[Show abstract][Hide abstract] ABSTRACT: Sclerosing cholangitis, an uncommon disorder in children, is progressive and is, therefore, an important indication for pediatric liver transplantation. This review summarizes current challenges in the diagnosis and treatment of this rare form of pediatric liver disease.
[Show abstract][Hide abstract] ABSTRACT: A 25-year-old woman was referred to our hospital with persistent upper abdominal pain. Preoperative imaging studies revealed a hilar bile duct stricture with portal venous encasement, and the patient underwent curative resection involving extended left hepatectomy and segmental portal vein resection. The pathological findings demonstrated a well-differentiated tubular adenocarcinoma of the bile duct with regional lymph node metastasis (stage IIIB according to the UICC TNM classification), as well as the overexpression of p53 proteins and the K-ras gene mutation in tumor cells. The patient has shown no evidence of recurrence in the 10 months since the operation. Although there are several reports of relatively young adults with cholangiocarcinoma, the majority of such patients demonstrate either an anomalous arrangement of the pancreaticobiliary duct system or primary sclerosing cholangitis. The absence of any morphological abnormalities in this patient's biliary system implicates de novo carcinogenesis as the most likely cause of the cholangiocarcinoma.
Surgery Today 04/2013; 44(7). DOI:10.1007/s00595-013-0574-9 · 1.21 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: BACKGROUND: The epidemiology and natural history of pediatric primary sclerosing cholangitis (PSC), autoimmune sclerosing cholangitis (ASC) and autoimmune hepatitis (AIH) are not well characterized. METHODS: Using multiple overlapping search strategies followed by detailed records review, we identified all cases of pediatric PSC, ASC, AIH and inflammatory bowel disease (IBD) in a geographically-isolated region of the United States. RESULTS: We identified 607 cases of IBD, 29 cases of PSC, 12 cases of ASC, and 44 cases of AIH. The mean age at diagnosis was 13.0 years for PSC, 11.3 years for ASC and 9.8 years for AIH. The incidence and prevalence of PSC was 0.2 and 1.5, of ASC was 0.1 and 0.6, and of AIH was 0.4 and 3.0 cases per 100,000 children, respectively. Mean duration of follow-up was 5.9 years. The probability of developing complicated liver disease within 5 years of diagnosis of liver disease was 37% (95%CI 21-58) for PSC, 25% (95%CI 7-70) for ASC and 15% (95%CI 7-33) for AIH. Five-year survival with native liver was 78% (95%CI 54-91) for PSC, 90% (95%CI 47-99) for ASC and 87% (95%CI 71-95) for AIH. Cholangiocarcinoma developed in 2/29(6.9%) PSC patients. PSC occurred in 9.9% ulcerative colitis (UC) and 0.6% Crohn's disease(CD) patients. ASC occurred in 2.3% UC and 0.9% CD patients. AIH occurred in 0.4% UC and 0.3% CD patients. Liver disease occurred in 39/607(6.4%) of IBD patients overall. CONCLUSION: Immune mediated liver diseases are important sources of morbidity in children. Using a population based design, this study quantifies the burden and natural history of immune mediated liver disease in children. (HEPATOLOGY 2013.).
Matthias Barral, Anthony Dohan, Matthieu Allez, Mourad Boudiaf, Marine Camus, Valérie Laurent, Christine Hoeffel, Philippe Soyer
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