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https://www.readbyqxmd.com/read/29025775/a-unique-case-of-nephrogenic-systemic-fibrosis-from-gadolinium-exposure-in-a-patient-with-normal-egfr
#1
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
#2
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...
October 9, 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
#3
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
#4
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
#5
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
#6
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
#7
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...
September 20, 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
#8
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...
September 16, 2017: Acta Biomaterialia
https://www.readbyqxmd.com/read/28926118/chop-induced-loss-of-intestinal-epithelial-stemness-contributes-to-bile-duct-ligation-induced-cholestatic-liver-injury
#9
Runping Liu, Xiaojiaoyang Li, Zhiming Huang, Derrick Zhao, Bhagyalaxmi Sukka Ganesh, Guanhua Lai, William M Pandak, Phillip B Hylemon, Jasmohan S Bajaj, Arun J Sanyal, Huiping Zhou
Impaired intestinal barrier function promotes the progression of various liver diseases including cholestatic liver disease. The close association of primary sclerosing cholangitis (PSC) with inflammatory bowel disease highlights the importance of the gut-liver axis. It has been reported that bile duct ligation (BDL)-induced liver fibrosis is significantly reduced in C/EBP homologous protein knock out (CHOP(-/-) ) mice. However, the underlying mechanisms remain unclear. In the current study, we demonstrate that BDL induces striking and acute hepatic ER stress responses after 1 day, which return to normal after 3 days...
September 19, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28922106/igg4-related-disease-a-patient-with-multiple-organ-involvement
#10
Miroslav Průcha, Lenka Sedláčková
IgG4-related diseases represent a heterogeneous group of conditions characterised by elevated serum IgG4 levels and fibrotic or sclerosing changes in the affected organs or systems accompanied by IgG4-positive plasma cells. A disease associated with IgG4 may affect virtually any organ - salivary glands, periorbital tissue, kidneys, lungs, meninges, aorta, prostate, pericardium or skin. Histopathological findings are uniform, characterised by a major lymphoplasmocytic infiltrate and the presence of IgG4-producing plasma cells, irrespective of the affected site...
2017: Prague Medical Report
https://www.readbyqxmd.com/read/28919271/-autoimmune-hepatitis-immunological-diagnosis
#11
Imane Brahim, Ikram Brahim, Raja Hazime, Brahim Admou
Autoimmune hepatopathies (AIHT) including autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune cholangitis (AIC), represent an impressive entities in clinical practice. Their pathogenesis is not perfectly elucidated. Several factors are involved in the initiation of hepatic autoimmune and inflammatory phenomena such as genetic predisposition, molecular mimicry and/or abnormalities of T-regulatory lymphocytes. AIHT have a wide spectrum of presentation, ranging from asymptomatic forms to severe acute liver failure...
September 14, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28913623/updated-strategies-for-the-management-pathogenesis-and-molecular-genetics-of-different-forms-of-ichthyosis-syndromes-with-prominent-hair-abnormalities
#12
REVIEW
Madiha Rasheed, Shaheen Shahzad, Afifa Zaeem, Imran Afzal, Asma Gul, Sumbal Khalid
Syndromic ichthyosis is rare inherited disorders of cornification with varied disease complications. This disorder appears in seventeen subtypes associated with severe systematic manifestations along with medical, cosmetic and social problems. Syndromic ichthyosis with prominent hair abnormalities covers five major subtypes: Netherton syndrome, trichothiodystrophy, ichthyosis hypotrichosis syndrome, ichthyosis hypotrichosis sclerosing cholangitis and ichthyosis follicularis atrichia photophobia syndrome. These syndromes mostly prevail in high consanguinity states, with distinctive clinical features...
September 14, 2017: Archives of Dermatological Research
https://www.readbyqxmd.com/read/28910870/-primary-mediastinal-large-b-cell-lymphoma-a-clinicopathologic-study-of-27-cases
#13
Q Y Shi, X Feng, H Chen, H H Ma, Z F Lu, Q L Shi, X J Zhou, Q Shen
Objective: To study the clinicopathologic characteristics and diagnostic criteria of primary mediastinal B-cell lymphoma (PMBL), and to distinguish PMBL from classic Hodgkin lymphoma(CHL) and systemic diffuse large B-cell lymphoma(DLBCL). Methods: The clinical features, histologic findings, results of immunohistochemical study and prgnosis in 27 PMBL cases were analyzed, with review of literature. Results: The age of patients ranged from 19 to 82 years (median age 34 years). All cases were located in the mediastinum and frequently accompanied by superior vein cava syndrome...
September 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28870031/-the-clinical-characteristics-of-346-patients-with-igg4-related-disease
#14
P P Zhang, J Z Zhao, M Wang, R E Feng, X W Liu, X M Lai, X J Li, J G Zeng, H J Shi, H D Zhu, W Xue, H Zhang, Y Y Chen, L Y Fei, X F Peng, F C Zeng, Y M Zhang, W Zhang
Objective: To analyze the clinical characteristics of IgG4-related disease (IgG4-RD)so as to improve the understanding of IgG4-RD in China. Methods: IgG4-RD patients were recruited from Peking Union Medical College Hospital between January 2011 and January 2016. All patients were followed-up for more than 6 months. The demographic characteristics, symptoms, organ involvements, laboratory examinations and treatment efficacy were evaluated and analyzed. Results: A total of 346 patients were finally enrolled, including 230 males (66...
