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https://www.readbyqxmd.com/read/28633199/diffuse-type-caroli-disease-with-characteristic-central-dot-sign-complicated-by-multiple-intrahepatic-and-common-bile-duct-stones
#1
Moon Joo Hwang, Tae Nyeun Kim
Caroli disease (CD) is a rare congenital malformation of the liver characterized by non-obstructive, segmental, cystic dilatation of the intrahepatic bile ducts (IHDs). The clinical course is usually asymptomatic for the first 5-20 years, and symptoms may seldom occur throughout the patient's life. Bile stagnation leads to recurrent episodes of cholangitis, stone formation, or liver abscesses, and biliary cirrhosis usually occurs years later. Here we report on a 42-year-old man diagnosed with diffuse-type CD with a characteristic central dot sign, who had multiple intrahepatic and common bile duct (CBD) stones...
June 20, 2017: Clinical Endoscopy
https://www.readbyqxmd.com/read/28630805/dermatitis-herpetiformis-as-the-initial-presentation-of-primary-biliary-cholangitis-in-a-male-with-gluten-sensitivity
#2
Cyriac Philips, Rajaguru Paramaguru, Divya A Indiran, Philip Augustine
Celiac disease is commonly associated with elevated liver enzymes that normalize on a gluten-free diet. Celiac disease is rarely described in patients with primary biliary cholangitis. Dermatitis herpetiformis is the skin manifestation of the celiac disease that is very rarely associated with primary biliary cirrhosis. We present the case of a 62-year-old man who presented with severe chronic pruritus, in whom a diagnosis of primary biliary cholangitis was made initially. However, in the presence of atypical skin lesions, not confirming to chronic cholestasis, an in-depth evaluation including histopathological examination led to the diagnosis of dermatitis herpetiformis associated with gluten sensitivity...
May 14, 2017: Curēus
https://www.readbyqxmd.com/read/28630443/chemical-chaperone-tudca-unlike-pba-mitigates-protein-aggregation-efficiently-and-resists-er-and-non-er-stress-induced-hepg2-cell-death
#3
Jagadeesh Kumar Uppala, Amina R Gani, Kolluru V A Ramaiah
Stress induced BSA (bovine serum albumin) protein aggregation is effectively mitigated in vitro by TUDCA (tauroursodeoxycholic acid) than by PBA (4- phenylbutyric acid), chemical chaperones approved by FDA for the treatment of biliary cirrhosis and urea cycle disorders respectively. TUDCA, unlike PBA, enhances trypsin mediated digestion of BSA. TUDCA activates PERK, an ER-resident kinase that phosphorylates the alpha-subunit of eukaryotic initiation factor2 (eIF2α) and promotes the expression of activated transcription factor 4 (ATF4) in HepG2 cells...
June 19, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28624334/seasonality-and-autoimmune-diseases-the-contribution-of-the-four-seasons-to-the-mosaic-of-autoimmunity
#4
REVIEW
Abdulla Watad, Shir Azrielant, Nicola Luigi Bragazzi, Kassem Sharif, Paula David, Itay Katz, Gali Aljadeff, Mariana Quaresma, Galya Tanay, Mohammad Adawi, Howard Amital, Yehuda Shoenfeld
Autoimmune diseases (ADs) are a heterogeneous groups of diseases that occur as a results of loss of tolerance to self antigens. While the etiopathogeneis remain obscure, different environmental factors were suggested to have a role in the development of autoimmunity, including infections, low vitamin D levels, UV radiation, and melatonin. Interestingly, such factors possess seasonal variation patterns that could influence disease development, severity and progression. Vitamin D levels which reach a nadir during late winter and early spring is correlated with increased disease activity, clinical severity as well as relapse rates in several disease entities including multiple sclerosis (MS), non-cutaneous flares of systemic lupus erythematosus (SLE), psoriasis, and rheumatoid arthritis (RA)...
June 14, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28614198/the-almost-normal-liver-biopsy-presentation-clinical-associations-and-outcome
#5
Thomas W Czeczok, John S Van Arnam, Laura D Wood, Michael S Torbenson, Taofic Mounajjed
Liver biopsies obtained for abnormal liver enzymes or unexplained ascites occasionally appear histologically almost normal. The differential diagnosis for these cases is challenging because literature addressing this topic is lacking. We aimed to establish a differential diagnosis and determine clinical associations and outcomes for almost-normal liver biopsies. Ninety-seven histologically almost-normal liver biopsies were collected from 2 institutions. All cases lacked significant inflammation, fatty change, biliary tract disease, vascular disease, nodular regenerative hyperplasia, iron overload, inherited metabolic or storage disorder, viral hepatitis, or fibrosis...
