ALBERT

Beschreibung: Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease in which impaired natural killer and cytotoxic T-cell function results in excessive immune activation. It is predominantly seen in children; most of the available data comes from the pediatric population so it cannot be generalized to adult HLH. Treatment of HLH usually involves either treating the underlying cause in the secondary form (i.e. malignancy with chemotherapy, rheumatologic with immune suppression) or chemotherapy and stem cell transplantation for primary, familial etiology, multiple courses of intensive chemotherapy, with stem cell transplantation for relapse and familial disease. Recently, increasing adult HLH cases have been reported. The goal of this study is to describe the association between patient factors, geography, hospital resource utilization, and mortality among adult HLH patients. Methods: We performed a retrospective cohort analysis of the National Inpatient Sample 2012, 2013 and 2014 Databases (HCUP-NIS). Patients were included in the study if they had a principal diagnosis of HLH and were older than 18 years. We used descriptive statistics to characterize the cohort in terms of personal demographic factors (age, race, sex, insurance type, community-level income level), hospital characteristics (size, region, teaching status, and urban or rural location), and admission timing (weekend or weekday). We performed univariate and multivariate regression to analyze the association of the following factors with length of stay and mortality: age, sex, Charlson index, hospital region (Northeast NE, Midwest MW, South, West), income, insurance, hospital size, weekend versus weekday, hospital location (rural versus urban), teaching status. All analyses applied the HCUP-NIS weights. Results: The cohort comprised 760 patients, the majority of whom were male (57.9%), aged 21-30 years (26.3%), white (56.3%), and treated in large (78.9%) and/or teaching (92.1%) hospitals, third quartile for median household income (30.4%), covered by private insurance (43.4%), and treated in the southern US (32.2%). Per hospitalization, the average total hospital charges were $210,526 (95% CI $176,251 to $244,801) and the average length of stay (ALOS) was 18 days (95% CI 16 to 20). On multivariate analysis, ALOS was significantly longer with patients at teaching hospitals (AMD 5.10 95% CI 0.57 to 9.64, p=0.03) or with self-pay status (AMD 29.05 95% CI 21.62 to 36.48, p

