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https://www.readbyqxmd.com/read/28224373/drug-induced-liver-injury-in-the-setting-of-glycogenic-hepatopathy
#1
Valmiki Maharaj, Matthew Fitz, Xianzdong Ding
Glycogenic hepatopathy (GH) is an underdiagnosed complication of uncontrolled type 1 diabetes mellitus (T1DM). It appears as an acute relapsing hepatitis with reversible transaminase elevations secondary to excessive hepatic glycogen accumulation. Patients are often asymptomatic but can present with abdominal pain, nausea and vomiting. Physical examination shows hepatomegaly without splenomegaly. GH is diagnosed by biopsy as it is clinically indistinguishable from non-alcoholic fatty liver disease (NAFLD), a more common cause of hepatic dysfunction in diabetics...
February 21, 2017: Journal of General Internal Medicine
https://www.readbyqxmd.com/read/28222652/results-from-the-belgian-mantle-cell-lymphoma-registry
#2
Vibeke Vergote, Ann Janssens, Marc André, Christophe Bonnet, Vanessa Van Hende, Eric Van Den Neste, Koen Van Eygen, Marie Maerevoet, Delphine Pranger, Wilfried Schroyens, Sarah Debussche, Vincent Maertens, Karolien Beel, Jan Lemmens, Charlotte Caron, Vanessa Delrieu, Isabelle Van Den Broeck, Gaetan Vanstraelen, Caroline Jacquy, Liesbeth Schauvlieghe, Hade De Samblanx, Vincent Madoe, Stef Meers, Dominique Boulet, Gregor Verhoef, Achiel Van Hoof
INTRODUCTION: Mantle cell lymphoma is a B-cell non-Hodgkin's lymphoma characterized by a t(11;14), resulting in overexpression of cyclin D1. Conventional chemotherapy obtains frequent (but short) remissions, leading to a poor median overall survival (OS) of 3-5 years. To obtain more information about the prevalence and current treatment of Mantle cell lymphoma (MCL) in Belgium, we collected data in a Belgian registry of MCL. MATERIALS AND METHODS: All Belgian MCL patients, t(11;14) and/or cyclin D1 positive, seen in hematology departments over a one-year period (April 2013-March 2014) were included...
February 22, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28218669/a-review-of-gaucher-disease-pathophysiology-clinical-presentation-and-treatments
#3
REVIEW
Jérôme Stirnemann, Nadia Belmatoug, Fabrice Camou, Christine Serratrice, Roseline Froissart, Catherine Caillaud, Thierry Levade, Leonardo Astudillo, Jacques Serratrice, Anaïs Brassier, Christian Rose, Thierry Billette de Villemeur, Marc G Berger
Gaucher disease (GD, ORPHA355) is a rare, autosomal recessive genetic disorder. It is caused by a deficiency of the lysosomal enzyme, glucocerebrosidase, which leads to an accumulation of its substrate, glucosylceramide, in macrophages. In the general population, its incidence is approximately 1/40,000 to 1/60,000 births, rising to 1/800 in Ashkenazi Jews. The main cause of the cytopenia, splenomegaly, hepatomegaly, and bone lesions associated with the disease is considered to be the infiltration of the bone marrow, spleen, and liver by Gaucher cells...
February 17, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28217237/-caught-by-the-eye-of-sound-epigastric-swelling-due-to-xiphisternal-tuberculosis
#4
Shabnam Bhandari Grover, Sumit Arora, Amit Kumar, Hemal Grover, Amit Katyan, Deepthi Mohan Nair
BACKGROUND: Common causes of an epigastric mass include hepatomegaly, pancreatic pseudocyst and epigastric hernia, less common causes being carcinoma of the stomach or pancreas, whereas diseases of the sternum presenting as an epigastric swelling is extremely uncommon. We report a case of tubercular infection of the sternum located in the xiphoid process resulting in its presentation as an epigastric swelling. CASE REPORT: A 30-year-old immunocompetent woman with complaints of an epigastric swelling and undocumented pyrexia for four months was referred for sonographic evaluation with a clinical suspicion of an incompletely treated liver abscess...
2017: Polish Journal of Radiology
https://www.readbyqxmd.com/read/28212414/decline-in-infection-related-morbidities-following-drug-mediated-reductions-in-the-intensity-of-schistosoma-infection-a-systematic-review-and-meta-analysis
#5
Gisele Andrade, David J Bertsch, Andrea Gazzinelli, Charles H King
BACKGROUND: Since 1984, WHO has endorsed drug treatment to reduce Schistosoma infection and its consequent morbidity. Cross-sectional studies suggest pre-treatment correlation between infection intensity and risk for Schistosoma-related pathology. However, evidence also suggests that post-treatment reduction in intensity may not reverse morbidity because some morbidities occur at all levels of infection, and some reflect permanent tissue damage. The aim of this project was to systematically review evidence on drug-based control of schistosomiasis and to develop a quantitative estimate of the impact of post-treatment reductions in infection intensity on prevalence of infection-associated morbidity...
