Citations

Abstract Rationale There is sparse published literature on the causes and outcomes of hospitalization of patients with hereditary hemorrhagic telangiectasia (HHT). Objectives To evaluate rates of various complications, comorbidities, and in-hospital outcomes of patients with HHT using a large, multihospital inpatient database. Methods We identified patients with HHT in the U.S. Nationwide Inpatient Sample between 2000 and 2012. Rates of hemorrhagic, neurological, hepatic, and cardiopulmonary complications among hospitalized patients with HHT were evaluated. We also studied procedure use rates for blood transfusion, endoscopy, and epistaxis treatment. Hospitalization outcomes, including in-hospital mortality, discharge status, charges, and length of stay, were evaluated. Measurements and Main Results: We identified 10,293 patients with HHT. The mean age of the HHT population was 60.7 years. Sixty percent of patients were female. More than 75% of HHT hospitalizations occurred in those older than 50 years of age. Patients with HHT had high rates of bleeding-related complications, including anemia (53.3%), epistaxis (16.2%), and gastrointestinal bleeding (10.8%). Overall, bleeding complications accounted for 62.7% of HHT-related complications. Thirty-eight percent of hospitalized patients with HHT received one or more transfusions of a blood product. Cardiopulmonary complications were present in 41.0% of the cases. Congestive heart failure was the second most common individual complication among patients with HHT, affecting 19.9% of patients. The in-hospital mortality rate was 1.9%. Conclusions In this large, nationwide study, we found that nearly two-thirds of patients hospitalized with HHT experienced a bleeding-related complication. Nearly 40% of hospitalized patients with HHT required transfusion of blood products. Cardiopulmonary complications, including congestive heart failure, were the second most common complication. The high burden of bleeding-related complications points to a significant unmet clinical need for these patients.

Purpose To (1) evaluate long‐term outcomes of osteochondral allograft (OCA) with regard to clinical outcome scores, reoperation and failure rates, and (2) examine if certain factors predispose patients to worse outcomes. Methods A comprehensive review was performed with specific inclusion criteria for studies with long‐term outcomes after OCA. Studies reported on patient clinical scores such as Hospital for Special Surgery score, Knee Society Score (knee and function score), and Lysholm score. Reoperation and failure rates were recorded for each study. Modified Coleman Methodology Scores assessed study methodological quality. Results Five studies with a total of 291 patients (55% male, 45% female) and average age of 34.8 years (range, 15 to 69 years) were included. Of all lesions, 67% were on the femoral condyles, 29% on the tibial plateau, and 4% were patellofemoral. All scores (Knee Society Function Score, Knee Society Knee Score, and Lysholm score) have significant mean improvement from preoperative to final follow‐up. The mean postoperative Hospital for Special Surgery score was 84.1. The mean failure rate was 25% at 12.3 years with a reoperation rate of 36%. A total of 72% of the failures were conversion to total (68%) or unicompartmental (4%) knee arthroplasty and 28% involved graft removal, graft fixation, and graft revision. Patellofemoral lesions (83%) had a significantly higher reoperation rate than lesions involving the tibial plateau or the femoral condyles (34%, P = .01). Conclusions Overall, OCA demonstrated significant improvements in clinical outcome scores and good durability with successful outcomes in 75% of the patients at 12.3 years after surgery. Patellofemoral lesions are associated with decreased clinical improvement and more frequent reoperations. The orthopaedic literature is limited by heterogeneity in surgical technique, lesion and patient characteristics, and reporting of nonstandardized outcome measures. Level of Evidence Level IV, systematic review of Level II and IV studies.

