circumcision for nonmedical reasons is a surgical approach which is historically long standing and accepted as the most performed procedure. The necessity of the procedure is usually for religious and traditional reasons alongside some medical ground related benefits to enable its social acceptability. The discussion of the subject from the aspect of ethics becomes necessary as there is no consensus about the benefits or harmfulness of nonmedical circumcision. Fundamental ethical discussions about circumcision, which contradicts legal acceptance criteria of any medical application, are related to the basic concepts of the existence of an individual such as sovereignty, the loss of bodily integrity, and privacy. The recent legal processes and the fact that the European Council and the American Academy of Pediatrics have put the issue on their agenda have increased the necessity of these ethical evaluations. The responsibility of consideration and evaluation of ethical permission of every circumcision procedure, besides discussing the necessity of circumcision for improvement and protection of health rests on the shoulders of the physicians because the dignity and intellectual identity of the profession require so.

  10. Posterior reversible encephalopathy syndrome in a B-cell acute lymphoblastic leukemia young adult patient treated with a pediatric-like chemotherapeutic schedule

    Directory of Open Access Journals (Sweden)

    Cristina Papayannidis

    2014-09-01

    Full Text Available We report here the case of a young adult affected by pre B-cell acute lymphoblastic leukemia (ALL, who developed, during a pediatric-like chemotherapy consolidation schedule with high dosage of Methotrexate, a severe neurological toxicity. Clinical presentation and neuroimaging data were diagnostic for posterior reversible encephalopathy syndrome (PRES. A complete resolution was quickly obtained with medical blood pressure control and anticonvulsants administration. To the best of our knowledge, this is the first case of PRES described in the adult ALL setting. Currently, the clinical management of this aggressive disease is moving towards a pediatric-like approach also in adult patients, due to the better outcome reached with intensive chemotherapeutic regimens in children population. However, therapy-related toxicities have to be taken into account, since their onset may adversely affect patients’ clinical outcome.

  11. Role of flexible bronchoscopy and bronchoalveolar lavage in the diagnosis of pediatric acquired immunodeficiency syndrome-related pulmonary disease.

    Science.gov (United States)

    Birriel, J A; Adams, J A; Saldana, M A; Mavunda, K; Goldfinger, S; Vernon, D; Holzman, B; McKey, R M

    1991-06-01

    Flexible fiberoptic bronchoscopy with bronchoalveolar lavage was performed in 16 pediatric patients with the acquired immunodeficiency syndrome (AIDS) and deterioration in pulmonary function suggestive of opportunistic infection. In 62% of the patients Pneumocystis carinii was identified. Culture results showed a pure growth of Pseudomonas aeruginosa for one patient in addition to the Pneumocystis carinii. Bronchoscopy with lavage was well tolerated, with few complications even among patients with significant tachypnea and hypoxia. Because of its relative safety and effectiveness, this procedure should be considered the first invasive measurement used for evaluation of parenchymal lung disease in this population of patients.

  12. Decreased ADAMTS 13 Activity is Associated With Disease Severity and Outcome in Pediatric Severe Sepsis

    Science.gov (United States)

    Lin, Jainn-Jim; Chan, Oi-Wa; Hsiao, Hsiang-Ju; Wang, Yu; Hsia, Shao-Hsuan; Chiu, Cheng-Hsun

    2016-01-01

    Abstract Decreased ADAMTS 13 activity has been reported in severe sepsis and in sepsis-induced disseminated intravascular coagulation. This study aimed to investigate the role of ADAMTS 13 in different pediatric sepsis syndromes and evaluate its relationship with disease severity and outcome. We prospectively collected cases of sepsis treated in a pediatric intensive care unit, between July 2012 and June 2014 in Chang Gung Children's Hospital in Taoyuan, Taiwan. Clinical characteristics and ADAMTS-13 activity were analyzed. All sepsis syndromes had decreased ADAMTS 13 activity on days 1 and 3 of admission compared to healthy controls. Patients with septic shock had significantly decreased ADAMTS 13 activity on days 1 and 3 compared to those with sepsis and severe sepsis. There was a significant negative correlation between ADAMTS 13 activity on day 1 and day 1 PRISM-II, PELOD, P-MOD, and DIC scores. Patients with mortality had significantly decreased ADAMTS 13 activity on day 1 than survivors, but not on day 3. Different pediatric sepsis syndromes have varying degrees of decreased ADAMTS 13 activity. ADAMTS 13 activity is strongly negatively correlated with disease severity of pediatric sepsis syndrome, whereas decreased ADAMTS 13 activity on day 1 is associated with increased risk of mortality. PMID:27100422

  13. Female fertility preservation in the pediatric and adolescent cancer patient population.

    Science.gov (United States)

    Algarroba, Gabriela N; Sanfilippo, Joseph S; Valli-Pulaski, Hanna

    2018-04-01

    The 5-year survival rate for childhood cancer is over 80%, thereby increasing the number of young women facing infertility in the future because of the gonadotoxic effects of chemotherapy and radiation. The gonadotoxic effects of childhood cancer treatment vary by the radiation regimen and the chemotherapeutic drugs utilized. Although the American Society of Clinical Oncology guidelines recommend fertility preservation for all patients, there are several barriers and ethical considerations to fertility preservation in the pediatric and adolescent female population. Additionally, the fertility preservation methods for pre- and postpubertal females differ, with only experimental methods available for prepubertal females. We will review the risk of chemotherapy and radiation on female fertility, the approach to fertility preservation in the pediatric and adolescent female population, methods of fertility preservation for both pre- and postpubertal females, barriers to fertility preservation, cost, and psychological and ethical considerations. Copyright © 2018 Elsevier Ltd. All rights reserved.

  14. Prevalence of the metabolic syndrome among North Indian adolescents using Adult Treatment Panel III and pediatric International Diabetic Federation definitions

    Directory of Open Access Journals (Sweden)

    Riyaz Ahmad Bhat

    2015-01-01

    Full Text Available Background and Objective: Childhood obesity is an important risk factor for the development of metabolic syndrome (MS in children and adolescent. Because of high prevalence of insulin resistance and MS in Indian adult population, studies are needed to identify the prevalence of these metabolic abnormalities in the adolescent population. The objective of this study was to estimate the prevalence of MS using pediatric International Diabetic Federation (IDF definition and compare it with estimates of Adult Treatment Panel III (ATP III definition among adolescents in Northern India. Materials and Methods: At a total of 899 adolescents attending school (aged 10-18 years participated in this population-based prospective study. All the clinical and biochemical assessment were done after proper consent. The MS was determined by the National Cholesterol Education Program ATP III definition modified for age and pediatric IDF definition. Results: The prevalence of MS was 3.5% according to ATP III criteria and 1.5% based on IDF criteria. No significant gender difference was observed in the distribution of MS. Hypertriglyceridemia was the most common and abdominal obesity the least common constituent of MS. Conclusion: This study provides the first estimates of MS using pediatric IDF definition in the adolescent population from Northern India.

  15. [Reporting ethics board approval in German medical theses and journals].

    Science.gov (United States)

    Zenz, Michael; Zenz, Julia; Grieger, Maximilian

    2018-06-05

    Since 1975, the Declaration of Helsinki of the World Medical Association (WMA) has clearly required ethics committee approval for research into humans. Nevertheless, this Declaration is violated quite often. As many English-language publications have addressed the theme of ethics board approval in theses and other published works, it is now to be investigated in Germany for the first time.From 2013 to 2014, a total of 1,482 medical theses at four selected universities in addition to three German-language scientific journals were reviewed. In 543 theses, reference to ethics approval would have been required according to the criteria of the Declaration of Helsinki.However, ethics approval was stated in only 58.7% of cases, and even less frequently if the prevailing doctoral regulations or instructions did not refer to the necessity of obtaining ethics approval. Theses on pediatrics mentioned ethics approval most frequently (78.6%), whereas the proportion of surgical papers was the lowest (34.9%). Among the journals, Der Nervenarzt mentioned ethics approval most frequently (59.4%) and Der Chirurg least frequently (30%).Our results point to significant deficits in mentioning ethics approval in medical theses and publications. These deficits could easily be compensated for by a thorough approach of the referees of doctoral regulations and by journal reviewers and editors.

  16. Pediatric Mïcrodose and Microtracer Studies Using 14C in Europe

    NARCIS (Netherlands)

    Turner, M.A.; Mooij, M.G.; Vaes, W.H.J.; Windhorst, A.D.; Hendrikse, N.H.; Knibbe, C.A.J.; Körgvee, L.T.; Maruszak, W.; Grynkiewicz, G.; Garner, R.C.; Tibbocl, D.; Park, B.K.; Wildt, S.N. de

    2015-01-01

    Important information gaps remain on the efficacy and safety of drugs in children. Pediatric drug development encounters several ethical, practical, and scientifîc chaflenges One barrier to the evaluation of medicines for children is a lack of Innovative methodologies that have been adapted to the

  17. Using information technology and social networking for recruitment of research participants: experience from an exploratory study of pediatric Klinefelter syndrome.

    Science.gov (United States)

    Close, Sharron; Smaldone, Arlene; Fennoy, Ilene; Reame, Nancy; Grey, Margaret

    2013-03-19

    Recruiting pediatric samples for research may be challenging due to parental mistrust of the research process, privacy concerns, and family time constraints. Recruitment of children with chronic and genetic conditions may further complicate the enrollment process. In this paper, we describe the methodological challenges of recruiting children for research and provide an exemplar of how the use of information technology (IT) strategies with social networking may improve access to difficult-to-reach pediatric research participants. We conducted a cross-sectional descriptive study of boys between the ages of 8 and 18 years with Klinefelter syndrome. This study presented unique challenges for recruitment of pediatric participants. These challenges are illustrated by the report of recruitment activities developed for the study. We reviewed the literature to explore the issues of recruiting children for research using conventional and IT approaches. Success rates of conventional recruitment approaches, such as brochures, flyers in medical offices, and physician referrals, are compared with IT-based outreach. The IT approaches included teleconferencing via a Klinefelter syndrome support group, services of a Web-based commercial recruitment-matching company, and the development of a university-affiliated research recruitment website with the use of paid advertising on a social networking website (Facebook). Over a 3-month period, dissemination of over 150 recruitment brochures and flyers placed in a large urban hospital and hospital-affiliated clinical offices, with 850 letters to physicians and patients were not successful. Within the same period, face-to-face recruitment in the clinical setting yielded 4 (9%) participants. Using Web-based and social networking approaches, 39 (91%) agreed to participate in the study. With these approaches, 5 (12%) were recruited from the national Klinefelter syndrome advocacy group, 8 (19%) from local and teleconference support groups, 10

  18. Global Ethics Applied: Global Ethics, Economic Ethics

    OpenAIRE

    Stückelberger, Christoph

    2016-01-01

    Global Ethics Applied’ in four volumes is a reader of 88 selected articles from the author on 13 domains: Vol. 1 Global Ethics, Economic Ethics; Vol. 2 Environmental Ethics; Vol. 3 Development Ethics, Political Ethics, Dialogue and Peace Ethics, Innovation and Research Ethics, Information and Communication Ethics; Vol. 4 Bioethics and Medical Ethics, Family Ethics and Sexual Ethics, Leadership Ethics, Theological Ethics and Ecclesiology, Methods of Ethics. It concludes with the extended Bibli...

  19. [Microalbuminuria in pediatric patients diagnosed with hemolytic uremic syndrome].

    Science.gov (United States)

    Cubillos C, María Paz; Del Salas, Paulina; Zambrano, Pedro O

    2015-01-01

    Hemolytic uremic syndrome (HUS) is characterized by the presence of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney failure. It is the leading cause of acute kidney failure in children under 3 years of age. A variable number of patients develop proteinuria, hypertension, and chronic renal failure. To evaluate the renal involvement in pediatric patients diagnosed with HUS using the microalbumin/creatinine ratio. Descriptive concurrent cohort study that analyzed the presence of microalbuminuria in patients diagnosed with HUS between January 2001 and March 2012, who evolved without hypertension and normal renal function (clearance greater than 90ml/min using Schwartz formula). Demographic factors (age, sex), clinical presentation at time of diagnosis, use of antibiotics prior to admission, and need for renal replacement therapy were evaluated. Of the 24 patients studied, 54% were male. The mean age at diagnosis was two years. Peritoneal dialysis was required in 45%, and 33% developed persistent microalbuminuria. Antiproteinuric treatment was introduce in 4 patients, with good response. The mean follow-up was 6 years (range 6 months to 11 years). The serum creatinine returned to normal in all patients during follow up. The percentage of persistent microalbuminuria found in patients with a previous diagnosis of HUS was similar in our group to that described in the literature. Antiproteinuric treatment could delay kidney damage, but further multicenter prospective studies are necessary. Copyright © 2015. Publicado por Elsevier España, S.L.U.

  20. Ethical, Legal, and Clinical Considerations when Disclosing a High-Risk Syndrome for Psychosis.

    Science.gov (United States)

    Mittal, Vijay A; Dean, Derek J; Mittal, Jyoti; Saks, Elyn R

    2015-10-01

    There are complex considerations when planning to disclose an attenuated psychosis syndrome (APS) diagnosis. In this review, we evaluate ethical, legal, and clinical perspectives as well as caveats related to full, non- and partial disclosure strategies, discuss societal implications, and provide clinical suggestions. Each of the disclosure strategies is associated with benefits as well as costs/considerations. Full disclosure promotes autonomy, allows for the clearest psychoeducation about additional risk factors, helps to clarify and/or correct previous diagnoses/treatments, facilitates early intervention and bolsters communication between providers but there are important considerations involving heritability, comorbidity, culture, and stigma. Non-disclosure advances nonmaleficence by limiting stigma and stress (which may inadvertently exacerbate the condition), and confusion (related to the rapidly evolving diagnosis) in a sensitive developmental period but is complicated by varying patient preferences and the possibility that, as new treatments without adverse effects become available, the risk with false positives no longer justifies the accompanying loss of autonomy. Partial disclosure balances ethical considerations by focusing on symptoms instead of labels, but evidence that laypersons may interpret this information as a pseudo-diagnosis and that symptoms alone also contribute to stigma limits the efficacy of this approach. In addition, there are notable societal considerations relating to disclosure involving conservatorship, the reach of insurance companies, and discrimination. We advocate a hybrid approach to disclosure and recommend future research aimed at understanding the effects of stigma on clinical course and a renewed focus on those help-seeking cases that do not transition but remain clinically relevant. © 2015 John Wiley & Sons Ltd.

  1. CAM for Pediatric Pain: What is State-of-the-Research?

    Directory of Open Access Journals (Sweden)

    Jennie C. I. Tsao

    2006-01-01

    Full Text Available Previously, we reviewed the evidence for the efficacy of CAM approaches for pediatric pain (volume 2; issue 2; 2005 using criteria developed by the American Psychological Association Division 12 Task Force. Our review focused on CAM modalities that had been tested with at least one controlled trial or multiple baseline study. In addition, only those trials in which children comprised the study sample were included. Thus, several CAM modalities were not included in our review. Key ethical and other reasons for the limited literature on CAM for pediatric pain as well as directions for future studies are discussed.

  2. Developing child autonomy in pediatric healthcare: towards an ethical model.

    Science.gov (United States)

    Martakis, Kyriakos; Brand, Helmut; Schröder-Bäck, Peter

    2018-06-01

    The changes initiated by the new National Civil and Commercial Code in Argentina underline the pediatric task to empower children's and adolescents' developing autonomy. In this paper, we have framed a model describing autonomy in child healthcare. We carried out a literature review focusing on i) the concept of autonomy referring to the absolute value of the autonomous individual, and ii) the age-driven process of competent decisionmaking development. We summarized our findings developing a conceptual model that includes the child, the pediatrician and the parents. The pediatricianchild relationship is based on different forms of guidance and cooperation, resulting in varying levels of activity and passivity. Parental authority influences the extent of autonomy, based on the level of respect of the child's moral equality. Contextual, existential, conceptual, and socialethical conditions shall be considered when applying the model to facilitate dialogue between pediatricians, children, parents and other actors. Sociedad Argentina de Pediatría.

  3. Genetics Home Reference: Potocki-Lupski syndrome

    Science.gov (United States)

    ... cause the disorder. Most cases of Potocki-Lupski syndrome result from a new (de novo) chromosomal duplication and occur in people with ... or Free article on PubMed Central Treadwell-Deering DE, ... of the Potocki-Lupski syndrome (duplication 17p11.2). J Dev Behav Pediatr. 2010 ...

  4. Tooth enamel hypoplasia in PHACE syndrome.

    Science.gov (United States)

    Chiu, Yvonne E; Siegel, Dawn H; Drolet, Beth A; Hodgson, Brian D

    2014-01-01

    Individuals with PHACE syndrome (posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, sternal cleft, and supraumbilical raphe syndrome) have reported dental abnormalities to their healthcare providers and in online forums, but dental involvement has not been comprehensively studied. A study was conducted at the third PHACE Family Conference, held in Milwaukee, Wisconsin, in July 2012. A pediatric dentist examined subjects at enrollment. Eighteen subjects were enrolled. The median age was 4.2 years (range 9 mos-9 yrs; 14 girls, 4 boys). Eleven of 18 patients had intraoral hemangiomas and five of these (50%) had hypomature enamel hypoplasia. None of the seven patients without intraoral hemangiomas had enamel hypoplasia. No other dental abnormalities were seen. Enamel hypoplasia may be a feature of PHACE syndrome when an intraoral hemangioma is present. Enamel hypoplasia increases the risk of caries, and clinicians should refer children with PHACE syndrome to a pediatric dentist by 1 year of age. © 2014 Wiley Periodicals, Inc.

  5. Parental preference or child well-being: an ethical dilemma.

    Science.gov (United States)

    Turner, Helen N

    2010-02-01

    An ethical dilemma that is not uncommon to encounter when caring for children occurs when parental preference does not appear to be in the child's best interest. Challenges facing the health care team are further amplified when the family's cultural background does not match that of the team. A case study will be used to illustrate the challenges of a pediatric palliative care ethical dilemma further complicated by cultural diversity. Review of the child's medical condition, patient/parent preferences, quality of life, and contextual features will be followed by an analysis and recommendations for resolution of this challenging situation. Copyright 2010 Elsevier Inc. All rights reserved.

  6. Alagille Syndrome

    Science.gov (United States)

    ... 3] Kamath BM, Loomes KM, Piccoli DA. Medical management of Alagille syndrome. Journal of Pediatric Gastroenterology and Nutrition. 2010;50(6): ... 30 a.m. to 5 p.m. eastern time, M-F Follow Us NIH… Turning Discovery Into ... Disease Urologic Diseases Endocrine Diseases Diet & Nutrition ...

  7. Pediatric Deceased Donation-A Report of the Transplantation Society Meeting in Geneva

    NARCIS (Netherlands)

    Martin, Dominique E.; Nakagawa, Thomas A.; Siebelink, Marion J.; Bramstedt, Katrina A.; Brierley, Joe; Dobbels, Fabienne; Rodrigue, James R.; Sarwal, Minnie; Shapiro, Ron; Dominguez-Gil, Beatriz; Danovitch, Gabriel; Sweet, Stuart C.; Trompeter, Richard S.; Moazam, Farhat; Bos, Michael A.; Delmonico, Francis L.

    The Ethics Committee of The Transplantation Society convened a meeting on pediatric deceased donation of organs in Geneva, Switzerland, on March 21 to 22, 2014. Thirty-four participants from Africa, Asia, the Middle East, Oceania, Europe, and North and South America explored the practical and

  8. High tidal volume decreases adult respiratory distress syndrome, atelectasis, and ventilator days compared with low tidal volume in pediatric burned patients with inhalation injury.

    Science.gov (United States)

    Sousse, Linda E; Herndon, David N; Andersen, Clark R; Ali, Arham; Benjamin, Nicole C; Granchi, Thomas; Suman, Oscar E; Mlcak, Ronald P

    2015-04-01

    Inhalation injury, which is among the causes of acute lung injury and acute respiratory distress syndrome (ARDS), continues to represent a significant source of mortality in burned patients. Inhalation injury often requires mechanical ventilation, but the ideal tidal volume strategy is not clearly defined in burned pediatric patients. The aim of this study was to determine the effects of low and high tidal volume on the number of ventilator days, ventilation pressures, and incidence of atelectasis, pneumonia, and ARDS in pediatric burned patients with inhalation injury within 1 year post burn injury. From 1986 to 2014, inhalation injury was diagnosed by bronchoscopy in pediatric burned patients (n = 932). Patients were divided into 3 groups: unventilated (n = 241), high tidal volume (HTV, 15 ± 3 mL/kg, n = 190), and low tidal volume (LTV, 9 ± 3 mL/kg, n = 501). High tidal volume was associated with significantly decreased ventilator days (p tidal volume significantly decreases ventilator days and the incidence of both atelectasis and ARDS compared with low tidal volume in pediatric burned patients with inhalation injury. Therefore, the use of HTV may interrupt sequences leading to lung injury in our patient population. Copyright © 2015 American College of Surgeons. Published by Elsevier Inc. All rights reserved.

  9. Noonan syndrome: A case report

    Directory of Open Access Journals (Sweden)

    Asokan S

    2007-09-01

    Full Text Available Noonan syndrome is a developmental disorder characterized by facial dysmorphia, short stature, cardiac defects and skeletal malformations. It may be sporadic or inherited as an autosomal dominant or recessive trait and occurs, one in 1000-2500 children. This is a case report of a 13 year-old girl who was referred by a general dental practitioner to a pediatric dentist for management. Full mouth dental rehabilitation was done and the child was brought to a dental institution for correction of orofacial and occlusal defects. Multidisciplinary treatment is the key to success in managing children with Noonan syndrome and the pediatric dentists play an important position to lead the health team.

  10. The Role of Genetic, Dietary and Lifestyle Factors in Pediatric Metabolic Syndrome: A Review of the Literature from Prenatal to Adolescence

    Directory of Open Access Journals (Sweden)

    Teresa Arora

    2017-04-01

    Full Text Available The metabolic syndrome (MetS is described as a cluster of health conditions that are associated with an increased risk of cardiovascular disease. The clinical diagnosis of MetS in pediatrics is challenging due to differing criteria, although the estimated prevalence continues to rise. The increased prevalence of childhood obesity and insulin resistance, in both developed and developing countries, is believed to be a major contributor to MetS diagnosis in children. We review the current literature surrounding genetic predisposition, maternal influence, epigenetics, environmental and lifestyle factors pertaining to pediatric MetS with a specific emphasis on obesity and insulin resistance. We highlight and discuss recent, key studies in prenatal through to adolescent populations and review evidence suggesting that children may be pre-disposed to obesity and insulin resistance, prenatally. We also discuss several key lifestyle drivers of these conditions including poor nutrition and dietary habits, insufficient physical activity, use of electronic devices, over-consumption of caffeinated and/or sugar-sweetened beverages, as well as the importance of sleep during childhood and adolescence in relation to metabolic health. We conclude with recommendations for preventable methods to tackle this growing pediatric public health issue, which, if current trends continue, will undoubtedly compromise the health and longevity of the next adult generation.

  11. Cogan's syndrome mimicking acute Lyme arthritis.

    Science.gov (United States)

    Schwegmann, J P; Enzenauer, R J

    1995-05-01

    A pediatric case of Cogan's syndrome mimicking acute Lyme arthritis is described. A 12-year-old black boy was admitted to the pediatric service for presumed right knee septic arthritis. Symptoms included acute pain and swelling with decreased range-of-motion. Although the patient's right knee symptoms and positive Lyme serology were consistent with a diagnosis of Lyme arthritis, the presence of sensorineural hearing loss and interstitial keratitis with inflammatory arthritis suggested a diagnosis of Cogan's syndrome. Subsequent Western blot analysis was negative for Borrelia burgdorferi antigens. The patient had dramatic clinical improvement of musculoskeletal and ophthalmologic complaints shortly after receiving high-dose corticosteroids, although residual sensorineural hearing loss persisted.

  12. Noninvasive Brain Stimulation in Pediatric ADHD: A Review

    Science.gov (United States)

    Rubio, Belen; Boes, Aaron D.; Laganiere, Simon; Rotenberg, Alexander; Jeurissen, Danique; Pascual-Leone, Alvaro

    2015-01-01

    Attention-deficit hyperactivity disorder (ADHD) is one of the most prevalent neurodevelopmental disorders in the pediatric population. The clinical management of ADHD is currently limited by a lack of reliable diagnostic biomarkers and inadequate therapy for a minority of patients that do not respond to standard pharmacotherapy. There is optimism that noninvasive brain stimulation may help to address these limitations. Transcranial magnetic stimulation (TMS) and transcranial direct current stimulation (tDCS) are two methods of noninvasive brain stimulation that modulate cortical excitability and brain network activity. TMS can be used diagnostically to probe cortical neurophysiology, while daily use of repetitive TMS or tDCS can induce long-lasting and potentially therapeutic changes in targeted networks. In this review we highlight research showing the potential diagnostic and therapeutic applications of TMS and tDCS in pediatric ADHD. We also discuss the safety and ethics of using these tools in the pediatric population. PMID:26661481

  13. Ethical Barriers in Adolescent Oncofertility Counseling.

    Science.gov (United States)

    Runco, Daniel V; Taylor, Julia F; Helft, Paul R

    2017-01-01

    Adult survivors of pediatric cancers are at substantial risk for infertility. Oncofertility is an emerging field in medicine that has focused on the fertility preservation of these patients. As the field continues to develop, there are several areas in which our practice has improved. However, several ethical concerns still exist involving beneficence, nonmaleficence, informed consent, adolescent assent, and posthumous use of reproductive tissues. Because the field is still developing, great disparities exist in available options depending on age, ability to pay, and geographic location. Such discrepancies in access may lead to health disparities in the adolescent patient population. As the science continues to make future fertility more feasible, the ethical questions will continue to be more complex. The purpose of this article is to review some of the developments regarding oncoferility and address future directions for research and inquiry in specific areas.

  14. Assessment of pediatric residents burnout in a tertiary academic centre

    Directory of Open Access Journals (Sweden)

    Roaa S. Jamjoom

    2018-03-01

    Full Text Available Objectives: To study burnout among pediatric residents at King Abdulazaiz University Hospital in Jeddah, Saudi Arabia. Methods: This is a cross-sectional survey that was administered to all pediatric residents enrolled in the Saudi Paediatric Board program (PGY1-PGY4 in a large tertiary academic hospital in the Western region of Saudi Arabia (King Abdulaziz University Hospital. The survey were sent via E-mail to 50 registered general pediatric residents. Results: Seventy percent of the pediatric residents completed the survey. More than 70% of residents experiencing severe burnout. Forty-three percent suffering emotional exhaustion, 71.8% experiencing depersonalization and 40.6% suffering from low accomplishment. Conclusion: Burnout syndrome appear to be a serious threat to resident well-being in our program. Moreover, pediatric residents in our institute experienced higher levels of depersonalization than their peers nationally and internationally.

  15. Characterization of the Pediatric Acute-Onset Neuropsychiatric Syndrome Phenotype

    Science.gov (United States)

    Patel, Priyal D.; McGuire, Joseph F.; Kennel, Allison; Mutch, P. Jane; Parker-Athill, E. Carla; Hanks, Camille E.; Lewin, Adam B.; Storch, Eric A.; Toufexis, Megan D.; Dadlani, Gul H.; Rodriguez, Carina A.

    2015-01-01

    Abstract Objective: Pediatric acute-onset neuropsychiatric syndrome (PANS) is a subtype of obsessive compulsive disorder (OCD) marked by an abrupt onset or exacerbation of neuropsychiatric symptoms. We aim to characterize the phenotypic presentation of youth with PANS. Methods: Forty-three youth (ages 4–14 years) meeting criteria for PANS were assessed using self-report and clinician-administered measures, medical record reviews, comprehensive clinical evaluation, and laboratory measures. Results: Youth with PANS presented with an early age of OCD onset (mean=7.84 years) and exhibited moderate to severe obsessive compulsive symptoms upon evaluation. All had comorbid anxiety and emotional lability, and scored well below normative means on all quality of life subscales. Youth with elevated streptococcal antibody titers trended toward having higher OCD severity, and presented more frequently with dilated pupils relative to youth without elevated titers. A cluster analysis of core PANS symptoms revealed three distinct symptom clusters that included core characteristic PANS symptoms, streptococcal-related symptoms, and cytokine-driven/physiological symptoms. Youth with PANS who had comorbid tics were more likely to exhibit a decline in school performance, visuomotor impairment, food restriction symptoms, and handwriting deterioration, and they reported lower quality of life relative to youth without tics. Conclusions: The sudden, acute onset of neuropsychiatric symptoms, high frequency of comorbidities (i.e., anxiety, behavioral regression, depression, and suicidality), and poor quality of life capture the PANS subgroup as suddenly and severely impaired youth. Identifying clinical characteristics of youth with PANS will allow clinicians to diagnose and treat this subtype of OCD with a more strategized and effective approach. PMID:25314221

  16. Clarifying perspectives: Ethics case reflection sessions in childhood cancer care.

    Science.gov (United States)

    Bartholdson, Cecilia; Lützén, Kim; Blomgren, Klas; Pergert, Pernilla

    2016-06-01

    Childhood cancer care involves many ethical concerns. Deciding on treatment levels and providing care that infringes on the child's growing autonomy are known ethical concerns that involve the whole professional team around the child's care. The purpose of this study was to explore healthcare professionals' experiences of participating in ethics case reflection sessions in childhood cancer care. Data collection by observations, individual interviews, and individual encounters. Data analysis were conducted following grounded theory methodology. Healthcare professionals working at a publicly funded children's hospital in Sweden participated in ethics case reflection sessions in which ethical issues concerning clinical cases were reflected on. The children's and their parents' integrity was preserved through measures taken to protect patient identity during ethics case reflection sessions. The study was approved by a regional ethical review board. Consolidating care by clarifying perspectives emerged. Consolidating care entails striving for common care goals and creating a shared view of care and the ethical concern in the specific case. The inter-professional perspectives on the ethical aspects of care are clarified by the participants' articulated views on the case. Different approaches for deliberating ethics are used during the sessions including raising values and making sense, leading to unifying interactions. The findings indicate that ethical concerns could be eased by implementing ethics case reflection sessions. Conflicting perspectives can be turned into unifying interactions in the healthcare professional team with the common aim to achieve good pediatric care. Ethics case reflection sessions is valuable as it permits the discussion of values in healthcare-related issues in childhood cancer care. Clarifying perspectives, on the ethical concerns, enables healthcare professionals to reflect on the most reasonable and ethically defensible care for the child

  17. Improvement of Pediatric Drug Development: Regulatory and Practical Frameworks.

    Science.gov (United States)

    Tsukamoto, Katusra; Carroll, Kelly A; Onishi, Taku; Matsumaru, Naoki; Brasseur, Daniel; Nakamura, Hidefumi

    2016-03-01

    A dearth in pediatric drug development often leaves pediatricians with no alternative but to prescribe unlicensed or off-label drugs with a resultant increased risk of adverse events. We present the current status of pediatric drug development and, based on our data analysis, clarify the problems in this area. Further action is proposed to improve the drug development that has pediatric therapeutic orphan status. We analyzed all Phase II/III and Phase III trials in ClinicalTrials.gov that only included pediatric participants (Performance index, an indicator of pediatric drug development, was calculated by dividing the annual number of pediatric clinical trials by million pediatric populations acquired from Census.gov. Effects of the 2 Japanese premiums introduced in 2010, for the enhancement of pediatric drug development, were analyzed by comparing mean performance index prepremiums (2006-2009) and postpremiums (2010-2014) among Japan, the European Union, and the United States. The European Union Clinical Trials Register and published reports from the European Medicines Agency were also surveyed to investigate the Paediatric Committee effect on pediatric clinical trials in the European Union. Mean difference of the performance index in prepremiums and postpremiums between Japan and the European Union were 0.296 (P 15% after 2008. Recruitment and ethical obstacles make conducting pediatric clinical trials challenging. An improved operational framework for conducting clinical trials should mirror the ever-improving regulatory framework that incentivizes investment in pediatric clinical trials. Technological approaches, enhancements in electronic medical record systems, and community approaches that actively incorporate input from physicians, researchers, and patients could offer a sustainable solution to recruitment of pediatric study participants. The key therefore is to improve pediatric pharmacotherapy collaboration among industry, government, academia, and

  18. Intrinsic brain networks normalize with treatment in pediatric complex regional pain syndrome

    Science.gov (United States)

    Becerra, Lino; Sava, Simona; Simons, Laura E.; Drosos, Athena M.; Sethna, Navil; Berde, Charles; Lebel, Alyssa A.; Borsook, David

    2014-01-01

    Pediatric complex regional pain syndrome (P-CRPS) offers a unique model of chronic neuropathic pain as it either resolves spontaneously or through therapeutic interventions in most patients. Here we evaluated brain changes in well-characterized children and adolescents with P-CRPS by measuring resting state networks before and following a brief (median = 3 weeks) but intensive physical and psychological treatment program, and compared them to matched healthy controls. Differences in intrinsic brain networks were observed in P-CRPS compared to controls before treatment (disease state) with the most prominent differences in the fronto-parietal, salience, default mode, central executive, and sensorimotor networks. Following treatment, behavioral measures demonstrated a reduction of symptoms and improvement of physical state (pain levels and motor functioning). Correlation of network connectivities with spontaneous pain measures pre- and post-treatment indicated concomitant reductions in connectivity in salience, central executive, default mode and sensorimotor networks (treatment effects). These results suggest a rapid alteration in global brain networks with treatment and provide a venue to assess brain changes in CRPS pre- and post-treatment, and to evaluate therapeutic effects. PMID:25379449

  19. Intrinsic brain networks normalize with treatment in pediatric complex regional pain syndrome

    Directory of Open Access Journals (Sweden)

    Lino Becerra

    2014-01-01

    Full Text Available Pediatric complex regional pain syndrome (P-CRPS offers a unique model of chronic neuropathic pain as it either resolves spontaneously or through therapeutic interventions in most patients. Here we evaluated brain changes in well-characterized children and adolescents with P-CRPS by measuring resting state networks before and following a brief (median = 3 weeks but intensive physical and psychological treatment program, and compared them to matched healthy controls. Differences in intrinsic brain networks were observed in P-CRPS compared to controls before treatment (disease state with the most prominent differences in the fronto-parietal, salience, default mode, central executive, and sensorimotor networks. Following treatment, behavioral measures demonstrated a reduction of symptoms and improvement of physical state (pain levels and motor functioning. Correlation of network connectivities with spontaneous pain measures pre- and post-treatment indicated concomitant reductions in connectivity in salience, central executive, default mode and sensorimotor networks (treatment effects. These results suggest a rapid alteration in global brain networks with treatment and provide a venue to assess brain changes in CRPS pre- and post-treatment, and to evaluate therapeutic effects.

  20. Effects of Adenotonsillectomy on Neurocognitive Function in Pediatric Obstructive Sleep Apnea Syndrome

    Directory of Open Access Journals (Sweden)

    Fumie Horiuchi

    2014-01-01

    Full Text Available Obstructive sleep apnea syndrome (OSAS in children does not only present with symptoms of sleep disturbances but also with associated symptoms such as growth failure, enuresis, academic learning difficulties, and behavioral problems, including attention deficit/hyperactivity disorder- (ADHD- like symptoms. We evaluated neurocognitive functions before and after adenotonsillectomy in a patient with OSAS. An 11-year-old boy suspected of having ADHD with nocturnal enuresis was referred for evaluation. He was found to have adenotonsillar hypertrophy. Presence of snoring was evident only after detailed medical interview. Polysomnography confirmed the diagnosis of OSAS, which was subsequently treated by adenotonsillectomy. The apnea/hypopnea index decreased from 21.9 at baseline to 1.8 after surgery, and the frequency of enuresis fell from almost nightly to 2-3 times per month. Neurocognitive and behavioral assessment after the treatment of OSAS showed significant improvement in cognitive functions, especially attention capacity and considerable amelioration of behavioral problems including ADHD-like symptoms. As the most common cause of pediatric OSAS is adenotonsillar hypertrophy, medical interview and oropharyngeal examination should always be performed in children suspected of having ADHD. The necessity of sleep evaluation for children with ADHD-like symptoms was also emphasized.

  1. Genetics Home Reference: congenital central hypoventilation syndrome

    Science.gov (United States)

    ... Kravis EM, Zhou L, Rand CM, Weese-Mayer DE. Congenital central hypoventilation syndrome: PHOX2B mutations and phenotype. Am J Respir Crit ... BA, Leurgans SE, Berry-Kravis EM, Weese-Mayer DE. Congenital central hypoventilation syndrome: PHOX2B genotype determines risk for sudden death. Pediatr ...

  2. Fibromyalgia and chronic fatigue syndrome in children.

    Science.gov (United States)

    Itoh, Yasuhiko; Shigemori, Tomoko; Igarashi, Tohru; Fukunaga, Yoshitaka

    2012-04-01

    Fibromyalgia (FM) is characterized by widespread persistent pain and the presence of multiple discrete tender points. Chronic fatigue syndrome (CFS) is a syndrome characterized by debilitating fatigue associated with a variable number of non-specific complaints. Because neither condition had necessarily been recognized in children until recently, those patients have been treated as having school refusal without being diagnosed as having either syndrome. There is a considerable overlap of clinical symptoms between these two syndromes. It is therefore controversial as to whether these syndromes have the same pathogenesis or not. The aim of the present study was to clarify the relationship between these syndromes in children. Fifteen patients with FM and 21 patients with CFS were investigated both clinically and immunologically. Immunological assessments included thorough analysis of autoantibodies using several techniques. Anti-nuclear antibody titers were higher and the prevalence of anti-Sa antibody was far more frequent in CFS patients than in FM patients. CFS and FM are different from each other at least in childhood, from an immunological aspect, although some patients could have both conditions. © 2011 The Authors. Pediatrics International © 2011 Japan Pediatric Society.

  3. A resuscitation "dilemma" theory-practice-ethics. Is there a theory-practice-ethics gap?

    Science.gov (United States)

    Mortell, Manfred

    2009-07-01

    The theory-practice-ethics gap - a new paradigm to contemplate. Practices based on tradition, rituals and outdated information are placed into a nonscientific paradigm called the theory-practice gap. Within this paradigm there is often a gap between theoretical knowledge and its application in practice. This theory-practice gap has always existed [Allmark, P., 1995. A classical view of the theory-practice gap in nursing. J. Adv. Nurs. 22 (1), 18-23; Hewison, A. et al., 1996. The theory-practice gap in nursing: a new dimension. J. Adv. Nurs. 24 (4), 754-761]. Its creation is often sited as a culmination of theory being idealistic and impractical, even if practical and beneficial, are often ignored. Most of the evidence relating to the non integration of theory and practice makes the assumption that environmental factors are responsible and will affect learning and practice outcomes, hence the "gap". In fact, it is the author's belief, that to "bridge the gap" between theory and practice an additional component is required, called ethics. A moral duty and obligation ensuring theory and practice integrate. In order to effectively implement new practices, one must deem these practices are worthy and relevant to their role as healthcare providers. Otherwise, we fall victims to providing nothing more than a lip service. This introduces a new concept which the author refers to as the theory-practice-ethics gap. This theory-practice-ethics gap must be considered when reviewing some of the unacceptable outcomes in health care practice. The author believes that there is a crisis of ethics where theory and practice integrate, and as a consequence, malfeasance. We are failing to fulfill our duty as healthcare providers and as patient advocates. One practice of major concern, which the author will endeavor to unfold relates to adult and pediatric resuscitation.

  4. Ethics Rounds: In the Eye of a Social Media Storm.

    Science.gov (United States)

    McKlindon, Donna; Jacobson, Jake A; Nathanson, Pamela; Walter, Jennifer K; Lantos, John D; Feudtner, Chris

    2016-09-01

    Social media, no stranger to health care environments, is increasingly used by patients, families, clinicians, and institutions to interact and engage in new ways. The ethical challenges related to the use of social media in the clinical setting are familiar, yet come with a novel twist, including the possibility of having a conflict "go viral". Health care clinicians and institutions must understand and embrace these technologies, while at the same time promoting policies and practices that ensure the ethically appropriate use of social media and address strategies for preventing and responding to a social media crisis. Copyright © 2016 by the American Academy of Pediatrics.

  5. Pediatric biobanking: a pilot qualitative survey of practices, rules and researcher opinions in ten European countries

    DEFF Research Database (Denmark)

    Salvaterra, Elena; Giorda, Roberto; Bassi, Maria Teresa

    2012-01-01

    Ethical, legal, and social issues related to the collection, storage, and use of biospecimens and data derived from children raise critical concerns in the international debate. So far, a number of studies have considered a variety of the individual issues crucial to pediatric biobanking such as ......Ethical, legal, and social issues related to the collection, storage, and use of biospecimens and data derived from children raise critical concerns in the international debate. So far, a number of studies have considered a variety of the individual issues crucial to pediatric biobanking...... such as decision making, privacy protection, minor recontact, and research withdrawal by focusing on theoretical or empirical perspectives. Our research attempted to analyze such issues in a comprehensive manner by exploring practices, rules, and researcher opinions regarding proxy consent, minor assent, specimens...

  6. Middle East respiratory syndrome in children. Dental considerations

    Directory of Open Access Journals (Sweden)

    Fares S. Al-Sehaibany

    2017-04-01

    Full Text Available As of January 2016, 1,633 laboratory-confirmed cases of Middle East Respiratory Syndrome Coronavirus (MERS-CoV infection and 587 MERS-related deaths have been reported by the World Health Organization globally. Middle East Respiratory Syndrome Coronavirus may occur sporadically in communities or may be transmitted within families or hospitals. The number of confirmed MERS-CoV cases among healthcare workers has been increasing. Middle East Respiratory Syndrome Coronavirus may also spread through aerosols generated during various dental treatments, resulting in transmission between patients and dentists. As MERS-CoV cases have also been reported among children, pediatric dentists are at risk of MERS-CoV infection. This review discusses MERS-CoV infection in children and healthcare workers, especially pediatric dentists, and considerations pertaining to pediatric dentistry. Although no cases of MERS-CoV transmission between a patient and a dentist have yet been reported, the risk of MERS-CoV transmission from an infected patient may be high due to the unique work environment of dentists (aerosol generation.

  7. Ethical decision-making in the dilemma of the intersex infant.

    Science.gov (United States)

    Lathrop, Breanna L; Cheney, Teresa B; Hayman, Annette B

    2014-03-01

    The Making Ethical Decisions about Surgical Intervention (MEDSI) tool is designed to guide health care professionals, patients, and families faced with ethically charged decisions regarding surgical interventions for pediatric patients. MEDSI is built on the principles of beneficence, nonmaleficence, and patient autonomy and created to promote truth-telling, compassion, respect for patient cultural and religious preferences, and appropriate follow up in the clinical setting. Following an overview of the 8 steps that compose MEDSI, the tool is applied to the management of intersex infants. The birth of a child with a disorder of sexual development (DSD) and ambiguous genitalia presents an ethically challenging situation for the family and health care team. The use of the MEDSI model is demonstrated in a case study involving the decision of surgical intervention in the management of an intersex child.

