Long-term Observation in Patients with Duchenne Muscular Dystrophy with Early Introduction of a Standing Program Using Knee–ankle–foot Orthoses

  • Fujimoto Akiko
    Department of Physical Rehabilitation, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan
  • Mizuno Katsuhiro
    Department of Physical Rehabilitation, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan Department of Rehabilitation Medicine, Tokai University School of Medicine, Isehara, Japan
  • Iwata Yasuyuki
    Department of Physical Rehabilitation, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan Department of Rehabilitation, National Hakone Hospital, Odawara, Japan
  • Yajima Hiroyuki
    Department of Physical Rehabilitation, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan
  • Nishida Daisuke
    Department of Physical Rehabilitation, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan Department of Rehabilitation Medicine, Tokai University School of Medicine, Isehara, Japan Department of Rehabilitation Medicine, Keio University School of Medicine, Tokyo, Japan
  • Komaki Hirofumi
    Department of Child Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan
  • Ishiyama Akihiko
    Department of Child Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan
  • Mori-yoshimura Madoka
    Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan
  • Tachimori Hisateru
    Endowed Course for Health System Innovation, Keio University School of Medicine, Tokyo, Japan Department of Clinical Data Science, Clinical Research and Education Promotion Division, National Center of Neurology and Psychiatry, Tokyo, Japan
  • Kobayashi Yoko
    Department of Physical Rehabilitation, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan Department of Rehabilitation, National Hakone Hospital, Odawara, Japan

抄録

<p>Objectives: This study investigated the outcomes of the early introduction of a standing program for patients with Duchenne muscular dystrophy (DMD).</p><p>Methods: This was a retrospective observational study of 41 outpatients with DMD aged 15–20 years. We introduced the standing program using knee–ankle–foot orthoses (KAFO) to slow the progression of scoliosis when ankle dorsiflexion became less than 0° in the ambulatory period.</p><p>Results: Thirty-two patients with DMD were offered the standing program with KAFO; 12 continued the program until the age of 15 years (complete group) and 20 discontinued the program before the age of 15 years (incomplete group). The non-standing program group included 9 patients. The standing program with KAFO was significantly associated with the Cobb angle at the age of 15 years after adjustment for the duration of corticosteroid use and DMD mutation type (P=0.0004). At the age of 15 years, significant correlations were found between the ankle dorsiflexion range of motion (ROM) and non-ambulatory period (P=0.0010), non-ambulatory period and Cobb angle (P<0.0001), Cobb angle and percent predicted forced vital capacity (P=0.0004), and ankle dorsiflexion ROM and Cobb angle (P=0.0066). In the complete group, the age at ambulation loss (log-rank P=0.0015), scoliosis progression (log-rank P=0.0032), and pulmonary dysfunction (log-rank P=0.0006) were significantly higher than in the non-standing program group.</p><p>Conclusions: The early introduction of a standing program for DMD patients may prolong the ambulation period and slow the progression of scoliosis and pulmonary dysfunction.</p>

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