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Dataset Information

Cushing syndrome in the McCune-Albright syndrome.


ABSTRACT:

Context

Cushing syndrome (CS) is a rare but potentially fatal feature of McCune-Albright syndrome (MAS). Optimal management, prognostic features, and long-term follow-up of this disorder have not been described.

Setting

The study was conducted at an academic tertiary care center.

Patients

A total of 112 patients participating in a natural history study at the National Institutes of Health (NIH) were evaluated, and 21 published cases were reviewed.

Interventions

Subjects received observation, medical management, or bilateral adrenalectomy.

Main outcome measures

We measured prevalence, prognostic factors, and natural history.

Results

The prevalence of CS among NIH patients was 7.1%. The median age at diagnosis was 3 months. Clinical features include

SUBMITTER: Brown RJ 

PROVIDER: S-EPMC2853983 | biostudies-literature | 2010 Apr

REPOSITORIES: biostudies-literature