Canapari Craig A, Barrowman Nick, Hoey Lynda, Walker Scott W, Townsend Elise, Tseng Brian S, Katz Sherri L
Yale New Haven Children's Hospital, Yale University, New Haven, Connecticut.
Pediatr Pulmonol. 2015 Jan;50(1):63-70. doi: 10.1002/ppul.23004. Epub 2014 Mar 18.
Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder associated with progressive muscle weakness and respiratory failure. Oral corticosteroids are the mainstay of treatment, but are associated with obesity with a central distribution. This study is designed to determine the relationship between body mass index, central adiposity, and lung function in subjects with DMD.
Retrospective fat distribution data was obtained in boys with DMD from studies using dual X-ray absorptiometry (DXA). Fat distribution data was reviewed at two tertiary academic institutions and compared with concurrent height, weight, body mass index (BMI), measures of lung function, and sleep study data when available. Truncal fat mass used as a measure of central adiposity.
Forty-four subjects (age 12.0 ± 3.4 years) were included. Mean BMI was 22.1 ± 5.9 kg/m(2) . Sixty-eight percent (30 patients) were on corticosteroid therapy. Truncal fat mass percentage was inversely correlated with forced vital capacity (% predicted FVC) (Pearson coefficient -0.37, P = 0.01). Linear regression showed that truncal fat distribution, but not total fat mass, age or corticosteroid use negatively predicted FVC (r(2) = 0.24, P = 0.048). BMI had a positive effect (P = 0.04). However, fat distribution did not predict the rate of change of lung function in a smaller sample. Fat distribution, BMI, or age did not predict measures of sleep disordered breathing.
Truncal fat distribution is a significant predictor of lower vital capacity in boys with DMD, whereas BMI has a positive effect.
杜氏肌营养不良症（DMD）是一种X连锁隐性疾病，与进行性肌无力和呼吸衰竭相关。口服皮质类固醇是主要治疗方法，但会导致以中心性分布为主的肥胖。本研究旨在确定DMD患者的体重指数、中心性肥胖与肺功能之间的关系。
通过双能X线吸收法（DXA）研究，获取DMD男孩的回顾性脂肪分布数据。在两家三级学术机构对脂肪分布数据进行审查，并与同期身高、体重、体重指数（BMI）、肺功能指标以及睡眠研究数据（如有）进行比较。使用躯干脂肪量作为中心性肥胖的指标。
纳入44名受试者（年龄12.0±3.4岁）。平均BMI为22.1±5.9kg/m²。68%（30例患者）接受皮质类固醇治疗。躯干脂肪量百分比与用力肺活量（预测FVC%）呈负相关（Pearson系数-0.37，P=0.01）。线性回归显示，躯干脂肪分布而非总脂肪量、年龄或皮质类固醇使用对FVC有负向预测作用（r²=0.24，P=0.048）。BMI有正向作用（P=0.04）。然而，在较小样本中，脂肪分布不能预测肺功能的变化率。脂肪分布、BMI或年龄不能预测睡眠呼吸障碍指标。
躯干脂肪分布是DMD男孩肺活量降低的重要预测因素，而BMI有正向作用。
