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https://www.readbyqxmd.com/read/29226107/cholangitis-diagnosis-treatment-and-prognosis
#1
REVIEW
Amir Houshang Mohammad Alizadeh
Cholangitis is a serious life-threatening situation affecting the hepatobiliary system. This review provides an update regarding the clinical and pathological features of various forms of cholangitis. A comprehensive search was performed in the PubMed, Scopus, and Web of Knowledge databases. It was found that the etiology and pathogenesis of cholangitis are heterogeneous. Cholangitis can be categorized as primary sclerosing (PSC), secondary (acute) cholangitis, and a recently characterized form, known as IgG4-associated cholangitis (IAC)...
December 28, 2017: Journal of Clinical and Translational Hepatology
https://www.readbyqxmd.com/read/29221270/analysis-of-the-clinical-differentiation-of-pulmonary-sclerosing-pneumocytoma-and-lung-cancer
#2
Jun Zhu
Background: Pulmonary sclerosing pneumocytoma (PSP) is a rare benign lung tumor. This study investigated the diagnostic experience of PSP and lung cancer. Methods: This study is a retrospective study. We observed the locations of lung lesions, imaging form and clinical symptoms, and recorded the surgical complications through comparing patients with PSP and lung cancer. Results: From December 2012 to February 2017, 187 PSP cases and 197 lung cancer cases were collected...
September 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29196804/igg4-related-disease-with-emphasis-on-the-biopsy-diagnosis-of-autoimmune-pancreatitis-and-sclerosing-cholangitis
#3
REVIEW
Sönke Detlefsen, Günter Klöppel
In 2011, chronic fibroinflammatory processes occurring simultaneously or metachronously in various organs and associated with elevated IgG4 serum levels and/or tissue infiltration with IgG4-positive plasma cells have been recognized as manifestations of a systemic disorder called IgG4-related disease (IgG4-RD). The histologic key findings are lymphoplasmacytic infiltration rich in IgG4-positive plasma cells combined with storiform fibrosis and obliterative phlebitis. Among the organs mainly affected by IgG4-RD are the pancreas and the extrahepatic bile ducts...
December 1, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/29141796/high-dose-biotin-as-treatment-for-progressive-multiple-sclerosis
#4
Gary Birnbaum, Jessica Stulc
BACKGROUND: Published data suggested high dose biotin improved patients with progressive MS. We wished to determine benefits and side effects of administering daily high dose biotin to patients with progressive multiple sclerosis in a large MS specialty clinic. METHODS: Forty-three patients with progressive multiple scleroses were prescribed pharmaceutical grade biotin as a single daily dose of 300mg/day. Brain MRIs were performed at baseline and after one year on biotin...
November 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29129978/a-case-of-peripheral-ulcerative-keratitis-associated-with-autoimmune-hepatitis
#5
Hamoon Eshraghi, Aria Mahtabfar, Mohammad H Dastjerdi
Purpose: To describe a case of peripheral ulcerative keratitis in the setting of autoimmune hepatitis and possible overlap syndrome with primary sclerosing cholangitis. Case Report: A 48-year-old African American female with autoimmune hepatitis with possible overlap syndrome with primary sclerosing cholangitis presented with tearing, irritation, and injection of the left eye that was determined to be peripheral ulcerative keratitis. The patient was treated with topical and systemic steroids, immunosuppressant drugs (azathioprine and mycophenolate mofetil), a biologic (rituximab), and surgery (conjunctival resection), and the peripheral ulcerative keratitis epithelialized but ultimately led to corneal perforation...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/29108474/molecular-testing-for-oncogenic-gene-alterations-in-pediatric-thyroid-lesions
#6
Sogol Mostoufi-Moab, Emmanuel Labourier, Lisa Sullivan, Virginia A LiVolsi, Yimei Li, Rui Xiao, Sylvie Beaudenon-Huibregtse, Ken Kazahaya, N Scott Adzick, Zubair W Baloch, Andrew J Bauer
BACKGROUND: Thyroid nodules are less common in pediatric patients (≤18 years) than in adults. The Bethesda System for Reporting Thyroid Cytopathology allows for individual risk stratification, but a significant number of nodules are indeterminate. Incorporating gene mutation panels and gene expression classifiers may aid in pre-operative diagnosis. The overall aim of this study was to assess the prevalence of oncogene alterations in a representative pediatric population and across a broad-spectrum of thyroid tumor diagnoses...