June 13, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28602220/inhibition-of-microrna-24-increases-liver-fibrosis-by-enhanced-menin-expression-in-mdr2-mice
#6
Chad Hall, Laurent Ehrlich, Fanyin Meng, Pietro Invernizzi, Francesca Bernuzzi, Terry C Lairmore, Gianfranco Alpini, Shannon Glaser
BACKGROUND: Liver transplantation remains the primary treatment for primary sclerosing cholangitis (PSC). Mdr2(-/-) mice provide a reliable in vivo model of PSC and develop characteristic biliary inflammation and fibrosis. We tested the hypothesis that the tumor suppressor protein menin is implicated in the progression of liver fibrosis and that menin expression can be regulated in the liver via microRNA-24 (miR-24). MATERIALS AND METHODS: Menin expression was measured in human PSC and Mdr2(-/-) mice...
May 11, 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/28597680/fishbone-impaction-in-the-colon
#7
Armando Peixoto, Marco Silva, Branca Órfão, Guilherme Macedo
Postoperative benign biliary stricture in the anastomotic site is one of the most common complications of biliary-enteric anastomosis, with a rate of 6.87% after 2-13 years of follow-up. If untreated, biliary strictures can induce other complications such as recurrent cholangitis, intrahepatic stones, pancreatitis and secondary biliary cirrhosis. We report our experience with extracorporeal shock wave lithotripsy (ESWL) in a patient with a massive symptomatic stone proximal to an anastomotic biliary stricture...
June 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28597679/successful-extracorporeal-shock-wave-lithotripsy-eswl-treatment-of-a-symptomatic-massive-biliary-stone-proximal-to-an-anastomotic-biliary-stricture
#8
Rosangela Muratori, Daniele Mandolesi, Maria Cristina Galaverni, Francesco Azzaroli
Postoperative benign biliary stricture in the anastomotic site is one of the most common complications of biliary-enteric anastomosis, with a rate of 6.87% after 2-13 years of follow-up. If untreated, biliary strictures can induce other complications such as recurrent cholangitis, intrahepatic stones, pancreatitis and secondary biliary cirrhosis. We report our experience with extracorporeal shock wave lithotripsy (ESWL) in a patient with a massive symptomatic stone proximal to an anastomotic biliary stricture...
June 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28583540/living-donor-liver-transplantation-for-progressive-familial-intrahepatic-cholestasis-type-1-two-reported-cases
#9
Y Oya, Y Sugawara, M Honda, D Yoshii, K Isono, S Hayashida, H Yamamoto, Y Inomata
BACKGROUND: Progressive familial intrahepatic cholestasis type 1 (PFIC1) is an inherited disease characterized by cholestatic features. We report two patients with PFIC1 who underwent liver retransplantation. CASE REPORT: One patient was a 3-year-old female who underwent liver transplantation for PFIC1. She presented with severe diarrhea and fatty liver, and went into liver failure. She therefore underwent liver retransplantation and external biliary diversion 8 years after the initial liver transplantation...
June 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28580726/the-first-case-of-domino-split-liver-transplantation-in-maple-syrup-urine-disease
#10
Uta Herden, Jun Li, Lutz Fischer, Florian Brinkert, Martin Blohm, René Santer, Bjoern Nashan, Enke Grabhorn
The enzymatic defect in MSUD results in accumulation of neurotoxic metabolites of BCAAs. LTX has shown to be a feasible strategy in patients non-responsive to diet. Because of sufficient enzyme activity in extrahepatic tissues in healthy people, the MSUD liver graft is a suitable domino organ. We present the first case of a technical challenging ex situ split of a MSUD domino organ transplanted into two pediatric recipients. The domino graft donor was a 21-year-old female (58 kg) suffering from MSUD with recurrent metabolic decompensation despite strict diet...
