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Clubfoot describes a congenital condition. If untreated, clubfoot can cause long-term functional issues. The Ponseti method is the gold-standard treatment; it emphasizes casting over surgery. We identified a high rate of major recurrence in patients with isolated clubfoot at our institution. We implemented a quality improvement intervention to address the recurrences., Methods: We established a clubfoot program that aimed to (1) develop a standardized treatment pathway; (2) improve care team education; (3) improve coordination of care with families; and (4) improve documentation. The purpose of this study was to outline our quality improvement intervention and evaluate its success. Data were retrospectively collected from isolated clubfeet before (2003-2007, phase I) and after (2012-2014, phase II) implementation of the clubfoot program. We compared the differences in treatment and major recurrence between the 2 phases using generalized logistic or linear mixed models. Modified Poisson regression models were used to evaluate the association between provider nonadherence and recurrence., Results: The pre- (phase I) and post- (phase II) implementation groups included 91 patients (131 feet) and 68 patients (101 feet), respectively. The incidence of major recurrence (odds ratio: 59.5, 95% confidence interval: 7.8-454.4, P < 0.0001) was lower during phase II compared to phase I. Nonadherence with the care pathway was associated with an increased risk of recurrence (risk ratio: 4.1, 95% confidence interval: 1.2-14.3, P = 0.0274)., Conclusions: The implementation of a clubfoot program was associated with a decrease in major clubfoot surgery and improved adherence to established guidelines for clubfoot management., Competing Interests: The authors have no financial interest to declare in relation to the content of this article., (Copyright © 2020 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: The relationship between Fassier-Duval (FD) rod placement and rod failure rates has not previously been quantified., Methods: Retrospective review was conducted on patients with osteogenesis imperfecta treated with FD rods between 2005 and 2017. Age at first surgery, sex, Sillence type of osteogenesis imperfecta, bisphosphonate treatment, location of rod (side of body and specific bone), and dates of surgeries, radiographs, and rod failures were collected. C-arm images determined rod fixation within the distal epiphysis at the time of surgery. C-arm variables included rod deviation (percent deviation from the midline of the distal epiphysis) and anatomical direction of deviation (anterior/posterior and medial/lateral). X-ray images were examined for rod failure, which was defined as bending, pulling out of the physis, protrusion out of the bone, and/or failure to telescope. Cox proportional hazards regression models were used to compare failure rates with location of placement within the distal epiphysis allowing for clustering of the data by side (left or right) and bone (femur or tibia)., Results: The cohort was 13 patients (11 female individuals and 2 male individuals) with a total of 66 rods and 75 surgeries. Mean time from the first surgery to the last follow-up visit was 8.9 years (SD=5 y). There was a 7% increase in hazard of failure per 1-mm increase in antero-posterior (AP) deviation [hazard ratio (HR), 1.07; 95% confidence interval (CI), 1.01-1.14; P=0.029)]. Similarly, there was a 9% increase in hazard of failure for every 1-mm increase in lateral deviation (HR, 1.09; 95% CI, 1.01-1.18; P=0.019). A 12% increase in hazard of failure per 10% increase in deviation from the midline for both AP and lateral radiograph views was also found, although this was only statistically significant for lateral deviation on the AP radiograph view (HR, 1.12; 95% CI, 1.01-1.25; P=0.030)., Conclusions: FD rod placement within the distal epiphysis has significant impact on increasing rod survival., Level of Evidence: Level III-therapeutic study.

We estimate the causal effects of acute fine particulate matter exposure on mortality, health care use, and medical costs among the US elderly using Medicare data. We instrument for air pollution using changes in local wind direction and develop a new approach that uses machine learning to estimate the life-years lost due to pollution exposure. Finally, we characterize treatment effect heterogeneity using both life expectancy and generic machine learning inference. Both approaches find that mortality effects are concentrated in about 25 percent of the elderly population.

Background: Cessation counseling and pharmacotherapy are recommended for hospitalized smokers, but better coordination between cessation counselors and providers might improve utilization of pharmacotherapy and enhance smoking cessation., Objective: To compare smoking cessation counseling combined with care coordination post-hospitalization to counseling alone on uptake of pharmacotherapy and smoking cessation., Design: Unblinded, randomized clinical trial PARTICIPANTS: Hospitalized smokers referred from primarily rural hospitals INTERVENTIONS: Counseling only (C) consisted of telephone counseling provided during the hospitalization and post-discharge. Counseling with care coordination (CCC) provided similar counseling supplemented by feedback to the smoker's health care team and help for the smoker in obtaining pharmacotherapy. At 6 months post-hospitalization, persistent smokers were re-engaged with either CCC or C., Main Measures: Utilization of pharmacotherapy and smoking cessation at 3, 6, and 12 months post-discharge., Key Results: Among 606 smokers randomized, 429 (70.8%) completed the 12-month assessment and 580 (95.7%) were included in the primary analysis. Use of any cessation pharmacotherapy between 0 and 6 months (55.2%) and between 6 and 12 months (47.1%) post-discharge was similar across treatment arms though use of prescription-only pharmacotherapy between months 6-12 was significantly higher in the CCC group (30.1%) compared with the C group (18.6%) (RR, 1.61 (95% CI, 1.08, 2.41)). Self-reported abstinence rates of 26.2%, 20.3%, and 23.4% at months 3, 6, and 12, respectively, were comparable across the two treatment arms. Of those smoking at month 6, 12.5% reported abstinence at month 12. Validated smoking cessation at 12 months was 19.3% versus 16.9% in the CCC and C groups, respectively (RR, 1.13 (95% CI, 0.80, 1.61))., Conclusion: Supplemental care coordination, provided by counselors outside of the health care team, failed to improve smoking cessation beyond that achieved by cessation counseling alone. Re-engagement of smokers 6 months post-discharge can lead to new quitters, at which time care coordination might facilitate use of prescription medications., Trial Registration: NCT01063972.

Background: The survival of Fassier-Duval (FD) telescoping rods as compared with static implants in children affected by osteogenesis imperfecta is not well characterized. The purpose of this study was to compare risk of lower extremity implant failure in FD rods versus static implants., Methods: Data were retrospectively collected from patients with osteogenesis imperfecta who underwent surgical treatment using either FD rods or static implants (Rush rods, flexible nails, or Steinmann pins) between 1995 and 2015. The timing of implant failure was the primary outcome variable of interest. Comparisons were limited to limbs with no previous history of implants. Cox-proportional hazards regression analyses were used to compare the hazard of implant failure across implants. Negative binomial regression analyses were used to compare the incidence of surgical procedures in the 2 implant groups., Results: The final cohort consisted of 64 limbs (n=21 patients). The static implant group (n=38) consisted of 24 Rush rods (63%), 14 flexible nails (37%), and 2 Steinmann pins (5%). The hazard of implant failure in the static implant group was 13.2 times [95% confidence interval (CI), 2.5-69.6; P=0.0024] the hazard of implant failure in the FD rod group. The hazard of implant failure among females was 4.8 (95% CI, 1.4-16.7; P=0.0125) times the hazard of implant failure among males. The total surgery rate in the static implant group was 7.8 (95% CI, 1.8-33.0; P=0.0056) times the total surgery rate in the FD group., Conclusions: Among surgically naive limbs, FD rods were associated with significantly improved probability of survival compared with static implants., Level of Evidence: Level II-retrospective study.

