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Splenomegaly

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https://www.readbyqxmd.com/read/29334440/multifactorial-splenomegaly
#1
Jay Allison, Rachel Sunne, Mark Huntington
Splenomegaly is an unusual finding among North Americans, but is commonly seen in many parts of the world. Increasingly, it can be encountered locally among recent immigrant and refugee populations. A broad differential diagnosis is required. We report the case of a 33-year-old refugee with multiple potential causes for splenomegaly. Empiric treatment of one of the infectious contributors to his splenomegaly resulted in a significant improvement of his quality of life, illustrating the importance of a thorough evaluation of potential causes of splenomegaly, especially in refugee and immigrant populations...
December 2017: South Dakota Medicine: the Journal of the South Dakota State Medical Association
https://www.readbyqxmd.com/read/29326879/characteristics-of-26-patients-with-type-3-gaucher-disease-a-descriptive-analysis-from-the-gaucher-outcome-survey
#2
Ida Vanessa D Schwartz, Özlem Göker-Alpan, Priya S Kishnani, Ari Zimran, Lydie Renault, Zoya Panahloo, Patrick Deegan
The Gaucher Outcome Survey (GOS) is an international disease-specific registry established in 2010 for patients with a confirmed diagnosis of Gaucher disease (GD), regardless of GD type or treatment status. Historically, there has been a limited understanding of type 3 GD (GD3) and its natural history in patients irrespective of their treatment status. Here, we describe the disease characteristics of patients with GD3 enrolled in GOS. As of October 2015, 1002 patients had been enrolled, 26 of whom were reported as GD3...
March 2018: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/29325068/emergence-of-non-falciparum-plasmodium-infection-despite-regular-artemisinin-combination-therapy-in-a-18-month-longitudinal-study-of-ugandan-children-and-their-mothers
#3
Martha Betson, Sarah Clifford, Michelle Stanton, Narcis B Kabatereine, J Russell Stothard
As part of a longitudinal cohort investigation of intestinal schistosomiasis and malaria in Ugandan children and their mothers on the shorelines of Lakes Victoria and Albert, we documented risk factors and morbidity associated with non-falciparum Plasmodium infections and the longitudinal dynamics of Plasmodium species in children. Host age, household location and P. falciparum infection were strongly associated with non-falciparum Plasmodium infections, and P. malariae infection was associated with splenomegaly...
January 6, 2018: Journal of Infectious Diseases
https://www.readbyqxmd.com/read/29324951/signs-and-symptoms-in-gaucher-disease-priority-nursing-diagnoses
#4
Márcia Koja Breigeiron, Vitória da Costa Moraes, Janice Carneiro Coelho
OBJECTIVE: Identify the signs and symptoms of patients with Gaucher Disease, inferring possible priority nursing diagnoses. METHOD: Cross-sectional study, developed in a specialized laboratory, between 2013 and 2015. The sample (n = 91) comprised the records of patients with genetic diagnosis for Gaucher Disease. The study respected research norms. RESULTS: Prevalence of female sex (57.1%), age at diagnosis between 0 and 10 years, and origin from the Southeast Region of Brazil were prevalent...
January 2018: Revista Brasileira de Enfermagem
https://www.readbyqxmd.com/read/29321547/polycythemia-vera-treatment-algorithm-2018
#5
REVIEW
Ayalew Tefferi, Alessandro M Vannucchi, Tiziano Barbui
Recently reported mature survival data have confirmed the favorable prognosis in polycythemia vera (PV), with an estimated median survival of 24 years, in patients younger than age 60 years old. Currently available drugs for PV have not been shown to prolong survival or alter the natural history of the disease and are instead indicated primarily for prevention of thrombosis. Unfortunately, study endpoints that are being utilized in currently ongoing clinical trials in PV do not necessarily target clinically or biologically relevant outcomes, such as thrombosis, survival, or morphologic remission, and are instead focused on components of disease palliation...
January 10, 2018: Blood Cancer Journal
https://www.readbyqxmd.com/read/29318565/a-case-of-severe-stenosis-of-hepatic-veins-and-inferior-vena-cava-with-stomal-variceal-bleeding-induced-by-oxaliplatin-based-chemotherapy
#6
Hayato Yamaguchi, Yoshihiro Furuichi, Yoshitaka Kasai, Hirohito Takeuchi, Yuu Yoshimasu, Katsutoshi Sugimoto, Ikuo Nakamura, Takao Itoi
A 27-year-old woman with colon cancer and liver metastasis was referred to our hospital. Colectomy and colostomy were performed to improve her ileus. Following 13 sessions of oxaliplatin-based chemotherapy (OC) with mFOLFOX6 + bevacizumab, thrombocytopenia and frequent peristomal bleeding occurred. Computed tomography showed severe ascites, splenomegaly, significant collateral veins around the stoma, and severe stenosis of the hepatic veins (HV) and inferior vena cava (IVC). Ultrasound elastography showed high liver (and spleen) stiffness values...
