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  • 1
    Electronic Resource
    Electronic Resource
    Hyperuricaemia in cyanotic congenital heart disease (1993)
    Springer
    European journal of pediatrics 152 (1993), S. 873-876 
    Details
    ISSN: 1432-1076
    Keywords: Hyperuricaemia ; Cyanotic congenital heart disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This study examines the exacerbating factors of hyeruricaemia in patients with cyanotic congenital heart disease (CCHD). We studied 59 CCHD patients aged 1 month-30 years. The following variables were assessed: serum uric acid levels, red blood cell count, haemoglobin, hematocrit, partial oxygen pressure and arterial oxygen saturation. Uric acid excretion and renal function were also measured in ten patients with serum levels of uric acid greater than 8 mg/dl (hyperuricaemia group). Serum uric acid level correlated significantly with age and severity of polycythaemia. However, it did not correlate with partial oxygen pressure or arterial oxygen saturation. Uric acid excretion was measured in hyperuricaemia group. Urinary uric acid excretion (24 h) was within normal limits in infants but markedly lower in patients over 15 years of age. The aetiology of hyperuricaemia and decreased uric acid fractional excretion and clearance in infants appears to be secondary to diminished excretion of uric acid in concert with uric acid overproduction. Hyperuricaemia in adolescents and adults with CCHD, however, results mainly from age-related impairment of uric acid excretion.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01957519
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  • 2
    Electronic Resource
    Electronic Resource
    Echocardiographic evaluation of the development of aortic valve prolapse in supracristal ventricular septal defect (1995)
    Springer
    European journal of pediatrics 154 (1995), S. 176-181 
    Details
    ISSN: 1432-1076
    Keywords: Key words Supracristal ventricular septal defect ; Aortic valve prolapse ; Aortic regurgitation ; Colour flow mapping
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The development and timing of aortic valve prolapse (AoVP) and aortic regurgitation (AR) was studied by two-dimensional echocardiography in 99 consecutive patients with supracristal ventricular septal defect (VSD). Thirty patients (30%) had aortic valve prolapse (VSD + AoVP group), and 31 patients (31%) had AoVP with AR (VSD + AoVP + AR group). In the VSD + AoVP group, AoVP was detected first by echocardiography at the age of 6.8 ± 4.2 years (mean ± SD). In the VSD + AoVP + AR group, the interval from detection of AoVP to the appearance of AR was 3.4 ± 2.0 years. The configuration of the prolapsed aortic valve was echocardiographically classified into two types: tear-drop type (small) prolapse and box type (large) prolapse. The frequency of tear-drop type prolapse was not significantly different between VSD + AoVP and VSD + AoVP + AR groups (43% versus 32%, respectively), indicating that even minor AoVP can result in AR. Four infants (4%) had AoVP at the ages of 1, 5, 7, and 11 months, respectively. All infants had tear-drop type prolapse. Two infants developed AR by colour flow mapping at the ages of 3 and 11 months, and the interval from prolapse to AR was only 2 and 4 months, respectively. Conclusion Aortic valce involement can develop under the age of 1 year in supracristal VSD. Regular evaluation by two-dimensional echocardiography with colour flow mapping is important in the follow-up of children with supracristal VSD.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01954266
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  • 3
    Electronic Resource
    Electronic Resource
    Echocardiographic evaluation of the development of aortic valve prolapse in supracristal ventricular septal defect (1995)
    Springer
    European journal of pediatrics 154 (1995), S. 176-181 
    Details
    ISSN: 1432-1076
    Keywords: Supracristal ventricular septal defect ; Aortic valve prolapse Aortic regurgitation ; Colour flow mapping
