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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This case describes a patient with cholesteryl ester storage disease who underwent liver transplantation for progressive cirrhosis, portal hypertension, ascites, and uncontrollable gastrointestinal bleeding. Four and one-half years posttransplant, her growth improved, cholesterol levels have returned to normal, and she is clinically well except for mild hypersplenism and an elevated blood urea nitrogen (BUN) and creatinine. Serum triglycerides remain elevated, but there have been no signs of progressive renal, intestinal, vascular, or pulmonary disease.
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PMID:Liver transplantation for cholesteryl ester storage disease. 207 31

The present study was undertaken to elucidate clinicopathological findings and operative results of HCC with HB-associated cirrhosis, compared with those in HCC patients with alcoholic and post-transfusion cirrhosis. The number of the HBV group was 26 cases, consisting of 17 in sAg(+), 4 in eAg(+) and 5 in eAb(+) subgroups. The number of the post-transfusion group was 7 and that of alcoholic group was 12. A high incidence of hypersplenism and esophageal varix in the eAg(+) subgroup was found. ICG R15 was the highest, KICG and ICG Rmax were the lowest in the eAg(+) subgroup. The mean diameter of tumors was the largest, 6.6 +/- 3.9 cm, in the sAg(+) subgroup and was the smallest, 2.2 +/- 1.7 cm, in the eAg(+) subgroup. The incidence of postoperative jaundice, hyperammoninemia and live dysfunction were the highest in the sAg(+) and eAg(+) subgroup. One and three-year survival rate were 76.9% and 48.1% in the sAg(+) subgroup, 60.0% and 30.0% in the eAb(+) subgroup, and the one-year survival rate in the eAg(+) subgroup was 50.0%. The three-year survival rate could not be calculated because 3 years had not passed since the operation. The prognosis was the poorest in the HBV group among all groups. This study suggests that in HBV-associated cirrhosis, hepatectomy might induce "acute on chronic" changes (acute hepatitis and fulminant hepatitis). Therefore we should select operative procedures by considering surgical risk and the etiology of liver cirrhosis in hepatectomy.
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PMID:Clinicopathological studies and operative results of hepatocellular carcinoma with liver cirrhosis, comparing HB-associated cirrhosis to alcoholic and post-transfusion cirrhosis. 215 51

Platelet abnormalities associated with hepatobiliary diseases include increased (thrombocytosis) and decreased (thrombocytopenia) numbers of platelets as well as abnormalities in function (thrombocytopathy or thrombasthenia). Hepatic diseases that are accompanied by platelet abnormalities include hepatitis, cirrhosis, portal hypertension, and neoplastic disorders both benign and malignant. The objective of this work is to examine the platelet abnormalities that occur with a variety of hepatobiliary disorders. Thrombocytosis is seen as a reactive entity following splenectomy. Thrombocytopenia is associated with hypersplenism, dysproteinemias and liver disease related disseminated intravascular coagulation (DIC). Qualitative platelet abnormalities are found in hepatic failure, liver diseases associated with high or low levels of lipid, and with medications given for a variety of hepatocellular diseases. Clinically common and significant platelet abnormalities associated with liver disease are thrombocytopenia secondary to portal hypertension and the thrombasthenias following metabolic changes and/or therapeutic interventions of liver disease.
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PMID:Platelet abnormalities in hepatobiliary diseases. 218 3

