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Prolonged remission of severe Cushing syndrome without adrenalectomy in an infant with McCune-Albright syndrome |
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| Authors: | Gillis David Rösler Ariel Hannon Tamara S Koplewitz Benjamin Z Hirsch Harry J |
| Affiliation: | a Department of Pediatrics, Pediatric Endocrine Unit, Hadassah-Hebrew University Medical Center, Jerusalem, Israel b Department of Endocrinology and Metabolism, Hadassah-Hebrew University Medical Center, Jerusalem, Israel c Department of Medical Imaging, Hadassah-Hebrew University Medical Center, Jerusalem, Israel d Department of Pediatrics, Shaare-Zedek Medical Center, Jerusalem, Israel e Department of Pediatrics, Division of Endocrinology, Children's Hospital of Pittsburgh, Pittsburgh, PA. |
| Abstract: | A 4 month-old girl presented with severe Cushing syndrome caused by McCune-Albright syndrome. After undergoing 19 months of pharmacologic suppression of cortisol production, she has been in clinical remission for more than 6 years. Adrenalectomy may be avoidable even in severe cases of Cushing syndrome associated with McCune-Albright syndrome. |
| Keywords: | ACTH, Adrenocorticotropin CRH, Corticotropin-releasing hormone c-AMP, Cyclic-adenosine monophosphate Gs-alpha, Stimulatory -alpha subunit of the signal-transducing guanosine nucleotide-binding protein MAS, McCune-Albright syndrome |
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