Genetic factors predictive of severe adolescent idiopathic scoliosis (AIS) are largely unknown. To identify genetic variation associated with severe AIS, we performed an exome-wide association study of 457 severe AIS cases and 987 controls. We find a missense SNP in SLC39A8 (p.Ala391Thr, rs13107325) associated with severe AIS (P = 1.60 × 10 -7 , OR = 2.01, CI = 1.54-2.62). This pleiotropic SNP was previously associated with BMI, blood pressure, cholesterol, and blood manganese level. We replicate the association in a second cohort (841 cases and 1095 controls) resulting in a combined P = 7.02 × 10 -14 , OR = 1.94, CI = 1.63-2.34. Clinically, the minor allele of rs13107325 is associated with greater spinal curvature, decreased height, increased BMI and lower plasma manganese in our AIS cohort. Functional studies demonstrate reduced manganese influx mediated by the SLC39A8 p.Ala391Thr variant and vertebral abnormalities, impaired growth, and decreased motor activity in slc39a8 mutant zebrafish. Our results suggest the possibility that scoliosis may be amenable to dietary intervention.

Case: A 19-year-old adolescent with Kniest dysplasia was incidentally found to have osteosarcoma of the proximal aspect of the humerus after having a chest radiograph for evaluation of an upper respiratory infection. He underwent chemotherapy and resection of the tumor, and there was no evidence of metastasis at the 16-month follow-up., Conclusion: Patients with osteochondrodysplasias often have multiple orthopaedic symptoms. Changes in chronic pain symptoms should be investigated to rule out insidious secondary causes of musculoskeletal pain, such as osteosarcoma.

Objectives: To use real-time ultrasonography to estimate the prevalence of persistent median arteries in a cohort of pediatric orthopedic patients., Methods: With Institutional Review Board approval, patients between the ages of 3 months and 19 years were recruited for this cross-sectional study. Variables of interest included demographics, underlying diagnoses, and the presence of a Doppler-confirmed median artery. Ultrasonographic examinations were performed on both upper extremities by a single investigator. Patient- and limb-level analyses were performed. A multivariable generalized logistic regression analysis was used to test the association between the prevalence of limb-specific persistent median arteries and age. A generalized estimating equation was used to adjust for the inclusion of multiple limbs from the same patient., Results: A total of 135 patients (270 limbs) were evaluated. The patient-specific prevalence rate (persistent median arteries present in 1 or both limbs) was 26.7%. Among these patients (n = 36), a persistent median artery was present bilaterally in 55.6% (n = 20). The limb-specific prevalence rate (proportion of limbs with a persistent median artery) was 20.7%. After adjusting for race, for every 1-year increase in age, the odds of a persistent median artery decreased by 4.4%. After adjusting for age, African Americans were significantly more likely to present with a persistent median artery (odds ratio, 3.78; 95% confidence interval, 1.25-11.48)., Conclusions: Ultrasonography can effectively visualize anatomic variants, such as persistent median arteries, in the pediatric population. The prevalence of persistent median arteries was higher than anticipated, especially among African American patients., (© 2018 by the American Institute of Ultrasound in Medicine.)

Idiopathic scoliosis (IS) is a structural lateral spinal curvature of ≥10° that affects up to 3% of otherwise healthy children and can lead to life-long problems in severe cases. It is well-established that IS is a genetic disorder. Previous studies have identified genes that may contribute to the IS phenotype, but the overall genetic etiology of IS is not well understood. We used exome sequencing to study five multigenerational families with IS. Bioinformatic analyses identified unique and low frequency variants (minor allele frequency ≤5%) that were present in all sequenced members of the family. Across the five families, we identified a total of 270 variants with predicted functional consequences in 246 genes, and found that eight genes were shared by two families. We performed GO term enrichment analyses, with the hypothesis that certain functional annotations or pathways would be enriched in the 246 genes identified in our IS families. Using three complementary programs to complete these analyses, we identified enriched categories that include stereocilia and other actin-based cellular projections, cilia and other microtubule-based cellular projections, and the extracellular matrix (ECM). Our results suggest that there are multiple paths to IS and provide a foundation for future studies of IS pathogenesis., (Copyright © 2018 Baschal et al.)

Background: The appropriate intervention for hip subluxation or dislocation in children affected by cerebral palsy (CP) remains controversial. The purpose of this retrospective study was to report radiographic and clinical outcomes following isolated femoral varus derotational osteotomy (VDRO) in children with CP hip dysplasia. Risk factors for resubluxation and avascular necrosis (AVN) were also examined., Methods: A cohort of 100 patients (199 hips) with CP treated with isolated VDRO between 2003 and 2009 was reviewed. All but 1 patient received bilateral surgery. Patients were followed for an average of 5.4 years (range, 1.03 to 10.20 y). Anteroposterior pelvic radiographs were used to assess migration percentage (MP), Shenton's line, and presence of AVN. Resubluxation was defined as a postoperative break in Shenton's line. Radiographic outcomes and risk analysis was performed in the 91 subjects (179 hips) with radiographic follow-up >1 year., Results: Significant improvement was observed in MP, and all hips had a reconstituted Shenton's line following surgery. Over the course of follow-up, 16% of hips were noted to have a repeat break in Shenton's line. Univariate risk analysis showed preoperative MP, Gross Motor Function Classification System (GMFCS) level, and age at surgery were risk factors for a recurrent line break. Preoperative MP and GMFCS level were found to be predictors of resubluxation in multivariate analysis. AVN was detected in 10 hips (5.7%). GMFCS level V patients were more at risk for resubluxation, but less at risk for AVN when compared with ambulatory (GMFCS I/II/II) patients and GMFCS level IV patients., Conclusions: Performing a VDRO without additional procedures provided a stable and concentrically reduced hip joint in this population of children with CP. Attention should be paid to initial ambulatory status during the postoperative period. Concomitant procedures such as pelvic osteotomy should be considered for patients of GMFCS level IV and V, as these patients were more at risk for recurrent subluxation., Level of Evidence: Level III-retrospective comparative study.

