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)

Thirty-four patients with primary generalized amyloidosis (PGA) and 14 with multiple-myeloma-related amyloidosis (MRA) were studied. The commonest clinical manifestations in PGA were nephrotic syndrome, hepatomegaly and congestive heart failure, and in MRA, low back pain, plasmacytoma and rheumatoid-arthritis-like syndrome. Eight patients with PGA had limited clinical expression of the disease, such as involvement of only kidneys, joints, parotid glands or gastrointestinal tract; in one patient amyloidosis was limited to lymph nodes. Low serum concentrations of total protein and albumin were common. M components were detected in the serum of 91% of patients with PGA and 92% of patients with MRA: 70% of the M components in PGA and 25% of those in MRA had lambda light chains. Bence Jones proteinemia was detected in 56% of the patients with PGA and in 77% of those with MRA. The serum concentration of immunoglobulins was decreased substantially in more than two thirds of the patients with PGA. Proteinuria (greater than 250 mg/24 h) was observed in 78% of patients with PGA and in 93% of patients with MRA. Bence Jones proteinuria was noted in 75 and 77% of patients, respectively. Plasmacytic infiltration of the bone marrow was found in 90% of the patients with PGA. The mean survival time of the patients with PGA was 28 months and of those with MRA, 29 months from the time of diagnosis.
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PMID:Clinical and laboratory findings in primary generalized and multiple-myeloma-related amyloidosis. 126 76

Nonbacterial thrombotic endocarditis is an uncommon, but well-described, complication of bone marrow transplantation. We describe a case of nonbacterial thrombotic endocarditis following autologous bone marrow transplantation that was marked by weight gain, hepatomegaly, ascites, and extreme hyperbilirubinemia leading to a clinical diagnosis of hepatic veno-occlusive disease. Autopsy revealed nonbacterial thrombotic endocarditis of the tricuspid and pulmonic valves, and passive congestion of the liver, but there was no evidence of veno-occlusive disease. We discuss the pathophysiology and clinical features of nonbacterial thrombotic endocarditis and review its occurrence in association with bone marrow transplantation. Nonbacterial thrombotic endocarditis is often difficult to detect clinically and should be a diagnostic consideration in patients who develop systemic emboli or congestive heart failure after bone marrow transplantation.
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PMID:Nonbacterial thrombotic endocarditis clinically mimicking veno-occlusive disease of the liver complicating autologous bone marrow transplantation. 144 13

Liver dysfunction in hyperthyroid patients has not been well characterized. We analyzed the clinical records of 43 patients with hyperthyroidism to define the spectrum of clinical and liver test abnormalities. The patients were divided into three categories: (a) 18 patients with uncomplicated hyperthyroidism (HT) (b) 19 with hyperthyroidism and congestive heart failure (HT/CHF), and (c) 6 with hyperthyroidism and concomitant unrelated liver disease (HT/ULD). Hepatomegaly and/or spenomegaly were noted in 15 of 19 (79%) patients with HT/CHF as compared to 6 of 18 (33%) patients with HT and 3 of 6 (50%) patients with HT/ULD. Four patients with HT/CHF had ascites. Serum aminotransferase levels greater than 250 IU/L were noted in only 1 of 37 (3%) patients without unrelated liver disease. Patients with HT/ULD or HT/CHF had markedly low prothrombin time. Serum bilirubin levels as high as 323 microM were noted in patients with HT. No characteristic liver histology due to hyperthyroidism was noted. Severe liver test abnormalities, including deep jaundice and prolonged prothrombin time, can occur in patients with hyperthyroidism alone or with HT/CHF. This makes the diagnosis of concomitant, unrelated liver disease difficult until the hyperthyroidism has been controlled.
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PMID:Hyperthyroidism and hepatic dysfunction. A case series analysis. 156

