Hyponatremia is a common problem in patients with advanced cirrhosis. It develops slowly (paralle... more Hyponatremia is a common problem in patients with advanced cirrhosis. It develops slowly (paralleling the rate of progression of the liver disease) and usually produces no neurological symptoms, although it may exacerbate hepatic encephalopathy. For patients awaiting liver transplantation a low serum sodium level is a strong predictor of pretransplant mortality, independent of the Model for End-stage Liver Disease score (MELD). The pathogenesis of hyponatremia is related to the hemodynamic changes and secondary neurohormonal adaptations that occur in patients with cirrhosis and ascites. The nonosmotic release of arginine vasopressin is the principle cause of the hyponatremia and vasopressin-receptor antagonists are a new class of drugs recently approved for treatment of cirrhotic hyponatremia. In this article we review the safety and efficacy of V2-receptor antagonists in patients with cirrhosis, ascites and hyponatremia.
Hypersplenism is a common manifestation of portal hypertension in the cirrhotic. More than half o... more Hypersplenism is a common manifestation of portal hypertension in the cirrhotic. More than half of cirrhotics will have low platelet counts, but neutropenia is much less common. Despite being common in the cirrhotic population, the presence of hypersplenism is of little clinical consequence. The presence of hypersplenism suggests more advanced liver disease and an increase in risk of complications, but there is no data showing that correcting the hypersplenism improves patient survival. In most series, the most common indications for treating the hypersplenism is to increase platelet and white blood cell counts to allow for use of drugs that suppress the bone marrow such as interferon alpha and chemotherapeutic agents. There are several approaches used to treat hypersplenism. Portosystemic shunts are of questionable benefit. Splenectomy, either open or laparoscopically, is the most effective but is associated with a significant risk of portal vein thrombosis. Partial splenic artery ...
Caroli’s disease is a rare indication for liver transplantation that accounts for 0.13% of all li... more Caroli’s disease is a rare indication for liver transplantation that accounts for 0.13% of all liver transplants performed in the United States. It was first described in 1958 by the French physician Jacques Caroli (19021979). The disease is characterized by segmental dilatation of the intrahepatic biliary tree, and it has an autosomal recessive inheritance pattern. In patients with coexistent congenital hepatic fibrosis, it is termed Caroli’s syndrome. The disease often develops in association with autosomal recessive polycystic kidney disease. Caroli’s disease belongs to the group of fibrocystic liver diseases that develop as a result of biliary ductal plate malformation. In the human embryo, liver development begins in the third week of gestation in the form of a hollow endodermal outgrowth from the ventral foregut or future duodenum into the mesodermal septum transversum. The progenitor hepatic parenchymal cells or hepatoblasts soon align themselves as doublelayered sheets in proximity with the vitelline capillary plexus to create what is termed the ductal plate. At 12 weeks of gestation, the ductal plate starts to remodel to form tubular and cylindrical structures accompanied by resorption of excess epithelial cells. The ductal plate remodeling persists throughout the fetal life, with a gradient directed from the hepatic hilum to the periphery that results in the formation of a network of bile ducts within the portal tracts. The gallbladder, cystic duct, and extrahepatic biliary tree develop from the caudal part of the embryonic endodermal projection. Defective ductal plate remodeling may involve all levels of the intrahepatic biliary tree, with large duct involvement in Caroli’s disease and smaller portal bile duct malformation in congenital hepatic fibrosis. The clinical course of Caroli’s disease is determined by the underlying pathologic abnormalities. Biliary cystic dilatation and narrowing predispose patients to cholestasis and recurrent bouts of acute cholangitis that may become complicated with intrahepatic biliary stones, septicemia, liver abscesses, and cholangiocarcinoma. With associated hepatic fibrosis, as seen in Caroli’s syndrome, portal hypertension develops that may lead to varices, ascites, and liver failure. Treatment of such patients is therefore directed to alleviation of cholestasis with ursodeoxycholic acid, management of acute cholangitis with analgesia and antibiotics, and management of complications related to portal hypertension. In the case of biliary obstruction, radiologic or endoscopic drainage procedures or laparoscopic or surgical deroofing is needed. In some patients with severe, localized, monolobar disease, hepatic resection may be helpful. However, for patients with extensive bilobar disease and/or recurrent bouts of cholangitis and those with complications related to portal hypertension, medical therapy and localized resection may not provide a cure. Such patients may require liver transplantation. Until recently, only small case series were available regarding the application of liver transplantation among patients with Caroli’s disease. We published our singlecenter experience of 33 patients and noted graft and patient survival comparable to the survival of patients who underwent transplantation for other etiologies. Most of our patients had clinical and/or histologic evidence of recurrent cholangitis and complications related to portal hypertension as indications for liver transplantation. A report from the European Liver Transplant Registry provided a limited description of 110 recipients with Caroli’s disease. Several patients had pretransplant septic complications, and posttransplant cumulative graft and patient survival was 68% and 76%, respectively.
