Bone demineralization in cystic fibrosis: evidence of imbalance between bone formation and degradation.
Bone turnover, collagen metabolism, and bone mineral status were investigated in 59 patients with cystic fibrosis and in 72 sex and age-matched control subjects. In all patients and control subjects serum concentrations of osteocalcin (OC), carboxy-terminal propeptide of type I procollagen (PICP), amino-terminal propeptide of type III procollagen (PIIINP), and cross-linked carboxy-terminal telopeptide of type I collagen (ICTP), and urinary values of cross-linked N-telopeptides of type I collagen (NTX), as well as total body bone mineral content (TBBM) were measured. Higher ICTP (microgram/L) and NTX (bone collagen equivalent/urinary creatinine (nmol/mmol) values were found in pre-pubertal, pubertal, and young adult patients than in control subjects (ICTP: 15.4 +/- 2.1 and 13.2 +/- 1.8, p < 0.001; 23.3 +/- 5.3 and 20.1 +/- 4.1, p < 0.02; 4.8 +/- 1.1 and 4.0 +/- 1.0, p < 0.05. respectively; NTX: 1047.5 +/- 528.6 and 227.8 +/- 71.8, p < 0.01; 997.8 +/- 391.7 and 376.3 +/- 91.0, p < 0.01; 993.2 +/- 398.0 and 73.9 +/- 28.5, p < 0.01, respectively). Lower OC and PICP levels (microgram/L) were showed in pubertal patients in comparison with control subjects (OC: 20.2 +/- 12.3 and 39.0 +/- 15.1, p < 0.01; PICP: 305.8 +/- 130.4 and 436.2 +/- 110.1, p < 0.02, respectively). Lower OC and higher PIIINP levels (microgram/L) were found in young adult patients than in control subjects (OC: 4.4 +/- 3.0 and 7.0 +/- 3.1, p < 0.05; PIIINP: 4.8 +/- 1.1 and 3.1 +/- 1.0, p < 0.001, respectively). TBBM (z score) was reduced in prepubertal, pubertal, and young adult patients (-0.8 +/- 0.4, -1.0 +/- 0.4, -1.1 +/- 0.5, respectively). Patients with cystic fibrosis have bone demineralization and imbalance between bone formation and degradation.
Baroncelli
G I
GI
Department of Pediatrics, University of Pisa, Italy.
De Luca
F
F
Magazzú
G
G
Arrigo
T
T
Sferlazzas
C
C
Catena
C
C
Bertelloni
S
S
Saggese
G
G
D000293:Adolescent; D000328:Adult; D000375:Aging / Q000502:physiology; D015415:Biomarkers / Q000737:chemistry; D001827:Body Height / Q000502:physiology; D015519:Bone Density / Q000502:physiology*; D001846:Bone Development / Q000502:physiology*; D001862:Bone Resorption / Q000503:physiopathology*; D016022:Case-Control Studies; D002648:Child; D003094:Collagen / Q000378:metabolism*; D003550:Cystic Fibrosis / Q000150:complications / Q000378:metabolism / Q000503:physiopathology*; D005260:Female; D050723:Fractures, Bone / Q000453:epidemiology / Q000209:etiology; D006801:Humans; D008107:Liver Diseases / Q000000981:diagnostic imaging / Q000378:metabolism; D008297:Male; D015995:Prevalence; D011627:Puberty / Q000502:physiology*; D018709:Statistics, Nonparametric; D014463:Ultrasonography
D015415:Biomarkers; D003094:Collagen