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)

Medical records of 12 dogs determined at necropsy as having had cardiomyopathy and of 5 live dogs with clinical, electrocardiographic and radiographic evidence of the disease were reviewed. Congestive cardiomyopathy was the most common form of the disease, affecting 15 of the 17 dogs. The dogs were primarily of large breeds and ranged in age from 2 to 8 years. Clinical findings included right and left congestive heart failure presenting as pulmonary congestion and edema, pleural effusion, hepatomegaly, and ascites. Thoracic radiographs showed moderate severe enlargement of all cardiac chambers and evidence of congestive heart failure. Atrial fibrillation was the predominant rhythmn; ventricular premature contractions and left ventricular hypertrophy were sometimes noted. At necropsy, biventricular dilation including dilation of the atrioventricular annular rings and accompanying massive atrial dilation was observed. Myocardial contractility was poor and had resulted in dilation of the heart chambers with minimal hypertrophic responses. The atrioventricular valve leaflets and chordae tendinae were usually near normal. Medical treatment included rest, digoxin, and diuretics, Medical or electrical cardioversion of atrial fibrillation to normal sinus rhythm was also attempted. Prognosis for congestive cardiomyopathy is very poor. The average survival time after onset of signs is 6-12 months; 1 dog in our study survived for 20 months. In contrast to congestive cardiomyopathy, the hypertrophic form is rare in the dog. Only two of the dogs studied had hypertrophic cardiomyopathy; one case was diagnosed at necropsy and one by angiocardiography. Both had features of idiopathic hypertrophic subaortic stenosis (IHSS) as reported in man.
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PMID:Cardiomyopathy in the dog. 12 94

Chest roentgenograms in 13 infants and angiocardiograms in 11 infants with persistent transitional circulation (PCT) syndrome were reviewed. Chest radiographs typically revealed congestive heart failure with pulmonary venous congestion, cardiomegaly, hepatomegaly, and pleural effusions. Angiocardiography demonstrated a right-to-left shunt through the ductus arteriosus, ventricular dilatation, poor myocardial function, and ventricular emptying. Cardiopulmonary abnormalities resolved over a period of 2-6 days in survivors. PTC syndrome as seen at the authors' institution has been an important and relatively common cause of congestive heart failure and cyanosis in the newborn. The incidence of congestive failure was higher than in other reported series.
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PMID:Persistent transitional circulation. Roentgenographic findings in thirteen infants. 13 73

A 3-month-old infant presented with multiple cutaneous haemangiomata, and was found to have a cardiac murmur and hepatomegaly. The latter increased strikingly over the next 3 months, and was accompanied by cardiomegaly. Other usual signs of congestive cardiac failure were not present, however, and an arteriogram showed the presence of a grossly abnormal vascular pattern in the liver, confirming the clinical suspecions of hepatic haemangiomatosis. Two short courses of steroid therapy resulted in a marked but temporary decrease in liver size. After the age of one year, however, there was gradual regression of all abnormal clinical signs without further therapy.
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PMID:Cutaneous and hepatic haemangiomata. 13 98

Of a total sixtytwo patients covering all the spectrum of genetic ASH, who were studied by heart catheterization, M-mode echocardiography and phonomechano cardiography, five patients (four with the obstructive variety of the disease) showed clinical evidence of chronic congestive heart failure with ankle edema and hepatomegaly (group I). Their data were compared with those of fifteen "obstructed" patients who were not in heart decompensation (group II). No statistically significant differences were found between groups I and II in terms of L.V. internal transverse dimensions and in terms of L.V. systolic function. Conversely a statistically significant difference was found between the two groups in terms of left atrial and right ventricular dimensions (P less than 0,001), which were markedly increased in groups I. These findings strongly suggest that in patients with ASH and congestive heart failure there is a reduction in L.V. compliance (or distensibility), whereas L.V. systolic function is essentially preserved. The persistence of severe L.V. outflow obstruction in four patients of I group gives further confirmation to these observations. The use of beta-blockers (in association or not with cardiac glycosides) seems therefore to preserve its validity in the treatment of patients with ASH and heart failure, particulary when severe L.V. outflow obstruction is present. Cardiac glycosides are indicated in the forms with little or no obstruction to L.V. ejection.
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PMID:[Congestive heart failure in genetic hypertrophic cardiomyopathies (ASH) (author's transl)]. 16 Mar 52

Five patients, 4 men and 1 woman, had adult-onset and slowly progressive weakness. There was distal wasting in 2, hepatomegaly in 3, and congestive heart failure in 2. Electromyography showed a mixed pattern with abundant fibrillations. Serum creatine phosphokinase was increased 5- to 45-fold. Blood glucose failed to respond to epinephrine or glucagon, and venous lactate did not rise after ischemic exercise. Muscle biopsy showed vacuolar myopathy affecting both fiber types. By electron microscopy the vacuoles corresponded to large pools of glycogen not limited by a membrane. Glycogen concentration was 3 to 5 times normal in muscle and 7 to 21 times normal in erythrocytes. In the presence of iodine, muscle glycogen showed a spectrum characteristic of phosphorylase-limit-dextrin. Debrancher activity was measured by a spectrophotometric assay and by a radioactive reverse reaction. The activity was lacking in muscle and erythrocytes of 4 patients according to both assays; in 1 patient the reverse reaction was not impaired. Though previously reported in only 5 patients, debrancher deficiency myopathy may not be rare and should be considered in the differential diagnosis of adult-onset hereditary myopathies.
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PMID:Debrancher deficiency: neuromuscular disorder in 5 adults. 28 18

