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https://www.readbyqxmd.com/read/27898593/coinfection-with-hiv-and-hepatitis-c-virus-in-229-children-and-young-adults-living-in-europe
#1
(no author information available yet)
OBJECTIVE: To characterize children, adolescents and young adults infected with HIV/hepatitis C virus (HCV) vertically or before age of 18 years and living in Europe regarding mode of acquisition, HCV genotype, clinical status and treatment. DESIGN: Retrospective, cross-sectional study using pooled data from 11 European paediatric HIV cohorts. METHODS: Patients aged more than 18 months and less than 25 years, with HIV/HCV acquired vertically or in childhood, were included...
January 2, 2017: AIDS
https://www.readbyqxmd.com/read/27898169/cross-sectional-analysis-of-progressive-familial-intrahepatic-cholestasis-in-puerto-rican-children
#2
Dellys M Soler, Antonio I Del Valle, David Fernandez-Lube, Benjamin L Shneider
OBJECTIVE: Specific inherited disorders may be more common in island communities. Prior case reports suggest that cholestatic liver diseases may constitute a group of these inherited disorders in Puerto Rico. A cross-sectional survey of liver diseases in children was conducted to assess this hypothesis. METHODS: A cross-sectional analysis was performed in patients with chronic cholestasis at "Hospital Pediátrico Universitario" in San Juan, Puerto Rico. Ten potential participants with high gamma-glutamyl transpeptidase (GGTP) cholestasis were identified...
December 2016: Puerto Rico Health Sciences Journal
https://www.readbyqxmd.com/read/27897243/diagnostic-performance-of-contrast-enhanced-ct-in-pyrrolizidine-alkaloids-induced-hepatic-sinusoidal-obstructive-syndrome
#3
Xuefeng Kan, Jin Ye, Xinxin Rong, Zhiwen Lu, Xin Li, Yong Wang, Ling Yang, Keshu Xu, Yuhu Song, Xiaohua Hou
Hepatic sinusoidal obstruction syndrome (HSOS) can be caused by pyrrolizidine alkaloids(PAs)-containing herbals. Since PAs exposure is obscure and clinical presentation of HSOS is unspecific, it is challenge to establish the diagnosis of PAs-induced HSOS. Gynura segetum is one of the most wide-use herbals containing PAs. The aim of our study is to describe the features of contrast-enhanced computed tomography (CT) in gynura segetum-induced HSOS, and then determine diagnostic performance of radiological signs...
November 29, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27896132/divergent-clinical-outcomes-of-alpha-glucosidase-enzyme-replacement-therapy-in-two-siblings-with-infantile-onset-pompe-disease-treated-in-the-symptomatic-or-pre-symptomatic-state
#4
Takashi Matsuoka, Yoshiyuki Miwa, Makiko Tajika, Madoka Sawada, Koichiro Fujimaki, Takashi Soga, Hideshi Tomita, Shigeru Uemura, Ichizo Nishino, Tokiko Fukuda, Hideo Sugie, Motomichi Kosuga, Torayuki Okuyama, Yoh Umeda
Pompe disease is an autosomal recessive, lysosomal glycogen storage disease caused by acid α-glucosidase deficiency. Infantile-onset Pompe disease (IOPD) is the most severe form and is characterized by cardiomyopathy, respiratory distress, hepatomegaly, and skeletal muscle weakness. Untreated, IOPD generally results in death within the first year of life. Enzyme replacement therapy (ERT) with recombinant human acid alpha glucosidase (rhGAA) has been shown to markedly improve the life expectancy of patients with IOPD...
December 2016: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/27893200/clinical-impact-of-overexpression-of-foxp3-and-wt1-on-disease-outcome-in-egyptian-acute-myeloid-leukemia-patients
#5
Magda Assem, Ahmed Osman, Eman Kandeel, Reham Elshimy, Hanan Nassar, Radwa Ali
Background: In the last decade, it has become clear that change of gene expression may alter the hematopoietic cell quiescent state and consequently play a major role in leukemogenesis. WT1 is known to be a player in acute myeloid leukemia (AML) and FOXP3 has a crucial role in regulating the immune response. Objectives: To evaluate the impact of overexpression of WT1and FOXP3 genes on clinical course in adult and pediatric AML patients in Egypt. Patients and methods: Bone marrow and peripheral blood samples were obtained from 97 de novo non M3 AML patients (63 adult and 34 pediatric)...
