Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Splanchnic hemodynamics and portal systemic shunting were measured in eight dogs with experimentally induced portal fibrosis and splenomegaly and in six normal dogs by the radioactive microsphere technique. Portal fibrosis and splenomegaly were produced by repeated intraportal injections of a mixture of killed nonpathogenic Escherichia coli and dog anti-E. coli serum. All E. coli-treated dogs developed intrahepatic presinusoidal portal hypertension (portal vein pressure 15.8 +/- 5.4 vs. 7.5 +/- 0.9 mmHg in controls, P less than 0.005; intrahepatic pressure 6.8 +/- 2.1 vs. 6.2 +/- 1.4 mmHg in controls, NS) within 2.5 mo, but no portal systemic shunt was demonstrated at this time (2.1 +/- 1.5 vs. 0.7 +/- 0.4%, NS). Portal venous inflow, the total blood flow within the portal system, was increased in the treated dogs (27.6 +/- 6.6 vs. 18.2 +/- 2.5 ml X min-1 X kg body wt-1, P less than 0.005). Total splanchnic arterial vascular resistance was reduced in these dogs (26.0 +/- 10.4 vs. 40.9 +/- 4.6 dyn X s X cm-5 X 10(3), P less than 0.01) as a result of reduced arteriolar resistance in the spleen, jejunum and ileum, colon, and omentum, in all of which blood flow increased. In these animals both portal venous flow (27.0 +/- 6.5 vs. 18.1 +/- 2.5 ml X min-1 X kg body wt-1, P less than 0.005) and intrahepatic portal vascular resistance (1.9 +/- 0.7 vs. 0.7 +/- 0.3 dyn X s X cm-5 X 10(3), P less than 0.005) were increased.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Splanchnic hemodynamics in portal hypertensive dogs with portal fibrosis. 329 82

Recently, it has been suggested that there is an immunological mechanism in the etiology of idiopathic portal hypertension (IPH) and that the spleen plays a very important role. In the present study, the extract of human IPH spleen with Freund's complete adjuvant (FCA) was injected into rabbits, which were then examined both immunologically and pathologically. The results of the study are summarized as follows: Portal venous pressure was significantly increased in rabbits sensitized for more than one hundred days. Portal venous pressure of 175 mm H2O or more was observed in approximately 80% of the animals. Splenomegaly of 3.0 g or more was noted in all sensitized rabbits. Maximum spleen weight was 8.0 g. Peripheral blood cell counts decreased as the duration of sensitization increased. In immunological examination, smooth muscle antibodies, anti-DNA antibodies and antimitochondrial antibodies, were noted in some rabbits. In histological examination, the livers of sensitized rabbits in the early stage showed the infiltration of small round cells and fibrosis into Glisson's capsule. These changes were found to increase in later stages. In some rabbits, fibrosis around the interlobular bile duct was observed. The spleens of sensitized rabbits in the early stage showed splenitis with infiltration of small round cells and plasma cells. Sinus hyperplasia and fibrosis in the red and white pulp increased progressively as the period of sensitization lengthened. These results are similar in many respects to the clinical and pathological features of IPH. Thus, it is strongly suggested that the immunological mechanism may affect the pathogenesis of IPH in human.
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PMID:[Experimental studies on idiopathic portal hypertension (IPH)--prolonged sensitization of rabbits with human spleen of IPH]. 378 35

The authors report 15 cases of nodular regenerative hyperplasia (NRH) of the liver observed in 10 women and 5 men during a 9 year period. Gastrointestinal bleeding due to ruptured esophageal varices revealed the liver disease in 11 cases. Hepatomegaly and splenomegaly were noted in 9 cases and ascites in 7. Anicteric cholestasis was demonstrated in 10 cases. Another disease, e. g. myelofibrosis and monoclonal gammapathy, was present in 11 patients. In 10 patients, portal diversion was performed; outcome being favorable with a follow-up of one to six years. The analysis of these cases and of the 113 previously published reports calls for the following comments: 1) In most cases, NRH is characterized by small-sized hepatocytic nodules scattered throughout the entire liver with no surrounding fibrosis; however this histological pattern may vary somewhat, with adjacent normal zones being found adjacent to typical cirrhotic fibrosis; although a precise morphometric study was not performed in our patients, obstruction of the tiny branches of intrahepatic portal veins was not observed. 2) Histological diagnosis of NRH is difficult and in most cases requires surgical biopsy specimens and specific coloration of the reticulin network. 3) NRH must be considered as a new cause of intrahepatic (sinusoidal or presinusoidal) portal hypertension and/or of chronic anicteric cholestasis. 4) A number of various conditions may be associated with NRH, the most frequent being Felty's syndrome and myeloproliferative disorders. 5) The pathogenesis of NRH remains unknown. 6) Portal diversion generally has a favorable outcome in this disease.
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PMID:[Nodular regenerative hyperplasia of the liver. Study of 15 cases and review of the literature]. 662 10

