tbuckley/cabot-search huggingface dataset:
Coagulation Testing in Patients Taking Direct Oral Anticoagulants
Authors: Namrah Siddiq, Srikanth Nagalla, Sung-hee Choi
Journal: JAMA Internal Medicine (2019)

Our website uses cookies to enhance your experience. By continuing to use our site, or clicking "Continue," you are agreeing to our Cookie Policy | Continue JAMA Internal Medicine HomeNew OnlineCurrent IssueFor Authors Podcast Publications JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry (1919-1959) JN Learning / CMESubscribeJobsInstitutions / LibrariansReprints & Permissions Terms of Use | Privacy Policy | Accessibility Statement 2023 American Medical Association. All Rights Reserved Search All JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Forum Archive JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry Input Search Term Sign In Individual Sign In Sign inCreate an Account Access through your institution Sign In Purchase Options: Buy this article Rent this article Subscribe to the JAMA Internal Medicine journal
FRI0601 SERUM IMMUNOGLOBIN G4 LEVEL IN INHEPATITIS B WAS HIGHER THAN AUTOIMMUNE LIVER DISEASE IN A CHINESE POPULATION
Authors: Zetao Liao, Yanli Zhang
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> High serum level of Immunoglobulin G4 (IgG4) was detected in autoimmune hepatitis (AIH), but little was known in other types of hepatitis such as hepatitis B, primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). <h3>Objectives</h3> The aim of this study is to evaluate the level of serum IgG4 in hepatitis B and autoimmune liver disease. <h3>Methods</h3> Hepatitis B and autoimmune liver disease patients were enrolled between January 1st and August 30th 2018. Diagnosis of Hepatitis B, AIH, PBC and PSC was basing on corresponding classification criteria, and established IgG4 related diseases were excluded. The levels of serum IgG4 were determined by nephelometric assay. The cut-off value of serum IgG4 was 2.01 g/L. Serum IgG4 among different liver diseases were compared. <h3>Results</h3> 161 HBV hepatitis and 112 autoimmune liver diseases (52 AIH, 57 PBC, 3 PSC) were enrolled in this study. The HBV hepatitis patients were younger than patients with autoimmune liver disease (44.79±12.56 vs 52.52 ± 11.16 years old). In total, 61 patients (22.34%) had elevated serum IgG4 level. IgG4 level in HBV hepatitis was significantly higher than in AIH, PBC, respectively (1.64±1.80 vs 1.11±1.16, 1.64±1.80 vs 0.96±0.87 g/L, P&lt;0.05). <h3>Conclusion</h3> <b>High</b> serum IgG4 was very common in liver diseases without IgG4 related diseases especially in HBV hepatitis. Differential diagnosis should be made in patients with hepatitis accompanied with high serum IgG4. <h3>References</h3> [1] No. <h3>Disclosure of Interests</h3> None declared
AB1153 EVALUATION OF DIAGNOSTIC DISCREPANCY USING THE ASAS CRITERIA AND F-18 NAF PET/CT IN PATIENTS WITH SUSPECTED AS
Authors: Seung Min Son, Jung Sub Lee, Tae Sik Goh
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> The latest ASAS diagnostic criteria allow early diagnosis of ankylosing spondylitis by using MRI. Nuclear imaging techniques may detect increased bone turnover not only during inflammation, but probably also paralleling postinflammatory reparative changes at starting point of new bone formation. <h3>Objectives</h3> We focus on diagnostic discrepancy to evaluate AS between fluorine-18 sodium fluoride (F-18 NaF) PET/CT and assessment of spondyloarthritis international society (ASAS) criteria in patients with chronic low back pain suspected ankylosing spondylitis <h3>Methods</h3> Sixty-eight patients with chronic lower back back pain over 3 months and limited lumbar movement were included. Among them, 49 patients who fulfilled ASAS diagnostic criteria were included as AS groups and 19 others were controlled. For clinical assessment, ESR, CRP, BASDAI, and BASFI scores were measured. For imaging assessment, conventional radiography for sacroillitis and F-18 NaF PET/CT were performed. We defined AS-positive lesions on PET/CT as uptake of sacroiliac joint, syndesmophyte, enthesitis lesions and facet joint. <h3>Results</h3> No significant differences were observed in the baseline demographic evaluation between two groups in terms of age, sex. follow-up period and clinical parameters including BASDAI, BASFI, ESR level. However, there were significant differences in terms of HLA-B27 positive value, CRP level and standard uptake value ratio (SUVR) of sacroiliac joint in patients fulfilled ASAS criteria. <h3>Conclusion</h3> In diagnosing AS, F-18 NaF PET/CT showed 79.59% sensitivity, 84.21% specificity and 80.88% accuracy. This shows the diagnostic value of the F-18 NaF PET/CT, which can be a good alternative to the diagnosis of early ankylosing spondylitis, and can evaluate whole body lesions in a single session. <h3>Disclosure of Interests</h3> None declared
