Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

From 1979 until 1981, 10 children aged 2 and a half to 15 years, were treated by mesocaval shunt with jugular vein interposition. In 6 cases, there were liver alterations in relation to Budd-Chiari's syndrome or cirrhosis, but the splenomesenterico-portal axis was uninvolved by thrombosis; in the other 4 cases, without any liver damage except fibrosis in one case, there was extensive thrombosis of the portal system. Thus the jugular graft may be simply set between the superior mesenteric vein and the vena cava, or may have to be anastomosed in an atypical position, between a pancreatic, jejunal, ileal vein and the renal vein or the vena cava. The right jugular vein is the longest and will be more suitable; it should be removed from a point situated above the opening of the facial tributary, so that the length of the graft would be approximately 7 cm. The anastomosis itself is rather a simple procedure if one takes care to mobilize the last segments of the duodenum and the pancreas. Mean fall in portal pressure of 10 mm Hg and a mean gradient of 3 mm Hg after completion of the anastomosis were demonstrated in most cases. Flow measures through the graft done in 8 cases gave a mean result of 800 ml per mn. Postoperative controls by means of ultrasonogram and angiography in all patients except the two most recent ones showed a patent anastomosis. But the longest follow-up is only 2 and a half years, and long term results are yet to be determined, especially in regards to the risk of late encephalopathy in cases with cirrhosis.
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PMID:[Experience with mesocaval shunt with jugular vein interposition for treatment of portal hypertension in children (author's transl)]. 708 45

Thirty-six patients were referred to the Liver Unit between 1971 and 1980 after unsuspected liver disease had been found at laparotomy. The preoperative diagnosis had been extrahepatic biliary obstruction in 16 patients and intra-abdominal malignancy in 15. Misdiagnosis resulted from insufficient attention to the history and physical signs in 31 patients and omission or misinterpretation of liver function tests and of other hepatobiliary investigations in the remaining 5 patients. The morbidity and mortality of the 36 patients within 1 month of operation was 61 per cent and 31 per cent respectively. All patients with viral or alcoholic hepatitis died, and severe complications, which included bacterial peritonitis, wound dehiscence and hepatic failure, developed in 13 of 15 in whom ascites due to cirrhosis or the Budd-Chiari syndrome was present before operation.
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PMID:Adverse effects of exploratory laparotomy in patients with unsuspected liver disease. 710 30

Drug injury of the liver presents as I. functional disorder without jaundice; 2. predominant cholostatic disease; 3. predominant hepatitic disease; 4. a combination of 2 und 3.--A predictable dose related direct toxic injury can be differentiated from an unpredictable dose independent indirect injury mostly of the hypersensitivity type. Special varieties are severe fatty liver, peliosis, Budd-Chiari-Syndrome, liver tumors and granulomas. The diagnosis can be proven by reexposition or better--by the in vitro lymphocyte stimulation test. --There is no specific treatment apart from stopping the responsible drug. Prognosis is good but in some cases a chronic course develops presenting as chronic aggressive hepatitis or cirrhosis.
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PMID:[Liver damage caused by drugs]. 716 50

A ratio between the respective sizes of the caudate and the right hepatic lobes has been described as an adjunct to diagnosing cirrhosis. A case of Budd-Chiari syndrome is reported that shows that this ratio may also be indicative of this condition and is not entirely specific for cirrhosis.
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PMID:Case report. Budd-Chiari syndrome. 724 May 18

The sixth case of post-gravidic Budd-Chiari syndrome is reported. In the acute stage of the disease heparin seems to have given immediate favourable results, and the value of long-term heparin treatment is discussed. Post-mortem findings seven years after the acute episode confirmed the evolution towards a certain type of cirrhosis characterized by macroscopically and histologically heterogenous lesions and by persistent liver congestion. Portal hypertension was associated with arterialization of the portal vein and considerable dilatation of the right diaphragmatic veins. The venous drainage of the liver had undergone complex alterations: the supra-hepatic veins were affected with fairly recent incomplete ostial thrombosis, many intra-hepatic veins were reduced to a vestigial reticulum and others were the seat of recent or old re-canalized thrombosis. Cancerous transformation was found to be present in the larger hepatic nodules.
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PMID:[Acute post-gravidic Budd-Chiari syndrome. One case (author's transl)]. 742 85

