A Sudden Death Due to Central Hypoventilation in A 3-Year-Old Boy with Idiopathic Hypothalamic Dysfunction
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- Kobayashi Hironori
- Endocrinology, Metabolism, and Genetics unit, Tokyo Metropolitan Kiyose Children's Hospital
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- Miyamoto Junko
- Endocrinology, Metabolism, and Genetics unit, Tokyo Metropolitan Kiyose Children's Hospital
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- Hasegawa Yukihiro
- Endocrinology, Metabolism, and Genetics unit, Tokyo Metropolitan Kiyose Children's Hospital
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抄録
Idiopathic hypothalamic dysfunction is a rare disorder associated with adipsia, obesity and other symptoms such as central hypoventilation without any hypothalamic structural lesion. We report the case of a 3-year-old boy who died suddenly due to central hypoventilation. His obesity index increased from 0% to 75% during the 7 months prior to 3 yr 0 mo. During this clinical course, adipsia, hyperthermia, sudoresis, mild central hypoventilation, blepharoptosia, a change of character, hypernatremia, and hypothalamic hypopituitarism were observed. No treatment was given for hypoventilation. He died from sudden respiratory arrest because of central hypoventilation at 3 yr 8 mo. MRI examination was performed twice at 3 yr 3 mo and 3 yr 4 mo and revealed neither specific lesion nor invisible pituitary stalk. At autopsy, local inflammation and gliosis of the hypothalamus were present. So far at least 14 patients with idiopathic hypothalamic dysfunction have been reported. Eight cases including ours had central hypoventilation. Six out of the eight had poor respiratory prognosis, and central hypoventilation in hypothalamic dysfunction could be potentially fatal. In conclusion the patients with hypothalamic dysfunction should be treated intensively if they are suspected of having central hypoventilation.<br>
収録刊行物
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- Clinical Pediatric Endocrinology
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Clinical Pediatric Endocrinology 12 (1), 7-11, 2003
日本小児内分泌学会
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詳細情報
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- CRID
- 1390282679460135296
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- NII論文ID
- 130004430928
- 110002984797
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- NII書誌ID
- AA11006467
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- DOI
- 10.1297/cpe.12.7
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- ISSN
- 13477358
- 09185739
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- 本文言語コード
- en
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- データソース種別
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- JaLC
- Crossref
- CiNii Articles
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- 抄録ライセンスフラグ
- 使用不可