AB0308 THE ASSOCIATION OF PARKINSON DISEASE WITH RHEUMATOID ARTHRITIS
Authors: Fulya Cosan, Sena Arda, Nuri Onat Demirci
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Parkinson disease (PD) is a progressive neurological disease, characterised with tremor, spasticity, arthrosis, dementia, loss of mobility and generalised pain. PD affects mainly elderly population. Activity of PD patients is restricted because of spasticity and severe arthrosis. Rheumatoid arthritis (RA) is a chronic inflammatory autoimmune disease which can cause disability in elderly population. Patients with can admit to rheumatology clinics with generalised pain or restricted mobilisation. In contrast, in some PD patients with generalised pain on joints, RA may be overlooked <h3>Objectives</h3> We aimed in this study to evaluate the characteristics of patients with RA and Parkinson patients. <h3>Methods</h3> 842 RA patients screened retrospective for PD from the patient files.10 patients with both RA and Parkinson disease were included to study. 3 female and 7 male patients with<b> mean age 76,4 (with a standard deviation 5,4) were evaluated. The characteristics of patientsare evaluated from the patient files.</b> <h3>Results</h3> All patients were older than 75 years. Mean RA duration time was 14 months and PD duration time was 34 months. All patients admitted outpatient clinic with leg pain and inability to walk. 4 of 10 patients had mild dementia. Median ESR was 36 mm/h and median CRP value was 0,68 mg/dl. 6 of 10 patients had positive ANA result with 1/100 ratio, but rheumatoid factor and anti CCP were negative. <h3>Conclusion</h3> PD is one of the disorder that may cause severe disability in very old ages. RA may be an additional factor for pain and loss of function in PD patients. Patients with generalised pain and difficulties with walking should be evaluated for PD especially in old ages. PD patients with generalised pain on joints should be evaluated for inflammatioy arthritis. <h3>Disclosure of Interests</h3> None declared
Decision Aids for Prostate Cancer Screening Choice
Authors: Jarno M. Riikonen, Gordon H. Guyatt, Tuomas P. Kilpeläinen, Samantha Craigie, Arnav Agarwal, Thomas Agoritsas, Rachel Couban, Philipp Dahm, Petrus Järvinen, Victor Montori, Nicholas Power, Patrick O. Richard, Jarno Rutanen, Henrikki Santti, Thomas Tailly, Philippe D. Violette, Qi Zhou, Kari A. O. Tikkinen
Journal: JAMA Internal Medicine (2019)

US guidelines recommend that physicians engage in shared decision-making with men considering prostate cancer screening.To estimate the association of decision aids with decisional outcomes in prostate cancer screening.MEDLINE, Embase, PsycINFO, CINAHL, and Cochrane CENTRAL were searched from inception through June 19, 2018.Randomized trials comparing decision aids for prostate cancer screening with usual care.Independent duplicate assessment of eligibility and risk of bias, rating of quality of the decision aids, random-effects meta-analysis, and Grading of Recommendations, Assessment, Development and Evaluations rating of the quality of evidence.Knowledge, decisional conflict, screening discussion, and screening choice.Of 19 eligible trials (12 781 men), 9 adequately concealed allocation and 8 blinded outcome assessment. Of 12 decision aids with available information, only 4 reported the likelihood of a true-negative test result, and 3 presented the likelihood of false-negative test results or the next step if the screening test result was negative. Decision aids are possibly associated with improvement in knowledge (risk ratio, 1.38; 95% CI, 1.09-1.73; I2 = 67%; risk difference, 12.1; low quality), are probably associated with a small decrease in decisional conflict (mean difference on a 100-point scale, -4.19; 95% CI, -7.06 to -1.33; I2 = 75%; moderate quality), and are possibly not associated with whether physicians and patients discuss prostate cancer screening (risk ratio, 1.12; 95% CI, 0.90-1.39; I2 = 60%; low quality) or with men's decision to undergo prostate cancer screening (risk ratio, 0.95; 95% CI, 0.88-1.03; I2 = 36%; low quality).The results of this study provide moderate-quality evidence that decision aids compared with usual care are associated with a small decrease in decisional conflict and low-quality evidence that they are associated with an increase in knowledge but not with whether physicians and patients discussed prostate cancer screening or with screening choice. Results suggest that further progress in facilitating effective shared decision-making may require decision aids that not only provide education to patients but are specifically targeted to promote shared decision-making in the patient-physician encounter.