January 10, 2016: Asian Pacific Journal of Cancer Prevention: APJCP
https://www.readbyqxmd.com/read/27882258/dysmorphic-facial-features-and-other-clinical-characteristics-in-two-patients-with-pex1-gene-mutations
#6
Mehmet Gunduz, Ozlem Unal
Peroxisomal disorders are a group of genetically heterogeneous metabolic diseases related to dysfunction of peroxisomes. Dysmorphic features, neurological abnormalities, and hepatic dysfunction can be presenting signs of peroxisomal disorders. Here we presented dysmorphic facial features and other clinical characteristics in two patients with PEX1 gene mutation. Follow-up periods were 3.5 years and 1 year in the patients. Case I was one-year-old girl that presented with neurodevelopmental delay, hepatomegaly, bilateral hearing loss, and visual problems...
2016: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/27882065/hepatic-alveolar-hydatid-cyst-a-brief-review-of-published-cases-from-iran-in-the-last-20-years
#7
REVIEW
Bita Geramizadeh, Mohammad Baghernezhad
CONTEXT: Echinococcus multilocularis is the cause of alveolar hydatid disease, which most commonly involves the liver in more than 90% of cases. This disease is endemic in northern Iran. However, there are very few published cases from Iran. In this article, we will review all of the published cases of hepatic alveolar echinococcosis from Iran regarding all aspects, including clinical, paraclinical, and treatment protocols. EVIDENCE ACQUISITION: In this brief review, the published cases of hepatic Alveolar Echinococcosis (AE) from Iran were retrieved for review via a search in PubMed, Scopus, Google Scholar, IranMedex, scientific information database (SID), Magiran, and Irandoc (1995 - 2015) using the keywords Echinococcus multilocularis and Iran, Alveolar hydatid cyst and Iran, liver and Alveolar hydatid cyst and Iran, and Hepatic alveolar hydatid cyst and Iran...
October 2016: Hepatitis Monthly
https://www.readbyqxmd.com/read/27876045/seroprevalence-of-avian-hepatitis-e-virus-and-avian-leucosis-virus-subgroup-j-in-chicken-flocks-with-hepatitis-syndrome-china
#8
Yani Sun, Taofeng Du, Baoyuan Liu, Shahid Faraz Syed, Yiyang Chen, Huixia Li, Xinjie Wang, Gaiping Zhang, En-Min Zhou, Qin Zhao
BACKGROUND: From 2014 to 2015 in China, many broiler breeder and layer hen flocks exhibited a decrease in egg production and some chickens developed hepatitis syndrome including hepatomegaly, hepatic necrosis and hemorrhage. Avian hepatitis E virus (HEV) and avian leucosis virus subgroup J (ALV-J) both cause decreasing in egg production, hepatomegaly and hepatic hemorrhage in broiler breeder and layer hens. In the study, the seroprevalence of avian HEV and ALV-J in these flocks emerging the disease from Shandong and Shaanxi provinces were investigated...
November 22, 2016: BMC Veterinary Research
https://www.readbyqxmd.com/read/27870387/primary-myelofibrosis-2017-update-on-diagnosis-risk-stratification-and-management
#9
Ayalew Tefferi
: Disease overview: Primary myelofibrosis (PMF) is a myeloproliferative neoplasm (MPN) characterized by stem cell-derived clonal myeloproliferation that is often but not always accompanied by JAK2, CALR or MPL mutation, abnormal cytokine expression, bone marrow fibrosis, anemia, splenomegaly, extramedullary hematopoiesis (EMH), constitutional symptoms, cachexia, leukemic progression and shortened survival. DIAGNOSIS: Diagnosis is based on bone marrow morphology...
December 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27870288/effect-of-dietary-supplementation-with-clay-based-binders-on-biochemical-and-histopathological-changes-in-organs-of-turkey-fed-with-aflatoxin-contaminated-diets
#10
A O Lala, O L Ajayi, A O Oso, M O Ajao, O O Oni, N Okwelum, O M O Idowu
This study was carried out to investigate the effect of dietary supplementation with molecular or nano-clay binders on biochemical and histopathological examination of organs of turkeys fed diets contaminated with aflatoxin B1. Two hundred and sixteen unsexed 1-day-old British United Turkeys were randomly allotted to nine diets in a 3 × 3 factorial arrangement of diets supplemented with no toxin binder, molecular toxin binder (MTB) and nano-clay toxin binder, each contaminated with 0, 60 and 110 ppb aflatoxin B1 respectively...
