0594 A Prospective Study of Ambulatory Blood Pressure And Heart Rate In Pediatric Narcolepsy With Cataplexy.

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      Introduction Narcolepsy with cataplexy (NC) is a unique disease associated with hypocretin deficiency in the lateral hypothalamus. The neural connections from this region connect to autonomic centers in the brain. We hypothesized that heart rate and blood pressure differ between pediatric NC patients and control group. Methods Values and circadian rhythmicity of 24-hour ambulatory blood pressure (ABP) and heart rate (HR) were compared among patients with NC before and after methylphenidate treatment (18 mg) and age-sex-BMI matched healthy controls. 50 patients (40 males, 10 females; mean age 10.4 ± 3.5 years (M+/-SD, range 5-17years) with irrepressible sleepiness for more than three months associated with cataplexy and human leukocyte antigen (HLA)DQB1*06:02 positive. 100 normal controls were a reference group. Results drug-free patients with NC had a lower daytime systolic blood pressure (SBP) and higher HR across whole day but comparable daytime diastolic blood pressure (DBP) than healthy controls. Methylphenidate treatment medication in NC subjects increases daytime SBP, DBP, and HR back to the normal range. Patients with NC before and after methylphenidate treatment had a higher rate of non-dipping in SBP than healthy controls. Conclusion NC patients with hypocretin deficiency have impaired cardiovascular function when compared with normal controls. Methylphenidate increases the values of BP and HR in NC patients. Support (If Any) National Foundation of Science of China 81420108002,81670087 (to F.H.) [ABSTRACT FROM AUTHOR]
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