Concomitant deletion of the short arm (del(1p13.3)) and amplification or gain (1q21) of chromosome 1 by fluorescence in situ hybridization are associated with a poor clinical outcome in multiple myeloma.

BACKGROUND: Chromosome 1 abnormalities in multiple myeloma (MM) are increasingly recognized as high risk-defining features. The authors report the prognostic value of del(1p13.3) by fluorescence in situ hybridization (FISH) at enrollment in subjects treated on total therapy clinical trials 2-6. METHODS: FISH probes were generated from specific BAC DNA clones for the AHCYL1 gene locus (1p13.3) and the CKS1B locus (1q21). RESULTS: A total of 1133 patients were included in this analysis. Although del(1p13.3) was detected in 220 (19.4%) patients, 1q21gain or 1q21amp were observed in 300 (26.5%) and 150 (13.2%) patients, respectively. Concomitant del(1p13.3) with 1q21 gain or amp was observed in 65 (5.7%) and 29 (2.5%) patients, respectively. There was enrichment of high-risk features such as International Staging System (ISS) stage 3 disease and gene expression profiling (GEP)70 high risk (HR) in the group with del(1p13.3). Presence of del(1p13.3) confers inferior progression-free survival (PFS) and overall survival (OS). On multivariate analysis, the presence of ISS stage 3 disease, GEP70 HR, 1q21gain, and 1q21amp were independent predictors of PFS or OS. CONCLUSIONS: The PFS and OS of patients with combined abnormalities of del (1p13.3)/1q21gain or amp was significantly worse compared to del(1p13.3) alone and 1q21gain or 1q21 amp alone, which identifies a subset of patients with poor clinical outcomes.

Mechanical Testing of a Novel Fastening Device to Improve Scoliosis Bracing Biomechanics for Treating Adolescent Idiopathic Scoliosis.

Velcro fastening straps are commonly used to secure a scoliosis brace around the upper body and apply corrective forces to the spine. However, strap loosening and tension loss have been reported that reduce spinal correction and treatment efficacy. A novel fastening device, or controlled tension unit (CTU), was designed to overcome these limitations. A scoliosis analog model (SAM) was used to biomechanically compare the CTU fasteners and posterior Velcro straps on a conventional brace (CB) as well as on a modified brace (MB) that included a dynamic cantilever apical pad section. Brace configurations tested were (1) CB with posterior Velcro straps, (2) CB with posterior CTU fasteners, (3) MB with posterior Velcro straps, and (4) MB with posterior CTU fasteners. MB configurations were tested with 0 N, 35.6 N, and 71.2 N CTU fasteners applied across the apical pad flap. Three-dimensional forces and moments were measured at both ends of the SAM. The CTU fasteners provided the same corrective spinal loads as Velcro straps when tensioned to the same level on the CB configuration and can be used as an alternative fastening system. Dynamically loading the apical flap increased the distractive forces applied to the spine without affecting tension in the fastening straps.

The Risk of Curve Progression and Surgery in African Americans With Adolescent Idiopathic Scoliosis.

STUDY DESIGN: Retrospective clinical cohort study. OBJECTIVE: To determine if certain risk factors (age, curve magnitude, skeletal maturity, gender, and curve pattern) traditionally associated with curve progression and surgical intervention in the general population apply equally to African Americans. SUMMARY OF BACKGROUND DATA: Currently, information is limited on the role that a patient's race plays in the risk of curve progression of adolescent idiopathic scoliosis (AIS), and existing studies have conflicting results. METHODS: Retrospective search of records identified patients who were African American, had been diagnosed with AIS, had a major curve Cobb angle of 10 degrees or more, and had at least two clinical visits with spinal radiographs at least 90 days apart to determine the risk factors for surgical treatment, and 2 years apart to determine the risk factors for curve progression. Patients with a medical condition likely to cause scoliosis were excluded. RESULTS: Of 738 African American patients with AIS, 223 were assessed for surgical risk factors, and 72 were assessed for curve progression risk factors. Fifty-six (29.17%) had progression of the major coronal curve, and 38 (17.04%) underwent surgery. Age at presentation and curve magnitude at presentation were significant risk factors for surgical intervention. Curve magnitude at presentation was a significant risk factor for curve progression. No significant relationships were found for gender or curve type as they relate to surgical intervention or curve progression. CONCLUSION: Age and curve magnitude at presentation were significantly associated with surgery, as is true in other scoliosis populations. Curve magnitude at presentation was associated with curve progression. In contrast to studies in other populations, however, no significant association was observed between curve progression and age at presentation, curve type, or gender, or between surgery and curve type or gender. LEVEL OF EVIDENCE: Level III, prognostic cohort study.

