In the introduction the author submits association, connotations, and definitions of basic ethical terms, along with a classification of ethics. The author presents an outline of ethical problems of medicine and in particular paediatrics with an explanation why society pays special attention to these problems and why ethical self-reflection of doctors in general and paediatricians in particular is necessary.
Yee, Jennifer; King, Andrew
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....
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.
...] 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...
Al-Youbi, Reem A.; Jan, Mohammed M.
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...
Cimaz, Rolando; Descloux, Elodie
APS is rare in the pediatric age, but it represents an interesting phenomenon because most of the known "second hit" risk factors such as atherosclerosis, smoking, hypertension, contraceptive hormonal treatment, and pregnancy are not present in childhood. This could also be the reason for the prevalence of some clinical manifestations rather than others in PAPS. On the other hand, the increased frequency of infectious processes in the childhood age is likely responsible for the relatively high prevalence of non-pathogenic and transient aPL. Such points raise the problem of a different diagnosis or monitoring approach in pediatric APS. Of particular interest is the special entity of neonatal APS, which represents an in vivo model of acquired autoimmune disease, in which transplacentally acquired aPL cause thrombosis in the newborn. International registries for pediatric and neonatal APS are currently in place; epidemiologic, clinical, and laboratory re-search will help to shed light on all the still obscure aspects of this fascinating but rare disorder in the very young. Finally, treatment is less aggressive overall in pediatric APS, given the reluctance to anticoagulate children over the long term. Studies on the outcome of pediatric APS and the relative risks of prolonged anticoagulation in children are necessary to determine the type and duration of anticoagulation therapy.
The diagnosis of a polyposis syndrome, such as juvenile polyposis, Peutz-Jeghers syndrome, and familial adenomatous polyposis, requires knowledge of the site, number, and histologic type of the polyps and an appreciation of relevant family history. Children and adolescents with polyposis syndromes are faced with not only the immediate complications of the polyps, such as intussusception or bleeding, but also the extraintestinal manifestations and the long-term risk for malignancy. This article reviews the diagnosis, clinical management, surveillance, and surgical options for children with polyposis syndromes and discusses genetics and appropriate screening programs.
Feudtner, Chris; Nathanson, Pamela G
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.
Coleman, Doriane Lambelet
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
Reem A. Al-Youbi
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.
Needham, Allison C; Kapadia, Mufiza Z; Offringa, Martin
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 local requirements while ensuring that the protection of human subjects is consistent across sites. In pediatrics, this requirement is complicated by pediatric-specific ethical concerns such as the acquisition of assent and consent and the need for pediatric expertise to assess the scholarly merit of the proposed research. Efforts to tackle these challenges have focused on the process of ethics review, which will improve efficiency. In addition to improving process, we suggest further research to fill gaps in the evidence base for recommendations and decisions made by REBs, specifically their effectiveness to protect human subjects. Evidence gathered will contribute to the successful development, adoption and implementation of harmonized guidance to apply ethics principles in order to protect children through research rather than from research.
Higgins, Victoria; Adeli, Khosrow
Pediatric overweight and obesity is an emerging public health priority as rates have rapidly increased worldwide. Obesity is often clustered with other metabolic abnormalities including hypertension, dyslipidemia, and insulin resistance, leading to increased risk of cardiovascular disease. This cluster of risk factors, termed the metabolic syndrome, has traditionally been reported in adults. However, with the increased prevalence of pediatric obesity, the metabolic syndrome is now evident in children and adolescents. This complex cluster of risk factors is the result of the pathological interplay between several organs including adipose tissue, muscle, liver, and intestine with a common antecedent - insulin resistance. The association of the metabolic syndrome with several systemic alterations that involve numerous organs and tissues adds to the complexity and challenge of diagnosing the metabolic syndrome and identifying useful clinical indicators of the disease. The complex physiology of growing and developing children and adolescents further adds to the difficulties in standardizing laboratory assessment, diagnosis, and prognosis for the diverse pediatric population. However, establishing a consensus definition is critical to identifying and managing children and adolescents at high risk of developing the metabolic syndrome. As a result, the examination of novel metabolic syndrome biomarkers which can detect these metabolic abnormalities early with high specificity and sensitivity in the pediatric population has been of interest. Understanding this complex cluster of risk factors in the pediatric population is critical to ensure that this is not the first generation where children have a shorter life expectancy than their parents. This review will discuss the pathophysiology, consensus definitions and laboratory assessment of pediatric metabolic syndrome as well as potential novel biomarkers.
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.
...] 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...
Graf, William D; Nagel, Saskia K; Epstein, Leon G; Miller, Geoffrey; Nass, Ruth; Larriviere, Dan
The use of prescription medication to augment cognitive or affective function in healthy persons-or neuroenhancement-is increasing in adult and pediatric populations. In children and adolescents, neuroenhancement appears to be increasing in parallel to the rising rates of attention-deficit disorder diagnoses and stimulant medication prescriptions, and the opportunities for medication diversion. Pediatric neuroenhancement remains a particularly unsettled and value-laden practice, often without appropriate goals or justification. Pediatric neuroenhancement presents its own ethical, social, legal, and developmental issues, including the fiduciary responsibility of physicians caring for children, the special integrity of the doctor-child-parent relationship, the vulnerability of children to various forms of coercion, distributive justice in school settings, and the moral obligation of physicians to prevent misuse of medication. Neurodevelopmental issues include the importance of evolving personal authenticity during childhood and adolescence, the emergence of individual decision-making capacities, and the process of developing autonomy. This Ethics, Law, and Humanities Committee position paper, endorsed by the American Academy of Neurology, Child Neurology Society, and American Neurological Association, focuses on various implications of pediatric neuroenhancement and outlines discussion points in responding to neuroenhancement requests from parents or adolescents. Based on currently available data and the balance of ethics issues reviewed in this position paper, neuroenhancement in legally and developmentally nonautonomous children and adolescents without a diagnosis of a neurologic disorder is not justifiable. In nearly autonomous adolescents, the fiduciary obligation of the physician may be weaker, but the prescription of neuroenhancements is inadvisable because of numerous social, developmental, and professional integrity issues.
Schachter, Asher D.
Idiopathic nephrotic syndrome is the most common glomerular disorder of childhood. Recurrence of nephrotic syndrome immediately following renal transplantation is rapid, results in a high rate of graft loss, and represents the most severe form of nephrotic syndrome. This review discusses the molecular heterogeneity of pediatric nephrotic syndrome across the spectrum of disease activity. A schema is offered for a molecular approach to pediatric nephrotic syndrome, including immune-mediated and...
Victoria Cheung BA
Full Text Available Background. Restless legs syndrome (RLS is a sleep disorder characterized by an urge to move or the presence of unpleasant sensations in the extremities. The prevalence of RLS is higher in children and adults with chronic kidney disease and in adults with glomerular disease. Objective. To determine the prevalence of RLS in children with nephrotic syndrome. Methods. We studied 50 children with nephrotic syndrome and 22 controls. The following surveys were administered: Pediatric Emory RLS questionnaire, Pediatric Daytime Sleepiness Scale, and Pediatric Sleep Questionnaire. Results. Children with nephrotic syndrome were 9.0 ± 4.4 years old, 27 were male, and 27 were in remission. The prevalence of RLS was similar in the nephrotic syndrome cases and controls, whether or not indeterminate cases were considered positive: 14.0% versus 13.6% including indeterminate cases, and 8.0% versus 9.1% excluding indeterminate cases. Conclusion. RLS is not more common in children with glomerular disease compared to healthy controls.
Daisy Amparo Martínez Delgado
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.
Kadom, Nadja; Rosman, N.P.; Jubouri, Shams; Trofimova, Anna; Egloff, Alexia M.; Zein, Wadih M.
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.)
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)
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.)
Ekhomu, Omonigho; Naheed, Zahra J
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.
Full Text Available Analysis of 5 cases of anemic syndrome is presented, due to which children were admitted to the hospital. Final clinical diagnoses were as follows: Crohn’s disease, Jaksch-Hayem anemia, acute lymphoblastic leucosis, hiatus hernia and chronic renal disease. These data indicated that any anemia can be observed as a symptom for a variety of diseases that may either be related to the primary damage to the blood system or not depend on it. Based on the data presented, it is concluded that the success of treatment activities and the quality of life of a child with anemia largely depend on the early detection of its cause, and the therapy should not be reduced to restoring the normal level of hemoglobin, but based on an understanding of the pathogenetic mechanisms of hematological disorders in the patient.
Stratakis, Constantine A
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.
Cameron, Miriam E.; And Others
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)
Cook, Alyssa F; Sobotka, Sarah A; Ross, Lainie F
The Accreditation Council for Graduate Medical Education requires residency programs to provide instruction in and evaluation of competency in ethics and professionalism. We examined current practices and policies in ethics and professionalism in pediatric training programs, utilization of newly available resources on these topics, and recent concerns about professional behavior raised by social media. From May to August 2012, members of the Association of Pediatric Program Directors identified as categorical program directors in the APPD database were surveyed regarding ethics and professionalism practices in their programs, including structure of their curricula, methods of trainee assessment, use of nationally available resources, and policies regarding social media. The response rate was 61% (122 of 200). Most pediatric programs continue to teach ethics and professionalism in an unstructured manner. Many pediatric program directors are unaware of available ethics and professionalism resources. Although most programs lack rigorous evaluation of trainee competency in ethics and professionalism, 30% (35 of 116) of program directors stated they had not allowed a trainee to graduate or sit for an examination because of unethical or unprofessional conduct. Most programs do not have formal policies regarding social media use by trainees, and expectations vary widely. Pediatric training programs are slowly adopting the educational mandates for ethics and professionalism instruction. Resources now exist that can facilitate curriculum development in both traditional content areas such as informed consent and privacy as well as newer content areas such as social media use. Copyright © 2013 Academic Pediatric Association. Published by Elsevier Inc. All rights reserved.
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.
Guerrero-Tinoco Gustavo Adolfo; Julio-Barrios Emil
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...
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)
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.)
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...
Cabalka, A K; Rosenblatt, H M; Towbin, J A; Price, J K; Windsor, N T; Martin, A B; Louis, P T; Frazier, O H; Bricker, J T
Clinical features of postpericardiotomy syndrome (PPS) occur in pediatric heart transplant recipients despite immunosuppression, which raises questions about the mechanism of PPS. We studied the clinical and immunologic characteristics of 15 pediatric heart transplant patients, ages 1.1 to 17.8 years (mean, 7.5 years); 7 had clinical evidence of PPS (PPS+), and 8 were without clinical features of PPS (PPS-). Indicators of PPS included fever, irritability, pericardial friction rub, leukocytosi...
Kalantari, Minoo; Kamali, Mohammad; Joolaee, Soodabeh; Shafarodi, Narges; Rassafiani, Mehdi
It is the responsibility of each occupational therapist to always act ethically and professionally in a clinical setting. However, there is little information available concerning the factors influencing ethical behavior of occupational therapists at work. Since no study has been conducted in Iran on this topic, this qualitative study aimed to identify the factors influencing ethical behavior of pediatric occupational therapists. Twelve pediatric occupational therapists participated in this study. The sampling was purposeful, and the interviews continued until reaching data saturation. All interviews were recorded and transcribed. The data were analyzed by qualitative content analysis, and the ethics of qualitative research was considered. The factors influencing ethical behavior were classified into four main categories including organizational factors, therapist related factors, client's family issues, and social factors. This study identified numerous factors influencing the ethical behavior of pediatric occupational therapists that could be used to train occupational therapists, human resources managers, professional policy makers, and could also be used to conduct future researches, and produce tools.
Moon, Margaret; Taylor, Holly A; McDonald, Erin L; Hughes, Mark T; Beach, Mary Catherine; Carrese, Joseph A
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.
Needham, Allison C.; Kapadia, Mufiza Z.; Offringa, Martin
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
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.
Kumra, Sanjiv; Ashtari, Manzar; Anderson, Britt; Cervellione, Kelly L.; Kan, Li
Objective: The authors examined the ethical and practical management issues resulting from the detection of incidental abnormal findings on magnetic resonance imaging (MRI) research studies in healthy pediatric volunteers. Method: A retrospective examination of the findings from 60 clinical reports of research MRI scans from a cohort of healthy…
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.
Valentine, Stacey L; Nadkarni, Vinay M; Curley, Martha A Q
To describe the recommendations from the Pediatric Acute Lung Injury Consensus Conference on nonpulmonary treatments in pediatric acute respiratory distress syndrome. Consensus conference of experts in pediatric acute lung injury. A panel of 27 experts met over the course of 2 years to develop a taxonomy to define pediatric acute respiratory distress syndrome and to make recommendations regarding treatment and research priorities. The nonpulmonary subgroup comprised three experts. When published data were lacking, a modified Delphi approach emphasizing strong professional agreement was utilized. The Pediatric Acute Lung Injury Consensus Conference experts developed and voted on a total of 151 recommendations addressing the topics related to pediatric acute respiratory distress syndrome, 30 of which related to nonpulmonary treatment. All 30 recommendations had strong agreement. Patients with pediatric acute respiratory distress syndrome should receive 1) minimal yet effective targeted sedation to facilitate mechanical ventilation; 2) neuromuscular blockade, if sedation alone is inadequate to achieve effective mechanical ventilation; 3) a nutrition plan to facilitate their recovery, maintain their growth, and meet their metabolic needs; 4) goal-directed fluid management to maintain adequate intravascular volume, end-organ perfusion, and optimal delivery of oxygen; and 5) goal-directed RBC transfusion to maintain adequate oxygen delivery. Future clinical trials in pediatric acute respiratory distress syndrome should report sedation, neuromuscular blockade, nutrition, fluid management, and transfusion exposures to allow comparison across studies. The Consensus Conference developed pediatric-specific definitions for pediatric acute respiratory distress syndrome and recommendations regarding treatment and future research priorities. These recommendations for nonpulmonary treatment in pediatric acute respiratory distress syndrome are intended to promote optimization and
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)
Fisher, Celia B.
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
Sauerland, Jeanie; Marotta, Kathleen; Peinemann, Mary Anne; Berndt, Andrea; Robichaux, Catherine
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.
Mercurio, M R
Institutional Ethics Committees are commonly available in hospitals with newborn intensive care units, and may serve as a valuable resource for staff and parents dealing with difficult ethical decisions. Many clinicians may be unaware of when the committee might be helpful, or how it functions. After a brief historical introduction, two cases are presented as illustrations of pediatric ethics committee function. The first involves consideration of cardiac surgery for an infant with ventricular septal defect and Trisomy 13. The second involves disagreement between staff and parents regarding possible provision of cardio-pulmonary resuscitation in a terminally ill newborn. Principles and considerations often brought to bear in committee deliberations are reviewed for each case. Neonatologists, staff and families should be aware of this potentially valuable resource, and are encouraged to use it for situations of moral distress, conflict resolution or ethical uncertainty.
Picchietti, Daniel L; Bruni, Oliviero; de Weerd, Al; Durmer, Jeffrey S; Kotagal, Suresh; Owens, Judith A; Simakajornboon, Narong
Specific diagnostic criteria for pediatric restless legs syndrome (RLS) were published in 2003 following a workshop at the National Institutes of Health. Due to substantial new research and revision of the adult RLS diagnostic criteria, a task force was chosen by the International Restless Legs Syndrome Study Group (IRLSSG) to consider updates to the pediatric diagnostic criteria. A committee of seven pediatric RLS experts developed a set of 15 consensus questions to review, conducted a comprehensive literature search, and extensively discussed potential revisions. The committee recommendations were approved by the IRLSSG executive committee and reviewed by the IRLSSG membership. The pediatric RLS diagnostic criteria were simplified and integrated with the newly revised adult RLS criteria. Specific recommendations were developed for pediatric application of the criteria, including consideration of typical words used by children to describe their symptoms. Pediatric aspects of differential diagnosis, comorbidity, and clinical significance were then defined. In addition, the research criteria for probable and possible pediatric RLS were updated and criteria for a related condition, periodic limb movement disorder (PLMD), were clarified. Revised diagnostic criteria for pediatric RLS have been developed, which are intended to improve clinical practice and promote further research. Copyright © 2013 The Authors. Published by Elsevier B.V. All rights reserved.
Milkovic, D.; Ranogajec Komor, M.
X-ray diagnostic of paranasal sinuses of children has some specifics. The sinuses are not completely developed under the age of 5-7 years; oral cavity and the retina of the eyes are directly exposed to ionising radiation. Children are more radiosensitive than other population. Therefore in our long-term research many efforts were done to improve the radiation protection of the children patients. In that purpose doses were measured on the children patients and phantom with thermoluminescent (TL) and radiophotoluminescent (RPL) dosimetric systems. Our experience shows that in addition to technical parameters human factors have significant influence on the radiation protection in X-ray diagnostic of sinuses. Therefore, compliance of ethical principles is very important. According to the IRPA Code of Ethics medical staff shall not allow to compromise their professional judgement and advice, i.e. medical indication for X-ray examination has to be decided according to best knowledge. Further, the Code of Ethics recommends that medical staff should strive to improve their own professional knowledge and skills. The dosimetric results in our study indicated quantitatively the positive role of continuous education and good teamwork for dose reduction. In accordance with the ethics in radiation protection it is necessary to use correct algorithm of diagnostic images as well as all devices for radiation protection. (author).
Benoit, J; Berdah, L; Carlier-Gonod, A; Guillou, T; Kouche, C; Patte, M; Schneider, M; Talcone, S; Chappuy, H
Children suffer most from today's increasing precariousness. In France, access to care is available for all children through various structures and existing measures. The support for foreign children is overseen by specific legislation often unfamiliar to caregivers. Pediatric emergencies, their location, organization, actors, and patient flow are a particular environment that is not always suitable to communication and may lead to situations of abuse. Communication should not be forgotten because of the urgency of the situation. The place of the child in the dialogue is often forgotten. Considering the triangular relationship, listening to the child and involving the parents in care are the basis for a good therapeutic alliance. Privacy and medical confidentiality in pediatric emergencies are governed by law. However, changes in treatments and medical practices along with the variety of actors involved imply both individual and collective limitations, to the detriment of medical confidentiality. Copyright © 2015 Elsevier Masson SAS. All rights reserved.
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.
Burns, Natalie S.; Stevens, Anne M.; Iyer, Ramesh S.
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.)
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)
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.)
Fémel, Maryline; Dosne, Sandrine; Akeb, Lila
An organ and tissue removal coordination department organised a drawing competition for the children in the paediatric unit, on the theme: "Donation, passing on the baton". This ethical reflection combining respect for the children, interaction with the parents and multi-disciplinary collaboration provided an opportunity for exchanges on the topic of organ donation and encouraged a different approach on the part of the medical team. Copyright © 2015 Elsevier Masson SAS. All rights reserved.
Streuli, Jürg C; Staubli, Georg; Pfändler-Poletti, Marlis; Baumann-Hölzle, Ruth; Ersch, Jörg
Our retrospective study presents and evaluates clinical ethics consultations (CECs) in pediatrics as a structure for implementing hospital-wide ethics. We performed a descriptive and statistical analysis of clinical ethics decision making and its implementation in pediatric CECs at Zurich University Children's Hospital. Ninety-five CECs were held over 5 years for 80 patients. The care team reached a consensus treatment recommendation after one session in 75 consultations (89 %) and on 82 of 84 ethical issues (98 %) after two or more sessions (11 repeats). Fifty-seven CECs recommended limited treatment and 23 maximal treatment. Team recommendations were agreed outright by parents and/or patient in 59 of 73 consultations (81 %). Initial dissensus yielded to explanatory discussion or repeat CEC in seven consultations (10 %). In a further seven families (10 %), no solution was found within the CEC framework: five (7 %) required involvement of the child protection service, and in two families, the parents took their child elsewhere. Eventual team-parent/patient consensus was reached in 66 of 73 families (90 %) with documented parental/patient decisions (missing data, n = 11). Patient preference was assessable in ten CECs. Patient autonomy was part of the ethical dilemma in only three CECs. The Zurich clinical ethics structure produced a 98 % intra-team consensus rate in 95 CECs and reduced initial team-parent dissensus from 21 to 10 %. Success depends closely on a standardized CEC protocol and an underlying institutional clinical ethics framework embodying a comprehensive set of transparently articulated values and opinions, with regular evaluation of decisions and their consequences for care teams and families.
Avard, Denise M; Knoppers, Bartha M
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.
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
Cuzzoni, Eva; De Iudicibus, Sara; Franca, Raffaella; Stocco, Gabriele; Lucafò, Marianna; Pelin, Marco; Favretto, Diego; Pasini, Andrea; Montini, Giovanni; Decorti, Giuliana
Idiopathic nephrotic syndrome represents the most common type of primary glomerular disease in children: glucocorticoids (GCs) are the first-line therapy, even if considerable interindividual differences in their efficacy and side effects have been reported. Immunosuppressive and anti-inflammatory effects of these drugs are mainly due to the GC-mediated transcription regulation of pro- and anti-inflammatory genes. This mechanism of action is the result of a complex multistep pathway that involves the glucocorticoid receptor and several other proteins, encoded by polymorphic genes. Aim of this review is to highlight the current knowledge on genetic variants that could affect GC response, particularly focusing on children with idiopathic nephrotic syndrome.
Sarnaik, Ajit A; Clark, Jeff A; Meert, Kathleen L; Sarnaik, Ashok P
Donation after cardiac death has been endorsed by professional organizations, including the American Academy of Pediatrics as a means of increasing the supply of transplantable organs. However, ethical concerns have been raised about donation after cardiac death, especially in children. This study explores the views of pediatric intensive care physicians on the ethics of pediatric donation after cardiac death. Internet survey. Physician members of the American Academy of Pediatrics Section of Critical Care. Physicians were emailed an anonymous survey consisting of four demographic items and 16 items designed to assess their views on the ethics of pediatric donation after cardiac death. Responses to ethics items were rated on a 5-point scale ranging from strongly disagree to strongly agree. Physicians were also given the opportunity to provide free-text comments regarding their views. Of the 598 eligible physicians, 264 (44.1%) responded to the survey. Of these, 193 (73.4%) were practicing in a transplant center and 160 (60.6%) participated in at least one donation after cardiac death procedure at the time of survey completion. Two hundred twenty (83.4%) agreed or strongly agreed that regarding donation after cardiac death, parents should be able to make decisions based on the best interests of their child. Two hundred twenty-two (84.1%) agreed or strongly agreed that it is not acceptable to harvest organs from a child before the declaration of death, consistent with the Dead Donor Rule. However, only 155 (59.1%) agreed or strongly agreed that the time of death in donation after cardiac death can be conclusively determined. Twenty-nine (11.0%) agreed or strongly agreed that the pediatric donation after cardiac death donor may feel pain or suffering during the harvest procedure. Most pediatric intensive care physicians agree that the Dead Donor Rule should be applied for donation after cardiac death and that donation after cardiac death can be consistent with the
Noordam, C.; Thoonen, G.H.J.; Burgt, C.J.A.M. van der
Noonan syndrome is a relatively common autosomal dominant condition characterised by cardiac defects, short stature, feeding difficulties during the first year of life, and learning and behavioural problems later in life. The diagnosis is clinical and in 50% of cases it can be confirmed by a
Meroni, Pier Luigi; Argolini, Lorenza Maria; Pontikaki, Irene
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.
Natasa Marcun Varda
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.
Martha Leonor Paradoa Pérez
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.
Thieleman, Kara J; Wallace, Cara; Cimino, Andrea N; Rueda, Heidi A
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.
Kay, Marsha; Eng, Katharine; Wyllie, Robert
Gastrointestinal polyps are commonly encountered during childhood and are one of the most common causes of rectal bleeding in this age group. Most polyps are benign and located in the colon, with the most frequent type being juvenile polyps. However, in older pediatric patients, if multiple polyps are present, in patients who have a positive family history, or if polyps are located outside of the colon, either adenomatous polyps or polyps associated with genetic abnormalities are more common. Imaging techniques such as ultrasound and computed tomographic colonoscopy have recently been utilized to identify simple juvenile colonic polyps in children with rectal bleeding in whom there is a high index of suspicion. Colonoscopy with polypectomy is still required for histologic evaluation and resection of the polyp. There have been significant advances in genetic testing and management of hereditary gastrointestinal cancer syndromes with onset in childhood or adolescence that may ultimately reduce long-term morbidity and mortality. In addition to enhanced gastrointestinal and extraintestinal malignancy screening for affected individuals, specific gene mutations within a given condition such as adenomatous polyposis coli may predict clinical course and timing of specific interventions such as colectomy. In other conditions such as phosphatase and tensin homolog hamartoma tumor syndrome, phenotype may not be predicted by genotype. Pediatricians, pediatric gastroenterologists, and adult gastroenterologists caring for children should understand how to differentiate benign polyps in the pediatric age group from those associated with a higher risk of complications including recurrence risk and risk of development of intestinal or extraintestinal malignancy. Recent advances in genetic testing, as well as development of consensus guidelines, are key in the identification, screening, and follow-up of children and adolescents with polyposis syndromes.
Greene, D J; Williams Iii, A C; Koller, J M; Schlaggar, B L; Black, K J
Previous studies of brain structure in Tourette syndrome (TS) have produced mixed results, and most had modest sample sizes. In the present multicenter study, we used structural magnetic resonance imaging (MRI) to compare 103 children and adolescents with TS to a well-matched group of 103 children without tics. We applied voxel-based morphometry methods to test gray matter (GM) and white matter (WM) volume differences between diagnostic groups, accounting for MRI scanner and sequence, age, sex and total GM+WM volume. The TS group demonstrated lower WM volume bilaterally in orbital and medial prefrontal cortex, and greater GM volume in posterior thalamus, hypothalamus and midbrain. These results demonstrate evidence for abnormal brain structure in children and youth with TS, consistent with and extending previous findings, and they point to new target regions and avenues of study in TS. For example, as orbital cortex is reciprocally connected with hypothalamus, structural abnormalities in these regions may relate to abnormal decision making, reinforcement learning or somatic processing in TS.
Siregar, R. S.; Daulay, K. R.; Siregar, B.; Ramayani, O. R.; Eyanoer, P. C.
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.
Cully, Jennifer L
Drug overdose and opioid-involved deaths continue to increase across the United States. An end-condition related to opioid abuse is neonatal abstinence syndrome (NAS). The implications for pregnant or post-partum women using an opioid, whether prescribed or illicit, can have significant effects on their infant. First described in the 1970s, NAS is defined as a postnatal drug withdrawal syndrome that primarily occurs in opioid-exposed infants. There are no nationally accepted, evidence-based treatment protocols for these infants. Pediatric dentists should be cognizant of potential dental development issues and developmental delays in children with NAS. Basic behavior guidance techniques, such as tell-show-do, may not be as effective. In addition, adherence to prescription drug monitoring programs can help identify patients who may have received multiple opioids, alerting the provider to possible misuse or abuse. The purpose of this paper was to provide background information on the incidences and potential dental implications for children with neonatal abstinence syndrome.
Benavides, Sandra; Kohler, Lisa A.; Souffrant, Garry
Purpose: The prevalence of metabolic syndrome in the pediatric population is increasing. Barriers, including the lack of consensus of a definition for metabolic syndrome and time constraints for the pediatrician, may limit the identification and diagnosis of metabolic syndrome in children. The objective of this pilot study was to evaluate the role…
Glover, Noreen M.; Glover, Samuel J.
Selective abortion of fetuses with Down syndrome is discussed in terms of abortion perspectives, genetic testing, legislation, and ethical principles. The ethical principles of autonomy, beneficence, nonmaleficence, fidelity, and justice are offered as guidelines for the examination of legal standards imposed by legislation. (Author/PB)
Coffino, Samantha W; Fryer, Robert H
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.
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.
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.
Stratakis, Constantine A.
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
Ingelse, Sarah A; Wösten-van Asperen, Roelie M; Lemson, Joris; Daams, Joost G; Bem, Reinout A; van Woensel, Job B
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.
Sarah A Ingelse
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.
Full Text Available Nephrotic syndrome (NS is a common renal disorder with significant tubulo-interstitial damage due to the combined effects of proteinuria and obstruction of efferent blood flow. Peritubular capillary (PTC loss has also been correlated with interstitial fibrosis. This study included 30 pediatric cases of idiopathic NS. Clinical details, including biochemical parameters, were recorded and renal biopsy slides were reviewed for histological features. PTCs were highlighted using anti-CD34 antibody and quantified with the help of image analysis software. Postmortem kidney biopsies from seven children were taken as controls for quantification of PTCs and interstitial fibrosis. Wherever possible, as ultrastructural examination of the renal biopsy was performed. Appropriate statistical methods were applied. Patients with minimal change disease (MCD had lower serum creatinine as compared with those with focal and segmental glomerulosclerosis (FSGS. Similarly, tubular atrophy and interstitial fibrosis were significantly lower in MCD than in FSGS. PTC density was lower in all groups of NS as compared with the controls. Biopsies with FSGS had a lower PTC density compared with both MCD and mesangioproliferative glomerulonephritis. PTC density showed a negative correlation with serum creatinine and degree of proteinuria. PTC loss appears to play an important role in the development of renal biopsy changes in pediatric NS. This aspect of the renal vasculature requires further study in idiopathic NS.
Ajit Ashok Sarnaik
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.
Sarnaik, Ajit A
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.
Livingston, Kristin; Glotzbecker, Michael; Miller, Patricia E; Hresko, Michael T; Hedequist, Daniel; Shore, Benjamin J
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
J Gordon Millichap
Investigators from University Hospital of Rennes; Necker Hospital, Paris; and University Paris Descartes, France used a questionnaire to study the transition and transfer from pediatric to adult health-care system in patients with Dravet syndrome and their families.
Tamburro, Robert F.; Kneyber, Martin C. J.
Objective: To provide an overview of the current literature on pulmonary-specific therapeutic approaches to pediatric acute respiratory distress syndrome to determine recommendations for clinical practice and/or future research. Data Sources: PubMed, EMBASE, CINAHL, SCOPUS, and the Cochrane Library
Matos, Jesus M; Gonzalez, Lorena; Kfoury, Elias; Echeverria, Angela; Bechara, Carlos F; Lin, Peter H
Objectives Thoracic outlet syndrome, a condition commonly reported in adults, occurs infrequently in the pediatric population. The objective of this study was to assess the outcome of surgical interventions of thoracic outlet syndrome in pediatric patients. Methods Clinical records of all pediatric patients with thoracic outlet syndrome who underwent operative repair from 2002 to 2015 in a tertiary pediatric hospital were reviewed. Pertinent clinical variables and treatment outcomes were analyzed. Results Sixty-eight patients underwent a total of 72 thoracic outlet syndrome operations (mean age 15.7 years). Venous, neurogenic, and arterial thoracic outlet syndromes occurred in 39 (57%), 21 (31%), and 8 (12%) patients, respectively. Common risk factors for children with venous thoracic outlet syndrome included sports-related injuries (40%) and hypercoagulable disorders (33%). Thirty-five patients (90%) with venous thoracic outlet syndrome underwent catheter-based interventions followed by surgical decompression. All patients underwent first rib resection with scalenectomy via either a supraclavicular approach (n = 60, 88%) or combined supraclavicular and infraclavicular incisions (n = 8, 12%). Concomitant temporary arteriovenous fistula creation was performed in 14 patients (36%). Three patients with arterial thoracic outlet syndrome underwent first rib resection with concomitant subclavian artery aneurysm repair. The mean follow-up duration was 38.4 ± 11.6 months. Long-term symptomatic relief was achieved in 94% of patients. Conclusions Venous thoracic outlet syndrome is the most common form of thoracic outlet syndrome in children, followed by neurogenic and arterial thoracic outlet syndromes. Competitive sports-related injuries remain the most common risk factor for venous and neurogenic thoracic outlet syndromes. Temporary arteriovenous fistula creation was useful in venous thoracic outlet syndrome patients in selective children. Surgical
Liu, Bin; Kaplan, Summer L; Zhuang, Hongming
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.
Cubillos C, María Paz; Del Salas, Paulina; Zambrano, Pedro O
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.
Jouvet, Philippe; Thomas, Neal J.; Willson, Douglas F.; Erickson, Simon; Khemani, Robinder; Smith, Lincoln; Zimmerman, Jerry; Dahmer, Mary; Flori, Heidi; Quasney, Michael; Sapru, Anil; Cheifetz, Ira M.; Rimensberger, Peter C.; Kneyber, Martin; Tamburro, Robert F.; Curley, Martha A. Q.; Nadkarni, Vinay; Valentine, Stacey; Emeriaud, Guillaume; Newth, Christopher; Carroll, Christopher L.; Essouri, Sandrine; Dalton, Heidi; Macrae, Duncan; Lopez-Cruces, Yolanda; Quasney, Michael; Santschi, Miriam; Watson, R. Scott; Bembea, Melania
Objective: To describe the final recommendations of the Pediatric Acute Lung Injury Consensus Conference. Design: Consensus conference of experts in pediatric acute lung injury. Setting: Not applicable. Subjects: PICU patients with evidence of acute lung injury or acute respiratory distress
Vries, M.C. de; Houtlosser, M.; Wit, J.M.; Engberts, D.P.; Bresters, D.; Kaspers, G.J.L.; Leeuwen, E. van
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
Hevia, Pilar; Nazal, Vilma; Rosati, María Pía; Quiroz, Lily; Alarcón, Claudia; Márquez, Sonia; Cuevas, Karen
Idiopathic nephrotic syndrome is the most common glomerular disease in childhood, affecting 1 to 3 per 100,000 children under the age of 16. It most commonly occurs in ages between 2 and 10. Its cause is unknown, and its histology corresponds to minimal change disease in 90% of cases, or focal segmental glomerulosclerosis. Steroid-resistant nephrotic syndrome represents 10-20% of idiopathic nephrotic syndrome in pediatrics. It has a poor prognosis, and its management is a significant therapeutic challenge. Half of patients evolve to end-stage renal disease within 5 years, and are additionally exposed to complications secondary to persistent NS and to the adverse effects of immunosuppressive therapy. The primary goal of treatment is to achieve complete remission, but even a partial remission is associated with a better renal survival than the lack of response. This paper is the result of the collaborative effort of the Nephrology Branch of the Chilean Society of Pediatrics with aims at helping pediatricians and pediatric nephrologists to treat pediatric idiopathic nephrotic syndrome. In this second part, handling of steroid-resistant nephrotic syndrome as well as nonspecific therapies are discussed. Copyright © 2015 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.
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.
Ingelse, Sarah A.; Wösten-van Asperen, Roelie M.; Lemson, Joris; Daams, Joost G.; Bem, Reinout A.; van Woensel, Job B.
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
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.
Eisenmann, Joey C
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 s...
Rothner, A David; Parikh, Sumit
To provide an overview of the clinical course for children and adolescents with migraine variants (M.V.), childhood periodic syndromes or the episodic syndromes that may be associated with migraine using the International Classification of Headache Disorders, 3rd Edition Beta version [ICHD-3] International Headache Society criteria for the diagnosis of each disorder. Migraine is a complex set of neurological symptoms. This review encompasses the subtypes of M.V. or episodic syndromes that may be associated with migraine within the children and adolescent population. The episodic syndromes that may be associated with migraine or migraine variant is multilayered neurological disorder in young children and adolescents. Within the these generally pediatric syndromes there are associated disorders described in this review, to provide a clinical overview and including the less common forms of migraine, such as acute confusional migraine, trauma-triggered migraine, and transient global amnesia. © 2015 American Headache Society.
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...
Hevia, Pilar; Nazal, Vilma; Rosati, María Pía; Quiroz, Lily; Alarcón, Claudia; Márquez, Sonia; Cuevas, Karen
Idiopathic nephrotic syndrome is the most common glomerular disease in childhood, affecting 1 to 3 per 100,000 children under the age of 16. It most commonly occurs in ages between 2 and 10. Its cause is unknown and its histology corresponds to minimal change disease in 90% of cases, or focal segmental glomerulosclerosis. 80 to 90% of cases respond to steroids (steroid-sensitive nephrotic syndrome) with good prognosis and long-term preservation of renal function over time. 70% of patients with SSNS have one or more relapses in their evolution, and of these, 50% behave as frequent relapsing or steroid-dependent, a group that concentrate the risk of steroid toxicity. Patients with steroid-resistant nephrotic syndrome have a poor prognosis and 50% of them evolve to end-stage renal disease. The goal of therapy is to induce and maintain remission of the disease, reducing the risk secondary to proteinuria while minimizing the adverse effects of treatments, especially with prolonged use of corticosteroids. This paper is the result of the collaborative effort of the Nephrology Branch of the Chilean Society of Pediatrics with aims at helping pediatricians and pediatric nephrologists to treat pediatric SNI. In this first part, recommendations of steroid-sensitive nephrotic syndrome are discussed. Copyright © 2015 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.
van den Berg, S A A; van 't Veer, N E; Emmen, J M A; van Beek, R H T
We present a case of iatrogenic Cushing's syndrome, induced by treatment with fluticasone furoate (1-2 dd, 27.5 µg in each nostril) in a pediatric patient treated for congenital HIV. The pediatric patient described in this case report is a young girl of African descent, treated for congenital HIV with a combination therapy of Lopinavir/Ritonavir (1 dd 320/80 mg), Lamivudine (1 dd 160 mg) and Abacavir (1 dd 320 mg). Our pediatric patient presented with typical Cushingoid features (i.e. striae of the upper legs, full moon face, increased body and facial hair) within weeks after starting fluticasone furoate therapy, which was exacerbated after increasing the dose to 2 dd because of complaints of unresolved rhinitis. Biochemical analysis fitted iatrogenic Cushing's syndrome, with a repeatedly low cortisol (iatrogenic Cushing's syndrome in patients treated for HIV due to the strong inhibition of CYP3 enzymes by Ritonavir. Upon discontinuation of fluticasone treatment, the pediatric patient improved both clinically and biochemically with normalisation of cortisol and ACTH within a couple of weeks. Fluticasone therapy may induce iatrogenic Cushing's syndrome in a patient treated with anti-retroviral therapy.Pharmacogenetic analysis, in particular CYP3A genotyping, provides useful information in patients treated for HIV with respect to possible future steroid treatment.Fluticasone furoate is not detected in the Siemens Immulite cortisol binding assay.