Background: To evaluate lower extremity strength and alignment among children who underwent submuscular plating (SMP)., Methods: Subjects who underwent SMP for a length unstable femoral fracture returned for isokinetic strength testing ≥2 years after surgery. Extensor and flexor strength deficits (percent difference) between the operative and nonoperative limbs were evaluated. Radiographic measurements of mechanical alignment [anatomic lateral distal femoral angle (aLDFA)] and qualitative measurements (The Pediatric Outcomes Data Collection Instrument, PODCI) were obtained from all subjects. The upper 97.5% confidence interval approach to establishing clinical equivalency was utilized to compare differences in strength and alignment between the operative and nonoperative limbs. An extensor strength deficit of >19% and an aLDFA discrepancy of >5 degrees were considered to be clinically significant., Results: The average age at surgery of the 10 subjects included in the study was 8.7 years. The hardware was placed an average of 27.9 mm from the distal femoral physis and was removed 6.4 months postsurgery. Among all subjects, the median PODCI scores were ≥97 according to all subscales. There was no significant difference in extension torque between the operative versus nonoperative limbs at 60 degrees/s (P=0.5400), 120 degrees/s (P=0.4214), or 180 degrees/s (P=0.8166). More importantly, extension strength deficits between the operative and nonoperative limbs were not clinically significant at 60 degrees/s [upper 97.5% confidence interval (CI), 10.9%], 120 degrees/s (upper 97.5% CI, 11.0%), or 180 degrees/s (upper 97.5% CI, 10.7%). The difference in aLDFA between the operative and nonoperative limb was less than the predefined clinically significant threshold of 5 degrees for all subjects., Conclusions: SMP achieves satisfactory clinical and functional results. In this series, extensor strength deficits and/or lower extremity malalignment were not clinically meaningful. High patient satisfaction can be expected after implant removal., Level of Evidence: Level IV-case series.

The growth of an organism is highly dependent on the acquisition of carbon and metals, and availability of these nutrients in the environment affects its survival. Organisms can obtain both nutrients simultaneously through proteins of the CitMHS superfamily. Bioinformatic studies suggested a CitMHS gene (Accession number ABS03965.1) in Kineococcus radiotolerans. Radio flux assays following 14-C radiolabelled citrate, either free or complexed to a variety of metal ions, in K. radiotolerans demonstrated internalization of the citrate when bound to select metal ions only, primarily in the form of calcium-citrate. A pH response was also observed, consistent with a permease (ATP independent) mechanism as noted for other CitMHS family members, with greater uptake at pH 7 compared to pH 10. These results confirm the ability of K. radiotolerans to transport complexed citrate., (© 2017 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.)

Background: Optimization of the electronic medical record (EMR) is essential to support the clinician and to improve the quality and efficiency of patient care. The present report describes the development and implementation of a standardized template that is embedded in the EMR and is focused on a comprehensive physical examination during the evaluation of pediatric supracondylar humeral fractures. We compared the completeness of physical examinations as well as the timing of detection and documentation of neurovascular injuries before and after implementation of the template. We hypothesized that the use of a template would increase the completeness of examinations and would lead to earlier documentation of neurovascular injuries., Methods: A multidisciplinary quality-improvement task force was created to address neurovascular documentation practices for patients who underwent operative treatment of supracondylar humeral fractures. Following a series of formative and process evaluations, a standardized EMR template was implemented. Neurovascular examination documentation practices that were in use before (pre-template group, n = 224) and after (template group, n = 300) the implementation of the template were compared. Logistic regression analyses of the 2 groups were used to compare the likelihood of a complete neurovascular examination and the timing of neurovascular injury identification., Results: There was significant improvement in the documentation of the vascular (odds ratio [OR], 70.7; 95% confidence interval [CI], 39.5 to 126.6; p < 0.0001), motor (OR, 17.6; 95% CI, 9.5 to 32.7; p < 0.0001), and sensory (OR, 23.9; 95% CI, 12.9 to 44.4; p < 0.0001) examinations in the template group. Neurological injuries were more likely to be identified preoperatively in the template group compared with the pre-template group (OR, 6.8; 95% CI, 1.7 to 27.1; p = 0.0067)., Conclusions: The incorporation of a standardized template in the EMR improved the completeness and timing of documentation of neurological injury. Standardized EMR templates developed by a clinically driven multidisciplinary task force have the potential to improve the quality of clinical documentation and to ease communication among providers., Level of Evidence: Level III. See Instructions for Authors for a complete description of levels of evidence.

Background: Idiopathic patellofemoral pain (PFP) has been linked to hip weakness and abnormal lower extremity mechanics. The effect of a strengthening intervention on balance has not been well studied among individuals with PFP., Hypothesis/purpose: The primary aim of this study was to evaluate changes in center of pressure displacement during the single limb squat following a nine-week physical therapy intervention among adolescent females with PFP., Study Design: Interventional and cross-sectional., Methods: Seven adolescent females with PFP (10 extremities) were included in the study. Center of Pressure (CoP) excursions during a single limb squat task were measured before and after a nine week of physical therapy intervention focused on strengthening of the hip and core. Seven asymptomatic females were matched to the PFP group on the basis of age and activity level, and were tested as a reference group. CoP trajectories were reduced into four variables: mean distance (MDIST), root-mean-square distance (RDIST), range (RANGE), and 95% confidence interval circle area (AREA-CC). Maximum knee flexion angle, peak knee power generation and absorption were also recorded. Linear mixed models were used to test for within and between group differences in CoP metrics., Results: Pre-intervention, CoP range, knee power absorption and generation were significantly decreased in the PFP group relative to the reference group. Post-intervention, the PFP group reported a significant decrease in symptom severity. There was also a significant (p<0.05) increase in MDIST, RDIST, RANGE, AREA-CC, peak knee flexion angle, peak power absorption and power generation. There was no difference (p>0.05) in knee flexion, knee power or CoP displacement between the two groups after the physical therapy intervention., Conclusion: Hip and core-strengthening resulted in a significant decrease in symptom severity as well as significant reductions in CoP displacement., Level of Evidence: 3.

To identify factors predictive of the risk of conversion from closed to open reduction. International Classification of Disease-9 codes were used to identify completely displaced pediatric supracondylar humerus fractures that were subjected to planned closed reduction and percutaneous pinning. Clinical and radiographic variables were retrospectively collected. Compared with posterior extension fractures, flexion (risk ratio: 34.1, 95% confidence interval: 8.1-143.6, P<0.0001) and posterolateral extension (risk ratio: 6.0, 95% confidence interval: 1.3-27.5, P=0.0221) fractures were significantly more likely to undergo conversion from closed to open reduction. The direction of displacement should be considered during the preoperative evaluation of supracondylar fractures.

