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DOI: 10.1055/s-2002-36065
Seltene familiäre Häufung solitärer Ösophagusleiomyome in Verbindung mit progressiver Nephropathie
Familial Leiomyoma of the Esophagus in Combination with NephropathyPublication History
Publication Date:
11 December 2002 (online)
![](https://www.thieme-connect.de/media/viszeralchirurgie/200206/lookinside/thumbnails/10.1055-s-2002-36065-1.jpg)
Zusammenfassung
Diffuse ösophageale Leiomyomatosen in Verbindung mit X-chromosomal vererbter progressiver Nephropathie (Alport-Syndrom) werden in der Literatur vereinzelt beschrieben. Dagegen stellen solitäre Ösophagusleiomyome in diesem Kontext eine Rarität dar. Wir berichten über das Auftreten von solitären Leiomyomen und progressiver Alport-Nephropathie bei 5 Mitgliedern einer Familie, wobei Leiomyome in 4 Generationen derselben Familie auftraten. Zwei Familienangehörige wurden in unserer Klinik operiert. Heredität und Therapiestrategien werden diskutiert.
Abstract
Diffuse esophageal leiomyomatosis associated with X-chromosomal Alport-like nephropathy has been reported by various authors. However, solitary esophageal leiomyomas in combination with Alport-Syndrome are a rare disorder. We report on patients from 4 consecutive generations of one family. Five patients presented with a combination of a solitary leiomyoma and progressive nephropathy. Two of them were operated on at our department.
Schlüsselwörter
Ösophagus - Leiomyom - Nephropathie - Alport-Syndrom - Koloninterposition
Key words
Esophageal leiomyoma - nephropathy - alport-syndrome - colonic interposition
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Prof. Dr. Johannes Scheele
Friedrich-Schiller-Universität Jena Klinik für Allgemeine und Viszerale Chirurgie
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