February 17, 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/28204893/gastrointestinal-system-manifestations-in-juvenile-systemic-lupus-erythematosus
#6
Hafize Emine Sönmez, Asuman Nur Karhan, Ezgi Deniz Batu, Yelda Bilginer, Ersin Gümüş, Hülya Demir, Aysel Yüce, Seza Özen
Systemic lupus erythematosus (SLE) is an autoimmune disease which may involve gastrointestinal system (GIS). The aim of this study was to present GIS manifestations of pediatric SLE patients. The medical files of 69 children with SLE followed between January 2011 and January 2016 were reviewed. All fulfilled the Systemic Lupus International Collaborating Clinics criteria. All patients (≤18 years of age) with GIS manifestations were included. GIS manifestations were observed in 19 (27.5%) out of 69 SLE patients and present at the time of SLE diagnosis in 13 (68...
February 16, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28203580/unusual-presentation-of-diffuse-large-b-cell-lymphoma-with-splenic-infarcts
#7
Vivek Kumar, Parita Soni, Vishangi Dave, Jonathan Harris
A 67-year-old man presented with a 3-day history of abdominal pain, fever, and significant weight loss over 2 months. Physical examination revealed left upper quadrant tenderness, hepatomegaly, splenomegaly, and bilateral pitting edema but peripheral lymphadenopathy was absent. Laboratory tests showed anemia, thrombocytopenia, elevated prothrombin time (PT), partial thromboplastin time (PTT), and increased lactate dehydrogenase (LDH). PTT was corrected completely in mixing study. Further workup for the cause of coagulopathy revealed decreased levels of all clotting factors except factor VIII and increase fibrinogen levels, which ruled out disseminated intravascular coagulation (DIC)...
January 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28202118/-clinical-and-laboratory-features-of-macrophage-activation-syndrome
#8
Li Guo, Mei-Ping Lu, Gui-Juan Dong, Li-Ping Teng, Yi-Ping Xu, Li-Xia Zou, Qi Zheng
OBJECTIVE: To study the clinical and laboratory features of macrophage activation syndrome (MAS) at the early stage of diagnosis, and to explore a method for early identification of MAS. METHODS: A retrospective analysis was performed for the demographic data, clinical and laboratory features, and treatment outcomes of 21 MAS patients. RESULTS: Of the 21 MAS patients, 14 had systemic juvenile idiopathic arthritis, 5 had Kawasaki disease (KD), and 2 had connective tissue disease (CTD) as primary diseases...
February 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28197978/managing-cardiovascular-risk-in-lysosomal-acid-lipase-deficiency
#9
REVIEW
James J Maciejko
Lysosomal acid lipase deficiency (LAL-D) is a rare, life-threatening, autosomal recessive, lysosomal storage disease caused by mutations in the LIPA gene, which encodes for lysosomal acid lipase (LAL). This enzyme is necessary for the hydrolysis of cholesteryl ester and triglyceride in lysosomes. Deficient LAL activity causes accumulation of these lipids in lysosomes and a marked decrease in the cytoplasmic free cholesterol concentration, leading to dysfunctional cholesterol homeostasis. The accumulation of neutral lipid occurs predominantly in liver, spleen, and macrophages throughout the body, and the aberrant cholesterol homeostasis causes a marked dyslipidemia...
February 14, 2017: American Journal of Cardiovascular Drugs: Drugs, Devices, and Other Interventions
https://www.readbyqxmd.com/read/28193996/a-case-report-of-primary-nasal-natural-killer-nk-t-cell-lymphoma-in-an-african-american-patient-presenting-with-hemophagocytic-syndrome
#10
Bowei Tan, Cherif Abdelmalek, James E O'Donnell, Thomas Toltaku, Rashid Chaudhry, Jen C Wang, Vladimir Gotlieb
BACKGROUND Extranodal natural killer/T-cell lymphoma, nasal type (ENKTCL) is generally an aggressive and rare non-Hodgkin lymphoma. It is most common in East Asians, Native Americans, and South Americans, but is rarely reported in blacks. CASE REPORT A 55-year-old African American male born in Grenada presented with a left nostril mass with facial swelling and biopsy subsequently confirmed a diagnosis of extranodal NK/T-cell lymphoma, nasal type (ENKTCL). Immunochemistry was positive for CD2, cytoplasmic CD3, CD7, CD 43, CD 56, granzyme B, and TIA-1...
