bluuebunny/crossref_metadata_2025_split huggingface dataset:
Analysis of JAK2V617F mutation in Chinese patients with myeloproliferative disorders
Authors: Chen Suning , Fei Hairong , Zhang Ri , Xue Yongquan , Pan Jinlan , Wu Yafang , Ceng Jiannong 
Published: February 2007

AbstractIt is now well‐recognized that the activating JAK2V617F mutation occurs in the majority of patients with polycythemia vera (PV) and approximately half of those with either essential thrombocythemia (ET) or myelofibrosis with myeloid metaplasia (MMM). Here we analyzed JAK2V617F mutation in 137 Chinese patients with myeloproliferative disorders by allele‐specific polymerase chain reaction (PCR). DNA was extracted from methanol/acetic acid‐fixed cells that had been routinely prepared for cytogenetic analysis. A single point mutation (Val617Phe) was identified in JAK2 in 42 (73.7%) of 57 patients with PV, 40 (58.8%) of 68 with ET, and eight (66.7%) of 12 with MMM. Am. J. Hematol., 2007. © 2007 Wiley‐Liss, Inc.
Nongastric marginal zone B‐cell lymphoma: Analysis of 247 cases
Authors: Oh Sung Yong , Ryoo Baek‐Yeol , Kim Won Seog , Park Yeon Hee , Kim Kihyun , Kim Hyun Jung , Kwon Jung Mi , Lee Jeeyun , Ko Young Hye , Ahn Yong Chan , Oh Suk Joong , Lee Soon Il , Kim Hyo‐Jin , Kwon Hyuk‐Chan , Bang Soo Mee , Kim Jung Han , Park Jinny , Lee Seung‐Sook , Kim Ho Young , Park Keunchil 
Published: February 2007

AbstractNongastric marginal zone B‐cell lymphoma (NG‐MZL) is a relatively uncommon indolent lymphoma. From 1990 to 2005, a total of 247 patients with histologically confirmed NG‐MZL were analyzed. Ann Arbor stage I/II disease was present in 78% (167 out of 215). One hundred eighty‐six patients out of two hundred eight were categorized into the low/low‐intermediate risk group (89%) according to International Prognostic Index (IPI). Eighty percent (172/215) were in low risk group according to Follicular Lymphoma International Prognostic Index (FLIPI). Complete and partial remissions (CR and PR) were achieved in 140 (92.7%) and 8 (5.3%) of the 151 stage I/II patients. Especially, radiation containing treatment achieved 96% CR rate (108 out of 113). In 38 patients with stage III/IV, CR and PR were achieved in 17 (44.7%) and 11 (26.3%), respectively. The estimated five‐year overall survival (OS) and progression‐free survival (PFS) were 93.8% and 70.1%, respectively. Although anthracycline‐containing regimen could achieve higher CR rate, it did not improve PFS. Stage III/IV, low hemoglobin, poor performance status, high/high‐intermediate IPI, poor risk FLIPI, and nodal MZL were poor prognostic factors for PFS. NG‐MZL is an indolent disease. FLIPI has strong power to predict the prognosis of NG‐MZL. Am. J. Hematol., 2007. © 2007 Wiley‐Liss, Inc.
Late‐onset cases of familial hemophagocytic lymphohistiocytosis with missense perforin gene mutations
Authors: Ueda Ikuyo , Kurokawa Yumi , Koike Kenichi , Ito Shuichi , Sakata Akifumi , Matsumora Tsutomu , Fukushima Takashi , Morimoto Akira , Ishii Eiichi , Imashuku Shinsaku 
Published: February 2007

AbstractSince the discovery of perforin gene mutations in familial hemophagocytic lymphohistiocytosis (FHL) type 2, heterogeneous features in FHL2 patients have been identified in a report of Feldmann et al. as the beginning. This study was conducted to determine the impact of characteristic gene mutations on late‐onset (age ≥ 7 years) hemophagocytic lymphohistiocytosis episodes. We analyzed perforin gene mutations in three late‐onset cases from our registry in Japan and an additional 10 cases from the literature. Of the 13 cases with onset ages of a median of 10 (range 7–49) years, nine had homozygous and four had compound heterozygous missense mutations of the perforin gene. None had homozygous nonsense mutations. Our data suggest that nonsense perforin gene mutations yield early onset and missense mutations late onset in FHL2 cases. Am. J. Hematol., 2007. © 2007 Wiley‐Liss, Inc.
Thrombotic thrombocytopenic purpura induced by trimethoprim‐sulfamethoxazole in a Jehovah's witness
Authors: Martin Michael G. , Whitlatch Nicole L. , Shah Bijal , Arepally Gowthami M. 
Published: February 2007