Martha Ortiz García
Full Text Available Disímiles son los problemas éticos de la práctica médica que en la especialidad de Pediatría tienen la peculiaridad de involucrar a la madre y a otros familiares, pero que trasciende al compromiso con la salud cubana y el país. Se abordan algunos dilemas éticos como el manejo del niño con anorexia, la atención al paciente con afecciones crónicas, aspectos del diagnóstico prenatal y las creencias religiosas. Como dificultades se señala la falta de información al paciente y sus familiares y el uso inadecuado de medicamentos e investigaciones. Finalmente se enfatiza en que la aplicación del método clínico debe ir acompañada de los aspectos éticos y en el buen funcionamiento de los comités de ética en los servicios de salud.There are different ethical problems in Pediatrics that have the peculiarity of involving the mother and other relatives, and that go beyond to the commitment with the Cuban health and the country. Some ethical dilemmas as the management of the child with anorexia, the attention to the patient with chronic affections, aspects of the prenatal diagnosis and religious believes are dealt with. The deficient information given to the patients and his relatives and the inadequate use of drugs and investigations are stressed as difficulties. Finally, emphasis is given to the fact that the application of the clinical method should be accompanied by the ethical aspects and the good functioning of the ethic committees in the health services.
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.
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.
Yang, Hye Ran; Yi, Dae Yong; Choi, Hyoung Soo
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.
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 ...
Gudrunardottir, Thora; Morgan, Angela T; Lux, Andrew L
-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...
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
Downs, Jenny; Forbes, David; Johnson, Michael; Leonard, Helen
Rett syndrome is a rare disorder caused by a mutation in the MECP2 gene. Those affected generally have severe functional impairments, and medical comorbidities such as scoliosis and poor growth are common. There is a paucity of information on the natural history of many rare disorders and an even greater deficit of evidence to guide best practice. The population-based and longitudinal Australian Rett Syndrome Database established in 1993 has supported investigations of the natural history of Rett syndrome and effectiveness of treatments. This paper reviews the disorder Rett syndrome and evidence for the management of scoliosis and poor growth within a clinical ethics framework. Compared with conservative management, we have shown that spinal fusion is associated with reduced mortality and better respiratory health. We have also shown that gastrostomy insertion is associated with subsequent weight gain. Family counselling for both procedures necessarily must include family perspectives and careful clinical attention to their needs and wishes. Vignettes describing family decision-making and experiences are presented to illustrate the principals of beneficence and autonomy in determining the best interests of the child and family. A blend of evidence-based practice with a strong clinical ethics framework has capacity to build existing strengths in families and reduce the negative impacts of disability and in so doing, optimise the health and wellbeing of those with Rett syndrome. © 2016 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).
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.
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
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.
Khatib, Hassan El; Naous, Amal; Ghanem, Soha; Dbaibo, Ghassan; Rajab, Mariam
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.
Mariucci, Elisabetta; Guidarini, Marta; Donti, Andrea; Lovato, Luigi; Wischmeijer, Anita; Angeli, Emanuela; Gargiulo, Gaetano D; Picchio, Fernando M; Bonvicini, Marco
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.
Perito, E R; Lustig, R H; Rosenthal, P
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.
Gavigan, Geneviève M; Kanigsberg, Nordau D; Ramien, Michele L
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.
Shirakami, Gotaro; Tazuke-Nishimura, Misako; Hirakata, Hideo; Fukuda, Kazuhiko
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.
Hefti, Erik; Blanco, Javier G
Children with Down syndrome (DS) have a 10- to 30-fold increased risk of developing acute myeloid leukemia or acute lymphoblastic leukemia. Patients with DS and leukemia are treated with the same chemotherapeutic agents as patients without DS. Treatment regimens for pediatric leukemia comprise multiple cytotoxic drugs including methotrexate, doxorubicin, vincristine, cytarabine, and etoposide. There have been reports of increased toxicity, as well as altered therapeutic outcomes in pediatric patients with DS and leukemia. This review is focused on the pharmacokinetics of cytotoxic drugs in pediatric patients with leukemia and DS. The available literature suggests that methotrexate and thioguanine display altered pharmacokinetic parameters in pediatric patients with DS. It has been hypothesized that the variable pharmacokinetics of these drugs may contribute to the increased incidence of treatment-related toxicities seen in DS. Data from a small number of studies suggest that the pharmacokinetics of vincristine, etoposide, doxorubicin, and busulfan are similar between patients with and without DS. Definitive conclusions regarding the pharmacokinetics of cytotoxic drugs in pediatric patients with leukemia and DS are difficult to reach due to limitations in the available studies.
Typical clinical sequelae include gastrointestinal disturbances, neuromuscular effects, cardiovascular complications, acute renal failure and death. Tumor lysis syndrome can also compromise the efficacy or administration of curative therapies. Available evidence suggests that the incidence of clinical TLS is approximately ...
de Weerd, Al; Arico, Irene; Silvestri, Rosalia
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
Acker, Karen P; Fetch, Audrey; Schnell, Stephanie A; Hammond, Jennifer; Herrera, Christina; Niedt, George; Ratner, Adam J; Lauren, Christine T
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.
Sakugawa, Yoko; Kamizato, Kota; Miyata, Yuji; Kakinohana, Manabu; Sugahara, Kazuhiro
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.
Tincani, Bruna J.; Mascagni, Bruno R.; Pinto, Roberto D. P.; Guaragna-Filho, Guilherme; Castro, Carla C. T. S.; Sewaybricker, Letícia E.; Viguetti-Campos, Nilma L.; Marques-de-Faria, Antonia P.; Maciel-Guerra, Andréa T.; Guerra-Júnior, Gil
OBJECTIVE: To identify clinical and laboratory data which differentiate Klinefelter syndrome (KS) patients according to age group. METHODS: The study included all cases of hypogonadism, gynecomastia and/or infertility whose karyotype was performed at a university hospital from January 1989 to December 2011, in a total of 105 subjects. The following data were retrospectively analyzed: age at first visit, ratio of arm span to height, pubic hair, gynecomastia, testicular volume, luteinizing horm...
Pellegrino, Edmund D
In this brief annual review of ethical issues in medicine, Pellegrino focuses on two issues, AIDS and surrogate mothers. The AIDS epidemic has generated debate over public health needs vs. individual rights, modification of sexual practices, screening programs to detect infected persons, confidentiality of test results, experimental therapies, and the duty of physicians to care for AIDS patients. Surrogate motherhood arrangements have become one of the more controversial of the new reproductive technologies. The publicity that accompanied the custody battle over New Jersey's "Baby M" intensified debate over the commercialization of childbearing and the regulation of reproduction. Pellegrino concludes that physicians, along with ethicists and policymakers, have an obligation to "lead society in careful and judicious deliberation" of the ethical issues raised by AIDS and by reproductive technologies.
Spackman, T. J.
The utilization of the Lixiscope in pediatrics was investigated. The types of images that can presently be obtained are discussed along with the problems encountered. Speculative applications for the Lixiscope are also presented.
Rasheed, Shabana; Teo, Harvey James Eu Leong; Littooij, Annemieke Simone
Imaging of pediatric patients involves many diverse modalities, including radiography, ultrasound imaging, computed tomography, magnetic resonance imaging, and scintigraphic and angiographic studies. It is therefore important to be aware of potential pitfalls that may be related to these modalities
Verrotti, Alberto; Pizzella, Vittorio; Trotta, Daniela; Madonna, Laura; Chiarelli, Francesco; Romani, Gian Luca
In recent years, great advances in the knowledge of neuromagnetism have permitted the application of Superconducting Quantum Interference Devices to the pathophysiologic study of the human brain. In particular, in pediatric neurology, the integration of biomagnetism with magnetic resonance imaging and other techniques for medical imaging have allowed for precise neuromagnetic measurements of the human brain. The more frequently used technique is magnetoencephalography. Recent data have illustrated the usefulness of magnetoencephalography in mapping activity of sensory and motor areas and in studying the spatiotemporal pattern of brain activation specific to somatosensory function. Moreover, magnetoencephalography is an important tool to localize epileptic activity; magnetic source imaging superimposes magnetoencephalographic localizations on the magnetic resonance imaging and yields improved spatial resolution as compared with surface electroencephalography. The role of magnetoencephalography in evaluating patients with epilepsy continues to evolve; in fact, it seems to be very useful in the localization of the epileptogenic zone in patients with partial epilepsy. This application of magnetoencephalography is essential in the selection of epileptic children candidates to surgical treatment of seizures.
Full Text Available Diagnosis of polycystic ovary syndrome (PCOS in adolescence still raises many questions. The problem is that the characteristics of normal puberty often coincide with PCOS symptoms, so a number of researchers suggest to apply more stringent requirements to diagnosing. We use a cautious approach to a final diagnosis of PCOS because of its interpretation as a global problem of somatic health — endocrine and metabolic status, cardiovascular and oncogenic risk. In addition, one of the main therapies for PCOS is application of combined oral contraceptives, which are undesirable to use in adolescent girls with oligo- and amenorrhea without careful examination and use of all the abilities of vitamine therapy, phytotherapy and gestagens.
Full Text Available Viral infections are an important cause of pediatric Acute Respiratory Distress Syndrome (ARDS. Numerous viruses, including respiratory syncytial virus (RSV and influenza A (H1N1 virus, have been implicated in the progression of pneumonia to ARDS; yet the incidence of progression is unknown. Despite acute and chronic morbidity associated with respiratory viral infections, particularly in ‘at risk’ populations, treatment options are limited. Thus, with few exceptions, care is symptomatic. In addition, mortality rates for viral related ARDS have yet to be determined. This review outlines what is known about ARDS secondary to viral infections including the epidemiology, the pathophysiology and diagnosis. In addition, emerging treatment options to prevent infection, and to decrease disease burden will be outlined. We focused on RSV and influenza A (H1N1 viral-induced ARDS, as these are the most common viruses leading to pediatric ARDS, and have specific prophylactic and definitive treatment options.
Molteni, Erika; Avantaggiato, Paolo; Formica, Francesca; Pastore, Valentina; Colombo, Katia; Galbiati, Sara; Arrigoni, Filippo; Strazzer, Sandra
Sleep patterns of pediatric patients in unresponsive wakefulness syndrome (UWS) have been poorly investigated, and the prognostic potential of polysomnography (PSG) in these subjects is still uncertain. The goal of the study was to identify quantitative PSG indices to be applied as possible prognostic markers in pediatric UWS. We performed PSG in 27 children and adolescents with UWS due to acquired brain damage in the subacute phase. Patients underwent neurological examination and clinical assessment with standardized scales. Outcome was assessed after 36 mo. PSG tracks were scored for sleep stages and digitally filtered. The spectral difference between sleep and wake was computed, as the percent difference at specific spectral frequencies. We computed (1) the ratio between percent power in the delta and alpha frequency bands, (2) the ratio between alpha and theta frequency bands, and (3) the power ratio index, during wake and sleep, as proposed in previous literature. The predictive role of several clinical and PSG measures was tested by logistic regression. Correlation was found between the differential measures of electroencephalographic activity during sleep and wake in several frequency bands and the clinical scales (Glasgow Outcome Score, Level of Cognitive Functioning Assessment Scale, and Disability Rating Scale) at follow-up; the Sleep Patterns for Pediatric Unresponsive Wakefulness Syndrome (SPPUWS) scores correlated with the differential measures, and allowed outcome prediction with 96.3% of accuracy. The differential measure of electroencephalographic activity during sleep and wake in the beta band and, more incisively, SPPUWS can help in determining the capability to recover from pediatric UWS well before the confirmation provided by suitable clinical scales. © 2016 American Academy of Sleep Medicine.
Selewski, David T; Troost, Jonathan P; Cummings, Danyelle; Massengill, Susan F; Gbadegesin, Rasheed A; Greenbaum, Larry A; Shatat, Ibrahim F; Cai, Yi; Kapur, Gaurav; Hebert, Diane; Somers, Michael J; Trachtman, Howard; Pais, Priya; Seifert, Michael E; Goebel, Jens; Sethna, Christine B; Mahan, John D; Gross, Heather E; Herreshoff, Emily; Liu, Yang; Carlozzi, Noelle E; Reeve, Bryce B; DeWalt, Darren A; Gipson, Debbie S
Nephrotic syndrome represents a condition in pediatric nephrology typified by a relapsing and remitting course, proteinuria and the presence of edema. The PROMIS measures have previously been studied and validated in cross-sectional studies of children with nephrotic syndrome. This study was designed to longitudinally validate the PROMIS measures in pediatric nephrotic syndrome. One hundred twenty seven children with nephrotic syndrome between the ages of 8 and 17 years participated in this prospective cohort study. Patients completed a baseline assessment while their nephrotic syndrome was active, a follow-up assessment at the time of their first complete proteinuria remission or study month 3 if no remission occurred, and a final assessment at study month 12. Participants completed six PROMIS measures (Mobility, Fatigue, Pain Interference, Depressive Symptoms, Anxiety, and Peer Relationships), the PedsQL version 4.0, and two global assessment of change items. Disease status was classified at each assessment: nephrotic syndrome active in 100% at baseline, 33% at month 3, and 46% at month 12. The PROMIS domains of Mobility, Fatigue, Pain Interference, Depressive Symptoms, and Anxiety each showed a significant overall improvement over time (p change, the domains of Mobility, Fatigue, Pain Interference, and Anxiety consistently changed in an expected fashion. With the exception of Pain Interference, change in PROMIS domain scores did not correlate with changes in disease activity. PROMIS domain scores were moderately correlated with analogous PedsQL domain scores. This study demonstrates that the PROMIS Mobility, Fatigue, Pain Interference, and Anxiety domains are sensitive to self-reported changes in disease and overall health status over time in children with nephrotic syndrome. The lack of significant anchoring to clinically defined nephrotic syndrome disease active and remission status may highlight an opportunity to improve the measurement of HRQOL in children
Middleton, Elizabeth; Macksey, Lynn Fitzgerald; Phillips, J Duncan
This case report describes a 2.5-year-old girl who was hospitalized with complaints of abdominal pain and vomiting for 2 days. Abdominal ultrasound revealed small bowel-to-small bowel intussusception. Diagnostic laparoscopic-assisted exploration of the abdomen revealed 4 separate intestinal intussusceptions along with multiple dark intraluminal masses within the small intestine. Laparoscopic reduction of the intussusceptions was unsuccessful. Laparotomy allowed palpation of the entire small intestine with extraction of the masses, which were found to be human hair (trichobezoars). The intussusceptions were reduced, and the multiple masses were removed through a single enterotomy. The child recovered following surgery and was discharged home to her family. The surgeon counseled the parents before discharging the patient and recommended follow-up counseling for their child. The parents were given information about trichophagia and strategies to reduce the behavior in their child. A comprehensive literature review revealed this to be the youngest reported case of intussusception and Rapunzel syndrome due to trichobezoars.
Player, Brittany; Harmelink, Matthew; Bordini, Brett; Weisgerber, Michael; Girolami, Michael; Croix, Michael
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.
Byrd, Gentry; Quinonez, Rocio B; Offenbacher, Steven; Keels, Martha Ann; Guthmiller, Janet M
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.
Verger, Judy T; Bradshaw, Darla J; Henry, Elizabeth; Roberts, Kathryn E
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.
de Vries Martine C
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
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
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
Zanatta, Aline Bedin; Lucca, Sergio Roberto de
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.
Aline Bedin Zanatta
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.
Pearson, Thomas E; Frizzola, Meg A; Priest, Marc A; Rochman, Monica F; Froehlich, Curtis D
Takotsubo syndrome is rare in pediatric patients but must be considered in patients with subarachnoid hemorrhage with pulmonary edema and cardiomyopathy. A systematic, collaborative approach is needed to facilitate emergent transfer of patients where extracorporeal cardiopulmonary resuscitation (e-CPR) is used as a lifesaving measure. Extracorporeal membrane oxygenation (ECMO) use in transport requires preplanning, role delineation, resources, and research efforts to be successful. We present an unusual transport case of successful e-CPR/ECMO treatment of Takotsubo syndrome in a 12-year-old boy with an isolated traumatic intracranial injury, cardiomyopathy with pulmonary edema, and multiple cardiac arrests. Copyright © 2018 Air Medical Journal Associates. Published by Elsevier Inc. All rights reserved.
Oguzkurt, Levent; Tercan, Fahri; Sener, Mesut
A 10-year-old boy presented to our clinic with left lower extremity swelling present for 1 year with deterioration of symptoms during the prior month. Laboratory investigation for deep vein thrombosis was negative. Venography and computed tomography scan of the pelvis showed compression of the left common iliac vein by the right common iliac artery. A diagnosis of iliac vein compression syndrome was made. After venography, endovascular treatment was planned. The stenosis did not respond to balloon dilatation and a 12 mm Wallstent was placed with successful outcome. The patient's symptoms improved but did not resolve completely, probably due to a chronically occluded left superficial femoral vein that did not respond to endovascular recanalization. To the best of our knowledge, this is the first case of successful endovascular treatment of iliac vein compression syndrome with stent placement in a pediatric patient
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.
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.
Full Text Available Introduction In children with nephrotic syndrome, it is probable to determine a hypothyroid state because of thyroxine (T4, tri-iodothyronine (T3 and thyroid-binding globulin loss in presence of proteinuria. Objectives: To examine thyroid function in pediatric cases of nephrotic syndrome. Methods: In a cross-sectional study, from march 2010 to march 2012, thyroid function tests were performed in 104 patients referred to the nephrology department of children’s medical center, because of nephrotic syndrome. Collected data analyzed with SPSS Statistics 17 and pResults: Sixty one cases identified as hypothyroid patients and were treated with supplementary levothyroxine. There were 41 (67.2% males and 20 (32.8% females with the mean age of 3.72±3.35 years. Our patients showed lowered T3 (68.3% and T4 (64.4% in comparison with normal values. Median TSH (Thyroid-stimulating hormone was 11.65±6.71 Micu/ml and 2.82±0.82 in the hypothyroid and euthyroid patients respectively. In all, TSH was negatively correlated with the total urinary protein content . Conclusions: According to this study, the occurrence of hypothyroidism in any child with nephrotic syndrome needs to be mentioned. It is proposed to systematically search hypothyroidism by measuring TSH and free T4 in these patients particularly when proteinuria is prolonged.
Sleep apnea - pediatric; Apnea - pediatric sleep apnea syndrome; Sleep-disordered breathing - pediatric ... During sleep, all of the muscles in the body become more relaxed. This includes the muscles that help keep ...
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
Ramstein, J J; Halpern, J; Gadzinski, A J; Brannigan, R E; Smith, J F
The successful treatment of boys with cancer has led to increasing attention to preserving their quality of life after completing cancer therapy. One of the top priorities for living a full life is keeping open the opportunity to have children. While sperm banking for males facing sterilizing cancer treatment can be effective, this approach requires subsequent use of reproductive procedures such as in vitro fertilization (IVF) or intrauterine insemination (IUI) to achieve a pregnancy. Advances in fertility preservation techniques may allow pre-pubertal boys to conceive using advanced stem cell technologies and stem cell transplantation in the future. This review summarizes the ethical positions of leading medical societies and explores the religious and moral stances of major religious institutions regarding these options. © 2017 American Society of Andrology and European Academy of Andrology.
Higgins, S S
Parents of children with complex or terminal heart conditions often face agonizing decisions about cardiac transplantation. There are differences in the level of involvement that parents prefer when making such decisions. The purpose of this study was to identify and describe parents' preferences for their roles in decisions related to cardiac transplantation. A prospective ethnographic method was used to study 24 parents of 15 children prior to their decision of accepting or rejecting the transplant option for their children. Findings revealed that the style of parent decision making ranged from a desire to make an independent, autonomous choice to a wish for an authoritarian, paternalistic choice. Nurses and physicians can best support families in this situation, showing sensitivity to the steps that parents use to make their decisions. An ethical model of decision making is proposed that includes respect for differences in beliefs and values of all persons involved in the transplantation discussion. Copyright 2001 by W.B. Saunders Company
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.
Kashikar-Zuck, Susmita; Parkins, Irina S; Graham, Thomas Brent; Lynch, Anne M; Passo, Murray; Johnston, Megan; Schikler, Kenneth N; Hashkes, Philip J; Banez, Gerard; Richards, Margaret M
Mood and anxiety disorders are common psychiatric conditions among adult patients with fibromyalgia syndrome, but little is known about whether psychiatric disorders are prevalent among pediatric patients with fibromyalgia. The primary objective of this study was to assess the prevalence of mood, anxiety, and behavioral disorders in a clinical sample of children and adolescents with juvenile primary fibromyalgia syndrome (JPFS) and assess the relationship between psychiatric disorders and JPFS symptom severity. Standardized psychiatric interviews were conducted with children and their parents/primary caregivers, and measures of symptom severity including pain intensity and physician global ratings were obtained for 76 children and adolescents diagnosed with JPFS (ages 11 to 18 y) in pediatric rheumatology clinics at 4 hospitals in the Midwest. A total of 67.1% of patients had at least 1 current and 71.5% had at least 1 lifetime DSM-IV (Diagnostic and Statistical Manual of Mental Disorders-fourth edition) psychiatric diagnosis. The most frequent psychiatric diagnosis was anxiety disorder (57.5% of JPFS patients). Although mood difficulties were also common, the presence of major depression was lower than has been reported for adults with fibromyalgia syndrome. Physicians' global assessment of functioning was significantly lower for patients with a current anxiety disorder. There were no significant differences in pain severity among patients with and without anxiety, mood, or behavioral disorders. There seems to be a high prevalence of anxiety disorders in patients with JPFS, and presence of anxiety disorder is associated with poorer physician-rated functioning. Future research should explore whether early anxiety symptoms are predictive of long-term functioning.
Full Text Available 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.
Hoekman, Daniël R; Rutten, Juliette M T M; Vlieger, Arine M; Benninga, Marc A; Dijkgraaf, Marcel G W
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.
Objective: To investigate the plasma contents of VEGF in pediatric patients with nephrotic syndrome. Methods: Plasma VEGF contents were determined with ELISA in 31 pediatric patients with nephrotic syndrome both before and after treatment as well as in 35 controls. Results: Before treatment, the plasma contents of VEGF in the patients were significantly higher than those in the controls (P<0.01). After one month of treatment, the levels remained significantly higher (P<0.05). Conclusion: Development of nephrotic syndrome in pediatric patients was closely related to the plasma levels of VEGF. (authors)
Samuel, Susan M; Flynn, Rachel; Zappitelli, Michael; Dart, Allison; Parekh, Rulan; Pinsk, Maury; Mammen, Cherry; Wade, Andrew; Scott, Shannon D
Treatment protocols for childhood nephrotic syndrome are highly variable between providers and care centres. We conducted a qualitative study to understand the complex multilevel processes that lead to practice variation and influence provider management of nephrotic syndrome. Focus groups with multidisciplinary pediatric nephrology care providers ( n = 67) from 10 Canadian pediatric nephrology centres that had more than 1 pediatric nephrologist were conducted between September 2013 and April 2015. Focus group discussions were guided by the Ottawa Model for Research Use. We used a semistructured interview guide to elicit participants' perspectives regarding 1) the work setting and context of the clinical environment, 2) reasons for variation at the provider level and 3) clinical practice guidelines for nephrotic syndrome. Focus group discussions were transcribed and analyzed concurrently with the use of qualitative content analysis. Emerging themes were grouped into 2 categories: centre-level factors and provider-level factors. At the centre level, the type of care model used, clinic structures and resources, and lack of communication and collaboration within and between Canadian centres influenced care variation. At the provider level, use of experiential knowledge versus empirical knowledge and interpretation of patient characteristics influenced provider management of nephrotic syndrome. Centre- and provider-level factors play an important role in shaping practice differences in the management of childhood nephrotic syndrome. Further research is needed to determine whether variation in care is associated with disparities in outcomes. Copyright 2017, Joule Inc. or its licensors.
Drake, Madeline; Cox, Peter
Spinal muscular atrophy (SMA) is a group of progressive and fatal neurodegenerative disorders that are characterized by destruction of the anterior horn cells of the spinal cord. In this case report we outline the medical and ethical issues involved in a 7-year-old boy with SMA type 2 who experienced acute respiratory failure. A review of the literature was conducted focusing particularly on the pathology, presentation, and outcomes of SMA and end-of-life decision-making in pediatrics. In a world where 40%-60% of deaths in pediatric intensive care units are a result of withdrawal or limitation of life-sustaining treatment, end-of-life decision-making has become an integral and difficult part of pediatric practice. Limitation or withdrawal of life-sustaining treatment in a cognitively normal child with SMA poses a significant medical and ethical dilemma. This difficult decision is influenced by confluence of parental, doctor, social, cultural, moral, religious, legal, and economic factors and more recently the media.
Boillot, O; Dawahra, M; Porcheron, J; Houssin, D; Boucaud, C; Gille, D; Kopp, C; Bodnar, D; Sann, L; Paliard, P
With improved results of liver transplantation, the number of candidates is increasing. However the scarcity of suitable grafts from cadaveric donors remains a limitation. In spite of the use of full size or reduced size grafts or partial grafts from split livers, some children still die while waiting for liver transplantation. We describe a successful orthotopic liver transplantation in a 10 months old female using the left lateral lobe (segments II and III) from her 27 years old father. The child suffered from biliary atresia, her condition was deteriorating with intractable ascites and increasing jaundice. The father asked us to give a part of his own liver to his daughter. The concept of this innovative therapy had already been submitted to a research-ethics consultation which gave us favorable conclusions. After careful donor evaluation, the left lateral lobe was harvested on July 22, 1992, including the left hepatic artery, left portal vein and left hepatic vein; hepatic artery for segment IV, which arose from the right structures, was preserved, The graft was immediately transplanted orthotopically after recipient total hepatectomy with inferior vena cava preservation. Cold ischemia time was 1 hour and 45 minutes, revascularization of the graft was homogeneous from the very beginning and its early function was excellent. Thirteen days after the operation, the donor was discharged in good condition. The child was reoperated at day 9 for a small biliary leak originating from the cut surface of the liver. After resolution of an episode of rejection and an intra-abdominal abscess, the child was discharged in good health with normal liver function 1 month post-transplant.(ABSTRACT TRUNCATED AT 250 WORDS)
Caraballo, Roberto; Bartuluchi, Marcelo; Cersósimo, Ricardo; Soraru, Alejandra; Pomata, Hugo
In this study we report the clinical outcomes of hemispherectomy for epilepsy in pediatric patients with special emphasis on the epileptic syndromes and their etiologies. We retrospectively studied 45 patients with medically refractory epilepsy with hemispheric lesions who underwent hemispherectomy at the "Hospital de Pediatría Prof. Dr. Juan P. Garrahan", Buenos Aires, Argentina between February 1990 and February 2010. Patients had been assessed using a standard protocol involving clinical, neuroradiological, neurophysiological, and neuropsychological teams. Twenty-seven males and 18 females with a mean age of 8.5 years (range, 2 months to 18 years) who underwent epilepsy surgery for refractory epilepsy were assessed. The mean time of follow-up was 9.5 years (range, 1 to 16 years). The following epileptic syndromes were recognized: West syndrome in 15 patients (33.5%), Rasmussen syndrome in 13 (29%), focal symptomatic epilepsy in 8 (17.5%), startle epilepsy in 6 (13.5%), Lennox-Gastaut syndrome in 2 (5%), and continuous spikes and waves during slow sleep in 1 (2%). The surgical specimens revealed malformations of cortical development in 18 patients (40%), Rasmussen encephalitis in 13 (29%), porencephalic lesions in 10 (22%), gliosis in 2 (4.4%), tumor in 1 (2.2%), and Sturge-Weber syndrome in 1 (2.2%). The outcome of hemispherectomy in pediatric patients is good for those with refractory epilepsies, such as West syndrome, Lennox-Gastaut syndrome, epileptic encephalopathy with continuous spikes and waves during slow sleep, and startle epilepsy arising from a hemispheric lesion associated with hemiplegia.
Mohri, Ikuko; Kato-Nishimura, Kumi; Kagitani-Shimono, Kuriko; Kimura-Ohba, Shihoko; Ozono, Keiichi; Tachibana, Naoko; Taniike, Masako
We conducted a retrospective chart review of children with restless legs syndrome (RLS) to evaluate the efficacy of oral iron treatment, which was administered open-label during the course of clinical care. In addition, we provided detailed clinical information about RLS in this pediatric cohort. The study included 30 consecutive Japanese children with RLS who visited the Pediatric Sleep Clinic at Osaka University Hospital, and consisted of 17 boys and 13 girls, aged 2-14 years (mean ± SD, 6.5 ± 2.8). All-night polysomnography was performed in 18 patients and serum ferritin levels were measured in all the patients. After the diagnosis of RLS, iron was administered at doses between 1.6 and 7.8 mg/kg/day (3.2 ± 1.3). Serum ferritin was re-evaluated 3-6 months after iron treatment, or when RLS symptoms had disappeared. The patient age at onset of RLS symptoms ranged from six months to 13 years (4.3 ± 3.6). A positive family history was recognized in 19 children (63.3%). Serum ferritin levels before therapy were 9-62 ng/ml (26.6 ± 12.8) and oral iron supplementation was reported to be highly effective in 17 children, effective in 10, and ineffective in three. The serum ferritin level at follow-up was 23-182 ng/ml (83.5 ± 49.8). The onset of treatment effect was within approximately three months. Iron treatment could be effective in Japanese pediatric RLS. Copyright © 2012 Elsevier B.V. All rights reserved.
Greenbaum, Larry A; Fila, Marc; Ardissino, Gianluigi; Al-Akash, Samhar I; Evans, Jonathan; Henning, Paul; Lieberman, Kenneth V; Maringhini, Silvio; Pape, Lars; Rees, Lesley; van de Kar, Nicole C A J; Vande Walle, Johan; Ogawa, Masayo; Bedrosian, Camille L; Licht, Christoph
Atypical hemolytic uremic syndrome (aHUS) is caused by alternative complement pathway dysregulation, leading to systemic thrombotic microangiopathy (TMA) and severe end-organ damage. Based on 2 prospective studies in mostly adults and retrospective data in children, eculizumab, a terminal complement inhibitor, is approved for aHUS treatment. Here we prospectively evaluated efficacy and safety of weight-based dosing of eculizumab in eligible pediatric patients with aHUS in an open-label phase II study. The primary end point was complete TMA response by 26 weeks. Twenty-two patients (aged 5 months-17 years) were treated; 16 were newly diagnosed, 12 had no prior plasma exchange/infusion during current TMA symptomatology, 11 received baseline dialysis and 2 had prior renal transplants. By week 26, 14 achieved a complete TMA response, 18 achieved hematologic normalization, and 16 had 25% or better improvement in serum creatinine. Plasma exchange/infusion was discontinued in all, and 9 of the 11 patients who required dialysis at baseline discontinued, whereas none initiated new dialysis. Eculizumab was well tolerated; no deaths or meningococcal infections occurred. Bone marrow failure, wrist fracture, and acute respiratory failure were reported as unrelated severe adverse events. Thus, our findings establish the efficacy and safety of eculizumab for pediatric patients with aHUS and are consistent with proposed immediate eculizumab initiation following diagnosis in children. Copyright © 2016 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.
Muhammet Ali Varkal
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.
Moore, Cynthia A.; Staples, J. Erin; Dobyns, William B.; Pessoa, André; Ventura, Camila V.; da Fonseca, Eduardo Borges; Ribeiro, Erlane Marques; Ventura, Liana O.; Neto, Norberto Nogueira; Arena, J. Fernando; Rasmussen, Sonja A.
Importance Zika virus infection can be passed prenatally from a pregnant woman to her fetus. There is sufficient evidence to conclude that intrauterine Zika virus infection is a cause of microcephaly and serious brain anomalies, but the full spectrum of anomalies has not been delineated. To inform pediatric healthcare providers who may be called upon to evaluate and manage affected infants and children, we review the most recent evidence to better characterize congenital Zika syndrome. Observations We reviewed published reports of congenital anomalies occurring in fetuses or infants with presumed or laboratory-confirmed intrauterine Zika virus infection. Congenital anomalies were considered in the context of the presumed pathogenetic mechanism related to the neurotropic properties of the virus. We conclude that congenital Zika syndrome is a recognizable pattern of structural anomalies and functional disabilities secondary to central and perhaps peripheral nervous system damage. Although many of the components of this syndrome such as cognitive, sensory and motor disabilities are shared by other congenital infections, there are five features that are rarely seen with other congenital infections or are unique to congenital Zika virus infection: severe microcephaly with partially collapsed skull; thin cerebral cortices with subcortical calcifications; macular scarring and focal pigmentary retinal mottling; congenital contractures; and marked early hypertonia and symptoms of extrapyramidal involvement. Conclusions and Relevance Although the full spectrum of adverse reproductive outcomes caused by Zika virus infection is not yet determined, a distinctive phenotype, the congenital Zika syndrome, has emerged. Recognition of this phenotype by healthcare providers for infants and children can help ensure appropriate etiologic evaluation as well as comprehensive clinical investigation to define the range of anomalies in an affected infant and determine essential follow
Panico, Flávia F; Troster, Eduardo J; Oliveira, Cindy S; Faria, Aline; Lucena, Michelle; João, Paulo R D; Saad, Everardo D; Foronda, Flávia A K; Delgado, Artur F; de Carvalho, Werther Brunow
Children admitted to PICUs often present with or develop respiratory failure that requires mechanical ventilation. We prospectively identified children admitted to three general PICUs, with the goal of identifying risk factors for mortality. Prospective multicenter observational study. Three general PICUs, two in São Paulo and one in Curitiba, Brazil. Children aged between 1 month and 15 years, consecutively admitted between August 2008 and July 2010, with acute lung injury or acute respiratory distress syndrome that developed at least 12 hours after invasive or noninvasive mechanical ventilation. None. We used logistic regression models to explore the relationship between death and independent variables. Of 3,046 patients admitted to the three PICUs, 1,658 patients underwent mechanical ventilation, and 84 fulfilled the acute lung injury/acute respiratory distress syndrome inclusion criteria and were analyzed. Nearly 60% were boys, and the median age was 31 months. Pressure control/assist control was the initial mode of mechanical ventilation in 86% of cases, and the median durations of mechanical ventilation and PICU stay were 12 and 15 days, respectively. None of the eight patients with acute lung injury died, whereas 33 of 76 of the remaining patients with acute respiratory distress syndrome died, for an overall mortality rate of 39.3% (95% CI, 28.8-50.6%). In different multivariate logistic regression model, the number of organ dysfunctions at admission, peak inspiratory pressure, airway pressure gradient on day 1, and the mean airway pressure gradient over the first 7 days of mechanical ventilation were significantly associated with mortality. Mortality is high in pediatric acute lung injury/acute respiratory distress syndrome. Mechanical ventilation-associated risk factors for death among such patients are potential targets for intervention.
Mittal, Vijay A; Dean, Derek J; Mittal, Jyoti; Saks, Elyn R
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.
Agarwal, Amit; Kumar, Pradeep; Gupta, Nomeeta
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.
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.
Naito, Makiko; Aoki, Sayaka; Kamide, Anri; Miyamura, Kohei; Honda, Manami; Nagai, Akira; Mezawa, Hidetoshi; Hashimoto, Keiji
Children with Down syndrome (DS), who are likely to suffer from a large number of musculoskeletal problems, tend to have a unique pattern of walking in clinical settings. Despite such apparent uniqueness, few studies have empirically investigated gait development pattern in DS children, especially at an earlier age. We therefore conducted gait analysis in young DS children who are prescribed insoles, to explore how their gait patterns develop, using the gait parameters identified by Sutherland et al. as determinants of gait maturity of typical children. Participants consisted of 63 DS children (31 boys) aged 1-6 years (mean, 4 years 1 month) with a diagnosis of flat feet who were prescribed orthotic insoles. A 2.4 m sheet-type gait analyzer was used to analyze gait pattern. We measured the following variables: walking velocity (cm/min), cadence (steps/min), step length (cm), and single-limb stance phase ratio (%), and examined their relationship with age on regression analysis. Walking velocity and step length were significantly and positively related to age. Cadence was also significantly, but negatively associated with age. In contrast, SLS phase ratio did not have a statistically significant relationship with age. Down syndrome children have unique gait development patterns. Although walking velocity, cadence, and step length were found to develop with age, as in typical children, SLS phase ratio did not change with age in DS children. Further studies with a larger sample are necessary to replicate these findings. © 2015 Japan Pediatric Society.
