Bone mineral density in children with neurofibromatosis 1

• Published in: Acta Paediatrica Vol. 96; no. 8; pp. 1220 - 1222
• Main Authors: Yilmaz, Kutluhan, Ozmen, Meral, Bora Goksan, Suleyman, Eskiyurt, Nurten
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01-08-2007; Blackwell
• Subjects: Absorptiometry, Photon; Adolescent; Biological and medical sciences; Body Composition - physiology; Bone Density - physiology; Bone Diseases, Metabolic - physiopathology; Bone mineral density; Child; Child, Preschool; DEXA; Exercise - physiology; Female; General aspects; Humans; Investigative techniques, diagnostic techniques (general aspects); Leg Bones - diagnostic imaging; Leg Bones - physiopathology; Male; Medical sciences; Neurofibromatosis; Neurofibromatosis 1 - complications; Neurofibromatosis 1 - physiopathology; Neurology; Osteoarticular system. Muscles; Radiodiagnosis. Nmr imagery. Nmr spectrometry; Spine - diagnostic imaging; Spine - physiopathology; Tumors of the nervous system. Phacomatoses; Human; Pediatrics; Skin disease; Nervous system diseases; Phacomatosis; DEXA; Neurofibromatosis; Bone mineral density; Benign neoplasm; Child; Neurofibromatosis Recklinghausen; Genetic disease
• ISSN: 0803-5253; 1651-2227
• DOI: 10.1111/j.1651-2227.2007.00401.x

Aim: Our aim was to detect the status of bone mineral density (BMD) in children with NF1, and thus to help the management of the skeletal complications of NF1.
Methods: Dual‐energy X‐ray absorptiometry (DEXA) was performed in lumbar spine, total body, proximal femur and forearm in 31 children (3.1–18 years) with NF1. Correlations among the BMD values of four regions were calculated statistically. Z‐scores of lumbar‐ and total body‐BMD were also evaluated in 24 patients at and older than 5 years.
Results: Eleven children had skeletal findings, including mild scoliosis in 5 patients. No case with total body‐Z score <–2 was detected. Lumbar‐Z score was lower than −2 in 3 out of 24 cases. Patients with any skeletal involvement of NF1 were likely to have a lumbar‐BMD lower than −2 in comparison with patients with no skeletal finding (odds ratio 4; 95% CI 0.01–4.62). Proximal femur‐BMD values (g/cm2), yet forearm‐BMDs, were correlated with both lumbar‐ and total body‐BMD, regardless of skeletal involvements of NF1.
Conclusions: Our findings suggest that lumbar‐ or proximal femur‐DEXA, rather than forearm‐ or total body‐DEXA, could reveal significantly decreased BMD in children with NF1, especially in those with skeletal involvement of NF1.