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Gastroenterology
Published in Kristen Davies, Shadaba Ahmed, Core Conditions for Medical and Surgical Finals, 2020
Primary sclerosing cholangitis: Autoimmune condition causing inflammation and fibrosis of the bile ducts. Patients may be asymptomatic but may develop liver failure. Associated with IBD (especially UC). Blood tests show positive ANCA. At risk of developing cholangiocarcinoma. Liver transplantation is the only effective treatment.
Mid Common Bile Duct Cholangiocarcinoma Involving the Portal Vein and Right Branch of the Hepatic Artery
Published in Savio George Barreto, Shailesh V. Shrikhande, Dilemmas in Abdominal Surgery, 2020
Charles W. Kimbrough, Timothy M. Pawlik
Overall, cholangiocarcinoma accounts for approximately 3% of gastrointestinal malignancies. The incidence is relatively low among Western nations and is highest in Southeast Asia. The majority of tumors develop sporadically and most known risk factors are associated with chronic inflammation of the biliary tract. Primary sclerosing cholangitis represents a major risk factor in the West, as up to 20% of patients can develop cholangiocarcinoma. Additional risk factors include chronic choledocholithiaisis, hepatolithiasis, choledochal cysts, parasitic liver flukes, and exposure to certain hepatotoxins. Cholangiocarcinoma is also linked to genetic disorders such as Lynch syndrome and biliary papillomatosis.
Non-viral liver disease
Published in Michael JG Farthing, Anne B Ballinger, Drug Therapy for Gastrointestinal and Liver Diseases, 2019
John ML Christie, Roger WG Chapman
The diagnosis is made by cholangiography since liver histology is only diagnostic in one-third of cases. The generally accepted diagnostic criteria of primary sclerosing cholangitis are: Generalized beading and stenosis of the biliary system on cholangiographyAbsence of choleocholithiasis or a history of bile duct surgeryExclusion of bile duct cancer, usually by prolonged follow-up
An update on novel pharmacological agents for primary sclerosing cholangitis
Published in Expert Opinion on Therapeutic Targets, 2022
Camilla Gallo, Bright Oworae Howardson, Laura Cristoferi, Marco Carbone, M. Eric Gershwin, Pietro Invernizzi
Primary sclerosing cholangitis is a rare, wide clinical spectrum, chronic cholestatic disease characterized by inflammation and fibrosis of the biliary tree that naturally evolves toward cirrhosis. Even if the pathogenesis of PSC is still poorly understood, latest evidence proved that many different factors are implied: genetic background predisposing toward autoimmune reactions, intestinal dysbiosis, and increased permeability of intestinal mucosa demonstrated to play a fundamental role. Latest knowledge on PSC etiopathogenesis promoted experimental application of bile acids modulators, anti-inflammatory agents, and antibiotics, to arrest the disease evolution. None of the completed trials or preliminary data of ongoing trials yet showed a transplant free survival extension, but promising preclinical and clinical results are on the way, suggesting clinical and biochemical improving. Fecal microbiota transplantation may be a strategy to face with PSC intestinal dysbiosis. Dominant focal stenosis must be therapeutically approached by interventional endoscopy.
Malignancy in elective cholecystectomy due to gallbladder polyps or thickened gallbladder wall: a single-centre experience
Published in Scandinavian Journal of Gastroenterology, 2021
Dennis Björk, Wolf Bartholomä, Kristina Hasselgren, David Edholm, Bergthor Björnsson, Linda Lundgren
In the group of patients with gallbladder polyps, one (1.5%) patient with pT2 gallbladder cancer was identified and required liver and lymph node resection. The polyp measured 17 × 10 mm on preoperative ultrasound. Another three (4.6%) patients were diagnosed with malignant disease < pT1b: two patients had high-grade dysplasia/carcinoma in situ and one patient had pT1a, without further need for liver surgery and lymph node resection (see Table 3). For the total cohort, the mean preoperative polyp size was 12.3 (±7.7) mm, while the mean diameter of the resected benign polyps was 11.3 (±6.3) mm. The preoperative measurement was predominantly acquired by ultrasound. Thirty-five (53.8%) patients had multiple polyps, and of these, 19 (29.2%) showed contrast enhancement on preoperative ultrasound. All polyps with malignancy/dysplasia showed contrast enhancement. Only 11 (17.0%) patients with preoperative findings indicative of polyps had findings consistent with true polyps in their pathology reports (see Table 2). Some patients with benign disease had multiple benign diagnoses in their pathology reports. The cohort included four patients with primary sclerosing cholangitis. Their polyps measured 3, 6, 8 and 12 mm at the time of surgery, but none were found to be malignant or dysplastic. The patient with a pT2 malignancy underwent liver and lymph node resection at the same time as the cholecystectomy.
Performance of digital single-operator cholangiopancreatoscope for biliopancreatic diseases in real life: results from a prospective multicenter study
Published in Scandinavian Journal of Gastroenterology, 2020
Arthur E. Laquière, Jocelyn Privat, Fabien Fumex, Philippe Grandval, Laurence Lecomte, Laurence Curel, Arthur Belle, Félix Trottier-Tellier, Romina Urena-Campos, Guillaume Penaranda, Christian Boustière, Bertrand Napoléon
Finally, 36 subjects could have a histologic analysis of POCP-guided samples.For 19/36 patients, the initial histologic analysis of POCP-guided sample diagnosed neoplastic strictures. Among them, the final diagnosis for one patient was a false positive. A final diagnosis of primary sclerosing cholangitis was confirmed by liver biopsy and follow up.For 17/36 patients, the initial histologic analysis of POCP-guided samples diagnosed non-malignancy strictures. Among them, the final diagnoses were false negatives for six patients (five patients had cholangiocarcinoma as final diagnosis and one had hepatic metastasis of colorectal cancer).