Ripperger, Tim; Bielack, Stefan S; Borkhardt, Arndt; Brecht, Ines B; Burkhardt, Birgit; Calaminus, Gabriele; Debatin, Klaus-Michael; Deubzer, Hedwig; Dirksen, Uta; Eckert, Cornelia; Eggert, Angelika; Erlacher, Miriam; Fleischhack, Gudrun; Frühwald, Michael C; Gnekow, Astrid; Goehring, Gudrun; Graf, Norbert; Hanenberg, Helmut; Hauer, Julia; Hero, Barbara; Hettmer, Simone; von Hoff, Katja; Horstmann, Martin; Hoyer, Juliane; Illig, Thomas; Kaatsch, Peter; Kappler, Roland; Kerl, Kornelius; Klingebiel, Thomas; Kontny, Udo; Kordes, Uwe; Körholz, Dieter; Koscielniak, Ewa; Kramm, Christof M; Kuhlen, Michaela; Kulozik, Andreas E; Lamottke, Britta; Leuschner, Ivo; Lohmann, Dietmar R; Meinhardt, Andrea; Metzler, Markus; Meyer, Lüder H; Moser, Olga; Nathrath, Michaela; Niemeyer, Charlotte M; Nustede, Rainer; Pajtler, Kristian W; Paret, Claudia; Rasche, Mareike; Reinhardt, Dirk; Rieß, Olaf; Russo, Alexandra; Rutkowski, Stefan; Schlegelberger, Brigitte; Schneider, Dominik; Schneppenheim, Reinhard; Schrappe, Martin; Schroeder, Christopher; von Schweinitz, Dietrich; Simon, Thorsten; Sparber-Sauer, Monika; Spix, Claudia; Stanulla, Martin; Steinemann, Doris; Strahm, Brigitte; Temming, Petra; Thomay, Kathrin; von Bueren, Andre O; Vorwerk, Peter; Witt, Olaf; Wlodarski, Marcin; Wössmann, Willy; Zenker, Martin; Zimmermann, Stefanie; Pfister, Stefan M; Kratz, Christian P
Heritable predisposition is an important cause of cancer in children and adolescents. Although a large number of cancer predisposition genes and their associated syndromes and malignancies have already been described, it appears likely that there are more pediatric cancer patients in whom heritable cancer predisposition syndromes have yet to be recognized. In a consensus meeting in the beginning of 2016, we convened experts in Human Genetics and Pediatric Hematology/Oncology to review the available data, to categorize the large amount of information, and to develop recommendations regarding when a cancer predisposition syndrome should be suspected in a young oncology patient. This review summarizes the current knowledge of cancer predisposition syndromes in pediatric oncology and provides essential information on clinical situations in which a childhood cancer predisposition syndrome should be suspected. © 2017 Wiley Periodicals, Inc.
Thapa, Rajoo; Biswas, Biawajit; Mallick, Debkrishna; Mukherjee, Swapan
Pharyngeal-cervical-brachial weakness is considered a variant of Guillain-Barré syndrome with limited oropharyngeal, neck, and upper limb muscle involvement. The authors report on a 7-year-old boy, who developed pharyngeal-cervical-brachial type of Guillain-Barré syndrome following an antecedent episode of acute hepatitis A virus infection, 2 weeks prior to admission. The presentation was characterized by acute onset dysphagia, loss of head control, and bilateral arm weakness. The diagnosis was confirmed by acute motor axonal changes in the arm and albuminocytologic dissociation of the cerebrospinal fluid. The child was treated with intravenous immunoglobulin, which resulted in gradual improvement over 3 weeks. Documented instances of this form of Guillain-Barré syndrome remain rare in the pediatric age group, with none existing following antecedent hepatitis A virus infection. The authors emphasize that acute hepatitis A virus infection be included in the triggers responsible for Guillain-Barré syndrome in children.
Ali-Panzarella, Andrea Z; Bryant, Tamika J; Marcovitch, Hannah; Lewis, Jeffery D
We highlight the need for a multidisciplinary approach to the diagnosis of medical child abuse, also known as factitious disorder imposed on another (FDIA) or Munchausen syndrome by proxy (MSP), and review our experience focusing on the variety of symptoms that often present to the pediatric gastroenterologist many months before the diagnosis is made. Recent literature on medical child abuse, mostly case reports, is markedly limited, highlighting a need for increased research on this topic. Articles agree on the value of a multidisciplinary approach to these cases and the importance of involving professionals outside the hospital setting. Given the technology-dependent nature of our current society, the use of social media to aid in making the diagnosis has emerged. Review of the literature shows that there are almost no data on long-term outcomes of the victims or perpetrators of MSP. Making the diagnosis of MSP involves a complicated process of piecing together inconsistencies among the history, examination, and clinical presentation. The diagnosis remains difficult and is not often considered during early presentation of symptoms. Once MSP is suspected, it is important that a multidisciplinary process is used, incorporating input from various sources: the outpatient care structure, the hospital, non-hospital agencies such as school and child protective services, and non-traditional sources such as social media. In our experience, a multidisciplinary approach augmented by thoughtful inpatient surveillance provides the greatest opportunity for confirming or excluding MSP. Pediatric gastroenterology is one of the most common services consulted prior to diagnosis and presents an opportunity for early intervention.
Hesselmark, Eva; Bejerot, Susanne
Pediatric Acute Neuropsychiatric Syndrome (PANS) and pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) are conditions marked by sudden onset of obsessive-compulsive disorder (OCD), tics, or avoidant/restrictive food intake in combination with multiple psychiatric symptoms. A diagnosis of PANS or PANDAS may be supported by the Cunningham Panel, a commercially available set of immunologic assays currently in clinical use. However, the relationship between Cunningham Panel results and patient symptoms remains unclear. This study was done to assess the diagnostic accuracy of the Cunningham Panel in patients with suspected PANS or PANDAS. All Swedish patients who had taken the Cunningham Panel prior to June 2014 (n=154) were invited and 53 patients participated in the study. Based on comprehensive psychiatric assessment (the reference standard of diagnosis), subjects were classified as PANS, PANDAS, or neither. Prior Cunningham Panel test results were collected from patient records, and new blood samples were similarly analyzed within the scope of this study. In addition, results were compared to healthy controls (n=21) and a test-retest reliability analysis was performed. Sensitivities of individual biomarkers in the Cunningham Panel ranged from 15 to 60%, and specificities from 28 to 92%. Positive predictive values ranged from 17 to 40%, and negative predictive values from 44 to 74%. A majority of the healthy controls had pathological Cunningham Panel results and test-retest reliability proved insufficient. Clinical use of the Cunningham Panel in diagnosing PANS or PANDAS is not supported by this study. Copyright © 2017 The Authors. Published by Elsevier B.V. All rights reserved.
Al-Agha, A; Ocheltree, A; Shata, N
Obesity and overweight among children and adolescents is increasing at an alarming rate, which lead to the increase in the incidence of their related co-morbidities. Our objectives are to establish the following: 1) the prevalence of hyperinsulinism among overweight and obese pediatric patients. 2) The prevalence of type 2 diabetes mellitus (T2DM) among those with hyperinsulinism. 3) The prevalence of metabolic syndrome (MS) and its components among T2DM pediatric patients. A retrospective cross-sectional study conducted on overweight and obese pediatric patients attending the pediatrics diabetes clinic at King Abdul-Aziz University Hospital, Jeddah, Saudi Arabia, from 2006 to 2010. Serum insulin level was measured for 387 patients (ages from 2 to 18 years). Those with hyperinsulinism underwent further investigations to assess the prevalence of T2DM and the prevalence of MS among T2DM patients. The overall prevalence of hyperinsulinism and T2DM were 44.7%, and 9.04%, respectively. Among children and adolescents with T2DM, 62.86% had a body mass index BMI≥85th percentile, 37.14% had a BMI ≥ 95th percentile, 14.29% had MS, 34.29% were hypertensive and 28.57% had dyslipidemia. Obesity and its co-morbidities were prevalent among Saudi pediatric patients. We recommend preventing excessive weight gain through the promotion of a healthy lifestyle, family educational seminars and the reinforcement of indoor exercises.
Seidel, Natan E; Arlen, Angela M; Smith, Edwin A; Kirsch, Andrew J
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.
Thabet, F; Durand, P; Chevret, L; Fabre, M; Debray, D; Brivet, M; Devictor, D
Idiopathic Reye syndrome is a rare disease revealed by unexplained encephalopathy and microvesicular liver steatosis. Some clinical and epidemiological studies mainly performed in English speaking countries questioned the reality of Reye syndrome because numerous know inherited metabolic diseases, and some of them unrecognized, could mimick this disorder. We focused in our study on severe forms of Reye syndrome admitted to a pediatric intensive care unit. Retrospective study over the last eleven years (1991-2001) included all the pediatric patients admitted to our tertiary referral center with the classical American Reye syndrome criteria (e.g. CDC). Extensive metabolic screening was performed in all cases, except for the ultimately dead patients. Fourteen patients (mean age 52 months) were included. Fever always occurred before their admission and aspirin (n = 12) or acetaminophen (n = 7) was prescribed. Median Glasgow scale was 7 on admission. Mean amoniac plasma level was 320 mumol/L and alanine-aminotransferase peak plasma level 1475 +/- 1387 IU/L. Mechanical ventilation was started in ten children and six of them underwent continuous venovenous hemofiltration. Three patients ultimately died and 11 survived with a mean five years follow-up without relapses or neurological impairment. Any of them demonstrated inherited metabolic disease except for one infant with hereditary fructose intolerance. Unlike widespread opinion, severe Reye syndrome without identified metabolic disorders seems to not disappear in our country. Reye syndrome remains a potentially life threatening disease and raises for aggressive treatment of brain edema. If aspirin and Reye syndrome association are not formally documented in France, cautiousness must be kept in mind and all the aspirin adverse effects notifications should be addressed to the public drugs survey network.
Kianifar, Hamidreza; Jafari, Seyed Ali; Kiani, Mohammadali; Ahanchian, Hamid; Ghasemi, Seyed Vahid; Grover, Zubin; Mahmoodi, Leili Zarif; Bagherian, Rita; Khalesi, Maryam
Irritable bowel syndrome (IBS) is a common gastrointestinal disorder in children. Recently, probiotics have been suggested as a treatment option for gastrointestinal disorders. The most effective species and the most appropriate doses are still unknown. The aim of this study was to assess the effects of Lactobacillus GG (LGG) for treating IBS in pediatric patients. In a controlled, double blind, randomized trial, patients with IBS diagnosed by Rome III criteria from August 2012 to September 2012 at Dr. Sheikh Hospital, Mashhad University of Medical Sciences, Iran, were assigned to one of two groups, i.e., intervention and control groups. For four weeks, the intervention group received a probiotic in capsule form that contained LGG at a concentration of 1×10(10) cfu/ml bacteria. For the same period, the control group received a placebo capsule that had the same shape and color but only contained inulin, which also was present in the LGG capsules. The primary outcome was any change in the severity of the patients' pain, and we used a five-point Likert scale to evaluate the severity of their pain. Secondary outcomes were ghanges of the functional scale, stool patterns, and associated problems. Fifty-two patients participated in the study, and 26 patients were assigned randomly to each of the two groups. The severity of the patients' pain decreased significantly in the intervention group after one, two, three, and four weeks of treatment, as indicated by P-values of 0.01, 0.00, 0.00, and 0.00, respectively. Also, there was significant improvement in the functional scale after two weeks of treatment (P-value ≤ 0.00). Lactobacillus GG at a concentration of 1×10(10) cfu/ml for a period of four weeks can lessen the severity of the patients' pain and improve the functional scale in patients with irritable bowel syndrome. Probiotics can have therapeutic effects for IBS patients.
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.
Saadiya Javed Khan
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.
Fresneau, Brice; Brugières, Laurence; Caron, Olivier; Moutel, Grégoire
In 2001, a French expert panel recommended that presymptomatic tests should not be carried out on minors in families affected by Li-Fraumeni syndrome (LFS), flying in the face of possible parental demands for such testing. We decided to investigate the legitimacy of such a recommendation. We conducted a national multicenter survey using self-administered questionnaires mailed to French oncogeneticists in 33 regional centers in France. We aimed to (1) determine the extent to which these doctors were confronted with parental requests for TP53 testing, (2) study how they responded to these requests and the arguments used and (3) assess the attitude of oncogeneticists concerning the normative framework regulating the prescription of tests for minors. Twenty oncogeneticists stated that they had managed at least one LFS family. Eleven of these doctors had been confronted with parental requests for testing and three had prescribed such tests on at least one occasion. The oncogeneticists gave balanced medical, psychological and ethical arguments, highlighting the dilemma they face in the decision-making process. This dilemma is due to the lack of a consensus concerning this recommendation, which aims to protect the minor by limiting presymptomatic tests to cases in which a clear medical benefit can be demonstrated but which prevents the unique situation of particular families from being taken into account. In conclusion, the recommendation has a normative status but first, from a clinical stance, it is difficult to dissociate it from the evaluation of individual family situations, and second, the benefit of a specific medical follow-up for TP53 mutation carriers is currently being investigated.
de Melo-Martín, Inmaculada; Sondhi, Dolan; Crystal, Ronald G
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.
Marraro, Giuseppe A; Chen, Chengshui; Piga, Maria Antonella; Qian, Yan; Spada, Claudio; Genovese, Umberto
Acute respiratory distress syndrome (ARDS) is a heterogeneous syndrome that lacks definitive treatment. The cornerstone of management is sound intensive care treatment and early anticipatory ventilation support. A mechanical ventilation strategy aiming at optimal alveolar recruitment, judicious use of positive end-respiratory pressure (PEEP) and low tidal volumes (VT) remains the mainstay for managing this lung disease. Several treatments have been proposed in rescue settings, but confirmation is needed from large controlled clinical trials before they be recommended for routine care. Non-invasive ventilation (NIV) is suggested with a cautious approach and a strict selection of candidates for treatment. Mild and moderate cases can be efficiently treated by NIV, but this is contra-indicated with severe ARDS. The extra-corporeal carbon dioxide removal (ECCO2 R), used as an integrated tool with conventional ventilation, is playing a new role in adjusting respiratory acidosis and CO2. The proposed benefits of ECCO2 R over extra-corporeal membrane oxygenation (ECMO) consist in a reduction of artificial surface contact, avoidance of pump-related side effects and technical complications, as well as lower costs. The advantages and disadvantages of inhaled nitric oxide (iNO) are better recognized today and iNO is not recommended for ARDS and acute lung injury (ALI) in children and adults because iNO results in a transient improvement in oxygenation but does not reduce mortality, and may be harmful. Several trials have found no clinical benefit from various surfactant supplementation methods in adult patients with ARDS. However, studies which are still controversial have shown that surfactant supplementation can improve oxygenation and decrease mortality in pediatric and adolescent patients in specific conditions and, when applied in different modes and doses, also in neonatal respiratory distress syndrome (RDS) of preemies. Management of ARDS remains supportive, aimed at
Gonçalves, Daniel; Meireles, Joana; Rocha, Ruben; Sampaio, Mafalda; Leão, Miguel
The syndrome of transient headache and neurologic deficits associated with cerebrospinal fluid lymphocytosis (HaNDL) is characterized by 1 or more episodes of severe headache, transient neurologic deficits, and lymphocytic pleocytosis in the cerebrospinal fluid. It is a benign and self limited disorder seldom reported in pediatric age. We report the case of a 14-year-old girl who suffered from 2 episodes of headache with transient focal neurologic deficits and pleocytosis consistent with the syndrome of HaNDL. This entity should be taken into account as a differential diagnosis in otherwise healthy children presenting with recurrent headache and acute neurologic deficits. Repeated use of invasive and expensive laboratory and imaging investigations can be avoided when the diagnosis of the syndrome of HaNDL is correctly established.
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
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.
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
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.
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
Venditto, Chelsea; Jager, Zachary; LoGiudice, John; Matloub, Hani
Compartment syndrome of the upper extremity is a surgical emergency that, when left untreated, can have dire consequences. Its causes are numerous, one of which is the uncommon entity hereditary angioedema, an autosomal dominant disease resulting in edema in a variety of potential locations, including the extremities. This is only the second time hereditary angioedema has been mentioned in the literature as a cause of compartment syndrome. We present a case of hereditary angioedema leading to hand and forearm compartment syndrome in a 13-year-old pediatric patient. Diagnosis of hereditary angioedema was made by our Rheumatology colleagues with physical exam and a thorough history, and confirmed by laboratory studies. Our patient presented with compartment syndrome of the hand and forearm and underwent hand and volar forearm fasciotomies. She was subsequently worked up for hereditary angioedema with laboratory results confirming the diagnosis. She was discharged after a 5-day hospitalization with prophylactic C1-inhibitor therapy. Hereditary angioedema is a rare but known cause of compartment syndrome of the upper extremity, and must be considered when patients present with compartment syndrome of unknown etiology. This disease can be diagnosed by laboratory studies and symptoms can be controlled with medical therapy.
Means, Casey; Aldape, Mark A; King, Ericka
Primary Sjögren syndrome is uncommon in children, and the standard clinical criteria used in diagnosis of adult Sjögren syndrome will miss many children with the disease. Floor of mouth ranulas have not been described in Sjögren syndrome. This study aims to describe a novel presentation of juvenile primary Sjögren syndrome, and to present a comprehensive systematic review of the literature regarding the presentation and diagnosis of Sjögren syndrome in children. Ovid MEDLINE. A MEDLINE literature search was performed using the following search terms: primary, Sjögren, disease, and children. Results were limited to human subjects and articles written in English between 1981 and 2014. Applicable articles were reviewed and qualitatively summarized. Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines (PRIMA). Initial MEDLINE search yielded 146 articles, 80 of which were excluded as not clinically pertaining to Sjögren syndrome. An additional 25 were excluded due to lack of pediatric-specific data. Systematic review of the literature revealed no reports of ranula in association with Sjögren syndrome. 6 papers were manually included from review of reference lists of included articles. Our review indicated that recurrent parotitis is the most commonly reported presenting symptom in children, followed by ocular and oral symptoms, musculoskeletal, and renal symptoms. Compared to adults, children are less likely to present with dry eyes and mouth. All studies were retrospective chart reviews, case series or case reports. This is the first report of a child presenting with floor of mouth ranulas in association with Sjögren syndrome. While recurrent parotitis is the most common presentation in children, other salivary gland and extra-salivary manifestations may be seen, and the clinician must maintain a high index of suspicion for underlying Sjögren syndrome. Copyright © 2017 Elsevier B.V. All rights reserved.
Ma, J R; Song, H M; Xiao, J; Tang, X Y; He, Y Y; Wei, M
Objective: To identify the clinical and immunological characteristics of pediatric antiphospholipid syndrome (APS) patients with pulmonary embolism. Method: Among 47 pediatric APS patients from Peking Union Medical College Hospital during the year of 2000 to 2015, 12 patients were diagnosed of pulmonary embolism, who were investigated and compared with APS patients without pulmonary embolism. Result: Twelve patients (among whom 6 cases were primary and the other 6 were secondary APS)had pulmonary embolism and all of them were non-shock type, which was the first presenting manifestation in 6 of them.Eight cases were misdiagnosed as infection, while 3 cases were missed.Among patients with pulmonary embolism, 10 patients suffered from deep vein thrombosis at the same time, mainly in lower extremities.2 cases had thrombotic recurrence, which happened only in primary APS patients, because of irregular monitoring of International Normalized Ratio, or not taking aspirin after quitting warfarin.Positive anticardiolipin (ACL) and lupus anticoagulant (LA) were found in 10 and 9 patients respectively.Four primary APS patients had positive anti-nuclear antibodies (ANA). During follow-up of 3-100 months (median 23 months) of primary APS, no one had evolved manifestations of systemic lupus erythematosus.Primary APS was more often seen in males (M∶F 5∶1 vs . 0∶6) and the patients were much younger ((15±1) vs . (17±0) years old) than those with secondary APS.Besides that, no statistically significant difference was seen between primary and secondary APS ( P all>0.05). Compared with APS patients without pulmonary embolism, pulmonary hypertension was more common in patients suffered from pulmonary embolism (3/12 vs . 0, P irregular.Positive anti-nuclear antibodies can be seen in primary APS patients, but no manifestations of lupus come out during follow-up.There is no significant difference between primary APS and secondary APS.Pulmonary hypertension is more common in APS
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.
Bagwell, Charles E; Chiu, Priscilla; Fecteau, Annie; Gow, Kenneth W; Mueller, Claudia M; Price, David; Zigman, Andrew F
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.
Jessica A Church
Full Text Available Tourette Syndrome (TS is a pediatric movement disorder that may affect control signaling in the brain. Previous work has proposed a dual-networks architecture of control processing involving a task-maintenance network and an adaptive control network (Dosenbach et al., 2008. A prior resting-state functional connectivity MRI (rs-fcMRI analysis in TS has revealed functional immaturity in both putative control networks, with “anomalous” correlations (i.e. correlations outside the typical developmental range limited to the adaptive control network (Church et al., 2009. The present study used functional MRI (fMRI to study brain activity related to adaptive control (by studying start-cues signals, and to task-maintenance (by studying signals sustained across a task set. Two hypotheses from the previous rs-fcMRI results were tested. First, adaptive control (i.e., start-cue activity will be altered in TS, including activity inconsistent with typical development (“anomalous”. Second, group differences found in task maintenance (i.e., sustained activity will be consistent with functional immaturity in TS. We examined regions found through a direct comparison of adolescents with and without TS, as well as regions derived from a previous investigation that showed differences between unaffected children and adults. The TS group showed decreased start-cue signal magnitude in regions where start-cue activity is unchanged over typical development, consistent with anomalous adaptive control. The TS group also had higher magnitude sustained signals in frontal cortex regions that overlapped with regions showing differences over typical development, consistent with immature task maintenance in TS. The results demonstrate task-related fMRI signal differences anticipated by the atypical functional connectivity found previously in adolescents with TS, strengthening the evidence for functional immaturity and anomalous signaling in control networks in adolescents
Genoni, Giulia; Menegon, Veronica; Secco, Gioel Gabrio; Sonzini, Michela; Martelli, Massimiliano; Castagno, Matteo; Ricotti, Roberta; Monzani, Alice; Aronici, Michele; Grossini, Elena; Di Mario, Carlo; Bona, Gianni; Bellone, Simonetta; Prodam, Flavia
Childhood obesity is associated with cardiovascular abnormalities but little is known on the potential correlation between early cardiovascular and metabolic alterations. Aims of this study were 1) to evaluate early cardiovascular abnormalities in a large population of obese children and adolescents compared with a normal weight counterpart, 2) to investigate their potential association with insulin resistance (IR), serum uric acid (sUA) and metabolic syndrome (MetS). This was a single-center case-control study. Eighty obese (OB) subjects (6-16years) and 20 normal weight (NW) matched controls were consecutively recruited. In the whole population we performed an anthropometric and a cardiovascular assessment. OB patients also underwent an OGTT and biochemical evaluations. OB children showed greater left atrial (LA) and ventricular (LV) dimensions and mass and higher carotid artery intima-media thickness (CIMT), compared with NW controls. The BMI z-score, waist circumference, IR and sUA were positively related with LA and LV dimensions and mass. OB subjects with MetS (46.3%) showed greater LA diameter (p=0.001) and LV area (p=0.01) and volume (p=0.04) compared with OB children without MetS. LA diameter and LV dimensions and mass were significantly dependent on the number of criteria for MetS. Mets, sUA and IR were significant predictors of left heart dimensions and mass in obese children. Obesity and MetS are associated with abnormal cardiovascular response during childhood. Hyperuricemia can be an early marker of cardiovascular dysfunction and the routine determination of circulating levels of sUA should be implemented during risk stratification among pediatric age. Copyright © 2017 Elsevier B.V. All rights reserved.
Pranzatelli, Michael R; Tate, Elizabeth D
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
Vázquez-Frias, R; Gutiérrez-Reyes, G; Urbán-Reyes, M; Velázquez-Guadarrama, N; Fortoul-van der Goes, T I; Reyes-López, A; Consuelo-Sánchez, A
There is evidence that patients with irritable bowel syndrome (IBS) have a low degree of inflammation in the intestinal mucosa. The aim of the study was to evaluate the profile of pro- and anti-inflammatory cytokines in plasma in Mexican pediatric patients with IBS. Fifteen patients with IBS according to Rome III criteria for childhood and 15 healthy children, matched by age and sex, were included in the study. Plasma levels of tumoral necrosis factor alpha (TNF-α), interleukins 10 and 12 (IL-10, IL-12) and transforming growth factor beta (TGF-β) were quantified and compared between groups. Plasma levels of IL-10 were lower in patients with IBS (86.07+21.3 pg/mL vs. 118.71+58.62 pg/mL: P=.045) and IL-12 levels were higher in patients with IBS compared to the control group of healthy children (1,204.2±585.9 pg/mL vs. 655.04±557.80 pg/mL; P=.011). The IL-10/IL-12 index was lower in patients with IBS (0.097±0.07 vs. 0.295±0.336; P=.025). Plasma concentration of TGF-β was higher in patients with IBS (545.67±337.69 pg/mL vs. 208.48±142.21 pg/mL; P=.001). There was no difference in plasma levels of TNF-α between groups. This study suggests that children with IBS have a state of altered immune regulation. This is consistent with the theory of low-grade inflammatory state in these patients. Further studies are needed to elucidate the role played by these cytokines, specifically TGF-β in the pathogenesis of IBS. Copyright © 2014 Asociación Mexicana de Gastroenterología. Published by Masson Doyma México S.A. All rights reserved.
Boesen, Magnus Spangsberg; Magyari, Melinda; Koch-Henriksen, Nils
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...... European countries. Referral bias and classification differences may account for this disparity, in particular the age-intervals and the definition of onset....
Scott, Halden F; Donoghue, Aaron J; Gaieski, David F; Marchese, Ronald F; Mistry, Rakesh D
Early detection of compensated pediatric septic shock requires diagnostic tests that are sensitive and specific. Four physical exam signs are recommended for detecting pediatric septic shock prior to hypotension (cold extremities, mental status, capillary refill, peripheral pulse quality); this study tested their ability to detect patients who develop organ dysfunction among a cohort of undifferentiated pediatric systemic inflammatory response syndrome patients. A prospective cohort of 239 pediatric emergency department patients physical exams on a standardized form. Abstraction of the medical record determined outcomes including organ dysfunction, intensive care unit stay, serious bacterial infection, and therapies. Organ dysfunction occurred in 13/239 (5.4%) patients. Presence of at least one sign was significantly associated with organ dysfunction (Relative Risk: 2.71, 95% CI: 1.05-6.99), and presence of at least two signs had a Relative Risk = 4.98 (95% CI: 1.82-13.58). The sensitivity of exam findings ranged from 8-54%, specificity from 84-98%. Signs were associated with increased risk of intensive care and fluid bolus, but not with serious bacterial infection, intravenous antibiotics or admission. Altered mental status and peripheral pulse quality were significantly associated with organ dysfunction, while abnormal capillary refill time and presence of cold, mottled extremities were not. Certain recommended physical exam signs were associated with increased risk of organ dysfunction, a rare outcome in this undifferentiated pediatric population with fever and tachycardia. Sensitivity was low, while specificity was high. Additional research into optimally sensitive and specific diagnostic strategies is needed.
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...
Kam, Madibèlè; Douamba, Sonia; Nagalo, Kisito; Dao, Lassina; Kouéta, Fla; Lougué, Claudine; Yé, Diarra
Pepper's syndrome is a neuroblastoma that metastasizes to the liver. It affects infants younger than six months of age. It can regress spontaneously and is associated with a favorable prognosis in 80% of cases. Given its rarity, we here report two cases of Pepper's syndrome observed at the Charles de Gaulle university pediatric hospital center, Ouagadougou (Burkina Faso). Our study involved two female infants in whom the disease manifested as an increase in abdominal volume, hepatomegaly and signs of respiratory distress. Ultrasound enabled diagnosis, which was based on the nodular appearance of the liver in both cases and determination of the primary tumor in one case. Urinary catecholamine test confirmed the diagnosis in one case. Both patients died from complications related to liver compression, chemotherapy (in one of the cases) and lack of treatment (in the other case).
Riyaz Ahmad Bhat
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.
Felipe Alves Mourato
Full Text Available OBJECTIVE:To determine the frequence and profile of congenital heart defects in Down syndrome patients referred to a pediatric cardiologic center, considering the age of referral, gender, type of heart disease diagnosed by transthoracic echocardiography and its association with pulmonary hypertension at the initial diagnosis.METHODS:Cross-sectional study with retrospective data collection of 138 patients with Down syndrome from a total of 17,873 records. Descriptive analysis of the data was performed, using Epi-Info version 7.RESULTS: Among the 138 patients with Down syndrome, females prevailed (56.1% and 112 (81.2% were diagnosed with congenital heart disease. The most common lesion was ostium secundum atrial septal defect, present in 51.8%, followed by atrioventricular septal defect, in 46.4%. Ventricular septal defects were present in 27.7%, while tetralogy of Fallot represented 6.3% of the cases. Other cardiac malformations corresponded to 12.5%. Pulmonary hypertension was associated with 37.5% of the heart diseases. Only 35.5% of the patients were referred before six months of age.CONCLUSIONS: The low percentage of referral until six months of age highlights the need for a better tracking of patients with Down syndrome in the context of congenital heart disease, due to the high frequency and progression of pulmonary hypertension.
Gil-Gómez, Raquel; Blasco-Alonso, Javier; Sánchez-Yáñez, Pilar; Rosa-Camacho, Vanessa; Milano Manso, Guillermo
Neopterin and biopterin are sub-products of redox reactions, which act as cofactors of enzymes responsible for nitric oxide production. The hypothesis is presented that plasma neopterin and biopterin evolve differently during the first days in a critically ill child. A single-centre prospective observational study was conducted on patients 7 days to 14 years admitted to our Paediatric Intensive Care Unit (PICU) and that met Systemic inflammatory response syndrome (SIRS) criteria. Neopterin and biopterin levels, as well as other acute phase reactants, were collected at admission and at 24 h. A total of 28 patients were included, of which 78.9% were male, The median age was 5.04 years (interquartile range [IQR] 1.47-10.26), and PRISM II 2.0% (IQR 1.1-5.0). Mechanical ventilation (MV) was used in 90% of patients, with a median duration of 6.0 hrs (IQR 3.7-102.0). The median length of stay in PICU was 5.0 days (IQR 2.7-18.7), maximum VIS mean of 0 (IQR 0-14). Baseline neopterin level was 2.3±1.2 nmol/l and at 24 h it was 2.3±1.4 nmol/l. Baseline biopterin was 1.3±0.5 nmol/l and 1.4±0.4 nmol/l at 24 h. Neopterin levels were significantly higher in patients with PICU length of stay > 6 days (P=.02), patients who needed MV >24 h (P=.023), and those who developed complications (P=.05). Neopterin correlates directly and is statistically significant with the duration of MV (rho=.6, P=.011), PICU length of stay (rho=.75, P<.0001), and VIS (rho=.73, P=.001). Additionally, biopterin directly correlates with the PRISM (rho=.61, P=.008). There is a higher neopterin level when there is a longer PICU stay, higher VIS score, longer time on MV, and occurrence of complications, indicating the involvement of an activation of the cellular immune system. Copyright © 2016 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.
Hsu, Derek Y; Brieva, Joaquin; Silverberg, Nanette B; Paller, Amy S; Silverberg, Jonathan I
Little is known about the epidemiology of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in children. We sought to determine the morbidity, mortality, and comorbid health conditions of SJS and TEN in US children. This was a cross-sectional study of the 2009 to 2012 Nationwide Inpatient Sample, which contains a representative 20% sample of all US hospitalizations. Sociodemographics, inflation-adjusted cost, length of stay, comorbidities, and mortality were analyzed using descriptive statistics and multivariate regression analyses. The incidences of SJS, SJS-TEN, and TEN were a mean 5.3, 0.8, and 0.4 cases per million children per year in the US, respectively. Prolonged length of stay and higher costs of care (SJS: 9.4 ± 0.6 days, $24,947 ± $3171; SJS-TEN: 15.7 ± 1.5 days, $63,787 ± $8014; TEN: 20.4 ± 6.3 days, $102,243 ± $37,588) were observed compared with all other admissions (4.6 ± 0.1 days, $10,496 ± $424). Mortality was 0% for SJS, 4% for SJS-TEN, and 16% for TEN. In regression models, predictors of mortality included renal failure (adjusted OR [aOR] 300.28, 95% confidence interval [CI] 48.59->999.99), malignancy (aOR 54.33, 95% CI 9.40-314.22), septicemia (aOR 30.45, 95% CI 7.91-117.19), bacterial infection (aOR 20.38, 95% CI 5.44-76.36), and epilepsy (aOR 5.56, 95% CI 1.37-26.2). Data regarding treatment were not available. Date of diagnosis of comorbidities was not present, precluding temporal analysis. Pediatric SJS/TEN poses a substantial health burden in the United States. Copyright © 2016 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.
Full Text Available Wolfram syndrome (WFS is inherited as an autosomal recessive disease with main clinical features of diabetes mellitus, optic atrophy, diabetes insipidus and deafness. However, various neurological defects may also be detected. The aim of this study was to evaluate aspects of brain structure and function using PET-CT (positron emission tomography and computed tomography and MRI (magnetic resonance imaging in pediatric patients with WFS. Regional changes in brain glucose metabolism were measured using standardized uptake values (SUVs based on images of (18F fluorodeoxyglucose (FDG uptake in 7 WFS patients aged 10.1-16.0 years (mean 12.9±2.4 and in 20 healthy children aged 3-17.9 years (mean 12.8±4.1. In all patients the diagnosis of WFS was confirmed by DNA sequencing of the WFS1 gene. Hierarchical clustering showed remarkable similarities of glucose uptake patterns among WFS patients and their differences from the control group. SUV data were subsequently standardized for age groups 13 years old to account for developmental differences. Reduced SUVs in WFS patients as compared to the control group for the bilateral brain regions such as occipital lobe (-1.24±1.20 vs. -0.13±1.05; p = 0.028 and cerebellum (-1.11±0.69 vs. -0.204±1.00; p = 0.036 were observed and the same tendency for cingulate (-1.13±1.05 vs. -0.15±1.12; p = 0.056, temporal lobe (-1.10±0.98 vs. -0.15±1.10; p = 0.057, parietal lobe (-1.06±1.20 vs. -0.08±1.08; p = 0.058, central region (-1.01±1.04 vs. -0.09±1.06; p = 0.060, basal ganglia (-1.05±0.74 vs. -0.20±1.07; p = 0.066 and mesial temporal lobe (-1.06±0.82 vs. -0.26±1.08; p = 0.087 was also noticed. After adjusting for multiple hypothesis testing, the differences in glucose uptake were non-significant. For the first time, regional differences in brain glucose metabolism among patients with WFS were shown using PET-CT imaging.
Cebrián, J; Sánchez, P
To review the clinical, psychiatric, and social characteristics of complex regional pain syndrome in children and adolescents treated in the last 4 years at our pediatric pain clinic. We analyzed the specialty of the initial treating physician, age, sex, initial diagnosis, pain intensity, degree of disability, fear of movement, clinical stage, history of trauma, time between onset and diagnosis, psychiatric illness, family support and behavior, chronic pain in near relatives, school grades and attendance, treatment given at the pediatric pain clinic, recurrences, and course of disease. The cases of 7 patients (4 female, 3 male) between the ages of 8 and 15 years were analyzed. Four had been referred by the child psychiatry department. The initial diagnosis was erroneous in all but 1 case. Pain intensity and associated disability were severe in 5 patients and 4 expressed intense fear of moving the limb. Five patients had initial stage I disease, 5 had a history of trauma, and 5 had been previously treated by immobilization of the limb and prescription of nonsteroidal anti-inflammatory drugs. The time between onset and diagnosis ranged from 2 to 18 months (mean [SD], 6.4 [3.5] months). In most cases psychiatric disease and concomitant social disability were present. Treatment prescribed at the pediatric pain clinic consisted of a combination of oral medication, psychologic and psychiatric counseling, and intensive physiotherapy for all but 2 children, who required regional nerve blocks. The clinical course was satisfactory for all but 1 patient, who developed severe disability. Complex regional pain syndrome affecting an upper limb is uncommon in children but not rare.
Hoekman, Daniël R.; Rutten, Juliette M. T. M.; Vlieger, Arine M.; Benninga, Marc A.; Dijkgraaf, Marcel G. W.
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.
Cox, Joanna H; Seri, Stefano; Cavanna, Andrea E
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
... DEPARTMENT OF HEALTH AND HUMAN SERVICES Request for Comments on Ethical Issues Associated with the... ethical issues associated with the development of medical countermeasures for children, including ethical considerations surrounding clinical research with children, ethical considerations surrounding pediatric medical...
MUHAMMAD NUMAN ALI KHAN; DANISH JAMIL,
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.
Ricardo Erazo Torricelli
Full Text Available Este trabajo revisa el conocimiento actual sobre el síndrome de Guillain-Barré (SGB en niños. El SGB se define como una parálisis flácida arrefléxica aguda y se clasifica en 4 subgrupos: polirradiculopatía aguda inflamatoria desmielinizante (AIDP, neuropatía axonal sensitivo-motora aguda (AMSAN, neuropatía axonal motora aguda (AMAN y síndrome de Miller-Fisher (SMF. La AIDP se asocia en un 30-50% a compromiso de pares craneales, lo cual no se observa en la AMAN. El SMF se caracteriza por ataxia, oftalmoplejía y arreflexia, pero puede presentar también compromiso de pares craneales. Datos recientes de la anatomía patológica y la fisiopatología del SGB destacan la importancia de la infección por Campylobacter jejuni en la generación de anticuerpos anti-gangliósidos (GM1 en AIDP, GQ1b en SMF y GD1a en AMAN que lesionan la mielina en AIDP y SMF y el axón en AMAN. El diagnóstico diferencial debe descartar enfermedades del sistema nervioso central (SNC (encefalitis, encefalomielitis, mielitis, síndromes miasténicos, neuropatías tóxicas por metales pesados, fármacos, substancias químicas o toxinas animales y cuadros miopáticos, especialmente la miositis aguda infecciosa benigna y la neuromiopatía del paciente en la unidad de cuidados intensivos. Es importante el tratamiento con inmunoglobulina en dosis total de 2 gramos por kilogramo a administrar en 48 horas. La plasmaféresis puede ser igualmente eficaz. El SGB tiene buen pronóstico en niños, con una recuperación total en el 85% de los casos. La rehabilitación es fundamental para lograr una recuperación más rápida e integral.This paper reviews the current knowledge about Guillain- Barré syndrome (GBS. GBS is defined as an acute, areflexic, flaccid paralysis, which is classified into 4 subgroups: acute inflammatory demyelinating polyneuropathy (AIDP, acute motor-sensory axonal neuropathy (AMSAN, acute motor axonal neuropathy (AMAN and Miller-Fisher syndrome (MFS
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.
Ettinger, Nicholas; Pearson, Matthew; Lamb, Fred S; Wellons, John C
In this report, the authors describe the case of a teenage boy who presented with hypertensive emergency, posterior reversible encephalopathy syndrome, and hydrocephalus due to fourth ventricle outlet obstruction. Posterior reversible encephalopathy syndrome is a well-characterized but uncommon syndrome in children that is generally triggered by severe hypertension. The unusual clinical picture of this patient, who had isolated cerebellar edema leading to obstructive hydrocephalus, has been rarely described in children.
