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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric radiology 25 (1995), S. 265-266 
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We present the case of a 6-year-old boy who had an omphalocele repaired at day 1 of life. He had a secundum atrial septal defect and an anomalous inferior vena cava of a type which has not been previously reported. Cine-MRI was a useful noninvasive tool for diagnosing the anomalous subaortic innominate vein and four immature vessels which make up the venous drainage systems of the lumbar region. The recognition of this malformation is important in planning and executing surgical repair or cardiac catheterization for postoperative patients with omphaloceles.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    International journal of clinical oncology 4 (1999), S. 372-377 
    ISSN: 1437-7772
    Keywords: Key words Radiotherapy ; Non-small cell lung cancer ; Early stage lung cancer
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background. Surgery has been regarded as the standard treatment for patients with non-small cell lung cancer in the early stage, while radiotherapy has become an effective alternative for medically inoperable patients and those who refuse surgery. Methods. We reviewed the records of 31 patients with stage I–II non-small cell lung cancer treated by radiotherapy between 1980 and 1997. There were 15 patients in stage I and 16 in stage II. The variables analyzed for influence on cause-specific survival and loco-regional control were: age, performance status, clinical stage, tumor size, tumor site, radiation field, radiation dose, and combination with chemotherapy. Results. The overall and cause-specific 1-, 2-, 3-, and 5-years survival rates were 71% and 77%; 63% and 73%; 34% and 48%; and 17% and 32%, respectively. Five-year survival rate for patients with peripheral tumor in the lung was 72%, with 70% loco-regional control, while the 5-year survival rate of patients whose tumor originated in the central region was 20%, with 25% loco-regional control. These differences had marginal significance on univariate analysis (P = 0.07), but only tumor site (central vs peripheral ) showed marginal significant influence on cause-specific survival (P = 0.08) and loco-regional control (P = 0.07) on multivariate analysis. There were no fatal complications, including radiation-induced myelopathy. Conclusion. The present series showed satisfactory results with definitive radiotherapy for patients with medically inoperable stage I–II non-small cell lung cancer, with results similar to those in recent reports of radiotherapy. The only significant variable was that patients with peripheral tumors had a better prognosis than patients with central tumors.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 157 (1998), S. 648-652 
    ISSN: 1432-1076
    Keywords: Key words Lactic acidaemia ; Thiamine ; Pyruvate dehydrogenase complex ; Thiamine pyrophosphate ; Sodium dichloroacetate
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Lactic acidaemia is sometimes associated with a defect of the pyruvate dehydrogenase complex (PDHC), catalysing the thiamine-dependent decarboxylation of pyruvate. The activity of PDHC for different thiamine pyrophosphate (TPP) concentrations was determined in 13 patients with lactic acidaemia, clinically responsive to thiamine treatment in order to assess the role of PDHC in the aetiology of thiamine-responsive lactic acidaemia. Culture of lymphoblastoid cells and skin fibroblasts and muscle biopsies were performed in these 13 patients. The activity of PDHC to sodium dichloroacetate (DCA), known as the activator of PDHC, was also examined. Three groups were identified according to PDHC activity. Group 1 (two patients) displayed very low PDHC activity, which was not increased by DCA. This PDHC activity increased at high TPP concentrations. Group 2 (five patients) displayed below normal PDHC activity at low TPP concentrations, increased by DCA. This PDHC activity became normal at high TPP concentrations. PDHC deficiency in these patients of groups 1 and 2 was due to a decreased affinity of PDHC for TPP. Group 3 included six patients with normal PDHC activity at low as well as high TPP concentrations. This PDHC activity was increased by DCA. Conclusion High concentrations of TPP may be required for maximal activity of PDHC in some patients with lactic acidaemia. The assay of PDHC activity, performed at a low concentration of TPP (1 × 10−4 mM) allows selection of patients with thiamine-responsive lactic acidaemia.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 156 (1997), S. 704-708 
    ISSN: 1432-1076
    Keywords: Key words HCV antibody ; HCV RNA ; HCV genotype ; Post-transfusion hepatitis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Twenty-two children with chronic hepatitis serologically positive for hepatitis C virus (HCV) were treated with interferon-α (IFN-α). Liver biopsy showed chronic active hepatitis in 13 and chronic persistent hepatitis in 9 patients. A sustained clearance of HCV was observed in 8/22 children 12 months after the administration of IFN-α for 26 weeks, associated with normalization of HCV core antibody. Of these eight patients six had HCV genotype III and two HCV genotype II or IV. Hepatitis relapsed in seven other patients after completion of IFN-α with an increase in HCV core antibody titre, five with HCV genotype II, and two with HCV genotype III or IV. A second course of IFN-α suppressed the reactivation of HCV in all seven patients. Three of seven responders who relapsed after the first course remained negative for HCV RNA 12 months after their second course of IFN-α. However, the remaining four patients with HCV genotype II again relapsed after completing their second course of IFN-α. Seven children with the HCV genotype II resistant to IFN, including 8 weeks of IFN-β administration, and showed no significant reduction in HCV core antibody titre. Conclusion The genotype of HCV (III) and a reduction in the core antibody titre appear to be useful parameters for predicting the response to IFN-α therapy.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Neuroradiology 37 (1995), S. 649-652 
    ISSN: 1432-1920
    Keywords: Carmofur ; 5-Fluorouracil ; Leukoencephalopathy ; Magnetic resonance imaging
