A Personalized Diagnostic and Treatment Approach for Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Adults.

OBJECTIVE: We assessed the clinical features and outcomes based on therapeutic options adopted during hospital stay for adult patients with macrophage activation syndrome and secondary hemophagocytic lymphohistiocytosis (MAS/sHLH). METHODS: We conducted a retrospective chart review of all adult patients (age ≥ 18 years) diagnosed with MAS/sHLH at our center between 2010 and 2015. Inclusion criteria for patients were diagnosis of MAS/sHLH during admission and patients meeting at least 5 out of 8 of Henter's criteria or at least 4 out of 6 of the criteria that were tested. RESULTS: Nineteen adult patients with MAS/sHLH met the inclusion criteria from January 2010 to October 2015 (median age 48 years; female 68.4%). Treatment had been personalized, depending on the clinical presentation and course of disease. Majority of the patients received anakinra, cyclosporine, intravenous immunoglobulins (IVIG), and steroids. Fourteen (74%) patients survived, with clinical improvement by the time of discharge. After excluding the three patients with underlying leukemia/lymphoma who opted for palliative care and subsequently died, the survival rate was 88%. CONCLUSION: A modified diagnostic and treatment protocol for adult patients with MAS/sHLH that incorporated graded introduction of medications based on clinical presentation and cytokine profile resulted in the best adult survival rate reported in literature.

Methodological rigor and reporting of clinical practice guidelines in patients with allergic rhinitis: QuGAR study.

BACKGROUND: There are several clinical practice guidelines about the management of allergic rhinitis (AR) being used by clinicians. OBJECTIVE: We sought to assess the methodological rigor and transparency of reporting of clinical practice guidelines for the management of AR. METHODS: We systematically searched MEDLINE, the TRIP database, and professional society Web sites for all guidelines about the management of AR published in English after the year 2000. Four reviewers independently assessed the rigor of development and reporting of included guidelines using the Appraisal of Guidelines for Research and Evaluation II instrument. RESULTS: Our search revealed 432 records, of which 34 full-text articles were assessed for eligibility and 10 fulfilled inclusion criteria. Overall methodological rigor and reporting of guidelines varied from fulfilling most of the Appraisal of Guidelines for Research and Evaluation II criteria to almost none. Across all guidelines, the best reported domain was clarity of presentation, and the least rigorously addressed domain was applicability of guidelines. Agreement beyond chance among the 4 appraisers was fair. CONCLUSIONS: Guideline users should be aware of the difference in the rigor of development and quality of reporting of guidelines about the management of AR. They should choose higher-quality guidelines to use in their practice and teaching. For most reviewed guidelines, there is room for improvement, particularly in the domains of applicability and implementation.

Depression screening optimization in an academic rural setting.

PURPOSE: Primary care plays a critical role in screening and management of depression. The purpose of this paper is to focus on leveraging the electronic health record (EHR) as well as work flow redesign to improve the efficiency and reliability of the process of depression screening in two adult primary care clinics of a rural academic institution in USA. DESIGN/METHODOLOGY/APPROACH: The authors utilized various process improvement tools from lean six sigma methodology including project charter, swim lane process maps, critical to quality tree, process control charts, fishbone diagrams, frequency impact matrix, mistake proofing and monitoring plan in Define-Measure-Analyze-Improve-Control format. Interventions included change in depression screening tool, optimization of data entry in EHR. EHR data entry optimization; follow up of positive screen, staff training and EHR redesign. FINDINGS: Depression screening rate for office-based primary care visits improved from 17.0 percent at baseline to 75.9 percent in the post-intervention control phase (p<0.001). Follow up of positive depression screen with Patient History Questionnaire-9 data collection remained above 90 percent. Duplication of depression screening increased from 0.6 percent initially to 11.7 percent and then decreased to 4.7 percent after optimization of data entry by patients and flow staff. RESEARCH LIMITATIONS/IMPLICATIONS: Impact of interventions on clinical outcomes could not be evaluated. ORIGINALITY/VALUE: Successful implementation, sustainability and revision of a process improvement initiative to facilitate screening, follow up and management of depression in primary care requires accounting for voice of the process (performance metrics), system limitations and voice of the customer (staff and patients) to overcome various system, customer and human resource constraints.

The prevalence of waterpipe tobacco smoking among the general and specific populations: a systematic review.

