Progressive, refractory macrophage activation syndrome as the initial presentation of anti-MDA5 antibody positive juvenile dermatomyositis: a case report and literature review.

BACKGROUND: Macrophage activation syndrome (MAS) is a severe and under-recognized complication of rheumatologic diseases. We describe a patient who presented with rapidly progressive, refractory MAS found to have anti-MDA5 antibody Juvenile Dermatomyositis (JDM) as her underlying rheumatologic diagnosis. CASE PRESENTATION: We describe a 14-year-old female who at the time of admission had a history of daily fevers for 6 weeks and an unintentional sixteen-pound weight loss. Review of systems was significant for cough, shortness of breath, chest pain, headaches, sore throat, muscle aches, rash, nausea, and loss of appetite. An extensive initial workup revealed findings consistent with an autoimmune process. While awaiting results of her workup she had clinical decompensation with multi-organ system involvement including pancytopenias, interstitial lung disease, hepatitis, cardiac involvement, gastrointestinal distension and pain, feeding intolerance, extensive mucocutaneous candidiasis, and neuropsychiatric decline. Due to her decompensation, significant interstitial lung disease, and likely underlying rheumatologic condition she was started on high dose pulse steroids and mycophenolate. An MRI was performed due to her transaminitis and shoulder pain revealing significant myositis. Intravenous immunoglobulin was then initiated. The myositis antibody panel sent early in her workup was significant for anti-MDA5 and anti-SSA-52 antibodies. Despite high dose pulse steroids, mycophenolate, and IVIG, her disease progressed requiring escalating therapies. Ultimately, she responded with resolution of her MAS as well as significant and steady improvement in her feeding intolerance, interstitial lung disease, cardiac dysfunction, myositis, arthritis, and cutaneous findings. CONCLUSIONS: JDM in the pediatric patient is rare, as is MAS. In patients with complex rheumatologic conditions and lack of response to treatment, it is important to continually assess the patient's clinical status with MAS in mind, as this may change the treatment approach. Without proper recognition of this complication, patients can have a significant delay in diagnosis leading to life-threatening consequences.

Stroke code simulation benefits advanced practice providers similar to neurology residents.

BACKGROUND: Advanced practice providers (APPs) are important members of stroke teams. Stroke code simulations offer valuable experience in the evaluation and treatment of stroke patients without compromising patient care. We hypothesized that simulation training would increase APP confidence, comfort level, and preparedness in leading a stroke code similar to neurology residents. METHODS: This is a prospective quasi-experimental, pretest/posttest study. Nine APPs and 9 neurology residents participated in 3 standardized simulated cases to determine need for IV thrombolysis, thrombectomy, and blood pressure management for intracerebral hemorrhage. Emergency medicine physicians and neurologists were preceptors. APPs and residents completed a survey before and after the simulation. Generalized mixed modeling assuming a binomial distribution was used to evaluate change. RESULTS: On a 5-point Likert scale (1 = strongly disagree and 5 = strongly agree), confidence in leading a stroke code increased from 2.4 to 4.2 (p < 0.05) among APPs. APPs reported improved comfort level in rapidly assessing a stroke patient for thrombolytics (3.1-4.2; p < 0.05), making the decision to give thrombolytics (2.8 vs 4.2; p < 0.05), and assessing a patient for embolectomy (2.4-4.0; p < 0.05). There was no difference in the improvement observed in all the survey questions as compared to neurology residents. CONCLUSION: Simulation training is a beneficial part of medical education for APPs and should be considered in addition to traditional didactics and clinical training. Further research is needed to determine whether simulation education of APPs results in improved treatment times and outcomes of acute stroke patients.

Exploring nursing students' decision-making skills whilst in a Second Life clinical simulation laboratory.

AIM: To explore nursing students' decision-making skills through the use of a 3D virtual environment such as Second Life. METHOD: An exploratory qualitative evaluation of the students' experience of learning decision-making skills whilst in a Second Life clinical simulation laboratory. A convenience sample of five third year student nurses entered a simulated world environment where they cared for six patients over 1h. The written communication text from the Second Life scenario was saved into a Microsoft Word document. Additionally a semi-structured tape-recorded one to one interview was conducted immediately after the Second Life simulation in order to explore the students' decision-making skills. RESULTS: The communication text illustrated that the majority of decisions (n=21) were made in response to a situation or a patient request, therefore 'reactive' rather than proactive (n=9). Only one student carried out a vital signs assessment on a newly admitted patient (Willie). The interviews produced two themes, performing decision-making and improving learning. The absence of 'visual cues' such as pre-operative checklists, vital sign observation charts and 'Nil by Mouth' signs may offer a rationale for why students were more reactive. CONCLUSION: Further work is required for students to practice decision-making skills. With further development the innovative 3D virtual worlds such as Second Life could provide this experience.

A review of cooling patients with severe cerebral insult in ICU (Part 1).

A review of the literature was undertaken to determine the existence of any evidence to support cooling strategies used in the ICU. The focus of the review was to examine previous findings on the specific problems of cooling patients with severe cerebral insult. Cooling methods that use external physical cooling strategies as well as the effects of antipyretics and vasoactive drugs were examined. Overall, it would appear that conclusions for practice remain unclear. The findings led to the implementation of a pilot study, the findings of which will be discussed in Part 2.

Cooling strategies for patients with severe cerebral insult in ICU (Part 2).

Critically ill patients who have sustained a severe cerebral insult will be actively cooled should they develop an elevated body core temperature. Patients who require therapeutic hypothermia for neuroprotection may require the same cooling strategies. A literature review suggested limited evidence to support cooling strategies currently used within one intensive care unit. An experimental approach was used to examine the effects of paracetamol and four external cooling strategies on patients with severe cerebral insult It is suggested that paracetamol is effective in reducing body core temperature and that fans may not. However, data obtained from the study of the four external cooling strategies were inconclusive.