To determine which variables most accurately define congestive heart failure (CHF) in infants, 41 patients (median age 2.5 months) were graded by four pediatric cardiologists for the presence and severity of CHF based on the following variables: amount of formula consumed per feeding, feeding time, history of diaphoresis or tachypnea, growth parameters, respiratory and heart rates, respiratory pattern, perfusion, presence of edema, diastolic filling sounds, and hepatomegaly. There were 19 patients graded as having no CHF, nine as mild, seven moderate, and six severe CHF. The most sensitive and specific variables (p less than 0.0001) for the presence of CHF were a history of less than 3.5 oz/feed, respiratory rate greater than 50/min, an abnormal respiratory pattern, diastolic filling sounds, and hepatomegaly. Moderate to severe CHF was present when patients took less than 3 oz/feed or greater than 40 min/feed, had an abnormal respiratory pattern with a resting respiratory rate greater than 60/min, and had a diastolic filling sound and moderate hepatomegaly. Severe CHF was accompanied by a heart rate greater than 170/min, decreased perfusion, and severe hepatomegaly. Thus, the grading of the severity of CHF in infants should include an accurate description of these historical and clinical variables.
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PMID:Grading the severity of congestive heart failure in infants. 161 22

It has been generally accepted that congestive heart failure does not lead to fulminant hepatic failure, unless it is associated with cardiac shock or low cardiac output. Only three cases have been reported, in which liver congestion is followed by fulminant hepatic failure without a history of shock or low cardiac output. Here we present a case of a 48-year-old man with dilated cardiomyopathy and pulmonary infarction, who developed fulminant hepatic failure from congestion. When he was admitted for the control of diabetes mellitus, hepatomegaly of 3-finger breadth and marked cardiomegaly without pulmonary congestion was noted. Diabetes was controlled using insulin. But 3 weeks after admission, he sometimes complained of back dullness because of pulmonary infarction. His heart gradually increased in size, and Jugular venous dilatation and pretibial pitting edema also worsened. Jaundice was noted and serum GOT and GPT increased. A large liver of 6-finger breath below the right costal margin was able to be felt. But within one week, the size of the liver markedly decreased and the signs of hepatic failure such as jaundice, hepatic encephalopathy and numerous petechiae appeared. Blood pressure was maintained and no hypotension or cardiac shock was noted. The patient died of fulminant hepatic failure on the 20th days after onset of the hepatic failure. The autopsy revealed liver atrophy with severe central lobular necrosis, and thrombus in the right main pulmonary artery which caused severe pulmonary infarction. The mechanism of fulminant hepatic failure not accompanied with low cardiac output is discussed.
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PMID:[A case of fulminant hepatic failure secondary to congestive heart failure]. 187 44

Two children are reported with hemangioendothelioma of the hepatobiliary system. One patient, a 7-month-old girl, presented with classic symptomatology of hepatomegaly and congestive heart failure, but did not have any cutaneous lesions. She was treated with systemic steroids and supportive therapy and did very well. The other patient, a 22-month-old boy, is the first patient reported with exclusive involvement of the extrahepatic biliary tree. His symptoms included hepatomegaly and obstructive jaundice. He was evaluated with an endoscopic retrograde cholangiopancreatogram and a transhepatic cholangiogram. He did very well after resection of the lesion and Roux-en-Y drainage of the proximal biliary tree and remains asymptomatic 2 years after surgery.
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PMID:Hemangioendothelioma of the hepatobiliary system: the classic and the unusual. 218 12

Since 1957, 30 patients with constrictive pericarditis have been treated surgically in Kyushu University Hospital. The surgical approaches were the left anterolateral thoracotomy in 17 patients (group I); the median sternotomy without cardiopulmonary bypass (CPB) in 6 patients (group II); and the median sternotomy with CPB in 7 patients (group III). The hospital mortality was 3.3 percent. The mean postoperative follow-up period was 11.7 years (longest 30 years). The actuarial survival rate at 5 years postoperatively was 88% in total cases (100% in group I, 82% in group II and 52% in group III), 88% at 10 years, 75% at 15 years and 67% at 20 years. Several patients in group III, who underwent complete pericardiectomy using CPB showed severe congestive heart failure and arrhythmia postoperatively. The comparative study between an poor result group (patients who died within 10 years post-operatively) and a good result group (patients who survived more than 10 years postoperatively revealed that preoperative hepatomegaly, atrial fibrillation and the interval between the onset of symptoms and the pericardiectomy influenced the survival rate significantly. These results suggested that pericardiectomy using CPB was a safe method for removing the calcified pericardium in the patient with severe constrictive pericarditis. However, a careful long term follow-up was necessary for the patient with severe myocardial damage even though the complete pericardiectomy was performed.
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PMID:[Surgical treatment of constrictive pericarditis]. 221 78