September 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/28868289/pathophysiological-mechanisms-in-sclerosing-skin-diseases
#15
REVIEW
Beate Eckes, Fang Wang, Pia Moinzadeh, Nicolas Hunzelmann, Thomas Krieg
Sclerosing skin diseases represent a large number of distinct disease entities, which include systemic sclerosis, localized scleroderma, and scleredema adultorum. These pathologies have a common clinical appearance and share histological features. However, the specific interplay between cytokines and growth factors, which activate different mesenchymal cell populations and production of different extracellular matrix components, determines the biomechanical properties of the skin and the clinical features of each disease...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28851259/does-transient-elastography-correlate-with-liver-fibrosis-in-patients-with-psc-laennec-score-based-analysis-of-explanted-livers
#16
Marcin Krawczyk, Joanna Ligocka, Mariusz Ligocki, Joanna Raszeja-Wyszomirska, Małgorzata Milkiewicz, Grzegorz Szparecki, Tomasz Ilczuk, Barbara Górnicka, Krzysztof Zieniewicz, Marek Krawczyk, Frank Lammert, Piotr Milkiewicz
BACKGROUND AND AIMS: Previous studies demonstrated a close correlation between transient elastography (TE) and liver histology in chronic liver diseases. Data on the accuracy of TE in primary sclerosing cholangitis (PSC) remains scarce. Here, we investigated the association between TE, serum marker of liver injury and histology of explanted livers in PSC patients. METHODS: Thirty patients were prospectively recruited. TE (Fibroscan(®)) and blood sampling were performed during evaluation for liver transplantation (LT); the second blood sampling was performed on the day of LT...
August 29, 2017: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/28816552/the-role-of-biopsy-in-lacrimal-gland-inflammation-a-clinicopathologic-study
#17
Panitee Luemsamran, Jack Rootman, Valerie A White, Nariman Nassiri, Manraj K S Heran
PURPOSE: To determine the causes of lacrimal gland inflammation based on histopathology and systemic evaluation. METHODS: This is a retrospective case series study. From the University of British Columbia Orbit Clinic between January 1976 and December 2008, we reviewed the medical records of 60 patients who presented with inflammatory features of the lacrimal gland (i.e., erythema, edema, or tenderness) in which the diagnoses were not possible clinically and on imaging alone...
August 17, 2017: Orbit
https://www.readbyqxmd.com/read/28802066/targeting-senescent-cholangiocytes-and-activated-fibroblasts-with-bcl-xl-inhibitors-ameliorates-fibrosis-in-mdr2-mice
#18
Anja Moncsek, Mohammed S Al-Suraih, Christy E Trussoni, Steven P O'Hara, Patrick L Splinter, Camille Zuber, Eleonora Patsenker, Piero V Valli, Christian D Fingas, Achim Weber, Yi Zhu, Tamar Tchkonia, James L Kirkland, Gregory J Gores, Beat Müllhaupt, Nicholas F LaRusso, Joachim C Mertens
Cholangiocyte senescence has been linked to primary sclerosing cholangitis (PSC). Persistent secretion of growth factors by senescent cholangiocytes leads to the activation of stromal fibroblasts (ASF), which are drivers of fibrosis. The activated phenotype of ASF is characterized by an increased sensitivity to apoptotic stimuli. Here, we examined the mechanisms of apoptotic priming in ASF and explored a combined targeting strategy to deplete senescent cholangiocytes and ASF from fibrotic tissue to ameliorate liver fibrosis...
August 12, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28757171/genetic-determinants-of-cholangiopathies-molecular-and-systems-genetics
#19
REVIEW
Matthias C Reichert, Rabea A Hall, Marcin Krawczyk, Frank Lammert
Familial cholangiopathies are rare but potentially severe diseases. Their spectrum ranges from fairly benign conditions as, for example, benign recurrent intrahepatic cholestasis to low-phospholipid associated cholelithiasis and progressive familial intrahepatic cholestasis (PFIC). Many cholangiopathies such as primary biliary cholangitis (PBC) or primary sclerosing cholangitis (PSC) affect first the bile ducts ("ascending pathophysiology") but others, such as PFIC, start upstream in hepatocytes and cause progressive damage "descending" down the biliary tree and leading to end-stage liver disease...
July 27, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28744088/igg4-related-disease-simulating-paraneoplastic-syndrome-role-of-18-fdg-pet-ct-imaging
#20
Madhuri S Mahajan, Sudeshna Maitra, Natasha Singh, Melvika Pereira
Immunoglobulin G4 (IgG4)-related systemic disease (IgG4-RSD) is a new systemic entity associated with autoimmune pancreatitis (AIP). Other organ involvements take the form of sclerosing cholangitis, sclerosing cholecystitis, sclerosing sialadenitis, retroperitoneal fibrosis, and interstitial nephritis. Recently, lung diseases related to IgG4 have been described to occur with or without other organ involvement. These diseases include interstitial lung disease (ILD), pulmonary inflammatory pseudotumor, and lymphomatoid granulomatosis...
April 2017: Indian Journal of Radiology & Imaging
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