Related Experiment Video
Updated: Sep 15, 2025
06:08
A Novel Method: Super-selective Adrenal Venous Sampling
Published on: September 15, 2017
23.6K
Unilateral Adrenalectomy for Pediatric Cyclical Cushing Syndrome with Novel PRKAR1A Variant Associated Carney Complex
Chris J Zhang1,2, Robert Stein1,3,4, Maha Saleh1,3,5
1Schulich School of Medicine and Dentistry, Western University, London, ON, Canada N6A 5W9.
JCEM Case Reports
|July 14, 2025
Abstract:
Primary pigmented nodular adrenocortical disease is a rare cause of Cushing syndrome accounting for less than 1% of cases. We present a 9-year-old boy who presented at age 4 with cyclical Cushing syndrome and was eventually diagnosed with a novel, previously unreported, unpublished variant in PRKAR1A associated with Carney complex. He was treated with unilateral left adrenalectomy. At 1-year follow-up, he continues to be in remission of his symptoms of Cushing syndrome.
