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)

Endocardial fibroelastosis is an uncommon congenital heart disease in dogs that may be manifested by signs of left-sided congestive heart failure. A three-month-old, male, Fila Brasileiro dog developed signs of generalised heart failure. Physical examination revealed normal temperature, ascites, and pale and cyanotic mucous membranes. The pup died just after radiography which revealed ascites, hepatomegaly, severe cardiac enlargement and pulmonary oedema. At necropsy, serosanguineous fluid in the thorax and abdomen, pulmonary oedema, right ventricular dilatation, hypertrophy and dilatation of the left ventricle, and mitral valve incompetence were observed. The histopathological examination demonstrated that the thickening of the endocardium of the left atrium and left ventricle was due to the presence of elastic and collagen fibres, although there were no signs of an inflammatory process.
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PMID:Endocardial fibroelastosis in a dog. 912 86

We report the case of a young man first seen by is in 1989 at the age of 20 years. The diagnoses of hypertrophic cardiomyopathy, Wolf-parkinson-White syndrome, congestive heart failure and pulmonary hypertension were made. One month later the patient had jaundice and hepatomegaly and a diagnosis of acute viral hepatitis A was established by laboratory findings. The ALT and AST levels were persistently elevated, seven times the normal mean, during six years. Two liver biopsies in 1991 and 1993 showed liver injury secondary to congestive heart failure (CHF) as the only abnormality. This case illustrates the importance of liver injury secondary to CHF as a cause of a marked and persistent increase of ALT and AST that resembles that of other liver diseases.
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PMID:[A case of elevated transaminases caused by congestive heart failure simulating chronic hepatitis]. 938 Sep 78

A 31-year-old woman was admitted because of persistent remittent fever. Tricuspid valve endocarditis due to Staphylococcus aureus was identified as the cause of fever. The patient had no history of intravenous drug abuse, oral contraceptives or predisposing cardiac disease. Huge hepatomegaly was found without any signs of congestive heart failure. Liver enzyme abnormalities were not detected throughout the entire course of therapy. The liver biopsy specimen revealed peliosis hepatis. Treatment with panipenem/betamipron was successful, although recurrent septic pulmonary embolism occurred. The cause of the huge hepatomegaly encountered in the present case may be attributable to peliosis due to severe infection.
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PMID:Tricuspid valve endocarditis in a non-drug addict associated with peliosis hepatis. 939 42

We retrospectively investigated the feasibility and the toxicity of autologous stem cell transplantation (ASCT) in 21 cases of systemic amyloidosis (AL). The conditioning regimens consisted of high-dose melphalan (HDM) alone (n = 18) or in combination with 12 Gy total body irradiation (n = 3). Toxic death rate was high: 9/21 patients (43%) died within the first month following ASCT, and 10/12 surviving patients achieved a response. With a median follow-up of 14 months, the OS and the EFS rates at 4 years were 57.1% (+/-10.8) and 29.9% (+/-14.5) respectively for the whole group. The major prognostic factor for both response and survival was the number of clinical manifestations at the time of ASCT, of the following five criteria, i.e. creatinine clearance < 30 ml/min, nephrotic syndrome with urinary protein excretion > 3000 mg/24 h, congestive heart failure, neuropathy, or hepatomegaly associated with alkaline phosphatase level > 200 IU/l. For patients presenting with two or more clinical manifestations the 4-year OS and EFS were both 11.1% compared with 91.7% and 46.3% respectively in patients with fewer than two clinical manifestations at the time of ACST. We conclude that ASCT is feasible in AL in a subset of patients with fewer than two clinical manifestations at the time of ASCT. Given the severe extra-haematological toxicity, ASCT should not be considered in other cases.
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PMID:Prognostic factors for survival and response after high-dose therapy and autologous stem cell transplantation in systemic AL amyloidosis: a report on 21 patients. 967 53

Imaging investigations and other findings observed in a term infant with a multicentric hepatic hemangioendothelioma, admitted to the Intensive Care Unit at the age of 13 days because of non specified feeding difficulties and dyspnoea, are presented. Physical examination revealed cardiac bruit and congestive heart failure with marked hepatomegaly; in addition there were multiple small skin hemangiomas. Echocardiography was negative, abdominal sonography showed multiple round lesions of mixed echogenicity in the liver, large vascular channels, a right hepatic artery and hepatic veins enlarged, a caliber of the aorta below the level of the superior mesenteric artery reduced. The infant was additionally investigated by whole-body scintigraphy with 99mTc-labeled red blood cells to determine the possibility of coexistence of other visceral hemangiomas and by MR, in which the tumor manifested as multiple well-circumscribed space-occupying nodules of high signal intensity on T2-weighted images with evidence of fast flow. The baby underwent furosemide and steroid therapy: serial two-dimensional US scans showed change in echogenicity, responding to therapy. Doppler sonography has proven to be also very useful in the monitoring therapy determining changes in flow pattern and velocity at the level of hepatic, cerebral and renal vessels: before therapy we observed a reduction of the diastolic flow until the zero line through the internal carotid artery and renal artery with an increase of the Resistance Index. It means that this important component can be compromised in the presence of a hepatic hemangioendothelioma.
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PMID:[A case of congenital hepatic hemangioendothelioma treated with prednisone: the echographic changes and Doppler study]. 967 4