November 6, 2017: Thyroid: Official Journal of the American Thyroid Association
https://www.readbyqxmd.com/read/29099539/igg4-related-disease-in-thymus-a-very-rare-case-of-chronic-fibrosis-mimicking-sarcoidosis
#7
Sara Simonetti, Noelia Pérez Muñoz, Josefa López Vivancos, Lluís Sanchez Sitjes, Javier Cesar Herranz Pérez, Nelson Leal Bohorquez, José Antonio Maestre Alcacer, Inessa Koptseva de García, Miguel Ángel Carrasco García
IgG4-related disease (IgG4-RD) is a multi-organ immune-mediated chronic fibroinflammatory condition, with unclear certain etiology. It is morphologically characterized by storiform fibrosis, dense IgG4-positive lymphoplasmacytic infiltrate, and obliterative phlebitis. It was recognized as a systemic condition as recently as 2003. IgG4-RD has been described in virtually every organ, forming sclerosing masses, and often mimicking tumors. Clinically, patients present unspecific symptoms and this condition is often recognized incidentally...
October 28, 2017: Tumori
https://www.readbyqxmd.com/read/29089545/activation-of-fas-fasl-pathway-and-the-role-of-c-flip-in-primary-culture-of-human-cholangiocarcinoma-cells
#8
Gianluca Carnevale, Guido Carpino, Vincenzo Cardinale, Alessandra Pisciotta, Massimo Riccio, Laura Bertoni, Lara Gibellini, Sara De Biasi, Lorenzo Nevi, Daniele Costantini, Diletta Overi, Andrea Cossarizza, Anto de Pol, Eugenio Gaudio, Domenico Alvaro
Intrahepatic cholangiocarcinoma (iCCA) represents a heterogeneous group of malignancies emerging from the biliary tree, often in the context of chronic bile ducts inflammation. The immunological features of iCCA cells and their capability to control the lymphocytes response have not yet been investigated. The aims of the present study were to evaluate the interaction between iCCA cells and human peripheral blood mononuclear cells (PBMCs) and the role of Fas/FasL in modulating T-cells and NK-cells response after direct co-culture...
October 31, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29083037/review-article-the-gut-microbiome-as-a-therapeutic-target-in-the-pathogenesis-and-treatment-of-chronic-liver-disease
#9
REVIEW
C A Woodhouse, V C Patel, A Singanayagam, D L Shawcross
BACKGROUND: Mortality from chronic liver disease is rising exponentially. The liver is intimately linked to the gut via the portal vein, and exposure to gut microbiota and their metabolites translocating across the gut lumen may impact upon both the healthy and diseased liver. Modulation of gut microbiota could prove to be a potential therapeutic target. AIM: To characterise the changes in the gut microbiome that occur in chronic liver disease and to assess the impact of manipulation of the microbiome on the liver...
October 30, 2017: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29045346/autoimmune-hepatitis-and-aih-overlap-with-sclerosing-cholangitis-immunophenotype-markers-in-children-and-adolescents
#10
Priscila Menezes Ferri, Ana Cristina Simões E Silva, Karen Cecília de Lima Torres, Soraya Luiza Campos Silva, Diego Júnior Queiroga de Aquino, Maria Luísa Marques Ferreira, Eleonora Druve Tavares Fagundes, Débora Marques de Miranda, Alexandre Rodrigues Ferreira
OBJECTIVE: The pathophysiology of autoimmune hepatitis (AIH) may involve the activation of immune cells and changes in the expression of cellular markers. The aim of this study was to characterize the immunophenotype markers of lymphocytes and monocytes in the peripheral blood of children and adolescents with type 1 AIH and AIH overlap with sclerosing cholangitis (overlap syndrome, OS). METHODS: This is a cross-sectional study of 20 children and adolescents diagnosed with type 1 AIH and 19 with OS...
