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

Twenty patients with anemia and massive splenomegaly were studied in order to elucidate the mechanism by which splenomegaly results in plasma volume expansion. In 18 patients, increased plasma volume accounted for most of the anemia. Fourteen patients had an exaggerated renin response to standing, mean 1967 +/- 613 (SE) ng angiotensin ll/100 ml plasma (p less than 0.05). The mean resting forearm blood flow was increased 3.47 +/- 0.32 (SE) ml/100 ml forearm tissue (p less than 0.001). The venous capacitance was normal, as contrasted to a marked decrease in venous capacitance in patients with anemia of comparable degree without splenomegaly. Cardiac indices were increased in 10 of 11 patients (range 4.1-8.1 liters/min/sq m). In nine of ten patients oxygen consumption was increased (range 147-231 ml/min/sq m). Splenectomy was performed on 14 patients. Splenic blood flow was elevated in four of four patients (range 750-2000 ml/min). Splenic A-V oxygen difference was exaggerated in seven of seven patients and in three of three patients splenic indocyanine-green dye dilution curve failed to show an early peak suggestive of A-V shunting in the spleen. Free portal pressure was elevated in 12 of 12 patients and decreased immediately after splenectomy. The intravascular albumin mass decreased in ten patients, was unchanged in three at 2-4 mo after splenectomy, and was accompanied by a rise in the plasma albumin concentration in nine. These data suggest that a flow-induced portal hypertension with expansion of the portal vascular space is an important early hemodynamic change. This finding, together with a decreased peripheral resistance, probably results in a decrease in effective intravascular volume, resulting in stimulation of the renin-angiotensin-aldosterone system and other renal hemodynamic changes necessary for salt and water retention. Splenectomy usually accomplishes a complete reversal of these abnormalities and correction of the anemia.
 ...
PMID:Mechanism of dilutional anemia in massive splenomegaly. 126 Jan 26

To clarify the effect of splenomegaly on portal hemodynamics in patients with portal hypertension and esophageal varices, manometric studies were carried out before and after splenectomy during an operation for esophageal varices. The 118 patients evaluated retrospectively had underlying liver cirrhosis (LC) (62), idiopathic portal hypertension (IPH) (42), and extrahepatic portal occlusion (EHO) (14). The weight of the spleen did not differ significantly among the three diagnostic groups: 640 +/- 473.5 g for LC, 780 +/- 414.6 g for IPH, and 683 +/- 457.2 g for EHO. Before splenectomy, portal pressure was significantly elevated in the patients with EHO (410 +/- 85.2 mm H2O) as compared to either the LC or IPH groups (348 +/- 64.1 and 348 +/- 73.5 mm H2O). Following splenectomy the reduction of portal pressure was significantly greater in the EHO group (29 +/- 15.5%) than in either the LC (18 +/- 17.4%) or IPH (19 +/- 17.0%) groups. Each group was subdivided according to severity of splenomegaly: marked (spleen weight > or = 500 g) or slight (spleen weight < 500 g). Patients with LC and marked splenomegaly showed a reduction in liver function parameters as shown by the prolongation of indocyanine retention rate at 15 min as compared to those with slight splenomegaly. Though it is not statistically significant, the average portal pressure tended to be higher among those with marked splenomegaly.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Portal venous pressure following splenectomy in patients with portal hypertension of differing etiology. 129 40

Splenic trauma with hematoma following extracorporeal shock wave lithotripsy (ESWL) is very rare. We reported a case of subcapsular hematoma of spleen with impending rupture following ESWL for ureteral calculus. This case was noted to have liver cirrhosis and splenomegaly, and received a total of 2000 shock waves under 18 kilovoltage. The subcapsular hematoma occurred 2 months later. Splenectomy was undertaken for a symptomatic huge subcapsular hematoma and thrombocytopenia. We reviewed the literature and concluded that portal hypertension with severe coagulopathy are contraindications for ESWL, even in case with ureteral calculus.
 ...
PMID:Subcapsular hematoma of spleen--a complication following extracorporeal shock wave lithotripsy for ureteral calculus. 129 57