In rats, surgical creation of a portacaval shunt leads to hepatic atrophy and lowered levels of cytochrome P450, the key component of liver enzymes involved with drug metabolism. These effects are largely attributable to diversion of portal blood away from the liver and not to decreased hepatic blood flow. The present study has established a simpler model of portal blood diversion in order to examine the role of portal blood constituents in the regulation of hepatic cytochrome P450. Portal vein ligation was performed on male Wistar rats in which portasystemic anastomoses had been produced by subcutaneous transposition of the spleen. Portal vein ligation resulted in portal hypertension, as evidenced by splenomegaly, and in hepatic atrophy. In liver of rats with portal vein ligation, microsomal cytochrome P450 levels were significantly less than in sham-operated control rats, but cytochrome b5, NADPH-cytochrome c reductase, and glucose-6-phosphatase were unaltered. The activities of four mixed function oxidases also were reduced significantly in the liver of rats with portal vein ligation, the changes being greatest for ethylmorphine N-demethylase, a prototype substrate for the phenobarbital-inducible isoenzyme of cytochrome P450. In contrast, the activity of microsomal heme oxygenase, the rate-limiting step in catabolism of heme to bilirubin, was enhanced after portal vein ligation. Experiments in pair-fed rats showed that the changes observed in liver from rats with portal vein ligation could not be attributed to caloric deprivation. Administration of phenobarbital increased liver mass, cytochrome P450 levels, and mixed function oxidase activities both in rats with portal vein ligation and in controls, indicating that the liver of the ligated rats retained considerable protein synthetic capacity. It appears that hepatic atrophy and lowering of cytochrome P450 levels that follow portal vein ligation are consequences of altered exposure of the liver to factors normally present in portal blood, and that the same alterations may also enhance heme oxygenase activity.
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PMID:Portal vein ligation selectively lowers hepatic cytochrome P450 levels in rats. 686 53

Splenectomy and splenic embolization have been advocated as definitive therapy in cirrhotic patients bleeding from varices. While splenomegaly is commonly associated with portal hypertension, no clear hemodynamic link between portal pressure and splenic enlargement has yet been established. In an effort to clarify the hemodynamic significance of splenomegaly in portal hypertensive patients the relationship between spleen size and portal pressure was retrospectively reviewed and the contribution of splenic inflow to portal hypertension prospectively studied. In 50 consecutive cirrhotic variceal bleeders studied angiographically, there was no correlation between spleen size and corrected sinusoidal pressure. Portal pressure was then prospectively measured before and after splenic vein clamping in 12 cirrhotic patients undergoing distal splenorenal shunt. No significant pressure drop occurred following elimination of splenic venous flow. On the basis of these data, there would appear to be no firm hemodynamic basis for splenectomy or splenic embolization alone in the unselective management of cirrhotic patients with variceal bleeding.
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PMID:Splenomegaly and variceal bleeding--hemodynamic basis and treatment implications. 772 Dec 48

The differences in sonographic measurements of the splanchnic vessels, their modifications during respiration, the spleen size and the presence of collaterals of the portal system among 110 cirrhotic patients with portal hypertension and 30 control subjects are analyzed to verify the usefulness of conventional sonography in the diagnosis of portal hypertension. We achieved a sensitivity above 70% and a specificity of at least 90% with the following signs: 1) dilation of portal, splenic and superior mesenteric veins, 2) limited variations of the latter two during respiration, and 3) splenomegaly. A higher sensitivity was achieved considering variations in splenic caliber during respiration under 33% (91%) or superior mesenteric vein during forced expiration above or equal than 7 mm (88%). These measurements could be found in 71.8% and 57.2% respectively. On the other hand, values of portal vein and spleen size were easily obtained. The usefulness of all measurements persists if we take into account only patients with Child-Pugh score < 7. Collateral circulation was demonstrated in 18%. Portal vein above 14 mm, variations in splenic caliber under 13% or superior mesenteric vein during forced expiration above or equal than 9 mm were obtained in any of control subjects but respectively in 33%, 46% and 69% of patients in portal hypertension group. We conclude that ultrasonography is a reliable and noninvasive tool in the diagnosis of portal hypertension in cirrhotic patients.
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PMID:[The usefulness of abdominal echography in the diagnosis of portal hypertension in cirrhotic patients]. 798 98

