Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 10-year-old boy with idiopathic scoliosis presented with exertional dyspnea, lower leg edema since two weeks prior to admission. Physical examination revealed neck venous engorgement, moist rales, mild hepatomegaly, ascites and lower leg edema in addition to the scoliosis. Chest x-ray films showed cardiomegaly, pulmonary congestion and pneumonic infiltration of both lower lobes with bilateral pleural effusion. Electrocardiograms showed right axis deviation and right atrial hypertrophy. Two-dimensional echocardiography revealed dilatation of the right heart chambers and pulmonary artery. He was treated initially with mechanical ventilation, antibiotics, bronchodilators, diuretics, vasodilators, and digoxin with improvement. Unfortunately the patient became comatose after an episode of cardiopulmonary arrest. He did not recover from this tragedy and was discharged against medical advice in comatose state. Scoliosis may be complicated with pneumonia and cor pulmonale in children. Early surgical correction of the scoliosis may prevent the development of cor pulmonale. Combined medical and surgical managements should be carried out aiming at scoliotic heart disease.
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PMID:[Idiopathic scoliosis and pneumonia complicated with cor pulmonale. Report of one case]. 227 31

Four patients with unstable diabetes mellitus and pronounced elevations of serum aminotransferases and alkaline phosphatases are reported. Thorough investigations revealed no cause for the abnormalities. The enzyme elevations were associated with hepatomegaly, and in some instances, abdominal pain and leg edema. Liver biopsies in all patients showed only marked accumulation of glycogen in the hepatocytes.
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PMID:Elevated aminotransferases and alkaline phosphatases in unstable diabetes mellitus without ketoacidosis or hypoglycemia. 250 26

In a 7-year period, transatrial membranotomy was performed in 11 patients with membranous obstruction of the inferior vena cava. There were 5 men and 6 women, ranging in age from 23 to 53 years. Clinical symptoms included jaundice in 4 patients, hepatomegaly in 4, leg edema or varicose veins in 10, and venous collaterals over the abdominal and chest wall in all 11 patients. Transatrial membranotomy was performed through a median sternotomy in all patients. When inferior vena cava venography revealed that the obstruction was accompanied by long segmental thrombosis, additional dilation was performed with a Hegar dilator. There was no surgical mortality. Early operative complications included pulmonary embolism in 2 patients and bleeding requiring reoperation in 1. In a mean follow-up period of 30.6 months (range, 2 to 88 months), 9 patients had no symptoms, transient pericardial constriction developed in 1 patient and resolved 1 month later, and restenosis of the inferior vena cava developed in another patient 1 year after the first operation. This latter patient received a second transatrial membranotomy followed by percutaneous balloon angioplasty of the inferior vena cava, with a satisfactory result at 8 months follow-up. We conclude that transatrial membranotomy is an effective and safe procedure for patients with membranous obstruction of the inferior vena cava.
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PMID:Transatrial membranotomy for Budd-Chiari syndrome. 240 Feb 84

Three patients with the Budd-Chiari syndrome are presented. This is a rare condition characterized by hepatomegaly, progressive and refractory ascites, distension of the abdominal wall veins, abdominal pain and leg oedema. These features are attributed to congestion of the liver and portal hypertension. The condition has been notoriously difficult to treat medically. Surgical measures are directed towards relieving the liver congestion and lowering pressure in the portal system by portal-systemic shunting operations. In some cases refractory ascites may be treated by peritoneovenous shunting with a Le Veen shunt. In a select group of patients orthotopic liver transplantation has proved to be worth while.
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PMID:Budd-Chiari syndrome. A report of 3 cases. 396 23

Thirteen patients with symptomatic polycystic liver disease who were selected for fenestration were reviewed. The main preoperative complaints that related to polycystic liver disease were severe abdominal pain, respiratory distress, clinical ascites, or leg edema. All symptoms disappeared after operation, and the number and size of the cysts were smaller. Five patients developed transient but massive ascites during the postoperative period, and long-term follow-up demonstrated a moderate recurrence of hepatomegaly in two patients. These postoperative complications and failures appeared to be at least partly related to a more extensive evolution of the disease in the patients in whom it developed.
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PMID:Fenestration in the management of polycystic liver disease. 774 Aug 6

The Japanese Ministry of Health and Welfare Research Committee on Aberrant Portal Blood Flow carried out an epidemiological survey and clinical study on Budd-Chiari syndrome in 1990. In the primary survey for determining the prevalence of the disease, a questionnaire was sent to all major hospitals throughout Japan and 160 cases seen in 1989 were compiled. More epidemiological details were obtained in 87 of these 160 cases. The number of patients with Budd-Chiari syndrome in this country was estimated to be about 300 (prevalence of 2.4/million) with about 20 new cases occurring every year. In the clinical study, 157 authentic cases of Budd-Chiari syndrome studied in 15 years (1975-89) were analyzed. There were 87 males (average age, 36.4 years) and 70 females (46.5 years), and the average period from the likely onset to the first medical consultation was 6.6 years, suggesting that these patients were mostly chronic cases. The main clinical features were hepatomegaly, leg edema, ascites and venous dilatation over the trunk. Abdominal pain was recorded in only four (2.5%). There were 16 (10.2%) with known identifiable etiologies. Of the patients 93% showed an obstructing lesion of various thickness in the hepatic portion of the inferior vena cava. Only nine (5.7%) had hepatic vein obstruction without caval lesions. Thus, the majority of Budd-Chiari syndrome patients in Japan are idiopathic, having an obstructing lesion in the inferior vena cava. The main causes of 33 deaths (21%) were liver failure, variceal bleeding and hepatocellular carcinoma. Hepatocellular carcinoma occurred in 10 (6.4%) in the 15-year period. However, the incidence of Budd-Chiari syndrome among all cases of hepatocellular carcinoma was less than 1% in the survey made by the Liver Cancer Study Group of Japan.
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PMID:Epidemiological and clinical features of Budd-Chiari syndrome in Japan. 775 74

