Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: EC:2.6.1.2 (alanine aminotransferase)
26,722 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Schistosomiasis mansoni is a widespread parasitic disease in the Brazilian territory that affects over 8 million individuals. Hepatosplenic schistosomiasis is a serious clinical presentation of this disease, associated with splenomegaly, liver fibrosis, and portal hypertension, and is responsible for approximately 7% of schistosomotic patients. The surgical treatment of portal hypertension in schistosomotic patients has distinct features when compared with cirrhotic patients, mostly because hepatic function is preserved in schistosomotic liver disease. Therefore, when attempting to reduce the portal pressure, the surgeon must be aware that the surgery might interfere with hepatic perfusion, and consequently with hepatic function. The aim of this study was to report the results achieved with splenectomy, division of the left gastric vein, devascularization of great gastric curvature, and postoperative endoscopic variceal sclerosis, as a surgical option to esophageal varices in hepatosplenic schistosomiasis. A total of 111 patients were studied, and the following is a list of inclusion criteria: age >16 years, history of gastrointestinal (GI) bleeding, presence of esophageal varices on preoperative endoscopy, hematocrit >22% and prothrombin enzymatic activity >50%, negative viral hepatitis on serologic tests (anti-HBV and anti-HCV), and definition, after liver biopsy, of exclusive schistosomotic liver disease. The following list includes exclusion criteria used: presence of liver disease other than schistosomotic, history of alcohol abuse, and preoperative thrombosis of the portal vein. The rebleeding rate was 14.4% during a mean 30-month follow-up period; portal vein thrombosis was 13.2%, and there was a global mortality of 5.4%. Gastric varices were present in 46.9% of the patients; for those patients, a gastrotomy and running suture of the varices achieved an eradication rate of the varices of 75.6%. The degree of periportal fibrosis was also analyzed. Periportal fibrosis staging revealed that patients with class II or III liver fibrosis had a significant increased risk of recurrent GI bleeding when compared with patients with class I liver fibrosis. Despite the elevation on alanine aminotransferase (ALT) and aspartate aminotransferase (AST), most other liver function tests showed no alteration or were corrected after surgery. We conclude that splenectomy, division of the left gastric vein, devascularization of great gastric curvature, and postoperative endoscopic variceal sclerosis showed good results globally and should be considered as therapeutic options in the treatment of hepatosplenic schistosomiasis.
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PMID:Surgical treatment of schistosomal portal hypertension. 1189 Mar 33

Ninety individuals (76 males and 14 females) were classified into four groups. G1 (Control) included 20 healthy individuals. G2 (Chronic hepatitis) included 20 patients, G3 (Liver cirrhosis group) included 30 patients, and G4 (HCC) included 20 patients with HCC. All groups were subjected to clinical examination, abdominal ultrasonography, complete blood picture, HCV antibodies, HBs Ag, and function tests (total and direct bilirubin, total plasma proteins and albumin, prothrombin time and concentration, and liver enzymes AST, ALT and ALP). Patients of G3 & 4 were classified according to Child-Pugh classification into A. B and C. Upper endoscopic examination was done for 36/50 patients with chronic hepatitis or HCC. Circulating VEGF levels were determined by ELISA. There was a statistically high significant levels of circulating VEGF in G1, 2 & 3 than in the controls. A statistically significant higher level of circulating VEGF in G4 than in G3 & G4, and a statistically negative significant between VEGF levels and platelet count in G2. No significant correlation between VEGF and the grade of esophageal varices in G3 & G4. and no significant correlation between VEGF and upper GIT bleeding or spider naevi (vascular skin changes) in G2. A statistically significant was in correlation between VEGF and degree of hepatic dysfunction.
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PMID:Vascular endothelial growth factor level in chronic liver diseases. 1251 23