Re: Dolz-Marco et al.: Choroidal and sub-retinal pigment epithelium caverns: multimodal imaging and correspondence with Friedman lipid globules (Ophthalmology. 2018;125:1287-1301)
Authors: Riccardo Sacconi, Enrico Borrelli, Alessandro Marchese, Francesco Gelormini, Flavia Pennisi, Alice Cerutti, Francesco Bandello, Giuseppe Querques
Journal: Ophthalmology (2019)
Impact of Bridge to Transplantation With Continuous-Flow Left Ventricular Assist Devices on Posttransplantation Mortality
Authors: Lauren K. Truby, Maryjane A. Farr, A. Reshad Garan, Raymond Givens, Susan W. Restaino, Farhana Latif, Hiroo Takayama, Yoshifumi Naka, Koji Takeda, Veli K. Topkara
Journal: Circulation (2019)

Background: Bridge to transplantation (BTT) with left ventricular assist devices (LVADs) is a mainstay of therapy for heart failure in patients awaiting heart transplantation (HT). Criteria for HT listing do not differ between patients medically managed and those mechanically bridged to HT. The objectives of the present study were to evaluate the impact of BTT with LVAD on posttransplantation survival, to describe differences in causes of 1-year mortality in medically and mechanically bridged patients, and to evaluate differences in risk factors for 1-year mortality between those with and those without LVAD at the time of HT. Methods: Using the United Network of Organ Sharing database, we identified 5486 adult, single-organ HT recipients transplanted between 2008 and 2015. Patients were propensity matched for likelihood of LVAD at the time of HT. Kaplan–Meier survival estimates were used to assess the impact of BTT on 1- and 5-year mortality. Logistic regression analysis was used to evaluate the odds ratio of 1-year mortality for patients BTT with LVAD compared with those with medical management across clinically significant variables at various thresholds. Results: Early mortality was higher in mechanically bridged patients: 9.5% versus 7.2% mortality at 1 year ( P &lt;0.001). BTT patients incurred an increased risk of 1-year mortality with an estimated glomerular filtration rate of 40 to 60 mL·min −1 ·1.73 m −2 (odds ratio, 1.69; P =0.003) and &lt;40 mL·min −1 ·1.73 m −2 (odds ratio, 2.16; P =0.005). A similar trend was seen in patients with a body mass index of 25 to 30 kg/m 2 (odds ratio, 1.88; P =0.024) and &gt;30 kg/m 2 (odds ratio, 2.11; P &lt;0.001). When patients were stratified by BTT status and the presence of risk factors, including age &gt;60 years, estimated glomerular filtration rate &lt;40 mL·min −1 ·1.73 m −2 , and body mass index &gt;30 kg/m 2 , there were significant differences in 1-year mortality between medium- and high-risk medically and mechanically bridged patients, with 1-year mortality in high-risk BTT patients at 17.6% compared with 10.4% in high-risk medically managed patients. Conclusions: Bridge to HT with LVAD, although necessary because of organ scarcity and capable of improving wait list survival, confers a significantly higher risk of early posttransplantation mortality. Patients bridged with mechanical support may require more careful consideration for transplant eligibility after LVAD placement.
Pneumonia in acute ischemic stroke patients requiring invasive ventilation: Impact on short and long-term outcomes
Authors: Etienne de Montmollin, Stephane Ruckly, Carole Schwebel, Francois Philippart, Christophe Adrie, Eric Mariotte, Guillaume Marcotte, Yves Cohen, Benjamin Sztrymf, Daniel da Silva, Fabrice Bruneel, Marc Gainnier, Maité Garrouste-Orgeas, Romain Sonneville, Jean-François Timsit
Journal: Journal of Infection (2019)
B1a B cells require autophagy for metabolic homeostasis and self-renewal
Authors: Alexander J. Clarke, Thomas Riffelmacher, Daniel Braas, Richard J. Cornall, Anna Katharina Simon
Journal: The Journal of Experimental Medicine (2018)

Specific metabolic programs are activated by immune cells to fulfill their functional roles, which include adaptations to their microenvironment. B1 B cells are tissue-resident, innate-like B cells. They have many distinct properties, such as the capacity to self-renew and the ability to rapidly respond to a limited repertoire of epitopes. The metabolic pathways that support these functions are unknown. We show that B1 B cells are bioenergetically more active than B2 B cells, with higher rates of glycolysis and oxidative phosphorylation, and depend on glycolysis. They acquire exogenous fatty acids and store lipids in droplet form. Autophagy is differentially activated in B1a B cells, and deletion of the autophagy gene Atg7 leads to a selective loss of B1a B cells caused by a failure of self-renewal. Autophagy-deficient B1a B cells down-regulate critical metabolic genes and accumulate dysfunctional mitochondria. B1 B cells, therefore, have evolved a distinct metabolism adapted to their residence and specific functional properties.
