Decline in activities of daily living as a manifestation of secondary adrenal insufficiency in a patient with severe motor and intellectual disabilities.

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  • 活動性低下の要因として二次性副腎皮質機能低下症が疑われた重症心身障害者の1例
  • 症例報告 活動性低下の要因として二次性副腎皮質機能低下症が疑われた重症心身障害者の1例
  • ショウレイ ホウコク カツドウセイ テイカ ノ ヨウイン ト シテ ニジセイ フクジン ヒシツ キノウ テイカショウ ガ ウタガワレタ ジュウショウ シンシン ショウガイシャ ノ 1レイ

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Abstract

This report concerns a 51-year-old male with severe motor and intellectual disabilities who presented with a decline in activities of daily living due to secondary adrenal insufficiency and was successfully treated with hydrocortisone. He was unable to crawl on his hands and legs or move with a walker for approximately 10 days. He also exhibited painful behavior in his lower limbs. Physical, radiologic and laboratory examinations did not reveal fractures, infections, or rheumatic diseases. The basal serum cortisol and plasma ACTH levels and the peak serum cortisol level following the rapid ACTH test confirmed that adrenal cortex function was preserved; however, adrenal insufficiency could not be excluded. The results of the CRH test and other hypopituitary hormone levels suggested a partially isolated ACTH deficiency. Soon after the administration of hydrocortisone, he was able to ambulate. Thorough examinations are necessary in patients with symptoms suggestive of hypoadrenalism because dynamic tests may miss mild secondary adrenal insufficiency and hormone replacement therapy can result in compete resolution of symptoms. In addition, adrenal insufficiency should be considered when patients present with musculoskeletal symptoms of unknown etiology. Even in patients with severe motor and intellectual disabilities, it is very important to consider diseases in addition to adrenal insufficiency that can be examined and treated noninvasively.

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