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Primary sclerosing cholangitis-What is the difference between east and west?

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World Journal of Gastroenterology 2008年第25期14卷 3974-3981页

作者： Ali Shorbagi yusuf bayraktar Hacettepe University School of Medicine Department of Internal Medicine Gastroenterology Clinic

Primary sclerosing cholangitis （PSC） is a chronic, progressive, cholestatic liver disease characterized by inflammation and fibrotic obliteration of the hepatic biliary tree. It is commonly associated with inflammatory bowel disease （IBD）. A number of complications can occur which require special consideration, the most important of which is the development of cholangiocellular carcinoma （CCC）. Unfortunately, no medical therapy is currently available for the underlying liver disease. Liver transplantation is an effective, life-extending option for patients with advanced PSC. Geographical variations between East and West include a second peak for age with a lower association with IBD in a .lapanese population and female predominance in a lone study from Turkey. The clinical and biochemical Mayo criteria may not be universally applicable, as different patients show variations regarding the initial presentation and natural course of the disease, Directing research towards explaining these geographical differences and understanding the pathogenesis of PSC is required in order to develop better therapies for this devastating disease.

关键词： Primary sclerosing cholangitis Endoscopicretrograde cholangiopancreatography Magneticresonance cholangiopancreatography Chola-ngiocellularcarcinoma Ursodeoxycholic acid

Hepatic venous outflow obstruction:Three similar syndromes

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World Journal of Gastroenterology 2007年第13期13卷 1912-1927页

作者： Ulas Darda bayraktar Soley Seren yusuf bayraktar Department of Internal Medicine Interfaith Medical Center Brooklyn NY 11230 United States Department of Internal Medicine Wayne State University Detroit MI United States Division of Gastroenterology Hacettepe University School of Medicine Ankara Turkey MD Series Editor

Our goal is to provide a detailed review of veno- occlusive disease (VOD), Budd-Chiari syndrome (BCS), and congestive hepatopathy (CH), all of which results in hepatic venous outflow obstruction. This is the first article in which all three syndromes have been reviewed, enabling the reader to compare the characteristics of these disorders. The histological findings in VOD, BCS, and CH are almost identical: sinusoidal congestion and cell necrosis mostly in perivenular areas of hepatic acini which eventually leads to bridging fibrosis between adjacent central veins. Tender hepatomegaly with jaundice and ascites is common to all three conditions. However, the clinical presentation depends mostly on the extent and rapidity of the outflow obstruction. Although the etiology and treatment are completely different in VOD, BCS, and CH; the similarities in clinical manifestations and liver histology may suggest a common mechanism of hepatic injury and adaptation in response to increased sinusoidal pressure.

关键词： Veno-occlusive disease Sinusoidal obstru-ction syndrome Budd-Chiari syndrome Congestivehepatopathy

Etiology and consequences of thrombosis in abdominal vessels

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World Journal of Gastroenterology 2006年第8期12卷 1165-1174页

作者： yusuf bayraktar Ozgur Harmanci Hacettepe University Faculty of Medicine Department of Gastroenterology06100 SihhiyeAnkaraTurkey

The thrombophilia which can be either congenital or acquired in adult life has major implications in the abdominal vessels. The resulting portal vein thrombosis, Budd-Chiari syndrome and mesenteric vein thrombosis have a variety of consequences ranging from acute abdomen to chronic hepatomegaly and even totally asymp- tomatic patient in whom the only finding is pancytopenia. The complications like esophageal varices, portal gastropathy, ascites, severe hypersplenism, liver failure requiring liver transplantation are well known. Interesting features of collateral venous circulation showing itself as pseudocholangiocarcinoma sign and its possible clinical reflection as cholestasis are also known from a long time. The management strategies for these complications of intraabdominal vessel thrombosis are not different from their counterpart which is cirrhotic portal hypertension, but the prognosis is unquestionably better in former cases. In this review we presented and discussed the abdominal venous thrombosis, etiology and the resulting clinical pictures. There are controversial issues both in nomenclature, and management including anticoagulation problems and follow up strategies. In light of the current knowledge, we discussed some controversial issues in literature and presented our experience and our proposals about this group of patients.

