1/29. Unsuspected bile duct paucity in donors for living-related liver transplantation: two case reports.Alagille's syndrome is a common cause of liver disease in children and may lead to the need for orthotopic liver transplantation. Alagille's syndrome is inherited in an autosomal dominant manner, with variable penetration, and may also be present in patients' parents, who may be considered potential donors for living-related transplantation. We report here on two cases in which the living-related donors for children with Alagille's syndrome had no liver function abnormalities or characteristic features of Alagille's syndrome. In both cases, the operation for living-related donation had to be aborted because of a paucity of bile ducts discovered intraoperatively. Given the variable presentation of Alagille's syndrome, we believe that it is necessary preoperatively to evaluate the biliary system of family members who are potential living-related donors for patients with this condition.- - - - - - - - - - ranking = 1keywords = bile duct (Clic here for more details about this article) |
2/29. A case report: alagille syndrome.alagille syndrome consists of 5 major features comprising paucity of interlobular bile ducts, characteristic facies, posterior embryotoxon, vertebral defects and peripheral pulmonic stenosis. The female patient in this report met 4 of the 5 major features except ocular abnormality. The first clinical presentations were prolonged jaundice and generalized ecchymoses. She was treated by plasma replacement and vitamin supplement, particularly vitamin K1, which produced clinical improvement. This report also reviews the literature of alagille syndrome.- - - - - - - - - - ranking = 0.2keywords = bile duct (Clic here for more details about this article) |
3/29. Laparoscopic findings in an adult case of alagille syndrome.Laparoscopic findings in a 32-year-old woman presenting with alagille syndrome are reported. The liver surface showed a segmental whitish discoloration on the surface of both lobes with a transverse sulcus which was not stained enough after an intravenous injection of indocyanine green (ICG). These findings suggested an acquired bile duct injury in addition to a congenital anomaly of the bile duct in this patient.- - - - - - - - - - ranking = 0.4keywords = bile duct (Clic here for more details about this article) |
4/29. Successful heterotopic segmental liver transplantation from a live donor to a patient with alagille syndrome.alagille syndrome is characterized by a paucity of bile ducts in the liver. The syndrome is associated with some or all the features of chronic cholestasis, cardiac disease, skeletal abnormalities, ocular defects and a distinctive facial appearance. The most common finding is chronic cholestasis, which causes intractable pruritus, xanthoma, deficiency of certain metabolic nutrients and growth retardation. Cardiac abnormalities are the most common cause of death in these patients. It is unusual to see the clinical picture of hepatic failure resulting in cirrhosis and requiring transplantation, but liver transplantation is indicated in alagille syndrome patients who have chronic cholestasis. If the disease is diagnosed in childhood, transplantation can improve significantly the patient's prognosis and the quality of life. In recent years, auxiliary liver transplantation has gained popularity for treating both acute and chronic liver disease. Heterotopic segmental liver transplantation is an alternative treatment modality for patients who do not require native liver removal. Individuals with alagille syndrome are good candidates for this type of treatment. J Pediatr Surg 36:667-671.- - - - - - - - - - ranking = 3.8122719586009keywords = cholestasis, bile duct (Clic here for more details about this article) |
5/29. Cholestatic syndromes of infancy and childhood.cholestasis results from structural and functional impairment of the hepatobiliary system, which is often the target of several environmental factors and disease processes. This review focuses on the clinical consequences of this impairment. When evaluating an infant or child with cholestasis, a broad differential diagnosis must be considered; viral infections, metabolic disorders, and toxic insults may often lead to cholestasis. In the infant, cholestasis associated with severe hepatic synthetic dysfunction points to life-threatening metabolic disorders. In this setting, early diagnosis and prompt treatment offer the only chance for survival. Fortunately, cholestasis in infants presents more frequently with initially normal liver synthetic function. In those infants without evidence of infection, evaluation for patency of the extra-hepatic biliary system is a high priority. biliary atresia comprises a significant portion of these patients and requires surgical intervention with portoenterostomy in an attempt to improve biliary flow. In a substantial group of infants and older children in whom the cause for cholestasis is not apparent, typical clinical and biochemical markers may allow the identification of specific genetic defects of syndromes that result from abnormal canalicular transporters.- - - - - - - - - - ranking = 6.0204532643349keywords = cholestasis (Clic here for more details about this article) |
