Association between obesity paradox in the all-cause mortality among patients with cardiac resynchronization therapy device.

BACKGROUND: Recent studies have demonstrated an obesity paradox, where obese patients with cardiovascular disease have a better outcome compared to those with normal weight. However, the effect of obesity and body mass index (BMI) on the outcome of patients with cardiac resynchronization therapy (CRT) devices remains unclear. The current study aims to investigate this relationship using all available published data. METHODS: We systematically reviewed studies from Medline and EMBASE databases from inception to January 2024. Eligible studies must investigate the association between BMI status and all-cause mortality in individuals with CRT devices. Relative risk (RR) or hazard ratio (HR) and 95% CIs were retrieved from each study and combined using the generic inverse variance method. RESULTS: A total of 12 cohort studies were included in the meta-analysis. Pooled analysis showed that overweight and obesity patients had lower all-cause mortality compared to those with normal body weight with the pooled risk ratios (RR) for overweight of 0.77 (95% CI 0.69-0.87, I2 47%) and for obesity of 0.81 (95% CI 0.67-0.97, I2 59%). Conversely, the underweight exhibited higher all-cause mortality than the group with normal weight, with a pooled RR of 1.37 (95% CI 1.14-1.64, I2 0%). Additionally, higher BMI as continuous data was associated with decreased all-cause mortality, with a pooled HR of 0.94 (95% CI 0.89-0.98, I2 72%). CONCLUSIONS: The pooled analyses observed an obesity paradox in patients with CRT, where overweight and obesity were associated with reduced all-cause mortality, while underweight individuals exhibited higher all-cause mortality. Further research is necessary to investigate the underlying mechanisms and their implications for clinical practice.

Hidradenitis Suppurativa and Risk of Coronary Artery Disease: A Systematic Review and Meta-Analysis.

Background: Patients with hidradenitis suppurativa (HS) may have a higher risk of coronary artery disease (CAD) due to the excessive inflammatory burden. However, data on this association is still relatively limited. Aims: To investigate the association between HS and risk of prevalent and incident CAD by combining result from all available studies using systematic review and meta-analysis technique. Materials and Methods: Potentially eligible studies were identified from Medline and EMBASE databases from inception to November 2021 using search strategy that comprised of terms for 'hidradenitis suppurativa' (HS) and 'coronary artery disease' (CAD). Eligible study must be cohort study that consisted of one cohort of patients with HS and another cohort of individuals without HS. The study must report incidence or prevalence of CAD in both groups. The retrieved point estimates with standard errors from each study were summarized into pooled result using random-effect model and generic inverse variance method. Meta-analyses of the prevalent and incident CAD were conducted separately. Results: A total of 876 articles were identified. After two rounds of independent review by three investigators, seven cohort studies (four incident studies and three prevalent studies) met the eligibility criteria and were analysed in the meta-analyses. The meta-analysis found a significantly elevated risk of both incident and prevalent CAD in patients with HS compared to individuals without psoriasis with the pooled risk ratio of 1.38 (95% CI, 1.21-1.58; I2 83%) and 1.70 (95% CI, 1.13-2.57; I2 89%), respectively. Limitations: Limited accuracy of diagnosis of HS and CSD as most included studies relied on diagnostic codes and high between-study statistical heterogeneity. Conclusions: The current systematic review and meta-analysis found a significantly increased risk of both prevalent and incident CAD among patients with HS.

Response to Acthar Gel in sarcoidosis uveitis: A prospective open label study.