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

Although idiopathic portal hypertension (IPH) is clinically characterized by portal hypertension and marked splenomegaly, we have experienced a case of spontaneous portal-systemic shunt without splenomegaly in whom the liver histology resembled IPH but with normal portal pressure. We admitted a 64 year old man who had suffered from hepatic encephalopathy for 2 years. Laparoscopy revealed a dark grey liver with a sharp edge and a concave surface. Examination of a liver biopsy specimen revealed peri-portal fibrosis consistent with IPH. A single, large, portal-systemic shunt was identified by percutaneous transhepatic portography. The shunt arose from the left gastric vein and flowed through the left renal vein into the inferior vena cava. No varices were identified. There were no morphological changes in the hepatic or portal veins. Portal vain pressure was normal. There was a slight difference between the portal pressure and the wedged hepatic vein pressure, suggesting a presinusoidal block. This case raises important questions concerning the aetiology of IPH and the relationship between portal hypertension and the development of collateral venous circulation.
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PMID:Portal-systemic shunting in a patient with normal portal vain pressures and histological evidence of idiopathic portal hypertension. 874 32

A prospective case controlled study was conducted to evaluate the value of real time ultrasound in establishing the diagnosis and the etiology of portal hypertension. Patients attending the outpatient department of a tertiary care centre were included. There were 324 consecutive patients with portal hypertension due to cirrhosis (n = 229), non cirrhotic portal fibrosis (NCPF: n = 64) and extrahepatic portal venous obstruction (EHPVO: n = 31). During this period, 146 patients with dyspepsia, 35 with splenomegaly and 32 with ascites due to varied causes served as negative and positive controls. Real time ultrasonography using a 3.5 MHz linear array scanner was performed in a fasting state in all subjects. Portal and splenic vein diameter greater than 10 mm, splenomegaly, hepatic and splenic hilar collaterals were suggestive of portal hypertension. Non visualization of the portal vein which was replaced by a cavernoma had a diagnostic accuracy of 98% in EHPVO. Splenic infarcts and absence of ascites were features of non cirrhotic portal hypertension (NCPF and EHPVO). Sonography had an overall diagnostic accuracy of 80%. A stepwise logistic regression with multivariate analysis using discriminate function showed that collaterals at the hepatic and splenic hilum, hepatomegaly, ascites and splenic infarcts were independent markers to differentiate cirrhotic from noncirrhotic causes of portal hypertension. The discriminate equation generated had a mismatch of 9.8%. Correlations between the sonographic signs demonstrated that the variceal grade correlated positively with the presence of splenic hilar collaterals and the liver size inversely correlated with presence of ascites. It was concluded that real time ultrasonography is an accurate method to establish the presence and etiology of portal hypertension.
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PMID:Sonographic signs in portal hypertension: a multivariate analysis. 878 73

Lethal disease in Schistosoma mansoni infections is mostly due to portal hypertension caused by hepatic periportal fibrosis. To evaluate the factors that may determine severe disease, livers and spleens were examined by ultrasound in a Sudanese population living in a village where S. mansoni is endemic. Early (FI), moderate (FII), or advanced (FIII) fibrosis was observed in 58%, 9%, and 3% of the population, respectively. Although FI affected 50%-70% of the children and adolescents, FII prevalence was low in subjects </=20 years old but increased sharply (45%-58%) in men 21-30 years old and was associated with the highest infections. Portal and splenic vein diameters were increased in one-third of persons with FII and in almost all with FIII disease. Severe disease, FII or FIII with portal hypertension, affected 6% of the population, was associated with splenomegaly, occurred mostly in adult men, and was clustered in a few pedigrees. These observations suggest that infection intensity and duration, gender-related factors, and inherited factors are important in fibrosis development.
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PMID:Susceptibility to periportal (Symmers) fibrosis in human schistosoma mansoni infections: evidence that intensity and duration of infection, gender, and inherited factors are critical in disease progression. 1047 61


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