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Splenomegaly

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https://www.readbyqxmd.com/read/29147042/insecticide-treated-nets-and-malaria-prevalence-papua-new-guinea-2008-2014
#1
Manuel W Hetzel, Justin Pulford, Yangta Ura, Sharon Jamea-Maiasa, Anthony Tandrapah, Nandao Tarongka, Lina Lorry, Leanne J Robinson, Ken Lilley, Leo Makita, Peter M Siba, Ivo Mueller
Objective: To investigate changes in malaria prevalence in Papua New Guinea after the distribution of long-lasting Insecticide-treated nets, starting in 2004, and the introduction of artemisinin-based combination therapy in 2011. Methods: Two malaria surveys were conducted in 2010-2011 and 2013-2014. They included 77 and 92 randomly selected villages, respectively. In each village, all members of 30 randomly selected households gave blood samples and were assessed for malaria infection by light microscopy...
October 1, 2017: Bulletin of the World Health Organization
https://www.readbyqxmd.com/read/29143912/determination-of-splenomegaly-by-coronal-oblique-length-on-ct
#2
Serra Ozbal Gunes, Yeliz Akturk
PURPOSE: The aims of this study were (a) to determine whether a coronal oblique length (COL) > 12 cm, which is often used to detect splenomegaly (SM) on ultrasound, can be used as a marker of SM on computed tomography (CT), (b) to compare the diagnostic accuracy of COL with other unidimensional linear measurements (ULM) in identifying SM, (c) to determine which ULM most closely correlates with splenic volume (SVol) according to the splenic index on CT, (d) to assess the relationship between SVol and patient's gender, age and body parameters (height, weight), and (e) to determine whether there is a difference between non-contrast-enhanced and contrast-enhanced CT images in identifying SM...
November 16, 2017: Japanese Journal of Radiology
https://www.readbyqxmd.com/read/29143319/the-full-spectrum-of-castleman-disease-273-patients-studied-over-20%C3%A2-years
#3
Eric Oksenhendler, David Boutboul, David Fajgenbaum, Adrien Mirouse, Claire Fieschi, Marion Malphettes, Laetitia Vercellino, Véronique Meignin, Laurence Gérard, Lionel Galicier
The spectrum of Castleman disease (CD) has considerably extended since its first description in 1956. Recently, an international collaborative working group has reached consensus on the diagnostic criteria and classification of CD. We herein report 273 patients with lymph node histopathology consistent with CD and investigate the newly established diagnostic criteria. Twenty of these patients with Castleman-like histopathology were removed from analyses, because they were diagnosed with an exclusionary disorder (18 with haematological malignancy)...
November 16, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/29141318/-ras-associated-autoimmune-leukoproliferative-disorder-a-report-of-2-cases-and-literature-review
#4
T Y He, C R Li, Y Xia, F F Liang, Y Luo, J Yang
Objective: To investigate the clinical features and genetic characteristics of cases with Ras-associated autoimmune leukoproliferative disorder(RALD). Method: Characteristics of clinical data and gene mutation of the first two cases in China with RALD were retrospectively analyzed. The related literature was searched by using search terms "NRAS" , "KRAS" or "RALD" . Result: Case1, a seven-year-seven-month old girl, was admitted due to "thrombocytopenia and splenomegaly for three years" . Palpation showed enlargement of submandibular lymph nodes and hepatosplenomegaly...
November 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29138852/anti%C3%A2-il%C3%A2-39-il%C3%A2-23p19-ebi3-polyclonal-antibodies-ameliorate-autoimmune-symptoms-in-lupus%C3%A2-like-mice
#5
Xiaoqian Wang, Yu Zhang, Zhiding Wang, Xiaoling Liu, Gaizhi Zhu, Gencheng Han, Guojiang Chen, Chunmei Hou, Tianxiao Wang, Beifen Shen, Yan Li, He Xiao, Ning Ma, Renxi Wang
The interleukin (IL)‑12 family cytokines have been examined as therapeutic targets in the treatment of several autoimmune diseases. Our previous study showed that a novel IL‑12 family cytokine, IL‑39 (IL‑23p19/Ebi3) mediates inflammation in lupus‑like mice. In the present study, the effect of anti‑mouse IL‑39 polyclonal antibodies on autoimmune symptoms in lupus‑like mice was investigated. Rabbit anti‑mouse IL‑39 polyclonal antibodies were produced by immunization with recombinant mouse IL‑39, and purified using protein A chromatography...
