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https://www.readbyqxmd.com/read/28327053/predictive-parameters-for-imatinib-failure-in-patients-with-chronic-myeloid-leukemia
#1
Danijela Lekovic, Mirjana Gotic, Natasa Milic, Biljana Zivojinovic, Jelica Jovanovic, Natasa Colovic, Violeta Milosevic, Andrija Bogdanovic
OBJECTIVE: Until recently, imatinib was the standard first-line treatment in chronic myeloid leukemia (CML). The inclusion of nilotinib and dasatinib as first-line options in CML raised a debate on treatment selection. The aim of our study was to analyze predictive parameters for imatinib response as the first-line treatment of CML patients. METHODS: The study included 168 consecutive patients with chronic phase Philadelphia-positive CML who were diagnosed and treated with Imatinib 400 mg once daily at a single university hospital...
March 22, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28320214/progression-of-liver-disease-in-children-and-adults-with-lysosomal-acid-lipase-deficiency
#2
Barbara K Burton, Nancy Silliman, Sachin Marulkar
BACKGROUND AND OBJECTIVE: Manifestations of the autosomal recessive disorder lysosomal acid lipase deficiency (LAL-D) include hepatomegaly, elevated serum liver enzymes, and progressive liver disease. We report an analysis of time to progression from first clinical manifestation to first documentation of hepatic fibrosis, cirrhosis, or liver transplantation from an observational study of pediatric and adult patients with LAL-D (clinical trial registration: NCT01528917). METHODS: Data were analyzed from 31 patients with available biopsy data and 1 patient without biopsy data who had undergone liver transplantation...
March 21, 2017: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/28316383/condition-factor-and-organosomatic-indices-of-parasitized-rattus-rattus-as-indicators-of-host-health
#3
Neelima Gupta, D K Gupta, P K Sharma
This study describes the influence of parasitism (Trypanosoma lewisi and Cysticercus fasciolaris) on the condition factor, hepato and splenosomatic indices of the common house rat, Rattus rattus Linnaeus, 1758 from Rohilkhand, Uttar Pradesh, India. Examination of R. rattus (n = 389) revealed T. lewisi (prevalence 12.40 %; intensity14 parasites/1000 RBC) from the blood and C. fasciolaris (larval Taenia taeniaeformis) (prevalence 46.70 %; intensity 2-4 par/host) from the liver. Condition factor (K) and organosomatic indices [hepatosomatic index, spleenosomatic index (SSI)] were evaluated in two groups (Group I non pregnant, Group II pregnant) of female rats which were further subdivided into four subgroups each (a Uninfected, b T...
March 2017: Journal of Parasitic Diseases: Official Organ of the Indian Society for Parasitology
https://www.readbyqxmd.com/read/28296808/waitlisted-candidates-with-polycystic-liver-disease-are-more-likely-to-be-transplanted-than-those-with-chronic-liver-failure
#4
Sahil D Doshi, Therese Bittermann, Thomas D Schiano, David Seth Goldberg
BACKGROUND: Polycystic liver disease (PCLD) is characterized by cystic replacement of the hepatic parenchyma, leading to hepatic dysfunction, portal hypertension, and hepatomegaly. Patients with liver dysfunction and/or symptomatic disease are eligible for liver transplantation. However, little is known about these patients' waitlist outcomes relative to others with chronic liver disease. METHODS: We used OPTN/UNOS data from February 27, 2002 to December 31, 2015 to compare waitlist outcomes of adult patients with PCLD to those with chronic liver failure (CLF) and hepatocellular carcinoma (HCC)...
March 15, 2017: Transplantation
https://www.readbyqxmd.com/read/28296775/giant-hepatic-metastasis-in-a-patient-with-coin-like-small-cell-lung-carcinoma-incidentally-diagnosed-at-autopsy-a-case-report
#5
Decebal Fodor, Simona Gurzu, Anca Otilia Contac, Ioan Jung
RATIONALE: Encephalopathy is a rare complication of hepatic metastases. In this paper we present a case of a patient with lung cancer and metastatic-related giant hepatomegaly. PATIENT CONCERNS: A 78-year-old previously healthy male was admitted in the Emergency room in hepatic coma. DIAGNOSES: The abdominal CT scan examination revealed a huge liver filled with solid nodules. INTERVENTIONS: No interventions were performed...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28296377/-melioidosis-report-of-the-first-case-in-a-northern-state-of-m%C3%A3-xico
#6
Carlos Boroel-Cervantes, Mónica Ibarra-Valdez, Sandra Miranda-Pacheco, Elías Sánchez-Camarena, Tamara Wolburgth-Franco, Andrés Ortìz-González, José Luis Domínguez-Ríos
The melioidosis, is an infection caused by Burkhordelia pseudomallei that comprises heterogeneous clinical syndromes with acute or chronic evolution. The objective of this article is to report a case with unusual presentation and outside the known epidemiological context. We present the case of a 48-year-old man who came to our hospital with fever and a history of an abscess in the right subscapular region, physical examination showed hepatomegaly, splenomegaly, fever, without pulmonary symptoms. Within study it reveal multiple abscesses in liver and spleen, requiring surgical exploration and antibiotics (meropenem/vancomycin)...
