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Hepatomegaly

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https://www.readbyqxmd.com/read/28437835/pten-downregulation-promotes-%C3%AE-oxidation-to-fuel-hypertrophic-liver-growth-after-hepatectomy-in-mice
#1
Ekaterina Kachaylo, Christoph Tschuor, Nicolas Calo, Nathalie Borgeaud, Perparim Limani, Anne-Christine Piguet, Jean-Francois Dufour, Michelangelo Foti, Rolf Graf, Pierre A Clavien, Bostjan Humar
In regenerating liver, hepatocytes accumulate lipids before the major wave of parenchymal growth. This transient, regeneration-associated steatosis (TRAS) is required for liver recovery, but its purpose is unclear. The tumor suppressor PTEN is a key inhibitor of the AKT-mTOR axis that regulates growth and metabolic adaptations after hepatectomy. In quiescent liver, PTEN causes pathological steatosis when lost, while its role in regenerating liver remains unknown. Here, we show that PTEN downregulation promotes liver growth in a TRAS-dependent way...
April 24, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28427965/t1-st2-deficient-mice-display-protection-against-leishmania-infantum-experimental-infection
#2
Khalid Eltahir Khalid, Manuela Sales, Laís Sacramento, Diego L Costa, Djalma de Souza, Vanessa Carregaro, Joao Santana da Silva
T1/ST2 is a surface marker selectively expressed on type 2 helper (TH2) effector cells. As Leishmania infection in susceptible BALB/c mice have ascribed to a polarized TH2 response, this study aim to investigate the T1/ST2 (the receptor for IL-33), as a typical TH2 marker in the postulation that a shift towards a beneficial TH1 response would occur in the absence of ST2. For this, ST2 knockout (ST2(-/-)) and WT BALB/c mice were experimentally infected in the retro-orbital sinus with L. infantum. We showed that ST2(-/-) animals displayed better control of parasite burden in both spleen and liver tissues at different time points of chronic phases, and reduced spleenomegaly and hepatomegaly compared with the wild-type (WT) mice...
April 17, 2017: Acta Tropica
https://www.readbyqxmd.com/read/28423235/hepatic-sinusoidal-obstruction-syndrome-during-maintenance-therapy-of-childhood-acute-lymphoblastic-leukemia-is-associated-with-continuous-asparaginase-therapy-and-mercaptopurine-metabolites
#3
Linea Natalie Toksvang, Silvia De Pietri, Stine N Nielsen, Jacob Nersting, Birgitte K Albertsen, Peder S Wehner, Steen Rosthøj, Päivi M Lähteenmäki, Daniel Nilsson, Tove A Nystad, Kathrine Grell, Thomas L Frandsen, Kjeld Schmiegelow
BACKGROUND: Hepatic sinusoidal obstruction syndrome (SOS) during treatment of childhood acute lymphoblastic leukemia (ALL) has mainly been associated with 6-thioguanine. The occurrence of several SOS cases after the introduction of extended pegylated asparaginase (PEG-asparaginase) therapy in the Nordic Society of Paediatric Haematology and Oncology (NOPHO) ALL2008 protocol led us to hypothesize that PEG-asparaginase, combined with other drugs, may trigger SOS during 6-thioguanine-free maintenance therapy...
April 19, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28422749/obesity-induced-hepatic-steatosis-is-mediated-by-endoplasmic-reticulum-stress-in-the-subfornical-organ-of-the-brain
#4
Julie A Horwath, Chansol Hurr, Scott D Butler, Mallikarjun Guruju, Martin D Cassell, Allyn L Mark, Robin L Davisson, Colin N Young
Nonalcoholic fatty liver disease (NAFLD), characterized by an excess accumulation of hepatic triglycerides, is a growing health epidemic. While ER stress in the liver has been implicated in the development of NAFLD, the role of brain ER stress - which is emerging as a key contributor to a number of chronic diseases including obesity - in NAFLD remains unclear. These studies reveal that chemical induction of ER stress in the brain caused hepatomegaly and hepatic steatosis in mice. Conversely, pharmacological reductions in brain ER stress in diet-induced obese mice rescued NAFLD independent of body weight, food intake, and adiposity...