关键词： Portal vein thrombosis Pseudocholangiocarcinoma sign Thrombophilia Budd-Chiari syndrome Mesenteric vein thrombosis

Cholangiocarcinoma:A compact review of the literature

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World Journal of Gastroenterology 2008年第42期14卷 6458-6466页

作者： Yucel Ustundag yusuf bayraktar Department of Internal Medicine Gastro-enterology Clinic School of Medicine Karaelmas University Department of Internal Medicine Gastro-enterology Clinic School of Medicine Hacettepe University

Cholangiocarcinoma(CC) is a devastating cancer aris-ing from biliary ***,the incidence of this disease is increasing in Western *** tumors progress insidiously,and liver failure,biliary sepsis,malnutrition and cancer cachexia are general modes of death associated with this *** date,no established therapy for advanced dis-ease has been established or ***,our knowledge in tumor biology is increasing dramatically and new drugs are under investigation for treatment of this notorious *** clinical practice,there are better diagnostic tools in use to facilitate an earlier diagnosis of CC,at least in those patients with known risk *** is resectable for cure in only a small percentage of *** staging for vas-cular and biliary extension of CC is very important in this *** and recently endosonography seem to protect against unnecessary laparotomies in these *** the last 15 years,aggressive surgical approaches,including combined liver resec-tions and vascular reconstructive surgical expertise,have improved survival in patients with *** is contraindicated in CC cases having primary sclerosing cholangitis(PSC).Although CC was previously consid-ered a contraindication to liver transplantation,new cautious protocols,including neo-adjuvant chemora-diation therapies and staging procedures before the transplantation,have made it possible to achieve long-term survival after liver transplantation in this *** ablative therapies with photodynamic therapy,intraductal high-intensity ultrasonography and chemo-therapy-impregnated plastic biliary endoprosthesis are important steps in the palliative management of extra-hepatic *** and chemo-embolization methods are also applicable for intra-hepatic CCs as palliative modes of *** need more prospec-tive randomized controlled trials to evaluate the role of the new emerging therapies for CC patients.

关键词： Cholangiocarcinoma Primary sclerosing cholangitis Radiofrequency ablation Chemoembolization

Experience of a single center with congenital hepatic fibrosis:A review of the literature

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World Journal of Gastroenterology 2010年第6期16卷 683-690页

作者： Ali Shorbagi yusuf bayraktar Faculty of Medicine Department of Internal MedicineGastroenterology ClinicHacettepe University

Congenital hepatic fibrosis(CHF) is an autosomal recessive inherited malformation defined pathologically by a variable degree of periportal fibrosis and irregularly shaped proliferating bile *** is one of the fibropolycystic diseases,which also include Caroli disease,autosomal dominant polycystic kidney disease,and autosomal recessive polycystic kidney disease. Clinically it is characterized by hepatic fibrosis,portal hypertension,and renal cystic *** is known to occur in association with a range of both inherited and non-inherited disorders,with multiorgan involvement,as a result of ductal plate *** of the similarities in the clinical picture,it is necessary to differentiate CHF from idiopathic portal hypertension and early liver cirrhosis,for which a liver biopsy is essential. Radiological tests are important for recognizing involvement of other organ *** regards to our experience at Hacettepe University,a total of 26 patients have been diagnosed and followed-up between 1974 and 2009 with a diagnosis of *** with Caroli syndrome was the most common diagnosis,with all such patients presenting with symptoms of recurrentcholangitis and symptoms related to portal hypertension. Although portal fibrosis is known to contribute to the ensuing portal hypertension,it is our belief that portal vein cavernous transformation also plays an important role in its *** all patients with CHF portal vein morphology should be evaluated by all means since portal vein involvement results in more severe and complicated portal *** associations include the Joubert and Bardet-Biedl syndromes.

关键词： Congenital hepatic fibrosis Fibropolycystic disorders Portal hypertension Bardet Biedl syndrome

Gaucher disease:New developments in treatment and etiology

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World Journal of Gastroenterology 2008年第25期14卷 3968-3973页

作者： Ozgur Harmanci yusuf bayraktar Department of Gastro-enterology Hacettepe University Faculty of Medicine

Gaucher disease (GD) is an autosomal recessive disease which if undiagnosed or diagnosed late results in devastating complications. Because of the heterozygous nature of GD, there is a wide spectrum of clinical presentation. Clinicians should be aware of this rare but potentially treatable disease in patients who present with unexplained organomegaly, anemia, massive splenomegaly, ascites and even cirrhosis of unknown origin. The treatment options for adult type GD include enzyme replacement treatment (ERT) and substrate reduction treatment (SRT) depending on the status of the patient. Future treatment options are gene therapy and "smart molecules" which provide specifi c cure and additional treatment options. In this review, we present the key issues about GD and new developments that gastroenterologists should be aware of.