December 2016: Journal of Animal Physiology and Animal Nutrition
https://www.readbyqxmd.com/read/27869648/hippo-signaling-interactions-with-wnt-%C3%AE-catenin-and-notch-signaling-repress-liver-tumorigenesis
#11
Wantae Kim, Sanjoy Kumar Khan, Jelena Gvozdenovic-Jeremic, Youngeun Kim, Jason Dahlman, Hanjun Kim, Ogyi Park, Tohru Ishitani, Eek-Hoon Jho, Bin Gao, Yingzi Yang
Malignant tumors develop through multiple steps of initiation and progression, and tumor initiation is of singular importance in tumor prevention, diagnosis, and treatment. However, the molecular mechanism whereby a signaling network of interacting pathways restrains proliferation in normal cells and prevents tumor initiation is still poorly understood. Here, we have reported that the Hippo, Wnt/β-catenin, and Notch pathways form an interacting network to maintain liver size and suppress hepatocellular carcinoma (HCC)...
November 21, 2016: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/27868197/expression-of-a-hepatitis-b-virus-pre-s2-deletion-mutant-in-the-liver-results-in-hepatomegaly-and-hepatocellular-carcinoma-in-mice
#12
Yuan-Chi Teng, Jenq Chyuan Neo, Jaw-Ching Wu, Yi-Fan Chen, Cheng-Heng Kao, Ting-Fen Tsai
Hepatocellular carcinoma (HCC) is the most common form of liver cancer and has a poor prognosis and a low survival rate; its incidence is on the rise. Hepatitis B virus (HBV) infection is one of the main causes of HCC. A high prevalence of pre-S deletions of HBV surface antigen, which encompass T-cell and/or B-cell epitopes, is found in HBV carriers; antiviral therapy and viral immune escape may cause and select for these HBV mutants. In particular, the presence of pre-S2 deletion mutants is an important risk factor associated with cirrhosis and HCC...
November 21, 2016: Journal of Pathology
https://www.readbyqxmd.com/read/27867168/clinical-experience-of-dual-phase-cone-beam-computed-tomography-during-hepatic-arteriography-to-apply-3d-dsa
#13
Yoshinori Takao, Akihiko Kakimi, Yutaka Katayama, Shohei Sasaki, Toshiyo Norimasa, Shinichiro Izuta, Daisuke Himoto, Takao Ichida
We report on the methods and experiences of the dual-phase cone beam computed tomography during hepatic arteriography (CBCTHA) to apply the 3D-DSA. A total of 32 ml contrast medium (150 mgI/ml) was injected at the rate of 2.0 ml/s for 16 s. The early phase scan was initiated 10 s after the start of contrast media injection. The delayed phase scan was started 40 s after that (24 s after the end of CM injection). When using the dual phase CBCTHA, it was able to obtain the classical hepatocellular carcinoma (HCC) images same as computed tomography during hepatic arteriography (CTHA)...
2016: Nihon Hoshasen Gijutsu Gakkai Zasshi
https://www.readbyqxmd.com/read/27864598/impact-of-fetal-counseling-on-outcome-of-antenatal-congenital-surgical-anomalies
#14
Shilpa Sharma, Ranjana Bhanot, Dipika Deka, Minu Bajpai, Devendra K Gupta
AIM: To analyze the impact of counseling on antenatal congenital surgical anomalies (ACSA). METHODS: Cases presenting with ACSA for fetal counseling and those presenting in post-natal period following diagnosis of ACSA (PACSA) for surgical opinion were analyzed for spectrum, presentation and outcome. RESULTS: 117 cases including ACSA(68);PACSA(49) were analyzed. Gestational age at diagnosis of ACSA;PACSA was 17-37;17-39 weeks (median 24;32 weeks)...
November 18, 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27858891/pediatric-visceral-leishmaniasis-in-northwest-of-iran
#15
Babak Abdinia, Mohammad Oliaei-Motlagh, Amir Teimouri-Dereshki
Leishmaniasis is one of the major health problems in Iran. Although the incidence of visceral leishmaniasis (VL) is reported almost everywhere, the northwestern Iran is one of the major endemic regions.To do this study, clinical, laboratory as well as disease characteristics of children admitted to Children Cure and Health Hospital, Tabriz University of Medical Sciences, were examined as the reference hospital for the treatment of VL in northwestern Iran.In this study, 156 children hospitalized in a pediatric hospital from 2000 to 2015 for VL were included...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27824644/rapid-fatal-acute-peripheral-t-cell-lymphoma-associated-with-igg-plasma-cell-leukemia-and-iga-hypergammaglobulinemia
#16
Nives Jonjić, Irena Seili Bekafigo, Dora Fučkar Čupić, Ksenija Lučin, Antica Duletić Načinović, Toni Valković
Simultaneous occurrence of T-cell and B-cell neoplasms is rare, and etiologic relationships between these 2 malignancies are poorly understood. We describe the case of a 66-year-old woman who was admitted to the hospital because of fever, hemoptysis, lymphadenopathy, and skin rash. Enlarged lymph nodes in axillary, pectoral, paratracheal, and periportal regions as well as slight hepatomegaly and splenomegaly were confirmed. A peripheral blood smear revealed rouleaux formation and numerous circulating plasma cells, with plasmacytoid lymphocytes...
