Impact of Task-Oriented Role Assignment on Neonatal Resuscitation Performance: A Simulation-Based Randomized Controlled Trial.

OBJECTIVE: Task-oriented role assignment (TORA) is the assignment of a specific role, a list of tasks, and a location to stand to each resuscitation team member. We performed this study to examine the impact of TORA training during a Neonatal Resuscitation Program (NRP) course on neonatal resuscitation team technical performance and behavioral skills. STUDY DESIGN: Participants were cluster randomized into either a standard NRP course (control) or an NRP course with TORA training. Both technical and behavioral skills were evaluated using standardized simulations. The simulations were video recorded and then scored by two blinded reviewers using previously validated instruments. RESULTS: A total of 65 participants (62 resident physicians and 3 nurses) participated in 108 simulations (48 controls, 60 TORA). Technical skill scores were similar between the two groups (control = 77.6% [±8.4] vs. TORA = 78.1% [±8.1]; p = 0.74). The TORA group had higher behavior skill scores in 8 of the 10 NRP key behavioral skills and had higher overall behavioral skill scores (control = 30.1 [±7.2] vs. TORA = 34.9 [±4.8]; p < 0.001). CONCLUSION: In resuscitation teams comprising mostly resident physicians, TORA training resulted in better behavioral skills during simulated neonatal resuscitation. Further study on the impact of TORA training on actual resuscitation performance in interdisciplinary teams is warranted.

Development of Pulmonary Hypertension During Treatment with Diazoxide: A Case Series and Literature Review.

Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in infancy. The mainstay of medical management for CHI is diazoxide. Diazoxide inhibits insulin release from the pancreas, but also causes smooth muscle relaxation and fluid retention so it is typically given with chlorothiazide. In July 2015, the FDA issued a drug safety communication warning that pulmonary hypertension (PH) had been reported in 11 infants being treated with diazoxide and that the PH resolved with withdrawal of diazoxide. All three of the cases in our hospital were admitted to the neonatal intensive care unit (NICU) for hypoglycemia. All patients received thorough radiologic and laboratory evaluations related to their diagnosis of CHI. All initially improved when diazoxide was initiated. Case 1 and case 3 were discharged from the NICU on diazoxide and chlorothiazide. Case 2 developed pulmonary hypertension while still in the NICU days after an increase in diazoxide dosing. Case 1 presented to the emergency room in respiratory distress shortly after discharge from the NICU with evidence of PH and heart failure. Case 3 presented to the emergency room after 2 weeks at home due to a home blood glucose reading that was low and developed PH and heart failure while an inpatient. Discontinuation of diazoxide led to resolution of all three patients' PH within approximately one week. The experience of our hospital indicates that pulmonary hypertension may be more common than previously thought in infants taking diazoxide. It is unclear if these symptoms develop slowly over time or if there is some other, as yet undescribed, trigger for the pulmonary hypertension. Our hospital's experience adds to the body of evidence and suggests these infants may benefit from more surveillance with echocardiography.

Diagnostic journey for individuals with fibrous dysplasia / McCune albright syndrome (FD/MAS).

BACKGROUND: Reducing delayed diagnosis is a significant healthcare priority for individuals with rare diseases. Fibrous Dysplasia/ McCune Albright Syndrome (FD/MAS) is a rare bone disease caused by somatic activation mutations of NASA. FD/MAS has a broad clinical phenotype reflecting variable involvement of bone, endocrine and other tissues, distribution and severity. The variable phenotype is likely to prolong the diagnostic journey for patients further. AIM: To describe the time from symptom onset to final diagnosis in individuals living with FDMAS. METHODS: We used the UK-based RUDY research database ( www.rudystudy.org ), where patients self-report their diagnosis of FD/MAS. Participants are invited to complete the diagnostic journey based on the EPIRARE criteria. RESULTS: 51 individuals diagnosed with FD/MAS were included in this analysis. Among them, 70% were female, and the median age was 51.0 years (IQR 34.5-57.5]. 12 (35%) individuals reported McCune Albright Syndrome, 11 (21.6%) craniofacial and 11(21.6%) for each of poly- and mono-ostotic FD and 6 (11.8%) did not know their type of FD/MAS. Pain was the commonest first symptom (58.8%), and 47.1% received another diagnosis before the diagnosis of FD/MAS. The median time to final diagnosis from the first symptom was two years with a wide IQR (1,18) and range (0-59 years). Only 12 (23.5%) of individuals were diagnosed within 12 months of their first symptoms. The type of FD/MAS was not associated with the reported time to diagnosis. Significant independent predictors of longer time to final diagnosis included older current age, younger age at first symptom and diagnosis after 2010. CONCLUSION: Individuals with FDMAS have a variable time to diagnosis that can span decades. This study highlights the need for further research on how to improve diagnostic pathways within Orthopaedic and Ear, Nose and Throat (ENT)/Maxillofacial services. Our data provides a baseline to assess the impact of novel NHS diagnostic networks on reducing the diagnostic odyssey.

