Recent advances in gene therapy and genome-engineering technologies offer the opportunity to correct sickle cell disease (SCD), a heritable disorder caused by a point mutation in the β-globin gene. The developmental switch from fetal γ-globin to adult β-globin is governed in part by the transcription factor (TF) BCL11A. This TF has been proposed as a therapeutic target for reactivation of γ-glo...

BACKGROUND/AIMS We experimented with different ablation methods and two types of microwave antennas to determine whether microwave ablation (MWA) increases intrahepatic pressure and to identify an MWA protocol that avoids increasing intrahepatic pressure. METHODS MWA was performed using either a single-step standard ablation or a stepwise increment ablation paired with either a 16-gauge (G) 2...

PURPOSE The goal of this study was the early diagnosis of ABCB11 spectrum liver disorders, especially those focused on benign recurrent intrahepatic cholestasis and progressive familial intrahepatic cholestasis. METHODS Fifty patients presenting neonatal cholestasis were evaluated to identify underlying etiologies. Genetic analysis was performed on patients suspected to have syndromic disease...

BACKGROUND Intrahepatic lymphocytes are believed to be directly involved in the immunopathogenesis of chronic liver diseases. Little is known about the trafficking of lymphocytes into the liver and their role in chronic hepatitis C infection. METHODS The expression of 4 chemokine receptors and an activation marker on multiple lymphocyte subsets in paired liver biopsy and peripheral blood spec...

BACKGROUND AND OBJECTIVES Hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular neoplasm of the liver. Its therapeutic management remains difficult to define in curative intent. The aim of this study was to report long-term results of surgically managed patients. METHODS From 1990 to 2006, nine patients (25-64 years) were retrospectively enrolled in this study. Intrahepatic disea...

BACKGROUND Intrahepatic stones, though common in East Asia, are uncommon in India. There is paucity of data from India regarding the treatment and long-term outcome of patients with intrahepatic stones. METHODS We retrospectively analyzed medical records of 35 patients with intrahepatic stones who had been treated surgically. Endoscopic biliary drainage had been used in patients who presented...

During liver development, hepatoblasts differentiate into hepatocytes or biliary epithelial cells (BEC). The BEC delineate the intrahepatic and extrahepatic bile ducts, and the gallbladder. The transcription factors that control the development of the biliary tract are unknown. Previous work has shown that the onecut transcription factor HNF6 is expressed in hepatoblasts and in the gallbladder ...

Background. The purpose of this retrospective study is to evaluate the color Doppler US (CDUS) findings of Budd-Chiari Syndrome (BCS) associated with hydatid disease of the liver (HDL) with a special emphasis on the intrahepatic venous collateralisation. Methods. Digitally stored CDUS videoclips or videotape records of the liver of 13 patients with HDL and BCS were retrospectively reviewed. A s...

BACKGROUND Ectopic intrahepatic gallbladder is a rare phenomenon. Gallbladder duplication is an even rarer phenomenon. Pathological processes are more common in congenital anomalies of the gall bladder than normal gallbladders due to poor drainage. CASE REPORT We present a case of duplex gallbladder with one component intrahepatic and the other extra-hepatic, the duo draining via a common cys...