关键词： Gaucher disease Enzyme replacementtreatment Substrate reduction treatment Genetherapy Liver fibrosis

Five years' experience with capsule endoscopy in a single center

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World Journal of Gastroenterology 2009年第16期15卷 1934-1942页

作者： Taylan Kav yusuf bayraktar Department of Medicine Division of GastroenterologyHacettepe University School of Medicine

Capsule endoscopy (CE) is a novel technology that facilitates highly effective and noninvasive imaging of the small bowel. Although its effi cacy in the evaluation of obscure gastrointestinal bleeding (OGIB) has been proven in several trials, data on uses of CE in different small bowel diseases are rapidly accumulating in the literature, and it has been found to be superior to alternative diagnostic tools in a range of such diseases. Based on literature evidence, CE is recommended as a first-line investigation for OGIB after negative bi-directional endoscopy. CE has gained an important role in the diagnosis and follow-up of Crohn's disease and celiac disease and in the surveillance of small bowel tumors and polyps in selected patients. Capsule retention is the major complication, with a frequency of 1%-2%. The purpose of this review was to discuss the procedure, indications, contraindications and adverse effects associated with CE. We also review and share our five-year experience with CE in various small bowel diseases. The recently developed balloon-assisted enteroscopies have both diagnostic and therapeutic capability. At the present time, CE and balloon-assisted enteroscopies are complementary techniques in the diagnosis and management of small bowel diseases.

关键词： Capsule endoscopy Small bowel diseases Obscure gastrointestinal bleeding Crohn's disease Celiac disease Indications Contraindications

Portal hypertension due to portal venous thrombosis:Etiology, clinical outcomes

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World Journal of Gastroenterology 2007年第18期13卷 2535-2540页

作者： Ozgur Harmanci yusuf bayraktar Hacettepe University Faculty of Medicine Department of Gastroenterology Sihhiye 06100 Ankara Turkey

The thrombophilia in adult life has major implications in the hepatic vessels. The resulting portal vein thrombosis has various outcomes and complications. Esophageal varices, portal gastropathy, ascites, severe hypersplenism and liver failure needing liver transplantation are known well. The newly formed collateral venous circulation showing itself as pseudocholangicarcinoma sign and its possible clinical reflection as cholestasis are also known from a long time. The management strategies for these complications of portal vein thrombosis are not different from their counterpart which is cirrhotic portal hypertension, but the prognosis is unquestionably better in former cases. In this review we present and discuss the portal vein thrombosis, etiology and the resulting dinical pictures. There are controversial issues in nomenclature, management （including anticoagulation problems）, follow up strategies and liver transplantation. In the light of the current knowledge, we discuss some controversial issues in literature and present our experience and our proposals about this group of patients.

关键词： Portal vein thrombosis Pseudocholangiocarcinomasign, Thrombophilia

Clinical characteristics of Caroli's syndrome

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World Journal of Gastroenterology 2007年第13期13卷 1934-1937页

作者： Ozlem Yonem yusuf bayraktar Hacettepe University Faculty of Medicine Department of Gastroenterology Sihhiye 06100 Ankara Turkey Hacettepe University Faculty of Medicine Department of Gastroenterology Sihhiye 06100 Ankara Turkey MD Series Editor

Caroli's syndrome is characterized by multiple segmental cystic or saccular dilatations of intrahepatic bile ducts associated with congenital hepatic fibrosis. The clinical features of this syndrome reflect both the characteristics of congenital hepatic fibrosis such as portal hypertension and that of Caroli's disease named as recurrent cholangitis and cholelithiasis. The diagnosis depends on both histology and imaging methods which can show the communication between the sacculi and the bile ducts. Treatment consists of symptomatic treatment of cholangitis attacks by antibiotics, some endoscopic, radiological and surgical drainage procedures and surgery. Liver transplantation seems the ultimate treatment for this disease. Prognosis is fairly good unless recurrent cholangitis and renal failure develops.

关键词： Caroli's syndrome Liver transplantation Endoscopic retrograde cholangiopancreatography