BACKGROUND: The objective of this study was to systematically review the medical literature for the prevalence of waterpipe tobacco use among the general and specific populations. METHODS: We electronically searched MEDLINE, EMBASE, and the ISI the Web of Science. We selected studies using a two-stage duplicate and independent screening process. We included cohort studies and cross sectional studies assessing the prevalence of use of waterpipe in either the general population or a specific population of interest. Two reviewers used a standardized and pilot tested form to collect data from each eligible study using a duplicate and independent screening process. We stratified the data analysis by country and by age group. The study was not restricted to a specific context. RESULTS: Of a total of 38 studies, only 4 were national surveys; the rest assessed specific populations. The highest prevalence of current waterpipe smoking was among school students across countries: the United States, especially among Arab Americans (12%-15%) the Arabic Gulf region (9%-16%), Estonia (21%), and Lebanon (25%). Similarly, the prevalence of current waterpipe smoking among university students was high in the Arabic Gulf region (6%), the United Kingdom (8%), the United States (10%), Syria (15%), Lebanon (28%), and Pakistan (33%). The prevalence of current waterpipe smoking among adults was the following: Pakistan (6%), Arabic Gulf region (4%-12%), Australia (11% in Arab speaking adults), Syria (9%-12%), and Lebanon (15%). Group waterpipe smoking was high in Lebanon (5%), and Egypt (11%-15%). In Lebanon, 5%-6% pregnant women reported smoking waterpipe during pregnancy. The studies were all cross-sectional and varied by how they reported waterpipe smoking. CONCLUSION: While very few national surveys have been conducted, the prevalence of waterpipe smoking appears to be alarmingly high among school students and university students in Middle Eastern countries and among groups of Middle Eastern descent in Western countries.

Survey instruments used in clinical and epidemiological research on waterpipe tobacco smoking: a systematic review.

BACKGROUND: The primary objective was to systematically review the medical literature for instruments validated for use in epidemiological and clinical research on waterpipe smoking. METHODS: We searched the following databases: MEDLINE, EMBASE, and ISI the Web of Science. We selected studies using a two-stage duplicate and independent screening process. We included papers reporting on the development and/or validation of survey instruments to measure waterpipe tobacco consumption or related concepts. Two reviewers used a standardized and pilot tested data abstraction form to collect data from each eligible study using a duplicate and independent screening process. We also determined the percentage of observational studies assessing the health effects of waterpipe tobacco smoking and the percentage of studies of prevalence of waterpipe tobacco smoking that have used validated survey instruments. RESULTS: We identified a total of five survey instruments. One instrument was designed to measure knowledge, attitudes, and waterpipe use among pregnant women and was shown to have internal consistency and content validity. Three instruments were designed to measure waterpipe tobacco consumption, two of which were reported to have face validity. The fifth instrument was designed to measure waterpipe dependence and was rigorously developed and validated. One of the studies of prevalence and none of the studies of health effects of waterpipe smoking used validated instruments. CONCLUSIONS: A number of instruments for measuring the use of and dependence on waterpipe smoking exist. Future research should study content validity and cross cultural adaptation of these instruments.

Successful use of cyclosporine as treatment for eosinophilic cystitis: a case report.

BACKGROUND: Eosinophilic cystitis is a rare inflammatory disorder characterized by eosinophilic infiltration of all layers of the urinary bladder wall. Due to lack of consensus and potential for side effect from various therapeutic options, treatment of the disease is often challenging. CASE PRESENTATION: A 64-year old woman with hypertensive nephropathy resulting in stage III chronic kidney disease, obstructive sleep apnea, and obstructive lung disease presented with a 4 month history of dysuria, urgency, frequency, and persistent hematuria. Based on eosinophilic infiltration on bladder wall biopsy in the absence of any evidence of infection, malignancy, or immune disorder, she was diagnosed with eosinophilic cystitis. Despite multiple medication regimens, her symptoms persisted, requiring high-dose prednisone with steroid-related side effects. After four months, she was started on cyclosporine, which led to symptomatic improvement and reduction in prednisone dosage. At that time, repeat urine cytology and cystoscopy did not reveal friable tissues or eosinophiluria. CONCLUSION: This case illustrates the utility of using cyclosporine to treat eosinophilic cystitis in adult patient with multiple comorbid conditions.

Latest approaches to the diagnosis and management of food allergies in children.

Adverse food reactions are a challenge for physicians. As the prevalence of this condition rises, it is important that paediatricians and other health care professionals adeptly diagnose this condition. We begin by discussing the relevant points in history and physical examination, then we discuss the recent effective diagnostic tests and techniques available for doctors and patients, along with several management options. Over the last decade, there have been major advancements in this field and novel mechanisms have been proposed which efficiently modulate immune mechanisms involved. Although results are only preliminary, they do however, indicate a promising future for patients with food allergies.

