著者
Akiko Fujimoto Katsuhiro Mizuno Yasuyuki Iwata Hiroyuki Yajima Daisuke Nishida Hirofumi Komaki Akihiko Ishiyama Madoka Mori-yoshimura Hisateru Tachimori Yoko Kobayashi
出版者
The Japanese Association of Rehabilitation Medicine
雑誌
Progress in Rehabilitation Medicine (ISSN:24321354)
巻号頁・発行日
vol.8, pp.20230038, 2023 (Released:2023-10-28)
参考文献数
50

Objectives: This study investigated the outcomes of the early introduction of a standing program for patients with Duchenne muscular dystrophy (DMD).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.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.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.
著者
Keisuke Yorimoto Yosuke Ariake Takako Saotome Madoka Mori-Yoshimura Tadashi Tsukamoto Yuji Takahashi Yoko Kobayashi
出版者
The Japanese Association of Rehabilitation Medicine
雑誌
Progress in Rehabilitation Medicine (ISSN:24321354)
巻号頁・発行日
vol.5, pp.20200011, 2020 (Released:2020-05-26)
参考文献数
9
被引用文献数
2

Objective: The aim of this study was to validate the usefulness of the measurement of lung insufflation capacity (LIC) using the LIC TRAINER (LT) in patients with amyotrophic lateral sclerosis (ALS). Methods: This retrospective study was conducted in the rehabilitation departments of the Japanese National Center of Neurology and Psychiatry and involved 20 ALS patients who underwent respiratory therapy between April 1, 2014, and December 2017. The vital capacity (VC), maximum insufflation capacity (MIC), and LIC measurements at the start of respiratory therapy were extracted from the medical records, and patients were divided into three groups: group A, VC could not be measured; group B, VC could be measured, but MIC was less than VC; and group C, MIC was larger than VC. LIC could be measured in all groups. In group C, paired t-tests were used to analyze whether there was a significant difference in the volumes measured using different methods. Results: LIC was 950, 1863±595, and 2980±1176 ml in groups A (n=1), B (n=10), and C (n=9), respectively. In groups A and B, LIC could be measured in all patients, even when VC or MIC could not be measured. In group C, the measured LIC value was significantly greater than MIC (p=0.003). Conclusion: LIC could be successfully measured using the LT. By using the LT, it was feasible to conveniently perform LIC measurements, suggesting that it could be a useful device for performing respiratory therapy in ALS patients.