Association Between Leg Muscle Thickness and Walking Test with the Haemophilia-Specific Functional Parameters.

OBJECTIVE: To evaluate the muscle thickness and walking test in people with haemophilia A (PWH) and their correlation to joint health and functional impairments. DESIGN: Cross-sectional study. RESULTS: 29 severe/moderate PWH were enrolled. Muscle thickness of quadriceps and medial gastrocnemius were measured using ultrasound. Joint health and functional capacity were assessed using Haemophilia Joint Health Score (HJHS), Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US), 6-Minute Walking test (6MWT), Haemophilia Quality of Life Questionnaire for Adults (Haem-A-QoL), and Haemophilia Activities List (HAL). Quadriceps muscle thickness significantly correlated with HJHS knee, HEAD-US knee, and HAL. Calf muscle thickness significantly correlated with the HJHS ankle. After adjusted age and BMI, calf muscle thickness was inversely associated with the HJHS ankle. 6MWT was found to significantly correlate with HJHS total, HEAD-US total, Haem-A-QoL, and HAL. CONCLUSION: Muscle thickness and the distance of 6MWT were linked to assessment of joint health, quality of life and activity participation in PWH. Ultrasound measurement of muscle thickness and walking test appear to be useful tools for the assessment of joint health and functional status in PWH.

Overview of Methamphetamine-Associated Pulmonary Arterial Hypertension.

TOPIC IMPORTANCE: The global surge in methamphetamine use is a critical public health concern, particularly due to its robust correlation with methamphetamine-associated pulmonary arterial hypertension (MA-PAH). This association raises urgent alarms about the potential escalation of MA-PAH incidence, posing a significant and imminent challenge to global public health. REVIEW FINDINGS: This comprehensive review meticulously explores MA-PAH, offering insights into its epidemiology, pathophysiology, clinical presentation, diagnostic intricacies, and management strategies. The pathogenesis, yet to be fully described, involves complex molecular interactions, including alterations in serotonin signaling, reduced activity of carboxylesterase 1, oxidative stress, and dysregulation of pulmonary vasoconstrictors and vasodilators. These processes culminate in the structural remodeling of the pulmonary vasculature, resulting in pulmonary arterial hypertension. MA-PAH exhibits a more severe clinical profile in functional class and hemodynamics compared with idiopathic pulmonary arterial hypertension. Management involves a multifaceted approach, integrating pulmonary vasodilators, cessation of methamphetamine use, and implementing social and rehabilitation programs. These measures aim to enhance patient outcomes and detect potential relapses for timely intervention. SUMMARY: This review consolidates our understanding of MA-PAH, pinpointing knowledge gaps for future studies. Addressing these gaps is crucial for advancing diagnostic accuracy, unraveling mechanisms, and optimizing treatment for MA-PAH, thereby addressing the evolving landscape of this complex health concern.

Methamphetamine-associated pulmonary arterial hypertension: data from the national biological sample and data repository for pulmonary arterial hypertension (PAH Biobank).

OBJECTIVE: This study compares the clinical and haemodynamic severity of methamphetamine-associated pulmonary arterial hypertension (MA-PAH) with idiopathic pulmonary arterial hypertension (IPAH) and connective tissue-associated pulmonary arterial hypertension (CTD-PAH). It also examines sex differences in clinical and physiological parameters among those with MA-PAH. DESIGN: This is a cross-sectional study using clinically derived data from the National Biological Sample and Data Repository for Pulmonary Arterial Hypertension (PAH biobank), a US-based registry, to compare clinical and physiological characteristics between males and females with MA-PAH. POPULATION: The analysis included 1830 patients enrolled in the PAH biobank, with a diagnosis of MA-PAH (n=42), IPAH (n=1073), or CTD-PAH (n=715). MAIN OUTCOME MEASURES: The study assessed and compared the clinical and haemodynamic parameters of patients with MA-PAH, IPAH and CTD-PAH. RESULTS: Among the patients analysed, 42 had MA-PAH, with 69.1% being female. There were no statistically significant differences in functional class among patients with MA-PAH, IPAH and CTD-PAH. The per cent predicted 6-min walk distance (6MWD) was comparable between the three groups. Patients with MA-PAH had similar mean pulmonary artery pressure and pulmonary vascular resistance to patients with IPAH but higher compared with patients with CTD-PAH. Male patients with MA-PAH exhibited a worse functional class and lower per cent predicted 6MWD, but no significant differences in haemodynamic findings were observed between the sexes. CONCLUSION: There were no differences in haemodynamic between MA-PAH and IPAH but we found that MA-PAH differed from CTD-PAH. The study did not find evidence of sex differences in MA-PAH. Further research is necessary to identify risk factors and underlying mechanisms of MA-PAH, particularly considering the increasing prevalence of methamphetamine use. Such investigations will contribute to the development of effective prevention and treatment strategies for this condition.

