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https://www.readbyqxmd.com/read/28438857/reduced-competitive-repopulation-capacity-of-multipotential-hematopoietic-stem-cells-in-the-bone-marrow-of-friend-virus-infected-fv2-resistant-mice
#1
Michael W Epperly, Xichen Zhang, Renee Fisher, Wen Hou, Darcy Franicola, Donna Shields, Michael Quickel, Pamela Hankey-Giblin, Hong Wang, Joel S Greenberger
BACKGROUND/AIM: The polycythemia form of Friend leukemia virus (FVP) causes splenomegaly and lethal erythroleukemia in Fv-2(s)-susceptible mouse strains. We sought to determine whether the hematopoietic stem cell (HSC) pool was expanded in Fv-2(r)-resistant mice. MATERIALS AND METHODS: The 120-day bone marrow transplantation competitive repopulation assay was used to determine whether FVP-infected Fv-2(r) C57BL/6 mice demonstrated expansion of the HSC pool compared to the pool of committed hematopoietic progenitor cells in the same marrow assayed in vitro...
May 2017: In Vivo
https://www.readbyqxmd.com/read/28436280/persistent-foot-ulcer-due-to-ruxolitinib-therapy-for-primary-myelofibrosis
#2
Michael Del Rosario, Henry Tsai, Constantin A Dasanu
Primary myelofibrosis is characterized by bone marrow fibrosis, splenomegaly and presence of JAK-2 V617F mutation in more than 90% of patients. Ruxolitinib is a Janus kinase inhibitor used for the treatment of primary myelofibrosis. We describe herein a persistent foot ulcer development attributed to ruxolitinib therapy. We are unaware of any previous reports of this phenomenon in the scientific literature. A thorough examination of the lower extremities is perhaps necessary before initiating this oral agent...
January 1, 2017: Journal of Oncology Pharmacy Practice
https://www.readbyqxmd.com/read/28427176/metronomic-regimen-as-an-effective-treatment-for-aggressive-t-lgl-leukemia-with-central-nervous-system-infiltration-clinical-experience-and-review-of-literature
#3
REVIEW
Yun Liu, Lei Fan, Huihui Zhao, Wei Xu, Jianyong Li
A 71-year-old man was diagnosed with T-Large granular lymphocytic (LGL) leukemia, which usually represents a relatively indolent clinical course. While the clinical manifestation of this patient we report herein was aggressive with lasting fever, splenomegaly and hemophagocytic lymphohistiocytosis (HLH). T-cell immunophenotype was CD3+CD4-CD8-CD5-CD7-TCRαβ+. After comprehensive evaluation, an adjusted chemotherapy regimen CEOP (cyclophosphamide, vincristine, etoposide, prednisone) with etoposide, a potential effective regimen for HLH was administrated to the patient...
February 27, 2017: Oncotarget
https://www.readbyqxmd.com/read/28415607/thymoquinone-exerts-potent-growth-suppressive-activity-on-leukemia-through-dna-hypermethylation-reversal-in-leukemia-cells
#4
Jiuxia Pang, Na Shen, Fei Yan, Na Zhao, Liping Dou, Lai-Chu Wu, Christopher L Seiler, Li Yu, Ke Yang, Veronika Bachanova, Eric Weaver, Natalia Y Tretyakova, Shujun Liu
Thymoquinone (TQ), a bioactive constituent of the volatile oil of Monarda fistulosa and Nigella sativa, possesses cancer-specific growth inhibitory effects, but the underlying molecular mechanisms remain largely elusive. We propose that TQ curbs cancer cell growth through dysfunction of DNA methyltransferase 1 (DNMT1). Molecular docking analysis revealed that TQ might interact with the catalytic pocket of DNMT1 and compete with co-factor SAM/SAH for DNMT1 inhibition. In vitro inhibitory assays showed that TQ decreases DNMT1 methylation activity in a dose-dependent manner with an apparent IC50 of 30 nM...
March 21, 2017: Oncotarget
https://www.readbyqxmd.com/read/28414188/a-case-of-splenomegaly-in-cbl-syndrome
#5
Rachel R Coe, Margaret L McKinnon, Maja Tarailo-Graovac, Colin J Ross, Wyeth W Wasserman, Jan M Friedman, Paul C Rogers, Clara D M van Karnebeek
INTRODUCTION: We present a child with unexplained splenomegaly to highlight this feature as a presenting sign of the RASopathy CBL syndrome and to draw attention to the power and utility of next generation genomic sequencing for providing rapid diagnosis and critical information to guide care in the pediatric clinical setting. CLINICAL REPORT: A 7-year-old boy presented with unexplained splenomegaly, attention deficit hyperactivity disorder, mild learning difficulties, easy bruising, mild thrombocytopenia, and subtle dysmorphic features...