Because of genetic heterogeneity present in idiopathic scoliosis, we previously defined clinical subsets (a priori) from a sample of families with idiopathic scoliosis to find genes involved with spinal curvature. Previous genome-wide linkage analysis of seven families with at least two individuals with kyphoscoliosis found linkage (P-value = 0.002) in a 3.5-Mb region on 5p13.3 containing only three known genes, IRX1, IRX2, and IRX4 In this study, the exons of IRX1, IRX2, and IRX4, the conserved noncoding elements in the region, and the exons of a nonprotein coding RNA, LOC285577, were sequenced. No functional sequence variants were identified. An intrafamilial test of association found several associated noncoding single nucleotide variants. The strongest association was with rs12517904 (P = 0.00004), located 6.5 kb downstream from IRX1 In one family, the genotypes of nine variants differed from the reference allele in all individuals with kyphoscoliosis, and two of three individuals with scoliosis, but did not differ from the reference allele in all other genotyped individuals. One of these variants, rs117273909, was located in a conserved noncoding region that functions as an enhancer in mice. To test whether the variant allele at rs117273909 had an effect on enhancer activity, zebrafish transgenesis was performed with overlapping fragments of 198 and 687 bp containing either the wild type or the variant allele. Our data suggests that this region acts as a regulatory element; however, its size and target gene(s) need to be identified to determine its role in idiopathic scoliosis., (Copyright © 2016 Justice et al.)

Adolescent idiopathic scoliosis (AIS) is a complex inherited spinal deformity whose etiology has been elusive. While common genetic variants are associated with AIS, they explain only a small portion of disease risk. To explore the role of rare variants in AIS susceptibility, exome sequence data of 391 severe AIS cases and 843 controls of European ancestry were analyzed using a pathway burden analysis in which variants are first collapsed at the gene level then by Gene Ontology terms. Novel non-synonymous/splice-site variants in extracellular matrix genes were significantly enriched in AIS cases compared with controls (P = 6 × 10(-9), OR = 1.7, CI = 1.4-2.0). Specifically, novel variants in musculoskeletal collagen genes were present in 32% (126/391) of AIS cases compared with 17% (146/843) of in-house controls and 18% (780/4300) of EVS controls (P = 1 × 10(-9), OR = 1.9, CI = 1.6-2.4). Targeted resequencing of six collagen genes replicated this association in combined 919 AIS cases (P = 3 × 10(-12), OR = 2.2, CI = 1.8-2.7) and revealed a highly significant single-gene association with COL11A2 (P = 6 × 10(-9), OR = 3.8, CI = 2.6-7.2). Importantly, AIS cases harbor mainly non-glycine missense mutations and lack the clinical features of monogenic musculoskeletal collagenopathies. Overall, our study reveals a complex genetic architecture of AIS in which a polygenic burden of rare variants across extracellular matrix genes contributes strongly to risk., (© The Author 2015. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Background: Despite being recognized as the gold standard in isolated clubfoot treatment, the Ponseti casting method has yielded variable results. Few studies have directly compared common predictors of treatment failure between institutions with high versus low failure rates., Questions/purposes: We asked: (1) is the provider's rigid adherence to the Ponseti method associated with a lower likelihood of unplanned clubfoot surgery, and (2) at the institution that did not adhere rigidly to Ponseti's principles, are any demographic or treatment-related factors associated with increased likelihood of unplanned clubfoot surgery?, Methods: After institutional review board approval, a consecutive series of patients with a diagnosis of isolated clubfoot who underwent treatment between January 2003 and December 2007 were identified. At Institution 1, 91 of 133 patients met the eligibility criteria and were followed for a minimum of 2 years compared with 58 of 58 patients at Institution 2. At Institution 1, 16 providers managed care using a conservative casting approach based on the Ponseti method. However, treatment was adapted by the provider(s). At Institution 2, one orthopaedic surgeon managed care with strict adherence to the Ponseti method. Surgical indications at both institutions included the presence of a persistent equinovarus foot position while standing. A chart review was used to collect data related to proportion of patients undergoing unplanned additional treatment for deformity recurrences after Ponseti casting, demographics, and treatment patterns., Results: The proportion of subjects who underwent unplanned major surgical intervention was greater (odds ratio [OR], 51.1; 95% CI, 6.8-384.0; p < 0.001) at Institution 1 (60 of 131, 47%) compared with Institution 2 (two of 91, 2%). There was no difference (p = 0.200) in the proportion of patients who underwent additional casting, repeat tendo Achilles lengthening, and/or anterior tibialis tendon transfer only (minor recurrence) at Institution 1 (nine of 131, 7%) compared with Institution 2 (11 of 91, 13%). At Institution 1, an increase in the number of revision casts (multiple vs no casts, hazard ratio [HR] = 3.9; 95% CI, 2.0-7.6; p < 0.001) and an increase in the number of cast-related complications (multiple vs no complications, HR = 2.8; 95% CI, 1.2-6.7; p = 0.019) were associated with increased risk of major surgery in the multivariate analysis., Conclusions: Rigid commitment to the Ponseti method in the conservative treatment of patients with isolated clubfoot was associated with a lower risk of subsequent unplanned surgical intervention. In addition, clubfoot treatment programs that use a care model that prioritizes continuity in care and dedication to the Ponseti method may decrease the proportion of patients who undergo unplanned surgical intervention., Level of Evidence: Level III, therapeutic study.

Background: Although venous thromboembolism (VTE) has been well studied in the pediatric trauma population, rates of VTE associated with elective pediatric orthopaedic procedures have not been addressed in current literature. The purpose of this retrospective study was to identify the incidence of VTE in the elective pediatric orthopaedic surgical population and delineate subsets of this population at greatest risk. This study may provide valuable data to begin the process of resolving the controversy surrounding deep vein thrombosis prophylaxis in the pediatric orthopaedic population., Methods: The Pediatric Health Information System was queried for patients admitted on an ambulatory or inpatient basis, aged below 18 years, from January 2006 to March 2011 during which an elective orthopaedic surgery was the principal procedure performed. Patients with diagnoses or procedures related to infection, trauma, malignancy, or coagulopathies were excluded. Patients admitted through the emergency department or whose orthopaedic procedure was not performed on the admission date were excluded. Age, sex, ethnicity, race, admission year, and all procedures/diagnoses were recorded. The presence of VTE at the index admission or any subsequent readmission within 90 days was recorded. All criteria were coded using ICD-9-CM codes. Generalized logistic regression analyses were used to identify factors related to VTE., Results: A total of 143,808 admissions (117,676 patients) matched the inclusion criteria. Thirty-three had a VTE during the index admission with an additional 41 at subsequent readmissions, for a total incidence of 0.0515% by admission and 0.0629% by patient. In the multivariable model, variables significantly (P<0.05) related to VTE included increasing age, admission type, diagnosis of metabolic conditions, obesity, and/or syndromes, and complications of implanted devices and/or surgical procedures. No procedure variables were significantly related to VTE in the multivariable model., Conclusions: The incidence of VTE in this cohort of pediatric patients undergoing elective orthopaedic surgery was 0.0515%. In children, underlying diagnosis seems to be a stronger predictor of VTE than procedures performed. Diagnosis with a metabolic condition, syndrome, and/or obesity, complications of implanted devices and/or surgical procedures, older age, and admission as an inpatient were significantly related to the development of a VTE., Level of Evidence: Level IV—case series.

