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Although liver biopsy is a very useful procedure used frequently in the diagnosis and management of liver dysfunction occurring after orthotopic liver transplantation, complications can occur with its use. An unusual complication of arterioportal fistula is reported here. Based upon this small series of an unusual event and the knowledge that the posttransplant liver may be more hypervascular than prior to OLTx and that it is uniquely susceptible to hepatic infarction and abscess formation, any attempt at fistula closure should be considered carefully prior to initiating the therapy (15). Unless a serious complication occurs [such as a transient biliary obstruction due to hemobilia as occurred in case 2, portal hypertension as also occurred in case 2, or systemic sepsis or other symptoms develop related directly to the fistula], simple observation may be the best choice of action. Should therapy be required, hepatic arterial embolization should be reserved for adults with intrahepatic fistulas. Primary surgical closure of intrahepatic fistula should be reserved for cases of extrahepatic fistula.
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PMID:Arterioportal fistula following liver biopsy. Three cases occurring in liver transplant recipients. 772 61

The optimal treatment of Budd-Chiari syndrome (BCS) remains an open question. It is still a matter of controversial discussion whether venous decompression or liver transplantation is superior. To elucidate the role and prognosis of both surgical options in our own experience, a consecutive series of 50 patients treated between 1981 and 1993 was retrospectively analyzed. Twelve patients had different types of portosystemic shunts or local decompressive procedures, and transplantation was performed in 43 cases, including five with previous conventional surgery. The overall mortality of 18 of 50 was conventional surgery. The overall mortality of 18 of 50 was concentrated within the early postoperative period, with no patient lost after 1 year. In the venous decompression group, the success rate was only 29%, and treatment failure was closely related to the finding of cirrhosis or technical problems like vascular thrombosis. After transplantation, early complications were rejection, primary nonfunction, or graft necrosis, and contributed significantly to the risk of sepsis. Thirty of 43 liver recipients are currently alive, including four rescued after failed decompressive surgery, with 1- and 10-year survival of 69%, and excellent recurrence-free rehabilitation. These results clearly indicate that patient selection plays a dominant prognostic role in the treatment of BCS. Venous decompression and liver transplantation should both be integrated in a common therapeutic concept, and the individual decision for the preferred approach must be based on the leading clinical symptom: portal hypertension or liver failure, together with the assessment of reversibility of hepatic damage, and the potential of cure of the underlying disease.
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PMID:Which is the best surgery for Budd-Chiari syndrome: venous decompression or liver transplantation? A single-center experience with 50 patients. 773 40

The TIPS (transjugular intrahepatic portosystemic shunt) procedure is a relatively new therapeutic treatment option for variceal bleeding secondary to portal hypertension. The TIPS procedure evolved in response to the need for a safe, nonoperative shunting procedure, and is becoming a practical alternative to surgically placed shunts. Possible complications of a TIPS include hemorrhage, postprocedure sepsis, shunt encephalopathy or decompensation. Development of shunt stenosis or occlusion is associated with re-bleeding. Three children (ages 10-13) with recurrent, significant variceal bleeding were referred for the TIPS procedure. Two had recurrent GI hemorrhage despite sclerotherapy. To date, all have maintained shunt patency for up to 1 year. None of these children have had significant re-bleeding episodes, and the only recurrence of varices was associated with an obstructed shunt. In this article, the author reviews both conventional treatment and the TIPS procedure for the child with bleeding varices. Nursing implications from a pediatric perspective are emphasized. Pediatric GI nurses must understand TIPS in order to prepare the child and family and to monitor for complications or shunt failure afterward.
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PMID:Transjugular intrahepatic portosystemic shunt (TIPS): a pediatric perspective. 785 7