April 13, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/28413133/vaccination-with-the-polymorphic-membrane-protein-a-reduces-chlamydia-muridarum-induced-genital-tract-pathology
#6
Tina Müller, Elisabeth Becker, Sonja Stallmann, Anna Waldhuber, Franziska Römmler-Dreher, Simone Albrecht, Fabian Mohr, Johannes H Hegemann, Thomas Miethke
Chlamydia trachomatis serovars D-K are one of the most frequent causes of sexually transmitted infections of the female genital tract, with possible complications such as hydrosalpinx, pelvic inflammatory disease, extra-uterine gravidity or infertility. We used the murine genital tract infection model with C. muridarum for vaccination studies and found that more than 70% of the infected mice suffered from uterus dilatations and/or hydrosalpinx. Systemic consequences of the vaginal infection were apparent by splenomegaly ten to fifteen days post infection...
April 13, 2017: Vaccine
https://www.readbyqxmd.com/read/28409239/macrophage-activation-syndrome-in-systemic-lupus-erythematosus-a-multicenter-case-control-study-in-china
#7
Ai-Chun Liu, Yue Yang, Meng-Tao Li, Yuan Jia, Sheng Chen, Shuang Ye, Xiang-Zong Zeng, Zhao Wang, Jin-Xia Zhao, Xiang-Yuan Liu, Jian Zhu, Yan Zhao, Xiao-Feng Zeng, Zhan-Guo Li
The objective of this study was to describe the clinical and laboratory characteristics, precipitating factors, treatment, and outcome of macrophage activation syndrome (MAS) complicating systemic lupus erythematosus (SLE). A multicenter case-control study was performed across six tertiary hospitals from 1997 to 2014. A total of 32 patients with SLE-associated MAS were enrolled. Sixty-four age- and sex-matched SLE patients diagnosed in the same period without MAS episodes were selected as controls. The most frequent clinical feature was fever, followed by splenomegaly...
April 13, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28409042/nephrologists-hate-the-dialysis-catheters-a-systemic-review-of-dialysis-catheter-associated-infective-endocarditis
#8
Kalyana C Janga, Ankur Sinha, Sheldon Greenberg, Kavita Sharma
A 53-year-old Egyptian female with end stage renal disease, one month after start of hemodialysis via an internal jugular catheter, presented with fever and shortness of breath. She developed desquamating vesiculobullous lesions, widespread on her body. She was in profound septic shock and broad spectrum antibiotics were started with appropriate fluid replenishment. An echocardiogram revealed bulky leaflets of the mitral valve with a highly mobile vegetation about 2.3 cm long attached to the anterior leaflet...
2017: Case Reports in Nephrology
https://www.readbyqxmd.com/read/28408792/thrombopoietin-receptor-agonist-mitigates-hematopoietic-radiation-syndrome-and-improves-survival-after-whole-body-ionizing-irradiation-followed-by-wound-trauma
#9
Juliann G Kiang, Min Zhai, Pei-Jun Liao, Connie Ho, Nikolai V Gorbunov, Thomas B Elliott
Ionizing radiation combined with trauma tissue injury (combined injury, CI) results in greater mortality and H-ARS than radiation alone (radiation injury, RI), which includes thrombocytopenia. The aim of this study was to determine whether increases in numbers of thrombocytes would improve survival and mitigate H-ARS after CI. We observed in mice that WBC and platelets remained very low in surviving RI animals that were given 9.5 Gy (60)Co-γ-photon radiation, whereas only lymphocytes and basophils remained low in surviving CI mice that were irradiated and then given skin wounds...
2017: Mediators of Inflammation
https://www.readbyqxmd.com/read/28406768/ultrasonographic-findings-of-liver-in-chronic-liver-disease-and-its-complications-and-their-association-with-the-duration-of-the-disease
#10
Sara Khalid Memon
OBJECTIVE: To evaluate the ultrasound abdomen findings in patients having viral chronic liver disease (CLD) and build correlation of these findings with the duration of disease. STUDY DESIGN: Cross-sectional analytical study. PLACE AND DURATION OF STUDY: Department of Medicine, Liaquat University Hospital, Hyderabad and Jamshoro, Pakistan, from February to July 2016. METHODOLOGY: Known cases of CLD were inducted. Data was collected using a standardized form which included details of patients, duration of disease and their ultrasound abdomen findings, like liver size, echo pattern and echogenicity, nodularity, intrahepatic vessel (IHV) obliteration, ascites and splenomegaly...
