{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T01:03:17Z","timestamp":1780362197341,"version":"3.54.1"},"reference-count":25,"publisher":"Wiley","license":[{"start":{"date-parts":[[2013,1,1]],"date-time":"2013-01-01T00:00:00Z","timestamp":1356998400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/3.0\/"}],"funder":[{"name":"Ministry of Health, Czech Republic","award":["NT11469-5"],"award-info":[{"award-number":["NT11469-5"]}]},{"name":"Ministry of Health, Czech Republic","award":["00064203\u20136001"],"award-info":[{"award-number":["00064203\u20136001"]}]},{"name":"Motol University Hospital","award":["NT11469-5"],"award-info":[{"award-number":["NT11469-5"]}]},{"name":"Motol University Hospital","award":["00064203\u20136001"],"award-info":[{"award-number":["00064203\u20136001"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["International Journal of Endocrinology"],"published-print":{"date-parts":[[2013]]},"abstract":"<jats:p>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\u201324 years (median, 14.5) who had been diagnosed at the age of 0.5\u201317 years (median, 8). Of these, 10 carried pathogenic mutations in either<jats:italic>DNAH5<\/jats:italic>or<jats:italic>DNAI1<\/jats:italic>. In children with PCD, body length\/height progressively decreased from<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M1\"><mml:mo>+<\/mml:mo><mml:mn mathvariant=\"normal\">0.40<\/mml:mn><mml:mo>\u00b1<\/mml:mo><mml:mn mathvariant=\"normal\">0.24<\/mml:mn><\/mml:math>SDS (the 1st birthday),<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M2\"><mml:mo>+<\/mml:mo><mml:mn mathvariant=\"normal\">0.16<\/mml:mn><mml:mo>\u00b1<\/mml:mo><mml:mn mathvariant=\"normal\">0.23<\/mml:mn><\/mml:math>SDS (3 years old), and<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M3\"><mml:mo>-<\/mml:mo><mml:mn mathvariant=\"normal\">0.13<\/mml:mn><mml:mo>\u00b1<\/mml:mo><mml:mn mathvariant=\"normal\">0.21<\/mml:mn><\/mml:math>SDS (5 years old) to<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M4\"><mml:mo>-<\/mml:mo><mml:mn mathvariant=\"normal\">0.54<\/mml:mn><mml:mo>\u00b1<\/mml:mo><mml:mn mathvariant=\"normal\">0.19<\/mml:mn><\/mml:math>SDS (7 years old;<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M5\"><mml:mi>P<\/mml:mi><mml:mo>=<\/mml:mo><mml:mn mathvariant=\"normal\">0.01<\/mml:mn><\/mml:math>versus<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M6\"><mml:mrow><mml:mn mathvariant=\"normal\">0<\/mml:mn><\/mml:mrow><\/mml:math>),<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M7\"><mml:mo>-<\/mml:mo><mml:mn mathvariant=\"normal\">0.67<\/mml:mn><mml:mo>\u00b1<\/mml:mo><mml:mn mathvariant=\"normal\">0.21<\/mml:mn><\/mml:math>SDS (9 years old;<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M8\"><mml:mi>P<\/mml:mi><mml:mo>=<\/mml:mo><mml:mn mathvariant=\"normal\">0.005<\/mml:mn><\/mml:math>versus<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M9\"><mml:mrow><mml:mn mathvariant=\"normal\">0<\/mml:mn><\/mml:mrow><\/mml:math>),<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M10\"><mml:mo>-<\/mml:mo><mml:mn mathvariant=\"normal\">0.52<\/mml:mn><mml:mo>\u00b1<\/mml:mo><mml:mn mathvariant=\"normal\">0.24<\/mml:mn><\/mml:math>SDS (11 years old;<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M11\"><mml:mi>P<\/mml:mi><mml:mo>=<\/mml:mo><mml:mn mathvariant=\"normal\">0.04<\/mml:mn><\/mml:math>versus<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M12\"><mml:mrow><mml:mn mathvariant=\"normal\">0<\/mml:mn><\/mml:mrow><\/mml:math>), and<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M13\"><mml:mo>-<\/mml:mo><mml:mn mathvariant=\"normal\">0.53<\/mml:mn><mml:mo>\u00b1<\/mml:mo><mml:mn mathvariant=\"normal\">0.23<\/mml:mn><\/mml:math>SDS (13 years old;<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M14\"><mml:mi>P<\/mml:mi><mml:mo>=<\/mml:mo><mml:mn mathvariant=\"normal\">0.03<\/mml:mn><\/mml:math>versus<mml:math xmlns:mml=\"http:\/\/www.w3.org\/1998\/Math\/MathML\" id=\"M15\"><mml:mrow><mml:mn mathvariant=\"normal\">0<\/mml:mn><\/mml:mrow><\/mml:math>). 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<jats:italic>DNAH5<\/jats:italic>or<jats:italic>DNAI1<\/jats:italic>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.<\/jats:p>","DOI":"10.1155\/2013\/731423","type":"journal-article","created":{"date-parts":[[2013,12,12]],"date-time":"2013-12-12T16:03:49Z","timestamp":1386864229000},"page":"1-5","source":"Crossref","is-referenced-by-count":18,"title":["Impaired Growth during Childhood in Patients with Primary Ciliary Dyskinesia"],"prefix":"10.1155","volume":"2013","author":[{"given":"Tamara","family":"Svobodov\u00e1","sequence":"first","affiliation":[{"name":"Department of Pediatrics, 2nd Faculty of Medicine, University Hospital Motol, Charles University, 150 06 Prague, Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jana","family":"Djakow","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, 2nd Faculty of Medicine, University Hospital Motol, Charles University, 150 06 Prague, Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela","family":"Zemkov\u00e1","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, 2nd Faculty of Medicine, University Hospital Motol, Charles University, 150 06 Prague, Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam","family":"Cipra","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Masaryk\u2019s Hospital, 400 11 Usti nad Labem, Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Petr","family":"Pohunek","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, 2nd Faculty of Medicine, University Hospital Motol, Charles University, 150 06 Prague, Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3365-5375","authenticated-orcid":true,"given":"Jan","family":"Lebl","sequence":"additional","affiliation":[{"name":"Department of 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