Characteristics and survival of patients diagnosed with cardiac sarcoidosis: A case series

BackgroundSarcoidosis is a multiorgan system granulomatous disease of unknown etiology. It is hypothesized that a combination of environmental, occupational, and/or infectious factors provoke an immunological response in genetically susceptible individuals, resulting in a diversity of manifestations throughout the body. In the United States, cardiac sarcoidosis (CS) is diagnosed in 5% of patients with systemic sarcoidosis, however, autopsy results suggest that cardiac involvement may be present in > 50% of patients. CS is debilitating and significantly decreases quality of life and survival. Currently, there are no gold-standard clinical diagnostic or monitoring criteria for CS.MethodsWe identified patients with a diagnosis of sarcoidosis who were seen at the Simmons Center from 2007 to 2020 who had a positive finding of CS documented with cardiovascular magnetic resonance (CMR) and/or endomyocardial biopsy as found in the electronic health record. Medical records were independently reviewed for interpretation and diagnostic features of CS including late gadolinium enhancement (LGE) patterns, increased signal on T2-weighted imaging, and non-caseating granulomas, respectively. Extracardiac organ involvement, cardiac manifestations, comorbid conditions, treatment history, and vital status were also abstracted.ResultsWe identified 44 unique patients with evidence of CS out of 246 CMR reports and 9 endomyocardial biopsy pathology reports. The first eligible case was diagnosed in 2007. The majority of patients (73%) had pulmonary manifestations, followed by hepatic manifestations (23%), cutaneous involvement (23%), and urolithiasis (20%). Heart failure was the most common cardiac manifestation affecting 59% of patients. Of these, 39% had a documented left ventricular ejection fraction of < 50% on CMR. Fifty eight percent of patients had a conduction disease and 44% of patients had documented ventricular arrhythmias. Pharmacotherapy was usually initiated for extracardiac manifestations and 93% of patients had been prescribed prednisone. ICD implantation occurred in 43% of patients. Patients were followed up for a median of 5.4 (IQR: 2.4–8.5) years. The 10-year survival was 70%. In addition to age, cutaneous involvement was associated with an increased risk of death (age-adjusted OR 8.47, 95% CI = 1.11–64.73).ConclusionCMR is an important tool in the non-invasive diagnosis of CS. The presence of LGE on CMR in a pattern consistent with CS has been shown to be a predictor of mortality and likely contributed to a high proportion of patients undergoing ICD implantation to decrease risk of sudden cardiac death.Clinical implicationsAdditional studies are necessary to develop robust criteria for the diagnosis of CS with CMR, assess the benefit of serial imaging for disease monitoring, and evaluate the effect of immunosuppression on disease progression

Serum metabolomic signatures of fatty acid oxidation defects differentiate host-response subphenotypes of acute respiratory distress syndrome

BACKGROUND: Fatty acid oxidation (FAO) defects have been implicated in experimental models of acute lung injury and associated with poor outcomes in critical illness. In this study, we examined acylcarnitine profiles and 3-methylhistidine as markers of FAO defects and skeletal muscle catabolism, respectively, in patients with acute respiratory failure. We determined whether these metabolites were associated with host-response ARDS subphenotypes, inflammatory biomarkers, and clinical outcomes in acute respiratory failure. METHODS: In a nested case-control cohort study, we performed targeted analysis of serum metabolites of patients intubated for airway protection (airway controls), Class 1 (hypoinflammatory), and Class 2 (hyperinflammatory) ARDS patients (N = 50 per group) during early initiation of mechanical ventilation. Relative amounts were quantified by liquid chromatography high resolution mass spectrometry using isotope-labeled standards and analyzed with plasma biomarkers and clinical data. RESULTS: Of the acylcarnitines analyzed, octanoylcarnitine levels were twofold increased in Class 2 ARDS relative to Class 1 ARDS or airway controls (P = 0.0004 and \u3c 0.0001, respectively) and was positively associated with Class 2 by quantile g-computation analysis (P = 0.004). In addition, acetylcarnitine and 3-methylhistidine were increased in Class 2 relative to Class 1 and positively correlated with inflammatory biomarkers. In all patients within the study with acute respiratory failure, increased 3-methylhistidine was observed in non-survivors at 30 days (P = 0.0018), while octanoylcarnitine was increased in patients requiring vasopressor support but not in non-survivors (P = 0.0001 and P = 0.28, respectively). CONCLUSIONS: This study demonstrates that increased levels of acetylcarnitine, octanoylcarnitine, and 3-methylhistidine distinguish Class 2 from Class 1 ARDS patients and airway controls. Octanoylcarnitine and 3-methylhistidine were associated with poor outcomes in patients with acute respiratory failure across the cohort independent of etiology or host-response subphenotype. These findings suggest a role for serum metabolites as biomarkers in ARDS and poor outcomes in critically ill patients early in the clinical course