March 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28402911/hassab-s-operation-for-joubert-syndrome-with-congenital-hepatic-fibrosis-a-case-report
#11
Koji Miyazawa, Yasuyuki Hara, Kenji Shimizu, Wataru Nakanishi, Kazuaki Tokodai, Chikashi Nakanishi, Shigehito Miyagi, Naoki Kawagishi, Noriaki Ohuchi
INTRODUCTION: Joubert syndrome is characterized by psychomotor developmental delay, hypotonia, oculomotor abnormalities, occasional retinal dystrophy and cystic kidneys, and frequent and often, striking breathing abnormalities, especially in the neonatal period, with panting tachypnea followed by apnea. We report a case of Joubert syndrome with hepatic fibrosis, portal hypertension, and pancytopenia treated by Hassab's operation. PRESENTATION OF CASE: Our patient was a 27-year-old woman with a history of tachypnea, muscle hypotonia, and psychomotor retardation shortly after birth and a diagnosis of Joubert syndrome at 2 years of age...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28399088/-the-forensic-medical-evaluation-of-traumatic-and-spontaneous-ruptures-of-the-organs-affected-by-the-tumours
#12
Yu I Pigolkin, O V Dolzhansky, E M Pal'tseva, M A Shilova, D N Fedorov, S E Boeva
The present article was designed to report the results of the analysis of the cases of traumatic and spontaneous ruptures of the organs affected by the tumours based on the original observations and the literature data. It is shown that the probability of the tumour rupture depends on its histological type, localization, the size, and the distance from the capsule of the affected organ, the degree of involvement of the major blood vessels, the severity of the necrotic changes, the presence of cysts in the neoplasm, and the regimens of radio- and chemotherapy...
2017: Sudebno-meditsinskaia Ekspertiza
https://www.readbyqxmd.com/read/28395746/derivation-of-the-human-induced-pluripotent-stem-cell-line-mui017-a-from-a-patient-with-homozygous-hemoglobin-constant-spring
#13
Wasinee Wongkummool, Warun Maneepitasut, Thongperm Munkongdee, Pirut Tong-Ngam, Amornrat Tangprasittipap, Saovaros Svasti, Narisorn Kitiyanant, Kittiphong Paiboonsukwong, Suthat Fucharoen, Alisa Tubsuwan
Hemoglobin Constant Spring (HbCS, HBA2: c.427T>C) is a common nondeletional α-thalassemia resulting from a nucleotide substitution at the termination codon of the HBA2 gene. Homozygosity for HbCS is characterized with mild anemia, jaundice, and splenomegaly. In this study, the human induced pluripotent stem cell line MUi017-A was successfully generated from peripheral blood CD34+ hematopoietic progenitors of a 52year old female with homozygous HbCS. The MUi017-A cell line exhibited embryonic stem cell characteristics with consistent expression of specific pluripotency markers and the capability of differentiating into the three germ layers...
April 2017: Stem Cell Research
https://www.readbyqxmd.com/read/28395559/management-of-myelofibrosis-jak-inhibition-and-beyond
#14
Maximilian Stahl, Amer M Zeidan
Myelofibrosis (MF) is characterized by bone marrow fibrosis with subsequent extramedullary hematopoiesis and abnormal cytokine expression leading to splenomegaly, constitutional symptoms and cytopenias. The discovery of the JAK2 V617F mutation in the majority of MF patients has been followed by significant progress in drug development for MF. Areas covered: In this article, we review advances in the understanding of the underlying disease biology, prognostic assessment and therapeutic modalities for MF. We provide clinical trial evidence behind using the JAK2 inhibitor ruxolitinib, erythropoiesis stimulating agents, androgens, immunomodulatory drugs, interferon, cytoreductive drugs and hypomethylating agents in MF...
April 26, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28389309/steroid-refractory-autoimmune-hemolytic-anemia-with-massive-splenomegaly
#15
Hiroshi Ureshino, Masaharu Miyahara
No abstract text is available yet for this article.
