The Relationship Between Fractures and DXA Measures of BMD in the Distal Femur of Children and Adolescents With Cerebral Palsy or Muscular Dystrophy

Children with limited or no ability to ambulate frequently sustain fragility fractures. Joint contractures, scoliosis, hip dysplasia, and metallic implants often prevent reliable measures of bone mineral density (BMD) in the proximal femur and lumbar spine, where BMD is commonly measured. Further, the relevance of lumbar spine BMD to fracture risk in this population is questionable. In an effort to obtain bone density measures that are both technically feasible and clinically relevant, a technique was developed involving dual-energy X-ray absorptiometry (DXA) measures of the distal femur projected in the lateral plane. The purpose of this study is to test the hypothesis that these new measures of BMD correlate with fractures in children with limited or no ability to ambulate. The relationship between distal femur BMD Z-scores and fracture history was assessed in a cross-sectional study of 619 children aged 6 to 18 years with muscular dystrophy or moderate to severe cerebral palsy compiled from eight centers. There was a strong correlation between fracture history and BMD Z-scores in the distal femur; 35% to 42% of those with BMD Z-scores less than −5 had fractured compared with 13% to 15% of those with BMD Z-scores greater than −1. Risk ratios were 1.06 to 1.15 (95% confidence interval 1.04–1.22), meaning a 6% to 15% increased risk of fracture with each 1.0 decrease in BMD Z-score. In clinical practice, DXA measure of BMD in the distal femur is the technique of choice for the assessment of children with impaired mobility. © 2010 American Society for Bone and Mineral Researc

Revised Pediatric Reference Data for the Lateral Distal Femur Measured by Hologic Discovery/Delphi Dual-Energy X-Ray Absorptiometry

BackgroundLateral distal femur (LDF) scans by dual energy x-ray absorptiometry (DXA) are often feasible in children for whom other sites are not measurable. Pediatric reference data for LDF are not available for more recent DXA technology.AimsTo assess older pediatric LDF reference data, construct new reference curves for LDF bone mineral density (BMD), and demonstrate the comparability of LDF BMD to other measures of BMD and strength assessed by DXA and by peripheral quantitative computed tomography (pQCT).MethodsLDF, spine and whole body scans of 821 healthy children, 5 to 18 years of age, recruited at a single center were obtained using a Hologic Delphi/Discovery system. Tibia trabecular and total BMD (3% site), cortical geometry (38% site) (cortical thickness, section modulus, strain strength index) were assessed by pQCT. Sex and race-specific reference curves were generated using LMS-ChartMaker and Z-scores calculated and compared by correlation analysis.ResultsZ-scores for LDF BMD based on published findings demonstrated overestimation or underestimation of the prevalence of low BMD-for-age depending on the region of interest considered. Revised LDF reference curves were generated. The new LDF Z-scores were strongly and significantly associated with weight, BMI, spine and whole body BMD Z-scores, and all pQCT Z-scores.ConclusionThese findings demonstrate the comparability of LDF measurements to other clinical and research bone density assessment modes, and enable assessment of BMD in children with disabilities, who are particularly prone to low trauma fractures of long bones, and for whom traditional DXA measurement sites are not feasible

Bone mineral density in mucopolysaccharidosis IVB

To date, the only published reports of bone mineral density (BMD) in MPS IV involve patients with MPS IVA; no reports exist describing BMD for MPS IVB. In this prospective study of BMD in three patients with MPS IVB, BMD was acquired by dual-energy X-ray absorptiometry (DXA) at whole body (WB), lumbar spine (LS), and lateral distal femur (LDF). Functional abilities, ambulatory status, medical history, and height z-score were evaluated. Three patients with MPS IVB (two females), aged 17.7, 31.4 and 31.7 years, were evaluated. Every patient was ambulatory and one sustained two fractures caused by trauma. Whole body and hip DXA scans were technically invalid in every patient due to the presence of prosthetic hip hardware. Lumbar spine was valid in only 1 patient due skeletal abnormalities, and was normal (Z-score of −0.8). The LDF was valid in every patient and was low at all three regions of interest: average LDF z-scores were −3.1 (range, −2.9 to −3.6), −2.3 (range, −2.0 to −2.5), and −2.1 (range, −2.0 to −2.3) for region 1–region 3, respectively. Patients with MPS IVB have low BMD of the lower extremities even with full-time ambulation. Routine body sites to measure by DXA were problematic; hip and WB were invalid due to artifact, and LS had limited utility. The LDF was the only body site consistently available on all patients. Patients did not experience low-energy fractures despite low BMD

Newly Diagnosed Children with Cancer Have Lower 25-Vitamin D Levels than Their Cancer-Free Peers: A Comparison across Age, Race, and Sex

