[Bone mass in patients with cystic fibrosis of the pancreas. Relationship with anthropometric parameters and genotype].
BACKGROUND
Cross-sectional study of bone mineral density (BMD) in children and adolescents with cystic fibrosis of the pancreas. The relationship of BMD values with nutritional status, respiratory function and the cystic transmembrane regulator genotype was also evaluated.


METHODS
BMD expressed as grams of hydroxyapatite/cm2 was measured by dual-energy X-ray absorptiometry in the lumbar spine (L2-L4) in 41 patients (21 males and 20 women; age range: 4-21 years) with cystic fibrosis of the pancreas and compared with that of 471 normal controls (256 males and 215 women; age range: 1-20 years). Twenty patients were prepubertal, 9 pubertal and 12 young adults.


RESULTS
Clinical repercussion of the disease evaluated by clinical and anthropometric data (weight, height and body mass index) and respiratory function was considered moderate. Height z score (mean [MSE]) was -0.53 (0.28), weight -0.81 (0.21) and body mass index -0.82 (0.12) BMD z score values (mean [MSE]) were -1.14 (0.17) and differed significantly (p < 0.001) from those of normal age- and sex-matched controls. No significant differences were observed between males and women or among prepubertal, pubertal and young adult patients. BMD z score values less than-1 z score were found in 53% and under -2 z score in 8%. Cystic transmembrane regulator genotype was studied in 36 patients (17 were F508/-, 10 F508/F508, 5 G542X/- and 4 diverse) and did not predict bone mineral status. A statistically significant correlation was found between BMD z score values and height z score, weight z score, body mass index z score and clinical assessment according to Shwachman criteria. A negative and statistically significant correlation was observed between BMD z score and functional score.


CONCLUSIONS
The decrease in BMD values in CF patients begins early in life and appears to be related to the degree of clinical expression of the disease.
Yeste
D
D
Departamento de Pediatría, Hospital Materno-Infantil Vall d'Hebron, Universidad Autónoma de Barcelona.
Liñán
S
S
Cobos
N
N
Gussinyé
M
M
del Río
L
L
Carrascosa
A
A
D000293:Adolescent; D000328:Adult; D000886:Anthropometry; D015519:Bone Density; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000235:genetics / Q000503:physiopathology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D005260:Female; D005838:Genotype; D006801:Humans; D008297:Male; D010182:Pancreatic Diseases / Q000235:genetics / Q000503:physiopathology
C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator
Masa ósea en pacientes con fibrosis quística de páncreas. Relación con parámetros antropométricos y genotipo.