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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical presentation and outcome of 32 children with primary sclerosing cholangitis (PSC) are reviewed, the largest North American series. The majority of patients were diagnosed in their second decade (median age: 13 years). Four children presented before the age of 2 years, but none in the neonatal period. Seventeen patients had inflammatory bowel disease (IBD), all with colitis, 14 ulcerative colitis, and 3 Crohn's disease. Eight patients presented with chronic liver disease before clinical onset of IBD. Only 8 of 32 patients were jaundiced at presentation. Fifteen of 32 had a normal serum alkaline phosphatase (ALP) level at presentation. Nine children presented with features similar to those of autoimmune hepatitis. Cholangiography was performed in all cases and classified by a scoring system specifically developed for pediatric patients. Intrahepatic disease predominated; in only three cases a common bile duct stricture was identified requiring stenting. Findings on the initial liver biopsy were classified according to Ludwig's criteria for staging PSC: there were 15 biopsies in stages 1 to 2 and 17 biopsies stages 3 to 4. HLA class I and II antigens were determined in 27 patients. An increased incidence of HLA B8 and DR2(15) but not DRw52a (DRB3*0101) was found. Anti-neutrophil cytoplasmic antibody (ANCA) was positive in 10 of 24 patients tested. Survival analysis indicated that a later age at presentation, splenomegaly, and prolonged prothrombin time (PT) at presentation were significant contributors to the prediction of poor outcome (i.e., death or listing for transplantation). Liver transplantation was successfully performed in seven children. Physicians must maintain a high index of suspicion of PSC in any child or young adult presenting with chronic liver disease, especially in the presence of IBD, even with a normal serum alkaline phosphatase level.
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PMID:Primary sclerosing cholangitis in 32 children: clinical, laboratory, and radiographic features, with survival analysis. 759 Jun 57

Diagnostic laparoscopy continues to have a role in the evaluation and diagnosis of acute and chronic liver diseases, primary and metastatic liver tumors, and peritoneal diseases. We retrospectively reviewed the records of 1794 diagnostic laparoscopies performed at our institution from 1987 to 1992 to identify the indications, results, and safety of this procedure in our training program. A definitive diagnosis was made in 91% of cases with biopsy performed in 93%. Chronic liver disease was evaluated in 890 patients, and a diagnosis was made in 98%. Four hundred thirty-seven patients were evaluated for suspected primary or metastatic carcinoma, and a diagnosis was made in 85%. Ascites was evaluated in 73 patients, and a diagnosis was made in 82%. One-hundred sixty-four patients were evaluated for abnormal liver function tests, and a diagnosis was made in 91%. HIV-related liver function test abnormalities were evaluated in 67 patients, and a diagnosis was made in 81%. One hundred sixty-three patients underwent diagnostic laparoscopy for the evaluation of hepatomegaly, splenomegaly, unexplained portal hypertension, fever of unknown origin, and cholestasis, and a diagnosis was made in 74% of cases. Eight major complications (including abdominal viscus perforation, hemobilia, splenic laceration, bleeding) and thirty-one minor complications were seen. Our findings confirm that diagnostic laparoscopy is a safe and valuable procedure in the evaluation of chronic liver disease.
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PMID:Diagnostic laparoscopy: a 5-year experience in a hepatology training program. 763 26

The association between chronic liver disease and plasmatic lipids and glucidic metabolism was studied. The liver was evaluated with biochemical variables, ultrasound and upper gastrointestinal endoscopy. 12 chronic liver diseases patients and 10 normal were studied. 83.3% of patients showed lipidic abnormalities, 66.6% on the cholesterol levels and 41.7%, reduced HDL. When patients received 100 gr of glucose the response was abnormal in 83.3%. These results were compared with other "liver function test". 75% had abnormal bilirubin, 58.3% abnormal albumin, prothrombin time 58.3%, aminotransferases 75%. Esophageal verices were found in 75% of cases and ultrasound abnormalities in 91.6% (27.3% cirrhosis, 54.4% diffuse abnormal patter and 18.2% splenomegaly). These findings show that lipidic and glucidic metabolism, and ultrasound, in chronic liver diseases are the more frequent abnormalities.
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PMID:[Metabolic markers of chronic liver disease]. 856 66

Ultrasound examination should be carried out as the first investigation in all patients with suspected hepatobiliary disease. It has a high accuracy in detecting signs of portal hypertension such as ascites, splenomegaly and collateral veins. The most common cause of portal hypertension in the Western world is cirrhosis. Although there is an overlap of ultrasound findings in normal liver, fatty liver, chronic liver disease with and without fibrosis, and cirrhosis, a correct diagnosis of cirrhosis by judgment of ultrasound findings is reported to be achieved in up to 80%. Diagnostic parameters used are size and outline of the liver, its reflectivity and attenuation of the echo-pattern. If used in combination with the Doppler duplex system, the information gained vastly increases. Areas where this technique is able to provide useful and reliable data have been defined in a consensus conference held in Bologna. Doppler flowmetry of hepatic vessels allows detection of presence, direction and characteristics of blood flow. The reliability of the quantitative measurements of blood flow in abdominal vessels is still questioned. When using the same equipment by skilled operators, however, duplex-Doppler data of the portal vein are of value in the assessment of the risk of variceal bleeding, in the evaluation of the progression of liver disease and in the evaluation of medical therapy for portal hypertension. Furthermore, the technique is of practical value in pre-and postoperative examinations for shunt surgery as well as in the pre-procedural work-up of transjugular intrahepatic portosystemic stent shunt (TIPS), during the TIPS procedure and in the post-TIPS followup.
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PMID:Ultrasound and duplex-Doppler in the diagnosis and follow-up of portal hypertension. 877 98