Study Design: A hypothesis-driven study was conducted in a familial cohort to determine the potential association between variants within the TBX6 gene and Familial Idiopathic Scoliosis (FIS)., Objective: To determine if variants within exons of the TBX6 gene segregate with the FIS phenotype within a sample of families with FIS., Summary of Background Data: Idiopathic Scoliosis (IS) is a structural curvature of the spine whose underlying genetic etiology has not been established. IS has been reported to occur at a higher rate than expected in family members of individuals with congenital scoliosis (CS), suggesting that the two diseases might have a shared etiology. The TBX6 gene on chromosome 16p, essential to somite development, has been associated with CS in a Chinese population. Previous studies have identified linkage to this locus in families with FIS, and specifically with rs8060511, located in an intron of the TBX6 gene., Methods: Parent-offspring trios from 11 families (13 trios, 42 individuals) with FIS were selected for Sanger sequencing of the TBX6 gene. Trios were selected from a large population of families with FIS in which a genome-wide scan had resulted in linkage to 16p., Results: Sequencing analyses of the subset of families resulted in the identification of five coding variants. Three of the five variants were novel; the remaining two variants were previously characterized and account for 90% of the observed variants in these trios. In all cases, there was no correlation between transmission of the TBX6 variant allele and FIS phenotype. However, an analysis of regulatory markers in osteoblasts showed that rs8060511 is in a putative enhancer element., Conclusions: Although this study did not identify any TBX6 coding variants that segregate with FIS, we identified a variant that is located in a potential TBX6 enhancer element. Therefore, further investigation of the region is needed.

Idiopathic scoliosis occurs in 3% of individuals and has an unknown etiology. The objective of this study was to identify rare variants that contribute to the etiology of idiopathic scoliosis by using exome sequencing in a multigenerational family with idiopathic scoliosis. Exome sequencing was completed for three members of this multigenerational family with idiopathic scoliosis, resulting in the identification of a variant in the HSPG2 gene as a potential contributor to the phenotype. The HSPG2 gene was sequenced in a separate cohort of 100 unrelated individuals affected with idiopathic scoliosis and also was examined in an independent idiopathic scoliosis population. The exome sequencing and subsequent bioinformatics filtering resulted in 16 potentially damaging and rare coding variants. One of these variants, p.Asn786Ser, is located in the HSPG2 gene. The variant p.Asn786Ser also is overrepresented in a larger cohort of idiopathic scoliosis cases compared with a control population (P = 0.024). Furthermore, we identified additional rare HSPG2 variants that are predicted to be damaging in two independent cohorts of individuals with idiopathic scoliosis. The HSPG2 gene encodes for a ubiquitous multifunctional protein within the extracellular matrix in which loss of function mutation are known to result in a musculoskeletal phenotype in both mouse and humans. Based on these results, we conclude that rare variants in the HSPG2 gene potentially contribute to the idiopathic scoliosis phenotype in a subset of patients with idiopathic scoliosis. Further studies must be completed to confirm the effect of the HSPG2 gene on the idiopathic scoliosis phenotype., (Copyright © 2015 Baschal et al.)

Adolescent idiopathic scoliosis (AIS) causes spinal deformity in 3% of children. Despite a strong genetic basis, few genes have been associated with AIS and the pathogenesis remains poorly understood. In a genome-wide rare variant burden analysis using exome sequence data, we identified fibrillin-1 (FBN1) as the most significantly associated gene with AIS. Based on these results, FBN1 and a related gene, fibrillin-2 (FBN2), were sequenced in a total of 852 AIS cases and 669 controls. In individuals of European ancestry, rare variants in FBN1 and FBN2 were enriched in severely affected AIS cases (7.6%) compared with in-house controls (2.4%) (OR = 3.5, P = 5.46 × 10(-4)) and Exome Sequencing Project controls (2.3%) (OR = 3.5, P = 1.48 × 10(-6)). Scoliosis severity in AIS cases was associated with FBN1 and FBN2 rare variants (P = 0.0012) and replicated in an independent Han Chinese cohort (P = 0.0376), suggesting that rare variants may be useful as predictors of curve progression. Clinical evaluations revealed that the majority of AIS cases with rare FBN1 variants do not meet diagnostic criteria for Marfan syndrome, though variants are associated with tall stature (P = 0.0035) and upregulation of the transforming growth factor beta pathway. Overall, these results expand our definition of fibrillin-related disorders to include AIS and open up new strategies for diagnosing and treating severe AIS., (© The Author 2014. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Background: Adolescent idiopathic scoliosis (AIS) is a complex genetic disorder that causes spinal deformity in approximately 3% of the population. Candidate gene, linkage, and genome-wide association studies have sought to identify genetic variation that predisposes individuals to AIS, but the genetic basis remains unclear. Copy number variants are associated with several isolated skeletal phenotypes, but their role in AIS, to our knowledge, has not been assessed., Questions/purposes: We determined the frequency of recurrent copy number rearrangements, chromosome aneuploidy, and rare copy number variants in patients with AIS., Methods: Between January 2010 and August 2014, we evaluated 150 patients with isolated AIS and spinal curvatures measuring 10° or greater, and 148 agreed to participate. Genomic copy number analysis was performed on patients and 1079 control subjects using the Affymetrix(®) Genome-wide Human SNP Array 6.0. After removing poor quality samples, 143 (97%) patients with AIS were evaluated for copy number variation., Results: We identified a duplication of chromosome 1q21.1 in 2.1% (N = 3/143) of patients with AIS, which was enriched compared with 0.09% (N = 1/1079) of control subjects (p = 0.0057) and 0.07% (N = 6/8329) of a large published control cohort (p = 0.0004). Other notable findings include trisomy X, which was identified in 1.8% (N = 2/114) of female patients with AIS, and rearrangements of chromosome 15q11.2 and 16p11.2 that previously have been associated with spinal phenotypes. Finally, we report rare copy number variants that will be useful in future studies investigating candidate genes for AIS., Conclusions: Copy number variation and chromosomal aneuploidy may contribute to the pathogenesis of adolescent idiopathic scoliosis., Clinical Relevance: Chromosomal microarray may reveal clinically useful abnormalities in some patients with AIS.

