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Hepatomegaly

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https://www.readbyqxmd.com/read/28718838/phyllanthus-niruri-standardized-extract-alleviates-the-progression-of-non-alcoholic-fatty-liver-disease-and-decreases-atherosclerotic-risk-in-sprague-dawley-rats
#1
Raghdaa Hamdan Al Zarzour, Mariam Ahmad, Mohd Zaini Asmawi, Gurjeet Kaur, Mohammed Ali Ahmed Saeed, Majed Ahmed Al-Mansoub, Sultan Ayesh Mohammed Saghir, Nasiba Salisu Usman, Dhamraa W Al-Dulaimi, Mun Fei Yam
Non-alcoholic fatty liver disease (NAFLD) is one of the major global health issues, strongly correlated with insulin resistance, obesity and oxidative stress. The current study aimed to evaluate anti-NAFLD effects of three different extracts of Phyllanthus niruri (P. niruri). NAFLD was induced in male Sprague-Dawley rats using a special high-fat diet (HFD). A 50% methanolic extract (50% ME) exhibited the highest inhibitory effect against NAFLD progression. It significantly reduced hepatomegaly (16%) and visceral fat weight (22%), decreased NAFLD score, prevented fibrosis, and reduced serum total cholesterol (TC) (48%), low-density lipoprotein (LDL) (65%), free fatty acids (FFAs) (25%), alanine aminotransferase (ALT) (45%), alkaline phosphatase (ALP) (38%), insulin concentration (67%), homeostatic model assessment of insulin resistance (HOMA-IR) (73%), serum atherogenic ratios TC/high-density lipoprotein (HDL) (29%), LDL/HDL (66%) and (TC-HDL)/HDL (64%), hepatic content of cholesterol (43%), triglyceride (29%) and malondialdehyde (MDA) (40%) compared to a non-treated HFD group...
July 18, 2017: Nutrients
https://www.readbyqxmd.com/read/28717104/dexamethasone-induced-hepatomegaly-and-steatosis-in-larval-zebrafish
#2
Guojun Yin, Liping Cao, Jinliang Du, Rui Jia, Takio Kitazawa, Akira Kubota, Hiroki Teraoka
Fish hepatobiliary syndrome, characterized by hepatomegaly and fatty liver, has been frequently reported in many cultured fish species and has caused a dramatic economic loss in China. Glucocorticoids are thought to be important non-nutritional factors for hepatomegaly and fatty liver development. In the present study, a dexamethasone-induced zebrafish model of fatty liver and hepatomegaly was established, and the role of glucocorticoid receptor (GR) in the development of hepatomegaly and fatty liver was investigated using developing zebrafish...
2017: Journal of Toxicological Sciences
https://www.readbyqxmd.com/read/28716242/a-novel-pathogenic-variant-in-an-iranian-ataxia-telangiectasia-family-revealed-by-next-generation-sequencing-followed-by-in-silico-analysis
#3
Mohammad Amin Tabatabaiefar, Paria Alipour, Azam Pourahmadiyan, Najmeh Fattahi, Laleh Shariati, Neda Golchin, Javad Mohammadi-Asl
Ataxia telangiectasia (A-T) is a neurodegenerative autosomal recessive disorder with the main characteristics of progressive cerebellar degeneration, sensitivity to ionizing radiation, immunodeficiency, telangiectasia, premature aging, recurrent sinopulmonary infections, and increased risk of malignancy, especially of lymphoid origin. Ataxia Telangiectasia Mutated gene, ATM, as a causative gene for the A-T disorder, encodes the ATM protein, which plays an important role in the activation of cell-cycle checkpoints and initiation of DNA repair in response to DNA damage...
August 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28698688/retro-orbital-and-disseminated-b-cell-lymphoma-in-a-yellow-collared-macaw-primolius-auricollis
#4
Kim Le, Hugues Beaufrère, Emily Brouwer, S Karlyn Bland, Sarah Wills, Shawn MacKenzie, Heather Chalmers, Chantale Pinard, R Darren Wood, Josepha DeLay, Dale A Smith
A yellow-collared macaw was presented with unilateral left exophthalmia. The complete blood cell count and biochemistry revealed a heterophilic leukocytosis and elevation in liver parameters, respectively. A computed tomography scan showed a contrast-enhancing retrobulbar mass and hepatomegaly. Cytology of the liver was consistent with a round cell tumor, most likely lymphoma. The bird died after 2 months of palliative care. Postmortem examination confirmed a retro-orbital and disseminated B-cell lymphoma.
