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Activity of the antimicrobial peptide in children with cystic fibrosis

[Cystic fibrosis]
Elena Kestutisovna Zhekaite; Leonid Klimov; Svetlana Dolbnya; Elena Ivanovna Kondratieva; Natalia Anatolievna Ilyenkova; Victoria Kuryaninova; Vladimir Viktorovich Chikunov; Elena Alexandrovna Enina;

100 patients with cystic fibrosis aged 0 to 18 years and 58 healthy children were examined in Moscow, Krasnoyarsk and Stavropol. The concentration of α-1-3-defensin (HNP1-3), cathelicidin (LL-37), and calcidiol in the blood serum was determined in all patients. A significant deterioration in the availability of vitamin D was revealed as children grow. It was shown that the level of HNP1-3 above 4270 pg/ml is detected significantly more often in adolescents and, conversely, in the group of patients younger than 3 years, the level of HNP1-3 less than 2980 pg/ml is determined more often than in older children (χ2 test, p=0.008). The activity of LL-37 is almost the same in all age groups. Patients with cystic fibrosis are characterized by significantly lower serum calcidiol levels –25.1 [16.6–38.6] ng/ml than healthy children 31.7 [23.3–42.7] ng/ml (p=0.003). Moreover, the level of HNP1-3 below 2980 pg/ml was significantly more often detected among healthy children – 28 (48.3 %) than in patients with CF – 26 (26.0 %; p = 0.017). A weak relationship was revealed between the level of vitamin D and LL-37 r=0.278, p=0.001. Patients with CF are characterized by an increase in LL-37 in chronic Pseudomonas aeruginosa infection, for HNP1-3 such a relationship is not obtained.

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Keywords: cystic fibrosis, antimicrobial peptides, cathelicidin, α-1-3-defensins, Pseudomonas aeruginosa, lung function, vitamin D


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