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John Knight - One of the best experts on this subject based on the ideXlab platform.
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intrahepatic Bile Duct Dilatation and cholestasis in autosomal recessive polycystic kidney disease demonstration with hepatobiliary scintigraphy
Clinical Nuclear Medicine, 1995Co-Authors: Karen Waters, Robert Howmangiles, Monica A Rossleigh, A H Lam, Roger F Uren, John KnightAbstract:Fifteen patients with the clinical and radiologic features of autosomal recessive polycystic kidney disease were investigated with radionuclide hepatobiliary scintigraphy. In nine patients (60%), cholestasis and intrahepatic Bile Duct Dilatation were demonstrated. A 10th child had scintigraphic evidence of cholestasis, but the Bile Ducts were not dilated. The other five children has normal h hepatobiliary scans. The authors conclude that intrahepatic Bile Duct Dilatation with cholestasis (Caroli's disease) is part of the clinical spectrum of autosomal recessive polycystic kidney disease and that hepatobiliary scintigraphy can be of value in determining the extent of hepatobiliary disease in this group of patients.
Hisami Ando - One of the best experts on this subject based on the ideXlab platform.
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diagnostic criteria for congenital biliary Dilatation 2015
Journal of Hepato-biliary-pancreatic Sciences, 2016Co-Authors: Yoshinori Hamada, Hisami Ando, Terumi Kamisawa, Takao Itoi, Naoto Urushihara, Tsugumichi Koshinaga, Takeshi Saito, Hideki Fujii, Yoshiki MorotomiAbstract:Background The Diagnostic Criteria for Pancreaticobiliary Maljunction 2013 were published by the Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM) in 2014. The committee of JSGPM for diagnostic criteria for pancreaticobiliary maljunction has established the standard diameter of the Bile Duct, and a definition of Dilatation of the Bile Duct was proposed in 2014. Methods The committee of JSGPM prepared the diagnostic criteria for congenital biliary Dilatation in 2014, and a final revised version was approved in 2015. Results Congenital biliary Dilatation is defined as a congenital malformation involving both local Dilatation of the extrahepatic Bile Duct, including the common Bile Duct, and pancreaticobiliary maljunction. However, cases associated with intrahepatic Bile Duct Dilatation can also be included. Various kinds of pathological conditions can occur on hepatobiliary systems and pancreas by Bile Duct Dilatation and pancreaticobiliary maljunction. For a diagnosis of congenital biliary Dilatation, both abnormal Dilatation of the Bile Duct and pancreaticobiliary maljunction must be evident by either imaging test or anatomical examination. Acquired or secondary Dilatation of the Bile Duct by obstruction due to biliary stones or malignancy should be strictly excluded. Conclusion Diagnostic criteria for congenital biliary Dilatation 2015 were established from Japan representing a world first.
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pancreaticobiliary maljunction without Bile Duct Dilatation in children distinction from choledochal cyst
Journal of Pediatric Gastroenterology and Nutrition, 2008Co-Authors: Yasuyuki Ono, Kenitiro Kaneko, Takahisa Tainaka, Wataru Sumida, Hisami AndoAbstract:ABSTRACTObjectives:Pancreaticobiliary maljunction without Bile Duct Dilatation (nondilated type) is rare in children, and its definition remains unclear. There is controversy over treatment between pediatric and adult patients. We reevaluated our previous definition of the nondilated type in childre
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pathologic changes in the common Bile Duct of an experimental model with pancreaticobiliary maljunction without biliary Dilatation
Pediatric Surgery International, 2000Co-Authors: Kenichiro Kaneko, Hisami Ando, Yoshio Watanabe, Takahiko Seo, Tohru HaradaAbstract:Pancreaticobiliary maljunction (PBM), a congenital anomaly, causes regurgitation of pancreatic juice into the biliary tract, where it exerts a hazardous influence. However, changes in the common Bile Duct (CBD) remain obscure due to a lack of suitable experimental models. Using cats, we have developed an experimental model of PBM without Bile-Duct Dilatation that allows the pure effects of PBM to be studied. Histologic and cellular kinetic changes in the CBD were analyzed in 6 controls and 9 experimental animals that survived for more than 6 months. CBD sections were stained with hematoxylin and eosin and a monoclonal antibody to the proliferating cell nuclear antigen (PCNA). Invaginations of the Bile-Duct epithelium or parietal sacculi increased, and peribiliary glands were well-developed. PCNA-positive cells significantly increased in the CBD, especially in the parietal sacculi and glands. It is concluded that PBM increases the cell cycle in CBD epithelium and subsequently developed peribiliary glands. These developed glands may be associated with the formation of protein plugs, often seen in patients with PBM.