March 2017: Revista Médica del Instituto Mexicano del Seguro Social
https://www.readbyqxmd.com/read/28292383/mucopolysaccharidoses-clinical-spectrum-and-frequency-of-different-types
#7
Huma Arshad Cheema, Hassan Suleman Malik, Muhammad Almas Hashmi, Zafar Fayyaz, Iqra Mushtaq, Nagina Shahzadi
OBJECTIVE: To determine the relative frequency and clinical features of different varieties of mucopolysaccharidosis. STUDY DESIGN: Descriptive study. PLACE AND DURATION OF STUDY: Department of Pediatric Gastroenterology, Hepatology and Nutrition, The Children's Hospital and The Institute of Child Health, Lahore, from January 2013 to December 2015. METHODOLOGY: All patients who had any feature suggestive of mucopolysaccharidosis were screened with detailed history, clinical examination and skeletal survey...
February 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28286788/primary-hepatic-amyloidosis-presenting-as-acute-on-chronic-liver-failure
#8
Madhumita Premkumar, Devaraja Rangegowda, Tanmay Vyas, Anand Kulkarni, Shrruti Grover, Rakhi Mahiwall, Shiv Kumar Sarin
Systemic amyloidosis of amyloid light chain associated protein (AL), also called primary amyloidosis, frequently involves the liver, but rarely causes clinically apparent liver disease. The more common presentation is with acute renal failure. Hepatomegaly and mild elevation of alkaline phosphatase are the most common clinical and biochemical findings, respectively. We report a case of systemic amyloidosis of AL that clinically presented as acute-on-chronic liver failure and resulted in a fatal clinical course in a 56-year-old man...
2017: ACG Case Reports Journal
https://www.readbyqxmd.com/read/28283841/clinical-and-molecular-variability-in-patients-with-phka2-variants-and-liver-phosphorylase-b-kinase-deficiency
#9
Deeksha S Bali, Jennifer L Goldstein, Keri Fredrickson, Stephanie Austin, Surekha Pendyal, Catherine Rehder, Priya S Kishnani
Glycogen storage disease (GSD) type IX is a rare disease of variable clinical severity affecting primarily the liver tissue. Individuals with liver phosphorylase b kinase (PhK) deficiency (GSD IX) can present with hepatomegaly with elevated serum transaminases, ketotic hypoglycemia, hyperlipidemia, and poor growth with considerable variation in clinical severity. PhK is a cAMP-dependent protein kinase that phosphorylates the inactive form of glycogen phosphorylase, phosphorylase b, to produce the active form, phosphorylase a...
March 12, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28272140/hiv-infected-children-with-hepatomegaly-and-ascites-is-there-something-more-than-an-infection
#10
Ankur Kumar Jindal, Jasmina Ahluwalia, Avinash Sharma, Sumeet Dhawan, Karalanglin Tiewsoh, Deepti Suri, Anindita Sinha, Akshay Saxena, Surjit Singh
No abstract text is available yet for this article.
March 27, 2017: AIDS
https://www.readbyqxmd.com/read/28258915/resurrection-of-whole-liver-radiotherapy-a-case-report
#11
Sabeena Choudhary, Swarupa Mitra, Manoj Kumar Sharma, Pooja Khullar, Upasna Saxena
This is a case report of a patient of lymphoma with liver metastasis who had persistent pain and jaundice despite multiple lines of chemotherapy. She underwent palliative liver irradiation. This is an uncommon modality due to fear of radiation induced hepatitis. We reviewed literature available on the use of liver irradiation for palliation and concluded that it can be safely used with good results. A 33-year-old female with Non-Hodgkin's Lymphoma (NHL) presented with obstructive jaundice. She underwent stenting followed by chemotherapy...