Risk factors for the development of hepatocellular carcinoma (HCC) were investigated in 397 patients who underwent non-shunt operation for esophageal varices due to underlying cirrhosis or pre-cirrhosis between September 1979 and May 1995. Ninety-five of these patients developed HCC. The clinical characteristics of patients at the time of surgery for varices, stages (F0-F4) of the progression of fibrosis, and grades (A0-A3) of necroinflammatory activity in liver biopsy tissue obtained at surgery in 170 patients based on the New Inuyama Classification (Int. Hepatol. Commun. 6 (1996) 112), were analyzed to investigate their relationship with the development of HCC. In addition, the levels of AST and ALT were followed every 3 months after surgery in 116 patients, and were divided into 2 groups at 80 IU/ml to compare the level of risk for the development of HCC. In liver biopsy tissue, group F4 (n=68/152, 45%) showed a significantly higher (P=0.0224) rate of appearance of HCC than group F3 (n=3/18, 17%). Group F4 also tended to show a higher cumulative HCC appearance rate of 55% compared with 37% for group F3 at 10 years after surgery (P=0.097). In regard to activity, the appearance rate of HCC in group A2+A3 (n=52/112, 51%) was significantly higher (P=0.0008) than that of HCC in group A1 (n=14/58, 25%). The cumulative appearance rate (60%) of HCC in group A2+A3 was significantly higher than that (31%) in group A1 at 10 years after surgery (P=0.0003). The appearance rate of HCC was significantly higher in the group (n=33/44, 75%) with a mean AST level >/=80 IU/ml than in the group (n=41/72, 57%) with a mean AST level <80 IU/ml (P=0.0496). A multivariate analysis of the risk factors for the development of HCC showed that necroinflammatory activity was a risk factor. These results suggested that the histopathologic classification (the New Inuyama Classification) of liver biopsy tissue from patients who underwent non-shunt operation for esophageal varices due to underlying cirrhosis or pre-cirrhosis is useful for predicting the development of HCC, to which the grades of necroinflammatory activity in particular are more closely related.
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PMID:Clinicalpathological analysis of risk factors for the development of hepatocellular carcinoma after surgery for esophageal varices due to underlying cirrhosis or pre-cirrhosis in the 397 patients. 1264 40

Since 1975, our experience in the treatment of biliary atresia with Kasai's technique has improved little by little, achieving 65% favourable outcome in the last five years. We define "good results" as the complete restoration of biliary flow and normalization of bilirrubin levels. The long-term evolution of these good results can be diverse. The objective of the present work is to analyze the outcome of patients in our series in whom a favourable initial response was achieved, as well as evaluating their present situation and future perspectives. The authors present a total of 17 patients operated by Kasai's technique since 1985, that constitutes the group with good results in our series. The controls were based on general analysis, liver function and periodic ultrasound explorations. All received a standardized medical treatment consisting of vitamin supplements (A, D3, E, K) minerals (zinc, calcium, phosphate, iron) ursodexoxicolic acid, luminal,as well as close control of calorie intake. In two patients the levels of bilirrubine were progressively increased with time, stabilizing at between 5/6 mgs/100 ml, with progressive hepatic hardening, appearance of splenomegalia, indirect signs of portal hypertension and a slight deterioration of hepatic function. One received a transplant at age 12 with Quick levels below 50%. The other, aged 16, continues with an acceptable hepatic function and good quality of life under recommendation of transplant. Eleven patients with ages ranging from fourteen months to seventeen years presented slight and firm hepatomegalia, moderate portal hypertension, GOT 71 +/- 8 mg/100 ml, GPT 97 +/- 11 mg/100 ml and normal bilirrubine levels. From this group, 3 patients, all under five years of age, experienced bleeding from esophageal varices which were controlled by sclerosis and medical treatment (propanolol and isosorbide dinitrate). Recently, one three year-old patient developed a hepatocarcinoma of rapid, mortal evolution. Since then, the determination of alfa-feto protein in follow-up controls has been introduced. Four other patients of 5, 6, 14, 16, years of age are completely assymptomatic with an excellent clinical evolution. In our experience,the patients that overcome the third year after surgery without serious complications seem destined to reach puberty with a good quality of life. However, some cases show signs of hepatic fibrosis and portal hypertension, 77% in our series. Only 23% of patients with a favorable initial evolution appear to present a complete normalization of their hepatic lesion in the long term.
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PMID:[Biliary atresias operated with favourable results: predictable outcome]. 1590 Nov 4

A 57-year-old woman was admitted due to a hemorrhage from esophageal varices. Laboratory tests showed liver dysfunction, elevated immunoglobulin G levels and positivity for anti-nuclear antibodies. Serum hepatitis B virus and hepatitis C virus markers were negative. The liver biopsy specimen was compatible with autoimmune hepatitis, and low-dose prednisolone was started. During the follow-up, her serum alanine aminotransferase levels continued to fluctuate between 40 and 60 IU/l. Nine years later, hepatocellular carcinoma 20 mm in diameter was detected. This case suggests that hepatocellular carcinoma develops even if serum alanine aminotransferase levels are maintained at less than twice the upper normal limit.
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PMID:Development of hepatocellular carcinoma in a woman with HBV- and HCV-negative autoimmune hepatitis with unsatisfactory response to Corticosteroid. 1625 9

