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Porto-systemic shunt – a rare cause of hyperandrogenism in children. Two case reports and review of literature

  • Mikołaj Danko ORCID logo EMAIL logo , Anna Malinowska , Elżbieta Moszczyńska , Joanna Pawłowska , Maria Szarras-Czapnik , Małgorzata Walewska-Wolf , Urszula Wątrobińska and Mieczysław Szalecki
Published/Copyright: July 11, 2020

Abstract

Objectives

The main cause of hyperandrogenism in children is congenital adrenal hyperplasia, adrenal and gonadal tumors, polycystic ovary syndrome (PCOs) and Cushing’s disease. In the last 20 years several descriptions of girls with hyperandrogenism and venous porto-systemic shunts appeared in literature.

Case presentation

First case is an eleven and a half-year-old girl, was admitted to Department of Endocrinology because of symptoms of hyperandrogenism. Laboratory tests revealed high serum testosterone, androstenedione, and dehydroepiandrosterone sulfate (DHEAS). The ammonia concentration was also increased. In the abdominal angio-CT scans persistent umbilical vein which connected portal and femoral vein was found. The second case was a seven-year-old boy with symptoms of precocious puberty. Blood tests also revealed high concentration of testosterone, androstenedione, DHEAS and ammonia. Imaging studies showed persistent ductus venosus.

Conclusion

Although pathophysiological relation is not clear, porto-systemic shunts should be considered as a cause of hyperandrogenism of unknown origin in children.


Corresponding author: Mikołaj Danko M.D, Department of Paediatric Endocrinology and Diabetology, The Children’s Memorial Health Institute, Warsaw, Poland; Department of Paediatrics, Nutrition and Metabolic Diseases, The Children’s Memorial Health Institute, Al. Dzieci Polskich 20, 04-730 Warsaw, Poland, Phone: +48 228151214, Fax: +48 228151212, E-mail:

Acknowledgment

The authors thank a lot to Mr Rafał Żegnałek and Ms Anna Małecka for their contribution to edit this manuscript.

  1. Research funding: None declared.

  2. Author contributions: Conceptualization, M.D., M.S., E.M. Data curation, M.D Formal analysis, M.D. M.S.,; Investigation, M.D. U.W., A.M.; Methodology, M.D.; Writing-original draft, M.D., M. W-F.; Writing-review & editing, M.S., M.S.-C., M. P.

  3. Competing interests: The authors have no conflicts of interest to declare.

  4. Informed consent: Informed consent was obtained from all individuals included in this study.

  5. Ethical approval: We state that the subject and his parents have given their written informed consent to publish their case.

References

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Received: 2020-03-17
Accepted: 2020-04-27
Published Online: 2020-07-11
Published in Print: 2020-08-27

© 2020 Walter de Gruyter GmbH, Berlin/Boston

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