Full Text Available Background. Our goal was to compare the carotid intimal-medial thickness (CIMT of untreated pediatric patients with metabolic syndrome (MS, heterozygous familial hyperlipidemia (heFH, and MS+heFH against one another and against a control group consisting of healthy, normal body habitus children. Methods. Our population consisted of untreated pediatric patients (ages 5–20 yrs who had CIMT measured in a standardized manner. Results. Our population included 57 with MS, 23 with heFH, and 10 with MS+heFH. The control group consisted of 84 children of the same age range. Mean CIMT for the MS group was 469.8 μm (SD = 67, 443.8 μm (SD = 61 for the heFH group, 478.3 μm (SD = 70 for the MS+heFH group, and 423.2 μm (SD = 45 for the normal control group. Significance differences between groups occurred for heFH versus MS (P=0.022, heFH versus control (P=0.038, MS versus control (P=9.0E-10, and MS+heFH versus control (P=0.003. Analysis showed significant negative correlation between HDL and CIMT (r=-0.32, P=0.03 but not for LDL, triglycerides, BP, waist circumference, or BMI. Conclusion. For pediatric patients, the thickest CIMT occurred for patients with MS alone or for those with MS+heFH. This indicates that MS, rather than just elevated LDL, accounts for more rapid thickening of CIMT in this population.
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.
Zidan, Ahmed S; Emam, Sherif E; Shehata, Tamer M; Ghazy, Fakhr-eldin S
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.
Okada, Shinichi; Inaga, Sumire; Kawaba, Yasuo; Hanada, Takuya; Hayashi, Atsushi; Nakane, Hironobu; Naguro, Tomonori; Kaidoh, Toshiyuki; Kanzaki, Susumu
Despite intensive treatment, steroid-resistant nephrotic syndrome (NS) often progresses to endstage renal disease. Therefore, a more accurate and quick histological diagnosis is required to properly treat such patients. The aim of this study was to introduce a novel approach to the histological diagnosis of pediatric NS by low vacuum scanning electron microscopy (LVSEM) and to describe the morphological differences in glomeruli between steroid-sensitive and steroid-resistant NS specimens. The subjects were three patients with steroid-sensitive NS and four patients with steroid-resistant NS. Conventional renal biopsy paraffin sections were stained with platinum-blue (Pt-blue) or periodic acid methenamine silver (PAM) and directly observed under LVSEM at magnifications between ×50 and ×10,000. The Pt-blue-stained sections showed three-dimensional structural alterations in glomerular podocytes and foot processes. PAM-stained sections showed changes in the structure and thickness of the glomerular basement membrane (GBM). Consequently, many round-shaped podocytes and elongated primary foot processes were exclusively recognized in steroid-resistant NS, although irregularities in foot process interdigitation, fusions, effacements, and microvillus transformations were observed in both steroid-sensitive and steroidresistant NS. Irregularities in thickness and the wrinkling of GBMs were clearly detected in steroid-resistant NS. The evaluation by LVSEM is probably useful for the renal histological diagnosis of pediatric NS.
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
Warris, Lidewij T.; van den Akker, Erica L. T.; Bierings, Marc B.; van den Bos, Cor; Zwaan, Christian M.; Sassen, Sebastiaan D. T.; Tissing, Wim J. E.; Veening, Margreet A.; Pieters, Rob; van den Heuvel-Eibrink, Marry M.
Although dexamethasone is highly effective in the treatment of pediatric acute lymphoblastic leukemia (ALL), it can cause serious metabolic side effects. Because studies regarding the effects of dexamethasone are limited by their small scale, we prospectively studied the direct effects of treating
Tiwari, Ashish; Goel, Manjusha; Pal, Pankaj; Gohiya, Poorva
Cushing syndrome, a systemic disorder, is the result of abnormally high blood level of cortisol or other glucocorticoids. The most common cause of Cushing syndrome is prolonged exogenous administration of glucocorticoid hormones. Prolonged use of topical corticosteroids, particularly in children, may cause Cushing syndrome and suppression of the hypothalamopituitory-adrenal axis, which is less common than that of oral or parenteral route. However, iatrogenic Cushing syndrome in the infantile age group due to topical steroid is very rare and only a few patients have been reported to date in the literature. Here we report a case of iatrogenic Cushing syndrome due to topical steroid application in a 5-month-old female child admitted to the hospital for repeated episodes of fever and cough.
Dreisinger, Naomi; Zapolsky, Nathan
The emergency department (ED) is an environment that is conducive to medical errors. The ED is a time-pressured environment where physicians aim to rapidly evaluate and treat patients. Quick thinking and problem-based solutions are often used to assist in evaluation and diagnosis. Error analysis leads to an understanding of the cause of a medical error and is important to prevent future errors. Research suggests mechanisms to prevent medical errors in the pediatric ED, but prevention is not always possible. Transparency about errors is necessary to assure a trusting doctor-patient relationship. Patients want to be informed about all errors, and apologies are hard. Apologizing for a significant medical error that may have caused a complication is even harder. Having a systematic way to go about apologizing makes the process easier, and helps assure that the right information is relayed to the patient and his or her family. This creates an environment of autonomy and shared decision making that is ultimately beneficial to all aspects of patient care.
Arbuckle, Robert; Abetz, Linda; Durmer, Jeffrey S; Ivanenko, Anna; Owens, Judith A; Croenlein, Jens; Bolton, Kate; Moore, Adam; Allen, Richard P; Walters, Arthur S; Picchietti, Daniel L
To develop a questionnaire to measure Pediatric Restless Legs Syndrome (P-RLS) symptoms and impact for use in clinical research. Questionnaire items were developed based on open-ended, qualitative interviews of 33 children and adolescents diagnosed with definite RLS (ages 6-17 years) and their parents. The draft questionnaire was then tested through cognitive debriefing interviews with 21 of the same children/adolescents and 15 of their parents. This involved the children and parents answering the draft items and then interviewing them about the child's ability to understand and interpret the questionnaire. Expert clinicians provided clinical guidance throughout. Draft severity questions were generated to measure the four-symptom and four-impact domains identified from the concept elicitation interviews: RLS sensations, move/rub due to RLS, relief from move/rub, pain, and impact of RLS on sleep, awake activities, emotions, and tiredness. RLS descriptions, symptoms, and impact were compared between those who had comorbid attention-deficit/hyperactivity disorder and those who did not. Revisions to several questions were made based on the cognitive debriefing interviews and expert clinician review, resulting in a severity scale with 17 morning and 24 evening items. Caution regarding self-administration in children ages 6-8 years is recommended. To complement the child/adolescent measures, a separate parent questionnaire was also developed. The P-RLS-SS was constructed based on detailed input from children and adolescents with RLS, their parents, and clinical experts, thus providing a scale with strong content validity that is intended to be comprehensive, clinically relevant, and important to patients. Validation of this scale is recommended.
Close, Sharron; Smaldone, Arlene; Fennoy, Ilene; Reame, Nancy; Grey, Margaret
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
Wocial, Lucia; Ackerman, Veda; Leland, Brian; Benneyworth, Brian; Patel, Vinit; Tong, Yan; Nitu, Mara
This paper describes a practice innovation: the addition of formal weekly discussions of patients with prolonged PICU stay to reduce healthcare providers' moral distress and decrease length of stay for patients with life-threatening illnesses. We evaluated the innovation using a pre/post intervention design measuring provider moral distress and comparing patient outcomes using retrospective historical controls. Physicians and nurses on staff in our pediatric intensive care unit in a quaternary care children's hospital participated in the evaluation. There were 60 patients in the interventional group and 66 patients in the historical control group. We evaluated the impact of weekly meetings (PEACE rounds) to establish goals of care for patients with longer than 10 days length of stay in the ICU for a year. Moral distress was measured intermittently and reported moral distress thermometer (MDT) scores fluctuated. "Clinical situations" represented the most frequent contributing factor to moral distress. Post intervention, overall moral distress scores, measured on the moral distress scale revised (MDS-R), were lower for respondents in all categories (non-significant), and on three specific items (significant). Patient outcomes before and after PEACE intervention showed a statistically significant decrease in PRISM indexed LOS (4.94 control vs 3.37 PEACE, p = 0.015), a statistically significant increase in both code status changes DNR (11 % control, 28 % PEACE, p = 0.013), and in-hospital death (9 % control, 25 % PEACE, p = 0.015), with no change in patient 30 or 365 day mortality. The addition of a clinical ethicist and senior intensivist to weekly inter-professional team meetings facilitated difficult conversations regarding realistic goals of care. The study demonstrated that the PEACE intervention had a positive impact on some factors that contribute to moral distress and can shorten PICU length of stay for some patients.
Yang, Wuyang; Xu, Risheng; Porras, Jose L; Takemoto, Clifford M; Khalid, Syed; Garzon-Muvdi, Tomas; Caplan, Justin M; Colby, Geoffrey P; Coon, Alexander L; Tamargo, Rafael J; Huang, Judy; Ahn, Edward S
OBJECTIVE Sickle cell disease (SCD) in combination with moyamoya syndrome (MMS) represents a rare complication of SCD, with potentially devastating neurological outcomes. The effectiveness of surgical revascularization in this patient population is currently unclear. The authors' aim was to determine the effectiveness of surgical intervention in their series of SCD-MMS patients by comparing stroke recurrence in those undergoing revascularization and those undergoing conservative transfusion therapy. METHODS The authors performed a retrospective chart review of patients with MMS who were seen at the Johns Hopkins Medical Institution between 1990 and 2013. Pediatric patients (age < 18 years) with confirmed diagnoses of SCD and MMS were included. Intracranial stroke occurrence during the follow-up period was compared between surgically and conservatively managed patients. RESULTS A total of 15 pediatric SCD-MMS patients (28 affected hemispheres) were included in this study, and all were African American. Seven patients (12 hemispheres) were treated with indirect surgical revascularization. The average age at MMS diagnosis was 9.0 ± 4.0 years, and 9 patients (60.0%) were female. Fourteen patients (93.3%) had strokes before diagnosis of MMS, with an average age at first stroke of 6.6 ± 3.9 years. During an average follow-up period of 11.6 years, 4 patients in the conservative treatment group experienced strokes in 5 hemispheres, whereas no patient undergoing the revascularization procedure had any strokes at follow-up (p = 0.029). Three patients experienced immediate postoperative transient ischemic attacks, but all recovered without subsequent strokes. CONCLUSIONS Indirect revascularization is suggested as a safe and effective alternative to the best medical therapy alone in patients with SCD-MMS. High-risk patients managed on a regimen of chronic transfusion should be considered for indirect revascularization to maximize the effect of stroke prevention.
Greenbaum, L.A.; Fila, M.; Ardissino, G.; Al-Akash, S.I.; Evans, J.; Henning, P.; Lieberman, K.V.; Maringhini, S.; Pape, L.; Rees, L.; Kar, N.C. van de; Walle, J. Vande; Ogawa, M.; Bedrosian, C.L.; Licht, C.
Atypical hemolytic uremic syndrome (aHUS) is caused by alternative complement pathway dysregulation, leading to systemic thrombotic microangiopathy (TMA) and severe end-organ damage. Based on 2 prospective studies in mostly adults and retrospective data in children, eculizumab, a terminal complement
Lundin, Catarina; Forestier, Erik; Klarskov Andersen, Mette
BACKGROUND: Children with Down syndrome (DS) have an increased risk for acute lymphoblastic leukemia (ALL). Although previous studies have shown that DS-ALL differs clinically and genetically from non-DS-ALL, much remains to be elucidated as regards genetic and prognostic factors in DS-ALL. METHODS...
Varela-Fascinetto, G; Greenawalt, S R; Villegas-Alvarez, F
Short bowel syndrome (SBS) continues to be one of the most challenging problems in pediatric surgery. Intestinal transplantation (IT) seems to be the best form of treatment for this pathology. However, it is thought that the development of an IT program may be more expensive than the present manner of treatment. To assess this item, and to identify potential candidates for IT, we reviewed the charts of all the patients with SBS treated at our Institute from 1989 to 1994. Nine patients were identified as carriers of SBS; six with intestinal atresia, two with midgut volvulus and one with post-traumatic mesenteric thrombosis. The small bowel remnants varied from 1 to 80 cm, seven patients had remnants shorter than 30 cm, and the ileocecal valve was resected in three. The overall morbidity and mortality was extremely high; four patients died within the first 3 months postresection and those still alive have had several complications: sepsis; hydroelectrolyte imbalances secondary to loose stools; thrombosis or infection of the catheter; TPN-related cholestasis, and malabsorption syndromes, etc. No patient survived with an intestinal remnant shorter than 15 cm. Of the five survivors, four have a weight/age deficit greater than 40%, two have rickets, one still depends on TPN and all, except one, require special enteral diets. Multiple central venous accesses had to be performed in every patient (mean 4.8). They all required multiple readmissions and have spent a considerable part of their lives as inpatients. The mean of the calculated cost per patient was $50,000 USD, while the minimal wage in Mexico is $1,616 USD/year (1). The shorter the segment of the retained bowel and the longer the survival, the higher the cost. These results may be further improved with the development of IT and, probably, with the same economic burden.
Johnson, Raymond M; Bergmann, Kelly R; Manaloor, John J; Yu, Xiaoqing; Slaven, James E; Kharbanda, Anupam B
Background. Pediatric Kawasaki disease (KD) and human immunodeficiency virus (HIV) + adult Kawasaki-like syndrome (KLS) are dramatic vasculitides with similar physical findings. Both syndromes include unusual arterial histopathology with immunoglobulin (Ig)A + plasma cells, and both impressively respond to pooled Ig therapy. Their distinctive presentations, histopathology, and therapeutic response suggest a common etiology. Because blood is in immediate contact with inflamed arteries, we investigated whether KD and KLS share an inflammatory signature in serum. Methods. A custom multiplex enzyme-linked immunosorbent assay (ELISA) defined the serum cytokine milieu in 2 adults with KLS during acute and convalescent phases, with asymptomatic HIV + subjects not taking antiretroviral therapy serving as controls. We then prospectively collected serum and plasma samples from children hospitalized with KD, unrelated febrile illnesses, and noninfectious conditions, analyzing them with a custom multiplex ELISA based on the KLS data. Results. Patients with KLS and KD subjects shared an inflammatory signature including acute-phase reactants reflecting tumor necrosis factor (TNF)-α biologic activity (soluble TNF receptor I/II) and endothelial/smooth muscle chemokines Ccl1 (Th2), Ccl2 (vascular inflammation), and Cxcl11 (plasma cell recruitment). Ccl1 was specifically elevated in KD versus febrile controls, suggesting a unique relationship between Ccl1 and KD/KLS pathogenesis. Conclusions. This study defines a KD/KLS inflammatory signature mirroring a dysfunctional response likely to a common etiologic agent. The KD/KLS inflammatory signature based on elevated acute-phase reactants and specific endothelial/smooth muscle chemokines was able to identify KD subjects versus febrile controls, and it may serve as a practicable diagnostic test for KD.
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.
Full Text Available Nephrotic syndrome is a chronic disease that increases the risk of skin, respiratory and urinary tract infection, while also increasing the chance for other diseases, like peritonitis and meningitis. A four year old patient with a history of nephrotic syndrome was admitted to emergency room (ER with the following symptoms: abdominal pain, fever, diarrhea and vomiting, associated to abdominal wall erythema, abdominal distension and peritoneal signs. In order to make a differential diagnosis of the infection, peritoneal fluid was extracted and, according to the characteristics found, treatment with broad-spectrum antibiotics was started. Cases in which different infections like pneumonia, abdominal wall peritonitis and cellulitis occur simultaneously have been reported rarely. Early diagnosis and dismissal of other causes of acute abdominal pain, as well as early introduction of antibiotics are fundamental in the treatment of these kind of infections.
Tack, L J W; Tatsi, C; Stratakis, C A; Lodish, M B
Chronic exposure to supraphysiologic levels of glucocorticoids (GCs) is associated with impaired bone mineral density, an increase in fracture rates, and, in growing children, compromised linear growth. GCs inhibit bone formation in part by decreasing the number of osteoblasts and by increasing bone resorption by stimulating osteoclasts. While GCs are used to treat many chronic diseases, it is difficult to isolate the effects of the steroids on the bone from the effects of the underlying disease itself. Investigation into the effects of GC exposure on the bone in endogenous Cushing syndrome have contributed to our understanding of bone microarchitecture, growth, healing, and regeneration. We now know that GCs negatively impact bone marrow derived-mesenchymal stromal cells. In children with Cushing syndrome, the potential reversibility of deleterious effects of chronic GC exposure on bone provides insight into the pathophysiology behind pure GC excess. © Georg Thieme Verlag KG Stuttgart · New York.
ARZANIAN, Mohammad Thaghi; SHAMSIAN, Bibi Shahin; KARIMZADEH, Parvaneh; KAJIYAZDI, Mohammad; MALEK, Fatima; HAMMOUD, Mohammad
Objective Posterior reversible encephalopathy syndrome (PRES) is a cliniconeuroradiological disease entity, which is represented by characteristic magnetic resonance imaging (MRI) findings of subcortical/cortical hyperintensity in T2-weighted sequences. It is more often seen in parietaloccipital lobes, and is accompanied by clinical neurological changes. PRES is a rare central nervous system (CNS) complication in patients with childhood hematologic-oncologic disese and shows very different neurological symptoms between patients, ranging from numbness of extremities to generalized seizure. In this article, we will review PRES presentation in hematologic-oncologic patients. Then, we will present our patient, a 7-year-old boy with Evans syndrome on treatment with cyclosporine, mycophenolate mofetil (MMF) and prednisone, with seizure episodes and MRI finding in favour of PRES. PMID:24949044
W Guite, Jessica; Sherry, David D; Jarvis, Esther W; O Lewen, Margaret; Khan, Sarosh; Wickham Kraemer, Francis
To characterize medication use by adolescents with chronic musculoskeletal pain syndromes before an initial multidisciplinary clinic visit. A cross-sectional sample of 120 adolescents and parents reported on standardized assessment measures, with medication use data extracted from the medical chart and categorized. On average, 3.2 medications were reported; 70% used more than one pain-specific medication including opioids (17%), nonopioids (31%), psychotropics/neuropathics (45%) and other medications (13%). Adolescents with complex regional pain syndrome consistently reported greatest use of opioid, psychotropic/neuropathic and other pain medications. A regression model explained 17% of the variance in pain medication use. Nonpain medication use and disability contributed unique variance - pain duration and intensity did not. Greater attention to factors contributing to prescriptive practices, medication use and long-term outcomes is warranted.
Mantero, Natalia M; Jaime, Lorena J; Nijamin, Tamara R; Laffargue, Jorge A; De Lillo, Leonardo; Grees, Susana A
Norwegian (crusted) scabies is a rare and extreme manifestation of scabies that can be observed mainly among immunosuppressed patients. Due to the high number of scabies mites present in each lesion, crusted scabies symptoms are much more intense than in usual scabies and it is thus highly contagious. A case study of a child with Down syndrome and Norwegian scabies who shows a good response to a treatment combining keratolytics, emollients, ivermectin and topical scabicides is described.
Full Text Available Introduction: Steroid treatment for corticosteroid-sensitive nephrotic syndrome (CSNS could cause bone and mineral metabolism alterations, preventable with calcium and Vitamin D. Objectives: We carried out a preliminary retrospective study along 36 months with the following objectives. 1 To evaluate the relationship between Cumulative Corticosteroid Doses (CCD and 25-0 Vitamin D serum concentration and with Bone Mineral Content (BMC; 2 To evaluate the relationship between CCD and Bone Mineral Densitomety (BMD. Methods: We included patients between 2 and 12 years of age with corticosteroid sensitive primary nephrotic syndrome (CSNS (first episode, relapsing nephrotic syndrome, corticosteroid dependent nephrotic syndrome normotensive, eutrophic and FG>:90ml/min/1.73 m2, who were divided into three groups: GROUP A: =3 or 4 relapses/year, GROUP C: CSNS, we measured: a Quarterly: calcemia, phosphatemia, alkaline phosphatase; b half-yearly: 25-OH cholecalcipherol levels, CCD; c annually BMD in children >6 years (score Z and BMC, bone age, PTHi. Results: We evaluated 29 patients, average age: 4.4 years. The BMD was performed on 11 patients and there were no significant differences among the groups (p=0.08. No significant differences were seen between chronologic age and average bone age (p=0.3. Change in 25-OH cholecalcipherol levels due to the increase of ergocalcipherol dose was significant (T:32.4 Q:<0.001. There were significant correlation in the three groups, between Vitamin D dose and Vitamin D serum levels (Pearson correlation R=0.9, between CCD and 25 OH cholecalcipherol dose: (Pearson correlation R=0.62 and between CCD and BMC (Pearson correlation R=0.44. Finally, in these three groups the average increase of vitamin D was: 1833IU. Conclusions: We found a relationship between CCD and hypovitaminosis D, which could be corrected increasing Vitamin D dose.
Lee, Dae Sik; Kim, Yeong Pil; Kim, Yeong Jin
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.
Lee, Dae Sik; Kim, Yeong Pil; Kim, Yeong Jin
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.
Benn, Peter; Iyengar, Sushma; Crowley, Terrence Blaine; Zackai, Elaine H; Burrows, Evanette K; Moshkevich, Solomon; McDonald-McGinn, Donna M; Sullivan, Kathleen E; Demko, Zachary
The 22q11.2 deletion syndrome is a variably expressed disorder that can include cardiac, palate, and other physical abnormalities, immunodeficiency, and hypocalcemia. Because of the extreme variability in phenotype, there has been no available estimate of the total medical expenditure associated with the average case. We have developed a model to estimate the cost from the time of diagnosis to age 20. Costs were based on patients seen at a specialty center but also considered those components of care expected to have been provided by external healthcare facilities. Expense was based on billed medical charges extracted from the electronic medical billing system for all patients with a diagnosis of DiGeorge or velocardiofacial syndrome from 1993-2015. Expenditures included maternal prenatal care directly related to an affected pregnancy, molecular/cytogenetic diagnosis, consultations, surgery, and/or other treatment and management. Most mental health services (except inpatient), therapy related to cognitive, behavioral, speech, pharmacy, and nonmedical costs (special education, vocational, respite, lost earnings) were not included. Data were available for 642 patients with 50.7% diagnosed prenatally or in the first year of life. The average cost for a patient was $727,178. Costs were highest for patients ascertained prenatally ($2,599,955) or in the first year of life ($1,043,096), those with cardiac abnormalities or referred for cardiac evaluation ($751,535), and patients with low T-cell counts ($1,382,222). This study demonstrates that there are significant medical costs associated with 22q11.2 deletion syndrome. © 2017 Natera. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc.
Nicholas T. Gamboa, B.S.
Full Text Available Turner syndrome (TS; 45,X0 is a relatively common chromosomal disorder that is associated with characteristic phenotypic stigmata: short stature, webbed neck, broad (“shield” chest with widely spaced nipples, cubitus valgus, ovarian dysgenesis (“streak ovary”, primary amenorrhea, renal anomalies, lymphedema of the hands or feet, and various vascular abnormalities. Abnormalities of the cardiovascular system are commonly reported in patient with TS, and vascular anomalies affecting various other organ systems are also frequently reported. To date, however, few reports of intracranial vascular malformations exist. The authors report the case of a patient with TS who was found to have multiple cerebral cavernous malformations on imaging.
Tieder, M; Manor, H; Peshin, J; Alon, U S
A 5-year-old girl presented with lower limb deformities, delayed ambulation, short stature, facial dysmorphism and scoliosis. Radiologic examination showed severe anterior and external bowing of the femurs and anterior and internal bowing of the tibia and fibula, with posterior and medial cortical thickening. Square iliac wings, horizontal sacrum and low-set L5 were also seen. The diagnosis of Weismann-Netter, Stuhl syndrome was established with the exclusion of abnormalities in mineral and vitamin D metabolism. This rare skeletal dysplasia should be included in the radiologic differential diagnosis of congenital deformities of the lower extremities.
Plaiasu, Vasilica; Ochiana, Diana; Motei, Gabriela; Anca, Ioana; Georgescu, Adrian
Patau syndrome (trisomy 13) is one of the most common chromosomal anomalies clinically characterized by the presence of numerous malformations with a limited survival rate for most cases. Babies are usually identified at birth and the diagnosis is confirmed with genetic testing. In this review we outline the clinical and cytogenetic aspects of trisomy 13 and associated phenotypes for 5 cases analyzed in the last 3 years, referred to our Clinical Genetics Department. For each child cytogenetic analysis was performed to determine the genetic variant; also, the patients were investigated for other associated malformations (cardiac, cerebral, renal, ocular anomalies). All 5 cases presented multiple malformations, including some but not all signs of the classical clinical triad suggestive of Patau syndrome. The cytogenetic investigation confirmed for each case the suspected diagnosis and also indicated the specific genetic variant, this being a valuable information for the genetic counselling of the families. The application of genetic analysis can increase diagnosis and prognosis accuracy and have an impact on clinical management.
Jonathan D. Santoro
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.
Sousse, Linda E; Herndon, David N; Andersen, Clark R; Ali, Arham; Benjamin, Nicole C; Granchi, Thomas; Suman, Oscar E; Mlcak, Ronald P
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.
Kaufmann, Liane; Zotter, Sibylle; Pixner, Silvia; Starke, Marc; Haberlandt, Edda; Steinmayr-Gensluckner, Maria; Egger, Karl; Schocke, Michael; Weiss, Elisabeth M; Marksteiner, Josef
By merging neuropsychological (CANTAB/cambridge neuropsychological test automated battery) and structural brain imaging data (voxel-based-morphometry) the present study sought to identify the neurocognitive correlates of executive functions in individuals with Asperger syndrome (AS) compared to healthy controls. Results disclosed subtle group differences regarding response speed on only one CANTAB subtest that is thought to tap fronto-executive network functions (SWM/spatial working memory). Across all participants, SWM performance was significantly associated with two brain regions (precentral gyrus white matter, precuneus grey matter), thus suggesting a close link between fronto-executive functions (SWM) and circumscribed fronto-parietal brain structures. Finally, symptom severity (ADOS total score) was best predicted by response speed on a set-shifting task (IES) thought to tap fronto-striatal functions (corrected R2 56%).
Maggini, Valentina; Lombardi, Niccolò; Lenti, Maria Carmela; Masi, Stefano; Trapani, Sandra; Pugi, Alessandra; Mugelli, Alessandro; Vannacci, Alfredo
We describe a case of Steven-Johnson Syndrome (SJS) associated with albuterol exposure in a 6-year-old male. A possible contributing role of albuterol in SJS occurrence in the present case is strongly suggested by the temporal relationship between the event and the initiation of drug therapy as well as by the positive rechallenge. To the best of our knowledge, albuterol had not been previously associated with SJS in medical literature. It can be therefore possible that physicians, pediatricians in particular, probably not aware of the possible risk of albuterol-induced SJS, might underestimate skin reactions in children taking the drug, thus underreporting this kind of severe adverse drug reaction. Copyright © 2015 Elsevier B.V. All rights reserved.
Mouksassi, M S; Marier, J F; Cyran, J; Vinks, A A
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.
Mehmet Burak Özkan
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.
Adriana Almeida Jesus
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
Full Text Available <b>Purpose:</b> Chronic day-to-day symptoms of orthostatic intolerance are the most notable features of postural orthostatic tachycardia syndrome (POTS. However, we have encountered patients with such symptoms and excessive tachycardia but with no symptoms during the tilt-table test (TTT. We aimed to investigate whether POTS patients with chronic orthostatic intolerance always present orthostatic symptoms during the TTT and analyze the factors underlying symptom manifestation during this test. <b>Methods:</b> We retrospectively examined patients who presented with POTS at the Gyeongsang Na tional University Hospital between 2008 and 2011. Diagnosis of POTS was based on chronic day-to-day orthostatic intolerance symptoms as well as excessive tachycardia during the TTT. The patients were divided two groups depending on the presentation of orthostatic symptoms during the TTT. Clinical data and the results of the TTT were compared between these groups. <b>Results:</b> In 22 patients, 7 patients (31.8% did not present orthostatic symptoms during the test. Diastolic blood pressure (BP was significantly lower in the symptom-positive group. The head-up tilt resulted in a significant increase in diastolic BP in the symptom-negative group (<i>P</i> =0.04, while systolic BP had a tendency to decrease in the symptom-positive group (<i>P</i> =0.06. <b>Conclusion:</b> Significant patients with POTS did not present orthostatic symptoms during the TTT despite having chronic daily symptoms. This finding may be important for establishing definitive diagnostic criteria for pediatric POTS. Development of symptoms during TTT might be related to low diastolic BP and abnormal compensatory responses to orthostasis.
Saps, M; van Tilburg, M A L; Lavigne, J V; Miranda, A; Benninga, M A; Taminiau, J A; Di Lorenzo, C
There is little published evidence of efficacy for the most commonly used treatments. Thus, there is an urgent need to conduct clinical trials on existing and novel therapies. In order to address these issues the Rome Foundation and members of the Pediatric Committee of the European Medicines Agency formed a subcommittee on clinical trials to develop guidelines for the design of clinical trials in children with irritable bowel syndrome (IBS). The following recommendations are based on evidence from published data when available and expert opinion. The subcommittee recommends randomized, double-blind, placebo-controlled, parallel-group, clinical trials to assess the efficacy of new drugs. The combined endpoints for abdominal pain are a decrease in intensity of at least 30% compared with baseline and to meet or exceed the Reliable Change Index (RCI) for the sample. Stool consistency is measured with the Bristol Stool Scale Form (BSFS). The subcommittee recommends as entry criteria for abdominal pain a weekly average of worst abdominal pain in past 24 h of at least 3.0 on a 0-10 point scale or at least 30 mm in 100 mm Visual Analog Scale. For stool endpoints the committee recommends an average stool consistency lower than 3 in the BSFS during the run-in period for clinical trials on IBS-C and an average stool consistency greater than 5 in the BSFS during the run-in period for clinical trials on IBS-D. Changes in stool consistency are the primary endpoints for both IBS with diarrhea (IBS-D) and IBS with constipation (IBS-C). © 2016 John Wiley & Sons Ltd.
Furtado, Andre; Zuccoli, Giulio [Section of Neuroradiology Children' s Hospital of Pittsburgh of UPMC, Department of Radiology, Pittsburgh, PA (United States); Hsu, Ariel [University of Pittsburgh Medical Center, Department of Radiology, Pittsburgh, PA (United States); La Colla, Luca [University of Parma, Department of Anesthesiology, Parma (Italy)
Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiological entity affecting both adults and children characterized by neurotoxicity often in setting of hypertension coupled with distinct brain magnetic resonance imaging features. Decreased serum albumin level has been suggested to correlate with the presence of vasogenic brain edema in adult PRES. Serum albumin has thus been hypothesized to protect against neurotoxicity in PRES by reducing vasogenic brain edema through its role in maintaining plasma osmotic pressure and endothelial integrity. The purpose of our study was to investigate if such correlation between decreased serum albumin level and PRES-related vasogenic edema could be found in children. We conducted a retrospective study of 25 pediatric patients diagnosed with PRES. Underlying clinical conditions, presenting symptoms, blood pressures, and serum albumin levels at onset of symptoms were collected. Brain MR imaging studies were reviewed. We used a quantitative method to evaluate the degree of vasogenic edema by measuring apparent diffusion coefficient (ADC) values of the T2-FLAIR hyperintense brain lesions. No significant correlation was found between serum albumin level and degree of PRES-related vasogenic edema. A significant correlation was found between elevated blood pressure and degree of vasogenic edema in the temporal lobes (p = 0.02 and 0.04, respectively) but not in the other cerebral lobes or cerebellum. Our initial results suggest blood pressure, not serum albumin level, as a main biomarker for brain edema in children with PRES. Thus, our study does not suggest a protective role of serum albumin against PRES-related neurotoxicity in children. (orig.)
Anegundi, Rajesh T; Tegginmani, Veeresh S; Battepati, Prashant; Tavargeri, Anand; Patil, Shruthi; Trasad, Vijay; Jain, Garima
Supernumerary teeth are one of the most widely reported and significant anomaly in patients affecting the primary and early mixed dentition. To describe the distribution and characteristics of the supernumerary teeth in South Indian population of paediatric patients. In a 12 year retrospective study, a total of 63,569 patients up to the age of 14 years who visited our department between June 2003 and May 2013 were revised.790 cases of supernumerary teeth were found. Patients were evaluated for age, sex, site, status of dentition, number, position, orientation and type of supernumerary teeth. Statistical analysis was carried out using chi square test. 790 subjects with supernumerary teeth (481 males and 309 females) were detected, revealing male-female ratio of 1.55:1.The most common supernumerary teeth were mesiodens (82.28%), the most common site was the anterior maxilla (92.53%)region. Majority of patients had a single erupted supernumerary oriented straight in the arch. Patients in mixed dentition stage reported with maximum number of supernumerary teeth. The prevalence of supernumerary teeth in non syndromic South Indian paediatric population is 1.24% with slight male predilection and conical mesiodens being the commonest.
Rajesh T Anegundi
Full Text Available Background: Supernumerary teeth are one of the most widely reported and significant anomaly in patients affecting the primary and early mixed dentition. Aim: To describe the distribution and characteristics of the supernumerary teeth in South Indian population of paediatric patients. Design: In a 12 year retrospective study, a total of 63,569 patients up to the age of 14 years who visited our department between June 2003 and May 2013 were revised.790 cases of supernumerary teeth were found. Patients were evaluated for age, sex, site, status of dentition, number, position, orientation and type of supernumerary teeth. Statistical analysis was carried out using chi square test. Results: 790 subjects with supernumerary teeth (481 males and 309 females were detected, revealing male-female ratio of 1.55:1.The most common supernumerary teeth were mesiodens (82.28%, the most common site was the anterior maxilla (92.53%region. Majority of patients had a single erupted supernumerary oriented straight in the arch. Patients in mixed dentition stage reported with maximum number of supernumerary teeth. Conclusions: The prevalence of supernumerary teeth in non syndromicSouth Indian paediatric population is 1.24% with slight male predilection and conical mesiodens being the commonest.
Greene, Deanna J; Church, Jessica A; Dosenbach, Nico U F; Nielsen, Ashley N; Adeyemo, Babatunde; Nardos, Binyam; Petersen, Steven E; Black, Kevin J; Schlaggar, Bradley L
Tourette syndrome (TS) is a developmental neuropsychiatric disorder characterized by motor and vocal tics. Individuals with TS would benefit greatly from advances in prediction of symptom timecourse and treatment effectiveness. As a first step, we applied a multivariate method - support vector machine (SVM) classification - to test whether patterns in brain network activity, measured with resting state functional connectivity (RSFC) MRI, could predict diagnostic group membership for individuals. RSFC data from 42 children with TS (8-15 yrs) and 42 unaffected controls (age, IQ, in-scanner movement matched) were included. While univariate tests identified no significant group differences, SVM classified group membership with ~70% accuracy (p classification that, in addition to an overall accuracy rate for the SVM, provides a confidence measure for the accurate classification of each individual. Our results support the contention that multivariate methods can better capture the complexity of some brain disorders, and hold promise for predicting prognosis and treatment outcome for individuals with TS. © 2016 The Authors. Developmental Science Published by John Wiley & Sons Ltd.
Martínez-Silvestrini, Julio A; Micheo, William F
Outpatient Sports Medicine Clinic. Three adolescent young female athletes who developed clinical findings compatible with complex regional pain syndrome (CRPS) after lower extremity trauma. Based on the clinical picture, the patients were identified and treated with physical therapy, which included desensitization techniques, stress loading, edema control, early mobilization and electrical stimulation. It was combined with pharmacotherapeutic measures such as non-steroidal anti-inflamatories, oral corticosteroids and gabapentin. One of the patients required serotonin selective reuptake inhibitors, opioid analgesics and acupuncture as an adjunctive treatment. All three patients had involvement of the lower extremities, with a degree of pain and edema exceeding the expected for the nature of their injury. They exhibited a good response to conservative treatment, with physical therapy playing a significant role on the treatment plan. One of the patients, whose diagnosis and treatment was delayed due to late referral, had a more severe presentation and protracted recovery. CRPS is a challenging disease that may affect young active and athletic patients. In this population, early clinical suspicion and aggressive treatment may lead to excellent clinical results and the avoidance of invasive procedures or long-term disability. Laboratory and imaging studies should be used to discard other conditions with clinical presentations similar to CRPS.
Christian J. Hendriksz MD
Full Text Available Hunter syndrome (mucopolysaccharidosis II [MPS II] is characterized by lysosomal glycosaminoglycan (GAG accumulation. Although a majority of patients with MPS II experience neurocognitive involvement, few data are available on cerebrospinal fluid (CSF GAG levels in these patients. This study measured GAG levels in CSF collected from 9 patients with MPS II, including 4 adults (aged ≥18 years with normal cognition, and 5 children, 3 of them with cognitive impairment. The CSF total GAG levels were generally higher in the 3 patients with cognitive impairment (range 842.9-2360.9 ng/mL versus those with normal cognitive status (range 356.8-1181.1 ng/mL. Heparan sulfate levels, as measured by mass spectrometry, generally followed a similar pattern, with patients with the severe phenotype having the highest values. These data, limited by small sample size, suggest CSF GAG levels and heparan sulfate levels may be higher in patients with cognitive impairment versus patients with cognitively intact MPS II.