  8. Detection of metabolic syndrome features among childhood cancer survivors: A target to prevent disease

    Directory of Open Access Journals (Sweden)

    Adriana Aparecida Siviero-Miachon

    2008-08-01

    Full Text Available Adriana Aparecida Siviero-Miachon1, Angela Maria Spinola-Castro1, Gil Guerra-Junior21Division of Pediatric Endocrinology, Department of Pediatrics, Federal University of Sao Paulo – UNIFESP/EPM, Brazil; 2Division of Pediatric Endocrinology, Department of Pediatrics, State University of Campinas – FCM/UNICAMP, BrazilAbstract: Along with the growing epidemic of obesity, the risk of atherosclerosis, cardiovascular disease morbidity, and mortality are increasing markedly. Several risk factors for cardiovascular disease, such as visceral obesity, glucose intolerance, arterial hypertension, and dyslipidemia commonly cluster together as a condition currently known as metabolic syndrome. Thus far, insulin resistance, and endothelial dysfunction are the primary events of the metabolic syndrome. Several groups have recommended clinical criteria for the diagnosis of metabolic syndrome in adults. Nonetheless, in what concerns children and adolescents, there are no unified definitions, and modified adult criteria have been suggested by many authors, despite major problems. Some pediatric disease states are at risk for premature cardiovascular disease, with clinical coronary events occurring very early in adult life. Survivors of specific pediatric cancer groups, particularly acute lymphocytic leukemia, central nervous system tumors, sarcomas, lymphomas, testicular cancer, and following bone marrow transplantation, may develop metabolic syndrome traits due to: hormonal deficiencies (growth hormone deficiency, thyroid dysfunction, and gonadal failure, drug or radiotherapy damage, endothelial impairment, physical inactivity, adipose tissue dysfunction, and/or drug-induced magnesium deficiency. In conclusion, some primary and secondary prevention remarks are proposed in order to reduce premature cardiovascular disease risk in this particular group of patients.Keywords: metabolic syndrome X, cardiovascular diseases, insulin resistance, obesity, growth hormone

  9. Gut Microbiota Profiling and Gut-Brain Crosstalk in Children Affected by Pediatric Acute-Onset Neuropsychiatric Syndrome and Pediatric Autoimmune Neuropsychiatric Disorders Associated With Streptococcal Infections.

    Science.gov (United States)

    Quagliariello, Andrea; Del Chierico, Federica; Russo, Alessandra; Reddel, Sofia; Conte, Giulia; Lopetuso, Loris R; Ianiro, Gianluca; Dallapiccola, Bruno; Cardona, Francesco; Gasbarrini, Antonio; Putignani, Lorenza

    2018-01-01

    Pediatric acute-onset neuropsychiatric syndrome (PANS) and pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections syndrome (PANDAS) are conditions that impair brain normal neurologic function, resulting in the sudden onset of tics, obsessive-compulsive disorder, and other behavioral symptoms. Recent studies have emphasized the crosstalk between gut and brain, highlighting how gut composition can influence behavior and brain functions. Thus, the present study investigates the relationship between PANS/PANDAS and gut microbiota ecology. The gut composition of a cohort of 30 patients with PANS/PANDAS was analyzed and compared to control subjects using 16S rRNA-based metagenomics. Data were analyzed for their α- and β-diversity; differences in bacterial distribution were detected by Wilcoxon and LEfSe tests, while metabolic profile was predicted via PICRUSt software. These analyses demonstrate the presence of an altered bacterial community structure in PANS/PANDAS patients with respect to controls. In particular, ecological analysis revealed the presence of two main clusters of subjects based on age range. Thus, to avoid age bias, data from patients and controls were split into two groups: 4-8 years old and >9 years old. The younger PANS/PANDAS group was characterized by a strong increase in Bacteroidetes; in particular, Bacteroides , Odoribacter , and Oscillospira were identified as potential microbial biomarkers of this composition type. Moreover, this group exhibited an increase of several pathways concerning the modulation of the antibody response to inflammation within the gut as well as a decrease in pathways involved in brain function (i.e., SCFA, D-alanine and tyrosine metabolism, and the dopamine pathway). The older group of patients displayed a less uniform bacterial profile, thus impairing the identification of distinct biomarkers. Finally, Pearson's analysis between bacteria and anti-streptolysin O titer reveled a

  10. Clinical correlations of dry eye syndrome and allergic conjunctivitis in Korean children.

    Science.gov (United States)

    Kim, Tae Hyung; Moon, Nam Ju

    2013-01-01

    Clinical patterns in pediatric patients with dry eye syndrome and allergic conjunctivitis were investigated. Children aged 6 to 15 years with dry eye symptoms were included. Slit-lamp examinations including tear film break-up time, Schirmer's test, and fluorescent staining were performed, and subjective symptoms were investigated. Patients with allergic conjunctivitis were subjected to skin prick tests. Tear film break-up time was shorter and the number of symptoms related to dry eyes was higher in pediatric patients with allergic conjunctivitis than in those without allergic conjunctivitis. Patients with allergic conjunctivitis who had higher numbers of positive allergens on the skin prick test also had shorter tear film break-up time. Because pediatric patients with dry eye syndrome tend to complain less about their symptoms than adult patients, dry eye syndrome is commonly overlooked. This study showed that dry eyes tended to be more severe with the presence of allergic conjunctivitis; the more allergens present, the more severe the dry eyes. More attention should be paid to the treatment of pediatric patients with dry eyes accompanied by allergies. Copyright 2013, SLACK Incorporated.

  11. A Qualitative Study Exploring Moral Distress Among Pediatric Resuscitation Team Clinicians: Challenges to Professional Integrity.

    Science.gov (United States)

    Thomas, Tessy A; Thammasitboon, Satid; Balmer, Dorene F; Roy, Kevin; McCullough, Laurence B

    2016-07-01

    Our study objectives were to explore moral distress among pediatric team clinicians within the context of resuscitation experiences, and determine whether there were any distinctively ethical perspectives on moral distress that could be conceptualized as challenges to professional integrity, rather than to previously described psychological responses of clinicians. Descriptive, exploratory qualitative study. A large tertiary pediatric academic hospital in Houston, TX. Twenty-five PICU resuscitation team clinicians were interviewed from December 2012 to April 2013. None. All clinicians reported experiencing moral distress during certain resuscitations. Twenty-one of 25 clinicians reflected and acknowledged that their sense of professional integrity had been challenged during those resuscitation events. Four main components of resuscitation experience that induced moral distress were identified: 1) experiences where there was lack of understanding of the big picture; 2) experiences where there was suboptimal team leadership; 3) experiences where there was variable meanings to the word "resuscitation"; and 4) experiences were there was uncertainty of role responsibility. The perception of moral distress exists among pediatric clinicians during resuscitations and could be conceptualized as challenges to professional integrity. This ethical framework offers an alternative approach to understanding and investigating the complex layers of moral distress.

  12. Prevalence of ''obesity disease'' and ''metabolic syndrome'' in obese pediatric outpatients at the University Hospital of Occupational and Environmental Health, Japan

    International Nuclear Information System (INIS)

    Araki, Shunsuke; Dobashi, Kazushige; Kubo, Kazuyasu; Kawagoe, Rinko; Yamamoto, Yukiyo; Kawada, Yasusada; Shirahata, Akira; Asayama, Kohtaro

    2008-01-01

    'Obesity Disease for Japanese Children' was defined in 2002, and very recently 'Metabolic Syndrome (MS) for Japanese Children' was also defined. We therefore aimed to determine the prevalence of these two among the obese pediatric outpatients at our university hospital. The subjects were 97 children, 58 boys and 39 girls, ranging in age from 5 to 15 years. A child was considered to be obese when the body weight exceeded 120% of the standard body weight. All the subjects exceeded 120% overweight, and 58 children (35 boys and 23 girls) were over 150% overweight. Eighty five children (53 boys and 32 girls) were diagnosed with obesity disease (87.6%). Sixteen children (12 boys and 4 girls) were diagnosed with metabolic syndrome, which was 16.5% of all the subjects and 18.8% of the children with obesity disease. Fourteen of the 16 children with MS were over 10 years old. Obesity disease is diagnosed when the child has an obesity disease score of more than 6. The obesity disease score was significantly correlated with the waist circumference and the visceral adipose tissue area measured by computed tomography. The mean score of the children with MS was significantly higher than that of the non-MS group (30.2 vs. 12.3 points). In this study, it was clear that about 90% of our clinic patients are in the obesity disease group, and need therapeutic interventions. The prevalence of MS in the pediatric age is very low compared with that of adults, but MS is a high-risk category of obesity disease. (author)

  13. Absence of family history and phenotype-genotype correlation in pediatric Brugada syndrome: more burden to bear in clinical and genetic diagnosis.

    Science.gov (United States)

    Daimi, Houria; Khelil, Amel Haj; Ben Hamda, Khaldoun; Aranega, Amelia; Chibani, Jemni B E; Franco, Diego

    2015-06-01

    Brugada syndrome (BrS) is an autosomal-dominant genetic cardiac disorder caused in 18-30 % of the cases by SCN5A gene mutations and manifested by an atypical right bundle block pattern with ST segment elevation and T wave inversion in the right precordial leads. The syndrome is usually detected after puberty. The identification of BrS in pediatric patients is thus a rare occurrence, and most of the reported cases are unmasked after febrile episodes. Usually, having a family history of sudden death represents the first reason to perform an ECG in febrile children. However, this practice makes the sporadic cases of cardiac disease and specially the asymptomatic ones excluded from this diagnosis. Here, we report a sporadic case of a 2-month-old male patient presented with vaccination-related fever and ventricular tachycardia associated with short breathing, palpitation and cold sweating. ECG changes were consistent with type 1 BrS. SCN5A gene analysis of the proband and his family revealed a set of mutations and polymorphisms differentially distributed among family members, however, without any clear genotype-phenotype correlation. Based on our findings, we think that genetic testing should be pursued as a routine practice in symptomatic and asymptomatic pediatric cases of BrS, with or without family history of sudden cardiac death. Similarly, our study suggests that pediatrician should be encouraged to perform an ECG profiling in suspicious febrile children and quickly manage fever since it is the most important factor unmasking BrS in children.

  14. The Prevalence of Hypomagnesaemia in Pediatric Patients with Mitral Valve Prolapse Syndrome and the Effect of Mg Therapy

    Directory of Open Access Journals (Sweden)

    Hamid Amoozgar

    2012-09-01

    Full Text Available Background: A paucity of data exists regarding the prevalence and relationship of hypomagnesaemia with clinical symptoms of mitral valve prolapse (MVP in pediatric patients. Objective: In this study we evaluated the prevalence of magnesium (Mg deficiency in pediatric patients with MVP syndrome and attempted to clarify the effect of Mg therapy on alleviating their symptoms. Methods: The present study was conducted from April 2010 to January 2012, and included 230 patients (90 males and 140 females with symptoms of mitral valve prolapse and mean age of 11.6±3.66. Serum magnesium (Mg level less than 1.5 mg/dl was defined as hypomagnesaemia. Patients with 2 mm leaflet displacement and maximum leaflet thickness of 5 mm in echocardiography were considered to have classic MVP, while those with leaflet thickness less than 5 mm were considered as non-classic MVP. Patients with hypomagnesaemia were orally treated with 4.5 mg/kg/day Mg chloride for 5 weeks followed by re-evaluation of symptoms of chest pain, palpitation, fatigue and dyspnea. Results: Hypomagnesaemia was found in 19 (8.2 % of 230 patients with mitral valve prolapse. The re-evaluation of patients with Hypomagnesaemia after 5 weeks of Mg therapy, showed statistically significant relief of chest pain (P=0.01. However, no significant changes was detected in regard to palpitation (P=0.06, fatigue (P= 0.5 and dyspnea (P=0.99. Conclusion: This study revealed that the prevalence of hypomagnesaemia in pediatric patients with mitral valve prolapse is relatively low compared to adults, but treatment with oral Mg in patient with hypomagnesaemia decreases chest pain.

  15. Orphan Hereditary Syndromes in the Practice of Pediatric Endocrinologist

    Directory of Open Access Journals (Sweden)

    M.O. Ryznychuk

    2015-05-01

    Above-mentioned syndromes are caused by different gene mutations, namely Perlman syndrome — by homozygous or heterozygous mutation in DIS3L2 gene on the chromosome 2q37, Proteus syndrome — mutation in AKT1 gene on chromosome 14q32.3, Sotos syndrome 1 is caused by heterozygous mutation in NSD1 gene in the 5q35 region, Sotos syndrome 2 — by heterozygous mutation in NFIX gene on chromosome 19p13.3. Berardinelli syndrome, which is divided into four types, has four different mutations, namely: type 1 is caused by mutations in AGPAT2 gene in locus 9q34, type 2 — in BSCα2gene in locus 11q13, type 3 — by mutations in CAV1 gene (locus 7q31, type 4 — by mutation in PTRF gene located on the chromosome 17.

  16. Disclosure of Adverse Events in Pediatrics.

    Science.gov (United States)

    2016-12-01

    Despite increasing attention to issues of patient safety, preventable adverse events (AEs) continue to occur, causing direct and consequential injuries to patients, families, and health care providers. Pediatricians generally agree that there is an ethical obligation to inform patients and families about preventable AEs and medical errors. Nonetheless, barriers, such as fear of liability, interfere with disclosure regarding preventable AEs. Changes to the legal system, improved communications skills, and carefully developed disclosure policies and programs can improve the quality and frequency of appropriate AE disclosure communications. Copyright © 2016 by the American Academy of Pediatrics.

  17. A comparison of pediatric and adult kidney donors for adult recipients.

    Science.gov (United States)

    Pugliese, M R; Ridolfi, L; Nanni Costa, A; Taddei, S; Venturoli, N; Petrini, F

    1999-01-01

    The high demand for organs for transplantation has made it necessary to consider using even the oldest and youngest of potential donors in order to increase the organ supply. In this retrospective study, the outcome of kidney transplantation using cadaveric pediatric donors was compared with that of an adult control series. Graft procurement took place in two regions of Italy (Emilia-Romagna and Piemonte) over an 11-year period. A group of pediatric donors (Actuarial patient and graft survival rates did not differ significantly between the two groups (patient survival 96% and 96% for pediatric donors versus 98% and 92% for adult donors at 1 and 5 years post-transplantation; graft survival 76% and 68% for pediatric donors versus 88% and 74% for adult donors 1 and 5 y post-transplantation). Complications were also evaluated, but no difference was found (the only exception being the creatinine level in the 5th year). Renal transplantation with cadaveric donors starting at 4 years of age gave results comparable to kidneys coming from adults. These data show that cadaveric pediatric donor kidneys may be used in adult recipients with good results. The ethical implications of the subject are extensively reviewed.

  18. Determination of Eligibility in Related Pediatric Hematopoietic Cell Donors: Ethical and Clinical Considerations. Recommendations from a Working Group of the Worldwide Network for Blood and Marrow Transplantation Association.

    Science.gov (United States)

    Bitan, Menachem; van Walraven, Suzanna M; Worel, Nina; Ball, Lynne M; Styczynski, Jan; Torrabadella, Marta; Witt, Volker; Shaw, Bronwen E; Seber, Adriana; Yabe, Hiromasa; Greinix, Hildegard T; Peters, Christina; Gluckman, Eliane; Rocha, Vanderson; Halter, Joerg; Pulsipher, Michael A

    2016-01-01

    Related donors for hematopoietic cell (HC) transplantation are a growing population in recent years because of expanding indications for allogeneic transplantation. The safety and welfare of the donor are major concerns for the transplantation community, especially for related sibling donors of young recipients who are children and, thus, not able to fully consent. Because donation of HC does not improve the donor's own physical health and carries a risk of side effects, careful assessment of medical risks specific to the individual donor, as well as consideration of ethical and legal aspects associated with donation from a child, must be considered. In addition, donor centers must balance the needs of both the donor and the recipient, understanding the inherent conflict parents may have as they can be overly focused on the very sick child receiving a transplant, rather than on the relatively less significant health or emotional problems that a sibling donor may have, which could impact risk with donation. Likewise, consideration must be made regarding the nature of the relationship of the sibling donor to the recipient and also aspects of performing research on pediatric HC donors. In this article, as members of the Donor Issues Committee of the Worldwide Network for Blood and Marrow Transplantation, we review key ethical concerns associated with pediatric donation and then give recommendations for screening potential child donors with underlying health conditions. These recommendations are aimed at protecting the physical and emotional well-being of childhood donors and arise out of the Third International Conference on Health and Safety of Donors sponsored by the Worldwide Network for Blood and Marrow Transplantation. Copyright © 2016 American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved.

  19. Establishing a surgical outreach program in the developing world: pediatric strabismus surgery in Guatemala City, Guatemala.

    Science.gov (United States)

    Ditta, Lauren C; Pereiras, Lilia Ana; Graves, Emily T; Devould, Chantel; Murchison, Ebony; Figueroa, Ligia; Kerr, Natalie C

    2015-12-01

    To report our experince in establishing a sustainable pediatric surgical outreach mission to an underserved population in Guatemala for treatment of strabismic disorders. A pediatric ophthalmic surgical outreach mission was established. Children were evaluated for surgical intervention by 3 pediatric ophthalmologists and 2 orthoptists. Surgical care was provided at the Moore Pediatric Surgery Center, Guatemala City, over 4 days. Postoperative care was facilitated by Guatemalan physicians during the second year. In year 1, patients 1-17 years of age were referred by local healthcare providers. In year 2, more than 60% of patients were prescreened by a local pediatric ophthalmologist. We screened 47% more patients in year 2 (132 vs 90). Diagnoses included congenital and acquired esotropia, consecutive and acquired exotropia, congenital nystagmus, Duane syndrome, Brown syndrome, cranial nerve palsy, dissociated vertical deviation, and oblique muscle dysfunction. Overall, 42% of the patients who were screened underwent surgery. We performed 21 more surgeries in our second year (58 vs 37), a 57% increase. There were no significant intra- or postoperative complications. Surgical outreach programs for children with strabismic disorders in the developing world can be established through international cooperation, a multidisciplinary team of healthcare providers, and medical equipment allocations. Coordinating care with local pediatric ophthalmologists and medical directors facilitates best practice management for sustainability. Copyright © 2015 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.

  20. Pediatric fibromyalgia

    Directory of Open Access Journals (Sweden)

    J. Ablin

    2012-09-01

    Full Text Available Fibromyalgia (FM is currently defined as chronic widespread pain (CWP with allodynia or hyperalgesia to pressure pain. It is classified as one of the large group of soft-tissue pain syndromes. Pain is the cardinal symptom of FM; however, most patients also experience additional symptoms such as debilitating fatigue, disrupted or non-restorative sleep, functional bowel disturbances, and a variety of neuropsychiatric problems, including cognitive dysfunction, anxiety and depressive symptoms. Its pathogenesis is not entirely understood, although it is currently believed to be the result of a central nervous system (CNS malfunction that increases pain transmission and perception. FMS usually involves females, and in these patients it often makes its first appearance during menopause. But it is often diagnosed both in young as well as elderly individuals. Pediatric FMS is a frustrating condition affecting children and adolescents at a crucial stage of their physical and emotional development. Pediatric FMS is an important differential diagnosis to be considered in the evaluation of children suffering from widespread musculoskeletal pain, and must be differentiated from a spectrum of inflammatory joint disorders such as juvenile idiopathic arthritis (JIA, juvenile ankylosing spondylitis, etc. The management of pediatric FMS is centered on the issues of education, behavioral and cognitive change (with a strong emphasis on physical exercise, and a relatively minor role for pharmacological treatment with medications such as muscle relaxants, analgesics and tricyclic agents.

  1. Pediatric Renal Transplantation in Oman: A Single-center Experience

    Directory of Open Access Journals (Sweden)

    Mohamed S. Al Riyami

    2018-01-01

    Full Text Available Objectives: This study sought to report 22 years experience in pediatric kidney transplantation in Oman. Methods: Electronic charts of all Omani children below 13 years of age who received a kidney transplant from January 1994 to December 2015 were reviewed. Data collected included patient demographics, etiology of end-stage kidney disease, modality and duration of dialysis, donor type, complication of kidney transplantation (including surgical complications, infections, graft rejection graft and patient survival, and duration of follow-up. Results: During the study period transplantation from 27 living related donors (LRDs, 42 living unrelated donors (LURDs, also referred to as commercial transplant, and one deceased donor were performed. The median age at transplantation was nine years for both groups. The most common primary diagnosis was congenital anomalies of the kidney and urinary tract in 32.8% of patients followed by familial nephrotic syndrome in 20.0% and polycystic kidney disease in 18.5%. Almost half the patients were on hemodialysis before transplantation, 35.7% were on peritoneal dialysis, and 14.2% received preemptive renal transplantation. Children who received LURD kidneys had high surgical complications (42.8% compared to the LRDs group (17.8%. Five patients from LURDs group had early graft nephrectomy and four patients developed non-graft function or delayed graft function. In addition, patients in the LURDs group had a higher incidence of hypertension and acute rejection. Graft and patient survival were both better in the LRDs than the LURDs group. Conclusions: Although our pediatric kidney transplant program is a young program it has had successful patient outcomes comparable to international programs. Our study provides evidence that in addition to legal and ethical issues with commercial transplant, it also carries significantly higher morbidity and reduced graft and patient survival.

  2. Syncope as initial symptom for nephrotic syndrome: a case report

    Science.gov (United States)

    Wu, Xuemei; Wang, Guangliang; Feng, Jiachun

    2015-01-01

    Although syncope and nephrotic syndrome are frequently encountered independently in pediatric practice, syncope as the initial symptom for nephrotic syndrome is rarely observed in the pediatric age group. In this report, we present the case of 3-year-old boy with nephrotic syndrome who presented with a history of three episodes of syncope before admission. The syncope occurred after excessive fluid loss or inadequate intake of fluids and was relieved spontaneously. History taking revealed that the early morning palpebral edema, and laboratory tests showed decreased plasma protein levels and elevated serum lipid levels. Nephrotic syndrome was diagnosed, but could not be confirmed histopathologically because the patient’s parent refused consent for biopsy. The patient was managed with fluid expansion, correction of acidosis, and improvement of microcirculation to prevent recurrence of syncope, and glucocorticoids were administered to prevent disease progression. PMID:26629237

  3. The contributions of the European Medicines Agency and its pediatric committee to the fight against childhood leukemia

    Directory of Open Access Journals (Sweden)

    Rose K

    2015-11-01

    Full Text Available Klaus Rose,1,* Philip D Walson,2,* 1klausrose Consulting, Pediatric Drug Development and More, Riehen, Switzerland; 2Department of Clinical Pharmacology, University Medical School, Goettingen, Germany *These authors contributed equally to this work Background: Although the diagnosis of childhood leukemia is no longer a death sentence, too many patients still die, more with acute myeloid leukemia than with acute lymphoblastic leukemia. The European Union pediatric legislation was introduced to improve pharmaceutical treatment of children, but some question whether the European Medicines Agency (EMA approach is helping children with leukemia. Some have even suggested that the decisions of EMA pediatric committee (PDCO are counterproductive. This study was designed to investigate the impact of PDCO-issued pediatric investigation plans (PIPs for leukemia drugs.Methods: All PIPs listed under “oncology” were downloaded from the EMA website. Non-leukemia decisions including misclassifications, waivers (no PIP, and solid tumors were discarded. The leukemia decisions were analyzed, compared to pediatric leukemia trials in the database http://www.clinicaltrials.gov, and discussed in the light of current literature.Results: The PDCO leukemia decisions demand clinical trials in pediatric leukemia for all new adult drugs without prioritization. However, because leukemia in children is different and much rarer than in adults, these decisions have resulted in proposed studies that are scientifically and ethically questionable. They are also unnecessary, since once promising new compounds are approved for adults, more appropriate, prioritized pediatric leukemia trials are initiated worldwide without PDCO involvement.Conclusion: EMA/PDCO leukemia PIPs do little to advance the treatment of childhood leukemia. The unintended negative effects of the flawed EMA/PDCO's standardized requesting of non-prioritized testing of every new adult leukemia drug in

  4. e-Government Ethics : a Synergy of Computer Ethics, Information Ethics, and Cyber Ethics

    OpenAIRE

    Arief Ramadhan; Dana Indra Sensuse; Aniati Murni Arymurthy

    2011-01-01

    Ethics has become an important part in the interaction among humans being. This paper specifically discusses applied ethics as one type of ethics. There are three applied ethics that will be reviewed in this paper, i.e. computer ethics, information ethics, and cyber ethics. There are two aspects of the three applied ethics that were reviewed, i.e. their definition and the issues associated with them. The reviewing results of the three applied ethics are then used for defining e-Government eth...

  5. The history of ethical decision making in neonatal intensive care.

    Science.gov (United States)

    Placencia, Frank X; McCullough, Laurence B

    2011-01-01

    Neonatal ethics has focused on 2 questions: is withholding potentially live-saving treatment from neonates ethically justified? and if so, who has the authority to decide? This article details how these questions developed and provides a description of the possible answers. In the first section, we review a selection of seminal articles by noted authors in the fields of ethics, medicine, and law. The second section provides a detailed account of the development of the Baby Doe Regulations and the impact they had on neonatal ethics, with particular attention to the emergence of the Best Interest Standard as a guideline for decision making. In the last section, we review the landmark position statements by the American Academy of Pediatric (AAP), and the focus on evidence-based decision making. We conclude that forgoing life-saving treatment is ethically justified. However, this requires a rigorous evidence-based process and is limited by the Best Interest Standard. The second question is more difficult to answer, but we feel that in light of legal limitations, physicians acting as both the infant advocate and a proxy for the state, decide what falls in the range of acceptable treatment options, with the parents free to choose within that range.

  6. Successful treatment of dwarfism secondary to long-term steroid therapy in steroid-dependent nephrotic syndrome.

    Science.gov (United States)

    Sun, Linlin; Chen, Dongping; Zhao, Xuezhi; Xu, Chenggang; Mei, Changlin

    2010-01-01

    Prolonged steroid therapy is generally used for steroid-dependent nephrotic syndrome in pediatric patients. However, dwarfism secondary to a long-term regimen and its successful reverse is rarely reported. The underlying mechanism of dwarfism is still poorly understood, as both long-term steroid use and nephrotic syndrome may interact or independently interfere with the process of growth. Here, we present a 17-year-old patient with dwarfism and steroid-dependent nephrotic syndrome and the successful treatment by recombinant human growth factor and cyclosporine A with withdrawal of steroid. We also briefly review the current understanding and the management of dwarfism in pediatric patients with nephrotic syndrome.

  7. Imaging of pediatric pituitary endocrinopathies

    Science.gov (United States)

    Chaudhary, Vikas; Bano, Shahina

    2012-01-01

    Accurate investigation of the hypothalamic-pituitary area is required in pediatric patients for diagnosis of endocrine-related disorders. These disorders include hypopituitarism, growth failure, diencephalic syndrome, delayed puberty, precocious puberty, diabetes insipidus, syndrome of inappropriate antidiuretic hormone (SIADH) secretion, and hyperpituitarism. Magnetic resonance imaging (MRI) is the modality of choice to visualize hypothalamic-pituitary axis and associated endocrinopathies. Neuroimaging can be normal or disclose abnormalities related to pituitary-hypothalamic axis like (i) congenital and developmental malformations; (ii) tumors; (iii) cystic lesions; and (iv) infectious and inflammatory conditions. Classical midline anomalies like septo-optic dysplasias or corpus callosum agenesis are commonly associated with pituitary endocrinopathies and also need careful evaluation. In this radiological review, we will discuss neuroendocrine disorders related to hypothalamic pituitary-axis. PMID:23087850

  8. A Clinical Roadmap to Investigate the Genetic Basis of Pediatric Pheochromocytoma: Which Genes Should Physicians Think About?

    Directory of Open Access Journals (Sweden)

    Bernardo Dias Pereira

    2018-01-01

    Full Text Available Pheochromocytoma is very rare at a pediatric age, and when it is present, the probability of a causative genetic mutation is high. Due to high costs of genetic surveys and an increasing number of genes associated with pheochromocytoma, a sequential genetic analysis driven by clinical and biochemical phenotypes is advised. The published literature regarding the genetic landscape of pediatric pheochromocytoma is scarce, which may hinder the establishment of genotype-phenotype correlations and the selection of appropriate genetic testing at this population. In the present review, we focus on the clinical phenotypes of pediatric patients with pheochromocytoma in an attempt to contribute to an optimized genetic testing in this clinical context. We describe epidemiological data on the prevalence of pheochromocytoma susceptibility genes, including new genes that are expanding the genetic etiology of this neuroendocrine tumor in pediatric patients. The clinical phenotypes associated with a higher pretest probability for hereditary pheochromocytoma are presented, focusing on differences between pediatric and adult patients. We also describe new syndromes, as well as rates of malignancy and multifocal disease associated with these syndromes and pheochromocytoma susceptibility genes published more recently. Finally, we discuss new tools for genetic screening of patients with pheochromocytoma, with an emphasis on its applicability in a pediatric population.

  9. Industry and Patient Perspectives on Child Participation in Clinical Trials: The Pediatric Assent Initiative Survey Report.

    Science.gov (United States)

    Lombardi, Donald; Squires, Liza; Sjostedt, Philip; Eichler, Irmgard; Turner, Mark A; Thompson, Charles

    2018-01-01

    Obtaining assent from children participating in clinical trials acknowledges autonomy and developmental ability to contribute to the consent process. This critical step in pediatric drug development remains poorly understood, with significant room for improving the clarity, efficiency, and implementation of the assent process. Beyond ethical necessity of informing children about their treatment, the assent process provides the advantages of including children in discussions about their diagnosis and treatment-allowing greater understanding of interventions included in the study. A formalized assent process acknowledges the child as a volunteer and provides a forum for questions and feedback. Legal, cultural, and social differences have historically prevented the development of clear, concise, and accessible materials to ensure children understand the clinical trial design. Published guidelines on obtaining pediatric assent are vague, with many decisions left to local institutional review boards and ethics committees, underscoring the need for collaboratively designed standards. To address this need, 2 surveys were conducted to quantify perspectives on assent in pediatric clinical trials. Two digital surveys were circulated in the United States and internationally (October 2014 to January 2015). The first survey targeted children, parents, and/or caregivers. The second polled clinical trial professionals on their organizations' experience and policies regarding pediatric assent. Forty-five respondents completed the child and parent/caregiver survey; 57 respondents completed the industry survey. Respondents from both surveys detailed experiences with clinical trials and the impediments to securing assent, offering potential solutions to attaining assent in pediatric patients. An important opportunity exists for standardized practices and tools to ensure pediatric patients make well-informed decisions regarding their participation in clinical trials, using materials

  10. [Pulmonary reperfusion syndrome after pulmonary stent implants in a patient with vascular tortuosity syndrome].

    Science.gov (United States)

    Berenguer Potenciano, M; Piris Borregas, S; Mendoza Soto, A; Velasco Bayon, J M; Caro Barri, A

    2015-01-01

    Vascular tortuosity syndrome is a rare genetic disorder that causes tortuosity and stenosis of the pulmonary, systemic and / or coronary circulations. As a result of treatment of pulmonary stenosis, symptoms of pulmonary edema, known as lung reperfusion syndrome, may occur. The case is presented of an adolescent patient with vascular tortuosity syndrome who presented with a pulmonary reperfusion syndrome after multiple stent implants in the left pulmonary artery. After the procedure, the patient immediately developed an acute pulmonary edema with severe clinical deterioration, which required assistance with extracorporeal membrane oxygenation for recovery. Copyright © 2014 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  11. IRRITATED BOWEL SYNDROME IN CHILDREN

    Directory of Open Access Journals (Sweden)

    V. F. Privorotskiy

    2012-01-01

    Full Text Available Irritated bowel syndrome is a significant and underestimated problem in childhood. This condition is not so good studied in pediatrics in comparison with adult practice. Pediatricians often diagnosed this disease in infants and young children without proper reasons. The authors analyze current opinions about etiology and pathogenesis, clinical presentation, diagnosticsand treatment of irritated bowel syndrome in children. An emphasis is made on diagnostic criteria, which allow suggesting and confirming the diagnosis.

  12. Use of conivaptan to allow aggressive hydration to prevent tumor lysis syndrome in a pediatric patient with large-cell lymphoma and SIADH.

    Science.gov (United States)

    Rianthavorn, Pornpimol; Cain, Joan P; Turman, Martin A

    2008-08-01

    The available treatment options for hyponatremia secondary to SIADH are limited and not completely effective. Conivaptan is a vasopressin 1a and 2 receptor antagonist recently approved by the US Food and Drug Administration (FDA) for treating euvolemic and hypervolemic hyponatremia in adult patients. However, data on efficacy and safety of conivaptan in pediatrics are limited. We report a case of a 13-year-old boy with extensively metastasized anaplastic large-cell lymphoma. He also developed hyponatremia due to syndrome of inappropriate antidiuretic hormone secretion (SIADH) prior to chemotherapy initiation. SIADH management in this case was complicated when fluid restriction was not safely attainable. Conivaptan played a significant role in this situation by allowing provision of a large amount of intravenous fluid prior to and during induction chemotherapy. It proved to be an important component in preventing uric acid nephropathy/tumor lysis syndrome. Conivaptan induced free-water clearance as indicated by increased urine output and decreased urine osmolality. The patient responded to conivaptan without any adverse effects.

  13. [Applying Ethics, Placating Ethics, or Applying ourselves to Ethics? A Critical View of Environmental Ethics as Applied Ethics].

    Science.gov (United States)

    Serani Merlo, Alejandro

    2016-01-01

    There is actually a pervasive tendency to consider environmental ethics and bioethics as specific cases pertaining to a supposed kind of ″applied ethics″. Application can be understood in two different meanings: a concrete sense, as in technical applications, and a psychological meaning, as when we mentally apply ourselves to a task. Ethics has been always thought as a practical knowledge, in a ″praxical″ sense and not in a ″poietic″ one. Ethics has to do with ″ends″ not with ″means″; in this sense ethics is ″useless″. Since ethics has to do with the ultimate meaning of things, ethical choices give meaning to all practical activities. In that sense ethics instead of being useless must be considered as ″over-useful″ (Maritain). Nowadays politics tend to instrumentalize ethics in order to political objectives. The consequence has been the reconceptualization of specific ethics as applied ethics. Environmental ethics and bioethics are then submitted to politics following the logic of technical applications. Environmental ethics and bioethics considered as applied ethics are at risk to becoming not only useless, but also meaningless.

  14. When ethics constrains clinical research: trial design of control arms in "greater than minimal risk" pediatric trials.

    Science.gov (United States)

    de Melo-Martín, Inmaculada; Sondhi, Dolan; Crystal, Ronald G

    2011-09-01

    For more than three decades clinical research in the United States has been explicitly guided by the idea that ethical considerations must be central to research design and practice. In spite of the centrality of this idea, attempting to balance the sometimes conflicting values of advancing scientific knowledge and protecting human subjects continues to pose challenges. Possible conflicts between the standards of scientific research and those of ethics are particularly salient in relation to trial design. Specifically, the choice of a control arm is an aspect of trial design in which ethical and scientific issues are deeply entwined. Although ethical quandaries related to the choice of control arms may arise when conducting any type of clinical trials, they are conspicuous in early phase gene transfer trials that involve highly novel approaches and surgical procedures and have children as the research subjects. Because of children's and their parents' vulnerabilities, in trials that investigate therapies for fatal, rare diseases affecting minors, the scientific and ethical concerns related to choosing appropriate controls are particularly significant. In this paper we use direct gene transfer to the central nervous system to treat late infantile neuronal ceroid lipofuscinosis to illustrate some of these ethical issues and explore possible solutions to real and apparent conflicts between scientific and ethical considerations.

  15. Surfactant from neonatal to pediatric ICU: bench and bedside evidence.

    Science.gov (United States)

    Boet, A; Brat, R; Aguilera, S S; Tissieres, P; De Luca, D

    2014-12-01

    Surfactant is a cornerstone of neonatal critical care for the treatment of respiratory distress syndrome of preterm babies. However, other indications have been studied for various clinical conditions both in term neonates and in children beyond neonatal age. A high degree of evidence is not yet available in some cases and this is due to the complex and not yet totally understood physiopathology of the different types of pediatric and neonatal lung injury. We here summarise the state of the art of the bench and bedside knowledge about surfactant use for the respiratory conditions usually cared for in neonatal and pediatric intensive care units. Future research direction will also be presented. On the whole, surfactant is able to improve oxygenation in infection related respiratory failure, pulmonary hemorrhage and meconium aspiration syndrome. Bronchoalveolar lavage with surfactant solution is currently the only means to reduce mortality or need for extracorporeal life support in neonates with meconium aspiration. While surfactant bolus or lavage only improves the oxygenation and ventilatory requirements in other types of postneonatal acute respiratory distress syndrome (ARDS), there seems to be a reduction in the mortality of small infants with RSV-related ARDS.

  16. Pediatric Acute Kidney Injury.

    Science.gov (United States)

    Fragasso, Tiziana; Ricci, Zaccaria; Goldstein, Stuart L

    2018-01-01

    Acute kidney injury (AKI) in children is a serious condition with an important impact on morbidity and mortality. Onset can be insidious and it is frequently unrecognized in the early phase when the therapeutic opportunities are theoretically more effective. The present review focuses on the most recent epidemiology studies and the progress in pediatric AKI (pAKI) research. Standardization of definition (presented in the Kidney Disease: Improving Global Outcomes) and novel biomarkers have been developed to help clinicians recognize kidney injury in a timely manner, both in adult and pediatric populations. Strengths and weaknesses of these diagnostic tools are discussed and the clinical scoring system (Renal Angina Index), which aims to provide a rational context for biomarker utilization, is also presented. Even if effective treatments are not currently available for established AKI, specific preventive approaches and some promising pharmacological treatments will be detailed. Renal replacement therapy is currently considered the most effective way to manage fluid balance when severe AKI occurs. Key Messages: Great efforts in pAKI research have today led to new strategies for early AKI detection and prevention strategies. Further studies have to be conducted in the next future in order to definitely improve the outcomes of pediatric patients experiencing this deadly syndrome. © 2018 S. Karger AG, Basel.

  17. IS ETHICAL HACKING ETHICAL?

    OpenAIRE

    MUHAMMAD NUMAN ALI KHAN; DANISH JAMIL,

    2011-01-01

    This paper explores the ethics behind ethical hacking and whether there are problems that lie with this new field of work. Since ethical hacking has been a controversial subject over the past few years, the question remains of the true intentions of ethical hackers. The paper also looks at ways in which future research could be looked intoto help keep ethical hacking, ethical.

  18. Perceived barriers to pediatrician and family practitioner participation in pediatric clinical trials: Findings from the Clinical Trials Transformation Initiative

    Directory of Open Access Journals (Sweden)

    Rachel G. Greenberg

    2018-03-01

    Of the 136 providers surveyed, 52/136 (38% had previously referred a pediatric patient to a trial, and only 17/136 (12% had ever been an investigator for a pediatric trial. Lack of awareness of existing pediatric trials was a major barrier to patient referral by providers, in addition to consideration of trial risks, distance to the site, and time needed to discuss trial participation with parents. Overall, providers perceived greater challenges related to parental concerns and parent or child logistical barriers than study implementation and ethics or regulatory barriers as barriers to their practice serving as a trial site. Providers who had previously been an investigator for a pediatric trial were less likely to be concerned with potential barriers than non-investigators. Understanding the barriers that limit pediatric providers from collaboration or inhibit their participation is key to designing effective interventions to optimize pediatric trial participation.

  19. Pediatric suppositories of sulpiride solid dispersion for treatment of Tourette syndrome: in vitro and in vivo investigations.

    Science.gov (United States)

    Zidan, Ahmed S; Emam, Sherif E; Shehata, Tamer M; Ghazy, Fakhr-eldin S

    2015-06-01

    Pharmaceutical development was adopted in the current study to propose a pediatric rectal formulation of sulpiride as a substitute to the available oral or parenteral formulations in the management of Tourette syndrome (TS). The goal was to formulate a product that is easy to use, stable, and highly bioavailable and to achieve a rapid clinical efficacy. Towards this aim, sulpiride solid dispersion (SD) with tartaric acid at a weight ratio of 1:0.25 was incorporated into different suppository bases, namely witepsol W25, witepsol H15, witepsol E75, suppocire NA, suppocire A, glycerogelatin, and polyethylene glycols. The formulae were evaluated in vitro using different pharmacotechnical methods such as visual, melting, weight and content uniformities, drug release, differential scanning calorimetry (DSC), Fourier transform infrared (FTIR), and X-ray diffraction (XRD) analyses. In vivo bioavailability was also assessed in rabbits to compare the bioavailability of either raw sulpiride-incorporated or its SD-incorporated witepsol H15-based suppositories to its oral suspension (reference). Sulpiride SD-incorporated witepsol H15 formulation showed acceptable in vitro characteristics with a bioavailability of 117% relative to oral dosing, which excel that in humans (27% after dosing of oral product). In addition, the proposed formula not only passed the 6-month stability study but also proposed a promising scale-up approach. Hence, it showed a great potential for pediatric product development to manage TS in rural areas.

  20. HYPERTROPHIC CARDIOMYOPATHY AS A PART OF INHERITED MALFORMATION SYNDROMES IN INFANTS

    Directory of Open Access Journals (Sweden)

    M.V. Tural'chuk

    2011-01-01

    Full Text Available The data of clinical and instrumental examination of two infantile patients with obstructive hypertrophic cardiomyopathy in association with marked multisystem involvement as a picture of inherited malformation syndromes are given.Key words: infants, hypertrophic cardiomyopathy, LEOPARD syndrome, Noonan syndrome.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2011; 10 (3: 166–169

  1. Activity of Ethics Committees in Europe on issues related to clinical trials in paediatrics: Results of a survey

    Czech Academy of Sciences Publication Activity Database

    Altavilla, A.; Giaquinto, C.; Giocanti, D.; Manfredi, C.; Aboulker, J.-P.; Bartoloni, F.; Cattani, E.; Giudice, M. L.; Mellado Peňa, M.J.; Nagler, R.; Peterson, C.; Vajnerová, Olga; Bonifazi, F.; Ceci, A.

    2009-01-01

    Roč. 11, 1-2 (2009), s. 79-87 ISSN 1389-2827 Grant - others:EC(XE) LSHBCT-2005-005126 Institutional research plan: CEZ:AV0Z50110509 Keywords : ethic committees * clinical trials * pediatrics Subject RIV: FP - Other Medical Disciplines

  2. The McGill Interactive Pediatric OncoGenetic Guidelines: An approach to identifying pediatric oncology patients most likely to benefit from a genetic evaluation.

