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

The features of the syndrome of mulibrey nanism, an autosomal recessive disorder of unknown pathogenesis, include severe growth failure, yellow pigmentation of the retina, evidence of pericardial constriction, J-shaped sella turcica, and fibrous dysplasia of bones. To date, 24 individuals from Finland and a boy from Egypt have been reported with the syndrome. The patient reviewed in this article is the first known affected child from the United States. It is important that physicians look for this disorder in children with severe growth failure and hepatomegaly because of the potential seriousness of undetected pericardial constriction.
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PMID:Growth failure with pericardial constriction. The syndrome of mulibrey nanism. 13 12

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

A short survey is given on description and evaluation of progress and developmental trend of laparoscopy. Important technical improvements are the cold light via the glass fibre light conductor, optics of high value, the colour photography with electronic flash-light, colour films and colour television, new accessory instruments and belongings. Now as ever hepatomegaly and splenomegaly, jaundice, ascites, portal hypertension, suspicion of cirrhosis and metastases are regarded as main indications. The laparoscopy deserves a greater consideration in unclear abdominal symptoms, in gynaecological diseases, for the proof of the affection of liver and spleen in lymphogranulomatosis, sarcoidosis, tuberculosis (with aimed liver biopsy). The endoscopic retrograde cholangiopancreatography in the differential diagnosis of the jaundice competes with the laparoscopy, but it is not able to supersede it. The laparoscopic judgment of the pancreas and the importance of visible changes of the fine structed. Among the contraindications of the laparoscopy the hiatal hernia has lost its significance.
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PMID:[Laparoscopy--current aspects]. 13 32

Radionuclide inferior vena cavagrams were done in 135 patients who had hepatomegaly, splenomegaly or a mass in the vicinity of the inferior vena cava (I.V.C.). 2-5mCi 66mTc phytate, 99mTc S colloid, 99mTc O4-, 99mTc-LIDA, 99mTc pyridoxyledene glutamate or 113mIn colloid were injected directly and rapidly into either a malleolar or a femoral vein while the patient lay supine under the 13.5'' detector head of a scinticamera. Rapid sequential scintiphotos were manually taken at approximately 1 sec. interval for 20-30 sec. Thus iliac vein, I.V.C., cardiopulmonary zone in infants, aorta and the arterial phase were visualized. 48% of these subjects had an abnormal I.V.C. and the depictions were interestingly varied, indicating that different patients responded in a different manner even to grossly similar pathologies. It became evident that this soft walled vessel could be compressed by both fluids and neoplastic tissue (Fig. 1, 2); the long I.V.C. channel could also be segmentally pushed away by a mass in its vicinity. (Fig. 2, 3, 4). An abnormal arterial flush usually differentiated between benign (Fig. 2) and malignant (Fig. 3) lesions, even when the mass was extra-hepatic (Fig. 4) and retiroperitoneal (Fig. 5). Such a systematic study of I.V.C. had not been possible earlier since the classical x-ray contrast inferior vena cavagram necessitates venous dissection, passage of a catheter, and the injection of large volume of fluid under an unphysiologically high pressure. The simplified radionuclide technique, however, permitted the study of neonates and critically ill subjects with massive ascites, while retaining a satisfactory reproducability.
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PMID:Frequency of inferior vena caval abnormalcy due to a juxtaposed pathology. 14 54

Mucopolysaccharidosis V (Scheie's syndrome, MPS-IS) is a very rare, autosomal recessively inherited metabolic disease. The degradation of dermatan sulphate and heparan sulphate is disturbed due to alpha-L-iduronidase deficiency, leading to intracellular storage and excessive urinary secretion of these substances. The characteristic clinical features are contractures (claw-like flexion of the fingers), umbilical and inguinal herniae, corneal opacity, hepatomegaly, myocardiopathy and minor skeletal malformations. A patient with Scheie's syndrome is now reported for the first time in Austria; the results of the clinical, biochemical, chromosomal, dermatoglyphic and electron optical investigations are described and discussed.
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PMID:[Mucopolysaccharidosis V (Ullrich-Scheie syndrome) (author's transl)]. 15 10

Peritoneoscopy revealed in 15 patients with malignant lymphomas splenomegaly (13 patients), hepatomegaly (10 patients, 8 of them with impressive or suspicious alterations of the liver surface) and enlargement of retroperitoneal lymph nodes or localised infiltration of a jejunal loop (3 patients). Aimed biopsy of liver, spleen or lymph nodes frequently established the diagnosis definitively. The various features of malignant lymphomas observable at peritoneoscopy are described. Based on these experiences peritoneoscopy is recommended in suspected malignant lymphomas as a real diagnostic aid.
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PMID:[The contribution of laparoscopy to diagnosis of malignant lymphomas (author's transl)]. 15 36

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

Famialial defect of hepatic fructose-1,6-diphosphatase. Diagnosis was suspected in a male newborn since a brother was also concerned by the disease. The disease may be therefore diagnosed early, when the onset is neonatal. In the neonatal distress syndromes due to hereditery disorders of metabolism, a semiologic field may be isolated in which symptoms begin after an interval of short duration with hypoglycemia, hepatomegaly, lactic acidosis and ketosis ("enlarged liver hypoglycemia"). This possibility leads first to a symptomatic treatment of hypoglycemia and acidoketosis, then to the feeding with human milk and simple functional tests for the diagnostic approach.
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PMID:[Neonatal lactic acidosis and hypoglycemia due to a congenital hepatic fructose-1,6-diphosphatase deficiency]. 16 61


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