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Inpatients with facioscapulohumeral muscular dystrophy in specialized institutions in Japan from 1999 to 2013—Clinical condition changes and causes of death
- Source :
- Rinsho Shinkeigaku. 59:716-722
- Publication Year :
- 2019
- Publisher :
- Societas Neurologica Japonica, 2019.
-
Abstract
- 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.
- Subjects :
- Adult
Male
Pediatrics
medicine.medical_specialty
Time Factors
Hospitals, Special
Severity of Illness Index
03 medical and health sciences
0302 clinical medicine
Japan
Cause of Death
medicine
Humans
Facioscapulohumeral muscular dystrophy
Mobility Limitation
Respiratory system
Muscular dystrophy
Aged
Heart Failure
Inpatients
Ventilators, Mechanical
Nutritional Support
business.industry
Age Factors
Middle Aged
medicine.disease
Muscular Dystrophy, Facioscapulohumeral
Hospitalization
Respiratory failure
Heart failure
Female
Neurology (clinical)
Respiratory Insufficiency
business
030217 neurology & neurosurgery
Subjects
Details
- ISSN :
- 18820654 and 0009918X
- Volume :
- 59
- Database :
- OpenAIRE
- Journal :
- Rinsho Shinkeigaku
- Accession number :
- edsair.doi.dedup.....9d56961bf5d4e5b5556377eda49feaca