Not Another Pleural Effusion: A Unique Case of Metastatic Endometrial Stromal Sarcoma.

The etiology of complicated pleural effusion can be vast. We present a unique case of an unsuspected metastatic endometrial stromal sarcoma (ESS) in an asymptomatic patient with an incidentally found complicated pleural effusion. A 69-year-old female with no pertinent past medical history was referred to pulmonology for an effusion noted on a routine chest X-ray. Her surgical history was significant for a hysterectomy. At the time of evaluation in the pulmonology clinic, the patient was asymptomatic with stable vital signs. Computed tomography of her chest showed a complex pleural effusion which was drained by cardiothoracic surgery. Fluid analysis results were positive for estrogen and progesterone receptor-positive mesenchymal tumor. Follow-up imaging was negative for any other metastasis. Appropriate management and drainage of this asymptomatic pleural effusion resulted in the diagnosis of a rare malignancy. Given the good clinical prognosis of mesenchymal tumors, the patient was appropriately treated and doing well. We present the case of a patient who was found to have a rare malignancy rather than a benign chronic pleural effusion, as previously suspected. This neoplasm represented a metastatic ESS, especially in this patient's setting of a hysterectomy.

An Unusual Case of Macrophage Activation Syndrome (MAS)- Hemophagocytic Lymphohistiocytosis (HLH) Triggered by Necrotizing Autoimmune Myopathy.

Macrophage activation syndrome (MAS)- hemophagocytic lymphohistiocytosis (HLH) secondary to inflammatory myopathies such as dermatomyositis (DM), polymyositis (PM), and necrotizing autoimmune myopathy is exceedingly rare in the medical literature. We present the complicated diagnosis and treatment of a 41-year-old female who presented with proximal muscle weakness and shock. Following an extensive critical care workup, she was diagnosed with MAS-HLH, triggered by a newly diagnosed necrotizing autoimmune myopathy. In this case report and literature review, we would like to highlight the importance of recognizing the clinical signs of MAS-HLH in rheumatological disorders and the necessity for rapid treatment.

Impact of Antifibrotic Treatment on Postoperative Complications in Patients with Interstitial Lung Diseases Undergoing Lung Transplantation: A Systematic Review and Meta-Analysis.

Antifibrotic treatment has been approved for reducing disease progression in fibrotic interstitial lung disease (ILD). As a result of increased bleeding risk, some experts suggest cessation of antifibrotics prior to lung transplantation (LT). However, extensive knowledge regarding the impact of antifibrotic treatment on postoperative complications remains unclear. We performed a comprehensive search of several databases from their inception through to 30 September 2021. Original studies were included in the final analysis if they compared postoperative complications, including surgical wound dehiscence, anastomosis complication, bleeding complications, and primary graft dysfunction, between those with and without antifibrotic treatment undergoing LT. Of 563 retrieved studies, 6 studies were included in the final analysis. A total of 543 ILD patients completing LT were included, with 161 patients continuing antifibrotic treatment up to the time of LT and 382 without prior treatment. Antifibrotic treatment was not significantly associated with surgical wound dehiscence (RR 1.05; 95% CI, 0.31-3.60; I2 = 0%), anastomotic complications (RR 0.88; 95% CI, 0.37-2.12; I2 = 31%), bleeding complications (RR 0.76; 95% CI, 0.33-1.76; I2 = 0%), or primary graft dysfunction (RR 0.87; 95% CI, 0.59-1.29; I2 = 0%). Finally, continuing antifibrotic treatment prior to LT was not significantly associated with decreased 1-year mortality (RR 0.80; 95% CI, 0.41-1.58; I2 = 0%). Our study suggests a similar risk of postoperative complications in ILD patients undergoing LT who received antifibrotic treatment compared to those not on antifibrotic therapy.

Osteoporosis increases the risk of rotator cuff tears: a population-based cohort study.

