tbuckley/cabot-search huggingface dataset:
Eczema apps conformance with clinical guidelines: a systematic assessment of functions, tools and content
Authors: L.S. Galen, X. Xu, M.J.A. Koh, S. Thng, J. Car
Journal: British Journal of Dermatology (2019)

Eczema is a prevalent complex skin condition requiring active disease monitoring and personalized education. No studies have assessed the quality of apps that aim to support eczema self-management.To evaluate the quality and comprehensiveness of English, Chinese and Spanish self-management eczema smartphone apps for patients and/or their caregivers.A systematic assessment of eczema apps from July 2018 to November 2018. The assessment criteria were based on conformance with international eczema guidelines. The following domains were assessed: consistency and comprehensiveness of eczema-specific educational information; quality and comprehensiveness of eczema-specific tracking functions; compliance with health information best practice principles.In total, 98 apps were assessed: 82 (84%) provided educational information; 38 (39%) tracking functions; and 13 (13%) both. We found that 34% (28/82) of apps provided misleading information, particularly regarding aspects of treatment and disease progression of eczema. Only 15% (12/82) provided international guideline supported information on pharmacological therapies and 16% (13/82) on nonpharmacological therapies. Among 38 apps with a tracking function, 82% (31/38) measured specific symptoms, disease severity or current skin condition and 89% (34/38) helped users to record medication usage including application of topicals. Environmental or dietary allergens were recorded by 34% (13/38). None of the included apps complied with all criteria for educational information, tracking functions or health information principles.Eczema apps have not yet reached their potential. The large variance in quality of eczema apps highlights the need for quality assurance mechanisms for health apps and guidance for clinicians that would enable them to make personalized recommendations for patients and caregivers. What's already known about this topic? There is limited information about the quality of eczema self-management smartphone apps on the global market. What does this study add? This systematic assessment evaluated all English, Chinese and Spanish language apps that support eczema self-management. The majority did not conform with information in guidelines and insufficiently support evidence-based self-management. The large variance in the quality of eczema apps highlights the need for mechanisms to ensure app quality and to guide personalized app selection for patients, caregivers and doctors.
PB1845 THE EFFICACY OF TRANEXAMIC ACID IN MINOR SURGERY WITH BLEEDING DISORDERS PATIENTS: 17 YEARS EXPERIENCE AT UNIVERSITY HOSPITAL
Authors: S. Al Jaouni, D. Farsi, S. Al Muhayawi
Journal: HemaSphere (2019)

Background: Tranexamic acid (TXA) is an antifibrinolytic agent commonly used to reduce blood loss in different medical disorders. Aims: To assess the efficacy and outcome to use TXA in dental work with patient with bleeding disorders at our institute over 17 years period. Methods: This is a retrospective study to assess the efficacy of the TXA in all dental works for patients with bleeding disorders treated at King Abdulaziz University Hospital (KAUH) at King Abdulaziz University, Jeddah, Kingdom of Saudi Arabia, between 2000‐2017, also to assess the requirements of transfusion after the procedures. Results: One hundred twenty one patients with bleeding disorders underwent dental and minor surgery over 17 years with patient age range 3‐46 years. Tropical oral TXA in majority of the cases only 5% used intravenous TXA in hospitalized patients. Less than 5% required transfusion after the procedures. Summary/Conclusion: Strong evidence supports the use of TXA in minor surgery with bleeding disorders in our institute over 17 years. Markedly decrease and minimize the need of transfusion after use TXA in dental works.