AbstractBackgroundChildhood obesity is underdiagnosed in primary care practices. Our study aimed to compare rates of documentation of diagnosis of obesity and counselling for nutrition and physical activity at an academic primary care practice prior to and following implementation of a body mass index (BMI)‐based electronic point of care clinical reminder tool.MethodsWe performed a retrospective record review of children aged 2–18 years undergoing well child visits during any of three calendar years (2009, 2011 and 2013). The electronic clinical reminder tool was implemented in September 2010. Records of children with BMI > 95th percentile were electronically searched for terms of documentation of diagnosis of obesity and nutrition and physical activity counselling. Multivariable logistic regression models were used to adjust for potential patient and provider confounders.ResultsAmong those with BMI ≥ 95th percentile, there was a statistically significant increase in documentation of diagnosis of obesity (45.5% in 2009 vs. 52.4% in 2011; P < 0.001; adjusted odds ratio 1.36; 95% CI 1.13–1.65) and in counselling for physical activity and nutrition (66.8% in 2009 vs. 75.2% in 2011; P < 0.001; adjusted odds ratio 1.33; 95% CI 1.06–1.66) following implementation of the tool. Compared with 2011, there was no statistically significant increase in documentation of obesity (50.4%) or nutrition and physical activity counselling (77.9%) in 2013.ConclusionsThe implementation of a point of care clinical reminder tool was associated with improvement in documentation of diagnosis of obesity and counselling for nutrition and physical activity. Further studies are needed to determine the impact of these automated tools on weight outcomes in children.

BackgroundClear cell sarcoma typically arises in deep soft tissues of the foot/ankle. Primary head and neck clear cell sarcoma is rare. We report three molecularly confirmed primary head and neck clear cell sarcoma and review the literature.MethodsHead and neck clear cell sarcoma with no primary elsewhere were retrieved from archival/consultation files. English language literature review of all reported head and neck clear cell sarcoma was performed.ResultsThree cases were identified. The tumors, all in men, presented on the scalp of a 33‐year‐old, face of a 20‐year‐old and tongue of a 44‐year‐old. Tumors ranged from 0.6 to 1.4 cm. All had typical features of clear cell sarcoma, including nests of tumor cells divided by fibrous septae. One had multinucleated wreath‐like giant cells. One had a focal junctional component. Immunohistochemistry was positive for S100 (three out of three), HMB45 (three out of three) and Melan‐A (two out of three). All had EWSR1 rearrangements by fluorescence in situ hybridization. Follow up available for one patient revealed no evidence of disease after wide excision and radiation. Seven additional reported cases were identified and tabulated.ConclusionHead and neck clear cell sarcoma is rare but should be considered in the differential diagnosis of nested tumors with fibrous septae. Molecular analysis should be considered for definitive diagnosis regardless of location.

SummaryObjectiveThe role of percutaneous adrenal biopsy in a high‐risk population for adrenal malignancy has not been fully investigated. Our aim was to describe the clinical presentation leading to the adrenal biopsy and evaluate the diagnostic performance, complications and non diagnostic rate of adrenal biopsy.DesignSingle‐centre, retrospective cohort study.Patients and MeasurementsMedical records of patients who underwent adrenal biopsy between 1994 and 2014 were reviewed. Adrenal biopsy outcome was compared to a predefined reference standard.ResultsBiopsy was performed in 418 patients [62% men, median age 69 years (range, 15–91)] on 419 adrenal lesions, median size 3·1 cm (range, 0·6–24). The main indication for adrenal mass biopsy was (349/419, 83%) suspected adrenal metastasis from a known or suspected extra‐adrenal primary source. Only 116 of 419, 28% of cases had prebiopsy biochemical testing for pheochromocytoma. Biopsy‐related complications occurred in 4% of the patients. Histology revealed a metastasis in 231 of 419 (55%), benign adrenal tissue in 137 of 419 (33%), adrenocortical carcinoma in eight of 419 (2%), other lesions in 23 of 419 (5%) including seven cases of pheochromocytoma and six cases of infectious process. Biopsy was nondiagnostic in 20 of 419 (5%). All adrenal masses with unenhanced radiodensity ≤10 HU (42/137, 31%) proved to be benign adrenal adenomas. Adrenal biopsy diagnosed malignancy with a sensitivity of 88·5%, specificity of 91·5%, positive predictive value of 93·4% and negative predictive value of 85·5%.ConclusionWhen used in the appropriate clinical setting, adrenal biopsy is a powerful tool in the diagnostic algorithm of the evaluation of adrenal masses with features suspicious for malignancy. Efforts to increase awareness to perform biochemical testing for pheochromocytoma prior to adrenal biopsy are needed.

