인문학
사회과학
자연과학
공학
의약학
농수해양학
예술체육학
복합학
DBpia AI
AI 에이전트
AI 아이디어
AI 리더
AI 뷰어
Citeasy
크롬 서지관리 다운로드
지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
초록·키워드
Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare genetic disorder characterized by premature aging, severe growth retardation, and metabolic abnormalities. This study aimed to evaluate the growth, nutritional status, and body composition of HGPS patients, with a focus on fat and muscle distribution, to provide insights into potential nutritional and therapeutic interventions. Eight HGPS patients (aged ≥ 3 years) and 18 age- and sex-matched healthy controls were enrolled. Physical assessments, dietary surveys, and laboratory tests were conducted, including dual-energy X-ray absorptiometry (DXA) to analyze bone density, fat distribution, and muscle mass. Genetic testing confirmed LMNA mutations in all patients. Data on growth parameters, dietary intake, and metabolic profiles were collected and compared with controls. HGPS patients exhibited severe growth retardation, with significant declines in height and weight by two months of age compared to controls. Dietary surveys revealed that 4 out of 8 subjects had sufficient energy intake, the energy intake was 91 ± 39% of the estimated basal metabolic rate. DXA analysis showed reduced bone density (Z-score: -2.82 ± 1.46), abnormal fat distribution (increased visceral fat and decreased subcutaneous fat, the T/L fat ratio and A/G fat ratio > 95th percentile for boys and girls in China), and significant reductions in total and limb muscle mass(< mean-2SD). HGPS patients experience profound growth retardation, metabolic dysfunction, and abnormal body composition, including reduced muscle mass and altered fat distribution. A nutrient-dense diet with increased protein intake, healthy fat intake, and supplementation with vitamin D, calcium, and zinc are essential to support growth and muscle maintenance. Tailored and individualised dietary interventions may further improve outcomes. Continuous monitoring and further research are needed to optimize interventions and enhance the quality of life for HGPS patients.
인공지능 문자 인식 모델을 통해 추출된 텍스트로, 일부 오타나 오류가 포함될 수 있으나 지속적으로 개선 중입니다.
오류를 발견하셨다면 해당 부분을 드래그한 후 ' 를 통해 신고해주세요.
오류를 발견하셨다면 해당 부분을 드래그한 후 ' 를 통해 신고해주세요.
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