February 28, 2017: Journal of the Egyptian National Cancer Institute
https://www.readbyqxmd.com/read/28257338/expansion-of-the-phenotypic-spectrum-in-three-families-of-methyl-cpg-binding-protein-2-duplication-syndrome
#12
Amita Moirangthem, Moni Tuteja Bhatia, Priyanka Srivastava, Kausik Mandal, Archana Rai, Shubha R Phadke
The methyl CpG-binding protein 2 duplication syndrome (OMIM #300260) is characterized by hypotonia, developmental delay, spasticity, seizures, and recurrent infections. It is fully penetrant in males and the females can have varied manifestations because of skewed X-inactivation. The size of the duplication can range from 0.2 Mb to over 100 Mb. Around 150 cases have been reported in the literature so far. Here, we report the unusual findings in three cases such as hepatomegaly, ataxia and females with mild intellectual disability that further expand the phenotypic spectrum of this disorder...
April 2017: Clinical Dysmorphology
https://www.readbyqxmd.com/read/28250985/factors-associated-with-growth-retardation-in-children-suffering-from-sickle-cell-anemia-first-report-from-central-africa
#13
Aimé Lukusa Kazadi, René Makuala Ngiyulu, Jean Lambert Gini-Ehungu, Jean Marie Mbuyi-Muamba, Michel Ntetani Aloni
Background. The aim of this study was to investigate and determine the risk factors associated with poor growth among SCA children. Methods. A cross-sectional study was conducted in Kinshasa, the capital's country. The nutritional status was assessed using the Z scores of the anthropometric indices. Results. We gathered data on the 256 patients, 138 females (53.9%), who entered the study. The mean age at presentation was 8.4 ± 4.9 years of age. Underweight, stunting, and wasting were found, respectively, in 47...
2017: Anemia
https://www.readbyqxmd.com/read/28247771/poems-syndrome-and-idiopathic-portal-hypertension-a-possible-association
#14
Sara Campos, Cláudia Agostinho, Maria Augusta Cipriano
A 48-year old female patient was admitted to the emergency department with upper gastrointestinal bleeding. Endoscopy showed large esophageal varices that were treated with band ligation. She had been treated with cyclophosphamide, melphalan, lenalidomide and corticosteroids for POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy or edema, M protein, skin changes). She had no other risk factors for chronic liver disease. Laboratory and radiological examinations could not confirm the etiology of portal hypertension...
March 1, 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28245189/the-variable-clinical-phenotype-of-three-patients-with-hepatic-glycogen-synthase-deficiency
#15
Çiğdem Seher Kasapkara, Zehra Aycan, Esma Açoğlu, Saliha Senel, Melek Melahat Oguz, Serdar Ceylaner
BACKGROUND: Glycogen synthase deficiency, also known as glycogenosis (GSD) type 0 is an inborn error of glycogen metabolism caused by mutations in the GYS2 gene, which is transmitted in an autosomal recessive trait. It is a rare form of hepatic glycogen storage disease with less than 30 cases reported in the literature so far. The disorder is characterized by fasting hyperketotic hypoglycemia without hyperalaninemia or hyperlactacidemia. It is a glycogenosis with lack of liver glycogen synthesis, therefore hepatomegaly is not observed in patients with glycogen synthase deficiency...
February 28, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28242381/antidiabetic-effect-of-sn158-through-ppar%C3%AE-%C3%AE-dual-activation-in-ob-ob-mice
#16
Yujung Jung, Yongkai Cao, Suresh Paudel, Goo Yoon, Seung Hoon Cheon, Gyu-Un Bae, Li Tai Jin, Yong Kee Kim, Su-Nam Kim
In this study, we aimed to demonstrate the antidiabetic potential of (E)-N-(4-(3-(5-bromo-4-hydroxy-2-methoxyphenyl)acryloyl) phenyl)-4-tert-butylbenzamide (SN158) through peroxisome proliferator-activated receptor (PPAR)-α/γ dual activation. SN158 interacted with both PPARα and PPARγ, and increased their transcriptional activities. Simultaneously, SN158 treatment led to an increase in adipogenic differentiation of 3T3-L1 preadipocytes and fatty acid oxidation in hepatocytes. In addition, glucose uptake in myotubes was significantly increased by SN158 treatment...
February 24, 2017: Chemico-biological Interactions
https://www.readbyqxmd.com/read/28224373/drug-induced-liver-injury-in-the-setting-of-glycogenic-hepatopathy
#17
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
#18
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
#19
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
#20
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
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