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)

This report reviews 164 cases derived from 2 Australian and 3 U.S. centres. There were 128 direct operations and 63 shunts. There were 16 deaths (10% mortality) but 3 were due to unrelated causes. Direct procedures; In the long term these appear of little value. 37 of 48 children having splenectomy subsequently rebled. Almost all children having ligation of varices and direct operations on the stomach rebled subsequently. Acute bleeding can almost always be controlled by conservative measures and direct operations would appear to offer no benefit over non-operative management except in the occasional case of catastrophic management that cannot be controlled conservatively. Shunts; A properly performed decompressive shunt offers the best hope of long term control for bleeding varices. Meso-caval shunts seem to give somewhat better results than splenorenal shunts. About two-thirds of the patients undergoing shunts remain free of any further bleeding. Non-operative management; 27 children have had no surgery performed and all are alive except for one child who subsequently died in a railroad accident. Bleeding episodes become less frequent after the age of 15 years and there are a number of reasons for this including the progressive development of natural shunts. We are not aware of any deaths or major complications from hypersplenism. Growth and development of all children in this series has been normal, although other have commented on a significant incidence of encephalopathy. Conclusions; 1. GIT bleeding becomes progressively less after the age of 15 years. 2. Direct operations have little place in treatment. 3. Decompressive shunts are the most effective method of controlling continuing bleeding but require a shunt of at least 1 cm diameter. 4. Splenomegaly and hypersplenism are not serious problems. 5. One can anticipate the progressive development of natural shunts. 6. Splenectomy should be avoided in this disease unless accompanied by a shunt.
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PMID:Extrahepatic portal hypertension--long-term results. 30 Dec 81

Acute and/or recurrent gastrointestinal bleeding due to ruptured gastric varices from an isolated thrombosed splenic vein is a distinct entity. Incidence of this syndrome is probably less than 1%. Typical clinical features of this syndrome include evidence of splenic hypertension without liver disease and no demonstrable cause of gastrointestinal hemorrhage. Diagnosis can easily be missed unless the surgeon is familiar with this syndrome. Typical findings at the time of surgery are an enlarged spleen, varicose veins usually involving the upper third of the stomach, and pancreatic and peripancreatic inflammation. Portal vein and portal pressure will be normal. Meso-portography is a convenient and safe procedure and will lend support to suspicion when a retrograde nonfilling of the splenic vein is present. Splenectomy offers the expectation of a long-range cure. A representative case of a 39-year-old man is discussed. He had at least six episodes of gastric bleeding in less than 3 years. At a previous laparotomy, the cause of bleeding could not be determined. A splenectomy in December 1970 has been able to control the gastric bleeding since then.
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PMID:Splenic vein thrombosis: an unusual case of gastric bleeding. 30 66

Three typical cases of segmental portal hypertension due to occlusion of the splenic vein are reported. This syndrome may be asymptomatic for a very long time and then present suddenly in the form of a serious picture of high digestive haemorrhage due to rupture of the varices of the fundus of the stomach as a result of hypertrophized submucous collateral drainage circulation. Useful for diagnosis are oesophagogastroduodenoscopy, which points to stomach varices, and splenoportography or superselective arteriography of the splenic artery with venous phase, which highlight pathognomonic dilatation and tortuosity of the gastroepiploic veins. Surgical exploration typically shows: presence of large epipolic vessels, splenomegaly, absence of changes in the liver and in the portal and mesenteric circulation. Resolutive treatment of choice is splenectomy.
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PMID:[Problems in emergency surgery: segmental portal hypertension]. 31 37

The frequency of splenic vein obstruction secondary to pancreatic or retroperitoneal diseases has been only recently appreciated. The diagnosis is important because it frequently results in development of gastric and duodenal varices. It is often assumed that the diagnosis should only be suspected in patients with splenomegaly. This report describes 19 patients in whom splenic vein thrombosis was diagnosed by angiography although clinically unsuspected. In 11, the spleen was normal in size although extensive gastric varices were present. In 3 patients the presenting problem was massive hematemesis. Review of the upper gastrointestinal examinations in these patients showed thickened gastric or duodenal folds although in the absence of esophageal varices, the diagnosis was not made prior to angiography. More liberal use of angiography in patients with an appropriate clinical background, such as a history of pancreatitis, may lead to earlier and more frequent diagnosis of splenic vein obstruction. Varices that result from splenic vein obstruction can be cured by splenectomy.
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PMID:Splenic vein thrombosis in patients with a normal size spleen. 57 63

