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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 145 (1986), S. 454-459 
    ISSN: 1432-1076
    Keywords: Cardiomyopathy ; Genetic metabolic disease ; Congenital heart defects
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This is a survey of genetic metabolic diseases in which cardiomyopathy is typical or can be the leading symptom in infancy. Apart from the well-known Pompe disease, several other storage disorders, mitochondrial disorders, and miscellaneous conditions (particularly the carnitine deficiency syndromes) may be seen in this way. Since prompt diagnosis may be mandatory for genetic counselling, and sometimes for specific treatment, guidelines for clinical, cardiological, and laboratory work-up are given.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: Keywords Thrombosis  ;  Activated protein C resistance  ;  Protein C deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  In many children, the pathogenesis of thrombo-embolism remains unexplained. This study examines the role of non-genetic risk factors in 37 children with venous or arterial thrombosis. Included were 17 patients with portal vein thrombosis following umbilical vein catheterisation, 6 with portal vein thrombosis and an uneventful neonatal period, 4 with deep vein␣thrombosis, 4 with renal vein thrombosis after kidney transplantation, 1 haemodialysis patient with thromboses of arteriovenous shunts, and 5 with arterial thromboses at various sites. In 25 of these 37 patients (68%) exogenic risk factors and particularly vascular manipulations (24/37) were related to the thrombotic event. Resistance to activated protein C was identified in 5 patients and protein C deficiency in 2 (7/37; 19%). This prevalence was significantly higher than that of the control group (14/243; 5.8%; χ2, P 〈 0.008). Conclusion Our data show that non-genetic and particular iatrogenic risk factors can often be identified in children with thrombosis, but activated protein C resistance and protein C deficiency are significant genetic risk factors in this age group.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 147 (1988), S. 558-558 
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 157 (1998), S. 885-889 
    ISSN: 1432-1076
    Keywords: Key words Pulmonary atresia ; Interventional heart catheterization ; Balloon valvuloplasty ; Stent implantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In neonates with pulmonary atresia and intact ventricular septum the aims of therapy are maintenance of pulmonary blood flow and right ventricular decompression in order to achieve right ventricular support of the pulmonary circulation. Recent developments in interventional heart catheterization with pulmonary radiofrequency-assisted balloon valvuloplasty and ductal stent implantation offer an alternative to the classical surgical approach. We report on a neonate with membranous pulmonary atresia and intact ventricular septum, in whom a large interatrial right-to-left shunt via the foramen ovale persisted after radiofrequency-assisted pulmonary balloon valvuloplasty on the 2nd day of life. The interatrial shunt prevented adequate right ventricular filling and antegrade pulmonary perfusion leading to severe cyanosis (transcutaneous oxygen saturation 40%). In order to increase pulmonary blood flow and raise left atrial pressure, the arterial duct was stented. After ductal stenting, prostaglandin was discontinued and the transcutaneous oxygen saturation remained stable around 89%. At follow up after 7 weeks the foramen ovale had decreased in size and only a small left-to-right shunt was present, documenting the effectiveness of this approach. Conclusion Based on the present case we propose a stepwise interventional approach for the neonate with pulmonary atresia and intact ventricular septum. If cyanosis persists after isolated pulmonary valvuloplasty despite adequate right ventricular function, ductal stent implantation can reduce interatrial shunting and thus improve oxygen saturation.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1540-8183
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: A newborn with pulmonary atresia with intact ventricular septum (PA-IVS) was treated with radiofrequency current perforation of the atretic pulmonary valve. As the right ventricle was hypoplastic (z-value of the tricuspid valve: —4) the arterial duct was stented with a Gianturco-Roubin GR II stent. Early postinterventionally, the patient became cyunotic and compromised blood flow across the stented ductus arteriosus despite adequate stent position was detected echographically. The newborn was treated successfully with the implantation of a Palmaz stent (Johnson & Johnson Interventional Systems, Warren, NJ, USA) into the obstructed Gianturco-Roubin GR II stent. The Gianturco-Roubin GR II stents might be associated with the risk of early stent stenosis after implantation in actively contracting tissues like the ductus arteriosus. In patients with early stent stenosis after ductal stenting, bailout implantation of a subsequent stent can be performed. Transcatheter procedures can be effective means for therapy of PA-IVS. (J Interven Cardiol 2000;13:39–44)
