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autoimmune liver diseases

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https://www.readbyqxmd.com/read/28326205/shigella-sonnei-bacteremia-presenting-with-profound-hepatic-dysfunction
#1
Oluwaseun Shogbesan, Andrew Rettew, Bilal Shaikh, Abdullateef Abdulkareem, Anthony Donato
Worldwide, Shigellosis is a significant public health issue, associated with nearly one million deaths annually. About half a million cases of Shigella infection are reported annually in the United States. Shigella bacteremia is uncommon and generally seen in children and immunocompromised adults. We present a case of a Shigella sonnei bacteremia with marked hepatic derangement in a 27-year-old previously healthy homosexual male with history of Roux-en-Y gastric bypass, who presented to the emergency room with a 4-day history of loose watery stool, abdominal cramps, nausea and vomiting, and yellow skin of 2-day duration...
2017: Case Reports in Gastrointestinal Medicine
https://www.readbyqxmd.com/read/28324773/haptoglobin-is-frequently-low-in-patients-with-myelofibrosis-clinical-relevance
#2
Paolo Strati, Lucia Masarova, Prithviraj Bose, Naval Daver, Naveen Pemmaraju, Srdan Verstovsek
A recent study, showing the absence of paroxysmal nocturnal hemoglobinuria clones in myelofibrosis, has reopened the debate around the role of decreased haptoglobin in this disease. We present here a large prospective analysis of the clinical significance of low haptoglobin in 152 patients with myelofibrosis. Low haptoglobin (<32mg/dL) was observed in 50 patients (33%). Decreased haptoglobin did not associate with low hemoglobin levels, positive Coombs test or abnormal liver function tests, suggesting it is not result of autoimmune hemolytic anemia or liver cirrhosis...
March 8, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28321568/gastrointestinal-and-liver-lesions-in-primary-childhood-sj%C3%A3-gren-syndrome
#3
Yasuyo Kashiwagi, Tatsuro Hatsushika, Norito Tsutsumi, Soken Go, Shigeo Nishimata, Hisashi Kawashima
Sjögren syndrome (SS) is characterized by lymphocytic infiltration of exocrine glands, mainly the lacrimal and salivary glands, leading to keratoconjunctivitis sicca and xerostomia. SS is one of the most common autoimmune rheumatic diseases in adults; however, few cases of primary childhood SS with gastrointestinal and liver lesions have been reported in the literature. We report five cases of primary childhood SS with gastrointestinal and liver lesions. Multiple gastric biopsies in four cases revealed atrophic gastritis in the antrum of the stomach or chronic gastritis...
March 20, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28321390/metabonomics-research-progress-on-liver-diseases
#4
REVIEW
Mengqian Yu, Ying Zhu, Qingwei Cong, Chunyan Wu
Metabolomics as the new omics technique develops after genomics, transcriptomics, and proteomics and has rapid development at present. Liver diseases are worldwide public health problems. In China, chronic hepatitis B and its secondary diseases are the common liver diseases. They can be diagnosed by the combination of history, virology, liver function, and medical imaging. However, some patients seldom have relevant physical examination, so the diagnosis may be delayed. Many other liver diseases, such as drug-induced liver injury (DILI), alcoholic liver disease (ALD) and nonalcoholic fatty liver disease (NAFLD), and autoimmune liver diseases, still do not have definite diagnostic markers; the diagnosis consists of history, medical imaging, and the relevant score...
2017: Canadian Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28317620/lupus-hepatitis-and-autoimmune-hepatitis-lupoid-hepatitis
#5
REVIEW
Avinash Adiga, Kenneth Nugent
Liver dysfunction occurs in approximately 50% of patients with systemic lupus erythematosus (SLE), and patients with SLE and elevated liver enzymes can present a complicated and difficult differential diagnosis. Lupus hepatitis and autoimmune hepatitis are 2 immunologic conditions involving the liver, which can have similar clinical, laboratory and systemic presentations, leading to difficulties in diagnosis. Physicians need to be aware of these 2 hepatic diseases as diagnosis and appropriate therapy need to occur early in the disease course to prevent progression to advanced liver disease...