A 3-week-old infant with hyperbilirubinemia and hepatomegaly secondary to hemangioendotheliomas of the liver was presented. Review of the literature produced 18 cases of associated jaundice in 182 infants with hepatic vascular tumors, an incidence of 10%. This disorder should be considered in the differential diagnosis of the infant with hepatomegaly and jaundice, particularly in the presence of gastrointestinal complaints, cutaneous hemangiomas, congestive heart failure, or abdominal bruit. Liver scan should be done in such infants to rule out vascular tumors. Although mortality appears to be high in jaundiced infants with such tumors, currently available therapeutic modalities may be of value in treating these infants.
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PMID:Hepatic hemangioendotheliomas and hyperbilirubinemia. 71 95

A 6-month-old girl was seen because of multiple cutaneous hemangiomas, hepatomegaly, and failure to thrive. A liver scan with technetium Tc 99m sulfur colloid showed two filling defects in the left lobe. Diffuse nodular hepatic lesions characteristic of hemangioendotheliomas were disclosed by celiac angiography and confirmed by biopsy. Congestive heart failure, present in many infants with this syndrome, was not manifest. Prednisone was administered, 2 mg/kg, on alternate days. Liver size and cutaneous hamangiomas promptly regressed in size and "catch up" linear growth occurred during six months of treatment. Congestive heart failure did not develop. Sixteen months after treatment, liver size and a second hepatic scan were normal.
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PMID:Hepatic hemangioendothelioma. Angiographic appearance and apparent prednisone responsiveness. 83 25

A calf into which a biolized, total artificial heart (TAH) had been implanted survived for 145 days. All measured physiological parameters except central venous pressure (CVP) were back to normal one month after implantation, and thereafter the animal's physiological development was similar to that of a normal calf. The intimal weight, which was 96 kilograms at implantation, reached 190 kilogram at the end of experiment, with a daily gain rate of 0.9 kilogram per day. After the nineteenth postoperative week, signs of congestive heart failure appeared, such as high venous pressure, ascites, and enlarged liver although the calf outwardly appeared well. On postoperative day 146, the animal started foaming at the mouth, and a convulsion occurred; then, the experiment was terminated after 3,494 hours of pumping. At autopsy, there were acute bilateral bronchopneumonia involving mostly both upper lobes, pulmonary edema, slight chronic pneumonitis, and hepatomegaly. There were no serious thrombotic deposits inside the cardiac prosthesis.
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PMID:Survival for 145 days with a total artificial heart. 83 53

For over 15 years, upper respiratory tract obstruction due to adenotonsillar hypertrophy has been known to cause hypoxia, hypercapnia, increased pulmonary vascular resistance and thereby cor pulmonale and congestive heart failure. This is now an uncommon but not rare entity and three recent cases prompted this report. The typical patient is dyspneic with retractions, cyanosis, occasional periods of apnea and somnolence. Edema and hepatomegaly and at times splenomegaly are common. X-rays show cardiomegaly, which on electrocardiogram is found to involved mainly the right ventricle. The strict definition of cor pulmonale is right ventricular hypertrophy secondary to lung disease or abnormal pulmonary function, a definition that may logically be stretched to include abnormal respiratory function secondary to upper airway pathology. The mechanisms by which this occurs are generally agreed upon. Hypoxia has been demonstrated to cause pulmonary vasoconstriction. Acidosis and hypercapnia are thought by some to have the same effect. Pressure across the pulmonary vascular bed is also increased, as predicted by Poiseuille's law, by the high rate of blood flow required to maintain tissue oxygenation with poorly oxygenated blood. Conditions producing hypoxia of hypercapnia or both lead to hypertrophy and eventually to dilatation of the right ventricle. Three cases of children who underwent cardiac catheterization while suffering from cor pulmonale due to adenotonsillar hypertrophy are reported. Right ventricular pressure averaged 44/5, PAO2 72, pH 7.32, and PACO2 52. All were clinically improved following adenotonsillectomy. Cardiac catheterization was repeated in one case, with right ventricular pressure dropping from 44/5 to 21/2, pulmonary vascular resistance from eight units to three, and PACO2 from 62 to 44.
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PMID:Cardiac and pulmonary failure secondary to adenotonsillar hypertrophy. 95 48

The clinical and hematological features of 100 patients with sickle cell anemia are reviewed. The heart was enlarged and a murmur was heard in nearly 80 percent of patients. Pneumonia and pulmonary infarction occurred in 43 percent and 12 percent of patients, respectively. Musculoskeletal involvement included the hand-foot syndrome (15 percent), leg ulcers (55 percent), aseptic necrosis ofbone (11 percent), and osteomyelitis (4 percent). Symptoms and signs related to the gastrointestinal system included jaundice (55 percent), hepatomegaly (50 percent), splenomegaly (23 percent), hepatitis (11 percent) and gallstones (9 percent). Three patients underwent cholecystectomy and three patients had their spleens removed. Pyelonephritis occurred in 17 patients, priapism in five and hematuria in seven. Nineteen women had 39 pregnancies, of which 35 resulted in the birth of healthy infants. At least 328 painful crises occurred in 73 patients. There were also 13 hemolytic crises, eight sequestration crises, and five aplastic crises. A trail of alkali therapy in 33 crises in children failed to produce beneficial effects greater than hydration and analgesics alone as used in the control group. Laboratory findings in the 100 patients were comparable to those previously reported in the literature. The renal concentrating defect in most patients was confirmed. There were six deaths: hepatic coma secondary to post-transfusion hepatitis, thrombosis of inferior vena cava, congestive heart failure, exsanguination from erosion of the pancreaticoduodenal artery, extensive bronchopneumonia, and pulmonary infarction.
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PMID:Sickle cell anemia- clinical manifestations in 100 patients and review of the literature. 113 Apr 36


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