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)

Portal hypertension occurs in a number of disorders other than cirrhosis and they are collectively called non-cirrhotic portal hypertension (NCPH). The common causes of NCPH include idiopathic portal hypertension (IPH), non-cirrhotic portal fibrosis (NCPF) and extrahepatic portal venous thrombosis (EHPVT). Other causes include schistosomiasis, hepatic venous outflow tract obstruction, veno-occlusive disease and congenital hepatic fibrosis. Patients with IPH and EHPVT present with upper gastrointestinal bleeding, splenomegaly, ascites after gastrointestinal bleeding, features of hypersplenism, growth retardation and jaundice due to portal biliopathy. The diagnosis is usually made by abdominal ultrasound, upper gastrointestinal endoscopy, normal liver function tests and normal liver histology. Variceal bleeding in NCPH has lower mortality as compared with cirrhosis because of better liver functions in NCPH. Treatment for NCPH includes primary prophylaxis for variceal bleeding and prevention of repeat bleeding using drugs like beta-blockers, endoscopic sclerotherapy and endoscopic band ligation of varices. In patients with uncontrolled variceal bleeding or symptomatic hypersplenism, porto-systemic shunt surgery or splenectomy are required.
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PMID:Non-cirrhotic portal hypertension versus idiopathic portal hypertension. 1247 38

Portal cavernoma is a network of veins whose caliber, initially millimetric or microscopic, is increased and which contain hepatopedal portal blood. It results from occlusion, thrombotic and always chronic, of the extra-hepatic portal system. Diagnosis is mainly done by imaging. Clinical signs of portal cavernoma are usually related to extra-hepatic portal hypertension (hematemesis due to rupture of oeso-gastric varices, splenomegaly, rectal bleeding from ano-rectal varices, growth retardation in children) and sometimes to the cause of portal hypertension (abdominal pain, venous bowel infarction). Occurrence of portal thrombosis is often the conjunction of a local cause and a prothrombotic disorder which must be systematically detected. Biliary consequences of cavernoma are related to compression of common bile duct and are usually asymptomatic. In case of jaundice or cholangitis, portal decompression by portosystemic shunt can be performed to treat both biliary symptoms and portal hypertension.
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PMID:[Portal cavenorma: diagnosis, aetiologies and consequences]. 1253 94

Acute variceal hemorrhage is the most lethal complication of cirrhosis. The reported mortality rate from a first episode of variceal hemorrhage is 17% to 57%. Management of varices can be categorized into three phases: 1) prevention of initial bleeding, 2) management of acute bleeding, and 3) prevention of rebleeding. Modalities for treatment include pharmacologic, endoscopic, and shunt therapy. For the prevention of first variceal hemorrhage, cirrhotic patients should undergo endoscopy to identify patients with large varices. Priority for screening for varices should be given to patients with low platelet count, splenomegaly, and advanced cirrhosis. Once large varices are identified, patients should be started on beta-blocker therapy, which reduces the risk of bleeding by 50%. If pharmacologic therapy is not tolerated or contraindicated, endoscopic band ligation should be performed, and surveillance of varices should be performed every 6 months thereafter. Shunt procedures are not indicated due to their higher rates of complications compared with medical therapy. For the management of acute variceal hemorrhage, patients should be started on prophylactic intravenous antibiotics and intravenous octreotide. Endoscopy should be performed to diagnose and treat variceal hemorrhage. Band ligation appears to be as effective as sclerotherapy, but with less complications. If hemostasis is not achieved, balloon tamponade can be used as a bridge to definitive therapy, which in this case would be a transjugular intrahepatic portosystemic shunt (TIPS). If TIPS is unavailable, a surgical shunt is indicated. Once an episode of acute bleeding has been controlled, variceal eradication is best accomplished with repeat band ligation every 10 to 14 days until varices are obliterated. Prevention of recurrent bleeding can be achieved with beta-blocker therapy. The addition of isosorbide mononitrate further reduces recurrent bleeding. This combination pharmacologic therapy has been shown to be superior to sclerotherapy and may be superior to band ligation. However, side effects of combination pharmacologic therapy may limit its effectiveness. Band ligation is preferred to sclerotherapy when considering endoscopic therapy due to less complications and lower cost. Surgical shunts should be used for prevention of rebleeding in patients who do not tolerate or are noncompliant with medical therapy and who have relatively preserved liver function. TIPS should be reserved for patients who have poor liver function and who have failed medical therapy.
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PMID:Current Management of Esophageal Varices. 1458 39

