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autoimmune liver diseases

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https://www.readbyqxmd.com/read/28230715/a-multireferral-centre-retrospective-cohort-analysis-on-the-experience-in-treatment-of-metastatic-uveal-melanoma-and-utilization-of-sequential-liver-directed-treatment-and-immunotherapy
#1
Malinda Itchins, Paolo A Ascierto, Alexander M Menzies, Meredith Oatley, Serigne Lo, Dariush Douraghi-Zadeh, Timmothy Harrington, Richard Maher, Antonio M Grimaldi, Alexander Guminski
Metastatic uveal melanoma is a rare malignancy with a poor prognosis. To date, systemic therapy has been ineffective; however, there are few data on the benefits of anti-CTLA4 or anti-PD-1 antibodies in sequence with liver-directed therapy. A retrospective cohort analysis was carried out on 37 consecutive patients managed in a tertiary referral centre examining the safety and efficacy of treatment; patterns of care; and impact on survival. The sequential treatment with transarterial chemotherapy (TAC), systemic immunotherapy (IT) and systemic chemotherapy was reviewed...
February 22, 2017: Melanoma Research
https://www.readbyqxmd.com/read/28229097/pediatric-hypovitaminosis-d-molecular-perspectives-and-clinical-implications
#2
Rafiu Ariganjoye
Vitamin D, a secosteroid, is essential for the development and maintenance of healthy bone in both the adult and pediatric populations. Low level of 25-hydroxy vitamin D (25-(OH)-D) is highly prevalent in children worldwide and has been linked to various adverse health outcomes including rickets, osteomalacia, osteomalacic myopathy, sarcopenia, and weakness, growth retardation, hypocalcemia, seizure and tetany, autism, cardiovascular diseases, diabetes mellitus, cancers (prostate, colon, breast), infectious diseases (viral, tuberculosis), and autoimmune diseases, such as multiple sclerosis and Hashimoto's thyroiditis...
2017: Global Pediatric Health
https://www.readbyqxmd.com/read/28225953/drug-reaction-with-eosinophilia-and-systemic-symptoms-dress-and-its-relation-with-autoimmunity-in-a-reference-center-in-mexico
#3
Juan Manuel Ruiz Matta, Silvia Méndez Flores, Judith Domínguez Cherit
BACKGROUND: Drug reaction with eosinophilia and systemic symptoms is a severe adverse drug reaction, with a reported mortality of 10%. Long-term outcomes involve organic failure and autoimmune diseases in some populations. OBJECTIVE: To evaluate the clinical prognosis of patients with drug reaction with eosinophilia and systemic symptoms. METHODS: We conducted a retrospective review at a referral hospital in Mexico City in a period of 22 years (1992-2013), looking up for records with diagnosis of DRESS according to RegiSCAR criteria...
January 2017: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/28225291/-a-contribution-to-the-differential-diagnostics-of-sclerosing-cholangitides
#4
Martin Blaho, Petr Dítě, Martina Bojková, Martin Rydlo, Tomáš Kupka, Pavel Svoboda, Pavel Klvaňa, Arnošt Martínek
Sclerosing cholangitides represent a group of chronic biliary obstructive diseases which include primary sclerosing cholangitis (PSC), IgG4 associated sclerosing cholangitis (IgG4-SC) and secondary sclerosing cholangitis (SSC). The manifestations of the diseases are similar, but their asymptomatic course is also frequent. IgG4-SC belongs to the group of IgG4 associated diseases and it is the most frequently related to type 1 autoimmune pancreatitis. Diagnosing of IgG4-SC is based on typical histopathological images, shape changes revealed by diagnostic imaging, serological tests, concurrent impairment of other organs and response to therapy, where IgG4-SC responds well to treatment with corticoids, whereas the only possibility for the remaining units is endoscopic intervention or liver transplantation...
2017: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/28223730/magnetic-resonance-elastography-is-accurate-in-detecting-advanced-fibrosis-in-autoimmune-hepatitis
#5
Jin Wang, Neera Malik, Meng Yin, Thomas C Smyrk, Albert J Czaja, Richard L Ehman, Sudhakar K Venkatesh
AIM: To assess the value of magnetic resonance elastography (MRE) in detecting advanced fibrosis/cirrhosis in autoimmune hepatitis (AIH). METHODS: In this retrospective study, 36 patients (19 treated and 17 untreated) with histologically confirmed AIH and liver biopsy performed within 3 mo of MRE were identified at a tertiary care referral center. Liver stiffness (LS) with MRE was calculated by a radiologist, and inflammation grade and fibrosis stage in liver biopsy was assessed by a pathologist in a blinded fashion...
