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Published ahead of print on February 1, 2007, doi:10.1164/rccm.200606-776OC

Am. J. Respir. Crit. Care Med., Volume 175, Number 9, May 2007, 951-957

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Submitted on June 12, 2006
Accepted on February 1, 2007

Low Bone Mineral Density in Young Children with Cystic Fibrosis

Isabelle Sermet-Gaudelus1*, Jean Claude Souberbielle2, Jean Charles Ruiz2, Stephanie Vrielynck1, Blandine Heuillon1, Imrana Azhar1, Aline Cazenave1, Ethel Lawson-Body2, Frederique Chedevergne1, and Gerard Lenoir1

1 Service de Pediatrie Generale, Centre de Ressources et de Competence en Mucoviscidose, Hopital Necker-Enfants Malades, Paris, France, 2 Laboratoire d'Explorations Fonctionnelles, Hopital Necker-Enfants Malades, Paris, France

* To whom correspondence should be addressed. E-mail: isabelle.sermet{at}nck.aphp.fr.

Rationale: Low bone mineral density is a frequent problem for adult patients with Cystic Fibrosis (CF). Only limited information is available for young patients. Objectives: The aim of this study was to evaluate bone mineral density (BMD) of children with CF younger than 6 years. Methods: BMD was measured at the lumbar spine (LS) after adjustment for height, sex and pubertal status in 25 CF children younger than 6 years, 53 prepubertal children aged 6 to 10 years and 36 adolescents aged 11 to 18 years. Nutritional status, body composition, pulmonary disease severity, corticosteroid usage, dietary calcium, caloric intake, and vitamin D status were evaluated as potential correlates of BMD. Measurements and main results: The mean LS Z score in the youngest group was significantly lower than normal (-0.96; SEM=0.3). It did not differ significantly from that of either the children aged 6 to 10 years (-0.91(0.2)) or the adolescents (-1.4(0.2)). LS Z score was positively correlated with fat-free mass (FFM) in multiple regression analysis. LS Z score was < - 1 in 34% of the patients with mild pulmonary disease and normal nutritional status. Conclusions: These data suggest that the origin of CF bone disease in early childhood may be independent of nutritional status or disease severity.
Key words: bone mineralization, Cystic Fibrosis, Fat free mass, Cystic Fibrosis Transmembrane Conductance Regulator




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