Cholesteryl ester storage disease (CESD) is caused by deficient lysosomal acid lipase (LAL) activity, predominantly resulting in cholesteryl ester (CE) accumulation, particularly in the liver, spleen, and macrophages throughout the body. The disease is characterized by microvesicular steatosis leading to liver failure, accelerated atherosclerosis and premature demise. Although CESD is rare, it is likely that many patients are unrecognized or misdiagnosed. Here, the findings in 135 CESD patients described in the literature are reviewed. Diagnoses were based on liver biopsies, LAL deficiency and/or LAL gene (LIPA) mutations. Hepatomegaly was present in 99.3% of patients; 74% also had splenomegaly. When reported, most patients had elevated serum total cholesterol, LDL-cholesterol, triglycerides, and transaminases (AST, ALT, or both), while HDL-cholesterol was decreased. All 112 liver biopsied patients had the characteristic pathology, which is progressive, and includes microvesicular steatosis, which leads to fibrosis, micronodular cirrhosis, and ultimately to liver failure. Pathognomonic birefringent CE crystals or their remnant clefts were observed in hepatic cells. Extrahepatic manifestations included portal hypertension, esophageal varices, and accelerated atherosclerosis. Liver failure in 17 reported patients resulted in liver transplantation and/or death. Genotyping identified 31 LIPA mutations in 55 patients; 61% of mutations were the common exon 8 splice-junction mutation (E8SJM(-1G>A)), for which 18 patients were homozygous. Genotype/phenotype correlations were limited; however, E8SJM(-1G>A) homozygotes typically had early-onset, slowly progressive disease. Supportive treatment included cholestyramine, statins, and, ultimately, liver transplantation. Recombinant LAL replacement was shown to be effective in animal models, and recently, a phase I/II clinical trial demonstrated its safety and indicated its potential metabolic efficacy.
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PMID:Cholesteryl ester storage disease: review of the findings in 135 reported patients with an underdiagnosed disease. 2348 21

ABO-incompatible living-donor liver transplantation (ABO-LDLT) is generally more difficult to perform than ABO-incompatible kidney transplantation. Despite introduction of rituximab, ABO-LDLT in non-responders is a still difficult issue. A 23-year-old woman with primary sclerosing cholangitis underwent LDLT. The recipient's blood type was 0(+) and the donor's was B(+). Rituximab was infused twice on preoperative day (POD) 14 and 7. Plasma exchange (PE) was performed on PODs 5, 3, 2, and 1. However, repeated PE failed to decrease the anti-B antibody titer. On the other hand, preoperative esophagogastroscopy revealed esophageal varices with red color sign. Therefore, simultaneous liver transplantation and Hassab operation were performed. The donor left lobe of the liver was orthotopically transplanted into the recipient following Hassab operation. Flow cytometry on the day of surgery showed that the frequencies of B cells (CD20+) and memory B cells (CD20+/CD27+) in the peripheral blood were 0.9% and 0.3%, respectively; flow cytometry of cells recovered from the spleen revealed that the frequencies of B cells and memory B cells were 2.5% and 2.4%, respectively. Acute cellular rejection occurred on POD 15, and was treated by steroid pulse therapy, leading to a decrease in the anti-B antibody titer. The liver was functioning well on POD 390 (AST 19, ALT 34). In non-responders to ABO-LDLT, anti-donor blood type antibody-producing cells remains in the spleen after the conventional preoperative regimen. Splenectomy is an option for ABO-LDLT non-responders.
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PMID:Discrepancy of B cell frequency between periphery and spleen after rituximab treatment in ABO-incompatible liver transplantation. 2463 32

Primary biliary cirrhosis (PBC) is an autoimmune, slowly progressive, cholestatic, liver disease characterized by a triad of chronic cholestasis, circulating anti-mitochondrial antibodies (AMA), and characteristic liver biopsy findings of nonsuppurative destructive cholangitis and interlobular bile duct destruction. About 10% of PBC patients, however, lack AMA. A variant, called PBC-autoimmune hepatitis (AIH) overlap, is characterized by the above findings of PBC together with findings of elevated serum alanine aminotransferase, elevated serum immunoglobulin G, and circulating anti-smooth muscle antibodies, with liver biopsy demonstrating periportal or periseptal, lymphocytic, piecemeal necrosis. PBC is hypothesized to be related to environmental exposure in genetically vulnerable individuals. It typically occurs in middle-aged females. Prominent clinical features include fatigue, pruritis, jaundice, xanthomas, osteoporosis, and dyslipidemia. The Mayo Risk score is the most widely used and best prognostic system. Ursodeoxycholic acid is the primary therapy. It works partly by reducing the concentration and injury from relatively toxic bile acids. PBC-AIH overlap syndrome is treated with ursodeoxycholic acid and corticosteroids, especially budesonide. Obeticholic acid and fibrate are promising new, but incompletely tested, therapies. Liver transplantation is the definitive therapy for advanced disease, with about 70% 10-year survival after transplantation. Management of pruritis includes local skin care, dermatologist referral, avoiding potential pruritogens, cholestyramine, and possibly opioid antagonists, sertraline, or rifaximin. Management of osteoporosis includes life-style modifications, administration of calcium and vitamin D, and alendronate. Statins are relatively safe to treat the osteopenia associated with PBC. Associated Sjogren's syndrome is treated by artificial tears, cyclosporine ophthalmic emulsion to stimulate tear production; and saliva substitutes, cholinergic agents, and scrupulous oral and dental care. Complications of cirrhosis from advanced PBC include esophageal varices, ascites, spontaneous bacterial peritonitis, hepatorenal syndrome, and hepatoma formation.
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PMID:Primary biliary cirrhosis: Pathophysiology, clinical presentation and therapy. 2595 76

