Pinto Isabel Carolina da Silva, Silva Cristiane Pereira da, Britto Murilo Carlos Amorim de
Instituto Materno Infantil Professor Fernando Figueira, Recife, PE, Brasil.
J Bras Pneumol. 2009 Feb;35(2):137-43. doi: 10.1590/s1806-37132009000200006.
To describe the profile of patients with cystic fibrosis (CF).
A prospective, cross-sectional study involving CF patients < 18 years of age, evaluated between March and July of 2006 at a referral center in northeastern Brazil. Nutritional assessment was performed using Z scores for height/age (H/A), weight/age (W/A) and weight/height (W/H), as well as %weight/height (%W/H) and body composition measurements. Socioeconomic and clinical data were obtained.
Twenty-one patients were evaluated, 12 (57.1%) of whom were female. Mean age at diagnosis was 3.8 +/- 3.9 years. The principal features at diagnosis were respiratory infection (85.7%), steatorrhea (66.7%) and nutritional deficit (47.6%). The mean Z scores for W/A, H/A and W/H were 0.73 +/- 0.28, 0.34 +/- 0.21 and 0.73 +/- 0.35, respectively. Mean %W/H was 94.52 +/- 1.58. The percentage of malnourished children assessed by Z score differed from that assessed by %W/H (nutritional deficit in 66.7% and 33.3%, respectively; p > 0.05). Socioeconomic status, clinical status and Shwachman score were better among well-nourished patients than among those classified as malnourished (p < 0.05 for Shwachman score).
Normal nutritional status was identified based on nutritional indicators (W/A, H/A and W/H), whereas nutritional deficit was identified by assessing body composition. Socioeconomic factors proved favorable, especially maternal education and per capita income. Age at diagnosis was higher than that reported in the literature, although the Shwachman score and the incidence of respiratory infections demonstrated that the patients presented good clinical status.
描述囊性纤维化（CF）患者的概况。
一项前瞻性横断面研究，纳入年龄小于18岁的CF患者，于2006年3月至7月在巴西东北部的一家转诊中心进行评估。使用身高/年龄（H/A）、体重/年龄（W/A）和体重/身高（W/H）的Z评分以及体重/身高百分比（%W/H）和身体成分测量进行营养评估。获取社会经济和临床数据。
评估了21例患者，其中12例（57.1%）为女性。诊断时的平均年龄为3.8±3.9岁。诊断时的主要特征为呼吸道感染（85.7%）、脂肪泻（66.7%）和营养缺乏（47.6%）。W/A、H/A和W/H的平均Z评分分别为0.73±0.28、0.34±0.21和0.73±0.35。平均%W/H为94.52±1.58。通过Z评分评估的营养不良儿童百分比与通过%W/H评估的不同（营养缺乏分别为66.7%和33.3%；p>0.05）。营养良好的患者在社会经济状况、临床状况和舒瓦茨曼评分方面优于被归类为营养不良的患者（舒瓦茨曼评分p<0.05）。
根据营养指标（W/A、H/A和W/H）确定营养状况正常，而通过评估身体成分确定营养缺乏。社会经济因素显示出有利影响，尤其是母亲的教育程度和人均收入。诊断时的年龄高于文献报道，尽管舒瓦茨曼评分和呼吸道感染的发生率表明患者的临床状况良好。