Background: Sternoclavicular dislocations are relatively infrequent and are generally divided into anterior and posterior disruptions, the former being the most common. While posterior sternoclavicular joint (PSCJ) injuries are very rare, they may be associated with life-threatening complications. The ideal management of these injuries, particularly in the adolescent population, has not been well described., Purpose: Through a meta-analysis of PSCJ injuries in the adolescent, we aimed to (1) describe the epidemiology of PSCJ injuries in relation to the mechanism of injury, associated complications, and treatment preferences; (2) compare the success of closed reduction when attempted <48 versus >48 hours after the initial injury; and (3) compare the outcomes of closed versus open treatment., Study Design: Meta-analysis., Methods: A thorough review of the English literature was performed to identify all cases of PSCJ dislocations or medial clavicular physeal fractures in patients aged 12 to 18 years. Patient-level data for 140 patients were extracted from 79 studies., Results: The mean age of the patients was 15.24 years. Forty-nine patients (35.00%) underwent closed treatment only, 42 (30.00%) open treatment alone, and 47 (33.57%) closed treatment followed by open treatment. Also, 55.8% of closed reductions performed within 48 hours were successful compared with 30.8% of those performed more than 48 hours after injury. After initial treatment, 92.31% of patients treated with closed reduction regained full function without recurrence as compared with 95.83% of patients treated operatively., Conclusion: Closed and open methods have proven highly effective for the treatment of PSCJ injuries. However, follow-up data reported in the literature vary considerably. Closed reduction is most effective when attempted less than 48 hours after the initial injury., (© 2014 The Author(s).)

Background Context: Blood loss in patients with adolescent idiopathic scoliosis (AIS) who are undergoing posterior spinal instrumentation and fusion (PSIF) varies greatly. The reason for this wide range is not clear. There are reports of unexpected massive hemorrhage during these surgeries. Many studies reflect authors' preferences for describing blood loss in terms of levels fused, weight, or percent blood volume., Purpose: We sought to define excessive blood loss clinically, determine its incidence in our study population, and identify associated variables. Results are intended to inform perioperative preparation for these cases. Results may be used to inform prospective study designs., Study Design: This was a retrospective uncontrolled case series., Patient Sample: A total of 311 consecutive AIS PSIF cases during the years 2005-2010 performed at Children's Hospital Colorado were studied., Outcome Measures: We measured estimated blood loss (EBL) and its association with multiple patient, surgical, and anesthetic variables., Methods: Thirty-one variables potentially related to blood loss were collected retrospectively from electronic medical records for analysis. When no cases of clearly excessive blood loss were identified on the basis of visual examination of EBL distribution, we chose to use the top 10% of blood loss cases as an arbitrary determinant of excessive blood loss. Three cut-off strategies captured the top 10% of EBL cases with little variation in who was selected: 1) >1,700 mL of EBL, 2) >50% EBL/estimated blood volume, and 3) >150 mL/level fused EBL. Variables were compared with the χ(2) test, Fisher exact, or t-tests, when appropriate. A generalized linear mixed logistic model was used to determine the probability of excessive blood loss based on the number of levels fused., Results: The average EBL was 89.17 mL/level fused (range, 45-133 mL). EBL fit a progressively wider distribution as surgical complexity (number of levels fused) increased. Number of levels fused (p<.0001), operative time (p=.0139), number of screws (p<.0001), and maximal preoperative Cobb angle (p=.0491) were significantly associated with excessive blood loss. The variable that was most strongly associated with excessive blood loss was the number of levels fused, with ≥12 levels having a probability of >10% of excessive hemorrhage., Conclusion: Excessive blood loss may be an arbitrary number until future research suggests otherwise. We show that the probability of exceeding one of our arbitrary definitions is approximately 10% when 12 or more levels are fused. If a 10% incidence of excessive blood loss is determined to be clinically relevant, teams might wish to pursue hematologic consultation and maximal blood conservation strategy when 12 or more levels are planned for fusion., (Copyright © 2014 Elsevier Inc. All rights reserved.)

The global epidemic of cardiovascular disease (CVD) calls for multidisciplinary and multiprofessional approaches to the management of this condition, with strategic emphasis on prevention, treatment, and control. In addition, there is increasing recognition that effective prevention and management of CVD requires a diverse workforce skilled in the social, environmental, and policy determinants of health. Nowhere are these approaches and strategies brought together and more closely aligned than in the field of preventive cardiovascular nursing. This executive summary of "Global Cardiovascular Prevention: A Call to Action for Nursing" includes key points from the 6 papers written by the Preventive Cardiovascular Nurses Association and published in July-August 2011 as a supplement to the Journal of Cardiovascular Nursing and the European Journal of Cardiovascular Nursing. This supplement addresses innovative efforts to stem the current global epidemic of CVD and emphasizes the need for effective team-based interventions for lifestyle and behavior changes across the life span. Social solutions, strategies for working with key players to develop interactive models, as well as coordinated multilevel policies, partnerships, and programs that are culturally relevant and context specific are examined. Such approaches are urgently needed to reduce death and disability from CVD in the United States and globally. Nurse leaders and other members of the healthcare team are well positioned internationally to meet these challenges.

Study Design: Model-independent linkage analysis and tests of association were performed for 22 single nucleotide polymorphisms in the CHD7 gene in 244 families of European descent with familial idiopathic scoliosis (FIS)., Objective: To replicate an association between FIS and the CHD7 gene on 8q12.2 in an independent sample of families of European descent., Summary of Background Data: The CHD7 gene on chromosome 8, responsible for the CHARGE syndrome, was previously associated with FIS in an independent study that included 52 families of European descent., Methods: Model-independent linkage analysis and intrafamilial tests of association were performed on the degree of lateral curvature considered as a qualitative trait (with thresholds of ≥10°, ≥15°, ≥20°, and ≥30°) and as a quantitative trait (degree of lateral curvature). Results from the tests of associations from this study and the previous study were combined in a weighted meta-analysis., Results: No significant results (P < 0.01) were found for linkage analysis or tests of association between genetic variants of the CHD7 and FIS in this study, failing to replicate the findings from the previous study. Furthermore, no significant results (P < 0.01) were found from meta-analysis of the results from the tests of association from this sample and from the previous sample., Conclusion: No association between the 22 genotyped single nucleotide polymorphisms in the CHD7 gene and FIS within this study sample was found, failing to replicate the earlier findings. Further investigation of the CHD7 gene and its potential association to FIS may be required., Level of Evidence: N/A.