July 2017: Canadian Veterinary Journal. la Revue Vétérinaire Canadienne
https://www.readbyqxmd.com/read/28697823/-clinical-feature-and-genetic-analysis-of-a-family-affected-by-congenital-bile-acid-synthesis-defect-type-2-identification-of-2-novel-mutations-in-akr1d1-gene
#5
Ying Cheng, Li Guo, Mei Deng, Yuan-Zong Song
Congenital bile acid synthesis defect type 2 (CBAS2) is an autosomal recessive disorder caused by biallelic mutations of AKR1D1 gene, which encodes the Δ4-3-oxo-steroid 5β-reductase. Cholestatic jaundice is the main clinical manifestation, accompanied by malabsorption of fat and fat-soluble vitamins. This paper reported the clinical and genetic features of a CBAS2 patient definitely diagnosed by AKR1D1 genetic analysis. An 8-month-old male infant was referred to the hospital with the complaint of jaundiced skin and sclera over 7 months...
July 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28695157/lipoprotein-lipase-deficiency-in-an-infant-with-chylomicronemia-hepatomegaly-and-lipemia-retinalis
#6
Dinesha Maduri Vidanapathirana, Thushara Rodrigo, Samantha Waidyanatha, Eresha Jasinge, Amanda J Hooper, John R Burnett
No abstract text is available yet for this article.
2017: Global Pediatric Health
https://www.readbyqxmd.com/read/28687948/role-of-hepatobiliary-scintigraphy-and-preoperative-liver-biopsy-for-exclusion-of-biliary-atresia-in-neonatal-cholestasis-syndrome
#7
Ankur Mandelia, Richa Lal, Nijagal Mutt
All diagnostic algorithms for Neonatal Cholestasis Syndrome (NCS) focus on differentiating numerous medical causes from Biliary Atresia (BA). No preoperative diagnostic algorithm has 100% diagnostic accuracy for BA and yet, timely diagnosis is crucial to optimize surgical outcome. Markers for high index of clinical suspicion for BA are: a "usually" well thriving infant with conjugated hyperbilirubinemia, raised gamma glutamyl transpeptidase, persistently "acholic" stools, firm hepatomegaly with dysmorphic, hypoplastic gall bladder...
July 8, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28685844/genetic-characterization-of-gsd-i-in-serbian-population-revealed-unexpectedly-high-incidence-of-gsd-ib-and-three-novel-slc37a4-variants
#8
Anita Skakic, Maja Djordjevic, Adrijan Sarajlija, Kristel Klaassen, Natasa Tosic, Bozica Kecman, Milena Ugrin, Vesna Spasovski, Sonja Pavlovic, Maja Stojiljkovic
Glycogen storage disease (GSD) type I is inborn metabolic disease characterized by accumulation of glycogen in multiple organs. We analyzed 38 patients with clinical suspicion of GSD I using Sanger and next-generation sequencing (NGS). We identified 28 GSD Ib and five Ia patients. In five patients, GSD III, VI, IX, cholesteryl-ester storage disease and Shwachman-Diamond syndrome diagnoses were set using NGS. Incidences for GSD Ia and GSD Ib were estimated at 1:172746 and 1:60461 live-births respectively. Two variants were identified in G6PC gene: c...
July 7, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28680700/ultrasonographic-and-clinicopathologic-features-of-segmental-dilatations-of-the-common-bile-duct-in-four-cats
#9
Heather N Spain, Dominique G Penninck, Cynthia Rl Webster, Evence Daure, Samuel H Jennings
CASE SERIES SUMMARY: This case series documents ultrasonographic and clinicopathologic features of four cats with marked segmental dilatations of the common bile duct (CBD). All cats had additional ultrasonographic changes to the hepatobiliary system, including hepatomegaly, tubular to saccular intra/extrahepatic biliary duct dilatation and biliary debris accumulation. Based on all available data the presence of extrahepatic biliary duct obstruction (EHBDO) was ruled out in 3/4 cases and was equivocal in one case...
