Prolonged remission of severe Cushing syndrome without adrenalectomy in an infant with McCune-Albright syndrome.
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.
Gillis
David
D
Department of Pediatrics, Pediatric Endocrine Unit, Hadassah-Hebrew University Medical Center, Jerusalem, Israel. dgillis@hadassah.org.il
Rösler
Ariel
A
Hannon
Tamara S
TS
Koplewitz
Benjamin Z
BZ
Hirsch
Harry J
HJ
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
D018663:Adrenergic Agents; D004791:Enzyme Inhibitors; D000616:Aminoglutethimide; D008797:Metyrapone