Three-Quarters Adrenalectomy for Infantile-Onset Cushing Syndrome due to Bilateral Adrenal Hyperplasia in McCune-Albright Syndrome

Hormone Research in Pædiatrics
Tomoyo ItonagaKenji Ihara

Abstract

Bilateral adrenalectomy is performed in cases with infantile-onset Cushing syndrome due to bilateral adrenal hyperplasia in McCune-Albright syndrome (MAS) because severe Cushing syndrome with heart failure and liver dysfunction can have a lethal outcome. This procedure can completely ameliorate hypercortisolism, although lifetime steroid replacement therapy and steps to prevent adrenal crisis are necessary. Recently, the efficacy of unilateral adrenalectomy has been reported in adult cases of bilateral macronodular adrenal hyperplasia, but there is no consensus regarding the appropriate surgical treatment for bilateral adrenal hyperplasia in MAS. A 6-month-old girl presented with café-au-lait spots, short stature, central obesity, a moon face, and hypertension. Endocrinological tests and imaging studies led to the diagnosis of ACTH-independent Cushing syndrome due to bilateral adrenal hyperplasia induced by MAS. "Three-quarters adrenalectomy", namely right-sided total adrenalectomy and left-sided half adrenalectomy, was carried out. An activating mutation of the GNAS1 gene (p.Arg201Cys) was identified in the adrenal tissues. Since the operation, our patient has been in a state of clinical remission for more than 2 years. Our or...Continue Reading

References

Dec 12, 1991·The New England Journal of Medicine·L S WeinsteinA M Spiegel
Dec 7, 2000·Endocrinology·L BreaultN Gallo-Payet
Jun 23, 2006·Journal of Pediatric Endocrinology & Metabolism : JPEM·Thomas M K Völkl, Helmuth G Dörr
Jan 25, 2008·World Journal of Surgery·Maurizio IacoboneGennaro Favia
Feb 17, 2010·The Journal of Clinical Endocrinology and Metabolism·Rebecca J BrownMichael T Collins
Mar 10, 2011·Acta Physiologica·P A InselA C Zambon
Jun 1, 2012·Orphanet Journal of Rare Diseases·Michael T CollinsErica Eugster
Jul 30, 2015·The Journal of Clinical Endocrinology and Metabolism·Lynnette K NiemanUNKNOWN Endocrine Society
Oct 10, 2015·The Journal of Clinical Endocrinology and Metabolism·Emmanuelle DebillonOlivier Chabre

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Citations

Jul 31, 2019·Hormone Research in Pædiatrics·Prapai DejkhamronVorasuk Shotelersuk

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