일시적인 뇌하수체기능저하증을 동반한 터너 증후군 1예

Abstract

Turner's syndrome is characterized by short stature and gonadal dysgenesis, and it is often associated with various systemic manifestations, such as cardiovascular, renal, thyroidal, gastrointestinal, and musculoskeletal disorders. Though very rare, it can also be accompanied by hypopituitarism. It is important to give a meticulous medical attention to short females with gonadal dysgenesis so that neither disease is neglected or gets delayed diagnosis. In this case, Turner's syndrome and hypopituitarism were diagnosed almost simultaneously, but hypopiuitarism was transient, and the normal pituitary function was recovered spontaneously without any treatment. Initial sella MRI showed mild congenital hypoplastic hypopituitarism, and combined pituitary function test was compatible with hypopituitarism, but after 5 years, though growth hormone deficiency was still present, otherwise normal pituitary function was noted without any change in MRI. Herein, we are reporting a case of Turner's syndrome with transient idiopathic hypopituitarism with the review of literature.