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International Journal of Endocrinology
Volume 2013 (2013), Article ID 731423, 5 pages
http://dx.doi.org/10.1155/2013/731423
Research Article

Impaired Growth during Childhood in Patients with Primary Ciliary Dyskinesia

1Department of Pediatrics, 2nd Faculty of Medicine, University Hospital Motol, Charles University, 150 06 Prague, Czech Republic
2Department of Pediatrics, Masaryk’s Hospital, 400 11 Usti nad Labem, Czech Republic

Received 28 August 2013; Accepted 19 November 2013

Academic Editor: Fabio Buzi

Copyright © 2013 Tamara Svobodová et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Primary ciliary dyskinesia (PCD) leads to recurrent/chronic respiratory infections, resulting in chronic inflammation and potentially in chronic pulmonary disease with bronchiectasis. We analyzed longitudinal data on body length/height and body mass index (BMI) for 29 children and young adults with PCD aging 1.5–24 years (median, 14.5) who had been diagnosed at the age of 0.5–17 years (median, 8). Of these, 10 carried pathogenic mutations in either DNAH5 or DNAI1. In children with PCD, body length/height progressively decreased from SDS (the 1st birthday), SDS (3 years old), and SDS (5 years old) to SDS (7 years old; versus ), SDS (9 years old; versus ), SDS (11 years old; versus ), and SDS (13 years old; versus ). These results reflect low growth rates during the childhood growth period. Thereafter, heights stabilized up to the age of 17 years. The growth deterioration was not dependent on sex or disease severity but was more pronounced in DNAH5 or DNAI1 mutation carriers. BMI did not differ from population standards, which suggests that nutritional deficits are not the cause of growth delay. We conclude that PCD leads to chronic deprivation with significant growth deterioration during childhood.