Abstract MP57: Associations of Pentraxin 3 with Frailty: The Cardiovascular Health Study (CHS)
Authors: Nancy S Jenny, Alice M Arnold, Lewis H Kuller, Jeremy D Walston, Russell P Tracy, Linda P Fried, Bruce M Psaty, Anne B Newman
Journal: Circulation (2016)

Background: Pentraxin 3 (PTX3) is an inflammatory biomarker primarily reflecting vascular damage. PTX3 is associated with cardiovascular disease (CVD); however, associations with frailty, another inflammation-related disease of aging, are unknown. Methods: At baseline, we examined these associations in 1,948 white and black men and women (mean age 73 y) in the Cardiovascular Health Study (CHS) who had measures of PTX3 and were free of dementia, Parkinson’s disease and depression. PTX3 was determined by immunoassay (coefficient of variation 10%). Robust regression models were minimally adjusted for age, sex, ethnicity and clinic site with additional adjustments for obesity, smoking, education, C-reactive protein (CRP) and prevalent subclinical or clinical CVD. Results: Levels of PTX3 ranged from 0.24 - 23.05 ng/ml, mean 2.36 ng/ml (standard deviation 2.00 ng/ml), in these older adults. PTX3 was significantly associated with unintentional weight loss (p&lt;0.001), slow walk time (p=0.04) and low grip strength (p=0.03), but not the other domains of frailty, exhaustion and physical activity, both p&gt;0.2. Increased PTX3 was also associated with frailty status comparing those who were not frail (0 domains of frailty, N=879) to those who were intermediate frail (presence of 1-2 domains of frailty, N=926) or frail (≥3 domains, N=143). In minimally adjusted models, the relative risk (RR) for intermediate frailty was 1.22 (95% confidence interval 1.04-1.44) and 1.70 (1.25-2.29) for prevalent frailty, comparing both to non-frail. Results were slightly attenuated but remained significant in fully adjusted models; RR 1.19 (1.01-1.41) for intermediate and 1.59 (1.16-2.18) for frail. As some biomarkers show sex-dependent associations, we stratified on sex. In fully adjusted models, increased PTX3 was associated with intermediate frailty (RR 1.3; 1.00-1.69) and frailty (RR 2.14; 1.24-3.67) in men, but did not appear to be associated in women (RR 1.13; 0.91-1.42 for intermediate and 1.28; 0.85-1.92 for frail); however, the interaction term was not significant (p=0.2). Conclusions: In these older adults, PTX3 was associated with some domains of frailty and frailty status. Associations were independent of the presence of clinical and/or subclinical CVD and CRP, a biomarker of general inflammation. PTX3 may reflect ongoing vascular damage in multiple aging-related diseases such as CVD and frailty. Further study is needed to evaluate the potential for PTX3 to act as a biomarker of healthy aging.
SP0085 WHOM TO IDENTIFY AND INVITE TO AN FLS? WHO IS THAT FRACTURE PATIENT?
Authors: Bo Abrahamsen
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Though osteoporosis can be diagnosed through the presence of fragility fractures in the absence of other aetiology such as myeloma or metastases, patients with osteoporosis are also at increased risk of high impact traumatic fractures. Further, distinguishing between fracture mechanisms through chart review is often difficult and may lead to patients with osteoporosis being missed. On the other hand, focusing on identifying and treating patients at high imminent fracture risk rather than milder degrees of osteoporosis makes for better cost utility. <h3>Objectives:</h3> To review the demographics of patients presenting with fragility fractures and the evidence for targeting osteoporosis assessment for each category of fracture in order to balance the need for maximum risk reduction with the requirement for an evidence based strategy. <h3>Methods:</h3> Narrative review of recent epidemiology studies and national and international guidelines <h3>Results:</h3> Based on epidemiology data from Iceland(1) and Denmark(2,3), the risk of subsequent fractures following a sentinel fracture event is critically dependent on the recency and the site of the initial fracture, with the risk of new fractures being particular