October 17, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29039048/sclerosing-and-obstructive-cholangiopathy-in-biliary-atresia-mechanisms-and-association-with-biliary-innate-immunity
#11
REVIEW
Kenichi Harada
Biliary atresia (BA) is histologically characterized by a progressive, sclerosing cholangitis and the obstruction of extrahepatic bile ducts. In terms of the etiology and pathogenesis of BA, several viral infections consisting of dsRNA, including Reoviridae, have been implicated. Human biliary epithelial cells (BECs) possess an innate immune system consisting of Toll-like receptors (TLRs). BECs have negative regulatory mechanisms of TLR tolerance to avoid an excessive inflammatory response to lipopolysaccharide (LPS), a TLR4 ligand; however, they lack the tolerance to poly(I:C) (a synthetic analog of viral dsRNA), a TLR3 ligand...
December 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/29032433/diffuse-mesangial-sclerosis-in-a-pdss2-mutation-induced-coenzyme-q10-deficiency
#12
Béla Iványi, Gábor Z Rácz, Péter Gál, Kitti Brinyiczki, István Bódi, Tibor Kalmár, Zoltán Maróti, Csaba Bereczki
BACKGROUND: A 7-month-old male infant was admitted because he was suffering from nephrotic syndrome, along with encephalomyopathy, hypertrophic cardiomyopathy, clinically suspected deafness and retinitis pigmentosa, and an elevated serum lactate level. METHODS: Coenzyme Q10 supplementation was started because of the clinical suspicion of primary CoQ10 deficiency. Despite intensive efforts, he passed away 4 weeks after admission. RESULTS: The results of genetic tests, available postmortem, explored two hitherto undescribed mutations in the PDSS2 gene...
October 14, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29025775/a-unique-case-of-nephrogenic-systemic-fibrosis-from-gadolinium-exposure-in-a-patient-with-normal-egfr
#13
Sadichhya Lohani, Jon Golenbiewski, Abhishek Swami, Alexandra Halalau
A 57-year-old woman presented with swelling and thickening of the skin of the lower extremities. Three months prior to presentation, patient had MRI with gadolinium as part of an evaluation for suspected pancreatic malignancy. Creatinine levels at the time of gadolinium exposure were 0.9-1.2 mg/dL, with a corresponding estimated glomerular filtration rate of 64 mL/min/1.73m(2) by modification of diet in renal disease equation. Twenty-four-hour urine creatinine clearance was performed as an outpatient following development of symptoms...
October 11, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28993983/skin-manifestations-associated-with-autoimmune-liver-diseases-a-systematic-review
#14
REVIEW
Benedetta Terziroli Beretta-Piccoli, Pietro Invernizzi, M Eric Gershwin, Carlo Mainetti
Autoimmune liver diseases, which include mainly autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, and the variant syndromes, are often associated with extrahepatic autoimmune diseases. However, the association with cutaneous diseases is less well described. In the present article, we provide a systematic literature review on skin manifestations linked to each of these four autoimmune liver diseases, excluding skin manifestations of systemic diseases. The association of autoimmune hepatitis with vitiligo is well known, with a particular striking association with type 2 autoimmune hepatitis, a condition occurring almost entirely in children and adolescents, much rarer and more aggressive than type 1 autoimmune hepatitis; probable associations are also identified with alopecia areata, psoriasis, and pyoderma gangrenosum...
December 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28973414/sclerotherapy-for-reticular-veins-in-the-lower-limbs-a-triple-blind-randomized-clinical-trial
#15
Matheus Bertanha, Rodrigo Gibin Jaldin, Regina Moura, Rafael Elias Farres Pimenta, Jamil Victor de Oliveira Mariúba, Carlos Eduardo Pinheiro Lúcio Filho, Giovana Piteri Alcantara, Carlos Roberto Padovani, Winston Bonetti Yoshida, Marcone Lima Sobreira
Importance: Reticular veins are subdermal veins located in the lower limbs and are mainly associated with aesthetic complaints. Although sclerotherapy is the treatment of choice for reticular veins in the lower limbs, no consensus has been reached regarding to the optimal sclerosant. Objective: To compare the efficacy and safety of 2 sclerosants used to treat reticular veins: 0.2% polidocanol diluted in 70% hypertonic glucose (HG) (group 1) vs 75% HG alone (group 2)...