PURPOSE: To investigate the response to Acthar Gel® in patients with moderate to severe sarcoidosis uveitis. METHODS: This is a prospective open-label study that enrolled patients with moderate to severe sarcoidosis uveitis to receive 80 units daily of Acthar Gel for ten days followed by maintenance treatment with 80 units twice weekly. The primary outcome was the proportion of patients meeting at least one of the following variables 1) improved visual acuity, 2) resolution of intraocular inflammation, 3) ability to taper ocular or oral steroids by at least 50% or 4) reduction of cystoid macular edema, with no worsening of any single measure and no need for additional sarcoidosis therapies at 24 weeks. RESULTS: A total of nine patients were enrolled in the study. Four patients completed the full 24-week course of Acthar Gel, and three of these met the primary endpoint. Among the five patients who did not complete the 24-week course of treatment, four discontinued the treatment due to worsening ocular inflammation. One patient discontinued treatment due to severe adverse effects. The most common adverse effects were fluid retention (77%), insomnia (44%), hypertension (44%) and hyperglycemia (44%). CONCLUSIONS: We observed a clinical response to Acthar Gel in some patients with moderate to severe sarcoidosis uveitis, but a substantial proportion either failed to respond or did not tolerate the therapy. These observations may serve as preliminary data for controlled trials of Acthar Gel, but they do not support its role prior to failure of other agents.

Anorexia nervosa and adrenal hormones: a systematic review and meta-analysis.

OBJECTIVE: Anorexia nervosa is a primary psychiatric disorder characterized by self-induced negative energy balance. A number of hormonal responses and adaptations occur in response to starvation and low body weight including changes in adrenocortical hormones. Our objective was to systematically review adrenocortical hormone levels in anorexia nervosa. DESIGN/METHODS: We searched MEDLINE and EMBASE for studies that reported at least one adrenocortical hormone, including dehydroepiandrosterone (DHEA), DHEA-sulphate (DHEA-S), progesterone, 17-hydroxyprogesterone, pregnenolone, cortisol (serum, urine, cerebrospinal fluid, and hair sample), aldosterone, androstenedione, and testosterone in patients with anorexia nervosa and normal-weight healthy controls from inception until October 2021. Means and standard deviations for each hormone were extracted from the studies to calculate a mean difference (MD). A pooled MD was then calculated by combining MDs of each study using the random-effects model. RESULTS: We included a total of 101 studies with over 2500 females with anorexia nervosa. Mean cortisol levels were significantly higher in anorexia nervosa as compared to normal-weight controls for multiple forms of measurement, including morning cortisol, 12-hour and 24-hour pooled serum cortisol, 24-hour urine cortisol, and after an overnight dexamethasone suppression test. In contrast, mean serum total testosterone and DHEA-S levels were significantly lower among patients with anorexia nervosa. CONCLUSIONS: Women with anorexia nervosa have higher cortisol levels and lower DHEA-S and testosterone levels compared to women without anorexia nervosa. This finding is important to consider when evaluating low-weight women for disorders involving the adrenal axis, especially Cushing's syndrome.

Increased Risk of Systemic Lupus Erythematosus in Patients with Chronic Urticaria: A Systematic Review and Meta-analysis.

Introduction: The association between systemic lupus erythematosus (SLE) and chronic urticaria (CU) has been suggested in the literature although the amount of evidence is still relatively limited. We aimed to combine all available studies on this association using systematic review and meta-analysis technique. Methods: Potentially eligible studies were identified from Medline and EMBASE from inception to February 2023 using search strategy that comprised of terms for "chronic urticaria" and "systemic lupus erythematosus". The eligible study must consist of one group of patients with CU and another group of comparators without CU and must compare the prevalence of SLE in each group and report effect size with 95% confidence intervals (95% CIs). We extracted such data from each study to calculate a pooled odds ratio using the generic inverse variance method with random-effect model. Funnel plot was used to evaluate publication bias. Newcastle-Ottawa Scale was used to appraise the methodological quality of the included studies. Results: A total of 5,155 articles were identified. After two rounds of independent review by four investigators, five studies met the eligibility criteria and were included in the meta-analysis. The meta-analysis found an increased prevalence of SLE among patients with CU compared with individuals without CU with the pooled odds ratio of 5.03 (95% CI, 2.57-9.85, I2 of 93%). Conclusion: This systematic review and meta-analysis found that patients with CU had a significantly increased risk of SLE compared to individuals without CU.