Obesity perceptions and documentation among primary care clinicians at a rural academic health center.

BACKGROUND: Obesity recognition in primary care is important to address the epidemic. We aimed to evaluate primary care clinician-reported documentation, management practices, beliefs and attitudes toward obesity compared to body mass index (BMI) calculation, obesity prevalence and actual documentation of obesity as an active problem in electronic health record in a rural academic center. METHODS: Our target population for previously validated clinician survey was 56 primary care providers working at 3 sites. We used calendar year 2012 data for assessment of baseline system performance for metrics of documentation of BMI in primary care visits, and proportion of visits in patients with obesity with obesity as a problem. Standard statistical methods assessed the data. RESULTS: Survey response rate was 91%. Average age of respondents was 48.9 years and 62.7% were females. 72.5% clinicians reported having normal BMI. The majority of clinicians reported regularly documenting obesity as an active problem, and utilized motivational interviewing and basic good nutrition and healthy exercise. Clinicians identified lack of discipline and exercise time, access to unhealthy food and psychosocial issues as major barriers. Most denied disliking weight loss discussion or patients taking up too much time. In 21,945 clinic visits and 11,208 annual preventive care visits in calendar year 2012, BMI was calculated in 93% visits but obesity documentation as an active problem only 27% of patients meeting BMI criteria for obesity. CONCLUSIONS: Despite high clinician-reported documentation of obesity as an active problem, actual obesity documentation rates remained low in a rural academic medical center.

End-of-life care beliefs among Muslim physicians.

OBJECTIVE: Physicians' religiosity affects their approach to end-of-life care (EOLC) beliefs. Studies exist about end-of-life care beliefs among physicians of various religions. However, data on Muslim physicians are lacking. This study explores the beliefs centering on aspects of end-of-life care among Muslim physicians in the US and other countries. DESIGN: A 25 item, online survey was created and distributed via Survey Monkey®. The survey was targeted toward Muslim physicians in the US and other countries. RESULTS: A total 461 Muslim physicians responded to our survey. The primary end point was if the Muslim physicians thought that making a patient DO NOT RESUSCITATE (DNR) is allowed in Islam?. Nearly 66.8 % of the respondents replied yes as compared to 7.38 % of the respondents who said no. Country of origin, country of practice, and if physicians had talked about comfort care in the past had the most impact on the yes vs. no response (p=0.0399, p=0.0092 and 0.0023 respectively). CONCLUSION: Muslim physicians' beliefs on EOLC issues are affected more by the area of practice, country of origin and previous experience in talking about comfort care than the religious beliefs.

Sleep duration and risk of lung cancer in the physicians' health study.

BACKGROUND: Lung cancer is the most common cancer and cancer related cause of death worldwide. However, the association between sleep duration and incident lung cancer has not been investigated in a prospective cohort study. METHODS: We prospectively examined the association between sleep duration and incident lung cancer in a cohort of 21,026 United States (US) male physicians. Self-reported sleep duration was ascertained during 2002 annual follow-up questionnaire. Incident lung cancer was ascertained through yearly follow-up questionnaires. Cox regression was used to estimate relative risk of incident lung cancer. RESULTS: The average age at baseline was 68.3±8.8 yr. During a mean follow up of 7.5 (±2.2) yr, 150 cases of lung cancer occurred. Using 7 h of sleep as the reference group, multivariable adjusted hazard ratios (95%CI) for lung cancer were 1.18 (0.77-1.82), 1.0 (ref), and 0.97 (0.67-1.41) from lowest to the highest category of sleep duration (P for quadratic trend 0.697), respectively. In a secondary analysis, smoking status did not modify the sleep duration-lung cancer association (P=0.78). There was no evidence for an interaction between sleep duration and sleep apnea on the risk of lung cancer either (P=0.65). CONCLUSIONS: Our data failed to show a higher risk of lung cancer in association with altered sleep duration among US male physicians.

Translating 10 lessons from lean six sigma project in paper-based training site to electronic health record-based primary care practice: challenges and opportunities.

Lean Six Sigma is a well-proven methodology to enhance the performance of any business, including health care. The strategy focuses on cutting out waste and variation from the processes to improve the value and efficiency of work. This article walks through the journey of "green belt" training using a Lean Six Sigma approach and the implementation of a process improvement project that focused on wait time for patients to be examined in an urban academic primary care clinic without requiring added resources. Experiences of the training and the project at an urban paper-based satellite clinic have informed the planning efforts of a data and performance team, including implementing a 15-minute nurse "pre-visit" at primary care sites of an accountable care organization.