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      Hypermobile Ehlers-Danlos syndrome (a.k.a. Ehlers-Danlos syndrome Type III and Ehlers-Danlos syndrome hypermobility type): Clinical description and natural history

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          Abstract

          The hypermobile type of Ehlers-Danlos syndrome (hEDS) is likely the most common hereditary disorder of connective tissue. It has been described largely in those with musculoskeletal complaints including joint hypermobility, joint subluxations/dislocations, as well as skin and soft tissue manifestations. Many patients report activity-related pain and some go on to have daily pain. Two undifferentiated syndromes have been used to describe these manifestations-joint hypermobility syndrome and hEDS. Both are clinical diagnoses in the absence of other causation. Current medical literature further complicates differentiation and describes multiple associated symptoms and disorders. The current EDS nosology combines these two entities into the hypermobile type of EDS. Herein, we review and summarize the literature as a better clinical description of this type of connective tissue disorder. © 2017 Wiley Periodicals, Inc.

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          School start times for adolescents.

          , , (2014)
          The American Academy of Pediatrics recognizes insufficient sleep in adolescents as an important public health issue that significantly affects the health and safety, as well as the academic success, of our nation's middle and high school students. Although a number of factors, including biological changes in sleep associated with puberty, lifestyle choices, and academic demands, negatively affect middle and high school students' ability to obtain sufficient sleep, the evidence strongly implicates earlier school start times (ie, before 8:30 am) as a key modifiable contributor to insufficient sleep, as well as circadian rhythm disruption, in this population. Furthermore, a substantial body of research has now demonstrated that delaying school start times is an effective countermeasure to chronic sleep loss and has a wide range of potential benefits to students with regard to physical and mental health, safety, and academic achievement. The American Academy of Pediatrics strongly supports the efforts of school districts to optimize sleep in students and urges high schools and middle schools to aim for start times that allow students the opportunity to achieve optimal levels of sleep (8.5-9.5 hours) and to improve physical (eg, reduced obesity risk) and mental (eg, lower rates of depression) health, safety (eg, drowsy driving crashes), academic performance, and quality of life. Copyright © 2014 by the American Academy of Pediatrics.
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            Ehlers-Danlos syndromes: Revised nosology, Villefranche, 1997

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              The effects of sleep deprivation on pain inhibition and spontaneous pain in women.

              Impaired central pain modulation is implicated in the pathophysiology of chronic pain. In this controlled experiment, we evaluated whether partial sleep loss altered endogenous pain inhibition and reports of spontaneous pain. Thirty-two healthy females were studied polysomnographically for 7 nights. On Nights 1-2 (Baseline), subjects slept undisturbed for 8 hours. After Night 2, subjects were randomized to Control (N = 12), Forced Awakening (FA, N = 10), or Restricted Sleep Opportunity (RSO, N = 10) conditions. Controls continued to sleep undisturbed. FA underwent 8 forced awakenings (one per hour) on Nights 3-5. RSO subjects were yoked to FA on total sleep time (TST), receiving partial sleep deprivation by delayed bedtime. On Night 6, both FA & RSO underwent 36 hours total sleep deprivation (TSD), followed by 11-hour recovery sleep (Night 7). Subjects completed twice-daily psychophysical assessments of mechanical pain thresholds and pain inhibition (Diffuse Noxious Inhibitory Controls), via use of a conditioning stimulus (i.e., cold pressor) paradigm. FA and RSO demonstrated 50% reductions in total sleep time and increases in nonpainful somatic symptoms during partial sleep deprivation. While sleep deprivation had no effect on pain thresholds, during partial sleep deprivation the FA group demonstrated a significant loss of pain inhibition and an increase in spontaneous pain; neither of the other 2 groups showed changes in pain inhibition or spontaneous pain during partial sleep deprivation. These data suggest that sleep continuity disturbance, but not simple sleep restriction, impairs endogenous pain-inhibitory function and increases spontaneous pain, supporting a possible pathophysiologic role of sleep disturbance in chronic pain.
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                Author and article information

                Journal
                American Journal of Medical Genetics Part C: Seminars in Medical Genetics
                Am. J. Med. Genet.
                Wiley
                15524868
                March 2017
                March 2017
                February 01 2017
                : 175
                : 1
                : 48-69
                Article
                10.1002/ajmg.c.31538
                28145611
                699ca227-df3e-421a-85cb-b26d979aca58
                © 2017

                http://doi.wiley.com/10.1002/tdm_license_1

                http://onlinelibrary.wiley.com/termsAndConditions

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