The present review has the objective to identify clinical epidemiologic characteristics and evolution for four children with Fasciola hepatica evaluated at the Pediatric Gastroenterology Unit at the Children's Health Institute, Lima, Peru, between 1988 and 1992. The first patient was a 9-year old girl who had fever, leg edema, collateral circulation and hepatomegaly, predominantly at the right lobe. The second was a 6-year old by who had upper gastrointestinal hemorrhage and fever; endoscopic diagnosis demonstrated hemobilia. The third case was a 12-year old boy who presented generalized edema. The fourth patient was a 9-year old boy with jaundice, fever and upper gastrointestinal hemorrhage. Esophageal varices and cirrhosis were present. In the four patients the diagnosis was made by the identification of parasite eggs in the feces. The first three cases responded satisfactorily to treatment with bithionol; the fourth patient died because of systemic failure.
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PMID:[Hepatic fascioliasis in children: uncommon clinical manifestations]. 962 20

This report describes a case of cardiac tamponade as the initial manifestation of unclassified connective tissue disease (UCTD). A 68-year-old Japanese woman was admitted to hospital because of dyspnea and edema. She had undergone a radical left mastectomy for the treatment of breast cancer 18 years before. On admission, bilateral leg edema, hepatomegaly, and a paradoxical pulse were noted on physical examination. The erythrocyte sedimentation rate was elevated and the C-reactive protein was 2.8 mg/dl. Antinuclear antibodies and anti-SS-A/Ro antibodies were present. The scl-70 and anticentromere antibodies were elevated. Chest radiography showed cardiomegaly. Echocardiography revealed a large pericardial effusion, but the pericardial fluid did not contain malignant cells or bacteria. She did not meet the diagnostic criteria for any known connective tissue diseases, so was diagnosed with cardiac tamponade due to UCTD. Prednisolone (30 mg/day) was administered, which resulted in a gradual resolution of the pericardial effusion. Although connective tissue diseases are known to cause pericardial effusion, cardiac tamponade as the initial manifestation of the disease in the absence of other symptoms is quite rare.
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PMID:Unclassified connective tissue disease presenting as cardiac tamponade: a case report. 1095 61

Budd-Chiari syndrome (BCS) is a disorder caused by occlusion of the hepatic vein or inferior vena cava. The clinical presentation include abdominal pain, hepatomegaly, ascites, leg edema, collateral venous dilatation of the body trunk, and portal hypertension. In addition, BCS can cause hepatocellular carcinoma (HCC) in some patients, although its pathogenesis is not yet completely understood. The average reported time lag from diagnosis of BCS to full-blown HCC ranges from several years to several decades. Hepatic carcinogenesis in patients with BCS perhaps reflects a prolonged and persistent liver injury in that it occurs in the primary inferior vena cava obstruction rather than the primary hepatic vein thrombosis. Among patients with BCS, membranous obstruction of the vena cava (MOVC) usually presents an insidious and chronic illness, whereas primary hepatic vein thrombosis presents an acute or subacute illness. We experienced a case of a patient with BCS, which progressed rapidly that HCC developed only nine months after the diagnosis of BCS. The factors causing this rapid progression are still unclear and remain to be investigated.
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PMID:Rapidly progressing Budd-Chiari syndrome complicated by hepatocellular carcinoma. 1461 90

A 48-year-old woman was hospitalized because of severe thrombocytopenia, leg edema, and fever. Intravenous immunoglobulin therapy was administered, but no efficacy was obtained. Her bone marrow was dry-tap, and fibrosis was found in the biopsy specimens. A positron emission tomographic study showed FDG-avid lymphadenopathy and hepatomegaly. Biopsy specimens of axillary lymph nodes showed Castleman's disease-like findings. Since she then developed severe proteinuria and massive pleural effusion, steroid therapy was started, providing temporary relief of symptoms other than the thrombocytopenia. However, rapid worsening of her general condition prompted us to attempt rituximab as salvage therapy. The pleural effusion, edema, and proteinuria disappeared soon after starting rituximab administration. Platelet counts also normalized and fibrosis of the bone marrow showed amelioration. Recently, a variant of multicentric Castleman's disease, termed the TAFRO syndrome, has been proposed, and our patient's features fit the diagnosis of this syndrome. Rituximab might be considered as a therapeutic option in such cases.
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PMID:[Efficacy of rituximab for TAFRO syndrome, a variant type of multicentric Castleman's disease]. 2468 40


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