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https://www.readbyqxmd.com/read/29452711/primary-sclerosing-cholangitis
#1
REVIEW
Jessica K Dyson, Ulrich Beuers, David E J Jones, Ansgar W Lohse, Mark Hudson
Primary sclerosing cholangitis is a rare, chronic cholestatic liver disease characterised by intrahepatic or extrahepatic stricturing, or both, with bile duct fibrosis. Inflammation and fibrosis of bile ducts and the liver are followed by impaired bile formation or flow and progressive liver dysfunction. Patients might be asymptomatic at presentation or might have pruritus, fatigue, right upper quadrant pain, recurrent cholangitis, or sequelae of portal hypertension. The key diagnostic elements are cholestatic liver biochemistry and bile duct stricturing on cholangiography...
February 13, 2018: Lancet
https://www.readbyqxmd.com/read/29451344/a-case-of-microscopic-multiple-sclerosing-pneumocytoma
#2
Hitomi Kawai, Norio Takayashiki, Haruo Otani, Shingo Sakashita, Masayuki Noguchi
Sclerosing pneumocytoma is a rare tumor of the lung, commonly affecting middle-aged women, and is mostly isolated. Although this tumor is thought to be derived from primitive respiratory epithelial cells, the characteristics of the precursor cells are still unknown. A 19-year-old woman presented with multiple nodules in the right lung. Partial resection of the right middle lobe was performed, and seven sclerosing pneumocytomas, including four that were microscopic, were detected. The latter showed a simple papillary pattern, and three of them consisted of only round cell-like cells (single population)...
February 16, 2018: Pathology International
https://www.readbyqxmd.com/read/29449577/neutralization-of-il-15-abrogates-experimental-immune-mediated-cholangitis-in-diet-induced-obese-mice
#3
José L Reyes, Danielle T Vannan, Tina Vo, Aliya Gulamhusein, Paul L Beck, Raylene A Reimer, Bertus Eksteen
Obesity is a global epidemic affecting chronic inflammatory diseases. Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease that can occur as an extraintestinal manifestation of inflammatory bowel disease (IBD). Previously we reported that patients with PSC who are obese have a higher risk of advanced liver disease. Currently it is unknown how obesity accelerates or worsens PSC. We evaluated the progression of PSC in an antigen-driven cholangitis mouse model of diet-induced obesity. Obesity was induced in our murine model of immune-mediated cholangitis (OVAbil)...
February 15, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29442349/18f-fdg-avid-sclerosing-angiomatoid-nodular-transformation-sant-of-spleen-on-pet-ct-a-rare-mimicker-of-metastasis
#4
Punit Sharma
Sclerosing Angiomatoid Nodular Transformation (SANT) is a rare benign vascular tumor of spleen. It consists of multiple angiomatoid nodules surrounded by dense fibrous tissue that often coalesces centrally to form a scar, which is considered to be a characteristic feature. These are usually asymptomatic and incidentally detected on imaging for other underlying pathology. SANTs can be 18F-Fluorodeoxyglucose (18F-FDG) avid on positron emission tomography-computed tomography (PET-CT) and thus can lead to false positive finding in oncological patients...
2018: Nuclear Medicine Review. Central & Eastern Europe
https://www.readbyqxmd.com/read/29437719/dieulafoy-s-lesion-in-the-oesophagus-a-rare-cause-of-upper-gastrointestinal-bleeding
#5
Divyesh Reddy Nemakayala, Manoj P Rai, Julie L Yam, Heather Laird-Fick
A Dieulafoy's lesion is defined as a dilated, aberrant, submucosal artery that erodes overlying mucosa in the absence of an underlying ulcer, aneurysm or intrinsic mural abnormality. It is a rare cause of upper gastrointestinal (GI) bleed with a very high mortality rate if it goes unidentified. It is most commonly located in the lesser curvature of the stomach but is extremely rare in the oesophagus. We are reporting a 55-year-old man who had massive haematemesis. Emergent endoscopy showed Dieulafoy's lesion in the distal oesophagus...