February 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28223722/to-screen-or-not-to-screen-celiac-antibodies-in-liver-diseases
#6
REVIEW
Janaína Luz Narciso-Schiavon, Leonardo Lucca Schiavon
Celiac disease (CD) is a systemic immune-mediated disorder triggered by dietary gluten in genetically predisposed individuals. The typical symptoms are anemia, diarrhea, fatigue, weight loss, and abdominal pain. CD has been reported in patients with primary sclerosing cholangitis, primary biliary cholangitis, autoimmune hepatitis, aminotransferase elevations, nonalcoholic fatty liver disease, hepatitis B, hepatitis C, portal hypertension and liver cirrhosis. We evaluate recommendations for active screening for CD in patients with liver diseases, and the effect of a gluten-free diet in these different settings...
February 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28222032/unexplained-cyanosis-caused-by-hepatopulmonary-syndrome-in-a-girl-with-apeced-syndrome
#7
Fatih Celmeli, Abdullah Kocabas, Ishak A Isik, Mesut Parlak, Kai Kisand, Serdar Ceylaner, Doga Turkkahraman
Autoimmune polyendocrinopathy, candidiasis and ectodermal dystrophy (APECED) is a rare but devastating primary immunodeficiency disease caused by loss-of-function mutations in autoimmune regulator (AIRE) gene on chromosome 21q22.3. The clinical spectrum of the disease is characterized by a wide heterogeneity because of autoimmune reactions toward different endocrine and non-endocrine organs. Here, we report a 17-year-old Turkish girl diagnosed with APECED at 9 years in whom a novel homozygote mutation in AIRE gene p...
February 21, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28217916/effects-of-allogeneic-mesenchymal-stem-cell-transplantation-in-the-treatment-of-liver-cirrhosis-caused-by-autoimmune-diseases
#8
Jun Liang, Huayong Zhang, Cheng Zhao, Dandan Wang, Xiaolei Ma, Shengnan Zhao, Shiying Wang, Lingying Niu, Lingyun Sun
AIM: There has been great interest in recent years to take advantage of mesenchymal stem cells (MSCs) to treat end-stage liver disease. This study is aimed to evaluate clinical therapeutic effects of allogeneic MSC transplantation in liver cirrhosis caused by autoimmune diseases. METHODS: The enrolled patients with liver cirrhosis were assigned to receive allogeneic MSC infusions through a peripheral vein. The primary objective of this study was to assess the safety and effectiveness of MSCT in patients with autoimmune diseases-induced cirrhosis...
February 20, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28217252/primary-mucosa-associated-lymphoid-tissue-lymphoma-of-the-liver-a-report-of-two-cases-and-review-of-the-literature
#9
Ifeyinwa E Obiorah, Lynt Johnson, Metin Ozdemirli
Mucosa-associated lymphoid tissue (MALT) lymphoma of the liver is a very rare condition and thus the diagnosis may be challenging. The clinical presentation is usually variable, ranging from minimal clinical symptoms to severe end stage liver disease. In this paper, we describe the clinicopathologic findings in two cases of primary hepatic MALT lymphoma. One case is an 80-year-old female with no underlying chronic liver disease and the second case is a 30-year-old female with autoimmune hepatitis complicated by MALT lymphoma...
January 28, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28207511/autoimmune-hepatitis-in-patients-with-human-immunodeficiency-virus-hiv-case-reports-of-a-rare-but-important-diagnosis-with-therapeutic-implications
#10
Leila Kia, Adam Beattie, Richard M Green
RATIONALE: Chronic liver disease is a major cause of morbidity and mortality in patients with HIV. However, autoimmune hepatitis (AIH) in patients with HIV has rarely been reported. Our aim was to evaluate a cohort of patients with HIV and AIH and identify clinical presentations and outcomes. PATIENT CONCERNS: Management of autoimmune hepatitis in context of human immunodeficiency virus, long-term outcomes, and safety in setting of underlying immunocompromised state...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28203580/unusual-presentation-of-diffuse-large-b-cell-lymphoma-with-splenic-infarcts
#11
Vivek Kumar, Parita Soni, Vishangi Dave, Jonathan Harris
A 67-year-old man presented with a 3-day history of abdominal pain, fever, and significant weight loss over 2 months. Physical examination revealed left upper quadrant tenderness, hepatomegaly, splenomegaly, and bilateral pitting edema but peripheral lymphadenopathy was absent. Laboratory tests showed anemia, thrombocytopenia, elevated prothrombin time (PT), partial thromboplastin time (PTT), and increased lactate dehydrogenase (LDH). PTT was corrected completely in mixing study. Further workup for the cause of coagulopathy revealed decreased levels of all clotting factors except factor VIII and increase fibrinogen levels, which ruled out disseminated intravascular coagulation (DIC)...
