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  • 1
    Electronic Resource
    Electronic Resource
    Atypical Kawasaki disease with peripheral gangrene and myocardial infarction: Therapeutic implications (1995)
    Springer
    European journal of pediatrics 154 (1995), S. 830-834 
    Details
    ISSN: 1432-1076
    Keywords: Atypical Kawasaki disease ; Giant coronary aneurysm ; Myocardial infarction ; Peripheral gangrene ; Prostaglandin E1
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe a 2-month-old girl with atypical Kawasaki disease (KD) complicated by peripheral gangrene and myocardial infarction. Peripheral ischaemia leading to gangrene is a rare but serious complication of KD in infants younger than 7 months of age. Treatment has been targeted at reducing arterial inflammation, arteriospasm and thrombosis. We report the first patient with incomplete KD and peripheral ischaemia in whom therapy with prostaglandin E1 (PGE1) as vasodilating and antithrombotic agent appeared successful, restoring hand and foot perfusion without significant long-term sequelae. However, PGE1 could have supported development of myocardial infarction by shunting blood away from ischaemic areas distal to a giant coronary artery aneurysm with beginning thrombosis. Conclusion Atypical KD with peripheral gangrene appears to react favourably to treatment with PGE1, but needs careful monitoring to detect early signs of cardiac ischaemia.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01959792
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Defective activation of the alternative pathway of complement in patients with homozygous C2 deficiency: studies in two unrelated families (1991)
    Springer
    European journal of pediatrics 150 (1991), S. 647-651 
    Details
    ISSN: 1432-1076
    Keywords: C2 deficiency ; Alternative pathway of complement ; Bacterial infections
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Selective homozygous deficiency of the second component of complement, C2, with increased susceptibility to infection was detected in five children of two unrelated families. Because the haemolytic activity of the alternative complement pathway (AP) was in the low normal range, we evaluated the AP activation pattern. Serum levels of factor B measured immunochemically and the haemolytic function of factor B were low normal. Levels of C3d were not increased. Activation products of factor B were undetectable indicating the absence of in vivo activation of AP. Activation of C3 in vitro by activators of the AP (zymosan A and lipopolysaccaride) was profoundly deficient in homozygous C2 deficiency while heterozygous carriers exhibited intermediate values. There was no correlation between serum levels of factor B and in vitro C3 activation. We conclude that defective AP activation may contribute to increased susceptibility to bacterial infections in some patients with homozygous C2 deficiency.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02072626
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
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