터너 증후군은 증후군은 X 염색체의 완전 혹은 불완전 결손으로 인한 저신장증과 성선무발생증을 특징으로 하는 여성에서 가장 흔한 성염색체 질환이다. 그 이외에도 특이적인 근골격계 기형, 심혈관계 질환, 신장기형, 갑상선 기능저하증, 내당능장애, 염증성 장 질환 등 전신적으로 다양한 임상 양상을 보일 수 있다. 드물지만 뇌하수체질환이 동반되기도 하는데, 갑상선 기능저하증과 성선 기능저하증으로 인해 이차적으로 뇌하수체 비후증이나 뇌하수체 선종이 발생한 경우가 있고, 뇌하수체 공터키안 혹은 뇌하수체기능저하증이 동반된 경우가 몇 차례 보고된 적 있다. 저자들은 터너 증후군 환자에서 원인 미상의 일시적인 뇌하수체기능저하증이 특별한 치료 없이 회복된 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.
터너 증후군은 증후군은 X 염색체의 완전 혹은 불완전 결손으로 인한 저신장증과 성선무발생증을 특징으로 하는 여성에서 가장 흔한 성염색체 질환이다. 그 이외에도 특이적인 근골격계 기형, 심혈관계 질환, 신장기형, 갑상선 기능저하증, 내당능장애, 염증성 장 질환 등 전신적으로 다양한 임상 양상을 보일 수 있다. 드물지만 뇌하수체질환이 동반되기도 하는데, 갑상선 기능저하증과 성선 기능저하증으로 인해 이차적으로 뇌하수체 비후증이나 뇌하수체 선종이 발생한 경우가 있고, 뇌하수체 공터키안 혹은 뇌하수체기능저하증이 동반된 경우가 몇 차례 보고된 적 있다. 저자들은 터너 증후군 환자에서 원인 미상의 일시적인 뇌하수체기능저하증이 특별한 치료 없이 회복된 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.
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 ...
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.
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.
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