1/430. Striking cholestatic liver disease: a distinct manifestation of advanced primary amyloidosis.In patients with systemic amyloidosis, amyloid fibrils are typically deposited in numerous organs, including the kidneys, heart, and liver. Although amyloid deposition in the liver is common in patients with systemic amyloidosis, clinical liver disease is relatively rare. The patient described here had cholestatic liver disease as the primary manifestation of primary systemic amyloidosis. review of the literature suggests that prominent liver disease with cholestasis is unusual but probably underreported in patients with amyloidosis and appears to be restricted to patients with the primary form of amyloidosis. Nonetheless, cholestatic hepatic amyloidosis is characterized by distinct clinical, laboratory, and pathologic features; recognition of this process is critical because it identifies patients with widespread organ involvement and portends a poor prognosis.- - - - - - - - - - ranking = 1keywords = cholestasis (Clic here for more details about this article) |
2/430. Fibropolycystic disease of the hepatobiliary system and kidneys.This complicated case of fibropolycystic disease of the hepatobiliary system and kidneys was ably and incisively analyzed by Professor Sheila Sherlock. Her clinical acumen was revealed by her ability to differentiate congenital hepatic fibrosis, Caroli's disease, and adult polycystic disease of the liver and kidney. Interesting histologic features of this case included hepatic fibrosis with intact limiting plates anc central veins and the presence of bile plugs in the ducts, but the absence of bile statsis in the parenchyma. A percutaneous transhepatic cholangiogram demonstrated the dilated intrahepatic and extrahepatic ducts. Washing out the "gunk" from the biliary tract by T-tube drainage has great limitations in this type of case. Therefore, Dr. Adson suggested irrigation of the biliary ductal system using tubed placed transhepatically, plus a wide choledojejunostomy. Dr. Sherlock questioned this surgical approach. The use of chenodeoxycholic acid for this "gunk" was suggested. In spite of the dilated ducts and pathologic changes in the liver, the patient was not jandiced and did not have stones in her biliary tract. The genetics of this patient's problems was discussed.- - - - - - - - - - ranking = 1.1820630978598keywords = extrahepatic, bile, duct (Clic here for more details about this article) |
3/430. An inborn error of bile acid synthesis (3beta-hydroxy-delta5-C27-steroid dehydrogenase deficiency) presenting as malabsorption leading to rickets.Deficiency of 3beta-hydroxy-delta5-C27-steroid dehydrogenase (3beta-HSDH), the enzyme that catalyses the second reaction in the principal pathway for the synthesis of bile acids, has been reported to present with prolonged neonatal jaundice with the biopsy features of neonatal hepatitis. It has also been shown to present between the ages of 4 and 46 months with jaundice, hepatosplenomegaly, and steatorrhoea (a clinical picture resembling progressive familial intrahepatic cholestasis). This paper reports two children with 3beta-HSDH deficiency who developed rickets during infancy and did not develop clinically evident liver disease until the age of 3 years. bile acid replacement resulted in considerable clinical and biochemical improvement. The importance of thorough investigation of fat soluble vitamin deficiencies in infancy is emphasised.- - - - - - - - - - ranking = 1.1507521459815keywords = cholestasis, bile (Clic here for more details about this article) |
4/430. Treatment of copper associated liver disease in childhood.BACKGROUND / AIMS: copper associated liver disease is accompanied with high liver copper concentrations and progressive liver disease in infancy or childhood. The disease is thought to be due to excessive dietary copper overload (copper-enriched water supply) and in addition to be based on a genetic predisposition. Treatment with penicillamine in Indian childhood disease, which probable has the same etiology, is effective when it is started early enough as well as in Wilson's disease. We aimed to describe the clinical features of copper associated liver disease and report our experience with different treatment options in German children. methods / RESULTS: Two boys presented at the age of 6 and 10 months with abdominal distension due to hepatosplenomegaly. The diagnosis of copper associated liver disease was made based on feeding