January 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28199778/a-national-retrospective-study-of-peld-as-a-predictor-of-change-to-second-line-therapy-in-children-with-autoimmune-hepatitis
#12
Andréanne N Zizzo, Carolina Jimenez-Rivera, Joseph Kim, Richard A Schreiber, Simon C Ling, Jason Yap, Jeff Critch, Najma Ahmed, Fernando Alvarez, Binita M Kamath
BACKGROUND & AIMS: Adult studies of autoimmune hepatitis (AIH) have shown that the model of end-stage liver disease (MELD) is associated with resistance to first-line treatment. Using a multi-centre retrospective database, we sought to determine if the pediatric end-stage liver disease (PELD) score would similarly predict treatment resistance in pediatric AIH. METHODS: One hundred and seventy-one children from 13 Canadian centres who fulfilled the International Autoimmune Hepatitis Group (IAIHG) criteria were included and assessed for change to second-line therapy within 24 months of primary treatment onset...
February 15, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28197333/a-case-of-primary-biliary-cirrhosis-mimicking-acute-hepatitis-b-in-the-clinic-republic-of-korea
#13
Woo Hyuk Kwon, Hong Min Park, Jeong Jun Park, Sung Hoon Lee, Yong Kyu Lee
Primary biliary cirrhosis (PBC) is a slowly progressive cholestatic autoimmune liver disease characterized by progressive bile duct injury. The most common symptoms of this disease include fatigue and pruritus. The diagnosis of PBC is based on cholestatic biochemical liver tests, presence of antimitochondrial antibodies, and characteristic histological biopsy findings. We report a case of a patient with PBS, who was initially suspected to be in the window period of hepatitis B by a private doctor in a local clinic based on the detection of isolated immunoglobulin M antibody against hepatitis B core antigen...
January 2017: Korean Journal of Family Medicine
https://www.readbyqxmd.com/read/28192452/increased-risk-of-dementia-in-patients-hospitalized-with-acute-kidney-injury-a-nationwide-population-based-cohort-study
#14
Hsin-Hsi Tsai, Ruoh-Fang Yen, Cheng-Li Lin, Chia-Hung Kao
PURPOSE: To determine whether acute kidney injury (AKI) is a risk factor for dementia. METHODS: This nationwide population-based cohort study was based on data from the Taiwan National Health Insurance Research Database for 2000-2011. The incidence and relative risk of dementia were assessed in 207788 patients hospitalized for AKI. The comparison control was selected using the propensity score based on age, sex, index year and comorbidities. RESULTS: During the 12-year follow-up, patients with AKI had a significantly higher incidence for developing dementia than did the controls (8...
2017: PloS One
https://www.readbyqxmd.com/read/28192189/mir-425-regulates-inflammatory-cytokine-production-in-cd4-t-cells-via-n-ras-upregulation-in-primary-biliary-cholangitis
#15
Ryo Nakagawa, Ryosuke Muroyama, Chisato Saeki, Kaku Goto, Yoshimi Kaise, Kazuhiko Koike, Masanori Nakano, Yasuo Matsubara, Keiko Takano, Sayaka Ito, Masayuki Saruta, Naoya Kato, Mikio Zeniya
BACKGROUND & AIM: Primary biliary cholangitis (PBC) is an autoimmune liver disease of unknown pathogenesis. Consequently, therapeutic targets for PBC have yet to be identified. Because CD4(+) T cells play a pivotal role in immunological dysfunction observed in PBC, we analysed microRNA (miRNA) and mRNA expression in CD4(+) T cells to investigate PBC pathogenesis and identify novel therapeutic targets. METHODS: We performed integral miRNA and mRNA analysis of 14 PBC patients and 10 healthy controls using microarray and quantitative real time polymerase chain reaction (qRT-PCR) with gene set enrichment analysis...
