IMPORTANCEReal-world data are limited on the patient-reported burden of adult atopic dermatitis (AD).OBJECTIVE To characterize the patient-reported burden of AD with regard to impact of disease severity and inadequate control in adults from clinical settings. DESIGN, SETTING, AND PARTICIPANTSIn this cross-sectional study using data from 6 academic medical centers in the United States collected by a self-administered internet-based questionnaire, 1519 adult patients with AD were stratified by AD severity as mild or moderate/severe using the Patient-Oriented Scoring Atopic Dermatitis (PO-SCORAD). Patients with moderate/severe disease using systemic immunomodulators/phototherapy were further stratified as having adequate or inadequate disease control. Strata were compared for all outcomes. MAIN OUTCOMES AND MEASURESOutcomes included validated measures and stand-alone questions assessing itch (pruritus numerical rating scale; PO-SCORAD itch visual analog scale), pain (numerical rating scale), sleep (PO-SCORAD sleep visual analog scale; sleep interference with function), anxiety and depression (Hospital Anxiety and Depression Scale), and health-related quality of life (Dermatology Life Quality Index). RESULTS Among the 1519 adult patients with AD, relative to mild AD (n = 689, 64% women; mean [SD] age, 46.5 [18.0] years), patients with moderate/severe AD (n = 830, 66.8% women; mean [SD] age, 45.1 [16.9] years) reported more severe itching and pain, greater adverse effects on sleep, higher prevalence of anxiety and depression (417 [50.2%] vs 188 [27.3%]), and greater health-related quality-of-life impairment. The 103 patients with moderate/severe AD with inadequate disease control despite treatment with systemic immunomodulators or phototherapy (55.7%) reported higher burdens of itch and sleeping symptoms vs patients with controlled disease including more days per week with itchy skin (5.7 vs 2.7) and higher proportions with itch duration greater than half a day (190 [22.8%] vs 20 [2.9%]). Sleep symptoms included trouble sleeping (3.9 vs 1.1 on the PO-SCORAD VAS), longer sleep latency (38.8 vs 21.6 minutes), more frequent sleep disturbances (2.6 vs 0.4 nights in past week), and greater need for over-the-counter sleep medications (324 [39%] vs 145 [21%]).CONCLUSIONS AND RELEVANCE Inadequate disease control was common among patients with moderate/severe AD, and was associated with a higher patient-reported burden than patients with controlled disease. Regardless of disease control, the burden of moderate/severe AD was higher than mild AD, suggesting a need for more effective therapies for moderate/severe disease.

Despite its rarity in the United States, sickle cell disease accounts for a disproportionate amount of healthcare utilization and costs. The majority of this is due to acute care for painful crises. A small subpopulation of patients accounts for most these costs due to frequent visits to emergency departments and acute care facilities. Previous investigations have found that these high utilizing patients are distinguished by both a more severe disease course and certain non-hematologic characteristics, which may include higher socioeconomic status and some psychiatric and psychological characteristics. This prospective observational cohort study was undertaken to test the ability of these characteristics to prospectively predict acute pain care outcomes, including visit frequency, total opioid doses, and pain improvement at the Johns Hopkins Sickle Cell Infusion Center (SCIC). Seventy-three participants were followed for 12 months and SCIC utilization and treatment outcomes were tabulated for 378 visits. Participants who visited the SCIC most frequently had markedly worse pain improvement despite higher within-visit opioid doses. Higher utilization was associated with indicators of greater illness severity, more aggressive treatment for sickle cell disease, higher baseline opioid doses, higher socioeconomic status, greater pain-related anxiety, and a history of psychiatric treatment. Overall, poor acute pain treatment response was associated with higher utilization and higher baseline opioid doses. The pattern of association between high utilization, poor acute care outcomes, and higher baseline opioid doses is discussed in terms of prior research and future directions.

