In this article we discuss the steps taken by the United States (US) and the European Union (EU) to meet the health care needs of children with rare diseases and suggest possible directions for future endeavors for further improvement. We reviewed 23 reports and nine legislative documents related to pediatric rare diseases and public policy. We assessed the outcome measures of access and satisfaction with medical services by utilizing the surveys done by the European Organization for Rare Diseases -Eurordis (n = 5,963). Comparable surveys were not available in the US. Our analyses of the existing policies and surveys indicate multiple differences between the US and EU. While the US policies seem to be aimed at disease diagnosis and neonatal screening, EU legislators appear to be focusing on access to existing specialized care. However, both systems have struggled with effectively promoting new treatments. Also, while Eurordis surveys have evaluated areas such as the access to medical services, access to social services and satisfaction with the services received in Europe, there are no comparable surveys in the United States. We conclude that better tools are needed to measure the quality of care, needs-assessment and outcome of pediatric rare diseases in both the EU and US. We suggest a better assessment of areas such as access to primary and specialty care, legal advocacy, comfort-care, end-of-life care, social and financial services, psychological support and quality outcome-measures.

Health care professionals who felt comfortable discussing options for end of life care with colleagues also felt more comfortable: initiating a discussion regarding a child's impending death with his/her family (r = 0.42), discussing options for terminal care with a family (r = 0.58), discussing death with families from a variety of ethnic/cultural backgrounds (r = 0.51), guiding parents in developmentally age-appropriate discussions of death with their children (r = 0.43), identifying and seeking advice from a professional role model regarding management concerns (r = 0.40), or interacting with a family following the death of a child (r = 0.51). Among all three disciplines, physicians were more likely to initiate discussions with regards to a child's impending death (F = 13.07; p = 0.007). Health care professionals that received formal grief and bereavement training were more comfortable discussing death. Significance of the results: The results demonstrated that consultation practices are associated with a higher level of comfort in discussing death and dying in pediatrics.

BackgroundAcute ankle injuries are one of the most common reasons for presenting to emergency departments, but only a small percentage of patients – approximately 15% – have clinically significant fractures. However, these patients are almost always referred for radiography. The Ottawa Ankle Rules (OARs) have been designed to reduce the number of unnecessary radiographs ordered for these patients. The objective of this study was to validate the OARs in the Iranian population.MethodsThis prospective survey was done among 200 patients with acute ankle injury from January 2004 to April 2004 in the Akhtar Orthopedics Hospital Emergency Department. Main outcome measures of this survey were: sensitivity, specificity, positive predictive value, negative predictive value, and likelihood ratios (positive and negative) of the OARs.ResultsSensitivity of the OARs for detecting 37 ankle fractures (23 in the malleolar zone and 14 in the midfoot zone) was 100% for each of the two zones, and 100% for both zones. Specificity of the OARs for detecting fractures was 40.50% for both zones, 40.50% for the malleolar zone, and 56.00% for the midfoot zone. Implementation of the OARs had the potential for reducing radiographs by 33%.ConclusionOARs are very accurate and highly sensitive tools for detecting ankle fractures. Implementation of these rules would lead to significant reduction in the number of radiographs, costs, radiation exposure and waiting times in emergency departments.

Residents indicated increased comfort in some areas of pediatric palliative care after the first year of their training. The underlying cause of this increased comfort is unclear at this time. The overall effect of longitudinal palliative care curricula on residents' level of comfort in caring for this population deserves further assessment.

We report a case of a child with Noonan phenotype and incidental radiographic findings of mediastinal neuroblastoma. Recent studies have reported an increased association of Noonan syndrome with some malignancies, and the case we present here is the first reported case to our knowledge of an association of neuroblastoma with Noonan syndrome.

SUMMARY Chronic pain in children is a poorly recognized entity that is challenging to treat and leaves many families frustrated. Often, lack of an identifiable etiology along with the complex biopsychosocial nature of this condition leads to a lengthy diagnostic odyssey and delayed treatment that exacerbates the existing problem. Effective treatment of chronic pain requires a team approach in order to deal with the various aspects of this condition. Combinations of medication along with nonpharmacologic treatments, such as physical therapy, psychological interventions and complementary therapies, are often the most effective ways of treating chronic pain rather than medication alone. Further research is needed to understand the complex biobehavioral processes involved in the development and maintenance of chronic pain. Development of targeted novel therapies as well as comparative studies of existing treatments will help to improve treatment for chronic pain in children.

BACKGROUND: Few studies exist on the ability of standardized preprinted order forms to improve patient care. OBJECTIVE: To examine resident‐perceived effects of introducing a pediatric admission order set (PAOS) on the quality of inpatient care. DESIGN: Cross‐sectional study. SETTING: University of California, Los Angeles (UCLA) Children's Hospital, a nonprofit, tertiary‐care teaching hospital and major referral center with approximately 3,000 admissions per year. PARTICIPANTS: A total of 97 pediatric residents (PL‐1, n = 34; PL‐2, n = 33; and PL‐3, n = 30) who did the vast majority of the inpatient admissions. MEASUREMENTS: Residents were asked to rate the PAOS overall and with respect to 9 specific dimensions using a 5‐point Likert scale. RESULTS: Overall, 89% of respondents approved of the PAOS, 58% reported using it ≥90% of the time, and all said that they would recommend it to their colleagues. Eighty‐four percent thought that it improved inpatient care, and 75% thought that medical errors were reduced. Eighty‐eight percent reported that the PAOS saved time; 93% said it was convenient; and most reported less need for clarification with secretaries (81%) and nurses (82%). In multivariate regression analyses, the only predictor of overall rating was whether the PAOS improved inpatient care (P = 0.04). Improved patient care, meanwhile, was predicted by whether the PAOS was comprehensive (P = 0.01), reduced medical errors (P = 0.01), and required less clarification with nurses (P = 0.01). CONCLUSIONS: A standardized admission order set is a simple, low‐cost intervention that residents believe may benefit patients by reducing medical errors and expediting high‐quality care. Journal of Hospital Medicine 2009;4:90–96. © 2009 Society of Hospital Medicine.

No abstract