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)

Budd-Chiari syndrome is a well described entity which can easily be confused with congestive hepatomegaly. The syndrome usually is caused by thrombosis of the hepatic veins, however it also can be caused by congenital fibrous webs that occlude hepatic vessels in the inferior vena cava. It leads to an enlarged liver with ascites, peripheral edema, and portal hypertension.
Clin Cardiol 1993 Apr
PMID:Balloon dilatation of inferior vena cava obstruction in Budd-Chiari syndrome. 837 Jan 88

Coronary arteries are frequently involved in systemic arteritis. The inflammatory infiltrate damages the intima and may trigger the occurrence of coronary thrombosis. We report an extreme example of how intimal inflammation in multiple sites of a coronary tree with and without atherosclerosis may trigger coronary thrombosis, in an elderly female patient who died of a clinically unrecognized systemic autoimmune-inflammatory disorder with necrotizing arteritis. The clinical picture was dominated by abdominal symptoms (peritonitis and possible chronic hepatic disease), renal failure and pulmonary X-ray opacities. A precise clinical diagnosis was not formulated, and the patient died of cardiac arrest 15 days after admission. Autopsy showed findings typical of Wegener's granulomatosis and of systemic arteritis with fibrinoid necrosis and multiorgan infarctions. Wegener's granulomatosis-polyarteritis nodosa overlap syndrome was pathologically diagnosed. Although there were no clinical signs of heart involvement, the coronary tree showed inflammation associated with multiple mural and occlusive thrombi. The atypical severe clinical presentation, the short course of the disease and the age of the patient probably contributed to the non proper clinical diagnosis. Old age does not preclude the occurrence of autoimmune disorders, whose course may be dramatically fatal. The abrupt occurrence of a systemic disease with renal failure, hepatomegaly, lung opacities and serositis should prompt analysis to consider these disorders. If properly diagnosed, cardiac involvement should be suspected in autoimmune disorders, even when clinically silent or masked by the systemic clinical picture. In our patient, the role that heart involvement played in the outcome, if any, remains unknown, even though the postmortem pathological identification of coronary mural and occlusive thrombi is generally sufficient to attribute the final cause of death to coronary thrombosis itself.
G Ital Cardiol 1998 Apr
PMID:Coronary artery inflammation and thrombosis in Wegener's granulomatosis-polyarteritis nodosa overlap syndrome. 961 52

Hyperlipidemia is recognized as one of the major risk factors for the development of coronary artery disease and progression of atherosclerotic lesions. Dietary therapy together with hypolipidemic drugs are central to the management of hyperlipidemia, which aims to prevent atherosclerotic plaque progression, induce regression, and so decrease the risk of acute coronary events in patients with pre-existing coronary or peripheral vascular disease. In patients at high risk of coronary artery disease but without evidence of atherosclerosis, treatment is designed to prevent the premature development of coronary artery disease, whereas in those with hypertriglyceridemia, treatment aims to prevent the development of hepatomegaly, splenomegaly, and pancreatitis. The 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase inhibitors, or statins, are the most potent lipid-lowering agents currently available, and their use in the treatment of hyperlipidemia provides the focus for this review. Particular emphasis is given to cerivastatin, a new HMG-CoA reductase inhibitor that combines potent cholesterol-lowering properties with significant triglyceride-reducing effects. Recently completed primary and secondary intervention trials have shown that the significant reductions in low-density lipoprotein (LDL) cholesterol achieved with statins result in significant reductions in morbidity and mortality associated with coronary artery disease as well as reductions in the incidence of stroke and total mortality. Such benefits occur early in the course of statin therapy and have led to suggestions that these drugs may possess antiatherogenic effects over and above their capacity to lower atherogenic lipids and lipoproteins. Experimental studies have also shown statin-induced improvements in endothelial function, decreased platelet thrombus formation, improvements in fibrinolytic activity, and reductions in the frequency of transient myocardial ischemia.
Am J Cardiol 1998 Aug 27
PMID:Current and future treatment of hyperlipidemia: the role of statins. 973 40

The first case of Q fever endocarditis that has been diagnosed in Mexico is presented. A 10-year-old girl with discrete subaortic stenosis (SAS) and patent ductus arteriosus (PDA) was seen in December of 1996 with fever, hepatomegaly and splenomegaly. She presented also anemia, leukopenia, hypergammaglobulinemia, positive rheumatoid factor, cryoglobulinemia, antinuclear and anticytoplasmic antibodies (anti-RNA-proteins and anti-DNA). An aortic valve vegetation was seen by echocardiogram. Blood-cultures were negative. Antibody test for Coxiella burnetii was positive. Treatment with doxicyclin was initiated as soon the diagnosis was done. PDA was closed, SAS was liberated and two aortic vegetations were resected. Endocarditis in Q fever occurs when there is predisposing heart disease and/or immunodeficiency. Effective therapy has not yet been established. The diagnosis of Q fever endocarditis is difficult; it should be considered, in case of clinical suspicion of endocarditis with negative blood-cultures.
Arch Inst Cardiol Mex
PMID:[Coxiella burnetii endocarditis. A report of the first case diagnosed in Mexico]. 981 Mar 69