January 9, 2018: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/29317889/clinical-and-laboratory-analysis-of-patients-with-leishmaniasis-a-retrospective-study-from-a-tertiary-care-center-in-new-delhi
#7
Nitin Gupta, Kamla Kant, Bijay Ranjan Mirdha
Background: Leishmaniasis manifests as visceral (VL), cutaneous (CL) or a dermal sequel of VL, known as Post kala-azar dermal leishmaniasis (PKDL). The aim of the study was to analyze the clinical and laboratory features of cases diagnosed with leishmaniasis. Methods: This hospital-based retrospective study included all cases of VL, PKDL, and CL diagnosed between Jan 2011 to Jan 2016 at All India Institute of Medical Sciences, New Delhi. Clinical and laboratory profile of the diagnosed cases were analyzed in detail...
October 2017: Iranian Journal of Parasitology
https://www.readbyqxmd.com/read/29314222/impact-of-clinically-evident-portal-hypertension-on-clinical-outcome-of-patients-with-hepatocellular-carcinoma-treated-by-transarterial-chemoembolization
#8
Nam Hee Kim, Taeheon Lee, Yong Kyun Cho, Byung Ik Kim, Hong Joo Kim
BACKGROUND AND AIM: To determine the impact of clinically evident portal hypertension (CEPH) on prognosis of hepatocellular carcinoma (HCC) patients with Child-Pugh A cirrhosis who underwent transarterial chemoembolization (TACE). METHODS: A retrospective data analysis was performed for a total of 388 treatment-naïve HCC patients with Child-Pugh A cirrhosis who underwent TACE as first-line treatment from January 2000 to June 2014. Cumulative occurrence rate of complete response (CR), progression free survival (PFS), and overall survival (OS) were compared between patients with CEPH and those without CEPH [esophageal/gastric varices or low platelet count (<100,000/mm3 ) associated with splenomegaly]...
January 3, 2018: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29314206/follicular-lymphoma-2018-update-on-diagnosis-and-management
#9
Arnold Freedman
DISEASE OVERVIEW: Follicular lymphoma is generally an indolent B cell lymphoproliferative disorder of transformed follicular center B cells. Follicular lymphoma (FL) is characterized by diffuse lymphadenopathy, bone marrow involvement, splenomegaly and less commonly other extranodal sites of involvement. In general, cytopenias can occur but constitutional symptoms of fever, nightsweats, and weight loss are uncommon. DIAGNOSIS: Diagnosis is based on histology of preferably a biopsy of a lymph node...
February 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29312869/gastrointestinal-tract-involvement-in-acute-pancreatitis-initial-findings-and-follow-up-by-magnetic-resonance-imaging
#10
Yi-Fan Ji, Xiao-Ming Zhang, Don G Mitchell, Xing-Hui Li, Tian-Wu Chen, Yong Li, Zhi-Guo Bao, Wei Tang, Bo Xiao, Xiao-Hua Huang, Lin Yang
Background: To study the initial and follow up patterns of gastrointestinal tract involvement in acute pancreatitis (AP) using magnetic resonance imaging (MRI). Methods: A total of 209 patients with AP undergoing abdominal MRI on 1.5 T MRI were compared to 100 control patients selected from our daily clinical caseload who underwent MRI over the same recruitment period and had no other disease which can cause abnormality of gastrointestinal tract. Initial and follow up MRI examinations of gastrointestinal tract abnormalities were noted for AP patients...
December 2017: Quantitative Imaging in Medicine and Surgery
https://www.readbyqxmd.com/read/29306106/quantitative-assessment-of-jak2-v617f-and-calr-mutations-in-philadelphia-negative-myeloproliferative-neoplasms
#11
Ambrus Gángó, Réka Mózes, Zsófia Boha, Béla Kajtár, Botond Timár, Péter Attila Király, Richárd Kiss, Viktória Fésüs, Noémi Nagy, Judit Demeter, Gábor Körösmezey, Zita Borbényi, Imelda Marton, Anita Szőke, Tamás Masszi, Péter Farkas, Judit Várkonyi, Márk Plander, Éva Pósfai, Miklós Egyed, Katalin Pál, Gáspár Radványi, Aryan Hamed, Judit Csomor, András Matolcsy, Donát Alpár, Csaba Bödör
BACKGROUND: Philadelphia negative myeloproliferative neoplasms (MPNs) are characterized by frequent mutations of driver genes including JAK2, CALR and MPL. While the influence of JAK2 V617F mutant allele burden on the clinical phenotype of MPN patients is well-described, the impact of CALR mutant allele burden on clinical features needs further investigation. PATIENTS AND METHODS: Quantitative assessment of JAK2 and CALR mutations was performed on diagnostic DNA samples from 425 essential thrombocythemia (ET) and 227 primary myelofibrosis patients using real-time quantitative PCR and fragment length analysis...