Children with cancer have a greater risk for vitamin D concerns because of compromised health before diagnosis, the disease itself, and treatments for the cancer. This IRB-approved retrospective, matched case–control study of children with and without cancer included three race categories: black, other, and Caucasian. This is the first study to directly compare serum 25-hydroxy vitamin D (25(OH)D) levels and status in newly diagnosed pediatric cancer patients with age-, sex-, and race-matched cancer-free children from the same geographic region of the US, all of whom are free from other conditions that negatively impact 25(OH)D levels. Univariable and multivariable ordinal logistic regressions were performed. In the 544 children (mean age of 8.5 years, 53% female), there were 136 newly diagnosed children with cancer and 408 matched non-cancer controls. Serum 25(OH)D levels at cancer diagnosis were lower (22.4 ng/mL) than in controls (30.1 ng/mL; p < 0.0001). Differences persisted across race (p < 0.001) and age (p < 0.001), but not sex. Older children exhibited lower 25(OH)D levels. Only 18.4% of the children with cancer had sufficient levels. Black children with cancer had the greatest rate of deficiency (39%). Race differences were evident: children of color (other and black) displayed higher levels of insufficiency; black children were most deficient

Bone Measurements by Peripheral Quantitative Computed Tomography (pQCT) in Children with Cerebral Palsy

OBJECTIVE: To use peripheral quantitative computed tomography (pQCT) to determine bone measurements in patients with cerebral palsy (CP) age 3 to 20 years and compare them with control subjects.STUDY DESIGN: A total of 13 (5 male) patients with CP, along with 2 sex- and age-matched controls for each, were included in a mixed-model analysis with matched pairs as random effects for pQCT bone measurements of the 20% distal tibia.RESULTS: Tibia length was similar in the CP and control groups (P = .57). Weight was marginally higher in the control group (P = .06). Cortical bone mineral content (BMC), area, thickness, polar strength-strain index (pSSI), and periosteal and endosteal circumferences were greater in the control group (P \u3c .05 for all). Relationships between bone measurements and weight showed that cortical BMC, area, periosteal circumference, and pSSI were greater at higher weights in the control group (group-by-weight interaction, P \u3c .05 for all). Cortical thickness was greater in the control group and was correlated with weight. Cortical volumetric bone mineral density (vBMD) was greater with higher weights in the CP group (group-by-weight interaction, P = .03).CONCLUSIONS: Bone strength, as indicated by pSSI, is compromised in children with CP due to smaller and thinner bones, not due to lower cortical bone density

Advanced Neuroimaging Approaches to Pediatric Brain Tumors

Central nervous system tumors are the most common pediatric solid tumors; they are also the most lethal. Unlike adults, childhood brain tumors are mostly primary in origin and differ in type, location and molecular signature. Tumor characteristics (incidence, location, and type) vary with age. Children present with a variety of symptoms, making early accurate diagnosis challenging. Neuroimaging is key in the initial diagnosis and monitoring of pediatric brain tumors. Conventional anatomic imaging approaches (computed tomography (CT) and magnetic resonance imaging (MRI)) are useful for tumor detection but have limited utility differentiating tumor types and grades. Advanced MRI techniques (diffusion-weighed imaging, diffusion tensor imaging, functional MRI, arterial spin labeling perfusion imaging, MR spectroscopy, and MR elastography) provide additional and improved structural and functional information. Combined with positron emission tomography (PET) and single-photon emission CT (SPECT), advanced techniques provide functional information on tumor metabolism and physiology through the use of radiotracer probes. Radiomics and radiogenomics offer promising insight into the prediction of tumor subtype, post-treatment response to treatment, and prognostication. In this paper, a brief review of pediatric brain cancers, by type, is provided with a comprehensive description of advanced imaging techniques including clinical applications that are currently utilized for the assessment and evaluation of pediatric brain tumors

The Utility of DXA Assessment at the Forearm, Proximal Femur, and Lateral Distal Femur, and Vertebral Fracture Assessment in the Pediatric Population: The 2019 Official Pediatric Positions of the ISCD.

Dual-energy X-ray absorptiometry (DXA) is widely used in the evaluation of bone fragility in children. Previous recommendations emphasized total body less head and lumbar spine DXA scans for clinical bone health assessment. However, these scan sites may not be possible or optimal for all groups of children with conditions that threaten bone health. The utility of DXA scans of the proximal femur, forearm, and radius were evaluated for adequacy of reference data, precision, ability of predict fracture, and applicability to all, or select groups of children. In addition, the strengths and limitations of vertebral fracture assessment by DXA were evaluated. The new Pediatric Positions provide guidelines on the use of these additional measures in the assessment of skeletal health in children