6/29. Genetic disorders and molecular mechanisms in cholestatic liver disease--a clinical approach.cholestasis may result from genetic or acquired defects in bile secretion. Cloning of hepatobiliary transporter genes has advanced our understanding of the molecular basis of bile formation and cholestasis. Hereditary mutations of transporter genes, exposure to cholestatic injury (eg, drugs, hormones, cytokines), or the combination of both can result in reduced expression and function of hepatobiliary transport systems. These molecular changes impair hepatic uptake and excretion of bile salts and other organic anions (eg, bilirubin). Other molecular changes contibuting to cholestasis include alterations of membrane fluidity, cytoskeleton, vesicle movement, and cell contacts. Transporter mutations can be diagnosed at the molecular genetic level. Gene therapy and hepatocyte transplantation could be used in the future to correct hereditary transport defects. Drugs used to treat cholestatic liver diseases (eg, ursodeoxycholic acid) stimulate and partially restore defective transporter expression and function. New information on the molecular mechanisms of cholestasis should lead to the development of novel drugs for cholestatic liver diseases.- - - - - - - - - - ranking = 3.6122719586009keywords = cholestasis (Clic here for more details about this article) |
7/29. alagille syndrome with colonic polyposis.alagille syndrome is a rare inherited condition, which typically manifests during the first year of life as an episode of prolonged cholestasis. Although the pattern of inheritance is autosomal dominant with almost complete penetrance, highly variable expression may delay the diagnosis, and with passing time the clinical findings may be more difficult to recognize. This has clinical implications, as patients with alagille syndrome are at risk for late complications such as hepatocellular carcinoma. We report a case of a 35-yr-old patient with alagille syndrome who was diagnosed with colonic polyposis raising the possibility of an association between the two.- - - - - - - - - - ranking = 1.204090652867keywords = cholestasis (Clic here for more details about this article) |
8/29. Proliferation to paucity: evolution of bile duct abnormalities in a case of alagille syndrome.alagille syndrome is an autosomal dominant disorder characterized by abnormalities in multiple organ systems, including the liver, and is caused by mutations in JAG1. Chronic cholestasis secondary to paucity of interlobular bile ducts is traditionally both a clinical and a pathologic hallmark of this disease at diagnosis. We describe the biliary changes on serial liver biopsies in a patient who presented with jaundice and extrahepatic stigmata of alagille syndrome. Her initial specimens at 6 and 10 months of age demonstrated interlobular bile duct proliferation and cholestasis, suggestive of distal biliary obstruction. A specimen at 2 years of age showed near-total absence of interlobular bile ducts, with the classic histologic appearance of bile duct paucity. We present this case to underscore the potential pitfalls in interpreting cholestatic liver morphology in the absence of clinical information. The progression of bile duct abnormalities is discussed in the context of the role postulated for JAG1 in postnatal liver growth and development.- - - - - - - - - - ranking = 4.0081813057339keywords = cholestasis, bile duct (Clic here for more details about this article) |
9/29. alagille syndrome.Syndromic paucity of bile ducts or "alagille syndrome" is characterized by peculiar facies, chronic cholestasis, posterior embryotoxon, butterfly-like vertebral arch defects and peripheral pulmonary artery hypoplasia or stenosis. We present a two-year-old female child with the 'partial' or 'incomplete' alagille syndrome. The child had three of the five major features of the syndrome. A brief review of literature of the syndrome is presented.- - - - - - - - - - ranking = 1.404090652867keywords = cholestasis, bile duct (Clic here for more details about this article) |
10/29. Hepatocellular carcinoma associated with arteriohepatic dysplasia in a 4-year-old girl.Hepatocellular carcinoma and obliterated hepatic bile duct were found at postmortem examination in a 4-year-old girl with arteriohepatic dysplasia (Alagille's syndrome). AFP level was extremely high. liver cirrhosis was present on percutaneous needle biopsy 9 months before she succumbed in progressive liver failure. Episodes of repeated gastrointestinal, life-threatening hemorrhages occurred during the last 6 months of her life. Histopathologic findings of the eyes were documented at autopsy.- - - - - - - - - - ranking = 0.2keywords = bile duct (Clic here for more details about this article) |
| | Next -> |