January 2017: JFMS open reports
https://www.readbyqxmd.com/read/28678091/prenatally-diagnosed-infant-aml
#10
Mary-Pat Schlosser, Shannon Bucking, Brigitte Lemyre, David Grynspan, Ruth Padmore, Donna Johnston
We report the first case of a fetus with acute myeloid leukemia, without Down syndrome, diagnosed in utero. A cordocentesis sample prepared to investigate hepatomegaly led to further evaluations revealing acute myeloid leukemia, monocytic type, in the fetus. Cytogenetic analysis showed mixed lineage leukemia duplication, no gene disruption or trisomy. Planned treatment included intrauterine exchange transfusion to extend gestation, low-dose chemotherapy at birth, and full chemotherapy once stable. Before any intervention, the child was delivered emergently for maternal condition and died 2 hours later...
July 3, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28674823/comparison-of-scrub-typhus-with-and-without-meningitis
#11
Winsley Rose, Urmi Ghosh, Anu Punnen, Rajiv Sarkar, John Jude Antony Prakash, Valsan Philip Verghese
OBJECTIVE: To compare the children admitted with scrub typhus with and without meningitis. METHODS: All children admitted with scrub typhus over a 62 mo period were reviewed. Statistical analysis was performed to compare those with and without meningitis for demographic, clinical, investigations and outcome parameters. RESULTS: Four hundred twenty seven children were admitted with scrub typhus and 63 (14.8%) had meningitis. The mean cerebrospinal fluid white blood cell (CSF WBC) count was 71 cells/cu...
July 4, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28656936/blastic-plasmacytoid-dendritic-cell-leukemia-in-a-black-malian
#12
Y L Diallo, J Cucherousset, O Samassekou, M Doumbere, Y Cissoko, D T Menta, N Ouologuem, M Ouologuem, M Konate, A T Sidibe
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, clinically aggressive hematologic malignancy, that most commonly manifests as cutaneous lesions. A 19-year-old Malian female was admitted to the Unit of Medicine of Hopital du Mali with anemia, fever, weakness, and weight loss. On physical examination she was wasted, pale, febrile (37.4°C), and had inguinal and axillary lymphadenopathies. The complete blood count found pancytopenia with Hemoglobin level of 4.8 g/dL, Leucocytes count of 1900/μL (neutrophil: 300/μL), and platelets count of 56 000/μL...
June 2017: Nigerian Journal of Clinical Practice
https://www.readbyqxmd.com/read/28652510/beta-blocker-and-steroid-therapy-in-the-treatment-of-infantile-hepatic-hemangioendothelioma
#13
Zeynep Canan Özdemir, Yeter Düzenli Kar, Nurullah Cihan Şöhret, Mahmut Kebapçı, Özcan Bör
Infantile hepatic hemangioendothelioma (IHHE) is the most common benign vascular liver tumor and typically occurs during the first 6 months of life. A 4-month-old male patient presented with abdominal distention. A physical examination revealed massive hepatomegaly. Liver enzyme levels were normal. The alpha fetoprotein level was 1,323 mg/dL (6-1,000). Abdominal magnetic resonance imaging (MRI) showed multiple, well-defined and hyperintense nodular lesions in the liver. MRI findings suggested IHHE. The thyroid stimulating hormone (TSH) level was high (177...
June 25, 2017: Drug Discoveries & Therapeutics
https://www.readbyqxmd.com/read/28642818/nivolumab-as-salvage-treatment-in-a-patient-with-hiv-related-relapsed-refractory-hodgkin-lymphoma-and-liver-failure-with-encephalopathy
#14
Jose D Sandoval-Sus, Francis Mogollon-Duffo, Ankita Patel, Nathan Visweshwar, Damian A Laber, Richard Kim, Michael V Jagal
BACKGROUND: We report the first case to our knowledge of a patient with relapsed/refractory classical hodgkin lymphoma and liver failure with encephalopathy along with human immunodeficiency virus/acquired immunodeficiency syndrome infection, successfully treated with nivolumab without major side effects and encouraging prolonged disease control. CASE PRESENTATION: In December 2015, at the time of the patient's progression from his Hodgkin lymphoma after fourth line treatment, he developed persistent fevers, abdominal distension, jaundice and worsening of his liver function tests...