February 14, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28151863/miliary-tuberculosis-epidemiologicaland-clinical-analysis-of-large-case-series-from-moderate-to-low-tuberculosis-endemic-country
#11
Ali Mert, Ferhat Arslan, Tülin Kuyucu, Emine Nur Koç, Mesut Ylmaz, Demet Turan, Sedat Altn, Filiz Pehlivanoglu, Gonul Sengoz, Dilek Yldz, Ilyas Dokmetas, Suheyla Komur, Behice Kurtaran, Tuna Demirdal, Hüseyin A Erdem, Oguz Resat Sipahi, Ayse Batirel, Emine Parlak, Recep Tekin, Özlem Güzel Tunçcan, Ilker Inanc Balkan, Osman Hayran, Bahadr Ceylan
The aim of this study was to determine the clinical features, and outcome of the patients with miliary tuberculosis (TB).We retrospectively evaluated 263 patients (142 male, 121 female, mean age: 44 years, range: 16-89 years) with miliary TB. Criteria for the diagnosis of miliary TB were at least one of the followings in the presence of clinical presentation suggestive of miliary TB such as prolonged fever, night sweats, anorexia, weight loss: radiologic criterion and pathological criterion and/or microbiological criterion; pathological criterion and/or microbiological criterion...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28148232/hepatic-tuberculosis-in-human-immunodeficiency-virus-co-infected-adults-a-case-series-of-south-african-adults
#12
Lilishia Gounder, Pravikrishnen Moodley, Paul K Drain, Andrew J Hickey, Mahomed-Yunus S Moosa
BACKGROUND: Although Mycobacterium tuberculosis (TB) infection may cause extrapulmonary disease in HIV-infected adults, HIV-associated hepatic TB has been poorly characterized. Our objective was to describe hepatic TB in HIV-infected adults. METHODS: Retrospective study of patients diagnosed with hepatic TB from 2005-2012 at Infectious Diseases Clinic, King Edward VIII Hospital, Durban, South Africa. RESULTS: Among twenty cases of histology-confirmed HIV-associated hepatic TB, median CD4 count was 47 cells/μl (inter-quartile range 27-107 cells/μl) and 75% (15/20) of patients had pre-existing pulmonary TB...
February 1, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28144463/drug-reaction-with-eosinophilia-and-systemic-symptoms-induced-by-valproic-acid-a-case-report
#13
Mahboubeh Darban, Bahador Bagheri
INTRODUCTION: Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare but life-threatening reaction to drugs such as carbamazepine and allopurinol. The condition is characterized by skin rashes, fever, hematological disturbances, lymphadenopathy, and organ failure, most probably hepatic dysfunction. To date, only a few cases of valproate-induced DRESS syndrome have been reported. CASE PRESENTATION: We report on the case of a 60-year-old man who had been treated with valproic acid some time before being referred to Kowsar Hospital, Semnan, Iran in December 2015...
September 2016: Iranian Red Crescent Medical Journal
https://www.readbyqxmd.com/read/28144188/clinical-manifestations-in-82-neurobrucellosis-patients-from-kosovo
#14
Shemsedin Dreshaj, Nexhmedin Shala, Gresa Dreshaj, Naser Ramadani, Albina Ponosheci
BACKGROUND: Central nervous system involvement is a serious complication of brucellosis with various incidence and various clinical presentations. PATIENTS AND METHODS: Hospitalized patients in University Clinical Centre, Clinic for Infectious diseases in Prishtina, with laboratory-confirmed brucellosis, were analyzed, a brucellosis-endemic region. Among the 648 confirmed cases with brucellosis during the period 1991- 2013, 82 patients (12.65%) were diagnosed with neurobrucellosis...
December 2016: Materia Socio-medica
https://www.readbyqxmd.com/read/28133237/-malignant-pleural-mesothelioma-with-excessive-inflammation-and-high-level-of-interleukin-6-a-case-report
#15
Tomoya Miura, Satoshi Iida, Takahiro Igaki, Hiroyuki Shiobara, Ryou Matsumoto, Katsutaka Mitachi, Teppei Miyakawa, Yoshiaki Ohata, Katsumasa Saitou, Takumi Irie, Shigeru Yamazaki
Malignant mesothelioma is a rare aggressive solid tumor that is invariably incurable. A 23-year-old female patient with ascites, anemia, and high levels of ferritin and CRP was diagnosed with pleural mesothelioma by exploratory laparotomy. She remained asymptomatic, but 7 years later, she developed intractable diarrhea and fever. Systematic chemotherapy with both cisplatin and pemetrexed was administered. However, the treatment was discontinued due to side effects, after which time the diarrhea, ascites, and fever became progressively more severe...