November 14, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29138744/multiple-myeloma-in-an-amur-tiger-panthera-tigris-altaica
#6
Alison M Lee, Naomi Guppy, John Bainbridge, Hanne Jahns
The Amur tiger (Panthera tigris altaica) is an endangered tiger subspecies. An adult zoo-bred female was found collapsed, and died despite supportive treatment. Hematology and biochemistry showed pancytopenia and hyperglobulinemia, and serum protein electrophoresis revealed a monoclonal band in the β-globulin region. Necropsy demonstrated hemoabdomen, multifocal lytic bone marrow lesions, splenomegaly, and hemorrhagic hepatic nodules, with left medial lobe rupture. There were mutifocal hemorrhages in the subcutis, lung, epicardium, and intestinal mucosa...
2017: Open veterinary journal
https://www.readbyqxmd.com/read/29136691/-tumors-of-lymphoid-and-hematopoietic-tissue-of-spleen-a-clinicopathologic-analysis-of-53-cases
#7
D B Chen, D H Shen, H Zhang, Y Wang, Q J Song, S M Yang, X Z Fang
Objective: To study the clinicopathologic features, diagnosis and differential diagnosis of the tumors of lymphoidand hematopoietic tissue of the spleen(TLTS). Methods: Fifty-three cases of TLTS were selected from the pathologic files from Peking University People's Hospital from April 2002 to April 2017. According to WHO classification of tumors of hematopoietic and lymphoid tissues (2008) and its updated classification (2016), the cases were studied by microscopy, immunohistochemistry and in situ hybridization, combined with the bone marrow biopsy and clinical examination...
November 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/29135986/splenomegaly-diagnostic-validity-work-up-and-underlying-causes
#8
Emelie Curovic Rotbain, Dennis Lund Hansen, Ove Schaffalitzky de Muckadell, Flemming Wibrand, Allan Meldgaard Lund, Henrik Frederiksen
PURPOSE: Our aim was to assess the validity of the ICD-10 code for splenomegaly in the Danish National Registry of Patients (DNRP), as well as to investigate which underlying diseases explained the observed splenomegaly. BACKGROUND: Splenomegaly is a common finding in patients referred to an internal medical department and can be caused by a large spectrum of diseases, including haematological diseases and liver cirrhosis. However, some patients remain without a causal diagnosis, despite extensive medical work-up...
2017: PloS One
https://www.readbyqxmd.com/read/29134664/a-phase-ii-trial-of-ruxolitinib-in-combination-with-azacytidine-in-myelodysplastic-syndrome-myeloproliferative-neoplasms
#9
Rita Assi, Hagop M Kantarjian, Guillermo Garcia-Manero, Jorge E Cortes, Naveen Pemmaraju, Xuemei Wang, Graciela Nogueras-Gonzalez, Elias Jabbour, Prithviraj Bose, Tapan Kadia, Courtney D Dinardo, Keyur Patel, Carlos Bueso-Ramos, Lingsha Zhou, Sherry Pierce, Romany Gergis, Carla Tuttle, Gautam Borthakur, Zeev Estrov, Rajyalakshmi Luthra, Juliana Hidalgo-Lopez, Srdan Verstovsek, Naval Daver
Ruxolitinib and azacytidine target distinct disease manifestations of myelodysplastic syndrome/myeloproliferative neoplasms (MDS/MPNs). Patients with MDS/MPNs initially received ruxolitinib BID (doses based on platelets count), continuously in 28-day cycles for the first 3 cycles. Azacytidine 25 mg/m(2) (Day 1-5) intravenously or subcutaneously was recommended to be added to each cycle starting cycle 4 and could be increased to 75 mg/m(2) (Days 1-5) for disease control. Azacytidine could be started earlier than cycle 4 and/or at higher dose in patients with rapidly proliferative disease or with elevated blasts...