Early Career Experience of Pediatric Orthopaedic Fellows: What to Expect and Need for Their Services.

INTRODUCTION: A dramatic increase in the number of pediatric orthopaedic fellows being trained has led to concerns that there may be an oversupply of pediatric orthopaedists. The purpose of this study was to determine whether this perception is accurate and whether the practice expectations of recent pediatric fellowship graduates are being met by surveying recent pediatric fellowship graduates about their early practice experiences. METHODS: A 36-question survey approved by the Pediatric Orthopaedic Society of North America (POSNA) leadership was electronically distributed to 120 recent graduates of pediatric orthopaedic fellowships; 81 responses were ultimately obtained (67.5% response rate). RESULTS: Almost all (91%) of the respondents were very or extremely satisfied with their fellowship experience. Half of the respondents had at least 1 job offer before they entered their fellowships. After completion of fellowships, 35% received 1 job offer and 62.5% received ≥2 job offers; only 2.5% did not receive a job offer. Most reported a practice consisting almost entirely of pediatric orthopaedics, and 93.5% thought this was in line with their expectations; 87% indicated satisfaction with their current volume of pediatric orthopaedics, and 85% with the complexity of their pediatric orthopaedic cases. Despite the high employment percentages and satisfaction with practice profiles, nearly a third (28%) of respondents replied that too many pediatric orthopaedists are being trained. CONCLUSIONS: Positive messages from this survey include the satisfaction of graduates with their fellowship training, the high percentage of graduates who readily found employment, and the satisfaction of graduates with their current practice environments; this indicates that the pediatric orthopaedic job environment is not completely saturated and there are continued opportunities for graduating pediatric fellows despite the increased number of fellows being trained. Although not determined by this study, it may be that the stable demand for pediatric orthopaedic services is being driven by the expansion of the scope of practice as well as subspecialization within the practice of pediatric orthopaedics.

Environmental and temporal factors affecting pediatric orthopaedic injuries.

Anecdotal evidence suggests that temporal and environmental factors may contribute to the variance in the volume of pediatric orthopaedic injuries (POI), and knowing the effect of these factors could help in the allocation of resources and personnel in pediatric emergency rooms. All POI presenting to a level 1 pediatric emergency department over a 3-year period were reviewed. Environmental data, including minimal, maximal, and average daily temperatures; amount of precipitation; and lunar phase, were obtained for the study region, as were day of the week and month and season of the year. Multiple logistic regression determined which variable or combination of variables might affect the rate of POI. In the 6770 POI seen over the study period (average 6.2 per day), the day of the week and lunar phase had no effect on the rate of POI, the presence of precipitation lowered the rate slightly, and temperature had a dramatic effect, with the highest number of injuries occurring in the average daily temperature range of 70° to 79°. Winter months of November through February had the lowest rates of POI, while May had the highest.

Progressive valgus deformity after curettage of benign lesions of the proximal tibia.

BACKGROUND: Progressive valgus deformity after proximal tibial metaphyseal fractures in pediatric patients has been well described, although the pathophysiology is not clear. We performed a retrospective study to evaluate the occurrence of progressive tibia valga after curettage of benign bone lesions of the tibia. METHODS: During a 6-year period, we performed curettage and bone grafting of benign bone lesions of the tibia in 20 patients aged 16 years and younger. Radiographs and clinical notes were reviewed. The diagnosis, age, sex, location of lesion, surgical approach, physeal arrest, limb-length discrepancy, deformity, and treatment were documented. RESULTS: Four patients subsequently developed progressive tibia valga (mean, 12 degrees; range, 7-21 degrees). Progressive valgus deformity occurred in 4 of 5 patients younger than 10 years old who had curettage of lesions of the proximal tibial metaphysis. Deformity did not occur in any of the 13 patients older than 10 years (range, 13-16 years) or in any of the 8 patients who had curettage of the distal tibia (age range, 6-14 years). In patients who developed tibia valga, the deformity progressed during the first 6 to 17 months postoperatively and then stabilized. One patient required corrective osteotomy at age 12 years for a symptomatic 21-degree deformity 3 years after curettage. The other 3 patients with tibia valga remained asymptomatic and demonstrated partial correction of their deformities, currently ranging from 3 to 7 degrees with a mean follow-up of 31 months (range, 19-47 months). CONCLUSIONS: Progressive tibia valga seem to be relatively common in patients younger than 10 years who have had curettage of the proximal tibial metaphysis. This potential sequela should be discussed thoroughly with the patient's parents before proceeding with surgery, and patients should be followed and evaluated for this postoperatively.