    Science.gov (United States)

    Goudie, Catherine; Coltin, Hallie; Witkowski, Leora; Mourad, Stephanie; Malkin, David; Foulkes, William D

    2017-08-01

    Identifying cancer predisposition syndromes in children with tumors is crucial, yet few clinical guidelines exist to identify children at high risk of having germline mutations. The McGill Interactive Pediatric OncoGenetic Guidelines project aims to create a validated pediatric guideline in the form of a smartphone/tablet application using algorithms to process clinical data and help determine whether to refer a child for genetic assessment. This paper discusses the initial stages of the project, focusing on its overall structure, the methodology underpinning the algorithms, and the upcoming algorithm validation process. © 2017 Wiley Periodicals, Inc.

  3. Posterior Reversible Encephalopathy Syndrome in Pediatric Cancer: Clinical and Radiologic Findings

    Directory of Open Access Journals (Sweden)

    Saadiya Javed Khan

    2017-12-01

    Full Text Available Purpose: Posterior reversible encephalopathy syndrome (PRES is associated with a range of medical conditions and medications. In this retrospective analysis, we present 19 pediatric patients with PRES who had undergone chemotherapy. Methods: We identified four female and 15 male patients diagnosed with PRES on the basis of clinical and radiologic features. Patient charts were reviewed from January 2013 to June 2016 after authorization from the institutional review board. Results: The average age of patients with PRES was 7 years. Primary diagnoses were non-Hodgkin lymphoma (n = 9, acute pre–B-cell leukemia (n = 5, relapsed pre–B-cell leukemia (n = 2, Hodgkin lymphoma (n = 2, and Ewing sarcoma (n = 1. PRES occurred during induction chemotherapy in 12 patients. Sixteen patients had hypertension when they developed PRES. Most of these patients (n = 13 were receiving corticosteroids on diagnosis of PRES. Common clinical features were hypertension, seizures, and altered mental status. With the exclusion of three patients, all others required antiepileptic therapy. Ten of these patients underwent additional magnetic resonance imaging. Ten patients are still alive. Conclusion: In patients who presented to our center with signs and symptoms of hypertension, seizures, visual loss, or altered mental status, PRES was mostly seen in those who were undergoing systemic and intrathecal chemotherapy. Approximately 40% of the patients had reversal of clinical and radiologic findings. Antiepileptic medications were discontinued after being seizure free for approximately 6 months.

  4. Carmi syndrome associated with gastric perforation

    African Journals Online (AJOL)

    a case of Carmi syndrome in a neonate who presented with an uncommon ... Departments of aPediatric Surgery and bGeneral Surgery, Fr Muller Medical. College ... Emergency laparotomy indicated a large ... evidence of peritonitis. Pyloric ...

  5. Review of Pediatric Pheochromocytoma and Paraganglioma

    Directory of Open Access Journals (Sweden)

    Reshma Bholah

    2017-07-01

    Full Text Available Pheochromocytoma (PCC and paraganglioma (PGL are rare chromaffin cell tumors which secrete catecholamines and form part of the family of neuroendocrine tumors. Although a rare cause of secondary hypertension in pediatrics, the presentation of hypertension in these patients is characteristic, and treatment is definitive. The gold standard for diagnosis is via measurement of plasma free metanephrines, with imaging studies performed for localization, identification of metastatic lesions and for surgical resection. Preoperative therapy with alpha-blocking agents, beta blockers, and potentially tyrosine hydroxylase inhibitors aid in a safe pre-, intra- and postoperative course. PCC and PGL are inherited in as much as 80% of pediatric cases, and all patients with mutations should be followed closely given the risk of recurrence and malignancy. While the presentation of chromaffin cell tumors has been well described with multiple endocrine neoplasia, NF1, and Von Hippel–Lindau syndromes, the identification of new gene mutations leading to chromaffin cell tumors at a young age is changing the landscape of how clinicians approach such cases. The paraganglioma–pheochromocytoma syndromes (SDHx comprise familial gene mutations, of which the SDHB gene mutation carries a high rate of malignancy. Since the inheritance rate of such tumors is higher than previously described, genetic screening is recommended in all patients, and lifelong follow-up for recurrent tumors is a must. A multidisciplinary team approach allows for optimal health-care delivery in such children. This review serves to provide an overview of pediatric PCC and PGL, including updates on the preferred methods of imaging, guidelines on gene testing as well as management of hypertension in such patients.

  6. Circulating dendritic cells in pediatric patients with nephrotic syndrome

    African Journals Online (AJOL)

    Background: Dendritic cells (DCs) represent one of the most extensively studied topics in immunology, because of their central role in the induction and regulation of adaptive immunity, and because of their therapeutic potential for manipulating immune responses. Objectives: To evaluate circulating DC levels in pediatric ...

  7. The Differencies in Adult and Pediatric Myelodysplastic Syndrome: A Review

    Directory of Open Access Journals (Sweden)

    Vasekova P

    2016-08-01

    Full Text Available Myelodysplastic syndrome (MDS represent very heterogenous group of clonal stem cell bone marrow disorders with ineffective haematopoesis leading to cytopenias in peripheral blood and increased risk of blastic transformation and evolution of acute myeloid leukemia. MDS is a disease of older age mostly, in children it seems to be very rare. There are several significant morphological, cytogenetic and prognostic differencies of the disease in adults and in children. Adult MDS patients most commonly manifest with symptoms of anemia, bleeding and infection are uncommon. In childhood, MDS manifests predominantly by neutropenia and thrombocytopenia. In addition, some pediatric MDS patients present also with constitutional disease’s signs and symptoms. Early and correct diagnosis in both age groups is essential for the choice of appropriate therapy and also for next life of patients. However, the diagnosis of MDS is challenging, complex and requiring close correlation of clinical symptoms, laboratory parameters and standardized examination of BM biopsies. The authors present an overview focused on biology of MDS in adults and children, on the differences in the incidence, clinical presentation and treatment. They summarize the possibilities and limits of histopathological diagnosis and differential diagnosis of the disease in different age groups. A major problem in the morphological diagnosis of MDS remains the determination, whether the myelodysplasia is due to clonal disorder. It might result also from some other factors, as significant dysplasia can also occur in reactive conditions, and vice versa, only discrete dysplasia is sometimes observed in MDS patients. Although histomorphological and immunohistochemical analysis of BM biopsy is invasive and time-consuming examination, it has its value in the diagnosis, differential diagnosis and evaluation of therapeutic effect.

  8. Microaspiration Syndrome in Pediatric Practice: Modern Features and Role in Bronchial Obstruction Syndrome Formation

    Directory of Open Access Journals (Sweden)

    S.I. Ilchenko

    2016-10-01

    Full Text Available The paper presents the data on research of aspiration syndrome prevalence among young children treated in the City children’s pulmonary department due to protracted course of obstructive bronchitis. The structure of microaspiration reasons in young children was studied (2010–2015. The most significant reasons of microaspiration syndrome development were revealed depending on children age. Modified questionnaire for the parents was used to collect anamnesis effectively. The children with perinatal impairment of nervous system, preterm children, and the children with morphological and physio­logical nasopharyngeal defects, with muscular dystonia are firstly in risk group for microaspiration syndrome. Microaspiration may manifest with frequent regurgitation, vomiting during cough, meal leaking from the nose, correlation of coughing fit with feeding, exacerbation or development of coughing in prone position, fit of night coughing and asphyxia, long-term hacking after cough attack. Pediatricians are recommended to observe attentively their patients during each feeding as dysphagy could be inconstant. Microaspiration syndrome is a challenging for diagnosis and requires complex approach, as mostly diagnostic procedures are invasive. Timely diagnosis and treatment of microaaspiration syndrome allow decrease respiratory diseases rate in young children and reduce obstructive bronchitis duration.

  9. Aromatase inhibitors in pediatrics.

    Science.gov (United States)

    Wit, Jan M; Hero, Matti; Nunez, Susan B

    2011-10-25

    Aromatase, an enzyme located in the endoplasmic reticulum of estrogen-producing cells, catalyzes the rate-limiting step in the conversion of androgens to estrogens in many tissues. The clinical features of patients with defects in CYP19A1, the gene encoding aromatase, have revealed a major role for this enzyme in epiphyseal plate closure, which has promoted interest in the use of inhibitors of aromatase to improve adult height. The availability of the selective aromatase inhibitors letrozole and anastrozole--currently approved as adjuvant therapy for breast cancer--have stimulated off-label use of aromatase inhibitors in pediatrics for the following conditions: hyperestrogenism, such as aromatase excess syndrome, Peutz-Jeghers syndrome, McCune-Albright syndrome and functional follicular ovarian cysts; hyperandrogenism, for example, testotoxicosis (also known as familial male-limited precocious puberty) and congenital adrenal hyperplasia; pubertal gynecomastia; and short stature and/or pubertal delay in boys. Current data suggest that aromatase inhibitors are probably effective in the treatment of patients with aromatase excess syndrome or testotoxicosis, partially effective in Peutz-Jeghers and McCune-Albright syndrome, but probably ineffective in gynecomastia. Insufficient data are available in patients with congenital adrenal hyperplasia or functional ovarian cysts. Although aromatase inhibitors appear effective in increasing adult height of boys with short stature and/or pubertal delay, safety concerns, including vertebral deformities, a decrease in serum HDL cholesterol levels and increase of erythrocytosis, are reasons for caution.

  10. Clinical Trials in Pediatrics and Neonatology: Reasons for Ups and Downs

    Directory of Open Access Journals (Sweden)

    A. A. Mosikian

    2016-01-01

    Full Text Available The predictability of results of pediatric clinical trials is often limited for a number of reasons. Among the main ones is the imperfect functioning of organ due to immature ontogeny of enzyme and organ systems in children, and the presence of special subpopulations of full-term newborns and preterm neonates sometimes being in a critical condition. The main task of a present-day pediatric investigational plan is to develop drugs and to elaborate doses that are «specifically designed», not simply «suitable» for neonates. Other reasons for limited predictability are as follows: adult data extrapolation constraint due to children’s anatomic and physiological features, the lack of clinical trial subjects resulting in inability to select an optimal dose by its escalation, the absence of consensus on the ethical aspect of pediatric clinical trials, the etiopathogenetic difference of some diseases and conditions depending on subject’s age, and prevalence of placebo-effect in children. Nowadays it is supposed to be very important to publish all, even failed, pediatric trials to improve the accuracy of pharmacological effects modeling in different subpopulations.

  11. Efficacy of levamisole in children with frequently relapsing and steroid-dependent nephrotic syndrome.

    Science.gov (United States)

    Ekambaram, Sudha; Mahalingam, Vijayakumar; Nageswaran, Prahlad; Udani, Amish; Geminiganesan, Sangeetha; Priyadarshini, Shweta

    2014-05-01

    To assess the efficacy of levamisole in frequently relapsing nephrotic syndrome and steroid-dependent nephrotic syndrome. Retrospective analysis of hospital case records. Pediatric nephrology department of a tertiary referral pediatric hospital. 62 children with frequently relapsing nephrotic syndrome and 35 children with steroid-dependent nephrotic syndrome. Case records of children who were diagnosed as steroid-dependant or frequently-relapsing nephrotic syndrome from June 2004 to June 2011, were reviewed. Levamisole was given daily (2 mg/kg/d) along with tapering doses of alternate day steroids after remission on daily steroids. Levamisole was effective in 77.3% children with a better (80.6%) efficacy in frequently relapsing nephrotic syndrome. A total of 34 children completed 1 year follow-up post levamisole therapy. The cumulative mean (SD) steroid dose 1-year before therapy was 4109(1154) mg/m2 and 1-year post therapy was 661 (11) mg/m2 (P<0.001). The relapses were also less during the period of post-levamisole therapy. Levamisole is an effective alternative therapy in frequently relapsing and steroid-dependent nephrotic syndrome.

  12. Ethical considerations in umbilical cord blood banking.

    Science.gov (United States)

    Fox, Nathan S; Chervenak, Frank A; McCullough, Laurence B

    2008-01-01

    Pregnant patients have the option at delivery of having their cord blood collected and stored for future use. At many hospitals, they have the option of donating their cord blood to the public banking system for future use by anyone who is an appropriate match (public banking). Patients also have the option of having their cord blood stored for a fee with a commercial/private company for future use within their family (private banking). Currently, private banking is not recommended by major obstetric and pediatric professional organizations. We applied current evidence of the risks and benefits of private and public cord blood banking and accepted ethical principles to answer the following two related questions: 1) Do obstetricians have an ethical obligation to comply with a request for private banking? and 2) Do obstetricians have an ethical obligation to routinely offer private banking to women who do not request it? The only situation where there is a known benefit to private banking is when public banking is not available and the patient currently has an affected family member who may benefit from cord blood therapy. We conclude that when presented with a request for private banking, obstetricians have an ethical obligation to explain the lack of proven benefit of this procedure. If the patient still requests private banking, it would be appropriate to comply, because there is minimal or no risk to the procedure. However, obstetricians are not ethically obligated to offer private banking, even when public banking is not available, except in the limited circumstance when the patient currently has an affected family member who may benefit from cord blood therapy.

  13. A practical guide for short-term pediatric surgery global volunteers.

    Science.gov (United States)

    Meier, Donald E; Fitzgerald, Tamara N; Axt, Jason R

    2016-08-01

    The tremendous need for increasing the quantity and quality of global pediatric surgical care in underserved areas has been well documented. Concomitantly there has been a significant increase in interest by pediatric surgeons in helping to relieve this problem through surgical volunteerism. The intent of the article is to serve as a practical guide for pediatric surgeons contemplating or planning a short-term global volunteer endeavor. The article is based on the authors' personal experiences and on the published experiences of other volunteers. The following aspects of volunteerism are discussed: ethical considerations, where and how to go, what and whom to take with you, what to expect in your volunteer locale, and what to do and what to avoid in order to enhance the volunteer experience. The points discussed in this guide will hopefully make the volunteer activity one that results in greatly improved immediate and long term surgical care for children and improves the chances that the activity will be a meaningful, pleasant, and productive experience for both the volunteer and the host physician. Copyright © 2016 Elsevier Inc. All rights reserved.

  14. Pediatric allergy and immunology in Japan.

    Science.gov (United States)

    Ebisawa, Motohiro; Nishima, Sankei; Ohnishi, Hidenori; Kondo, Naomi

    2013-11-01

    The Japanese Society of Pediatric Allergy and Clinical Immunology (JSPACI) was started in 1966 and currently has 3613 members as of August 1, 2012. The number of pediatricians specializing in allergies who have been certified by the Japanese Society of Allergology is 817. Among these, there are 125 training directors and training facilities for allergy and clinical immunology. The JSPACI first published an asthma guideline specific for children in 2000, and this has been revised every 3 yrs, contributing to better control of pediatric asthma. Food allergy management guidelines were first developed in 2005, which have helped to improve the care of food allergy patients. Among 514 pediatric training programs by the Japanese Society of Pediatrics, there are 312 facilities routinely performing oral food challenges. Among these, there were already 53 facilities performing oral immunotherapy at the end of 2011, treating 1400 cases of food allergy. The prevalence of pediatric allergic diseases has increased in Japan over the past 50 yrs. A number of International Study of Asthma and Allergies in Childhood surveys have been conducted in the past at specific times. The prevalence of wheezing among children aged 13-14 yrs in 2002 was 13.0%. Multi-year surveys found a 1.5- to 2-fold increase every 10 yrs until 2002. However, according to the latest data in 2012, asthma prevalence seems to have slightly decreased in Japan. Food allergy mainly associated with infantile atopic eczema among infants younger than 1 yr of age is the most common form as with other developed countries. The estimated food allergy prevalence based on data from several surveys is 5-10% among infants (0-6 yrs) and 1-2% among schoolchildren (6-15 yrs). A variety of patients suffering from primary deficiency syndrome have been actively analyzed. Previously, antibody defects and well-defined syndromes with immunodeficiency were analyzed, but recent research is focusing on not only acquired immune

  15. A Case of Pediatric Abdominal Wall Reconstruction: Components Separation within the Austere War Environment

    Directory of Open Access Journals (Sweden)

    Ian Valerio, MD, MS, MBA, FACS

    2014-07-01

    Full Text Available Summary: Reconstructive surgeons supporting military operations are required to definitively treat severe pediatric abdominal injuries in austere environments. The safety and efficacy of using a components separation technique to treat large ventral hernias in pediatric patients in this setting remains understudied. Components separation technique was required to achieve definitive closure in a 12-month-old pediatric patient in Kandahar, Afghanistan. Her course was complicated by an anastomotic leak after small bowel resection. Her abdominal was successfully reopened, the leak repaired, and closed primarily without incident on postinjury day 9. Abdominal trauma with a large ventral hernia requiring components separation is extremely rare. A pediatric patient treated with components separation demonstrated minimal complications, avoidance of abdominal compartment syndrome, and no mortality.

  16. Pediatric Miller Fisher Syndrome Complicating an Epstein-Barr Virus Infection.

    Science.gov (United States)

    Communal, Céline; Filleron, Anne; Baron-Joly, Sandrine; Salet, Randa; Tran, Tu-Anh

    2016-10-01

    Miller Fisher syndrome, a variant of Guillain-Barré syndrome, is an acute inflammatory demyelinating polyradiculoneuropathy that may occur weeks after a bacterial or viral infection. Campylobacter jejuni and Haemophilus influenzae are frequently reported etiological agents. We describe a boy with Miller Fisher syndrome following Epstein-002DBarr virus primary infectious mononucleosis. He presented with bilateral dysfunction of several cranial nerves and hyporeflexia of the limbs but without ataxia. Miller Fisher syndrome was confirmed by the presence of anti-GQ1b antibodies in a blood sample. Epstein-Barr virus was identified by polymerase chain reaction and serology. Epstein-Barr virus should be considered as a Miller Fisher syndrome's causative agent. The physiopathology of this condition may involve cross-reactive T-cells against Epstein-Barr virus antigens and gangliosides. Copyright © 2016 Elsevier Inc. All rights reserved.

  17. Gut-directed hypnotherapy in children with irritable bowel syndrome or functional abdominal pain (syndrome): A randomized controlled trial on self exercises at home using CD versus individual therapy by qualified therapists

    NARCIS (Netherlands)

    J.M.T.M. Rutten (Juliette); A.M. Vlieger (Arine M.); C. Frankenhuis (Carla); E.K. George (Elvira K.); M. Groeneweg (Michael); O.F. Norbruis (Obbe); W.E. Tjon A ten; H. Van Wering (Herbert); M.G.W. Dijkgraaf (Marcel); M.P. Merkus; M.A. Benninga (Marc)

    2014-01-01

    textabstractBackground: Irritable bowel syndrome (IBS) and functional abdominal pain (syndrome) (FAP(S)) are common pediatric disorders, characterized by chronic or recurrent abdominal pain. Treatment is challenging, especially in children with persisting symptoms. Gut-directed hypnotherapy (HT)

  18. Docosahexaenoic Acid Levels in Blood and Metabolic Syndrome in Obese Children: Is There a Link?

    Science.gov (United States)

    Lassandro, Carlotta; Banderali, Giuseppe; Radaelli, Giovanni; Borghi, Elisa; Moretti, Francesca; Verduci, Elvira

    2015-08-21

    Prevalence of metabolic syndrome is increasing in the pediatric population. Considering the different existing criteria to define metabolic syndrome, the use of the International Diabetes Federation (IDF) criteria has been suggested in children. Docosahexaenoic acid (DHA) has been associated with beneficial effects on health. The evidence about the relationship of DHA status in blood and components of the metabolic syndrome is unclear. This review discusses the possible association between DHA content in plasma and erythrocytes and components of the metabolic syndrome included in the IDF criteria (obesity, alteration of glucose metabolism, blood lipid profile, and blood pressure) and non-alcoholic fatty liver disease in obese children. The current evidence is inconsistent and no definitive conclusion can be drawn in the pediatric population. Well-designed longitudinal and powered trials need to clarify the possible association between blood DHA status and metabolic syndrome.

  19. Gut-directed hypnotherapy in children with irritable bowel syndrome or functional abdominal pain (syndrome): a randomized controlled trial on self exercises at home using CD versus individual therapy by qualified therapists

    NARCIS (Netherlands)

    Rutten, Juliette M. T. M.; Vlieger, Arine M.; Frankenhuis, Carla; George, Elvira K.; Groeneweg, Michael; Norbruis, Obbe F.; Tjon A ten, Walther; van Wering, Herbert; Dijkgraaf, Marcel G. W.; Merkus, Maruschka P.; Benninga, Marc A.

    2014-01-01

    Irritable bowel syndrome (IBS) and functional abdominal pain (syndrome) (FAP(S)) are common pediatric disorders, characterized by chronic or recurrent abdominal pain. Treatment is challenging, especially in children with persisting symptoms. Gut-directed hypnotherapy (HT) performed by a therapist

  20. [Transition from pediatric to adult health care services for adolescents with chronic diseases: Recommendations from the Adolescent Branch from Sociedad Chilena de Pediatría].

    Science.gov (United States)

    Zubarew, Tamara; Correa, Loreto; Bedregal, Paula; Besoain, Carolina; Reinoso, Alejandro; Velarde, Macarena; Valenzuela, María Teresa; Inostroza, Carolina

    2017-01-01

    The Adolescent Branch from Sociedad Chilena de Pediatría supports the implementation of planned programs for transition from child to adult health centers, oriented to adolescents with chronic diseases, in order to ensure an appropriate follow-up and a high-quality health care. Recommendations for care are set out in the FONIS and VRI PUC project carried out by the Division of Pediatrics of the Universidad Católica de Chile: “Transition process from pediatric to adult services: perspectives of adolescents with chronic diseases, caregivers and health professionals”, whose goal was to describe the experience, barriers, critical points, and facilitators in the transition process. Critical points detected in this study were: existence of a strong bond between adolescents, caregivers and the pediatric team, resistance to transition, difficulty developing autonomy and self-management among adolescents; invisibility of the process of adolescence; and lack of communication between pediatric and adult team during the transfer. According to these needs, barriers and critical points, and based on published international experiences, recommendations are made for implementation of gradual and planned transition processes, with emphasis on the design and implementation of transition policies, establishment of multidisciplinary teams and transition planning. We discuss aspects related to coordination of teams, transfer timing, self-care and autonomy, transition records, adolescent and family participation, need for emotional support, ethical aspects involved, importance of confidentiality, need for professional training, and the need for evaluation and further research on the subject.

  1. Clinical trial simulations in pediatric patients using realistic covariates: application to teduglutide, a glucagon-like peptide-2 analog in neonates and infants with short-bowel syndrome.

    Science.gov (United States)

    Mouksassi, M S; Marier, J F; Cyran, J; Vinks, A A

    2009-12-01

    Teduglutide, a synthetic glucagon-like peptide-2 (GLP-2) analog with activity relating to the regeneration, maintenance, and repair of the intestinal epithelium, is currently being evaluated for the treatment of short-bowel syndrome (SBS), Crohn's disease, and other gastrointestinal disorders. On the basis of promising results from teduglutide studies in adults with SBS and from studies in neonatal and juvenile animal models, a pediatric multiple-dose phase I clinical study was designed to determine the safety, efficacy, and pharmacokinetics of teduglutide in pediatric patients with SBS who have undergone resection for necrotizing enterocolitis, malrotation, or intestinal atresia. This report details the application of clinical trial simulations coupled with a novel approach using generalized additive modeling for location, scale, and shape (GAMLSS) that facilitates the simulation of demographic covariates specific to the targeted patient populations. The goal was to optimize phase I dosing strategies and the likelihood of achieving target exposure and therapeutic effect.

  2. Improving the Level and Quality of Ethics Review in Chinese Medicine and Integrative Medicine.

    Science.gov (United States)

    Zhang, Cheng-Bin; Li, En-Chang

    2018-04-01

    Three features of ethics review in Chinese medicine (CM) and integrative medicine (IM) were put forward in this paper. It is consistent with the principles of ethical review in Western medicine; it has to be compliant with the laws of CM and IM; emphasis should be laid on the review of clinical practice facts and experience. Three problems were pointed out. The characteristics of CM and IM are not distinctive enough, operation procedures need to be refined and effectiveness remains to be improved. Based on the mentioned above, seven measures were proposed to improve the level and quality of ethics review in CM and IM, including better brand awareness, considerable tolerance, treatment based on disease differentiation and syndrome differentiation, scientific review and toxicity and side effects of CM, perfection of the ethics review system, reasonable procedures of ethics review and more specialized ethics review workers.

  3. Anesthesia in a pediatric patient with ROHADD syndrome.

    Science.gov (United States)

    Esparza Isasa, E; Palomero Rodríguez, M A; Acebedo Bambaren, I; Medrano Viñas, C; Gil Mayo, D; Domínguez Pérez, F; Pestaña Lagunas, D

    2018-05-01

    Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome is a rare entity that is characterised by its onset in healthy children at 2-4 years of age. It is a complex syndrome that includes, among other symptoms, rapid weight gain with hyperphagia, hypothalamic dysfunction, central hypoventilation, and autonomic dysregulation. The case is presented of a 10-year-old boy with a diagnosis of ROHHAD syndrome undergoing insertion of a port-a-cath under general anaesthesia, who developed complications during the anaesthetic procedure related to his illness. The peri-operative management of these patients represents a challenge for the anaesthetist, given the involvement of multiple systems and the frequent respiratory comorbidities associated with them. A summary is presented of some of the implications and anaesthetic considerations that must be taken into account in the management of these patients. Copyright © 2018 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.

  4. Risk factors associated with iatrogenic opioid and benzodiazepine withdrawal in critically ill pediatric patients: a systematic review and conceptual model.

    Science.gov (United States)

    Best, Kaitlin M; Boullata, Joseph I; Curley, Martha A Q

    2015-02-01

    Analgesia and sedation are common therapies in pediatric critical care, and rapid titration of these medications is associated with iatrogenic withdrawal syndrome. We performed a systematic review of the literature to identify all common and salient risk factors associated with iatrogenic withdrawal syndrome and build a conceptual model of iatrogenic withdrawal syndrome risk in critically ill pediatric patients. Multiple databases, including PubMed/Medline, EMBASE, CINAHL, and the Cochrane Central Registry of Clinical Trials, were searched using relevant terms from January 1, 1980, to August 1, 2014. Articles were included if they were published in English and discussed iatrogenic withdrawal syndrome following either opioid or benzodiazepine therapy in children in acute or intensive care settings. Articles were excluded if subjects were neonates born to opioid- or benzodiazepine-dependent mothers, children diagnosed as substance abusers, or subjects with cancer-related pain; if data about opioid or benzodiazepine treatment were not specified; or if primary data were not reported. In total, 1,395 articles were evaluated, 33 of which met the inclusion criteria. To facilitate analysis, all opioid and/or benzodiazepine doses were converted to morphine or midazolam equivalents, respectively. A table of evidence was developed for qualitative analysis of common themes, providing a framework for the construction of a conceptual model. The strongest risk factors associated with iatrogenic withdrawal syndrome include duration of therapy and cumulative dose. Additionally, evidence exists linking patient, process, and system factors in the development of iatrogenic withdrawal syndrome. Most articles were prospective observational or interventional studies. Given the state of existing evidence, well-designed prospective studies are required to better characterize iatrogenic withdrawal syndrome in critically ill pediatric patients. This review provides data to support the

  5. Case Study of High-Dose Ketamine for Treatment of Complex Regional Pain Syndrome in the Pediatric Intensive Care Unit.

    Science.gov (United States)

    Pasek, Tracy Ann; Crowley, Kelli; Campese, Catherine; Lauer, Rachel; Yang, Charles

    2017-06-01

    Complex regional pain syndrome (CRPS) is a life-altering and debilitating chronic pain condition. The authors are presenting a case study of a female who received high-dose ketamine for the management of her CRPS. The innovative treatment lies not only within the pharmacologic management of her pain, but also in the fact that she was the first patient to be admitted to our pediatric intensive care unit solely for pain control. The primary component of the pharmacotherapy treatment strategy plan was escalating-dose ketamine infusion via patient-controlled-analgesia approved by the pharmacy and therapeutics committee guided therapy for this patient. The expertise of advanced practice nurses blended exquisitely to ensure patient and family-centered care and the coordination of care across the illness trajectory. The patient experienced positive outcomes. Copyright © 2017 Elsevier Inc. All rights reserved.

  6. Docosahexaenoic Acid Levels in Blood and Metabolic Syndrome in Obese Children: Is There a Link?

    Directory of Open Access Journals (Sweden)

    Carlotta Lassandro

    2015-08-01

    Full Text Available Prevalence of metabolic syndrome is increasing in the pediatric population. Considering the different existing criteria to define metabolic syndrome, the use of the International Diabetes Federation (IDF criteria has been suggested in children. Docosahexaenoic acid (DHA has been associated with beneficial effects on health. The evidence about the relationship of DHA status in blood and components of the metabolic syndrome is unclear. This review discusses the possible association between DHA content in plasma and erythrocytes and components of the metabolic syndrome included in the IDF criteria (obesity, alteration of glucose metabolism, blood lipid profile, and blood pressure and non-alcoholic fatty liver disease in obese children. The current evidence is inconsistent and no definitive conclusion can be drawn in the pediatric population. Well-designed longitudinal and powered trials need to clarify the possible association between blood DHA status and metabolic syndrome.

  7. Pathophysiology and Management of Acute Respiratory Distress Syndrome in Children.

    Science.gov (United States)

    Heidemann, Sabrina M; Nair, Alison; Bulut, Yonca; Sapru, Anil

    2017-10-01

    Acute respiratory distress syndrome (ARDS) is a syndrome of noncardiogenic pulmonary edema and hypoxia that accompanies up to 30% of deaths in pediatric intensive care units. Pediatric ARDS (PARDS) is diagnosed by the presence of hypoxia, defined by oxygenation index or Pao 2 /Fio 2 ratio cutoffs, and new chest infiltrate occurring within 7 days of a known insult. Hallmarks of ARDS include hypoxemia and decreased lung compliance, increased work of breathing, and impaired gas exchange. Mortality is often accompanied by multiple organ failure. Although many modalities to treat PARDS have been investigated, supportive therapies and lung protective ventilator support remain the mainstay. Copyright © 2017 Elsevier Inc. All rights reserved.

  8. Cauda equina syndrome as the initial presenting clinical feature of medulloblastoma: a case report

    Directory of Open Access Journals (Sweden)

    Al-Otaibi Faisal

    2012-05-01

    Full Text Available Abstract Introduction Medulloblastoma is one of the most common pediatric brain malignancies. The usual presenting clinical features are related to posterior fossa syndrome or/and hydrocephalus. Cauda equina syndrome is a very rare presentation for this disease. Case presentation We describe the case of a three-year-old boy with cauda equina syndrome as the initial presenting clinical feature for medulloblastoma. He was initially diagnosed as having a spinal tumor by magnetic resonance imaging scan. Subsequently, a cranial magnetic resonance imaging scan revealed a posterior fossa tumor with features of dissemination. He had substantial improvement after treatment. This case report is complemented by a literature review related to this unusual presentation. Conclusions Medulloblastoma primarily presenting with cauda equina syndrome is very rare. However, spinal drop metastasis should be considered in the pediatric age group to avoid suboptimal management.

  9. Magnetic resonance imaging of pediatric soft-tissue vascular anomalies

    International Nuclear Information System (INIS)

    Navarro, Oscar M.

    2016-01-01

    Magnetic resonance (MR) imaging can be used in the management of pediatric soft-tissue vascular anomalies for diagnosing and assessing extent of lesions and for evaluating response to therapy. MR imaging studies often involve a combination of T1- and T2-weighted images in addition to MR angiography and fat-suppressed post-contrast sequences. The MR imaging features of these vascular anomalies when combined with clinical findings can aid in diagnosis. In cases of complex vascular malformations and syndromes associated with vascular anomalies, MR imaging can be used to evaluate accompanying soft-tissue and bone anomalies. This article reviews the MR imaging protocols and appearances of the most common pediatric soft-tissue vascular anomalies. (orig.)

  10. Pediatric siMS score: A new, simple and accurate continuous metabolic syndrome score for everyday use in pediatrics.

    Science.gov (United States)

    Vukovic, Rade; Milenkovic, Tatjana; Stojan, George; Vukovic, Ana; Mitrovic, Katarina; Todorovic, Sladjana; Soldatovic, Ivan

    2017-01-01

    The dichotomous nature of the current definition of metabolic syndrome (MS) in youth results in loss of information. On the other hand, the calculation of continuous MS scores using standardized residuals in linear regression (Z scores) or factor scores of principal component analysis (PCA) is highly impractical for clinical use. Recently, a novel, easily calculated continuous MS score called siMS score was developed based on the IDF MS criteria for the adult population. To develop a Pediatric siMS score (PsiMS), a modified continuous MS score for use in the obese youth, based on the original siMS score, while keeping the score as simple as possible and retaining high correlation with more complex scores. The database consisted of clinical data on 153 obese (BMI ≥95th percentile) children and adolescents. Continuous MS scores were calculated using Z scores and PCA, as well as the original siMS score. Four variants of PsiMS score were developed in accordance with IDF criteria for MS in youth and correlation of these scores with PCA and Z score derived MS continuous scores was assessed. PsiMS score calculated using formula: (2xWaist/Height) + (Glucose(mmol/l)/5.6) + (triglycerides(mmol/l)/1.7) + (Systolic BP/130)-(HDL(mmol/l)/1.02) showed the highest correlation with most of the complex continuous scores (0.792-0.901). The original siMS score also showed high correlation with continuous MS scores. PsiMS score represents a practical and accurate score for the evaluation of MS in the obese youth. The original siMS score should be used when evaluating large cohorts consisting of both adults and children.

  11. Pediatric MS

    Science.gov (United States)

    ... Pediatric MS Share this page Facebook Twitter Email Pediatric MS Pediatric MS Pediatric MS Support Pediatric Providers ... system through the Pediatric MS Support Group . Treating pediatric MS In 2018 the U.S. Food and Drug ...

  12. EFFICACY OF HYOSCINE BUTYLBROMIDE IN TREATMENT OF IRRITABLE BOWEL SYNDROME IN CHILDREN: PLACEBO-CONTROLLED TRIAL

    Directory of Open Access Journals (Sweden)

    K.V. Arifullina

    2008-01-01

    Full Text Available The activity of hyoscine butylbromide (buscopan was evaluated in a placebobcontrolled trial, on pediatric patients with algid type of irritable bowel syndrome. Hyoscine butylbromide favored to the increase of quality of life in pediatric patients, alleviation of clinical symptoms of disease, reliable decrease of malonic dialdehyde and increase of antioxidant activity of blood plasma significantly superior to placebo. Clinical efficacy of hyoscine butylbromide accompanies to its good tolerance and safety.Key words: children, irritable bowel syndrome, hyoscine butylbromide, placebo controlled trial.

  13. Nationwide survey of Arima syndrome: revised diagnostic criteria from epidemiological analysis.

    Science.gov (United States)

    Itoh, Masayuki; Iwasaki, Yuji; Ohno, Kohsaku; Inoue, Takehiko; Hayashi, Masaharu; Ito, Shuichi; Matsuzaka, Tetsuo; Ide, Shuhei; Arima, Masataka

    2014-05-01

    We have never known any epidemiological study of Arima syndrome since it was first described in 1971. To investigate the number of Arima syndrome patients and clarify the clinical differences between Arima syndrome and Joubert syndrome, we performed the first nationwide survey of Arima syndrome, and herein report its results. Furthermore, we revised the diagnostic criteria for Arima syndrome. As a primary survey, we sent out self-administered questionnaires to most of the Japanese hospitals with a pediatric clinic, and facilities for persons with severe motor and intellectual disabilities, inquiring as to the number of patients having symptoms of Arima syndrome, including severe psychomotor delay, agenesis or hypoplasia of cerebellar vermis, renal dysfunction, visual dysfunction and with or without ptosis-like appearance. Next, as the second survey, we sent out detailed clinical questionnaires to the institutes having patients with two or more typical symptoms. The response rate of the primary survey was 72.7% of hospitals with pediatric clinic, 63.5% of national hospitals and 66.7% of municipal and private facilities. The number of patients with 5 typical symptoms was 13 and that with 2-4 symptoms was 32. The response rate of the secondary survey was 52% (23 patients). After reviewing clinical features of 23 patients, we identified 7 Arima syndrome patients and 16 Joubert syndrome patients. Progressive renal dysfunction was noticed in all Arima syndrome patients, but in 33% of those with Joubert syndrome. It is sometimes difficult to distinguish Arima syndrome from Joubert syndrome. Some clinicians described a patient with Joubert syndrome and its complications of visual dysfunction and renal dysfunction, whose current diagnosis was Arima syndrome. Thus, the diagnosis of the two syndromes may be confused. Here, we revised the diagnostic criteria for Arima syndrome. Copyright © 2013 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights

  14. Coexistence of Mayer-Rokitansky-Küster-Hauser Syndrome and Turner Syndrome: A Case Report.

    Science.gov (United States)

    Białka, Agnieszka; Gawlik, Aneta; Drosdzol-Cop, Agnieszka; Wilk, Krzysztof; Małecka-Tendera, Ewa; Skrzypulec-Plinta, Violetta

    2016-04-01

    Turner syndrome is a common chromosomal disorder, with an incidence of 1 in 2000 live-born female infants. Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) affects 1 in 4500 female births and, rarely, it might be associated with gonadal dysgenesis. A 17-year-old girl was referred to our clinic with short stature and primary amenorrhea. The patient was diagnosed with Turner syndrome and underwent estrogen therapy. At the age of 24 years, just after the patient's sexual initiation, the first complete gynecological examination was performed. A blind-ending vagina was revealed and the patient was diagnosed with MRKH. Early diagnosis of coexistence of MRKH and Turner syndrome, although very difficult, might prevent patients from developing serious complications. Copyright © 2016 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  15. Ethical aspects of registry-based research in the Nordic countries

    Directory of Open Access Journals (Sweden)

    Ludvigsson JF

    2015-11-01

    Full Text Available Jonas F Ludvigsson,1,2 Siri E Håberg,3 Gun Peggy Knudsen,3 Pierre Lafolie,4,5 Helga Zoega,6 Catharina Sarkkola,7 Stephanie von Kraemer,7 Elisabete Weiderpass,1,7–10 Mette Nørgaard11 1Department of Medical Epidemiology and Biostatistics, Karolinska Institutet, Stockholm, 2Department of Pediatrics, Örebro University Hospital, Örebro, Sweden; 3Norwegian Institute of Public Health, Oslo, Norway; 4Department of Medicine, Clinical Pharmacology Unit, 5The Stockholm Regional Ethical Review Board, Karolinska Institutet, Stockholm, Sweden; 6Center of Public Health Sciences, Faculty of Medicine, University of Iceland, Reykjavík, Iceland; 7Genetic Epidemiology Group, Folkhälsan Research Center, Helsinki, Finland; 8Department of Community Medicine, Faculty of Health Sciences, University of Tromsø, 9The Arctic University of Norway, Tromsø, 10Department of Research, Cancer Registry of Norway, Oslo, Norway; 11Department of Clinical Epidemiology, Aarhus University Hospital, Aarhus, Denmark Abstract: National health care registries in the Nordic countries share many attributes, but different legal and ethical frameworks represent a challenge to promoting effective joint research. Internationally, there is a lack of knowledge about how ethical matters are considered in Nordic registry-based research, and a lack of knowledge about how Nordic ethics committees operate and what is needed to obtain an approval. In this paper, we review ethical aspects of registry-based research, the legal framework, the role of ethics review boards in the Nordic countries, and the structure of the ethics application. We discuss the role of informed consent in registry-based research and how to safeguard the integrity of study participants, including vulnerable subjects and children. Our review also provides information on the different government agencies that contribute registry-based data, and a list of the major health registries in Denmark, Finland, Iceland, Norway, and

  16. Ethical, legal and social implications of prenatal and preimplantation genetic testing for cancer susceptibility.

    Science.gov (United States)

    Wang, C-W; Hui, E C

    2009-01-01

    With the progress in cancer genetics and assisted reproductive technologies, it is now possible for cancer gene mutation carriers not only to reduce cancer mortality through the targeting of surveillance and preventive therapies, but also to avoid the birth of at-risk babies through the choice of different means of reproduction. Thus, the incidence of hereditary cancer syndromes may be decreased in the future. The integration of cancer genetic testing and assisted reproductive technologies raises certain ethical, legal and social issues beyond either genetic testing or assisted reproductive technology itself. In this paper, the reproductive decisions/choices of at-risk young couples and the ethical, legal and social concerns of prenatal genetic testing and preimplantation genetic diagnosis for susceptibility to hereditary cancer syndromes are discussed. Specifically, three ethical principles related to the integration of cancer genetic testing and assisted reproductive technologies, i.e. informed choice, beneficence to children and social justice, and their implications for the responsible translation of these medical techniques into common practice of preventive medicine are highlighted.

  17. Medical ethics and ethical dilemmas.

    Science.gov (United States)

    Iyalomhe, G B S

    2009-01-01

    Ethical problems routinely arise in the hospital and outpatient practice settings and times of dilemma do occur such that practitioners and patients are at cross-roads where choice and decision making become difficult in terms of ethics. This paper attempts a synopsis of the basic principles of medical ethics, identifies some ethical dilemmas that doctors often encounter and discusses some strategies to address them as well as emphasizes the need for enhanced ethics education both for physicians and patients particularly in Nigeria. Literature and computer programmes (Medline and PsychoInfo databases) were searched for relevant information. The search showed that the fundamental principles suggested by ethicists to assist doctors to evaluate the ethics of a situation while making a decision include respect for autonomy, beneficence, non-maleficence and justice. Although the above principles do not give answers as to how to handle a particular situation, they serve as a guide to doctors on what principles ought to apply to actual circumstances. The principles sometimes conflict with each other leading to ethical dilemmas when applied to issues such as abortion, contraception, euthanasia, professional misconduct, confidentiality truth telling, professional relationship with relatives, religion, traditional medicine and business concerns. Resolution of dilemmas demand the best of the doctor's knowledge of relevant laws and ethics, his training and experience, his religious conviction and moral principles as well as his readiness to benefit from ethics consultation and the advice of his colleagues. Ethics education should begin from the impressionable age in homes, continued in the medical schools and after graduation to ensure that doctors develop good ethical practices and acquire the ability to effectively handle ethical dilemmas. Also, education of patients and sanction of unethical behaviour will reduce ethical dilemmas.

  18. N-Acetylcysteine in the Treatment of Pediatric Tourette Syndrome: Randomized, Double-Blind, Placebo-Controlled Add-On Trial.