Atypical Femoral Fracture in Hypophosphatasia: A Systematic Review.

Objective: To summarize the characteristics of all reported patients with hypophosphatasia (HPP) who sustained atypical femoral fracture (AFF) and identify all available evidence to quantify the rate of coexistence between HPP and AFF. Methods: Potentially eligible articles were identified from the MEDLINE and EMBASE databases from its inception to September 2022, using a search strategy consisting of terms related to "Hypophosphatasia" and "Atypical femoral fracture." Eligible articles must report one of the following information: (1) individual data of patients diagnosed with HPP and AFF, (2) prevalence of HPP among patients with AFF, or (3) prevalence of AFF among patients of HPP. Characteristics of patients reported in each study were extracted. Results: A total of 148 articles were identified. After the systematic review, 24 articles met the eligibility criteria. A total of 28 patients with AFF and HPP were identified. The mean ± SD age of the reported patients was 53.8 ± 12.5 years, and 22 patients (78.6%) were female. Nine patients (32.1%) received antiresorptive medication (bisphosphonate and/or denosumab), and two patients (7.1%) received teriparatide prior to the development of AFF. Seven (25.0%) and eighteen (64.3%) patients sustained unilateral and bilateral AFF, respectively (laterality not reported in three cases). Thirteen patients (46.4%) had a history of fractures at other sites. Four (14.3%) and seven (25.0%) patients received asfotase alfa and teriparatide after sustaining AFF. Two studies reported the prevalence of AFF among patients with HPP of approximately 10%. One study reported one HPP patient in a cohort of 72 patients with AFF. Conclusions: Based on the limited evidence, AFF occurred in up to 10% of patients with HPP. Based on the 28 case reports, about two-thirds did not receive antiresorptive treatment, suggesting that the HPP itself could potentially be a risk factor for AFF.

Association between statin use & risk of diffuse large B-cell lymphoma: A systematic review & meta-analysis.

Background & objectives: Statin use has been shown to be associated with a decreased risk of several types of cancer, however, the data on diffuse large B-cell lymphoma (DLBCL) are still inconclusive. This study aimed to systematically summarize all available data on this association and conduct a meta-analysis on the same. Methods: A systematic review was performed using EMBASE and MEDLINE databases from inception upto October 2019 with a search strategy that included terms such as 'statin' and 'DLBCL'. Eligible studies included either case-control or cohort studies that reported the association between statin use and the risk of DLBCL. Relative risk, odds ratio (OR), hazard: risk ratio or standardized incidence ratio of this association and standard error were extracted and combined for calculating the pooled effect estimate using random-effects, generic inverse variance method. Results: A total of 1139 articles were screened. Of these six studies satisfied the inclusion criteria and were included for the meta-analysis. Statin use was associated with a significantly reduced risk of DLBCL with the pooled OR of 0.70 (95% confidence interval, 0.56-0.88; I[2]=70%). The funnel plot (fairly symmetric) was not suggestive of the presence of a publication bias. Interpretation & conclusions: The present systematic review and meta-analysis found that statin use is associated with a 30 per cent reduced odds of DLBCL. However, the pooled analysis utilized data from observational studies so causation cannot be concluded upon. Hence, it suggested that randomized-controlled studies are still needed to confirm this potential benefit.

Inpatient epidemiology, healthcare utilization, and association with comorbidities of Turner syndrome: A National Inpatient Sample study.

