tbuckley/cabot-search huggingface dataset:
Proton radiotherapy for rhabomyosarcoma: Preliminary results from a multicenter prospective study.
Authors: Torunn I. Yock, Jackie Szymonifka, Alison M. Friedmann, Anita Mahajan, Beow Yong Yeap, Shannon M. MacDonald, Hallie Bieber Kasper, David Grosshans, Karen Chayt Marcus, Nancy Tarbell
Journal: Journal of Clinical Oncology (2012)

9585 Background: Pediatric rhabdomyosarcoma (RMS) is commonly cured with chemotherapy and radiation. However, late effects of radiotherapy (RT) can be disabling. Proton RT irradiates less normal tissue, which should result in fewer late side effects of treatment. The purpose of this study was to describe the disease control and side effect profile of proton RT in RMS patients (pts). Methods: Eligible pts included those with localized disease and metastatic embryonal RMS if age 2-10. All pts were treated with VAC (vincristine, actinomycin, cyclophosphamide) based chemotherapy and proton RT, median dose 50.4 Gy (36-50.4 Gy RBE ). Concurrent enrollment in COG protocols was allowed. All pathology/imaging was reviewed at the treating institution. Results: 47 pts with RMS were prospectively enrolled from January 2005 to June 2011 and evaluable for analysis. Median age was 3.1 yrs, (range 0.6-15.6 years; M/F ratio 23:24). There were 1, 7, 37, and 2 Group I, II, III and IV and 14, 12, 19 and 2, Stage I-IV pts respectively, and 33 with embryonal 14 with alveolar/other. Most common sites included PM (48.9%), orbital (23.4%) bladder/prostate (6.4%), H&amp;N non-PM (6.4%), extremities (4.3%), trunk/abdomen 2.1%), perineal/anal region (2.1%) and other (6.4%). Median follow-up is 15.2 months. One/two-year overall survival (OS) and progression-free survival (PFS) for the entire group is 94/81% (OS) and 79/73% (PFS). 2-year OS for stage I,II/III,IV pts is 91/66% (OS, p=0.114) and two-year PFS for these pts is 86/57%, respectively, (p=0.083. 16 (34%) had grade 3/4 acute toxicities attributable to the radiation during treatment, the most common of which was mucositis/oral pain (12.8%), anorexia (4.3%), and erythema (4.3%). Among the 24 pts analyzable for late toxicities with at least 2 yrs of follow up, there were no grade 3 or 4 late toxicities attributable to radiation. Conclusions: Early results of this prospective trial demonstrate comparable disease outcomes and thus far limited late effects in a young pediatric RMS population. However, additional follow up is needed to determine if protons truly reduce rates and severity of late effects compared with photon cohorts published in the literature.
FRI0237 OMEGA-3 AND OMEGA-6 FATTY ACIDS IN SJöGREN’S SYNDROME: CLINICAL IMPLICATIONS AND THEIR ASSOCIATION WITH INFLAMMATION
Authors: Gabriela Hernandez-Molina, Carlos Castrejón-Morales, Omar Granados-Portillo, Ivette Cruz-Bautista, Narlly Ruiz-Quintero, Iliana Manjarrez, Diego Hernández-Ramírez, Guadalupe Lima, Miguel Astudillo-Angel, Luis Llorente
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Essential ω-3 and ω-6 fatty acids (FA) are natural modulators of inflammation activity; however, their role in primary Sjögren's syndrome (pSS) is unknown. <h3>Objectives:</h3> To evaluate the intake and serum levels of ω-3 and ω-6 FA in patients with pSS, and to correlate them with ocular and oral signs and symptoms, disease activity as well as with the presence of a panel of inflammatory chemokines/cytokines in saliva and tears. <h3>Methods:</h3> We included 108 patients with pSS according to EULAR/ACG criteria. We excluded patients under ω-3 and ω-6 supplementation. Dietary information was obtained from a semiquantitative food frequency questionnaire of one-day reminder (applied by a trained interviewer), and processed using computerized nutritional analysis software. Fasting blood samples were collected. We measured the serum levels of ω-3 (α-linolenic acid [α-L], eicosapentaenoic acid [EPA], docosahexaenoic acid [DHA]) and ω-6 (linoleic acid [LA], arachidonic acid [AA]) using gas chromatography flame ionization. We assessed the ESSDAI score, ESPPRI, OSDI, tear film break-up time, Schirmer-I test and the SICCA Ocular Staining Score (OSS). In a subgroup of patients, we