    Science.gov (United States)

    Bloch, Michael H; Panza, Kaitlyn E; Yaffa, Alisa; Alvarenga, Pedro G; Jakubovski, Ewgeni; Mulqueen, Jilian M; Landeros-Weisenberger, Angeli; Leckman, James F

    2016-05-01

    Current pharmacological treatments for Tourette Syndrome (TS), such as antipsychotic agents and α-2 agonists, are moderately effective in the treatment of tics, but have substantial side effects that limit their use. N-acetylcysteine (NAC) modulates glutamatergic systems, and has been used safely as an antioxidant agent with minimal side effects for decades. NAC has been increasingly studied for the treatment of other obsessive-compulsive spectrum disorders. We aim to examine the efficacy of NAC for the treatment of pediatric TS in a double-blind, placebo-controlled, add-on study. Thirty-one children and adolescents 8-17 years of age with TS were randomly assigned to receive NAC or matching placebo for 12 weeks. Our primary outcome was change in severity of tics as measured by the Yale Global Tic Severity Scale (YGTSS), Total tic score. Secondary measures assessed comorbid obsessive-compulsive disorder (OCD), depression, anxiety, and attention-deficit/hyperactivity disorder (ADHD). Linear mixed models in SAS were used to examine differences between NAC and placebo. Of 31 randomized subjects, 14 were assigned to placebo (two females; 11.5 + 2.8 years) and 17 to active NAC (five females; 12.4 + 1.4 years) treatment. No significant difference between NAC and placebo was found in reducing tic severity or any secondary outcomes. We found no evidence for efficacy of NAC in treating tic symptoms. Our findings stand in contrast to studies suggesting benefits of NAC in the treatment of other obsessive-compulsive spectrum disorders in adults, including OCD and trichotillomania, but are similar to a recent placebo-controlled trial of pediatric trichotillomania that found no benefit of NAC.

  19. Designing a Pediatric Severe Sepsis Screening Tool

    Directory of Open Access Journals (Sweden)

    Robert eSepanski

    2014-06-01

    Full Text Available We sought to create a screening tool with improved predictive value for pediatric severe sepsis and septic shock that can be incorporated into the electronic medical record and actively screen all patients arriving at a pediatric Emergency Department (ED. Gold standard severe sepsis cases were identified using a combination of coded discharge diagnosis and physician chart review from 7,402 children who visited a pediatric ED over two months. The tool’s identification of severe sepsis was initially based on International Consensus Conference on Pediatric Sepsis (ICCPS parameters that were refined by an iterative, virtual process that allowed us to propose successive changes in sepsis detection parameters in order to optimize the tool’s predictive value based on receiver operating curve (ROC characteristics. Age-specific normal and abnormal values for heart rate (HR and respiratory rate (RR were empirically derived from 143,603 children seen in a second pediatric ED over three years. Univariate analyses were performed for each measure in the tool to assess its association with severe sepsis and to characterize it as an early or late indicator of severe sepsis. A split-sample was used to validate the final, optimized tool. The final tool incorporated age-specific thresholds for abnormal HR and RR and employed a linear temperature correction for each category. The final tool’s positive predictive value was 48.7%, a significant, nearly three-fold improvement over the original ICCPS tool. False positive Systemic Inflammatory Response Syndrome (SIRS identifications were nearly six-fold lower.

  20. The Ethics of Doing Ethics.

    Science.gov (United States)

    Hansson, Sven Ove

    2017-02-01

    Ethicists have investigated ethical problems in other disciplines, but there has not been much discussion of the ethics of their own activities. Research in ethics has many ethical problems in common with other areas of research, and it also has problems of its own. The researcher's integrity is more precarious than in most other disciplines, and therefore even stronger procedural checks are needed to protect it. The promotion of some standpoints in ethical issues may be socially harmful, and even our decisions as to which issues we label as "ethical" may have unintended and potentially harmful social consequences. It can be argued that ethicists have an obligation to make positive contributions to society, but the practical implications of such an obligation are not easily identified. This article provides an overview of ethical issues that arise in research into ethics and in the application of such research. It ends with a list of ten practical proposals for how these issues should be dealt with.

  1. Is Exercise Stress Testing a Cost-Saving Strategy For Risk Assessment of Pediatric Wolff-Parkinson-White Syndrome Patients?

    Science.gov (United States)

    Moltedo, Jose M.; Iyer, Ramesh V.; Forman, Howard; Fahey, John; Rosenthal, Geoffrey; Snyder, Christopher S.

    2006-01-01

    Background: In Wolff-Parkinson-White syndrome (WPW) patients the loss of pre-excitation in a single heartbeat during exercise stress testing (EST) is a predictor of low risk of sudden death. The purpose of this study was to: 1) assess the frequency of loss of pre-excitation in a single heartbeat during exercise testing, and 2) compare the cost of EST versus trans-catheter electrophysiology study (EPS) in the risk assessment of WPW patients. Methods: A retrospective review of 50 cases of patients with WPW who underwent EST was conducted including demographics, history of supraventricular tachycardia, associated congenital heart disease, maximum heart rate achieved, and loss of pre-excitation in a single heartbeat. Hospital costs of EST and EPS were compared. Results: Of the 50 patients who underwent EST, 4 (8%), lost pre-excitation in a single heartbeat during EST. No differences were found regarding gender, age at diagnosis or EST, history of supraventricular tachycardia, presence of congenital heart disease or maximal heart rate. A cost comparison, utilizing the cost data: EST ($62.75) and EPS ($5,597) found EST to be a cost-saving approach in WPW patients. With 4 patients losing pre-excitation during EST, the cost saving of EST was $22,388 for this population of WPW patients. Conclusions: A frequency of 8% loss of pre-excitation was found in a pediatric sample that underwent EST. Additionally, EST was shown to be a cost-saving strategy in risk assessment of pediatric WPW patients. PMID:21845141

  2. [Continuing education in ethics: from clinical ethics to institutional ethics].

    Science.gov (United States)

    Brazeau-Lamontagne, Lucie

    2012-01-01

    The mandate of the Ethics Committee of the Conseil de médecins, dentistes et pharmaciens (CMDP) at the Centre hospitalier universitaire de Sherbrooke (CHUS), Sherbrooke, Quebec is three-fold: to guide the clinical decision; to address the institutional ethical function; to create the program for continuing education in ethics (Formation éthique continue or FEC). Might FEC be the means of bridging from individual ethics to institutional ethics at a hospital? To take the FEC perspectives considered appropriate for doctors and consider them for validation or disproving in the context of those of other professionals. Situate the proposed FEC mandate in a reference framework to evaluate (or triangulate) the clinical decision and the institutional ethic. CONVICTION: Sustainable professional development for doctors (DPD) includes ethics; it cannot be ignored. Without constant attention to upgrading one's abilities in professional ethics, these suffer the same fate as other professional aptitudes and competences (for example, techniques and scientific knowledge): decay.

  3. Medically assisted reproduction and ethical challenges

    International Nuclear Information System (INIS)

    Kaeaeriaeinen, Helena; Evers-Kiebooms, Gerry; Coviello, Domenico

    2005-01-01

    Many of the ethical challenges associated with medically assisted reproduction are societal. Should the technique be restricted to only ordinary couples or could it be used also to single females or couples of same sex? Should the future child be entitled to know the identity of the gamete donor? Should there be age limits? Can embryos or gametes be used after the death of the donor? Can surrogate mothers be part of the process? Can preimplantation diagnostics be used to select the future baby's sex? In addition, there are several clearly medical questions that lead to difficult ethical problems. Is it safe to use very premature eggs or sperms? Is the risk for some rare syndromes caused by imprinting errors really increased when using these techniques? Do we transfer genetic infertility to the offspring? Is the risk for multiple pregnancies too high when several embryos are implanted? Does preimplantation diagnosis cause some extra risks for the future child? Should the counselling of these couples include information of all these potential but unlikely risks? The legislation and practices differ in different countries and ethical discussion and professional guidelines are still needed

  4. Pediatric cardiology. Clinical and practical experiences with heart diseases of children, juveniles and young adults

    International Nuclear Information System (INIS)

    Haas, Nikolaus A.

    2011-01-01

    The book on pediatric cardiology covers the following chapters: (I) Fundamentals and diagnostics: pediatric cardiologic anamnesis, electrocardiograms, thorax X-radiography, MRT and CT of the heart, nuclear medical diagnostics, exercise tests, heart catheter examination, electrophysiological tests. (II) Leading symptoms: Cyanosis, cardiac murmur, thorax pain, palpitation, syncopes. (III) Disease pictures: congenital heart defects, acquired heart defects, cardiomyopathies, heart rhythm disturbances, heart insufficiency, arterial hypertension, pulmonary hypertension, other heart involving syndromes. (IV) Therapy: Catheter interventional therapy, post-surgical pediatric cardiac therapy, surgery involving the life-support machine, mechanical cardiovascular support systems, initial treatment of newborns with critical heart defects, heart transplantation, vaccination of children with heart diseases, medicinal therapy.

  5. Ethical Orientations for Understanding Business Ethics.

    Science.gov (United States)

    Lewis, Phillip V.; Speck, Henry E., III

    1990-01-01

    Argues that history provides the necessary framework in which both to discuss and to seek answers to the three necessary and sequential questions about business ethics: (1) What is ethics and what does it mean to be ethical? (2) Why be ethical?; and (3) How can one be ethical? (SG)

  6. Revisiting eco-ethics and econ-ethics

    Directory of Open Access Journals (Sweden)

    Otto Kinne

    2002-11-01

    Full Text Available Modern humanity can survive only if traditional ethics are extended to include eco-ethics and econ-ethics. Success or failure in developing and implementing these new ethical constructs will affect the fate of our species Homo sapiens and that of millions of other forms of life. In the long run failure to accept and apply eco-ethics and econ-ethics would reduce the capacities of Planet Earth to support life.

  7. Assessing and addressing moral distress and ethical climate, part 1.

    Science.gov (United States)

    Sauerland, Jeanie; Marotta, Kathleen; Peinemann, Mary Anne; Berndt, Andrea; Robichaux, Catherine

    2014-01-01

    There is minimal research exploring moral distress and its relationship to ethical climate among nurses working in acute care settings. Objectives of the study were to explore moral distress, moral residue, and perception of ethical climate among registered nurses working in an academic medical center and develop interventions to address study findings. A mixed-methods design was used. Two versions of Corley and colleagues' Moral Distress Scale, adult and pediatric/neonatal, were used in addition to Olson's Hospital Ethical Climate Survey. Participants were invited to respond to 2 open-ended questions. This article reports the results for those nurses working in adult acute and critical care units. The sample (N = 225) was predominantly female (80%); half held a bachelor of science in nursing or higher, were aged 30 to 49 years, and staff nurses (77.3%). The mean item score for moral distress intensity ranged from 3.79 (SD, 2.21) to 2.14 (SD, 2.42) with mean item score frequency ranging from 2.86 (SD, 1.88) to 0.23 (SD, 0.93). The mean score for total Hospital Ethical Climate Survey was 94.39 (SD, 18.3) ranging from 23 to 130. Qualitative comments described bullying, lateral violence, and retribution. Inadequate staffing and perceived incompetent coworkers were the most distressing items. Almost 22% left a previous position because of moral distress and perceived the current climate to be less ethical compared with other participants. Findings may potentially impact nurse retention and recruitment and negatively affect the quality and safety of patient care. Interventions developed focus on the individual nurse, including ethics education and coping skills, intraprofessional/interprofessional approaches, and administrative/policy strategies.

  8. Oral Rehabilitation and Management for Secondary Sjögren's Syndrome in a Child.

    Science.gov (United States)

    Fidalgo, Tatiana Kelly da Silva; Nogueira, Carla; Andrade, Marcia Rejane Thomas Canabarro; Valente, Andrea Graciene Lopez Ramos; Tannure, Patricia Nivoloni

    2016-01-01

    The aim of this paper is to describe a rare case report of a pediatric patient with secondary Sjögren's syndrome (SSS). A 12-year-old female child was referred to the Pediatric Dentistry Clinic with the chief complaint of tooth pain, dry mouth, and tooth sensibility. The patient was submitted to orthodontic treatment prior to syndrome diagnosis. The clinical treatment consisted of the interruption of orthodontic treatment and restoring the oral condition with dental treatment and the use of artificial saliva in an innovative apparatus. Dental therapy involved the control of dental caries, periodontal disease, and opportunistic fungal infections and the use of fluoride-rich solutions. The present clinical case describes clinical and laboratory aspects of SSS in pediatric patients. The management of the oral findings promoted an improvement in the oral health status and quality of life of the child.

  9. Rumination Syndrome and Dental Erosions in Children.

    Science.gov (United States)

    Monagas, Javier; Ritwik, Priyanshi; Kolomensky, Andrew; Acosta, Julio; Kay, Danielle; Clendaniel, Lindsey; Hyman, Paul E

    2017-06-01

    Rumination syndrome is the effortless regurgitation of recently ingested food with subsequent reswallowing or spitting out. Dental erosion (DE) affects 2% to 5% of the population. DE is defined as loss of tooth structure by a chemical process that does not involve bacteria. Our objective was to compare the frequency of DE among children with rumination syndrome with healthy controls. We enrolled 30 patients 4 to 21 years of age diagnosed with rumination syndrome, and 30 age- and sex-matched healthy control subjects. Patients were evaluated by pediatric dentists for presence of DE with Taji et al a validated grading system. Patients with rumination were more likely to have DE (P syndrome, 23 (77%) had DE, compared with 4 (13%) control subjects. DEs are more frequent in patients with rumination syndrome.

  10. Assessment and management of pain in pediatric otolaryngology.

    Science.gov (United States)

    Rodríguez, Maria Claudia; Villamor, Perla; Castillo, Tatiana

    2016-11-01

    Pain is a disease by itself and it's a public health concern of major implication in children, not just because of the emotional component of the child and his family, but also due to the potential morbidity and mortality involving it. A proper assessment of pain it's a challenge in the pediatric population, due to their lack of understanding and verbalization of hurt. Additionally, a satisfactory treatment of pediatric pain can be arduous due to a lack of clinical knowledge, insufficient pediatric research, and the fear to opioid side effects and addiction. The aim of this review is to address the current definitions of pain, its physiological mechanisms and the consequences of its inadequate management, as well as, to guide the clinicians in the assessment and management of pain in the pediatric population at otolaryngology services. Narrative review by selective MeSH search terms: Children, Pediatrics, Otolaryngology, Pain measurement, Pain Management, Analgesics and Analgesia, from databases: MEDLINE/PubMed, Cochrane, ISI, Current Contents, Scielo and LILACS, between January 2000 and May 2016. 129 articles were reviewed according to the requirements of the objectives. Pain measurement is a challenge in children as there are no physical signs that constitute an absolute or specific indicator of pain, and its diagnosis must rely on physiological, behavioral and self-report methods. Regarding treatment, a suitable alternative are the non-pharmacological cognitive/behavioral therapies helped by pharmacological therapies tailored to the severity of pain and the child's age. We provide evidence-based recommendations on pain treatment, including non-opioid analgesics, opioid analgesics and adjuvant medicines to improve the management of pain in children in otolaryngology services. We present a global review about assessment and management of pain in pediatric otolaryngology, which leads to future specific reviews on each topic. Research gaps on pain assessment and

  11. Work-hour restrictions as an ethical dilemma for residents.

    Science.gov (United States)

    Carpenter, Robert O; Austin, Mary T; Tarpley, John L; Griffin, Marie R; Lomis, Kimberly D

    2006-04-01

    We propose that the standardized work-hour limitations have created an ethical dilemma for residents. A survey tool was designed to assess factors that influence the number of hours residents work and report. The program directors of pediatrics, internal medicine, and general surgery at our institution supported their residents' participation. A voluntary, anonymous survey of these residents was performed. One hundred seventy of 265 eligible residents were surveyed. Eighty-one percent of residents surveyed responded. Eighty percent of respondents reported exceeding work-hour restrictions at least once within the past 6 months. The factor of greatest influence measured was concern for patient care (80%). Forty-nine percent of respondents admitted underreporting their work hours. The Accreditation Council for Graduate Medical Education work-hour restrictions have created an ethical dilemma for residents. Our data show that a significant number of residents feel compelled to exceed work-hour regulations and report those hours falsely.

  12. Supporting in grief and burnout of the nursing team from pediatric units in Chilean hospitals.

    Science.gov (United States)

    Vega, Paula Vega; Rodriguez, Rina González; Galdamez, Natalie Santibáñez; Molina, Camila Ferrada; Orellana, Javiera Spicto; Villanueva, Antonia Sateler; Melo, Jose Bustos

    2017-01-01

    Objective To know the levels of Burnout Syndrome and perception of grief support in nursing teams of oncology and pediatric intensive care in public hospitals in Chile. Method A study of descriptive cross-sectional design. The Maslach Burnout Inventory and the Grief Support Health Care Scale were applied to university-level and technical nursing professionals between March and November 2015. An analysis to compare the means was performed with use of the Student's t-test, and the level of significance was set at 5%. Results The study included 153 professionals. Results show 4% of professionals have Burnout Syndrome (BS) and 89% are at risk of having it. Oncology professionals are at higher risk as they present higher levels of emotional exhaustion and lower levels of personal fulfillment. Half of professionals have a high level of perception of grief support, which is greater in the oncology team. Conclusion Professionals working in pediatric oncology units are at increased risk of Burnout Syndrome and grief support may be a mediating factor in this process.

  13. Rituximab for nephrotic syndrome in children.

    Science.gov (United States)

    Iijima, Kazumoto; Sako, Mayumi; Nozu, Kandai

    2017-04-01

    Idiopathic nephrotic syndrome is the most common chronic glomerular disease in children. At least 20 % of children with this syndrome show frequent relapses and/or steroid dependence during or after immunosuppressive therapies, a condition defined as complicated frequently relapsing/steroid-dependent nephrotic syndrome (FRNS/SDNS). Approximately 1-3 % of children with idiopathic nephrotic syndrome are resistant to steroids and all immunosuppressive agents, a condition defined as refractory steroid-resistant nephrotic syndrome (SRNS); these SRNS children have a high risk of end-stage renal failure. Rituximab, a chimeric anti-CD20 monoclonal antibody, has been shown to be effective for patients with complicated FRNS/SDNS and refractory SRNS. This review describes the recent results of rituximab treatment applied to pediatric nephrotic syndrome, as well as those of our recent study, a multicenter, double-blind, randomized, placebo-controlled trial of rituximab for childhood-onset complicated FRNS/SDNS (RCRNS01). The overall efficacy and safety of rituximab for this disease are discussed.

  14. Cardiomyopathies in Noonan syndrome and the other RASopathies

    Science.gov (United States)

    Gelb, Bruce D.; Roberts, Amy E.; Tartaglia, Marco

    2015-01-01

    Noonan syndrome and related disorders (Noonan syndrome with multiple lentigines, Costello syndrome, cardiofaciocutaneous syndrome, Noonan syndrome with loose anagen hair, and other related traits) are autosomal dominant traits. Mutations causing these disorders alter proteins relevant for signaling through RAS. Thus, these traits are now collectively called the RASopathies. While the RASopathies have pleiomorphic features, this review will focus on the hypertrophic cardiomyopathy observed in varying percentages of all of these traits. In addition, inherited abnormalities in one pathway gene, RAF1, cause pediatric-onset dilated cardiomyopathy. The pathogeneses for the RASopathy-associated cardiomyopathies are being elucidated, principally using animal models, leading to genotype-specific insights into how signal transduction is perturbed. Based on those findings, small molecule therapies seem possible for RASopathy-associated cardiomyopathies. PMID:26380542

  15. Cleaved caspase-3 in lung epithelium of children who died with acute respiratory distress syndrome

    NARCIS (Netherlands)

    Bem, Reinout A.; van der Loos, Chris M.; van Woensel, Job B. M.; Bos, Albert P.

    2010-01-01

    OBJECTIVE: To investigate the extent of cleaved caspase-3 immunostaining in lung epithelial cells in children with acute respiratory distress syndrome. DESIGN: Observational study in sixteen children who died with acute respiratory distress syndrome and diffuse alveolar damage. SETTING: Pediatric

  16. Machine Ethics: Creating an Ethical Intelligent Agent

    OpenAIRE

    Anderson, Michael; Anderson, Susan Leigh

    2007-01-01

    The newly emerging field of machine ethics (Anderson and Anderson 2006) is concerned with adding an ethical dimension to machines. Unlike computer ethics -- which has traditionally focused on ethical issues surrounding humans' use of machines -- machine ethics is concerned with ensuring that the behavior of machines toward human users, and perhaps other machines as well, is ethically acceptable. In this article we discuss the importance of machine ethics, the need for machines that represent ...

  17. Prader–Willi syndrome: clinical problems in transition from pediatric to adult care

    Directory of Open Access Journals (Sweden)

    Crinò A

    2016-07-01

    Full Text Available Antonino Crinò,1 Danilo Fintini,1 Sarah Bocchini,1 Chiara Carducci,1 Graziano Grugni,2 1Autoimmune Endocrine Diseases Unit, Bambino Gesù Children’s Hospital, Research Institute, Palidoro, Rome, 2Division of Auxology, Italian Auxological Institute, Research Institute, Piancavallo, Verbania, Italy Abstract: Prader–Willi syndrome (PWS represents the most common form of genetic obesity. Thanks to the advances in medical care and technology, many persons with PWS live longer and survive to adulthood. Currently, because of the many physical and behavioral manifestations, transitional health care is not easy for these patients and is considered a very important issue. Moreover, very few studies have examined these transitional problems in young adults with PWS. In recent years, there has been great interest in improving transition planning and support for young people with PWS reaching adulthood. In this article, we underline the main clinical problems in transition and give some advice to make this period less difficult and easier for adolescents with PWS. Special attention should be paid to obesity, diabetes mellitus, hypertension, osteoporosis, and sleep apnea during the period of transition. In PWS, for an effective transition from childhood to adulthood, a multidisciplinary team is needed, and should maintain the same approach to food, environment, and psychiatric issues. For comprehensive care, it is necessary to involve adult endocrinologists and other medical specialists in conjunction with the pediatric team. Parental involvement is, however, a great help for supervising adolescents with PWS during this particular period. Keywords: Prader–Willi, transition period, intellectual disabilities

  18. Association of Wolfram syndrome with Fallot tetralogy in a girl.

    Science.gov (United States)

    Korkmaz, Hüseyin A; Demir, Korcan; Hazan, Filiz; Yıldız, Melek; Elmas, Özlem N; Özkan, Behzat

    2016-06-01

    Wolfram syndrome (DIDMOAD: diabetes insipidus, diabetes mellitus, optic atrophy and deafness) is a rare neurodegenerative disorder. Mutations of the WFS1 (wolframin) on chromosome 4 are responsible for the clinical manifestations in majority of patients with Wolfram syndrome. Wolfram syndrome is also accompanied by neurologic and psychiatric disorders, urodynamic abnormalities, restricted joint motility, cardiovascular and gastrointestinal autonomic neuropathy, hypergonadotrophic hypogonadism in males and diabetic microvascular disorders. There are very limited data in the literature regarding cardiac malformations associated in children with Wolfram syndrome. A 5-year-old girl with Wolfram syndrome and tetralogy of Fallot is presented herein. Sociedad Argentina de Pediatría.

  19. A newborn with very rare von Voss-Cherstvoy syndrome and literature review

    Directory of Open Access Journals (Sweden)

    Sharma D

    2016-07-01

    Full Text Available Deepak Sharma,1 Basudev Gupta,2 Sweta Shastri,3 Pradeep Sharma4 1Department of Pediatrics, Pt. Bhagwat Dayal Sharma Post Graduate Institute of Medical Sciences, Rohtak, 2Department of Pediatrics, Civil Hospital, Palwal, Haryana, 3Department of Pathology, N.K.P. Salve Medical College, Nagpur, Maharashtra, 4Department of Medicine, Mahatma Gandhi Medical College and Research Institute, Jaipur, Rajasthan, India Introduction: von Voss-Cherstvoy syndrome is a part of a group of syndromes with radial and hematologic abnormalities, and until now approximately ten cases have been reported in the literature. This syndrome is characterized by a triad of radial ray defects, occipital encephalocele, and urogenital abnormalities.Case presentation: We report a neonate from Indian ethnicity who was diagnosed with von Voss-Cherstvoy syndrome. The neonate had radial ray defect, occipital encephalocele, tetralogy of Fallot, and bilateral agenesis of kidney, ureter, and bladder. The neonate was suspected to have von Voss-Cherstvoy syndrome on the basis of clinical features, which was further confirmed by fibroblast analysis showing somatic mosaicism for del(13q.Conclusion: von Voss-Cherstvoy syndrome is a very rare syndrome that can be suspected on the basis of typical clinical features and confirmed by fibroblast analysis showing somatic mosaicism for del(13q. This adds a second case of this chromosome anomaly described in this syndrome. Keywords: von Voss-Cherstvoy syndrome, radial ray defects, occipital encephalocele, urogenital abnormalities, somatic mosaicism for del(13q

  20. Management of moyamoya syndrome in patients with Noonan syndrome.

    Science.gov (United States)

    Gupta, Mihir; Choudhri, Omar A; Feroze, Abdullah H; Do, Huy M; Grant, Gerald A; Steinberg, Gary K

    2016-06-01

    A few isolated reports have described an association between Noonan syndrome and cerebrovascular abnormalities, including moyamoya syndrome. These reports have been limited to pediatric patients presenting with recurrent transient ischemic attacks (TIA) or headaches. Management has primarily been pharmacologic, with only one prior report of surgical revascularization to our knowledge. We report four cases of Noonan syndrome patients presenting with headaches and/or sensorimotor strokes in childhood that caused unilateral sensorimotor impairment. Cerebral angiography and MRI revealed bilateral moyamoya syndrome. All patients underwent successful bilateral extracranial-to-intracranial revascularization. The first patient was a 10-year-old girl who presented following a hemorrhagic stroke and recovered well after indirect bypass. The second patient was an adult with a history of childhood stroke whose symptoms progressed in adulthood. She underwent a direct bypass and improved, but continued to experience TIA at her 4 year follow-up. The third patient was a 7-year-old girl with headaches and a new onset TIA who failed pharmacological therapy and subsequently underwent bilateral indirect bypass. The fourth patient was a 24-year-old woman with worsening headaches and an occluded left middle cerebral artery from unilateral moyamoya syndrome. A left sided direct bypass was completed given delayed MRI perfusion with poor augmentation. To our knowledge these are the first reported surgical cases of combined Noonan and moyamoya syndrome. These cases highlight the need to recognize moyamoya syndrome in patients with Noonan syndrome. Early surgical revascularization should be pursued in order to prevent symptom progression. Copyright © 2015 Elsevier Ltd. All rights reserved.

  1. KUDESAN EFFICACY IN ADOLESCENTS WITH METABOLIC SYNDROME

    Directory of Open Access Journals (Sweden)

    M.B. Kolesnikova

    2011-01-01

    Full Text Available Metabolic abnormalities in metabolic syndrome affect the functioning of practically all organs and systems, and most seriously — cardio-vascular system. Cardio-vascular abnormalities in metabolic syndrome manifest as arterial hypertension, Riley-Day syndrome and endothelial dysfunction that can lead to decrease of adaptive and reserve capabilities. Co-enzyme Q10 possesses cardioprotective,  stress-protective and anti-ischaemic activity. Clinical study performed on 40 children aged 10 to 17 years with constitutive obesity, complicated metabolic syndrome, has proven validity of co-enzyme Q10 treatment in patients with metabolic syndrome. The use of co-enzyme Q10 15 mg/day during 30 days has lead to improvement of psycho-emotional condition, decrease in anxiety complaints, sleep improvement, decrease in asthenic syndrome symptoms, improvement in electrophysiological heart indices Key words: metabolic syndrome, co-enzyme Q10. (Voprosy sovremennoi pediatrii — Current Pediatrics. — 2011; 10 (5: 102–106.

  2. Gorlin Syndrome with Bilateral Polydactyly: A Rare Case Report

    Science.gov (United States)

    Acharya, Sonu; Panda, Swagatika; Sahoo, Sujit Ranjan; Ray, Prayas

    2013-01-01

    ABSTRACT Gorlin's syndrome is a rare disorder transmitted as an autosomal dominant trait. It is characterized by multiple disorders involving multiple systems. We present a case of 11-year-old male child presenting with multiple odontogenic keratocyst to the dental clinic. Retrograde diagnosis of Gorlin-Goltz syndrome was made after clinical and radiological investigation. How to cite this article: Acharya S, Panda S, Dhull KS, Sahoo SR, Ray P. Gorlin Syndrome with Bilateral Polydactyly: A Rare Case Report. Int J Clin Pediatr Dent 2013;6(3):208-212. PMID:25206225

  3. Paraneoplastic Cushing Syndrome Due To Wilm's Tumor.

    Science.gov (United States)

    Faizan, Mahwish; Manzoor, Jaida; Saleem, Muhammad; Anwar, Saadia; Mehmood, Qaiser; Hameed, Ambreen; Ali, Agha Shabbir

    2017-05-01

    Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity. Few case reports are available in the literature exploring the option of preoperative chemotherapy as well as upfront nephrectomy. We report a rare case of paraneoplastic Cushing syndrome due to a Wilm's tumor. Based on gradual decrease of postoperative weight, blood pressure, serum adrenocorticotropic hormone, and plasma cortisol levels, along with histological confirmation of Wilm's tumor, paraneoplastic Cushing syndrome due to Wilm's tumor was confirmed.

  4. Paraneoplastic cushing syndrome due to wilm's tumor

    International Nuclear Information System (INIS)

    Faizan, M.; Anwar, S.; Hameed, A.; Manzoor, J.; Saleem, M.; Mehmood, Q.; Ali, A. S.

    2017-01-01

    Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity. Few case reports are available in the literature exploring the option of preoperative chemotherapy as well as upfront nephrectomy. We report a rare case of paraneoplastic Cushing syndrome due to a Wilm's tumor. Based on gradual decrease of postoperative weight, blood pressure, serum adrenocorticotropic hormone, and plasma cortisol levels, alongwith histological confirmation of Wilm's tumor, paraneoplastic Cushing syndrome due to Wilm's tumor was confirmed. (author)

  5. Abdominal and lower back pain in pediatric idiopathic stabbing headache.

    Science.gov (United States)

    Kakisaka, Yosuke; Ohara, Tomoichiro; Hino-Fukuyo, Naomi; Uematsu, Mitsugu; Kure, Shigeo

    2014-01-01

    Idiopathic stabbing headache (ISH) is a primary headache syndrome characterized by transient, sharp, stabbing pains located in the first division of the trigeminal nerve. Reports of pediatric ISH are rare, and extracephalic pain in pediatric ISH is extremely rare. Here we report the case of a 7-year-old male patient suffering from frequent, short, stabbing headache, which was occasionally associated with abdominal and lower back pain. Various investigations were normal. He was diagnosed with ISH, and valproic acid was administered to relieve his headache and accompanying symptoms. Our case demonstrates that abdominal and lower back pain may occur in pediatric ISH. This case may provide new evidence linking ISH and migraine by showing that extracephalic symptoms accompanying ISH are similar to those of migraine. We hypothesize that the mechanism underlying the headache and abdominal and lower back pain associated with ISH may be similar to that of a migraine headache. Accumulating additional cases by asking specific questions regarding the presence of the unusual symptoms presented in our case may help to establish a detailed clinical profile of these unfamiliar and peculiar symptoms in the pediatric ISH population.

  6. Business ethics in ethics committees?

    Science.gov (United States)

    Boyle, P

    1990-01-01

    The "Ethics committees" column in this issue of the Hastings Center Report features an introduction by Cynthia B. Cohen and four brief commentaries on the roles hospital ethics committees may play in the making of institutional and public health care policy in the 1990s. The pros and cons of a broader, more public role for ethics committees in reconciling the business and patient care aspects of health care delivery are debated by Cohen in "Ethics committees as corporate and public policy advocates," and by Philip Boyle in this article. Boyle is an associate for ethical studies at The Hastings Center.

  7. Engineer Ethics

    International Nuclear Information System (INIS)

    Lee, Dae Sik; Kim, Yeong Pil; Kim, Yeong Jin

    2003-03-01

    This book tells of engineer ethics such as basic understanding of engineer ethics with history of engineering as a occupation, definition of engineering and specialized job and engineering, engineer ethics as professional ethics, general principles of ethics and its limitation, ethical theory and application, technique to solve the ethical problems, responsibility, safety and danger, information engineer ethics, biotechnological ethics like artificial insemination, life reproduction, gene therapy and environmental ethics.

  8. Perceived barriers to pediatrician and family practitioner participation in pediatric clinical trials: Findings from the Clinical Trials Transformation Initiative.

    Science.gov (United States)

    Greenberg, Rachel G; Corneli, Amy; Bradley, John; Farley, John; Jafri, Hasan S; Lin, Li; Nambiar, Sumathi; Noel, Gary J; Wheeler, Chris; Tiernan, Rosemary; Smith, P Brian; Roberts, Jamie; Benjamin, Daniel K

    2018-03-01

    Despite legislation to stimulate pediatric drug development through clinical trials, enrolling children in trials continues to be challenging. Non-investigator (those who have never served as a clinical trial investigator) providers are essential to recruitment of pediatric patients, but little is known regarding the specific barriers that limit pediatric providers from participating in and referring their patients to clinical trials. We conducted an online survey of pediatric providers from a wide variety of practice types across the United States to evaluate their attitudes and awareness of pediatric clinical trials. Using a 4-point Likert scale, providers described their perception of potential barriers to their practice serving as a site for pediatric clinical trials. Of the 136 providers surveyed, 52/136 (38%) had previously referred a pediatric patient to a trial, and only 17/136 (12%) had ever been an investigator for a pediatric trial. Lack of awareness of existing pediatric trials was a major barrier to patient referral by providers, in addition to consideration of trial risks, distance to the site, and time needed to discuss trial participation with parents. Overall, providers perceived greater challenges related to parental concerns and parent or child logistical barriers than study implementation and ethics or regulatory barriers as barriers to their practice serving as a trial site. Providers who had previously been an investigator for a pediatric trial were less likely to be concerned with potential barriers than non-investigators. Understanding the barriers that limit pediatric providers from collaboration or inhibit their participation is key to designing effective interventions to optimize pediatric trial participation.

  9. A case of splenomegaly in CBL syndrome

    NARCIS (Netherlands)

    Coe, Rachel R.; McKinnon, Margaret L.; Tarailo-Graovac, Maja; Ross, Colin J.; Wasserman, Wyeth W.; Friedman, Jan M.; Rogers, Paul C.; van Karnebeek, Clara D. M.

    2017-01-01

    We present a child with unexplained splenomegaly to highlight this feature as a presenting sign of the RASopathy CBL syndrome and to draw attention to the power and utility of next generation genomic sequencing for providing rapid diagnosis and critical information to guide care in the pediatric

  10. Improving Ethical Attitudes or Simply Teaching Ethical Codes? The Reality of Accounting Ethics Education

    Science.gov (United States)

    Cameron, Robyn Ann; O'Leary, Conor

    2015-01-01

    Ethical instruction is critical in accounting education. However, does accounting ethics teaching actually instil core ethical values or simply catalogue how students should act when confronted with typical accounting ethical dilemmas? This study extends current literature by distinguishing between moral/ethical and legal/ethical matters and then…

  11. Hypocretin-1 Deficiency in a Girl With ROHHAD Syndrome

    NARCIS (Netherlands)

    Dhondt, K.; Verloo, P.; Verhelst, H.; Coster, R. van; Overeem, S.

    2013-01-01

    Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) is a rare and complex pediatric syndrome, essentially caused by dysfunction of 3 vital systems regulating endocrine, respiratory, and autonomic nervous system functioning. The clinical spectrum

  12. The duty of the physician to care for the family in pediatric palliative care: context, communication, and caring.

    Science.gov (United States)

    Jones, Barbara L; Contro, Nancy; Koch, Kendra D

    2014-02-01

    Pediatric palliative care physicians have an ethical duty to care for the families of children with life-threatening conditions through their illness and bereavement. This duty is predicated on 2 important factors: (1) best interest of the child and (2) nonabandonment. Children exist in the context of a family and therefore excellent care for the child must include attention to the needs of the family, including siblings. The principle of nonabandonment is an important one in pediatric palliative care, as many families report being well cared for during their child's treatment, but feel as if the physicians and team members suddenly disappear after the death of the child. Family-centered care requires frequent, kind, and accurate communication with parents that leads to shared decision-making during treatment, care of parents and siblings during end-of-life, and assistance to the family in bereavement after death. Despite the challenges to this comprehensive care, physicians can support and be supported by their transdisciplinary palliative care team members in providing compassionate, ethical, and holistic care to the entire family when a child is ill.

  13. Applications of Genomic Sequencing in Pediatric CNS Tumors.

    Science.gov (United States)

    Bavle, Abhishek A; Lin, Frank Y; Parsons, D Williams

    2016-05-01

    Recent advances in genome-scale sequencing methods have resulted in a significant increase in our understanding of the biology of human cancers. When applied to pediatric central nervous system (CNS) tumors, these remarkable technological breakthroughs have facilitated the molecular characterization of multiple tumor types, provided new insights into the genetic basis of these cancers, and prompted innovative strategies that are changing the management paradigm in pediatric neuro-oncology. Genomic tests have begun to affect medical decision making in a number of ways, from delineating histopathologically similar tumor types into distinct molecular subgroups that correlate with clinical characteristics, to guiding the addition of novel therapeutic agents for patients with high-risk or poor-prognosis tumors, or alternatively, reducing treatment intensity for those with a favorable prognosis. Genomic sequencing has also had a significant impact on translational research strategies in pediatric CNS tumors, resulting in wide-ranging applications that have the potential to direct the rational preclinical screening of novel therapeutic agents, shed light on tumor heterogeneity and evolution, and highlight differences (or similarities) between pediatric and adult CNS tumors. Finally, in addition to allowing the identification of somatic (tumor-specific) mutations, the analysis of patient-matched constitutional (germline) DNA has facilitated the detection of pathogenic germline alterations in cancer genes in patients with CNS tumors, with critical implications for genetic counseling and tumor surveillance strategies for children with familial predisposition syndromes. As our understanding of the molecular landscape of pediatric CNS tumors continues to advance, innovative applications of genomic sequencing hold significant promise for further improving the care of children with these cancers.

  14. A practical approach to risk-benefit estimation in pediatric drug research.

    Science.gov (United States)

    Koren, Gideon

    2015-02-01

    One of the most difficult challenges in pediatric drug research is in exposing children to risk, often without a balanced chance of benefits. While the concept of risk is similar in adult research, the adult patient can decide for himself/herself on an acceptable level of risk, whereas children have to accept the decisions of their guardians. This paper attempts to put the complexities of estimating risk in pediatric drug research into their practical perspective, and to familiarize the reader with the way such processes are conducted in different parts of the world. Although there are regional differences, all authorities typically quantify risks of pediatric research in general, and drug research in particular, in three levels: those experienced in day-to-day life; risks slightly above this 'baseline' risk; and risks substantially above 'baseline risk'. Proportionally, the diligence of the ethics process depends on these levels, as well as on the potential benefits (or lack of) to the child involved in the research. Importantly, risk is context dependent, and a particular intervention may be effective or safe in one setting but not in another, based on local experience, staffing levels, and similar variabilities.

  15. Ethics rounds: An appreciated form of ethics support.

    Science.gov (United States)

    Silén, Marit; Ramklint, Mia; Hansson, Mats G; Haglund, Kristina

    2016-03-01

    Ethics rounds are one way to support healthcare personnel in handling ethically difficult situations. A previous study in the present project showed that ethics rounds did not result in significant changes in perceptions of how ethical issues were handled, that is, in the ethical climate. However, there was anecdotal evidence that the ethics rounds were viewed as a positive experience and that they stimulated ethical reflection. The aim of this study was to gain a deeper understanding of how the ethics rounds were experienced and why the intervention in the form of ethics rounds did not succeed in improving the ethical climate for the staff. An exploratory and descriptive design with a qualitative approach was adopted, using individual interviews. A total of 11 healthcare personnel, working in two different psychiatry outpatient clinics and with experience of participating in ethics rounds, were interviewed. The study was based on informed consent and was approved by one of the Swedish Regional Ethical Review Boards. The participants were generally positive about the ethics rounds. They had experienced changes by participating in the ethics rounds in the form of being able to see things from different perspectives as well as by gaining insight into ethical issues. However, these changes had not affected daily work. A crucial question is whether or not increased reflection ability among the participants is a good enough outcome of ethics rounds and whether this result could have been measured in patient-related outcomes. Ethics rounds might foster cooperation among the staff and this, in turn, could influence patient care. By listening to others during ethics rounds, a person can learn to see things from a new angle. Participation in ethics rounds can also lead to better insight concerning ethical issues. © The Author(s) 2014.

  16. Collaborative Efforts Driving Progress in Pediatric Acute Myeloid Leukemia

    Science.gov (United States)

    Zwaan, C. Michel; Kolb, Edward A.; Reinhardt, Dirk; Abrahamsson, Jonas; Adachi, Souichi; Aplenc, Richard; De Bont, Eveline S.J.M.; De Moerloose, Barbara; Dworzak, Michael; Gibson, Brenda E.S.; Hasle, Henrik; Leverger, Guy; Locatelli, Franco; Ragu, Christine; Ribeiro, Raul C.; Rizzari, Carmelo; Rubnitz, Jeffrey E.; Smith, Owen P.; Sung, Lillian; Tomizawa, Daisuke; van den Heuvel-Eibrink, Marry M.; Creutzig, Ursula; Kaspers, Gertjan J.L.

    2015-01-01

    Diagnosis, treatment, response monitoring, and outcome of pediatric acute myeloid leukemia (AML) have made enormous progress during the past decades. Because AML is a rare type of childhood cancer, with an incidence of approximately seven occurrences per 1 million children annually, national and international collaborative efforts have evolved. This overview describes these efforts and includes a summary of the history and contributions of each of the main collaborative pediatric AML groups worldwide. The focus is on translational and clinical research, which includes past, current, and future clinical trials. Separate sections concern acute promyelocytic leukemia, myeloid leukemia of Down syndrome, and relapsed AML. A plethora of novel antileukemic agents that have emerged, including new classes of drugs, are summarized as well. Finally, an important aspect of the treatment of pediatric AML—supportive care—and late effects are discussed. The future is bright, with a wide range of emerging innovative therapies and with more and more international collaboration that ultimately aim to cure all children with AML, with fewer adverse effects and without late effects. PMID:26304895

  17. Refeeding syndrome in a small-for-dates micro-preemie receiving early parenteral nutrition.

    Science.gov (United States)

    Mizumoto, Hiroshi; Mikami, Masamitsu; Oda, Hirotsugu; Hata, Daisuke

    2012-10-01

    This report describes a small-for-date extremely low birth weight infant who manifested bradycardic events, respiratory failure, and hemolytic jaundice during her first week of life. These complications were attributed to severe hypophosphatemia and hypokalemia. Inadequate supply and refeeding syndrome triggered by early aggressive parenteral nutrition were responsible for electrolyte abnormalities. © 2012 The Authors. Pediatrics International © 2012 Japan Pediatric Society.