high after major osteoporotic fractures(1) and pelvic fractures but less so after lower leg fractures(2). In Danish women(2), 29.5% of patients suffering a pelvic fracture went on to sustain a hip fracture in the next ten years, compared with 25.9% after a vertebral fracture but only 12.5% after a lower leg fracture. Further, despite the high recurrent fracture risk in the FLS setting, it is important to appreciate that the majority of hip fracture patients have not consulted with a prior fracture in the last ten years prior to their hip fracture(3). The following issues will be addressed in more detail 1) Demographics of fracture patients with particular emphasis on age, sex and BMD. 2) Which fracture types are indicators of elevated risk of subsequent major osteoporotic fractures? 3) Which fracture types will respond to osteoporosis treatment? 4) What is the role of DXA in FLS? 5) Identifying vertebral fractures <h3>Conclusion:</h3> FLS patients are at elevated risk of sustaining additional fractures both in the long term and in the short term, with risks being particularly high in the first years after the sentinel fracture and especially if the initial fracture is a pelvic fracture or a major osteoporotic fracture. <h3>References:</h3> [1] Kanis, Osteoporos Int 2018, 29:1747–1757 [2] Hansen, Osteoporos Int 2015, 26:513–519 [3] Frederiksen, Osteoporos Int 2018; 29:109–114 <h3>Disclosure of Interests:</h3> None declared
PS1406 WEEKLY CARFILZOMIB, LENALIDOMIDE AND DEXAMETHASONE UNTIL PROGRESSION IN RELAPSED REFRACTORY MULTIPLE MYELOMA
Authors: C. Gruchet, V. Richez, S. Guidez, C. Tomowiak, G. Fouquet, A. Machet, N. Moya, F. Sabirou, A. Levy, A. Bobin, H. Gardeney, S. Bouyer, F. Bridoux, V. Javaugue, I. Azais, G. Durand, H. Avet Loiseau, X. Leleu
Journal: HemaSphere (2019)

Background: Carfilzomib, Lenalidomide and Dexamethasone association (KRd) has led to approval in early RRMM. On ASPIRE International phase 3 study Carfilzomib was used on a twice a week basis at 27 mg/m 2 and limited to 18 months exposure. We have reported already that KRd on a weekly basis at 56 mg/m 2 had similar and safe properties. In this abstract we report the long‐term exposure data on KRd weekly given at 56 mg/m 2 until progression. Aims: We aimed to evaluate the efficacy of KRd given on a prolong duration beyond 18months, and to validate the safety profile of continuous exposure to Carfilzomib. Methods: 28 patients were prospectively recruited. Carfilzomib 20/56 mg/m 2 was administered on days 1,8,15 on 28 days cycles. Lenalidomide (25 mg/day) was given 21/28 days and Dexamethasone was administered weekly. Patients were treated until progression. Results: With a median follow‐up at start of KRd of 30 months, 50% of patients relapsed and 39% died. 24/28 patients received 1 prior line of treatment. 8/28 patients are still on treatment with duration &gt; 24 month and 6/28 with duration &gt; 30 months. The median number of cycles was 15. ORR and CBR was 85.7% and 89.3%, whom 46% ≥ CR; with a median DOR of 13 months and 43% having more than 18 months. 6 patients had negative MRD at 10 –6 and normalized PET CT. Median of OS is not reached, and the 30 month‐expected OS from the start of KRd was 56%. The median PFS and EFS was at 29 months, and the 30 month‐expected PFS and EFS was 45%. PFS and EFS being superimposable speaks to that there was no safety concern related to prolonged exposure to K. Only 4 patients stopped KRd for safety issues. Hematologic and non‐hematologic adverse events ≥ grade 3 were reported in 16/28 and 10/28 patients. Adverse events ≥ grade 3 seen in ≥10% of patients were neutropenia, thrombocytopenia, vomiting and pyrexia. Of note, 5 patients (18%) were 3 65 years old and these patients showed similar data compared to the studied cohort. Summary/Conclusion: KRd weekly at 20/56 mg/m 2 is effective and safe to early RRMM patients, provides improved safety profile to patients allowing treating patients until progression. Further studies are warranted to confirm this data on a larger early RRMM population and validate the concept of long duration of treatment using Carfilzomib combination.