We aimed to investigate the prevalence, resource utilization, and comorbidities of patients with Turner syndrome (TS) hospitalized in the United States. We identified patients within the Nationwide Inpatient Sample database from the year 2017 to 2019. A propensity-matched cohort of non-TS patients from the same database was constructed to serve as comparators. There were 9845 TS patients, corresponding to inpatient prevalence of 10.4 per 100,000 admissions. The most common admission diagnosis was sepsis (27.9%). TS patients had higher inpatient mortality (adjusted odds ratio 2.16, 95% confidence interval 1.57-2.96) and morbidity, including shock, ICU admission, acute kidney injury, systemic inflammatory response syndrome, acute respiratory distress syndrome, and multi-organ failure. Increased risk of comorbidities, such as stroke, myocardial infarction, autoimmune diseases, and non-variceal gastrointestinal bleeding, was observed. TS patients had longer length of stay (LOS; 5.1 days vs. 4.5 days, p < 0.01) and displayed a mean additional $5382 (p < 0.01) in total hospital costs and a mean additional $20,083 (p < 0.01) in total hospitalization charges. In conclusion, hospitalization of patients with TS was associated with a significantly higher inpatient morbidity, mortality, expenditures, and longer LOS compared to non-TS patients. Patients with TS had a higher risk of cardiovascular complications, autoimmune diseases, and gastrointestinal bleeding.

Bone Mineral Density in Neurofibromatosis Type 1: A Systematic Review and Meta-Analysis.

To assess BMD in patients with neurofibromatosis type 1 (NF1) using systematic review and meta-analysis technique. Potentially eligible studies were identified from Medline and EMBASE databases from inception to February 2023 using search strategy that comprised terms for "Bone mineral density" and Neurofibromatosis type 1″. Eligible study must include adult or pediatric patients with NF1. The study must report mean Z-score with variance of total body, lumbar spine, femoral neck or total hip BMD of the studied patients. Point estimates with standard errors were retrieved from each study and were combined using the generic inverse variance method. A total of 1,165 articles were identified. After systematic review, 19 studies were included. The meta-analysis revealed that patients with NF1 had negative mean Z-scores for total body BMD (pooled mean Z-score -0.808; 95%CI, -1.025 to -0.591) and BMD at lumbar spine (pooled mean Z-score -1.104; 95%CI, -1.376 to -0.833), femoral neck (pooled mean Z-score -0.726; 95%CI, -0.893 to -0.560) and total hip (pooled mean Z-score -1.126; 95%CI, -2.078 to -0.173). The subgroup meta-analysis in pediatric patients aged < 18 years revealed that patients with NF1 had negative mean Z-scores for lumbar spine BMD (pooled mean Z-score -0.938; 95%CI, -1.299 to -0.577) and femoral neck BMD (pooled mean Z-score -0.585; 95%CI, -0.872 to -0.298). The current meta-analysis found that patients with NF1 had low Z-scores although the degree of low BMD may not be of clinical significance. The results do not support the role of early BMD screening in children and young adults with NF1.

Increased Prevalence of Gastroesophageal Reflux Disease Among Patients With Rheumatoid Arthritis: A Systematic Review and Meta-analysis.

OBJECTIVE: This study was conducted to determine the association between gastroesophageal reflux disease (GERD) and rheumatoid arthritis (RA) by pooling the evidence from all available studies. METHODS: Potentially eligible studies were identified from MEDLINE and EMBASE database from inception to April 2021 employing a search strategy that consisted of terms for "Rheumatoid Arthritis" and "Gastroesophageal Reflux Disease". Eligible studies for the meta-analysis were recruited with conditions of being cohort studies that included rheumatoid arthritis and without rheumatoid arthritis individuals. Together with this, prevalence of GERD in both groups and the odds ratio (OR) comparing the prevalence of GERD between the two cohorts have been reported. The retrieved point estimates with standard errors from each study were pooled into the final result by the random-effect model and generic inverse variance method as described by DerSimonian and Laird. RESULTS: A total of 3,646 articles were identified. After two rounds of independent review by two investigators, five cohort studies were included in the meta-analysis as they met the eligibility criteria. The pooled analysis demonstrated a significant association between RA and GERD with the pooled odds ratio of 1.98 (95% CI, 1.49 - 2.65). High statistical heterogeneity with I2 of 83% was observed. The funnel plot was symmetric and publication bias was not observed. CONCLUSION: This systematic review and meta-analysis found a significant association between GERD and RA.

