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Splenomegaly

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https://www.readbyqxmd.com/read/28629642/splenic-vein-reconstruction-is-unnecessary-in-pancreatoduodenectomy-combined-with-resection-of-the-superior-mesenteric-vein-portal-vein-confluence-according-to-short-term-outcomes
#1
Haruyoshi Tanaka, Akimasa Nakao, Kenji Oshima, Kiyotsugu Iede, Yukiko Oshima, Hironobu Kobayashi, Yasunori Kimura
BACKGROUND: Superior mesenteric vein-portal vein confluence resection combined with pancreatoduodenectomy (SMPVrPD) is occasionally required for resection of pancreatic head tumors. It remains unclear whether such situations require splenic vein (SV) reconstruction for decompression of left-sided portal hypertension (LSPH). METHODS: The data from 93 of 104 patients who underwent pancreatoduodenectomy (PD) for pancreatic head malignancies were reviewed. Surgical outcomes in three groups-standard PD (control group), PD combined with vascular resection and SV preservation (SVp group), and SMPVrPD with SV resection (SVr group)-were compared...
June 16, 2017: HPB: the Official Journal of the International Hepato Pancreato Biliary Association
https://www.readbyqxmd.com/read/28629605/ct-findings-in-11-patients-with-tafro-syndrome-a-variant-of-multicentric-castleman-s-disease
#2
T Kiguchi, C Sato, K Takai, Y Nakai, Y Kaneko, M Matsuki
AIM: To assess detailed computed tomography (CT) findings in patients with the recently described thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome, in order to contribute to imaging interpretation in the challenging diagnosis of this disease. MATERIALS AND METHODS: The institutional review board approved this retrospective study and waived the need for informed consent. Eleven patients (six men, five women; mean age, 52.5 years) with confirmed TAFRO syndrome were included in this study...
June 16, 2017: Clinical Radiology
https://www.readbyqxmd.com/read/28626451/dependency-of-b-1-cells-in-the-maintenance-of-splenic-interleukin-10-producing-cells-and-impairment-of-macrophage-resistance-in-visceral-leishmaniasis
#3
Angélica Fernandes Arcanjo, Dirlei Nico, Gabriellen Menezes Migliani de Castro, Yasmin da Silva Fontes, Paula Saltarelli, Debora Decote-Ricardo, Marise P Nunes, Antônio Ferreira-Pereira, Clarisa B Palatnik-de-Sousa, Célio G Freire-de-Lima, Alexandre Morrot
Visceral leishmaniasis is a neglected disease caused by Leishmania protozoa parasites transmitted by infected sand fly vectors. This disease represents the second in mortality among tropical infections and is associated to a profound immunosuppression state of the host. The hallmark of this infection-induced host immunodeviation is the characteristic high levels of the regulatory interleukin-10 (IL-10) cytokine. In the present study, we investigated the role of B-1 cells in the maintenance of splenic IL-10 levels that could interfere with resistance to parasite infection...
2017: Frontiers in Microbiology
https://www.readbyqxmd.com/read/28623190/primary-tuberculosis-of-cystic-duct-lymph-node
#4
Aamir Ghazanfar, Afifa Asghar, Naqeeb Ullah Khan, Iram Hassan
Tuberculosis (TC) is very common and significant cause of morbidity and mortality worldwide. Isolated cystic duct lymph node TC cases without involvement of gallbladder are exceedingly rare. It is difficult to diagnose preoperatively because of lack of characteristic signs and symptoms of TC. We report a man aged 45 years who presented with right upper abdominal pain since 1week. It was associated with nausea and postprandial fullness. There was no evidence of jaundice and lymphadenopathy. Abdominal examination showed moderate right upper quadrant tenderness with positive Murphy's sign and splenomegaly but no signs of peritonism...
June 16, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28622862/identification-of-lawsonia-intracellularis-putative-hemolysin-protein-a-and-characterization-of-its-immunoreactivity
#5
Jehyung Kim, Gayeon Won, Suyeon Park, John Hwa Lee
Despite the recent global increase in fatal endemic outbreaks of proliferative enteropathy (PE) caused by the obligate intracellular bacterium Lawsonia intracelluralis (LI) in the swine industry, development of effective prevention strategies or immunodiagnostic tests has been delayed due to the difficulty of cultivating this pathogen in vitro. Although several genetic analyses have been performed at the level of gene transcription after the complete genome sequence of LI was made available, the mechanism of LI infection and virulence genes remain unidentified...