INTRODUCTION: Osteoporosis has been demonstrated to be a risk factor for rotator cuff retears after surgery; however, no studies have directly investigated the association between osteoporosis and the development of rotator cuff tears. To investigate whether osteoporosis is associated with an increased risk of rotator cuff tears. MATERIALS AND METHODS: We conducted a population-based, matched-cohort study with a 7-year follow-uTwo matched cohorts (n = 3511 with osteoporosis and 17,555 without osteoporosis) were recruited from Taiwan's Longitudinal Health Insurance Dataset. Person-year data and incidence rates were evaluated. A multivariable Cox model was used to derive an adjusted hazard ratio (aHR) after controlling for age, sex, and various prespecified comorbidities. Age and sex were added in the model to test for interaction with osteoporosis. RESULTS: Women constituted 88.5% of the cohorts. During follow-up of 17,067 and 100,501 person-years for the osteoporosis and nonosteoporosis cohorts, 166 and 89 rotator cuff tears occurred, respectively. The cumulative incidence of rotator cuff tears was significantly higher in the osteoporosis cohort than in the nonosteoporosis cohort (p < 0.001, log-rank). The Cox model revealed a 1.79-fold increase in rotator cuff tears in the osteoporosis cohort, with an aHR of 1.79 (95% confidence interval, 1.55-2.05). Effect modification of sex and age on rotator cuff tears was not found in patients with osteoporosis. CONCLUSION: This population-based study supports the hypothesis that compared with individuals without osteoporosis, those with osteoporosis have a higher risk of developing rotator cuff tears.

The Association Between Active Transportation and Serum Total 25-Hydroxyvitamin D Levels Among US Childbearing-Aged Women.

BACKGROUND: Serum total 25-hydroxyvitamin D (25[OH]D) and physical activity (PA) both play important roles in maternal-fetal health. However, a high prevalence of vitamin D and PA insufficiency has been observed in women of childbearing age. Active transportation may increase overall PA levels and potentially boost serum 25(OH)D levels. METHODS: Data from the National Health and Nutrition Examination Survey between 2007 and 2014 were used. A total of 5601 women aged 18-49 years were included. Transportation PA (TPA) was quantified as metabolic equivalents of task and serum 25(OH)D levels was measured. Multivariable logistic regression models adjusted for potential confounders were conducted. RESULTS: The corresponding adjusted odds ratios associated with vitamin D insufficiency (<50 nmol/L) were 1.09 (95% confidence interval, 0.87-1.37) for 1 to 499 MET minutes per week of TPA, 0.69 (0.52-0.91) for 500 to 1000 MET minutes per week of TPA, and 0.95 (0.72-1.26) for >1000 MET minutes per week of TPA, respectively, compared with no TPA. Using vitamin D deficiency (<30 nmol/L) as the outcome led to similar results. The association between TPA and serum 25(OH)D levels was more robust in high sedentary time. CONCLUSIONS: A moderate level of TPA is related to lower odds of suboptimal vitamin D status among women of childbearing age.

A Case Report of the Hospital-Acquired Coronavirus Disease 2019 Pneumonia: Recognition of the Potential Exposure, Atypical Presentation, and Timeline on the Course of the Illness.

This study presented a case of coronavirus disease 2019 pneumonia in a patient in isolation for 103 days. Severe acute respiratory syndrome coronavirus 2 was positive in her last 4 days. Despite policies of temperature checks for employees and visitors, and employees facing patients wearing a mask, the transmission of severe acute respiratory syndrome coronavirus 2 from an asymptomatic carrier was inevitable.

Outcomes of C difficile infection in solid-organ transplant recipients: The National Inpatient Sample (NIS) 2015-2016.

BACKGROUND: Clostridioides (formerly Clostridium) difficile infection (CDI) is one of the leading causes of morbidity and mortality worldwide. Solid organ transplant (SOT) recipients are at an increased risk for CDI. A recent study showed an overall improvement in mortality amongst hospitalized individuals with CDI, but it is unclear if this benefit extends to SOT recipients. METHODS: We scrutinized the 2015 and 2016 National Inpatient Sample (NIS), the largest all-payer inpatient database in the United States for CDI data in patients with SOT. SOT was defined as any recipient who had received a heart, lung, liver, intestinal, kidney, pancreas, or combined thoracic and/or abdominal organ transplantation. Baseline characteristics, comorbidities, and concomitant diagnosis of pneumonia or urinary tract infection were adjusted for in our analysis. Primary outcomes included inpatient mortality, hospital length of stay and total hospital charges. RESULTS: A total of 105 780 hospital discharges of SOT recipients were included. The incidence of CDI was 3554 (3.36%) among SOTs. CDI was associated with a higher inpatient mortality (OR 1.85, 95% CI 1.56-2.20, P < .01), longer length of hospital stay (mean difference 5.07 days, 95% CI 4.43-5.71, P < .01) and higher total hospital charges (mean difference 43 958 US dollars, P < .01). CONCLUSION: Our study found that CDI is associated with poorer overall outcomes among hospitalized SOT recipients. However, there was a possible improving trend of the outcomes when compare to previous studies.

Associations of serum 25(OH) vitamin D levels with clinical and radiological outcomes in multiple sclerosis, a systematic review and meta-analysis.