Between July 1986 and April 1989, 334 hospitalized adult Ethiopian patients with chronic liver disease were studied according to a protocol to define their clinical features and to identify risk factors with the aim of preventive intervention. Of these, 14 had chronic hepatitis, 208 cirrhosis and 112 hepatocellular carcinoma (HCC). Both clinical and histological diagnostic criteria were employed. A detailed questionnaire was used to document demographic and clinical data. A common clinical presentation among patients with chronic hepatitis was darkening of the face and hands with or without hypertrichosis of the face and blisters over the dorsi of the hands. This overt or latent form of porphyrea cutanea tarda (PCT) responds to chloroquine. Patients with cirrhosis of the liver commonly present for the first time with ascites, splenomegaly, haematemesis and/or melena from oesophageal varices, and mental changes due to hepatic encephalopathy. Overt or latent forms of PCT are also common features. Peculiar to these cirrhotics is the rarity of spider naevi, gynaecomastia, testicular atrophy, Dupuytren's contracture, parotid gland enlargement and clubbing of the fingers. Exhaustion, loss of appetite, rapid loss of weight, right upper quadrant and/or epigastric pain (all often of less than 6 months' duration, a big, hard, tender and grossly nodular liver with bruit, signs of portal hypertension, and/or hepatic encephalopathy, in a young male with a rapid down hill course characterize the Ethiopian patient with HCC. Serum anti-nuclear factor, anti-mitochondrial anti-bodies and anti-smooth muscle anti-bodies were absent in those with chronic hepatitis and were uncommon in the cirrhotics and HCC cases. One or more hepatitis B virus markers were found in 86% of chronic hepatitis, 88% cirrhosis and 78% HCC and the HBsAg carrier state was found in 36%, 29% and 23%, respectively. Among the HBsAg carriers, HBeAg positivity was less common than anti-HBe but anti-HDV was significantly higher than in the healthy general population. Alphafetoprotein (AFP) levels greater than 500 mg/ml were present in 16 (8%) cirrhotics and 58 (52%) patients with HCC. Histologically, 3 of the chronic hepatitis patients had progressed to cirrhosis, 8 of the cirrhotic patients had chronic active hepatitis and 85% of HCC cases occurred in a background of macronodular cirrhosis. Three cirrhotics developed HCC during follow-up.(ABSTRACT TRUNCATED AT 400 WORDS)
 ...
PMID:Chronic liver disease in Ethiopia: a clinical study with emphasis on identifying common causes. 131

Clinically, idiopathic portal hypertension (IPH) is characterized by overt splenomegaly with pancytopenia, portal hypertension and relatively mild abnormalities in liver function tests. Although its etiology is still undetermined, the liver pathology is characterized by occlusive changes of the intrahepatic portal radicles, portal and periportal fibrosis, irregularly distributed parenchyma atrophies and absent of regeneration nodules. The disease is relatively benign and does not progress to cirrhosis. Differential diagnosis between IPH and liver cirrhosis is mandatory. We now report a case with histologically proven IPH, including clinical course, laboratory data, roentgenographic findings of hepatic venogram and celiac angiogram, hepatic hemodynamic features and intravariceal pressure of esophageal varix which has never been reported in Taiwan.
 ...
PMID:Idiopathic portal hypertension: a case report. 132 89

Vascular invasion is not a prominent feature of cholangiocarcinoma (CCC), in contrast to hepatocellular carcinoma (HCC), which frequently shows extensive vascular tumor thrombi. We report an autopsy case of CCC with extensive portal tumor thrombi and portal hypertension. A 57-yr-old man presented with abdominal pain. Liver imaging revealed no tumors, but showed intrahepatic portal venous obstruction. HCC with portal tumor thrombi was suspected clinically. His clinical course was rapid; he died of hepatic failure 50 days after admission. At autopsy, the liver (2,700 g) was studded with diffuse whitish yellow granular areas with flecks of coalescent granules. Intrahepatic portal veins were diffusely occluded by tumor thrombi. Microscopically, the tumor was poorly differentiated adenocarcinoma with mucin; tumor cells were immunohistochemically positive for carcinoembryonic antigen, CA 19-9, DU-PAN-2, and biliary type cytokeratins, but negative for alpha-fetoprotein. Tumor cells were diffuse in the liver, and there were numerous tumor thrombi in the small portal veins. Hepatic veins and small arteries were occasionally occluded by tumor thrombi. There was ascites, splenomegaly and tumor thrombi in the gastric and esophageal veins, suggesting that portal hypertension had been present. This tumor seemed to have marked affinity to invade portal veins. It must be stressed that there are CCCs with extensive portal tumor thrombi and resultant portal hypertension.
 ...
PMID:Extensive portal tumor thrombi with portal hypertension in an autopsy case of intrahepatic cholangiocarcinoma. 132 98