Increase in portal venous pressure after anatomical or functional obstruction of portal venous system represents the most important complication of liver cirrhosis. Important sequels of portal hypertension are not dependable of etiology of liver disease. They are: increased collateral circulation in portal system and low pressure venous system (esophageal and gastric varices, portal hypertensive gastropathy and colopathy, hemorrhoids, collateral circulation through anterior abdominal wall, increased lymphatic flow, ascites, splenomegaly with occasional hypersplenismus, hepatic encephalopathy, hepatorenal syndrome.
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PMID:[Complications of liver cirrhosis]. 1513 48

Left-sided portal hypertension due to splenic vein stenosis is a very rare disease. We report a case of this condition in a 21-year-old woman who suffered from a first episode of tarry stool passage with fresh blood vomiting. Panendoscopy showed isolated gastric varices while sonography showed a normal liver but the presence of splenomegaly with prominent collateral circulations. Further imaging studies, including abdominal computed tomography, splenoportography and percutaneous transhepatic portography, revealed a focal stenotic proximal splenic vein resulting in left-sided portal hypertension. The collateral circulation ran from the short gastric veins via the left gastric veins into the main portal vein. The intraportal venous pressure was within normal limits. Splenectomy was performed and near normal wedge liver biopsy pathology confirmed non-cirrhotic extrahepatic portal hypertension. The patient had no further variceal bleeding after surgery.
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PMID:Isolated gastric varices due to focal splenic vein stenosis. 1534 Jun 66

Live donors are becoming an increasingly important source of donor organs in liver transplantation; however, long-term functional aspects of recovery from donor right hepatectomy are unknown. We analyzed donor outcomes at 1-year follow-up. We performed a single-center retrospective analysis of 70 right hepatectomy donors. Six-week and 1-year postoperative follow-up results were compared to preoperative baseline values. Ultrasonography was performed in all donors at 6 weeks and as clinically indicated. All donors were alive and well at the end of the study period. Of 66 right hepatic donors, only 22 (32%) were fully compliant with a 1-year follow-up visit. All those not compliant were contacted by phone. All complications except 1 (late finding of portal vein thrombosis) occurred in the perioperative (90-day) period. The incidence of bile leak was 4.3%, incisional hernia 20%, and autologous transfusion 1.0%. There were no aborted procedures. In those compliant with full 1-year follow-up, total bilirubin, aspartate aminotransferase, and alanine aminotransferase were normal in 97%. A total of 5 donors were noted to have persistence of asymptomatic thrombocytopenia beyond the perioperative period (90 days). These were investigated with Doppler sonography. Sonography was unremarkable in 3 of the 5, while 2 had abnormal findings: splenomegaly alone in 1, and splenomegaly with portal vein thrombosis in the other. Magnetic resonance angiography was performed in both, and the patient with portal vein thrombosis underwent endoscopy, which failed to reveal varices. Neither has clinical portal hypertension. Both remain asymptomatic albeit with stable thrombocytopenia. In conclusion, the majority of complications after donor right hepatectomy occur in the perioperative period. Later findings may include asymptomatic thrombocytopenia, with an incidence possibly as high as 23%, though the significance of this finding remains uncertain. Larger-scale studies are needed to confirm the true incidence and clinical significance of persistent thrombocytopenia in the donor hepatectomy population. Strategies to improve compliance with 1-year follow-up visits need to be developed.
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PMID:One-year morbidity after donor right hepatectomy. 1549 45

Fibropolycystic liver disease encompasses a spectrum of related lesions of the liver and biliary tract that are caused by abnormal embryologic development of the ductal plates. These lesions (congenital hepatic fibrosis, biliary hamartomas, autosomal dominant polycystic disease, Caroli disease, choledochal cysts) can be clinically silent or can cause signs and symptoms such as cholangitis, portal hypertension, gastrointestinal bleeding, infections, and space-occupying masses. The different types of fibropolycystic liver disease demonstrate characteristic findings at computed tomography (CT) and magnetic resonance (MR) imaging. Patients with congenital hepatic fibrosis typically have imaging evidence of liver morphologic abnormalities, varices, splenomegaly, renal lesions, and other associated ductal plate abnormalities. Biliary hamartomas usually manifest as multiple cysts that are nearly uniform in size and measure up to 15 mm in diameter. Autosomal dominant polycystic disease typically manifests as an enlarged and diffusely cystic liver. In Caroli disease, cystic or fusiform dilatation of the intrahepatic ducts is seen, as well as the "central dot sign," which corresponds to a portal vein branch protruding into the lumen of a dilated bile duct. Choledochal cyst manifests as a fusiform or cystic dilatation of the extrahepatic bile duct. Awareness of these CT and MR imaging features is essential in detecting and differentiating between various fibropolycystic liver diseases and can assist in proper management.
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PMID:Fibropolycystic liver disease: CT and MR imaging findings. 1588 16