Prevalence of concomitant angioedema in chronic spontaneous urticaria: A systematic review and meta-analysis.

BACKGROUND: Angioedema (AE) is a condition associated with considerable morbidity and mortality that can significantly affect quality of life. AE often occurs in patients with CSU although the true prevalence remains unknown. Therefore, we conducted this systematic review and meta-analysis to summarize the available data. OBJECTIVE: This study is conducted with the aim of retrieving data from all published studies and create the pooled prevalence of AE in CSU patients. METHODS: Narrative reviews of AE and CSU, a systematic review, and a meta-analysis were conducted. The Ovid Medline and Embase databases were systematically searched per the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) recommendations. Studies were eligible if they were in English and measured the prevalence of AE in CSU in adults or children. Two reviewers independently extracted data and appraised each study's quality. Estimated prevalence and 95% confidence interval (CI) values were pooled using random-effects meta-analysis. RESULTS: Seventeen studies from 16 countries were included. The pooled prevalence of AE in patients with CSU was 36.5% (95%CI, 30.9-42.5%; I2 = 96%). The pooled estimated prevalence of AE in patients with CSU was 44.0% (95%CI, 34.1-54.5%) in Europe, 44.5% (95%CI, 28.5-61.8%), America, and 29.4% (95%CI, 24.7-34.7%) in Asia. CONCLUSIONS: Our systematic review and meta?analysis showed that AE affects over one-third of CSU patients, although the prevalence from individual study varied considerably, ranging from 5 to 67 percent. Subgroup-analysis found that AE is more prevalent in Europe and America than in Asia.

Food-Dependent Exercise-Induced Wheals/Angioedema, Anaphylaxis, or Both: A Systematic Review of Phenotypes.

BACKGROUND: Food-dependent exercise-induced allergic reactions can manifest with wheals, angioedema, and anaphylaxis, alone or in combination. OBJECTIVE: To systematically review the clinical manifestation, culprit foods and exercise, augmenting factors, comorbidities, and treatment options of each phenotype. METHODS: Using predefined search terms, we assessed and analyzed the relevant literature until June 2021. Preferred Reporting Items for Systematic Reviews and Meta-Analysis recommendations were applied to this systematic review. RESULTS: A total of 231 studies with 722 patients were included. The most common phenotype was anaphylaxis with wheals, angioedema, or both, reported in 80% of patients. This was associated with a higher number of anaphylactic episodes, augmenting factors, and use of on-demand antihistamine compared with the least common phenotype, anaphylaxis without wheals or angioedema, reported in 4% of patients. Anaphylaxis with wheals/angioedema was also associated with distinct characteristics compared with stand-alone wheals, angioedema, or both, in 17% of patients. Patients with anaphylaxis were older at the time of disease onset, less often had a history of atopy, showed more positive results in response to food and exercise provocation tests, had a more restricted spectrum of culprit foods, and more often used on-demand epinephrine. CONCLUSIONS: The three phenotypes of allergic reactions to food and exercise differ in clinical characteristics, triggers, and response to treatment. Knowledge of these differences may help with patient education and counseling as well as disease management.

Antineutrophil Cytoplasmic Autoantibodies-Associated Vasculitides and Risk of Stroke: A Systematic Review and Meta-analysis.