obtained tears and saliva samples that were frozen at -86°C until assayed. Once defrosted, the levels of CXCL8, CXCL10, CCL2, IL-22 and IL-21 in saliva and CXCL8, CXCL10, CCL2 and CXCL9 in tears were measured by Luminometry. <h3>Results:</h3> The median age was 56.12 ± 13.7 years, 94.4% women and median disease duration 10 years. The FA ω-3 and ω-6 intake was 0.43 g/day and 3 g/d, respectively. The levels of α-L were 6.6 μg/mL, DHA 26 μg/mL, total ω-3 (α-L +DHA) 25.4 μg/mL, LA 168.5 μg/mL, AA 34 μg/mL, and total ω-6 (LA+AA) 205 μg/mL. We did not find a correlation among serum levels and food intake. Thus, further analyses were focused on serum results. We found a negative correlation between α-L and the OSDI (ρ= -0.42, p= 0.01) and ESSDAI (ρ= -0.26, p=0.03) as well of DHA and ESSDAI (ρ= -0.30, p= 0.01). The rest of the variables were not associated. In tears, there was a positive correlation of AA and CXCL9 (ρ= 0.48, p= 0.04), whereas in saliva, we observed a negative correlation between α-L, DHA and total ω3 (α-L+DHA) with CCL2. We also observed a negative correlation between total ω6 (LA+AA) and IL-21, and the ω6/ω3 ratio with IL-22. <h3>Conclusion:</h3> Our pSS patients had deficient FA omega intake. We observed lower ocular symptoms, lower ESSDAI scores and salivary levels of CCL2 among patients with higher levels of FA ω-3. Our study suggest that low serum levels of ω-3 might be implicated in the perpetuation of chronic inflammation. <h3> Disclosure of Interests:</h3> None declared
4 Relationship between childhood overweight/obesity indicators and vigorous physical activity among NHANSE III boys (8 to 11 years old): different interpretation between body mass index and subcutaneous adiposity size and shape
Authors: H Kim, E Petherick, I Varela-Silva
Journal: British Journal of Sports Medicine (2019)

Aim The aim of this study was to investigate whether the relationship between BMI and vigorous physical activity (VPA) differs when both the overall and regional distribution of subcutaneous adiposity (SA) are considered as alternative fatness indicators of BMI. Methods The sample data were obtained from the third National Health and Nutrition Examination Survey (NHANES III). Four skinfolds (triceps, subscapular, supraspinal, thigh), BMI, age, race/ethnicity, height, and VPA (i.e., times per week exercise made you sweat) data of 1,028 boys (8–11 yrs) were extracted. The overall and regional distribution of the four skinfolds were calculated into Subcutaneous Adiposity Size and Shape (SASS) variables based on the description provided in Healy and Tanner’s method (1): transform all four skinfolds into logarithms, 2) calculate average (SA-size estimation), 3) calculate difference between the average and each log-transformed skinfold, 4) run principal component analysis (SA-shape estimation). The samples were categorized into three groups based on participants’ VPA levels (very-active: VPA&gt;5 times, active: 2&lt;VPA&lt;4 times, non-active: VPA&lt;1 time). Then, MANCOVA/ANCOVA (BMI) were conducted separately to ascertain whether the fatness indicators provide consistent interpretations of the relationship with VPA. Results The MANCOVA showed significant interaction between the SASS and VPA (p&lt;0.05). The MANCOVA Bonferroni-adjustment results indicated that SA-size (F(2,938)=5.528, p&lt;0.005; partial η2=0.012) and subscapular-to-thigh SA-shape was significantly associated with VPA (F(2,938)=9.587,p&lt;0.0005; partial η2=0.020), but not triceps-supraspinal SA-shape (F(2,938)=1.485,p=0.227; partial η2=0.003). This means that the very active and active boys had significantly larger SA-size and larger thigh and smaller subscapular SA-shape than the non-active boys. The ANCOVA result showed no BMI difference between the VPA groups (F(2,1024)=1.296, p=0.274). Conclusion It was concluded that the interpretations made via SASS and BMI when associated with VPA are different. Future studies need to further investigate whether tackling childhood obesity by focusing on BMI-based obesity is affective approach to reduce body fat. Reference Healy MJR, Tanner JM. Size and shape in relation to growth and form. Symp Zool Soc Lond 1981;46:19–35.