Forty-three operative procedures were performed on a population of 250 patients with myeloproliferative disorders, including polycythemia vera, myeloid metaplasia (MM) and chronic myelogenous leukemia (CML). The overall operative mortality was approximately 7% and the incidence of excessive bleeding which could be related to coagulopathy was 5%. Twenty-one patients with MM or CML underwent splenectomy for palliation of symptoms related to the enlarged spleen or hematologic problems. Eighty-four percent of the latter group were improved. Adverse hematologic effects which could be attributed to splenectomy in these patients were confined to two patients who developed marked thrombocytosis. Among the 23 patients with MM, 9 had portal hypertension. Three underwent portacaval shunt and one a splenorenal shunt for bleeding varices. One of the patients died of hepatic necrosis. Estimated hepatic blood flow determinations (EHBF) in 4 patients with portal hypertension demonstrated a marked absolute increase and an increase in the ratio of EHBF/Cardiac Index. Absence of any evidence of intrahepatic or extrahepatic obstruction in these patients and the demonstration that splenectomy relieved portal hypertension defined at surgery in 4 patients, suggests that augmented adhepatic flow contributes to portal hypertension in some cases. The review leads to the conclusions that: 1) Operative procedures in prepared patients with myeloproliferative disorders are not associated with prohibitive mortality and morbidity rates. 2) Splenectomy is indicated for patients with increasing transfusion requirements and symptomatic splenomegaly or hypersplenism and should be performed early in the course of disease. 3) When associated portal hypertension and bleeding varices are present, hemodynamic studies should be carried out to define if splenectomy alone, or a portal systemic decompressive procedure is indicated.
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PMID:Myeloproliferative disorders. 105 48

A case of upper gastrointestinal tract hemorrhage secondary to esophageal varices in a patient with Felty's syndrome prompted a review of the pathogenesis and treatment of this condition. Six previously reported cases of this association were found. The clinical picture is that of long-standing rheumatoid arthritis with severe articular and extraarticular manifestations including splenomegaly, depression of the blood elements, mild liver function abnormalities, portal hypertension without cirrhosis or portal vein obstruction, an elevated splenic blood flow, and a reduction in portal hypertension by simple splenectomy. The presence of portal hypertension with varices may be another indication of splenectomy in patients with Felty's syndrome.
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PMID:Esophageal varices in Felty's syndrome: A case report and review of the literature. 108 37

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

A patient is described presenting with an acute lower gastrointestinal haemorrhage as a result of extensive colonic varices. Further investigation revealed that there were no oesophageal varices or splenomegaly. Liver biopsy showed grade II fatty change only, with no other specific or significant pathological features. Transhepatic portography showed a raised portal pressure (20 mm/Hg) but the portal system was patent throughout. There was an abnormal leash of vessels in the caecum thought to represent a variceal plexus. This patient was diagnosed as having idiopathic colonic varices. This case is discussed together with nine other reports of idiopathic colonic varices from the published literature. Four of these reports describe idiopathic colonic varices in more than one member of the same family. Possible modes of inheritance, aetiology of variceal change, natural history, and prognosis are discussed.
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PMID:Familial and idiopathic colonic varices: an unusual cause of lower gastrointestinal haemorrhage. 142 83

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.
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PMID:Duplex Doppler ultrasound signs of portal hypertension: relative diagnostic value of examination of paraumbilical vein, portal vein and spleen. 152 Jan 64

The case of a 31-year-old pregnant woman with an isolated splenic vein thrombosis, diagnosed with Doppler ultrasonography is presented. Routine ultrasound examination in the fourth month of the pregnancy revealed massive splenomegaly of unknown origin. Doppler ultrasonography subsequently revealed splenic venous thrombosis with absence of blood flow. Noteworthy gastric varices were present at the endoscopic examination. On splenectomy, a spleen weighing 2,600 was removed, and numerous venous collaterals were found in the perigastric region. The postoperative course was satisfactory, pregnancy evolved normally, and a healthy female baby was delivered at term. The patient remained in excellent health with normal clinical and laboratory data.
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PMID:Splenic vein thrombosis. Diagnosed with Doppler ultrasonography. 177 91


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