Ozgur Harmanci

Is Congenital Hepatic Fibrosis a Pure Liver Disease

OBJECTIVES:An association between congenital hepatic fibrosis (CHF) and several different conditions is being increasingly recognized. We aimed to investigate, prospectively, these associated disorders and the clinical consequences for patients with CHF.MATERIALS AND METHODS:CHF was diagnosed using liver biopsy, abdominal ultrasound (US), Doppler US, upper endoscopy, and abdominal computed tomography (CT) in 19 patients (13 women, 6 men). CT portography and splenoportography with digital subtraction angiography were performed if indicated. Endoscopic retrograde cholangiopancreatography (ERCP) was performed to investigate the extent of portal vein involvement of the common bile duct if it existed, to remove a stone located in the common bile duct when documented, and to confirm the diagnosis of Carolis syndrome. Cranial MRI was done when clinical findings suggested brain involvement.RESULTS:The mean age of the patients was 29.47 ± 12.06, ranging from 13 to 57. CHF-associated diseases were Carolis syndrome, polycystic kidney disease, cavernous transformation of the portal vein, Jouberts syndrome, von Meyenburg complex, polydactyly, medullary sponge kidney, and pancreatic duct atrophy. In two cases, cholangiocarcinoma had developed. There was only one case with pure CHF. Portosystemic shunt, TIPS, or splenectomy were performed in some cases to control bleeding from esophageal varices. Papillotomy and stone extraction from the common bile duct were performed in four patients with Carolis syndrome complicated by cholangitis. Three patients died of complications of CHF. Two patients with Carolis syndrome underwent liver transplantation.CONCLUSION:In this prospective study, it seems that CHF is not a pure liver disease but rather a multiorgan disorder involving the brain, portal vein, kidneys, and bile ducts. In most cases, the clinical picture includes other organ involvement, rather than purely the liver parenchyma.

JAK2V617F Mutation in Patients with Portal Vein Thrombosis

In a retrospective cohort, we investigated the presence of the JAK2V617F mutation in chronic non-cirrhotic portal vein thrombosis (PVT) patients, irrespective of the presence or absence of myeloproliferative diseases (MPDs). We identified 25 patients in whom thrombophilia workup was completed. The diagnoses of MPDs were made according to the World Health Organization (WHO) criteria. JAK2V617F mutation analysis was performed by allele-specific polymerase chain reaction (PCR). There were 9 male and 16 female patients. Prior to JAK2V617F analysis, there were one or more thrombophilic risk factors in 19 patients (76%). The JAK2V617F mutation analysis revealed the presence of this mutation (all in the heterozygote state) in six patients (24%; two male, four female). Five of the six cases with prior clinical diagnosis of MPDs were found to have wild-type JAK2. We found that the addition of JAK2V617F analysis into the thrombophilia workup in patients with chronic PVT contributes to a 4% increase in the diagnosis of thrombophilic conditions.

How can portal vein cavernous transformation cause chronic incomplete biliary obstruction

Biliary disease in the setting of non-cirrhotic portal vein thrombosis (and similarly in portal vein cavernous transformation) can become a serious problem during the evolution of disease. This is mostly due to portal biliary ductopathy. There are several mechanisms that play a role in the development of portal biliary ductopathy, such as induction of fibrosis in the biliary tract (due to direct action of dilated peribiliary collaterals and/or recurrent cholangitis), loss of biliary motility, chronic cholestasis (due to fibrosis or choledocholithiasis) and increased formation of cholelithiasis (due to various factors). The management of cholelithiasis in cases with portal vein cavernous transformation merits special attention. Because of a heterogeneous clinical presentation and concomitant pathophysiological changes that take place in biliary anatomy, diagnosis and therapy can become very complicated. Due to increased incidence and complications of cholelithiasis, standard treatment modalities like sphincterotomy or balloon sweeping of bile ducts can cause serious problems. Cholangitis, biliary strictures and hemobilia are the most common complications that occur during management of these patients. In this review, we specifically discuss important issues about bile stones related to bile duct obstruction in non-cirrhotic portal vein thrombosis and present evidence in the current literature.

Potential role of lycopene in the treatment of hepatitis C and prevention of hepatocellular carcinoma.

Hepatitis C virus (HCV) infection and hepatocellular carcinoma (HCC) are growing health problems around the world. Oxidative stress plays a significant role in the initiation and progression of hepatocellular damage and possibly in the development of HCC in HCV infected patients. In vitro, animal and clinical studies suggest that lycopene, a nonprovitamin A carotenoid and a potent antioxidant, may attenuate the liver injury and possibly prevent the development of HCC. In this article, we discuss the relationship between HCV infection and oxidative stress and review the potential role of lycopene in the treatment of HCV and prevention of HCC.

The etiologic distribution of thrombophilic factors in chronic portal vein thrombosis.

Goals We aimed to prospectively investigate the full etiologic contributors to portal vein thrombosis. Background Portal vein thrombosis in the absence of liver disease is a rare cause of portal hypertension with a different clinical course and management strategy. The etiologic distribution of this interesting clinical picture is important as far as diagnostic and management issues are concerned. Study After the application of exclusion criteria, 59 patients were included in the study who had normal liver functions, normal liver histology and studied the thrombophilia factors of both acquired factors and congenital factors like protein C, protein S, antithrombin levels with the mutations. Results In all, 23.7% of the patients were found to have acquired thrombophilia factors like myeloproliferative disorders and cyst hydatid disease, whereas 22.1% of the patient population was found to harbor no identifiable cause of thrombophilia, which we termed as idiopathic. One or more causes of thrombophilia were identified in 46 patients. There were 27 patients with protein C deficiency, 18 patients with protein S deficiency. The antithrombin deficiency was found in 17 patients. The factor V Leiden mutation was found in 7 patients. There was 1 patient with homozygote mutation, whereas the remaining 6 patients were heterozygotes. There were 3 patients with prothrombin mutation who were heterozygote for this mutation. Conclusions Complete investigation of thrombophilia is crucial to delineate the outline of thrombophilic risk factors to estimate the rethrombosis risk and for further management concerns.