AbstractThe aim of this study was to investigate correlations between early subclinical findings (10‐ and 90‐day histology and gene expression data) and late outcomes (transplant glomerulopathy and graft loss) in positive crossmatch kidney transplants (+XMKTx). We compared 34 +XMKTx (19 receiving eculizumab and 15 receiving standard of care without eculizumab) to 13 −XMKTx (between August 2001 and August 2011). At 10 days, light microscopy identified subclinical inflammation in only 18% of +XMKTx, while intragraft gene expression identified inflammation in 79% (gene sets for activated macrophages, dendritic cells, NK cells or T cells). Inflammation persisted at 90 days and was associated with the development of transplant glomerulopathy by 2 years and graft loss. In contrast, endothelial cell (EC) changes present at 90 days by either electron microscopy or gene expression were not associated with transplant glomerulopathy or graft loss in this cohort. Eculizumab treatment did not appear to alter inflammation or EC changes. Therefore, intragraft inflammation might be an appropriate surrogate marker of progression and also a target of therapy to prevent chronic antibody‐mediated rejection.

Abstract Background: Human papillomavirus (HPV) is a causative agent in cervical cancer as well as anal, oropharyngeal, penile, and vaginal/vulvar cancers. Licensed, effective HPV vaccines have been available in the U.S. since mid-2006 for females and late 2011 for males and are recommended up to age 26. Despite this, in 2014, only 39.7% of U.S. girls and 21.6% of boys 13 to 17 years of age completed the three-dose vaccine series. ‘Lack of knowledge’ was a primary reason cited among parents for not vaccinating their children. HPV vaccination rates are lower still among 19-26 year olds, particularly among the uninsured. We assessed awareness and knowledge of HPV disease, HPV-related cancers, and HPV vaccines among working, uninsured adults and used 2014 data from the Health Information National Trends Survey (HINTS cycle 4) as a national benchmark against which to compare our data. Methods: Self-administered surveys were offered to patients attending appointments at the Volunteers in Medicine (VIM) clinic in Jacksonville, FL between September 10, 2014 and October 24, 2014. VIM provides free primary care for working individuals without insurance living and/or working in Duval County. Patients were eligible to participate if they were at least 18 years of age, not in acute distress, and had not completed a survey during pilot-testing. Surveys were available in English and Spanish and contained a validated HPV knowledge measure assessing disease knowledge and vaccine knowledge. Additionally, HPV-related cancer knowledge was assessed: 5 HPV-related (cervical, anal, throat, penile, vaginal/vulvar) and 4 not currently known to be HPV-related (lung, breast, prostate, skin). High knowledge was defined as having a knowledge score in the upper quartile of positive values. Odds ratios (OR) and 95% confidence intervals (CI) were estimated using logistic regression to assess the association of demographic characteristics with having high knowledge of HPV, HPV vaccines, and HPV-related cancer. Additional models assessed individual cancers. During the study period 356 patients were eligible to participate; of these, 55 declined (84% participation rate). Of the 301 surveys, 5 surveys were excluded from analysis due to excessive missing data; a total of 296 surveys were analyzed. Results: 50.3% of participants (n=149) had heard of HPV, 37.2% (n=110) correctly indicated that HPV is sexually transmitted, and 32.1% (n=95) had heard of the HPV vaccine. In HINTS 4, these estimates were 63.6%, 44.0%, and 62.7%, respectively. In adjusted models, high HPV disease knowledge was associated with race (OR non-white = 0.39 (0.19, 0.78)) and education (OR &gt;high school = 2.34 (1.14, 4.84)) while high HPV vaccine knowledge was associated with race (OR non-white = 0.42 (0.21, 0.82)), education (OR &gt;high school = 5.04 (2.30, 11.06)), and sex (OR male = 0.34 (0.12, 0.94)). Of the HPV-related cancers, 43.9% answered correctly for cervical, 9.1% for anal, 11.1% for mouth/throat, 11.5% for penile, and 30.7% for vaginal/vulvar cancer. Males were less likely than females to know that HPV is a causative agent for cervical (OR=0.41 (0.22, 0.75)) and vaginal/vulvar cancers (OR=0.43 (0.22, 0.86)). Conclusion: Using estimates obtained in HINTS 4 as a benchmark, awareness and knowledge of HPV, HPV-related cancers, and HPV vaccines were low among working, uninsured adult patients. In addition to improving access to HPV vaccination (e.g. volunteer clinics), the findings demonstrate a need for strategies to educate medically underserved adults about HPV. Maximizing access to HPV vaccination, including catch-up vaccination for adults up to age 26 are consistent with the recommendations of the President's Cancer Panel, the American College of Obstetricians and Gynecologists, the American College of Physicians, and the Advisory Committee on Immunization Practices. Citation Format: Carmen Radecki Breitkopf, Lila Finney Rutten, Debra Jacobson, Patrick Wilson, Monica Albertie, Robert M. Jacobson, Gerardo Colon-Otero. Awareness and knowledge of HPV, HPV-related cancers, and HPV vaccines in a working, uninsured clinic population. [abstract]. In: Proceedings of the Eighth AACR Conference on The Science of Health Disparities in Racial/Ethnic Minorities and the Medically Underserved; Nov 13-16, 2015; Atlanta, GA. Philadelphia (PA): AACR; Cancer Epidemiol Biomarkers Prev 2016;25(3 Suppl):Abstract nr B91.