Introduction Venous thromboembolism (VTE) is common in sickle cell disease (SCD); however, the risk factors associated with VTE in patients with sickle variant syndromes are not known. The primary aim of this study was to determine hematologic and clinical risk factors for VTE in adults with hemoglobin SC or Sβ+ thalassemia genotypes. Materials and Methods We conducted a retrospective cross-sectional analysis of patients with hemoglobin SC and Sβ+ thalassemia genotypes followed at the Sickle Cell Center for Adults from 2008 to 2012. Data on baseline hematologic parameters and SCD-specific comorbidities were collected from review of electronic records. Results A total of 116 patients, 85 (73%) with hemoglobin SC disease and 31 (27%) with Sβ+-thalassemia, were included for analysis. Thirty-two (28%) patients had a verified history of non-catheter related VTE. Mean baseline hemoglobin levels were higher among individuals with a history of VTE compared to those without (11.7 g/dL vs. 11.0 g/dL, p=0.003). In addition, the prevalence of surgical splenectomy was higher among patients with VTE compared to those without (25.0% vs. 4.8%, p=0.001). On multivariate analysis, elevated baseline hemoglobin (odds ratio [OR] 2.45 (95% confidence interval [CI] 1.42–4.23) and history of surgical splenectomy (OR 5.76 [CI 1.43–23.22) were independently associated with VTE risk. Conclusions Higher baseline hemoglobin is a risk factor for non-catheter-related VTE in patients with hemoglobin SC or Sβ+ thalassemia genotypes. Surgical splenectomy, which is a known risk factor for VTE in other hemoglobinopathies such as β-thalassemia intermedia, is also associated with VTE in sickle variant syndromes. Future studies are needed to validate these findings and to investigate the mechanisms of hypercoagulability observed in patients with hemoglobin SC and Sβ+ thalassemia.

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Objectives Sickle cell disease (SCD) is associated with high healthcare utilization rates and poor outcomes in a subset of patients, although the underlying factors that predict this phenotype are poorly understood. Prior studies suggest that comorbid avascular necrosis (AVN) contributes to high healthcare utilization. We sought to clarify whether AVN independently predicts acute care utilization in adults with SCD and to identify characteristics of those with AVN that predict higher utilization. Methods We reviewed the medical records of 87 patients with SCD with symptomatic AVN and compared acute care utilization and clinical characteristics with 87 sex- and age-matched patients with SCD without symptomatic AVN. Patients with ≥2 years of follow-up were included. Outcomes were compared using bivariate analysis and multivariate regression. Results Our study included 1381 follow-up years, with a median of 7 years per patient. The AVN cohort had greater median rates of urgent care visits (3.2/year vs 1.3/year; P = 0.0155), admissions (1.3/year vs 0.4/year; P = 0.0002), and admission days (5.1 days/year vs 1.8 days/year; P = 0.0007). History of high utilization (odds ratio [OR] 4.28; P = 0.001), acute chest syndrome (OR 3.12; P = 0.005), pneumonia (OR 3.20; P = 0.023), hydroxyurea therapy (OR 2.23; P = 0.0136), and long-term transfusion (OR 2.33; P = 0.014) were associated with AVN. In a median regression model, AVN, acute chest syndrome, and pneumonia were independently associated with greater urgent care visits and admissions. Conclusions Symptomatic AVN was found to be an independent risk factor for acute care utilization in patients with SCD. Because this is a potentially modifiable factor, further studies are urgently needed to determine whether AVN prevention/early treatment interventions will alter utilization and improve outcomes for patients with SCD.

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Superficial angiomyxoma (SA) is a rare benign soft‐tissue tumor, arising sporadically or as the earliest manifestation of Carney's complex. When it arises sporadically, the breast is infrequently involved with only few cases reported in the literature. Key imaging findings include T2 signal hyperintensity on MRI and hypervascularity. In this study, we report the clinical, radiological, surgical, and histopathologic findings of a case of sporadic SA of the breast in a 16‐year‐old girl.

In the U.S., pulmonary embolism (PE) is a common cause of cardiovascular death. Right heart thrombus (RHT) occurs in approximately 4% of patients with PE, and when concurrent is associated with increased 30-day PErelated and all-cause mortality. The consensus on optimal management of acute massive or high-risk PE is unclear, and even less so for concurrent RHT. In this report, we review a successful multidisciplinary coordination of vacuum-assisted thrombectomy (VAT) of a complex pacemaker lead-associated RHT in a patient with concurrent acute PE and significant comorbidities, using the AngioVac system (Vortex Medical, Norwell, MA). VAT is a reasonable treatment option that should be considered particularly for patients who are poor surgical or thrombolytic candidates. Procedural success and patient outcomes can be further optimized through multidisciplinary collaboration such as with the Pulmonary Embolism Response Team (PERT) model.