April 2017: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/28316856/giant-splenorenal-shunt-in-a-young-patient-with-autoimmune-hepatitis-primary-biliary-cholangitis-overlap-syndrome-and-portal-vein-thrombosis
#6
F Chegai, A U Cavallo, M Forcina, V Giuricin, F Castellani, L Greco, M Manuelli, T M Manzia, G Sergiacomi
We present a case of giant Splenorenal Shunt (SRS) associated with portal vein thrombosis in a 37-year-old woman with a twelve-year history of autoimmune hepatitis/primary biliary cholangitis overlap syndrome. At the moment of the CT examination laboratory tests showed creatinine 1.5 mg/dl, bilirubin 1.5 mg/dl, INR 3, and Na 145 mmol/l and the Model End-Stage Liver Disease score was 24. Extensive calcified thrombosis causing complete occlusion of the portal vein lumen and partially occluding the origin of the superior mesenteric vein was present and a small calcified thrombus in the Splenic Vein lumen was also evident...
2017: Case Reports in Radiology
https://www.readbyqxmd.com/read/28299346/serum-cytokine-levels-and-their-relation-to-clinical-features-in-patients-with-autoimmune-liver-diseases
#7
Dilyara Akberova, Andrei P Kiassov, Diana Abdulganieva
Serum cytokine levels were explored in a combined group of patients with autoimmune liver diseases (AILDs) and separately in patients with autoimmune hepatitis (AIH) and overlap syndrome. Overall, 60 patients with AILD, among them 32 patients with AIH and 28 patients with overlap syndrome, were included in the cross-sectional study. Serum cytokine levels were measured at baseline and compared to those of 21 healthy controls. Patients with AILD had significantly higher levels of IL-6 (0.70 (range 0.17-99.86) in patients with AILD compared to 0...
2017: Journal of Immunology Research
https://www.readbyqxmd.com/read/28297794/-research-advances-in-autoimmune-liver-diseases-in-2016
#8
B Li, Q X Wang, X Ma
Autoimmune liver diseases are a group of abnormal autoimmune-mediated inflammatory hepatobiliary injuries, mainly including autoimmune hepatitis(AIH), primary biliary cholangitis(PBC), and primary sclerosing cholangitis (PSC). The diagnosis and treatment of autoimmune liver diseases, an important type of non-viral liver disease, have become a prominent issue in hepatology. In 2016, many new advances have been achieved in the clinical and basic research on autoimmune liver diseases, including the phase 3 clinical trial of obeticholic acid, the proposal of UK-PBC risk score, and the research on gut microbiota associated with PSC...
February 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/28296758/the-incidence-and-clinical-characteristics-by-gender-differences-in-patients-with-kikuchi-fujimoto-disease
#9
In Young Jung, Hea Won Ann, Jung Ju Kim, Se Ju Lee, Jinnam Kim, Hye Seong, Dong Hyun Oh, Yong Chan Kim, Eun Jin Kim, Su Jin Jeong, Nam Su Ku, Jun Yong Choi, Young Goo Song, June Myung Kim
Kikuchi-Fujimoto disease (KFD) is a rare, self-limiting disorder that typically affects the cervical lymph nodes (LNs). Although initially described in young women, KFD also occurs in men. There are no reports on the clinical manifestations and characteristics of male KFD patients. Therefore, this study was conducted to assess the incidence of KFD among males, as well as the most frequent clinical characteristics of these patients. A retrospective, cross-sectional study was performed at a tertiary hospital of patients pathologically confirmed as having KFD from LN biopsy specimens...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28296656/severity-and-outcome-of-acute-on-chronic-liver-failure-is-dependent-on-the-etiology-of-acute-hepatic-insults-analysis-of-368-patients
#10
Shalimar, Saurabh Kedia, Soumya J Mahapatra, Baibaswata Nayak, Deepak Gunjan, Bhaskar Thakur, Subrat K Acharya
BACKGROUND: Acute-on-chronic liver failure (ACLF) may be precipitated by various hepatic insults. The present study evaluated the outcomes of ACLF with different acute insults. PATIENTS AND METHODS: A total of 368 ACLF patients were included. Data collected included etiologies of acute hepatic insult and underlying chronic liver disease, and organ failure. Model for end-stage liver disease (MELD), chronic liver failure consortium (CLIF)-C ACLF, and acute physiology and chronic health evaluation (APACHE) II scores were calculated...