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Carmofur, a derivative of 5-fluorouracil, has recently been noted to have an infrequent but serious association with leukoencephalopathy. To our knowledge, there has been no report of early MRI findings in this leukoencephalopathy. We describe a case in which diffuse high signal intensity of the entire cerebral white matter, including the corpus callosum, was seen on T2-weighted magnetic resonance images. Although similar findings can be seen in many other diseases, carmofur-induced leukoencephalopathy should be suspected in a patient treated with carmofur. It is important to know the clinical and MRI characteristics of this condition, for early diagnosis and better prognosis.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1920
    Keywords: Key words Spinocerebellar ataxia type 6 ; Cerebellar cortical atrophy ; Magnetic resonance imaging
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe the MRI findings in three Japanese patients with spinocerebellar ataxia type 6 (SCA6) in which a polymorphic CAG repeat was identified in the gene encoding the α1A voltage-dependent P/Q-type Ca2+ channel subunit (CACNL1A4). All showed slowly progressive cerebellar ataxia and mild pyramidal signs. Neuroradiologically, they had moderate cerebellar atrophy, most prominently in the superior vermis, whereas the brain stem appeared to be spared. No abnormal signal intensity was identified.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Neuroradiology 37 (1995), S. 649-652 
    ISSN: 1432-1920
    Keywords: Key words Carmofur ; 5-Fluorouracil ; Leukoencephalopathy ; Magnetic resonance imaging
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Carmofur, a derivative of 5-fluorouracil, has recently been noted to have an infrequent but serious association with leukoencephalopathy. To our knowledge, there has been no report of early MRI findings in this leukoencephalopathy. We describe a case in which diffuse high signal intensity of the entire cerebral white matter, including the corpus callosum, was seen on T2-weighted magnetic resonance images. Although similar findings can be seen in many other diseases, carmofur-induced leukoencephalopathy should be suspected in a patient treated with carmofur. It is important to know the clinical and MRI characteristics of this condition, for early diagnosis and better prognosis.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-1971
    Keywords: Key words: Atrial natriuretic peptide — Brain natriuretic peptide — Cyclic guanosine monophosphate — Tetralogy of Fallot — Surgically repair — Dobutamine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. We examined the plasma concentrations of atrial and brain natriuretic peptides (ANP and BNP) and cyclic guanosine monophosphate (cGMP) during dobutamine infusion and their relationship with hemodynamic parameters in 14 patients with surgically repaired tetralogy of Fallot (TOF). Dobutamine was infused at an initial dose of 5 μg/kg/min and increased by 5 μg/kg/min up to 20 μg/kg/min. The plasma ANP, BNP, and cGMP concentrations were determined before infusion, at the end of each stage, and 15 minutes after discontinuing dobutamine infusion. The plasma concentrations of ANP, BNP, and cGMP were elevated in all patients before dobutamine infusion. The ANP, BNP, and cGMP concentrations decreased in 11 of the 14 patients during dobutamine infusion. In contrast, the plasma ANP and BNP concentrations increased in the remaining 3 patients without a change in the cGMP concentration. The right ventricular pressure and volume were significantly elevated in these patients. The plasma cGMP concentration correlated with the ANP concentration (r= 0.62, p 〈 0.01) but not the BNP concentration. The plasma ANP concentration during dobutamine infusion correlated with right ventricular systolic pressure (r= 0.71, p 〈 0.05), mean right atrial pressure (r= 0.29, p 〈 0.05), and mean pulmonary capillary wedge pressure (r= 0.32, p 〈 0.05). The BNP concentration correlated with right ventricular volume (r= 0.61, p 〈 0.05) and systolic pressure (r= 0.46, p 〈 0.05). In conclusion, rapid changes in ANP, BNP, and cGMP concentrations during dobutamine infusion reflect the changes in atrial and ventricle pressure and volume overload. In surgically repaired TOF, the ANP concentration is affected by right ventricular systolic pressure, right atrial pressure, and pulmonary capillary pressure. Furthermore, the BNP concentration reflects right ventricular pressure and volume overload.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-1971
    Keywords: Key words: Duchenne's muscular dystrophy — Signal-averaged electrocardiogram — Fast-Fourier transform — Ventricular tachycardia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. To establish the criteria for an abnormal signal-averaged electrocardiogram (SA-ECG) in patients with Duchenne's muscular dystrophy (DMD), we used the technique of fast-Fourier transformation and studied the effects of analyzed signal phase and attenuation of low frequency components by filter processing. Twenty-three patients with DMD and twenty age-matched healthy volunteers were studied. An abnormal SA-ECG was defined as an area ratio of high frequency components/low frequency components over the 97th percentile of normal controls. Frequency domain analysis of the terminal 40 ms of the QRS complex and 80 ms of the initial ST segment was most predictive for distinguishing DMD patients at risk for ventricular tachycardia (VT). Although filter processing reduced the power of low frequency components and increased the area ratios, the sensitivity for the prediction of VT was not significantly enhanced. The area ratios of 40–100 Hz/0–40 Hz were superior to the area ratios of 20–50 Hz/0–20 Hz for predicting patients with VT.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 156 (1996), S. 30-32 
    ISSN: 1432-1076
    Keywords: Key words Allergy  ;  Kawasaki disease  ;  Epidemic
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To evaluate the possible trend towards developing allergic disease in children who had suffered from Kawasaki disease (KD), we evaluated data related to allergy that were collected by parental questionnaire on 1,165 children who had suffered from KD. Comparisons were made with 5,825 sex- and age-matched control children. The incidence of household pets and of cigarette smoking in the family were significantly lower in the children with a history of KD than in those of control children. A family history of allergy was significantly more common in the children with a history of KD (71%) versus the controls (56%) (P 〈 0.001). The incidence of atopic dermatitis and of allergic rhinitis was significantly higher (by approximately 1.7 times) in the KD children versus the control group (P 〈 0.01), even in subgroups with no family history of allergy. Conclusion We suggest that a genetic predisposition to atopy may be associated with a susceptibility to KD. Patients with KD tended to develop atopic dermatitis and allergic rhinitis.
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