Impaired Growth during Childhood in Patients with Primary Ciliary Dyskinesia
Joint Authors
Lebl, Jan
Cipra, Adam
Svobodová, Tamara
Zemková, Daniela
Pohunek, Petr
Djakow, Jana
Source
International Journal of Endocrinology
Issue
Vol. 2013, Issue 2013 (31 Dec. 2013), pp.1-5, 5 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2013-12-12
Country of Publication
Egypt
No. of Pages
5
Main Subjects
Abstract EN
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 +0.40±0.24 SDS (the 1st birthday), +0.16±0.23 SDS (3 years old), and -0.13±0.21 SDS (5 years old) to -0.54±0.19 SDS (7 years old; P=0.01 versus 0), -0.67±0.21 SDS (9 years old; P=0.005 versus 0), -0.52±0.24 SDS (11 years old; P=0.04 versus 0), and -0.53±0.23 SDS (13 years old; P=0.03 versus 0).
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.
American Psychological Association (APA)
Svobodová, Tamara& Djakow, Jana& Zemková, Daniela& Cipra, Adam& Pohunek, Petr& Lebl, Jan. 2013. Impaired Growth during Childhood in Patients with Primary Ciliary Dyskinesia. International Journal of Endocrinology،Vol. 2013, no. 2013, pp.1-5.
https://search.emarefa.net/detail/BIM-494206
Modern Language Association (MLA)
Svobodová, Tamara…[et al.]. Impaired Growth during Childhood in Patients with Primary Ciliary Dyskinesia. International Journal of Endocrinology No. 2013 (2013), pp.1-5.
https://search.emarefa.net/detail/BIM-494206
American Medical Association (AMA)
Svobodová, Tamara& Djakow, Jana& Zemková, Daniela& Cipra, Adam& Pohunek, Petr& Lebl, Jan. Impaired Growth during Childhood in Patients with Primary Ciliary Dyskinesia. International Journal of Endocrinology. 2013. Vol. 2013, no. 2013, pp.1-5.
https://search.emarefa.net/detail/BIM-494206
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-494206