Prospective Acid Reflux Study of Iran (PARSI): Methodology and study design

<p>Abstract</p> <p>Background</p> <p>Gastroesophageal reflux disease is a common and chronic disorder but long term, prospective studies of the fate of patients seeking medical advice are scarce. This is especially prominent when looking at non-erosive reflux disease (NERD) patients.</p> <p>Methods</p> <p>We designed a prospective cohort to assess the long term outcome of GERD patients referring to gastroenterologists. Consecutive consenting patients, 15 years of age and older, presenting with symptoms suggestive of GERD referring to our outpatient clinics undergo a 30 minute interview. Upper gastrointestinal endoscopy is performed for them with protocol biopsies and blood samples are drawn. Patients are then treated according to a set protocol and followed regularly either in person or by telephone for at least 10 years.</p> <p>Discussion</p> <p>Our data show that such a study is feasible and follow-ups, which are the main concern, can be done in a fairly reliable way to collect data. The results of this study will help to clarify the course of various subgroups of GERD patients after coming to medical attention and their response to treatment considering different variables. In addition, the basic symptoms and biological database will fuel further molecular epidemiologic studies.</p

Trends in the Incidence of Hepatocellular Carcinoma in Washington DC: A Single Institutional Cohort Study (1959–2013)

The African American (AA) community in Washington DC is at an elevated risk for hepatocellular carcinoma (HCC) that has a dismal prognosis. The recent rapid increase in the incidence and diagnosis of HCC and liver metastases (LM) in DC prompted us to evaluate the past six decades of this incidence and some of its underlying causes using a single institutional cohort in a hospital located in the center of the city. Electronic medical and pathology records of 454 liver cancer patients from 1959 to 2013 at Howard University Hospital (HUH) were reviewed. Demographic, clinical and pathology characteristics were examined, and statistical analysis was performed using Wilcoxon rank-sum test. Incidence of HCC rose substantially between 1959 and 2013, increasing eight-fold from 1.05 to 8.0 per 100,000 AAs. The rate of increase in the last decade was highest at 550%. Cases were disproportionately male (67.2%), and median age at diagnosis was 57 years. Towards the last decade, the most common etiology for HCC was nonalcoholic fatty liver disease (NAFLD) followed by NAFLD/HCV combination. Liver cancer was clustered in the eastern region of DC in wards 4, 5, 7, and 8. Cases of liver metastases clinically diagnosed and confirmed by biopsies increased 96.4% from 1959 to 1968 to 2009-2013. This study confirms that HCC incidence has been increasing (initially driven by HCV, and NAFLD in the latter decades) more rapidly in DC than previously believed, highlighting the impact of case definitions especially regarding NAFLD in the context of changing diagnostic approaches including the revised ICD10. The rising burden, disproportionate population distribution, and low survival rate among AAs emphasize the importance of prevention and early detection as a public health imperative

Global Epidemiology of Nonalcoholic Fatty Liver Disease and Perspectives on US Minority Populations

Background: Non-alcoholic fatty liver disease (NAFLD) is a clinical syndrome predicted to be the next global epidemic affecting millions of people worldwide. The natural course of this disease including its subtype, non-alcoholic steatohepatitis (NASH), is not clearly defined especially in the African-American segment of the US population. Aims: To conduct a review of the global epidemiology of NAFLD with emphasis on US minority populations. Methods: A thorough search of evidence-based literature was conducted using the Pubmed database and commercial web sources such as Medscape and Google Scholar. Results: NAFLD and its subtype NASH are becoming the principal cause of chronic liver disease across the world. In the US, Hispanics are the most disproportionately affected ethnic group with hepatic steatosis, and elevated aminotransferase levels, whereas African-Americans are the least affected. Genetic disparities involved in lipid metabolism seem to be the leading explanation for the lowest incidence and prevalence of both NAFLD and NASH in African-Americans. Conclusions: The unprecedented rise in the prevalence of NAFLD globally requires an initiation of population cohort studies with long-term follow-up to determine the incidence and natural history of NAFLD and its underrepresentation in African-Americans. Future studies should also focus on the delineation of the interplay between genetic and environmental factors that trigger the development of NAFLD and NASH