Francalanci Michela, Terlizzi Vito, Fevola Cristina, Di Rosa Giulia, Pierattini Valentina, Roselli Elena, Bonomi Paolo, Cavicchi Maria Chiara, Galici Valeria, Neri Anna Silvia, Bianchimani Chiara, Campana Silvia, Dolce Daniela, Ravenni Novella, Camera Erica, Orioli Tommaso, Taccetti Giovanni
Meyer Children's Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Department of Paediatric Medicine, 50139 Florence, Italy.
Meyer Children's Hospital IRCCS, Professional Dietetics, 50139 Florence, Italy.
Children (Basel). 2023 Jan 30;10(2):252. doi: 10.3390/children10020252.
Improved therapy in CF has led to an overall improvement in nutritional status. The objectives of our study are: to cross-sectionally assess nutritional status and serum levels of fat-soluble vitamins; to retrospectively evaluate the efficacy of modulators on nutritional status and fat-soluble vitamin levels.
In patients younger than 2 years of age, we evaluated growth, in patients aged 2-18 years, we assessed BMI z-scores, and in adults, we assessed absolute BMI values. Levels of 25(OH)D, vitamins A, and E were measured.
A cross-sectional analysis was conducted on 318 patients, 109 (34.3%) with pancreatic sufficiency. Only three patients were under 2 years old. In 135 patients aged 2-18 years, the median BMI z-score was 0.11, and 5 (3.7%) patients had malnutrition (z-score ≤ 2SD). In 180 adults, the median BMI was 21.8 kg/m. Overall, 15 (13.7%) males (M) and 18 (25.3%) females (F) were underweight (18 < BMI > 20); 3 (2.7%) M and 5 (7.0%) F had a BMI < 18. Suboptimal 25(OH)D levels were found in patients with pancreatic insufficiency. The prevalence of deficiency of vitamins A and E is low. After one year of treatment with modulators, the increase in BMI was more consistent (M: 1.58 ± 1.25 kg/m F: 1.77 ± 1.21 kg/m) in elexacaftor/tezacaftor/ivacaftor (ETI)-treated patients compared with other modulators, with a significant increase in levels of all fat-soluble vitamins.
Malnutrition is present in a limited number of subjects. The prevalence of subjects with suboptimal 25(OH)D levels is high. ETI showed a beneficial effect on nutritional status and circulating levels of fat-soluble vitamins.
囊性纤维化（CF）治疗方法的改进使营养状况得到了全面改善。我们研究的目的是：横断面评估营养状况和脂溶性维生素的血清水平；回顾性评估调节剂对营养状况和脂溶性维生素水平的疗效。
对于2岁以下的患者，我们评估其生长情况；对于2至18岁的患者，我们评估BMI z评分；对于成年人，我们评估绝对BMI值。测量25(OH)D、维生素A和维生素E的水平。
对318例患者进行了横断面分析，其中109例（34.3%）胰腺功能正常。只有3例患者年龄在2岁以下。在135例2至18岁的患者中，BMI z评分中位数为0.11，5例（3.7%）患者存在营养不良（z评分≤2SD）。在180例成年人中，BMI中位数为21.8 kg/m²。总体而言，15例（13.7%）男性和18例（25.3%）女性体重过轻（18＜BMI＞20）；3例（2.7%）男性和5例（7.0%）女性的BMI＜18。胰腺功能不全的患者25(OH)D水平欠佳。维生素A和维生素E缺乏的患病率较低。与其他调节剂相比，接受依列卡福/替扎卡福/依伐卡福（ETI）治疗的患者在使用调节剂治疗一年后，BMI的增加更为一致（男性：1.58±1.25 kg/m²；女性：1.77±1.21 kg/m²），所有脂溶性维生素水平均显著升高。
营养不良仅存在于少数受试者中。25(OH)D水平欠佳的受试者患病率较高。ETI对营养状况和脂溶性维生素的循环水平显示出有益作用。