Background: Increased incidence of cardiovascular disease, including stroke, has been consistently observed in patients with chronic inflammatory diseases, although data on antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) are still limited due to the relative rarity of the disease. Methods: Two investigators independently searched published studies indexed in MEDLINE and EMBASE database from inception to June 2019 using the search terms related to AAV and stroke. The eligible study must be cohort study that consisted of cohort of patients with AAV and cohort of patients without AAV. The study must follow the participants for incident stroke. The magnitude of difference in the incidence of stoke between the cohorts must be reported. Pooled effect estimates were calculated by combining the effect estimate of each eligible study using generic inverse variance method. Statistical heterogeneity was assessed using the Cochran's Q test and I2 statistics. All analyses were conducted using RevMan 5.3 software from the Cochrane Collaboration. Results: A total of six cohort studies fulfilled the eligibility criteria and were included into the meta-analysis. Patients with AAV had a higher risk of developing incident stroke than individuals without AAV with the a pooled risk ratio of 2.02 (95% CI, 1.02-4.00; I2 of 89%). Funnel revealed no suggestive evidence of publication bias. Conclusion: A significantly higher risk of incident stroke among patients with AAV than individuals without AAV was demonstrated by this meta-analysis.

Dynamic parameters for fluid responsiveness in mechanically ventilated children: A systematic review.

Objective: Fluid administration is the initial step of treatment of unstable pediatric patients. Evaluation of fluid responsiveness is crucial in mechanically ventilated children to avoid fluid overload, which increases mortality. We aim to review and compare the diagnostic performance of dynamically hemodynamic parameters for predicting fluid responsiveness in mechanically ventilated children. Design: A systematic review was performed using four electronic databases, including PubMed, EMBASE, Scopus, and Central, for published articles from 1 January 2010 to 31 December 2020. Studies were included if they described diagnostic performance of dynamic parameters after fluid challenge was performed in mechanically ventilated children. Settings: Pediatric intensive and cardiac intensive care unit, and operative room. Patients: Children aged 1 month to 18 years old who were under mechanical ventilation and required an intravenous fluid challenge. Measurements and Main Results: Twenty-seven studies were included in the systematic review, which included 1,005 participants and 1,138 fluid challenges. Respiratory variation in aortic peak velocity was reliable among dynamic parameters for predicting fluid responsiveness in mechanically ventilated children. All studies of respiratory variation in aortic peak velocity showed that the area under the receiver operating characteristic curve ranged from 0.71 to 1.00, and the cutoff value for determining fluid responsiveness ranged from 7% to 20%. Dynamic parameters based on arterial blood pressure (pulse pressure variation and stroke volume variation) were also used in children undergoing congenital heart surgery. The plethysmography variability index was used in children undergoing neurological and general surgery, including the pediatric intensive care patients. Conclusions: The respiratory variation in aortic peak velocity exhibited a promising diagnostic performance across all populations in predicting fluid responsiveness in mechanically ventilated children. High sensitivity is advantageous in non-cardiac surgical patients and the pediatric intensive care unit because early fluid resuscitation improves survival in these patients. Furthermore, high specificity is beneficial in congenital heart surgery because fluid overload is particularly detrimental in this group of patients. Systematic Review Registration: https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=206400.

Prevalence, Clinical Manifestations, Treatment, and Clinical Course of Chronic Urticaria in Elderly: A Systematic Review.

Purpose: Data specific to the epidemiology, clinical features, and management of chronic urticaria (CU) in the geriatric population remain limited and not well understood. We aim to systematically review the prevalence, clinical manifestations, treatment, and clinical course of elderly patients with CU. Patients and methods: Original articles that included data of elderly (aged >60 years) with CU that were published until February 2021 were searched in PubMed, Scopus, and Embase using predfefined search terms. Related articles were evaluated according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses recommendations. Results: Among the included 85 studies and 1,112,066 elderly CU patients, most (57.4%) were women. The prevalence of elderly CU in the general population ranged from 0.2-2.8%, and from 0.7-33.3% among all CU patients. Compared to adult CU, elderly CU patients had a higher percentage of wheal alone (73.9%), and lower rate of positive autologous serum skin test and atopy. Gastrointestinal diseases were the most common comorbidity (71.9%), and there was a high rate of malignancies and autoimmune diseases. Second generation H1-antihistamines were commonly used, and achievement of complete control was most often reported. Omalizumab was prescribed in 59 refractory patients, and a significant response to treatment was reported in most patients. The treatment of comorbidities also yielded significant improvement in CU. Conclusion: Elderly CU was found to be different from adult CU in both clinical and laboratory aspects. H1- antihistamines are effective as first-line therapy with minimal side-effects at licensed doses. Treatment of secondary causes is important since the elderly usually have age-related comorbidities.

