Biomedical subjects
Publications and source records attributed to R Mazzieri.
Through in situ hybridization of a cDNA probe to metaphase chromosomes, we localized the gene for the human urokinase receptor (PLAUR) on chromosome 19. RBG-banding permitted subchromosomal localization of the PLAUR gene to 19q13.
Basic fibroblast growth factor, a potent angiogenesis inducer, stimulates urokinase (uPA) production by vascular endothelial cells. In both basic fibroblast growth factor-stimulated and -nonstimulated bovine capillary endothelial and human umbilical vein endothelial cells single-chain uPA binding is mediated by a membrane protein with a Mr of 42,000. Exposure of bovine capillary or endothelial human umbilical vein endothelial cells to pmolar concentrations of basic fibroblast growth factor results in a dose-dependent, protein synthesis-dependent increase in the number of membrane receptors for uPA (19,500-187,000) and in a parallel decrease in their affinity (KD = 0.144-0.790 nM). With both cells, single-chain uPA binding is competed by synthetic peptides whose sequence corresponds to the receptor-binding sequence in the NH2-terminal domain of uPA. Exposure of bovine capillary endothelial cells to transforming growth factor beta 1, which inhibits uPA production and upregulates type 1 plasminogen activator inhibitor, the major endothelial cell plasminogen activator inhibitor, has no effect on uPA receptor levels. These results show that basic fibroblast growth factor, besides stimulating uPA production by vascular endothelial cells, also increases the production of receptors, which modulates their capacity to focalize this enzyme on the cell surface. This effect may be important in the degradative processes that occur during angiogenesis.
Fourty-four patients (aged 10 months to 15 years) were assessed in a double-blind study to observe the correlation between myocardial uptake of 67-gallium and endomyocardial biopsy in the detection of moderate to severe myocardial inflammation. The sensitivity and specificity of gallium-67 imaging were 87 and 81%, respectively. Based on these findings, immunosuppressive therapy can be assigned to children with dilated cardiomyopathy and positive myocardial uptake, since moderate and severe myocardial inflammation may be detected by this non-invasive method.
PURPOSE: To analyse hemodynamic parameters (left ventricles ejection fraction, cardiac index, mean pulmonary wedge pressure and left ventricle diastolic diameter, in a group of children with active myocarditis (diagnosed by endomyocardial biopsy) pre and post treatment with conventional therapy and immunosuppressive drugs (isolated prednisone or prednisone associated with azathioprine or cyclosporine). PATIENTS AND METHODS: Forty-four pediatric patients with active myocarditis were studied. Twenty males and 24 females from 10 months to 15 years old (median = 1.3 years). All patients were submitted to hemodynamic study and endomyocardial biopsy. The hemodynamic parameters mentioned above were analysed before and after the proposed therapy. The patients were distributed in group according to the admission in the protocol, group I (9 pts)--conventional therapy (CT); group II (12 pts)--CT plus prednisone; group III (16 pts)--CT plus prednisone plus azathioprine; group IV (13 pts)--CT plus prednisone plus cyclosporine. RESULTS: Forty-four patients were submitted to four different groups of therapeutic protocol. The hemodynamic parameters were analysed in each of them. Left ventricle ejection fraction were no significantly different in the pre and post therapeutic scheme in group I and II, they were significantly higher (p less than 0.05) in group III and IV. The same happened with cardiac index. The mean pulmonary wedge pressure no presented statistical differences in group I and II before and after treatment but significantly lower lends were observed in group III and IV. The same behavior were noted in the left ventricle end diastolic diameter. CONCLUSION: The association of azathioprine or cyclosporine to prednisone presented better results in the left ventricle function, when compared with conventional drugs or isolated use of prednisone, based in the analysis of same hemodynamic parameters.
The report of these two cases show the accentuated and progressive increase in the ventricular contractility in the late postoperative period in patients who underwent surgical correction of left coronary artery with origin at the pulmonary trunk. Male and female infants, both developed congestive heart failure not controlled by medical treatment. The diagnosis was made by EKG and confirmed by coronary angiogram. They were surgically treated by translocation of the left coronary artery from the pulmonary artery to the aorta. Nine and seven years later, respectively both patients are free of symptoms with marked recovery of the left ventricle function without alteration in height and weight and in excellent clinical conditions. The early surgical treatment in these cases, even with unfavourable clinical status is the only way to change the natural history of this pathology, with an increase of the ventricular contractility.
Female infant, presenting with flexion spasms, skin hypochromatic spots and systolic ejection murmur. A clinical diagnosis of tuberous sclerosis, supported by computerized brain tomography and neurosonographic data, was made. Echocardiographic and cineangiocardiographic studies disclosed an associated tumor in left ventricle outlet. After resection of the rhabdomyoma, the patient is asymptomatic with neural manifestations controlled under valproic acid.
PURPOSE: This study was designed to compare 67Ga imaging and endomyocardial biopsy (EB) in children with severe dilated cardiomyopathy (DC), as well as to evaluate the results in a group of patients with active myocarditis submitted to immunosuppressive therapy. PATIENTS AND METHODS: Forty-four pediatric patients with severe DC were studied. Twenty males and 24 females from 10 months to 15 year old (median = 2.6 years). All patients were submitted to a protocol including 67Ga uptake and EB. In patients submitted to immunosuppressive therapy these procedures were repeated after six months. RESULTS: In 32 patients (72.7%) the EB revealed presence of inflammatory process; 21 (65.6%) of these had a positive 67Ga uptake and 11 (34.4%) negative. Twelve patients with no evidence of inflammatory process in the EB, nine (75%) presented negative 67Ga uptake. However, when the intensity of myocardial inflammatory was analysed (mild, moderate and severe) and correlated with 67Ga imaging, was observed that the majority of patients with negative 67Ga uptake (11 patients) had mild inflammatory infiltration (nine patients). In this way the 67Ga uptake demonstrated a good correlation in the diagnosis of moderate and severe inflammatory process in children with DC. This is important because the use of immunosuppressive drugs is indicated only in these group. CONCLUSION: The 67Ga imaging is a noninvasive diagnostic method with a good sensitivity to the diagnostic method with a good sensitivity to the diagnosis of AM in children with severe DC, demonstrating to be very useful in the therapeutic approach.
The authors report a case of a child, 10 years old with a primary rhabdomyosarcoma of the heart that obstructed both caval veins. The clinical picture that lasted just three months before the admission was characterized by persistent fever, accentuated loss of weight and a very deteriorated general aspect. We emphasize the clinical aspects and other subsidiary elements that easily allowed to make the precise diagnosis in life as well as the therapeutic measures and follow up, besides a review of the literature, where it was confirmed the rarity of this pathological entity.
PURPOSE: To analyse the histological evolution of endomyocardial biopsies from children with active myocarditis, submitted or not to immunosuppressive therapy. PATIENTS AND METHODS: Four groups of patients were compared, clinically treated as follows: group I--anticongestive drugs (4 patients); group II--prednisone (5 patients); group III--prednisone plus azathioprine (9 patients); group IV--prednisone and cyclosporine (5 patients). RESULTS: No patient from group I presented any histological improvement during a mean period of 9 months, while evident histological improvement occurred in 25% of patients from group I, 67% from group III and 80% from group IV. The microscopical aspect of resolving myocarditis was only observed in patients from groups III and IV, after treatment. CONCLUSION: The immunosuppressive therapy with azathioprine or cyclosporine plus prednisone leads to decrease of active myocarditis intensity in a higher proportion of cases than the treatment with only prednisone or no immunosuppressive drugs.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.