title: Prolonged remission of severe Cushing syndrome without adrenalectomy in an infant with McCune-Albright syndrome.
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.
authors.affiliation: Department of Pediatrics, Pediatric Endocrine Unit, Hadassah-Hebrew University Medical Center, Jerusalem, Israel. dgillis@hadassah.org.il
authors.forename: David
authors.initials: D
authors.lastname: Gillis
authors.forename: Ariel
authors.initials: A
authors.lastname: Rösler
authors.forename: Tamara S
authors.initials: TS
authors.lastname: Hannon
authors.forename: Benjamin Z
authors.initials: BZ
authors.lastname: Koplewitz
authors.forename: Harry J
authors.initials: HJ
authors.lastname: Hirsch
mesh_terms: D000315:Adrenalectomy; D018663:Adrenergic Agents / Q000627:therapeutic use*; D000616:Aminoglutethimide / Q000627:therapeutic use*; D002648:Child; D003480:Cushing Syndrome / Q000188:drug therapy* / Q000209:etiology*; D004791:Enzyme Inhibitors / Q000627:therapeutic use*; D005260:Female; D005359:Fibrous Dysplasia, Polyostotic / Q000150:complications*; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008797:Metyrapone / Q000627:therapeutic use*; D012074:Remission Induction; D012720:Severity of Illness Index; D013997:Time Factors
chemical_list: D018663:Adrenergic Agents; D004791:Enzyme Inhibitors; D000616:Aminoglutethimide; D008797:Metyrapone