Long-term follow-up study in Budd-Chiari syndrome: single-center experience in 22 years.

Goals: We aimed to present our long-term surveillance experience in patients with Budd-Chiari syndrome (BCS), and we retrospectively evaluated the natural history, results of thrombophilia studies, and the factors related to mortality. Background: Primary BCS is a rare form of vascular disease, secondary to underlying thrombophilia. Because of its rarity and heterogeneous nature, there is a scarcity of knowledge about the natural history of the disease. Study and Results: In 22 years, a total of 62 patients with primary BCS were followed in our tertiary hospital. We identified an acquired cause of BCS in 40 out of 62 patients (64.5%), whereas in 6 patients (9.7%), we found no identifiable cause. One or more thrombophilia causes were identified in 56 patients (90.3%). In 19 patients with myeloproliferative disease, 15 had Janus tyrosine kinase 2 mutation analysis and Janus tyrosine kinase 2 positivity was found in 10 patients. In regression analysis, portal vein thrombosis was found to be the only indicator of mortality, with an estimated instantaneous risk of 8.4. Conclusions: In this study, we present one of the largest series of BCS in the English literature. We have shown that the multifactorial nature of underlying thrombophilia should be thoroughly investigated. In a patient with BCS, a clinician should be alert for the development or coexistence of portal vein thrombosis due to its deleterious effect on mortality.

Red cell distribution width can predict intestinal atrophy in selected patients with celiac disease.

Red cell distribution width (RDW) has been shown as a distinctive marker of mortality and morbidity in a wide spectrum of conditions related to systemic inflammation or deficiency of antioxidant nutrients.

Hepatic granuloma in Turkish adults: A report of 13 cases

BACKGROUND Hepatic granuloma (HG) is a well defined histopathological finding with an heterogenous clinical presentation. Diagnosis of a specific clinical entity is not possible every time. Descriptive studies may shed light on the various etiologies also common and distinctive findings among these patients. METHODS We reviewed the results of the liver biopsies of 592 patients. Characteristics of the patients with HG were extracted from the hospital charts. Laboratory studies included biochemical tests, hepatitis C virus (HCV) antibody, Brucella agglutination tests, tuberculin skin test. According to the diagnostic clues further tests (thoracic computed tomography (CT), ultrasonography, organ biopsy in addition to liver, antimitochondrial antibody, hepatitis B surface (HBs) antigen, venereal disease research laboratory (VDRL)) were performed. RESULTS HG was found in 13 of the 592 patients (2.2%). Primary biliary cirrhosis (three cases) was the most frequent cause followed by sarcoidosis, miliary tuberculosis and BCGitis (Bacillus Calmette Guerin) (two cases each). Two patients with HG could not be diagnosed. Only three patients had remarkable physical examination findings. Alkaline phosphatase and gamma-glutamyl transpeptidase were the most frequently elevated enzymes. Abdominal ultrasonography provided no specific diagnostic clue in any patient. Localization of the HGs was portal in 6 patients, parenchymal in 5 patients and both portal and parenchymal in 2 patients. Three exitus were due to BCGitis, miliary tuberculosis and fungal infection. CONCLUSIONS Tuberculosis is still among the most common etiologic factors. BCGitis has a fulminant rather than an indolent course. Abdominal ultrasonography could be used to rule out obstructive jaundice rather than to reach a specific diagnosis. Involvement of portal area by HG in most of the cases might cause obstruction of the biliary canaliculi and elevation of the cholestatic enzymes. Follow up of the difficult cases may be the best approach since the presence of HG was not proved as a bad prognostic factor for any disease.

Postinfantile Giant Cell Hepatitis Due to Hepatitis E Virus Along with the Presence of Autoantibodies

Giant cell hepatitis (GCH) encompasses a group of disorders with varying clinical presentations, etiological factors, and histological findings [1]. When found in adults it is called “postinfantile giant cell hepatitis (PGCH) [2]; this clinical picture is so rare that only about 100 cases have been reported in the English literature in the last 20 years. Drugs [3], autoimmune diseases [4], and several viral infections [5] have been implicated as causes of GCH. Here we present the first patient in the literature with PGCH testing positive for antibody to hepatitis E virus (antiHEV) but carrying some autoimmune features as well. We also aimed to make a differential diagnosis between acute hepatitis E infection and autoimmune hepatitis in light of the pertinent literature.

Misdiagnosis of gossypiboma as hydatid cyst

A 51-year-old woman was admitted for diabetes control. She was found to have a firm, mobile, large epigastric mass during physical examination. Laboratory findings were within normal range. She had undergone cholecystectomy at another hospital 8 years ago. On computerized tomography (CT) of abdomen showed a large intra-abdominal cystic mass measuring 102 cm× 106 cm×181 cm, originating from duodenopyloric junction, which compressed duodenum and pylorus. The appearance of the mass was consistent with type II hydatid cyst (Fig. 1). The patient was advised albendazole therapy; 2 months later, the cyst was treated by puncture, aspiration, injection of a helminthicide, and reaspiration (PAIR) technique. Analysis of cystic fluid was not consistent with hydatid cyst. A post-treatment CT scan showed a reduced size of the cyst; however it returned to its original size within 3 months after treatment. The patient underwent a diagnostic laparotomy which detected four sponges within the cyst (Fig. 2).