February 9, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28191014/extrahepatic-autoimmune-diseases-in-patients-with-autoimmune-liver-diseases-a-phenomenon-neglected-by-gastroenterologists
#16
REVIEW
Liping Guo, Lu Zhou, Na Zhang, Baoru Deng, Bangmao Wang
Autoimmune liver diseases (AILDs) often coexist with other extrahepatic autoimmune diseases (EHAIDs). The spectrum of EHAIDs in patients with AILDs is similar, whereas the incidence is different. Notably, autoimmune thyroid disease and Sjogren's syndrome are the most common EHAIDs. Associated extrahepatic diseases may predate the appearance of AILDs or coincide with their onset. More frequently, they may appear during the course and even occur years after the diagnosis of AILDs. Importantly, associated EHAIDs may influence the natural course and prognosis of AILDs...
2017: Gastroenterology Research and Practice
https://www.readbyqxmd.com/read/28184367/novel-immunotherapies-for-autoimmune-hepatitis
#17
REVIEW
Shamir Cassim, Marc Bilodeau, Catherine Vincent, Pascal Lapierre
Autoimmune hepatitis (AIH) is a multifactorial autoimmune disease of unknown pathogenesis, characterized by a loss of immunological tolerance against liver autoantigens resulting in the progressive destruction of the hepatic parenchyma. Current treatments are based on non-specific immunosuppressive drugs. Although tremendous progress has been made using specific biological agents in other inflammatory diseases, progress has been slow to come for AIH patients. While current treatments are successful in the majority of patients, treatment discontinuation is difficult to achieve, and relapses are frequent...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28178440/comorbidities-in-patients-with-psoriatic-arthritis
#18
REVIEW
Amir Haddad, Devy Zisman
Epidemiological studies have shown that patients with psoriatic arthritis (PsA) are often affected by numerous comorbidities that carry significant morbidity and mortality. Reported comorbidities include diabetes mellitus, obesity, metabolic syndrome, cardiovascular diseases, osteoporosis, inflammatory bowel disease, autoimmune eye disease, non-alcoholic fatty liver disease, depression, and fibromyalgia. All health care providers for patients with PsA should recognize and monitor those comorbidities, as well as understand their effect on patient management to ensure an optimal clinical outcome...
January 30, 2017: Rambam Maimonides Medical Journal
https://www.readbyqxmd.com/read/28176894/autoimmune-hepatitis-current-challenges-and-future-prospects
#19
REVIEW
Yoshio Aizawa, Atsushi Hokari
Autoimmune hepatitis (AIH) is a chronic progressive liver disease characterized by high levels of aminotransferases and autoantibodies, hypergammaglobulinemia, and interface hepatitis. AIH affects all races and all ages worldwide, regardless of sex, although a preponderance of females is a constant finding. The etiology of AIH has not been completely elucidated, but immunogenetic background and environmental parameters may contribute to its development. The most important genetic factor is human leukocyte antigens (HLAs), especially HLA-DR, whereas the role of environmental factors is not completely understood...
2017: Clinical and Experimental Gastroenterology
https://www.readbyqxmd.com/read/28176332/low-dose-interleukin-2-promotes-stat5-phosphorylation-treg-survival-and-ctla-4-dependent-function-in-autoimmune-liver-diseases
#20
Hannah C Jeffery, Louisa E Jeffery, Philipp Lutz, Margaret Corrigan, Gwilym J Webb, Gideon M Hirschfield, David H Adams, Ye Htun Oo
CD4(+) CD25(high) CD127(low) FOXP3(+) regulatory T cells (Treg) are essential for the maintenance of peripheral tolerance. Impaired Treg function and an imbalance between effector and regulatory T cells contribute to the pathogenesis of autoimmune diseases. We recently reported that the hepatic microenvironment is deficient in IL-2, a cytokine essential for Treg survival and function. Consequently, few liver-infiltrating Treg demonstrate STAT5 phosphorylation. To establish the potential of IL-2 to enhance Treg therapy, we investigated the effects of very low dose Proleukin (VLDP) on the phosphorylation of STAT5 and the subsequent survival and function of Treg and T effector cells from the blood and livers of patients with autoimmune liver diseases...
February 8, 2017: Clinical and Experimental Immunology
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