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  • Title: Cushing's syndrome caused by nodular adrenal hyperplasia in children with McCune-Albright syndrome.
    Author: Kirk JM, Brain CE, Carson DJ, Hyde JC, Grant DB.
    Journal: J Pediatr; 1999 Jun; 134(6):789-92. PubMed ID: 10356155.
    Abstract:
    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.
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