June 2017: Veterinary Microbiology
https://www.readbyqxmd.com/read/28622624/primary-myelofibrosis-older-age-and-high-jak2v617f-allele-burden-are-associated-with-elevated-plasma-high-sensitivity-c-reactive-protein-levels-and-a-phenotype-of-progressive-disease
#6
Giovanni Barosi, Margherita Massa, Rita Campanelli, Gabriela Fois, Paolo Catarsi, Gianluca Viarengo, Laura Villani, Valentina Poletto, Tiziana Bosoni, Umberto Magrini, Robert P Gale, Vittorio Rosti
We measured plasma levels of high-sensitivity C-reactive protein (hs-CRP) in 526 subjects with primary myelofibrosis (PMF). Thirty-eight percent had an elevated hs-CRP level (≥0.3mg/dL). Elevated hs-CRP levels were associated with a progressive disease phenotype, including anemia, high white blood cell count, low platelet count, increased splenomegaly, increased risk of blast transformation, and worse survival. Age≥52years, but no other demographic characteristics, was associated with an elevated hs-CRP level in multivariable logistic regression (odds ratio [OR], 4...
June 7, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28622623/a-phase-2-study-of-momelotinib-a-potent-jak1-and-jak2-inhibitor-in-patients-with-polycythemia-vera-or-essential-thrombocythemia
#7
Srdan Verstovsek, Stephane Courby, Martin Griesshammer, Ruben A Mesa, Carrie Baker Brachmann, Jun Kawashima, Julia D Maltzman, Lixin Shao, Yan Xin, Daniel Huang, Ashish Bajel
Momelotinib is a potent inhibitor of JAK1 and JAK2 that demonstrated efficacy in patients with primary and secondary myelofibrosis. This phase 2, open-label, randomized study evaluated the efficacy and safety of oral once-daily momelotinib (100mg and 200mg) for the treatment of polycythemia vera (PV) and essential thrombocythemia (ET). The primary endpoint for PV was overall response rate (ORR), defined as the proportion of patients with hematocrit <45%, white blood cell count <10×10(9)/L, platelet count ≤400×10(9)/L, and resolution of palpable splenomegaly, each lasting ≥4 weeks...
May 30, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28622305/the-thrombopoietin-mpl-axis-is-activated-in-the-gata1-low-mouse-model-of-myelofibrosis-and-is-associated-with-a-defective-rps14-signature
#8
M Zingariello, L Sancillo, F Martelli, F Ciaffoni, M Marra, L Varricchio, R A Rana, C Zhao, J D Crispino, A R Migliaccio
Myelofibrosis (MF) is characterized by hyperactivation of thrombopoietin (TPO) signaling, which induces a RPS14 deficiency that de-regulates GATA1 in megakaryocytes by hampering its mRNA translation. As mice carrying the hypomorphic Gata1(low) mutation, which reduces the levels of Gata1 mRNA in megakaryocytes, develop MF, we investigated whether the TPO axis is hyperactive in this model. Gata1(low) mice contained two times more Tpo mRNA in liver and TPO in plasma than wild-type littermates. Furthermore, Gata1(low) LSKs expressed levels of Mpl mRNA (five times greater than normal) and protein (two times lower than normal) similar to those expressed by LSKs from TPO-treated wild-type mice...
June 16, 2017: Blood Cancer Journal
https://www.readbyqxmd.com/read/28620611/dogs-with-acute-myeloid-leukemia-have-clonal-rearrangements-in-t-and-b-cell-receptors
#9
Tracy Stokol, Gabrielle A Nickerson, Martha Shuman, Nicole Belcher
Clonality testing for rearrangements in the complementarity-determining region 3 of the immunoglobulin heavy chain of B lymphocytes (B cell receptor) and the T cell receptor of T lymphocytes helps distinguish between clonal and non-clonal expansions of lymphocytes. There are rare reports of clonally rearranged T and B cell receptors in dogs with acute myeloid leukemia (AML). Our objective was to determine the frequency of clonally rearranged T and B cell receptors in dogs with AML. Archived slides from historical cases of AML (from January 2010 to June 2013) and slides or liquid specimens [blood, bone marrow (BM), body cavity fluid, or tissue aspirates] from cases of AML diagnosed between June 2013 and February 2017 were used in the study...