PB1973 CONTRIBUTION OF NGS IN THE DIAGNOSIS OF HEREDITARY SPHEROCYTOSIS: CASE REPORT
Authors: G. Verstraete, C. Graux, E. Collinge, J. Depaus, H. Vellemans, P. Bianchi, B. Gulbis
Journal: HemaSphere (2019)

Background: Hereditary spherocytosis (HS) is the most frequent cause of non‐immune haemolytic anaemia (HA) among individuals of Northern European ancestry; its estimated prevalence is 1:2000. HS results from various molecular defects leading to decreased production or abnormality of one or more red blood cell (RBC) membrane proteins i.e., ankyrin, protein 4.2, band 3 and spectrin. The majority of HS cases (75%) are inherited in an autosomal dominant manner. Especially because of the spherical shape, RBCs are trapped in the spleen and have a shortened life span. Among other factors, according to the importance of the deficiency or protein abnormality it conducts to a clinical expression that may range from asymptomatic to severe chronic HA. Most often a phenotypic diagnosis can be made but next‐generation gene sequencing (NGS) has a place in difficult cases i.e. unsolved cases of HA, transfusion prior to diagnosis and discrepancy between clinical phenotype and diagnosis. Aims: We report here the case of a 18‐year‐old patient with a suspected HS, prominent splenomegaly and unknown personal or family history. Methods: Case report: The patient complained of fatigue for several months and grade 2 dyspnea. In the past, he repeatedly presented with biliary colic pain. He apparently had no personal or family medical history. Two years ago, he nevertheless had jaundice secondary to haemolytic anaemia. The evolution was spontaneously favourable and no further investigation was undertaken. He has a twin brother and a 20‐year‐old sister, both in good health. The clinical examination found a prominent splenomegaly. Haemoglobinopathy and most frequent RBC enzyme deficiencies were excluded. Given the high clinical suspicion of HS, his parents and siblings (although asymptomatic) were screened for HS. Results: The main biological data are reported in the Table 1 . Molecular genetic analyses were performed: NGS targeted sequencing panel, containing panel 40 genes associated to haemolytic anemia, and confirmation by Sanger identified a nonsense mutation on the cytoplasmic domain of Band3. The mutation is novel and drastic; it causes a stop codon at 75 residues. The mutation is transmitted by the mother. NGS targeted sequencing analysis did not demonstrate any other abnormalities that could explain the phenotypic variability observed in the propositus and his mother. Given the profound anaemia associated to the voluminous spleen (main diameter of 25 cm by CT‐scan evaluation) and given the presence of symptomatic gallstones, the patient underwent a splenectomy and a cholecystectomy. After the procedure, he recovered from fatigue and the haemoglobin level returned to normal with no signs of residual haemolysis. Summary/Conclusion: This case reports a HS related to a new nonsense mutation in SLC4A1 . The transmission is autosomal dominant but the penetrance is heterogeneous within the same family. image
Treatment pattern trends of erythropoiesis-stimulating agents used for chemotherapy-induced anemia by U.S. community oncologists from 2008 to 2011.
Authors: Zhaohui Wang, Ann Katler, Karen Todoroff, Kimberly A. Bergstrom
Journal: Journal of Clinical Oncology (2013)

e20649 Background: In 2007 the FDA and national treatment (tx) guidelines recommended restricting use of erythropoiesis-stimulating agents (ESAs) to treat chemo-induced anemia (CIA). This study examines ESA tx started for CIA in non myeloid cancer (NMC) patients (pts) by U.S. community oncologists from 2008 to 2011. Methods: Adult NMC pts with CIA receiving initial ESA [epoetin α (EPO) or darbepoetin α (D-EPO)] tx from 2008 to 2011 were identified using an oncology drug dispensing database. Chemo drug combinations (drug-co) prescribed prior to CIA, Hb levels that triggered the start (1st Hb) and end (last Hb) for initial ESA tx, time to tx w/ ESA and ESA dose and duration were evaluated. Results: 523 oncology practices across 48 states prescribed initial tx w/ EPO or D-EPO for 4,489, 3,845, 2,813, and 1,393 NMC pts w/ CIA in 2008, 2009, 2010, 2011, respectively. Average age was 65, 66, 66, and 67 yrs in 2008, 2009, 2010, 2011, respectively. For all pts w/ CIA, carboplatin-paclitaxel, gemcitabine, and carboplatin-etoposide were the top 3 out of 2,605 drug-co given to 7.5, 4.2 and 3.7% of pts respectively. The Table summarizes data recorded for pts evaluated with CIA and initial ESA tx. Hb starting and ending values declined while time to treat with initial ESA tx increased from 2008 to 2011. No significant change of total dose of EPO or D-EPO was observed from 2008 to 2011. Conclusions: Since restrictions on ESA use were recommended, fewer CIA pts have received ESA TX, and the Hb value for initiation has declined. Additionally, ESA tx was initiated later in the course of chemotherapy from 2008 to 2011 but total time on ESA therapy remained relatively constant. [Table: see text]
