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)

Twelve patients diagnosed as having hepatic hemangiomas during 1960 to 1982 at the Royal Children's Hospital, Melbourne are reviewed. This rare benign vascular tumor can present considerable problems in both diagnosis and management and has a high reported mortality. Our experience over this 23-year period demonstrates some of the difficulties in diagnosis and suggests a plan of management. Eight patients were diagnosed when five weeks of age or less, the youngest was 24 hours old. One was diagnosed at four months of age. In the remaining three patients, the diagnosis was made at postmortem. Ten patients had prominent hepatomegaly, nine had congestive cardiac failure and in one of these the onset was delayed four weeks. Thrombocytopenia was present in five and jaundice in four patients. Four patients had associated cutaneous or visceral hemangiomas. Seven patients underwent selective hepatic arteriography, and two of these had prior ultrasound examinations of the liver. Management of congestive cardiac failure included steroids, radiotherapy, hepatic resection, and in one patient, hepatic artery ligation. One patient with diffuse hepatic hemangiomas did not require any specific therapy and resolved spontaneously over two years. The four patients in whom hepatic resection was performed survived.
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PMID:Diagnosis and management of massive hepatic hemangiomas in childhood. 351 Dec 16

A 65-year-old male patient underwent two bypass operations because of coronary artery disease. After the second operation he developed congestive heart failure with breathlessness, ankle swelling, hepatomegaly and poor exercise tolerance. Echocardiographic and haemodynamic findings were characteristic of constrictive pericarditis. Pericardiectomy was performed three months after the second operation. The retrosternal space was replaced with fibrotic, patchy hyalinic tissue, and the pericardium was thick and rigid. Histologically, the thickened pericardium showed dense fibrosis and foreign-body granulomas with large multinuclear giant cells and irregular crystals. This report indicates that foreign body reaction following coronary artery bypass operation may result in constrictive pericarditis with severe heart failure.
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PMID:Constrictive pericarditis following coronary bypass reoperation. Fibrotic pericardium and a foreign body reaction. 353 48

The vascular origin lesions of the liver (capillary hemangioma/infantile hemangioendothelioma) that present in infancy or early childhood often have a typical clinical picture of hepatomegaly and congestive heart failure. These lesions rarely present as asymptomatic hepatomegaly, simulating a primary hepatic malignancy. These lesions may also simulate a primary or secondary hepatic malignancy on cross-sectional imaging or angiography. Scintigraphic evaluations with technetium-99m-labeled red blood cells offers an accurate method of identification of these lesions, and allows differentiation from other common primary or secondary hepatic masses in infancy or childhood. This scintigraphic method may also be used to follow these patients after medical, radiation, or embolization therapy. Experience with seven patients with these tumors is reported and compared with eight children with other primary or secondary liver tumors also evaluated by this method.
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PMID:Technetium-99m-labeled red blood cells in the evaluation of hemangiomas of the liver in infants and children. 362 94

The factors associated with survival in 40 neonates (age less than 28 days) with critical aortic stenosis undergoing either open (22 patients) or closed (18 patients) transventricular aortic valvotomy were reviewed. Significant adverse correlates with survival included evidence of poor perfusion preoperatively (low pH, greater than Grade 2/6 soft ejection systolic murmur) and marked congestive heart failure (hepatomegaly, cardiomegaly, elevated left atrial pressure). Congenital mitral stenosis (anulus less than 11 mm), a small aortic anulus (less than 6.5 mm), and failure to achieve an adequate aortic orifice (greater than 6 mm), at operation were identified as factors associated with increased mortality. Initial perioperative survival was better with closed aortic valvotomy. However, there was no significant difference in overall operative survival between closed (9/18, 50%) and open (8/22, 36%) aortic valvotomy (p = 0.26). The incidence of early reoperation (less than 1 year of age) was greater in perioperative survivors undergoing closed valvotomy (7/13, 54%) rather than open valvotomy (1/10, 10%) (p less than 0.05). In conclusion, long-term survival among patients with critical neonatal aortic valve stenosis remains disturbingly low (13/40, 32%) and has not significantly improved over the past 20 years.
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PMID:Critical aortic stenosis. Survival and management. 365 53

One hundred sixty-eight patients with primary systemic amyloidosis (AL) were identified. Median survival after diagnosis was 12 months and ranged from 4 months for patients presenting with congestive heart failure to 50 months for those presenting with peripheral neuropathy only. Utilizing the proportional-hazards model in a stepwise multivariate fashion to evaluate the simultaneous influence of putative risk factors as of diagnosis revealed that congestive heart failure, urine light chain, hepatomegaly, and multiple myeloma were the major factors adversely affecting survival during the first year after diagnosis. Serum creatinine, multiple myeloma, orthostatic hypotension, and monoclonal serum protein were the most important variables adversely affecting survival for patients surviving 1 year. These models were used to categorize patients according to the variables in the models into low-, moderate-, and high-risk groups for the first year after diagnosis and separately for subsequent years. The influence of these variables on survival is important in stratification of patients randomized to prospective clinical trials.
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PMID:Primary systemic amyloidosis: multivariate analysis for prognostic factors in 168 cases. 371 98