2017: Frontiers in Veterinary Science
https://www.readbyqxmd.com/read/28620416/antiphospholipid-syndrome-multiple-manifestations-in-a-single-patient-a-high-suspicion-is-still-needed
#10
Uroosa Ibrahim, Shiksha Kedia, Gwenalyn Garcia, Jean Paul Atallah
Antiphospholipid Syndrome (APS) is an autoimmune disorder with clinical and laboratory features of vascular thrombosis, pregnancy loss, and persistent antiphospholipid antibodies (aPLs). The pathophysiology is thought to involve the activation of endothelial cells, monocytes, platelets, and complement by aPLs. Disease can range from asymptomatic to rapidly fatal catastrophic APS. We present a case of a 34-year-old male referred for pancytopenia and splenomegaly. On examination, he had decreased sensation and 4/5 power in the left upper extremity...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28614781/cell-lines-generated-from-a-chronic-lymphocytic-leukemia-mouse-model-exhibit-constitutive-btk-and-akt-signaling
#11
Simar Pal Singh, Saravanan Y Pillai, Marjolein J W de Bruijn, Ralph Stadhouders, Odilia B J Corneth, Henk Jan van den Ham, Alice Muggen, Wilfred van IJcken, Erik Slinger, Annemieke Kuil, Marcel Spaargaren, Arnon P Kater, Anton W Langerak, Rudi W Hendriks
Chronic lymphocytic leukemia (CLL) is characterized by the accumulation of mature CD5+ B cells in blood. Spontaneous apoptosis of CLL cells in vitro has hampered in-depth investigation of CLL pathogenesis. Here we describe the generation of three monoclonal mouse cell lines, EMC2, EMC4 and EMC6, from the IgH.TEμ CLL mouse model based on sporadic expression of SV40 large T antigen. The cell lines exhibit a stable CD5+CD43+IgM+CD19+ CLL phenotype in culture and can be adoptively transferred into Rag1-/- mice...
May 26, 2017: Oncotarget
https://www.readbyqxmd.com/read/28612224/extrahepatic-portal-venous-obstruction-what-should-be-the-mainstay-of-treatment
#12
Richa Lal, Moinak Sen Sarma, Manish K Gupta
The two cornerstones of management for Extrahepatic portal vein obstruction (EHPVO) are endotherapy and surgery [Porto-systemic shunts (PSS)/Mesorex bypass (MRB)]. Endotherapy is the mainstay of treatment for acute variceal bleed control and has also been used extensively for secondary prophylaxis till variceal eradication is achieved. However, long-term follow-up beyond endoscopic eradication of esophageal varices (EEEV) indicates that there are numerous delayed bleed and non bleed sequelae of EHPVO, which merit surgery as a definitive procedure to decompress the hypertensive portal venous system...
June 14, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28611971/diagnosis-and-management-of-hepatobiliary-complications-in-autosomal-recessive-polycystic-kidney-disease
#13
REVIEW
Andrew Wehrman, Alyssa Kriegermeier, Jessica Wen
Autosomal recessive polycystic kidney disease (ARPKD) is a congenital hepatorenal fibrocystic disease. The hepatic manifestations of ARPKD can range from asymptomatic to portal hypertension and massively dilated biliary system that results in liver transplantation. Hepatic complications of ARPKD typically present with signs of portal hypertension (splenomegaly and thrombocytopenia) or cholangitis. Liver disease in ARPKD does not always correlate with severity of renal disease. Management of ARPKD-related liver disease is largely treating specific symptoms, such as antibiotics for cholangitis or endoscopic treatment for variceal bleeding...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28611770/relevance-of-cd6-mediated-interactions-in-the-regulation-of-peripheral-t-cell-responses-and-tolerance
#14
Marta Consuegra-Fernández, Mario Martínez-Florensa, Fernando Aranda, José de Salort, Noelia Armiger-Borràs, Teresa Lozano, Noelia Casares, Juan José Lasarte, Pablo Engel, Francisco Lozano
The CD6 lymphocyte receptor has been involved in the pathophysiology of different autoimmune disorders and is now considered a feasible target for their treatment. In vitro data show the relevance of CD6 in the stabilization of adhesive contacts between T-cell and antigen-presenting cells, and the modulation of T-cell receptor signals. However, the in vivo consequences of such a function are yet undisclosed due to the lack of suitable genetically modified animal models. Here, the in vitro and in vivo challenge of CD6-deficient (CD6(-/-)) cells with allogeneic cells was used as an approach to explore the role of CD6 in immune responses under relative physiological stimulatory conditions...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28609263/image-diagnosis-a-gastric-signet-ring-adenocarcinoma-of-type-linitis-plastica-mimicking-splenomegaly-in-a-patient-with-chronic-lymphocytic-leukemia
#15
Leonid L Yavorkovsky, Shazia Ali
No abstract text is available yet for this article.
