Cushing's syndrome caused by nodular adrenal hyperplasia in children with McCune-Albright syndrome.
McCune-Albright syndrome consists of fibrous dysplasia of bone, café-au-lait skin pigmentation, and endocrine dysfunction (usually precocious puberty). Other endocrine abnormalities occur in a minority of patients, and of these, Cushing's syndrome is the least often recognized. We present 5 children (4 girls) with features of McCune-Albright syndrome who had Cushing's syndrome in the infantile period (<6 months). In 2 children spontaneous resolution occurred, but the remaining 3 required bilateral adrenalectomy. In addition, all 4 girls have experienced precocious puberty, and 3 children demonstrated radiologic evidence of nephrocalcinosis. Understanding of the underlying defect causing McCune-Albright syndrome emphasizes the importance of searching for other endocrine dysfunction in these children.
Kirk
J M
JM
Hospital for Sick Children, London, United Kingdom; Queen's University of Belfast, Belfast, United Kingdom.
Brain
C E
CE
Carson
D J
DJ
Hyde
J C
JC
Grant
D B
DB
D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000150:complications* / Q000601:surgery; D000315:Adrenalectomy; D019080:Cafe-au-Lait Spots / Q000097:blood / Q000473:pathology; D002648:Child; D003480:Cushing Syndrome / Q000209:etiology*; D005260:Female; D005357:Fibrous Dysplasia of Bone / Q000097:blood / Q000473:pathology; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011629:Puberty, Precocious / Q000097:blood / Q000473:pathology; D013577:Syndrome
D006854:Hydrocortisone