BACKGROUND Aspartate aminotransferase-to-platelet ratio index (APRI), aspartate aminotransferase-to-alanine aminotransferase ratio (AAR), FIB-4, fibrosis index (FI), and King scores might be alternatives to the use of upper gastrointestinal endoscopy for the diagnosis of esophageal varices (EVs) in liver cirrhosis. This study aimed to evaluate their diagnostic accuracy in predicting the presence and severity of EVs in liver cirrhosis. MATERIAL AND METHODS All patients who were consecutively admitted to our hospital and underwent upper gastrointestinal endoscopy between January 2012 and June 2014 were eligible for this retrospective study. Areas under curve (AUCs) were calculated. Subgroup analyses were performed according to the history of upper gastrointestinal bleeding (UGIB) and splenectomy. RESULTS A total of 650 patients with liver cirrhosis were included, and 81.4% of them had moderate-severe EVs. In the overall analysis, the AUCs of these non-invasive scores for predicting moderate-severe EVs and presence of any EVs were 0.506-0.6 and 0.539-0.612, respectively. In the subgroup analysis of patients without UGIB, their AUCs for predicting moderate-severe varices and presence of any EVs were 0.601-0.664 and 0.596-0.662, respectively. In the subgroup analysis of patients without UGIB or splenectomy, their AUCs for predicting moderate-severe varices and presence of any EVs were 0.627-0.69 and 0.607-0.692, respectively. CONCLUSIONS APRI, AAR, FIB-4, FI, and King scores had modest diagnostic accuracy of EVs in liver cirrhosis. They might not be able to replace the utility of upper gastrointestinal endoscopy for the diagnosis of EVs in liver cirrhosis.
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PMID:Diagnostic Accuracy of APRI, AAR, FIB-4, FI, and King Scores for Diagnosis of Esophageal Varices in Liver Cirrhosis: A Retrospective Study. 2668 74

In patients with upper gastrointestinal bleeding (UGIB), identifying those with esophageal variceal hemorrhage prior to endoscopy would be clinically useful. This retrospective study of a large cohort of patients with UGIB used logistic regression analyses to evaluate the platelet count, aspartate aminotransferase (AST) to platelet ratio index (APRI), AST to alanine aminotransferase (ALT) ratio (AAR) and Lok index (all non-invasive blood markers) as predictors of variceal bleeding in (1) all patients with UGIB and (2) patients with cirrhosis and UGIB. 2233 patients admitted for UGIB were identified; 1034 patients had cirrhosis (46%) and of these, 555 patients (54%) had acute UGIB due to esophageal varices. In all patients with UGIB, the platelet count (cut-off 122,000/mm(3)), APRI (cut-off 5.1), AAR (cut-off 2.8) and Lok index (cut-off 0.9) had area under the curve (AUC)s of 0.80 0.82, 0.64, and 0.80, respectively, for predicting the presence of varices prior to endoscopy. To predict varices as the culprit of bleeding, the platelet count (cut-off 69,000), APRI (cut-off 2.6), AAR (cut-off 2.5) and Lok Index (0.90) had AUCs of 0.76, 0.77, 0.57 and 0.73, respectively. Finally, in patients with cirrhosis and UGIB, logistic regression was unable to identify optimal cut-off values useful for predicting varices as the culprit bleeding lesion for any of the non-invasive markers studied. For all patients with UGIB, non-invasive markers appear to differentiate patients with varices from those without varices and to identify those with a variceal culprit lesion. However, these markers could not distinguish between a variceal culprit and other lesions in patients with cirrhosis.
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PMID:Prediction of esophageal varices and variceal hemorrhage in patients with acute upper gastrointestinal bleeding. 2691 6


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