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)

The disease course is described in 21 patients with low serum concentrations of alpha1-antitrypsin of the phenotype Z (genotype pi ZZ). 13 of these patients have long-standing disease characterized by bronchitis or dyspnea beginning before the age of 40 and progressing to emphysema (11 patients) and to corpulmonale (7 patients). The remaining 8 patients are children with hepatopathy characterized by prolonged jaundice at birth, persistent hepatomegaly and persistently elevated liver enzymes. In 2 children, the evolution to cirrhosis was ascertained by biopsy.
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PMID:[Severe alpha 1-antitrypsin deficiency: clinical observations of 21 patients]. 31 May 77

This report represents the Brompton Hospital experience of infants and neonates with critical pulmonary valve stenosis and aortic valve stenosis. There were 25 patients with critical pulmonary valve stenosis and 8 with critical aortic valve stenosis. Critical pulmonary stenosis: Among the 25 patients up to one year of age, 11 were girls and the commonest presenting symptom was cyanosis seen in 15, the commonest physical sign being a systolic ejection murmur with a single 2nd sound. A more detailed analysis of the symptoms, signs and findings at special investigation will be presented. The evolution of a satisfactory technique of operation will be indicated in these critically ill patients, and the late follow-up will be presented. Critical aortic stenosis: There were 8 patients in this group all of whom were male. Dyspnoea was the commonest presenting symptom with a systolic ejection murmur and hepatomegaly as the commonest signs. All patients in this group underwent open aortic valvotomy and/or valvuloplasty with 3 deaths. A late follow-up of the survivors will be presented. In summary, a series of 33 patients up to the age of one year with critical obstruction of either the pulmonary or aortic valves will be presented. (his is one of the larger series to be reported in this age group.
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PMID:[Critical outflow valve obstruction under the age of one year (author's transl)]. 108 66

In this article, the clinical and epidemiological characteristics of 137 children with cardiomyopathy admitted to the Hacettepe Pediatric Cardiology Unit were studied and the prognosis was evaluated after a follow-up period of 24 +/- 5 months. It was found that the highest proportion of patients were residents of Ankara, followed by the Northern and the Central Anatolian regions, and consanguinity between their parents was more common than the proportion for Turkey as a whole. Most of the patients had dilated cardiomyopathy (78.9%) and the age at which symptoms appeared varied according to the type of cardiomyopathy. A high proportion of patients came to the hospital with complaints of dyspnea and a decrease in effort capacity. The most common findings on the physical examination were hepatomegaly and tachycardia. Electrocardiographic and echocardiographic evaluations were made for all patients and cardiac catheterization and endomyocardial biopsy were performed when necessary. The outcome was as follows: 34.5% improved, 45.7% remained stable, 9.5% deteriorated and 10.3% died.
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PMID:Cardiomyopathies in children. Clinical, epidemiological and prognostic evaluation. 208 76

Immunotherapy with interleukin (IL)-2 possesses great potential in the treatment of immune-mediated diseases and cancers. However, only a few reports on a small number of children have appeared in the literature. From March 1988 to March 1989, 11 children and adolescents were treated with IL-2. They included 1 patient with hepatocellular carcinoma, 1 with hepatoblastoma, 6 with childhood atopic dermatitis, and 3 with juvenile rheumatoid arthritis. The dosages ranged from 10,000 to 50,000 U/kg every 8 hours by intravenous drip. The following side effects were observed: anorexia, fever, and chillness (100%), general malaise (82%), irritability (64%), diarrhea (100%), nausea and vomiting (73%), weight gain (82%), edema (82%), abdominal distension (73%), oliguria (82%), cough (91%), dyspnea (27%), pleural effusion (40%), hypotension (82%), skin eruption (82%), oral ulcer (18%), enlarged liver (73%) liver function abnormalities (82%), renal function impairment (36%), electrolyte imbalance (73%), anemia (91%), thrombocytopenia (54%), leukopenia (18%), and eosinophilia (73%). Immunologically, numbers of natural killer cells were increased and natural killer and lymphokine-activated killer cell activities were augmented after IL-2 treatment. There was a tendency for serum levels of IL-2 and receptor IL-2 to decrease, especially in patients with atopic eczema. Ten patients (91%) completed one course (9 to 12 days) of therapy, and the remaining patient interrupted the treatment because of intolerable adverse effects. Clinically, complete remission for 3 months was obtained in 1 juvenile rheumatoid arthritis patient, transient improvement (2 to 6 weeks) in all atopic dermatitis patients, minor response in the hepatoblastoma patient, and no response in the patient with hepatocellular carcinoma.
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PMID:Interleukin-2 immunotherapy in children. 217 36