In 61 patients with class IV (NYHA) of chronic congestive cardiac failure treated for 2 weeks with digoxin (0.290 +/- 0.108 mg/d) and furosemide (13.0 +/- 4.1 mg/d), for 2 weeks with digoxin, furosemide and isosorbide dinitrate (44.5 +/- 9.8 mg/d) or nifedipine (42.0 +/- 12.2 mg/d), for 4 weeks with digoxin, furosemide, isosorbide or nifedipine and captopril (angiotensin converting enzyme inhibitor) (75.1 +/- 24.4 mg/d), and for the last 2 weeks with digoxin, furosemide, isosorbide or nifedipine without captopril, after each stage the clinical state, exercise tolerance and haemodynamic parameters determined echocardiographically were assessed. Ten weeks of treatment by this method caused regression of pulmonary congestion in 80%, oedema in 63.3% and hepatomegaly in 33.3% of the patients. Moreover, 60.7% of the patients returned to class III, 13.1% to class II, and 26.2% remained in class IV (NYHA). In the group treated with digoxin, furosemide, nifedipine with captopril (n = 30) a significant rise was observed of the value of the ejection fraction and cardiac index in relation to the treatment with digoxin and furosemide and the treatment with digoxin, furosemide, nifedipine (p less than 0.05). No drug improved significantly the tolerance of submaximal exercise. During the treatment with captopril no clinical improvement was achieved in 4 cases, and worsening occurred in 3 cases of severe cardiac failure (7 of 61 patients, 11.5%). The obtained results showed that vasodilating drugs are safe in congestive cardiac failure and in many cases of severe failure captopril contributed to rapid clinical and haemodynamic improvement.
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PMID:[Results of treatment for severe congestive heart failure with digoxin, furosemide and vasodilating agents]. 227 87

To develop an improved animal model of congestive heart failure, 11 female farm pigs (wt, 42-46 kg) underwent rapid ventricular pacing at 230 beats/min for 7 days with a modified Medtronic unipolar pacemaker connected to an apical pacing lead. After 7 days the pacemaker was turned off, anesthesia induced, the chest opened, and cardiac hemodynamic and dimensional studies were performed. Results were subsequently compared with data from 12 control pigs that received no pacing. Two pigs died before measurements could be determined. Cardiac output in the paced animals (0.061 +/- 0.018 l.min-1.kg-1) was significantly less (P less than 0.05) than in control pigs (0.085 +/- 0.016 l.min-1.kg-1), when compared at the same resting heart rate. Left ventricular (LV) end-diastolic pressure (23.2 +/- 7.7 vs. 8.6 +/- 3.6 mmHg, P less than 0.01) and right ventricular (RV) end-diastolic pressure (9.0 +/- 3.1 vs. 4.4 +/- 1.7 mmHg, P less than 0.01) were significantly greater in the paced pigs. Significant increases in both septal-lateral LV end-diastolic dimension (60.3 +/- 3.9 vs. 52.1 +/- 7.2 mm, P less than 0.01) and RV end-diastolic dimension (47.2 +/- 5.7 vs. 40.8 +/- 4.7 mm, P less than 0.05) indicated biventricular dilation in the paced pigs. They also exhibited a significantly greater heart weight-to-total body weight ratio and clinical evidence of congestive heart failure, with hepatomegaly and ascites. These results demonstrate that 1 wk of rapid ventricular pacing at 230 beats/min produces a realistic model of congestive heart failure in the pig.
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PMID:Rapid ventricular pacing in pigs: an experimental model of congestive heart failure. 233 89

Two patients with similar symptoms referred for diagnosis and treatment of hepatic failure subsequently proved to have cardiomyopathy as the cause of their hepatic decompensation. Except for fatigue and edema, symptoms of congestive heart failure were absent and no history of dyspnea, orthopnea, or paroxysmal nocturnal dyspnea could be elicited. Hepatomegaly was present in both patients, but neck venous distension and hypotension were not apparent, and both patients were able to lie flat. The diagnosis of cardiomyopathy was made by echocardiogram showing global hypokinesis and low ejection fractions; right atrial pressures were markedly increased. Liver biopsies demonstrated centrilobular necrosis and congestion. Treatment for heart failure led to a prompt response in both patients with rapid return of all hepatic parameters toward normal. Paradoxically, our patients had striking evidence of hepatic failure and a notable absence of symptoms and signs of congestive heart failure. An awareness of this unique presentation may avoid prolonged evaluations in such critically ill patients.
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PMID:Cardiomyopathy unrecognized as a cause of hepatic failure. 236


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