Radiographic vertebral morphology: A diagnostic tool in pediatric osteoporosis
ABSTRACT To assess the value of spinal radiographs in determining the significance of reductions in bone mass or density in chronically ill children.
A pediatric scoring method for assessment of osteoporotic vertebral changes, developed on the basis of radiographs of 70 healthy controls and established adult scoring methods, was subsequently used to assess 32 pediatric patients with suspected secondary osteoporosis. Radiographic findings were correlated with bone mineral density (BMD), clinical data, and biochemistry.
Thirty-two patients (median age 14.1 years) were included. Assessment of spinal radiographs with the developed scoring method found previously undiagnosed spinal compression deformities in 11 patients (34%) of whom 9 were asymptomatic and 8 had lumbar spine (size-corrected) BMD measurements within +/-2.0 SD of the age- and sex-specific norms. Fracture history and cumulative glucocorticoid (GC) dose did not differ between those with and without compression deformities.
Vertebral compression fractures can be documented in a significant number of chronically ill children and are poorly predicted by single BMD measurements and clinical history. Assessment of vertebral morphology is recommended as an additional tool in the diagnostic workup of pediatric osteoporosis.
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ABSTRACT: Several factors can compromise optimal bone mass accrual during childhood and predispose to osteoporosis later in life. Patients with haemophilia are already at risk of low bone mass during childhood, partly due to reduced physical activity related to joint bleeds and haemarthrosis. The introduction of primary prophylaxis with regular infusions of the deficient coagulation factor has enabled reduction or prevention of haemophilic arthropathy. Finnish children with severe haemophilia start prophylaxis early and are encouraged to participate in physical activities. We hypothesized that prophylactic therapy would ensure normal childhood bone mass development and carried out a case-control study in 29 children with haemophilia (2 mild, 6 moderate, 21 severe) and 58 age-matched controls. Their bone health was determined by fracture history, blood and urine biochemistry, bone densitometry and spinal imaging. Bone mineral density was lower in children with haemophilia but there was no evidence for significantly increased fracture rate. The patients had significantly higher urinary calcium excretion and higher serum calcium concentration, and reduced bone resorption as compared with the controls. Our findings suggest primary skeletal pathology, resulting in increased urinary calcium loss and altered bone metabolism, which may over time contribute to the development of osteoporosis in patients with haemophilia.British Journal of Haematology 03/2011; 153(3):364-71. DOI:10.1111/j.1365-2141.2011.08639.x · 4.96 Impact Factor
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ABSTRACT: Shwachman-Diamond syndrome (SDS) is an autosomal recessive disorder characterized by exocrine pancreatic insufficiency and bone marrow dysfunction. These result in malabsorption and hematological abnormalities. A skeletal dysplasia is also an integral feature of SDS. The present study assessed prevalence and determinants of osteopenia and osteoporosis in patients with SDS and disease-causing mutations in the SBDS gene. Eleven patients (8 males) aged from 5 to 37 years (median 16.7 years) with a genetically confirmed diagnosis of SDS were assessed for fracture history, bone mineral content (BMC), lean tissue mass (LTM) and bone mineral density (BMD) (Hologic Discovery A), osteoporotic vertebral changes, and for blood biochemistry and hematological parameters. Iliac crest bone biopsies were obtained from four patients for histology and histomorphometry. The main findings were: (1) markedly reduced BMD Z-scores at the lumbar spine (median -2.1, range -4.4 to -0.8), proximal femur (median -1.3, range -2.2 to -0.7) and, whole body (median -1.0, range -2.8 to +0.6), and reduced Z-scores for height-adjusted BMC/LTM ratio (median -0.9, range -3.6 to +1.1); (2) vertebral compression fractures in three patients; and (3) blood biochemistry suggestive of mild vitamin D and vitamin K deficiency. Bone biopsies in four patients showed significant low-turnover osteoporosis with reduced trabecular bone volume, low numbers of osteoclasts and osteoblasts, and reduced amount of osteoid. The results suggest that in addition to the skeletal dysplasia, SDS is associated with a more generalized bone disease characterized by low bone mass, low bone turnover and by vertebral fragility fractures. Osteoporosis may result from a primary defect in bone metabolism, and could be related to the bone marrow dysfunction and neutropenia.Bone 01/2008; 41(6):965-72. DOI:10.1016/j.bone.2007.08.035 · 4.46 Impact Factor
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ABSTRACT: DXA scanner derived images of the spine are used for vertebral fracture detection in adults. It is unknown whether the method could be used in pediatrics. This study evaluated the diagnostic accuracy of DXA images in vertebral fracture assessment (VFA) in children. The study included 65 children (37 males; median age 12.1 years) with primary or secondary osteoporosis. Data on clinical history were collected from hospital records. Patients were assessed for spinal compression fractures by standard spinal radiographs and by bone densitometry (Hologic Discovery A) derived VFA images. The visibility and morphology of each vertebra in VFA images was assessed by two readers and by a semi-computerized software developed for the DXA scanner. The findings were compared with those in spinal radiographs and correlated with clinical parameters. The visibility of vertebrae in VFA images was good in T8-L4 but compromised in the upper thoracic region (T4-T7) and was constantly inferior to that in standard radiographs. A total of 25 vertebral fractures were diagnosed in radiographs, but only 9 (36%) of these also in VFA images. The semi-computerized software could not accurately detect vertebrae in most of the children; accuracy increased with increasing age, height and BMD but was not sufficient to detect vertebral fractures. The utility of DXA scanner derived images of the spine in vertebral fracture detection in children is limited by compromised visibility and poor diagnostic accuracy. The semi-computerized software is not suitable for pediatric use. These limitations should be kept in mind when assessing pediatric patients for osteoporosis.Bone 10/2007; 41(3):353-9. DOI:10.1016/j.bone.2007.05.012 · 4.46 Impact Factor