November 14, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/29132487/delta-beta-thalassaemia-in-a-pathan-family
#10
Saqib Qayyum Ahmad, Saerah Iffat Zafar, Hamid Saeed Malik, Suhaib Ahmed
Delta-beta-thalassaemia (δβ-thalassaemia) is a rare type of thalassaemia which mostly results from deletion of δ and β genes with preservation of γ genes. δβ-thalassaemia is classified into (δβ)+ and (δβ)0 types. The (δβ)0-thalassemia is further divided into GγAγ(δβ)0-thalassaemia and Gγ(Aγδβ)0-thalassaemia. In heterozygous state, (δβ)0mutations give rise to phenotype resembling β-thalassaemia trait but with raised Hb-F, ranging from 5 to 20%, without a rise in Hb-A2. In homozygotes, the clinical picture is usually that of thalassaemia intermedia and the patients have 100% Hb-F...
November 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/29131032/anti-psoriasis-effects-and-mechanisms-of-%C3%AE-8-quinolinoxy-zinc-phthalocyanine-mediated-photodynamic-therapy
#11
Han-Qing Liu, Ying-Ming Wang, Wan-Fang Li, Chao Li, Zhi-Huan Jiang, Jie Bao, Jin-Feng Wei, Hong-Tao Jin, Ai-Ping Wang
BACKGROUND/AIMS: The aim of this study was to determine the anti-psoriasis effects of α-(8-quinolinoxy) zinc phthalocyanine (ZnPc-F7)-mediated photodynamic therapy (PDT) and to reveal its mechanisms. METHODS: HaCaT cells were used to observe the influence of ZnPc-F7-PDT on cell proliferation in vitro. The in vivo anti-psoriasis effects of ZnPc-F7-PDT were evaluated using a mouse vagina model, a propranolol-induced cavy psoriasis model and an imiquimod (IMQ)-induced nude mouse psoriasis model...
November 9, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/29130929/tnf-regulates-transcription-of-nlrp3-inflammasome-components-and-inflammatory-molecules-in-cryopyrinopathies
#12
Matthew D McGeough, Alexander Wree, Maria E Inzaugarat, Ariela Haimovich, Casey D Johnson, Carla A Peña, Raphaela Goldbach-Mansky, Lori Broderick, Ariel E Feldstein, Hal M Hoffman
The NLRP3 inflammasome is a protein complex responsible for caspase-1-dependent maturation of the proinflammatory cytokines IL-1β and IL-18. Gain-of-function missense mutations in NLRP3 cause the disease spectrum known as the cryopyrin-associated periodic syndromes (CAPS). In this study, we generated Nlrp3-knockin mice on various KO backgrounds including Il1b/Il18-, caspase-1-, caspase-11- (Casp1/11-), and Tnf-deficient strains. The Nlrp3L351P Il1b-/- Il18-/- mutant mice survived and grew normally until adulthood and, at 6 months of age, exhibited marked splenomegaly and leukophilia...
November 13, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/29124835/clinical-characteristics-and-outcomes-of-castleman-disease-a-multicenter-study-of-185-chinese-patients
#13
Xuanye Zhang, Huilan Rao, Xiaolu Xu, Zhihua Li, Bing Liao, Hongmei Wu, Mei Li, Xiuzhen Tong, Juan Li, Qingqing Cai
Castleman disease (CD) is a rare lymphoproliferative disorder. To assess the clinical features, outcomes, and prognostic factors of this disease, we retrospectively analyzed 185 HIV-negative CD patients from four medical centers in southern China. The median age was 37 years. 121 patients (65.4%) were classified as unicentric CD (UCD) and 64 patients (34.6%) were classified as multicentric CD (MCD). The histology subtype was hyaline-vascular for 132 patients (71.4%), plasma cell for 50 patients (27%) and mixed type for 3 patients (1...
