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Titolo:
THE BONE-MINERAL STATUS OF PATIENTS WITH MARFAN-SYNDROME
Autore:
KOHLMEIER L; GASNER C; BACHRACH LK; MARCUS R;
Indirizzi:
VET ADM MED CTR,GRECC 182B,MUSCUKOSKELETAL RES LAB,3801 MIRANDA AVE PALO ALTO CA 94304 VET ADM MED CTR,GRECC 182B,MUSCUKOSKELETAL RES LAB PALO ALTO CA 94304 STANFORD UNIV,MED CTR,MARFAN SYNDROME CLIN PALO ALTO CA 94304 STANFORD UNIV,MED CTR,DEPT MED PALO ALTO CA 94304 STANFORD UNIV,MED CTR,DEPT PEDIAT PALO ALTO CA 94304
Titolo Testata:
Journal of bone and mineral research
fascicolo: 10, volume: 10, anno: 1995,
pagine: 1550 - 1555
SICI:
0884-0431(1995)10:10<1550:TBSOPW>2.0.ZU;2-D
Fonte:
ISI
Lingua:
ENG
Soggetto:
CONNECTIVE-TISSUE; CALCIUM-BINDING; FIBRILLIN; GENE; CHROMOSOME-15; EXPRESSION; DISORDERS; COLLAGEN; DECORIN;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
28
Recensione:
Indirizzi per estratti:
Citazione:
L. Kohlmeier et al., "THE BONE-MINERAL STATUS OF PATIENTS WITH MARFAN-SYNDROME", Journal of bone and mineral research, 10(10), 1995, pp. 1550-1555

Abstract

Osteopenia at the hip and low total body calcium content have been reported in women,vith Marfan syndrome, Using dual X-ray absorptiometry (DXA), we evaluated the lumbar spine L2-L4 and proximal femur bone mineral density (BMD, g/cm(2)) in 32 women and 16 children with Marfan syndrome. The women were 38 +/- 10 (SD) years old (23-58 years); their mean height was 178.6 +/- 8 cm. The children (9 boys and 5 girls) were 9.9-17.5 years old, Children were tall for their ages but of normal weight, All subjects were moderately active without previous nontraumatic fracture, In the women, BMD was reduced at L2-L4, femoral neck (fnk), trochanter (tr), and intertrochanter (intr) (p < 0.0001-0.006), compared with age-predicted values, Z stores for L2-L4 and for the fnk, tr, and intr, were -0.59 +/- 1.06, -1.25 +/- 0.99, -1.03 +/- 1.10, and -1.31 +/- 0.91, respectively, The average hip axis length (HAL) of 11.5+/- 0.093 cm was at the 80th percentile for women, No significant change,vas observed in 1 year follow-up BMD measurements in 13 women (fnk= -0.23 +/- 2.3%/year; L2-L4 = -0.43 +/- 1.57%/year), In Marfan children, BMD correlated with age, height, and pubertal development, Femoral neck BMD was reduced (Z = -0.74 +/- 1.22, p < 0.05) with a nonsignificant trend toward decreased BMD at L2-L4 (Z = -0.33 +/- 1.48). Resorption markers in Marfan women were normal and did not correlate with bone status. We conclude that women with Marfan syndrome have both axialand peripheral osteopenia as well as an increased HAL, This combination of findings likely increases substantially their long-term risk forhip fracture. Presence of osteopenia in Marfan children indicates that the skeletal deficits of Marfan syndrome may reflect inadequate boneacquisition.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 03/12/20 alle ore 15:45:01