We studied the incidence of AF in patients with endomyocardial fibrosis (EMF) and its influence on prognosis and associated clinical events. One hundred and sixty consecutive patients with EMF were followed for a mean period of 4 years. Their mean age was 39.7 years. There were 114 women. During follow-up there were 56 deaths. Eighty-eight patients (55%) were submitted to surgical intervention. AF was observed in 58 cases (36.2%). The presence of AF was associated with a greater prevalence of dyspnea, peripheral edema, hepatomegaly, lower left ventricular ejection fraction, lower right ventricular systolic pressure (37.8 vs 45.6 mmHg, P=0.0392), and greater incidence of tricuspid regurgitation (86.0 vs 63.2%, P=0.004). AF was more frequent among patients in whom the disease involved the right ventricle, particularly those with intense fibrosis. Overall, patients with AF had a higher mortality rate than those who did not have AF (43.1 vs 30.3%, P=0.0195), but among those submitted to surgery, AF did not have an impact on survival. In conclusion, AF is frequent among patients with EMF. It is more prevalent among patients with right ventricular involvement and its presence is associated with a greater incidence of heart failure. AF is associated with worse prognosis, but surgery potentially reverses this bad evolution.
Int J Cardiol 1998 Nov 30
PMID:Atrial fibrillation in endomyocardial fibrosis is a marker of worse prognosis. 988 Jan 97

A 5-month-old boy with a VACTERL syndrome underwent cardiac surgery for correction of a common arterial trunk and closure of an atrial septal defect. A prominent Eustachian valve was mistaken for the atrial septum and surgically closed. Thirty months later, after gradual shrinking of the foramen ovale with associated reduction of the right-to-left shunt, the boy presented with acute symptoms of a lower inflow obstruction, characterized by hepatomegaly and engorged abdominal vein pattern (Medusa's head). The boy was reoperated successfully after the condition had been recognized.
Pediatr Cardiol
PMID:Acute inferior cardiac inflow obstruction resulting from inadvertent surgical closure of a prominent Eustachian valve mistaken for an atrial septal defect. 998 97

Cardiac cachexia has recently been identified as an independent risk factor for mortality in chronic congestive heart failure. The aims of our study were to further identify the clinical or biochemical predictors or correlates of the cachexia, and to quantitate the magnitude of wasting. We undertook an anthropometric comparison of 30 patients with congestive heart failure, aged 56 (13) years, with ten age- and sex-matched healthy volunteers and 16 patients with essential hypertension. In comparison to the healthy volunteers, the heart failure patients exhibited a trend towards a lower body mass index, 21 (2.7) versus 23 (3.8) kg/m2, the 95% confidence interval for the difference being -0.54 to 5.4. However, the mid-upper arm circumference, of 24 (3.8) cm in the heart failure patients, was significantly (P<0.02) lower than the 27 (2.0) cm in the healthy volunteer group, with a 95% confidence interval for the difference being 1.18 to 4.82 cm. The triceps, mid-thigh, scapula and abdominal skinfold thicknesses were separately and significantly (P<0.05) diminished in the heart failure patients compared to the healthy controls. The sum of the four skin fold thicknesses, with a value of 68 (13) mm in the healthy volunteers, was highly significantly greater (P<0.001) than the value of 35.6 (9) mm in the heart failure patients. The 95% confidence interval for this difference was 22.7 to 41.3 mm. The patients with essential hypertension differed significantly from the heart failure patients in all of these parameters (P<0.01), but were not statistically different from the healthy controls in the anthropometric parameters. Among the heart failure patients, those with tricuspid regurgitation (n = 12) had a worse clinical, biochemical and cachexia profile compared to patients without the tricuspid regurgitation (n = 18). The values (tricuspid regurgitation versus no regurgitation) were New York Heart Association Class, 3.5 (0.65) versus 2.7 (0.75), P<0.01; ejection fraction of 34 (9) versus 43 (13)%, not significant; greater hepatomegaly of 159 (31) versus 135 (29) mm, P<0.05; more severe hypoalbuminemia, 24.5 (2.7) versus 28.5 (6.8) g/l, P<0.05; and worse hyponatremia, 128 (4) versus 133 (5) mmol/l, P<0.05. The tricuspid regurgitation group had a significantly more severe reduction in abdominal and scapula skin fold thickness (P<0.01) than that found in patients without tricuspid regurgitation. The sum of the four skin fold thicknesses was significantly lower (P<0.05) in tricuspid regurgitation, 30.9 (8) mm, than in heart failure without associated regurgitation, 38.0 (9.6). The 95% confidence interval for the difference was 0.8 to 13.4 mm. It is concluded that significant diminution of muscle bulk and subcutaneous fat occurs in chronic heart failure. Tricuspid regurgitation may be an accentuating and accelerating risk factor for cardiac cachexia, on account of a greater hypoalbuminemia and hyponatremia, which, presumably, results from the associated protein-losing enteropathy.
Int J Cardiol 1999 Sep 30
PMID:Anthropometric evaluation of cachexia in chronic congestive heart failure: the role of tricuspid regurgitation. 1057 94