April 20, 2017: JCI Insight
https://www.readbyqxmd.com/read/28421832/experimental-induction-of-chicken-aa-amyloidosis-in-white-layer-chickens-by-inoculation-with-inactivated-vaccines
#5
Wazir Ahmad Habibi, Takuya Hirai, Mohammad Hakim Niazmand, Naoko Okumura, Ryoji Yamaguchi
We investigated the amyloidogenic potential of inactivated vaccines and the localized production of serum amyloid A (SAA) at the injection site in white layer chickens. Hens in the treated group were injected intramuscularly three times with high doses of inactivated oil-emulsion Salmonella Enteritidis vaccine and multivalent viral and bacterial inactivated oil-emulsion vaccines at two week intervals. Chickens in the control group did not receive any inoculum. In the treated group, emaciation, and granulomas were present, while several chickens died between 4-6 weeks after the first injection...
April 19, 2017: Avian Pathology: Journal of the W.V.P.A
https://www.readbyqxmd.com/read/28416659/deubiquitinase-yod1-potentiates-yap-taz-activities-through-enhancing-itch-stability
#6
Youngeun Kim, Wantae Kim, Yonghee Song, Jeong-Rae Kim, Kyungjoo Cho, Hyuk Moon, Simon Weonsang Ro, Eunjeong Seo, Yeon-Mi Ryu, Seung-Jae Myung, Eek-Hoon Jho
Hippo signaling controls the expression of genes regulating cell proliferation and survival and organ size. The regulation of core components in the Hippo pathway by phosphorylation has been extensively investigated, but the roles of ubiquitination-deubiquitination processes are largely unknown. To identify deubiquitinase(s) that regulates Hippo signaling, we performed unbiased siRNA screening and found that YOD1 controls biological responses mediated by YAP/TAZ. Mechanistically, YOD1 deubiquitinates ITCH, an E3 ligase of LATS, and enhances the stability of ITCH, which leads to reduced levels of LATS and a subsequent increase in the YAP/TAZ level...
April 17, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28416007/disseminated-alveolar-echinococcosis-resembling-metastatic-malignancy-a-case-report
#7
Laura Caire Nail, Ezequiel Rodríguez Reimundes, Christelle Weibel Galluzzo, Dan Lebowitz, Yasmine Lucile Ibrahim, Johannes Alexander Lobrinus, François Chappuis
BACKGROUND: Alveolar echinococcosis is a potentially lethal zoonosis caused by larval forms of the tapeworm Echinococcus multilocularis. Humans are aberrant intermediate hosts who become infected by ingestion of egg-contaminated food or water or via physical contact with domestic or wild animals that carry the parasite in their small intestine. In humans, the disease usually affects the liver and can spread to other organs causing metastatic infiltration. In this report, we describe an advanced presentation of human alveolar echinococcosis mimicking metastatic malignancy...
April 18, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28411097/-acute-liver-failure-related-to-inherited-metabolic-diseases-in-young-children
#8
Filipa Dias Costa, Rita Moinho, Sandra Ferreira, Paula Garcia, Luísa Diogo, Isabel Gonçalves, Carla Pinto
INTRODUCTION: Pediatric acute liver failure (ALF) due to inherited metabolic diseases (IMD) is a rare life-threatening condition with a poor prognosis. Early intervention may be lifesaving. OBJECTIVE: To describe clinical presentation, investigation and outcomes of ALF related to IMD in young children. MATERIAL AND METHODS: Retrospective review of the medical records of children aged up to 24 months, admitted to a tertiary pediatric and neonatal Intensive Care Unit during a 27-year period, fulfilling the ALF criteria, with documented metabolic etiology...
April 11, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/28396719/case-of-hepatocellular-carcinoma-in-a-patient-with-hereditary-tyrosinemia-in-the-post-newborn-screening-era
#9
Essam M Imseis, John S Bynon, Chad Thornhill
Hereditary tyrosinemia type 1 (HT-1) is a metabolic disorder caused by a defect in tyrosine degradation. Without treatment, symptoms of hepatomegaly, renal tubular dysfunction, growth failure, neurologic crises resembling porphyrias, rickets and possible hepatocellular carcinoma can develop. The use of 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione and early diagnosis through newborn screening initiatives have resulted in a sharp decline in morbidity and mortality associated with this disease. We present a case report of a 7-year-old patient with HT-1 who was born prior to the addition of tyrosinemia to the newborn screening in her birth area...