Liver disease, particularly alcoholic cirrhosis, is associated with a number of interesting chemical changes which result in structural and metabolic abnormalities of the erythrocyte membrane leading to microscopically observable cell shape changes and hemolytic anemia varying from very mild to potentially lethal. Increase in unesterified serum cholesterol owing to lecithin cholesterol acyl transferase (LCAT) deficiency in cirrhosis leads to expansion of the lipid bilayer and macrocytosis without megaloblastic changes in precursors. Substitutions of phosphatidyl choline (PC) moieties in the erythrocyte lipid bilayer lead to echinocytes (disaturated PC) or to stomatocytes (diunsaturated PC). In some patients, high density lipoprotein (HDL) abnormalities lead to erythrocyte surface changes causing rapid formation of echinocytes. The rapidity and reversibility of these changes suggest blockade of metabolic transport channels critical to the maintenance of erythrocyte membrane shape. Metabolic changes involving vitamin E deficiency leading to lipid peroxidation and pyruvate kinase instability leading to adenosine triphosphate (ATP) reduction have also been invoked to explain hemolysis associated with acute liver damage. The most severe hemolysis in liver disease is associated with acanthocytes (spur cells) and a marked imbalance in cholesterol-phospholipid ratio. These patients usually have hypersplenism, as well as rigid erythrocyte membrane transformations which are irreversible. Any of the other erythrocyte membrane shape changes described appear to be reversible if the liver disease abates, but they too may become irreversible if bits of projecting membrane are repeatedly removed by the macrophages of an enlarged spleen.
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PMID:Mechanisms of hemolysis in liver disease. 218 63

We conducted a prospective, randomized trial to study the efficacy and tolerance of long-term versus short-term treatment with recombinant interferon alfa-2a in patients with chronic hepatitis B. Ten patients were randomly assigned to a 6-month interferon regimen, and 10 patients were assigned to a 3-week interferon trial. Eleven patients (five assigned to long-term treatment and six to short-term treatment) did not complete interferon therapy: eight had either severe thrombocytopenia or neutropenia; one had pronounced fatigue in relationship to administration of interferon; one had spontaneous bacterial peritonitis and sepsis and died; and one had a massive fatal variceal hemorrhage during interferon therapy. Most of the serious hematologic complications occurred in patients with cirrhosis and hypersplenism. In one patient, seroconversion to hepatitis B virus DNA negativity occurred before the onset of treatment. Four of the five patients able to complete the 6-month interferon regimen and only one of four patients able to complete the 3-week trial had seroconversion to hepatitis B virus DNA negativity. Thus, we conclude that the therapeutic response was better among patients who were able to complete a 6-month interferon trial. In patients with cirrhosis and hypersplenism, development of either severe thrombocytopenia or leukopenia associated with interferon therapy precluded completion of treatment.
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PMID:Long-term versus short-term treatment with recombinant interferon alfa-2a in patients with chronic hepatitis B: a prospective, randomized treatment trial. 221 80

Splenectomy for massive splenomegaly and hypersplenism carries a significant morbidity and mortality. We have used partial splenic embolization (PSE) as an effective alternative to splenectomy. Ten PSE procedures were performed on nine patients without mortality and with minimal morbidity. The age of the patients ranged from 8 months to 32 years (mean 14 years). The causes of splenomegaly and hypersplenism included cystic fibrosis with cirrhosis (2), tyrosinemia and cirrhosis (1); thalassemia (1), hemophilia with Human Immune Deficiency Virus infection (2), chronic hepatitis with portal hypertension (1), malignant histiocytosis (1), and Wiskott-Aldrich Syndrome (1). All procedures were performed under local anesthesia with sedation. A percutaneous femoral artery approach to the splenic artery was used to deliver Ivalon sponge particles (280-800 microns) into the spleen. Splenic infarction was assessed by postembolization angiograms. All of the patients except one demonstrated improvement of hematologic parameters. In one patient, however, cytopenia improved only after a second embolization. In the total series, there was an early mean rise of 8,600/mm3 in the leukocyte count (range 2,900-14,900) and 212,000/mm3 in the platelet count (range 30,000-718,000). Follow-up ranged from 4 months to 7 years. Improvement of the blood picture has been persistent in seven of the eight patients who showed initial improvement. Transient procedural complications included fever (5), pleural effusion (2), pneumonia (1), and splenic abscess (1). One patient had paralytic ileus lasting for 10 days and one patient developed a streptococcal peritonitis 3 weeks after embolization. No patient developed pancreatitis or vascular compromise of other abdominal viscera.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Partial splenic embolization. An effective alternative to splenectomy for hypersplenism. 226 5

