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)

Extra-hepatic obstruction of the portal vein is a well known cause of hypertension in childhood and 55 out of the 97 patients (57 per cent) seen with this condition presented before they were 15 years old. However, nearly half our cases (43 per cent) presented in adult life. Haematemesis was the commonest mode of presentation in both the adult and childhood group although splenomegaly was common, especially in the children. The severity and frequency of haematemesis increased during puberty in the children. Twelve children and three adults have now not bled for between five and 30 years, 10 of them ceasing spontaneously and five following surgery. Intra-abdominal infection or septicaemia precipitated the portal venous obstruction in 38 patients (39 per cent), though the exact cause of such obstruction in patients who had no cirrhosis was obscure in about half the cases. Ascites was present in 18/51 (35 per cent) of the children and 24/35 (69 per cent) of the adults. Its presence was associated with an increased mortality (p less than 0.01). Nineteen out of 42 (45 per cent) patients with ascites also had portal-systemic encephalopathy. Twenty-four patients died, nine having presented during childhood, variceal haemorrhage was responsible for death in 19 and infection in five. Sixty-four patients underwent 114 operations for variceal haemorrhage. Mortality was greater in the surgical group compared with those managed conservatively. Surgery is therefore indicated only in the rare case where bleeding cannot be controlled by medical means.
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PMID:The aetiology, presentation and natural history of extra-hepatic portal venous obstruction. 53 23

A 39-year-old male with bleeding esophageal varices due to portal hypertension was observed. The patient had taken an arsenical preparation during a period of 12 yr because of psoriasis and subsequently developed keratotic changes of the palms and soles of his feet and an epithelioma of the scrotum. Physical examination was unremarkable except for splenomegaly and skin lesions. Liver function tests were normal; a needle biopsy of the liver (right lobe) showed nonspecific changes. Combined hepatic and umbilicoportal catheterization revealed, on splenography and portography, huge esophageal varices and patent portal vein; dilation, distortion, and cut-off of many intrahepatic portal branches were found. A marked gradient existed between the free portal venous pressure (25 mm Hg) and the wedged hepatic venous pressure (9.5 mm Hg). Hepatic blood flow, portal PO2, cardiac output, cardiac index, and blOOD volume were within normal range. Arteriographies did not reveal arteriovenous shunts in the splanchnic or splenic vessels. A splenorenal shunt were performed and a wedged biopsy of the liver (left lobe) revealed nonspecific changes. Three years later the patient had not experienced any episode of hemorrhage or hepatic encephalopathy but developed an epithelioma of the tongue. No known cause could be incriminated in the pathogenesis of the portal hypertension. However, there was unequivocal chronic arsenic intoxication. Toxic hepatitis, cirrhosis, noncirrhotic portal hypertension, and hemangiosarcoma of the liver have been reported with the intake of arsenicals. Thus, it is suggested that in this patient, presinusoidal portal hypertension was secondary to chronic arsenical intake associated with marked intrahepatic vascular changes seen on portography.
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PMID:Noncirrhotic presinusoidal portal hypertension associated with chronic arsenical intoxication. 112 3

Plain films of the abdomen in advanced states of schistosomiasis reveal massive splenomegaly without evidence of splenic calcification. The liver may be enlarged in the early stages but ultimately decreases and becomes small. Ascites is evident in far advanced disease. Chest films are negative in the early stages, but in advanced disease, dilated pulmonary arteries, right ventricular enlargement, and dilatation of the azygous vein may be recognized. Granulomata are seen as multiple small rounded densities scattered throughout both lung fields. The routine barium swallow will reveal unsuspected esophageal varices. Nine patients were studied preoperatively by panhepatic angiography and 14 post-operatively following splenorenal shunt. Thrombosis of the shunt and hepatic encephalopathy were common postoperative complications.
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PMID:Radiographic evaluation of patients with schistosomiasis. 120 69

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)
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PMID:Chronic liver disease in Ethiopia: a clinical study with emphasis on identifying common causes. 131

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.
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PMID:Portal hypertension in schistosomiasis: pathophysiology and treatment. 134 92

DIC in patients affected by cirrhosis, accompanied by portal hypertension and splenomegaly, has been suspected in the past. The main aim of this study is to ascertain the incidence of this phenomenon. We carried out coagulation and fibrinolytic tests in 113 cirrhotic patients and 20 healthy control persons. We found chronic consumption coagulopathy at analysis level in 28 cases (24.8%) with a decrease of fibrinogen, factor V, kallikrein, platelets, prothrombin complex activity, increase of PDF, partial thromboplastic time and euglobulin lysis. 25 cases had active cirrhosis, with ascites, variceal bleeding and/or hepatic encephalopathy; 3 were non-active cirrhosis. Only 7 patients had clinical DIC. We observed that coagulation disorders increased with more active cirrhosis.
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PMID:[The incidence of consumption coagulopathy in liver cirrhosis]. 256 20

