국내 근육디스트로피 환자의 임상양상, 장애 및 사회경제학적 실태
Clinical Features, Disability and Socioeconomic Status of Patients With Muscular Dystrophy in Korea
3
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
Background: Since 2001, the Ministry of Health and Welfare in Korea has designated muscular dystrophy (MD) to be a rare and intractable disease, and has ensured that patients with this condition obtain support from the National Health Insurance Corporation for their medical expenditure. However, the health-related and socioeconomic status of Korean patients with MD has yet to be established. Methods: We selected 441 patients with MD who received medical services at 17 neuromuscular centers during 2005. The medical records of selected patients were analyzed, and the subtype of MD was classified by its clinical course and diagnostic tests. A total of 95 patients or their family members participated in this health-related and socioeconomic status survey. Results: Medical record analysis showed similar clinical and diagnostic characteristic data to those published previously in other countries: male predominance, being young at onset, and muscular weakness of the extremities as a predominant symptom in most patients. The diagnostic tests for MD were based on laboratory and electrophysiological studies. The most frequent form of MD among our cohort was Duchenne/Becker muscular dystrophy (42%). Our survey revealed the effect of the patients’ profound disability on their activities of daily living. One-half of the patients were dissatisfied with the medical expenditure support service that was made available to them, and most patients suffered from a financial burden. The most important medical services to be developed in the future are expansion of the public health service or development of a rehabilitation hospital. Conclusions: This is the first multicenter-based epidemiologic study on the health-related and socioeconomic status of patients with MD in Korea. The findings indicate that medical coverage and public health service are currently inadequate and hence should be expanded in the future.
Abstract
Background: Since 2001, the Ministry of Health and Welfare in Korea has designated muscular dystrophy (MD) to be a rare and intractable disease, and has ensured that patients with this condition obtain support from the National Health Insurance Corporation for their medical expenditure. However, the health-related and socioeconomic status of Korean patients with MD has yet to be established. Methods: We selected 441 patients with MD who received medical services at 17 neuromuscular centers during 2005. The medical records of selected patients were analyzed, and the subtype of MD was classified by its clinical course and diagnostic tests. A total of 95 patients or their family members participated in this health-related and socioeconomic status survey. Results: Medical record analysis showed similar clinical and diagnostic characteristic data to those published previously in other countries: male predominance, being young at onset, and muscular weakness of the extremities as a predominant symptom in most patients. The diagnostic tests for MD were based on laboratory and electrophysiological studies. The most frequent form of MD among our cohort was Duchenne/Becker muscular dystrophy (42%). Our survey revealed the effect of the patients’ profound disability on their activities of daily living. One-half of the patients were dissatisfied with the medical expenditure support service that was made available to them, and most patients suffered from a financial burden. The most important medical services to be developed in the future are expansion of the public health service or development of a rehabilitation hospital. Conclusions: This is the first multicenter-based epidemiologic study on the health-related and socioeconomic status of patients with MD in Korea. The findings indicate that medical coverage and public health service are currently inadequate and hence should be expanded in the future.
