NHO Toneyama National Hospital

Japan

NHO Toneyama National Hospital

Japan
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Takanoha S.,NCNP | Kuroda K.,National Hospital Organization NHO | Kon K.,NHO Aomori National Hospital | Mamiya S.,Akita | And 18 more authors.
Brain and Development | Year: 2012

Although muscular dystrophy patients often have feeding difficulty and need long-term enteral nutrition, only a few reports have described gastrostomy feeding in these patients. This study was designed to evaluate the efficacy and tolerance of gastrostomy feeding in patients with muscular dystrophy. We performed a retrospective, multicenter study on 144 patients with muscular dystrophy who received gastrostomy feeding between 2007 and 2009 in 25 neuromuscular centers in Japan. There were 77 Duchenne muscular dystrophy (median age at gastrostomy placement 26. years, range 13-47. years), 40 myotonic dystrophy (median age 54.5. years, range 13-70. years), 11 Fukuyama congenital muscular dystrophy (median age 22. years, range 13-29. years), 5 limb girdle muscular dystrophy (median age 62. years, range 43-78. years), and 5 facioscapulohumeral muscular dystrophy (median age 52. years, range 28-67. years) patients. Many benefits including amelioration of malnutrition, swallowing difficulty and respiratory status were observed after the introduction of gastrostomy feeding. Especially in patients with Duchenne muscular dystrophy, mean body weight significantly increased after gastrostomy placement. Although most complications, which are commonly observed in other populations, were tolerable, respiratory failure and peritonitis were important concerns. These findings suggest that gastrostomy placement at an appropriate time is advisable in patients with muscular dystrophy. © 2011 The Japanese Society of Child Neurology.


Saito T.,NHO Toneyama National Hospital | Tatara K.,Tokushima Bunri University
IRYO - Japanese Journal of National Medical Services | Year: 2013

The network system for trouble information of long term used artificial ventilator has been operated since 2009. Members of the system are risk managers and medical engineers of 26 institutes belong to the National Hospital Organization and the National Center of Neurology and Psychiatry, specialize in treatment of muscular dystrophy patients. The system works for speedy sharing information forproblem about long term used artificial ventilator. As the means of information transmission, e-mail was used effectively. Information about problem of ventilation sent to network office from member of network was transmitted to other members immediately by coodinator. Meanwhile, the information was sent to dealer of ventilation for confirmation of problem. If resolution for problem was found, that was also transmitted without delay. Through this network, some cases of problem were resolved quickly. The network system is effective for safety provision of artificial ventilation.


Nozaki S.,Health Science University | Kawai M.,NHO Higashisai tama National Hospital | Kimura T.,NHO Dohoku National Hospital | Adachi K.,NHO Tokushima National Hospital | And 5 more authors.
IRYO - Japanese Journal of National Medical Services | Year: 2011

The objective of this study are to evaluate whether simple oral phase exercise improves oral phase function and whether the exercise with a hot pack is useful in patients with myotonic muscular dystrophy type 1 (DM 1). The subjects were 15 DMlpatients (51 + 11 yo) who were not receiving respiratory care during the daytime and could take food orally by themselves, and have myotonia in hands or the tongue clinically,and without a history of a jaw dislocation. The study design consisted of a short-term crossover study, and long term open study. In the crossover study,the patients were divided into two groups.In group 1,7 patients (51±15 yo) performed the following : oral phase exercise with a hot pack (HP(+)) first, pause, and then oral phase exercise without a hot pack (HP(-)), for every 2 weeks.In group 11,8 patients (51 ±9 yo) performed the following: HP(~) first, pause,and then HP(+), for every 2 weeks. The oral phase exercise consisted of therapist-assisted exercise (5 times a week) and self-training (before each meal every day). The therapist-assisted exercise consisted of mouth opening and closing (lOtimes a day) with or without a hot pack.The self-training consisted of mouth opening and closing to the maximum degree by oneself (10 times a day).In the long-term open study after the short-term crossover study, 12 patients (52±12yo) performed therapist-assisted training (2 times a week) with a hot pack and self-training for 3 months.Outcomes were evaluated by measuring mouth width when smiling and puckering the lip, the distance between the top incisors and the bottom incisors at the maximum degree of mouth opening, and the greatest occlusal force. In the long-term open study,mouth width when smiling significantly increased after3 months of exercise compared with that at the start (p= 0.02). In the short-term crossover study, there was no significant difference in the outcomes between HP(+) and HP (-). Three months of oral phase exercise in DM 1 was effective for improving the oral movement.

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