Besbas, Nesrin; Gulhan, Bora; Soylemezoglu, Oguz; Ozcakar, Z Birsin; Korkmaz, Emine; Hayran, Mutlu; Ozaltin, Fatih
Atypical hemolytic uremic syndrome (aHUS) is a devastating disease with significant morbidity and mortality. Its genetic heterogeneity impacts its clinical presentation, progress, and outcome, and there is no consensus on its clinical management. To identify the characteristics of aHUS in Turkish children, an industry-independent registry was established for data collection that includes both retrospective and prospective patients. In total, 146 patients (62 boys, 84 girls) were enrolled; 53 patients (36.3%) were less than 2 years old at initial presentation. Among the 42 patients (37.1%) whose mutation screening was complete for CFH, CFI, MCP, CFB, C3, DGKE, and CHFR5 genes, underlying genetic abnormalities were uncovered in 34 patients (80.9%). Sixty-one patients (41.7%) had extrarenal involvement. During the acute stage, 33 patients (22.6%) received plasma therapy alone, among them 17 patients (51.5%) required dialysis, and 4 patients (12.1%) were still on dialysis at the time of discharge. In total, 103 patients (70.5%) received eculizumab therapy, 16 of whom (15.5%) received eculizumab as a first-line therapy. Plasma therapy was administered to 84.5% of the patients prior to eculizumab. In this group, renal replacement therapy was administered to 80 patients (77.7%) during the acute period. A total of 3 patients died during the acute stage. A total of 101 patients (77.7%) had a glomerular filtration rate >90 mL/min/1.73 m 2 at the 2-year follow-up. The Turkish aHUS registry will increase our knowledge of patients with aHUS who have different genetic backgrounds and will enable evaluation of the different treatment options and outcomes.
Full Text Available Treatment options for irritable bowel syndrome (IBS and inflammatory bowel disease (IBD are notoriously either inadequate (IBS or loaded with potentially serious side effects and risks (IBD. In recent years a growing interest for effective and safer alternatives has focused on the potential role of probiotics and their metabolic substrates, prebiotics. It is in fact conceivable that the microbiome might be targeted by providing the metabolic fuel needed for the growth and expansion of beneficial microorganisms (prebiotics or by administering to the host such microorganisms (probiotics. This review presents a concise update on currently available data, with a special emphasis on children.Data for prebiotics in IBS are scarce. Low doses have shown a beneficial effect, while high doses are counterproductive. On the contrary, several controlled trials of probiotics have yielded encouraging results. A meta-analysis including 9 randomized clinical trials in children showed an improvement in abdominal pain for Lactobacillus GG, Lactobacillus reuteri DSM 17938 and the probiotic mixture VSL#3. The patients most benefitting from probiotics were those with predominant diarrhea or with a post-infectious IBS. In IBD, the use of prebiotics has been tested only rarely and in small scale clinical trials, with mixed results. As for probiotics, data in humans from about 3 dozens clinical trials offer mixed outcomes. So far none of the tested probiotics has proven successful in Crohn’s disease, while in ulcerative colitis a recent meta-analysis on 12 clinical trials (1 of them in children showed efficacy for the probiotic mixture VSL#3 in contributing to induce and to maintain remission. It is evident that this is a rapidly evolving and promising field; more data are very likely to yield a better understanding on what strains and in what doses should be used in different specific clinical settings.
Full Text Available Abstract Introduction Reports on children with Ramsay Hunt syndrome are limited in the literature, resulting in uncertainty regarding the clinical manifestations and outcome of this syndrome. Treatment for Ramsay Hunt syndrome is usually with antivirals, although there is no evidence for beneficial effect on the outcome of Ramsay Hunt syndrome in adults (insufficient data on children exists. Here, we report a case of Ramsay Hunt syndrome occurring in a child who inadvertently received a lower dose of aciclovir and steroid administered for shorter than is usual. Our patient made a full recovery. Case presentation A 13-year-old African boy presented to our out-patients department with an inability to move the right side of his face for one week. He had previously been seen by the doctor on call, who prescribed aciclovir 200 mg three times per day and prednisone 20 mg once daily, both orally for five days, with a working diagnosis of Bell's palsy. After commencement of aciclovir-prednisone, while at home, our patient had headache, malaise, altered taste, vomiting after feeds, a ringing sound in his right ear as well as earache and ear itchiness. Additionally, he developed numerous fluid-filled pimples on his right ear. On presentation, a physical examination revealed a right-sided lower motor neuron facial nerve palsy and a healing rash on the right pinna. On direct questioning, our patient admitted having had chicken pox about three months previously. Based on the history and physical examination, Ramsay Hunt syndrome was diagnosed. Our patient was lost to follow-up until 11 months after the onset of illness; at this time, his facial nerve function was normal. Conclusions This case report documents the clinical manifestations and outcome of pediatric Ramsay Hunt syndrome; a condition with few case reports in the literature. In addition, our patient made a full recovery despite inadvertently receiving a lower dose of aciclovir and steroid
Howard V. Gimbel
Full Text Available The management of ectopia lentis in Marfan’s syndrome is challenging. Multiple disease-associated factors conspire to deprive these patients of adequate vision. While optical correction with glasses and contact lenses is usually advocated early on, the irregular astigmatism and even partial aphakia that accompanies advanced cases generally warrant surgical intervention. Several surgical strategies have been devised to manage these challenging cases, including the combination of endocapsular or pars plana lensectomy and iris or scleral fixation of the intraocular lens (IOL or IOL-bag complex. All of the reported cases have been implanted with IOLs that correct for myopia only. With toric lenses, it is now possible to correct for corneal astigmatism in these patients as well, provided that the capsular bag is maintained and can be properly centered. We report the combination of scleral-fixated Cionni endocapsular rings and toric IOLs in a pediatric patient with bilateral ectopia lentis secondary to Marfan’s syndrome.
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.
Liu Yue; Peng Yun; Gao Peiyi; Nie Binbin; Lu Chuankai; Zhang Liping; Ji Zhiying; Yin Guangheng; Yu Tong; Shan Baoci
Objectives: To identify the related abnormalities of gray matter in pediatric patients with Tourette syndrome (TS) by using the optimized voxel-based morphometry (VBM). Methods: Three dimensional T 1 WI was acquired in 31 TS children (28 boys, 3 girts, mean age 8 years, range 4-15 years) and 50 age- and sex-matched controls on a 1.5 Tesla Philips scanner. Images were pre-processed and analyzed using a version of VBM 2 in SPM 2. The whole brain gray matter volume was compared between the study and control group by using t-test. Multivariate linear regression analysis was used for analyzing the correlation between the change of grey matter volume within each brain region (mm 3 ) and YGTSS score and course of disease of TS patients. Statistical analyses were performed by using SPSS 13.0. Results: Using VBM, significant increases in gray matter volumes in left superior parietal lobule, right cerebellar hemisphere and left parahippocampal gyrus were detected in TS patients, and the volume changes were 4059, 2126 and 84 mm 3 (t=3.93, 3.71, 3.58, P<0.05) respectively. Compared to the control group, decreased grey matter volumes were found in medulla and left pons, and the volume changes were 213 and 117 mm 3 (t=3.53, 3.48, P<0.05)respectively. Tic severity was not correlated with any volume changes of gray matter in brain (P>0.05, a small volume correction, KE ≥ 10 voxel). Tic course was negatively correlated with the gray matter volume of left parahippocampal gyrus (Beta =-0.391, P=0.039). Conclusions: Using VBM technique, the gray matter abnormalities can be revealed in TS patients without obvious lesions on conventional MR imaging. The increasing volume of temporal and parietal lobes and cerebellar may be an adaptive anatomical change in response to experiential demand. The gray matter volume of the parahippocampal gyrus may be used as one potential objective index for evaluating the prognosis of TS. (authors)
Prithi Rajendra Inamdar
Full Text Available Context: Idiopathic nephrotic syndrome (NS is the most common glomerular nephropathy in pediatrics, with great variation in patient characteristics in different regions of the world. Difficult childhood NS poses a great diagnostic and management challenge for the treating nephrologists. The histopathological features of NS have been extensively studied by various authors from different regions of the world with a wide variation in the histopathological distribution. We aimed to describe the spectrum of histopathological diagnosis in childhood NS from North Karnataka, India, where there is a high incidence of consanguinity. Aims: The aim of this study is to study the spectrum of histopathology in children who underwent a renal biopsy in our tertiary care pediatric nephrology center. Settings and Design: Retrospective observational study at a tertiary care pediatric nephrology center over 1 year from January 2013 to June 2015. Subjects and Methods: The medical records of all children diagnosed as NS were retrospectively reviewed for demographic data, clinical course, and histopathological diagnosis. Statistical Analysis Used: Descriptive statistics. Results: Twenty-five children underwent biopsy in the study period. Seventeen (68% were male as compared to 8 (32% females. Thirteen (52% of biopsied patients showed minimal change NS, 6 (24% had focal segmental glomerulosclerosis, and 3 (14% patients showed IgA nephropathy while 1 patient had mesangioproliferative glomerulonephritis, IgM nephropathy, and Finnish type NS each. Conclusions: Minimal change nephritic syndrome is the major biopsy finding in our part of the country even in steroid-resistant NS. This finding needs to be confirmed with studies with bigger sample size.
Hills, Christine; Moller, James H; Finkelstein, Marsha; Lohr, Jamie; Schimmenti, Lisa
To analyze the cases submitted to the Pediatric Cardiac Care Consortium (PCCC) database from 1982 to 2002 to determine the frequency and distribution of congenital heart disease (CHD) found in this population, to review the literature for previously published accounts of CHD in this population, and to review current genotype-phenotype associations for cri du chat (CDC) syndrome with CHD. We performed a retrospective review of the 98422 CHD cases submitted to the PCCC between 1982 and 2002, to find patients who had a noncardiac diagnosis of CDC syndrome. A total of 21 patients (15 female and 6 male patients) were identified. Although some patients had multiple cardiac anomalies, they were categorized according to primary diagnoses on the basis of the most hemodynamically significant component. The patient groups were ventricular septal defect (n = 6), patent ductus arteriosus (n = 6), tetralogy of Fallot (n = 5), pulmonary valve atresia with ventricular septal defect (n = 2), pulmonary valve stenosis (n = 1), and double-outlet right ventricle (n = 1). Eighteen of the 21 patients underwent surgical repair of their defects. There was 1 late operative death. To determine whether the observed frequency of these cardiac defects among patients with CDC syndrome was comparable to that of the general population of patients with CHD, data for all cases submitted to the PCCC from 1982 to 2002 were used. Use of these numbers to determine expected frequencies for these defects showed significantly greater proportions of patients with these specific lesions among the patients with CDC syndrome. Currently there is no clear understanding of the genomic cause of the prevalence of these defects in the population with CDC syndrome, although CHD has been noted among patients with other deletion syndromes.
Jose A. Peña-Zarza
Full Text Available Objective. To assess the screening tools in snoring patients. Material and Methods. A retrospective review of data was conducted from children between 2 and 15 years old who were referred on suspicion of obstructive sleep apnea-hypopnea (OSAH between June 2008 and June 2011. We excluded patients with significant comorbidities. Pediatric Sleep Questionnaire (PSQ, physical exam (PE, and pulse-oximetry data were collected and correlated with the results of the nightly polygraph at home. Results. We selected 98 patients. The 22-item version of the PSQ had sensitivity of 96% and specificity of 36.8%. The overall value of the clinic predictor of OSAH (PSQ and PE together exhibited an increased specificity 57.6% with 94.6% of sensitivity. The nocturnal home oximetry method used alone was very specific, 92.1%, but had a lower sensitivity, 77.1%. The set of clinical assessment tools used together with pulse-oximetry screening provided excellent specificity 98.1% and a positive predictive value 94.1% globally. The performance of this screening tool is related with the severity of OSAH and accuracy is better in moderate and severe cases. Conclusion. The combination of clinical assessment and pulse-oximetry screening can provide a sufficient diagnostic approach for pediatric patients with suspected OSAH at least in moderate and severe cases.
Esposito, Ciro; Escolino, Maria; Bagnara, Vincenzo; Eckoldt-Wolke, Felicitas; Baglaj, Maciej; Saxena, Amulya; Patkowski, Dariusz; Schier, Felix; Settimi, Alessandro; Martelli, Helene; Savanelli, Antonio
The management of intersex patients with Y-chromosome Turner or Morris syndrome remains a challenge. We report our experience with a multicenter European survey. We collected the data on 18 patients (mean age 10.2 years, range 2-17 years) with Morris (10 patients) or Turner (8 patients) syndrome harboring the Y chromosome who were treated in 1 of 6 European centers of pediatric surgery between 1997 and 2013. All patients were evaluated by use of a multidisciplinary diagnostic protocol. All patients received a bilateral gonadectomy via laparoscopy; only 1 center performed ovarian cryopreservation. Seven patients received a concomitant genitoplasty. Operative notes and histology were reviewed for details. No conversions to laparotomy and no complications were recorded. For the patients receiving only the gonadectomy, the length of hospital stay was 24-48 hours, whereas for the patients receiving an associated genitoplasty, it was 6-10 days. Specimens were negative for tumors in 83.3% of cases, whereas in 3 patients (16.6%), benign abnormalities (Sertoli cell hyperplasia in 1 patient and ovotestis in 2 patients) were recorded. A malignant tumor was not recorded in our series. If the risk of malignancy is considered as the main indication for surgery in case of Turner or Morris syndrome, on the basis of our study, this indication should be reevaluated. However, based on the non-negligible rate of benign abnormalities reported in our series (16.6%), the performance of cryopreservation to preserve fertility and the possibility of performing genitoplasty during the same anesthetic procedure represent additional valid indications for surgery. Copyright © 2015 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.
Ngan, Olivia Miu Yung; Yi, Huso; Wong, Samuel Yeung Shan; Sahota, Daljit; Ahmed, Shenaz
While non-invasive prenatal testing (NIPT) for fetal aneuploidy is commercially available in many countries, little is known about how obstetric professionals in non-Western populations perceive the clinical usefulness of NIPT in comparison with existing first-trimester combined screening (FTS) for Down syndrome (DS) or invasive prenatal diagnosis (IPD), or perceptions of their ethical concerns arising from the use of NIPT. A cross-sectional survey among 327 obstetric professionals (237 midwives, 90 obstetricians) in Hong Kong. Compared to FTS, NIPT was believed to: provide more psychological benefits and enable earlier consideration of termination of pregnancy. Compared to IPD, NIPT was believed to: provide less psychological stress for high-risk women and more psychological assurance for low-risk women, and offer an advantage to detect chromosomal abnormalities earlier. Significant differences in perceived clinical usefulness were found by profession and healthcare sector: (1) obstetricians reported more certain views towards the usefulness of NIPT than midwives and (2) professionals in the public sector perceived less usefulness of NIPT than the private sector. Beliefs about earlier detection of DS using NIPT were associated with ethical concerns about increasing abortion. Participants believing that NIPT provided psychological assurance among low-risk women were less likely to be concerned about ethical issues relating to informed decision-making and pre-test consultation for NIPT. Our findings suggest the need for political debate initially on how to ensure pregnant women accessing public services are informed about commercially available more advanced technology, but also on the potential implementation of NIPT within public services to improve access and equity to DS screening services.
Goetghebeur, Mireille M; Wagner, Monika; Khoury, Hanane; Rindress, Donna; Grégoire, Jean-Pierre; Deal, Cheri
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. 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. 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. 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 decision-making.
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.
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.
O'Gorman Clodagh S
Full Text Available Abstract Background Turner Syndrome women are at high risk of vascular disease and the assessment of early risk factors in Turner Syndrome girls is an emerging focus of research. Our objective was to evaluate endothelial function (EF, a preclinical measure of atherosclerosis, in Turner Syndrome girls compared with controls. Methods A cross-sectional case-control study of Turner Syndrome girls and healthy controls. Subjects underwent fasting insulin and glucose with calculation of HOMA-IR, fasting lipid profile, anthropometrics, and EF testing using peripheral arterial tonometry (PAT. Subjects, aged 10-18 years, had karyotype-confirmed Turner Syndrome; growth hormone (GH, thyroxine and estrogen use were not exclusion criteria. Controls were age- and BMI-matched healthy girls. Fifteen Turner Syndrome and 15 controls were recruited. Results Turner Syndrome girls had lower height, higher HDL and higher waist:height ratio than controls. PAT-hyperemia ratio (RH-PAT scores were lower in Turner Syndrome (1.64 ± 0.34 vs. 2.08 ± 0.32, p = 0.002 indicating impaired EF. Among Turner Syndrome, RH-PAT did not vary with estrogen therapy or with karyotype 45,XO compared with other karyotypes. However, endothelial function was better in GH-treated compared with GH-untreated Turner Syndrome (1.80 ± 0.36 vs. 1.4 + 0.22, p = 0.02 although there were no differences in HOMA-IR, adiponectin or IGF-1. Conclusion Girls with Turner Syndrome exhibit impaired endothelial function compared with controls, which may explain higher risk for vascular disease. GH may protect endothelial function in Turner Syndrome.
Grioni, Daniele; Pavan, Fabio; Prunotto, Giulia; Canonico, Francesco; Grandi, Carlo de; Rovelli, Attilio
Despite a wide number of studies trying to define clinical, physiopathological, and neuroradiological features of posterior reversible encephalopathy syndrome (PRES), the true nature of symptoms is still not fully understood. We studied a standard cohort of 24 pediatric patients, affected by hemato-oncological diseases, with a neuroradiological diagnosis consistent with PRES identified from 2006 to 2013. Ten of them developed PRES after hematopoietic stem cell transplantation. We analyzed the sequence of clinical, radiological, and electrophysiological data. In all the patients who were recorded at the onset of the first symptoms, electroencephalograms showed focal nonconvulsive seizures or status epilepticus (SE). We found a sensitivity of 100% for electroencephalogram (EEG) with a good correlation between clinical signs and the localization of seizures, whereas computed tomography scans showed a sensitivity of 50% only. Following prompt treatment, intensive care unit admission rate was only 8%. PRES is a multifactorial neurologic event with focal nonconvulsive seizures or SE as the main feature in pediatric patients. Clinical manifestations are epileptic in nature, and prompt EEG recording is useful for diagnosis and supports an earlier treatment, potentially preventing the appearance of complications such as generalized seizures or refractory SE. Georg Thieme Verlag KG Stuttgart · New York.
Saps, M.; van Tilburg, M. A. L.; Lavigne, J. V.; Miranda, A.; Benninga, M. A.; Taminiau, J. A.; Di Lorenzo, C.
There is little published evidence of efficacy for the most commonly used treatments. Thus, there is an urgent need to conduct clinical trials on existing and novel therapies. In order to address these issues the Rome Foundation and members of the Pediatric Committee of the European Medicines Agency
Araki, Shunsuke; Dobashi, Kazushige; Kubo, Kazuyasu; Kawagoe, Rinko; Yamamoto, Yukiyo; Kawada, Yasusada; Shirahata, Akira; Asayama, Kohtaro
'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)
Daimi, Houria; Khelil, Amel Haj; Ben Hamda, Khaldoun; Aranega, Amelia; Chibani, Jemni B E; Franco, Diego
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.
Madden, Kate; Burns, Michele M; Tasker, Robert C
To identify available assessment tools for sedative/hypnotic iatrogenic withdrawal syndrome and delirium in PICU patients, the evidence supporting their use, and describe areas of overlap between the components of these tools and the symptoms of anticholinergic burden in children. Studies were identified using PubMed and EMBASE from the earliest available date until July 3, 2016, using a combination of MeSH terms "delirium," "substance withdrawal syndrome," and key words "opioids," "benzodiazepines," "critical illness," "ICU," and "intensive care." Review article references were also searched. Human studies reporting assessment of delirium or iatrogenic withdrawal syndrome in children 0-18 years undergoing critical care. Non-English language, exclusively adult, and neonatal intensive care studies were excluded. References cataloged by study type, population, and screening process. Iatrogenic withdrawal syndrome and delirium are both prevalent in the PICU population. Commonly used scales for delirium and iatrogenic withdrawal syndrome assess signs and symptoms in the motor, behavior, and state domains, and exhibit considerable overlap. In addition, signs and symptoms of an anticholinergic toxidrome (a risk associated with some common PICU medications) overlap with components of these scales, specifically in motor, cardiovascular, and psychiatric domains. Although important studies have demonstrated apparent high prevalence of iatrogenic withdrawal syndrome and delirium in the PICU population, the overlap in these scoring systems presents potential difficulty in distinguishing syndromes, both clinically and for research purposes.
Mearig, Judith S.
The article summarizes some major issues involved including social acceptance of and expectations for individuals with Down syndrome; valuing of physical appearance; relevance of intellectual functioning; infliction of avoidable pain or trauma; origins and import of medical professionals' opinions; and the individual's desire for and reactions to…
Miller, Richard B
... 220 11. Ethical Issues in Pediatric Research 238 : ⁄ 275 ⁄ 301 268 AcknowledgmentsACKNOWLEDGMENTS The remotest origin...
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 ...
Cattalini, Marco; Soliani, Martina; Iannone, Florenzo; Galeazzi, Mauro
Autoinflammatory diseases are caused by inflammasome dysregulation leading to overproduction of proinflammatory cytokines and a pathological delay in the inflammation switching off. The progress of cellular biology has partially clarified pathogenic mechanisms behind monogenic autoinflammatory diseases, whereas little is known about the polygenic ones. Although the genetic susceptibility of periodic fever, aphthous stomatitis, pharyngitis, and adenopathy (PFAPA) syndrome is still obscure, the presence of overlapping symptoms with monogenic periodic fevers, the recurrence in family members, the important role played by dysregulated interleukin- (IL-) 1β secretion during flares, the overexpression of inflammasome-associated genes during attacks, and, last but not least, the therapeutic efficacy of IL-1β blockade strongly indicate a potential genetic involvement in its pathogenesis, probably linked with environmental factors. PFAPA syndrome has a typical inception in the pediatric age, but a delayed onset during adulthood has been described as well. Treatments required as well as effectiveness of tonsillectomy remain controversial, even if the disease seems to have a self-limited course mostly in children. The purpose of this review is to provide an overview of this complex polygenic/multifactorial autoinflammatory disorder in which the innate immune system undoubtedly plays a basic role. PMID:26357457
Lin, J.C.; Spinella, P.C.; Fitzgerald, J.C.; Tucci, M.; Bush, J.L.; Nadkarni, V.M.; Thomas, N.J.; Weiss, S.L.; Lemson, J.; Sherring, C.; Bushell, T.
OBJECTIVES: To describe the epidemiology, morbidity, and mortality of new or progressive multiple organ dysfunction syndrome in children with severe sepsis. DESIGN: Secondary analysis of a prospective, cross-sectional, point prevalence study. SETTING: International, multicenter PICUs. PATIENTS:
Wallace, Scott A; Ignacio, Romeo C; Klugh, Arnett; Gates, Gregory; Henry, Marion C W
Osteoblastoma is an uncommon primary bone tumor that usually presents as a painful lesion in a long bone or in the spine. Osteoblastoma has been reported only twice in the literature in conjunction with systemic fibromatosis. The authors report the case of an 8-year-old girl with suspected Klippel-Trenaunay-Weber syndrome, a rare syndrome of systemic fibromatosis, who presented with a painless thoracic rib lesion that was found to be an osteoblastoma.
... Text Size Email Print Share What is a Pediatric Ophthalmologist? Page Content Article Body If your child ... treat your child. What Kind of Training Do Pediatric Ophthalmologists Have? Pediatric ophthalmologists are medical doctors who ...
... 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 ...
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
Dahlem, P.; van Aalderen, W. M. C.; Bos, A. P.
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%),
Brouwer, Marije; Kaczor, Christopher; Battin, Margaret P; Maeckelberghe, Els; Lantos, John D; Verhagen, Eduard
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.
Loos, Sebastian; Aulbert, Wiebke; Hoppe, Bernd; Ahlenstiel-Grunow, Thurid; Kranz, Birgitta; Wahl, Charlotte; Staude, Hagen; Humberg, Alexander; Benz, Kerstin; Krause, Martin; Pohl, Martin; Liebau, Max C; Schild, Raphael; Lemke, Johanna; Beringer, Ortraud; Müller, Dominik; Härtel, Christoph; Wigger, Marianne; Vester, Udo; Konrad, Martin; Haffner, Dieter; Pape, Lars; Oh, Jun; Kemper, Markus J
In 2011 Escherichia coli O104:H4 caused an outbreak with >800 cases of hemolytic uremic syndrome (HUS) in Germany, including 90 children. Data on the intermediate outcome in children after HUS due to E. coli O104:H4 have been lacking. Follow-up data were gathered retrospectively from the medical records of patients who had been included in the German Pediatric HUS Registry during the 2011 outbreak. Seventy-two of the 89 (81%) patients were included after a median follow-up of 3.0 (0.9-4.7) years. Hypertension and proteinuria were present in 19% and 28% of these patients, respectively. Of 4 patients with chronic kidney disease (CKD) > stage 2 at short-term follow-up, 1 had a normalized estimated glomerular filtration rate, and 3 (4%) had persistent CKD > stage 2. In 1 of these patients, CKD improved from stage 4 to 3; 1 who had CKD stage 5 at presentation received kidney transplantation; and 1 patient required further hemodialysis during follow-up. One patient (1.4%) still had major neurological symptoms at the latest follow-up. Dialysis during the acute phase (P = .01), dialysis duration (P = .01), and the duration of oligo-/anuria (P = .005) were associated with the development of renal sequelae. Patients treated with eculizumab (n = 11) and/or plasmapheresis (n = 13) during the acute phase of HUS had comparable outcomes. The overall outcome of pediatric patients after HUS due to E. coli O104:H4 was equivalent to previous reports on HUS due to other types of Shiga toxin-producing E. coli (STEC). Regular follow-up visits in patients are recommended after STEC-HUS. © The Author 2017. Published by Oxford University Press for the Infectious Diseases Society of America. All rights reserved. For permissions, e-mail: email@example.com.
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.
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 ...
Faure, C.; Patey, N.; Gauthier, C.; Brooks, E.M.; Mawe, G.M.
In adults, irritable bowel syndrome (IBS) and functional dyspepsia (FD) are chronic conditions that often start during childhood. We investigated mucosal serotonin (5-HT) signalling in children with the idea that data from subjects with a shorter history may improve our understanding of underlying pathophysiological mechanisms.
Russo-Ponsaran, Nicole M.; Yesensky, Jessica; Hessl, David; Berry-Kravis Elizabeth
Fragile X syndrome (FXS) is the most common inherited cause of intellectual disability and the most common known genetic cause of autism. FXS is associated with psychiatric impairments, including anxiety disorders. There is a paucity of well-developed measures to characterize anxiety in FXS. However, such scales are needed to measure therapeutic…
Jonasson, Haukur Ingi
How relevant is ethics to project management? The book - which aims to demystify the field of ethics for project managers and managers in general - takes both a critical and a practical look at project management in terms of success criteria, and ethical opportunities and risks. The goal is to help the reader to use ethical theory to further identify opportunities and risks within their projects and thereby to advance more directly along the path of mature and sustainable managerial practice.
Sternberg, Robert J.
Ethical impotence occurs when one wants to act ethically but feels powerless to do anything about the perceived unethical behavior. One may feel that one's actions will have no impact or that those actions actually will have harmful consequences to oneself and/or others. Ethical impotence can be understood in terms of an eight-step model of…
... area in medicine that doesn't have an ethical aspect. For example, there are ethical issues relating to End of life care: Should ... orders? Abortion: When does life begin? Is it ethical to terminate a pregnancy with a birth defect? ...
den Hartog, D.N.
High-profile cases of leaders’ ethical failure in different settings and sectors have led to increased attention to ethical leadership in organizations. In this review, I discuss the rapidly developing field of ethical leadership from an organizational behavior/psychology perspective, taking a
Contemporary medical ethics is far from the traditional concept of "In-Sul (benevolent art)" or "Yul-Li (倫, ethics), which emphasizes so much the personality or the character of a doctor. Nowadays, medical ethics should be considered as "professional ethics" which regulates the acts and medical practices of ordinary doctors in their daily practice. The key concepts of the professional ethics are "autonomy", "integrity", and "professional standard" established by medical organizations such as medical societies or associations. Most of Korean doctors have not been familiar with the concept of professional ethics or professionalism, which is due to the modern history of Korea. However, the concept of professional ethics is really critical to Korean doctors from the perspective of professional dignity and social respect to this profession. The current healthcare system of Korea is suffering from many problems of both private and public sector. Nonetheless, the professional ethics is urgently demanded for that very reason.
Hansson, Sven Ove
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.
Kristen M. Merino
Full Text Available Monocytes/macrophages are a diverse group of cells that act as first responders in innate immunity and then as mediators for adaptive immunity to help clear infections. In performing these functions, however, the macrophage inflammatory responses can also contribute to pathogenesis. Various monocyte and tissue macrophage subsets have been associated with inflammatory disorders and tissue pathogeneses such as occur during HIV infection. Non-human primate research of simian immunodeficiency virus (SIV has been invaluable in better understanding the pathogenesis of HIV infection. The question of HIV/SIV-infected macrophages serving as a viral reservoir has become significant for achieving a cure. In the rhesus macaque model, SIV-infected macrophages have been shown to promote pathogenesis in several tissues resulting in cardiovascular, metabolic, and neurological diseases. Results from human studies illustrated that alveolar macrophages could be an important HIV reservoir and humanized myeloid-only mice supported productive HIV infection and viral persistence in macrophages during ART treatment. Depletion of CD4+ T cells is considered the primary cause for terminal progression, but it was reported that increasing monocyte turnover was a significantly better predictor in SIV-infected adult macaques. Notably, pediatric cases of HIV/SIV exhibit faster and more severe disease progression than adults, yet neonates have fewer target T cells and generally lack the hallmark CD4+ T cell depletion typical of adult infections. Current data show that the baseline blood monocyte turnover rate was significantly higher in neonatal macaques compared to adults and this remained high with disease progression. In this review, we discuss recent data exploring the contribution of monocytes and macrophages to HIV/SIV infection and progression. Furthermore, we highlight the need to further investigate their role in pediatric cases of infection.
Paulmann, Maren; Mockenhaupt, Maja
Fever is a symptom that often accompanies skin eruptions, especially in children. It can be a sign of an infectious condition presenting with exanthems or it may precede an exanthematous eruption. Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe reactions affecting skin and mucosa with blisters and erosions. High fever occurs in these conditions, frequently before the skin and/or mucosa is affected.
Zawitkowska, Joanna; Odój, Teresa; Drabko, Katarzyna; Zaucha-Prażmo, Agnieszka; Rudnicka, Julia; Romiszewski, Michał; Matysiak, Michał; Kwiecińska, Kinga; Ćwiklińska, Magdalena; Balwierz, Walentyna; Owoc-Lempach, Joanna; Derwich, Katarzyna; Wachowiak, Jacek; Niedźwiecki, Maciej; Adamkiewicz-Drożyńska, Elżbieta; Trelińska, Joanna; Młynarski, Wojciech; Kołtan, Andrzej; Wysocki, Mariusz; Tomaszewska, Renata; Szczepański, Tomasz; Płonowski, Marcin; Krawczuk-Rybak, Maryna; Ociepa, Tomasz; Urasiński, Tomasz; Mizia-Malarz, Agnieszka; Sobol-Milejska, Grażyna; Karolczyk, Grażyna; Kowalczyk, Jerzy
Children with Down syndrome (DS) have a 20-fold increased risk of developing leukemia compared with the general population. The aim of the study was to analyze the outcome of patients diagnosed with Down syndrome and acute lymphoblastic leukemia (ALL) in Poland between the years 2003 and 2010. A total of 1848 children were diagnosed with ALL (810 females and 1038 males). Of those, 41 (2.2%) had DS. The children were classified into three risk groups: a standard-risk group-14 patients, an intermediate-risk group-24, a high-risk group-3. All patients were treated according to ALLIC 2002 protocol. The median observation time of all patients was 6.1 years, and in patients with DS 5.3 years. Five-year overall survival (OS) was the same in all patients (86% vs 86%, long-rank test, p = .9). The relapse-free survival (RFS) was calculated as 73% in patients with DS and 81% in patients without DS during a median observation time (long-rank test, p = .3). No statistically significant differences were found in the incidence of nonrelapse mortality between those two groups of patients (p = .72). The study was based on children with ALL and Down syndrome who were treated with an identical therapy schedule as ALL patients without DS, according to risk group. This fact can increase the value of the presented results.
Staaf, Johan; Labmayr, Viktor; Paulmichl, Katharina; Manell, Hannes; Cen, Jing; Ciba, Iris; Dahlbom, Marie; Roomp, Kirsten; Anderwald, Christian-Heinz; Meissnitzer, Matthias; Schneider, Reinhard; Forslund, Anders; Widhalm, Kurt; Bergquist, Jonas; Ahlström, Håkan; Bergsten, Peter; Weghuber, Daniel; Kullberg, Joel
Adolescents with obesity have increased risk of type 2 diabetes and metabolic syndrome (MetS). Pancreatic fat has been related to these conditions; however, little is known about associations in pediatric obesity. The present study was designed to explore these associations further. We examined 116 subjects, 90 with obesity. Anthropometry, MetS, blood samples, and oral glucose tolerance tests were assessed using standard techniques. Pancreatic fat fraction (PFF) and other fat depots were quantified using magnetic resonance imaging. The PFF was elevated in subjects with obesity. No association between PFF and body mass index-standard deviation score (BMI-SDS) was found in the obesity subcohort. Pancreatic fat fraction correlated to Insulin Secretion Sensitivity Index-2 and Homeostatic Model Assessment of Insulin Resistance in simple regression; however, when using adjusted regression and correcting for BMI-SDS and other fat compartments, PFF correlated only to visceral adipose tissue and fasting glucose. Highest levels of PFF were found in subjects with obesity and MetS. In adolescents with obesity, PFF is elevated and associated to MetS, fasting glucose, and visceral adipose tissue but not to beta-cell function, glucose tolerance, or BMI-SDS. This study demonstrates that conclusions regarding PFF and its associations depend on the body mass features of the cohort.
Vitamin D Deficiency; Stem Cell Transplant Complications; Pediatric Cancer; Blood Disorder; Pediatric Acute Myeloid Leukemia; Pediatric Acute Lymphoid Leukemia; Myelodysplastic Syndromes; Sickle Cell Anemia in Children; Aplastic Anemia; Thalassemia in Children
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.
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
Gordon, I. [London, Great Ormond Street Hospital for Children (United Kingdom)
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.
Berio, A; Trucchi, R; Meliota, M
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.
Vida, Vladimiro L; Padrini, Maddalena; Boccuzzo, Giovanna; Agnoletti, Gabriella; Bondanza, Sara; Butera, Gianfranco; Chiappa, Enrico; Marasini, Maurizio; Pilati, Mara; Pongiglione, Giacomo; Prandstraller, Daniela; Russo, Maria Giovanna; Castaldi, Biagio; Santoro, Giuseppe; Spadoni, Isabella; Stellin, Giovanni; Milanesi, Ornella
To analyze the clinical status of patients with "uncorrected" scimitar syndrome in a multicenter Italian study. The natural history of scimitar syndrome was analyzed in 44 affected individuals (from 9 Italian centers). The median age at diagnosis was 1.05 years (range, 1 day-41 years). Thirty-three patients (75%) had an isolated form; 11 patients (25%) had associated congenital heart diseases. Twenty-two patients (50%) were symptomatic at diagnosis, including respiratory symptoms (n=20) and congestive heart failure (n=6). Patients with associated congenital heart defects had a higher prevalence of congestive heart failure (4 of 11 [36.4%] vs 2 of 33 [6.1%]; P=.027), pulmonary arterial hypertension (7 of 11 [63.6%] vs 2 of 33 [6.1%]; P=.027) than patients with isolated forms. Ten patients (22.7%) underwent correction of associated cardiac defects, leaving the anomalous pulmonary venous drainage intact. The median length of follow-up after diagnosis was 6.4 years (range, 0.2-27.5 years). Two patients died, both with associated cardiac defects and severe pulmonary arterial hypertension. Of 42 survivors, 39 (92.8%) were asymptomatic at the last follow-up visit; 3 patients still complained respiratory symptoms. There was no difference between isolated and associated forms of the disease. In most patients, scimitar syndrome presented as an isolated lesion with a benign outcome. Nonetheless, when associated with other cardiac defects and pulmonary arterial hypertension, there was an increased risk of congestive heart failure and mortality. Correction of associated cardiac defects (transforming "associated" into "isolated" forms), together with the therapeutic occlusion of anomalous arterial supply to the lung, led to a benign outcome comparable to that in primarily isolated forms. Copyright © 2013 Sociedad Española de Cardiología. Published by Elsevier Espana. All rights reserved.
Full Text Available Abstract The coexistence of systemic lupus erythematosus (SLE in patients with congenital human immunodeficiency virus (HIV infection is rare. This is a case report of a child diagnosed with SLE at nine years of age. She initially did well on non-steroidal anti-inflammatory agents, hydroxychloroquine, and steroids. She then discontinued her anti-lupus medications and was lost to follow-up. At 13 years of age, her lupus symptoms had resolved and she presented with intermittent fevers, cachexia, myalgias, arthralgias, and respiratory symptoms. Through subsequent investigations, the patient was ultimately diagnosed with congenitally acquired immunodeficiency syndrome (AIDS.
Miranda-Lora, América L; Vilchis-Gil, Jenny; Molina-Díaz, Mario; Flores-Huerta, Samuel; Klünder-Klünder, Miguel
To estimate the heritability, parental transmission and environmental contributions to the phenotypic variation in type 2 diabetes mellitus and metabolic syndrome-related traits in families of Mexican children and adolescents. We performed a cross-sectional study of 184 tri-generational pedigrees with a total of 1160 individuals (99 families with a type 2 diabetes mellitus proband before age 19). The family history of type 2 diabetes mellitus in three generations was obtained by interview. Demographic, anthropometric, biochemical and lifestyle information was corroborated in parents and offspring. We obtained correlations for metabolic traits between relative pairs, and variance component methods were used to determine the heritability and environmental components. The heritability of early-onset of type 2 diabetes mellitus was 0.50 (p2-h insulin, and cholesterol (p0.50) for blood pressure, HbA1c and HDL-cholesterol after multivariate adjustment (ptype 2 diabetes mellitus and insulin resistance, were significantly correlated only through the mother and others, such as hypertriglyceridemia, were significantly correlated only through the father. This study demonstrates that type 2 diabetes mellitus and metabolic syndrome-related traits are highly heritable among Mexican children and adolescents. Furthermore, several cardiometabolic factors have strong heritability and/or high environmental contributions that highlight the complex architecture of these alterations. Copyright © 2017 Elsevier B.V. All rights reserved.
