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Bone mineral density in children with neurofibromatosis 1

Kutluhan Yilmaz1, Meral Ozmen, Suleyman Bora Goksan

  • 1Pediatrician and Pediatric Neurologist, Department of Pediatrics, Gaziantep University, Faculty of Medicine, Turkey. drkutluhan@yahoo.com

Acta Paediatrica (Oslo, Norway : 1992)
|July 5, 2007
PubMed

Insights

Children with neurofibromatosis type 1 (NF1) may have decreased bone mineral density (BMD). Lumbar or proximal femur DEXA scans are recommended for detecting low BMD in NF1 patients, particularly those with skeletal issues.

Area of Science:

  • Pediatric Endocrinology
  • Skeletal Dysplasias
  • Medical Imaging

Background:

  • Neurofibromatosis type 1 (NF1) is a genetic disorder associated with various skeletal complications.
  • Bone mineral density (BMD) assessment is crucial for managing these complications.

Purpose of the Study:

  • To evaluate bone mineral density (BMD) status in children diagnosed with NF1.
  • To identify optimal imaging methods for detecting skeletal complications in pediatric NF1.

Main Methods:

  • Dual-energy X-ray absorptiometry (DEXA) was performed on the lumbar spine, total body, proximal femur, and forearm in 31 children with NF1 (ages 3.1-18 years).
  • Statistical analysis was used to calculate correlations between BMD values across different regions.
  • Z-scores for lumbar and total body BMD were assessed in 24 patients aged 5 years and older.

Main Results:

  • Eleven children exhibited skeletal findings, including mild scoliosis in five.
  • No patient had a total body-Z score below -2.
  • Three out of 24 patients had a lumbar-Z score below -2.
  • Patients with skeletal involvement were more likely to have a lumbar-BMD below -2 (OR 4).
  • Proximal femur BMD correlated with lumbar and total body BMD, unlike forearm BMD.

Conclusions:

  • Lumbar or proximal femur DEXA is more effective than forearm or total body DEXA in identifying decreased BMD in children with NF1.
  • These findings aid in the management of skeletal complications in pediatric NF1.
Abstract

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