March 14, 2017: Journal of Clinical Gastroenterology
https://www.readbyqxmd.com/read/28294314/generation-of-precursor-immature-and-mature-murine-b1-cell-lines-from-c-myc-bcl-xl-overexpressing-pre-bi-cells
#11
Inge Wolf, Corinne Bouquet, Friederike Naumann, Fritz Melchers
Deregulated expression of c-myc and bcl-xL is long known to generate transformed B cells in humans and mice. We overexpressed these genes to induce in vitro and in vivo differentiation of fetal liver-derived mouse pre-BI cells to B1-lineage pre-BII-like, immature and mature B-cell lines and to Ig-secreting cells. In vitro, doxycycline-controlled c-myc/bcl-xL-overexpressing CD19(+) CD93(+) c-kikt(+) IgM(-) pre-BI cells differentiate to and survive as CD19(+) CD93(+) c-kit(-) IgM(+) immature B1 cells. Timed CpG-stimulation of these oncogene-overexpressing pre-B or immature B1 cells generates either CD19(+) CD93(low) c-kit(-) IgM(-) SLC(-) pre-BII-like, or IgM(+) MHCII(+) CD73(+) CD80(+) CD40(+) mature B1-cell lines and IgM-secreting B1 cells in vitro and fixes their state of differentiation...
March 10, 2017: European Journal of Immunology
https://www.readbyqxmd.com/read/28293081/autoantibody-profiles-in-autoimmune-hepatitis-and-chronic-hepatitis-c-identifies-similarities-in-patients-with-severe-disease
#12
Kawa Amin, Aram H Rasool, Ali Hattem, Taha Am Al-Karboly, Taher E Taher, Jonas Bystrom
AIM: To determine how the auto-antibodies (Abs) profiles overlap in chronic hepatitis C infection (CHC) and autoimmune hepatitis (AIH) and correlate to liver disease. METHODS: Levels of antinuclear Ab, smooth muscle antibody (SMA) and liver/kidney microsomal-1 (LKM-1) Ab and markers of liver damage were determined in the sera of 50 patients with CHC infection, 20 AIH patients and 20 healthy controls using enzyme linked immunosorbent assay and other immune assays...
February 28, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28293027/genetics-of-primary-sclerosing-cholangitis-and-pathophysiological-implications
#13
REVIEW
Xiaojun Jiang, Tom H Karlsen
Primary sclerosing cholangitis (PSC) is a chronic disease leading to fibrotic scarring of the intrahepatic and extrahepatic bile ducts, causing considerable morbidity and mortality via the development of cholestatic liver cirrhosis, concurrent IBD and a high risk of bile duct cancer. Expectations have been high that genetic studies would determine key factors in PSC pathogenesis to support the development of effective medical therapies. Through the application of genome-wide association studies, a large number of disease susceptibility genes have been identified...
March 15, 2017: Nature Reviews. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28292360/acute-on-chronic-liver-failure-causes-clinical-characteristics-and-predictors-of-mortality
#14
Abbas Ali Tasneem, Nasir Hassan Luck
OBJECTIVE: To determine the causes, characteristics and predictors of mortality in patients with acute-on-chronic liver failure (ACLF). STUDY DESIGN: Cross-sectional study. PLACE AND DURATION OF STUDY: Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, from July 2014 to June 2016. METHODOLOGY: All patients with acute-on-chronic liver disease (ACLD) with ages > 12 were included...
January 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28292174/extrahepatic-manifestations-of-primary-biliary-cholangitis
#15
REVIEW
Sara L Chalifoux, Peter G Konyn, Gina Choi, Sammy Saab
Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by progressive destruction of the intrahepatic bile ducts, leading to cholestasis. PBC is known to have both hepatic and extrahepatic manifestations. Extrahepatic manifestations are seen in up to 73% of patients with PBC, with the most common being Sjogren's syndrome, thyroid dysfunction and systemic sclerosis. It is thought that patients with PBC are at increased risk of developing these extrahepatic manifestations, almost all of which are autoimmune, because patients with autoimmune disease are at higher risk of developing another autoimmune condition...