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a new model for pancreaticobiliary maljunction without Bile Duct Dilatation demonstration of cell proliferation in the gallbladder epithelium
Journal of Surgical Research, 1996Co-Authors: Kenitiro Kaneko, Osamu Murahashi, Katsumasa Hiraiwa, Norihiro Niimi, Takashi Umeda, Hisami Ando, Moazzem HossainAbstract:Patients with pancreaticobiliary maljunction without Bile Duct Dilatation (PBMWBDD) develop gallbladder carcinoma frequently. No models of PBMWBDD exist, and no previous studies have clearly demonstrated changes in the gallbladder relating to carcinogenesis. We, therefore, examined the cell kinetics of the gallbladder epithelium in a new experimental model of PBMWBDD. A cat model was produced by performing choledocho-pancreatic side-to-side Ductal anastomosis in nine animals. Five cats, in which the choledocho-pancreatic Ducts were exposed only, served as controls. After 6 months, the gallbladders of these cats were removed and stained with anti-proliferating cell nuclear antigen (PCNA) antibody (PC10, Dako). The labeling index (LI) was determined from the percentage of positive nuclei in three microscopic fields. The diameter of the common Bile Duct was not different between the models and the controls. In the models, the number of PCNA positive cells was significantly increased. The mean (± standard deviation) PCNA LI was 28.1 ± 12.2% in the models and 4.3 ± 1.6% in the controls (P< 0.01). These studies clearly indicate that this model is suitable for studying PBMWBDD and that PBMWBDD has a prominent proliferating effect on the gallbladder epithelium which may play an important role in gallbladder carcinogenesis.
Blanche M Chavers - One of the best experts on this subject based on the ideXlab platform.
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morbidity from congenital hepatic fibrosis after renal transplantation for autosomal recessive polycystic kidney disease
American Journal of Transplantation, 2002Co-Authors: Khalid Khan, Sara Jane Schwarzenberg, Harvey L Sharp, Arthur J Matas, Blanche M ChaversAbstract:Presentation of autosomal recessive polycystic kidney disease (ARPKD) ranges from severe renal impairment and a high mortality rate in infancy to older children and adolescents with minimal renal disease and complications of congenital hepatic fibrosis (CHF), cholangitis and portal hypertension. Renal transplantation improves prognosis but it is unclear whether CHF in transplanted children follows the same clinical course as in older children with less severe renal disease. The aim of this study was to evaluate morbidity from CHF in ARPKD post renal transplantation. Data were analyzed for six males and eight females, transplanted for ARPKD (mean age 8.3 years, range 1-22.3 years) at the University of Minnesota between 1972 and 1998. Follow-up was for a mean of 14.5 years (range 3.1-33.6 years). One and 5 years patient survival rates were 93% and 86%, respectively. Overall five patients (36%) died; 4/5 deaths were related to CHF. Causes of death were hepatic failure immediately post transplant (n = 1), septicemia related to Bile Duct Dilatation (n = 3) and multiorgan failure (n = 1). One and 5years graft survival rates were 87% and 70%, respectively. One patient had a combined liver-kidney transplant and two were re-transplanted. Initial signs of CHF were splenomegaly (n = 5), hepatosplenomegaly (n = 4) and gastrointestinal bleed (n = 2). Progression of CHF through childhood included hypersplenism (n = 7), esophageal varices with gastrointestinal bleeding (n = 5) and Bile Duct Dilatation (n = 5). Portal hypertension was treated with portosystemic shunt (n = 3), sclerotherapy (n = 2), banding of varices (n = 1) and transjugular intrahepatic portosystemic shunt (n = 1). Of the nine survivors (mean age 12.8 years) 78% have functioning grafts (one liver-kidney transplant), 63% have portal hypertension and 22% have asymptomatic biliary Dilatation. Complications of CHF developed in 79% of children who received a renal transplant for ARPKD. Mortality related to CHF occurred in 29% and accounted for 80% (4/5) of the deaths.