Association between diabetic peripheral neuropathy and sarcopenia: A systematic review and meta-analysis.

AIM: The present study comprehensively investigated the relationship between diabetic peripheral neuropathy (DPN) and sarcopenia by identifying all eligible studies and summarizing their results. METHODS: Records were identified through MEDLINE and EMBASE database searching from inception to March 9, 2022. We included all cross-sectional studies investigating the association between DPN and sarcopenia among patients with diabetes. Data from eligible studies, including point estimates and standard errors, were pooled together using the generic inverse variance method. RESULTS: Of 2989 retrieved articles, five studies met the inclusion criteria and were allowed for meta-analysis. The pooled analysis found a significant association between DPN and sarcopenia with the pooled odds ratio of 1.62 (95% confidence interval: 1.30-2.02; I2 0%). The funnel plot was relatively symmetric and was not suggestive of the presence of publication bias. CONCLUSIONS: The current study discovered a significant association between DPN and sarcopenia in patients with diabetes. However, given summarized data from cross-sectional studies, the temporality between DPN and sarcopenia could not be established. Geriatr Gerontol Int 2022; 22: 785-789.

Risk of Sudden Death in a General Unbiased Epidemiological Cohort of Sarcoidosis.

Background Sarcoidosis is an inflammatory, noncaseating, granulomatous disorder of unknown cause that can affect any body system and is associated with cardiovascular disease including sudden cardiac death (SCD). Cardiac involvement in sarcoidosis is associated with higher risk of SCD, but the SCD risk in the general sarcoidosis population is unknown. We aimed to determine the risk of SCD in people with sarcoidosis versus the matched general population. Methods and Results A population-based cohort of sarcoidosis and age- and sex-matched comparators from January 1, 1976 to December 31, 2013 was used; presence of other comorbidities in the comparator group was not an exclusion criterion. Mortality, including time, place, and cause of death were measured and manually adjudicated for SCD events. Incidence rates are reported per 100 000 person-years, and Cox models were used for group comparisons. Of the 345 incident cases of sarcoidosis (171 men; 50%) there were 58 reported deaths; 10 were definite/probable SCD versus 57 all-cause and 9 SCDs in comparators. Median follow-up was 12.9 years (interquartile range, 6.0-23.4 years) . Incidence rate of SCD in sarcoidosis was 192 (95% CI, 92-352) versus 155 (95% CI, 71-294) in comparators (hazard ratio [HR], 1.28 (95% CI, 0.52-3.17). Nocturnal deaths were more frequent in sarcoidosis 57 (95% CI, 12-168) versus 17 (95% CI, 0.4-95) (HR, 3.76 [95% CI, 0.39-36.47]). No significant differences were detected between the groups by sex, age, calendar year of diagnosis, or disease duration. Conclusions In a population-based cohort of patients with sarcoidosis, the risk for SCD compared with matched comparators was not increased. There were more nocturnal deaths among patients with sarcoidosis, yet this was statistically insignificant.

Mortality Risk After Atypical Femoral Fracture: A Systematic Review and Meta-analysis.