history, standard liver function parameters, liver biopsy and assessment of dry weight copper concentration and urinary excretion of copper. Both had micronodular cirrhosis, ballooning of hepatocytes and mallory bodies. In child A improvement of liver function was observed after introduction of penicillamine therapy and copper-reduced diet. The treatment was stopped after 18 months, when normalisation of copper concentration in the liver had occured. In child B acute liver failure developed despite initiation of treatment. The boy was transplanted successfully. Both children are presently healthy 10 years after transplantation and 4 years after begin of chelating therapy, respectively. CONCLUSIONS: We conclude, that early chelating therapy with penicillamine can be successful in children with copper associated liver disease. In case of delayed diagnosis and acute liver failure liver transplantation is necessary. Our case reports highlight the urgent need of rapid diagnosis of copper associated liver disease in order to initiate early chelating therapy. copper associated liver disease should obviously be considered in liver disease of unknown origin. Possible causes of excessive dietary copper intake should be ascertained.- - - - - - - - - - ranking = 0.0065250117911242keywords = duct (Clic here for more details about this article) |
5/430. Langerhans' cell histiocytosis as a cause of periportal abnormal signal intensity on MRI.Three cases of hepatic Langerhans' cell histiocytosis (LCH) manifesting as periportal abnormal signal intensity on magnetic resonance images are described. One case also demonstrated intrahepatic bile duct dilatation due to secondary sclerosing cholangitis. Hepatic involvement of LCH could be included in the differential diagnosis of periportal abnormal signal intensity in children.- - - - - - - - - - ranking = 0.15160367774257keywords = bile duct, bile, duct (Clic here for more details about this article) |
6/430. Hepatic manifestations of familial patent ductus venosus in adults.BACKGROUND: The ductus venosus connects the umbilical vein to the inferior vena cava during fetal life and subsequently closes rapidly after birth. It is known as patent ductus venosus when it remains patent in adulthood. patients: A 43 year old man with a history of panhypopituitarism presented with recurrent bouts of pedal oedema associated with fatigue, hypoalbuminaemia, and elevated prothrombin time. An ultrasound examination of his abdomen with Doppler revealed notable attenuation of the main portal vein with diminished intrahepatic branches; a computed tomography scan with angiography revealed a large collateral vein within the liver consistent with a patent ductus venosus. Sequential liver biopsies showed a considerable reduction in the calibre and number of the portal veins. His younger brother, who was diagnosed with alcohol related cirrhosis, suffered from intermittent bouts of encephalopathy and was found to have the same vascular lesion. A third brother was found to have a patent ductus venosus as well as two large hepatic masses consistent with focal nodular hyperplasia. CONCLUSION: The syndrome of familial patent ductus venosus has only previously been described in three infant brothers who presented with hepatic encephalopathy and fatty degeneration of the liver. This report documents three brothers with a patent ductus venosus presenting in adulthood with different manifestations of liver disease. The presence of the same vascular anomaly in three brothers is highly suggestive of a recessive genetic trait with an anatomical manifestation of patent ductus venosus.- - - - - - - - - - ranking = 0.07830014149349keywords = duct (Clic here for more details about this article) |
7/430. Electrophysiologic recovery after vitamin e-deficient neuropathy.A case report is presented of an electrophysiologic recovery from vitamin e-deficient neuropathy after treatment with water-soluble vitamin e in a patient with chronic hepatobiliary disease. The patient was a 64-year-old man who had experienced progressive difficulty in ambulation, with ataxia, over the previous 3 years. The symptoms were associated with pain, tingling sensation in the extremities, and reduced fine motor activity. The patient had chronic hepatobiliary disease, with recurrent cholangitis and external drainage of bile acid through a T-tube for more than 20 years. vitamin e level was barely detectable (<0.5 mg/L). Sensory conduction was absent in both sural