January 2, 2018: Leukemia Research
https://www.readbyqxmd.com/read/29305630/chronic-myeloid-leukemia-patients-in-tunisia-epidemiology-and-outcome-in-the-imatinib-era-a-multicentric-experience
#12
Raihane Ben Lakhal, Hela Ghedira, Hatem Bellaaj, Yosra Ben Youssef, Samia Menif, Zeineb Manai, Manel Bedoui, Amel Lakhal, Fehmi M'Sadek, Moez Elloumi, Abderrahmane Khélif, Neila Ben Romdhane, Mohamed Adnène Laatiri, Tarek Ben Othmen, Balkis Meddeb
Data are limited in developing countries regarding the clinicopathologic features and response to therapy of chronic myeloid leukemia (CML) in the era of imatinib (IM). The objective of this study is to report on the clinicoepidemiologic features of CML in Tunisia, to evaluate the long-term outcome of patients in chronic (CP) or accelerated phase (AP) treated with IM 400 mg daily as frontline therapy, and to determine imatinib's efficacy and safety. From October 2002 to December 2014, 410 CML patients were treated with IM in six Tunisian departments of hematology...
January 6, 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29304824/patient-characteristics-associated-with-response-to-nsaid-monotherapy-in-children-with-systemic-juvenile-idiopathic-arthritis
#13
Anjali Sura, Christopher Failing, Julie Sturza, Jasmine Stannard, Meredith Riebschleger
BACKGROUND: Systemic juvenile idiopathic arthritis (sJIA) is an auto-inflammatory disease characterized by fever, arthritis, and ≥1 of rash, generalized lymphadenopathy, hepato/splenomegaly, and serositis. Non-steroidal anti-inflammatory drugs (NSAIDs) are among the initial treatments of sJIA, but there is currently no evidence indicating which children should undergo a trial of NSAID monotherapy and which should not. Our objective is to identify presentation characteristics which are associated with response and lack of response to a trial of NSAID monotherapy...
January 5, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29302559/peripheral-t-cell-lymphoma-not-otherwise-specified
#14
Kunal Kishor Jha, Suresh K Gupta, Harpreet Saluja, Nuwadatta Subedi
The peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) belongs to a heterogeneous class of aggressive neoplasms. Although several morphologic subtypes of this tumor have been described, no particular genetic, immunological, or distinct clinical features define this disease. Patients can experience night sweats, fever, lymphadenopathy, weight loss, splenomegaly, and/or skin changes. Common laboratory tests reveal that patients have anemia, thrombocytosis, lymphocytosis, eosinophilia, hypergammaglobulinemia, or increased lactate dehydrogenase...
April 2017: Journal of Family Medicine and Primary Care
https://www.readbyqxmd.com/read/29299089/hamartoma-of-the-spleen-splenoma-with-calcifications-in-a-child-with-beta-thalassemia-a-case-report
#15
Dario Giambelluca, Dario Picone, Giuseppe Lo Re, Salvatore Pappalardo, Placido Romeo
Splenic hamartoma (or splenoma) is a rare, benign, vascular tumor, often incidentally found at imaging, surgery or autopsy. Albeit usually asymptomatic and rare in children, when it occurs in the pediatric population it is more commonly symptomatic. We report a case of a 15-year-old girl with iron-deficiency anemia and beta-thalassemia, who had a large (10 × 8 × 7 cm) splenic lesion with calcifications, incidentally found during follow-up for splenomegaly and histologically characterized as hamartoma with calcified areas...