PLAIASU, Vasilica; OCHIANA, Diana; MOTEI, Gabriela; ANCA, Ioana; GEORGESCU, Adrian
ABSTRACT Introduction: Patau syndrome (trisomy 13) is one of the most common chromosomal anomalies clinically characterized by the presence of numerous malformations with a limited survival rate for most cases. Babies are usually identified at birth and the diagnosis is confirmed with genetic testing. Materials and methods: In this review we outline the clinical and cytogenetic aspects of trisomy 13 and associated phenotypes for 5 cases analyzed in the last 3 years, referred to our Clinical Genetics Department. For each child cytogenetic analysis was performed to determine the genetic variant; also, the patients were investigated for other associated malformations (cardiac, cerebral, renal, ocular anomalies). Discussion: All 5 cases presented multiple malformations, including some but not all signs of the classical clinical triad suggestive of Patau syndrome. The cytogenetic investigation confirmed for each case the suspected diagnosis and also indicated the specific genetic variant, this being a valuable information for the genetic counselling of the families. Conclusion: The application of genetic analysis can increase diagnosis and prognosis accuracy and have an impact on clinical management. PMID:21977150
, assessments and networking of the Forum of Ethics Review Committees of Asia and the Pacific (FERCAP), a Non-Governmental Organisation. The work provides a critical reflection on the spread and uptake of ethics, contributing particularly to literatures in medical anthropology, organisational studies...... to participate in global research, the pre-requisite of ethical review has necessitated a growth in capacity building exercises. The chapters aim to elucidate ethnographically the activities and implications of 'capacity building' activities in biomedical research ethics, through following the trainings......, and development anthropology. Drawing on material from ethnographic fieldwork with the NGO in Sri Lanka, Thailand, the Philippines, Taiwan and mainland China over 12 months between March 2009 and November 2010, it advances an argument that the uptake of ethics through forms such as the Ethics Review Committee...
Some ethical questions about molecular biology and human radiation studies are raised. The questions relate to the following: genetic epidemiology leading to possible stigmatization of certain groups; protection of medical information, including samples, and respect for privacy; effect of genetic characterization on standards and procedures relating to occupational exposure; exclusion of vulnerable groups from research studies. On the positive side, there is increased funding within Canada for studies of ethical, legal and social issues, and internationally ethical standards are being developed
Iyalomhe, G B S
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.
In this book, a neuroradiologist and a neuropediatrician have combined forces to provide the widest possible knowledge in investigating cranial and cerebral disorders in infancy and childhood. Based on more than 20,000 pediatric CT examinations, with a follow-up time often exceeding ten years, the book aims to bridge interdisciplinary gaps and help radiologists, pediatricians and neurosurgeons solve the various problems of pediatric neuroradiology that frequently confront them. For each disease, the etiology, clinical manifestations, pathological lesions and radiological presentations are discussed, supported by extensive illustrations. Malformative, vascular, traumatic, tumoral, infectious and metabolic diseases are reviewed. Miscellaneous conditions presenting particular symptoms or syndromes are also studied, such as hydrocephalus and neurological complications of leukemia. The combined expertise and experience contained in this volume make it an outstanding reference work in the field of pediatric neuroradiology. (orig./MG)
Jacobs, Howard; Singhi, Samata; Gladstein, Jack
Comorbid conditions frequently occur in pediatric headaches and may significantly affect their management. Comorbidities that have been associated with pediatric headaches include attention-deficit or hyperactivity disorder, autism, developmental disabilities, depression, anxiety, epilepsy, obesity, infantile colic, atopic disorders, inflammatory bowel disease, and irritable bowel syndrome. The goal of this article is to review these comorbidities associated with pediatric headache, thereby empowering child neurologists to identify common triggers and tailor management strategies that address headache and its comorbidities. Copyright © 2016 Elsevier Inc. All rights reserved.
Savage, Martin O; Storr, Helen L
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.
Benz-Bohm, Gabriele; Richter, Ernst
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).
Hussain, Shaun A; Zhou, Raymond; Jacobson, Catherine; Weng, Julius; Cheng, Emily; Lay, Johnson; Hung, Phoebe; Lerner, Jason T; Sankar, Raman
There is a great need for safe and effective therapies for treatment of infantile spasms (IS) and Lennox-Gastaut syndrome (LGS). Based on anecdotal reports and limited experience in an open-label trial, cannabidiol (CBD) has received tremendous attention as a potential treatment for pediatric epilepsy, especially Dravet syndrome. However, there is scant evidence of specific utility for treatment of IS and LGS. We sought to document the experiences of children with IS and/or LGS who have been treated with CBD-enriched cannabis preparations. We conducted a brief online survey of parents who administered CBD-enriched cannabis preparations for the treatment of their children's epilepsy. We specifically recruited parents of children with IS and LGS and focused on perceived efficacy, dosage, and tolerability. Survey respondents included 117 parents of children with epilepsy (including 53 with IS or LGS) who had administered CBD products to their children. Perceived efficacy and tolerability were similar across etiologic subgroups. Eighty-five percent of all parents reported a reduction in seizure frequency, and 14% reported complete seizure freedom. Epilepsy was characterized as highly refractory with median latency from epilepsy onset to CBD initiation of five years, during which the patient's seizures failed to improve after a median of eight antiseizure medication trials. The median duration and the median dosage of CBD exposure were 6.8 months and 4.3mg/kg/day, respectively. Reported side effects were far less common during CBD exposure, with the exception of increased appetite (30%). A high proportion of respondents reported improvement in sleep (53%), alertness (71%), and mood (63%) during CBD therapy. Although this study suggests a potential role for CBD in the treatment of refractory childhood epilepsy including IS and LGS, it does not represent compelling evidence of efficacy or safety. From a methodological standpoint, this study is extraordinarily vulnerable
Full Text Available Pediatric dysrhythmias are rare but important causes of admission to pediatric clinics and emergency departments. Due to the development of successful surgical treatment of congenital heart diseases and improvements in the diagnostic tools, pediatric dysrhythmias are more frequently diagnosed. Although pediatric dysrhythmias are may be asymptomatic, they may manifest with weakness, dizziness, decrease in the effort capacity, easy fatigability, irregularity in heartbeats, palpitations, syncope and cardiac arrest. Since dysrhythmias may give rise to significant hemodynamic outcomes, their recognition by pediatricians and family physicians is vital for the patients. This review aims to contribute to the correct diagnosis and management of the cases with frequently encountered pediatric dysrhythmias.
Carlo, Waldemar F; Cnota, James F; Dabal, Robert J; Anderson, Jeffrey B
The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) was established in 2008 to improve outcomes of hypoplastic left heart syndrome (HLHS) during the interstage period. They evaluated changes in patient variables and practice variation between early and late eras. Data including demographic, operative, discharge, and follow-up variables from the first 100 patients (6/2008-1/2010) representing 18 centers were compared with the most recent 100 patients (1/2014-11/2014) from these same centers. Prenatal diagnosis increased from 69% to 82% (P = .05). There were no differences in gestational age or weight at Norwood. A composite of any preoperative risk factor occurred more frequently in the early era (59% vs. 34%, P < .01). While mean age at Norwood was similar (8.3 vs. 6.6 days, P = .2), the standard deviation was significantly lower in the recent era (10.4-6.4 days, P = .04). Use of RV-PA conduit increased (67%-84%, P < .01). Rates of complete discharge communication with both the primary care physician (31%-97%, P < .01) and primary cardiologist (44%-97%, P < .01) increased substantially. There were limited changes in feeding strategies. Use of home monitoring program increased (76%-99%, P < .01) with all participants in the late era monitoring both oxygen saturation and weight. Among NPC-QIC centers contributing patients to both eras, there were significant changes in preoperative risk factors, surgical strategy, discharge communication, and interstage care. Further study is required to determine an association between these changes and decreased mortality. © 2017 Wiley Periodicals, Inc.
Dooly, Melinda; Moore, Emilee; Vallejo, Claudia
Qualitative research, especially studies in educational contexts, often brings up questions of ethics because the study design involves human subjects, some of whom are under age (e.g. data collected in primary education classrooms). It is not always easy for young researchers to anticipate where ethical issues might emerge while designing their…
The Handbook of Economics and Ethics portrays an understanding of economic methodology in which facts and values, though distinct, are closely interconnected in a variety of ways. From theory building to data collection, and from modelling to policy evaluation, this encyclopaedic Handbook is at the intersection of economics and ethics.
Chambers, David W
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.
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.
Research ethics has become integrated into what it means to conduct good science. This thesis is about the nature of that integration, which I argue is not neutral, carrying with it ideas of duty, moral obligations, organisational mechanisms, and processes of monitoring. For developing countries...... to participate in global research, the pre-requisite of ethical review has necessitated a growth in capacity building exercises. The chapters aim to elucidate ethnographically the activities and implications of 'capacity building' activities in biomedical research ethics, through following the trainings......, assessments and networking of the Forum of Ethics Review Committees of Asia and the Pacific (FERCAP), a Non-Governmental Organisation. The work provides a critical reflection on the spread and uptake of ethics, contributing particularly to literatures in medical anthropology, organisational studies...
Rønn, Kira Vrist
.e., the search for an ideal ethical framework for intelligence actions). The article concludes that there are many holes to fill for future studies on intelligence ethics both in external and internal discussions. Thus, the article is an invitation – especially, to moral philosophers and political theorists......Questions concerning what constitutes a morally justified conduct of intelligence activities have received increased attention in recent decades. However, intelligence ethics is not yet homogeneous or embedded as a solid research field. The aim of this article is to sketch the state of the art...... of intelligence ethics and point out subjects for further scrutiny in future research. The review clusters the literature on intelligence ethics into two groups: respectively, contributions on external topics (i.e., the accountability of and the public trust in intelligence agencies) and internal topics (i...
Sheldon, Claire A; Kwon, Young Joon; Liu, Grant T; McCormack, Shana E
Pseudotumor cerebri syndrome (PTCS) is defined by the presence of elevated intracranial pressure (ICP) in the setting of normal brain parenchyma and cerebrospinal fluid (CSF). Headache, vision changes, and papilledema are common presenting features. Up to 10% of appropriately treated patients may experience permanent visual loss. The mechanism(s) underlying PTCS is unknown. PTCS occurs in association with a variety of conditions, including kidney disease, obesity, and adrenal insufficiency, suggesting endocrine and/or metabolic derangements may occur. Recent studies suggest that fluid and electrolyte balance in renal epithelia is regulated by a complex interaction of metabolic and hormonal factors; these cells share many of the same features as the choroid plexus cells in the central nervous system (CNS) responsible for regulation of CSF dynamics. Thus, we posit that similar factors may influence CSF dynamics in both types of fluid-sensitive tissues. Specifically, we hypothesize that, in patients with PTCS, mitochondrial metabolites (glutamate, succinate) and steroid hormones (cortisol, aldosterone) regulate CSF production and/or absorption. In this integrated mechanism review, we consider the clinical and molecular evidence for each metabolite and hormone in turn. We illustrate how related intracellular signaling cascades may converge in the choroid plexus, drawing on evidence from functionally similar tissues.
Bembea, Melania M; Jouvet, Philippe; Willson, Douglas; Thomas, Neal J
This article describes the methodology used for the Pediatric Acute Lung Injury Consensus Conference. Consensus conference of international experts in pediatric acute respiratory distress syndrome using the Research ANd Development/University of California, Los Angeles appropriateness method and an expert recommendations process developed by the French-speaking intensive care society. Topics related to pediatric acute respiratory distress syndrome were divided into nine subgroups with a review of the literature. A group of 27 experts met three times over the course of 2 years and collaborated in their respective subgroups to define pediatric acute respiratory distress syndrome and to make recommendations regarding treatment and future research priorities. The consensus conference resulted in summary of recommendations published in Pediatric Critical Care Medicine, the present Pediatric Acute Lung Injury Consensus Conference methodology article, articles on the nine pediatric acute respiratory distress syndrome subtopics, and a review of pediatric acute respiratory distress syndrome pathophysiology published in this supplement of Pediatric Critical Care Medicine. The methodology described involved experts from around the world and the use of modern information technology. This resulted in recommendations for pediatric acute respiratory distress syndrome management, the identification of current research gaps, and future priorities.
Diamant, Ulla-Britt; Jensen, Steen M; Winbo, Annika; Stattin, Eva-Lena; Rydberg, Annika
Measurements of the Q-T interval are less reliable in children than in adults. Identification of superior diagnostic tools is warranted. This study aimed to investigate whether a vectorcardiogram (VCG) recorded from three orthogonal leads (X, Y, Z) according to Frank is superior to a 12-lead electrocardiogram (ECG) in providing a correct long Q-T syndrome (LQTS) diagnosis in children. This LQTS group consisted of 35 genetically confirmed carriers of mutations in the KCNQ1 (n = 29) and KCNH2 (n = 6) genes. The control group consisted of 35 age- and gender-matched healthy children. The mean age was 7 years in the LQTS group and 6.7 years in the control group (range, 0.5-16 years). The corrected Q-T interval (QT(c)) was measured manually (QT(man)) by one author (A.W.). The 12-lead ECG automatic measurements (QT(ECG)) and interpretation (QT(Interpret)) of QT(c) were performed with the Mac5000 (GE Medical System), and the VCG automatic measurements (QT(VCG)) were performed with the Mida1000, CoroNet (Ortivus AB, Sweden). By either method, a QT(c) longer than 440 ms was considered prolonged and indicative of LQTS. Of the 35 children with genetically confirmed LQTS, 30 (86 %) received a correct diagnosis using QT(VCG), 29 (82 %) using QT(man), 24 (69 %) using QT(ECG), and 17 (49 %) using QT(Interpret). Specificity was 0.80 for QT(VCG), 0.83 for QT(man), 0.77 for QT(ECG), and 0.83 for QT(Interpret). The VCG automatic measurement of QT(c) seems to be a better predictor of LQTS than automatic measurement and interpretation of 12-lead ECG.
Jung, Jinsei; Park, Sera; Kim, Sung Huhn; Moon, In Seok; Hwang, Kyu Rin; Lee, Jeon Mi; Bang, Mi Young; Choi, Jae Young
Cornelia de Lange syndrome (CdLS) is a multiple developmental disorder including hearing loss. The hearing impairment in CdLS patients is not only sensorineural but also conductive hearing loss (CHL). The aim of this study was to elucidate hearing loss causes in CdLS patients and evaluate the effect of ventilation tube (v-tube) insertion in the cases of CHL. Thirty-two patients clinically diagnosed with CdLS were enrolled and analyzed with retrospective case review. Audiologic evaluations and imaging studies such as a temporal bone computed tomogram or brain magnetic resonance imaging (MRI) were performed for all patients. Hearing rehabilitation such as ventilation tube insertion, hearing aid fitting, or cochlear implantation was chosen depending on the audiological condition. Among 32 CdLS patients who underwent auditory brainstem response test, 81.2% presented hearing loss. Imaging studies showed that only middle ear lesions without inner ear anomalies were identified in 56.3%. Notably, the soft tissue lesion in middle ear was identified even in the neonatal MRI. When 7 patients were thought to have CHL due to otitis media with effusion, v-tube insertion was applied first. However, v-tube insertion rarely improved CHL postoperatively. Moreover, middle ear lesion was not fluid effusion but soft tissue lesion according to the intraoperative finding. These lesions were not eradicated even after revision surgery of v-tube insertion. V-tube insertion is not effective to improve hearing or eradicate otitis media with effusion in CdLS patients. Copyright © 2016 Elsevier Inc. All rights reserved.
Full Text Available Background: This study aimed to determine the short- and long-term effects of consumption of grape and pomegranate juices on markers of endothelial function and inflammation in adolescents with metabolic syndrome (MetS. Methods: In a non-pharmacologic randomized controlled trial, 30 individuals were randomly assigned to two groups of drinking natural grape or pomegranate juice for 1 month. Measurements of inflammatory factors [Hs-CRP, sE-selectin, sICAM-1, sVCAM, and interleukin 6 (IL-6] and flow-mediated dilation (FMD were made at baseline, 4 hours after first juice consumption and after one month of juice consumption. Results: The percent changes of FMD were significant in both groups in the short- and long-term. Hs-CRP had a nonsignificant decrease. sE selectin had a significant decrease after 4 hours in total and in the pomegranate juice group, followed by a significant decrease after 1 month in both groups. After 4 hours, sICAM-1 significantly decreased in the pomegranate juice group, and after 1 month it decreased in total and pomegranate juice group. Interleulkin-6 (IL-6 had a significant constant decrease at 4-hour and 1-month measurements after drinking pomegranate juice, and in both groups after 1 month. Significant negative correlations of changes in sICAM-1 and sE-selectin with changes in FMD were found in both periods of follow-up; and at 1 month for IL-6. Conclusions: Decline in inflammation was associated with improvement in FMD without changes in conventional risk factors. Daily consumption of natural antioxidants may improve endothelial function in adolescents with MetS.
Faure, Christophe; Patey, Natalie; Gauthier, Cindy; Brooks, Elice M; Mawe, Gary M
In adults, irritable bowel syndrome (IBS) and functional dyspepsia (FD) are chronic conditions that often start during childhood. We investigated mucosal serotonin (5-HT) signaling in children with the idea that data from subjects with a shorter history may improve our understanding of underlying pathophysiological mechanisms. Ninety-eight children undergoing gastroscopy or colonoscopy were studied prospectively. Biopsy specimens were evaluated for inflammation, enterochromaffin cell numbers, 5-HT content, and messenger RNA (mRNA) levels for the synthetic enzyme, tryptophan hydroxylase 1, and the serotonin transporter (SERT) were assessed by quantitative real-time reverse-transcription polymerase chain reaction. Data from 12 children with IBS and 17 with FD were compared with age-matched controls (12 with rectal biopsies and 12 with gastric biopsies) and with subjects with organic disorders. In patients with FD, a small number of immune cells were observed in the gastric mucosa in half of the patients, but no abnormalities with respect to the 5-HT pathway were identified. In patients with IBS, no differences were detected between patients and controls regarding intraepithelial lymphocytes and CD3+ cells in the lamina propria although all patients showed at least a slight inflammatory infiltrate. In the IBS samples, higher 5-HT content (P < .01) and lower SERT mRNA (P < .05) were detected as compared with controls. Severe inflammation in the colonic mucosa had a high impact on 5-HT signaling with a significant decrease in enterochromaffin cells (P < .01) and 5-HT content (P < .01) and a high SERT mRNA expression (P < .01). These results confirm the role of 5-HT signaling in IBS in children and argue against such a role in FD. Copyright 2010 AGA Institute. Published by Elsevier Inc. All rights reserved.
George N. García Rodríguez
descriptive study was conducted among all the patients with Ehlers Danlos syndrome that were seen at the Orthopedics and Traumatology department of "José Martí" Teaching Pediatric Hospital (Sancti Spiritus, Cuba from July 2001 to July 2006. A minimum follow-up time of 6 months was considered for the validation of the results. RESULTS. 41 patients affected with 72 diseases of orthopedic origin were studied. The frequency was approximately 1.7 diseases per patient, with a non significant predominance of females (n = 24. One of the most important perinatal antecedents was the presence of diverse hip dysplasia degrees. The presence of other non-orthopedic affections was not remarkable. The main orthopedic findings were the flexible flat foot (37, the genus recurvatum (11 and kyphoscoliosis (9. Aesthetic surgery and the corrective orthopedic surgery were the most used. CONCLUSIONS. The categorization of this disease and its timely treatment is an efficient method of control that would help to prevent the articular degeneration that generally precedes osteoarthritis.
Nelu BURCEA; Ion CROITORU
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 eleme...
Dye, Thomas J; Jain, Sejal V; Simakajornboon, Narong
Restless legs syndrome (RLS) and periodic limb movement disorder (PLMD) are thought to center around a genetically mediated sensitivity to iron insufficiency. Previous studies have shown the effectiveness of short-term iron therapy in children with low iron storage. Little is known, however, about long-term iron treatment in children with RLS and PLMD. Therefore, we performed this study to assess the long-term effect of iron therapy in children with RLS and PLMD. A retrospective chart review was performed for children who met the following criteria: A) diagnosed as having either RLS or PLMD, B) started on iron supplementation, C) followed up for >2 years in a sleep clinic. Baseline values for iron, ferritin, and periodic limb movement of sleep index (PLMS index) were defined in the three months leading up to the initiation of iron therapy. Values were also computed for follow-up periods of 3-6 months, 1-2 years, and >2 years. Serum iron and ferritin levels and PLMS index were compared between baseline and all subsequent follow-ups. In total, 105 patients met inclusion criteria, of whom 64 were diagnosed with PLMD alone, seven with RLS alone, and 35 with both RLS and PLMD. The average age was 10.2 ± 5.3 years. Compared to the baseline (27.4 ± 12.1 ng/ml), the average ferritin values at 3-6 months (45.62 ± 21.2 ng/ml, p 2 years (54.7 ± 40.5 ng/ml, p 2 years (10 ± 14.5/h, p 2 years after iron therapy initiation in our RLS/PLMD cohort with a long-term follow-up. Iron therapy appears to lead to long-lasting improvements in children with RLS/PLMD. Copyright © 2016 Elsevier B.V. All rights reserved.
Andrés, A M; Miguel, M; De la Torre, C; Barrena, S; Ramírez, M; Hernández, F; Martínez, L; Leal, N; Ramos, E; Prieto, G; López Santamaría, M; Tovar, J A
Chronic Intestinal Pseudo Obstruction (CIPO) and Berdon Syndrome (BS) are motility disorders with still unclear pathophysiology, and challenging diagnosis and management. Patient and methods. 26 patients (8M/18F) treated of CIPO (21) or BS (5) at our institution between 1982-2009 were retrospectively reviewed and clinical, diagnostic, therapeutic and follow-up data were analyzed. 77% had a neonatal onset by the 3rd month of life (5 had a prenatal diagnosis of megacystis). Abdominal distension (87%), recurrent suboclusive episodes (70%) and malnourishment (60%) were the main symptoms followed by vomits, chronic diarrhea, constipation and dysfagia. The urinary tract was involved in 12 patients (46%): 8 had megacystis, 8 had vesicoureteral reflux. Arrythmias were seen in 2, deafness in 1, hydrocephalia in 1 and malrotation in 5. Radiological studies were consistent for CIPO in all of them: the disease was limited to the esophagus in 1, 3 had segmentary involvement of the small bowel, 6 had microcolon, and the rest had all the digestive tract involved. Anorectal manometry ruled out aganglionosis in 12, esophageal manometry showed aperistalsis in 9 and antro-duodenal manometry confirmed the diagnosis in 9 (visceral myopathy in 4 y neuropathy in 5). Rectal biopsies (16) and muscular biopsies (5) were normal in all of them. Full thickness biopsies (in 18, after surgery) showed myopathy in 12 and neuropathy in 6. Prokinetics and antibiotics for bacterial overgrowth were employed in 100%, 17 required long-time parenteral nutrition (PN), 21 required surgery and 7 were transplanted (4 isolated small bowel, 3 multivisceral). Symptoms improved in 9/15 with an ileostomy. 19 weaned from PN. After a median follow-up of 7.9 years (range 5m-17a), 3 were lost, 17/23 patients are alive and only 2 on home PN. Six died, 3 after being transplanted. CIPO and/or BS have a wide clinical spectrum and a complex diagnosis; however, the knowledge of the disease and an appropriate treatment
... gov/pubmed/26718656 . Ruderman NB, Shulman GI. Metabolic syndrome. In: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Adult and Pediatric . 7th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 43. Review ... NIH MedlinePlus Magazine Read more Health ...
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.
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
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 using a CD with that of individual HT (iHT) performed by qualified therapists. This noninferiority randomized clinical trial with a follow-up of 1 year after the end of treatment was conducted from July 15, 2011, through June 24, 2013, at 9 secondary and tertiary care centers throughout the Netherlands. A total of 303 children were eligible to participate. Of those, 260 children (aged 8-18 years) with IBS or FAP(S) were included in this study. Children were randomized (1:1 ratio) to home-based HT with a CD (CD group) or iHT performed by qualified therapists (iHT group). No children withdrew from the study because of adverse effects. The CD group was instructed to perform exercises 5 times per week or more for 3 months. The iHT group consisted of 6 sessions during 3 months. Primary outcomes were treatment success directly after treatment and after 1-year follow-up. Treatment success was defined as a 50% or greater reduction in pain frequency and intensity scores. The noninferiority limit was set at 50% treatment success in the CD group, with a maximum of 25% difference in treatment success with the iHT group after 1-year follow-up. Modified intention-to-treat analyses were performed. A total of 132 children were assigned to the CD group and 128 to the iHT group; 250 children were analyzed (126 in the CD group and 124 in the iHT group) (mean [SD] age, 13.4 [2.9] years in the CD group and 13.3 [2.8] years in the iHT group; 94 female [74.6%] in the CD group and 85 [68.5%] in the iHT group). Directly after treatment, 46 children (36.8%) in the CD group and 62 (50.1%) in the iHT group were successfully treated. After 1-year follow-up, the 62.1% treatment success in the CD group
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.”
... recommendation of the Pediatric Ethics Subcommittee from its meeting on May 11, 2011, regarding the Institutional Review Board process for clinical investigations that involve both an FDA regulated product and research... Committee will meet to discuss pediatric-focused safety reviews, as mandated by the Best Pharmaceuticals for...
Olivares Bøgeskov, Benjamin Miguel
This paper presents the result from our research on how nurse managers use and occasionally misuse inconclusive ethical arguments to engage their personnel in current reforms. The Danish health care system has undergone a series of reforms inspired by New Public Management theories, which have......, paying special attention to the way in which ethical arguments are used in relation to engagement. Our research shows that ethical arguments are extremely common, and they are used either to elicit engagement, or to demand engagement considering the result of a duty. However, most interestingly...... it was possible for us to find recurrence of fallacious arguments of different kinds. Based on these findings, I will argue that the use of fallacious arguments in order to generate engagement is in reality an abusive use of ethics, which raises important questions. I argue that depending on the degree...
Altınörs, Nur; Haberal, Mehmet
The aim of this study was to review and discuss the great variety of ethical issues related to organ donation, organ procurement, transplant activities, and new ethical problems created as a result of technologic and scientific developments. An extensive literature survey was made, and expert opinions were obtained. The gap between demand and supply of organs for transplant has yielded to organ trafficking, organ tourism, and commercialism. This problem seems to be the most important issue, and naturally there are ethical dilemmas related to it. A wide number of ideas have been expressed on the subject, and different solutions have been proposed. The struggle against organ trafficking and commercialism should include legislation, efforts to increase deceased-donor donations, and international cooperation. China's policy to procure organs from prisoners sentenced to death is unethical, and the international community should exert more pressure on the Chinese government to cease this practice. Each particular ethical dilemma should be taken separately and managed.
What are the characteristics of an ethical dilemma? And how do we handle them in the area of early childhood education? These are some of the questions that will be dealt with in this chapter.......What are the characteristics of an ethical dilemma? And how do we handle them in the area of early childhood education? These are some of the questions that will be dealt with in this chapter....
BLACKORBY, Charles; BOSSERT, Walter; DONALDSON, David
This paper reviews the welfarist approach to population ethics. We provide an overview of the critical-level utilitarian population principles and their generalized counterparts, examine important properties of these principles and discuss their relationships to other variable-population social-evaluation rules. We illustrate the difficulties arising in population ethics by means of an impossibility result and present characterizations of the critical-level generalized-utilitarian principles ...
Case Presentation. Grand Rounds in Pediatric Nephrology. Elisabeth M. Hodson. Centre for Kidney Research, The Children's Hospital at Westmead, Locked Bag 4001, Westmead NSW 2145, Australia .... nephrotic syndrome (SSNS) with minimal change disease ... and reduced renal function while severe SLE nephropathy.
Frankovich, Jennifer; Thienemann, Margo; Pearlstein, Jennifer; Crable, Amber; Brown, Kayla; Chang, Kiki
Abrupt, dramatic onset obsessive-compulsive disorder (OCD) and/or eating restriction with at least two coinciding symptoms (anxiety, mood dysregulation, irritability/aggression/oppositionality, behavioral regression, cognitive deterioration, sensory or motor abnormalities, or somatic symptoms) defines pediatric acute-onset neuropsychiatric syndrome (PANS). Descriptions of clinical data in such youth are limited. We reviewed charts of 53 consecutive patients evaluated in our PANS Clinic; 47 met PANS symptom criteria but not all met the requirement for "acute onset." Patients meeting full criteria for PANS were compared with patients who had a subacute/insidious onset of symptoms. Nineteen of 47 (40%) patients in the study had acute onset of symptoms. In these patients, autoimmune/inflammatory diseases and psychiatric disorders were common in first-degree family members (71% and 78%, respectively). Most acute-onset patients had a relapsing/remitting course (84%), prominent sleep disturbances (84%), urinary issues (58%), sensory amplification (66%), gastrointestinal symptoms (42%), and generalized pain (68%). Inflammatory back pain (21%) and other arthritis conditions (28%) were also common. Suicidal and homicidal thoughts and gestures were common (44% and 17%, respectively) as were violent outbursts (61%). Group A streptococcus (GAS) was the most commonly identified infection at onset (21%) and during flares (74%). Rates of the above-mentioned characteristics did not differ between the acute-onset group and the subacute/insidious-onset groups. Low levels of immunoglobulins were more common in the subacute/insidious-onset group (75%) compared with the acute-onset group (22%), but this was not statistically significant (p=0.06). In our PANS clinic, 40% of patients had acute onset of symptoms. However, those with and without acute onset of symptoms had similar symptom presentation, rates of inflammatory conditions, somatic symptoms, and violent thoughts and behaviors
Wu, Jiali; Gu, Meizhen; Chen, Shumei; Chen, Wei; Ni, Kun; Xu, Hongming; Li, Xiaoyan
This study aimed to retrospectively investigate the factors related to pediatric obstructive sleep apnea-hypopnea syndrome (OSAHS) with attention deficit hyperactivity disorder (ADHD) in children younger than 6 years and those older than 6 years.A total of 437 children who were hospitalized due to OSAHS between January 2014 and December 2014 were retrospectively reviewed. The children were further divided into OSAHS group and OSAHS + ADHD group. The general characteristics, OSA-18 quality of life, intention-hyperactivity score, and polysomnographic parameters (apnea-hypopnea index and the lowest oxygen saturation) were collected and compared between groups.There were 298 boys and 139 girls with the male to female ratio of 2.14:1. ADHD was found in 146 children including 105 boys and 41 girls with the male to female ratio of 2.56:1. Of these children, 31.62% and 35.46% had concomitant ADHD in children aged 4 to 5 years and those aged 6 to 11 years, respectively. In children aged 4 to 5 years, the incidence of allergic rhinitis was significantly higher (P = .016) and the adenoid hypertrophy was more severe (P = .001) in those with concomitant ADHD. In children aged 6 to 11 years, the tonsil hypertrophy was more severe in those with concomitant ADHD (P = .019). In children with concomitant ADHD, OSA-18 score was higher than in those with OSAHS alone (P ADHD than in those with OSAHS alone.As high as 30% of OSAHS children have concomitant ADHD, and the incidence of ADHD in OSAHS children is increasing over age. Boys are more likely to develop OSAHS and incidence of ADHD in OSAHS boys is higher than in OSAHS girls. In addition, risk factors of ADHD also vary between age groups. The ADHD is related to the severity of allergic rhinitis and adenoid hypertrophy in children aged 4 to 5 years, and to the severity of tonsil hypertrophy in children aged 6 to 11 years. Hypoxia may be an important factor causing ADHD. OSAHS should be treated as early as possible to
Giselle Serrano Ricardo
Full Text Available El síndrome de Marfán es una enfermedad hereditaria del tejido conectivo, que se describe en niños y en adultos, causada por una mutación en el gen que codifica la glicoproteína fibrilina tipo 1. Afecta múltiples órganos y sistemas, fundamentalmente cardiovascular, esquelético, oftalmológico, piel y tegumentos. Se presenta una revisión de los aspectos más actuales del diagnóstico, y la atención multidisciplinaria para lograr una reducción de la morbilidad y mortalidad en los pacientes pediátricos. Se concluye que el uso precoz de betabloqueadores e inhibidores del receptor AT-1 de la angiotensina II (losartán, constituyen actualmente los pilares fundamentales de la terapéutica farmacológica, pues disminuyen la frecuencia de complicaciones cardiovasculares, las cuales determinan el pronóstico de la enfermedad. La cirugía programada de la raíz aórtica, especialmente con preservación valvular, permite mejorar la expectativa de vida al evitar la alta mortalidad de los eventos agudos. Alternativas prometedoras son los procederes híbridos y el intervencionismo endovascular.Marfan syndrome is a hereditary disease of the connective tissue caused by mutation of type 1 fibrillin glycoprotein-coding gene in children and adults. This disease affects organs and systems, mainly cardiovascular, skeletal, ophthalmologic systems, skin and teguments. The review of the most current aspects of diagnosis, and the multidisciplinary care to reduce morbidity and mortality of pediatric patients were presented. It was concluded that the early use of betablockers and angiotensin II AT-1 receptor blocker (losarfan are the fundamental pillars of drug therapy, since they reduce the frequency of cardiovascular complications that determine the disease prognosis. The scheduled surgery of the aortic root, particularly valve preservation, allows improving the life expectancies because it prevents high mortality from acute events. Hybrid procedures and
Deborah Oughton started with a view of the work in progress by the ICRP TG 94 on ethics, from the historical context and the principles-based ethics in RP, to continue with an overview of the ethical theories and with the main area of elaboration which concerns the common values, to conclude with considerations about the implementation in different area such as biomedicine, nuclear safety and workers, ecological aspects, and environmental health and society. By reading again the ICRP and IAEA publications on the ethical aspects in the protection of environment from the effects of ionizing radiation, the presentation covers the various and different cultures within the history of environmental ethics, the perception of Nature and the theories of environmental ethics, in particular by focusing on anthropocentrism, biocentrism and ecocentrism, as philosophical worldwide views, and on conservation, biodiversity, sustainability, environmental justice and human dignity, as primary principles of environmental protection. The influence of western Christianity, with a view of man dominating over every creeping thing on earth, and of the non-western ideas, the human perception of Nature has been analyzed and discussed to conclude that, in reality then, the anthropocentrism, biocentrism and ecocentrism, as reflected in many cultures and religions, they all support the need to protect the environment and to recognise and preserve the diversity. Three challenges were then discussed in the presentation: the ecosystem approach and ecological economics, for example in the case of Fukushima by asking what is the economic cost of marine contamination; the ecosystem changes with attention to what harms, as in the case of the environment in the contaminated areas around Chernobyl; and the environmental consequences of remediation, which can be considered a source of controversy for environmental ethics and policy
Olsen Leif A
Full Text Available Abstract Background Time and communication are important aspects of the medical consultation. Physician behavior in real-life pediatric consultations in relation to ethical practice, such as informed consent (provision of information, understanding, respect for integrity and patient autonomy (decision-making, has not been subjected to thorough empirical investigation. Such investigations are important tools in developing sound ethical praxis. Methods 21 consultations for inguinal hernia were video recorded and observers independently assessed global impressions of provision of information, understanding, respect for integrity, and participation in decision making. The consultations were analyzed for the occurrence of specific physician verbal and nonverbal behaviors and length of time in minutes. Results All of the consultations took less than 20 minutes, the majority consisting of 10 minutes or less. Despite this narrow time frame, we found strong and consistent association between increasing time and higher ratings on all components of ethical practice: information, (β = .43, understanding (β = .52, respect for integrity (β = .60, and decision making (β = .43. Positive nonverbal behaviors by physicians during the consultation were associated particularly with respect for integrity (β =.36. Positive behaviors by physicians during the physical examination were related to respect for children's integrity. Conclusion Time was of essence for the ethical encounter. Further, verbal and nonverbal positive behaviors by the physicians also contributed to higher ratings of ethical aspects. These results can help to improve quality of ethical practice in pediatric settings and are of relevance for teaching and policy makers.
Vries, Martine Charlotte de
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
Full Text Available Chronic pain is a growing problem in children, with prevalence as high as 30.8%. Acupuncture has been found to be useful in many chronic pain conditions, and may be of clinical value in a multidisciplinary treatment program. The basic principles of acupuncture are reviewed, as well as studies exploring basic mechanisms of acupuncture and clinical efficacy. Conditions commonly treated in the pediatric pain clinic, including headache, abdominal pain, fibromyalgia, juvenile arthritis, complex regional pain syndrome, cancer pain, as well as perioperative pain studies are reviewed and discussed. Areas in need of further research are identified, and procedural aspects of acupuncture practice and safety studies are reviewed. Acupuncture can be an effective adjuvant in the care of pediatric patients with painful conditions, both in a chronic and an acute setting. Further studies, including randomized controlled trials, as well as trials of comparative effectiveness are needed.
Golianu, Brenda; Yeh, Ann Ming; Brooks, Meredith
Chronic pain is a growing problem in children, with prevalence as high as 30.8%. Acupuncture has been found to be useful in many chronic pain conditions, and may be of clinical value in a multidisciplinary treatment program. The basic principles of acupuncture are reviewed, as well as studies exploring basic mechanisms of acupuncture and clinical efficacy. Conditions commonly treated in the pediatric pain clinic, including headache, abdominal pain, fibromyalgia, juvenile arthritis, complex regional pain syndrome, cancer pain, as well as perioperative pain studies are reviewed and discussed. Areas in need of further research are identified, and procedural aspects of acupuncture practice and safety studies are reviewed. Acupuncture can be an effective adjuvant in the care of pediatric patients with painful conditions, both in a chronic and an acute setting. Further studies, including randomized controlled trials, as well as trials of comparative effectiveness are needed. PMID:27417472
Olivares Bøgeskov, Benjamin Miguel
This paper presents the result from our research on how nurse managers use and occasionally misuse inconclusive ethical arguments to engage their personnel in current reforms. The Danish health care system has undergone a series of reforms inspired by New Public Management theories, which have...... promised better services for lower costs. Despite the positive intention, such reforms have not always been received as such by front line nurses, who often see an opposition between these processes with their focus on efficiency and the fundamental values of nursing. In this climate, nurse managers......, paying special attention to the way in which ethical arguments are used in relation to engagement. Our research shows that ethical arguments are extremely common, and they are used either to elicit engagement, or to demand engagement considering the result of a duty. However, most interestingly...