AbstractWe report a case of Thrombotic Thrombocytopenic Purpura occurring as an allergic response to trimethoprim‐sulfamethoxazole therapy (Bactrim™, Septra®) in a Jehovah's Witness patient. The patient presented with fulminant microangiopathic hemolytic anemia and thrombocytopenia within 48 hr of initiating therapy with trimethoprim‐sulfamethoxazole. Other symptoms of drug hypersensitivity included nausea, vomiting, urticarial rash, and leukopenia. Because of her religious faith, the patient was supported without plasma therapy with use of intravenous immunoglobulin, steroids, rituximab, and erythropoietin. Am. J. Hematol., 2007. © 2007 Wiley‐Liss, Inc.
Towards understanding the schizophrenia code: An expanded convergent functional genomics approach
Authors: Le‐Niculescu H. , Balaraman Y. , Patel S. , Tan J. , Sidhu K. , Jerome R.E. , Edenberg H.J. , Kuczenski R. , Geyer M.A. , Nurnberger J.I. , Faraone S.V. , Tsuang M.T. , Niculescu A.B. 
Published: February 2007

AbstractIdentifying genes for schizophrenia through classical genetic approaches has proven arduous. Here, we present a comprehensive convergent analysis that translationally integrates brain gene expression data from a relevant pharmacogenomic mouse model (involving treatments with a psychomimetic agent—phencyclidine (PCP), and an anti‐psychotic—clozapine), with human genetic linkage data and human postmortem brain data, as a Bayesian strategy of cross validating findings. Topping the list of candidate genes, we have three genes involved in GABA neurotransmission (GABRA1, GABBR1, and GAD2), one gene involved in glutamate neurotransmission (GRIA2), one gene involved in neuropeptide signaling (TAC1), two genes involved in synaptic function (SYN2 and KCNJ4), six genes involved in myelin/glial function (CNP, MAL, MBP, PLP1, MOBP and GFAP), and one gene involved in lipid metabolism (LPL). These data suggest that schizophrenia is primarily a disorder of brain functional and structural connectivity, with GABA neurotransmission playing a prominent role. These findings may explain the EEG gamma band abnormalities detected in schizophrenia. The analysis also revealed other high probability candidates genes (neurotransmitter signaling, other structural proteins, ion channels, signal transduction, regulatory enzymes, neuronal migration/neurite outgrowth, clock genes, transcription factors, RNA regulatory genes), pathways and mechanisms of likely importance in pathophysiology. Some of the pathways identified suggest possible avenues for augmentation pharmacotherapy of schizophrenia with other existing agents, such as benzodiazepines, anticonvulsants and lipid modulating agents. Other pathways are new potential targets for drug development. Lastly, a comparison with our earlier work on bipolar disorder illuminates the significant molecular overlap between schizophrenia and bipolar disorder. © 2007 Wiley‐Liss, Inc.
Ultrastructural comparison of the morphology of three different platelet and fibrin fiber preparations
Authors: Pretorius Etheresia , Briedenhann Sharon , Marx Jorika , Smit Eureka , Van Der Merwe Chris , Pieters Marlien , Franz Carl 
Published: February 2007

AbstractThe aim of the current study was to investigate the ultrastructural morphology of three different sources of fibrin networks and platelets, namely, lypholized human platelet‐rich plasma (LPRP), freshly prepared human platelet‐rich plasma (FPRP), and human platelet concentrate (HPC). The ultrastructural morphology of the three different fibrin networks was studied using the scanning electron microscope (SEM). Turbidity curves were drawn at 405 nm at room temperature and fibrinogen concentrations were measured. Scanning electron micrographs showed that all clots produced thick major fibrin fibers as well as a well‐defined fine fibrin network, which appeared to be a superimposed process that occurred after the major fibrin network was established. These features were decidedly more pronounced in the HPC specimens. Turbidity curves of the three types of plasma showed differences in LPRP and FPRP. Fibrinogen concentrations of all three preparations were in the normal ranges. Because of the great similarity between LPRP, HPC, and FPRP, we suggest that LPRP could be used successfully to study morphological changes in fibrin fibers and platelets, which may occur after exposure to certain therapeutic agents. However, functionality studies such as turbidity curves should concurrently be included. We therefore conclude that from a basic science point of view, LPRP is a valuable research tool and that such results may add information that could be valuable for clinical application. Anat Rec 290:188–198, 2007. © 2007 Wiley‐Liss, Inc.
Increased expression of TRAIL receptor 3 on eosinophils in Churg‐Strauss syndrome
Authors: Mitsuyama Hideo , Matsuyama Wataru , Watanabe Masaki , Shirahama Yuko , Higashimoto Ikkou , Wada Takashi , Osame Mitsuhiro , Arimura Kimiyoshi 
Published: February 2007