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The development and timing of aortic weve prolapse (AoVP) and aortic regugitation (AR) was studied by two limensional echocardiography in 99 consecutive patients with supracristal ventricular septal defect (VSD). Thirty patients (30%) had aortic valve prolapse (VSD+AoVP group), and 31 patients (31%) had AoVP with AR (VSD+AoVP+AR group). In the VSD+AoVP group, AoVP was detected first by echocardiography at the age of 6.8±4.2 years (mea±SD). In the VSD+AoVP+AR group, the interval from detection of AoVP to the appearance of Al was 3.4±2.0 years. The configuration of the prolapsed aortic valve was echocardiographically classified into two types: teardrop type (small) prolapse and box type (large) prolapse. The frequency of tear-drop tyrolapse was not significantly different between VSD+AoVP and VSD+AoVP-AR groups (43% versus 32%, respectively), indicating that even minor AoVP can result, AR. Four infants (4%) had AoVP at the ages of 1, 5, 7, and 11 months, respectively. All infants had tear-drop type prolapse. Two infants developed AR by colour flow mapping at the ages of 3 and 11 months, and the interval from prolapse to AR was only 2 and 4 months, respectively. Conclusion Aortic valce, involement can develop under the age of 1 year in supracristal VSD. Regular evaluation by two-dimensional echocardiography with colour flow mapping is important in the followup of children with supracristal VSD.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01954266
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  • 4
    Electronic Resource
    Electronic Resource
    Efficacy of interferons in treating children with chronic hepatitis C (1997)
    Springer
    European journal of pediatrics 156 (1997), S. 704-708 
    Details
    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
    URL: http://dx.doi.org/10.1007/s004310050694
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  • 5
    Electronic Resource
    Electronic Resource
    Effect of acidosis on Ca2+-activated K+ channels in cultured porcine coronary artery smooth muscle cells (1998)
    Springer
    Pflügers Archiv 436 (1998), S. 509-514 
    Details
    ISSN: 1432-2013
    Keywords: Key words Acidosis ; Smooth muscle ; Coronary artery ; Ca2+-activated K+ channels
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Although acidosis induces vasodilation, the vascular responses mediated by large-conductance Ca2+-activated K+ (KCa) channels have not been investigated in coronary artery smooth muscle cells. We therefore investigated the response of porcine coronary arteries and smooth muscle cells to acidosis, as well as the role of KCa channels in the regulation of muscular tone. Acidosis (pH 7.3–6.8), produced by adding HCl to the extravascular solution, elicited concentration-dependent relaxation of precontracted, endothelium-denuded arterial rings. Glibenclamide (20 µM) significantly inhibited the vasodilatory response to acidosis (pH 7.3-6.8). Charybdotoxin (100 nM) was effective only at pH 6.9–6.8. When we exposed porcine coronary artery smooth muscle cells to a low-pH solution, KCa channel activity in cell-attached patches increased. However, pretreatment of these cells with 10 or 30 µM O, O′-bis(2-aminophenyl)ethyleneglycol-N,N,N′,N′-tetraacetic acid tetrakis(acetoxymethyl)ester (BAPTA-AM), a Ca2+ chelator for which the cell membrane is permeable, abolished the H+-mediated activation of KCa channels in cell-attached patches. Under these circumstances H+ actually inhibited KCa channel activity. When inside-out patches were exposed to a [Ca2+] of 10–6 M [adjusted with ethyleneglycolbis(β-aminoethylester)-N,N,N′,N′-tetraacetic acid (EGTA) at pH 7.3], KCa channels were activated by H+ concentration dependently. However, when these patches were exposed to a [Ca2+] of 10–6 M adjusted with BAPTA at pH 7.3, H+ inhibited KCa channel activity. Extracellular acidosis had no significant direct effect on KCa channels, suggesting that extracellular H+ exerts its effects after transport into the cell, and that KCa channels are regulated by intracellular H+ and by cytosolic free Ca2+ modulated by acute acidosis. These results indicate that the modulation of KCa channel kinetics by acidosis plays an important role in the determination of membrane potential and, hence, coronary arterial tone.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004240050665
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  • 6
    Electronic Resource
    Electronic Resource
    Age-related criteria for signal-averaged electrocardiographic late potentials in children (1994)
    Springer
    Pediatric cardiology 15 (1994), S. 107-111 
    Details
    ISSN: 1432-1971
    Keywords: Signal-averaged electrocardiography ; Criteria ; Normal values