Artificial neural networks were used in the diagnosis of chronic liver disease based on liver scintiscanning. One hundred and thirty-seven patients with chronic liver disease (12 with chronic persistent hepatitis, 39 with chronic aggressive hepatitis, and 86 with cirrhosis) and 25 healthy controls were studied. Sixty-five subjects (10 healthy controls, 20 patients with chronic hepatitis, and 35 patients with cirrhosis of the liver) were used in the establishment of a neural network. Liver scintiscans were taken starting 20 min after the intravenous injection of 111 MBq of Tc-99m-phytate. The neural network was used to evaluate five items judged from information on liver scintiscans: the ratio of the sizes of the left and right lobes, splenomegaly, radioactivity in the bone marrow, deformity of the liver and distribution of radioactivity in the liver. The neural network was designed to distinguish between three liver conditions (healthy liver, chronic hepatitis and cirrhosis) on the basis of these five items. The diagnostic accuracy with the neural network was 86% for patients with chronic hepatitis and 93% for patients with cirrhosis. With conventional scoring, the accuracy was 77% for patients with chronic hepatitis and 87% for patients with cirrhosis. Our findings suggest that artificial neural networks may be useful for the diagnosis of chronic liver diseases from liver scintiscans.
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PMID:Diagnosis of chronic liver disease from liver scintiscans by artificial neural networks. 921 85

Chronic liver disease is often accompanied by hypoxaemia. We investigated the clinical factors that were related to the arterial oxygen tension (PaO2) in 40 women, all non-smokers with chronic liver disease. They were positive for hepatitis C virus (HCV) antibody and had no evidence of cardiopulmonary disease. Arterial blood was collected from patients at rest (> 15 min) for analysis of blood gases. We determined the correlation between blood gas tension and the clinical variables, i.e. the presence or absence of skin manifestations such as cutaneous spider nevi and palmar erythema, the presence or absence of splenomegaly, vital capacity, forced expiratory volume in one second, V25/body height, serum alanine aminotransferase (AST), serum asparate aminotransferase (ALT), serum cholinesterase, serum gamma-globulin/total protein, excretion of indocyanine green at 15 min (15-min retention rate, ICG level), blood level of ammonia, blood level of endotoxin, plasma level of glucagon and the serum level of type IV collagen-7S. The mean level of PaO2 was 78 +/- 11 (range: 43-95) torr. The mean alveolar-arterial oxygen tension gradient (A-aDO2) was 19 +/- 13 (range: 2-60) torr. Multiple regression analysis used PaO2 and A-aDO2 as objective variables, and the clinical findings as explanatory variables. The explanatory variables that were significantly correlated with blood gas values were ICG level, blood level of endotoxin and presence of skin manifestations. The ICG level showed a high correlation with blood gas values; the ICG level increased, the PaO2 decreased (r = -0.69), while the A-aDO2 showed a high positive correlation (r = +0.78, P < 0.001). Findings suggest that a reduction in hepatic blood flow and hepatocellular function interfere with the inactivation of vasoactive substances such as endotoxin by the liver, leading to the development of skin manifestations, the dilatation of intrapulmonary capillaries and the induction of hypoxaemia.
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PMID:Clinical factors that affect blood gases in non-smoking women with chronic liver disease. 951 26

Recent advances in serodiagnosis of hepatotropic viruses have revolutionized the approach to diagnosis and understanding of chronic liver disease (CLD). There are few studies on CLD in children from India. The present study was planned to define the clinical spectrum of CLD in children, its histopathology and seroepidemiology. Forty children with clinical features satisfying the criteria for diagnosing chronic liver disease were studied. All underwent routine laboratory investigations, liver function tests and ultrasound scan of the abdomen. Liver biopsy, upper GI endoscopy and other special investigations were done wherever indicated. The most common presenting features were jaundice (70%), fever (67%), and abdominal distention (60%). On examination hepatomegaly and icterus (80% each) and splenomegaly (67%) were the commonest findings. Serum transaminases were raised in 62.5% of children while prothrombin time was prolonged in 75% patients. Oesophageal and/or gastric varices were seen in 13 out of 29 patients subjected to upper GI endoscopy. Hepatitis B surface antigen (HbsAg) was positive in 5 children (12.5%) while 3 (7.5%) tested positive for anti HCV antibody. The commonest histopathological diagnosis was infantile cholangiopathy (20%) followed by cryptogenic cirrhosis and idiopathic chronic active hepatitis (17.5% each). The study suggests that the incidence of chronic hepatitis B and C is rather low in childhood. However larger and longer studies are required to delineate the exact incidence of these conditions in childhood and their progression in adolescence and early adulthood.
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PMID:Clinical spectrum of chronic liver disease in north Indian children. 961 2