OBJECTIVE: To summarize all available data using systematic review and meta-analysis to estimate the 1-year mortality risk after atypical femoral fracture (AFF) and risk ratio of mortality after AFF versus typical femoral fracture (TFF). METHODS: Potentially eligible studies were identified from MEDLINE and Embase databases from inception to February 2022 using a search strategy that comprised the terms for "atypical femoral fracture" and "mortality." An eligible study must consist of a cohort of patients with AFF. Then, the study must report the 1-year mortality rate after the AFF or report effect estimates with 95% confidence intervals, comparing the incident mortality between patients with AFF and TFF. Point estimates with standard errors were retrieved from each study and combined using the generic inverse variance method. RESULTS: A total of 8967 articles were identified. After 2 rounds of independent review by 3 investigators, we identified 7 studies reporting the 1-year mortality rate of AFFs and 3 studies comparing the mortality rate of AFF with that of TFF. Pooled analysis revealed a pooled 1-year mortality rate after an AFF of 0.10 (95% confidence interval, 0.05-0.16; I2 = 93.3%). Two studies compared the mortality risks of AFF with those of TFF and revealed conflicting results. CONCLUSION: The 1-year mortality rate after an AFF was approximately 10%. However, evidence is insufficient to conclude whether there was a difference in mortality risk between AFF and TFF.

Neurosarcoidosis: an update on diagnosis and therapy.

INTRODUCTION: Clinically overt granulomatous involvement of the nervous system (i.e. neurosarcoidosis) can be seen in up to 10% of patients with sarcoidosis. Establishing a diagnosis of neurosarcoidosis is often challenging due to the heterogeneity of clinical presentations that are sometimes nonspecific, and inaccessibility of tissue confirmation. Recommended treatments are based on expert opinions that are derived from clinical experience and limited data from retrospective studies, as data from randomized controlled studies are limited. AREA COVERED: In this article, we comprehensively review all available literature on epidemiology, clinical presentations, diagnosis, treatment, and outcomes of neurosarcoidosis. We also offer our opinions on diagnostic approach and treatment strategy. EXPERT OPINION: Given the invasive nature and the limited sensitivity of biopsy of the nervous system, diagnosis of neurosarcoidosis is usually made when ancillary tests (such as magnetic resonance imaging and cerebrospinal fluid analysis) are compatible, and alternative diagnoses are reasonably excluded in patients with established extraneural sarcoidosis. Several factors must be taken into consideration to formulate the initial treatment strategy, including the extent of the disease, severity, functional impairment, comorbidities, and patient's preference. In addition, treatment regimen of neurosarcoidosis should be formulated with an emphasis on long-term strategy.

Food-Dependent Exercise-Induced Wheals, Angioedema, and Anaphylaxis: A Systematic Review.

BACKGROUND: Food-dependent exercise-induced wheals, angioedema, and anaphylaxis remain insufficiently characterized. OBJECTIVE: We systematically reviewed the literature on clinical manifestations, laboratory investigations, culprit foods, triggering exercise, comorbidities, and treatment outcomes. METHODS: Using predefined search terms and Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) recommendations, we searched 3 electronic databases to identify relevant literature published before July 2021. RESULTS: Of 722 patients (median age 25 years; 55.4% male) from 231 studies (43 cohort studies, 15 cases series, and 173 case reports), 79.6% and 3.7% had anaphylaxis with and without wheals and/or angioedema, respectively. The remaining 16.6% had wheals and/or angioedema without anaphylaxis. The duration from eating to exercising and from exercising to symptom onset ranged from 5 minutes to 6 hours (median 1 hour) and from 5 minutes to 5 hours (median 30 minutes), respectively, and virtually all patients exercised within 4 hours after eating and developed symptoms within 1 hour after exercising. Wheat was the most common culprit food. Running was the most common trigger exercise. Most patients were atopic, and 1 in 3 had a history of urticaria. Aspirin and wheat-based products were the most frequent augmenting factors. On-demand antihistamines, corticosteroids, and epinephrine were commonly used and reported to be effective. Patients who stopped eating culprit foods before exercise no longer developed food-dependent exercise-induced allergic reactions. CONCLUSIONS: Food-dependent exercise-induced allergic reactions are heterogeneous in their clinical manifestations, triggers, and response to treatment. Patients benefit from avoidance of culprit foods before exercise, which highlights the need for allergological diagnostic workup and guidance.