Simulation versus live tissue training randomised trial for ECMO proficiency: is one better than the other?

Introduction: Extracorporeal membrane oxygenation (ECMO) is a classic low-volume high-risk procedure that requires just in time and/or refresher training through animal or simulation modalities. This manuscript evaluated the performance of ECMO personnel trained with both modalities to determine which is better suited for ECMO skills training. Methods: Participants (physicians, nurses and respiratory/medical technicians) completed a series of ECMO scenarios with synthetic tissue cannulation task trainer as well as a live tissue model. Objective performance quality was based on task completion using a validated ECMO skills assessment tool. Results: Thirty-eight individuals completed this study. Participants completed individual scenario tasks 3 min faster using the simulator (26 min vs 29 min; p=0.03). No differences were seen in percentage of individual tasks completed. In the group scenarios, participants completed a higher percentage of critical tasks using the simulator (97%) versus the animal model (91%; p=0.05), but no differences were seen in task completion times. Additionally, no differences were seen in either lab-based or participants' prelab cognitive scores. Conclusions: Regardless of their self-assessment or experience, participants' objective performances were similar among both animal and simulation labs. Task completion times were quicker with simulation model. The distinction between simulation versus animal model may be less important as both demonstrate benefit in development of and/or maintaining skill competency. In the era of questioning the need for and costs of live tissue training, expanding the role of simulation may achieve similar training goals.

Rare musculoskeletal diseases in adults: a research priority setting partnership with the James Lind Alliance.

BACKGROUND: Osteogenesis imperfecta, fibrous dysplasia/McCune-Albright syndrome and X-linked hypophosphatemia are three rare musculoskeletal diseases characterised by bone deformities, frequent fractures and pain. Little high-quality research exists on appropriate treatment and long-term management of these conditions in adults. This is further worsened by limited research funding in rare diseases and a general mismatch between the existing research priorities and those of the patients. This partnership adopted the James Lind Alliance approach to identify the top 10 research priorities for rare musculoskeletal diseases in adults through joint patient, carer and healthcare professional collaboration. RESULTS: The initial survey for question collection recruited 198 respondents, submitting a total of 988 questions. 77% of the respondents were patients with a rare musculoskeletal disease. Following out-of-scope question exclusion, repeating query grouping and scientific literature check for answers, 39 questions on treatment and long-term management remained. In the second public survey, 220 respondents, of whom 85% were patients with a rare musculoskeletal disease, their carers, relatives or friends, prioritised these uncertainties, which allowed selection of the top 25. In the last stage, patients, carers and healthcare professionals gathered for a priority setting workshop to reach a consensus on the final top 10 research priorities. These focus on the uncertainties surrounding appropriate treatment and holistic long-term disease management, highlighting several aspects indirect to abnormal bone metabolism, such as extra-skeletal symptoms, psychological care of both patients and their families and disease course through ageing. CONCLUSIONS: This James Lind Alliance priority setting partnership is the first to investigate rare bone diseases. The priorities identified here were developed jointly by patients, carers and healthcare professionals. We encourage researchers, funding bodies and other stakeholders to use these priorities in guiding future research for those affected by rare musculoskeletal disorders.

Development of In-situ Simulation Lab for Training Gynecology Residents in Basic Laparoscopic and Hysteroscopic Operative Skills.

INTRODUCTION: Mounting evidence suggests that practice on simulators leads to improved operative skills and patient safety. With restrictions on resident work hours resulting in less exposure to procedures, simulation is the key to developing operative skills during residency and beyond. Residency programs struggle with implementing a simulation program due to timing and availability of residents. Despite having a large centralized simulation space at our institution, we identified lack of dedicated gynecologic simulation curriculum and simulator accessibility as our greatest barriers to utilizing simulation training in gynecology resident education. We sought to design a space within the resident work area dedicated to gynecologic simulation training with specific curriculum and objectives for each work station based on residency year level. METHODS: We created four workstations in a room within the Ob/Gyn clinic, in close proximity to the resident offices. Two virtual reality simulators, the LapVR (CAE, Montreal, Canada) ($84,996.00) and Simbionix Hystsim (3D Systems, (formerly Simbionix), CO, USA) ($95,741.10), were acquired from our institution's simulation center and placed in this training space to allow for enhanced resident access. The two other work stations consisted of an FLS trainer box and monitor ($1580) and another low fidelity laparoscopic box trainer and monitor ($450). Specific objectives for each station with corresponding evaluation checklists were written for each residency year level. Dedicated time to meet the written objectives was given to residents each week during their benign gynecology rotation. Supervision and assistance with task completion was provided by staff mentors assigned during those shifts. RESULTS: Residents who had this simulation lab available to them during their gynecology rotation participated in a minimum of seven hours of simulation time in addition to the time they spent on their own in the lab. These residents felt this was a meaningful increase in the amount of laparoscopic and hysteroscopic simulation exposure by having access to this in-situ GYN Simulation Training Laboratory with a defined gynecologic curriculum. Multiple staff members also took advantage of the simulation lab to practice their skills as well. CONCLUSIONS: We created an in-situ Gyn Simulation Training Lab that allowed for both improved accessibility by the residents and ease of implementation of simulation curriculum into pre-existing resident didactic time. It is our opinion that the time residents spend engaged in surgical simulation will improve surgical skills and confidence thereby enhancing patient safety. Additionally, the creation of this in situ simulation lab assists in meeting the Accreditation Council for Graduate Medical Education (ACGME) requirements for incorporation of simulation into OB/GYN resident education.