November 2016: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/28116801/assessment-of-treatment-response-in-non-alcoholic-steatohepatitis-using-advanced-magnetic-resonance-imaging
#16
S C Lin, E Heba, R Bettencourt, G Y Lin, M A Valasek, O Lunde, G Hamilton, C B Sirlin, R Loomba
BACKGROUND: Magnetic resonance imaging-derived measures of liver fat and volume are emerging as accurate, non-invasive imaging biomarkers in non-alcoholic steatohepatitis (NASH). Little is known about these measures in relation to histology longitudinally. AIM: To examine any relationship between MRI-derived proton-density fat-fraction (PDFF), total liver volume (TLV), total liver fat index (TLFI), vs. histology in a NASH trial. METHODS: This is a secondary analysis of a 24-week randomised, double-blind, placebo-controlled trial of 50 patients with biopsy-proven NASH randomised to oral ezetimibe 10 mg daily (n = 25) vs...
January 24, 2017: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28107068/successful-treatment-of-suspected-pulmonary-arterial-hypertension-in-a-mealy-amazon-parrot-amazona-farinose
#17
Sean M Brady, Anne Burgdorf-Moisuk, Sarah Silverman, Raymund F Wack
A 25-year-old, male mealy Amazon parrot (Amazona farinose) with a history of polycythemia, hepatomegaly, and epistaxis was evaluated for progressive lethargy and anorexia. Clinical laboratory testing revealed severe polycythemia (71%), hypophosphatemia (1.6 mg/dL), and mild hypokalemia (2.8 mEq/L). Radiographs showed marked hepatomegaly and loss of air sac space. Despite supportive treatments, the bird's condition deteriorated, and it developed ataxia, was unable to fly, and became oxygen dependent. An echocardiogram, including an air bubble study, revealed a right-to-left atrial shunt and presumed pulmonary arterial hypertension...
December 2016: Journal of Avian Medicine and Surgery
https://www.readbyqxmd.com/read/28105246/hepatobiliary-ultrasonographic-abnormalities-in-adult-patients-with-sickle-cell-anaemia-in-steady-state-in-ile-ife-nigeria
#18
Oluwatosin O Oguntoye, Dennis A Ndububa, Musah Yusuf, Rahman A Bolarinwa, Oluwagbemiga O Ayoola
BACKGROUND: Sickle cell anaemia (SCA) is associated with structural manifestations in the hepatobiliary axis. This study aimed to investigate the hepatobiliary ultrasonographic abnormalities in adult patients with sickle cell anaemia in steady state attending the Haematology clinic of a federal tertiary health institution in Ile-Ife, Nigeria. MATERIAL/METHODS: Basic demographic data as well as right upper abdominal quadrant ultrasonography of 50 consecutive sickle cell anaemia patients were compared with those of 50 age- and sex-matched subjects with HbAA as controls...
2017: Polish Journal of Radiology
https://www.readbyqxmd.com/read/28100235/solitary-fibrous-tumour-of-the-liver-report-on-metastasis-and-local-recurrence-of-a-malignant-case-and-review-of-literature
#19
REVIEW
Nelson Chen, Kellee Slater
BACKGROUND: Solitary fibrous tumours (SFT) are neoplasms of mesenchymal origin that predominantly arise from the pleura. SFT of the liver (SFTL) are a rare occurrence with little number of cases reported in English literature. Malignant cases of hepatic SFT are an even rarer occurrence. For this reason, the prognostic evaluation of SFTLs is unknown and difficult to measure. METHODS: A search on English literature on "Solitary Fibrous Tumour of the Liver" was conducted on common search engines (PubMed, Google)...
January 18, 2017: World Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28098109/visceral-leishmaniasis-in-adults-with-nephropathy
#20
H Kaaroud El Jeri, A Harzallah, S Barbouch, M M Bacha, R Kheder, S Turki, S Trabelsi, E Abderrahim, F Ben Hamida, T Ben Abdallah
The aim of this study is to evaluate the features of visceral leishmaniasis (VL) in adults with nephropathy, who were not infected with the human immunodeficiency virus. This is a retrospective study of 14 adults hospitalized between 2000 and 2014, with VL and renal involvement. Clinical, biological, and therapeutic data were collected from the patients' medical files. Eleven women and three men, most of whom were from the North of the country, with a mean age of 40.5 years were studied. Lupus was present in five cases, the Sicca syndrome in three cases, diabetes in one case, renal failure on dialysis in two cases, and there were three renal transplant recipients...
January 2017: Saudi Journal of Kidney Diseases and Transplantation
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