February 7, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29436376/breast-carcinoma-in-sclerosing-adenosis-a-clinicopathological-and-immunophenotypical-analysis-on-206-lesions
#6
Bao-Hua Yu, Shao-Xian Tang, Xiao-Li Xu, Yu-Fan Cheng, Rui Bi, Ruo-Hong Shui, Xiao-Yu Tu, Hong-Fen Lu, Xiao-Yan Zhou, Wen-Tao Yang
AIMS: To fully elucidate the clinicopathological features of breast carcinoma in sclerosing adenosis (SA-BC). METHODS: Clinical and histological characteristics of 206 SA-BCs from 180 patients were retrospectively evaluated. Immunohistochemical phenotype was examined. The clinicopathological relevance of the topographical pattern of SA-BCs was analysed. RESULTS: Overall, up to 46 patients (25.6%) had contralateral cancer, either SA associated or not...
February 7, 2018: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/29436365/teaching-case-2-2018-sclerosing-myxopapillary-ependymoma-mimicking-whorling-sclerosing-meningioma
#7
Julia Lang, Thomas Czech, Irene Slavc, Dominik Reisinger, Sophie Bartsch, Johannes A Hainfellner, Christine Haberler, Ellen Gelpi
No abstract text is available yet for this article.
February 13, 2018: Clinical Neuropathology
https://www.readbyqxmd.com/read/29435321/the-gut-microbiota-bile-acids-and-their-correlation-in-primary-sclerosing-cholangitis-associated-with-inflammatory-bowel-disease
#8
J Torres, C Palmela, H Brito, X Bao, H Ruiqi, P Moura-Santos, J Pereira da Silva, A Oliveira, C Vieira, K Perez, S H Itzkowitz, J F Colombel, L Humbert, D Rainteau, M Cravo, C M Rodrigues, J Hu
Background: Patients with primary sclerosing cholangitis associated with inflammatory bowel disease (PSC-IBD) have a very high risk of developing colorectal neoplasia. Alterations in the gut microbiota and/or gut bile acids could account for the increase in this risk. However, no studies have yet investigated the net result of cholestasis and a potentially altered bile acid pool interacting with a dysbiotic gut flora in the inflamed colon of PSC-IBD. Aim: The aim of this study was to compare the gut microbiota and stool bile acid profiles, as well as and their correlation in patients with PSC-IBD and inflammatory bowel disease alone...
February 2018: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/29434141/takayasu-arteritis-coexisting-with-sclerosing-osteomyelitis
#9
Tsuyoshi Shirai, Riiza Hanaoka, Yusuke Goto, Ikuho Kojima, Yusho Ishii, Yousuke Hoshi, Yoko Fujita, Yuko Shirota, Hiroshi Fujii, Tomonori Ishii, Hideo Harigae
We report a rare case of a 27-year-old woman with Takayasu arteritis (TAK) complicated by diffuse sclerosing osteomyelitis. She first presented with sclerosing osteomyelitis of the right mandible without evidence of arteritis in the carotid arteries. Eight months later, she complained of left neck pain, and imaging studies revealed the presence of arteritis in the left carotid artery. She was diagnosed with TAK, and immunosuppressive treatment was initiated, which was effective for both the arteritis and the osteomyelitis...
February 9, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29433373/expression-of-angiogenic-factors-in-sclerosing-stromal-tumours-of-the-ovary
#10
Tomoko Hirakawa, Yasushi Kawano, Chiharu Mizoguchi, Kaei Nasu, Hisashi Narahara
Sclerosing stromal tumours (SSTs) are rare benign tumours and are generally observed in individuals in their teens to 20 s. Many cases are suspected as malignant tumours pre-operatively, owing to their high vascularity on diagnostic imaging. Herein, we aimed to evaluate the expressions of various angiogenic factors in SSTs. The expressions of vascular endothelial growth factor (VEGF), basic fibroblast growth factor (bFGF) and hepatocyte growth factor (HGF) were examined by immunohistochemical staining. Upon immunohistochemistry, overexpressions of VEGF, bFGF, and HGF in the ovarian stroma were observed in SSTs...