November 9, 2017: Cancer Science
https://www.readbyqxmd.com/read/29123652/autoimmune-lymphoproliferative-syndrome-more-than-a-fascinating-disease
#14
REVIEW
Karen Bride, David Teachey
Autoimmune lymphoproliferative syndrome (ALPS) is an inherited syndrome characterized by abnormal lymphocyte survival caused by failure of apoptotic mechanisms to maintain lymphocyte homeostasis. This failure leads to the clinical manifestations of non-infectious and non-malignant lymphadenopathy, splenomegaly, and autoimmune pathology, most commonly, autoimmune cytopenias. Since ALPS was first characterized in the early 1990s, insights in disease biology have improved both diagnosis and management of this syndrome...
2017: F1000Research
https://www.readbyqxmd.com/read/29114065/bim-suppresses-the-development-of-sle-by-limiting-myeloid-inflammatory-responses
#15
FuNien Tsai, Philip J Homan, Hemant Agrawal, Alexander V Misharin, Hiam Abdala-Valencia, G Kenneth Haines, Salina Dominguez, Christina L Bloomfield, Rana Saber, Anthony Chang, Chandra Mohan, Jack Hutcheson, Anne Davidson, G R Scott Budinger, Philippe Bouillet, Andrea Dorfleutner, Christian Stehlik, Deborah R Winter, Carla M Cuda, Harris Perlman
The Bcl-2 family is considered the guardian of the mitochondrial apoptotic pathway. We demonstrate that Bim acts as a molecular rheostat by controlling macrophage function not only in lymphoid organs but also in end organs, thereby preventing the break in tolerance. Mice lacking Bim in myeloid cells (LysM(Cre)Bim(fl/fl)) develop a systemic lupus erythematosus (SLE)-like disease that mirrors aged Bim(-/-) mice, including loss of marginal zone macrophages, splenomegaly, lymphadenopathy, autoantibodies (including anti-DNA IgG), and a type I interferon signature...
November 7, 2017: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/29113155/poems-syndrome-presentation-with-progressive-weakness-in-upper-and-lower-limbs-a-case-report
#16
Chuang-Jie Cao, Cheng-Yun Dou, Ke-Hua Zhou, Jin-Bo Liu, Hong Lai
Polyneuropathy, organomegaly, endocrinopathy, M proteins, and skin changes (POEMS) syndrome is a rare variant of plasma cell disorders with multiple systemic manifestations. A 50-year-old female patient presented with progressive weakness in her upper and lower limbs; tingling, numbness and burning in her feet; polyneuropathy (demyelinating in the majority of cases of POEMS syndrome); monoclonal plasma cell disorder (typicallyλ-restricted in cases of POEMS syndrome); sclerotic lesions on the spine and pelvis; organomegaly, including hepatomegaly, splenomegaly and lymphadenopathy; edema; pleural effusion; adrenal, thyroidal, pituitary, gonadal and pancreatic endocrinopathy; skin changes, including hyperpigmentation, dry skin and hypertrichosis; thrombocytosis; pulmonary hypertension; low vitamin B12 and weight loss...