  18. Non-invasive prenatal testing for fetal chromosome abnormalities: review of clinical and ethical issues

    Directory of Open Access Journals (Sweden)

    Gekas J

    2016-02-01

    Full Text Available Jean Gekas,1,2 Sylvie Langlois,3 Vardit Ravitsky,4 François Audibert,5 David Gradus van den Berg,6 Hazar Haidar,4 François Rousseau2,7 1Prenatal Diagnosis Unit, Department of Medical Genetics and Pediatrics, Faculty of Medicine, Université Laval, Québec City, QC, Canada; 2Department of Medical Biology, CHU de Québec, Québec City, QC, Canada; 3Department of Medical Genetics, University of British Columbia, Vancouver, BC, Canada; 4Bioethics Program, Department of Social and Preventive Medicine, School of Public Health, University of Montreal, Montreal, QC, Canada; 5Department of Obstetrics and Gynecology, Hospital Sainte-Justine, Montreal, QC, Canada; 6Department of Social and Preventive Medicine, Faculty of Medicine, Université Laval, Québec City, QC, Canada; 7Department of Molecular Biology, Medical Biochemistry and Pathology, Faculty of Medicine, Université Laval, Québec City, QC, Canada Abstract: Genomics-based non-invasive prenatal screening using cell-free DNA (cfDNA screening was proposed to reduce the number of invasive procedures in current prenatal diagnosis for fetal aneuploidies. We review here the clinical and ethical issues of cfDNA screening. To date, it is not clear how cfDNA screening is going to impact the performances of clinical prenatal diagnosis and how it could be incorporated in real life. The direct marketing to users may have facilitated the early introduction of cfDNA screening into clinical practice despite limited evidence-based independent research data supporting this rapid shift. There is a need to address the most important ethical, legal, and social issues before its implementation in a mass setting. Its introduction might worsen current tendencies to neglect the reproductive autonomy of pregnant women. Keywords: prenatal diagnosis, Down syndrome, non-invasive prenatal testing, cell-free fetal DNA, informed consent, reproductive autonomy

  19. Business Ethics and Military Ethics : A Study in Comparative Applied Ethics

    OpenAIRE

    Shaw, William H.

    2012-01-01

    In the past three decades, philosophers have delved into applied ethics, pursuing a surprisingly wide range of practically oriented normative questions, and a number of fields of applied ethical research and teaching are flourishing. There have, however, been few comparative studies of different fields in applied ethics, but such studies can, I believe, teach us something. Accordingly, this essay compares and contrasts business ethics and military ethics as distinct disciplinar...

  20. Amelogenesis Imperfecta with Distal Renal Tubular Acidosis: A Novel Syndrome?

    Science.gov (United States)

    Misgar, R A; Hassan, Z; Wani, A I; Bashir, M I

    2017-01-01

    Amelogenesis imperfecta (AI) is a heterogeneous group of inherited dental enamel defects. It has rarely been reported in association with multiorgan syndromes and metabolic disorders. The metabolic disorders that have been reported in association with AI include hypocalciuria, impaired urinary concentrating ability, and Bartter-like syndrome. In literature, only three cases of AI and distal renal tubular acidosis (dRTA) have been described: two cases in adults and a solitary case in the pediatric age group. Here, we report a child with AI presenting with dRTA; to the best of our knowledge, our reported case is the only second such case in pediatric age group. Our case highlights the importance of recognizing the possibility of renal abnormalities in patients with AI as it will affect the long-term prognosis.

  1. Amelogenesis imperfecta with distal renal tubular acidosis: A novel syndrome?

    Directory of Open Access Journals (Sweden)

    R A Misgar

    2017-01-01

    Full Text Available Amelogenesis imperfecta (AI is a heterogeneous group of inherited dental enamel defects. It has rarely been reported in association with multiorgan syndromes and metabolic disorders. The metabolic disorders that have been reported in association with AI include hypocalciuria, impaired urinary concentrating ability, and Bartter-like syndrome. In literature, only three cases of AI and distal renal tubular acidosis (dRTA have been described: two cases in adults and a solitary case in the pediatric age group. Here, we report a child with AI presenting with dRTA; to the best of our knowledge, our reported case is the only second such case in pediatric age group. Our case highlights the importance of recognizing the possibility of renal abnormalities in patients with AI as it will affect the long-term prognosis.

  2. Strategic Planning for Research in Pediatric Critical Care.

    Science.gov (United States)

    Tamburro, Robert F; Jenkins, Tammara L; Kochanek, Patrick M

    2016-11-01

    To summarize the scientific priorities and potential future research directions for pediatric critical care research discussed by a panel of experts at the inaugural Strategic Planning Conference of the Pediatric Trauma and Critical Illness Branch of the Eunice Kennedy Shriver National Institute of Child Health and Human Development. Expert opinion expressed during the Strategic Planning Conference. Not applicable. Chaired by an experienced expert from the field, issues relevant to the conduct of pediatric critical care research were discussed and debated by the invited participants. Common themes and suggested priorities were identified and coalesced. Of the many pathophysiologic conditions discussed, the multiple organ dysfunction syndrome emerged as a topic in need of more study that is most relevant to the field. Additionally, the experts offered that the interrelationship and impact of critical illness on child development and family functioning are important research priorities. Consequently, long-term outcomes research was encouraged. The expert group also suggested that multidisciplinary conferences are needed to help identify key knowledge gaps to advance and direct research in the field. The Pediatric Critical Care and Trauma Scientist Development National K12 Program and the Collaborative Pediatric Critical Care Research Network were recognized as successful and important programs supported by the branch. The development of core data resources including biorepositories with robust phenotypic data using common data elements was also suggested to foster data sharing among investigators and to enhance disease diagnosis and discovery. Multicenter clinical trials and innovative study designs to address understudied and poorly understood conditions were considered important for field advancement. Finally, the growth of the pediatric critical care research workforce was offered as a priority that could be spawned in many ways including by expanded

  3. Combining multicriteria decision analysis, ethics and health technology assessment: applying the EVIDEM decisionmaking framework to growth hormone for Turner syndrome patients

    Directory of Open Access Journals (Sweden)

    Grégoire Jean-Pierre

    2010-04-01

    Full Text Available Abstract Objectives To test and further develop a healthcare policy and clinical decision support framework using growth hormone (GH for Turner syndrome (TS as a complex case study. Methods The EVIDEM framework was further developed to complement the multicriteria decision analysis (MCDA Value Matrix, that includes 15 quantifiable components of decision clustered in four domains (quality of evidence, disease, intervention and economics, with a qualitative tool including six ethical and health system-related components of decision. An extensive review of the literature was performed to develop a health technology assessment report (HTA tailored to each component of decision, and content was validated by experts. A panel of representative stakeholders then estimated the MCDA value of GH for TS in Canada by assigning weights and scores to each MCDA component of decision and then considered the impact of non-quantifiable components of decision. Results Applying the framework revealed significant data gaps and the importance of aligning research questions with data needs to truly inform decision. Panelists estimated the value of GH for TS at 41% of maximum value on the MCDA scale, with good agreement at the individual level (retest value 40%; ICC: 0.687 and large variation across panelists. Main contributors to this panel specific value were "Improvement of efficacy", "Disease severity" and "Quality of evidence". Ethical considerations on utility, efficiency and fairness as well as potential misuse of GH had mixed effects on the perceived value of the treatment. Conclusions This framework is proposed as a pragmatic step beyond the current cost-effectiveness model, combining HTA, MCDA, values and ethics. It supports systematic consideration of all components of decision and available evidence for greater transparency. Further testing and validation is needed to build up MCDA approaches combined with pragmatic HTA in healthcare decisionmaking.

  4. AKI in Children Hospitalized with Nephrotic Syndrome.

    Science.gov (United States)

    Rheault, Michelle N; Zhang, Lei; Selewski, David T; Kallash, Mahmoud; Tran, Cheryl L; Seamon, Meredith; Katsoufis, Chryso; Ashoor, Isa; Hernandez, Joel; Supe-Markovina, Katarina; D'Alessandri-Silva, Cynthia; DeJesus-Gonzalez, Nilka; Vasylyeva, Tetyana L; Formeck, Cassandra; Woll, Christopher; Gbadegesin, Rasheed; Geier, Pavel; Devarajan, Prasad; Carpenter, Shannon L; Kerlin, Bryce A; Smoyer, William E

    2015-12-07

    Children with nephrotic syndrome can develop life-threatening complications, including infection and thrombosis. While AKI is associated with adverse outcomes in hospitalized children, little is known about the epidemiology of AKI in children with nephrotic syndrome. The main objectives of this study were to determine the incidence, epidemiology, and hospital outcomes associated with AKI in a modern cohort of children hospitalized with nephrotic syndrome. Records of children with nephrotic syndrome admitted to 17 pediatric nephrology centers across North America from 2010 to 2012 were reviewed. AKI was classified using the pediatric RIFLE definition. AKI occurred in 58.6% of 336 children and 50.9% of 615 hospitalizations (27.3% in stage R, 17.2% in stage I, and 6.3% in stage F). After adjustment for race, sex, age at admission, and clinical diagnosis, infection (odds ratio, 2.24; 95% confidence interval, 1.37 to 3.65; P=0.001), nephrotoxic medication exposure (odds ratio, 1.35; 95% confidence interval, 1.11 to 1.64; P=0.002), days of nephrotoxic medication exposure (odds ratio, 1.10; 95% confidence interval, 1.05 to 1.15; Pchildren with nephrotic syndrome. Nephrotoxic medication exposure was common in this population, and each additional nephrotoxic medication received during a hospitalization was associated with 38% higher risk of AKI. AKI was associated with longer hospital stay after adjustment for race, sex, age at admission, clinical diagnosis, and infection (difference, 0.45 [log]days; 95% confidence interval, 0.36 to 0.53 [log]days; Pchildren hospitalized with nephrotic syndrome and should be deemed the third major complication of nephrotic syndrome in children in addition to infection and venous thromboembolism. Risk factors for AKI include steroid-resistant nephrotic syndrome, infection, and nephrotoxic medication exposure. Children with AKI have longer hospital lengths of stay and increased need for intensive care unit admission. Copyright © 2015 by the

  5. Mechanisms Linking Ethical Leadership to Ethical Sales Behavior.

    Science.gov (United States)

    Wu, Yu-Chi

    2017-06-01

    This study investigated the relationship between ethical leadership and ethical sales behavior. A total of 248 matched surveys with participant responses from insurance agents and their customers were collected. The insurance agents were asked to rate the ethical leadership of their leaders, the ethical climate in their organization, and their individual moral identity. Customers were asked to rate the perceived ethical sales behavior of the insurance agents. This empirical study utilized moderated mediation techniques to analyze the data. Results indicated that ethical climate mediated the relationship between ethical leadership and ethical sales behavior when moral identity was high, however, did not when moral identity was low. The research framework including contextual effects (i.e., ethical climate) and individual differences in moral judgment (i.e., moral identity) can provide a comprehensive picture of how ethical leadership influences ethical sales behavior. Theoretical and practical implications of these findings are also discussed.

  6. A four-part working bibliography of neuroethics: part 3 - "second tradition neuroethics" - ethical issues in neuroscience.

    Science.gov (United States)

    Martin, Amanda; Becker, Kira; Darragh, Martina; Giordano, James

    2016-09-19

    Neuroethics describes several interdisciplinary topics exploring the application and implications of engaging neuroscience in societal contexts. To explore this topic, we present Part 3 of a four-part bibliography of neuroethics' literature focusing on the "ethics of neuroscience." To complete a systematic survey of the neuroethics literature, 19 databases and 4 individual open-access journals were employed. Searches were conducted using the indexing language of the U.S. National Library of Medicine (NLM). A Python code was used to eliminate duplications in the final bibliography. This bibliography consists of 1137 papers, 56 books, and 134 book chapters published from 2002 through 2014, covering ethical issues in neuroimaging, neurogenetics, neurobiomarkers, neuro-psychopharmacology, brain stimulation, neural stem cells, neural tissue transplants, pediatric-specific issues, dual-use, and general neuroscience research issues. These works contain explanations of recent research regarding neurotechnology, while exploring ethical issues in future discoveries and use.

  7. Hunter′s syndrome: A case report

    Directory of Open Access Journals (Sweden)

    N S Savitha

    2015-01-01

    Full Text Available Hunter′s syndrome or mucopolysaccharidosis (MPS type II is an X-linked recessive mucopolysaccharide disorder caused by a defect in the metabolism of glycosaminoglycans (GAGs characterized by involvement of nervous, cardiovascular, respiratory, and mucoskeletal systems along with numerous oral manifestations. This is a case report of a 13-year-old boy referred to the Department of Pediatric Dentistry with a chief complaint of irregularly placed teeth from a general physician. Here we highlight the pivotal role of pediatric dentists in diagnosis and treatment planning for patients diagnosed with such systemic conditions and the provision of advanced dental care in the management of the same.

  8. Virtual Pediatric Hospital

    Science.gov (United States)

    ... Thoracopaedia - An Imaging Encyclopedia of Pediatric Thoracic Disease Virtual Pediatric Hospital is the Apprentice's Assistant™ Last revised ... pediatric resources: GeneralPediatrics.com | PediatricEducation.org | SearchingPediatrics.com Virtual Pediatric Hospital is curated by Donna M. D' ...

  9. Oral Rehabilitation and Management for Secondary Sjögren’s Syndrome in a Child

    Directory of Open Access Journals (Sweden)

    Tatiana Kelly da Silva Fidalgo

    2016-01-01

    Full Text Available The aim of this paper is to describe a rare case report of a pediatric patient with secondary Sjögren’s syndrome (SSS. A 12-year-old female child was referred to the Pediatric Dentistry Clinic with the chief complaint of tooth pain, dry mouth, and tooth sensibility. The patient was submitted to orthodontic treatment prior to syndrome diagnosis. The clinical treatment consisted of the interruption of orthodontic treatment and restoring the oral condition with dental treatment and the use of artificial saliva in an innovative apparatus. Dental therapy involved the control of dental caries, periodontal disease, and opportunistic fungal infections and the use of fluoride-rich solutions. The present clinical case describes clinical and laboratory aspects of SSS in pediatric patients. The management of the oral findings promoted an improvement in the oral health status and quality of life of the child.

  10. The evidence-based approach to adult-onset idiopathic nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Pietro A. Canetta

    2015-09-01

    Full Text Available Adult-onset nephrotic syndrome differs from its pediatric counterpart in several important ways. Most importantly, nephrotic syndrome in adults is more etiologically heterogeneous compared to children, and thus treatment approaches rely heavily on the histologic diagnosis provided by renal biopsy. The evidence-based approach to treatment of adult nephrotic syndrome has been critically examined by the Kidney Disease Improving Global Outcomes (KDIGO guidelines in glomerulonephritis, published in 2012. Here, we examine the strengths and limits of those guidelines and review recent work that expands the evidence-based approach.

  11. Pediatric allergy and immunology in Brazil.

    Science.gov (United States)

    Rosario-Filho, Nelson A; Jacob, Cristina M; Sole, Dirceu; Condino-Neto, Antonio; Arruda, Luisa K; Costa-Carvalho, Beatriz; Cocco, Renata R; Camelo-Nunes, Inês; Chong-Neto, Herberto J; Wandalsen, Gustavo F; Castro, Ana P M; Yang, Ariana C; Pastorino, Antonio C; Sarinho, Emanuel S

    2013-06-01

    The subspecialty of pediatric allergy and immunology in Brazil is in its early years and progressing steadily. This review highlights the research developed in the past years aiming to show the characteristics of allergic and immunologic diseases in this vast country. Epidemiologic studies demonstrated the high prevalence of asthma in infants, children, and adolescents. Mortality rates and average annual variation of asthma hospitalization have reduced in all pediatric age groups. Indoor aeroallergen exposure is excessively high and contributes to the high rates of allergy sensitization. Prevalence of food allergy has increased to epidemic levels. Foods (35%), insect stings (30%), and drugs (23%) are the main etiological agents of anaphylaxis in children and adolescents. Molecular diagnosis of primary immunodeficiencies (PID) showed a high incidence of fungal infections including paracoccidioidomycosis in X-linked hyper-IgM syndrome, and the occurrence of BCG adverse reactions or other mycobacterial infections in patients with chronic granulomatous disease. Education in pediatric allergy and immunology is deficient for medical students, but residency programs are effective in training internists and pediatricians for the practice of allergy. The field of PID requires further training. Last, this review is a tribute to Prof. Dr. Charles Naspitz, one of the pioneers of our specialty in Brazil. © 2013 John Wiley & Sons A/S. Published by Blackwell Publishing Ltd.

  12. Pediatric Specialists

    Science.gov (United States)

    ... Healthy Children > Family Life > Medical Home > Pediatric Specialists Pediatric Specialists Article Body ​Your pediatrician may refer your child to a pediatric specialist for further evaluation and treatment. Pediatric specialists ...

  13. [Anesthesia for a pediatric patient with Coffin-Siris syndrome].

    Science.gov (United States)

    Shirakami, Gotaro; Tazuke-Nishimura, Misako; Hirakata, Hideo; Fukuda, Kazuhiko

    2005-01-01

    A 3-year-old boy with Coffin-Siris syndrome was scheduled to undergo diagnostic laparoscopy, inguinal herniorrhaphy and orchiopexy at an ambulatory setting and same-day admission. Following anesthesia induction with inhalational sevoflurane, upper airway obstruction and hypoxemia developed. Hypoxemia was resolved immediately by manual positive pressure ventilation, although the stomach became bulged. Operation was finished uneventfully. However, he had massive bronchial secretion during anesthesia. He was admitted as planned and discharged on postoperative day 2. Since patients with Coffin-Siris syndrome have potential airway and pulmonary dysfunctions, careful perianesthesia airway and respiratory managements are essential.

  14. Dexamethasone, Intravenous Immunoglobulin, and Rituximab Combination Immunotherapy for Pediatric Opsoclonus-Myoclonus Syndrome.

    Science.gov (United States)

    Pranzatelli, Michael R; Tate, Elizabeth D

    2017-08-01

    Although pulse-dose dexamethasone is increasingly favored for treating pediatric opsoclonus-myoclonus syndrome (OMS), and multimodal immunotherapy is associated with improved clinical response, there have been no neuroimmunologic studies of dexamethasone-based multimodal disease-modifying therapy. In this observational retrospective study, 19 children with OMS (with or without associated neuroblastoma) underwent multibiomarker evaluation for neuroinflammation. Nine children of varying OMS severity, duration, and treatment status were treated empirically with pulse dexamethasone, intravenous immunoglobulin (IVIg), and rituximab combination immunotherapy (DEXIR-CI). Another 10 children on dexamethasone alone or with IVIg at initial evaluation only provided a comparison group. Motor severity (total score) was scored rater-blinded via videotapes using the validated OMS Evaluation Scale. DEXIR-CI was associated with a 69% reduction in group total score (P = 0.004) and was clinically well tolerated. Patients given the dexamethasone combination exhibited significantly lowered B cell frequencies in cerebrospinal fluid (-94%) and blood (-76%), normalizing the cerebrospinal fluid B cell percentage. The number of patients with positive inflammatory markers dropped 87% (P = 0.002) as did the number of markers. Cerebrospinal fluid oligoclonal bands were positive in four of nine pretreatment patients but zero of six post-treatment patients. In the comparison group, partial response to dexamethasone alone or with IVIg was associated with multiple positive markers for neuroinflammation despite an average of seven months of treatment. Multimechanistic dexamethasone-based combination immunotherapy increases the therapeutic armamentarium for OMS, providing a viable option for less severely affected individuals. Partial response to dexamethasone with or without IVIg is indicative of ongoing neuroinflammation and should be treated promptly and accordingly. Copyright © 2017

  15. A Qualitative Study into Dependent Relationships and Voluntary Informed Consent for Research in Pediatric Oncology.

    Science.gov (United States)

    Dekking, Sara A S; van der Graaf, Rieke; Schouten-van Meeteren, Antoinette Y N; Kars, Marijke C; van Delden, Johannes J M

    2016-04-01

    In pediatric oncology, many oncologists invite their own patients to participate in research. Inclusion within a dependent relationship is considered to potentially compromise voluntariness of consent. Currently, it is unknown to what extent those involved in pediatric oncology experience the dependent relationship as a threat to voluntary informed consent, and what they see as safeguards to protect voluntary informed consent within a dependent relationship. We performed a qualitative study among key actors in pediatric oncology to explore their experiences with the dependent relationship and voluntary informed consent. We conducted three focus groups and 25 semi-structured, in-depth interviews with pediatric oncologists, research coordinators, Research Ethics Committee members, parents of children with cancer, and adolescents with cancer. Professionals regarded the dependent relationship both as a potential threat to and as a positive influence on voluntary decision making. Parents and adolescents did not feel as though dependency upon the oncologist influenced their decisions. They valued the involvement of their own physician in the informed consent process. The professionals suggested three strategies to protect voluntariness: emphasizing voluntariness; empowering families; involvement of an independent person. Although the dependent relationship between pediatric oncologists, patients and parents may be problematic for voluntary informed consent, this is not necessarily the case. Moreover, the involvement of treating physicians may even have a positive impact on the informed consent process. Although we studied pediatric oncology, our results may also apply to many other fields of pediatric medicine where research and care are combined, for example, pediatric rheumatology, neurology and nephrology. Clinical trials in these fields are inevitably often designed, initiated and conducted by medical specialists closely involved in patient care.

  16. Professional Ethics of Software Engineers: An Ethical Framework.

    Science.gov (United States)

    Lurie, Yotam; Mark, Shlomo

    2016-04-01

    The purpose of this article is to propose an ethical framework for software engineers that connects software developers' ethical responsibilities directly to their professional standards. The implementation of such an ethical framework can overcome the traditional dichotomy between professional skills and ethical skills, which plagues the engineering professions, by proposing an approach to the fundamental tasks of the practitioner, i.e., software development, in which the professional standards are intrinsically connected to the ethical responsibilities. In so doing, the ethical framework improves the practitioner's professionalism and ethics. We call this approach Ethical-Driven Software Development (EDSD), as an approach to software development. EDSD manifests the advantages of an ethical framework as an alternative to the all too familiar approach in professional ethics that advocates "stand-alone codes of ethics". We believe that one outcome of this synergy between professional and ethical skills is simply better engineers. Moreover, since there are often different software solutions, which the engineer can provide to an issue at stake, the ethical framework provides a guiding principle, within the process of software development, that helps the engineer evaluate the advantages and disadvantages of different software solutions. It does not and cannot affect the end-product in and of-itself. However, it can and should, make the software engineer more conscious and aware of the ethical ramifications of certain engineering decisions within the process.

  17. haemolyticuraemic syndrome in children from south India

    African Journals Online (AJOL)

    Background. Haemolytic-uraemic syndrome (HUS) occurring without a diarrhoeal prodrome is termed D- HUS and has a poorer prognosis than D+ HUS, with high mortality and potential for long-term renal and non-renal morbidity. Methods. We studied nine children with D- HUS from the Pediatric Nephrology division of the ...

  18. Characteristics of pediatric multiple sclerosis: The Turkish pediatric multiple sclerosis database.

    Science.gov (United States)

    Yılmaz, Ünsal; Anlar, Banu; Gücüyener, Kıvılcım

    2017-11-01

    To document the clinical and paraclinical features of pediatric multiple sclerosis (MS) in Turkey. Data of MS patients with onset before age 18 years (n = 193) were collected from 27 pediatric neurology centers throughout Turkey. Earlier-onset (<12 years) and later-onset (≥12 years) groups were compared. There were 123 (63.7%) girls and 70 (36.3%) boys aged 4-17 years, median 14 years at disease onset. Family history of MS was 6.5%. The first presentation was polysymptomatic in 55.4% of patients, with brainstem syndromes (50.3%), sensory disturbances (44%), motor symptoms (33.2%), and optic neuritis (26.4%) as common initial manifestations. Nineteen children had facial paralysis and 10 had epileptic seizures at first attack; 21 (11%) were initially diagnosed with acute disseminated encephalomyelitis (ADEM). Oligoclonal bands were identified in 68% of patients. Magnetic resonance imaging revealed periventricular (96%), cortical/juxtacortical (64.2%), brainstem (63%), cerebellum (51.4%), and spinal cord (67%) involvement. Visual evoked potentials (VEP) were abnormal in 52%; serum 25-hydroxyvitamin D levels were low in 68.5% of patients. The earlier-onset group had a higher rate of infection/vaccination preceding initial attack, initial diagnosis of ADEM, longer interval between first 2 attacks, and more disability accumulating in the first 3 years of the disease. Brainstem and cerebellum are common sites of clinical and radiological involvement in pediatric-onset MS. VEP abnormalities are frequent even in patients without history of optic neuropathy. Vitamin D status does not appear to affect the course in early disease. MS beginning before 12 years of age has certain characteristics in history and course. Copyright © 2017 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

  19. The development of computer ethics: contributions from business ethics and medical ethics.

    Science.gov (United States)

    Wong, K; Steinke, G

    2000-04-01

    In this essay, we demonstrate that the field of computer ethics shares many core similarities with two other areas of applied ethics. Academicians writing and teaching in the area of computer ethics, along with practitioners, must address ethical issues that are qualitatively similar in nature to those raised in medicine and business. In addition, as academic disciplines, these three fields also share some similar concerns. For example, all face the difficult challenge of maintaining a credible dialogue with diverse constituents such as academicians of various disciplines, professionals, policymakers, and the general public. Given these similarities, the fields of bioethics and business ethics can serve as useful models for the development of computer ethics.

  20. Social pediatrics: weaving horizontal and vertical threads through pediatric residency.

    Science.gov (United States)

    van den Heuvel, Meta; Martimianakis, Maria Athina Tina; Levy, Rebecca; Atkinson, Adelle; Ford-Jones, Elizabeth; Shouldice, Michelle

    2017-01-13

    Social pediatrics teaches pediatric residents how to understand disease within their patients' social, environmental and political contexts. It's an essential component of pediatric residency training; however there is very little literature that addresses how such a broad-ranging topic can be taught effectively. The aim of this study was to determine and characterize social pediatric education in our pediatric residency training in order to identify strengths and gaps. A social pediatrics curriculum map was developed, attending to 3 different dimensions: (1) the intended curriculum as prescribed by the Objectives of Training for Pediatrics of the Royal College of Physicians and Surgeons of Canada (RCPSC), (2) the formal curriculum defined by rotation-specific learning objectives, and (3) the informal/hidden curriculum as reflected in resident and teacher experiences and perceptions. Forty-one social pediatric learning objectives were extracted from the RCPSC Objectives of Training for Pediatrics, most were listed in the Medical Expert (51%) and Health Advocate competencies (24%). Almost all RCPSC social pediatric learning objectives were identified in more than one rotation and/or seminar. Adolescent Medicine (29.2%), Pediatric Ambulatory Medicine (26.2%) and Developmental Pediatrics (25%) listed the highest proportion of social pediatric learning objectives. Four (10%) RCPSC social pediatric objectives were not explicitly named within learning objectives of the formal curriculum. The informal curriculum revealed that both teachers and residents viewed social pediatrics as integral to all clinical encounters. Perceived barriers to teaching and learning of social pediatrics included time constraints, particularly in a tertiary care environment, and the value of social pediatrics relative to medical expert knowledge. Despite the lack of an explicit thematic presentation of social pediatric learning objectives by the Royal College and residency training program

  1. Commentary: pediatric eHealth interventions: common challenges during development, implementation, and dissemination.

    Science.gov (United States)

    Wu, Yelena P; Steele, Ric G; Connelly, Mark A; Palermo, Tonya M; Ritterband, Lee M

    2014-07-01

    To provide an overview of common challenges that pediatric eHealth researchers may encounter when planning, developing, testing, and disseminating eHealth interventions along with proposed solutions for addressing these challenges. The article draws on the existing eHealth literature and the authors' collective experience in pediatric eHealth research. The challenges associated with eHealth interventions and their proposed solutions are multifaceted and cut across a number of areas from eHealth program development through dissemination. Collaboration with a range of individuals (e.g., multidisciplinary colleagues, commercial entities, primary stakeholders) is the key to eHealth intervention success. To ensure adequate resources for design, development, and planning for sustainability, a number of public and private sources of funding are available. A study design that addresses ethical concerns and security issues is critical to ensure scientific integrity and intervention dissemination. Table I summarizes key issues to consider during eHealth intervention development, testing, and dissemination. © The Author 2014. Published by Oxford University Press on behalf of the Society of Pediatric Psychology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  2. Young children with functional abdominal pain (FAP) and irritable bowel syndrome (IBS) followed in pediatric gastroenterology (PED-GI) vs primary pediatric care (PED): Differences in outcomes

    Science.gov (United States)

    The American Academy of Pediatrics suggests that children with recurrent abdominal pain without alarm signs be managed in pediatric rather than specialty care. However, many of these children are seen in tertiary care. In a longitudinal examination of physical and psychological symptoms, we hypothes...

  3. Anesthetic management of a patient with Bartter’s syndrome: A case report

    Directory of Open Access Journals (Sweden)

    Harun Aydoğan

    2012-09-01

    Full Text Available Bartter’s syndrome is a rare disorder characterized bysevere hypochloremic metabolic alkalosis, hypokalemia,hyponatremia, hyperaldosteronism, elevation in plasmarenin activity and insensitivity to angiotensin II. Electrolyteimbalances such as hypomagnesemia, hyponatremiamight make anesthetic management difficult in Barttersyndrome. A 4 years old patient underwent surgery foringuinal hernia with caudal anesthesia using bupivacaine0.25%. In this case report, it was aimed to discuss the anestheticmanagement of a patient with Bartter syndrome.Key words: Regional anesthesia, Bartter syndrome, inguinalhernia surgery, pediatric anesthesia

  4. Consensus paper on post-operative pediatric cerebellar mutism syndrome

    DEFF Research Database (Denmark)

    Gudrunardottir, Thora; Morgan, Angela T; Lux, Andrew L

    2016-01-01

    -operative pediatric CMS" was formed, preliminary recommendations for diagnostic and follow-up procedures were created, two working groups on a new scoring scale and risk prediction and prevention were established, and areas were identified where further information is needed. DISCUSSION: The consensus process...... to provide a more solid foundation for future clinical and research work. It is thought as a consensus for moving forward and hopefully paves the way to developing a standard approach to this challenging problem with the advent of better scoring methods and ultimate goal of reducing the risk of CMS....

  5. Imaginative ethics--bringing ethical praxis into sharper relief.

    Science.gov (United States)

    Hansson, Mats G

    2002-01-01

    The empirical basis for this article is three years of experience with ethical rounds at Uppsala University Hospital. Three standard approaches of ethical reasoning are examined as potential explanations of what actually occurs during the ethical rounds. For reasons given, these are not found to be satisfying explanations. An approach called "imaginative ethics", is suggested as a more satisfactory account of this kind of ethical reasoning. The participants in the ethical rounds seem to draw on a kind of moral competence based on personal life experience and professional competence and experience. By listening to other perspectives and other experiences related to one particular patient story, the participants imagine alternative horizons of moral experience and explore a multitude of values related to clinical practice that might be at stake. In his systematic treatment of aesthetics in the Critique of Judgement, Kant made use of an operation of thought that, if applied to ethics, will enable us to be more sensitive to the particulars of each moral situation. Based on this reading of Kant, an account of imaginative ethics is developed in order to bring the ethical praxis of doctors and nurses into sharper relief. The Hebraic and the Hellenic traditions of imagination are used in order to illuminate some of the experiences of ethical rounds. In conclusion, it is argued that imaginative ethics and principle-based ethics should be seen as complementary in order to endow a moral discourse with ethical authority. Kantian ethics will do the job if it is remembered that Kant suggested only a modest, negative role of principle-based deliberation.

  6. Peter Koslowski’s Ethics and Economics or Ethical Economy

    DEFF Research Database (Denmark)

    Rendtorff, Jacob Dahl

    2016-01-01

    This paper presents the concept of ethical economy (Wirtschaftsethik) and the relation between ethics and economics on the basis of the work of the German ethical economist Peter Koslowski. The concept of ethical economy includes three levels: micro, meso and macro levels; and it also deals...... with the philosophical analysis of the ethical foundations of the economy. After the discussion of these elements of the ethical economy, the paper presents some possible research topics for a research agenda about economic ethics or ethical economy....

  7. Presenting Symptoms in Pediatric Restless Legs Syndrome Patients

    NARCIS (Netherlands)

    de Weerd, Al; Arico, Irene; Silvestri, Rosalia

    2013-01-01

    Objective: The diagnosis restless legs syndrome (RLS) in children depends on the history told by the child and his parents. The description of symptoms given by the child him or herself is most important. Additional criteria are, among others, the results of polysomnography (PSG). Description of the

  8. A Mixed Presentation of Serotonin Syndrome vs Neuroleptic Malignant Syndrome in a 12-Year-Old Boy.

    Science.gov (United States)

    Sun, Christie; Sweet, Hannah; Minns, Alicia B; Shapiro, Desiree; Jenkins, Willough

    2018-04-24

    Neuroleptic malignant syndrome (NMS) and serotonin syndrome (SS) are serious medical conditions associated with commonly prescribed psychiatric medications. Although the mechanisms differ, they can be clinically difficult to distinguish. We report a case of a pediatric patient with complicated psychiatric history that developed features of both syndromes in the setting of polypharmacy. A 12-year-old boy with a history of developmental delay, attention-deficit hyperactivity disorder, and posttraumatic stress disorder presented to the emergency department with behavior changes consisting of delayed reactions, gait instability, drooling, and slowed movements. Ten days before presentation, his outpatient psychiatrist had made multiple medication changes including discontinuation of cyproheptadine (an appetite stimulant) and initiation of aripiprazole. On arrival, the patient was noted to be tachycardia and hypertensive for age. He was disoriented, intermittently agitated, and tremulous with increased tonicity, clonus in the lower extremities, and mydriasis. He was supportively treated with lorazepam and intravenous fluids while discontinuing potential offending agents. His course was complicated by hypertension and agitation managed with dexmedetomidine infusion and benzodiazepines. His mental status, tremors, and laboratory values began to improve over the next 2 days, and eventually transitioned to the inpatient psychiatric unit on hospital day 7. Diagnosis of NMS or SS can be difficult when there is overlap between syndromes, particularly in the setting of multiple potential offending agents or underlying developmental delay. In addition, pediatric patients may present atypically as compared with adult patients with the same condition. The use of antipsychotic medications for young children with behavioral problems has risen dramatically in the last decade, increasing their risk for developing SS or NMS.

  9. Ethics of End of Life Decisions in Pediatrics: A Narrative Review of the Roles of Caregivers, Shared Decision-Making, and Patient Centered Values

    Directory of Open Access Journals (Sweden)

    Jonathan D. Santoro

    2018-04-01

    Full Text Available Background: This manuscript reviews unique aspects of end of life decision-making in pediatrics. Methods: A narrative literature review of pediatric end of life issues was performed in the English language. Results: While a paternalistic approach is typically applied to children with life-limiting medical prognoses, the cognitive, language, and physical variability in this patient population is wide and worthy of review. In end of life discussions in pediatrics, the consideration of a child’s input is often not reviewed in depth, although a shared decision-making model is ideal for use, even for children with presumed limitations due to age. This narrative review of end of life decision-making in pediatric care explores nomenclature, the introduction of the concept of death, relevant historical studies, limitations to the shared decision-making model, the current state of end of life autonomy in pediatrics, and future directions and needs. Although progress is being made toward a more uniform and standardized approach to care, few non-institutional protocols exist. Complicating factors in the lack of guidelines include the unique facets of pediatric end of life care, including physical age, paternalism, the cognitive and language capacity of patients, subconscious influencers of parents, and normative values of death in pediatrics. Conclusions: Although there have been strides in end of life decision-making in pediatrics, further investigation and research is needed in this field.

  10. Ethics of End of Life Decisions in Pediatrics: A Narrative Review of the Roles of Caregivers, Shared Decision-Making, and Patient Centered Values.

    Science.gov (United States)

    Santoro, Jonathan D; Bennett, Mariko

    2018-04-26

    Background: This manuscript reviews unique aspects of end of life decision-making in pediatrics. Methods: A narrative literature review of pediatric end of life issues was performed in the English language. Results: While a paternalistic approach is typically applied to children with life-limiting medical prognoses, the cognitive, language, and physical variability in this patient population is wide and worthy of review. In end of life discussions in pediatrics, the consideration of a child’s input is often not reviewed in depth, although a shared decision-making model is ideal for use, even for children with presumed limitations due to age. This narrative review of end of life decision-making in pediatric care explores nomenclature, the introduction of the concept of death, relevant historical studies, limitations to the shared decision-making model, the current state of end of life autonomy in pediatrics, and future directions and needs. Although progress is being made toward a more uniform and standardized approach to care, few non-institutional protocols exist. Complicating factors in the lack of guidelines include the unique facets of pediatric end of life care, including physical age, paternalism, the cognitive and language capacity of patients, subconscious influencers of parents, and normative values of death in pediatrics. Conclusions: Although there have been strides in end of life decision-making in pediatrics, further investigation and research is needed in this field.

  11. Docosahexaenoic acid levels in blood and metabolic syndrome in obese children: is there a link?

    OpenAIRE

    Lassandro, C.; Banderali, G.; Radaelli, G.; Borghi, E.; Moretti, F.; Verduci, E.

    2015-01-01

    Prevalence of metabolic syndrome is increasing in the pediatric population. Considering the different existing criteria to define metabolic syndrome, the use of the International Diabetes Federation (IDF) criteria has been suggested in children. Docosahexaenoic acid (DHA) has been associated with beneficial effects on health. The evidence about the relationship of DHA status in blood and components of the metabolic syndrome is unclear. This review discusses the possible association between DH...

  12. An official American Thoracic Society/International Society for Heart and Lung Transplantation/Society of Critical Care Medicine/Association of Organ and Procurement Organizations/United Network of Organ Sharing Statement: ethical and policy considerations in organ donation after circulatory determination of death.

    Science.gov (United States)

    Gries, Cynthia J; White, Douglas B; Truog, Robert D; Dubois, James; Cosio, Carmen C; Dhanani, Sonny; Chan, Kevin M; Corris, Paul; Dark, John; Fulda, Gerald; Glazier, Alexandra K; Higgins, Robert; Love, Robert; Mason, David P; Nakagawa, Thomas A; Shapiro, Ron; Shemie, Sam; Tracy, Mary Fran; Travaline, John M; Valapour, Maryam; West, Lori; Zaas, David; Halpern, Scott D

    2013-07-01

    Donation after circulatory determination of death (DCDD) has the potential to increase the number of organs available for transplantation. Because consent and management of potential donors must occur before death, DCDD raises unique ethical and policy issues. To develop an ethics and health policy statement on adult and pediatric DCDD relevant to critical care and transplantation stakeholders. A multidisciplinary panel of stakeholders was convened to develop an ethics and health policy statement. The panel consisted of representatives from the American Thoracic Society, Society of Critical Care Medicine, International Society for Heart and Lung Transplantation, Association of Organ Procurement Organizations, and the United Network of Organ Sharing. The panel reviewed the literature, discussed important ethics and health policy considerations, and developed a guiding framework for decision making by stakeholders. A framework to guide ethics and health policy statement was established, which addressed the consent process, pre- and post mortem interventions, the determination of death, provisions of end-of-life care, and pediatric DCDD. The information presented in this Statement is based on the current evidence, experience, and clinical rationale. New clinical research and the development and dissemination of new technologies will eventually necessitate an update of this Statement.

  13. McCune-Albright syndrome, natural history and multidisciplinary management in a series of 14 pediatric cases.

    Science.gov (United States)

    Agopiantz, Mikael; Journeau, Pierre; Lebon-Labich, Béatrice; Sorlin, Arthur; Cuny, Thomas; Weryha, Georges; Leheup, Bruno

    2016-02-01

    McCune-Albright syndrome is a rare disorder characterized by endocrine disorders, café-au-lait spots and fibrous dysplasia of bone that occurs early in life. A series of 14 pediatric cases were followed between 1994 and 2013 by the competence center for rare endocrine diseases and constitutional bone diseases at CHU de Nancy (France). The diagnosis is based on the presence of at least two symptoms. The mean follow-up was 6 years (1-17 years). The sex ratio was six girls per boy. The incidence was 0.28 cases/million population/year. Mean age at diagnosis was 6 years. A mutation in the GNAS gene was found in 33% of patients tested. Gonadal involvement (13/14 cases), including early peripheral puberty and ovarian cysts in girls (82%) occurred on average at 4 years of age. Bone involvement (10/14 cases) appeared on average at 5 years of age and was most often multiple (80%) with fracture risk, and the skull, with a neurosensory risk. Clinical definition and methods of screening and monitoring can be improved to allow for an earlier intervention. It must be multidisciplinary and take into account the disability and quality of life of the patient. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  14. Successful management of Churg-Strauss syndrome using omalizumab as adjuvant immunomodulatory therapy: first documented pediatric case.

    Science.gov (United States)

    Iglesias, E; Camacho Lovillo, M; Delgado Pecellín, I; Lirola Cruz, M J; Falcón Neyra, M D; Salazar Quero, J C; Bernabeu-Wittel, J; González Valencia, J P; Neth, O

    2014-03-01

    Churg-Strauss syndrome (CSS) is an anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis; it is extremely rare in childhood and defined according to the Chapel-Hill Consensus as an eosinophil-rich and granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small to medium-sized vessels. Children commonly have a history of asthma and sinusitis whilst clinical presentation typically involves pulmonary tract and less frequently skin, heart, gastrointestinal tract, and peripheral nerves. Cardiopulmonary disease is higher in children and prognosis is worse. It is associated with significant eosinophilia and raised serum IgE-levels. ANCA are only found in 25% of childhood cases. Here we report the case of a 10-year-old girl who presented to us with vomiting, abdominal pain, and weight loss, paresthesias of lower extremities and breathlessness as well as a history of asthma, sinusitis and allergic rhinitis. She was treated with corticosteroids, cyclophosphamide, intravenous immunoglobulin, mycophenolate mofetil (MMF), and rituximab. However, remission was only achieved after initiation of omalizumab therapy, a recombinant humanized anti-IgE antibody. To the best of our knowledge this is the first pediatric patient suffering from CSS successfully managed with adjuvant anti-IgE therapy resulting in the control of respiratory as well as gastrointestinal symptoms. © 2013 Wiley Periodicals, Inc.

  15. Rett syndrome: an overlooked diagnosis in women with stereotypic hand movements, psychomotor retardation, Parkinsonism, and dystonia?

    Science.gov (United States)

    Roze, Emmanuel; Cochen, Valérie; Sangla, Sophie; Bienvenu, Thierry; Roubergue, Anne; Leu-Semenescu, Smaranda; Vidaihet, Marie

    2007-02-15

    Rett syndrome is an X-linked neurodevelopmental disorder resulting in profound psychomotor retardation. It is usually diagnosed by a pediatrician or pediatric neurologist. Adult neurologists may, therefore, overlook the possibility of Rett syndrome in women with psychomotor retardation of unknown etiology. We report the case of a woman diagnosed with Rett syndrome at age 49 years. This report emphasizes the diagnostic value of movement disorders, including hand stereotypies, Parkinsonism, and dystonia, in adults with Rett syndrome.

