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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 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]