AbstractObjectiveProlonged survival of eosinophils plays an important role in the pathogenesis of Churg‐Strauss syndrome (CSS); however, its detailed molecular mechanism is still unclear. TRAIL and its receptors are expressed on a variety of cells, including eosinophils. In this study, we examined the expression of TRAIL receptors on eosinophils from patients with CSS.MethodsTRAIL receptor expression was assessed on eosinophils from healthy volunteers, patients with CSS, patients with asthma, and patients with hypereosinophilia due to parasitic infection. TRAIL‐induced apoptosis of eosinophils was compared between the patients with CSS and patients with asthma. RNA interference was used to assess the effects of suppression of TRAIL receptor 3.ResultsExpression of TRAIL receptor 3, a decoy receptor that acts as an antiapoptotic receptor, on eosinophils from patients with CSS was significantly higher than that in the other subjects. Moreover, in CSS, serum TRAIL receptor 3 levels showed a significant positive correlation with peripheral eosinophil counts, tissue‐infiltrating eosinophils stained positive for this receptor, and peripheral T cells expressed TRAIL on their surface. Compared with asthma patients, eosinophils from CSS patients showed a significantly lower percentage of recombinant TRAIL, less autologous T cell–induced apoptosis, and decreased level of active caspase 3. Suppression of TRAIL receptor 3 through RNA interference significantly increased the recombinant TRAIL–induced apoptosis of eosinophils from CSS patients.ConclusionIncreased expression of TRAIL receptor 3 on eosinophils from patients with CSS was observed. These alterations in TRAIL receptor 3 expression might be involved in the molecular pathogenesis of CSS eosinophilia.
Prevalence of the metabolic syndrome in patients with gout: The Third National Health and Nutrition Examination Survey
Authors: Choi Hyon K. , Ford Earl S. , Li Chaoyang , Curhan Gary 
Published: February 2007

AbstractObjectiveTo determine the prevalence of metabolic syndrome among patients with gout and to examine the association between the 2 conditions in a nationally representative sample of US adults.MethodsUsing data from 8,807 participants age ≥20 years in the Third National Health and Nutrition Examination Survey (1988–1994), we determined the prevalence of metabolic syndrome among individuals with gout and quantified the magnitude of association between the 2 conditions. We used both the revised and original National Cholesterol Education Program Adult Treatment Panel III (NCEP/ATP III) criteria to define metabolic syndrome.ResultsThe prevalence (95% confidence interval [95% CI]) of metabolic syndrome according to revised NCEP/ATP III criteria was 62.8% (51.9–73.6) among individuals with gout and 25.4% (23.5–27.3) among individuals without gout. Using 2002 census data, ∼3.5 million US adults with a history of gout have metabolic syndrome. The unadjusted and age‐ and sex‐adjusted odds ratios (95% CI) of metabolic syndrome for individuals with gout were 4.96 (3.17–7.75) and 3.05 (2.01–4.61), respectively. With the original NCEP/ATP criteria, the corresponding prevalences were slightly lower, whereas the corresponding odds ratios were slightly higher. The stratified prevalences of metabolic syndrome by major associated factors of gout (i.e., body mass index, hypertension, and diabetes) remained substantially and significantly higher among those with gout than those without gout (all P values <0.05).ConclusionThese findings indicate that the prevalence of metabolic syndrome is remarkably high among individuals with gout. Given the serious complications associated with metabolic syndrome, this frequent comorbidity should be recognized and taken into account in long‐term treatment and overall health of individuals with gout.
A patient‐centered perspective on surgery avoidance for hip or knee arthritis: Lessons for the future
Authors: Ballantyne Peri J. , Gignac Monique A. M. , Hawker Gillian A. 
Published: February 2007