November 2017: Oncology Letters
https://www.readbyqxmd.com/read/29112083/characteristics-of-liver-disease-in-100-individuals-with-joubert-syndrome-prospectively-evaluated-at-a-single-center
#17
Anna Strongin, Theo Heller, Dan Doherty, Ian A Glass, Melissa A Parisi, Joy Bryant, Peter Choyke, Baris Turkbey, Kailash Daryanani, Deniz Yildirimli, Meghana Vemulapalli, Jim C Mullikin, May C Malicdan, Thierry Vilboux, William A Gahl, Meral Gunay-Aygun
BACKGROUND & AIMS: Joubert Syndrome (JS) is a rare, inherited, ciliopathy defined by cerebellar and brainstem malformations and is variably associated with liver, kidney, and ocular dysfunction. This study characterizes the hepatic findings in JS and identifies factors associated with probable portal hypertension. METHODS: 100 individuals with JS were prospectively evaluated at the National Institutes of Health Clinical Center. Laboratory tests, imaging, and DNA sequencing were performed...
November 3, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29111186/plasmodium-falciparum-found-in-the-bone-marrow-of-a-child-in-manado-city-east-indonesia-a-case-report
#18
Suryadi N N Tatura, Stefanus Gunawan, Janno Bernadus, Sianne Sandjoto
In Indonesia, there are at least 1.3 million cases of malaria each year and Plasmodium falciparum appears to be the most common Plasmodium. The finding of Plasmodium is important for the diagnosis and management of malaria. This is a case of a 4-year-and-9-month-old male who lived in Manado, East Indonesia. He presented with a prolonged fever, was pale in appearance, and was easily fatigued over the last 3 weeks. Hepato-splenomegaly was found on the initial physical examination. Preliminary laboratory findings found pancytopenia and severe anemia...
October 2017: Asian Pacific Journal of Tropical Medicine
https://www.readbyqxmd.com/read/29110361/hairy-cell-leukemia-2018-update-on-diagnosis-risk-stratification-and-treatment
#19
Xavier Troussard, Edouard Cornet
DISEASE OVERVIEW: Hairy cell leukemia (HCL) and HCL-like disorders, including HCL variant (HCL-V) and splenic diffuse red pulp lymphoma (SDRPL), are a very heterogeneous group of mature lymphoid B-cell disorders, characterized by the identification of hairy cells, a specific genetic profile, a different clinical course and the need for appropriate treatment. DIAGNOSIS: Diagnosis of HCL is based on morphological evidence of hairy cells, an HCL immunologic score of 3 or 4 based on the CD11C, CD103, CD123, and CD25 expression, the trephine biopsy which makes it possible to specify the degree of tumoral medullary infiltration and the presence of BRAF V600E somatic mutation...
December 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/29108288/baseline-factors-associated-with-response-to-ruxolitinib-an-independent-study-on-408-patients-with-myelofibrosis
#20
Francesca Palandri, Giuseppe Alberto Palumbo, Massimiliano Bonifacio, Mario Tiribelli, Giulia Benevolo, Bruno Martino, Elisabetta Abruzzese, Mariella D'Adda, Nicola Polverelli, Micaela Bergamaschi, Alessia Tieghi, Francesco Cavazzini, Adalberto Ibatici, Monica Crugnola, Costanza Bosi, Roberto Latagliata, Ambra Di Veroli, Luigi Scaffidi, Federico de Marchi, Elisa Cerqui, Barbara Anaclerico, Giovanna De Matteis, Marco Spinsanti, Elena Sabattini, Lucia Catani, Franco Aversa, Francesco Di Raimondo, Umberto Vitolo, Roberto Massimo Lemoli, Renato Fanin, Francesco Merli, Domenico Russo, Antonio Cuneo, Maria Letizia Bacchi Reggiani, Michele Cavo, Nicola Vianelli, Massimo Breccia
In patients with Myelofibrosis (MF) treated with ruxolitinib (RUX), the response is unpredictable at therapy start. We retrospectively evaluated the impact of clinical/laboratory factors on responses in 408 patients treated with RUX according to prescribing obligations in 18 Italian Hematology Centers. At 6 months, 114 out of 327 (34.9%) evaluable patients achieved a spleen response. By multivariable Cox proportional hazard regression model, pre-treatment factors negatively correlating with spleen response were: high/intermediate-2 IPSS risk (p=0...
October 3, 2017: Oncotarget
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