Our aim was to analyze patients diagnosed with left-sided portal hypertension prospectively and to document the complications at follow-up. Twenty-four patients with isolated splenic vein thrombosis (diagnosed by ultrasonography or angiography or intraoperatively) and/or isolated fundal varices (diagnosed by endoscopy or endosonography) were involved in this study. Demographics, clinical presentation, diagnostic and therapeutic procedures, and morbidity and mortality were recorded in their follow-up. There were 11 and 13 left-sided portal hypertension cases associated with pancreatic diseases and nonpancreatic disorders, respectively. Chronic abdominal pain and gastrointestinal bleeding were the two most common complaints. All patients except one had isolated esophageal (2 cases) or fundal (21 cases) varices. Thirteen patients had splenomegaly on ultrasonography. On Doppler sonography, the splenic vein could be evaluated in 21 of the 24 patients (9 and 6 had complete and partial occlusion, respectively, and 6 had patent blood flow). Urgent intervention with therapeutic endoscopy and splenectomy was performed for two patients each. Medical therapy was begun for three patients according to the underlying diseases. Three patients underwent elective surgery. Two patients were lost to follow-up after the first visit and the mean follow-up of the remaining 22 patients after diagnosis of left-sided portal hypertension was 20 months. Only one patient (with pancreas cancer) had gastrointestinal bleeding at follow-up. All patients with pancreas and gastric cancer died within 2-12 months. Left-sided portal hypertension has various etiologies. It may be difficult to diagnose this entity both endoscopically and radiologically. Treatment should be directed at the underlying diseases. Recurrent hemorrhage due to left-sided portal hypertension is not usual and the prognosis depends mainly on the underlying etiology.
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PMID:Report of 24 left-sided portal hypertension cases: a single-center prospective cohort study. 1590 78

Hemorrhage from gastric varices due to left-sided portal hypertension is an unusual presentation for pancreatic endocrine tumor. A case of pancreatic endocrine tumor presenting with gastric variceal hemorrhage secondary to left-sided portal hypertension associated with splenic vein occlusion is presented. A 53-year-old man with hemorrhage from isolated gastric varices was referred to our hospital. Laboratory studies revealed normal liver function. Surveys to identify the cause of gastric varices by an abdominal CT, MRCP, and abdominal angiography revealed splenic vein occlusion secondarily attributed to the pancreatic tail tumor and splenomegaly. The pancreatic tumor was suspected to be a resectable endocrine tumor. A distal pancreatectomy, splenectomy, partial resection of the gastric fundus, and limited lymph node dissection were performed. By the histological examination, the diagnosis of nonfunctioning pancreatic endocrine tumor with malignant potential was determined. Three years after the surgery, the patient is doing well and reveals no sign of recurrence. In this case, the unusual presentation for pancreatic endocrine tumors such as a gastric variceal hemorrhage had an advantage that led to early presentation prior to the development of metastases with possible curative surgery.
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PMID:Nonfunctioning pancreatic endocrine tumor presenting with hemorrhage from isolated gastric varices. 1644 73

A 53-year-old woman who had been diagnosed with rheumatoid arthritis was found to have thrombocytopenia, splenomegaly, and gastric varices. She was diagnosed as having idiopathic portal hypertension on the basis of liver biopsy and angiography. Treatment with prednisolone was not sufficiently effective for thrombocytopenia. After transabdominal devascularization with splenectomy, thrombocytopenia subsided and gastric varices disappeared. In this case, the autoimmune mechanism as well as hypersplenism was suspected of being involved in the mechanism of thrombocytopenia.
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PMID:A case of idiopathic portal hypertension associated with rheumatoid arthritis. 1663 28


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