Liver changes and complications in adult polycystic kidney disease.
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Biomedical subjects
Publications and source records attributed to D Franco.
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A limited liver resection was performed in two patients with cirrhosis and a hepatocellular carcinoma situated in segment VIII (anterosuperior subsegment of the right lobe). One of the patient had previously bled from esophageal varices. Resection of segment VIII was performed following the anatomical planes of section after complete mobilization of the right lobe of the liver. Both patients were alive and free of recurrence 14 and 30 months after surgery. Hepatocellular carcinomas are thus treatable by limited anatomic liver resection even when they are situated in the vicinity of the major hepatic veins and the vena cava.
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Two patients with nodular hyperplasia of the liver developed a chronic disabling encephalopathy after an interposition mesocaval shunt. Both had a low total hepatic blood flow-rate postoperatively. Encephalopathy disappeared following surgical occlusion of the shunt. These observations emphasize the risk of postshunt encephalopathy in patients with non-cirrhotic intrahepatic portal hypertension.
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Natural killer (NK) cell activity against two types of target cells was found to be low in patients with inactive alcoholic cirrhosis (AC). This defect was significantly more pronounced in AC patients with severe malnutrition than in those with mild or moderate malnutrition. This was not due to modifications of the kinetics of NK activity. The sera from AC patients had no inhibitory effect on the NK activity of normal subjects. Lymphocytes and macrophages from AC patients did not exert major suppressive effect on the NK activity of normal subjects. Interferon boosted the NK activity of cells from AC patients, but to a lesser degree than cells from normal controls. The findings show that a deficit of NK activity is clearly associated with inactive AC. This seems to be another consequence of AC on cellular immunity, and might be related to the protein calorie malnutrition often present in AC.
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Seven patients (6 women and 1 man) with a Budd-Chiari syndrome were treated by a portal systemic shunt (6 mesocaval and 1 portoatrial shunts). Budd-Chiari syndrome occurred after treatment by estrogen-progestational drugs in 3 cases and following delivery in one. It was due to a myeloproliferative syndrome in 2 cases and to narrowing of the ostium of the hepatic veins in one. All patients had ascites before operation and 5 had an increased serum activity of transaminases. One patient was operated in emergency for progressive massive liver cytolysis. There was no operative mortality. In all patients ascites cleared after operation and transaminases returned to normal values. There was no chronic encephalopathy. Six patients are alive and well with a follow-up of 5 to 66 months. One patient died of acute myeloblastic leukemia 19 months after mesocaval shunt. A liver biopsy was obtained in 2 patients 18 months and 2 years after operation. Hemorrhagic necrosis and sinusoidal dilatation had disappeared and there was annular fibrosis. These results suggest that portal-systemic shunting is a good treatment of the Budd-Chiari syndrome. Since the natural history of this syndrome is still poorly known, a surgical shunt should be rapidly decided upon.
Severe malnutrition and related impaired cell-mediated immunity are commonly found in cirrhotic patients with intractable ascites and may be responsible for the poor prognosis of these patients. The effects of LeVeen peritoneovenous shunting of ascites on protein reserves and cell-mediated immunity were studied in 31 cirrhotic patients with intractable ascites over a period of 1 year following operation. Arm-muscle circumference, serum albumin, and transferrin levels markedly increased and became close to the normal values. In the same period, delayed hypersensitivity improved. Although the lymphocyte count and the absolute T cell concentration in peripheral blood remained low after peritoneovenous shunting, the in vitro lymphoblastic transformation in response to phytohemagglutinin was augmented. An increased capacity to eat normal meals and the resumption of a good appetite due to the discontinuation of a salt-restricted diet seemed to be the most important factors in the dramatic improvement in the nutritional status of these patients. Restriction from heavy alcohol use may have also contributed, although the patients had already stopped drinking for several months before operation. Improvement in cell-mediated immunity might have been secondary to the increase in protein reserves.
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Forty one patients with chronic liver disease and intractable ascites were treated by a portal-systemic shunt. Operative mortality was 4.8 p. 100. Ascites was definitely cleared in 97 p. 100 of the 39 survivors. Fourteen patients later developed encephalopathy which was severe in 6 (15.3 p. 100). One-year survival rate was 72 p. 100. Late deaths were mainly due to complications of liver disease. These results suggest that portal diversion is an efficient and permanent treatment of intractable ascites in cirrhotic patients. Operative mortality and overall survival rates are similar to those observed after peritoneo-venous shunting. The peritoneo-venous shunt is preferable as the first-choice treatment of intractable ascites in cirrhotics because of the risk of encephalopathy. Portal diversion should be proposed for patients with previous variceal hemorrhage and for those in whom peritoneo-venous shunt has failed.
The effect of various types of portal diversion (portacaval, mesocaval and pancreatico-splenocaval anastomoses, portacaval transposition and arterialization) on liver atrophy and post-shunt encephalopathy was studied in the rat. Among all diversions, only portacaval anastomosis produced dramatic liver atrophy and encephalopathy. Moreover, portacaval anastomosis was also the only portal diversion which induced low body weight gain. There was no correlation between blood ammonia levels and encephalopathy. Liver atrophy was always correlated to a decrease of hepatic blood flow. Diminution of liver blood flow was only slight following partial (either mesenteric or pancreatico-splenic) diversion of portal blood and nil after portacaval transposition or anastomosis. These results suggest that: (1) pancreatic (insulin-rich) blood is not essential for maintenance of liver trophicity. Hemodynamic factors seem to be predominant in the pathogenesis of post-shunt liver atrophy. (2) Post-shunt encephalopathy arises only when total diversion of the portal blood and liver atrophy are associated.
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Secondary arterialization of the hepatic stump of the portal vein has been performed in rats 7 days after portacaval anastomosis (PCA) at a date when the electrocorticographic changes of postshunt encephalopathy were evident. A complete regression of encephalopathy ensued. This correlated with an increase in the weight of the liver and estimated hepatic blood flow. These results confirm the efficiency of liver arterialization, primary or secondary, in the treatment of postshunt encephalopathy. In the groups of rats studied in this experiment a significant correlation was always found among encephalopathy, liver weight: body weight ratio, and hepatic blood flow. This reinforces the hypothesis that a decrease in hepatic blood flow is the main factor in the generation of liver atrophy, decrease of hepatic function, and encephalopathy following portacaval shunt. Primary arterialization of the liver in humans has not gained much favor because of technical difficulties, a high mortality rate, and the absence of clear-cut evidence of its efficiency. Our results suggest that arterialization of the liver might better be kept as a secondary procedure for those cases in which encephalopathy develops after PCA.
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