Doxorubicin (DXR; Adriamycin) was administered i.v. to 20 Sprague-Dawley rats at 2.5 mg/kg once a week for 8 weeks. The rats were then observed for 2 to 5 weeks post treatment until they developed signs of general and cardiotoxicity (lassitude, ascites, marked ECG changes), at which time 11 of these animals and 11 age-matched controls were killed for evaluation of isometric contractile activity changes in isolated right ventricular papillary muscles. At necropsy the animals were examined for evidence of congestive heart failure (enlarged liver, s.c. edema). Baseline contractile changes in preparations from DXR-treated rats, compared with controls, were a decrease in maximum rate of tension development and prolongation in time to peak tension, time to half-relaxation from peak tension and duration of tension. Both developed tension and tension duration in the DXR-exposed muscles were reduced less than those in controls by a decrease in the stimulus interval, and some DXR preparations exhibited a positive staircase rather than the negative staircase normally observed in rats. Increasing extracellular Ca++ produced substantially greater changes in peak tension and maximum rate of tension development, as well as time to half-relaxation and tension duration in the DXR muscles; however, the effects of isoproterenol on these four parameters were blunted in the DXR muscles (although decreased isoproterenol sensitivity was borderline in the case of tension duration and time to half-relaxation). The results suggest that long-term DXR treatment leads to a loss of adrenergic support in the rat heart, and that calcium deficiency, rather than overload, occurs in cells that are still functionally active.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Doxorubicin cardiotoxicity: contractile changes after long-term treatment in the rat. 394 92

Three cases of congenital cerebral arteriovenous malformation are presented. They all developed congestive heart failure in the first day of life. The main clinical findings were a continuous murmur heard over the scalp, an ejection murmur at the upper left sternal border, gallop rhythm, cardiomegaly and hepatomegaly. Diagnosis was confirmed in all by carotideal arterography. Two had aneurysm of the vena cerebri interna Galeni and in the other, the aneurysm was localized in the area irrigated by the sylvian artery. The two former died after surgery was considered not feasible. The latter had his malformation resected. Two years after operation he is doing well.
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PMID:[Cerebral arteriovenous malformation (author's transl)]. 621 Nov 15

A new essential hypereosinophilic syndrome has been reported in a 53 years old man who presented an important blood and bone marrow hypereosinophilia of unknown etiology since 5 years. The evolution of the clinical course included neurological accidents (hemiplegia, spasmodic quadriplegia, seizures) congestive heart failure, hepatomegaly, transient renal involvement, pulmonary infiltrates with pleural hemorrhage, episodes of diarrhea and fever with weight loss. The cytological study of the eosinophiles showed the existence of abnormal inclusions which were confirmed by the electron microscope. These and cytoenzymological abnormalities led to discussion of the relationship between the essential hypereosinophilic syndrome and eosinophilic leukemia.
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PMID:[An essential hypereosinophilic syndrome. Cytological, cyto-enzymological and ultrastructural studies (author's transl)]. 645 47

An infant died at 8 months of age with a history of developmental regression, hypotonia, severe weakness, cardiomegaly, congestive heart failure, and hepatomegaly. A diagnosis of Pompe's disease (glycogenosis type II) was established by muscle biopsy at 5 months of age. Vacuolar myopathy involved muscle fibers of histochemical type I more than type II. Many vacuoles were filled with glycogen. In addition, increased amounts of neutral lipid were demonstrated by oil red O stain, electron microscopy, and quantitative analysis. Acid alpha-1,4-glucosidase activity was demonstrated to be deficient. Biochemical studies failed to determine the cause of the lipid accumulation, but demonstrated a low total concentration of carnitine in the muscle (6.37 nmole/mg of protein), associated with elevated activities of carnitine palmityl-transferase and palmityl-coenzyme A dehydrogenase. Palmityl-coenzyme A synthetase activity was in the normal range.
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PMID:Lipid storage myopathy in infantile Pompe's disease. 646 16

The distinctive sonographic appearance of benign vascular tumors of the liver (one hemangioendothelioma and two mesenchymal hamartomas) in three infants is presented. Each child had hepatomegaly and congestive heart failure, and all had confirmatory arteriography. The sonographic pattern of a complex mass, dilated proximal aorta, and enlarged nutritive and draining vascular structures should alert the radiologist and clinician to this diagnosis.
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PMID:Benign vascular tumors of the liver in infants: sonographic appearance. 697 23


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