April 4, 2017: American Journal of Medicine
https://www.readbyqxmd.com/read/28387450/severe-demyelination-in-a-patient-with-a-late-infantile-form-of-niemann-pick-disease-type-c
#16
Tsuyoshi Kodachi, Shizuko Matsumoto, Masashi Mizuguchi, Hitoshi Osaka, Nobuyuki Kanai, Eiji Nanba, Kousaku Ohno, Takanori Yamagata
Niemann-Pick disease type C (NPC) is a cholesterol storage disease caused by defective cellular cholesterol transportation. The onset and progression of NPC are variable, and autopsy findings have mainly been reported for the adult and juvenile forms of this disease. Here we report the clinical and pathological findings from a 9-year-old female patient with the late infantile form of NPC due to NPC1 gene mutation. She had notable splenomegaly at 4 months of age. She lost the ability to speak at 18 months of age...
April 7, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28385371/wandering-spleen-an-overview
#17
REVIEW
David C Reisner, Constantine M Burgan
An ectopic (or "wandering") spleen results from abnormal ligamentous laxity failing to fixate the spleen in its normal location in the left upper quadrant. Ligamentous laxity can be acquired due to conditions, such as splenomegaly or pregnancy, but is often congenital. Because of this laxity, there is an elongated vascular pedicle, which is prone to torsion and resultant splenic infarction. These patients generally present with abdominal pain and other nonspecific symptoms making the diagnosis very difficult to make clinically...
February 16, 2017: Current Problems in Diagnostic Radiology
https://www.readbyqxmd.com/read/28377281/novel-sphingosine-kinase-1-inhibitor-lcl351-reduces-immune-responses-in-murine-dss-induced-colitis
#18
Michael J Pulkoski-Gross, Joachim D Uys, K Alexa Orr-Gandy, Nicolas Coant, Agnieszka B Bialkowska, Zdzislaw M Szulc, Aiping Bai, Alicja Bielawska, Danyelle M Townsend, Yusuf A Hannun, Lina M Obeid, Ashley J Snider
Sphingosine-1-phosphate (S1P) is a biologically active sphingolipid metabolite which has been implicated in many diseases including cancer and inflammatory diseases. Recently, sphingosine kinase 1 (SK1), one of the isozymes which generates S1P, has been implicated in the development and progression of inflammatory bowel disease (IBD). Based on our previous work, we set out to determine the efficacy of a novel SK1 selective inhibitor, LCL351, in a murine model of IBD. LCL351 selectively inhibits SK1 both in vitro and in cells...
April 2, 2017: Prostaglandins & Other Lipid Mediators
https://www.readbyqxmd.com/read/28370285/identification-and-characterization-of-a-nationwide-danish-adult-common-variable-immunodeficiency-cohort
#19
Lena Westh, Trine Hyrup Mogensen, Lars Skov Dalgaard, Jens Magnus Bernth Jensen, Terese Katzenstein, Ann-Brit Eg Hansen, Olav Ditlevsen Larsen, Steen Terpling, Thyge Lynghøj Nielsen, Carsten Schade Larsen
In this study we identified all adults living in Denmark diagnosed with common variable immunodeficiency (CVID) and characterized them according to clinical presentation and EUROclass classification. Using a retrospective, cross-sectional design, possible CVID patients were identified in the Danish National Patient Register and Centers in Denmark treating patients with primary immunodeficiencies. The CVID diagnosis was verified by review of medical records. One-hundred-seventy-nine adults with CVID were identified...
April 2, 2017: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/28367662/adverse-drug-reactions-after-intravenous-rituximab-infusion-are-more-common-in-hematologic-malignancies-than-in-autoimmune-disorders-and-can-be-predicted-by-the-combination-of-few-clinical-and-laboratory-parameters-results-from-a-retrospective-multicenter-study
#20
Giovanni D'Arena, Vittorio Simeon, Luca Laurenti, Michele Cimminiello, Idanna Innocenti, Michele Gilio, Angela Padula, Maria Luigia Vigliotti, Sonya De Lorenzo, Giacomo Loseto, Anna Passarelli, Matteo Nicola Dario Di Minno, Marco Tucci, Vincenzo De Feo, Fiorella D'Auria, Francesco Silvestris, Giovanni Di Minno, Pellegrino Musto
Rituximab is an effective treatment for CD20 + B-cell malignancies and autoimmune disorders. However, adverse drug reactions (ADRs) may occur after rituximab infusion, causing, in rare cases, its discontinuation. In this multicenter, retrospective study, among 374 patients treated with rituximab i.v., 23.5% experienced ADRs. Mean follow-up was 20.6 months (range 8-135). Overall, ADRs were significantly more frequent in non-Hodgkin lymphomas (NHL) and chronic lymphocytic leukemias (25-35.9%), than in autoimmune diseases (9...
April 3, 2017: Leukemia & Lymphoma
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