Streptokinase is a thrombolytic agent used very frequently for the early treatment of acute myocardial infarction. A 35-year-old male with inferior acute myocardial infarction was admitted to the Coronary Care Unit and treated with systemic streptokinase. At the time of admission, he was a healthy male and he was not receiving any hepatotoxic agent. Six hours after thrombolysis, he developed high fever, painful hepatomegaly, jaundice and coluric urine. Leucocytosis with left deviation was observed in the hemogram and the liver function tests showed slight enzymatic elevation and hyperbilirubinemia. This condition was progressively improving and the patient was free of symptoms on the eighth day of evolution. Jaundice as secondary effect of streptokinase has been previously communicated in very few medical reports.
Rev Esp Cardiol 1999 Nov
PMID:[Streptokinase-induced jaundice]. 1061 14

This study was to evaluate the clinical effects of percutaneous balloon angioplasty of Budd-Chiari syndrome (BCS) caused by inferior vena cava (IVC) obstruction. Between 1993 and 1999, 28 men and 14 women with mean age of 44+/-12 years underwent percutaneous balloon angioplasty for primary BCS. Color Doppler ultrasound and venography showed membranous and segmental obstruction of IVC in 29 and 13 patients, respectively. Fourteen patients also had left- and/or mid-hepatic vein obstruction. Angioplasty of IVC was successful in 41 patients (97.6%), resulting in a reduction of pressure gradient between IVC and the right atrium from 15.0+/-2.5 to 5.5+/-0.8 mmHg (P<0.01). A stent was placed in the site of obstruction in the patient with unsuccessful balloon angioplasty. Patients with successful angioplasty or stent placement had significant improvement in clinical symptoms indicated by a reduction in hepatomegaly and the degree of ascites. No specific attempt was made to treat the occluded left- and/or mid-hepatic vein due to the presence of potent right hepatic vein. Over the follow-up period of 32+/-12 months, restenosis of IVC occurred in only one patient (2.4%), which was redilated successfully. Percutaneous balloon angioplasty is a safe and effective therapy for Budd-Chiari syndrome caused by IVC obstruction, therefore should be the first choice of treatment for this condition.
Int J Cardiol 2002 May
PMID:Percutaneous balloon angioplasty of inferior vena cava in Budd-Chiari syndrome-R1. 1200 92

This is the case of a patient who presented with severe right-sided heart failure due to diastolic dysfunction that caused a dilemma of differential diagnosis between restrictive cardiomyopathy and constrictive pericarditis. Restrictive cardiomyopathy was diagnosed based on noninvasive and invasive hemodynamic testing. However, the patient did not respond to therapy and succumbed to worsening heart failure and multiple comorbidities. Clinical features of right heart failure with edema, ascites, jugular venous distention, and tender hepatomegaly are commonly seen in clinical practice. When systolic function is determined to be normal, diastolic causes of heart failure must be ruled out. These include myocardial disorders with a broad range of pathologies leading to restrictive physiology, of which amyloidosis is a prototype. Pericardial disorders leading to diastolic heart failure are usually in the form of constrictive physiology, when pericardial tamponade is ruled out. Differentiation between restrictive and constrictive pathologies is often difficult and requires careful attention to hemodynamic and Doppler echocardiographic features. We report a case of severe right heart failure illustrating some of the complexities in decision-making and the importance of meticulous hemodynamic and ancillary testing in the diagnosis and treatment of this often fatal condition.
Cardiol Rev
PMID:Constrictive pericarditis versus restrictive cardiomyopathy: challenges in diagnosis and management. 1547 69


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