nerves. Other sensory and motor conduction studies in the upper and lower extremities showed decreased amplitude. The patient was treated with water-soluble vitamin e. After 4 months of therapy, his ambulation function improved, but pain and tingling sensation in both hands remained. Sensory nerve action potentials appeared in both sural nerves, and amplitudes of other sensory nerves were increased. In a second follow-up study after 9 months, all of the evaluated parameters in the nerve conduction studies, as well as the vitamin e level, were normal. The authors conclude that vitamin e-deficient neuropathy is reversible and electrophysiologic recovery can occur with water-soluble vitamin e therapy.- - - - - - - - - - ranking = 0.049725464569677keywords = bile, duct (Clic here for more details about this article) |
8/430. Mesenchymal hamartoma of the liver--new insight into histogenesis.BACKGROUND/PURPOSE: Mesenchymal hamartoma (MH) of the liver is thought to develop from the ductal plates of the prenatal liver. This immunohistochemical study was performed to gain insight into the pathophysiology of its development. methods: Specimens from four MHs with adjacent liver, in one case from a biopsy and from the resected lesion after 6 years follow-up, were investigated with immunostaining on cryostatsectionswith antibodies against cytokeratins, vimentin, desmin and alpha-actin, as well as von willebrand factor (factor viii), fibroblast growth factor (FGF) receptors, FGF-1 (acidic FGF), FGF-2 (basic FGF), and the proliferation-associated Ki67 antigen. RESULTS: Fibrous tissue of MH stained positive not only for vimentin, but also for desmin and alpha-actin, whereas cytokeratins and factor viii showed specific staining in biliary cysts and endothelial cells, respectively. All mesenchymal cells expressed proteins of the FGF receptor family. Although FGF-1 was only scarcely detectable, there was an accumulation of FGF-2 in borderline areas of liver to MH. Multiple Ki67-positive mesenchymal cells could be identified in these regions in all three MHs. However, we could not detect any proliferative activity in the MHs after follow-up. CONCLUSIONS: The proliferative process in MH is still active during early childhood. FGF-2 may have a role in promoting this process. The positivity for desmin and alpha-actin of the lesions suggests that fat-storing (Ito) cells of the immature liver may be involved in the development of MH.- - - - - - - - - - ranking = 0.0065250117911242keywords = duct (Clic here for more details about this article) |
9/430. Obstructive jaundice caused by a huge liver cyst riding on the hilum: report of a case.A 71-year-old man presented to our hospital with obstructive jaundice, found to be caused by a huge liver cyst which was centrally located and riding on the hilum. Percutaneous transhepatic cyst drainage was performed, following which obstruction of the bile duct was relieved and the jaundice subsided. As jaundice recurred after removal of the drainage tube, the patient underwent deroofing, since when he has remained well. Only 13 cases of liver cysts producing obstructive jaundice have been reported in the English literature, most of which were characteristically enormous, located centrally, and riding on the hilum. Liver cysts possessing such features are likely to cause obstructive jaundice by compressing the hepatic hilum. Cyst drainage is helpful for ameliorating the jaundice and making an accurate diagnosis; however, subsequent deroofing or injection therapy is necessary to prevent recurrence.- - - - - - - - - - ranking = 0.15342645952021keywords = bile duct, bile, duct, obstruction (Clic here for more details about this article) |
10/430. Myeloma and severe cholestasis.amyloidosis is a frequent complication of multiple myeloma. Liver involvement is common in amyloidosis. Hepatic dysfunction and liver chemistry abnormalities are often mild or absent and obstructive jaundice is rare. We report on a 44-year-old patient with multiple myeloma and rapidly deteriorating liver involvement with severe intrahepatic cholestasis. autopsy showed widespread amyloidosis primarily involving the liver. This unusual cholestatic manifestation of hepatic amyloidosis has an uniformly poor prognosis, with death occurring within a few months. We discuss the clinical and pathologic aspects.- - - - - - - - - - ranking = 5keywords = cholestasis (Clic here for more details about this article) |
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