Five patients with porto-systemic shunts, four spontaneous and one surgically created, presenting severe recurrent hepatic encephalopathy rebel to clinical treatment were angiographically examined. A spontaneous splenic-renal veins shunt was observed in four cases and a mesocaval shunt was identified in the remaining patient. Two thirds embolization of the spleen was able to control hepatic encephalopathy in two patients. Percutaneous transhepatic portography and selective embolization of the shunt was performed in two other patients with good results. Percutaneous transcaval embolization of the mesocaval shunt succeeded to control hepatic encephalopathy in the remaining patient. Splenic embolization is able to control hepatic encephalopathy in patients with splenomegaly and spontaneous porto-systemic shunt. Direct embolization of the shunt is also able to control hepatic encephalopathy as should be expected.
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PMID:Embolization to reverse severe recurrent hepatic encephalopathy. 320 7

A personal case series triggers an examination of surgical risk in patients with uncomplicated cirrhosis of the liver. After a general introduction the conditions that increase surgical risk in cirrhotic patients are analysed. These include generally poor resistance, altered haemostasis, a tendency towards cholestasis, water retention and hepatic encephalopathy. The conditions most often requiring surgical treatment are then considered. They include associated pathologies (cholelithiasis, hernias, tooth extractions, bleeding haemorrhoids etc) and complications of cirrhosis variceal bleeding, intractable ascites, splenomegaly, hepatocytoma).
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PMID:[Internal medicine evaluation of the surgical risk in cirrhosis patients]. 382 13

Two hundred and thirty six patients with amoebic liver abscess were investigated for cholestasis, its mechanism and the natural course of the disease. Cholestasis was seen in 29% of cases and it presented with some unusual features: it was frequently seen in young men (mean age 38.6 +/- 6.3 years) (87%) with acute onset (69%) and was associated with signs of peritonism, or peritonitis (28%), splenomegaly (12%) and hepatic encephalopathy (coma 13%). Raised diaphragm was seen only in 37% of cases. Alcoholism may have contributed to the cholestasis in 37% of cases. Multiple (43%) and single (32%) large liver abscesses, especially on the inferior surface of the liver (25%), were common in jaundiced patients with amoebic liver abscess, while size and number of abscesses were directly related to the raised serum bilirubin concentrations. Bromsulphalein excretion (BSP) was found to be significantly reduced (p less than 0.01) in patients with jaundice (60%). Retrograde injection of contrast media into the common bile duct during six necropsies showed compression by amoebic liver abscess on the hepatic ducts. The mortality (43%) and the complications were significantly higher (p less than 0.001) in patients with jaundice. The aspiration/surgical drainage of amoebic liver abscess together with a combination of metronidazole and di-iodohydroxyquinoline was more effective than either metronidazole alone, or dehydroemetine with chloroquine.
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PMID:Cholestasis in amoebic liver abscess. 396 31

In the absence of cirrhosis, most children with portal hypertension and bleeding esophageal varices have extrahepatic portal vein occlusion. In the past 2 yr this clinical picture has been mimicked by two children with hepatoportal sclerosis causing their variceal hemorrhage. Hepatoportal sclerosis has been well described in adults. It is manifested by splenomegaly, portal hypertension, and variable ascites and hepatomegaly. Liver histology is initially normal but subsequently shows periportal fibrosis without cirrhosis. Hepatic manometrics indicate a presinusoidal block, but angiography demonstrates a patient portal vein. Typically there is abrupt narrowing of the intrahepatic portal branches, giving a "withered tree" appearance. These findings are illustrated by two children who presented with esophageal variceal bleeding at 21 and 20 mo of age, respectively. They are the youngest reported cases of hepatoportal sclerosis. The etiology of hepatoportal sclerosis is uncertain, but the disease appears to be only slowly progressive. Control of variceal bleeding by central portosystemic shunts in this condition is associated with a 50% incidence of hepatic encephalopathy. Therefore alternate methods of therapy need be considered. Endoscopic injection sclerotherapy successfully controlled variceal bleeding in one child reported here. Hepatoportal sclerosis is a distinct entity and must be considered in the differential diagnosis of portal hypertension in infants and children.
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PMID:Hepatoportal sclerosis in childhood: a mimic of extrahepatic portal vein obstruction. 725 29


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