Germline p53 mutation in a Micronesian child with adrenocortical carcinoma and subsequent osteosarcoma.

In 1990, an 18-month-old Micronesian girl was initially diagnosed with a right adrenocortical carcinoma. More than a decade later (2003), she was diagnosed with metastatic osteosarcoma with the primary in her right proximal fibula. Given this child's remarkable history of malignancy, she underwent testing for a genetic mutation that is associated with increased cancer formation. One such cancer syndrome is called Li-Fraumeni syndrome where approximately 70% of patients carry a genetic mutation in the p53 tumor suppressor gene. Patients with LFS are at risk for developing cancers of the breast, soft tissues, brain, bone, adrenal gland, and blood cells. Mutational analysis of our patient did reveal the presence of a germline mutation of the p53 tumor suppressor gene. She was found to have a base pair change (A-->C) at nucleotide 394 resulting in a lysine to glutamine amino acid change at codon 132 (K132Q), which remarkably has never been described in association with either adrenocortical carcinoma or osteosarcoma.

Ebola Virus Disease Simulation Case Series: Patient With Ebola Virus Disease in the Prodromal Phase of Illness (Scenario 1), the "Wet" Gastrointestinal Phase of Illness (Scenario 2), and the Late, Critically Ill Phase of Disease (Scenario 3).

INTRODUCTION: As part of an international response to the Ebola virus disease (EVD) outbreak, the US Department of Defense has deployed thousands of personnel to help train and augment international health care workers. The transmission risk of this deadly virus to health care workers has been extreme, demonstrating the importance of safe practices while caring for these patients. Medical simulation training is well recognized as an integral component for disease outbreak preparedness. Therefore, the US Government created a program of instruction that outlines a formalized EVD training program, using high-fidelity simulation, which projects both an understanding of the disease and its transmission risks. METHODS: Two 5-day training courses were established to provide training to the 65-member Department of Defense Ebola Response Team, which would be activated during a stateside Ebola outbreak. This training consisted of Ebola-specific protocols, personal protective equipment familiarization, and scenario-based certification for physicians, nurses, and public health trainers. Simulation was used to replicate the work environment inside an Ebola treatment unit. RESULTS: Three comprehensive clinical scenarios covering a wide spectrum of EVD presentations were designed around details of published cases to provide the most realistic and relevant EVD training available. The authors conducted 10 iterations of the 3 EVD clinical scenarios totaling more than 1100 hours of simulation training. CONCLUSIONS: Quality practical exercises to include specialized task performance and collective teamwork training relied heavily on dedicated facilities and realistic medical simulation resulting in valuable lessons learned. In future iterations, these characteristics would be imperative to a successful training course.

Heteroleptic copper dipyrromethene complexes: synthesis, structure, and coordination polymers.

The synthesis of neutral [Cu(dpm)2] and [Cu(dpm)(acac)] (dpm = dipyrromethene, acac = acetylacetonato) complexes is presented. The formation of the asymmetric metal complexes was monitored by electronic absorption and infrared spectroscopy. Two of the complexes investigated, containing pyrdpm ligands (pyrdpm = pyridyldipyrromethene), form 1-dimensional coordination polymers. The coordination polymers formed by these complexes have been characterized by single-crystal X-ray diffraction, differential scanning calorimetry, and thermogravimetric analysis. The complexes possess square pyramidal coordination geometries with the apical position occupied by the meso-pyridyl donor of a neighboring complex in the crystal lattice. The features of these coordination complexes that facilitate formation of extended solids have been probed. Symmetric [Cu(pyrdpm)2] complexes are unable to form coordination solids due to steric hindrance at the metal center. Use of cyano donors in complexes such as [Cu(cydpm)(acac)] (cydpm = cyanodipyrromethene) in lieu of pyridyl donors also fail to form network solids. Through these systematic studies, both the basic coordination chemistry of these complexes and the fundamental design requirements for synthesizing this novel class of coordination polymers have been defined.