PB1978 HEREDITARY ELLIPTOCYTOSIS WITH NEONATAL TRANSIENT POIKILOCYTOSIS VS HEREDITARY PYRO POIKILOCYTOSIS - THE SAME PRESENTATION... A DISTINCT CLINICAL COURSE
Authors: D. Luís, S. Afonso, B. Loureiro, J. Silva Gomes, L. Relvas, C. Bento, J. Azevedo, T. Maia Magalhães, M.L. Ribeiro
Journal: HemaSphere (2019)

Background: Hereditary Elliptocytosis (HEllip) is a congenital haemolytic anemia (HA) of predominantly dominant transmission, with heterogenous phenotype, caused by quantitative / qualitative alterations of erythrocyte membrane proteins. Most cases do not present anemia, only ellipticals in the peripheral blood smear (PBS);10 to 20% have HA of variable severity, and may present with splenomegaly. Hereditary pyro poikilocytosis (PPH) is a serious form of HEllip, the most severe phenotypes of which are associated with mutations in the self-association contacts of αβ-spectrin. In the neonatal period, a transient poikilocytosis (PTN), with significant HA and marked anisopoikilocytosis, can be observed and be confounded with PPH. Clinical evolution allows the differentiation. PTN is associated with the predominance of HbF in this age group, with reduced affinity for the 2,3-DPG erythrocyte enzyme. From the first year of life, with the decline of HbF, the child will present a lighter phenotype typical of HEllip. Aims: Description of a clinical case of a rare form of congenital hemolytic anemia. Methods: Description in the form of a clinical case Results: Male infant, referred to the consultation at 7 months for microcytic / hypocromic anemia with poor response to iron oral suplementation. The research of his personal background revealed a hospitalizationin Neonatal Intensive Care Unit due to neonatal asphyxia and septic shock in second day of life. At this point he presented worsening of anemia (Hb) 9.2 g/dL, reticulocytosis and evidence of haemolysis, PBS with severe anisopoikilocytosis, fragmented RBC, with numerous elliptocytes and micro spherocytes. At this time he was medicated with oral iron, with a poor response in the Hb value. Due to the lack of response to the established oral iron, it was referenced to the consultationat 7 months. Hb studies (High performance liquid chromatography – HPLC) were normal and screening tests for hereditary spherocytosis were negative. Due to haemolytic conditions, with marked microcytosis, and the presence of marked anisopoikilocytosis in PBS, the most probable initial hypothesis was PPH. Sequencing of the STAPA1 gene excluded polymorphisms, such as αLELY. With about 18 monthsof age initiated spontaneous improvement of anemia and normalization of haemolysis parameters. In the last evaluation, he maintains excellent development, with Hb 11.3 g/dL, MCV 75.9fL, MCH 22.5pg, RDW 21 and reticulocytes 70G/L. Summary/Conclusion: The authors intend to alert to the difficulty to distinguish between these two entities, which, despite having a similar presentation in the neonatal period, have a different clinical course, emphasizing the importance of a careful follow-up during the first months/year of life, avoiding parent's distress.