May 2017: Journal of Radiology Case Reports
https://www.readbyqxmd.com/read/29296743/hmga2-collaborates-with-jak2v617f-in-the-development-of-myeloproliferative-neoplasms
#16
Koki Ueda, Kazuhiko Ikeda, Takayuki Ikezoe, Kayo Harada-Shirado, Kazuei Ogawa, Yuko Hashimoto, Takahiro Sano, Hiroshi Ohkawara, Satoshi Kimura, Akiko Shichishima-Nakamura, Yuichi Nakamura, Yayoi Shikama, Tsutomu Mori, Philip J Mason, Monica Bessler, Soji Morishita, Norio Komatsu, Kotaro Shide, Kazuya Shimoda, Shuhei Koide, Kazumasa Aoyama, Motohiko Oshima, Atsushi Iwama, Yasuchika Takeishi
High-mobility group AT-hook 2 (HMGA2) is crucial for the self-renewal of fetal hematopoietic stem cells (HSCs) but is downregulated in adult HSCs via repression by MIRlet-7 and the polycomb-recessive complex 2 (PRC2) including EZH2. The HMGA2 messenger RNA (mRNA) level is often elevated in patients with myelofibrosis that exhibits an advanced myeloproliferative neoplasm (MPN) subtype, and deletion of Ezh2 promotes the progression of severe myelofibrosis in JAK2V617F mice with upregulation of several oncogenes such as Hmga2...
June 27, 2017: Blood Advances
https://www.readbyqxmd.com/read/29295842/unopposed-il-18-signaling-leads-to-severe-tlr9-induced-macrophage-activation-syndrome-in-mice
#17
Charlotte Girard-Guyonvarc'h, Jennifer Palomo, Praxedis Martin, Emiliana Rodriguez, Sabina Troccaz, Gaby Palmer, Cem Gabay
The term macrophage activation syndrome (MAS) defines a severe, potentially fatal disorder characterized by overwhelming inflammation and multiorgan involvement. Interleukin (IL)-18 is a pro-inflammatory cytokine belonging to the IL-1 family, the activity of which is regulated by its endogenous inhibitor IL-18 binding protein (IL-18BP). Elevated IL-18 levels have been reported in patients with MAS. Herein we show that upon repeated toll-like receptor (TLR)9 stimulation with CpG, IL-18BP-/- mice display severe MAS manifestations, including increased weight loss, splenomegaly, anemia, thrombocytopenia, hyperferritinemia and bone marrow hemophagocytosis as compared to wild-type mice...
January 2, 2018: Blood
https://www.readbyqxmd.com/read/29285715/gene-mutations-and-clinical-phenotypes-in-15-chinese-children-with-cryopyrin-associated-periodic-syndrome-caps
#18
Caifeng Li, Xiaohua Tan, Junmei Zhang, Shipeng Li, Wenxiu Mo, Tongxin Han, Weiying Kuang, Yifang Zhou, Jianghong Deng
The aim of our study is to explore the features of clinical manifestations and genetic mutations in Chinese CAPS patients. Fifteen confirmed patients with CAPS were enrolled. The onset time ranges from 2 days after birth to 6 years and 1 month. Recurrent urticaria rash (93.3%) with fever (100%) were two dominant characteristics of these patients that were presented as either acute or chronic process. Systemic involvements were found in all patients except for one with only rash and fever. The top three symptoms were fever (100%), rash (93...
December 2, 2017: Science China. Life Sciences
https://www.readbyqxmd.com/read/29284921/splenic-localization-of-primary-hydatid-cyst-in-a-27-year-old-sportsman-treated-by-pair-technique-imaging-anatomy-assessment
#19
Dzenan Jahic, Eldan Kapur, Edin Begic, Enver Zerem
Introduction: In this paper, we report our experience with a case of primary hydatid cyst involving only the spleen in a 27-year-old sportsman treated by PAIR technique. Case Report: Five years before, a 27-year-old handball player being admitted to our hospital, it was detected the cyst in his spleen which size was 35 x 30 mm in diameter, by abdominal ultrasound during a systematic examination. There was no pain or any other symptoms at that time. Tests on the presence of echinococcus cysts were negative...
December 2017: Acta Informatica Medica: AIM
https://www.readbyqxmd.com/read/29282357/risk-factors-for-arterial-versus-venous-thrombosis-in-polycythemia-vera-a-single-center-experience-in-587-patients
#20
S Cerquozzi, D Barraco, T Lasho, C Finke, C A Hanson, R P Ketterling, A Pardanani, N Gangat, A Tefferi
In a recent International Working Group on Myeloproliferative Neoplasms Research and Treatment (IWG-MRT) study, prior arterial events and hypertension were predictors of subsequent arterial thrombosis whereas prior venous events and age ≥65 years predicted venous thrombosis in polycythemia vera (PV). In the current study, we sought to validate the above findings and identify additional predictors of arterial versus venous thrombosis. At a median follow up of 109 months, thrombosis after diagnosis occurred in 128 (22%) patients; 82 (14%) arterial and 57 (10%) venous events...
December 27, 2017: Blood Cancer Journal
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