Hyponatremia is a common problem in patients with advanced cirrhosis. It develops slowly (paralle... more Hyponatremia is a common problem in patients with advanced cirrhosis. It develops slowly (paralleling the rate of progression of the liver disease) and usually produces no neurological symptoms, although it may exacerbate hepatic encephalopathy. For patients awaiting liver transplantation a low serum sodium level is a strong predictor of pretransplant mortality, independent of the Model for End-stage Liver Disease score (MELD). The pathogenesis of hyponatremia is related to the hemodynamic changes and secondary neurohormonal adaptations that occur in patients with cirrhosis and ascites. The nonosmotic release of arginine vasopressin is the principle cause of the hyponatremia and vasopressin-receptor antagonists are a new class of drugs recently approved for treatment of cirrhotic hyponatremia. In this article we review the safety and efficacy of V2-receptor antagonists in patients with cirrhosis, ascites and hyponatremia.
Hypersplenism is a common manifestation of portal hypertension in the cirrhotic. More than half o... more Hypersplenism is a common manifestation of portal hypertension in the cirrhotic. More than half of cirrhotics will have low platelet counts, but neutropenia is much less common. Despite being common in the cirrhotic population, the presence of hypersplenism is of little clinical consequence. The presence of hypersplenism suggests more advanced liver disease and an increase in risk of complications, but there is no data showing that correcting the hypersplenism improves patient survival. In most series, the most common indications for treating the hypersplenism is to increase platelet and white blood cell counts to allow for use of drugs that suppress the bone marrow such as interferon alpha and chemotherapeutic agents. There are several approaches used to treat hypersplenism. Portosystemic shunts are of questionable benefit. Splenectomy, either open or laparoscopically, is the most effective but is associated with a significant risk of portal vein thrombosis. Partial splenic artery ...
Caroli’s disease is a rare indication for liver transplantation that accounts for 0.13% of all li... more Caroli’s disease is a rare indication for liver transplantation that accounts for 0.13% of all liver transplants performed in the United States. It was first described in 1958 by the French physician Jacques Caroli (19021979). The disease is characterized by segmental dilatation of the intrahepatic biliary tree, and it has an autosomal recessive inheritance pattern. In patients with coexistent congenital hepatic fibrosis, it is termed Caroli’s syndrome. The disease often develops in association with autosomal recessive polycystic kidney disease. Caroli’s disease belongs to the group of fibrocystic liver diseases that develop as a result of biliary ductal plate malformation. In the human embryo, liver development begins in the third week of gestation in the form of a hollow endodermal outgrowth from the ventral foregut or future duodenum into the mesodermal septum transversum. The progenitor hepatic parenchymal cells or hepatoblasts soon align themselves as doublelayered sheets in proximity with the vitelline capillary plexus to create what is termed the ductal plate. At 12 weeks of gestation, the ductal plate starts to remodel to form tubular and cylindrical structures accompanied by resorption of excess epithelial cells. The ductal plate remodeling persists throughout the fetal life, with a gradient directed from the hepatic hilum to the periphery that results in the formation of a network of bile ducts within the portal tracts. The gallbladder, cystic duct, and extrahepatic biliary tree develop from the caudal part of the embryonic endodermal projection. Defective ductal plate remodeling may involve all levels of the intrahepatic biliary tree, with large duct involvement in Caroli’s disease and smaller portal bile duct malformation in congenital hepatic fibrosis. The clinical course of Caroli’s disease is determined by the underlying pathologic abnormalities. Biliary cystic dilatation and narrowing predispose patients to cholestasis and recurrent bouts of acute cholangitis that may become complicated with intrahepatic biliary stones, septicemia, liver abscesses, and cholangiocarcinoma. With associated hepatic fibrosis, as seen in Caroli’s syndrome, portal hypertension develops that may lead to varices, ascites, and liver failure. Treatment of such patients is therefore directed to alleviation of cholestasis with ursodeoxycholic acid, management of acute cholangitis with analgesia and antibiotics, and management of complications related to portal hypertension. In the case of biliary obstruction, radiologic or endoscopic drainage procedures or laparoscopic or surgical deroofing is needed. In some patients with severe, localized, monolobar disease, hepatic resection may be helpful. However, for patients with extensive bilobar disease and/or recurrent bouts of cholangitis and those with complications related to portal hypertension, medical therapy and localized resection may not provide a cure. Such patients may require liver transplantation. Until recently, only small case series were available regarding the application of liver transplantation among patients with Caroli’s disease. We published our singlecenter experience of 33 patients and noted graft and patient survival comparable to the survival of patients who underwent transplantation for other etiologies. Most of our patients had clinical and/or histologic evidence of recurrent cholangitis and complications related to portal hypertension as indications for liver transplantation. A report from the European Liver Transplant Registry provided a limited description of 110 recipients with Caroli’s disease. Several patients had pretransplant septic complications, and posttransplant cumulative graft and patient survival was 68% and 76%, respectively.
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Papers by Shahid Habib