Clinics in liver disease
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Successful liver transplantation in a child is often a hard-won victory, requiring all the combined expertise of a dedicated pediatric transplant team. This article outlines the considerable challenges still facing pediatric liver transplant physicians and surgeons. In looking to the future, where should priorities lie to enhance the success already achieved? First, solutions to the donor shortage must be sought aggressively by increasing the use of from split-liver transplants, judicious application of living-donor programs, and increasing the donation rate, perhaps by innovative means. ⋯ In the United States and in other countries with the medical infrastructure to support liver transplantation, however, health care professionals must strive to be sure that the policies governing candidacy for transplantation and allocation of organs are applied justly and uniformly to all children whose lives are threatened by liver disease. In the current regulatory climate that increasingly takes medical decisions out of the hands of physicians, pediatricians must be even more prepared to protect the unique and often complicated needs of children both before and after transplantation. Only in this way can the challenges of the present and the future be met.
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Hepatorenal syndrome is a functional renal failure that occurs in cirrhotic patients with advanced liver disease and ascites. The diagnostic criteria and clinical types of this syndrome have recently been revised. ⋯ Liver transplantation is the best treatment for HRS, but its applicability is low because of the short survival of these patients. New therapies, such as the use of systemic vasoconstrictors or TIPS, seem promising, but prospective investigations are needed to delineate their role in the management of cirrhotic patients with HRS.
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Clinics in liver disease · May 1998
ReviewRecurrence of primary biliary cirrhosis, autoimmune cholangitis and primary sclerosing cholangitis after liver transplantation.
Given the usually prolonged natural history of primary biliary cirrhosis, autoimmune cholangitis, and primary sclerosing cholangitis, and the relatively recent introduction of orthotopic liver transplantation, our understanding of recurrence of these autoimmune diseases after orthotopic liver transplantation has been slow to evolve. Present data suggest that after orthotopic liver transplantation, patients with primary biliary cirrhosis will have persistence of serum antimitochondrial antibodies, develop histologic lesions suggestive of recurrent primary biliary cirrhosis with a frequency in the 8% to 16% range at 2 to 5 years after orthotopic liver transplantation, but will demonstrate little if any symptomatic disease as a consequence. ⋯ Recurrence of primary sclerosing cholangitis remains the most controversial, however, these patients probably develop nonanastomotic intrahepatic and extrahepatic strictures more frequently than patients without primary sclerosing cholangitis, with a frequency in the 20% to 25% range at 3 to 5 years. With longer patient follow-up and additional studies, it is hoped that our understanding of recurrent autoimmune biliary diseases will grow considerably in the future.
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Portal hypertension is a common clinical syndrome associated with chronic liver diseases, and is characterized by a pathological increase in portal pressure that leads to the formation of portosystemic collaterals resulting in shunting of portal blood into the systemic circulation. The increase in portal pressure is due to an increase in vascular resistance and an elevated portal blood flow. The site of increased resistance is variable, and dependent upon the disease process. ⋯ Another important factor contributing to the increased portal pressure is elevated portal blood flow. Peripheral vasodilatation initiates the classical profile of decreased systemic resistance, expanded plasma volume, elevated splanchnic blood flow, and elevated cardiac index, which characterize the hyperdynamic circulatory state. This hyperdynamic circulation is responsible for various complications of portal hypertension.