Karen Waters - One of the best experts on this subject based on the ideXlab platform.
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intrahepatic Bile Duct Dilatation and cholestasis in autosomal recessive polycystic kidney disease demonstration with hepatobiliary scintigraphy
Clinical Nuclear Medicine, 1995Co-Authors: Karen Waters, Robert Howmangiles, Monica A Rossleigh, A H Lam, Roger F Uren, John KnightAbstract:Fifteen patients with the clinical and radiologic features of autosomal recessive polycystic kidney disease were investigated with radionuclide hepatobiliary scintigraphy. In nine patients (60%), cholestasis and intrahepatic Bile Duct Dilatation were demonstrated. A 10th child had scintigraphic evidence of cholestasis, but the Bile Ducts were not dilated. The other five children has normal h hepatobiliary scans. The authors conclude that intrahepatic Bile Duct Dilatation with cholestasis (Caroli's disease) is part of the clinical spectrum of autosomal recessive polycystic kidney disease and that hepatobiliary scintigraphy can be of value in determining the extent of hepatobiliary disease in this group of patients.
Masao Tanaka - One of the best experts on this subject based on the ideXlab platform.
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long term results of treatment for pancreaticobiliary maljunction without Bile Duct Dilatation
Archives of Surgery, 2006Co-Authors: Jiro Ohuchida, Kazuo Chijiiwa, Masahide Hiyoshi, Kiichiro Kobayashi, Hiroyuki Konomi, Masao TanakaAbstract:Hypothesis Resection of the gallbladder together with the dilated Bile Duct is the preferred treatment for pancreaticobiliary maljunction (PBM) with Bile Duct Dilatation, whereas this treatment for PBM without Bile Duct Dilatation is still controversial. Design Retrospective study of 196 patients from January 1979 to November 2004. Setting Two university hospitals. Patients One hundred ninety-six patients with PBM, 152 (78%) with and 44 (22%) without Bile Duct Dilatation, formed the basis of this study. Main Outcome Measures The effects of cholecystectomy on long-term results in the patients without Bile Duct Dilatation. Results Significant differences were observed in patients without Bile Duct Dilatation: patients were older, carcinoma of the gallbladder was more prevalent (19 patients [43.2%] without Dilatation vs 9 patients [5.9%] with Dilatation), and pancreatic cancer and pancreatitis were also more frequent. Most of their gallbladder carcinomas were found at stage IV (63%). The outcome was very poor in stage IV, whereas 5 patients in stage I and II lived for more than 5 years after surgery. Of the 44 patients without Bile Duct Dilatation, 23 with carcinoma of the gallbladder or pancreas died and the other 2 were lost to follow-up. The remaining 19 patients were alive at the study's conclusion after cholecystectomy without Bile Duct resection. None of them had Bile Duct carcinoma at the time of surgery or during the mean follow-up period of 9 years after surgery. Conclusions Prophylactic cholecystectomy without Bile Duct resection is the best treatment option for patients with PBM without Bile Duct Dilatation. Possible association of gallbladder carcinoma should be kept in mind at the time of treatment of patients with PBM when the Bile Duct is not dilated.