  16. The Responsive Amygdala: Treatment-induced Alterations in Functional Connectivity in Pediatric Complex Regional Pain Syndrome

    Science.gov (United States)

    Simons, LE; Pielech, M; Erpelding, N; Linnman, C; Moulton, E; Sava, S; Lebel, A; Serrano, P; Sethna, N; Berde, C; Becerra, L; Borsook, D

    2014-01-01

    The amygdala is a key brain region with efferent and afferent neural connections that involve complex behaviors such as pain, reward, fear and anxiety. This study evaluated resting state functional connectivity of the amygdala with cortical and subcortical regions in a group of chronic pain patients (pediatric complex regional pain syndrome) with age-gender matched controls before and after intensive physical-biobehavioral pain treatment. Our main findings include (1) enhanced functional connectivity from the amygdala to multiple cortical, subcortical, and cerebellar regions in patients compared to controls, with differences predominantly in the left amygdala in the pre-treated condition (disease state); (2) dampened hyperconnectivity from the left amygdala to the motor cortex, parietal lobe, and cingulate cortex after intensive pain rehabilitation treatment within patients with nominal differences observed among healthy controls from Time 1 to Time 2 (treatment effects); (3) functional connectivity to several regions key to fear circuitry (prefrontal cortex, bilateral middle temporal lobe, bilateral cingulate, hippocampus) correlated with higher pain-related fear scores and (4) decreases in pain-related fear associated with decreased connectivity between the amygdala and the motor and somatosensory cortex, cingulate, and frontal areas. Our data suggest that there are rapid changes in amygdala connectivity following an aggressive treatment program in children with chronic pain and intrinsic amygdala functional connectivity activity serving as a potential indicator of treatment response. PMID:24861582

  17. How Should Ethical Theories Be Dealt with in Engineering Ethics?

    Science.gov (United States)

    Ohishi, Toshihiro

    Contemporary engineering ethics scholars deal with contesting several ethical theories without criticizing them radically and try to use them to solve ethical problems. In this paper I first show that a conflict between ethical theories is not superficial, and pragmatic methods are adopted in engineering ethics. Second, I claim that the way to deal with contesting ethical theories in contemporary engineering ethics has an unacceptable side which does not accord with my argument that a conflict between ethical theories is not superficial and pragmatic methods are adopted in engineering ethics. Finally, I conclude that this inconsistency in contemporary engineering ethics should be corrected to make contemporary engineering ethics consistent.

  18. Needs assessment for collaborative network in pediatric clinical research and education.

    Science.gov (United States)

    Ishiguro, Akira; Sasaki, Hatoko; Yahagi, Naohisa; Kato, Hitoshi; Kure, Shigeo; Mori, Rintaro

    2017-01-01

    A collaborative network for pediatric research has not been fully established in Japan. To identify the network infrastructure, we conducted a survey on the support and education for clinical research currently available in children's hospitals. In November 2014, a 27-question survey was distributed to 31 hospitals belonging to the Japanese Association of Children's Hospitals and Related Institutions (JACHRI) to assess clinical research support, research education, research achievements, and their expectations. All the hospitals responded to the survey. Overall, 74.2% of hospitals had clinical research support divisions. Although all hospitals had ethics committees, manager, intellectual property management unit, biostatistician, and English-language editor. Seven hospitals had education programs for clinical research. The number of seminars and workshops for clinical research had significant correlations with the number of physicians (r = 0.927), pediatricians (r = 0.922), and clinical trial management physicians (r = 0.962). There was a significant difference in the number of clinical trials initiated by physicians between hospitals with research education programs and those without (P leader to establish a collaborative network for clinical research. Important factors for creating a collaborative system for pediatric research in Japan were identified. Human resources to support clinical research are a key factor to improve clinical research education and research achievements. © 2016 Japan Pediatric Society.

  19. Pediatric Uveitis.

    Science.gov (United States)

    Chan, Nicole Shu-Wen; Choi, Jessy; Cheung, Chui Ming Gemmy

    2018-01-01

    Pediatric uveitis differs from adult-onset uveitis and is a topic of special interest because of its diagnostic and therapeutic challenges. Children with uveitis are often asymptomatic and the uveitis is often chronic, persistent, recurrent, and resistant to conventional treatment. Anterior uveitis is the most common type of uveitis in children; the prevalence of intermediate, posterior, and panuveitis varies geographically and among ethnic groups. Regarding etiology, most cases of pediatric uveitis are idiopathic but can be due to systemic inflammatory disorders, infections, or a manifestation of masquerade syndrome. Ocular complications include cataracts, hypotony or glaucoma, band keratopathy, synechiae formation, macular edema, optic disc edema, choroidal neovascular membranes, and retinal detachment. These complications are often severe, leading to irreversible structural damage and significant visual disability due to delayed presentation and diagnosis, persistent chronic inflammation from suboptimal treatment, topical and systemic corticosteroid dependence, and delayed initiation of systemic disease‒modifying agents. Treatment for noninfectious uveitis is a stepwise approach starting with corticosteroids. Immunomodulatory therapy should be initiated in cases where quiescence cannot be achieved without steroid dependence. Patients should be monitored regularly for complications of uveitis along with systemic and ocular adverse effects from treatments. The goals are to achieve steroid-free durable remission, to reduce the risk of sight-threatening complications from the uncontrolled ocular inflammation, and to avoid the impact of lifelong burden of visual loss on the child and their family. Multidisciplinary management will ensure holistic care of affected children and improve the support for their families. Copyright 2018 Asia-Pacific Academy of Ophthalmology.

  20. Problemas de la ética médica: sus manifestaciones en el manejo de pacientes pediátricos inmunodeprimidos Problems of the medical ethics: its manifestations in the management of immunodepression in pediatric patients

    Directory of Open Access Journals (Sweden)

    Miriam C. Sánchez-Segura

    2010-12-01

    Full Text Available La Bioética nació a partir de 1970, y desde su surgimiento, comenzó a tener enorme impacto en la práctica de la medicina y en la investigación. Más que un influjo de la ciencia sobre la ética se debe hablar de un desfasaje entre ambas, pues la ciencia progresó con tanta rapidez que la ética no podía dar respuesta a los problemas que planteaba. Por esto, algunos autores señalaron que si no se le añadía ciencia a la ética, esta sería algo vano, inconsistente. Entonces se realizó una nueva revisión de la fundamentación y sistematización de la ética, que cobijó particularmente a la ética científica y, desde luego, a la ética médica. En este trabajo se exponen algunos de los problemas de la ética médica que pueden surgir en el manejo de pacientes pediátricos inmunodeprimidos y de qué forma pueden cumplirse en ellos los principios de autonomía, beneficencia y justicia, que constituyen la trinidad de la Bioética Médica, con la finalidad de lograr un mejor tratamiento y seguimiento de los enfermos y la rápida incorporación de ellos y de sus familiares a la vida social.The Bioethics appeared from 1970 and from then it has a great impact in the medical practice and in research. More than a influence science on the ethics it is necessary to speak of a gapamong them since the science has progressed so fast that the ethics could not to give an answer to related problems. Thus, some authors have indicated that if the science is not added to ethics, this will be something in vain and inconsistent. Then a new review of the foundation and systematization of the ethics was carried out covering particularly the scientific ethics and of course, the medical ethics. In present paper are exposed some of the problems of medical ethics that may to appear in the management of immunodepression pediatric patients and way to fulfill in them the autonomy, charity and justice principles that are the Trinity of the Medical Bioethics to achieve

  1. Staphylococcal superantigens; toxic shock syndrome toxin-1 and enterotoxins in pediatric otitis media effusion: a brief report

    Directory of Open Access Journals (Sweden)

    Noorbakhsh S

    2013-03-01

    Full Text Available Background: Staphylococcal superantigens (SAg's may have some role in otitis media with effusion (OME. The aim of this study was the search of staphylococcal SAg's in middle ear effusion of children with OME.  Methods: This cross sectional-analytic study was done in ENT & pediatric wards upon 64 children with otitis media with effusion (OME between 1-15 years, (mean age=7.42+4 years of Rasoul Akram University Hospital, Tehran, Iran in 2009-2011. Fifty six percent (36 of cases were male, 43.8% (28 were female. Staphylococcal SAg's; Toxic Shock Syndrome Toxin-1 (TSST-1, Staphylococcal enterotoxin A, B, C, D (Enzyme immune assay, AB Cam, USA were detected in middle ear effusion samples after conventional culture.Results: None type of SAg's found in 39% of OME cases, enterotoxin B found in: 22%; enterotoxin A: 17%, enterotoxin C: 15.6%, enterotoxin D: 12.5%, Toxic Shock Syndrome Toxin-1 (TSST-1: 7.8% Mean age of cases with positive TSST-1, enterotoxin A, B, C, and D was: 1, 5, 8.6, 9.6 and 9.6 years respectively. Positive TSST had no agreement with positive enterotoxin A and C but had weak agreement with type B and D. Mean age of cases with positive TSST was one years which had significant difference with (7.9 years in cases with negative TSST test (P<0.0001.Conclusion: At least one or more type of staphylococcal toxins had found in middle ear effusion of 70% of OME cases with negative culture for Staphylococcus aureus. Even in culture negative cases, staphylococcal toxins might have some immunologic role in middle ear effusion forming. Finding the SAg's (at least one type are important for treatment of immunosuppressive or corticosteroid in cases with resistant OME.

  2. Annual Costs of Care for Pediatric Irritable Bowel Syndrome, Functional Abdominal Pain, and Functional Abdominal Pain Syndrome

    NARCIS (Netherlands)

    Hoekman, Daniël R.; Rutten, Juliette M. T. M.; Vlieger, Arine M.; Benninga, Marc A.; Dijkgraaf, Marcel G. W.

    2015-01-01

    To estimate annual medical and nonmedical costs of care for children diagnosed with irritable bowel syndrome (IBS) or functional abdominal pain (syndrome; FAP/FAPS). Baseline data from children with IBS or FAP/FAPS who were included in a multicenter trial (NTR2725) in The Netherlands were analyzed.

  3. [Dimensions of work ethic as predictors of strategies to cope with stress].

    Science.gov (United States)

    Grabowski, Damian; Pollak, Anita; Czerw, Agnieszka

    2017-10-17

    The article presents the mutual relations between the components of work ethic and the strategies of coping with stress used by employees of different branches. Work ethic was presented as a syndrome of the following attitudes: perceiving work as a moral value, treating work as a central value in life, and the belief in the importance of hard work that leads to success. This ethic also consists of the following components: unwillingness to waste time, disapproval of spare time (anti-leisure), willingness to delay gratification, willingness to act honestly at work (morality/ethic), and being independent (self-reliance). Coping strategies were presented as 3 dimensions (obtained by application of factor analysis of the questionnaire scales COPE (Coping Orientations to Problems Experienced)): proactive cognitive operations, avoidance of action and seeking support. The study conducted on 360 employees of different branches shows that the dimensions of the work ethic are moderately related to strategies emphasizing proactive cognitive operations and poorly related to seeking support and avoidance of action. At the same time, the relations between work ethic and avoidance of action are negative (higher work ethic is linked with lower tendency to avoid action). Predictors of proactive cognitive operations are unwillingness to waste time, treating work as a central value in life, willingness to act honestly at work (morality/ethic) and being independent (self-reliance). Med Pr 2017;68(6):711-724. This work is available in Open Access model and licensed under a CC BY-NC 3.0 PL license.

  4. The use of the Berlin definition for acute respiratory distress syndrome during infancy and early childhood : multicenter evaluation and expert consensus

    NARCIS (Netherlands)

    De Luca, Daniele; Piastra, Marco; Chidini, Giovanna; Tissieres, Pierre; Calderini, Edoardo; Essouri, Sandrine; Medina Villanueva, Alberto; Vivanco Allende, Ana; Pons-Odena, Marti; Perez-Baena, Luis; Hermon, Michael; Tridente, Ascanio; Conti, Giorgio; Antonelli, Massimo; Kneyber, Martin

    2013-01-01

    A new acute respiratory distress syndrome (ARDS) definition has been recently issued: the so-called Berlin definition (BD) has some characteristics that could make it suitable for pediatrics. The European Society for Pediatric Neonatal Intensive Care (ESPNIC) Respiratory Section started a project to

  5. Pediatric neurocritical care.

    Science.gov (United States)

    Murphy, Sarah

    2012-01-01

    Pediatric neurocritical care is an emerging multidisciplinary field of medicine and a new frontier in pediatric critical care and pediatric neurology. Central to pediatric neurocritical care is the goal of improving outcomes in critically ill pediatric patients with neurological illness or injury and limiting secondary brain injury through optimal critical care delivery and the support of brain function. There is a pressing need for evidence based guidelines in pediatric neurocritical care, notably in pediatric traumatic brain injury and pediatric stroke. These diseases have distinct clinical and pathophysiological features that distinguish them from their adult counterparts and prevent the direct translation of the adult experience to pediatric patients. Increased attention is also being paid to the broader application of neuromonitoring and neuroprotective strategies in the pediatric intensive care unit, in both primary neurological and primary non-neurological disease states. Although much can be learned from the adult experience, there are important differences in the critically ill pediatric population and in the circumstances that surround the emergence of neurocritical care in pediatrics.

  6. Ethics Management: How to Achieve Ethical Organizations and Management?

    Directory of Open Access Journals (Sweden)

    Carita Lilian Snellman

    2015-12-01

    Full Text Available The last decades’ serious organizational scandals that mainly stem from corruption and conflicting interests but also from bribery, favoritism and other wrongdoings have ac-centuated the need for finding instruments for achieving more ethical organizations and management. Ethics management is particularly important in the public sector because public employees and holders of public office are responsible for increasing wellbeing and providing common good for all citizens. Only accountable management striving for integrity through ethical practices and decision making will guarantee ethical organiza-tional behavior. In spite of increasing research on ethics in general and ethics manage-ment in particular, increase in organizational scandals indicates that there is knowledge gap concerning ethical instruments that help to solve ethical problems. The aim of this paper is to shed light on ethical theories and instruments, and wrongdoings in public sec-tor organizations. The main questions are; why is there so much wrongdoing; how can it be reduced; and how can more ethical organization and management be achieved. This is a review paper aiming to provide a review of ethical theories and instruments and dis-cuss serious wrongdoings and the role of ethics in the public sector. The paper contrib-utes to the fields of management and organization, ethics, and public management.

  7. Wolfram Syndrome. Case report.

    Science.gov (United States)

    Tarała, Wojciech; Drachal, Elzbieta; Mazur, Artur; Korczowski, Bartosz; Szadkowska, Agnieszka; Zmysłowska, Agnieszka; Młynarski, Wojciech

    2016-01-01

    Wolfram syndrome is a rare neurodegenerative and genetic disorder, characterized by insulin-dependent diabetes mellitus, caused by non-autoimmune loss of β cells, as well as optic atrophy; the disease is also known as DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness). Patients that demonstrate diabetes mellitus are also affected by: optic atrophy in the first decade of their life, diabetes insipidus and sensorineural deafness in the second decade, and urinary tract and neurological abnormalities in the third decade of their life. Patients with Wolfram syndrome usually die due to central respiratory failures caused by brain stem atrophy in their third or at the beginning of their fourth decade of life. The authors present a case of two female siblings with diagnosed Wolfram syndrome that have been diagnosed with diabetes mellitus, optic atrophy, and urological abnormalities. Early diagnosis and adequate hormonal supplementation can improve their quality of life. © Polish Society for Pediatric Endocrinology and Diabetology.

  8. Is Duane retraction syndrome part of the VACTERL association?

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    Akar S

    2013-03-01

    Full Text Available Serpil Akar,1 Birsen Gokyigit,1 Isilay Kavadarli,2 Ahmet Demirok11Pediatric Ophthalmology and Strabismus Department, Prof Dr N Resat Belger Beyoglu Education and Research Eye Hospital, Istanbul, 2Department of Ophthalmology, Gaziantep, Kilis State Hospital, Kilis, TurkeyAbstract: We report here a patient with type 1 Duane’s retraction syndrome and multiple congenital abnormalities as a result of the VACTERL association. The presented combination of Duane’s retraction syndrome and the VACTERL association has not been reported in the literature. The present case was instructive for reviewing the continuous spectrum of ocular anomalies that accompany the VACTERL association.Keywords: Duane’s retraction syndrome, VACTERL association, combination

  9. Ethical Ideology and Ethical Judgments of Accounting Practitioners in Malaysia

    Directory of Open Access Journals (Sweden)

    Suhaiza Ismail

    2011-09-01

    Full Text Available The paper intends to explore the ethical ideology and ethical judgments of accounting practitioners in Malaysia. The objectives of this study are twofold. First, the paper intends to examine the factors that contribute to the different ethical ideology among Malaysian accounting practitioners. Second, it aims to investigate the influence of demographic factors and ethical ideology on ethical judgments of accounting practitioners. The study used Forsyth’s (1980 Ethics Position Questionnaire instrument to examine the ethical ideology of the accountants and adopted ethics vignettes used by Emerson et al. (2007 to assess the ethical judgments of the respondents. From the statistical analysis, this study found that age and gender have a significant impact on ethical judgment but not on ethical ideology. In addition, idealism and relativism have a significant influence on ethical judgment, especially in a legally unethical situation.

  10. Radiation-sensitive genetically susceptible pediatric sub-populations

    Energy Technology Data Exchange (ETDEWEB)

    Kleinerman, Ruth A. [National Cancer Institute, NIH, DHHS, Radiation Epidemiology Branch, Division of Cancer Epidemiology and Genetics, Rockville, MD (United States)

    2009-02-15

    Major advances in pediatric cancer treatment have resulted in substantial improvements in survival. However, concern has emerged about the late effects of cancer therapy, especially radiation-related second cancers. Studies of childhood cancer patients with inherited cancer syndromes can provide insights into the interaction between radiation and genetic susceptibility to multiple cancers. Children with retinoblastoma (Rb), neurofibromatosis type 1 (NF1), Li-Fraumeni syndrome (LFS), and nevoid basal cell carcinoma syndrome (NBCCS) are at substantial risk of developing radiation-related second and third cancers. A radiation dose-response for bone and soft-tissue sarcomas has been observed in hereditary Rb patients, with many of these cancers occurring in the radiation field. Studies of NF1 patients irradiated for optic pathway gliomas have reported increased risks of developing another cancer associated with radiotherapy. High relative risks for second and third cancers were observed for a cohort of 200 LFS family members, especially children, possibly related to radiotherapy. Children with NBCCS are very sensitive to radiation and develop multiple basal cell cancers in irradiated areas. Clinicians following these patients should be aware of their increased genetic susceptibility to multiple primary malignancies enhanced by sensitivity to ionizing radiation. (orig.)

  11. Surgical body modification and altruistic individualism: a case for cyborg ethics and methods.

    Science.gov (United States)

    Frank, Arthur W

    2003-12-01

    Three cases of pediatric surgical body modification--limb lengthening, normalization of genitalia, and craniofacial surgery--are considered through the moral language used by those who experience these surgeries. This language has been described as altruistic individualism. Decision making remains individualist, but it also shows considerable concern for others; egoism is complementary with altruism. The altruistic individualist is one of many incompatible identities that are predicted and described by the figure of the cyborg. Cyborgs suggest both ethics and qualitative methods appropriate to surgically shaped children.

  12. Chronicle of pediatric radiology

    International Nuclear Information System (INIS)

    Benz-Bohm, Gabriele; Richter, Ernst

    2012-01-01

    The chronicle of pediatric radiology covers the following issues: Development of pediatric radiology in Germany (BRD, DDR, pediatric radiological accommodations); development of pediatric radiology in the Netherlands (chronology and pediatric radiological accommodations); development of pediatric radiology in Austria (chronology and pediatric radiological accommodations); development of pediatric radiology in Switzerland (chronology and pediatric radiological accommodations).

  13. Translational research in pediatrics III: bronchoalveolar lavage.

    Science.gov (United States)

    Radhakrishnan, Dhenuka; Yamashita, Cory; Gillio-Meina, Carolina; Fraser, Douglas D

    2014-07-01

    The role of flexible bronchoscopy and bronchoalveolar lavage (BAL) for the care of children with airway and pulmonary diseases is well established, with collected BAL fluid most often used clinically for microbiologic pathogen identification and cellular analyses. More recently, powerful analytic research methods have been used to investigate BAL samples to better understand the pathophysiological basis of pediatric respiratory disease. Investigations have focused on the cellular components contained in BAL fluid, such as macrophages, lymphocytes, neutrophils, eosinophils, and mast cells, as well as the noncellular components such as serum molecules, inflammatory proteins, and surfactant. Molecular techniques are frequently used to investigate BAL fluid for the presence of infectious pathologies and for cellular gene expression. Recent advances in proteomics allow identification of multiple protein expression patterns linked to specific respiratory diseases, whereas newer analytic techniques allow for investigations on surfactant quantification and function. These translational research studies on BAL fluid have aided our understanding of pulmonary inflammation and the injury/repair responses in children. We review the ethics and practices for the execution of BAL in children for translational research purposes, with an emphasis on the optimal handling and processing of BAL samples. Copyright © 2014 by the American Academy of Pediatrics.

  14. Arterial Stiffness in Children: Pediatric Measurement and Considerations

    Science.gov (United States)

    Savant, Jonathan D.; Furth, Susan L.; Meyers, Kevin E.C.

    2014-01-01

    Background Arterial stiffness is a natural consequence of aging, accelerated in certain chronic conditions, and predictive of cardiovascular events in adults. Emerging research suggests the importance of arterial stiffness in pediatric populations. Methods There are different indices of arterial stiffness. The present manuscript focuses on carotid-femoral pulse wave velocity and pulse wave analysis, although other methodologies are discussed. Also reviewed are specific measurement considerations for pediatric populations and the literature describing arterial stiffness in children with certain chronic conditions (primary hypertension, obesity, diabetes, chronic kidney disease, hypercholesterolemia, genetic syndromes involving vasculopathy, and solid organ transplant recipients). Conclusions The measurement of arterial stiffness in children is feasible and, under controlled conditions, can give accurate information about the underlying state of the arteries. This potentially adds valuable information about the functionality of the cardiovascular system in children with a variety of chronic diseases well beyond that of the brachial artery blood pressure. PMID:26587447

  15. A Case of Bardet-Biedl Syndrome

    Directory of Open Access Journals (Sweden)

    katayon Etemadi

    2007-09-01

    Full Text Available Etemadi K1, Khazaii MR2 1. MSC of Human Genetic, Molecular Medicine and Genetic department, Medical school, Hamadan University of medical sciences. 2. Assistant professor of Pediatric Urology Abstract Background: The Bardet Biedl syndrome is a heterogenous and autosomal recessive disorder. Primary features are: retinitis pigmentosa, obesity, polydactyly, mental retardation, renal abnormalities and hypogonadism. Renal failure is the major cause of death in homozygote patients, with chronic glomerolopathy that cause chronic renal disease. Secondary features are: speech disorder delay, developmental delay, polyuria, diabetes mellitus and hypertension. The diagnosis of Bardet- Biedl syndrome is established by clinical findings. Twelve genes are known to be associated with Bardet Biedl syndromes: BBS1, BBS2… BBS12. Case presentation: In this article we report a four and half year old boy that have Bardet Biedl syndrome as a result of a consanguine marriage (third degree. Conclusion: A monogenic syndrome such as Bardet Biedl has a lot of symptoms. These symptoms are out put of a mutation in locus of a recessive allel. Therefore people like to marry consanguinly have to do genetic counseling before marriage. Because analysis of family history will reduced the risk of such syndromes.

  16. WOLFF–PARKINSON–WHITE SYNDROME IN CHILDREN: CLINICAL COURSE, DIAGNOSTICS, TREATMENT

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    T.K. Kruchina

    2011-01-01

    Full Text Available Wolff–Parkinson–White (WPW syndrome — is the most common cause of tachycardia in children. The clinical significance of WPW udden cardiac death. Data are presented on the principles of diagnosis of various types of WPW syndrome and characteristics of various types of tachycardia occurring in this disease. At present there is a radical method of treatment of the WPW syndrome — radiofrequency ablation of atrioventricular additional connections. Antiarhythmic therapy remains relevant in arresting attacks of tachycardia, as well as in the treatment of young children who have the age limits for radiofrequency ablation. The principles of choice of treatment and relief of the attack algorithm tachycardia syndrome WPW are described. Key words: Wolff–Parkinson–White syndrome, paroxysmal atrioventricular reciprocal tachycardia, children. (Pediatric Pharmacology. — 2011; 8 (5: 49–53.

  17. Ethical Awareness and Ethical Orientation of Turkish Teachers

    Science.gov (United States)

    Gökçe, Asiye Toker

    2013-01-01

    This study inquires ethical evaluation of teachers, investigating their moral reasoning to ethical decision making, in Turkey. Specifically three hypotheses were tested: Overall ethical awareness of teachers is high; Teachers will identify reasons for ethical evaluation related to philosophical values such as justice, deontology, utilitarianism,…

  18. Nutrition support of the pediatric patient with AIDS.

    Science.gov (United States)

    Bentler, M; Stanish, M

    1987-04-01

    Maintaining optimal nutrition in the pediatric patient with Acquired Immune Deficiency Syndrome (AIDS) is challenging, but it may be one of the most effective therapies. Patients experience numerous complications that compromise nutritional status. Infection, fever, diarrhea, feeding problems, and decreased intake all contribute to malnutrition, which in turn predisposes the patient even more to infection and malabsorption. Nutrition assessment should be done routinely so that new problems may be identified and treated. High-calorie, high-protein feedings, vitamin supplementation, and, when necessary, gavage feedings or parenteral nutrition are recommended to improve nutritional status and prevent further deficits. Maintaining optimal nutrition in the pediatric patient with Acquired Immune Deficiency Syndrome (AIDS) poses a significant challenge to the health care team. Patients may experience numerous complications that compromise nutritional status. The patient is at high risk for opportunistic infections, especially of the lungs, central nervous system, gastrointestinal (GI) tract, and skin. Such infections are common causes of morbidity and mortality. Impaired nutritional status may further impair the patient's immunocompetence. A study by Kotler and Gaety demonstrated severe progressive malnutrition in adult AIDS patients, with the lowest measures of lean body mass occurring in those patients close to death at the time of the study. While no studies of children with AIDS have been done to date, we have subjectively observed feeding problems, weight loss, and malnutrition in most of the patients we have seen.

  19. 'I'm more sick than my doctors think': ethical issues in managing somatization in developing countries.

    Science.gov (United States)

    Chandra, Prabha S; Satyanarayana, Veena A

    2013-02-01

    Several ethical issues confront the healthcare professional who is managing somatization in developing countries where cost constraints, low literacy, poverty, poor nutrition and infections and inadequate access to healthcare are common. The paper discusses these in the context of the ethical principles of autonomy, beneficence, non-maleficence and justice. Some of the ethical issues in managing somatization include being influenced by patient distress rather than rational medical decision-making, inadequate attention to the cultural meaning of symptoms, psychologizing versus medicalizing, the ethics of nomenclature and labels, communicating ethically with patients, and managing them adequately given lack of evidence and training. An ethical approach to managing somatization in this context would include using an integrated and simultaneous medical and psychiatric approach. To ensure patient beneficence, the medical, psychological and social assessment should be undertaken side-by-side as much as possible and should be cost effective. Respecting patient autonomy by using adequate communication methods and the patient's cultural model of the illness as part of management is also integral to ethical practice. In the developing world, issues of equity are also an important ethical concern. When more serious illnesses are the health priority, functional syndromes may not get equal importance or resources.

  20. An ethics model to develop an ethical organisation

    Directory of Open Access Journals (Sweden)

    Hendrik R. Lloyd

    2010-11-01

    Research purpose: The main aim of the paper is to analyse the impact of business ethics in the Eastern Cape Motor Industry Cluster (ECMIC and the different perceptions regarding such ethics. This is based on the main research question, namely, whether a business ethics model should be developed to assist in creating an ethical organisation. Motivation of the study: The motivation for this study is based on the question of whether there is a dedicated drive within the motor industry to establish an ethical organisation and, if such is the case, what benefits would accrue to the organisations in ECMIC. Research design, approach and method: An empirical study was conducted within ECMIC to test the proposed ethics intervention model. A questionnaire, as the main measuring instrument, was developed and 150 questionnaires were distributed. Statistical hypothesis testing was used, with a significance level set at 5%. The aim of the hypothesis testing was to test whether the percentage responses in certain categories were significantly higher than a pre-determined test-value. Main findings: The research results substantiate the fact that the majority of the surveyed organisations do not implement specific ethics interventions. Nevertheless, the majority of respondents acknowledge the importance of ethical behaviour in the organisation, especially with regard to their financial positions. Practical/managerial implications: From this study it became clear that the implementation of a code of ethics would create a platform for ethical behaviour in organisations. It is also concluded from this study that all organisations must strive towards creating an ethical organisation which would have long-term rewards for the organisation, especially from a financial perspective. Contribution/value-add: This study highlights the fact that although ethics and ethical behaviour in organisations are emphasised and receive wide-spread media coverage, not enough action is linked to this task

  1. Ehlers-Danlos syndrome(s) mimicking child abuse: Is there an impact on clinical practice?

    Science.gov (United States)

    Castori, Marco

    2015-12-01

    Ehlers-Danlos syndrome is a heterogeneous group of heritable connective tissue disorders characterized by increased fragility of various non-ossified tissues. It is usually ascertained due to abnormal skin texture, scarring complications, vascular fragility, or chronic symptoms, such as fatigue and musculoskeletal pain. Sometimes, Ehlers-Danlos syndrome remains undetected until the patient, usually in the pediatric age, shows extensive or severe mucocutaneous injuries after only minor traumas. In this scenario, the misdiagnosis of Ehlers-Danlos syndrome with child abuse is a possibility, as occasionally reported in the literature. Recently, more attention was posed by lay people between the possible association of Ehlers-Danlos syndrome and bone fragility. Literature and personal experience show a strong association between Ehlers-Danlos syndrome, generalized joint hypermobility and reduced bone mass density in older children and adults, especially fertile women. The existence of a true increased risk of fracture in Ehlers-Danlos syndrome is still a matter of debate in children and adults with little and conflicting evidence. In case of suspected child abuse, Ehlers-Danlos syndrome is certainly on the differential for bruising, especially in EDS types with marked cutaneous and capillary involvement. In suspected child abuse cases, careful examination of the index case and her/his extended family is routine, as well as exclusion of other disorders such as osteogenesis imperfecta. The hypothesis of Ehlers-Danlos syndrome as an alternative explanation for infantile fractures remains speculative. © 2015 Wiley Periodicals, Inc.

  2. Williams-Beuren's Syndrome: A Case Report.

    Science.gov (United States)

    Zamani, Hassan; Babazadeh, Kazem; Fattahi, Saeid; Mokhtari-Esbuie, Farzad

    2012-01-01

    Williams-Beuren syndrome is a rare familial multisystem disorder occurring in 1 per 20,000 live births. It is characterized by congenital heart defects (CHD), skeletal and renal anomalies, cognitive disorder, social personality disorder and dysmorphic facies. We present a case of Williams syndrome that presented to us with heart murmur and cognitive problem. A 5-year-old girl referred to pediatric cardiologist because of heart murmurs. She had a systolic murmur (2-3/6) in right upper sternal border with radiation to right cervical region. She also had a bulge forehead. Angiography showed mild supra valvular aortic stenosis and mild multiple peripheral pulmonary stenosis. Fluorescent in situ hybridization (FISH) was performed and the result was: 46.XX, ish del (7q11.2) (ELN X1) (7q22 X2) ELN deletion compatible with Williams syndrome. Peripheral pulmonary artery stenosis is associated with Noonan syndrome, Alagille syndrome, Cutis laxa, Ehler-Danlos syndrome, and Silver-Russel syndrome. The patient had peripheral pulmonary artery stenosis, but no other signs of these syndromes were present, and also she had a supravalvular aortic stenosis which was not seen in other syndromes except Williams syndrome. Conclusion. According to primary symptoms, paraclinical and clinical finding such as dysmorphic facies, cognitive disorder and congenital heart defect, Williams syndrome was the first diagnosis. We suggest a more attention for evaluating heart murmur in childhood period, especially when the patient has abnormal facial features or mental problem.

  3. Williams-Beuren's Syndrome: A Case Report

    Directory of Open Access Journals (Sweden)

    Hassan Zamani

    2012-01-01

    Full Text Available Williams-Beuren syndrome is a rare familial multisystem disorder occurring in 1 per 20,000 live births. It is characterized by congenital heart defects (CHD, skeletal and renal anomalies, cognitive disorder, social personality disorder and dysmorphic facies. We present a case of Williams syndrome that presented to us with heart murmur and cognitive problem. A 5-year-old girl referred to pediatric cardiologist because of heart murmurs. She had a systolic murmur (2-3/6 in right upper sternal border with radiation to right cervical region. She also had a bulge forehead. Angiography showed mild supra valvular aortic stenosis and mild multiple peripheral pulmonary stenosis. Fluorescent in situ hybridization (FISH was performed and the result was: 46.XX, ish del (7q11.2 (ELN X1 (7q22 X2 ELN deletion compatible with Williams syndrome. Peripheral pulmonary artery stenosis is associated with Noonan syndrome, Alagille syndrome, Cutis laxa, Ehler-Danlos syndrome, and Silver-Russel syndrome. The patient had peripheral pulmonary artery stenosis, but no other signs of these syndromes were present, and also she had a supravalvular aortic stenosis which was not seen in other syndromes except Williams syndrome. Conclusion. According to primary symptoms, paraclinical and clinical finding such as dysmorphic facies, cognitive disorder and congenital heart defect, Williams syndrome was the first diagnosis. We suggest a more attention for evaluating heart murmur in childhood period, especially when the patient has abnormal facial features or mental problem.

  4. Four Roles of Ethical Theory in Clinical Ethics Consultation.

    Science.gov (United States)

    Magelssen, Morten; Pedersen, Reidar; Førde, Reidun

    2016-09-01

    When clinical ethics committee members discuss a complex ethical dilemma, what use do they have for normative ethical theories? Members without training in ethical theory may still contribute to a pointed and nuanced analysis. Nonetheless, the knowledge and use of ethical theories can play four important roles: aiding in the initial awareness and identification of the moral challenges, assisting in the analysis and argumentation, contributing to a sound process and dialogue, and inspiring an attitude of reflexivity. These four roles of ethical theory in clinical ethics consultation are described and their significance highlighted, while an example case is used as an illustration throughout.

  5. Etiology of pediatric acute liver failure

    Directory of Open Access Journals (Sweden)

    GUO Jing

    2017-10-01

    Full Text Available Pediatric acute liver failure (PALF is a complex syndrome with rapid progression, and the cause of PALF is age-dependent. This article analyzes the common causes of PALF in clinical practice, including infection factors, inherited metabolic factors, poisoning and drugs, abnormal perfusion, and autoimmune diseases, among which infection factors are the most common cause. With the improvement in diagnosis and treatment techniques, the diagnostic rate of PALF caused by inherited metabolic diseases and autoimmune diseases keeps increasing. Due to the small number of PALF patients, there lacks experience in etiological diagnosis. This article summarizes related reports, in order to provide a reference for screening the causes of PALF.

  6. Scrub Typhus - A Major Cause of Pediatric Intensive Care Admission and Multiple Organ Dysfunction Syndrome: A Single-Center Experience from India.

    Science.gov (United States)

    Giri, Prabahs Prasun; Roy, Joydeb; Saha, Agnisekhar

    2018-02-01

    Scrub typhus has been globally recognized as an emerging infectious disease contributing significantly to pyrexia of unknown origin (PUO) and a potential cause of multiorgan dysfunction syndrome (MODS). We studied the incidence of scrub typhus as a cause of pediatric intensive care unit (PICU) admission and MODS in our hospital and its clinical and laboratory characteristics to measure the incidence of MODS caused by scrub typhus. This study was done in a pediatric teaching hospital in Kolkata, India. Records of patients admitted with PUO from March 2012 to December 2015 were reviewed. Rathi-Goodman-Aghai scoring system was used to identify potential ST patients and confirmed by serological testing. Clinical characteristics, laboratory findings, and treatment response were noted of those needing PICU admissions. Ninety-seven cases of scrub typhus have been identified during that period. PICU admission was needed in 30 of them (31%) which contributed 8.43% of total PICU admissions. Among these 30 patients, 16 (53%) developed MODS which contributed 18.29% of total MODS admitted in PICU. Septic shock was the most common manifestation in as many as 18 (60%) patients followed by encephalopathy in 13 (43%). Patients were treated with either doxycycline alone or in combination with azithromycin. Mean time to complete defervescence was 32 h after first dose of doxycycline. The outcome was excellent without a single mortality. Scrub typhus is an important cause of MODS in this part of the world, especially in fevers associated with features as identified and not responding to conventional antibiotics.

  7. Airway Management in a Patient with Wolf-Hirschhorn Syndrome.

    Science.gov (United States)

    Gamble, John F; Kurian, Dinesh J; Udani, Andrea G; Greene, Nathaniel H

    2016-01-01

    We present a case of a 3-month-old female with Wolf-Hirschhorn syndrome (WHS) undergoing general anesthesia for laparoscopic gastrostomy tube placement with a focus on airway management. WHS is a rare 4p microdeletion syndrome resulting in multiple congenital abnormalities, including craniofacial deformities. Microcephaly, micrognathia, and glossoptosis are common features in WHS patients and risk factors for a pediatric airway that is potentially difficult to intubate. We discuss anesthesia strategies for airway preparation and management in a WHS patient requiring general anesthesia with endotracheal intubation.

  8. Pediatric inpatient hospital resource use for congenital heart defects.

    Science.gov (United States)

    Simeone, Regina M; Oster, Matthew E; Cassell, Cynthia H; Armour, Brian S; Gray, Darryl T; Honein, Margaret A

    2014-12-01

    Congenital heart defects (CHDs) occur in approximately 8 per 1000 live births. Improvements in detection and treatment have increased survival. Few national estimates of the healthcare costs for infants, children and adolescents with CHDs are available. We estimated hospital costs for hospitalizations using pediatric (0-20 years) hospital discharge data from the 2009 Healthcare Cost and Utilization Project Kids' Inpatient Database (KID) for hospitalizations with CHD diagnoses. Estimates were up-weighted to be nationally representative. Mean costs were compared by demographic factors and presence of critical CHDs (CCHDs). Up-weighting of the KID generated an estimated 4,461,615 pediatric hospitalizations nationwide, excluding normal newborn births. The 163,980 (3.7%) pediatric hospitalizations with CHDs accounted for approximately $5.6 billion in hospital costs, representing 15.1% of costs for all pediatric hospitalizations in 2009. Approximately 17% of CHD hospitalizations had a CCHD, but it varied by age: approximately 14% of hospitalizations of infants, 30% of hospitalizations of patients aged 1 to 10 years, and 25% of hospitalizations of patients aged 11 to 20 years. Mean costs of CHD hospitalizations were higher in infancy ($36,601) than at older ages and were higher for hospitalizations with a CCHD diagnosis ($52,899). Hospitalizations with CCHDs accounted for 26.7% of all costs for CHD hospitalizations, with hypoplastic left heart syndrome, coarctation of the aorta, and tetralogy of Fallot having the highest total costs. Hospitalizations for children with CHDs have disproportionately high hospital costs compared with other pediatric hospitalizations, and the 17% of hospitalizations with CCHD diagnoses accounted for 27% of CHD hospital costs. © 2014 Wiley Periodicals, Inc.

  9. Are Business Ethics Ethical? Do company ethics live up to what they claim?

    OpenAIRE

    Berge-Venter, Maud-Ellen

    2013-01-01

    The following Master's thesis is an analysis of the terms used in both the ethical guidelines and values espoused by companies, weighed up against classical philosophical texts and normative ethical theories, as well as traditional business ethics.

  10. Pediatrics in 21st Century and Beyond.

    Science.gov (United States)

    Singh, Meharban

    2016-11-01

    Pediatrics is a dynamic discipline and there is awareness and hope for actualizing outstanding achievements in the field of child health in 21 st century and beyond. Improved lifestyle and quality of children's health is likely to reduce the burden of adult diseases and enhance longevity because seeds of most adult diseases are sown in childhood. Identification and decoding of human genome is expected to revolutionize the practice of pediatrics. The day is not far off when a patient will walk into doctor's chamber with an electronic or digital health history on a CD or palmtop and a decoded genomic constitution. There will be reduced burden of genetic diseases because of selective abortions of "defective" fetuses and replacement of "bad" genes with "good" ones by genetic engineering. Availability of totipotent stem cells and developments in transplant technology are likely to revolutionize the management of a variety of hematologic cancers and life-threatening genetic disorders. The possibility of producing flawless designer babies by advances in assisted reproductive technologies (ARTs) is likely to be mired by several ethical and legal issues.The availability of newer vaccines by recombinant technology for emerging infective and for non-infective lifestyle diseases is likely to improve survival and quality of life. There is going to be a greater focus on the "patient" having the disease rather than "disease" per se by practicing holistic pediatrics by effective utilization of alternative or complementary strategies for health care. Due to advances in technology, pediatrics may get further dehumanized. A true healer cannot simply rely on technology; there must be a spiritual bond between the patient and the physician by exploiting the concept of psycho-neuro-immunology and body-mind interactions. In the years to come, physicians are likely to play "god" but medicine can't achieve immortality because anything born must die in accordance with nature's recycling

  11. Commentary: Pediatric eHealth Interventions: Common Challenges During Development, Implementation, and Dissemination

    Science.gov (United States)

    Steele, Ric G.; Connelly, Mark A.; Palermo, Tonya M.; Ritterband, Lee M.

    2014-01-01

    Objective To provide an overview of common challenges that pediatric eHealth researchers may encounter when planning, developing, testing, and disseminating eHealth interventions along with proposed solutions for addressing these challenges. Methods The article draws on the existing eHealth literature and the authors’ collective experience in pediatric eHealth research. Results and conclusions The challenges associated with eHealth interventions and their proposed solutions are multifaceted and cut across a number of areas from eHealth program development through dissemination. Collaboration with a range of individuals (e.g., multidisciplinary colleagues, commercial entities, primary stakeholders) is the key to eHealth intervention success. To ensure adequate resources for design, development, and planning for sustainability, a number of public and private sources of funding are available. A study design that addresses ethical concerns and security issues is critical to ensure scientific integrity and intervention dissemination. Table I summarizes key issues to consider during eHealth intervention development, testing, and dissemination. PMID:24816766

  12. The prevalence of metabolic syndrome and its components among ...

    African Journals Online (AJOL)

    Background: The incidence of metabolic syndrome (MetS), is rapidly increasing in developing countries. However, the epidemiology of MetS is not well reported in the pediatric and young adult population. We determined the prevalence of MetS and its components among overweight and obese Nigerian adolescents and ...