AbstractObjectiveResearch indicates that there is a discrepancy between need and patient preference for total joint arthroplasty (TJA), an efficacious and cost‐effective treatment for severe hip or knee arthritis. To understand this discrepancy, we conducted qualitative research to assess the illness perceptions and preferred accommodations and coping strategies of patients with advanced osteoarthritis who had expressed a preference to avoid TJA.MethodsIn‐depth interviews were conducted with a community sample of 29 men and women who were medically assessed as appropriate candidates for TJA but who had expressed a preference to avoid surgery. Inductive content analysis of text data was used to examine how patients' illness perceptions and preferred coping strategies related to surgery preference.ResultsParticipants frequently rejected the medicalization of arthritis, normalizing the experience of functional decline and defining it as age normative. Participants drew on a broad set of previous experiences with informal and formal care to make decisions about how to manage their condition. Previous negative encounters in medical and surgical care, including those from a distant past or those experienced vicariously, combined with the perception (reinforced by physicians and others) that doing nothing was a viable option deterred arthritis‐related help seeking in the health care system.ConclusionIndividuals with arthritis may benefit from additional counseling regarding effective medical and surgical treatments. Physicians may better meet patient needs by gauging patient preferences for a combination of self‐management strategies and medical interventions.
Proxy‐reported health‐related quality of life of patients with juvenile idiopathic arthritis: The pediatric rheumatology international trials organization multinational quality of life cohort study
Authors: Oliveira Sheila , Ravelli Angelo , Pistorio Angela , Castell Esteban , Malattia Clara , Prieur Anne Marie , Saad‐Magalhães Claudia , Murray Kevin J. , Bae Sang‐Cheol , Joos Rik , Foeldvari Ivan , Duarte‐Salazar Carolina , Wulffraat Nico , Lahdenne Pekka , Dolezalova Pavla , de Inocencio Jaime , Kanakoudi‐Tsakalidou Florence , Hofer Michael , Nikishina Irina , Ozdogan Huri , Hashkes Philip J. , Landgraf Jeanne M. , Martini Alberto , Ruperto Nicolino 
Published: February 2007

AbstractObjectiveTo investigate the proxy‐reported health‐related quality of life (HRQOL) and its determinants in patients with juvenile idiopathic arthritis (JIA).MethodsIn this multinational, multicenter, cross‐sectional study, HRQOL of patients with JIA was assessed through the Child Health Questionnaire (CHQ) and was compared with that of healthy children of similar age from the same geographic area. Potential determinants of HRQOL included demographic data, physician's and parent's global assessments, measures of joint inflammation, Childhood Health Assessment Questionnaire (CHAQ), and erythrocyte sedimentation rate.ResultsA total of 6,639 participants (3,324 with JIA and 3,315 healthy) were enrolled from 32 countries. The mean ± SD physical and psychosocial summary scores of the CHQ were significantly lower in patients with JIA than in healthy children (physical: 44.5 ± 10.6 versus 54.6 ± 4.0, P < 0.0001; psychosocial: 47.6 ± 8.7 versus 51.9 ± 7.5, P < 0.0001), with the physical well‐being domain being most impaired. Patients with persistent oligoarthritis had better HRQOL compared with other subtypes, whereas HRQOL was similar across patients with systemic arthritis, polyarthritis, and extended oligoarthritis. A CHAQ score >1 and a pain intensity rating >3.4 cm on a 10‐cm visual analog scale were the strongest determinants of poorer HRQOL in the physical and psychosocial domains, respectively.ConclusionWe found that patients with JIA have a significant impairment of their HRQOL compared with healthy peers, particularly in the physical domain. Physical well‐being was mostly affected by the level of functional impairment, whereas the intensity of pain had the greatest influence on psychosocial health.
Characterization of B cell lymphoma in patients with Sjögren's syndrome and hepatitis C virus infection
Authors: Ramos‐Casals Manuel , Civita Luca la , de Vita Salvatore , Solans Roser , Luppi Mario , Medina Francisco , Caramaschi Paola , Fadda Patrizia , de Marchi Ginevra , Lopez‐Guillermo Armando , Font Josep 
Published: February 2007

AbstractObjectiveTo characterize the clinical and immunologic patterns of expression, response to therapy, and outcome of patients with Sjögren's syndrome (SS) and associated hepatitis C virus (HCV) infection who developed B cell lymphoma.MethodsVarious international reference centers constituted a multicenter study group with the purpose of creating a registry of patients with SS‐HCV who developed B cell lymphoma. A protocol form was used to record the main characteristics of SS, chronic HCV infection, and B cell lymphoma.ResultsTwenty‐five patients with SS‐HCV with B cell lymphoma were included in the registry. There were 22 (88%) women and 3 (12%) men (mean age 55, 58, and 61 years at SS, HCV infection, and lymphoma diagnosis, respectively). The main extraglandular SS manifestations were cutaneous vasculitis in 15 (60%) patients and peripheral neuropathy in 12 (48%); the main immunologic features were positive rheumatoid factor (RF) in 24 (96%) and type II cryoglobulins in 20 (80%). The main histologic subtypes were mucosa‐associated lymphoid tissue (MALT) lymphoma in 11 (44%) patients, diffuse large B cell lymphoma in 6 (24%), and follicular center cell lymphoma in 6 (24%). Fifteen (60%) patients had an extranodal primary location, most frequently in the parotid gland (5 patients), liver (4 patients), and stomach (4 patients). Twelve (52%) of 23 patients died after a median followup from the time of lymphoma diagnosis of 4 years, with lymphoma progression being the most frequent cause of death. Survival differed significantly between the main types of B cell lymphoma.ConclusionPatients with SS‐HCV and B cell lymphoma are clinically characterized by a high frequency of parotid enlargement and vasculitis, an immunologic pattern overwhelmingly dominated by the presence of RF and mixed type II cryoglobulins, a predominance of MALT lymphomas, and an elevated frequency of primary extranodal involvement in organs in which HCV replicates (exocrine glands, liver, and stomach).
Mindfulness meditation alleviates depressive symptoms in women with fibromyalgia: Results of a randomized clinical trial
Authors: Sephton Sandra E. , Salmon Paul , Weissbecker Inka , Ulmer Christi , Floyd Andrea , Hoover Katherine , Studts Jamie L. 
Published: February 2007