2017: Journal for Immunotherapy of Cancer
https://www.readbyqxmd.com/read/28639269/hepatobiliary-and-pancreatic-giant-hepatomegaly-from-hepatic-hemagiomatosis
#15
J M Costa, D Costa, F Vieira, C Rolanda
No abstract text is available yet for this article.
July 2017: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28632778/over-expressing-the-soluble-gp130-fc-does-not-ameliorate-methionine-and-choline-deficient-diet-induced-non-alcoholic-steatohepatitis-in-mice
#16
Helene L Kammoun, Tamara Louise Allen, Darren Colin Henstridge, Michael James Kraakman, Lone Peijs, Stefan Rose-John, Mark Anthony Febbraio
Non-alcoholic steatohepatitis (NASH) is a liver disease with the potential to lead to cirrhosis and hepatocellular carcinoma. Interleukin-6 (IL-6) has been implicated in the pathogenesis of NASH, with the so-called IL-6 'trans-signaling' cascade being responsible for the pro-inflammatory actions of this cytokine. We aimed to block IL-6 'trans-signaling', using a transgenic mouse that overexpresses human soluble glycoprotein130 (sgp130Fc Tg mice) fed a commonly used dietary model of inducing NASH (methionine and choline deficient-diet; MCD diet) and hypothesized that markers of NASH would be ameliorated in such mice...
2017: PloS One
https://www.readbyqxmd.com/read/28629605/ct-findings-in-11-patients-with-tafro-syndrome-a-variant-of-multicentric-castleman-s-disease
#17
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/28627441/clinical-and-genetic-characteristics-of-17-chinese-patients-with-glycogen-storage-disease-type-ixa
#18
Jiangwei Zhang, Yuheng Yuan, Mingsheng Ma, Yan Liu, Weimin Zhang, Fengxia Yao, Zhengqing Qiu
Glycogen storage disease (GSD) type IXa is caused by PHKA2 mutation, which accounts for about 75% of all the GSD type IX cases. Here we first summarized the clinical data and analyzed the PHKA2 gene of 17 Chinese male patients suspected of having GSD type IXa. Clinical symptoms of our patients included hepatomegaly, growth retardation, and liver dysfunction. The clinical and biochemical manifestations improved and even disappeared with age. We detected 14 mutations in 17 patients, including 8 novel mutations; exons 2 and 4 were hot spots in this research...
June 13, 2017: Gene
https://www.readbyqxmd.com/read/28620611/dogs-with-acute-myeloid-leukemia-have-clonal-rearrangements-in-t-and-b-cell-receptors
#19
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/28617828/erad-defects-and-the-hfe-h63d-variant-are-associated-with-increased-risk-of-liver-damages-in-alpha-1-antitrypsin-deficiency
#20
Philippe Joly, Hélène Vignaud, Julie Di Martino, Mathias Ruiz, Roman Garin, Lioara Restier, Abdelouahed Belmalih, Christelle Marchal, Christophe Cullin, Benoit Arveiler, Patricia Fergelot, Aaron D Gitler, Alain Lachaux, Julien Couthouis, Marion Bouchecareilh
BACKGROUND: The most common and severe disease causing allele of Alpha 1-Antitrypsin Deficiency (1ATD) is Z-1AT. This protein aggregates in the endoplasmic reticulum, which is the main cause of liver disease in childhood. Based on recent evidences and on the frequency of liver disease occurrence in Z-1AT patients, it seems that liver disease progression is linked to still unknown genetic factors. METHODS: We used an innovative approach combining yeast genetic screens with next generation exome sequencing to identify and functionally characterize the genes involved in 1ATD associated liver disease...
2017: PloS One
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