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Hämolytisch-urämisches Syndrom (Gasser)

Bericht über zwei Kinder mit ungewöhnlichem Verlauf

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Zusammenfassung

Es wird über zwei Kinder mit hämolytisch-urämischem Syndrom (Gasser) berichtet. Fall 1 war durch einen ungewöhnlichen schubweisen Verlauf gekennzeichnet. Im letzten Schub zeigte der Gerinnungsstatus eine Verbrauchscoagulopathie an. Die Therapie mit Corticoiden und zuletzt mit Heparin war erfolglos. Pathologisch-anatomisch fanden sich Fibringerinnsel in den Glomerulumcapillaren beider Nieren und eine in mehreren Schüben abgelaufene Herdglomerulitis. Bei Fall 2 entwickelte sich — möglicherweise infolge meningealer Thrombenbildung — ein Hydrocephalus hyporesorptivus. Die derzeitigen Vorstellungen über die Pathogenese des Krankheitsbildes und die therapeutischen Möglichkeiten werden dargelegt.

Summary

This is a report on two children with the hemolytic-uremic syndrome (Gasser). Case 1 had an unusual course with recurrent exacerbations. During the last episode coagulation studies indicated intravascular coagulation. Therapy with corticosteroids and heparin was without success. The post mortem examination revealed fibrin clots in the glomerular capillaries of the kidneys and a focal glomerulitis which had progressed in several stages. Case 2 developed hyporesorptive hydrocephalus — possibly due to meningeal fibrin clots. The current concepts of pathogenesis and therapy of the hemolytic-uremic syndrome are discussed.

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Authors and Affiliations

  1. Universitäts-Kinderklinik Freiburg i. Br., Germany

    H. Wehinger, H. U. Zollinger, W. Schenck & W. Künzer

  2. Ludwig Aschoff-Haus, Pathologisches Institut der Universität Freiburg, Germany

    H. Wehinger, H. U. Zollinger, W. Schenck & W. Künzer

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  1. H. Wehinger
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  2. H. U. Zollinger
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  3. W. Schenck
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  4. W. Künzer
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Wehinger, H., Zollinger, H.U., Schenck, W. et al. Hämolytisch-urämisches Syndrom (Gasser). Klin Wochenschr 46, 874–881 (1968). https://doi.org/10.1007/BF01746247

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