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

102 patients with myocardial infarction (MI) were examined by three clinicians, who independently recorded the following symptoms and signs: dyspnoea, a displaced apex beat, S3-gallop, rales, neck vein distension, hepatomegaly, and dependent oedema. Chest X-ray, radionuclide ventriculography, and (in 40 patients) right heart catheterization were carried out immediately after the physical examination. The clinicians frequently disagreed as to the presence of physical signs of heart failure in individuals. Moreover, these signs were of limited value in identifying patients with pulmonary vascular congestion on chest X-ray, reduced left or right radionuclide ventricular ejection fractions, enlarged ventricular volumes or haemodynamic evidence of ventricular dysfunction. We conclude that clinicians frequently disagree in the recognition of physical signs of heart failure, and that these signs have an unpredictable relationship to radiographic, radionuclide and haemodynamic measures of ventricular performance in patients with MI. Nevertheless, physical signs are useful in identifying patients with high risk of cardiac death.
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PMID:Symptoms and signs of heart failure in patients with myocardial infarction: reproducibility and relationship to chest X-ray, radionuclide ventriculography and right heart catheterization. 259 93

We reviewed the clinical data on 23 patients operated on for constrictive pericarditis. Mean age was 35 years and 17 were males. The chief complaint was dyspnea (87%). Outstanding physical findings were venous hypertension (96%), hepatomegaly (78%), ascitis (57%), pulsus paradoxus (57%) and Kussmaul sign (43%). The ECG, although always abnormal was non contributory. Chest X ray findings included cardiomegaly (48%) and pericardial calcification (35%). Half of the cases showed pericardial thickening and abnormal septal motion on M mode echocardiography. An equalization of diastolic pressures and the dip and plateau sign was confirmed at cardiac catheterization in all cases. The etiology could be established in only 6 patients (tuberculosis 3, traumatic 2 and septic 1). Surgical mortality was 9% (2 patients). Follow up was available on 10 patients at a mean of 35 months, 9 in functional class I and 1 in FC II.
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PMID:[Constrictive pericarditis: diagnostic aspects and surgical results in 23 cases]. 264 22

Between July 1983 and February 1984, eight children with adenovirus Type 3 infection, proven by virus isolation from sputum, stool or nasopharyngeal swabs and a fourfold increase in complement fixation antibody titers against the virus, were treated in our department. All eight patients had fever lasting at least 7 days, hepatomegaly, diffuse pulmonary infiltrates and abnormal liver function tests. Seven of the patients exhibited dyspnea and pulmonary wheezing. Six of the patients developed changes in state of consciousness, and three had repeated convulsions. EEG patterns in three of the patients were compatible with encephalopathy. Other clinical manifestations included: follicular tonsillitis in two patients, diarrhea in two, pneumothorax in one, and shock with disseminated intravascular coagulation in one. The spectrum of adenovirus Type 3 infection reported here has been described previously only in the viral hemorrhagic fevers. This adenovirus Type 3 infection shares the potential for disseminated disease that has been described previously for Type 7, simulating Reye's syndrome.
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PMID:Adenovirus type 3 infection with systemic manifestation in apparently normal children. 302 30

HIV infection was present in 18 out of 40 (45%) consecutive malnourished children aged 2 to 29 months in pediatric wards of Bujumbura, Burundi. No difference was observed within and between the seropositive and seronegative groups for sex and anthropometric measures. HIV seropositive cases could be explained by a HIV seropositive mother (83%) or by a transfusion history (17%). The onset of marasmus was earlier in the HIV seropositive group (5 cases observed less than 6 months old compared to none of the other group, Fischer's exact test: P = 0.026). A more complex clinical picture was seen in the HIV seropositive cases (12/18 compared to 4/22, Fischer's exact test: P = 0.004) with the presence of hepatomegaly, adenopathy, thrush, dyspnoea and skin disorders. No difference was observed concerning fever and diarrhoea. HIV seropositive group tended to show a higher hospitalisation frequency and did not well respond to high protein-energy diet: 7 were discharged without gain weight compared to none of the other group (Fischer's exact test: P = 0.011). These results suggest a high rate of vertical transmission mother-child for HIV infection and a frequent association of malnutrition and HIV infection in hospitalized children in Burundi. Marasmic children less than 6 months old should be highly suspected of HIV infection.
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PMID:[Malnutrition and HIV infection in children in a hospital milieu in Burundi]. 313 33

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


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