Background Context: Pedicle screws have shown to be a safe and effective method of spinal fixation, offering superior multiplanar correction compared with hooks or sublaminar wires in selected situations. Though only food and drug administration (FDA) approved in the adolescent population, they are commonly used in an off-label manner in the preadolescent population., Purpose: To determine if the complication rate of the off-label use of pedicle screws for spinal fixation in the preadolescent 0- to 12-year-old population is comparable with the complication rate in the FDA-approved 13- to 18-year-old population., Study Design/setting: Retrospective medical record and radiograph review., Patient Sample: A total of 726 pediatric patients who underwent a spinal fusion procedure at a single tertiary institution between January 2003 and December 2008 were reviewed., Outcome Measures: Incidence of instrumentation failure, infection, neurological complication, and total complications., Methods: The study population was divided into two groups based on age: the younger group included 0- to 12-year olds and the older group included 13- to 18-year olds at the time of surgery. Groups were further subdivided based on diagnosis: "A," neuromuscular scoliosis; "B," idiopathic scoliosis, and "C," other spinal deformities. Rates of neurovascular complications, infections, and instrumentation complications were compared statistically between the younger and the older groups. Only patients with greater than or equal to 1-year follow-up and greater than or equal to 2-year follow-up were included in the calculations for infection and instrumentation complication rates, respectively., Results: There were 206 patients (33% males, 67% females) in the younger group (0 to 12 years) and 520 (41% males, 59% females) in the older group (13 to 18 years). Overall, younger group had a 13.6% complication rate compared with 16.9% in the older group. Younger subjects showed a 13.4% complication rate because of instrumentation-related complications, 0.5% for neurovascular complications, and an infection rate of 9.2%. The older group showed a 15.4% complication rate because of instrumentation-related complications, 1.92% for neurovascular complications, and an infection rate of 11.0%. Complication rates were statistically insignificant between the two groups. Other complications in the younger group included one patient with aspiration pneumonia, two with ileus, and one with pulmonary and other complications in the older group included one patient with aspiration pneumonia, two with ileus, three with superior mesenteric artery syndrome, and three with wound dehiscence., Conclusions: The complication rates in the young pediatric population associated with the off-label use of pedicle screws for spinal fusions are not statistically different from the complication rates associated with the FDA-approved adolescent population., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Adherence is critical to the overall management of individuals at risk for and with cardiovascular disease. It forms an interplay between the patient, provider, and health care system and includes barriers that have been encountered within all 3 domains. Improving adherence to exercise, diet, and medication as well as focusing on addictive disorders such as smoking cessation requires patient, provider, and health care system approaches. The use of the cognitive/behavioral elements of health behavior change and communication strategies such as motivational interviewing and coaching serve to enhance overall adherence. Continuous quality improvement initiatives at the system level of change also increase the likelihood that teams will succeed in helping individuals change their behavior. Cardiac rehabilitation programs offer a unique opportunity for health care professionals to play a key role in supporting individuals through the health behavior change process.

Objective: Custom genotyping of markers in families with familial idiopathic scoliosis were used to fine-map candidate regions on chromosomes 9 and 16 in order to identify candidate genes that contribute to this disorder and prioritize them for next-generation sequence analysis., Methods: Candidate regions on 9q and 16p-16q, previously identified as linked to familial idiopathic scoliosis in a study of 202 families, were genotyped with a high-density map of single nucleotide polymorphisms. Tests of linkage for fine-mapping and intra-familial tests of association, including tiled regression, were performed on scoliosis as both a qualitative and quantitative trait., Results and Conclusions: Nominally significant linkage results were found for markers in both candidate regions. Results from intra-familial tests of association and tiled regression corroborated the linkage findings and identified possible candidate genes suitable for follow-up with next-generation sequencing in these same families. Candidate genes that met our prioritization criteria included FAM129B and CERCAM on chromosome 9 and SYT1, GNAO1, and CDH3 on chromosome 16., (Copyright © 2012 S. Karger AG, Basel.)

Many developing countries use food-price subsidies or controls to improve nutrition. However, subsidizing goods on which households spend a high proportion of their budget can create large wealth effects. Consumers may then substitute towards foods with higher non-nutritional attributes (e.g., taste), but lower nutritional content per unit of currency, weakening or perhaps even reversing the subsidy's intended impact. We analyze data from a randomized program of large price subsidies for poor households in two provinces of China and find no evidence that the subsidies improved nutrition. In fact, it may have had a negative impact for some households. (JEL I38; O12; Q18).

The worldwide personal and societal costs related to diseases of the vascular system are enormous. International research efforts have focused on discovering ways to implement prevention strategies shown to be both effective and cost-efficient. Teams comprising health care professionals with expertise in nursing, dietetics, physical activity, and behavioral skills have shown high levels of success in preventive efforts, particularly in high-risk and vulnerable populations. Used appropriately, team-based, nurse-directed case management has the potential to effect positive change in both primary and secondary prevention of cardiac and other vascular diseases.

Nonadherence and poor or no persistence with taking antihypertensive medications results in uncontrolled high blood pressure, poor clinical outcomes and preventable health care costs. Factors associated with nonadherence are multilevel and relate not only to the patient, but also to the provider, health care system, health care organization, and community. National guideline committees have called for more aggressive approaches to implement strategies known to improve adherence and technologies known to enable changes at the systems level including improved communication among providers and patients. Improvements in adherence and persistence are likely to be achieved by supporting patient self-management, a team approach to patient care, technology-supported office practice systems, better methods to measure adherence, and less clinical inertia. Integrating high blood pressure control into health care policies that emphasize and improve prevention and management of chronic illness remains a challenge. Four strategies are proposed: focusing on clinical outcomes; empowering informed, activated patients; developing prepared proactive practice teams; and advocating for health care policy reform. With hypertension remaining the most common reason for office visits, the time is now., (Copyright © 2011 Wiley Periodicals, Inc. Published by Elsevier Inc. All rights reserved.)

The triple curve pattern (three lateral curvatures of equal severity) has been recognized as a distinct and unique clinical subtype of scoliosis. As part of a large study of familial idiopathic scoliosis (FIS), a subset of five families with a triple curve pattern (at least one member of each family having a triple curve) was evaluated to determine if this curve pattern was linked to any of the markers previously genotyped as part of the STRP-based previous linkage screen. Model independent linkage analysis (SIBPAL, v4.5) of the initial genomic screen identified candidate regions on chromosomes 6 and 10 when FIS was analyzed both as qualitative and quantitative traits in single- and multipoint linkage analyses. Additional fine mapping analyses of this subgroup with SNPs corroborated the findings in these regions (P < 0.001). These regions have been previously linked to FIS, however, this is the first time these regions have been implicated in a clinically well-defined subgroup and may suggest a unique genetic etiology for the formation of a triple curve., ((c) 2010 Wiley-Liss, Inc.)