Introduction of anti-TNF therapy has not yielded expected declines in hospitalisation and intestinal resection rates in inflammatory bowel diseases: a population-based interrupted time series study
Authors: Sanjay K Murthy, Jahanara Begum, Eric I Benchimol, Charles N Bernstein, Gilaad G Kaplan, Jeffrey D McCurdy, Harminder Singh, Laura Targownik, Monica Taljaard
Journal: Gut (2019)

Objectives To better understand the real-world impact of biologic therapy in persons with Crohn’s disease (CD) and ulcerative colitis (UC), we evaluated the effect of marketplace introduction of infliximab on the population rates of hospitalisations and surgeries and public payer drug costs. Design We used health administrative data to study adult persons with CD and UC living in Ontario, Canada between 1995 and 2012. We used an interrupted time series design with segmented regression analysis to evaluate the impact of infliximab introduction on the rates of IBD-related hospitalisations, intestinal resections and public payer drug costs over 10 years among patients with CD and 5 years among patients with UC, allowing for a 1-year transition. Results Relative to what would have been expected in the absence of infliximab, marketplace introduction of infliximab did not produce significant declines in the rates of CD-related hospitalisations (OR at the last observation quarter 1.06, 95% CI 0.811 to 1.39) or intestinal resections (OR 1.10, 95% CI 0.810 to 1.50), or in the rates of UC-related hospitalisations (OR 1.22, 95% CI 1.07 to 1.39) or colectomies (OR 0.933, 95% CI 0.54 to 1.61). The findings were similar among infliximab users, except that hospitalisation rates declined substantially among UC patients following marketplace introduction of infliximab (OR 0.515, 95% CI 0.342 to 0.777). There was a threefold rise over expected trends in public payer drug cost among patients with CD following infliximab introduction (OR 2.98,95% CI 2.29 to 3.86), suggesting robust market penetration in this group, but no significant change among patients with UC (OR 1.06, 95% CI 0.955 to 1.18). Conclusions Marketplace introduction of infliximab has not yielded anticipated reductions in the population rates of IBD-related hospitalisations or intestinal resections, despite robust market penetration among patients with CD. Misguided use of infliximab in CD patients and underuse of infliximab in UC patients may largely explain our study findings.
Publisher Correction: Immune induction strategies in metastatic triple-negative breast cancer to enhance the sensitivity to PD-1 blockade: the TONIC trial
Authors: Leonie Voorwerk, Maarten Slagter, Hugo M. Horlings, Karolina Sikorska, Koen K. van de Vijver, Michiel de Maaker, Iris Nederlof, Roelof J. C. Kluin, Sarah Warren, SuFey Ong, Terry G. Wiersma, Nicola S. Russell, Ferry Lalezari, Philip C. Schouten, Noor A. M. Bakker, Steven L. C. Ketelaars, Dennis Peters, Charlotte A. H. Lange, Erik van Werkhoven, Harm van Tinteren, Ingrid A. M. Mandjes, Inge Kemper, Suzanne Onderwater, Myriam Chalabi, Sofie Wilgenhof, John B. A. G. Haanen, Roberto Salgado, Karin E. de Visser, Gabe S. Sonke, Lodewyk F. A. Wessels, Sabine C. Linn, Ton N. Schumacher, Christian U. Blank, Marleen Kok
Journal: Nature Medicine (2019)
PS953 SURVIVAL OUTCOME OF SECOND ALLOGENEIC‐HCT IN ADULT ALL PATIENTS WHO RELAPSED AFTER PREVIOUS ALLOGENEIC‐HCT
Authors: S.Y. Yoon, J.‐H. Yoon, C. Kim, J. Lee, G.J. MIn, K.‐S. Eom, H.‐J. Kim, J.W. Lee, S. Lee
Journal: HemaSphere (2019)

Background: Allogeneic hematopoietic cell transplantation (allo‐HCT) is a potentially curative option for adult patients with acute lymphoplastic leukemia (ALL). However, no standard therapy is elucidated for patients who relapsed after allo‐HCT. Aims: We analyzed patients treated with second allo‐HCT and tried to find out risk factors for survival outcomes. Methods: We retrospectively analyzed 35 adult ALL patients (median 35 years old, range 16–66) treated with second allo‐HCT in second remission from 2010 to 2017, all of them relapsed after previous allo‐HCT (median 12.8 months, range 2.9–99.4). After second remission, most patients underwent second allo‐HCT from available donor within 6 months, but some patients with Ph‐positive ALL responding to salvage tyrosine kinase inhibitors or with isolated EMR were delayed for second allo‐HCT. Results: Of 35 patients, 3 patients were T‐ALL, 16 were Philadelphia chromosome (Ph)‐negative, and 16 were Ph‐positive B‐ALL. Extramedullary relapse (EMR) were observed in 11 patients, and 5 of them were isolated EMR without hematological