ABSTRACT Measles is a highly contagious, acute viral illness. Immune cells within the airways are likely first targets of infection, and these cells traffic measles virus (MeV) to lymph nodes for amplification and subsequent systemic dissemination. Infected immune cells are thought to return MeV to the airways; however, the mechanisms responsible for virus transfer to pulmonary epithelial cells are poorly understood. To investigate this process, we collected blood from human donors and generated primary myeloid cells, specifically, monocyte-derived macrophages (MDMs) and dendritic cells (DCs). MDMs and DCs were infected with MeV and then applied to primary cultures of well-differentiated airway epithelial cells from human donors (HAE). Consistent with previous results obtained with free virus, infected MDMs or DCs were incapable of transferring MeV to HAE when applied to the apical surface. Likewise, infected MDMs or DCs applied to the basolateral surface of HAE grown on small-pore (0.4-μm) support membranes did not transfer virus. In contrast, infected MDMs and DCs applied to the basolateral surface of HAE grown on large-pore (3.0-μm) membranes successfully transferred MeV. Confocal microscopy demonstrated that MDMs and DCs are capable of penetrating large-pore membranes but not small-pore membranes. Further, by using a nectin-4 blocking antibody or recombinant MeV unable to enter cells through nectin-4, we demonstrated formally that transfer from immune cells to HAE occurs in a nectin-4-dependent manner. Thus, both infected MDMs and DCs rely on cell-to-cell contacts and nectin-4 to efficiently deliver MeV to the basolateral surface of HAE. IMPORTANCE Measles virus spreads rapidly and efficiently in human airway epithelial cells. This rapid spread is based on cell-to-cell contact rather than on particle release and reentry. Here we posit that MeV transfer from infected immune cells to epithelial cells also occurs by cell-to-cell contact rather than through cell-free particles. In addition, we sought to determine which immune cells transfer MeV infectivity to the human airway epithelium. Our studies are based on two types of human primary cells: (i) myeloid cells generated from donated blood and (ii) well-differentiated airway epithelial cells derived from donor lungs. We show that different types of myeloid cells, i.e., monocyte-derived macrophages and dendritic cells, transfer infection to airway epithelial cells. Furthermore, cell-to-cell contact is an important component of successful MeV transfer. Our studies elucidate a mechanism by which the most contagious human respiratory virus is delivered to the airway epithelium.