Martins Janine Pruinelli, Forte Gabriele Carra, Simon Miriam Isabel Souza Dos Santos, Epifanio Matias, Pinto Leonardo Araújo, Marostica Paulo José Cauduro
Universidade Federal de Ciências da Saúde de Porto Alegre (UFCSPA), Porto Alegre, RS, Brasil.
Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS, Brasil.
Rev Assoc Med Bras (1992). 2018 Nov;64(11):1032-1037. doi: 10.1590/1806-9282.64.11.1032.
to assess the progression of pediatric cystic fibrosis (CF) patients' nutritional status during the first 12 months after diagnosis and to establish its association with neonatal screening and clinical variables. Patients were recruited from two reference centers in Southern Brazil.
Retrospective cohort study was carried out with all the patients diagnosed between 2009 and 2014. Anthropometric, clinic and neonatal screening were collected from medical files. Analysis of anthropometric markers over time was performed by generalized estimating equations. A multivariate regression analysis model to predict the Δ percentile body mass index (BMI) (BMI percentile difference between one year after the treatment and BMI percentile at diagnosis) was done.
Forty-seven patients were included in the study. Analysis of nutritional data over the period between six months and one year after diagnosis showed significant improvement of BMI, weight/age and weight/height percentiles and Z scores. The neonatal screening was associated with a significant increase of 31.2 points in ΔBMI percentile at the one-year evaluation (p<0.05). On the other hand, a one-point increase of initial BMI percentile was associated with a reduction of 0.6 points in ΔBMI percentile.
This study demonstrated the role of neonatal screening in the nutritional status of patients diagnosed with CF in the first year after diagnosis. Early diagnosis can significantly contribute to the achievement of appropriate anthropometric indicators and important nutritional recovery of CF patients.
评估小儿囊性纤维化(CF)患者确诊后前12个月的营养状况进展,并确定其与新生儿筛查及临床变量之间的关联。患者来自巴西南部的两个参考中心。
对2009年至2014年期间确诊的所有患者进行回顾性队列研究。从医疗档案中收集人体测量学、临床及新生儿筛查数据。通过广义估计方程对人体测量指标随时间的变化进行分析。建立了一个多元回归分析模型来预测Δ体重指数百分位数(BMI)(治疗后一年的BMI百分位数与确诊时的BMI百分位数之差)。
47例患者纳入研究。对确诊后6个月至1年期间的营养数据进行分析,结果显示BMI、体重/年龄及体重/身高百分位数和Z评分均有显著改善。在一年评估时,新生儿筛查与ΔBMI百分位数显著增加31.2分相关(p<0.05)。另一方面,初始BMI百分位数每增加1分,ΔBMI百分位数就减少0.6分。
本研究证明了新生儿筛查在CF确诊患者确诊后第一年营养状况中的作用。早期诊断可显著有助于实现适当的人体测量指标,并促进CF患者重要的营养恢复。