Hasselbalch, Gry; Tranberg, Pernille
Respect for privacy and the right to control one’s own data are becoming key parameters to gain a competitive edge in today’s business world. Companies, organisations and authorities which view data ethics as a social responsibility, giving it the same importance as environmental awareness...... concerned about a lack of control over their data, but they also have begun to act. In addition, they describe alternative business models, advances in technology and a new European data protection regulation, all of which combine to foster a growing market for data-ethical products and services...... and respect for human rights, are tomorrow’s winners. Digital trust is paramount to digital growth and prosperity. This book combines broad trend analyses with case studies to examine companies which use data ethics to varying degrees. The authors make the case that citizens and consumers are no longer just...
Hasselbalch, Gry; Tranberg, Pernille
Respect for privacy and the right to control one’s own data are becoming key parameters to gain a competitive edge in today’s business world. Companies, organisations and authorities which view data ethics as a social responsibility, giving it the same importance as environmental awareness...... and respect for human rights, are tomorrow’s winners. Digital trust is paramount to digital growth and prosperity. This book combines broad trend analyses with case studies to examine companies which use data ethics to varying degrees. The authors make the case that citizens and consumers are no longer just...... concerned about a lack of control over their data, but they also have begun to act. In addition, they describe alternative business models, advances in technology and a new European data protection regulation, all of which combine to foster a growing market for data-ethical products and services...
Full Text Available Abstract The WHO defines pediatric palliative care as the active total care of the child's body, mind and spirit, which also involves giving support to the family. Its purpose is to improve the quality of life of young patients and their families, and in the vast majority of cases the home is the best place to provide such care, but for cultural, affective, educational and organizational reasons, pediatric patients rarely benefit from such an approach. In daily practice, it is clear that pediatric patients experience all the clinical, psychological, ethical and spiritual problems that severe, irreversible disease and death entail. The international literature indicates a prevalence of incurable disease annually affecting 10/10,000 young people from 0 to 19 years old, with an annual mortality rate of 1/10,000 young people from birth to 17 years old. The needs of this category of patients, recorded in investigations conducted in various parts of the world, reveal much the same picture despite geographical, cultural, organizational and social differences, particularly as concerns their wish to be treated at home and the demand for better communications between the professionals involved in their care and a greater availability of support services. Different patient care models have been tested in Italy and abroad, two of institutional type (with children staying in hospitals for treating acute disease or in pediatric hospices and two based at home (the so-called home-based hospitalization and integrated home-based care programs. Professional expertise, training, research and organization provide the essential foundations for coping with a situation that is all too often underestimated and neglected.
Weiss, Matthew J; Hornby, Laura; Witteman, William; Shemie, Sam D
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
The Italian Society for Pediatric Nephrology (SINePe) consensus document on the management of nephrotic syndrome in children: Part I - Diagnosis and treatment of the first episode and the first relapse.
Pasini, Andrea; Benetti, Elisa; Conti, Giovanni; Ghio, Luciana; Lepore, Marta; Massella, Laura; Molino, Daniela; Peruzzi, Licia; Emma, Francesco; Fede, Carmelo; Trivelli, Antonella; Maringhini, Silvio; Materassi, Marco; Messina, Giovanni; Montini, Giovanni; Murer, Luisa; Pecoraro, Carmine; Pennesi, Marco
This consensus document is aimed at providing an updated, multidisciplinary overview on the diagnosis and treatment of pediatric nephrotic syndrome (NS) at first presentation. It is the first consensus document of its kind to be produced by all the pediatric nephrology centres in Italy, in line with what is already present in other countries such as France, Germany and the USA. It is based on the current knowledge surrounding the symptomatic and steroid treatment of NS, with a view to providing the basis for a separate consensus document on the treatment of relapses. NS is one of the most common pediatric glomerular diseases, with an incidence of around 2-7 cases per 100000 children per year. Corticosteroids are the mainstay of treatment, but the optimal therapeutic regimen for managing childhood idiopathic NS is still under debate. In Italy, shared treatment guidelines were lacking and, consequently, the choice of steroid regimen was based on the clinical expertise of each individual unit. On the basis of the 2015 Cochrane systematic review, KDIGO Guidelines and more recent data from the literature, this working group, with the contribution of all the pediatric nephrology centres in Italy and on the behalf of the Italian Society of Pediatric Nephrology, has produced a shared steroid protocol that will be useful for National Health System hospitals and pediatricians. Investigations at initial presentation and the principal causes of NS to be screened are suggested. In the early phase of the disease, symptomatic treatment is also important as many severe complications can occur which are either directly related to the pathophysiology of the underlying NS or to the steroid treatment itself. To date, very few studies have been published on the prophylaxis and treatment of these early complications, while recommendations are either lacking or conflicting. This consensus provides indications for the prevention, early recognition and treatment of these complications
Hoeyer, Klaus; Jensen, Anja Marie Bornø
of treatment norms, we must move close to everyday work practices and appreciate the importance of material–technical treatment options as well as the interplay of professional ethics and identity. The cardiac treatment of brain-dead donors may thereby illuminate how treatment norms develop on the ground...
I.A.M. Robeyns (Ingrid)
textabstractThe capability approach is one of the most recent additions to the landscape of normative theories in ethics and political philosophy. Yet in its present stage of development, the capability approach is not a full-blown normative theory, in contrast to utilitarianism, deontological
U.S. Department of Health & Human Services — The NIH Study of Normal Brain Development is a longitudinal study using anatomical MRI, diffusion tensor imaging (DTI), and MR spectroscopy (MRS) to map pediatric...
... NICHD Research Information Find a Study More Information Preeclampsia and Eclampsia About NICHD Research Information Find a ... or visit the Centers for Disease Control and Prevention’s Safe Child website . What is pediatric critical care? ...
The National Institute of Child Health and Human Development (NICHD) works with NCI Enterprise Vocabulary Services (EVS) to provide standardized terminology for coding pediatric clinical trials and other research activities.
Yan, Weiyuan; Wang, Lijie
To evaluate the clinical value of the pulse indicator continuous cardiac output (PiCCO) system in patients with severe acute pancreatitis (SAP) complicated with acute respiratory distress syndrome (ARDS). Two cases of SAP with ARDS were monitored using PiCCO during comprehensive management in the Pediatric Intensive Care Unit (PICU) of Shengjing Hospital, China Medical University. To guide fluid management, the cardiac index (CI) was measured to assess cardiac function, the global end-diastolic volume index (GEDVI) was used to evaluate cardiac preload, and the extravascular lung water index (EVLWI) was used to evaluate the pulmonary edema. Case 1 was diagnosed with type L2 acute lymphoblastic leukemia (intermediate risk) and received the sixth maintenance phases of chemotherapy this time. After a 1-week dosage of chemotherapeutic drugs (pegaspargase and mitoxantrone), he suffered SAP combined with ARDS. Except comprehensive treatment (life supporting, antibiotic, etc.) and applying continuous veno-venous hemodiafiltration (CVVHDF) to remove inflammatory mediators. PiCCO monitor was utilized to guide fluid management. During the early stage of PiCCO monitoring, the patient showed no significant manifestations of pulmonary edema in the bedside chest X-ray (bedside ultrasound showed left pleural effusion), and had an oxygenation index 223 mmHg (1 mmHg = 0.133 kPa), GEDVI 450 ml/m², and ELVWI 7 ml/kg. We increased cardiac output to increase tissue perfusion and dehydration speed of CVVHDF was set at 70 ml/h. Two hours later, GEDVI significantly increased to 600 ml/m² and ELVWI significantly increased to 10 ml/kg, the oxygenation index declined to 155 mmHg, the bedside chest X-ray showed a significant decrease of permeability (right lung) and PEEP was adjusted to 5 cmH₂O (1 cmH₂O = 0.098 kPa), indicating circulating overload. ARDS subsequently occurred, upon which the fluid infusion was halted, the dehydration rate of CVVHDF raised (adjusted to 100-200 ml/h). On
Sharath Asokan; Sivakumar Nuvvula
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 pe...
Klinich, Kathleen D.; Reed, Matthew P.
Anthropometry is the measurement of human size, shape, and physical capabilities. Most pediatric anthropometry data are gathered to describe child growth patterns, but data on body size, mass distribution, range of motion, and posture are used to develop crash test dummies and computational models of child occupants. Pediatric anthropometry data are also used to determine child restraint dimensions, so they will accommodate the applicable population of child occupants.
Slover, Robin; Kent, Sheryl
Pediatric headaches are common, and many may never require intervention by a health care provider. However, migraines can become more difficult to treat, especially if they become chronic daily headaches. Pediatric headache is a subjective and unique experience that requires attention to both psychological and physiologic components in diagnosis and treatment. A biopsychosocial, multidisciplinary approach, including both medication management and psychological treatment, is considered essential for effective management.
Gadgil, Pradnya; Udani, Vrajesh
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.
Patel, Anita K; Bell, Michael J; Traube, Chani
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.
Curzer, Howard J.; Sattler, Sabrina; DuPree, Devin G.; Smith-Genthôs, K. Rachelle
The ethics assessment industry is currently dominated by the second version of the Defining Issues Test (DIT2). In this article, we describe an alternative assessment instrument called the Sphere-Specific Moral Reasoning and Theory Survey (SMARTS), which measures the respondent's level of moral development in several respects. We describe eight…
With this essay I treat some problems raised by the new developments in science and technology, that is, those about Computer Ethics to show how and how far Applied Ethics differs from traditional ethics. I take up backgrounds on which Computer Ethics rests, particularly historical conditions of morality. Differences of conditions in time and space explain how Computer Ethics and Applied Ethics are not any traditional ethics in concrete cases. But I also investigate the normative rea...
Colaneri, Natalie; Sheldon, Mark; Adesman, Andrew
Given the pervasiveness of psychotropic medication in the youth population and an increasingly competitive culture regarding educational performance, children, teenagers, and/or their parents may increasingly seek psychotropic substances in an effort to enhance a student's cognitive abilities and/or academic performance. Physicians must become aware of this very important and clinically relevant issue and work to ensure that medications remain in the hands of patients seeking wellness and not enhancement. The current article highlights findings on the pervasiveness of stimulant misuse and diversion in youth, the motivations and effects of stimulant use, health and legal consequences associated with use, and physician perceptions and preventive practices. Ethical concerns regarding pharmacological cognitive enhancement in pediatrics are also outlined - including coercion for nonusers, inequities in access, and threats to an individual's sense of self with regard to authenticity and autonomy. Pharmacological cognitive enhancement in pediatrics will become a larger, clinically relevant issue in the coming years. Physicians who care for children and adolescents must become more aware of this issue. Given the myriad health, legal, and ethical concerns, clinicians should discourage use of pharmaceuticals for enhancement purposes in the pediatric population.
... Imaging Encyclopedia of Pediatric Thoracic Disease Virtual Pediatric Hospital is the Apprentice's Assistant Last revised on February ... GeneralPediatrics.com | PediatricEducation.org | SearchingPediatrics.com Virtual Pediatric Hospital is curated by Donna M. D'Alessandro, M. ...
Steinberg, J J
The U.S. Environmental Protection Agency (EPA) held the first meeting on environmental ethics sponsored by the Scientific Advisory Panel and Board on 10-11 December 1998 in Arlington, Virginia (1). The report from the meeting will more completely inform scientists and the community of current issues. This editorial should serve as an initial brief of this meeting [which was held on the fiftieth anniversary of the Declaration of Human Rights (adopted by the United Nations on 10 December 1948)].
Decamp, Matthew; Joffe, Steven; Fernandez, Conrad V; Faden, Ruth R; Unguru, Yoram
Shortages of essential drugs, including critical chemotherapy drugs, have become commonplace. Drug shortages cost significant time and financial resources, lead to adverse patient outcomes, delay clinical trials, and pose significant ethical challenges. Pediatric oncology is particularly susceptible to drug shortages, presenting an opportunity to examine these ethical issues and provide recommendations for preventing and alleviating shortages. We convened the Working Group on Chemotherapy Drug Shortages in Pediatric Oncology (WG) and developed consensus on the core ethical values and practical actions necessary for a coordinated response to the problem of shortages by institutions, agencies, and other stakeholders. The interdisciplinary and multiinstitutional WG included practicing pediatric hematologist-oncologists, nurses, hospital pharmacists, bioethicists, experts in emergency management and public policy, legal scholars, patient/family advocates, and leaders of relevant professional societies and organizations. The WG endorsed 2 core ethical values: maximizing the potential benefits of effective drugs and ensuring equitable access. From these, we developed 6 recommendations: (1) supporting national polices to prevent shortages, (2) optimizing use of drug supplies, (3) giving equal priority to evidence-based uses of drugs whether they occur within or outside clinical trials, (4) developing an improved clearinghouse for sharing drug shortage information, (5) exploring the sharing of drug supplies among institutions, and (6) developing proactive stakeholder engagement strategies to facilitate prevention and management of shortages. Each recommendation includes an ethical rationale, action items, and barriers that must be overcome. Implemented together, they provide a blueprint for effective and ethical management of drug shortages in pediatric oncology and beyond.
Moreno Lax, Alejandro
Three ethics exist as a condition of possibility of any possible ethics, following a material and biological foundation. This content argument (not logical-formal) supposes a refutation of the naturalistic fallacy that the analytical philosophy attributes to Hume, in three areas of the ethical human experience: body, society and nature. These are: the ethics of the species [J. Habermas], the ethics of liberation [E. Dussel] and the ethics of the responsibility [H. Jonas]. This material argument is a philosophical foundation to considering for three types of applied ethics: medical bioethics, development ethics and environmental ethics.
Arief Ramadhan; Dana Indra Sensuse; Aniati Murni Arymurthy
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...
Pattanaik, Debendra; Lieberman, Jay Adam
The aims of this study are to update the clinician on current understanding of angioedema as it presents in the pediatric population and to review proper diagnostic techniques and treatment modalities for various types of angioedema. Angioedema is still best classified by whether it is likely histaminergic or kinin-mediated. New guidelines have been published around the world to help diagnose and treat both forms (urticaria/angioedema and hereditary angioedema). The vast majority of the studies on treatment have been conducted in the adult population; however, there are data available in the pediatric population. In the realm of hereditary angioedema, there are multiple new therapies that have been studied in the pediatric population (down to 2 years in some studies) in recent years and offer the clinician options for treatment. Angioedema (whether occurring with or without urticaria) is common in the pediatric population. The majority of the recent studies has been conducted in hereditary angioedema, and now, the clinician should have various options to treat all forms of angioedema. Many treatment options, especially for hereditary angioedema, are further being examined specifically in the pediatric population.
Korthals, M.J.J.A.A.; Bogers, R.J.
In this book we begin with two contributions on the ethical issues of working in organizations. A fruitful side effect of this start is that it gives a good insight into business ethics, a branch of applied ethics that until now is far ahead of ethics for life scientists. In the second part, ethics
Furman, Gail C.
This article proposes the concept of an ethic of community to complement and extend other ethical frames used in education e.g. the ethics of justice, critique, and care. Proceeding from the traditional definition of ethics as the study of moral duty and obligation, ethic of community is defined as the moral responsibility to engage in communal…
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)
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.)
Burch, Ezra A.; Orbach, Darren B.
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.)
Full Text Available Abstract We report on a case of autoimmune thyroiditis in a 6-month-old patient with cortico-resistant nephrotic syndrome. Normal serum levels of thyroid hormons and thyroid-stimulating hormone were detected with high titers of circulant antithyroid antibodies and a dysomogeneous ultrasound appearance of the gland, typical of autoimmune thyroiditis. The research of maternal thyroid antibodies was negative. This is the first case of autoimmune thyroiditis found in such a young patient with pre-existing nephrotic syndrome ever described in literature. This association is random because nephrotic syndrome does not have an autoimmune pathogenesis and the genes involved in autoimmune thyroiditis are not related to those of nephrotic syndrome.
Kellen, Roselyn; Silverberg, Nanette B
Because rosacea is uncommon in the pediatric population, care must be taken to exclude other papulopustular disorders. Children can present with vascular, papulopustular, and/or ocular findings. Importantly, ocular symptoms can appear before the cutaneous symptoms of rosacea, leading to misdiagnosis. Rosacea is a clinical diagnosis, but histopathologic examination typically reveals dilated vessels, perivascular lymphohistiocytic infiltrates in the upper dermis, elastosis, and disorganization of the upper dermal connective tissue. Treatment involves avoiding known triggers and utilizing topical and/or systemic therapies. Although treatment can control flares, pediatric rosacea often persists into adulthood.
Martin O Savage
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.
Mehmet Burak Özkan; Meltem Ceyhan Bilgici; Murathan Şahin; Gurkan Genc
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 ...
Claire E Wainwright
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.
Søndergaard, Dorte Marie
Human subjects and social relations are crucial in research psychologists’ ethical considerations. Lists of ethical criteria - including how to anonymize data, avoid causing harm, handle asymmetries – are pivotal. A situated ethics inspired by new materialism and poststructuralism would, however,...
This article focuses on the question of unification versus relationality in ethics. It compares two different ethical approaches from Late Antiquity, highlighting the contrast between Plotinian (Neoplatonic) ethics as striving for perfect unification of the human soul with the divinity...
Palmer, Clare; Sandøe, Peter
This chapter describes and discusses different views concerning our duties towards animals. First, we explain why it is necessary to engage in thinking about animal ethics and why it is not enough to rely on feelings alone. Secondly, we present and discuss five different kinds of views about the ...... the nature of our duties to animals. They are: contractarianism, utilitarianism, the animal rights view, contextual views, and a respect for nature view. Finally, we briefly consider whether it is possible to combine elements from the presented views, and how to make up one’s mind....
The development of applied ethics in recent decades has had great significance for philosophy and society. In this article, I try to characterise this field of philosophical inquiry. I also discuss the relation of applied ethics to social policy and to professional ethics. In the first part, I address the following questions: What is applied ethics? When and why did applied ethics appear? How do we engage in applied ethics? What are the methods? In the second part of the article, I introduce...
Kesgin, Ahmet; KESGİN, Ahmet
Ethics is philosophical thought which human behavior’s scope of moral is subject. However, by effectuating the principles of moral is to be in the life. In this regard, although not exactly the same is to be fulfilled the function of the concept of morality. There are ussually common oponion which ethics, a philosophical topic, is three kinds. These are descriptive ethics, normative ethics and metaethics. The main characteristics of these types of ethical are: Descriptive ethics depict existi...
Farooqi IS, O'Rahilly S. Genetic syndromes associated with obesity. In: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Adult and Pediatric . 7th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 28. ...
means ostracism, neglect, loss of family, abuse, or orphan status 2. In spite of the now estimated 40 million ... The human virus is difficult to be cultured in animals except chimpanzee. It differs from the simian virus 4. .... 1994 revised HIV pediatric classification system: clinical categories13. Category N: not symptomatic.
Harrison, J P; Franklin, M E
Trichotillomania (TTM) is an impulse control disorder characterized by chronic hair-pulling, distress, and impairment. Although the negative effects of TTM are documented and often readily evident, there remains a paucity of psychopathology and treatment research on this disorder, particularly in pediatric populations. In an effort to improve assessment of pediatric TTM, several TTM-specific instruments for youth have now been developed to reliably identify symptoms and examine related phenomenology. Instrument development has now yielded instruments to evaluate TTM and related symptoms in the context of clinical trials of youth, and the first randomized controlled trial of any treatment for pediatric TTM was recently published. Using the initial pediatric TTM studies as building blocks, future research is now needed to create a stronger body of knowledge about the relative and combined efficacy of potential interventions for TTM in youth, as well as to examine the effects of TTM phenomenology and comorbidity on treatment outcome. Dissemination efforts must also be heightened for this knowledge to best reach these vulnerable populations.
Barut, Kenan; Sahin, Sezgin; Kasapcopur, Ozgur
The aim of this review is to define childhood vasculitis and to highlight new causative factors and treatment modalities under the guidance of recently published studies. Childhood vasculitis is difficult to diagnose because of the wide variation in the symptoms and signs. New nomenclature and classification criteria were proposed for the diagnosis of pediatric vasculitis. Recently, progress has been made toward understanding the genetic susceptibility to pediatric vasculitis as it was in other diseases. Various radiological techniques provide great opportunities in establishing the diagnosis of pediatric vasculitis. Mild central nervous system disease can accompany Henoch-Schonlein purpura and can go unnoticed. Antineutrophilic cytoplasmic antibody-associated vasculitis is rare in children. Increased severity of the disease, subglottic stenosis, and renal disease are described more frequently among children. Biological therapies are used with success in children as in adults. Future studies, whose aims are to evaluate treatment responses, prognosis and to design guidelines for activity, and damage index of vasculitis for children are required. Henoch-Schonlein purpura and Kawasaki disease are the most frequent vasculitides of children. Experience from adult studies for treatment and prognosis are usually used because of low incidence of other vasculitides in children. Multicenter studies of pediatric vasculitis should be conducted to detail treatment responses and prognosis in children.
[Case report: Iatrogenic shoulder pain syndrome following spinal accessory nerve injury during lateral cervical neck dissection for tongue cancer: the role of rehabilitation and ethical-deontological issues].
Ronconi, Gianpaolo; Spagnolo, Antonio Gioacchino; Ferriero, Giorgio; Giovannini, Silvia; Amabile, Eugenia; Maccauro, Giulio; Ferrara, Paola Emilia
The shoulder pain syndrome is the most frequent complication of lateral cervical neck dissection and may be caused by iatrogenic injury to the spinal accessory nerve, causing pain and functional limitation of the upper limb and of the cervical spine. Interdisciplinary collaboration and early rehabilitation can reduce the consequences of disability and the possible issues that can arise due to inadequate management of the problem.
This work is about the ethics of education, and about philosophy as a discipline that can help us to help children look at ethics afresh. The study and practice of ethics is about morals and uncertainties and, as such, poses problems for the research community. The philosopher Ricoeur challenges research as only one way to find meaning in the…
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)
Drumwright, Minette; Prentice, Robert; Biasucci, Cara
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…
Full Text Available The article presents the data concerning definition, components and the manifestations of emotional burnout syndrome in general and in medical teachers, in particular. The author has analyzed the history of the question for the last decades, evaluated the findings, systemized the main approaches to the detection and liquidation of symptoms of emotional burnout syndrome. Negative personality traits were identified both in the teacher and medical student contributing to the development of emotional burnout in the teacher. The author proposes the ways to solve this problem in higher medical school.
Vaughan, Casey; Attlmayr, Bernhard; Dalton, Lucy; Upile, Navdeep; Xie, Carol; De, Su
Craniosynostosis is defined as premature fusion of the cranial suture lines and is part of a syndrome in 15% to 40% of the patients. There is limited literature available regarding these children's ability to smell. Most of them will undergo numerous surgical procedures, some of which may alter their sense of smell, potentially leading to significant social as well as safety implications. Ethical approval was obtained for this pilot study. Children with syndromic craniosynostosis were recruited and underwent anterior rhinoscopy, prior to performing a smell test utilizing the Sensonic pediatric Smell wheel. The results were compared to an age-matched control group. Eight children with syndromic craniosynostosis participated in the study. Of a possible total score of 11, their mean average score was 6.6 and the median was 6. In comparison, the mean average score for the control group was 7.5 and the median was 7. Although the study group was small, this pilot study demonstrates that children with syndromic craniosynostosis have a similar ability to identify smells to an age-matched cohort. Further research can now be undertaken to see whether or not midface advancement procedures affect these children's sense of smell.
Campisi, Paolo; Forte, Vito
Tracheotomy refers to a surgical incision made into a trachea. Tracheostomy, on the other hand, refers to a surgical procedure whereby the tracheal lumen is positioned in close proximity to the skin surface. Tracheostomy is an uncommon procedure in the pediatric population. When required tracheostomy is typically performed as an open surgical procedure under general anesthesia with the patient intubated. However, it may need to be performed under local anesthesia or over a rigid bronchoscope in the patient with a precarious airway. Over the past half century, the primary indication for pediatric tracheostomy has shifted from acute infectious airway compromise to the need for prolonged ventilatory support in neurologically compromised children. The surgical technique, choice of tracheostomy tube, and post-operative care requires a nuanced approach in infants and young children. This article will review these topics in a comprehensive fashion. Copyright © 2016 Elsevier Inc. All rights reserved.
Beggs, Jeri Mullins
The ineffectiveness of business ethics education has received attention from the popular press and the Association to Advance Collegiate Schools of Business after repeated ethics scandals. One possibility is that teaching ethics is different from other content areas because ethics is best learned when the student does not know it is being taught.…
This e-book on Emerging Technologies and Ethics includes a collection of essays which explore the future and ethics of emerging information and communication technologies. Articles in the collection include an overview of the legal implications which may be relevant to the ethical aspects of emerging technologies and also ethical issues arising from the mass-take up of mobile technologies.
Lieberman, Jethro K.
The evolution of ethics in law is followed from the harshness of caveat emptor to the humanistic ethics of the 1970s, including a renewal of formal ethics in the post-Watergate era. The impact on universities and individual disciplines of legalizing ethical conduct is examined cautiously. (JMF)
The title of this reflection evokes several contents that may encompass from ethics in research; fraud in science; ethics in medical advertising and relations between sponsors and science; and, finally, papers related to ethic content. This paper is limited to the ethic responsibilities of the medical writers or "scriptwriters."
Stroke in childhood has gained increasingly more attention and is accepted as an important disease in childhood. The reasons for this severe event and the consequences for the rest of the life are totally different than for adults. This is also true for the diagnosis and therapy. This paper gives a comprehensive overview on the characteristics of pediatric stroke to assist radiologists in making a rapid and safe diagnosis in order to identify the underlying disease. (orig.) [de
Riddle, M A; Kastelic, E A; Frosch, E
This paper reviews research in pediatric psychopharmacology over the past decade. The authors first discuss social, economic, and other influences on pediatric psychopharmacology research and prescribing patterns including changing models of childhood psychopathology, increased government funding, and changes in industry regulations. Definitions are offered for current research terminology including efficacy, effectiveness, and adverse events. Design trends and new approaches to outcome measurement are also presented. New data from the last 10 years of research is reviewed for each major class of psychotropic agents. Criteria for inclusion in the review are presented and include aspects of study design (placebo-controlled, large sample size), source of funding (government funded vs. industry), and vision (creative applications). Data for short-term efficacy, long-term efficacy, effectiveness, and safety and adverse events are discussed for each class of medication, although for many, there remains little empirical data. Findings for stimulants, selective serotonin reuptake inhibitors, neuroleptics, alpha-adrenergic agonists, mood stabilizers, buproprion, secretin, naltrexone, immune therapies, and natural supplements are all presented. Finally, the authors offer some speculations regarding the future of pediatric psychopharmacology research.
Full Text Available ... News Physician Resources Professions Site Index A-Z Children's (Pediatric) Nuclear Medicine Children’s (pediatric) nuclear medicine imaging ... the limitations of Children's Nuclear Medicine? What is Children's (Pediatric) Nuclear Medicine? Nuclear medicine is a branch ...
Full Text Available ... Physician Resources Professions Site Index A-Z Children's (Pediatric) Nuclear Medicine Children’s (pediatric) nuclear medicine imaging uses ... limitations of Children's Nuclear Medicine? What is Children's (Pediatric) Nuclear Medicine? Nuclear medicine is a branch of ...
... discharge; Heart valve surgery - children - discharge; Heart surgery - pediatric - discharge; Heart transplant - pediatric - discharge ... Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics . 20th ed. Philadelphia, PA: Elsevier; 2016:chap 434. ...
Full Text Available ... Resources Professions Site Index A-Z Children's (Pediatric) Nuclear Medicine Children’s (pediatric) nuclear medicine imaging uses small ... of Children's Nuclear Medicine? What is Children's (Pediatric) Nuclear Medicine? Nuclear medicine is a branch of medical ...
... Professions Site Index A-Z Children's (Pediatric) Nuclear Medicine Children’s (pediatric) nuclear medicine imaging uses small amounts ... Children's Nuclear Medicine? What is Children's (Pediatric) Nuclear Medicine? Nuclear medicine is a branch of medical imaging ...
Full Text Available ... Professions Site Index A-Z Children's (Pediatric) Nuclear Medicine Children’s (pediatric) nuclear medicine imaging uses small amounts ... Children's Nuclear Medicine? What is Children's (Pediatric) Nuclear Medicine? Nuclear medicine is a branch of medical imaging ...
Business ethics is a controversial topic. In my article I would like to explore where the limits of business ethics are and to what extent it can become part of the economic world. I would like to explore the question what the relationship between ethics and business is and whether what business ethicists consider ethics is real ethics in the fundamental sense of the world. The concept of business ethics will be discussed compared to general ethical theories and consequences drawn. I would li...
Luiz Martins da Silva
The purpose of this article is to dream of – in the sense ofestablishing – the utopian perspective of a scenario that remains utopian but which nonetheless provides the first indications that we may be entering a new paradigm, that of communication-ethics, that is to say, the ethical dimension of communication, which in this case is not restricted to technological advancements but concerns communication with ethics and as ethics, to conclude that without ethics, there is no commun...
The very term 'professional ethics' is puzzling with respect to what both 'professional' and 'ethics' might mean. I argue (1) that professionalism is ambiguous as to whether or not it is implicitly committed to ethical practice; (2) that to be 'professionally' ethical is at best ambiguous, if not in fact bizarre; and (3) that, taken together, these considerations suggest that professional ethics is something to be avoided rather than lauded.
Korthals, M.J.J.A.A.; Bogers, R.J.
In this book we begin with two contributions on the ethical issues of working in organizations. A fruitful side effect of this start is that it gives a good insight into business ethics, a branch of applied ethics that until now is far ahead of ethics for life scientists. In the second part, ethics of activities directly connected with doing scientific research are discussed, like experimenting with animals and human beings, publishing, patenting, getting funds and selecting one’s research th...
Ethics is a relevant value in business and management consulting. The presence of recognized ethics tends to reduce the need for informative or legal-contractual precautions in the formalization of relationships, for both of the parts involved in a negotiation. Management Consulting on ethics will develop more and more. Law will consider more and more ethics in business and management consulting. The ethics of corporations influences their workers and behaviour with the customers. It is an e...
Raimondi, A J
Pediatric neurosurgery as a subspecialty dealing primarily with such congenital and perinatal pathology as the dysraphic state, and various clinical entities having in common ventriculomegaly, is fast disappearing from the scene of neurosurgery in the industrialized world. Pari passu with this, one observes ever closer collaborative work between pediatric neurosurgeons and specialists in other pediatric disciplines such as oncology, radiology, orthopedics, and maxillofacial surgery: truly multidisciplinary activities. In addition, paramedical and specialized nursing personnel are participating actively, even in the decision-making and treatment-delivery aspects of pediatric neurosurgical care. The pediatric neurosurgeon is no longer the sole decision-maker, nor the automatic captain of the ship. Very probably, as in the whole history of human activities, new instrumentation will change somewhat what we do and with whom we collaborate. However, for the immediate future the major changes most probably will be expressions of socioeconomic readjustments, of ethical redefinitions, and of the to-and-fro movement into and out of pediatric neurosurgery by both neurosurgeons and ancillary medical personnel.
... AAPD AAPD Publications Advertising Brochures Journals & Publications Full Journal Archives Access Pediatric Dentistry Today Practice Management and Marketing Newsletter Pediatric Dentistry Journal Open Access Articles Oral ...
Dyment, Paul G.
Discusses controversial issues that have arisen in children's sports, including infant exercise programs, trampolines, amenorrhea in the adolescent athlete, coed contact sports, and sport participation by children with Down Syndrome. Policy statements are included from the American Academy of Pediatrics. (JD)
Mohamed S. Al Riyami
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.
Sawai, Toshihiro; Nangaku, Masaomi; Ashida, Akira; Fujimaru, Rika; Hataya, Hiroshi; Hidaka, Yoshihiko; Kaname, Shinya; Okada, Hirokazu; Sato, Waichi; Yasuda, Takashi; Yoshida, Yoko; Fujimura, Yoshihiro; Hattori, Motoshi; Kagami, Shoji
Atypical hemolytic uremic syndrome (aHUS) is rare and comprises the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Recently, abnormalities in the mechanisms underlying complement regulation have been focused upon as causes of aHUS. The prognosis for patients who present with aHUS is very poor, with the first aHUS attack being associated with a mortality rate of ~25 %, and with ~50 % of cases resulting in end-stage renal disease requiring dialysis. If treatment is delayed, there is a high risk of this syndrome progressing to renal failure. Therefore, we have developed diagnostic criteria for aHUS to enable its early diagnosis and to facilitate the timely initiation of appropriate treatment. We hope these diagnostic criteria will be disseminated to as many clinicians as possible and that they will be used widely.
Velasquez Mendez, Monica Patricia; Ramirez Gomez, Luis Alberto
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
Bioethics became applied ethics when it was assimilated to moral philosophy. Because deduction is the rationality of moral philosophy, subsuming facts under moral principles to deduce conclusions about what ought to be done became the prescribed reasoning of bioethics, and bioethics became a theory comprised of moral principles. Bioethicists now realize that applied ethics is too abstract and spare to apprehend the specificity, particularity, complexity and contingency of real moral issues. Empirical ethics and contextual ethics are needed to incorporate these features into morality, not just bioethics. The relevant facts and features of problems have to be identified, investigated and framed coherently, and potential resolutions have to be constructed and assessed. Moreover, these tasks are pursued and melded within manifold contexts, for example, families, work and health care systems, as well as societal, economic, legal and political backgrounds and encompassing worldviews. This naturalist orientation and both empirical ethics and contextual ethics require judgment, but how can judgment be rational? Rationality, fortunately, is more expansive than deductive reasoning. Judgment is rational when it emanates from a rational process of deliberation, and a process of deliberation is rational when it uses the resources of non-formal reason: observation, creative construction, formal and informal reasoning methods and systematic critical assessment. Empirical ethics and contextual ethics recognize that finite, fallible human beings live in complex, dynamic, contingent worlds, and they foster creative, critical deliberation and employ non-formal reason to make rational moral judgments. © 2017 John Wiley & Sons Ltd.
Gallagher, Ann; Tschudin, Verena
In this article we consider the nature of ethical leadership in nursing. An appreciation of the basis of such leadership requires an understanding of responsibility and of key intellectual and ethical qualities or virtues. We examine some of the educational and practice strategies to promote ethical leadership. We argue that there are different levels of ethical leadership. All members of the nursing workforce are ethical leaders in so far as they demonstrate a commitment to ethical practice in their everyday work and act as ethical role models for others. Nurse managers are responsible for influencing their team and for acting as arbiters between organisational and professional values. Nurse educators are role models and ethical leaders as they ensure that the explicit and hidden curriculum demonstrate a commitment to professional values. Nurses who assume political roles have an obligation to lead on ethical agenda compatible with the values of nursing.
Full Text Available Business ethics is a controversial topic. In my article I would like to explore where the limits of business ethics are and to what extent it can become part of the economic world. I would like to explore the question what the relationship between ethics and business is and whether what business ethicists consider ethics is real ethics in the fundamental sense of the world. The concept of business ethics will be discussed compared to general ethical theories and consequences drawn. I would like to show the contradictions inherent to the connection of business and ethics are no coincidence. At the end a possible relationship between ethics and business sphere will be suggested.
Tidwell, A.S.; Solano, M.; Schelling, S.H.
In this article, some of the common and not-so-common neuropediatric disorders were discussed. As in the full-grown animal, abnormalities of the CNS in the pediatric animal patient may be classified according to the type of insult present (eg, malformation, injury, neoplasia, inflammation, or degeneration). To recognize the imaging manifestations of such disorders, an appreciation of normal anatomy, the pathological response of nervous system tissue to insult, and the principles of image interpretation is required. These fundamentals may then be applied to any CNS disease, regardless of frequency and to any animal patient, regardless of age
Eka Agustia Rini
there is no cure for cri-du-chat syndrome. The most successful approach in the management of children with CDCS requires a multidisciplinary team approach. 4 The case presented below will remind us how to reveal, suspect and diagnose Cri-Du-Chat syndrome, a rare case in pediatric.
Flite, Cathy A; Harman, Laurinda B
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.
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.
This article focuses on the question of unification versus relationality in ethics. It compares two different ethical approaches from Late Antiquity, highlighting the contrast between Plotinian (Neoplatonic) ethics as striving for perfect unification of the human soul with the divinity...... - and Biblical ethics as a relational ethics, where alterity remains operative in the encounter with the deity, and where the primary ethical demand is to relate properly to fellow creatures and God as other. The latter demand is exemplified by the figure of Job, whose righteousness is interpreted as his...
Kirkpatrick, J.A. Jr.
Computed tomography has made possible the excellent and basic work having to do with the characteristics of the trachea, its caliber, shape, and length in children. Another group of articles has to do with interventional pediatric radiology. This year there were a number of articles of which only a sample is included, dealing with therapeutic procedures involving drainage of abscesses, angioplasty, nephrostomy, therapeutic embolization, and the removal of esophageal foreign bodies. Obviously, there is no reason to think that techniques developed for the adult may not be applicable to the infant or child; also, there is no reason to believe that processes peculiar to the child should not be amenable to intervention, for instance, use of embolization of hepatic hemangioma and transluminal balloon valvuloplasty for pulmonary valvular stenosis. Among the reports and reviews, the author would add that sonography remains a basic imaging technique in pediatric radiology and each year its application broadens. For example, there is an excellent article having to do with sonography of the neonatal and infant hip and evaluation of the inferior vena cava and the gallbladder. Nuclear medicine continues to play a significant role in diagnosis, which is featured in two articles concerned with problems of the hip
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 ethical issues pertaining to pediatric deceased donation and developed recommendations for policy and practice. Their expertise was inclusive of pediatric intensive care, internal medicine, and surgery, nursing, ethics, organ donation and procurement, psychology, law, and sociology. The report of the meeting advocates the routine provision of opportunities for deceased donation by pediatric patients and conveys an international call for the development of evidence-based resources needed to inform provision of best practice care in deceased donation for neonates and children.