February 13, 2018: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/29432923/peribiliary-cysts-presenting-as-primary-sclerosing-cholangitis
#11
Aravind Gokul Tamilarasan, Jessica Yang, Alice Unah Lee
No abstract text is available yet for this article.
February 9, 2018: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29432345/18f-fdg-pet-ct-findings-in-a-patient-with-igg4-related-sclerosing-mesenteritis
#12
Zhanli Fu, Guoqian Chen, Meng Liu, Ziao Li, Qian Li
A large mass in the abdomen was revealed by ultrasonography on a 68-year-old woman presenting with abdominal pain and weight loss. To exclude the malignancy, an F-FDG PET/CT was performed, which showed a solitary soft tissue mass with heterogeneous F-FDG avidity at the root of small bowel mesentery. Because of the elevation of inflammatory markers and serum IgG4 level, IgG4-related sclerosing mesenteritis was suspected, which was proved by the subsequent biopsy. The mass gradually shrunk and ultimately disappeared in 6 months after a low-dose steroid therapy...
February 9, 2018: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29431255/short-term-side-effects-and-patient-reported-outcomes-of-bleomycin-sclerotherapy-in-vascular-malformations
#13
Joana M Mack, Gresham T Richter, David Becton, Omar Salem, Sarah E M Hill, Shelley E Crary
BACKGROUND: Vascular malformations (VM) are congenital lesions that can be debilitating and cause significant aesthetic and functional limitations. The chemotherapeutic agent bleomycin has been utilized as a sclerosant, directly injected percutaneously into the VM. Unfortunately, little is known about the benefits and short-term side effects of bleomycin with intralesional injections. PROCEDURE: An IRB approved, retrospective chart review was performed on patients with VM who had been treated with intralesional bleomycin...
February 12, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29430973/a-case-of-twin-ivf-pregnancy-complicated-with-sclerosing-encapsulating-peritonitis-diagnosed-at-caesarean-delivery
#14
Ebru Yuce, Nilüfer Akgun, Ayse Altındis Bal, Muberra Namli Kalem, Reyhan Bayrak, Candan Iltemir Duvan
No abstract text is available yet for this article.
February 12, 2018: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/29430861/clinical-course-of-type-1-autoimmune-pancreatitis-patients-without-steroid-treatment-a-japanese-multicenter-study-of-97-patients
#15
Kensuke Kubota, Terumi Kamisawa, Kenji Hirano, Yoshiki Hirooka, Kazushige Uchida, Tsukasa Ikeura, Hideyuki Shiomi, Hirotaka Ohara, Kyoko Shimizu, Norikazu Arakura, Atsushi Kanno, Junichi Sakagami, Takao Itoi, Tetsuhide Ito, Toshiharu Ueki, Takayoshi Nishino, Kazuo Inui, Nobumasa Mizuno, Hitoshi Yoshida, Masanori Sugiyama, Eisuke Iwasaki, Atsushi Irisawa, Kazuichi Okazaki, Shigeyuki Kawa, Toru Shimosegawa, Yoshifumi Takeyama, Tsutomu Chiba
BACKGROUND: Sporadic autoimmune pancreatitis (AIP) cases showing remission without steroid treatment have been reported, however, the clinical course of these patients has not been clarified. AIM: This study sought to clarify the clinical course in AIP patients with hesitation for steroid treatment. METHODS: We collected clinical data for AIP patients from high-volume centers in Japan. Data for AIP patients with and those without steroid treatment (steroid treatment vs...
February 12, 2018: Journal of Hepato-biliary-pancreatic Sciences
https://www.readbyqxmd.com/read/29429186/-igg4-related-sclerosing-disease-with-initial-ear-symptom-a-case-report
#16
M Hu, W Wang, Y Cheng
No abstract text is available yet for this article.
