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10.5664/jcsm.6500

http://scihub22266oqcxt.onion/10.5664/jcsm.6500
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C5337592!5337592 !28095967
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suck abstract from ncbi


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pmid28095967
      J+Clin+Sleep+Med 2017 ; 13 (3 ): 441-453
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  • Intravenous Immunoglobulin Therapy in Pediatric Narcolepsy: A Nonrandomized, Open-Label, Controlled, Longitudinal Observational Study #MMPMID28095967
  • Lecendreux M ; Berthier J ; Corny J ; Bourdon O ; Dossier C ; Delclaux C
  • J Clin Sleep Med 2017[Mar]; 13 (3 ): 441-453 PMID28095967 show ga
  • STUDY OBJECTIVES: Previous case reports of intravenous immunoglobulins (IVIg) in pediatric narcolepsy have shown contradictory results. METHODS: This was a nonrandomized, open-label, controlled, longitudinal observational study of IVIg use in pediatric narcolepsy with retrospective data collection from medical files obtained from a single pediatric national reference center for the treatment of narcolepsy in France. Of 56 consecutively referred patients with narcolepsy, 24 received IVIg (3 infusions administered at 1-mo intervals) in addition to standard care (psychostimulants and/or anticataplectic agents), and 32 continued on standard care alone (controls). RESULTS: For two patients in each group, medical files were unavailable. Of the 22 IVIg patients, all had cerebrospinal fluid (CSF) hypocretin ? 110 pg/mL and were HLA-DQB1*06:02 positive. Of the 30 control patients, 29 were HLA-DQB1*06:02 positive and of those with available CSF measurements, all 12 had hypocretin ? 110 pg/mL. Compared with control patients, IVIg patients had shorter disease duration, shorter latency to sleep onset, and more had received H1N1 vaccination. Mean (standard deviation) follow-up length was 2.4 (1.1) y in the IVIg group and 3.9 (1.7) y in controls. In multivariate-adjusted linear mixed-effects analyses of change from baseline in Ullanlinna Narcolepsy Scale (UNS) scores, high baseline UNS, but not IVIg treatment, was associated with a reduction in narcolepsy symptoms. On time-to-event analysis, among patients with high baseline UNS scores, control patients achieved a UNS score < 14 (indicating remission) less rapidly than IVIg patients (adjusted hazard ratio 0.18; 95% confidence interval: 95% confidence interval: 0.03, 0.95; p = 0.043). Shorter or longer disease duration did not influence treatment response in any analysis. CONCLUSIONS: Overall, narcolepsy symptoms were not significantly reduced by IVIg. However, in patients with high baseline symptoms, a subset of IVIg-treated patients achieved remission more rapidly than control patients. COMMENTARY: A commentary on this article appears in this issue on page 363.
  • |Child [MESH]
  • |Female [MESH]
  • |France [MESH]
  • |Humans [MESH]
  • |Immunoglobulins, Intravenous/*therapeutic use [MESH]
  • |Longitudinal Studies [MESH]
  • |Male [MESH]
  • |Narcolepsy/*drug therapy [MESH]
  • |Retrospective Studies [MESH]


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