Summary
A case of sudden and unexpected death of a boy with Down-Syndrome is reported. Death was due to acute excessive haemorrhage from the lungs. There were no cardiac anomalies. Histology revealed in addition to mediahyperplasia, intimaproliferation and occlusions, multiple foci of anomalous ectatic blood vessels predominantly in the lungs, some of which ruptured and bled into adjacent airways. Evidence of previous less severe bleeding episodes was present. Further investigation showed a familial occurrence of the blood vessel anomaly.
Zusammenfassung
Es wird über den plötzlichen und unerwarteten Tod eines mongoloiden Knaben berichtet. Die Todesursache war Verblutung aus der Lunge. Die Histologie zeigte an den kleineren Gefäßen von Lunge, Niere, Leber und Gehirn multifokale Mediahyperplasie, Intimaproliferation, Obliterationen und multiple Angiektasien. In den Lungen fanden sich zusätzliche frische Rupturen von angiektatischen Gefäßen mit Einbruch der Blutungen in benachbarte Luftwege, außerdem Residuen älterer Blutungen. Nachforschungen ergaben eine autopisch gesicherte familiäre Häufung von Gefäßmißbildungen.
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Ogbuihi, S., Zink, P. Tödliche Lungenblutung bei komplexer pulmonaler Angiodysplasie in einem Fall von Mongolismus. Z Rechtsmed 101, 255–263 (1988). https://doi.org/10.1007/BF00200231
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DOI: https://doi.org/10.1007/BF00200231
Key words
- Down-Syndrome
- Pulmonary blood vessel anomalies, Down-Syndrome
- sudden unexpected death, haemorrhage from dysplastic pulmonary blood vessels