Beschreibung: Introduction: Comprehensive management for patients with hemophilia has drastically improved outcomes, quality of care, and longevity. Because of increases in life span, patients with hemophilia may be at risk for other chronic conditions including cardiovascular disease (CVD). Though initially it was thought that hemophilia might have been protective for cardiovascular disease further research has shown that CVD remains a significant risk for the aging hemophilia population. This study aims to determine the prevalence of risk factors and outcomes for CVD in hospitalized adult and pediatric patients with the discharge diagnosis of Hemophilia A or B compared to patients without Hemophilia. We examine longitudinal changes over the previous decade. WMethods: The Healthcare Cost and Utilization Project's Nationwide Inpatient Sample (HCUP-NIS) was utilized for analysis of years 2007 and 2017. The NIS uses a stratified probability sample of 20% of all inpatient discharges (representing more than 97% of the US population). Hemophilia-A and B were identified using ICD-9 code 286.0 and 286.1, ICD-10 codes D66 and D67 respectively and sampling weights were applied to generate nationally representative estimates. Cardiovascular risk factors and outcomes were determined by evaluating ICD-9 codes for 2007 data and ICD-10 codes for 2017 data. For comparative historical data, 2007 NIS data from a prior published study [Goel et al., Hemophilia (2012), 18, 688-692] were used. The NIS is a de-identified, publicly available data set. This study was deemed exempt from review from the Johns Hopkins Institutional Review Board. This analysis was conducted in accordance with the HCUP data use agreement guidelines. Results: In 2017, there were 10,555 admissions with Hemophilia A or B listed as one of all diagnoses. The mean age of hemophilia patients was 44.31 years compared to 49.57. years for all admissions. The most prevalent risk factor in 2017 was hypertension (32.4% for admissions with hemophilia as compared to 35.3% for all admissions) followed by hyperlipidemia (19.4% compared to 27.5%), diabetes (17.4% compared to 22.8%) and obesity (10.8% compared to 14.4%). CVD outcomes, in descending order of frequency were atherosclerotic coronary artery disease (11.6% for admissions with hemophilia compared to 16.9% for all admissions), heart failure (10.2% compared to 14.2%), acute myocardial infarction (AMI) (2.2% compared to 3.9%), and stroke (2.2% compared to 2.4% respectively). Comparing to 10 year prior data, in 2007, there were 9,737 admissions with Hemophilia A or B listed as one of all diagnoses. The mean age of hemophilia patients was 30.89 years compared to 47.16 years for all admissions. The most prevalent risk factor in 2007 was hypertension (27.0% in admissions with hemophilia compared to 36.7% for all admissions); followed by diabetes (11.2% compared to 18.5%), hyperlipidemia (9.5% compared to 17%), and obesity (3.6% compared to 5.8%). CVD outcomes, in descending order of frequency were, atherosclerotic coronary artery disease (10.1% compared to 16.7%), heart failure (6.6% compared to 10.8%), AMI (2.1% compared to 2.4%), and stroke (2.0% compared to 1.7%). Between 2007 and 2017 the crude prevalence rates of all CVD risk factors as well as CVD outcomes generally increased for admissions with hemophilia as well as all-cause hospitalizations. Conclusions: The frequency of all CVD risk factors (obesity, diabetes, hypertension, and hyperlipidemia) as well as CVD outcomes (atherosclerosis, congestive heart failure, AMI, and stroke) increased between 2007 and 2017 in hospitalized patients both with and without hemophilia. While the unadjusted prevalence rates for all CVD risk factors and CVD outcomes were less in hospitalized patients with hemophilia compared to the general hospitalized population in both 2007 and 2017, CVD remains a significant risk for the hemophilia population. An improved understanding of the various risk factors will help to improve CVD outcomes in the aging hemophilia population. Disclosures Takemoto: Genentech: Membership on an entity's Board of Directors or advisory committees; Novartis: Other: DSMB Aplastic Anemia Trial.

Beschreibung: Introduction Venous thromboembolism (VTE) (deep vein thrombosis [DVT] and pulmonary embolism [PE]) is a cause of significant morbidity and mortality. Over the last decade, there has been an increase in awareness and major advances in early diagnosis and treatment of VTE. This study sought to estimate the mortality and associated diagnoses in hospitalized patients with a primary diagnosis of DVT or PE using a nationally representative database. Methods The 2017 Healthcare Cost and Utilization Project's Nationwide Inpatient Sample (HCUP-NIS) was used for analysis. The NIS uses a stratified probability sample of 20% of all inpatient discharges, representing over 97% of the US population. Sampling weights were applied to hospital discharges for DVT and PE using applicable ICD-10 codes to generate nationally representative estimates. Pearson's chi-squared test and the Mann-Whitney U test were used for comparisons to assess statistical significance. Results Of the nearly 36 million hospital admissions in 2017, 579,860 had DVT included in the index list of diagnoses during the hospitalization, and 105,635 had DVT as the primary admission diagnosis. Within the primary DVT admissions (median age (interquartile range (IQR)): 64 years (51-77)), 102,505 were acute DVT, and 3,130 were chronic DVT. There were 376,140 admissions with PE as one of all diagnoses and 188,245 with PE as the primary admission diagnosis. Among primary PE admissions (median age (IQR): 64 years (52-75)), 16,205 (8.6%) were saddle PE (Figure 1a). Overall, there were 826,155 people diagnosed with PE or DVT as one of any diagnoses, and 129,845 were diagnosed with both DVT and PE. Mortality The all-cause mortality in admissions with a primary diagnosis of DVT (0.8%) was significantly lower than for all other NIS admissions at 1.96% (p