June 5, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28570655/circulating-fgf19-closely-correlates-with-bile-acid-synthesis-and-cholestasis-in-patients-with-primary-biliary-cirrhosis
#11
Zhanyi Li, Bingliang Lin, Guoli Lin, Yuankai Wu, Yusheng Jie, Xiangyong Li, Brian Ko, Yutian Chong, Jian Luo
BACKGROUND AND AIM: Bile acid (BA) synthesis in the liver is regulated by Fibroblast Growth Factor 19 (FGF19) secreted from the ileum as an enterohepatic feedback mechanism. Although FGF19 mRNA is absent in normal liver, FGF19 gene expression was reported to increase in response to both extrahepatic and intrahepatic cholestasis. The impact of upregulated FGF19 expression on BA synthesis is unclear and the overall role of circulating FGF19 and BA synthesis under cholestatic conditions needs to be further investigated...
2017: PloS One
https://www.readbyqxmd.com/read/28567454/distal-pancreatectomy-with-splenorenal-shunt-to-preserve-spleen-in-a-cirrhotic-patient
#12
Giuseppe Maria Ettorre, Giovanni Battista Levi Sandri, Marco Colasanti, Edoardo de Werra, Pasquale Lepiane
At pancreatic ductal adenocarcinoma is an aggressive malignancy with a high recurrence rate. Due to its high potentials of local invasion and distant metastasis, surgical resection is the only means for possible long-term survival. Surgical treatment comprises a distal pancreatectomy with or without splenectomy. Surgery has been conventionally contraindicated for patients with cirrhosis and portal vein hepato-biliary hypertension. Splenorenal shunt was first described by Warren and colleagues, to prevent death from bleeding esophageal varices in a patient with a patent portal vein hypertension...
May 2017: Annals of Hepato-Biliary-Pancreatic Surgery
https://www.readbyqxmd.com/read/28566919/xanthoma-striatum-palmare-in-a-patient-of-primary-biliary-cirrhosis-with-autoimmune-hepatitis
#13
Min-Young Yang, Jeong-Min Kim, Gun-Wook Kim, Hoon-Soo Kim, Byung-Soo Kim, Moon-Bum Kim, Hyun-Chang Ko
No abstract text is available yet for this article.
June 2017: Annals of Dermatology
https://www.readbyqxmd.com/read/28554673/secondary-sclerosing-cholangitis-in-cardiac-surgical-patients-a-complication-with-a-dismal-prognosis
#14
Ina Schade, Dejan Radakovic, Jörg Hoffmann, Sebastian-Patrick Sommer, Ullrich Stefenelli, Christoph Schimmer, Rainer G Leyh, Ivan Aleksic
OBJECTIVES: Secondary sclerosing cholangitis in critically ill patients is a rapidly progressing disease leading to biliary fibrosis and cirrhosis. We describe the course of sclerosing cholangitis in critically ill patients after cardiac surgery and compare this with matched patients. METHODS: A retrospective search for "secondary sclerosing cholangitis" and "liver and/or hepatic failure" in all adult patients (aged 18-93 years) who underwent cardiac surgery from April 2007 to March 2016 identified 192 of 8625 patients...
April 27, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28547740/biliary-duodenal-anastomosis-using-magnetic-compression-following-massive-resection-of-small-intestine-due-to-strangulated-ileus-after-living-donor-liver-transplantation-a-case-report
#15
Ryusuke Saito, Hiroyuki Tahara, Seiichi Shimizu, Masahiro Ohira, Kentaro Ide, Kohei Ishiyama, Tsuyoshi Kobayashi, Hideki Ohdan
BACKGROUND: Despite the improvements of surgical techniques and postoperative management of patients with liver transplantation, biliary complications are one of the most common and important adverse events. We present a first case of choledochoduodenostomy using magnetic compression following a massive resection of the small intestine due to strangulated ileus after living donor liver transplantation. CASE PRESENTATION: The 54-year-old female patient had end-stage liver disease, secondary to liver cirrhosis, due to primary sclerosing cholangitis with ulcerative colitis...
December 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/28546061/primary-biliary-cholangitis-advances-in-management-and-treatment-of-the-disease
#16
Pietro Invernizzi, Annarosa Floreani, Marco Carbone, Marco Marzioni, Antonio Craxi, Luigi Muratori, Umberto Vespasiani Gentilucci, Ivan Gardini, Antonio Gasbarrini, Paola Kruger, Francesco Saverio Mennini, Virginia Ronco, Elena Lanati, Pier Luigi Canonico, Domenico Alvaro
Primary Biliary Cholangitis, previously known as Primary Biliary Cirrhosis, is a rare disease, which mainly affects women in their fifth to seventh decades of life. It is a chronic autoimmune disease characterized by a progressive damage of interlobular bile ducts leading to ductopenia, chronic cholestasis and bile acids retention. Even if the disease usually presents a long asymptomatic phase and a slow progression, in many patients it may progress faster toward cirrhosis and its complications. The 10year mortality is greater than in diseases such as human immunodeficiency virus/Hepatitis C Virus coinfection and breast cancer...