SAT0458 EVALUATION OF DISABILITY IN THE COLOMBIA CHIKUNGUNYA EPIDEMIC WITHIN A COLOMBIAN COPCORD STUDY
Authors: Daniel Arsanios Martin, Juan Rueda, Ana María Santos, Ignacio Angarita, Jesús G. Ballesteros, Francy Cuervo, Viviana Reyes, Diana Padilla-Ortiz, John Londono
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> During the 2014-2015 a Chikungunya virus (CHIKV) epidemic reached Colombia. Concurrently, the Community Oriented Program for the Control of Rheumatic Diseases (COPCORD) was being developed in Colombia to establish rheumatic diseases prevalence. Since CHIKV generates rheumatic manifestations, the presence of CHIKV infection was included. <h3>Objectives</h3> Based on the population entered in this program this study was developed with the aim to describe the disability of CHIKV infection through four questionnaires (HAQ-DI, EQ-5D, EQ-5D VAS and VAS for pain). <h3>Methods</h3> World Health Organization (WHO) criteria for CHIKV diagnosis and ELISA IgG and IgM serology were performed to identify CHIKV infection. Four groups of patients were defined: true positives (WHO + and + serology), true negatives (WHO - and - serology), false positives (WHO + and - serology), false negatives (WHO - and + serology). Three scores of disability and one of pain were applied to evaluate disability. A descriptive analysis was made using the media and standard deviation for continuous variables and percentage for the categorical variables, the Odds ratio was calculated with a confidence interval of 95% and a p&lt; 0.05% for statistical significance <h3>Results</h3> From 548 patients with clinical suspicion of CHIKV infection, 295 were positive for CHIKV IgG and/or CHIKV IgM. Functionality evaluated by HAQ-DI showed a media of 0.17 (SD±0.45), with highest score after 7 weeks since the beginning of symptoms and the lowest in patients with CHIKV IgM positive. In the EQ-5D (Figure 1), between 72% and 92% of patients didn't report problems on the mobility, self-care, usual activities, anxiety and depression dimensions. In contrast pain and discomfort dimensions were affected from moderate to extreme (44.7% and 8.6%). There weren't significant differences between true positives, true false, false negative within all the variables, except on the anxiety and depression dimensions where the false positives had a higher significant score in comparison to the other groups (n: 10, 38.5%; p=0.00; OR:4.17, IC:1.8-9.57). In the EQ-5D-VAS, media for VAS was 75.56 (SD±21.18) where the highest scores of VAS were found on the false negatives. In pain VAS, false positives as well as the duration greater than 7 weeks where associated with several pain (n: 10, 2.9%; OR: 0.2; IC 1.25–6.33 y n: 27, 21.3%; p=0.018; OR 2.02, IC: 1.12-3.66 respectively). <h3>Conclusion</h3> In general, the disability of CHIKV infected patients was mild to moderate, finding the most affected dimensions to be pain, discomfort, anxiety and depression. The false positives and the population with anxiety and depression are related with higher score of disability. <h3>Disclosure of Interests</h3> None declared
THU0533 IMPAIRED PLATELET FUNCTIONS IN PATIENTS TREATED WITH COLCHICINE
Authors: Özlem Şatırer, Selcan Demir, Erdal Sag, Armağan Keskin, Yelda Bilginer, Şule Ünal, Seza Özen
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Colchicine has been used in the treatment of Familial Mediterranean Fever (FMF) since 1972. Apart from the inhibiting mitosis in all cells, colchicine has an anti-inflammatory effect by inhibiting activation and migration of neutrophils. Colchicine is a safe drug at recommended doses, but it can cause rare side effects including hematological findings such as lymphopenia, thrombocytopenia and neutropenia. <h3>Objectives</h3> In this study we aimed to define the adverse effect of colchicine on platelet function and its clinical relevance. <h3>Methods</h3> A total of 220 FMF patients between June 2016-2017, followed at Hacettepe University Pediatric Rheumatology Department and were on colchicine treatment for at least one year, were included to the study. <h3>Results</h3> Among the selected 220 FMF patients, 100 of them (54% female) described hematological symptoms when questioned in detail. The mean age of these patients was 11.74 ± 4.86 years. The mean cumulative colchicine exposure was 5.7±3.8 years. The most common referral symptom was frequent epistaxis (79%) followed by easy bruising (69%), and menstrual disorder including