  13. Onset of action and seizure control in Lennox-Gaustaut syndrome: focus on rufinamide

    OpenAIRE

    Saneto, Russell P; Anderson, Gail D

    2009-01-01

    Russell P Saneto1, Gail D Anderson21Division of Pediatric Neurology, Seattle Children’s Hospital/University of Washington, Seattle, Washington, USA; 2Department of Pharmacy, University of Washington, Seattle, Washington, USAAbstract: Lennox-Gaustaut syndrome is an electroclinical epilepsy syndrome characterized by the triad of electroencephalogram showing diffuse slow spike-and-wave discharges and paroxysmal fast activity, multiple intractable seizure types, and cognitive impairment...

  14. Outcomes of renal replacement therapy in boys with prune belly syndrome

    DEFF Research Database (Denmark)

    Yalcinkaya, Fatos; Bonthuis, Marjolein; Erdogan, Beyza Doganay

    2018-01-01

    BACKGROUND: As outcome data for prune belly syndrome (PBS) complicated by end-stage renal disease are scarce, we analyzed characteristics and outcomes of children with PBS using the European Society for Pediatric Nephrology/European Renal Association-European Dialysis and Transplant Association...

  15. Outcomes of renal replacement therapy in boys with prune belly syndrome

    DEFF Research Database (Denmark)

    Yalcinkaya, Fatos; Bonthuis, Marjolein; Erdogan, Beyza Doganay

    2018-01-01

    BACKGROUND: As outcome data for prune belly syndrome (PBS) complicated by end-stage renal disease are scarce, we analyzed characteristics and outcomes of children with PBS using the European Society for Pediatric Nephrology/European Renal Association-European Dialysis and Transplant Association (...

  16. Pediatric radiological diagnostics in suspected child abuse

    International Nuclear Information System (INIS)

    Erfurt, C.; Schmidt, U.; Hahn, G.; Roesner, D.

    2009-01-01

    Advanced and specialized radiological diagnostics are essential in the case of clinical suspicion of pediatric injuries to the head, thorax, abdomen, and extremities when there is no case history or when ''battered child syndrome'' is assumed on the basis of inadequate trauma. In particular, the aim of this sophisticated diagnostic procedure is the detection of lesions of the central nervous system (CNS) in order to initiate prompt medical treatment. If diagnostic imaging shows typical findings of child abuse, accurate documented evidence of the diagnostic results is required to prevent further endangerment of the child's welfare. (orig.) [de

  17. Diagnostic Dilemma of Cardiac Syncope in Pediatric Patients

    Directory of Open Access Journals (Sweden)

    Ranya A Hegazy

    2008-02-01

    Full Text Available Aims Syncope is defined as temporary loss of consciousness and postural tone resulting from an abrupt transient decrease in cerebral blood flow. The present work aimed at determining how diagnostic tests are used in the evaluation of pediatric syncope at a tertiary pediatric referral center and to report on the utility and the yield of these tests.Settings and Design Retrospective study conducted at a tertiary referral arrhythmolology serviceMethods and Material The clinical charts of 234 pediatric patients presenting with a primary complaint of syncope with an average age of 7.48 ± 3.82(3.5-16 years were reviewed by the investigators.Statistical analysis used Statistical Package of social science (SPSS version 9,0 was used for analysis of data.Results The commonest trigger for syncope in the study population was early following exercise (n=65 and the commonest prodrome was palpitation, noted in 25 patients. A murmur was present in 19 of our patients (8.3% while 10.7% (n=25 had abnormal ECGs. Of the 106 echocardiograms done, 14 (13.2% were abnormal. Only two of them were missed by ECG. All patients were offered ambulatory 24 hour ECG. One patient with sick sinus syndrome was diagnosed only with Holter.Conclusions Clues to the presence of cardiac syncope may include acute onset of syncope, frequent episodes, low difference between blood pressure readings in supine and erect positions (after standing for 2 minutes and most importantly an abnormal 12 lead ECG. Transthoracic echo and Holter monitoring have low yield in pediatric syncope.

  18. The Ethical Dilemmas and Ethical Choices in Atonement

    Institute of Scientific and Technical Information of China (English)

    陈晶

    2017-01-01

    Atonement, as the representative work of British writer Ian McEwan, is of high educational significance. This article wil adopt Ethical Literary Criticism to analyze the ethical dilemmas and ethical choices of two characters in the novel: Briony and Robbie. By putting the characters into diversified ethical problems, the writer has strengthened the necessity of reflection and atonement in real life, and also the moral responsibilities one should bear. The analysis of characters' ethical problems can better highlight the writer's moral value.

  19. [Is personalism or utilitarianism an adequate foundation of medical ethics?].

    Science.gov (United States)

    Biesaga, T

    1998-01-01

    The article rejects utilitarianism as a proper theory for medical ethics. Utilitarians lavishly use various slogans of effective action, development and better civilization. However, the principle of prosperity of humanity in the utilitarian interpretation makes the value of the human person subject to society. Social interest threatens the individual here because it defines his/her value of life. The drift towards maximalization of benefits and prosperity of humanity strikes the seriously ill, e.g. babies with brain damages, Down's syndrome, etc., people after accidents and with serious brain defects, the terminally ill. The principle of quality of life (lebensunwertes Leben) used by utylitarians allows them to argue, that euthanasia, abortion is in the interest of the patient. Some utilitarians openly admit that such ideas as "universal happiness", "prosperity", "benefit" are empty ideas, fictions to which one cannot attribute any contents. So utilitarianism, not defining its fundamental ideas, can easily change medical ethics in a theory of elimination of the uncomfortable people. Therefore, as a theory utilitarianism cannot serve as the basis for medical ethics.

  20. Use and reimbursement of off-label drugs in pediatric anesthesia: the Italian experience.

    Science.gov (United States)

    Salvo, Ida; Landoni, Giovanni; Mucchetti, Marta; Cabrini, Luca; Pani, Luca

    2014-06-01

    Most of the drugs used in anesthesia are off-label in children even if they present solid clinical evidence in adults. This lack of authorization is caused by multiple factors including the difficulty in conducting research in this area (due to the ethical concerns and/or the low number of available participants, the high variability of the outcome measures) and the lack of economic interest of the pharmaceutical companies (due to the limited market). Define a list of medicinal products commonly used off-label in pediatrics anesthesia to be reimbursed by Italian National Health System. We hereby describe the methodological framework used to allow reimbursed use of a list of medicinal products, widely used off-label in pediatric patients, ensuring the best therapeutic results with the lowest possible risk for children. A task force of pediatric anesthesiologists from Italy petitioned the Italian Medicines Agency (AIFA) to allow a number of commonly utilized but off-label drugs for pediatric anesthesia to be reimbursed for specific indications. For each drug, both the supporting literature and expert opinion were used, and the resulting list of drugs allowed to be used/reimbursed officially by AIFA was significantly expanded. This paper documents one approach to the problem of off-label use of drugs for pediatric patients that can be a model for future efforts. Continuous efforts are needed from government institutions and sponsors on drug development and on drug approval process in pediatrics, as research on drug effectiveness and safety is mandatory in children as in adults. At the same time, clinicians must become more familiar with the drug-approval process, participate to sponsored trials, and perform ztrials themselves. © 2014 John Wiley & Sons Ltd.

  1. Ethical Conduct of Research in Children: Pediatricians and Their IRB (Part 1 of 2).

    Science.gov (United States)

    Rose, Carlos D

    2017-05-01

    As human experimentation continues to grow into an ever more complex and sophisticated endeavor, the relevant ethical and regulatory structures become more intricate. When pediatricians and general practitioners are invited by pharmaceutical companies to enroll their offices in a clinical trial or a multicenter observational study or when they develop their own research questions, they frequently find themselves at a loss in the human research environment. The legal and regulatory complexity may have an unintended deterring effect at a time when office-based high quality pediatric research is urgently needed to support evidence-based medicine. Unfortunately, in many instances, unaware practitioners become involved in low-risk research activities without knowing it and become entangled in legal, auditing, and compliance procedures. This paper, written in 2 parts, aims at providing a general guidance on the principles that regulate human research with a focus on pediatrics. Part 1 discusses the history, the legal framework, and the consent process and highlights some practical aspects of initial protocol submission, continued review, and institutional review board determinations with the main focus on multicenter clinical trials (industry-sponsored research). Part 2 focuses on pediatric research regulation, also known as subpart-D, and minimal risk research, which encompasses many research activities aimed at addressing questions that may emerge in pediatricians' practices (investigator-initiated research). Copyright © 2017 by the American Academy of Pediatrics.

  2. Canadian Guidelines for Controlled Pediatric Donation After Circulatory Determination of Death—Summary Report*

    Science.gov (United States)

    Hornby, Laura; Rochwerg, Bram; van Manen, Michael; Dhanani, ; Sonny; Sivarajan, V. Ben; Appleby, Amber; Bennett, Mary; Buchman, Daniel; Farrell, Catherine; Goldberg, Aviva; Greenberg, Rebecca; Singh, Ram; Nakagawa, Thomas A.; Witteman, William; Barter, Jill; Beck, Allon; Coughlin, Kevin; Conradi, Alf; Cupido, Cynthia; Dawson, Rosanne; Dipchand, Anne; Freed, Darren; Hornby, Karen; Langlois, Valerie; Mack, Cheryl; Mahoney, Meagan; Manhas, Deepak; Tomlinson, Christopher; Zavalkoff, Samara; Shemie, Sam D.

    2017-01-01

    Objectives: Create trustworthy, rigorous, national clinical practice guidelines for the practice of pediatric donation after circulatory determination of death in Canada. Methods: We followed a process of clinical practice guideline development based on World Health Organization and Canadian Medical Association methods. This included application of Grading of Recommendations Assessment, Development, and Evaluation methodology. Questions requiring recommendations were generated based on 1) 2006 Canadian donation after circulatory determination of death guidelines (not pediatric specific), 2) a multidisciplinary symposium of national and international pediatric donation after circulatory determination of death leaders, and 3) a scoping review of the pediatric donation after circulatory determination of death literature. Input from these sources drove drafting of actionable questions and Good Practice Statements, as defined by the Grading of Recommendations Assessment, Development, and Evaluation group. We performed additional literature reviews for all actionable questions. Evidence was assessed for quality using Grading of Recommendations Assessment, Development, and Evaluation and then formulated into evidence profiles that informed recommendations through the evidence-to-decision framework. Recommendations were revised through consensus among members of seven topic-specific working groups and finalized during meetings of working group leads and the planning committee. External review was provided by pediatric, critical care, and critical care nursing professional societies and patient partners. Results: We generated 63 Good Practice Statements and seven Grading of Recommendations Assessment, Development, and Evaluation recommendations covering 1) ethics, consent, and withdrawal of life-sustaining therapy, 2) eligibility, 3) withdrawal of life-sustaining therapy practices, 4) ante and postmortem interventions, 5) death determination, 6) neonatal pediatric donation

  3. Canadian Guidelines for Controlled Pediatric Donation After Circulatory Determination of Death-Summary Report.

    Science.gov (United States)

    Weiss, Matthew J; Hornby, Laura; Rochwerg, Bram; van Manen, Michael; Dhanani, Sonny; Sivarajan, V Ben; Appleby, Amber; Bennett, Mary; Buchman, Daniel; Farrell, Catherine; Goldberg, Aviva; Greenberg, Rebecca; Singh, Ram; Nakagawa, Thomas A; Witteman, William; Barter, Jill; Beck, Allon; Coughlin, Kevin; Conradi, Alf; Cupido, Cynthia; Dawson, Rosanne; Dipchand, Anne; Freed, Darren; Hornby, Karen; Langlois, Valerie; Mack, Cheryl; Mahoney, Meagan; Manhas, Deepak; Tomlinson, Christopher; Zavalkoff, Samara; Shemie, Sam D

    2017-11-01

    Create trustworthy, rigorous, national clinical practice guidelines for the practice of pediatric donation after circulatory determination of death in Canada. We followed a process of clinical practice guideline development based on World Health Organization and Canadian Medical Association methods. This included application of Grading of Recommendations Assessment, Development, and Evaluation methodology. Questions requiring recommendations were generated based on 1) 2006 Canadian donation after circulatory determination of death guidelines (not pediatric specific), 2) a multidisciplinary symposium of national and international pediatric donation after circulatory determination of death leaders, and 3) a scoping review of the pediatric donation after circulatory determination of death literature. Input from these sources drove drafting of actionable questions and Good Practice Statements, as defined by the Grading of Recommendations Assessment, Development, and Evaluation group. We performed additional literature reviews for all actionable questions. Evidence was assessed for quality using Grading of Recommendations Assessment, Development, and Evaluation and then formulated into evidence profiles that informed recommendations through the evidence-to-decision framework. Recommendations were revised through consensus among members of seven topic-specific working groups and finalized during meetings of working group leads and the planning committee. External review was provided by pediatric, critical care, and critical care nursing professional societies and patient partners. We generated 63 Good Practice Statements and seven Grading of Recommendations Assessment, Development, and Evaluation recommendations covering 1) ethics, consent, and withdrawal of life-sustaining therapy, 2) eligibility, 3) withdrawal of life-sustaining therapy practices, 4) ante and postmortem interventions, 5) death determination, 6) neonatal pediatric donation after circulatory

  4. Nootropic and antiamnestic effects of tenoten (pediatric formulation) in immature rat pups.

    Science.gov (United States)

    Voronina, T A; Molodavkin, G M; Borodavkina, M V; Kheyfets, I A; Dugina, Yu L; Sergeeva, S A

    2009-09-01

    The antiamnestic effects of tenoten (pediatric formulation) was demonstrated on the model of scopolamine-induced amnesia of passive avoidance reflex and the nootropic effect of this preparation was demonstrated on the model of incomplete conditioning and in rat pups with experimental attention deficit syndrome. The efficiency of the preparation was comparable to that of piracetam and phenibut and even surpassed it by some parameters.

  5. The Contribution of Islamic Ethics Towards Ethical Accounting Practices

    Directory of Open Access Journals (Sweden)

    Rochania Ayu Yunanda

    2011-12-01

    and also increase public confidence in the profession. However, the efforts to integrate ethical values in educational system will not work well if there are no moral commitments implanted in the individuals.  Islam with its divine values plays the notable role to embed cognitive ethical values. It emphasizes on the unity of God, the accountability to God and the concept of maslahah (public benefits to be the foundations of ethics. Incorporating Islamic ethics into the system will be a significant contribution towards generating ethical accounting education. This paper attempts to elucidate how the Islamic ethics contribute its role towards ethical accountants as the products of accounting education.

  6. Pediatric Asthma

    Science.gov (United States)

    ... Science Education & Training Home Conditions Asthma (Pediatric) Asthma (Pediatric) Make an Appointment Refer a Patient Ask a ... meet the rising demand for asthma care. Our pediatric asthma team brings together physicians, nurses, dietitians, physical ...

  7. Heritage ethics: Toward a thicker account of nursing ethics.

    Science.gov (United States)

    Fowler, Marsha D

    2016-02-01

    The key to understanding the moral identity of modern nursing and the distinctiveness of nursing ethics resides in a deeper examination of the extensive nursing ethics literature and history from the late 1800s to the mid 1960s, that is, prior to the "bioethics revolution". There is a distinctive nursing ethics, but one that falls outside both biomedical and bioethics and is larger than either. Were, there a greater corpus of research on nursing's heritage ethics it would decidedly recondition the entire argument about a distinctive nursing ethics. It would also provide a thicker account of nursing ethics than has been afforded thus far. Such research is dependent upon identifying, locating, accessing and, more importantly, sharing these resources. A number of important heritage ethics sources are identified so that researchers might better locate them. In addition, a bibliography of heritage ethics textbooks and a transcript of the earliest known journal article on nursing ethics in the US are provided. © The Author(s) 2015.

  8. Circulating dendritic cells in pediatric patients with nephrotic syndrome

    African Journals Online (AJOL)

    EL-HAKIM

    nephrotic syndrome, circulating DCs were measured by flowcytometry. Results: Circulating DC count ... parents or caregivers of each child before enrollment in the study. ..... role in initiating the primary immune response. On the basis of the ...

  9. Pain Amplification Syndrome: A Biopsychosocial Approach.

    Science.gov (United States)

    Namerow, Lisa B; Kutner, Emily C; Wakefield, Emily C; Rzepski, Barbara R; Sahl, Robert A

    2016-08-01

    Pediatric neurologists frequently encounter patients who present with significant musculoskeletal pain that cannot be attributed to a specific injury or illness, which can often be defined as pain amplification syndrome (PAS). PAS in children and adolescents is the result of a heightened pain sensitivity pathway, which is intensified by significant biological, psychological, and social contributors. Appropriate assessment and multimodal intervention of PAS are crucial to treatment success, including neurology and behavioral health collaborative treatment plans to restore patient function and reduce pain perception. Pediatric neurologists are imperative in the identification of patients with PAS, providing the family assurance in diagnosis and validation of pain, and directing patients to the appropriate multidisciplinary treatment pathway. Copyright © 2016 Elsevier Inc. All rights reserved.

  10. Research ethics in dissertations: ethical issues and complexity of reasoning.

    Science.gov (United States)

    Kjellström, S; Ross, S N; Fridlund, B

    2010-07-01

    Conducting ethically sound research is a fundamental principle of scientific inquiry. Recent research has indicated that ethical concerns are insufficiently dealt with in dissertations. To examine which research ethical topics were addressed and how these were presented in terms of complexity of reasoning in Swedish nurses' dissertations. Analyses of ethical content and complexity of ethical reasoning were performed on 64 Swedish nurses' PhD dissertations dated 2007. A total of seven ethical topics were identified: ethical approval (94% of the dissertations), information and informed consent (86%), confidentiality (67%), ethical aspects of methods (61%), use of ethical principles and regulations (39%), rationale for the study (20%) and fair participant selection (14%). Four of those of topics were most frequently addressed: the majority of dissertations (72%) included 3-5 issues. While many ethical concerns, by their nature, involve systematic concepts or metasystematic principles, ethical reasoning scored predominantly at lesser levels of complexity: abstract (6% of the dissertations), formal (84%) and systematic (10%). Research ethics are inadequately covered in most dissertations by nurses in Sweden. Important ethical concerns are missing, and the complexity of reasoning on ethical principles, motives and implications is insufficient. This is partly due to traditions and norms that discount ethical concerns but is probably also a reflection of the ability of PhD students and supervisors to handle complexity in general. It is suggested that the importance of ethical considerations should be emphasised in graduate and post-graduate studies and that individuals with capacity to deal with systematic and metasystematic concepts are recruited to senior research positions.

  11. Teaching Business Ethics or Teaching Business Ethically?

    Science.gov (United States)

    Stablein, Ralph

    2003-01-01

    Notes that one of the most important contexts for ethical decision-making is the nature and operation of "contemporary capitalisms." Suggests that rather than issuing a call for teaching business ethics, the author emphasizes the need for more ethical business teaching. (SG)

  12. Ethics and Ethical Theories from an Islamic Perspective

    Directory of Open Access Journals (Sweden)

    AL-HASAN AL-AIDAROS

    2013-12-01

    Full Text Available With the collapse of many organizations, many researchers are increasingly paying attention to such phenomenon. But ethical issues are not always clear cut; there are many grey areas that need to be threaded with care by organizations. To determine whether an action or decision is ethically carried out, ethical theories, developed mainly by Western scholars, are the current theoretical framework organizations have at their disposal. Theories such as relativism, utilitarianism, egoism, deontology, the divine command theory, and the virtue ethics, are all products of Western understanding of what ethics are and how they are applicable to help one’s decision making process. Despite their utility, this paper intends to argue that the Western concepts and understanding of what ethics are limited and incomprehensive in explaining what is right and what is wrong. In its place, this paper argues that to understand the concepts of ethics that can extend beyond time and space. It has to be analysed from an Islamic perspective. Toward this purpose, this paper will compare and contrast between Islamic and Western perspectives of ethics, and highlight the main weaknesses and limitations of the former. Then, an argument on why Islam can provide the best understanding of ethics will be made.

  13. Pediatric Sinusitis

    Science.gov (United States)

    ... ENTCareers Marketplace Find an ENT Doctor Near You Pediatric Sinusitis Pediatric Sinusitis Patient Health Information News media interested in ... sinuses are present at birth. Unlike in adults, pediatric sinusitis is difficult to diagnose because symptoms of ...

  14. Peter Koslowski’s Ethics and Economics or Ethical Economy: A Framework for a research agenda in business ethics

    Directory of Open Access Journals (Sweden)

    Jacob Dahl Rendtorff

    2016-03-01

    Full Text Available This paper presents the concept of ethical economy (Wirtschaftsethik and the relation between ethics and economics on the basis of the work of the German ethical economist Peter Koslowski. The concept of ethical economy includes three levels: micro, meso and macro levels; and it also deals with the philosophical analysis of the ethical foundations of the economy. After the discussion of these elements of the ethical economy, the paper presents some possible research topics for a research agenda about economic ethics or ethical economy.

  15. Pediatric imaging essentials. Radiography, ultrasound, CT, and MRI in neonates and children

    International Nuclear Information System (INIS)

    Riccabona, Michael

    2014-01-01

    For all radiologists treating infants and children, knowledge of best practices in pediatric imaging is essential to safely obtaining high-quality images and achieving accurate diagnoses. This practical text covers current guidelines and key topics in the field, including choice of modality, equipment and dosages, child-specific diseases, typical imaging findings, differential diagnostic aspects and safety factors. This book is invaluable for all clinicians and radiologists who diagnose and manage this sensitive population. Special features: - Explores the use of all standard imaging modalities in children as compared to adults, especially with regard to ultrasound, CT, and MRI - Supplies more than 600 high-quality images to help in interpreting findings, including imaging of suspected child abuse - Shows how to adapt examination protocols and equipment requirements for the specialized needs of pediatric patients - Describes important safety protection measures in children utilizing the ALARA principle of radiation exposure (''As Low As Reasonably Achievable'') - Summarizes a wide array of pediatric diseases and disorders in a concise, checklist format, including clinical features, imaging findings, differential diagnosis, associated syndromes, and treatment recommendations - Includes lists of indications, summary tables, imaging protocols, case studies, and quiz questions to test your knowledge This book provides a fundamental understanding of imaging in infants and children and is an ideal, practice-oriented reference for residents, fellows in pediatric radiology, and general radiologists. It is also written for pediatricians, pediatric surgeons, and other interested doctors and specialists who want to know more about imaging specifics in the pediatric age group.

  16. Pediatric imaging essentials. Radiography, ultrasound, CT, and MRI in neonates and children

    Energy Technology Data Exchange (ETDEWEB)

    Riccabona, Michael (ed.) [Graz University Hospital (Austria). Div. of Pediatric Radiology

    2014-03-01

    For all radiologists treating infants and children, knowledge of best practices in pediatric imaging is essential to safely obtaining high-quality images and achieving accurate diagnoses. This practical text covers current guidelines and key topics in the field, including choice of modality, equipment and dosages, child-specific diseases, typical imaging findings, differential diagnostic aspects and safety factors. This book is invaluable for all clinicians and radiologists who diagnose and manage this sensitive population. Special features: - Explores the use of all standard imaging modalities in children as compared to adults, especially with regard to ultrasound, CT, and MRI - Supplies more than 600 high-quality images to help in interpreting findings, including imaging of suspected child abuse - Shows how to adapt examination protocols and equipment requirements for the specialized needs of pediatric patients - Describes important safety protection measures in children utilizing the ALARA principle of radiation exposure (''As Low As Reasonably Achievable'') - Summarizes a wide array of pediatric diseases and disorders in a concise, checklist format, including clinical features, imaging findings, differential diagnosis, associated syndromes, and treatment recommendations - Includes lists of indications, summary tables, imaging protocols, case studies, and quiz questions to test your knowledge This book provides a fundamental understanding of imaging in infants and children and is an ideal, practice-oriented reference for residents, fellows in pediatric radiology, and general radiologists. It is also written for pediatricians, pediatric surgeons, and other interested doctors and specialists who want to know more about imaging specifics in the pediatric age group.

  17. Histiocytoid Sweet Syndrome in a Child without Underlying Systemic Disease.

    Science.gov (United States)

    Yeom, Seung Dohn; Ko, Hye Soo; Moon, Jong Hyuk; Kang, Min Ji; Byun, Ji Won; Choi, Gwang Seong; Shin, Jeonghyun

    2017-10-01

    Sweet syndrome (acute, febrile, neutrophilic dermatosis) is characterized by the acute onset of an eruption of painful nodules or erythematous or violaceous plaques on the limbs, face and neck. These symptoms are accompanied by fever. The diagnostic features include histopathological findings of dermal neutrophilic infiltration without leukocytoclastic vasculitis or peripheral blood leukocytosis. Sweet syndrome is associated with infection, malignancies, autoimmune disease, pregnancy, and drugs. Patients with Sweet syndrome demonstrate a complete and rapid response to systemic steroid administration. Recently, a distinct variant of Sweet syndrome was reported, termed "histiocytoid Sweet syndrome", in which the infiltration of myeloperoxidase-positive histiocytoid mononuclear cells are observed (in contrast to the infiltration of neutrophils). The other clinical features are similar to those of classic Sweet syndrome. Pediatric Sweet syndrome is uncommon, and the histiocytoid type is even rarer. To date, four cases of histiocytoid Sweet syndrome have been reported in children. Herein, we describe a case of histiocytoid Sweet syndrome in an otherwise healthy 10-year-old boy with no underlying systemic disease in whom non-steroidal, anti-inflammatory drug treatment was successful.

  18. Hutchinson-Gilford Progeria Syndrome

    Directory of Open Access Journals (Sweden)

    Gopal G

    2014-08-01

    Full Text Available Hutchinson-Gilford Progeria syndrome (HGPS is a rare pediatric genetic syndrome associated with a characteristic aged appearance very early in life, generally leading to death in the second decade of life. Apart from premature aging, the other notable characteristics of children with HGPS include extreme short stature, prominent superficial veins, poor weight gain, alopecia, as well as various skeletal and cardiovascular pathologies associated with advanced age. The pattern of inheritance of HGPS is uncertain, though both autosomal dominant and autosomal recessive modes have been described. Recent genetic studies have demonstrated mutations in the LMNA gene in children with HGPS. In this article, we report a 16 years old girl who had the phenotypic features of HGPS and was later confirmed to have LMNA mutation by genetic analysis.

  19. (The Ethics of) Teaching Science and Ethics: A Collaborative Proposal.

    Science.gov (United States)

    Kabasenche, William P

    2014-12-01

    I offer a normative argument for a collaborative approach to teaching ethical issues in the sciences. Teaching science ethics requires expertise in at least two knowledge domains-the relevant science(s) and philosophical ethics. Accomplishing the aims of ethics education, while ensuring that science ethics discussions remain grounded in the best empirical science, can generally best be done through collaboration between a scientist and an ethicist. Ethics as a discipline is in danger of being misrepresented or distorted if presented by someone who lacks appropriate disciplinary training and experience. While there are exceptions, I take philosophy to be the most appropriate disciplinary domain in which to gain training in ethics teaching. Science students, who must be prepared to engage with many science ethics issues, are poorly served if their education includes a misrepresentation of ethics or specific issues. Students are less well prepared to engage specific issues in science ethics if they lack an appreciation of the resources the discipline of ethics provides. My collaborative proposal looks at a variety of ways scientists and ethicists might collaborate in the classroom to foster good science ethics education.

  20. (The Ethics of Teaching Science and Ethics: A Collaborative Proposal

    Directory of Open Access Journals (Sweden)

    William P. Kabasenche

    2014-10-01

    Full Text Available I offer a normative argument for a collaborative approach to teaching ethical issues in the sciences. Teaching science ethics requires expertise in at least two knowledge domains—the relevant science(s and philosophical ethics. Accomplishing the aims of ethics education, while ensuring that science ethics discussions remain grounded in the best empirical science, can generally best be done through collaboration between a scientist and an ethicist. Ethics as a discipline is in danger of being misrepresented or distorted if presented by someone who lacks appropriate disciplinary training and experience. While there are exceptions, I take philosophy to be the most appropriate disciplinary domain in which to gain training in ethics teaching. Science students, who must be prepared to engage with many science ethics issues, are poorly served if their education includes a misrepresentation of ethics or specific issues. Students are less well prepared to engage specific issues in science ethics if they lack an appreciation of the resources the discipline of ethics provides. My collaborative proposal looks at a variety of ways scientists and ethicists might collaborate in the classroom to foster good science ethics education.

  1. Behavioral Ethics and Teaching Ethical Decision Making

    Science.gov (United States)

    Drumwright, Minette; Prentice, Robert; Biasucci, Cara

    2015-01-01

    Business education often renders students less likely to act ethically. An infusion of liberal learning in the form of behavioral ethics could improve this situation by prompting students to develop higher levels of professionalism that encompass ethics, social responsibility, self-critical reflection, and personal accountability. More…

  2. Somatic mosaicism underlies X-linked acrogigantism syndrome in sporadic male subjects

    NARCIS (Netherlands)

    A.F. Daly (Adrian); B. Yuan (Bo); Fina, F. (Frederic); J.-H. Caberg (Jean-Hubert); G. Trivellin (Giampaolo); L. Rostomyan (Liliya); W.W. de Herder (Wouter); L.A. Naves (Lucianna); D. Metzger (Daniel); T. Cuny (Thomas); Rabl, W. (Wolfgang); N.S. Shah (Nalini Samir); M-L. Jaffrain-Rea (Marie-Lise); Chiara Zatelli, M. (Maria); F.R. Faucz (Fabio R.); E. Castermans (Emilie); Nanni-Metellus, I. (Isabelle); Lodish, M. (Maya); A. Muhammad (Ammar); Palmeira, L. (Leonor); Potorac, I. (Iulia); G. Mantovani (Giovanna); S.J.C.M.M. Neggers (Bas); Klein, M. (Marc); A. Barlier (Anne); P. Liu (Pengfei); Ouafik, L. (L'houcine); V. Bours (Vincent); Lupski, J.R. (James R.); C.A. Stratakis (Constantine); A. Beckers (Albert)

    2016-01-01

    textabstractSomatic mosaicism has been implicated as a causative mechanism in a number of genetic and genomic disorders. X-linked acrogigantism (XLAG)syndrome is a recently characterized genomic form of pediatric gigantism due to aggressive pituitary tumors that is caused by submicroscopic

  3. Expertise, Ethics Expertise, and Clinical Ethics Consultation: Achieving Terminological Clarity

    Science.gov (United States)

    Iltis, Ana S.; Sheehan, Mark

    2016-01-01

    The language of ethics expertise has become particularly important in bioethics in light of efforts to establish the value of the clinical ethics consultation (CEC), to specify who is qualified to function as a clinical ethics consultant, and to characterize how one should evaluate whether or not a person is so qualified. Supporters and skeptics about the possibility of ethics expertise use the language of ethics expertise in ways that reflect competing views about what ethics expertise entails. We argue for clarity in understanding the nature of expertise and ethics expertise. To be an ethics expert, we argue, is to be an expert in knowing what ought to be done. Any attempt to articulate expertise with respect to knowing what ought to be done must include an account of ethics that specifies the nature of moral truth and the means by which we access this truth or a theoretical account of ethics such that expertise in another domain is linked to knowing or being better at judging what ought to be done and the standards by which this “knowing” or “being better at judging” is determined. We conclude with a discussion of the implications of our analysis for the literature on ethics expertise in CEC. We do think that there are clear domains in which a clinical ethics consultant might be expert but we are skeptical about the possibility that this includes ethics expertise. Clinical ethics consultants should not be referred to as ethics experts. PMID:27256848

  4. Large ethics.

    Science.gov (United States)

    Chambers, David W

    2008-01-01

    This essay presents an alternative to the traditional view that ethics means judging individual behavior against standards of right and wrong. Instead, ethics is understood as creating ethical communities through the promises we make to each other. The "aim" of ethics is to demonstrate in our own behavior a credible willingness to work to create a mutually better world. The "game" of ethics then becomes searching for strategies that overlap with others' strategies so that we are all better for intending to act on a basis of reciprocal trust. This is a difficult process because we have partial, simultaneous, shifting, and inconsistent views of the world. But despite the reality that we each "frame" ethics in personal terms, it is still possible to create sufficient common understanding to prosper together. Large ethics does not make it a prerequisite for moral behavior that everyone adheres to a universally agreed set of ethical principles; all that is necessary is sufficient overlap in commitment to searching for better alternatives.

  5. Perspectives on Applied Ethics

    OpenAIRE

    2007-01-01

    Applied ethics is a growing, interdisciplinary field dealing with ethical problems in different areas of society. It includes for instance social and political ethics, computer ethics, medical ethics, bioethics, envi-ronmental ethics, business ethics, and it also relates to different forms of professional ethics. From the perspective of ethics, applied ethics is a specialisation in one area of ethics. From the perspective of social practice applying eth-ics is to focus on ethical aspects and ...

  6. Airway Management in a Patient with Wolf-Hirschhorn Syndrome

    Directory of Open Access Journals (Sweden)

    John F. Gamble

    2016-01-01

    Full Text Available We present a case of a 3-month-old female with Wolf-Hirschhorn syndrome (WHS undergoing general anesthesia for laparoscopic gastrostomy tube placement with a focus on airway management. WHS is a rare 4p microdeletion syndrome resulting in multiple congenital abnormalities, including craniofacial deformities. Microcephaly, micrognathia, and glossoptosis are common features in WHS patients and risk factors for a pediatric airway that is potentially difficult to intubate. We discuss anesthesia strategies for airway preparation and management in a WHS patient requiring general anesthesia with endotracheal intubation.

  7. Ethics, health policy, and Zika: From emergency to global epidemic?

    Science.gov (United States)

    Jamrozik, Euzebiusz; Selgelid, Michael J

    2018-05-01

    Zika virus was recognised in 2016 as an important vector-borne cause of congenital malformations and Guillain-Barré syndrome, during a major epidemic in Latin America, centred in Northeastern Brazil. The WHO and Pan American Health Organisation (PAHO), with partner agencies, initiated a coordinated global response including public health intervention and urgent scientific research, as well as ethical analysis as a vital element of policy design. In this paper, we summarise the major ethical issues raised during the Zika epidemic, highlighting the PAHO ethics guidance and the role of ethics in emergency responses, before turning to ethical issues that are yet to be resolved. Zika raises traditional bioethical issues related to reproduction, prenatal diagnosis of serious malformations and unjust disparities in health outcomes. But the epidemic has also highlighted important issues of growing interest in public health ethics, such as the international spread of infectious disease; the central importance of reproductive healthcare in preventing maternal and neonatal morbidity and mortality; diagnostic and reporting biases; vector control and the links between vectors, climate change, and disparities in the global burden of disease. Finally, there are controversies regarding Zika vaccine research and eventual deployment. Zika virus was a neglected disease for over 50 years before the outbreak in Brazil. As it continues to spread, public health agencies should promote gender equity and disease control efforts in Latin America, while preparing for the possibility of a global epidemic. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  8. Decision-making in pediatrics: a practical algorithm to evaluate complementary and alternative medicine for children.

    Science.gov (United States)

    Renella, Raffaele; Fanconi, Sergio

    2006-07-01

    We herein present a preliminary practical algorithm for evaluating complementary and alternative medicine (CAM) for children which relies on basic bioethical principles and considers the influence of CAM on global child healthcare. CAM is currently involved in almost all sectors of pediatric care and frequently represents a challenge to the pediatrician. The aim of this article is to provide a decision-making tool to assist the physician, especially as it remains difficult to keep up-to-date with the latest developments in the field. The reasonable application of our algorithm together with common sense should enable the pediatrician to decide whether pediatric (P)-CAM represents potential harm to the patient, and allow ethically sound counseling. In conclusion, we propose a pragmatic algorithm designed to evaluate P-CAM, briefly explain the underlying rationale and give a concrete clinical example.

  9. Pediatric Aspects of Headache Classification in the International Classification of Headache Disorders-3 (ICHD-3 beta version).

    Science.gov (United States)

    McAbee, Gary N; Morse, Anne Marie; Assadi, Mitra

    2016-01-01

    This analysis looks at the applicability of the International Classification of Headache Disorders-3 beta (ICHD-3 beta) to various headache syndromes of children and adolescents. Areas of similarities and differences between adult and pediatric headaches are addressed as they relate to the ICHD-3 beta.

  10. Anesthetic considerations for a pediatric patient with Wolf-Hirschhorn syndrome: a case report.

    Science.gov (United States)

    Tsukamoto, Masanori; Yamanaka, Hitoshi; Yokoyama, Takeshi

    2017-09-01

    Wolf-Hirschhorn syndrome is a rare hereditary disease that results from a 4p chromosome deletion. Patients with this syndrome are characterized by craniofacial dysgenesis, seizures, growth delay, intellectual disability, and congenital heart disease. Although several cases have been reported, very little information is available on anesthetic management for patients with Wolf-Hirschhorn syndrome. We encountered a case requiring anesthetic management for a 2-year-old girl with Wolf-Hirschhorn syndrome. The selection of an appropriately sized tracheal tube and maintaining intraoperatively stable hemodynamics might be critical problems for anesthetic management. In patients with short stature, the tracheal tube size may differ from what may be predicted based on age. The appropriate size ( internal diameter ) of tracheal tubes for children has been investigated. Congenital heart disease is frequently associated with Wolf-Hirschhorn syndrome. Depending on the degree and type of heart disease, careful monitoring of hemodynamics is important.

  11. Nephrotic Syndrome in a Child Suffering from Tetralogy of Fallot: A Rare Association

    Directory of Open Access Journals (Sweden)

    Pépé Mfutu Ekulu

    2015-01-01

    Full Text Available Nephrotic syndrome is an uncommon complication of tetralogy of Fallot and has been rarely reported in pediatric population. We describe a 4-year-old female Congolese child who was referred for investigation for persistent dyspnea, edema, and cyanosis and nephrotic range proteinuria. Our patient presented with a tetralogy of Fallot and nephrotic syndrome. Conclusion. This case reminds us that children with tetralogy of Fallot may develop nephrotic proteinuria.

  12. Allometric considerations when assessing aortic aneurysms in Turner syndrome: Implications for activity recommendations and medical decision-making.

    Science.gov (United States)

    Corbitt, Holly; Maslen, Cheryl; Prakash, Siddharth; Morris, Shaine A; Silberbach, Michael

    2018-02-01

    In Turner syndrome, the potential to form thoracic aortic aneurysms requires routine patient monitoring. However, the short stature that typically occurs complicates the assessment of severity and risk because the relationship of body size to aortic dimensions is different in Turner syndrome compared to the general population. Three allometric formula have been proposed to adjust aortic dimensions, all employing body surface area: aortic size index, Turner syndrome-specific Z-scores, and Z-scores based on a general pediatric and young adult population. In order to understand the differences between these formula we evaluated the relationship between age and aortic size index and compared Turner syndrome-specific Z-scores and pediatric/young adult based Z-scores in a group of girls and women with Turner syndrome. Our results suggest that the aortic size index is highly age-dependent for those under 15 years; and that Turner-specific Z-scores are significantly lower than Z-scores referenced to the general population. Higher Z-scores derived from the general reference population could result in stigmatization, inappropriate restriction from sports, and increasing the risk of unneeded medical or operative treatments. We propose that when estimating aortic dissection risk clinicians use Turner syndrome-specific Z-score for those under fifteen years of age. © 2017 Wiley Periodicals, Inc.

  13. Pediatric Dentistese

    Directory of Open Access Journals (Sweden)

    Sharath Asokan

    2017-01-01

    Full Text Available Successful practice of pediatric dentistry depends on the establishment of a good relationship between the dentist and the child. Such a relationship is possible only through effective communication. Pediatric dentistry includes both an art and a science component. The focus has been mostly on the technical aspects of our science, and the soft skills we need to develop are often forgotten or neglected. This paper throws light on the communication skills we need to imbibe to be a successful pediatric dentist. A new terminology “Pediatric Dentistese” has been coined similar to motherese, parentese, or baby talk. Since baby talk cannot be applied to all age groups of children, pediatric dentistese has been defined as “the proactive development-based individualized communication between the pediatric dentist and the child which helps to build trust, allay fear, and treat the child effectively and efficiently.”

  14. Prevalence of atopic disorders and immunodeficiency in patients with ectodermal dysplasia syndromes

    Science.gov (United States)

    Mark, Barry J.; Becker, Bradley A.; Halloran, Donna R.; Bree, Alanna F.; Sindwani, Raj; Fete, Mary D.; Motil, Kathleen J.; Srun, Sopheak W.; Fete, Timothy J.

    2013-01-01

    Background Ectodermal dysplasia (ED) syndromes are a diverse group of disorders that affect multiple ectodermally derived tissues. Small studies and case reports suggest an increase in atopy and primary immunodeficiencies (PIDs) among patients with ED syndromes. Objective To determine the prevalence of clinical symptoms suggestive of atopy or immunodeficiency among a large cohort of children with ED syndromes. Methods A 9-page questionnaire was mailed to families who were members of the National Foundation for Ectodermal Dysplasias. The surveys were completed by parents of children younger than 18 years with a diagnosis of an ED syndrome or carrier state. Portions of the questionnaire were adapted from previously validated questionnaires developed by the International Study of Asthma and Allergies in Childhood (ISAAC). Results We received 347 completed questionnaires (41%). When compared with the 13- to 14-year-old children surveyed by ISAAC, we found both all-aged and age-matched children with ED syndromes, respectively, had significantly higher rates of asthma (32.2% and 37.2% vs 16.4%), rhinitis symptoms (76.1% and 78.3% vs 38.9%), and eczema (58.9% and 48.9% vs 8.2%). The prevalence of physician-diagnosed food allergies (20.7%) and PIDs (6.1%) in these ED patients also exceeded known rates in the general pediatric population. Conclusion This large-scale, retrospective study demonstrates a greater reported prevalence of symptoms suggestive of atopic disorders and PIDs among children with ED syndromes than the general pediatric population. A combination of genetic and environmental factors in ED syndromes may contribute to breaches of skin and mucosal barriers, permitting enhanced transmission and sensitization to irritants, allergens, and pathogens. PMID:22626597

  15. Outpatient management of pediatric acute mastoiditis.

    Science.gov (United States)

    Alkhateeb, Ahmed; Morin, Francis; Aziz, Haya; Manogaran, Mayuri; Guertin, William; Duval, Melanie

    2017-11-01

    Evaluate the Montreal Children's Hospital experience with outpatient management of uncomplicated acute mastoiditis with parenteral antibiotic therapy alone and determine if it is a safe alternative to inpatient management. A retrospective review of pediatric patients diagnosed with acute mastoiditis at a tertiary care pediatric hospital between 2013 and 2015 was performed. Patients with syndromes, immunodeficiency, cholesteatoma, chronic otitis media, cochlear implant in the affected ear, or incidental mastoid opacity were excluded. 56 children age 6 months to 15 years old were treated for acute mastoiditis, including 29 hospitalizations and 27 outpatients. Patients managed as outpatient with daily intravenous ceftriaxone had a 93% cure rate. Eighteen hospitalized and one outpatient had complications of acute mastoiditis. Children with complications were more likely to be febrile (p = 0.045). Two patients failed outpatient therapy and were admitted; one for myringotomy and piperacillin-tazobactam treatment and one required a mastoidectomy. 4/27 children treated as outpatient underwent myringotomy and tube insertion, 2 underwent myringotomy and tube along with admission and 21 did not require tube insertion. The average total duration of intravenous antibiotic therapy was respectively 4.9 and 18.9 days in the outpatient and hospitalized group. The average duration of admission was 5.9 days. Outpatient medical therapy of uncomplicated pediatric mastoiditis is safe, successful, and efficient. Benefits include efficient use of surgical beds, cost savings and patient and family convenience. Careful patient selection and close monitoring are keys for successful outcome. Copyright © 2017 Elsevier B.V. All rights reserved.

