Umławska Wioleta, Krzyżanowska Monika, Zielińska Anna, Sands Dorota
Katedra Biologii Czlowieka Uniwersytetu Wroclawskiego.
Pediatr Endocrinol Diabetes Metab. 2012;18(4):137-42.
Somatic development and respiratory function are the most important prognostic factors for the course of the disease and the expected lifespan of patients suffering from cystic fibrosis (cF). Aim of the study was to evaluate nutritional status and pulmonary function in children and adolescents suffering from cystic fibrosis. Pulmonary function was evaluated in relation to several factors connected to the course of the disease.
Anthropometric and spirometric data were extracted from the medical histories of 89 children diagnosed and treated at the institute of Mother and child in Warsaw. Molecular DnA was analyzed in all the patients in order to determine the type of CFTR gene mutation. Anthropometric parameters were expressed in terms of standard deviations away from age-specific and sex-specific reference for the population of Warsaw.
the studied children were significantly shorter than healthy children. Ten of the children (11.2%) had severe growth disturbances. nutritional status was also adversely affected. Almost 50% of the children were mal-nourished. the children suffered from a deficit in muscularity and adiposity. The percent of predicted FeV1 was highest in those children who were well-nourished, and lowest in the severely malnourished patients. the effect of the nutritional status on pulmonary function was observed.
chronic infection by P. aeruginosa had a marked adverse effect on pulmonary function.
躯体发育和呼吸功能是囊性纤维化(CF)患者疾病进程和预期寿命的最重要预后因素。本研究的目的是评估患有囊性纤维化的儿童和青少年的营养状况和肺功能。根据与疾病进程相关的几个因素对肺功能进行了评估。
从华沙母婴研究所诊断和治疗的89名儿童的病历中提取人体测量和肺量计数据。对所有患者的分子DNA进行分析,以确定CFTR基因突变类型。人体测量参数以偏离华沙人群年龄和性别特异性参考值的标准差表示。
研究中的儿童明显比健康儿童矮。其中10名儿童(11.2%)有严重的生长障碍。营养状况也受到不利影响。近50%的儿童营养不良。这些儿童存在肌肉量和脂肪量不足的情况。预测FEV1百分比在营养良好的儿童中最高,在严重营养不良的患者中最低。观察到营养状况对肺功能的影响。
铜绿假单胞菌的慢性感染对肺功能有显著的不利影响。