Reversible severe ovarian enlargement in an infant with significant insulin resistance

Lai Lillian, MD; Mikhchi Amir, MD; Ryabets-Lienhard Anna, DO; Geffner Mitchell E, MD; Cheung Clement, MD, PhD; Giuffre Danielle, MD

Radiology Case Reports (Jul 2021)

Reversible severe ovarian enlargement in an infant with significant insulin resistance

Lai Lillian, MD,
Mikhchi Amir, MD,
Ryabets-Lienhard Anna, DO,
Geffner Mitchell E, MD,
Cheung Clement, MD, PhD,
Giuffre Danielle, MD

Affiliations

Lai Lillian, MD: Department of Radiology, Children's Hospital Los Angeles and University of Southern California, Keck School of Medicine, 4650 Sunset Blvd, Mailstop #81, Los Angeles, CA, USA; Corresponding author.
Mikhchi Amir, MD: Department of Radiology, Children's Hospital Los Angeles and University of Southern California, Keck School of Medicine, 4650 Sunset Blvd, Mailstop #81, Los Angeles, CA, USA
Ryabets-Lienhard Anna, DO: Division of Endocrinology, Children's Hospital Los Angeles and University of Southern California, Keck School of Medicine, Los Angeles, CA, USA; The Saban Research Institute, Children's Hospital Los Angeles and University of Southern California, Keck School of Medicine, Los Angeles, CA, USA
Geffner Mitchell E, MD: Division of Endocrinology, Children's Hospital Los Angeles and University of Southern California, Keck School of Medicine, Los Angeles, CA, USA; The Saban Research Institute, Children's Hospital Los Angeles and University of Southern California, Keck School of Medicine, Los Angeles, CA, USA
Cheung Clement, MD, PhD: Division of Endocrinology, Children's Hospital Los Angeles and University of Southern California, Keck School of Medicine, Los Angeles, CA, USA
Giuffre Danielle, MD: Division of Endocrinology, Children's Hospital Los Angeles and University of Southern California, Keck School of Medicine, Los Angeles, CA, USA

Journal volume & issue: Vol. 16, no. 7
pp. 1760 – 1765

Abstract

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The extent, severity, and radiological findings of ovarian growth in infants with genetic syndromes of insulin resistance have not been fully described. We report a rare case of reversible massive ovarian enlargement in a female infant with a congenital insulin resistance syndrome, likely Rabson-Mendenhall syndrome given the less clinically severe course. The patient presented with neonatal diabetes with hyperinsulinemia and hyperglycemia due to congenital insulin resistance. She developed increasing severe bilateral ovarian enlargement which peaked at 4 months of age, followed by gradual decrease in size of the ovaries following treatment with insulin-sensitizing drugs and improved hyperinsulinemia. The ovarian enlargement is postulated to be secondary to the trophic effects of insulin acting in a gonadotropin-independent mechanism. Hyperinsulinemia in congenital insulin resistance can also result in hypertrophy of other organs. Understanding the pathophysiology behind massive ovarian enlargement in the setting of congenital insulin resistance syndromes can help guide appropriate therapy.

Published in Radiology Case Reports

ISSN: 1930-0433 (Online)
Publisher: Elsevier
Country of publisher: United States
LCC subjects: Medicine: Medicine (General): Medical physics. Medical radiology. Nuclear medicine
Website: https://www.sciencedirect.com/journal/radiology-case-reports

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