Splenomegaly was studied retrospectively at the University of California, San Francisco, School of Medicine, on all patients (N = 2,056) for the years 1913 to 1962. The patients were classified into several diagnostic groups, and these groups were tested for statistical significance (chi(2)) with many clinical and laboratory variables to determine their predictive value. Hematologic disorders were associated with 57% of cases of splenomegaly and 81% of cases of massive splenomegaly. Among patients with splenomegaly, 19% had infectious diseases, 11% had hepatic diseases, and 9% had congestive or inflammatory disorders. The residual 4% were considered to have primary splenic disorders or a disorder of unknown cause. The commonest diseases associated with splenomegaly were hematologic (acute and chronic leukemias), infectious (malaria, endocarditis, and tuberculosis), hepatic (chronic liver disease), congestive (congestive heart failure), inflammatory (thyrotoxicosis), and other (cancers not metastatic to the spleen). The diseases most frequently associated with massive splenomegaly were the chronic leukemias. The disease with the highest incidence of massive splenomegaly was myelofibrosis (23 of 29 patients, 78%). Splenectomy was performed in 154 patients (7%), primarily for hematologic amelioration and hepatic hypersplenism. Hematologic diseases showed significant associations with lymphadenopathy, generalized lymphadenopathy, massive splenomegaly, and cytoses (P .001) and with progressive splenic enlargement (P < .02). Infectious diseases showed significant association with fever, and hepatic diseases showed significant association with abnormal results of liver function tests (P < .001). The findings of this retrospective study should be validated prospectively.
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PMID:Splenomegaly in 2,505 patients in a large university medical center from 1913 to 1995. 1913 to 1962: 2,056 patients. 973 88

Splenomegaly was studied retrospectively at the University of California, San Francisco (UCSF), School of Medicine in 301 patients from 1963 to 1995 and compared with the UCSF service of the San Francisco General Hospital Medical Center (SFGH) in 148 patients from 1979 to 1994. The combined 449 patients were classified into several diagnostic groups and were studied by means of several clinical and laboratory associations. Hepatic disease in the percentage of patients at UCSF (with those at SFGH given in parentheses) was associated with splenomegaly in 29% (41%), hematologic disease, 32% (16%); infectious diseases, 16% (36%); congestive or inflammatory disease, 10% (4%); primary splenic disease, 6% (1%); other, 5% (1%); and cause unknown, 2% (1%). Massive splenomegaly occurred in 27% of the patients of the combined series, particularly in patients with hematologic diseases. The acquired immunodeficiency syndrome (AIDS) occurred in more than half of the patients with infectious diseases at SFGH and was four times frequent than in the patients at UCSF. The commonest diseases associated with splenomegaly were hematologic (lymphoma), hepatic (chronic liver disease), infectious diseases (AIDS and endocarditis), congestive (congestive heart failure), primary splenic (splenic vein thrombosis), and other (malignancy not metastatic to the spleen). In 11 patients with AIDS and massive splenomegaly, Mycobacterium avium complex occurred in 8 (73%). Splenectomy was performed in 117 patients (26%), primarily for hematologic amelioration. I conclude that for splenomegaly of unknown origin, the invasive procedure of choice for patients with hematologic associations may be a bone marrow biopsy; for hepatic association, a liver biopsy; and for infectious disease associations, a lymph node biopsy, before any consideration of a diagnostic splenectomy.
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PMID:Splenomegaly in 2,505 patients at a large university medical center from 1913 to 1995. 1963 to 1995: 449 patients. 973 89

Laparoscopy is a relatively safe invasive procedure. However, the rate of bleeding complications during this procedure is still debatable. Moreover, it is not clear whether portal hypertension may increase the risk of this event. The authors analyzed retrospectively the records of 1,000 consecutive patients with chronic liver disease undergoing laparoscopy and guided direct-vision hepatic biopsy, and they examined the rate of bleeding complications from the trocar site after insertion of the Veress needle or after liver biopsy. A total of 400 of 1,000 patients had liver cirrhosis. Of these, 22.7% had splenomegaly, 13.0% had laparoscopic signs of portal hypertension, and 8.2% had esophageal varices. Bleeding occurred in 0.9% of patients from the trocar site, in 0.2% from the biopsy site, and in 0% from the Veress needle site. These figures were independent of the presence of advanced liver disease, with or without portal hypertension. Bleeding complications occur rarely during laparoscopy with guided liver biopsy and do not seem to be related per se to the presence of liver cirrhosis and portal hypertension. If anything, bleeding complications represent an unpredictable event in most cases.
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PMID:Are liver cirrhosis and portal hypertension associated with an increased risk of bleeding during laparoscopy? A retrospective analysis of 1,000 consecutive cases. 1096 46


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