prolonged or heavy menstrual bleeding (21.8% among female patients). Among these 100 patients, 36 of them had prolonged bleeding time and impaired platelet aggregation test. Patients who had abnormal platelet function tests (the group with abnormal bleeding time) were receiving higher colchicine doses (median 0.05 vs 0.03 mg/kg/day; p:0.001) compared to the patients who had normal platelet function tests (bleeding time normal group) However there were no significant difference in terms of cumulative colchicine exposure (median 6.5 vs 4.5 years; p:0.07) and total platelet counts (median 288500 vs 279000/mm3; p:0.61). Patients with abnormal platelet function tests also had more epistaxis (47% vs 7%; p&lt;0.001) bruising (51% vs 3%; p&lt;0.001) and dysmenorrhea (among female patients 100% vs 26%; p&lt;0.001). Colchicine was not reduced in these patients and no life-threatening event was observed. <h3>Conclusion</h3> In our study, we have shown prolonged bleeding time for the first time in the literature. Colchicine may cause microtubule inhibition in platelets as well as in other cells and impair platelet function. Further prospective studies are needed to clarify the significance of this side effect. <h3>Reference</h3> [1] Padeh S, Gerstein M, Berkun Y. Colchicine is a safe drug in children with familial Mediterranean fever. J Pediatr 2012;161(6):1142-1146. <h3>Disclosure of Interests</h3> Özlem Şatırer: None declared, Selcan Demir: None declared, Erdal Sag: None declared, Armağan Keskin: None declared, Yelda Bilginer: None declared, Şule Ünal: None declared, Seza Özen Consultant for: Seza Ozen is receiving consultancy fees from Novartis, Speakers bureau: Roche
Risk of suicide and repeat self-harm after hospital attendance for non-fatal self-harm in Sri Lanka: a cohort study
Authors: Duleeka Knipe, Chris Metcalfe, Keith Hawton, Melissa Pearson, Andrew Dawson, Shaluka Jayamanne, Flemming Konradsen, Michael Eddleston, David Gunnell
Journal: The Lancet Psychiatry (2019)

Evidence from high income countries (HICs) suggests that individuals who present to hospital after self-harm are an important target for suicide prevention, but evidence from low and middle-income countries (LMICs) is lacking. We aimed to investigate the risk of repeat self-harm and suicide, and factors associated with these outcomes, in a large cohort of patients presenting to hospital with self-harm in rural Sri Lanka.
Unexpected human cases of cutaneous anthrax in Latium region, Italy, August 2017: integrated human–animal investigation of epidemiological, clinical, microbiological and ecological factors
Authors: Emanuele Nicastri, Francesco Vairo, Paola Mencarini, Antonio Battisti, Chiara Agrati, Eleonora Cimini, Stefania Carrara, Silvia D’Arezzo, Rosanna Adone, Antonella Vulcano, Marco Iannetta, Alessandro Capone, Nicola Petrosillo, Antonio Fasanella, Giuseppe Ippolito
Journal: Eurosurveillance (2019)

On 31 August, a veterinarian and a farmworker were hospitalised for skin lesions. Both had been exposed to a dead cow on 19 August on a farm near Rome, where eight further cattle died of confirmed anthrax later the same month. At admission, the first case showed a black depressed eschar and another smaller lesion on one hand. The second case presented deep infection of the skin, with involvement of both arms. Anthrax diagnosis was confirmed by detection of B. anthracis DNA in eschar fragments from both patients. T-cell specific immunity was studied by flow cytometry and Elispot assay after stimulation with B. anthracis secretome in blood samples collected from Case 1. Immunoglobulin production was detected by complement fixation assay. In Case 1, specific CD4 + T-cell activation was detected, without antibody production. Specific antibodies were detected only in the second patient with severe cutaneous illness. Both patients recovered. The two human anthrax cases were epidemiologically linked, but anthrax was not suspected at admission in either case. The veterinarian had initially unrecognised professional exposure and the exposed farmworker did initially not report exposure to affected animals. A One Health strategy integrating human and animal investigations was essential to confirm the diagnosis.
Abstract 10046: Sex Differences in Risk Factors, Perceived Risk, and Healthcare Provider Discussion of Risk and Risk Modification among Young Patients with Acute Myocardial Infarction: The VIRGO Study
Authors: Erica C Leifheit-Limson, Gail D'Onofrio, Mitra Daneshvar, Mary L Geda, John A Spertus, Harlan M Krumholz, Judith H Lichtman
Journal: Circulation (2012)

Background: Prevention, detection, and treatment of cardiovascular (CV) risk factors require that individuals are educated about their CV risk, perceive themselves to be at risk, and are given guid...