참고문헌 (30)
- Hoffman EP, "Dystrophin: the protein product of the Duchenne muscular dystrophy locus", Cell, 51, pp.919~928 (1987)
- 문재호, "뒤시엔느형 근디스트로피 환자의 포괄적 임상고찰", Annals of Rehabilitation Medicine, 25(2), pp.241~248 (2001), 대한재활의학회
- 나상준, "Dystrophinopathy 환자의 임상적, 면역조직화학적 및 유전학적 분석", 대한신경과학회지, 22(5), pp.508~515 (2004), 대한신경과학회
- Park SY, "Molecular genetic analysis of dystrophin gene in Duchenne", Becker muscular dystrophy, 2004, pp.12, 50~58
- Choi BO, "Clinical significance of CTG repeat expansion in Korean myotonic dystrophy patients", J Korean Neurol Assoc, 17, pp.548~553 (1999)
- Oh Seung Hun, "Clinical and Pathological Characteristics of Four Korean Patients with Limb-Girdle Muscular Dystrophy type 2B", Journal of Korean Medical Science, 19(3), pp.447~452 (2004), 대한의학회
- Jin-Hong Shin, "Mutations of CAPN3 in Korean Patients with Limb-Girdle Muscular Dystrophy", Journal of Korean Medical Science, 22(3), pp.463~469 (2007), 대한의학회
- Emery AE, "Population frequencies of inherited neuromuscular diseases--a world survey", Neuromuscul Disord, 1, pp.19~29 (1991)
- Radhakrishnan K, "Descriptive epidemiology of selected neuromuscular disorders in Benghazi, Libya", Acta Neurol Scand, 75, pp.95~100 (1987)
- El-Tallawy HN, "Epidemiological study of muscular disorders in Assiut, Egypt", Neuroepidemiology, 25, pp.205~211 (2005)
- Nakagawa M, "Epidemiology of progressive muscular dystrophy in Okinawa, Japan. Classification with molecular biological techniques", Neuroepidemiology, 10, pp.185~191 (1991)
- Calvert LD, "Britton JR. Trends in survival from muscular dystrophy in England and Wales and impact on respiratory services", Respir Med, 100, pp.1058~1063 (2006)
- Bushby K, "107th ENMC international workshop: the management of cardiac involvement in muscular dystrophy and myotonic dystrophy. 7th-9th June 2002, Naarden, the Netherlands", Neuromuscul Disord, 13, pp.166~172 (2003)
- Yasuma F, "A new lease on life for patients with Duchenne muscular dystrophy in Japan", Am J Med, 117, pp.363~ (2004)
- Sewry CA, "Immunocytochemical analysis of human muscular dystrophy", Microsc Res Tech, 48, pp.142~154 (2000)
- Zatz M, "The 10 autosomal recessive limb-girdle muscular dystrophies", Neuromuscul Disord, 13, pp.532~544 (2003)
- 김대성, "근이영양증에서 면역조직화학염색의 진단적 의의", 대한신경과학회지, 24(1), pp.1~13 (2006), 대한신경과학회
- Bostrom K, "Sickness impact in people with muscular dystrophy: a longitudinal study over 10 years", Clin Rehabil, 19, pp.686~694 (2005)
- Kohler M, "Quality of life, physical disability, and respiratory impairment in Duchenne muscular dystrophy", Am J Respir Crit Care Med, 172, pp.1032~1036 (2005)
- Natterlund B, "Activities of daily living and quality of life in persons with muscular dystrophy", J Rehabil Med, 33, pp.206~211 (2001)
- Natterlund B, "Disability, coping and quality of life in individuals with muscular dystrophy: a prospective study over five years", Disabil Rehabil, 22, pp.776~785 (2000)
- Eggers S, "Social adjustment in adult males affected with progressive muscular dystrophy", Am J Med Genet, 81, pp.4~12 (1998)
- Antonini G, "Health-related quality of life in myotonic dystrophy type 1 and its relationship with cognitive and emotional functioning", J Rehabil Med, 38, pp.181~185 (2006)
- Chen JY, "Family function in families of children with Duchenne muscular dystrophy", Fam Community Health, 30, pp.296~304 (2007)
- Parker AE, "Analysis of an adult Duchenne muscular dystrophy population", Qjm, 98, pp.729~736 (2005)
- Boyer F, "Factors relating to carer burden for families of persons with muscular dystrophy", J Rehabil Med, 38, pp.309~315 (2006)
- Bushby K, "Social deprivation in Duchenne muscular dystrophy: population based study", Bmj, 323, pp.1035~1036 (2001)
- 임상희, "근디스트로피 환자의 교육 및 직업 실태", Annals of Rehabilitation Medicine, 32(1), pp.51~55 (2008), 대한재활의학회
- Ouyang L, "Health care utilization and expenditures for children and young adults with muscular dystrophy in a privately insured population", J Child Neurol, 23, pp.883~888 (2008)
- Davidoff A, "Effects of the State Children’s Health Insurance Program Expansions on children with chronic health conditions", Pediatrics, 116, pp.34~42 (2005)