Life-threatening portal hypertension (PHN) in patients with chronic myeloproliferative disorders may result from increased portal flow caused by marked splenomegaly or an increased resistance to portal flow from either a large vein thrombosis or an intrahepatic obstruction usually associated with agnogenic myeloid metaplasia (AMM). The former cause is correctable by splenectomy alone, whereas the latter requires portal-systemic shunt surgery. Few data exist regarding the outcome of portal-systemic shunt surgery in patients with AMM and intrahepatic obstruction. During the past 25 years, 13 patients with chronic myeloproliferative disorders underwent portal-systemic shunt surgery at our institution. The cause of PHN was intrahepatic obstruction in ten patients and hepatic vein thrombosis in three. Ten of the thirteen patients had AMM as initial diagnosis. Only one patient had intraoperative complications, and four patients had either sepsis or thrombosis during the postoperative period. Twelve patients survived the postoperative period and had a median postsurgical survival of 3 years (range, 0.25 to 19 years). The long-term complications of the operation were very few and included hepatic encephalopathy (one patient), portal vein thrombosis (one patient), and shunt occlusion (one patient). The procedure was successful in alleviating complications of PHN in all but one patient. Deterioration of hepatic function and subsequent hepatomegaly were unusual. Portal-systemic shunt surgery seems to be a useful option in patients with AMM and life-threatening PHN from intrahepatic obstruction.
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PMID:Outcome of portal-systemic shunt surgery for portal hypertension associated with intrahepatic obstruction in patients with agnogenic myeloid metaplasia. 803 84

Hypersplenism is defined as the association of anemia, leukopenia, or thrombocytopenia with bone marrow hyperplasia and splenomegaly. Hypersplenism is common in liver cirrhosis and frequent in patients with portal hypertension. The effects of portacaval shunt are variable; hypersplenism hardly ever improves but rarely develops after surgery. Since the spleen is a major component of the mononuclear phagocyte system, splenectomy reduces antibody synthesis. Although splenectomy abolishes hypersplenism, it may lead to sepsis. Recently, partial splenic embolization, using gelform injected directly into the splenic artery, has been performed in patients with cirrhosis. Partial splenic embolization induces an increase in the number of circulating blood cells. In addition, the levels of albumin, hepaplastintest, cholesterol and cholinesterase are increased significantly after treatment. Partial splenic embolization rarely causes problems and may actually be beneficial.
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PMID:[Hypersplenism in liver cirrhosis]. 811 16

Extrahepatic portal vein obstruction (EHPVO) affects 20-30% of all patients with portal hypertension in India. The etiopathogenesis of this disease is unknown. In retrospective studies, umbilical vein cannulation and sepsis have been alleged to cause portal thrombosis. This prospective study was undertaken to detect clinically and by serial sonography whether thrombosis and consequent obstruction of the splenoportal venous system develops after umbilical vein catheterization and sepsis. Forty-seven children who had undergone exchange transfusion for hyperbilirubinemia, belonging to two different age groups, were studied. Twenty-two new-borns (Group A) were studied within 4 weeks of birth and later at 3-month intervals until the age of 12 months and subsequently at 24 months. Another group of 25 children (Group B), 1- to 5-year-olds who had earlier undergone exchange transfusion and 15 healthy newborns (Group C) were also screened. In Group A, seven (29%) neonates had septicemia, and in five, the splenic vein could not be initially visualized. The splenic vein was not seen in five of the 15 newborns in Group A who had no umbilical sepsis. Twenty-one of the 22 neonates in Group A were followed-up, and the splenic vein was well visualized and found patent on subsequent ultrasound in all of them. In Group B, four (16%) children had umbilical sepsis. None of these or the other Group B children showed any thrombosis or obstruction of the splenoportal system. The portal vein and its branches and the splenic vein were visualized in all healthy (Group C) neonates.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Do umbilical vein catheterization and sepsis lead to portal vein thrombosis? A prospective, clinical, and sonographic evaluation. 814 94

Orthotopic liver transplantation (OLT) is a life-saving procedure for end-stage liver failure. We reviewed 39 children (24 girls, 15 boys) who received OLT for biliary atresia from 1987 to 1991. Twenty had unsuccessful portoenterostomy, 6 were referred too late for a drainage operation, and the remaining 13 achieved bile drainage but developed portal hypertension. At transplant 37 had decompensated liver disease with varices (28), ascites (24), encephalopathy (17), and gastrointestinal bleeding (12). The median weight and age at transplant were 8 kg and 12.6 months, respectively. The median waiting time was 65 days. Forty-eight grafts (30 reduced and 18 whole) were performed; graft loss was 33% and 27%, respectively. Of the 30 segmental grafts, 15 were reduced conserving the left lateral segment and hepatic vein (Brisbane technique)--13 were from the left lobe and 2 from the right lobe. The overall subject survival rate is 72%. Eleven deaths occurred: primary nonfunction (3), sepsis (3), perioperative bleed (3), and other causes (2). Early complications included: hepatic artery thrombosis (5), hepatic vein thrombosis (2), bowel perforation (3), biliary leak (3), and acute rejection (8). Later complications were chronic rejection (4) and biliary stricture requiring reconstruction (3). Follow-up at 12 months confirms good quality of life for both child and family with catch up growth and normal development. Technical advances in reduction hepatectomy have allowed us to treat small babies under 1 year with an urgent requirement for OLT, with comparable results to those obtained with whole grafts. In conclusion, in the future size and age need not be a contraindication to OLT in children with biliary atresia.
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PMID:Liver transplantation in babies and children with extrahepatic biliary atresia. 822 94

