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Low Bone Mineral Density in Young Children with Cystic Fibrosis

¹ Service de Pédiatrie Générale, Centre de Ressources et de Compétence en Mucoviscidose, and ² Laboratoire d'Explorations Fonctionnelles, Hôpital Necker-Enfants Malades, Paris, France

Correspondence and requests for reprints should be addressed to I. Sermet-Gaudelus, M.D., Ph.D., Service de Pédiatrie Générale, 149 rue de Sévres, 75015 Paris, France. E-mail: isabelle.sermet{at}nck.aphp.fr

Rationale: Low bone mineral density (BMD) 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 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 children with CF 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 children aged 6 to 10 years (–0.91; SEM, 0.2) or adolescents (-1.4; SEM, 0.2). LS z score was positively correlated with fat-free mass in multiple regression analysis. LS z score was less than –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

AT A GLANCE COMMENTARY Scientific Knowledge on the Subject Low bone mineral density is a frequent problem for adult patients with cystic fibrosis. Studying very young patients may help to clarify the underlying pathogenesis. What This Study Adds to the Field The origin of cystic fibrosis bone disease may lie in a primary defect, independent of nutritional status or disease severity.

 

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