PB1971 STEPWISE APPROACH TO UNDIAGNOSED ANEMIA IN CHILDREN: A MODEL FOR LIMITED RESOURCES SETTINGS
Authors: M. Elalfy, S. Elhabashy, I. Ragab, N. Mowafy, M. Sallam, R. Russo, A. Iolascon, N. Hussein
Journal: HemaSphere (2019)

Background: Undiagnosed inherited anemia is a unique clinical entity in tertiary hematology centers. Reaching confirmed diagnosis is challenging and requires series of complex expensive investigations.The high prevalence of iron deficiency and thalassemia carriers makes the diagnosis of other classes of anemia more challenging. Aims: we aimed at plotting a stepwise diagnostic approach in children with undiagnosed anemia which can be applied in restricted resource settings. Methods: A cross‐sectional study involved 45 still undiagnosed anemia patients after routine panel of investigations. They were classified based on mean corpuscular volume (MCV) into 3 groups; microcytic (n = 19),normocytic (n = 14) and macrocytic group(n = 12). An algorithm was plotted for each category including 4 levels of investigations. The fourth level analysis included genetic based analysis in appropriate setting. Results: After applying a systematic approach; 24 (54%) were probably diagnosed, 8 (18%) confirmed diagnosis,3 (6%) combined diagnosis and 10 (21%) patients remain undiagnosed. By primary, second, third levels; 9 patients were (5 microcytic,3 normocytic,1 macrocytic),8 (4 microcytic,2 normocytic,2 macrocytic) and 18 patients (5 microcytic, 8 normocytic,5 macrocytic) were diagnosed respectively; while 10 patients necessitated a fourth line level (8 microcytic and 2 normocytic).The diagnoses attained in microcytic group were hemoglobinopathies (42%),iron refractory iron deficiency anemia (IRIDA) (15%), membrane defect (10%), sideroblastic anemia (10%),hypotransferrenemia (5%), 15 % combined diagnosis (iron deficiency anemia and beta thalassemia minor, one sickle cell disease with pyropoikilocyosis and one alpha thalassemia and hereditary spherocytosiswhile15% remained undiagnosed. In normocytic group, the diagnosis washerediatary spherocytosis (20%), pyruvate kinase deficiency(15%), CDA type II (28%), beta thalassemia major (7%),Diamond Blackfan anemia (7%), vitamin B12 deficiency (15%) and 30% with no confirmed diagnosis,. In the macrocytic group, vitamin B12 deficiency (25%), PK deficiency(8%), sideroblastic anemia(8%) CDA II (16%) and 40% undiagnosed;by applying the four level of investigation, diagnosis was changed in 2,7 and 7 in microcytic, normocytic and macrocytic respectively. Summary/Conclusion: Setting a stepwise approach with different levels of may help to reach diagnosis of difficult anemia without performing unnecessary investigation showed that microcytic anemia was more frequent.High frequency of B12 deficiency presented either as normocytic or macrocytic was reported in this study. 40% remained with indefinite diagnosis after series of investigations
PB2246 CLINICAL FEATURES AND TREATMENT OUTCOMES OF IMMUNE THROMBOCYTOPENIC PURPURA IN INFANTS; A SINGLE CENTER RETROSPECTIVE STUDY
Authors: K. M. Park, E. J. Yang, Y. T. Lim
Journal: HemaSphere (2019)

Background: Immune thrombocytopenic purpura (ITP) in children less than one year of age is less well characterized compared to ITP in toddlers and school‐age children. Aims: Since children of different ages may have differing clinical courses, better delination of the natural history of ITP in infants is needed. Methods: We retrospectively reviewed the admission records of 248 consecutive pediatric patients between 1 month and 15 year of age admitted and treated for acute ITP at Pusan National University Children's Hospital from 2009 through 2017. All of these patients less than 1 year of age were identified and enrolled. We investigated their demographics, clinical features, laboratory examinations, respond to treatment and long‐term outcomes and compared to those of children 1 to 10 years of age. Results: Ninety nine infants were identified. Male to female ratio was highest in infants and decreased with age. Seventy nine (79.8%) of the 99 infant were found to be under 6 months old. The median platelet counts at diagnosis was 6 x 10 9 /L (0∼76). Minor bleeding (bleeding score 0‐2) was significantly dominant in infant compared to older ages. Eighty two out of 85 patients (96.5%) achieved complete remission after initial intravenous immunoglobulin(IVIG) treatment. The relapse rate after initial CR was significantly lower than older ages (p = 0.003 ). The platelet count after IVIG treatment in infant showed more rapid rising compared to older ages (p = 0.02 ), and the mean time to platelet counts above 100x10 9 /L was 6.4+4.5 days. Follow up information at 6 months was available for 85 infants. Chronic ITP at 6 month was seen less frequently in infants (4.7%) than in children 1 to 10 years of age. (23.2%. P &lt; 0.01 ). Summary/Conclusion: Infants with acute ITP respond more favorably to IVIG treatment and are less likely to develop chronic ITP compared to children 1 to 10 years of age.