Early chemotherapeutic intervention to avoid thyroidectomy in pediatric Langerhans cell histiocytosis with thyroid involvement accompanying tracheal stenosis: a report of two cases.

Thyroid involvement is rare in pediatric Langerhans cell histiocytosis (LCH). It may cause airway narrowing, leading to acute-onset respiratory distress. Severe cases may require emergent surgical interventions such as thyroidectomy, which should be avoided in children due to higher rates of complication, particularly in infancy. There is currently no consensus on the indications for surgical treatment in LCH with thyroid involvement. In this report, we describe the cases of two children who presented with tracheal stenosis caused by thyroid LCH, both of which were successfully treated by early induction of chemotherapy, and one of which was also treated for a shorter duration. Mutation analysis detected in-frame deletions of BRAF exon 12 in both cases. These cases suggest that timely diagnosis and administration of chemotherapy may alleviate severe airway obstruction and reduce the need for thyroidectomy in pediatric patients with thyroid LCH.

Translation and validation of the Japanese version of the measure of moral distress for healthcare professionals.

OBJECTIVES: Moral distress occurs when professionals cannot carry out what they believe to be ethically appropriate actions because of constraints or barriers. We aimed to assess the validity and reliability of the Japanese translation of the Measure of Moral Distress for Healthcare Professionals (MMD-HP). METHODS: We translated the questionnaire into Japanese according to the instructions of EORTC Quality of Life group translation manual. All physicians and nurses who were directly involved in patient care at nine departments of four tertiary hospitals in Japan were invited to a survey to assess the construct validity, reliability and factor structure. Construct validity was assessed with the relation to the intention to leave the clinical position, and internal consistency was assessed with Cronbach's alpha. Confirmatory factor analysis was conducted. RESULTS: 308 responses were eligible for the analysis. The mean total score of MMD-HP (range, 0-432) was 98.2 (SD, 59.9). The score was higher in those who have or had the intention to leave their clinical role due to moral distress than in those who do not or did not have the intention of leaving (mean 113.7 [SD, 61.3] vs. 86.1 [56.6], t-test p < 0.001). The confirmatory factor analysis and Cronbach's alpha confirmed the validity (chi-square, 661.9; CMIN/df, 2.14; GFI, 0.86; CFI, 0.88; CFI/TLI, 1.02; RMSEA, 0.061 [90%CI, 0.055-0.067]) and reliability (0.91 [95%CI, 0.89-0.92]) of the instrument. CONCLUSIONS: The translated Japanese version of the MMD-HP is a reliable and valid instrument to assess moral distress among physicians and nurses.

End-of-life care in the pediatric intensive care unit: Survey in Japan.

BACKGROUND: End-of-life (EOL) care is an important topic in critical care medicine, but EOL discussions with families can be difficult and stressful for intensivists. The aim of this study was to clarify the current practices and barriers facing pediatric intensive care unit (PICU) EOL care and to identify the requisites for excellent PICU EOL care in Japan. METHODS: A survey was conducted in 29 facilities across Japan in 2016. The questionnaire consisted of 19 multiple-choice questions and one open-ended question. RESULTS: Twenty-seven facilities responded to the survey. Only 19% had educational programs on EOL care for fellows or residents. Although 21 hospitals (78%) had a multidisciplinary palliative care team, only eight of these teams were involved in EOL care in PICUs. Mental health care for health-care providers provided by a psychiatrist was rare (4%). The free comments were categorized as individual, team, environment, legal/ethics, or culture. Commonly raised individual issues included "lack of experience and knowledge about EOL care", "fear of making the decision to end care", and "reluctance to be involved in EOL care because of its complex process". Team issues included "insufficient frequency of conferences" and "non-multidisciplinary approach". Legal and ethics issues were "lack of legal support" and "fear of lawsuits". CONCLUSIONS: This study is the first to investigate the current conditions and barriers in PICU EOL care in Japan. Most of the facilities involved were not satisfied with current practices. A need was identified for relevant educational programs, as well as the importance of multidisciplinary and legal support.

Simulation-based training for determination of brain death by pediatric healthcare providers.

BACKGROUND: Low competency for determination of brain death (BD) and unfamiliarity with Japanese BD (JBD) criteria among pediatricians were highlighted in previous nationwide studies. Because the JBD criteria were amended in 2010 to allow organ donation from pediatric brain-dead donors, we created a 2-day training course to assess knowledge and improve skill in the determination and diagnosis of pediatric BD. METHODS: The course consisted of two modules: a multistation round session and a group discussion session, and was bookended by a before and after 20-question test. In the multistation round session, participants rotated between stations staffed by expert faculty members. For hands-on skill development, we used the Sim Junior 3G™ simulation mannequin (Laerdal Medical, Wappingers Falls, NY, USA) for structured simulations. In the group discussion session, we implemented simulation-based role playing to practice decision making in prepared scenarios of complicated clinical situations. We investigated the participants' impressions of the course by self-scoring and questionnaires. RESULTS: Of 147 pediatric healthcare providers from multiple specialties who participated in this course, 145 completed the entire process. The course was evaluated in three aspects with self-scoring and questionnaires: (1) value (4.58 ± 0.64; range 1-5); (2) time schedule (2.40 ± 0.61; range 1-3); and (3) difficulty (2.89 ± 0.43; range 1-5). Finally, participants scored the entire course program (9.64 ± 1.69; range 1-11). Various positive feedbacks were obtained from a total of 93 participants. Post-test scores (83.6 %) were significantly higher than pre-test scores (62.9 %). CONCLUSION: This simulation-based course represents an effective method to train pediatric healthcare providers in determining BD in Japan and may improve baseline knowledge of BD among participants.

Cyclooxygenase inhibition in ventilator-induced lung injury.

BACKGROUND: We tested the hypothesis that inhibition of cyclooxygenase (COX) attenuates in vivo ventilator-induced lung injury (VILI) in a prospective, randomized laboratory investigation in a university-affiliated laboratory. Adult male rats were anesthetized and randomized with or without nonselective COX inhibition (ibuprofen) and were subjected to injurious mechanical ventilation (positive end-expiratory pressure = 0; peak inspiratory pressure = 21 mm Hg). METHODS: We investigated the profile of VILI (respiratory mechanics, cytokines, eicosanoids), expression of COX enzymes, and activation of nuclear factor (NF)-κB in ibuprofen- versus vehicle-treated animals. Injurious ventilation caused lung injury (i.e., decrement in compliance, tissue edema, and elevated inflammatory cytokines, eicosanoids, and COX-2). RESULTS: Pretreatment with ibuprofen that effectively inhibited eicosanoid synthesis and COX-2 activity increased survival and attenuated lung edema and decrement in respiratory mechanics. Ibuprofen had no modulatory effect on ventilator-induced activation of NF-κB or inflammatory cytokines (tumor necrosis factor-α, interleukin [IL]-1ß, IL-6, GRO/KC [growth-related oncogene/keratinocyte chemoattractant]). COX activity seems important in the pathogenesis of VILI in the in vivo rat. Inhibition of COX provides significant protection (i.e., survival, pulmonary function) in VILI, but without affecting levels of important mediators (tumor necrosis factor-α, IL-1ß, IL-6, GRO/KC) or activation of NF-κB. CONCLUSIONS: These data confirm that nonselective COX inhibition provides partial protection against VILI and that the NF-κB signaling pathway is not exclusively eicosanoid dependent. Studies of COX inhibition in ventilator-associated lung injury might benefit from multimodal targeting that includes a comprehensive focus on inflammatory cytokines and NF-κB.