Inpatients with facioscapulohumeral muscular dystrophy in specialized institutions in Japan from 1999 to 2013-Clinical condition changes and causes of death
We analyzed the registration data of inpatients with facioscapulohumeral muscular dystrophy (FSHD) receiving care at 27 specialized institutions for muscular dystrophy in Japan from 1999 to 2013 using data from October 1 of each year. The number of inpatients of each year ranged from 63 to 72 (67.1 ± 3.3) throughout the study period. Those aged over 50 years gradually increased during the study period, while the oldest inpatient was 82.8 years old. Most could not walk. The rate of respirator dependency increased from 21.0% in 1999 to 71.0% in 2013, while the rate of patients receiving oral nutrition was 98.4% in 1999 and then reduced to 75.4% in 2013. There were 36 death cases reported in the database, including 15 patients with respiratory failure and 4 with heart failure. Our findings indicate that FSHD patients in a severe condition are impacted by respiratory and nutritional problems and their prognosis for survival is related to respiratory failure.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2019 |
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| Erschienen: |
2019 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:59 |
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| Enthalten in: |
Rinsho shinkeigaku = Clinical neurology - 59(2019), 11 vom: 08. Nov., Seite 716-722 |
| Sprache: |
Japanisch |
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| Beteiligte Personen: |
Saito, Toshio [VerfasserIn] |
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| Links: |
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| Themen: |
Cause of death |
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| Anmerkungen: |
Date Completed 10.02.2020 Date Revised 10.02.2020 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.5692/clinicalneurol.cn-001229 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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NLM302610197 |
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| 245 | 1 | 0 | |a Inpatients with facioscapulohumeral muscular dystrophy in specialized institutions in Japan from 1999 to 2013-Clinical condition changes and causes of death |
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| 520 | |a We analyzed the registration data of inpatients with facioscapulohumeral muscular dystrophy (FSHD) receiving care at 27 specialized institutions for muscular dystrophy in Japan from 1999 to 2013 using data from October 1 of each year. The number of inpatients of each year ranged from 63 to 72 (67.1 ± 3.3) throughout the study period. Those aged over 50 years gradually increased during the study period, while the oldest inpatient was 82.8 years old. Most could not walk. The rate of respirator dependency increased from 21.0% in 1999 to 71.0% in 2013, while the rate of patients receiving oral nutrition was 98.4% in 1999 and then reduced to 75.4% in 2013. There were 36 death cases reported in the database, including 15 patients with respiratory failure and 4 with heart failure. Our findings indicate that FSHD patients in a severe condition are impacted by respiratory and nutritional problems and their prognosis for survival is related to respiratory failure | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a cause of death | |
| 650 | 4 | |a database of wards for inpatients with muscular dystrophy | |
| 650 | 4 | |a facioscapulohumeral muscular dystrophy (FSHD) | |
| 650 | 4 | |a nutritional support | |
| 650 | 4 | |a respiratory failure | |
| 700 | 1 | |a Kuru, Satoshi |e verfasserin |4 aut | |
| 700 | 1 | |a Takahashi, Toshiaki |e verfasserin |4 aut | |
| 700 | 1 | |a Suzuki, Mikiya |e verfasserin |4 aut | |
| 700 | 1 | |a Ogata, Katsuhisa |e verfasserin |4 aut | |
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