May 8, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28545547/clonorchis-sinensis-granulin-identification-immunolocalization-and-function-in-promoting-the-metastasis-of-cholangiocarcinoma-and-hepatocellular-carcinoma
#17
Caiqin Wang, Huali Lei, Yanli Tian, Mei Shang, Yinjuan Wu, Ye Li, Lu Zhao, Mengchen Shi, Xin Tang, Tingjin Chen, Zhiyue Lv, Yan Huang, Xiaoping Tang, Xinbing Yu, Xuerong Li
BACKGROUND: Long-term infections by Clonorchis sinensis are associated with cholangitis, cholecystitis, liver fibrosis, cirrhosis, and even liver cancer. Molecules from the worm play vital roles in disease progress. In the present study, we identified and explored molecular characterization of C. sinensis granulin (CsGRN), a growth factor-like protein from C. sinensis excretory/secretory products (CsESPs). METHODS: The encoding sequence and conserved domains of CsGRN were identified and analysed by bioinformatics tools...
May 25, 2017: Parasites & Vectors
https://www.readbyqxmd.com/read/28545082/clinical-endoscopic-management-and-outcome-of-post-endoscopic-sphincterotomy-bleeding
#18
Wei-Chen Lin, Hsaing-Hung Lin, Chien-Yuan Hung, Shou-Chuan Shih, Cheng-Hsin Chu
Post-endoscopic sphincterotomy bleeding is a common complication of biliary sphincterotomy, and the incidence varies from 1% to 48%. It can be challenging to localize the bleeder or to administer various interventions through a side-viewing endoscope. This study aimed to evaluate the risk factors of post-endoscopic sphincterotomy bleeding and the outcome of endoscopic intervention therapies. We retrospectively reviewed the records of 513 patients who underwent biliary sphincterotomy in Mackay Memorial Hospital between 2011 and 2016...
2017: PloS One
https://www.readbyqxmd.com/read/28540784/safety-and-efficacy-of-radiofrequency-assisted-alpps-ralpps-in-patients-with-cirrhosis-related-hepatocellular-carcinoma
#19
Qiang Wang, Jun Yan, Xiaobin Feng, Geng Chen, Feng Xia, Xiaowu Li, Kuansheng Ma, Ping Bie
BACKGROUND AND AIMS: Associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) has high morbidity and mortality. In this study, the safety and efficacy of a modification of ALPPS (radiofrequency-assisted ALPPS, RALPPS) were assessed in patients with hepatocellular carcinoma (HCC). MATERIALS AND METHODS: Patients who were diagnosed with HCC and were considered to have an insufficient future liver remnant (FLR) were enrolled. In stage I, a radiofrequency ablation (RFA) device was used to cauterise along the planned transection plane to form a coagulum avascular area...
March 23, 2017: International Journal of Hyperthermia
https://www.readbyqxmd.com/read/28538834/characteristics-of-liver-fibrosis-with-different-etiologies-using-a-fully-quantitative-fibrosis-assessment-tool
#20
Q Wu, X Zhao, H You
This study aimed to test the diagnostic performance of a fully quantitative fibrosis assessment tool for liver fibrosis in patients with chronic hepatitis B (CHB), primary biliary cirrhosis (PBC) and non-alcoholic steatohepatitis (NASH). A total of 117 patients with liver fibrosis were included in this study, including 50 patients with CHB, 49 patients with PBC and 18 patients with NASH. All patients underwent liver biopsy (LB). Fibrosis stages were assessed by two experienced pathologists. Histopathological images of LB slices were processed by second harmonic generation (SHG)/two-photon excited fluorescence (TPEF) microscopy without staining, a system called qFibrosis (quantitative fibrosis) system...
May 18, 2017: Brazilian Journal of Medical and Biological Research, Revista Brasileira de Pesquisas Médicas e Biológicas
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