BACKGROUND: Vitamin D supplementation is recommended for patients with multiple sclerosis (MS). However, a recent meta-analysis based on low-quality trials suggested no evidence of supplementation benefit. A systematic review and meta-analysis of high-quality observational cohort studies should provide us further evidences. METHODS: MEDLINE, EMBASE, and WEB-of-SCIENCE databases were systematically searched to identify eligible studies published before October 2018. Prospective cohort studies assessing the associations of serum 25(OH)D levels with MS relapses, radiological inflammatory lesions, or changes in expanded disability status scale in adults (≥18 years) with MS were included. Pooled RRs were calculated using fixed-effect or random-effects model depending on heterogeneity. RESULTS: Thirteen studies and 3498 patients were included. Each 25 nmol/L increase in serum 25(OH)D levels was associated with a reduction in (1) clinical relapse rate [RR = 0.90; 95% confidence interval (CI) = 0.83-0.99], (2) gadolinium-enhancing lesions (RR = 0.69; 95% CI = 0.60-0.79), (3) new/enlarging T2 lesions (RR = 0.86; 95% CI = 0.77-0.95), and (4) new active lesions (RR = 0.81; 95% CI = 0.74-0.89) in the magnetic resonance imaging(MRI). CONCLUSIONS: Serum 25(OH)D levels are associated with a modest decrease in relapse rate and radiological inflammatory activities in patients with MS. The association with disability worsening remains inconclusive.

Non-steroidal anti-inflammatory drugs and risk of venous thromboembolism: a systematic review and meta-analysis.

OBJECTIVE: The aim of this study was to integrate and examine the association between NSAID use and venous thromboembolism (VTE). METHODS: We conducted a systematic review and meta-analysis of studies that reported odds ratios, relative risks, hazard ratios or standardized incidence ratios for VTE among NSAID users compared with non-users. Pooled risk ratios and 95% CIs were calculated using a random effects generic inverse variance model. RESULTS: Six studies with 21 401 VTE events were identified and included in the data analysis. The pooled risk ratio of VTE in NSAID users was 1.80 (95% CI 1.28, 2.52). CONCLUSION: Our study demonstrated a statistically significant increased risk of VTE among NSAID users. This finding has important public health implications given the prevalence of NSAID use in the general population.

Risk of coronary artery disease in patients with systemic sclerosis: a systematic review and meta-analysis.

Several chronic inflammatory disorders, such as rheumatoid arthritis and systemic lupus erythematosus, have been shown to increase coronary artery disease (CAD) risk but the data on systemic sclerosis (SSc) is unclear. We conducted a systematic review and meta-analysis of observational studies that reported odds ratio, relative risk, hazard ratio, or standardized incidence ratio comparing CAD risk in patients with SSc versus non-SSc participants. Pooled risk ratio and 95 % confidence intervals were calculated using a random effect, generic inverse variance method. Four studies were identified and included in our data analysis. The pooled risk ratio of CAD in patients with SSc was 1.82 (95 % CI, 1.40 to 2.36). The statistical heterogeneity of this meta-analysis was moderate with an I (2) of 73 %. Our study demonstrated a statistically significant increased CAD risk among patients with SSc.

Risk of potentially rabid animal exposure among foreign travelers in Southeast Asia.

BACKGROUND: Each year millions of travelers visit Southeast Asia where rabies is still prevalent. This study aimed to assess the risk of rabies exposure, i.e., by being bitten or licked by an animal, among travelers in Southeast Asia. The secondary objective was to assess their attitudes and practices related to rabies. METHODOLOGY/PRINCIPAL FINDINGS: Foreign travelers departing to the destination outside Southeast Asia were invited to fill out the study questionnaire in the departure hall of Bangkok International Airport. They were asked about their demographic profile, travel characteristics, pre-travel health preparations, their possible exposure and their practices related to rabies during this trip. From June 2010 to February 2011, 7,681 completed questionnaires were collected. Sixty-two percent of the travelers were male, and the median age was 32 years. 34.0% of the participants were from Western/Central Europe, while 32.1% were from East Asia. Up to 59.3% had sought health information before this trip. Travel clinics were the source of information for 23.6% of travelers. Overall, only 11.6% of the participants had completed their rabies pre-exposure prophylaxis, and 15.3% had received only 1-2 shots, while 73.1% had not been vaccinated at all. In this study, the risk of being bitten was 1.11 per 100 travelers per month and the risk of being licked was 3.12 per 100 travelers per month. Among those who were bitten, only 37.1% went to the hospital to get post exposure treatment. Travelers with East Asian nationalities and longer duration of stay were significantly related to higher risk of animal exposure. Reason for travel was not related to the risk of animal exposure. CONCLUSIONS: Travelers were at risk of being exposed to potentially rabid animals while traveling in Southeast Asia. Many were inadequately informed and unprepared for this life-threatening risk. Rabies prevention advice should be included in every pre-travel visit.