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学科主题临床医学
Presentations of Primary Hypersomnia in Chinese Children
Han, Fang1; Lin, Ling2; Li, Jing1; Aran, Adi1,2; Dong, Song X.1; Zhao, Long1; Li, Ming3; Li, Qian Y.; Yan, Han1; Wang, Jie S.1; Gao, Hui Y.1; Li, Mei1; Gao, Zhan C.1; Strohl, Kingman P.4,5; Mignot, Emmanuel2
关键词Narcolepsy Childhood Hypocretin Mslt hladbqb1*0602
刊名SLEEP
2011-06-01
34期:5页:627-632A
收录类别SCI
文章类型Article
WOS标题词Science & Technology
类目[WOS]Clinical Neurology ; Neurosciences
研究领域[WOS]Neurosciences & Neurology
关键词[WOS]NARCOLEPSY-CATAPLEXY ; CHILDHOOD NARCOLEPSY ; KOREAN PATIENTS ; HYPOCRETIN ; ONSET ; HLA ; DIAGNOSIS
英文摘要

Objective: To retrospectively describe childhood presentations of primary hypersomnia with an emphasis on narcolepsy-cataplexy in a Chinese population.

Methods: A total of 417 children (< 18 years old) successively presenting with complaints of hypersomnia without anatomic cause or sleep apnea risk were evaluated using the Stanford Sleep Inventory, human leukocyte antigen (HLA) DQB1*0602 typing, and MSLT recordings. CSF hypocretin-1 was measured or HLA positive with clear-cut cataplexy) was further examined for presentations prior to , around, or after puberty.

Results: Narcolepsy with (n = 361) or without (n = 17) cataplexy presented at an earlier age and with increased male predominance when compared to idiopathic hypersomnia (n = 39, P < 0.01). Nearly 70% of those with narcolepsy/hypocretin deficiency (n = 271) had disease onset before age 10 y, and 15% had onset before age 6, an unusually young age distribution. Onset was prior to puberty in 78% of cases. Clinical features were decreased and the number of sleep onset REM periods (SOREMPs) increased in frequency with age/puberty, but MSLT diagnosis criteria (MSL <= 8 min, >= 2 SOREMPs) were similarly positive across groups. Familial clustering was present in only 1.7% of probands.

Conclusion: In children presenting with a complaint of primary hypersomnia to a sleep clinic in China, 86% (361/417) meet criterial for narcolepsy with cataplexy. Puberty did not affect positivity on the MSLT as a diagnostic feature. Sleep paralysis was the only symptom that increased with increasing age. In addition, narcolepsy with cataplexy in our clinic population appeared to begin at a younger age than usually reported in other studies.

语种英语
WOS记录号WOS:000291145800013
项目编号81070069 ; GZ538 ; HL 047380 ; NIH-NS23724
资助机构NSFC ; Sino-German Center for Research Promotion ; VA Research Service ; NIH
引用统计
被引频次:23[WOS]   [WOS记录]     [WOS相关记录]
文献类型期刊论文
条目标识符http://ir.bjmu.edu.cn/handle/400002259/57705
专题北京大学第二临床医学院_呼吸科
作者单位1.Cleveland Louis Stokes VA Med Ctr, Cleveland, OH USA
2.Beijing Univ, Peoples Hosp, Dept Pulm Med, Beijing 100044, Peoples R China
3.Stanford Univ, Ctr Narcolepsy, Howard Hughes Med Inst, Palo Alto, CA 94304 USA
4.Beijing Union Med Coll Hosp, Beijing Union Med Coll, Dept Endocrinol, Beijing 100730, Peoples R China
5.Case Western Reserve Univ, Dept Med, Div Pulm Crit Care & Sleep Med, Cleveland, OH 44106 USA
推荐引用方式
GB/T 7714
Han, Fang,Lin, Ling,Li, Jing,et al. Presentations of Primary Hypersomnia in Chinese Children[J]. SLEEP,2011,34(5):627-632A.
APA Han, Fang.,Lin, Ling.,Li, Jing.,Aran, Adi.,Dong, Song X..,...&Mignot, Emmanuel.(2011).Presentations of Primary Hypersomnia in Chinese Children.SLEEP,34(5),627-632A.
MLA Han, Fang,et al."Presentations of Primary Hypersomnia in Chinese Children".SLEEP 34.5(2011):627-632A.
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