March 28, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28388738/riboflavin-responsive-multiple-acyl-coa-dehydrogenase-deficiency-associated-with-hepatoencephalomyopathy-and-white-matter-signal-abnormalities-on-brain-mri
#10
Päivi Vieira, Päivi Myllynen, Marja Perhomaa, Hannu Tuominen, Riikka Keski-Filppula, Seppo Rytky, Leila Risteli, Johanna Uusimaa
Multiple acyl-CoA dehydrogenase deficiency (MADD) is a rare inborn error of metabolism affecting both fatty acid and amino acid oxidation. It can manifest at any age, but riboflavin-responsiveness has mainly been described in less severely affected patients. We describe an infant with severe MADD presenting with profound hypotonia and hepatomegaly. Treatment with riboflavin improved his muscle strength, liver size, and biochemical markers. A homozygous mutation of electron transfer flavoprotein dehydrogenase (ETFDH) was found...
April 7, 2017: Neuropediatrics
https://www.readbyqxmd.com/read/28386144/self-healing-juvenile-cutaneous-mucinosis
#11
Beata Kołodziejczyk, Agnieszka Gazda, Elżbieta Hernik, Izabela Szczygielska, Lidia Rutkowska-Sak, Marta Legatowicz Koprowska
Girl, aged 4 years old, began the disease with pain of the lower extremities, fever up to 38°C and signs of upper airway infection. Then the patient developed oedema and redness of the whole face, thickened skin, subcutaneous nodular foldings of the frontal, occipital, cervical and axillary regions, extensor areas of the joints; fine, hard whitish nodules in the frontal region and over interphalangeal joints of the hands, pruritus; oedemas of the ankles, knees and joints of the hands, cervical lymphadenopathy and hepatomegaly...
2017: Reumatologia
https://www.readbyqxmd.com/read/28375199/stevens-johnson-syndrome-and-toxic-epidermal-necrolysis-in-childhood-onset-systemic-lupus-erythematosus-patients-a-multicenter-study
#12
Ana Paula Sakamoto, Clovis Artur Silva, Claudia Saad-Magalhães, Aline Nicácio Alencar, Rosa Maria Rodrigues Pereira, Katia Kozu, Cassia Maria Passarelli Lupoli Barbosa, Maria Teresa Terreri
OBJECTIVE: To assess Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in a large population of childhood-onset systemic lupus erythematosus (cSLE) patients. METHODS: Multicenter study including 852 cSLE patients followed in Pediatric Rheumatology centers in São Paulo, Brazil. SJS was defined as epidermal detachment below 10% of body surface area (BSA), overlap SJS-TEN 10-30% and TEN greater than 30% of BSA. RESULTS: SJS and TEN was observed in 5/852 (0...
March 26, 2017: Acta Reumatológica Portuguesa
https://www.readbyqxmd.com/read/28374935/prenatal-sonographic-findings-in-a-case-of-wolman-s-disease
#13
Matthew J Blitz, Burton Rochelson, Monica Sood, Martin G Bialer, Nidhi Vohra
No published case of Wolman's disease has described the prenatal sonographic findings. We present a case in which a third-trimester sonographic examination demonstrated fetal hepatomegaly and bilateral adrenal echogenicity suggestive of diffuse calcification. Wolman's disease, also known as lysosomal acid lipase (LIPA) deficiency, is a rare autosomal-recessive disorder characterized by complete absence of the LIPA enzyme. The diagnosis of Wolman's disease was made postnatally by biochemical testing, which indicated absence of LIPA enzyme activity and gene sequencing, which confirmed homozygosity for the G66V mutation within the LIPA gene...
April 4, 2017: Journal of Clinical Ultrasound: JCU
https://www.readbyqxmd.com/read/28367431/a-3-year-old-girl-with-recurrent-infections-and-autoimmunity-due-to-a-stat1-gain-of-function-mutation-the-expanding-clinical-presentation-of-primary-immunodeficiencies
#14
Juan Carlos Aldave Becerra, Enrique Cachay Rojas
We report a 3-year-old Peruvian girl, born to non-consanguineous parents. At the age of 8 months, she had a severe pneumonia complicated with empyema that required thoracic drainage and mechanical ventilation. Although no microorganisms were isolated, the patient recovered with broad-spectrum antibiotics. Since that date, she has presented multiple episodes of pneumonia and recurrent episodes of bronchospasm. At 1 year 5 months of age, the patient began with recurrent episodes of oropharyngeal, vaginal, and skin candidiasis, which improved transiently after using oral azole drugs...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28327053/predictive-parameters-for-imatinib-failure-in-patients-with-chronic-myeloid-leukemia
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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