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary This study examined the age-related criteria of signal-averaged electrocardiographic (SA-ECG) parameters in children. SA-ECGs were obtained in 82 healthy volunteers in six groups depending on age (group 1:1 day to 〈1 month; group 2:1 month to 〈1 year; group 3:1 to 〈6 years; group 4:6 to 〈12 years; group 5:12 to 〈20 years; group 6:20 to 〈40 years). To examine the effect of heart rate on the parameters of SA-ECG, right atrial pacings were performed in 4 children with a ventricular septal defect aged 1–7 years. The root mean square voltage (RMS) was high; and the filtered QRS (f-QRS) duration and the duration of the low amplitude signal (LAS) were low during childhood, especially in infants (group 2), compared with those in adults. Late duration (LD) had no significant difference among age groups. The criteria for ventricular late potential were as follows: RMS〈25 μV, LAS〉35 ms, f-QRS duration 〉110 ms, and LD〉35 ms for those age 1 day to 〈1 month; RMS〈40 μV, LAS〉30 ms, f-QRS duration 〉100 ms, and LD〉35 ms for those age 1 month to 〈1 year; RMS〈20 μV, LAS〉40 ms, f-QRS duration 〉130 ms, and LD〉35 ms for those age 1 to 〈20 years; and RMS〈15 μV, LAS〉45 ms, f-QRS duration 〉135 ms, and LD〉35 ms for those age 20 to 〈40 years. RMS and LAS correlated with f-QRS duration (r=−0.78 and 0.76, respectively;p〈0.05), suggesting that these parameters are associated with the thickness of the ventricular muscle and the ventricular conduction time. Right atrial pacing had no effect on the measured SA-ECG parameters. The age-related differences in SA-ECG parameters might be due to age-related differences in the thickness of the ventricular muscle and the ventricular conduction time but are not due to differences in the heart rate. The age difference of each parameter on SA-ECG should be considered for ventricular late potentials.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00796320
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  • 7
    Electronic Resource
    Electronic Resource
    Hyperuricemia in an infant with Taussig-Bing anomaly and interruption of the aortic arch (1994)
    Springer
    Pediatric cardiology 15 (1994), S. 249-251 
    Details
    ISSN: 1432-1971
    Keywords: Cyanotic congenital heart disease ; Hyperuricemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Hyperuricemia is commonly recognized in adolescents and adults with cyanotic congenital heart disease. We report a case of a male infant with hyperuricemia, Taussig-Bing anomaly, and interruption of the aortic arch. The patient underwent correction of interrupted aortic arch and pulmonary arterial banding at the age of 7 days. Hyperuricemia appeared when he was 2 months old (max 17.7 mg/dl) and persisted until he underwent a Jatene operation at the age of 10 months. The hyperuricemia improved gradually after the disappearance of hypoxia and polycythemia. The laboratory findings suggest that hyperuricemia can result from uric acid overproduction due to secondary polycythemia, impairment of uric acid excretion by the kidney, or the acceleration of anaerobic metabolism. Allopurinol and benzbromarone together were partially effective treatments for hyperuricemia in this patient with cyanotic congenital heart disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00795737
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  • 8
    Electronic Resource
    Electronic Resource
    Plasma Concentrations of Atrial and Brain Natriuretic Peptides and Cyclic Guanosine Monophosphate in Response to Dobutamine Infusion in Patients with Surgically Repaired Tetralogy of Fallot (1999)
    Springer
    Pediatric cardiology 20 (1999), S. 343-350 
    Details
    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
    URL: http://dx.doi.org/10.1007/s002469900481
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  • 9
    Electronic Resource
    Electronic Resource
    Primary Mitral Valve Sarcoma in Infancy (1998)
    Springer
    Pediatric cardiology 19 (1998), S. 174-177 
    Details
    ISSN: 1432-1971
    Keywords: Key words: Cardiac tumor in infancy — Primary cardiac tumor — Sarcoma of mitral valve — Hemiparesis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Primary cardiac sarcoma is a rare tumor, found especially in children, particularly in the left side of the heart. This report describes a rare case of primary mitral valve sarcoma in a 7-month-old male infant with hemiparesis and heart murmur who underwent mitral valve replacement because of excessive invasion of the mitral valve by the tumor. The patient remains well, free of recurrence and cerebral metastasis, and without adjuvant therapy 18 months after the operation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002469900274
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  • 10
    Electronic Resource
    Electronic Resource
    Anomalous inferior vena cava in association with omphalocele: A case report (1995)
    Springer
    Pediatric radiology 25 (1995), S. 265-266 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF02011095
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