  16. What is known about pediatric antiphospholipid syndrome?

    Science.gov (United States)

    Meroni, Pier Luigi; Argolini, Lorenza Maria; Pontikaki, Irene

    2016-10-01

    Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by vascular thrombosis and/or pregnancy morbidity associated with the persistent presence of antiphospholipid antibodies (aPL) including lupus anticoagulant (LA), anticardiolipin antibodies (aCL), and anti-β2 glycoprotein I antibodies (aβ2GPI). APS is considered as the most common acquired hypercoagulation state of autoimmune origin in children. Unfortunately, data about incidence, prevalence, thrombosis risk and effective treatment in paediatric APS are limited and unmethodical. Expert commentary: This review summarizes recent clinical, laboratory and therapy characterization of paediatric APS and emphasizes the differences between paediatric and adult populations.

  17. Scepticism about the virtue ethics approach to nursing ethics.

    Science.gov (United States)

    Holland, Stephen

    2010-07-01

    Nursing ethics centres on how nurses ought to respond to the moral situations that arise in their professional contexts. Nursing ethicists invoke normative approaches from moral philosophy. Specifically, it is increasingly common for nursing ethicists to apply virtue ethics to moral problems encountered by nurses. The point of this article is to argue for scepticism about this approach. First, the research question is motivated by showing that requirements on nurses such as to be kind, do not suffice to establish virtue ethics in nursing because normative rivals (such as utilitarians) can say as much; and the teleology distinctive of virtue ethics does not transpose to a professional context, such as nursing. Next, scepticism is argued for by responding to various attempts to secure a role for virtue ethics in nursing. The upshot is that virtue ethics is best left where it belongs - in personal moral life, not professional ethics - and nursing ethics is best done by taking other approaches.

  18. Code of ethics: principles for ethical leadership.

    Science.gov (United States)

    Flite, Cathy A; Harman, Laurinda B

    2013-01-01

    The code of ethics for a professional association incorporates values, principles, and professional standards. A review and comparative analysis of a 1934 pledge and codes of ethics from 1957, 1977, 1988, 1998, 2004, and 2011 for a health information management association was conducted. Highlights of some changes in the healthcare delivery system are identified as a general context for the codes of ethics. The codes of ethics are examined in terms of professional values and changes in the language used to express the principles of the various codes.

  19. Business Ethics

    OpenAIRE

    Duong, Thi

    2016-01-01

    The goal of this paper is to present examples of business ethics issues. What is business ethics, things concerned in this field are and why it is needed and important when doing business? The concept of business ethics has connotations to provision, rules and standards in directing the behavior of actors in the business. Business ethics involves compliance with the law, the implementation of ethical responsibilities of a business, the protection of the rights of those who are related to the ...

  20. Bowel-associated dermatosis-arthritis syndrome in an adolescent with short bowel syndrome.

    Science.gov (United States)

    Pereira, Ester; Estanqueiro, Paula; Almeida, Susana; Ferreira, Ricardo; Tellechea, Oscar; Salgado, Manuel

    2014-09-01

    Bowel-associated dermatosis-arthritis syndrome (BADAS) is a neutrophilic dermatosis, characterized by the occurrence of arthritis and skin lesions related to bowel disease with or without bowel bypass. We report an unusual case of BADAS in a 15-year-old white male with congenital aganglionosis of the colon and hypoganglionosis of the small intestine and multiple bowel surgeries in childhood complicated by short bowel syndrome. He presented with recurrent peripheral polyarthritis, tenosynovitis, and painful erythematous subcutaneous nodules located on the dorsolateral regions of the legs and on the dorsa of the feet. Histological examination disclosed a neutrophilic dermatosis confirming the diagnosis of BADAS.Although an uncommon disease, especially at pediatric age, it is important to evoke the diagnosis of BADAS in children and adolescents with bowel disease, because treatment options and prognosis are distinct from other rheumatologic conditions.

  1. A 'good' ethical review: audit and professionalism in research ethics

    DEFF Research Database (Denmark)

    Douglas-Jones, Rachel

    2015-01-01

    How does one conduct, measure and record a ‘good’ ethical review of biomedical research? To what extent do ethics committees invoke professionalism in researchers and in themselves, and to what extent do they see competence as adherence to a set of standard operating procedures for ethical review......? Drawing on ethnographic fieldwork with the Forum of Ethics Review Committees of Asia and the Pacific (FERCAP), a capacity-building NGO that runs ethics committee trainings and reviews in the Asia Pacific region, I develop an analysis of ethical review and its effects. I focus on a ‘second-order audit’ run...... readings of ‘ethics’. I begin and end with a reflection on the ethical effects of a measurement practice that takes ethics itself as its object....

  2. Chinese insurance agents in "bad barrels": a multilevel analysis of the relationship between ethical leadership, ethical climate and business ethical sensitivity.

    Science.gov (United States)

    Zhang, Na; Zhang, Jian

    2016-01-01

    The moral hazards and poor public image of the insurance industry, arising from insurance agents' unethical behavior, affect both the normal operation of an insurance company and decrease applicants' confidence in the company. Contrarily, these scandals may demonstrate that the organizations were "bad barrels" in which insurance agents' unethical decisions were supported or encouraged by the organization's leadership or climate. The present study brings two organization-level factors (ethical leadership and ethical climate) together and explores the role of ethical climate on the relationship between the ethical leadership and business ethical sensitivity of Chinese insurance agents. Through the multilevel analysis of 502 insurance agents from 56 organizations, it is found that organizational ethical leadership is positively related to the organizational ethical climate; organizational ethical climate is positively related to business ethical sensitivity, and organizational ethical climate fully mediates the relationship between organizational ethical leadership and business ethical sensitivity. Organizational ethical climate plays a completely mediating role in the relationship between organizational ethical leadership and business ethical sensitivity. The integrated model of ethical leadership, ethical climate and business ethical sensitivity makes several contributions to ethics theory, research and management.

  3. Brief report: reporting practices of methodological information in four journals of pediatric and child psychology.

    Science.gov (United States)

    Raad, Jennifer M; Bellinger, Skylar; McCormick, Erica; Roberts, Michael C; Steele, Ric G

    2008-08-01

    To replicate Sifers, Puddy, Warren, and Roberts (2002) examining reporting rates of demographic, methodological, and ethical information in articles published during 1997, and to compare these rates to those found in articles published during 2005, in order to determine whether and how reporting practices of these variables have changed over time. We examined reporting demographic, methodological, and ethical information in articles in four journals: Journal of Pediatric Psychology, Journal of Clinical Child and Adolescent Psychology, Journal of Abnormal Child Psychology, and Child Development. Reporting rates during 2005 were compared to articles published during 1997. These four journals improved on many of the 23 variables compared to Sifers et al. including increases in the reporting of ethnicity, attrition, child assent procedures, socioeconomic status, reliability, and reward/incentive offered to participants. Improvements in descriptive information have implications for interpretation, replication, and generalizability of research findings.

  4. Anesthetic considerations for a pediatric patient with Wolf-Hirschhorn syndrome: a case report

    OpenAIRE

    Tsukamoto, Masanori; Yamanaka, Hitoshi; Yokoyama, Takeshi

    2017-01-01

    Wolf-Hirschhorn syndrome is a rare hereditary disease that results from a 4p chromosome deletion. Patients with this syndrome are characterized by craniofacial dysgenesis, seizures, growth delay, intellectual disability, and congenital heart disease. Although several cases have been reported, very little information is available on anesthetic management for patients with Wolf-Hirschhorn syndrome. We encountered a case requiring anesthetic management for a 2-year-old girl with Wolf-Hirschhorn ...

  5. Recent Clinical Drug Trials Evidence in Marfan Syndrome and Clinical Implications.

    Science.gov (United States)

    Singh, Michael N; Lacro, Ronald V

    2016-01-01

    Marfan syndrome is a genetic disorder of connective tissue with principal manifestations in the cardiovascular, ocular, and skeletal systems. Cardiovascular disease, mainly progressive aortic root dilation and aortic dissection, is the leading cause of morbidity and mortality. The primary aims of this report were to examine the evidence related to medical therapy for Marfan syndrome, including recently completed randomized clinical trials on the efficacy of β-blockers and angiotensin II receptor blockers for the prophylactic treatment of aortic enlargement in Marfan syndrome, and to provide recommendations for medical therapy on the basis of available evidence. Medical therapy for Marfan syndrome should be individualized according to patient tolerance and risk factors such as age, aortic size, and family history of aortic dissection. The Pediatric Heart Network trial showed that atenolol and losartan each reduced the rate of aortic dilation. All patients with known or suspected Marfan syndrome and aortic root dilation should receive medical therapy with adequate doses of either β-blocker or angiotensin receptor blocker. The Pediatric Heart Network trial also showed that atenolol and losartan are more effective at reduction of aortic root z score in younger subjects, which suggests that medical therapy should be prescribed even in the youngest children with aortic dilation. For patients with Marfan syndrome without aortic dilation, the available evidence is less clear. If aortic dilation is severe and/or progressive, therapy with a combination of β-blocker and angiotensin receptor blocker should be considered, although trial results are mixed with respect to the efficacy of combination therapy vs monotherapy. Copyright © 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

  6. Methodological and ethical aspects of the sexual maturation assessment in adolescents

    Directory of Open Access Journals (Sweden)

    Eliane Rodrigues de Faria

    2013-09-01

    Full Text Available OBJECTIVE To analyze methodological and ethical aspects in the sexual maturation assessment of adolescents. DATA SOURCES Books and theses, articles and legislations on the Medline, SciELO, Science Direct databases, besides institutional documents of the World Health Organization and the Pediatric Societies of Brazil and São Paulo, considering the period from 1962 to 2012. The following keywords were used in Portuguese and English: "sexual maturation", "self-assessment", "ethics", "OBJECTIVE assessment of sexual maturation", "puberty", "adolescent", and "adolescentdevelopment". DATA SYNTHESIS The sexual maturation assessment is used in populatinal studies and in clinical daily care. The direct evaluation is performed by a specialized physician, whereas the self-assessment is carried out by the adolescent. This evaluation should be carefully performed in the appropriate place, taking into account the ethical aspects. The patient should not be constrained and the physician must respect the privacy and the confidentiality. Before this evaluation and independently of the used method, the adolescent should receive information and explanation about the procedure and the tools that will be applied. Furthermore, the patient has the right to want or not an adult close to him. CONCLUSIONS Validation studies showed that self-assessment is inferior to clinical assessment and should, therefore, be performed only when the direct examination by physicians is not possible.

  7. Pediatric MDS: GATA screen the germline.

    Science.gov (United States)

    Stieglitz, Elliot; Loh, Mignon L

    2016-03-17

    In this issue of Blood, Wlodarski and colleagues demonstrate that as many as 72% of adolescents diagnosed with myelodysplastic syndrome (MDS) and monosomy 7 harbor germline mutations in GATA2. Although pediatric MDS is a very rare diagnosis, occurring in 0.8 to 4 cases per million, Wlodarski et al screened >600 cases of primary or secondary MDS in children and adolescents who were enrolled in the European Working Group on MDS consortium over a period of 15 years. The overall frequency of germline GATA2 mutations in children with primary MDS was 7%, and 15% in those presenting with advanced disease. Notably, mutations in GATA2 were absent in patients with therapy-related MDS or acquired aplastic anemia.

  8. [Discussion forum on medical ethics. A1. Basic forms in ethics].

    Science.gov (United States)

    Löw, R

    1990-05-01

    Medical ethics should not be subsumed under the classical types of ethical reasoning (e.g. Aristotle, Kant) nor the modern ethical versions of utilitarianism, deontology or ethics of discourse. All of them may contribute to medical ethics; but these should be goaled by general ethics in the meaning of how to lead a senseful life in its whole.

  9. Practice paper of the Academy of Nutrition and Dietetics abstract: ethical and legal issues of feeding and hydration.

    Science.gov (United States)

    Schwartz, Denise Baird; Posthauer, Mary Ellen; O'Sullivan Maillet, Julie

    2013-07-01

    It is the position of the Academy of Nutrition and Dietetics that individuals have the right to request or refuse nutrition and hydration as medical treatment. Registered dietitians should work collaboratively as part of an interprofessional team to make recommendations on providing, withdrawing, or withholding nutrition and hydration in individual cases and serve as active members of institutional ethics committees. This practice paper provides a proactive, integrated, systematic process to implement the Academy's position. The position and practice papers should be used together to address the history and supporting information of ethical and legal issues of feeding and hydration identified by the Academy. Elements of collaborative ethical deliberation are provided for pediatrics and adults and in different conditions. The process of ethical deliberation is presented with the roles and responsibilities of the registered dietitian and the dietetic technician, registered. Understanding the importance and applying concepts dealing with cultural values and religious diversity is necessary to integrate clinical ethics into nutrition care. Incorporating screening for quality-of-life goals is essential before implementing the Nutrition Care Process and improving health literacy with individual interactions. Developing institution-specific policies and procedures is necessary to accelerate the practice change with artificial nutrition, clinical ethics, and quality improvement projects to determine best practice. This paper supports the "Position of the Academy of Nutrition and Dietetics: Ethical and Legal Issues of Feeding and Hydration" published in the June 2013 issue of the Journal of the Academy of Nutrition and Dietetics. Copyright © 2013 Academy of Nutrition and Dietetics. Published by Elsevier Inc. All rights reserved.

  10. Reflective equilibrium and empirical data: third person moral experiences in empirical medical ethics.

    Science.gov (United States)

    De Vries, Martine; Van Leeuwen, Evert

    2010-11-01

    In ethics, the use of empirical data has become more and more popular, leading to a distinct form of applied ethics, namely empirical ethics. This 'empirical turn' is especially visible in bioethics. There are various ways of combining empirical research and ethical reflection. In this paper we discuss the use of empirical data in a special form of Reflective Equilibrium (RE), namely the Network Model with Third Person Moral Experiences. In this model, the empirical data consist of the moral experiences of people in a practice. Although inclusion of these moral experiences in this specific model of RE can be well defended, their use in the application of the model still raises important questions. What precisely are moral experiences? How to determine relevance of experiences, in other words: should there be a selection of the moral experiences that are eventually used in the RE? How much weight should the empirical data have in the RE? And the key question: can the use of RE by empirical ethicists really produce answers to practical moral questions? In this paper we start to answer the above questions by giving examples taken from our research project on understanding the norm of informed consent in the field of pediatric oncology. We especially emphasize that incorporation of empirical data in a network model can reduce the risk of self-justification and bias and can increase the credibility of the RE reached. © 2009 Blackwell Publishing Ltd.

  11. Ehlers-Danlos syndrome in a Zimbabwean child.

    Science.gov (United States)

    Olaosebikan, A; Wolf, B

    1993-01-01

    An isolated case of Ehler-Danlos syndrome, Type 1, in a two year old Zimbabwean boy is described. The patient presented with failure to thrive and inability to stand. Examination revealed hyperextensibility of the joints and skin, umbilical and inguinal hernias and a perimembranous ventricular septal defect. To the best of our knowledge this is the first pediatric case described in the African literature.

  12. [The ethics of principles and ethics of responsibility].

    Science.gov (United States)

    Cembrani, Fabio

    2016-01-01

    In his brief comment, the author speculates if ethics in health-care relationship it still has a practical sense.The essay points out the difference between principles ethics and ethics of responsibility, supporting the latter and try to highlight its constitutive dimensions.

  13. Ethical considerations on the value of patient knowledge in long-term care: a qualitative study of patients suffering from Korsakoff 's syndrome.

    Science.gov (United States)

    van den Hooff, Susanne L; Goossensen, Anne

    2015-05-01

    This study explores experiences of patients suffering from Korsakoff's syndrome. It contributes to improved reflection on the value of patient knowledge. An ethics of care perspective states the importance of moving to patients in their vulnerable state of being, and to figure out patients' individual needs necessary to provide good care. The information given by patients suffering from Korsakoff's syndrome might be mistaken, invented and even not true. The value of these patients' experiences and knowledge had not been researched to date. Data from six in-depth interviews were analysed concurrently through the constant comparative method. The principles of voluntariness, confidentiality and anonymity were respected during the research process. Four important themes within patients' knowledge emerged: health condition, involuntary admission into the nursing home, the daily routine and interpretation of the caring abilities of healthcare professionals. The findings show that our respondents were able to provide a window into their inner perspective. This study deepens our understanding of the value of patient knowledge in situations where patients see the world in a different manner as do healthcare professionals. The conversations revealed personal subjective knowledge which our respondents create and live by. Healthcare professionals are encouraged to discover and affirm the understandings of these patients, and not be prepossessed due to the diagnosis or general nursing home rules. Understanding patients' unique knowledge through appreciation of the complexity and richness of different views on the situation can assist to give a more humane response to individual needs and pain. Health staff in healthcare institutions are encouraged to evaluate the - unintended - effect of rigidly applied nursing home rules, and the government is challenged to rethink its policy about care, and provide more resources permitting relational care that nurtures the dignity of

  14. Christian ethic of love and Hindu ethic of dharma: comparative analysis

    OpenAIRE

    Pavenkov Oleg Vladimirovich

    2014-01-01

    This article is devoted to comparative analysis of two ethic systems: Hindu ethic of dharma and Christian ethic of love. If Christian ethic is the ethic of love, love is in the center of Christian moral values, then Hindu ethic is an example of ethic of law. The moral behavior of gods and humans is determined by Karma and cycle of samsara, which is impossible in Christianity. However forgiveness, self-restraint, non-stealing, purity are common moral obligations for Christians and Hindus.

  15. Nigerian Building Professionals’ Ethical Ideology and Perceived Ethical Judgement

    Directory of Open Access Journals (Sweden)

    John Oko Ameh

    2010-10-01

    Full Text Available In recent years, Nigeria is often cited in the international media in connection with corruption and other unethical practices. The professionals in the Nigerian building industry are not immune from the national trend in ethical erosion. Moral philosophy or ethical ideology has been used to explain individuals’ reasoning about moral issues and consequent behaviour. This study examines building industry professionals’ ethical ideologies with a view to understanding their ethical behaviour in professional practice.  In carrying out this investigation, building professionals in clients’ organisations, contracting and consultancy organisations within the industry were asked to respond to the Ethics Position Questionnaire (EPQ designed by Forsyth in order to determine their idealism and relativism level. Subsequently, they were classified into one of four groups, representing different ethical ideologies. The result indicates that the dominant ethical ideology of building industry professionals is situationism. The study predicts that the attitude of building industry professionals in practice, given the current socio-political and economic situation of Nigeria would possibly be unethical because of the extreme influence situational factors have on their behaviour. This finding is a bold step and necessary benchmark for resolving ethical issues within the industry and should be of interest to policy makers. It is also useful for intra professional ethical comparison.

  16. Furthering the sceptical case against virtue ethics in nursing ethics.

    Science.gov (United States)

    Holland, Stephen

    2012-10-01

    In a recent article in this journal I presented a sceptical argument about the current prominence of virtue ethics in nursing ethics. Daniel Putman has responded with a defence of the relevance of virtue in nursing. The present article continues this discussion by clarifying, defending, and expanding the sceptical argument. I start by emphasizing some features of the sceptical case, including assumptions about the nature of sceptical arguments, and about the character of both virtue ethics and nursing ethics. Then I respond to objections of Putman's such as that, according to virtue ethics, virtue is relevant to the whole of a human life, including one's behaviour in a professional context; and that eudaimonia should be central in explaining and motivating a nurse's decision to enter the profession. Having argued that these objections are not compelling, I go on to discuss an interesting recent attempt to reassert the role of virtue ethics in the ethics of professions, including nursing. This centres on whether role-specific obligations - e.g. the obligations that arise for a moral agent qua lawyer or mother - can be accommodated in a virtue ethics approach. Sean Cordell has argued that the difficulty of accommodating role-specific obligations results in an 'institution-shaped gap' in virtue ethics. He suggests a way of meeting this difficulty that appeals to the ergon of institutions. I endorse the negative point that role-specific obligations elude virtue ethics, but argue that the appeal to the ergon of institutions is unsuccessful. The upshot is further support for scepticism about the virtue ethics approach to nursing ethics. I end by gesturing to some of the advantages of a sceptical view of virtue ethics in nursing ethics. © 2012 Blackwell Publishing Ltd.

  17. What is the Business of Ethics in Business Ethics?

    DEFF Research Database (Denmark)

    Lystbæk, Christian Tang

    For the last decades, business ethics have spread from a new brand of a few socially and environmentally benevolent companies to the corporate mainstream. Thus, today, business ethics have become a central concern for both business managers and researchers in order to manage the cultural value base...... of the organization, stakeholder relations, etc.. Throughout the history of business ethics, though, and especially in the last decade, a series of studies have criticized the dominant view of business ethics for being instrumental and reductive. This critique often dismisses business ethics altogether. This paper...... addresses these “movements” or approaches to business ethics. It argues that business ethics is caught between two conceptions of what it is for. The first movement promotes the idea that it can be a reassuring and satisfying set of ideas that reminds us how to do the right thing in order to manage...

  18. Acquired retinal pigmentary degeneration in a child with 13q deletion syndrome.

    Science.gov (United States)

    Aguilera, Zenia P; Belin, Peter J; Cavuoto, Kara M; Jayakar, Parul; McKeown, Craig A

    2015-10-01

    Orbeli syndrome, or 13q deletion syndrome, is a rare condition caused by a distal deletion in the long arm of chromosome 13. The syndrome is characterized by severe physical malformations and developmental delays and has been associated with numerous ocular manifestations. We report the case of a 10-year-old boy with 13q deletion syndrome, who was evaluated for impaired vision and found to have bilateral retinal pigmentary changes resembling those seen in retinitis pigmentosa. There has only been one other case of retinal pigment variation in association with 13q deletion syndrome; however, this represents the first case of bilateral symmetric retinal pigmentary changes with corresponding rod and cone dysfunction on electroretinography. Copyright © 2015 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.

  19. Christian ethic of love and Hindu ethic of dharma: comparative analysis

    Directory of Open Access Journals (Sweden)

    Pavenkov Oleg Vladimirovich

    2014-10-01

    Full Text Available This article is devoted to comparative analysis of two ethic systems: Hindu ethic of dharma and Christian ethic of love. If Christian ethic is the ethic of love, love is in the center of Christian moral values, then Hindu ethic is an example of ethic of law. The moral behavior of gods and humans is determined by Karma and cycle of samsara, which is impossible in Christianity. However forgiveness, self-restraint, non-stealing, purity are common moral obligations for Christians and Hindus.

  20. OBESITY AND METABOLIC SYNDROME IN CHILDREN AND YOUTH: A HEALTH RISK WE CANNOT AFFORD

    OpenAIRE

    Serge P. von Duvillard

    2012-01-01

    Ample observational and empirical evidence has been provided that indicates that childhood metabolic syndrome risk factors inevitably lead to significantly more profound health risk factors of developing potent adulthood metabolic syndrome. Much of these data has been provided from medical, nutritional, health, pediatric, physical education and associated communities. Perhaps the most visible and observable health risk factor among children (here referred to as youth) is the childhood obesit...

  1. Pathophysiology, risk factors, and screening methods for prediabetes in women with polycystic ovary syndrome

    OpenAIRE

    Gourgari, Evgenia; Spanakis,Elias; Dobs,Adrian

    2016-01-01

    Evgenia Gourgari,1 Elias Spanakis,2 Adrian Sandra Dobs3 1Division of Pediatric Endocrinology, Georgetown University School of Medicine, Washington, DC, 2Division of Endocrinology, University of Maryland School of Medicine, 3Department of Endocrinology, Johns Hopkins University School of Medicine, Baltimore, MD, USA Abstract: Polycystic ovary syndrome (PCOS) is a syndrome associated with insulin resistance (IR), obesity, infertility, and increased cardiometabolic risk. This is a des...

  2. Behcet's syndrome involving the gastrointestinal tract - a diagnostic dilemma in childhood

    International Nuclear Information System (INIS)

    Stringer, D.A.; Daneman, A.; Cleghorn, G.J.; Durie, P.R.; Hamilton, J.R.

    1986-01-01

    Behcet's syndrome is very rare in children, especially those under 10 years of age. Clinical and radiological features are described in 4 children, including 2 under the age of 5 years, with the syndrome. As in other pediatric cases reported, the incomplete form of Behcet's syndrome was present in each case. All 4 patients had oral and genital mucosal effects, arthritis and gastrointestinal and dermatological manifestations. Ophthalmological symptoms occurred in only 1 patient. Radiologically, the 4 cases demonstrated the spectrum of gastrointestinal involvement, from minimal irregularity and thickening of the terminal ileum to gross irregularity and deformity of the terminal ileum and cecum. Because of the difficulty in differentiating Behcet's syndrome from other forms of inflammatory bowel disease it is suggested that in children with gastrointestinal involvement, 3 major criteria be present before the diagnosis of Behcet's syndrome is made. (orig.)

  3. Agricultural Ethics, Environmental Ethics, and Bioethics : the Major Issues of Agricultural Ethics

    OpenAIRE

    畠中, 和生

    2002-01-01

    It is the aim of the paper is to make the major issues of agricultural ethics clear by comparing them with the ones of environmental ethics and bioethics. The main topics in this paper are following. 1. The major issues of the debate over agricultural ethics are (1) agricultural threats to public health and safety; (2) government responsibility for controlling agricultural resource depletion; (3) agricultural contributions to ecological disturbance; (4) government responsibility for preservin...

  4. Beyond a code of ethics: phenomenological ethics for everyday practice.

    Science.gov (United States)

    Greenfield, Bruce; Jensen, Gail M

    2010-06-01

    Physical therapy, like all health-care professions, governs itself through a code of ethics that defines its obligations of professional behaviours. The code of ethics provides professions with a consistent and common moral language and principled guidelines for ethical actions. Yet, and as argued in this paper, professional codes of ethics have limits applied to ethical decision-making in the presence of ethical dilemmas. Part of the limitations of the codes of ethics is that there is no particular hierarchy of principles that govern in all situations. Instead, the exigencies of clinical practice, the particularities of individual patient's illness experiences and the transformative nature of chronic illnesses and disabilities often obscure the ethical concerns and issues embedded in concrete situations. Consistent with models of expert practice, and with contemporary models of patient-centred care, we advocate and describe in this paper a type of interpretative and narrative approach to moral practice and ethical decision-making based on phenomenology. The tools of phenomenology that are well defined in research are applied and examined in a case that illustrates their use in uncovering the values and ethical concerns of a patient. Based on the deconstruction of this case on a phenomenologist approach, we illustrate how such approaches for ethical understanding can help assist clinicians and educators in applying principles within the context and needs of each patient. (c) 2010 John Wiley & Sons, Ltd.

  5. ETHICS, VALUES AND BELONGING. BUILDING AN ETHICAL CULTURE

    OpenAIRE

    David King

    2013-01-01

    What is an ethical culture, and why should companies bother about it? An ethical company is a business that helps people know the right thing to do, understand their code of ethics and uphold the organisation’s principles. As a result, the organisation protects itself against the kind of ethical contraventions and scandals that have affected many sectors, putting businesses in the news for all the wrong reasons and undermining the trust of customers, employees and investors. How can companies...

  6. Role of sleep quality in the metabolic syndrome

    Directory of Open Access Journals (Sweden)

    Koren D

    2016-08-01

    Full Text Available Dorit Koren,1,2 Magdalena Dumin,1 David Gozal2,3 1Section of Adult and Pediatric Endocrinology, Diabetes, and Metabolism, Department of Medicine, 2Section of Pediatric Sleep Medicine, 3Section of Pulmonology, Department of Pediatrics, Pritzker School of Medicine, Biological Sciences Division, The University of Chicago, Chicago, IL, USA Abstract: Emerging evidence has assigned an important role to sleep as a modulator of metabolic homeostasis. The impact of variations in sleep duration, sleep-disordered breathing, and chronotype to cardiometabolic function encompasses a wide array of perturbations spanning from obesity, insulin resistance, type 2 diabetes, the metabolic syndrome, and cardiovascular disease risk and mortality in both adults and children. Here, we critically and extensively review the published literature on such important issues and provide a comprehensive overview of the most salient pathophysiologic pathways underlying the links between sleep, sleep disorders, and cardiometabolic functioning. Keywords: sleep apnea, circadian clock, insulin resistance, obesity, cardiovascular risk

  7. Pediatric oncologist willingness to offer germline TP53 testing in osteosarcoma.

    Science.gov (United States)

    Shaul, Eliana; Roth, Michael; Lo, Yungtai; Geller, David S; Hoang, Bang; Yang, Rui; Malkin, David; Gorlick, Richard; Gill, Jonathan

    2018-03-15

    Li-Fraumeni syndrome (LFS) is a cancer predisposition syndrome caused by mutations in the tumor-suppressor gene TP53. Osteosarcoma is a sentinel cancer in LFS. Prior studies using Sanger sequencing platforms have demonstrated that 3% of individuals with osteosarcoma harbor a mutation in TP53. New data from next-generation sequencing have demonstrated that 3.8% of patients with osteosarcoma have a known pathogenic variant, and an additional 5.7% carry exonic variants of unknown significance in TP53. Pediatric oncologists were e-mailed an anonymous 18-question survey assessing their willingness to offer TP53 germline testing to a child with osteosarcoma with or without a family history, and they were evaluated for changes in their choices with the prior data and the new data. One hundred seventy-seven pediatric oncologists (22%) responded to the survey. Respondents were more likely to offer TP53 testing to a patient with a positive family history (77.4% vs 12.4%; P offer TP53 testing once they were provided with the new data (25.4% vs 12.4%; P = .0038). The proportion of providers who responded that they were unsure increased significantly when they were presented with the new data (25.4% vs 10.2%; P = .0002). Potential implications for other family members and the possibility that surveillance imaging would detect new malignancies at an earlier stage were important factors influencing a provider's decision to offer TP53 testing. Recent data increase the proportion of providers willing to offer testing, and this suggests concern on the part of pediatric oncologists that variants of unknown significance may be disease-defining in rare cancers. Cancer 2018;124:1242-50. © 2018 American Cancer Society. © 2018 American Cancer Society.

  8. Future global ethics: environmental change, embedded ethics, evolving human identity.

    NARCIS (Netherlands)

    D.R. Gasper (Des)

    2014-01-01

    markdownabstract__Abstract__ Work on global ethics looks at ethical connections on a global scale. It should link closely to environmental ethics, recognizing that we live in unified social-ecological systems, and to development ethics, attending systematically to the lives and interests of

  9. CSF neurofilament light chain is elevated in OMS (decreasing with immunotherapy) and other pediatric neuroinflammatory disorders.

    Science.gov (United States)

    Pranzatelli, Michael R; Tate, Elizabeth D; McGee, Nathan R; Verhulst, Steven J

    2014-01-15

    Using a panel of seven brain cell-specific biomarkers in cerebrospinal fluid (CSF), pediatric opsoclonus-myoclonus syndrome (OMS) (n=234) was compared to pediatric non-inflammatory neurological controls (n=84) and other inflammatory neurological disorders (OIND) (n=44). Only CSF NFL was elevated in untreated OMS versus controls (+83%). It was 87% higher in OIND than in OMS. On combination treatment with front-loaded ACTH, IVIg, rituximab, median CSF NFL decreased by 60% to control levels. These biochemical data suggest neuronal/axonal injury in some children with OMS without indicators of astrogliosis, and reduction on sufficient immunotherapy. Copyright © 2013 Elsevier B.V. All rights reserved.

  10. Balancing Ethics and Quality in Educational Research--The Ethical Matrix Method

    Science.gov (United States)

    Tangen, Reidun

    2014-01-01

    This paper addresses ethical issues in educational research with a focus on the interplay between research ethics and both internal and external quality of research. Research ethics is divided into three domains: (1) ethics "within" the research community; (2) ethics concerning relationships with "individuals and groups directly…

  11. Ethical difficulties in nursing, educational needs and attitudes about using ethics resources.

    Science.gov (United States)

    Leuter, Cinzia; Petrucci, Cristina; Mattei, Antonella; Tabassi, Gianpietro; Lancia, Loreto

    2013-05-01

    Ethical difficulties arise in healthcare practices. However, despite extensive research findings that demonstrate that most nurses are involved in recurrent ethical problems, institutions are not always able to effectively support nursing care professionals. The limited availability of ethics consultation services and traditional nursing training fails to meet the frequent and strong requests by health workers to support their ethical dilemmas. A questionnaire was administered to 374 nurses attending a specialist training and a lifetime learning programme in Italy. The respondents reported a high frequency of ethically sensitive situations, and they described the poor development of ethics support and a scarcity of ethics training programmes. The results suggest the importance of promoting ethics services that include consultation and ethics training. A need for systematic ethics educational activities was identified for improving the capacity of nurses to manage ethical issues in patient care.

  12. Burnout syndrome: understanding of medical teaching professionals

    Directory of Open Access Journals (Sweden)

    Jaqueline Brito Vidal Batista

    2017-04-01

    Full Text Available This study aimed to investigate the understanding of medical teaching professionals about Burnout Syndrome. This is a qualitative, exploratory study, consisting of ten teaching physicians, who work at the hospital of a higher education institution. The data were collected from May to June 2013, through a form with questions pertinent to the proposed research objective, after approval by the Research Ethics Committee (Protocol No. 84022, and analyzed qualitatively, through the content analysis technique (Bardin. Among the 10 participants in the study, eight had adequate knowledge about Burnout Syndrome, while others showed insufficient knowledge. From the empirical material analysis, five thematic categories emerged: Syndrome characterized by physical and psychological exhaustion due to work stress; Physical and psychological signs and symptoms of Burnout Syndrome; Burnout syndrome and its implications for the worker’s health; The most vulnerable workers who develop Burnout Syndrome and Relation of Burnout Syndrome to the work of the teaching physician. The study showed that most participants in the research adequately understand Burnout Syndrome, but the subject is still little explored in academia. Therefore, intervention measures are necessary with the professionals of the risk group and new studies that contribute to expand the knowledge about that syndrome, aiming to improve the quality of life of the workers. Keywords: Worker’s Health; Professional Exhaustion; Doctors; Professors; Work Conditions.   DOI: http://dx.doi.org/10.3823/2397

  13. 'Wicked' ethics: Compliance work and the practice of ethics in HIV research.

    Science.gov (United States)

    Heimer, Carol A

    2013-12-01

    Using ethnographic material collected between 2003 and 2007 in five HIV clinics in the US, South Africa, Uganda, and Thailand, this article examines "official ethics" and "ethics on the ground." It compares the ethical conundrums clinic staff and researchers confront in their daily work as HIV researchers with the dilemmas officially identified as ethical issues by bioethicists and people responsible for ethics reviews and compliance with ethics regulations. The tangled relation between ethical problems and solutions invites a comparison to Rittel and Webber's "wicked problems." Official ethics' attempts to produce universal solutions often make ethics problems even more wickedly intractable. Ethics on the ground is in part a reaction to this intractability. Copyright © 2012 Elsevier Ltd. All rights reserved.

  14. BUSINESS ETHICS

    Directory of Open Access Journals (Sweden)

    Nelu BURCEA

    2014-12-01

    Full Text Available Through this study we seek to explore the concept of business ethics, in those aspects that we consider to be essential and concrete. We started from a few questions: Could the two concepts be compatible? If not, why not? If yes, could they be complementary? How real is the use of ethics in the profits of a business? How can be business ethics be exemplified and what principles are essential in doing business? How does the business environment react to the concept? These are some of the elements that will form the basis of this scientific study. Lately, business ethics has been becoming an increasingly popular topic. Set against the global economic crisis, the companies’ credibility could become a major concern. Business ethics also becomes a challenge for training and informing employees and employers, in order to make not only economical, but also ethical decisions regarding their profits. In the study we shall also address the ethical standards required in a business world interested in fundamental values that can make the difference in 21st century business. Also, according to a study conducted by the authors, we shall address the two most important ethical values that prove to be essential to a business.

  15. Ethical aspects in tissue research: thematic analysis of ethical statements to the research ethics committee

    Science.gov (United States)

    2012-01-01

    Background Many studies have been published about ethics committees and the clarifications requested about the submitted applications. In Finland, ethics committees require a separate statement on ethical aspects of the research in applications to the ethics committee. However, little is known about how researchers consider the ethical aspects of their own studies. Methods The data were collected from all the applications received by the official regional ethics committee in the Hospital District of Northern Savo during 2004–2009 (n = 688). These included a total of 56 studies involving research on tissue other than blood. The statements by the researchers about the ethics about their own research in these applications were analyzed by thematic content analysis under the following themes: recruitment, informed consent, risks and benefits, confidentiality and societal meaning. Results The researchers tended to describe recruitment and informed consent process very briefly. Usually these descriptions simply stated who the recruiter was and that written consent would be required. There was little information provided on the recruitment situation and on how the study recruiters would be informed. Although most of the studies were clinical, the possibility was hardly ever discussed that patients could fail to distinguish between care and research. Conclusion The written guidelines, available on the webpages of the ethics committee, do not seem to be enough to help researchers achieve this goal. In addition to detailed guidelines for researchers, investigators need to be taught to appreciate the ethical aspects in their own studies. PMID:22873761

  16. After Ethics

    DEFF Research Database (Denmark)

    Shepherd, Nick; Haber, Alejandro

    While books on archaeological and anthropological ethics have proliferated in recent years, few attempt to move beyond a conventional discourse on ethics to consider how a discussion of the social and political implications of archaeological practice might be conceptualized differently....... The conceptual ideas about ethics posited in this volume make it of interest to readers outside of the discipline; in fact, to anyone interested in contemporary debates around the possibilities and limitations of a discourse on ethics. The authors in this volume set out to do three things. The first is to track...... the historical development of a discussion around ethics, in tandem with the development and “disciplining” of archaeology. The second is to examine the meanings, consequences and efficacies of a discourse on ethics in contemporary worlds of practice in archaeology. The third is to push beyond the language...

  17. ROHHAD Syndrome: Reasons for Diagnostic Difficulties in Obesity.

    Science.gov (United States)

    Kocaay, Pınar; Şıklar, Zeynep; Çamtosun, Emine; Kendirli, Tanıl; Berberoğlu, Merih

    2014-12-01

    A very rare syndrome of rapid-onset obesity with hypoventilation, hypothalamic dysfunction and autonomic dysregulation (ROHHAD) has been recently described as causing morbidity due to hypothalamic dysfunction and respiratory arrest. Its prognosis is poor and often cardiac arrest occurs due to alveolar hypoventilation. This disorder can mimic genetic obesity syndromes and several endocrine disorders. We present a 13-year-old female patient who was reported to be healthy until the age of 3 years. She was admitted to our emergency department, presenting with respiratory distress. Features matching ROHHAD syndrome such as rapid-onset obesity, alveolar hypoventilation, central hypothyroidism, hyperprolactinemia, Raynaud phenomenon and hypothalamic hypernatremia were detected in the patient. In addition to these features, the patient was found to have hypergonadotropic hypogonadism and megaloblastic anemia. Because of its high mortality and morbidity, the possibility of ROHHAD syndrome needs to be considered in all pediatric cases of early- and rapid-onset obesity associated with hypothalamic-pituitary endocrine dysfunction.

  18. Is mandatory research ethics reviewing ethical?

    Science.gov (United States)

    Dyck, Murray; Allen, Gary

    2013-08-01

    Review boards responsible for vetting the ethical conduct of research have been criticised for their costliness, unreliability and inappropriate standards when evaluating some non-medical research, but the basic value of mandatory ethical review has not been questioned. When the standards that review boards use to evaluate research proposals are applied to review board practices, it is clear that review boards do not respect researchers or each other, lack merit and integrity, are not just and are not beneficent. The few benefits of mandatory ethical review come at a much greater, but mainly hidden, social cost. It is time that responsibility for the ethical conduct of research is clearly transferred to researchers, except possibly in that small proportion of cases where prospective research participants may be so intrinsically vulnerable that their well-being may need to be overseen.

  19. Early onset intellectual disability in chromosome 22q11.2 deletion syndrome.

    Science.gov (United States)

    Cascella, Marco; Muzio, Maria Rosaria

    2015-01-01

    Chromosome 22q11.2 deletion syndrome, or DiGeorge syndrome, or velocardiofacial syndrome, is one of the most common multiple anomaly syndromes in humans. This syndrome is commonly caused by a microdelection from chromosome 22 at band q11.2. Although this genetic disorder may reflect several clinical abnormalities and different degrees of organ commitment, the clinical features that have driven the greatest amount of attention are behavioral and developmental features, because individuals with 22q11.2 deletion syndrome have a 30-fold risk of developing schizophrenia. There are differing opinions about the cognitive development, and commonly a cognitive decline rather than an early onset intellectual disability has been observed. We report a case of 22q11.2 deletion syndrome with both early assessment of mild intellectual disabilities and tetralogy of Fallot as the only physic manifestation. Copyright © 2015 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

  20. Pediatric donation after circulatory determination of death (pDCD): A narrative review.

    Science.gov (United States)

    Weiss, Matthew J; Sherry, Wendy; Hornby, Laura

    2018-04-14

    Pediatric donation after circulatory death (pDCD) is an established pathway for organ donation. It remains, however, a relatively rare event worldwide, and most clinicians outside of the pediatric intensive care unit (PICU) are unfamiliar with it. The goal of this review is to introduce the processes and concepts of pDCD. While most children die in circumstances that would not allow pDCD, many children that die after withdrawal of life sustaining therapy (WLST) may be eligible for donation of some organs. The potential benefits of this practice to patients on the wait list are well known, but donation can also be an opportunity to honor a patient's or family's desire to altruistically improve the lives of others. Offering the possibility of donation requires careful attention to ethical principles to ensure that conflicts of interest are avoided and that the family is free to make an independent, fully informed decision. Doing so allows families and decision makers the autonomy to decide if donation is something they wish to incorporate into end-of-life care. Copyright © 2018 Elsevier Ltd. All rights reserved.