2017: Permanente Journal
https://www.readbyqxmd.com/read/28606225/-clinical-characteristics-and-prognostic-analysis-of-children-and-adolescents-over-10-years-of-age-with-acute-lymphoblastic-leukemia
#16
Jun Wu, Ai-Dong Lu, Le-Ping Zhang
OBJECTIVE: To explore the clinical characteristics and prognosis of children and adolescents over 10 years of age with acute lymphoblastic leukemia (ALL). METHODS: A total of 86 newly diagnosed ALL children and adolescents over 10 years of age (62 cases of B-ALL and 24 cases of T-ALL) were enrolled. Clinical characteristics, therapeutic effect and prognostic factors were retrospectively analyzed. Event-free survival (EFS) and overall survival (OS) rates were estimated by the Kaplan-Meier method...
June 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28602585/janus-kinase-2-inhibitor-fedratinib-in-patients-with-myelofibrosis-previously-treated-with-ruxolitinib-jakarta-2-a-single-arm-open-label-non-randomised-phase-2-multicentre-study
#17
Claire N Harrison, Nicolaas Schaap, Alessandro M Vannucchi, Jean-Jacques Kiladjian, Ramon V Tiu, Pierre Zachee, Eric Jourdan, Elliott Winton, Richard T Silver, Harry C Schouten, Francesco Passamonti, Sonja Zweegman, Moshe Talpaz, Joanne Lager, Zhenming Shun, Ruben A Mesa
BACKGROUND: Myelofibrosis is a chronic myeloproliferative neoplasm characterised by splenomegaly, cytopenias, bone marrow fibrosis, and debilitating symptoms including fatigue, weight loss, and bone pain. Mutations in Janus kinase-2 (JAK2) occur in approximately 50% of patients. The only approved JAK2 inhibitor for myelofibrosis is the dual JAK1 and JAK2 inhibitor, ruxolitinib. 58-71% of patients treated with ruxolitinib in clinical trials so far have not achieved the primary endpoint of 35% or more reduction in spleen volume from baseline assessed by MRI or CT...
June 8, 2017: Lancet Haematology
https://www.readbyqxmd.com/read/28600151/splenomegaly-myeloid-lineage-expansion-and-increased-osteoclastogenesis-in-osteogenesis-imperfecta-murine
#18
Brya G Matthews, Emilie Roeder, Xi Wang, Hector Leonardo Aguila, Sun-Kyeong Lee, Danka Grcevic, Ivo Kalajzic
Osteogenesis imperfecta (OI) is a disease caused by defects in type I collagen production that results in brittle bones. While the pathology is mainly caused by defects in the osteoblast lineage, there is also elevated bone resorption by osteoclasts resulting in high bone turnover in severe forms of the disease. Osteoclasts originate from hematopoietic myeloid cells, however changes in hematopoiesis have not been previously documented in OI. In this study, we evaluated hematopoietic lineage distribution and osteoclast progenitor cell frequency in bone marrow, spleen and peripheral blood of osteogenesis imperfecta murine (OIM) mice, a model of severe OI...
June 7, 2017: Bone
https://www.readbyqxmd.com/read/28596664/bullous-pyoderma-gangrenosum-associated-with-hairy-cell-leukemia-and-its-complete-response-to-cladribine-therapy
#19
Ankur Jain, Deepesh Lad, Gaurav Prakash, Alka Khadwal, Pankaj Malhotra, Amanjit Bal, Nabhajit Mallik, Narendar Kumar, Subhash Varma
Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis seen in association with systemic disorders including hematologic malignancies. Hairy cell leukemia (HCL) however, is an unusual association of PG. We describe a 49-year old lady who presented to our hematology clinic with easy fatiguability and ulcerative skin lesions of 6 months duration. Examination revealed pallor and massive splenomegaly. Indurated, ulcerated lesion with undermined edges and necrotic base was observed on left thigh. Investigations revealed pancytopenia and bone marrow examination identified typical hairy cells...
June 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28596542/leukocyte-bim-deficiency-does-not-impact-atherogenesis-in-ldlr-mice-despite-a-pronounced-induction-of-autoimmune-inflammation
#20
Lieve Temmerman, Marijke M Westra, Ilze Bot, Bart J M van Vlijmen, Niek Van Bree, Martine Bot, Kim L L Habets, Tom G H Keulers, Johan van der Vlag, Thomas G Cotter, Theo J C van Berkel, Erik A L Biessen
Proapoptotic Bcl-2 family member Bim is particularly relevant for deletion of autoreactive and activated T and B cells, implicating Bim in autoimmunity. As atherosclerosis is a chronic inflammatory process with features of autoimmune disease, we investigated the impact of hematopoietic Bim deficiency on plaque formation and parameters of plaque stability. Bim (-/-) or wild type bone marrow transplanted ldlr (-/-) mice were fed a Western type diet (WTD) for 5 or 10 weeks, after which they were immunophenotyped and atherosclerotic lesions were analyzed...
June 8, 2017: Scientific Reports
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