A total of 70 patients with chronic hepatitis and 60 with liver cirrhosis (LC) were examined. The hemorrhagic syndrome (HS) was identified in 16% of patients with chronic active hepatitis, in 26% with compensated and in 76% with decompensated LC. Intravital study of intravascular blood coagulation and liver microcirculation with the aid of fibrin determination according to D. D. Zerbino made it possible to establish local intravascular coagulation (LIC-syndrome). Study into the hemostatic and fibrinolytic systems, analysis of liver function, hemocoagulation and the intensity of the LIC-syndrome permitted one to arrive at conclusions about complex impairments of the hemostatic system in patients with chronic diffuse liver diseases characterized by a tendency towards blood hypocoagulation in the systemic blood flow with the risk of the development of the HS and a tendency towards hypercoagulation (with the risk of thrombosis development) in the vascular bed of the liver. It has been shown that the main cause of the HS occurring in chronic diffuse liver diseases lies in derangements of the thrombocytic component of the hemostatic system: thrombocytopenia as a consequence of hypersplenism and consumption of the most active thrombocytes in the process of the LIC. Activated fibrinolysis starts to exert an inhibitory action on hemocoagulation in patients with chronic diffuse liver diseases in the stage of decompensated LC. In compensated hepatopathies, the influence of fibrinolysis on coagulation and the development of the HS was immaterial.
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PMID:[The mechanisms of disorders in the hemostatic system of patients with chronic diffuse liver diseases]. 233 28

Patients with extrahepatic portal vein thrombosis may present from infancy through adulthood with variceal bleeding. Physiologically, such patients differ from patients with cirrhosis and variceal bleeding in that they have a normal liver and maintain good portal perfusion through hepatopedal collaterals. Complete evaluation of these patients requires identification of the bleeding site by endoscopy, definition of the anatomy by angiography, and confirmation of a normal liver by biopsy examination. Causative factors, including hypercoagulable states, should be evaluated. Therapeutic options range from noninterventive, through ablative procedures, to shunt operations. The goal should be definitive control of bleeding and return to a normal lifestyle. Distal splenorenal shunt offers the best option if technically feasible, but if no shuntable veins are patent, ablative procedures and sclerotherapy may be required. A noninterventive, noninvestigational approach is inappropriate in patients who can be offered definitive therapy. Splenectomy for hypersplenism should not be done in these patients.
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PMID:Management of variceal bleeding in patients with extrahepatic portal vein thrombosis. 236 72

It was discovered that eight patients with complications of non-cirrhotic portal hypertension had received an arsenical preparation for psoriasis as Fowler's solution some years age. Seven of them were admitted for bleeding oesophageal varices. Upon admission, splenomegaly and hypersplenism were present. Liver tests were normal and palmar skin keratosis and melanosis were noted. Liver biopsy of six patients showed features of incomplete septal cirrhosis. Malignant skin lesions were present in half of the patients. One patient died from lung carcinoma and another from an ovarium neoplasm. Chronic arsenic intake should be actively looked for in all patients with psoriasis and non-cirrhotic portal hypertension. They should be followed up for many years for development of malignant lesions in skin, lung and liver. Liver abnormalities present in the biopsies are often minor and may escape detection.
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PMID:Arsenic and non-cirrhotic portal hypertension. A report of eight cases. 180 30

10 patients with liver cirrhosis (LC) and previous variceal bleeding have been studied. Platelet count was markedly depressed. Giant platelet percentage (MTI) was significantly increased, reflecting bone marrow compensatory hyperactivity. Platelet associated IgG (PAIgG) was elevated. Such elevation, however, was inconstant and did not correlate with platelet count. Beta-thromboglobulin was markedly increased when referred to circulating platelet number, but its level did not accurately reflect platelet activation, because of decreased liver cell function. Platelet Factor 4 was always zero, thus confirming that high values of this protein represent only a laboratory artifact, due to platelet activation in vitro. 5 patients underwent spleno renal distal shunt, which transiently improved hypersplenism only in one case. Despite this, MTI became perfectly normal in all patients, suggesting a decrease in the thrombopoietic stimulus. PAIgG dramatically fell in the two patients with the highest pre-operative values. Since thrombocytopenia persisted, the non-specific nature of PAIgG in LC seems to be further supported.
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PMID:Thrombocytopenia in liver cirrhosis complicated by variceal haemorrhage: lack of increase in platelet count after spleno renal distal shunt. 243 15


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