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Biochimica et Biophysica Acta (BBA)/Protein Structure and Molecular 952 (1988), S. 20-26 
    ISSN: 0167-4838
    Keywords: Active site ; Methionine sulfoxide ; Oxidation ; Perborate ; Proteinase ; Subtilisin
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Medicine
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 0014-5793
    Keywords: Amino acid sequence ; Cysteine-containing subtilisin-like proteinase ; Homology ; Secondary structure prediction ; Subtilisin ; Thermitase
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Physics
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1435-1285
    Keywords: Key words Supraventricular tachycardia – infancy – childhood – pharmacological therapy – endocardial mapping – radiofrequency catheter ablation ; Schlüsselwörter Supraventrikuläre Tachykardien – Säuglinge – Kinder – pharmakologische Therapie –¶endokardiales Mapping –¶Hochfrequenzstromablation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Supraventrikuläre Tachykardien sind die häufigste symptomatische Tachyarrhythmie im Säuglings- und Kindesalter. Die klinische Symptomatik ist neben der zugrundeliegenden Rhythmusstörung abhängig vom Lebensalter des Kindes sowie von der vorliegenden kardialen Anatomie. Neugeborene und Säuglinge mit paroxysmalen atrioventrikulären Reentry-Tachykardien entwickeln aufgrund der hohen Kammerfrequenzen rasch Zeichen der Herzinsuffizienz. Ältere Kinder und Jugendliche hingegen klagen häufig über Palpitationen. Kinder mit chronisch-permanenten Tachykardien wie der ektopen Vorhoftachykardie und der permanenten Form der junktionalen Reentry-Tachykardie entwickeln zu einem beträchtlichen Anteil eine sekundäre Form der dilatativen Kardiomyopathie, die sogenannte „Tachymyopathie”.¶   Adenosin ist inzwischen Mittel der ersten Wahl in der Behandlung akuter Episoden atrioventrikulärer Reentry-Tachykardien in jeder Altersstufe. Zusätzlich erlaubt die Substanz im Zweifel die Diagnose primärer atrialer Tachykardien. Die Langzeit-Behandlung von atrioventrikulären Reentry-Tachykardien im Säuglings- und Kindesalter richtet sich nach dem Lebensalter sowie der Klinik des Patienten. Bei Neugeborenen und Säuglingen tritt bei der Mehrzahl der Patienten eine Spontanresolution der Tachykardien mit dem Ende des ersten Lebensjahres ein. Aus diesem Grund ist in dieser Altersklasse eine pharmakologische Therapie zu empfehlen. Im Gegensatz dazu ist die Aussicht auf ein spontanes Sistieren der Tachykardieneigung bei Kindern 〉1 Jahr äußerst gering. Daher stellt die Hochfrequenzstromablation des anatomischen Substrats der Tachykardien eine sinnvolle Alternative zur meist mehrjährigen medikamentösen Therapie dar. Die Ergebnisse der Ablationsbehandlung bei Kindern und Jugendlichen mit einem strukturell normalen Herz sind mit denen erwachsener Patienten vergleichbar. Bei Patienten mit einem angeborenen Herzfehler und supraventrikulären Tachykardien auf der Grundlage einer akzessorischen atrioventrikulären Leitungsbahn bzw. basierend auf einem AV-Knoten-Reentry-Mechanismus sollte die Hochfrequenzstromablation des anatomischen Substrats der Tachykardien bereits im Rahmen der präoperativen Herzkatheteruntersuchung erwogen werden.¶   Nach Korrekturoperation eines angeborenen Herzfehlers sind atriale Reentry-Tachykardien als signifikanter Risikofaktor für eine erhöhte spät-postoperative Morbidität und auch Letalität identifiziert worden. Eine pharmakologische Behandlung ist hier häufig nicht ausreichend wirksam, bei einem Teil der Patienten wird eine gleichzeitig bestehende Sinusknotendysfunktion erheblich verstärkt. Die Ablationsbehandlung mit konventionellem endokardialem Mapping mittels multipolarer Elektrodenkatheter mit dem Ziel der Lokalisierung der kritischen Zone des Reentry-Circuits ist mit einer zur Zeit nicht zufriedenstellenden Erfolgsrate sowie einer beträchtlichen Rezidivrate belastet. Innerhalb der nächsten Jahre ist es durch den Einsatz moderner Mappingverfahren wie dem elektroanatomischen Mapping sowie dem Non-Contact Mapping zu erwarten, dass die Ergebnisse der Ablationstherapie von atrialen Reentry-Tachykardien bei jungen Patienten nach Korrekturoperation angeborener Herzfehler erheblich verbessert werden können.