Background: Coronary heart disease (CHD) is a significant cause of health and economic burden. Secondary prevention programs play a pivotal role in the treatment and management of those affected by CHD although participation rates are poor due to patient, provider, health system and societal-level barriers. As such, there is a need to develop innovative secondary prevention programs to address the treatment gap. Telephone-delivered care is convenient, flexible and has been shown to improve behavioural and clinical outcomes following myocardial infarction (MI). This paper presents the design of a randomised controlled trial to evaluate the efficacy of a six-month telephone-delivered secondary prevention program for MI patients (ProActive Heart)., Methods: 550 adult MI patients have been recruited over a 14 month period (December 2007 to January 2009) through two Brisbane metropolitan hospitals, and randomised to an intervention or control group (n = 225 per group). The intervention commences within two weeks of hospital discharge delivered by study-trained health professionals ('health coaches') during up to 10 x 30 minute scripted telephone health coaching sessions. Participants also receive a ProActive Heart handbook and an educational resource to use during the health coaching sessions. The intervention focuses on appropriate modification of CHD risk factors, compliance with pharmacological management, and management of psychosocial issues. Data collection occurs at baseline or prior to commencement of the intervention (Time 1), six months follow-up or the completion of the intervention (Time 2), and at 12 months follow-up for longer term outcomes (Time 3). Primary outcome measures include quality of life (Short Form-36) and physical activity (Active Australia Survey). A cost-effective analysis of the costs and outcomes for patients in the intervention and control groups is being conducted from the perspective of health care costs to the government., Discussion: The results of this study will provide valuable new information about an innovative telephone-delivered cost-effective secondary prevention program for MI patients.

World food prices have increased dramatically in recent years. We use panel data from 2006 to examine the impact of these increases on the consumption and nutrition of poor households in two Chinese provinces. We find that households in Hunan suffered no nutrition declines. Households in Gansu experienced a small decline in calories, though the decline is on par with usual seasonal effects. The overall nutritional impact of the world price increase was small because households were able to substitute to cheaper foods and because the domestic prices of staple foods remained low due to government intervention in grain markets.

This paper provides the first real-world evidence of Giffen behavior, i.e., upward sloping demand. Subsidizing the prices of dietary staples for extremely poor households in two provinces of China, we find strong evidence of Giffen behavior for rice in Hunan, and weaker evidence for wheat in Gansu. The data provide new insight into the consumption behavior of the poor, who act as though maximizing utility subject to subsistence concerns. We find that their elasticity of demand depends significantly, and nonlinearly, on the severity of their poverty. Understanding this heterogeneity is important for the effective design of welfare programs for the poor. (JEL D12, O12).

Simultaneous avulsions of the tibial tubercle and patellar ligament have been reported, but are rare. We present an 11-year-old boy who was initially diagnosed with a Type IIIA tibial tubercle avulsion fracture after falling off of his bicycle. Intraoperatively, following exposure and fixation of the tibial tubercle fragment, knee range of motion under live fluoroscopy revealed a stationary patella with no tension in the patellar ligament. The incision was extended and a distal avulsion of the patellar ligament from the tibial tubercle was identified, with a small flap of tibial tubercle periosteum remaining attached. Transosseous suture fixation of the avulsed periosteal flap was achieved with 2 No. 5 ethibond sutures placed through connecting drill holes. Postoperatively, the patient was placed in a long leg cast with the knee in extension for 6 weeks. The injury to healed and the patient returned to full activities, although he required 2 courses of physical therapy and 4.5 months to regain full range of motion. Preoperative diagnosis of simultaneous tibial tubercle fracture and patellar ligament avulsion can be difficult. Palpation of the patellar ligament for gaps may not be possible due to a large knee effusion. Most patients do not tolerate quadriceps testing on examination and do not have patella alta on radiographs. In retrospect, a clue to this diagnosis in our patient was the preoperative radiographic finding of multiple calcified fragments below the patella. The calcified fragments likely represent the avulsed tibial tubercle periosteum attached to the distal patellar ligament. In addition, the large tibial tubercle fragment is separated and rotated superiorly, a finding that was also noted in two other similar case reports.

Exercise training is increasingly recognized as a viable treatment option for patients with heart failure (HF). For exercise to be a maximally effective treatment, it is important that patients adhere to the exercise prescription. In this review, the current state of adherence monitoring and intervention in randomized HF trials will be summarized, along with recommendations for advancing understanding of adherence in this population. Barriers to exercise participation and strategies to enhance adherence to exercise-training programs will be explored. Finally, directions for future research on exercise adherence in HF patients will be provided.

Idiopathic scoliosis in the adolescent period is a complex genetic disorder of high prevalence and extreme variability. Epidemiological approaches have confirmed the genetic basis of this disorder and have suggested multiple modes of heritability. The current challenge is to identify the genetic determinants of this condition to distinguish individuals who are at risk for severe progression. This review summarizes the literature establishing the genetic basis of this disorder. Studies focused on the identification of genes responsible for the scoliotic phenotype are reviewed, with an emphasis on recent works that have utilized contemporary strategies to isolate genes related to complex diseases. To date, molecular studies have isolated critical regions on chromosomes and X of potential importance to the etiology of scoliosis. An analysis of these works will aid in an understanding of seemingly conflicting results, and will help us determine the focus of future work.

We report six children with pigmented villonodular synovitis. They ranged in age from seven to fifteen years. In four patients, the knee was involved. One patient had involvement of the ankle, and one had diffuse involvement along a metacarpal. In five cases, the diagnosis was not suspected clinically or radiographically, and the delay in making the correct diagnosis was as long as two years. Clinical diagnosis in these five patients was usually bacterial synovitis or juvenile rheumatoid arthritis. We feel that the diagnoses of pigmented villonodular synovitis should be considered in any child with chronic joint effusion.

People distrust managed care organizations (MCOs) more than traditional health plans. This phenomenon has become known as "managed-care backlash." Using a model of the interaction between insurers, physicians, and patients, this paper identifies two possible motivations for MCO backlash. The first, which comes from traditional health plans' superior ability to credibly commit to providing better than least-cost care, is efficiency promoting. The second, which arises when patients are able to obtain higher-benefit treatments through reneging on their initial insurance contracts through "doctor shopping," may reduce efficiency.