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Adrenocorticotropin Insensitivity Syndromes

Departments of Endocrinology (A.J.L.C.), St. Bartholomew’s and the Royal London School of Medicine and Dentistry, West Smithfield, London EC1A 7BE, United Kingdom; and Children’s Hospital (A.W.), Technical University Dresden, D-01307 Dresden, Germany

I. Introduction

II. Familial Glucocorticoid Deficiency

A. Clinical presentation

B. Investigation

C. Treatment

D. Pathogenesis

III. Triple A Syndrome

A. Clinical features

B. Treatment

C. Pathogenesis

D. Mapping of triple A syndrome gene

E. Candidate genes

IV. Acquired ACTH Resistance

V. Conclusions