AbstractObjectiveDepressive symptoms are common among patients with fibromyalgia, and behavioral intervention has been recommended as a major treatment component for this illness. The objective of this study was to test the effects of the Mindfulness‐Based Stress Reduction (MBSR) intervention on depressive symptoms in patients with fibromyalgia.MethodsThis randomized controlled trial examined effects of the 8‐week MBSR intervention on depressive symptoms in 91 women with fibromyalgia who were randomly assigned to treatment (n = 51) or a waiting‐list control group (n = 40). Eligible patients were at least 18 years old, willing to participate in a weekly group, and able to provide physician verification of a fibromyalgia diagnosis. Of 166 eligible participants who responded to local television news publicizing, 49 did not appear for a scheduled intake, 24 enrolled but did not provide baseline data, and 2 were excluded due to severe mental illness, leaving 91 participants. The sample averaged 48 years of age and had 14.7 years of education. The typical participant was white, married, and employed. Patients randomly assigned to treatment received MBSR. Eight weekly 2.5‐hour sessions were led by a licensed clinical psychologist with mindfulness training. Somatic and cognitive symptoms of depression were assessed using the Beck Depression Inventory administered at baseline, immediately postprogram, and at followup 2 months after the conclusion of the intervention.ResultsChange in depressive symptoms was assessed using slopes analyses of intervention effects over time. Depressive symptoms improved significantly in treatment versus control participants over the 3 assessments.ConclusionThis meditation‐based intervention alleviated depressive symptoms among patients with fibromyalgia.
HLA–DRB1 and persistent chronic inflammation contribute to cardiovascular events and cardiovascular mortality in patients with rheumatoid arthritis
Authors: Gonzalez‐Gay Miguel A. , Gonzalez‐Juanatey Carlos , Lopez‐Diaz Maria J. , Piñeiro Angela , Garcia‐Porrua Carlos , Miranda‐Filloy Jose A. , Ollier William E. R. , Martin Javier , Llorca Javier 
Published: February 2007

AbstractObjectiveCardiovascular (CV) disease is the most common cause of mortality in patients with rheumatoid arthritis (RA). We assessed the contribution of epidemiologic features, clinical features, routine laboratory markers of inflammation, and HLA–DRB1 alleles to CV mortality in patients with RA prospectively followed at a single referral center in Spain.MethodsPatients fulfilling the 1987 American College of Rheumatology classification criteria for RA seen at the rheumatology outpatient clinic of Hospital Xeral‐Calde, Lugo between March and September 1996 were included. HLA–DRB1 phenotype, epidemiologic data, and clinical data were assessed at that time. Patients were prospectively followed and clinical records were examined until patient's death or September 1, 2005.ResultsA total of 182 consecutive patients were assessed. Compared with the general Spanish population, the age‐ and sex‐standardized mortality ratio by CV cause was 1.78. CV mortality adjusted by age at disease onset and sex was associated with chronic inflammation determined by C‐reactive protein level (CRP; hazard ratio [HR] 1.14, P < 0.001) and erythrocyte sedimentation rate (ESR; HR 1.05, P = 0.003). Patients with HLA–DRB1*04 shared epitope alleles (HR 4.15, P = 0.030), in particular those HLA–DRB1*0404 positive (HR 6.65, P = 0.002), had increased risk of CV mortality. Increased risk of CV events was also associated with CRP level (HR 1.09, P = 0.001), ESR (HR 1.03, P = 0.003), and HLA–DRB1*0404 (HR 4.47, P = 0.002).ConclusionOur results suggest that a chronically high inflammatory response in genetically predisposed individuals promotes an increased risk of CV events and CV mortality in RA.