Choe, Meeryo C; Blume, Heidi K
Head injuries are common in pediatrics, and headaches are the most common complaint following mild head trauma. Although moderate and severe traumatic brain injuries occur less frequently, headaches can complicate recovery. There is currently an intense spotlight on concussion and there has been a corresponding increase in the number of children seeking care for headache after mild traumatic brain injury or concussion. Understanding the natural history of, and recognition of factors that are associated with posttraumatic headache will help providers and families to limit disability and may prompt earlier intervention to address disabling headaches. While there are few studies on the treatment of posttraumatic headache, proper evaluation and management of posttraumatic headaches is essential to prevent further injury and to promote recovery. In this article, we will review the current definitions and epidemiology of pediatric posttraumatic headache and discuss current recommendations for the evaluation and management of this syndrome in children and adolescents. © The Author(s) 2015.
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)
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
Full Text Available Although ethics training is one of the core components of psychiatric education, it is not sufficiently addressed in the curricula of many educational institutions. It is shown that many of the psychiatry residents received no ethics training in both residency and medical school. Predictably, over half of the psychiatry residents had faced an ethical dilemma that they felt unprepared to meet, and nearly all of them indicated ethics education would have helped them to solve this dilemma. In addition to learning about the fundamental topics of ethics like confidentiality, boundary violations, justice, benefience and nonmaleficence, psychiatrists must also learn to deal with other hidden ethical dilemmas which are mostly due to the changing world order. It is obvious that residency training should include a well developed ethics curriculum. However, some still believe that ethical principles cannot be taught and are formed in one’s early moral development. Accepting the fact that teaching ethics is difficult, we believe that it is getting easier with the new methods for teaching in medicine. These methods are clinical supervisions, rol-models, case studies, role playing, small group discussions, team based learning and “let’s talking medicine” groups which is a useful methods for discussing ethics dilemmas on daily practice and C.A.R.E (Core Beliefs, Actions, Reasons, Experience which is a special training method for teaching ethics. In this review, the need of ethics training in residency curriculum will be discussed and new methods for teaching ethics will be proposed.
Kesselheim, Jennifer C; Najita, Julie; Morley, Debra; Bair, Elizabeth; Joffe, Steven
To evaluate the relationship between recently trained paediatricians' ethics knowledge and exposure to a formal ethics or professionalism curriculum during residency. We conducted a cross-sectional survey of recently trained paediatricians which included a validated 23-item instrument called the Test of Residents' Ethics Knowledge for Pediatrics. The sample included paediatricians who completed medical school in 2006-2008, whose primary specialty was paediatrics or a paediatric subspecialty, and who completed paediatric residency training in 2010-2011. This sample was stratified based on residency programme variables: presence of a formal curriculum in ethics or professionalism, programme size and American Board of Pediatrics certifying exam passage rate. Paediatricians were randomly selected from each stratum for survey participation. Among the 370 responding paediatricians (55%), the mean knowledge score was 17.3 (SD 2.2) out of a possible 23. Presence of a formal curriculum in ethics and/or professionalism was not significantly associated with knowledge. Knowledge was lowest on items about parental requests for a child to undergo genetic testing (2 items, 44% and 85% incorrect), preserving patient confidentiality over email (55% incorrect), decision-making regarding life-sustaining technologies (61% incorrect), and decision-making principles such as assent and parental permission (2 items, 47% and 49% incorrect). This study highlights several areas in which paediatricians' knowledge may be low and that are amenable to targeted educational interventions. These findings should prompt discussion and research among ethicists and educators about how ethics and professionalism curricula can more consistently influence paediatricians' knowledge. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.
Luiz Martins da Silva
Full Text Available The purpose of this article is to dream of – in the sense ofestablishing – the utopian perspective of a scenario that remains utopian but which nonetheless provides the first indications that we may be entering a new paradigm, that of communication-ethics, that is to say, the ethical dimension of communication, which in this case is not restricted to technological advancements but concerns communication with ethics and as ethics, to conclude that without ethics, there is no communication and by extension, no genuine mankind nor genuine humanity. Communication and ethics therefore appear inseparable; one cannot exist without the other. The audacity of this ethical visionary lies in managing to perceive more than just technological marvels, but also to appreciate the paradigm of anthropoethics entering the realm of the concrete, that is to say, ethics for mankind and for humanity, as conceived of by Apel, Habermas and Morin.
Luiz Martins da Silva
Full Text Available The purpose of this article is to dream of – in the sense of establishing – the utopian perspective of a scenario that remains utopian but which nonetheless provides the first indications that we may be entering a new paradigm, that of communication-ethics, that is to say, the ethical dimension of communication, which in this case is not restricted to technological advancements but concerns communication with ethics and as ethics, to conclude that without ethics, there is no communication and by extension, no genuine mankind nor genuine humanity. Communication and ethics therefore appear inseparable; one cannot exist without the other. The audacity of this ethical visionary lies in managing to perceive more than just technological marvels, but also to appreciate the paradigm of anthropoethics entering the realm of the concrete, that is to say, ethics for mankind and for humanity, as conceived of by Apel, Habermas and Morin.
The relationship between ethics and philosophy and jurisdiction is described; different kinds of ethics are presented. The increasing pressure of liberal points of view has boosted the ethics of utility. The ethics of care oppose a too rational utilitarianism, taking into consideration relationships such as the caregiver-patient relationship. In the multicultural society ethics of care and virtue ethics are being criticised for not giving universal answers to ethical dilemmas. Can one still define "doing good"? Is "doing good" so culturally biased that it no longer provides the basis for ethical conduct? An accurate procedural assessment of values, sometimes interpreted quite differently in different cultures, could be a tool to judge values in a less relativistic way.
Notes that it is essential that business organizations establish organizational systems that require satisfactory ethical business behaviors from everyone concerned, regardless of differences in personal outlooks. Outlines what needs to be done in order to effectively teach business ethics. (SG)
Provides a philosophical-ethical "toolkit" for analyzing central ethical issues evoked by our use of new media, including privacy, copyright, violent and sexual content online, and cross-cultural communication online....
Leibson, Tom; Koren, Gideon
Pediatric drug research is gradually becoming more and more accepted as the norm for assessing whether a drug is safe and efficacious for infants and children. The process of informed consent and assent for these trials presents a major challenge. The aim of this review is to map historical, ethical and legal aspects relevant to the challenges of informed consent in the setting of pediatric drug research. The impact of age, level of maturity and life circumstances on the process of obtaining informed consent as well as the relations between consent and assent are discussed. There appears to be a lack of regulatory clarity in the area of pediatric clinical trials; while numerous statements have been made regarding children's rights to autonomy and their ability to care for themselves and for younger ones, the ever changing status of adolescence is still difficult to translate to informed consent. This may delay scientific and clinical advancement for children who are at the very junction of being independent and not needing parental permission. Obtaining consent and assent for pediatric clinical trials is a delicate matter, as both parent and child need to agree to participate. The appropriate transfer of information to guardians and the children, especially concerning potential risks and benefits, is at the heart of informed consent, as it serves to protect both patient and physician. As many adults lack health literacy, one must ensure that guardians receive relevant information at a level and in forms they can understand regarding the trials their children are asked to participate in.
Battin, Tim; Riley, Dan; Avery, Alan
The regulatory scope of Human Research Ethics Committees can be problematic for a variety of reasons. Some scholars have argued the ethics approval process, for example, is antithetical to certain disciplines in the humanities and social sciences, while others are willing to give it qualified support. This article uses a case study to cast the…
Sternberg, Robert J.
Is there an ethical giftedness, and if so, what does it look like? In this article, I consider why ethical behavior is much harder to come by than one would expect. Ethically gifted individuals are able to complete a series of eight steps to action, the failure of any one of which may result in a person, even one who is ethically well trained, to…
Sternberg, Robert J.
What is, or should be, the role of ethics in giftedness? In this article, I consider why ethical behavior is much harder to come by than one would expect. Ethical behavior requires completion of a series of eight steps to action, the failure of any one of which may result in a person, even one who is ethically well trained, to act in a manner that…
Nedelko, Zlatko; Potočan, Vojko
The main purpose of this paper is to examine how employeesʼ personal ethics, expressed through their personal values and attitudes toward social and environmental issues, are associated with the ethics of organizations in public administration. The authors introduce their own theoretical model that examines the relations between employeesʼ personal ethics - expressed through employeesʼ personal values and attitudes toward natural and social environments - and the ethics of public administrati...
Tsukamoto, Katusra; Carroll, Kelly A; Onishi, Taku; Matsumaru, Naoki; Brasseur, Daniel; Nakamura, Hidefumi
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 (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 Japan and the United States were 0.560 (P Japan was more active after the introduction of these premiums, even reaching the level of the European Union. The Pediatric Regulation and the Paediatric Committee promoted pediatric drug development in the European Union. The registered number of clinical trials that includes at least 1 participants 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 community. Expanding the regulatory steps taken in the European Union
Up to one in three young people with chronic fatigue syndrome (CFS/ME) also has depressive symptoms. It is not known how best to treat young people with this comorbidity. This case report seeks to describe and discuss the use of a cognitive behavioral approach for depression and low self-esteem in a 16-year-old girl with CFS/ME. Therapy was effective in remediating the young person's mood difficulties, but appeared to exacerbate their CFS/ME symptoms. Therefore, it is crucial that CFS/ME and mood treatments are designed and trialed to ensure a complementary approach. Good communication and joint working between involved professionals is also important, and ideally, treatments for mood and for CFS/ME would be provided by the same team to facilitate this. © 2015 Wiley Periodicals, Inc.
Jeurissen, R.J.M.; Ven, van de B.W.
This article presents a response to the following papers: "Ethical Marketing," by P.E. Murphy, G.R. Laczniak, N.E. Bowie, and T.A. Klein, "Marketing Ethics: Cases and Readings," edited by P.E. Murphy and G.R. Laczniak, "Advertising Ethics" by E.H. Spence and B. van Heekeren, and "Corporate Social
If the goal of teaching ethics is to affect behavior and ultimately produce thoughtful, ethical people, then the best process to do this is by embedding ethical practices in student discussions. One teacher demonstrates how she has done this in her classrooms.
Full Text Available It approaches the librarian ethics comprehending the Librarianship constitution from a systemic view. In this way, with the objective to raise issues to discuss professional ethics, it places the librarian in the work world and points approaches between exertion and relation context of the professionals themselves with the human rights and alteration ethics.
van de Poel, I.R.; van der Poel, Ibo; Verbeek, Peter P.C.C.
Engineering ethics and science and technology studies (STS) have until now developed as separate enterprises. The authors argue that they can learn a lot from each other. STS insights can help make engineering ethics open the black box of technology and help discern ethical issues in engineering
Eveslage, Thomas; D'Angelo, Paul
Suggests that publications advisors have sound, practical reasons for addressing ethical decision making. Presents a brief review of the Supreme Court's message in "Hazelwood." Surveys staffs and advisors of award-winning high school newspapers concerning ethical issues facing the student press. Finds that advisors believe ethics to be…
Anker, Thomas Boysen; Sandøe, Peter; Kamin, Tanja
into account the ethical dimensions of health branding: this article presents a conceptual analysis of potential ethical problems in health branding. The analysis focuses on ethical concerns related to the application of three health brand elements (functional claims, process claims, and health symbols...
Full Text Available Most authors believe that ethics is set of moral principles and values which leads a person or a group toward what is good or bad. Ethics sets the standards about what is good, and what is bad in behaving and decision making. Principles are the rules or the laws that create ethical codex.
Sugnet, Chris, Ed.
Representatives of five library integrated system vendors express their views on ethics and the marketplace, emphasizing the need for ethical behavior by librarians, consultants, and vendors. Four sidebars are included: one on the need for customer data rights standards; others containing the codes of ethics of three professional consultants'…
Making ethics relevant to students in a business communications course continues to be a challenge. Classroom practitioners have long noted the difficulties in surmounting the contradictions students sense in business ethics instruction. Furthermore, students often perceive ethics to be largely irrelevant to the skills necessary for success in…
Intended for professionals and others in the field of philanthropy, this book applies ethics and ethical decision-making to fund raising. Its primary aim is to enhance the level of ethical fund raising throughout the nonprofit sector by equipping those involved with frameworks for understanding and taking principled actions and preventing…
Identifies common ethical dilemmas that arise in performance technology consultant-client relationships and the difficulties both parties have in resolving them. Questions of integrity v ethics, legality v ethics, conflict of interest issues, contracts and fee issues, and ownership issues are addressed. (MER)
Medlin, E. Lander
Ethics is defined as a set of guidelines and/or rules for the conduct of individual behavior in an organization or civil society. This ethical code of conduct is intended to guide policies, practices, and decision-making for employees on behalf of the organization. This article explores the importance of ethics, the basis for making ethical…
Full Text Available Five clinical observations in pediatric patients with Bloch — Sulzberger syndrome are presented. The observation had been performed for six months. The differential diagnosis depending on the stage of the disease was done. The article contains variants of treating patients of this category.
Cameron, Robyn Ann; O'Leary, Conor
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…
Goldhagen, Jeffrey; Mercer, Raul; Webb, Elspeth; Nathawad, Rita; Shenoda, Sherry; Lansdown, Gerison
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.
Have, H ten; Ang, T W
The Global Ethics Observatory, launched by the United Nations Educational, Scientific, and Cultural Organization in December 2005, is a system of databases in the ethics of science and technology. It presents data on experts in ethics, on institutions (university departments and centres, commissions, councils and review boards, and societies and associations) and on teaching programmes in ethics. It has a global coverage and will be available in six major languages. Its aim is to facilitate the establishment of ethical infrastructures and international cooperation all around the world. PMID:17209103
Birkvad Bernth, Camilla; Houmøller Mortensen, Kasper; Calles, Mark Benjamin; Wind, Martin; Saalfeldt, Rie
This project seeks to dissect the advent of CSR in business practice from the view of ethics. We perform this by laying out the theories of CSR, business ethics, deontology and utilitarianism. We then use the case studies of Lundbeck, The Body Shop, and British American Tobacco, to dissect some productions of CSR. These are compared to the above mentioned theories on ethics. We conclude that business ethics and CSR are only as good as the ethical base upon which they are built. This projec...
Kidney function in obese adolescents with or without metabolic syndrome in a nationally-representative sample of pediatric population: First report from the Middle East and North Africa: The CASPIAN-III Study
Full Text Available Background : Obesity in accordance with metabolic syndrome (MetS confronts populations at the higher risk of morbidity and mortality of chronic diseases including, chronic kidney diseases (CKD. The renal complication of obesity and MetS has been less debated in young adolescents. The objective of this study was to assess the kidney function in obese adolescents with or without MetS. Materials and Methods: The data used in this study were collected as part of a national study entitled Childhood and Adolescence Surveillance and Prevention of Adult Non-communicable disease Study. The present study was conducted on a sub-sample of 113 obese adolescents (body mass index >95 th percentile aged between 10 years and 16 years selected by convenient sampling from the whole population studied. Anthropometric indexes and blood pressure were examined. A 12-h fasting serum was obtained for each participant to measure blood glucose, lipid profile, quantitative C-reactive protein (hs-CRP, Cystatin-c, urea, and creatinine. Fasting spot urine was collected to determine microalbumin and creatinine. Based on the study findings, participants were assigned into two groups with and without MetS. Results: The mean of microalbuminuria was in similar ranges in two groups and while the mean glomerular filtration rate (GFR calculated by Bokenkamp′s, updated and combined Schwartz′s formulas were significantly lower in MetS + obese group in comparison with obese group. The similar result was not achieved by Filler′s formula. Among MetS components, waist circumference had a correlation with hs-CRP ( P = 0.04; r = 0.15. GFR was calculated based on the Schwartz formula and Cystatin-c formulas had no significant correlation with any MetS components. Conclusion: Our findings suggest that MetS can increase the risk of kidney dysfunction in obese adolescents. More studies are suggested in this regard in the pediatric population.
Rollin, Bernard E
Most veterinarians hold a 'pediatric' rather than 'garage mechanic' view of their function. In recent years, sophisticated medical modalities have allowed veterinarians to keep animals alive, and increased value of companion animals in society has increased demand for such treatment. But whereas humans can choose to trade current suffering for extended life, animals seem to lack the cognitive apparatus required to do so. Thus, veterinarians must guard against keeping a suffering animal alive for too long. Clients may be emotionally tied to the animal and blind to its suffering. Part of the veterinarian's role, therefore, is to lead the client to 'recollect' quality of life issues. A second major role for the veterinarian in treating geriatric or chronically ill animals is control of pain and distress. Unfortunately, pain and distress have historically been neglected in both human and veterinary medicine for ideological reasons. It is ethically necessary to transcend this ideology which leads to both bad medicine and bad ethics.
Addison, Courtney Page
of gene therapy, and the authority of its practitioners. The politics of ethics can also be discerned in practice: the UK research ethics system structures scientific work but cannot account for the various, complex, and on-going ethical dilemmas that patients and practitioners face when undertaking gene....... However, social scientists have yet to devote much attention to this ethically contentious and medically complex field. This project aimed to identify and explore social and ethical factors shaping gene therapy practice in clinical settings. It is based on six months of participant observation in a London...... children’s hospital (the UKCH), thirty-two interviews with key actors in the gene therapy field, and scientific and policy document analysis. One of the main interests of this research is with the politics of ethics. The thesis shows that ‘ethical boundary work’ was central to establishing the credibility...
This article examines the work that goes in to ‘making room’ for ethics, literally and figuratively. It follows the activities of a capacity building Asia-Pacific NGO in training and recognising ethics review committees, using multi-sited field materials collected over 12 months between 2009...... and 2010. Two queries drive this article: First, how are spaces made for ethical review –politically, infrastructurally, materially – as committee members campaign for attention to ethics and access to resources in which to conduct their meetings? Second, how are the limits of ‘local circumstance......’ negotiated during a review of the committee’s work: what does the implementation of standards in the area of ethics look like? I then discuss what standards of ethics practice mean for more fraught questions of the universal in bioethics. Rather than regarding ethics systems as backgrounds to global health...
Devettere, R J
Most contemporary accounts of clinical ethics do not explain why clinicians should be ethical. Those few that do attempt an explanation usually claim that clinicians should be ethical because ethical behavior provides an important good for the patient--better care. Both these approaches ignore the customary traditional reason for being ethical, namely, the good of the moral agent. This good was commonly called 'happiness'. The following article shows how the personal happiness of the moral agent provided a major reason for being ethical in the ancient philosophical and biblical traditions and how it continues to play a role in the more modern rights-based, Kantian and utilitarian theories. This history suggests that the personal happiness of the clinician, rightly understood, is a legitimate and important goal of clinical ethics.
Camargo, Aline; Liu, Li; Yousem, David M
The purpose of this study is to assess medical ethics knowledge among trainees and practicing radiologists through an online survey that included questions about the American College of Radiology Code of Ethics and the American Medical Association Code of Medical Ethics. Most survey respondents reported that they had never read the American Medical Association Code of Medical Ethics or the American College of Radiology Code of Ethics (77.2% and 67.4% of respondents, respectively). With regard to ethics education during medical school and residency, 57.3% and 70.0% of respondents, respectively, found such education to be insufficient. Medical ethics training should be highlighted during residency, at specialty society meetings, and in journals and online resources for radiologists.
Full Text Available Ethics and politics are normally considered domains that do not mix, in fact, domains that have little to do with one another. In this article, I provide four factual fictions that show how at the university, research ethics and politics are intertwined. Politics appears to be used for the sole purpose of constructing and maintaining control over the research process and its products. Ultimately, even ethics reviews of proposed research studies are caught up in the politics of power. URN: urn:nbn:de:0114-fqs0403357
Here, we report a case of Carmi syndrome in a neonate who presented with an uncommon complication of gastric perforation. Ann Pediatr Surg 9:122–123 c 2013 Annals of. Pediatric ... Here, we report these three uncommon conditions, that is, CPA and EB ... membrane resulting from minor mechanical friction or trauma.
... Samuels M, Stevens CA, Berry-Kravis EM, Weese-Mayer DE. PHOX2B mutation-confirmed congenital central hypoventilation syndrome: ... Citation on PubMed Axelrod FB, Chelimsky GG, Weese-Mayer DE. Pediatric autonomic disorders. Pediatrics. 2006 Jul;118( ...
Full Text Available OBJETIVO: Determinar a ocorrência de síndrome de abstinência em crianças internadas em UCI Pediátrica em uso de fentanil e midazolam. MÉTODOS: Avaliadas 36 crianças internadas na UCI Pediátrica do Hospital São Paulo - Universidade Federal de São Paulo, no período de março a setembro de 1997, com idade variando de 5 dias a 22 meses (22 masc : 14 fem que fizeram uso de fentanil e midazolam por mais de 24 horas. Utilizado o Escore Neonatal de Abstinência adaptado por Finnegan que determina a ocorrência de síndrome de abstinência em crianças menores de 2 anos. Escore maior ou igual a 8 é considerado como síndrome de abstinência. Correlacionados a síndrome de abstinência com a dose total acumulada, velocidade de infusão, dose diária e tempo de utilização do fentanil e do midazolam. RESULTADOS: Determinada síndrome de abstinência em 18 (50% das 36 crianças. Aplicado o teste estatístico de Mann Whitney para comparar os grupos com e sem síndrome de abstinência. Dose total acumulada de fentanil (5732.7 ± 5114.91 vs. 624.2 ± 591.2mcg, pPURPOSE: 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 determines the occurrence of abstinence syndrome in was used to children 2 years old or less. Sustain larger or equal for 8 is considered as abstinence syndrome. Correlated the abstinence syndrome with the accumulated total dose, infusion velocity, daily dose and time of use of the fentanyl and midazolam. RESULTS: Certain abstinence syndrome in 18 (50% of the 36 children. Applied Mann Whitney's statistical
Airth-Kindree, Norah M M; Kirkhorn, Lee-Ellen C
We offer an educational innovation called Ethical Grand Rounds (EGR) as a teaching strategy to enhance ethical decision-making. Nursing students participate in EGR-flexible ethical laboratories, where they take stands on ethical dilemmas, arguing for--or against--an ethical principle. This process provides the opportunity to move past normative ethics, that is, an ideal ethical stance in accord with ethical conduct codes, to applied ethics, what professional nurses would do in actual clinical practice, given the constraints that exist in contemporary care settings. EGR serves as a vehicle to translate "what ought to be" into "what is."
Dyck, Murray; Allen, Gary
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.
Full Text Available ... Videos About Us News Physician Resources Professions Site Index A-Z Children's (Pediatric) Nuclear Medicine Children’s (pediatric) ... molecular information. In many centers, nuclear medicine images can be superimposed with computed tomography (CT) or magnetic ...
Afsar, Fatma S; Diniz, Gulden; Aktas, Safiye
Dermatopathology is a subspecialty of pathology and dermatology involving correlation of clinical information with microscopic observations of skin biopsies to provide diagnostic information. Pediatric dermatology is a subspecialty of dermatology for which specific points need to be known for evaluating and managing skin disorders in children. The histopathological approach and other important factors for definitive diagnoses in pediatric dermatopathology are reviewed. Skin diseases in children are not necessarily smaller versions of those that develop in adults and some diaseases may be confined to pediatric age group. An experienced team of dermatology and pathology increases the success of skin biopsies in pediatric dermatology besides the excellent technical skills. The histopathologic findings of skin lesions in children should be evaluated by pediatric pathologists, who have a specific interest for pediatric dermatopathology, in close collaboration with pediatric dermatologists. Sociedad Argentina de Pediatría.
Full Text Available ... patient story here Images × Image Gallery Radiologist and patient consultation. View full size with caption Related Articles and Media General Nuclear Medicine Children's (Pediatric) CT (Computed Tomography) Epilepsy Images related to Children's (Pediatric) Nuclear Medicine ...
... navigate their brain tumor diagnosis. WATCH AND SHARE Brain tumors and their treatment can be deadly so ... Pediatric Central Nervous System Cancers Read more >> Pediatric Brain Tumor Foundation 302 Ridgefield Court, Asheville, NC 28806 ...
Scan for mobile link. Children's (Pediatric) Voiding Cystourethrogram A children’s (pediatric) voiding cystourethrogram uses fluoroscopy – a form of real-time x-ray – to examine a child’s bladder ...
American Pediatric Surgical Association Search for: Login Resources + For Members For Professionals For Training Program Directors For Media For ... Surgical Outcomes Surveys & Results Publications Continuing Education + ExPERT Pediatric Surgery NaT Annual Meeting CME MOC Requirements Residents / ...
Heart surgery - pediatric; Heart surgery for children; Acquired heart disease; Heart valve surgery - children ... Ginther RM, Forbess JM. Pediatric cardiopulmonary bypass. In: ... Care . 5th ed. Philadelphia, PA: Elsevier; 2017:chap 37. LeRoy S, ...
... a protein found in wheat, rye, and barley. Pediatric Celiac Disease If your child has celiac disease, ... physician. Established by the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) Celiac Disease Eosinophilic ...
... Find an ENT Doctor Near You Pediatric Thyroid Cancer Pediatric Thyroid Cancer Patient Health Information News media interested in ... and neck issues, should be consulted. Types of thyroid cancer in children: Papillary : This form of thyroid cancer ...
Infographic explaining NCI-COG Pediatric MATCH, a cancer treatment clinical trial for children and adolescents, from 1 to 21 years of age, that is testing the use of precision medicine for pediatric cancers.
Turner, Helen N
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.
Valenzuela, Carlos Y
Therapeutic abortion is proposed when a pregnancy threatens a woman's life and the fetus is not viable ex utero. As the intention is not to kill the fetus, this action should be named "therapeutic interruption of pregnancy". However, in some cases the fetus directly hampers the mother's health. Thus, the removal of the cause of the disease coincides with killing the fetus. Therapeutic abortion has been proposed for several situations. A) When pregnancy and not the fetus, impairs maternal life (e.g. ovular infection, ectopic pregnancy, decompensation of a preexisting disease or diseases of pregnancy as pre-eclampsia/eclampsia, HELLP and Ballantyne syndromes, choriocarcinoma). B) A risk for maternal survival caused by the embryo or fetal genetic constitution: autoimmune diseases of the mother generated by fetal antigens, some types of eclampsia with or without HELLP syndrome due to an immune or exaggerated inflammatory response of the mother, Ballantyne syndrome associated to eclampsia due to fetal-maternal genetic incompatibility, the classic fetus-maternal genetic incompatibility, embryo or fetus diseases caused by their genomic constitution, mainly hydatidiform mole and the triploid, or fetal cancer. Scientific knowledge and a prudential Medical Ethics are capable to solve most cases.
The Annals of Pediatric Surgery is striving to fill an important niche that provides focus to clinical care, technical innovation and clinical research. The Annals of Pediatric Surgery has the responsibility to serve not only pediatric surgeons in the Middle East and North Africa but also should be an important conduit for scientific ...
Full Text Available ... in the body. jaundice in newborns and older children. epilepsy . location, anatomy and function of the thyroid gland. ... General Nuclear Medicine Children's (Pediatric) CT (Computed Tomography) Epilepsy Images related to Children's (Pediatric) Nuclear Medicine Videos related to Children's (Pediatric) ...
The fields of radiodiagnosis application in pediatrics are considered. The improvement of roentgenologic methods and application of various contrast proparations enable to study and precisely differentiate congenital and acquired diseases. The scope of roentgenology application in pediatrics extends due to differentiation of pediatric specialities. New methods of investigation with decreasing radiation exposure to minimal are realized [ru
Full Text Available ... Resources Professions Site Index A-Z Children's (Pediatric) Nuclear Medicine Children’s (pediatric) nuclear medicine imaging uses small amounts ... of Children's Nuclear Medicine? What is Children's (Pediatric) Nuclear Medicine? Nuclear medicine is a branch of medical imaging ...
Hence, the crux of the matter – ethical issues in examination management! What then do we mean by ethical issues? Ethical Issues. Ethics, according to Collins Concise Dictionary of 21st Century (2001), is a moral principle or a set of moral values held by an individual or group. Succinctly put, ethic is a set of principles that ...
Brey, Philip A.E.
In this essay, a new approach for the ethical study of emerging technology ethics will be presented, called anticipatory technology ethics (ATE). The ethics of emerging technology is the study of ethical issues at the R&D and introduction stage of technology development through anticipation of
Full Text Available The Slovak Association of Social Anthropologists initiated recently a discussion about the ethics in the ethnology, social and cultural anthropology. In January 2009 the association organized the seminar “Ethics in ethnology/social anthropology which brought vivid response in the academic community in Slovakia. The paper will deal with the question which are the most frequent ethic problems in field work such as for example the selection of research topic from the ethic point of view, ethic regulations during the conducting of field work, the protection of respondent’s personal data during the elaboration of data and archiving, the publication of research data etc. The author will inform about approaches and react to the current discussion about the possibilities how to solve the ethic questions in the field work.
Vistisen, Peter; Jensen, Thessa; Poulsen, Søren Bolvig
This paper addresses the challenge of attaining ethical user stances during the design process of products and services and proposes animation-based sketching as a design method, which supports elaborating and examining different ethical stances towards the user. The discussion is qualified...... by an empirical study of Responsible Research and Innovation (RRI) in a Triple Helix constellation. Using a three-week long innovation workshop, U- CrAc, involving 16 Danish companies and organisations and 142 students as empirical data, we discuss how animation-based sketching can explore not yet existing user...... dispositions, as well as create an incentive for ethical conduct in development and innovation processes. The ethical fulcrum evolves around Løgstrup’s Ethical Demand and his notion of spontaneous life manifestations. From this, three ethical stances are developed; apathy, sympathy and empathy. By exploring...
Diebler, C.; Dulac, O.
In this book, a neuroradiologist and a neuropediatrician have combined forces to provide the widest possible knowledge in investigating cranial and cerebral disorders in infancy and childhood. Based on more than 20,000 pediatric CT examinations, with a follow-up time often exceeding ten years, the book aims to bridge interdisciplinary gaps and help radiologists, pediatricians and neurosurgeons solve the various problems of pediatric neuroradiology that frequently confront them. For each disease, the etiology, clinical manifestation, pathological lesions and radiological presentations are discussed, supported by extensive illustrations. Malformative, vascular, traumatic, tumoral, infectious and metabolic diseases are reviewed. Miscellaneous conditions presenting particular symptoms or syndromes are also studied, such as hydrocephalus and neurological complications of leukemia. Contents: Cerebral and cranial malformations; neurocutaneous syndromes; inherited metabolic diseases; infectious diseases - vascular disorders; intracranial tumors; cranial trauma - miscellaneous and subject index
"Demography is, explicitly and not, imbued with an [ethical] content.... As demography involves both public policies and individual choices, the [ethical] slant should be [examined]. Thus, what we have on the one hand is an [ethical] state, which dictates its citizens' personal behaviour and, on the other, a state based on liberty, backed up by three shared values: human rights, pluralism and equality. This article looks at how today these may be reinterpreted when making decisions regarding the population." (EXCERPT)
Henn, N.; Train, E.; Chagnoux, H.; Heinzle, P.; Daubresse, M.; Bret-Rouzaut, N.; Fradin, J.
7 articles in this data sheet, they concern: political stakes and stakes of the industrial petroleum sector towards ethical questions; establishment of associations attending to human and environmental questions; examples of of ethical, environmental and safety policy in an industrialized country (ExxonMobil) and in a developing country (TotalFina); synthesis of the ethical problems that the petroleum industry encounter in industrialized and developing countries; considerations on the communication stakes in petroleum companies with the general public. (O.M.)
Rivero-Serrano, Octavio; Durante-Montiel, Irene
Medical practice has been traditionally ruled by the principles of medical ethics and the scientific aspects that define it. However, today's medical practice is largely influenced by other aspects such as: economic interests, abuse of therapeutics, defensive medicine, unnecessary surgeries and conflicts of interests without excluding alterations in the application of the informed consent, the relation with the pharmaceutical industry, respect of confidentiality, organizational ethics, and the ethical practice that escapes the will of the medical professional.
Ethics can be defined as a reflection on nature and a definition of “the good”. Individuals value qualities and things dissimilarly, most visibly, but they also value their goods in different ways, in different relations to each other, for different reasons, and to different ends. These differences are very applicable to sustainability. In other words, sustainability cannot be achieved without attention to its ethical dimensions. The aim of this research is to examine the ethical aspects of s...
The Constitution and Declaration of Independence simultaneously hold two different views of human nature, an individualist and a collectivist view...the term “ethic” from the perspective of a philosophical ethicist, as opposed to a legal or financial sense. The Joint Ethics Regulation discusses...ethical perspective . 21 In that sense, some could argue that the military ethic should be informed (or at least aware) of the consequentialist school of
Full Text Available This paper makes the review of the literature dedicated to relevant social issues that have been addressed by business practices and the business ethics literature, especially during the past century. The review of practical literature is undertaken from the perspective of the practitioner and demonstrates that the business ethics literature has been lax in the sense that it mostly addresses specific managerial problems and personal ethics within the business environment.
Wasson, Nicholas R; Deer, Jeremy D; Suresh, Santhanam
Pediatric patients with liver dysfunction and renal failure may exhibit many comorbidities. There are often associated congenital syndromes to be taken into account. Liver and renal transplantation offer a solution and substantial improvement in quality of life. Anesthetic management of pediatric liver and renal transplantation has not been well described. There are key differences between adults and children undergoing these procedures, and acknowledgment of some key principles provide a solid foundation to optimize perioperative outcomes. This article provides an overview of the perioperative management and considerations in pediatric patients undergoing liver and renal transplantation. Copyright © 2017 Elsevier Inc. All rights reserved.
Schiffman, Joshua D
Molecular epidemiology is the study of genetic and environmental risk for disease, with much effort centered on cancer. Childhood leukemia occurs in nearly a third of all patients newly diagnosed with pediatric cancer. only a small percentage of these new cases of childhood leukemia are associated with high penetrant hereditary cancer syndromes. Childhood leukemia, especially acute lymphoblastic leukemia, has been associated with a dysregulated immune system due to delayed infectious exposure at a young age. Identical twins with childhood leukemia suggest that acute lymphoblastic leukemia begins in utero and that the concordant presentation is due to a shared preleukemia subclone via placental transfer. Investigation of single nucleotide polymorphisms within candidate genes find that leukemia risk may be attributed to population-based polymorphisms affecting folate metabolism, xenobiotic metabolism, DNA repair, immunity, and B-cell development. More recently, genome-wide association studies for leukemia risk has led investigators to genes associated with B-cell development. When describing leukemia predisposition due to hereditary cancer syndromes, the following 6 categories become apparent on the basis of biology and clinical presentation: (1) genetic instability/DNA repair syndromes, (2) cell cycle/differentiation syndromes, (3) bone marrow failure syndromes, (4) telomere maintenance syndromes, (5) immunodeficiency syndromes, and (6) transcription factor syndromes and pure familial leukemia. understanding the molecular epidemiology of childhood leukemia can affect the treatment and tumor surveillance strategies for these high risk patients and their family members. Copyright © 2016 American Federation for Medical Research.
Ray, Susan L
The purpose of this article is to discuss an external whistleblowing event that occurred after all internal whistleblowing through the hierarchy of the organization had failed. It is argued that an organization that does not support those that whistle blow because of violation of professional standards is indicative of a failure of organizational ethics. Several ways to build an ethics infrastructure that could reduce the need to resort to external whistleblowing are discussed. A relational ethics approach is presented as a way to eliminate the negative consequences of whistleblowing by fostering an interdependent moral community to address ethical concerns.
Full Text Available The ethics is the inner law of the individual. Its application is controlled and sacked by the self-awareness and the surrounding (by ethical codecs. The self-awareness is the inner law produced by everyday life, and is therefore changeable and adaptable to the outer reality. The ethics is a common field of marketing research, within the processes of ethical dilemmas and the marketing research process itself (identified target segments: the public the consumer, the subject ordering marketing research and marketing researchers.
In IT divisions and organizations, the need to execute in a competitive and complex technical environment while demonstrating personal integrity can be a significant personal and organizational challenge. Supplying concrete guidelines for those at an ethical crossroads, Ethics in IT Outsourcing explores the complex challenges of aligning IT outsourcing programs with ethical conduct and standards. This one-stop reference on the ethical structure and execution of IT outsourcing incorporates an easy-to-apply checklist of principles for outsourcing executives and managers. It examines certificatio
Ethics is a branch of philosophy. Its object is the study of both moral and immoral behaviour in order to make well founded judgements and to arrive at adequate recommendations. The Collins English Dictionary provides the following definitions of the word ethic: Ethic: a moral principle or set of moral values held by an individual or group; Ethics(singular): the philosophical study of the moral value of human conduct and of the rules and principles that ought to govern it; Ethics(pleural): a social, religious or civil code of behaviour considered correct, especially that of a particular group, profession or individual; Ethics(pleural): the moral fitness of a decision, course of action, etc. Ethics has a two-fold objective: Firstly it evaluates human practices by calling upon moral standards; it may give prescriptive advice on how to act morally in a specific kind of situation. This implies analysis and evaluation. Sometimes this is known as Normative ethics. The second is to provide therapeutic advice, suggesting solutions and policies. It must be based on well-informed opinions and requires a clear understanding of the vital issues. In the medical world, we are governed by the Hippocratic Oath. Essentially this requires medical practitioners (doctors) to do good, not harm. There is great interest and even furore regarding ethics in radiation protection