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Pediatric Radiology

, Volume 40, Issue 3, pp 371–371 | Cite as

Polycystic ovaries in leprechaunism

  • Denise Drohobyczer
  • Richard BellahEmail author
Clinical Image

Keywords

Polycystic Ovary Precocious Puberty Hyperandrogenism Pancreatic Islet Cell Left Ovary 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
A 2-month-old girl presented with increasing abdominal girth and failure to thrive. Pelvic US showed an enlarged right ovary containing multiple cysts (Fig. 1). The left ovary appeared the same. A slightly enlarged uterus with pubertal configuration and prominent endometrium is also shown (Fig. 2, arrows).
Fig. 1

Pelvic US shows enlarged right ovary with multiple cysts

Fig. 2

US shows enlarged uterus with prominent endometrium

Dr. William Donohue of the Hospital for Sick Children in Toronto first reported leprechaunism (also known as Donohue syndrome) in 1954. Physical features included growth retardation, lipoatrophy, acanthosis nigricans, hypertrichosis, abnormal facies, breast tissue hyperplasia, and enlarged genitalia. Endocrine dysfunction was suggested by abnormal glucose levels and profound insulin resistance. Autopsies revealed pancreatic islet cell hyperplasia and cystic ovaries [1].

Leprechaunism is now classified under a group of endocrinopathies resulting in HAIR-AN syndrome (Hyperandrogenism, Insulin Resistance, Acanthosis Nigricans). Hyperinsulinemia stimulates hyperandrogenism, which manifests as precocious puberty in infancy [2]. While the clinical literature has briefly mentioned large ovaries in association with sexual precocity, the imaging appearance has not been previously shown.

References

  1. 1.
    Barbieri RL, Ryan KJ (1983) Hyperandrogenism, insulin resistance, and acanthosis nigricans syndrome: a common endocrinopathy with distinct pathophysiologic features. Am J Obstet Gynecol 147:90–101PubMedGoogle Scholar
  2. 2.
    Elsas LJ, Endo F, Strumlauf E et al (1985) Leprechaunism: an inherited defect in a high-affinity insulin receptor. Am J Hum Genet 37:73–88PubMedGoogle Scholar

Copyright information

© Springer-Verlag 2009

Authors and Affiliations

  1. 1.Department of RadiologyThe Children’s Hospital of PhiladelphiaPhiladelphiaUSA

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