November 2016: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/27823643/an-overview-of-hematopoietic-stem-cell-transplantation-related-thrombotic-complications
#17
REVIEW
Ugur Sahin, Pinar Ataca Atilla, Erden Atilla, Selami Kocak Toprak, Taner Demirer
Thrombotic episodes are far less common than bleeding complications after hematopoietic stem cell transplantation (HSCT). However, they lead to significant morbidity and mortality. These complications are classified into four groups, including venous thromboembolic events (VTE), catheter-induced thrombosis (CIT), transplant-associated thrombotic microangiopathy (TA-TMA) and sinusoidal obstruction syndrome (SOS) or veno-occlusive disease (VOD). The frequency of VTE is increased among patients undergoing HSCT due to some acquired conditions including underlying malignancy, infections, administration of myeloablative conditioning regimens and/or total body irradiation, prolonged hospitalizations leading to immobility and presence of central venous catheters...
November 2016: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/27821514/autoimmune-hemolytic-anemia-in-systemic-lupus-erythematosus-at-diagnosis-differences-between-pediatric-and-adult-patients
#18
N W S Gormezano, D Kern, O L Pereira, G C X Esteves, A M E Sallum, N E Aikawa, R M R Pereira, C A Silva, E Bonfá
OBJECTIVE: To determine the overall prevalence of autoimmune hemolytic anemia (AIHA), and to compare clinical and laboratory features in a large population of children and adult lupus patients at diagnosis. METHODS: This retrospective study evaluated the medical charts of 336 childhood-onset systemic lupus erythematosus (cSLE) and 1830 adult SLE (aSLE) patients followed in the same tertiary hospital. Demographic data, clinical features and disease activity were recorded...
November 7, 2016: Lupus
https://www.readbyqxmd.com/read/27818180/rac1-mediated-dna-damage-and-inflammation-promote-nf2-tumorigenesis-but-also-limit-cell-cycle-progression
#19
Yuhao Shi, Saumya R Bollam, Shannon M White, Sean Z Laughlin, Garrett T Graham, Mandheer Wadhwa, Hengye Chen, Chan Nguyen, Jeremie Vitte, Marco Giovannini, Jeffery Toretsky, Chunling Yi
Merlin encoded by the Nf2 gene is a bona fide tumor suppressor that has been implicated in regulation of both the Hippo-Yap and Rac1-Pak1 pathways. Using genetically engineered murine liver models, we show that co-deletion of Rac1 with Nf2 blocks tumor initiation but paradoxically exacerbates hepatomegaly induced by Nf2 loss, which can be suppressed either by treatment with pro-oxidants or by co-deletion of Yap. Our results suggest that while Yap acts as the central driver of proliferation during Nf2 tumorigenesis, Rac1 primarily functions as an inflammation switch by inducing reactive oxygen species that, on one hand, induce nuclear factor κB signaling and expression of inflammatory cytokines, and on the other activate p53 checkpoint and senescence programs dampening the cyclin D1-pRb-E2F1 pathway...
November 21, 2016: Developmental Cell
https://www.readbyqxmd.com/read/27805337/tuberous-sclerosis-with-disseminated-malignancy
#20
Achintya S, Ragesh R, Neeraj Nischal, Manish Soneja, Aanchal Kakkar, Mehar C Sharma, Geethu Anthony, Surabhi Vyas, S K SharmaSenior Professor And Head Department Of Medicine All India Institute Of Medical Sciences Ansari Nagar New Delhi
Tuberous sclerosis is characterized by typical skin and clinical manifestations with predilection to neoplasia. We describe the case of a 42-year old female who presented with a mass and pain in right lumbar region with constitutional symptoms and generalized body aches since last one year. She had adenoma sebaceum, subungual fibromas and hepatomegaly. CT chest, abdomen and MRI revealed mass in the right renal fossa with wide spread extensions in the abdomen, left renal angiomyolipomas (AMLs) and intracerebral lesions...
November 2016: Journal of the Association of Physicians of India
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