    Notes: Summary Supraventricular tachycardias are the most frequent forms of symptomatic tachyarrhythmias in infants, children and adolescents. Clinical symptoms depend on age and underlying cardiac anatomy. Newborn babies and infants with paroxysmal atrioventricular reentrant tachycardias usually present with signs of congestive heart failure due to rapid heart rate. In older children and adolescents, palpitations are the leading symptom. Patients with chronic-permanent tachycardias (i.e., atrial ectopic tachycardia, permanent form of junctional reciprocating tachycardia) often develop a secondary form of dilated cardiomyopathy, the so-called „tachymyopathy”.¶   Adenosine has evolved as the drug of choice in any age group for the termination of atrioventricular reentrant tachycardia of any origin. In addition, it serves as a diagnostic tool in primary atrial tachycardias. Long-term management of atrioventricular reentrant tachycardia in infancy and childhood is age dependent. In newborn babies and infants, pharmacological therapy is advised due to the high spontaneous cessation rate of those tachycardias at the end of the first year of life. In contrast to this, the probability of spontaneous cessation of tachycardia in children 〉1 year of age is very low. Therefore, radiofrequency catheter ablation of the anatomical substrate of the tachycardia is a rational alternative to long-lasting antiarrhythmic therapy. Results in children with a structurally normal heart are comparable to those achieved in adults. In patients with congenital heart disease and supraventricular tachycardias, catheter ablation during preoperative cardiac catheterization is recommended.¶   Atrial reentrant tachycardias have been identified as one major risk factor for late postoperative morbidity and mortality in young patients. Pharmacological therapy is often not sufficient to control the tachycardia. In addition, underlying sinus node dysfunction may be aggravated in a considerable portion of the patients affected. Catheter ablation based on conventional endocardial mapping techniques by multipolar electrode catheters with the aim of identifying the critical region of the reentrant circuit is associated with an impaired success rate and a considerable recurrence rate. It may be assumed that, using the modern mapping techniques currently available (electroanatomical mapping and non-contact mapping), results of radiofrequency catheter ablation of atrial reentrant tachycardias after surgical correction of congenital heart disease will be significantly improved within the next few years.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1435-1285
    Keywords: Key words Angioplasty – aortopulmonary shunt – congenital heart disease – cyanosis – pulmonary atresia ; Schlüsselwörter Zyanose – Pulmonalatresie – angeborene Herzfehler – aortopulmonaler Shunt – Angioplastie
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Wir berichten über ein Neugeborenes mit Pulmonalatresie und Ventrikelseptumdefekt und Transposition der großen Gefäße, bei dem im Alter von 5 Tagen ein modifizierter Blalock-Taussig-Shunt angelegt wurde. Nach Beendigung der Prostaglandintherapie fiel die arterielle Sauerstoffsättigung von zuvor 80% auf 60%. Die Herzkatheteruntersuchung zeigte eine operationstechnisch bedingte Stenose der Arteria subclavia proximal des Abgangs des modifizierten Blalock-Taussig-Shunts. Der Patient wurde erfolgreich mittels Angioplastie und Stentimplantation behandelt.
    Notes: Summary A newborn with pulmonary atresia with ventricular septal defect and transposition of the great arteries was palliated with a modified Blalock-Taussig shunt at 5 days of age. Three days postoperatively arterial oxygen saturations dropped significantly. Thrombosis of the shunt was suspected. Angiography revealed stenosis of the proximal subclavian artery related to the creation of the shunt. The patient was successfully treated with angioplasty and stent implantation into the stenotic sublavian artery. Follow-up angiography 3 months after stent deployment showed a completely patent subclavian artery and unobstructed blood flow across the modified Blalock-Taussig shunt.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1435-1285
    Keywords: Schlüsselwörter Hämangioendotheliom – Gastrointestinaltrakt – Embolisation – Herzinsuffizienz – arteriovenöse Malformation ; Key words Hemangioendothelioma – gastrointestinal tract – embolization – congestive heart failure – arteriovenous malformation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary We report on a newborn (birth weight 3600 g) with high-output cardiac failure due to a large hepatic hemangioendothelioma requiring mechanical ventilation. One day post-partum therapy with steroids, furosemide, and digitalis was initiated. Despite this, the clinical condition deteriorated. Therefore, selective coil-embolization of the arterial vessels supplying the hemangioendothelioma was performed using a venous approach with passage to the arterial side via the ductus arteriosus. After the embolization the patient improved quickly and he was extubated 4 days postinterventionally. Follow-up sonography 4 months postinterventionally showed dramatic regression of the hepatic tumor. We conclude that the coil-occlusion of hepatic hemangioendothelioma should be performed early in symptomatic newborns as arterial puncture and its associated complications can be avoided by using a venous approach with passage to the arterial side via the ductus arteriosus. In addition, in this age group, access to the portal system can be gained through the umbilical vein to occlude portal venous supplies to the hepatic hemangioendothelioma.
    Notes: Zusammenfassung Wir berichten über ein Neugeborenes (Geburtsgewicht 3660 g), welches postpartal wegen eines hepatischen Hämangioendothelioms und dem damit verbundenen arteriovenösen Shuntvolumen kardiorespiratorisch dekompensierte. Eine Therapie der Herzinsuffizienz mit Digitalis und Diuretika sowie eine Behandlung mit Kortikosteroiden wurde am 1. Lebenstag begonnen, konnte die Symptomatik jedoch nicht verbessern. Aus diesem Grund wurde eine interventionelle Therapie des Hämangioendothelioms mittels Coil-Okklusion der arteriellen Tumorgefäße durchgeführt. Die Symptomatik des Patienten besserte sich daraufhin rasch, er konnte 4 Tage nach der Embolisation extubiert werden. Die sonographische Kontrolle nach 4 Monaten zeigte eine deutliche Regression des Tumors. Schlußfolgerung: Die interventionelle Coil-Okklusion hepatischer Hämangioendotheliome sollte bei symptomatischen Neugeborenen früh erwogen werden, da in dieser Altersgruppe über einen venösen Zugang und Passage über einen Ductus arteriosus die arterielle Punktion vermieden werden kann. Eventuelle Zuströme aus dem Pfortadergefäßsystem können in dieser Altersgruppe über die Nabelvene erreicht und therapiert werden.
    Type of Medium: Electronic Resource
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