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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2015.1
- 수록면
- 911 - 916 (6page)
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초록· 키워드
오류제보하기
Patients with Marfan syndrome (MFS) presents with primary skeletal manifestations such as tall stature, chest wall abnormality, and scoliosis. These primary skeletal manifestations affect the growth pattern in MFS. Therefore, it is not appropriate to use normal growth charts to evaluate the growth status of MFS. We aimed to develop disease-specific growth charts for Korean MFS patients and to use these growth charts for understanding the growth patterns in MFS and managing of patients with MFS. Anthropometric data were available from 187 males and 152 females with MFS through a retrospective review of medical records. Disease-specific growth charts were generated and 3, 25, 50, 75, and 97 percentiles were calculated using the LMS (refers to λ, μ, and σ, respectively) smoothing procedure for height and weight. Comparisons between MFS patients and the general population were performed using a one-sample t-test. With regard to the height, the 50th percentile of MFS is above the normative 97th percentile in both genders. With regard to the weight, the 50 percentile of MFS is above the normative 75th percentile in male and between the normative 50th percentile and the 75th percentile in female. The diseasespecific growth charts for Korean patients with MFS can be useful for monitoring growth patterns, planning the timing of growth-reductive therapy, predicting adult height and recording responses to growth-reductive therapy.
목차
등록된 정보가 없습니다.
참고문헌 (17)
참고문헌 신청
Pyeritz RE / 1979 / The Marfan syndrome : diagnosis and management / N Engl J Med 300:772~777
Lee NC / 2005 / Intrafamilial phenotype variation in Marfan syndrome ascertained by intragenic linkage analysis / J Formos Med Assoc 104:964~967
Judge DP / 2005 / Marfan’s syndrome / Lancet 366:1965~1976
Gray JR / 1998 / Life expectancy in British Marfan syndrome populations / Clin Genet 54:124~128
Erkula G / 2002 / Growth and maturation in Marfan syndrome / Am J Med Genet 109:100~115
Lee NC / 2005 / Intrafamilial phenotype variation in Marfan syndrome ascertained by intragenic linkage analysis / J Formos Med Assoc 104:964~967
Judge DP / 2005 / Marfan’s syndrome / Lancet 366:1965~1976
Gray JR / 1998 / Life expectancy in British Marfan syndrome populations / Clin Genet 54:124~128
Erkula G / 2002 / Growth and maturation in Marfan syndrome / Am J Med Genet 109:100~115
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