In heavily infected young patients, there is a "non-congestive" phase of the disease with splenomegaly which can improve after chemotherapy. A strong correlation between hepatosplenic form and worm burden in young patients has been repeatedly shown. The pattern of vascular intrahepatic lesions, seems to depend on two mechanisms: (a) egg embolization, with a partial blocking of the portal vasculature; (b) the appearance of small portal collaterals along the intrahepatic portal system. The role played by hepatitis B virus (HBV) and C virus infections in the pathogenesis of liver lesions is variably considered. Selective arteriography shows a reduced diameter of hepatic artery with thin and arched branches outlining vascular gaps. A rich arterial network, as described in autopsy cases, is usually not seen in vivo, except after splenectomy or shunt surgery. An augmented hepatic arterial flow was demonstrated in infected animals. These facts suggest that the poor intrahepatic arterial vascularization demonstrated by selective arteriography in humans is due to a "functional deviation" of arterial blood to the splenic territory. The best results obtained in treatment of portal hypertension were: esophagogastric devascularization and splenectomy (EGDS), although risk of rebleeding persists; classical (proximal) splenorenal shunt (SRS) should be abandoned; distal splenorenal shunt may complicate with hepatic encephalopathy, although later and in a lower percentage than in SRS. Propranolol is currently under investigation. In our Department, schistosomatic patients with esophageal varices bleeding are treated by EGDS and, if rebleeding occurs, by sclerosis of the varices.
 ...
PMID:Portal hypertension in schistosomiasis: pathophysiology and treatment. 134 92

Systemic mastocytosis is a rare disease of mast cell proliferation with cutaneous and multi-visceral involvement. Portal hypertension and ascites are rare manifestations of systemic mastocytosis. We report a case of systemic mastocytosis presenting with extensive nodular cutaneous lesions and hepatic dysfunction, manifested by portal hypertension (ascites, splenomegaly) and derangement of metabolic function (hyperammonemia, hypoalbuminemia, hypocholesterolemia), a picture resembling that of a common cirrhotic form. The correct diagnosis was established only after tissue sections were appropriately stained for mast cells. On the basis of our and other observations we suggest that systemic mastocytosis be added to the list of infiltrative diseases of the liver with potential evolution to portal hypertension and compromise of biochemical functions.
 ...
PMID:[Systemic mastocytosis with portal hypertension and hepatocellular failure]. 143 8

Splenomegaly is a common finding in patients with portal hypertension. In the present study the relation between spleen size and blood flow in the splenic and portal vein was evaluated in 33 patients with alcoholic liver cirrhosis and portal hypertension using pulsed Doppler sonography (Ultramark 9, ATL, Solingen, FRG). There was a significant positive correlation between hilar spleen diameter (HD) and splenic vein diameter (r = .73, p less than .001) as expected as the consequence of portal hypertension. However, a positive correlation between HD and splenic vein flow (SBF) was found (r = .67, p less than .001). Furthermore, there was no negative correlation between HD and flow velocity in the splenic vein (r = .01, n.s.). Portal blood flow (830 +/- 360 ml/min) was fairly constant in spite of considerable variations in SBF (range: 120 to 1200 ml/min). The data of the present study indicate that splenomegaly in patients with liver cirrhosis and portal hypertension is not simply the consequence of portal congestion resulting in decreased SBF. Rather, increased SBF serves to maintain portal blood flow and thereby contributes to portal hypertension. In few patients (15%) SBF increased to more than 11/min may be an important factor for the severity of portal hypertension. Surgical shunt treatment should be adjusted in these patients.
 ...
PMID:[Splenic size and duplex sonography determination of blood flow in the vena lienalis and vena portae in liver cirrhosis]. 151 Dec 15

The sonographic parameters in portal hypertension (PHT) were examined in a consecutive population of 118 patients who had PHT diagnosed using specific endoscopic, sonographic and Doppler signs. A patent or enlarged paraumbilical vein was found in 85.6% of patients overall and 82.5% of patients with varices indicating a relatively high sensitivity. A portal vein diameter greater than or equal to 13mm was found in only 41.1% and greater than or equal to 15mm in only 20% of patients. A thrombosed portal vein and reversed portal vein flow were present in 3.4% and 5.3% of patients respectively. These signs have only been reported in the context of PHT and are felt to be specific for PHT, but both have a very low sensitivity. Portal vein velocities were highly variable suggesting that this is not a useful predictor of PHT. Splenomegaly was found in only 53.5% of patients demonstrating its poor sensitivity as a sign of PHT. Varices were found in 73.3% of patients overall, and in 100% of patients with a patent or enlarged paraumbilical vein combined with ascites. No other statistically significant correlation between varices and sonographic findings was demonstrated. We conclude that the presence of a patent or enlarged paraumbilical vein is a practical, useful and sensitive ultrasound sign to look for in the diagnosis of PHT.
 ...
PMID:Duplex Doppler ultrasound signs of portal hypertension: relative diagnostic value of examination of paraumbilical vein, portal vein and spleen. 152 Jan 64


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>