721 patients with liver cirrhosis were regularly screened by sonography and determination of alpha fetoprotein during a period of eleven years (1.1.1982-1.1.1993). In 137 of them hepatocellular carcinoma (HCC) was diagnosed; 28 (20.4%) had a unilocular HCC with a diameter up to 5 cm. Diagnosis was regularly verified by sonographic guided puncture, in rare cases by laparoscopy and biopsy. Beside a diameter of 5 cm the tumor should be localized at least 5 mm from the main structures in the hilus, and not in the centre of the liver; furthermore multilocular hepatocellular carcinomas and intra- and extrahepatic metastases were contraindications. Child-Pugh-classification should be A+B and urea synthesis rate at least 6 g per day. In 21 patients (75%) a portal hypertension was diagnosed; 19 (68%) had bled from esophageal varices; in case of one bleeding a therapeutic sclerotherapy and in case of recurrent variceal hemorrhage an elective shunt operation were performed. Surgical resection was carried out with controlled hypotension and temporary occlusion of the hepatoduodenal ligament. Tumor was removed by segmentectomy or bisegmentectomy and in rare cases by enucleation. There were 3 clinical deaths (10.7%); causes of death were liver failure and (2) sepsis (1). All patients could be followed up to January 1, 1993; there were 12 further deaths of liver failure, tumor recurrence or second tumor. 13 patients are still living. Thus the live expectancy for one year was 80, for 5 years 50 and for 10 years 30%. There is no doubt, that it is possible to detect hepatocellular carcinoma in patients with liver cirrhosis early by regular sonography and determination of alpha-fetoprotein.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Single hepatocellular carcinoma (phi < or = 5 cm) in liver cirrhosis. Early diagnosis and surgical removal]. 826 41

Twenty-five patients with liver cirrhosis and portal hypertension were admitted for creation of a transjugular intrahepatic portosystemic shunt (TIPS). The procedure was successful in 22 patients (technical success 88%). The mean portal pressure gradient was lowered from 24.5 mmHg before to 11.4 mmHg after TIPS. Two early and three late occlusions were observed (primary patency rate 78%). The rate of secondary interventions was 41%. Five times a hepatic vein stenosis was dilated and stented, two times an occluded shunt was recanalized, two times a new shunt was created parallel to an occluded (secondary patency rate within a maximum of 16 months 95%). In two patients sepsis occurred which was effectively treated with antibiotics, two patients died shortly after TIPS due to hepatorenal syndrome and hepatic failure, respectively. There was no recurrent bleeding. Two patients developed hepatic encephalopathy; both improved after protein restriction. The authors conclude that TIPS is an alternative procedure to shunt surgery, especially for patients who cannot benefit from sclerotherapy.
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PMID:[Clinical experiences with the transjugular intrahepatic portocaval shunt (TIPS)]. 837 9

Nitric oxide is a highly reactive, diffusible gas that is produced by many tissues, and it exerts a range of physiological and pathophysiological effects. The liver is one organ clearly influenced by nitric oxide, and acute versus chronic exposure to this substance has been associated with distinct patterns of liver disease. Bacterial infections, including the sepsis syndrome, acutely increase nitric oxide systemically and may lead to acute hepatic dysfunction. We review etiology, diagnosis, and treatment of cholestasis associated with these infections, because this condition in particular has been linked to nitric oxide. Chronic increases in nitric oxide may cause the hyperdynamic circulation seen in cirrhosis and portal hypertension. Therefore, we also review etiology, diagnosis, and treatment of the hepatorenal and hepatopulmonary syndromes, because both these syndromes occur in end-stage liver disease, and they may be linked to nitric oxide as well. An appreciation of nitric oxide and its evolving role in hepatology may be important to understand the pathogenesis of and treatment strategies for these different types of liver disease.
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PMID:Nitric oxide and liver disease. 853 73


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