March 16, 2017: Gut and Liver
https://www.readbyqxmd.com/read/28287856/enzymatic-kinetics-regarding-reversible-metabolism-of-cs-0777-a-sphingosine-1-phosphate-receptor-modulator-via-phosphorylation-and-dephosphorylation-in-humans
#16
Shin-Ichi Inaba, Maki Yamaguchi-Goto, Kaoru Tanaka-Takanaka, Kiyoaki Yonesu, Hidetaka Sakurai, Kazuishi Kubota, Takashi Izumi
1. CS-0777, a candidate compound for autoimmune diseases, becomes phosphorylated active metabolite, M1, by fructosamine 3-kinase (FN3K), FN3K-related protein (FN3K-RP); and M1 reverted back to CS-0777 by alkaline phosphatase (ALP) in the body. We performed enzyme kinetic analysis of phosphorylation of CS-0777 by FN3K, FN3K-RP, human erythrocytes and human platelets; and dephosphorylation of M1 by various ALP isozymes and human liver, kidney, lung and small intestine microsomes. 2. The Michaelis constants of human FN3K, FN3K-RP, and erythrocytes for CS-0777 phosphorylation were in the range from 498 μM to 1060 μM...
March 13, 2017: Xenobiotica; the Fate of Foreign Compounds in Biological Systems
https://www.readbyqxmd.com/read/28281846/autoimmune-liver-diseases-and-inflammatory-bowel-diseases-in-children-current-issues-and-future-perspectives
#17
Sabrina Cardile, Tommaso Alterio, Manila Candusso, Andrea Pietrobattista, Daniela Liccardo, Maria Sole Basso, Bronislava Papadatou, Fiammetta Bracci, Daniela Knafelz, Giuliano Torre
Inflammatory bowel diseases (IBDs) represent a group of intestinal disorders with a chronic and relapsing inflammation of the gut, and with a potential risk of systemic involvement of other organs and systems. Over the pediatric age, an incidence higher than 20% of developing extraintestinal manifestation during follow-up has been reported. The liver and the biliary system are frequently involved, and primary sclerosing cholangitis (PSC) is the most predominant entity with an incidence rate of 6.4-7.8% in children...
March 10, 2017: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/28280376/single-donor-islet-transplantation-in-type-1-diabetes-patient-selection-and-special-considerations
#18
REVIEW
Jacob A Tatum, Max O Meneveau, Kenneth L Brayman
Type 1 diabetes mellitus is an autoimmune disorder of the endocrine pancreas that currently affects millions of people in the United States. Although the disease can be managed with exogenous insulin administration, the ultimate cure for the condition lies in restoring a patient's ability to produce their own insulin. Islet cell allotransplantation provides a means of endogenous insulin production. Though far from perfected, islet transplants are now a proven treatment for type 1 diabetics. However, proper patient selection is critical for achieving optimal outcomes...
2017: Diabetes, Metabolic Syndrome and Obesity: Targets and Therapy
https://www.readbyqxmd.com/read/28276834/drug-induced-liver-injury-due-to-antibiotics
#19
Einar S Björnsson
Drug-induced liver injury (DILI) is an important differential diagnosis in patients with abnormal liver tests and normal hepatobiliary imaging. Of all known liver diseases, the diagnosis of DILI is probably one of the most difficult one to be established. In all major studies on DILI, antibiotics are the most common type of drugs that have been reported. The clinical phenotype of different types of antibiotics associated with liver injury is highly variable. Some widely used antibiotics such as amoxicillin-clavulanate have been shown to have a delayed onset on liver injury and recently cefazolin has been found to lead to liver injury 1-3 weeks after exposure of a single infusion...
February 20, 2017: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/28273779/concurrent-autoimmune-hepatitis-in-multiple-sclerosis
#20
Gonçalo Cação, Ernestina Santos, Ana Martins Silva
BACKGROUND: Autoimmune hepatitis (AIH) is a rare and chronic inflammatory disorder associated with extrahepatic autoimmune diseases, including, infrequently, multiple sclerosis (MS). Short Reports: We report five cases of MS and AIH association. One patient developed AIH while under interferon beta-1b and the remaining while off disease-modifying therapy, although after methylprednisolone bolus in three. All presented a liver biopsy compatible with AIH. Hepatitis resolution was achieved with immunosuppressive treatment, but one patient died after a fulminant hepatitis requiring liver transplant...
February 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
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