Unique ID issued by UMIN | UMIN000017322 |
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Receipt number | R000019733 |
Scientific Title | Clinical features of long-term survivors over 40 years old with Duchenne muscular dystrophy |
Date of disclosure of the study information | 2015/04/29 |
Last modified on | 2015/04/29 10:30:27 |
Clinical features of long-term survivors over 40 years old with Duchenne muscular dystrophy
Clinical features of long-term survivors over 40 years old with Duchenne muscular dystrophy
Clinical features of long-term survivors over 40 years old with Duchenne muscular dystrophy
Clinical features of long-term survivors over 40 years old with Duchenne muscular dystrophy
Japan |
Duchenne muscular dystrophy (DMD)
Neurology | Pediatrics |
Others
YES
To elucidate the clinical condition and treatment of patients with DMD over 40 years old.
Others
To explore the key to long survive of DMD patients
Method and result of gene analysis.
Muscle biopsy findings.
Age of loss of independent ambulation.
Status of cardiomyopathy ( EF of echocardiograph and plasma BNP).
Kinds of agents used for treatment for cardiomyopathy.
History of steroid therapy (used, not used, or now using).
History of spinal fusion operation (done, not done).
Central nervous symptom (mental retardation, autistic disorder, epilepsy, and so on).
Other matter worthy of special mention.
Observational
40 | years-old | <= |
Not applicable |
Male
DMD patients aged over 40 year of age at the time of the search on July 1, 2013. They are recruited based on the Muscular dystrophy wards database of 2012.
DMD patients under 40 years of age at the time of the database search in July 2013 are excluded.
118
1st name | |
Middle name | |
Last name | Toshio Saito |
National Hospital Organization Toneyama National Hospital
Division of Child Neurology, Department of Neurology
5-1-1 Toneyama, Toyonaka, Osaka, 560-8552 Japan
06-6853-2001
saitot@toneyama.go.jp
1st name | |
Middle name | |
Last name | Toshio Saito |
National Hospital Organization Toneyama National Hospital
Division of Child Neurology, Department of Neurology
5-1-1 Toneyama, Toyonaka, Osaka, 560-8552 Japan
06-6853-2001
saitot@toneyama.go.jp
National Hospital Organization
Ministry of Health, Labour and Welfare of Japan
National Center of Neurology and Psychiatry
NO
旭川医療センター Asahikawa Medical Center
八雲病院 Yakumo Hospital
青森病院 Aomori National Hospital
あきた病院 Akita National Hospital
西多賀病院 Nishitaga National Hospital
精神・神経医療研究センター National Center of Neurology and Psychiatry
東埼玉病院East Saitama National Hospital
下志津病院Shimoshizu National Hospital
箱根病院National Hakone Hospital
新潟病院Niigata National Hospital
医王病院Iou National Hospital
長良医療センターNagara Medical Center
鈴鹿病院Suzuka National Hospital
兵庫中央病院Hyogo-cyuo National Hospital
奈良医療センターNara Cedical Center
宇多野病院Utano Hospital
刀根山病院 Toneyama National Hospital
松江病院 Matsue Medical Center
広島西医療センター Hiroshima-Nishi Medical Center
徳島病院Tokushima National Hospital
南九州病院Minami Kyushu National Hospital
大牟田病院 Oomuta Hospital
長崎神経医療センター Nagasaki Kawatana Medical Center
熊本再春荘病院 Kumamoto Saishunso National Hospital
西別府病院 Nishibeppu National
宮崎東病院 Miyazaki Higashi Hospital
沖縄病院 Okinawa National Hospital
2015 | Year | 04 | Month | 29 | Day |
Partially published
Mean age at study time is 43.6(SD 3.0) years.
Mean age of loss of independent ambulation is 10.6 (SD1.5) years.
Mean age of introduction of artificial ventilation is 24.1 (SD 5.5) years.
Cases with NPPV and those with TIV are comparable.
Mean EF of echocardiograph is 45.0 (SD 15.4) %.
Twenty one cases are administered >=3 agents for cardiomyopathy, while 9 cases no agent.
Seventy three percent of patients preserved oral nutritional function.
Completed
2013 | Year | 07 | Month | 01 | Day |
2013 | Year | 08 | Month | 01 | Day |
2013 | Year | 12 | Month | 31 | Day |
2013 | Year | 12 | Month | 31 | Day |
2013 | Year | 12 | Month | 31 | Day |
2015 | Year | 06 | Month | 30 | Day |
Age in July 2013 (years)
Mean (SD) = 43.6(3.0), Range = 40.2-51.0
Number: (age>=40-41), 12; (>=41-43), 18; (>=43-46), 12; (>=46), 13
Age at loss of independent ambulation (years)
Mean (SD) = 10.6(1.5) , Range = 7.0-15.0
Number: (age=7 years), 1; (8), 1; (9), 9; (10), 17; (11), 13; (12), 11; (14), 2; (15), 1
Age at introduction of artificial ventilation (years)
Mean (SD) = 24.1(5.5) , Range = 15.0-40.0
Respiratory method
Number: (NPPV), 27; (TIV), 28
Cardioprotective Agent
Number: (Beta-blocker), 18; (ACEI), 29; (ARB), 4; (Diuretics), 26; (Antiarrhythmic agent), 11; (Digitalis), 18; (Pimobendan), 2
Number of agents for cardiomyopathy
Number: (Number of agents = 0), 9; (1), 13; (2), 12; (3), 8; ( 4), 13
Nutritional method
Number: (Oral nutritional supply), 40; (Oral nutritional supply + gastrostomy feeding), 1; (Gastrostomy feeding), 6; (Tube feeding), 8
2015 | Year | 04 | Month | 29 | Day |
2015 | Year | 04 | Month | 29 | Day |
Value
https://center6.umin.ac.jp/cgi-open-bin/ctr_e/ctr_view.cgi?recptno=R000019733
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