February 7, 2018: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
https://www.readbyqxmd.com/read/29428100/clinical-analysis-of-liver-transplantation-in-autoimmune-liver-diseases
#17
Cheng-Peng Zhong, Zhi-Feng Xi, Qiang Xia
BACKGROUND: Autoimmune liver diseases (ALDs) consist of autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), IgG4-associated cholangitis and overlap syndromes. Patients with these diseases may gradually progress to end-stage liver diseases and need liver transplantation. The present study aimed to explore the prognosis of patients with ALDs after liver transplantation. METHODS: The clinical data of 80 patients with ALD (24 cases of AIH, 35 of PBC, 15 of PSC and 6 of AIH-PBC overlap syndromes) who underwent liver transplantation in Renji Hospital, Shanghai Jiao Tong University School of Medicine from June 2004 to September 2016 were collected retrospectively...
February 2018: Hepatobiliary & Pancreatic Diseases International: HBPD INT
https://www.readbyqxmd.com/read/29426275/primary-renal-hybrid-low-grade-fibromyxoid-sarcoma-sclerosing-epithelioid-fibrosarcoma-an-unusual-pediatric-case-with-ewsr1-creb3l1-fusion
#18
Yingting Mok, Yin Huei Pang, Jain Sudhanshi Sanjeev, Chik Hong Kuick, Kenneth Tou-En Chang
Low-grade fibromyxoid sarcoma (LGFMS) and sclerosing epithelioid fibrosarcoma (SEF) are rare tumors with distinct sets of morphological features, both characterized by MUC4 immunoreactivity. Tumors exhibiting features of both entities are considered hybrid LGFMS-SEF lesions. While the majority of LGFMS cases are characterized by FUS-CREB3L2 gene fusions, most cases of pure SEF show EWSR1 gene rearrangements. In the largest study of hybrid LGFMS-SEF tumors to date, all cases exhibited FUS rearrangements, a similar genetic profile to LGFMS...
January 1, 2018: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29425397/cholangiocyte-derived-exosomal-long-noncoding-rna-h19-promotes-cholestatic-liver-injury-in-mouse-and-human
#19
Xiaojiaoyang Li, Runping Liu, Zhiming Huang, Emily C Gurley, Xuan Wang, Juan Wang, Hongliang He, Hu Yang, Guanhua Lai, Luyong Zhang, Jasmohan S Bajaj, Melanie White, William M Pandak, Phillip B Hylemon, Huiping Zhou
Cholestatic liver injury is an important clinical problem with limited understanding of disease pathologies. Exosomes are small extracellular vesicles released by a variety of cells including cholangiocytes. Exosome-mediated cell-cell communication can modulate various cellular functions by transferring a variety of intracellular components to target cells. Our recent studies indicate that the long non-coding RNA H19 is mainly expressed in cholangiocytes and its aberrant expression is associated with significant down-regulation of small heterodimer partner (SHP) in hepatocytes and cholestatic liver injury in multidrug resistance 2 knockout (Mdr2-/- ) mice...
February 9, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29420106/syncytial-variant-of-nodular-sclerosing-hodgkin-lymphoma-in-children-a-prognostic-factor
#20
Noa Granot, Ayelet Ben-Barak, Yael Fisher, Hana Golan, Myriam Weyl Ben-Arush
Nodular sclerosing Hodgkin lymphoma (HL) has an excellent prognosis in children. The syncytial variant (SV) of HL in adults represents a clinic pathologic entity with a worse outcome. We report the clinical features and the course of the disease of three children with refractory HL. The three patients with SV were analyzed in a retrospective multi-institutional study conducted in Israel in 51 children diagnosed with refractory or recurrent HL between 1997 and 2014. All the three children developed multiple recurrences soon after diagnosis...
February 8, 2018: Pediatric Hematology and Oncology
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