We reviewed 37 living related liver transplantations (LRLT) performed by our department during the last 27 months on children with end-stage liver disease. The patients were 15 boys and 22 girls aged 7 months to 15 years with biliary atresia (27), cryptogenic cirrhosis (3), Budd-Chiari syndrome (2), progressive intrahepatic cholestasis (2), protoporphyria (1), Wilson's disease (1), and fulminant hepatitis (1). The donors were 14 fathers and 23 mothers. Grafts were made from the left lateral segment (19), left lateral segment with partial S4 (11), left lobe (6), and right lobe (1). After graft harvesting all donors resumed normal liver function and normal life. The recipient underwent total hepatectomy with preservation of the inferior vena cava. FK506 and low-dose steroids were used for immunosuppression. The survival rate was 90% (27/30) in elective cases and 57% (4/7) in emergency cases. Six recipients had functioning grafts but died of extrahepatic complications. Hepatic vein stenosis occurred in 3 cases at 3 months after LRLT and was successfully treated by balloon dilatation. Portal vein stenosis occurred in 1 case at 8 months after LRLT and was also safely dilated. We incurred no hepatic artery thrombosis after introducing microsurgery techniques. Among 12 viral, 5 bacterial, and 3 fungal postoperative infections, 1 Candida pneumonia and 1 EBV-associated lymphoma were lethal. Three patients with ABO-blood group compatible grafts and one with an incompatible graft developed acute rejection, which was controlled in evey case by steroid bolus and/or increasing the dose of FK506. There were no definite episodes of rejection in ABO-identical cases. Children with moderate growth retardation (> or = -1.5 SD of normal growth) caught up in growth soon after LRLT, but those with severe retardation (<-1.5 SD) were slow to attain age-normal height. Appropriate timing, meticulous surgical procedures, and comprehensive management of complications are crucial for successful outcome with LRLT. LRLT is a promising option for alleviating the shortage of livers for pediatric transplantation and may be regarded as an independent modality to supplement cadaver donation.
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PMID:Living related liver transplantation in children. 751 49

Orthotopic liver transplantation (OLT) is nowadays accepted as the best therapy for end-stage liver disease. The difficulties involved are even greater in children than in adults, and it is debatable whether exclusively pediatric programs are warranted. The aim of this paper is to analyze our experience at the Children's Hospital "La Paz", with the first consecutive 100 OLT in children, 61% of whom weighed less than 20 kg. Since 1986, 220 pediatric patients were evaluated as candidates, 100 OLT were performed in 78 patients and 13 died on the waiting list, currently maintained below 15 cases. Indications were: cholestasis (45), metabolic disease (18), fulminant hepatic failure (3), primary liver tumors (2) and cirrhosis (10). Mean age was 66 months (range = 7 to 216) with a mean weight of 21 kg (range = 6 to 60), twenty patients weighed less than 13 kilograms. OLT was performed by standard technique. Reduced or segmental grafts were necessary in 8 instances. Twenty-two patients were retransplanted and 2 received three grafts. Indications for retransplantation were: hepatic artery thrombosis (8), primary nonfunction (4), chronic rejection (7), portal thrombosis (2) and Budd-Chiari recurrence (1). Acute rejection was observed in 52 patients, and eight cases developed a chronic rejection. These episodes were treated with "bolus" of steroids, monoclonal antibodies (OKT-3) and FK-506. Surgical complications included: hepatic artery thrombosis 12%, portal vein thrombosis 3% and biliary fistula or stenosis 13%. The incidence of primary non-function was 7%. Actuarial survival rate at 5 years was 75%.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Our first 100 consecutive pediatric liver transplants. 761 86

A 49-year-old male with Budd-Chiari syndrome complicated by liver cirrhosis and intractable ascites is reported. The left hepatic vein was stenosed by a short subocclusive ostial web; the right and medial hepatic veins were thrombosed. A spontaneous intrahepatic portosystemic shunt had developed between the left portal and left hepatic veins. After ineffective balloon angioplasty, the left hepatic venous outflow was restored by placement of a 10-mm-diameter Wallstent across the web via a femoral approach. The hepatic venous pressure dropped from 29 to 12 mmHg. Rapid clinical improvement followed. The patient underwent liver transplantation 3 months later in stable condition.
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PMID:Treatment of Budd-Chiari syndrome by metallic stent as a bridge to liver transplantation. 764 99

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

We treated 33 cases of Budd-Chiari syndrome caused by localized occlusive lesions of the inferior vena cava (IVC) with balloon dilatation and stent deployment. Of the 33, 21 were male and 12 female. Complete "membranous" occlusion was found in 18, incomplete "membrane" in 6, and localized stenosis in 9. Seven cases recurred after transcardiac and membranotomy or cavoatial shunt or mesoatial shunt. Pure stent deployment was carried out in 24 cases, combined transcardiac and transfemoral approaches were used in 6, stent was placed during radical correction of IVC lesions in 3. Unrelieved intractable ascites were 5 cases because of hepatic venous occlusion or secondary cirrhosis, 3 of them were resolved by mesocaval anastomosis. The IVC pressures before and after stenting were 2.70 +/- 0.69kPa (1kPa = 0.98cmH2O) and 1.55 +/- 0.50kPa (P < 0.01) respectively. All but one (waiting for possible messocaval shunt) had excellent effect 6-23 months following the above mentioned therapy. We conclude that dilation and stenting plus necessary or additional surgery bring about a prosperous means for treating Budd-Chiari syndrome with localized lesions.
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PMID:[Treatment of Budd-Chiari syndrome with balloon dilatation and intraluminal stent]. 776 78


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