relapse. At the time of second allo‐HCT, 17 were in complete remission (CR) and 18 were in CR without full hematological recovery. Among 16 Ph‐positive ALL patients, 9 were in complete molecular response, 2 were in major molecular response, and 5 were in poor molecular response. After median follow‐up of 38.2 months (range 7.5–86.3), 3‐year overall survival (OS) and disease‐free survival (DFS) were 31.9% and 26.0%, respectively. Cumulative inicidence of non‐relapse mortality (NRM) and relapse (CIR) at 3 years were 29.1% and 45.4%, respectively. Acute GVHD was observed in 17 (12 with grade I‐II, 4 with grade III, 1 with grade IV) and chronic GVHD was observed in 14 (mild 7, moderate 4, severe 3) out of 31 patients who were alive at least 100 days after HCT. We identified late relapse &gt; 6 months and early allo‐HCT within 6 months (48.1% vs. 12.5% ; HR = 0.27, 95%CI 0.1–0.7, p = 0.014) and chronic GVHD (49.0% vs. 27.8% ; HR = 0.28, 95%CI 0.1–0.8, p = 0.021) showed favorable 3‐year OS in the entire cohort. Among patients with Ph‐positive subgroup, post‐HCT complete molecular response was related with superior OS (66.7% vs. 0.0% ; HR = 0.09, 95%CI 0.01–0.69, p = 0.021). Summary/Conclusion: Our data suggested that second allo‐HCT might be a feasible choice for some patients relapsed after previous allo‐HCT as a curative treatment option when allo‐HCT is performed earlier in late relapse case. Safe salvage therapy and additional post‐HCT preemptive therapy should be considered for better transplant outcomes.
PB2248 FIRST KOREAN CASE OF THROMBOCYTHEMIA 1 WITH THPO MUTATION (C.13+1G&gt;A, SPLICING) DIAGNOSED BY TARGETED EXOME SEQUENCING
Authors: N. Jung, Y. J. Shim, H. S. Kim
Journal: HemaSphere (2019)

Background: Thrombocythemia is a kind of myeloproliferative disorder characterized by excessive platelet production and increased numbers of circulating platelets. Thrombocythemia is also associated with thrombotic or hemorrhagic episodes and leukemic transformation. Thrombocythemia 1 (THCYT1) is a very rare autosomal hereditary thrombocythemia caused by heterozygous mutation in the thrombopoietin gene (THPO) on chromosome 3q27. Aims: We introduce the first Korean boy who was confirmed as thrombocythemia 1 with a THPO gene mutation by next generation sequencing. Methods: We performed targeted exome sequencing to find out genetic cause to induce thrombocytosis. Library preparation was performed by the TruSight One sequencing panel (Illumina, USA). Massively parallel sequencing was conducted by NextSeq (Illumina, USA). An identified variant was confirmed by Sanger sequencing of the patient and his parents. Results: A 25 day‐old male infant who had congenital absent distal phalanges of left hand was referred to our medical center for the evaluation of leukocytosis – white blood cell (WBC) count 32,700/ μL. His older sister had a history of persistent thrombocytosis with unknown etiology from her age of 5 months in another hospital. In his laboratory test, WBC count gradually increased to 57,610/μL and the monocyte count in peripheral blood increased over 1,000/μL. His bone marrow study showed increased myeloid series with some blasts (5.1%). At first, we suspected that he had juvenile myelomonocytic leukemia (JMML), but his clinical and genetic evaluations were insufficient for the diagnostic criteria of JMML. He was followed up for laboratory tests at the outpatient clinic regularly. At his 4 months of age, leukocytosis resolved spontaneously. On the other hand, platelet count increased to more than 1,000,000/μL and sustained. The second bone marrow study revealed megakaryocyte hyperplasia. The genetic evaluations for ET (JAK2, MPL, and CALR gene sequencing) were performed, and there was no pathogenic variant in them. Based on his history and laboratory findings, we performed targeted exome sequencing for his family. Finally, at the 11 months of his age, the patient was confirmed as THCYT1 with a THPO gene mutation (NM_000460.2:c.13+1G&gt;A, splicing) which is also found in his father, as mosaicism, and his sister who was had a history of thrombocytosis. Also, of the 4 of his family, thrombocytosis was only detected in his older sister. The pedigree suggested that the disease was in herited in an autosomal dominant manner. He has been followed over a period of 15 months up to now with treatment of low dose acetyl salicylic acid. No thrombotic or hemorrhagic events developed during the follow up period. Summary/Conclusion: This is the first report of thrombocythemia 1 patient with THPO gene mutation in Korea. image