Bone metabolism and inflammatory characteristics in 14 cases of chronic nonbacterial osteomyelitis.

BACKGROUND: Chronic nonbacterial osteomyelitis (CNO) is a multifocal autoinflammatory disease that often impairs daily life in children. This study aimed to investigate the bone metabolic and inflammatory characteristics of patients with CNO, and to assess the differences between responders and nonresponders to conservative treatment. METHODS: We investigated the clinical symptoms; laboratory data including inflammatory and bone metabolic biomarkers; and imaging findings from plain radiography, magnetic resonance imaging (MRI), fluorodeoxyglucose-positron emission tomography (FDG-PET), and dual-energy x-ray absorption (DEXA) in 14 patients with CNO. All patients underwent first-line treatment comprising systemic nonsteroidal anti-inflammatory drugs with or without bisphosphonate. According to the response to the first-line treatment, the patients were divided into the clinical remission/partial response group and the no response group. The differences in bone metabolic and inflammatory characteristics between the two groups were assessed. RESULTS: All patients had low bone mineral density assessed with DEXA. The bone metabolic biomarkers (bone-specific alkaline phosphatase and tartrate-resistant acid phosphatase 5b) were increased in boys of all ages and in young girls. Multiple inflammatory regions were detected in all patients by using FDG-PET including asymptomatic regions. The no response group had higher immunoglobulin G (IgG) and a greater number of bone inflammatory lesions detected on MRI than the clinical remission/partial response group. CONCLUSION: Our data indicate the involvement of abnormal bone turnover, necessity of whole-body scanning, and association of higher serum IgG levels and greater numbers of inflammatory lesions with prolonged disease activity in patients with CNO.

Novel 2 radiographical measurements for atlantoaxial instability in children with Down syndrome.

STUDY DESIGN: A retrospective case-control study. OBJECTIVE: To assess the usefulness and reproducibility of 2 novel safe and simple radiographical measurements for atlantoaxial instability in children with Down syndrome. SUMMARY OF BACKGROUND DATA: In pediatric atlantoaxial instability, early diagnosis is important for improved outcomes because of poor postoperative recovery in progressed neurological symptoms. Conventional dynamic radiography of the cervical spine is associated with a potential risk of worsening neurological symptoms. METHODS: We retrospectively reviewed the medical records of 50 patients (24 boys and 26 girls) with atlantoaxial instability associated with Down syndrome. Of the 50 children, 11 had undergone and 4 had been scheduled for surgery (surgical group). In this investigation, in addition to the atlas-dens interval (ADI) and space available for spinal cord (SAC), we measured C1 inclination angle and C1/4 SAC ratio on lateral radiographs of the cervical spine in the neutral position. To assess the diagnostic abilities of these indices to determine indication for surgery, receiver operating characteristic analysis of each index was performed, and their diagnostic abilities were compared using the area under the receiver operating characteristic curve. Moreover, we assessed reproducibility of our 2 proposed indices. RESULTS: The discriminatory abilities of C1/4 SAC ratio (area under the receiver operating characteristic curve, 1.00) and C1 inclination angle (0.91) were comparable with those of ADI (0.98) and SAC (0.95). For the interobserver and intraobserver reliability of the novel indices, the correlation coefficients were in the range from 0.88 to 0.99. Correlation was observed between the ADI and C1/4 SAC ratio (r = 0.507, P < 0.01) and between the ADI and C1 inclination angle (r = 0.407, P < 0.01). CONCLUSION: The diagnostic abilities of the 2 novel radiographical measurements were comparable with those of ADI and SAC. Moreover, these novel measurements can be obtained safely on lateral radiographs of the cervical spine in the neutral position. LEVEL OF EVIDENCE: 4.