September 27, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28970729/patients-with-inflammatory-bowel-disease-have-increased-risk-of-autoimmune-and-inflammatory-diseases
#16
Morten L Halling, Jens Kjeldsen, Torben Knudsen, Jan Nielsen, Lars Koch Hansen
AIM: To investigate whether immune mediated diseases (IMD) are more frequent in patients with inflammatory bowel disease (IBD). METHODS: In this population based registry study, a total of 47325 patients with IBD were alive and registered in the Danish National Patient Registry on December 16, 2013. Controls were randomly selected from the Danish Civil Registration System (CRS) and matched for sex, age, and municipality. We used ICD 10 codes to identify the diagnoses of the included patients...
September 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28945060/benefits-of-micronized-purified-flavonoid-fraction-as-adjuvant-therapy-on-the-inflammatory-response-after-sclerotherapy
#17
Vadim Y Bogachev, Boris V Boldin, Victor N Lobanov
BACKGROUND: Sclerotherapy is the treatment of choice for telangiectasias. The aim of this study was to investigate the systemic and local inflammatory response after sclerotherapy in women with chronic venous disease (CVD) and to assess the effects of micronized purified flavonoid fraction (MPFF) in combination with sclerotherapy on markers of inflammation and endothelial dysfunction. METHODS: 60 women with primary CVD CEAP class C1 were randomly assigned sclerotherapy in combination with MPFF treatment (N=30) or sclerotherapy alone (control) group (N=30)...
September 22, 2017: International Angiology: a Journal of the International Union of Angiology
https://www.readbyqxmd.com/read/28943450/pathological-features-of-primary-sclerosing-cholangitis-identified-by-bile-proteomic-analysis
#18
C Rupp, K A Bode, Y Leopold, P Sauer, D N Gotthardt
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown origin. Previous bile proteomic analyses in patients with PSC have revealed changes in disease activity specific to malignant transformation. In this study, we established a reference bile duct-derived bile proteome for PSC that can be used to evaluate biliary pathophysiology. Samples were collected from patients with PSC or with choledocholithiasis (control) (n=6 each). Furthermore, patients with PSC-associated cholangiocarcinoma (CC) and with CC without concomitant PSC were analyzed...
September 22, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28929808/ethanol-gel-sclerotherapy-of-venous-malformations-effectiveness-and-safety
#19
Veronika I Teusch, Walter A Wohlgemuth, Simone Hammer, Armin P Piehler, René Müller-Wille, Holger Goessmann, Wibke Uller
OBJECTIVE: In the treatment of venous malformations, ethanol may be administered in a gelified form to increase local effects and reduce systemic ones. The purpose of this prospective study was to evaluate the efficacy and safety of a commercially available viscous ethanol gel in the treatment of venous malformations. SUBJECTS AND METHODS: Thirty-one patients (mean age, 23.4 years; age range, 6.6-46.5 years) with venous malformations were prospectively scheduled for two ethanol-gel sclerotherapy sessions...
December 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/28927932/chitosan-doxycycline-hydrogel-an-mmp-inhibitor-sclerosing-embolizing-agent-as-a-new-approach-to-endoleak-prevention-and-treatment-after-endovascular-aneurysm-repair
#20
Fatemeh Zehtabi, Pompilia Ispas-Szabo, Djahida Djerir, Lojan Sivakumaran, Borhane Annabi, Gilles Soulez, Mircea Alexandru Mateescu, Sophie Lerouge
The success of endovascular repair of abdominal aortic aneurysms remains limited due to the development of endoleaks. Sac embolization has been proposed to manage endoleaks, but current embolizing materials are associated with frequent recurrence. An injectable agent that combines vascular occlusion and sclerosing properties has demonstrated promise for the treatment of endoleaks. Moreover, the inhibition of aneurysmal wall degradation via matrix metalloproteinases (MMPs) may further prevent aneurysm progression...
December 2017: Acta Biomaterialia
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