Twenty-six persons from five families comprising 34 members residing in different areas of Saptari district of the eastern region of Nepal developed symptoms of epidemic dropsy over 6-8 weeks. Seventeen patients were studied during July-August 1996. The age of affected individuals varied from 3 to 75 years. Members who had not consumed food cooked in mustard oil or who were not residing with the family were spared. Mustard oil, which was used for cooking, was found to be contaminated with oil of Argemone mexicana seeds. Sanguinarine was detected in all mustard oil samples collected from the homes of affected families. Gastrointestinal symptoms were present in 82 per cent of cases a week or so prior to the onset of pedal oedema. Pitting oedema of the lower limbs, fever, and darkening of the skin were the most consistent features, found in all cases. Other prominent features such as local erythema (82 per cent) and tenderness (88 per cent) of the lower limbs were present in most cases. Two striking features not previously noted were perianal itching (100 per cent) and severe carditis (35 per cent) with congestive cardiac failure (29 per cent). Other unique features noted were 'sarcoid' skin changes (18 per cent), bilateral pleural effusion, and Roth's spots and subhyloid haemorrhages in the fundus in one patient. Other important findings were anaemia (88 per cent), hepatomegaly (41 per cent), pneumonia (35 per cent) and ascites (12 per cent). There were no deaths due to epidemic dropsy. In the majority of cases, oedema, cutaneous changes, and carditis showed a marked improvement in 2-3 weeks and patients were well after 6-8 weeks of follow-up.
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PMID:Epidemic dropsy in the eastern region of Nepal. 1019 86

Amyloidosis should be considered in any patient older than 40 years who has nephrotic syndrome, congestive heart failure (not on an ischemic basis), idiopathic peripheral neuropathy, or unexplained hepatomegaly. When a patient has one of these problems, immunoelectrophoresis and immunofixation of the serum and urine should be done for the detection of a monoclonal light chain. If a monoclonal light chain is found, a diagnosis usually can be established by amyloid stains performed on a bone marrow biopsy specimen or a subcutaneous fat aspirate. The presence or absence of cardiac involvement with amyloid is the most important prognostic factor. Treatment can range from observation to oral chemotherapy to hematopoietic stem cell transplantation. A practical understanding of the mechanisms underlying this disease can lead to prompt diagnosis and early therapeutic intervention.
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PMID:Amyloidosis: recognition, confirmation, prognosis, and therapy. 1031 82

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.
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PMID:Anthropometric evaluation of cachexia in chronic congestive heart failure: the role of tricuspid regurgitation. 1057 94

Primary (AL, amyloid light-chain) amyloidosis is a plasma cell disorder in which deposits of amyloid light-chain protein cause progressive organ failure. It is important to recognise that amyloidosis is a dynamic process and chemotherapy-induced reduction of the activity of the plasma cell clone reduces the supply of the amyloid precursor protein and can result in a major regression of the deposits. The most common target organ is the kidney and renal amyloidosis manifests as proteinuria or nephrotic syndrome. Proteinuria is seen in three quarters of patients. Amyloid related nephrotic syndrome and renal failure are potentially reversible. Fatigue, congestive heart failure, hepatomegaly, peripheral neuropathy, orthostatic hypotension, carpal tunnel syndrome and macroglossia are other common features. The median survival is one to two years. Conventional-dose melphalan as standard treatment can prolong the median duration of survival by about ten months, but the clinical response rates with improvement of impaired organ function are low. Up-front high-dose chemotherapy with autologous peripheral blood stem cell transplantation is much more effective and can result in a major improvement in the clinical condition of patients. However, the toxicity related to this treatment can be relevant due to impaired organ function. Conventional-dose chemotherapy consisting of vincristine, doxorubicin and dexamethasone or high-dose dexamethasone or interferon-alpha are other possible approaches to treatment. The improvement of patient condition with an effective conventional-dose chemotherapy may increase the tolerability of high-dose chemotherapy and reduce transplantation related problems.
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PMID:Novel approaches to the treatment of primary amyloidosis. 1106 Aug 11

Congestive heart failure was diagnosed in a 27-year-old Indian ringneck parakeet with exercise-induced dyspnea. A grade IV/VI holosystolic murmur that radiated to the right sternal area was auscultated over the left side of the sternum. Radiography revealed progressive cardiomegaly, hepatomegaly, pulmonary edema, and accumulation of fluid within the coelomic cavity. Echocardiography revealed biatrial enlargement and enlargement of the right ventricle. Doppler recording revealed high velocity left and right atrioventricular valve regurgitation. Treatment with digoxin and furosemide alleviated clinical signs for approximately 10 months. Gross postmortem examination revealed cardiac enlargement and eccentric hypertrophy of both ventricles on cross-section. Pulmonary congestion and edema, hepatomegaly, hepatic congestion, and ascites were also evident. Histologic examination of the heart revealed myxomatous degeneration of the left atrioventricular valve, muscular hypertrophy of the right atrioventricular valve, and biventricular chronic myofiber degeneration and necrosis.
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PMID:Congestive heart failure associated with myxomatous degeneration of the left atrioventricular valve in a parakeet. 1120 63


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