Distal Femoral Valgus and Recurrent Traumatic Patellar Instability: Is an Isolated Varus Producing Distal Femoral Osteotomy a Treatment Option?

Background: Genu valgum, a risk factor for recurrent patellofemoral instability, can be addressed with a varus producing distal femoral osteotomy (DFO). The purpose of this study is to report 3-year clinical and radiographic outcomes on a series of skeletally mature adolescents with traumatic patellofemoral instability and genu valgum who underwent a varus producing DFO.

Methods: Consecutive patients (n=11) who underwent an isolated DFO for recurrent traumatic patellar instability over a 4-year study period (2009 to 2012) were reviewed.

Toe Walking: A Neurological Perspective After Referral From Pediatric Orthopaedic Surgeons.

Background: Toe walking (TW) in children is often idiopathic in origin. Our purpose was to determine the incidence of a neurological etiology for TW in patients seen in the neurology clinic after referral from pediatric orthopaedic surgeons.

Methods: We performed an Institutional Review Board approved retrospective review of 174 patients referred to the neurology clinic from orthopaedic surgeons at an academic pediatric tertiary care center between January 2010 and September 2015.

Comparison of Percentile Weight Gain of Growth-Friendly Constructs in Early-Onset Scoliosis.

Study Design: Multicenter retrospective cohort.

Objective: To compare improvement in nutritional status seen in early-onset scoliosis (EOS) patients following treatment with various growth-friendly techniques, especially in underweight patients (<20th weight percentile).

Background: Thoracic insufficiency resulting from EOS can lead to severe cardiopulmonary disease.

CELSR2 is a candidate susceptibility gene in idiopathic scoliosis.

A Swedish pedigree with an autosomal dominant inheritance of idiopathic scoliosis was initially studied by genetic linkage analysis, prioritising genomic regions for further analysis. This revealed a locus on chromosome 1 with a putative risk haplotype shared by all affected individuals. Two affected individuals were subsequently exome-sequenced, identifying a rare, non-synonymous variant in the CELSR2 gene.

CT-guided percutaneous biopsy of sclerotic bone lesions: diagnostic outcomes.

Objective: To determine the diagnostic yield of CT-guided percutaneous biopsy of densely sclerotic bone lesions.

Materials And Methods: We retrospectively analyzed CT-guided percutaneous bone biopsies performed at our institution from September 2008 through August 2011 (329 cases) and from September 2012 through August 2015 (324 cases) after adoption of a battery-powered drill system (OnControl). Bone lesions were included in the analysis if they were >70% sclerotic by visual inspection, had a density > 2 times that of adjacent trabecular bone, and had an attenuation of ≥250 HU.

Summary of the first inaugural joint meeting of the International Consortium for scoliosis genetics and the International Consortium for vertebral anomalies and scoliosis, March 16-18, 2017, Dallas, Texas.

Scoliosis represents the most common musculoskeletal disorder in children and affects approximately 3% of the world population. Scoliosis is separated into two major phenotypic classifications: congenital and idiopathic. Idiopathic scoliosis is defined as a curvature of the spine of 10° or greater visualized on plane radiograph and does not have associated vertebral malformations (VM).

Imaging of the Pediatric Temporomandibular Joint.

Imaging of the temporomandibular joint in pediatric patients is a critical component in the evaluation and treatment of children with temporomandibular joint symptoms. MRI can provide detailed joint anatomy and identify inflammation, sometimes before symptom onset. Ultrasound scan is a convenient emerging modality to evaluate the joint and guide therapeutic injections.

A missense variant in the coil1A domain of the keratin 25 gene is associated with the dominant curly hair coat trait (Crd) in horse.

Background: Curly horses present a variety of curl phenotypes that are associated with various degrees of curliness of coat, mane, tail and ear hairs. Their origin is still a matter of debate and several genetic hypotheses have been formulated to explain the diversity in phenotype, including the combination of autosomal dominant and recessive alleles. Our purpose was to map the autosomal dominant curly hair locus and identify the causal variant using genome-wide association study (GWAS) and whole-genome sequencing approaches.

Take Flight: the evolution of an Orton Gillingham-based curriculum.

Thirty years ago in this journal, Aylett Royall Cox reported on the development of Alphabetic Phonics, a revision of the existing Orton Gillingham treatment for children with dyslexia. This paper continues that discussion and reports on the evolution of that curriculum as it is represented in a comprehensive dyslexia treatment program informed by intervention research. The paper describes the curriculum and reports data from a hospital-based learning disabilities clinic that provides qualified support for treatment efficacy and the value of added comprehension instruction.

Expectant use of CIC in newborns with spinal dysraphism: Report of clinical outcomes.

Purpose: Report urologic outcomes among newborns with spinal dysraphism managed within an expectant clean intermittent catheterization (CIC) program.

Methods: Newborns were followed clinically and with serial ultrasound (US). Urodynamics (UD) and dimercaptosuccinic acid (DMSA) renal scan were obtained at 3-6 months, 1 year, 3 years, then as needed.

A trauma-informed approach supports health and development in children and youth with spina bifida.

Children with developmental disabilities may be at greater risk for experiencing maltreatment and traumatic events, threats to their physical and psychological safety and well-being. Young children and youth with spina bifida benefit from being considered in light of this risk, and may be especially vulnerable given the complexity of their neurodevelopmental condition. A trauma-informed approach brings together evidence from the neurosciences, epidemiology and psychology to promote improved developmental, behavioral, physical and mental health status.

Automated event detection algorithm for two squatting protocols.

Introduction: Squatting biomechanics assessed using motion analysis relies on the identification of specific events: start of descent, transition between descent/ascent and end of ascent. Automated identification reduces the time needed to process trials while allowing consistency across studies. The purpose of this study was to develop criteria for the identification of events and apply them to two squatting protocols in pathological patient and typically developing (TD) groups.

How Is Pulmonary Function and Exercise Tolerance Affected in Patients With AIS Who Have Undergone Spinal Fusion?

Study Design: Prospectively enrolled AIS patients who underwent spinal fusion, with 2 year follow-up.

Objectives: To evaluate the cardiovascular fitness and activity level in patients with AIS pre- and post-spinal fusion and to determine if initial curve magnitude or pulmonary function is predictive of exercise capacity.

Summary Of Background Data: Researchers have tried to link pulmonary function testing (PFT) to exercise capacity; the results are mixed.

Seasonal Variation and Weather Changes Related to the Occurrence and Severity of Acute Hematogenous Osteomyelitis in Children.

Background: Acute hematogenous osteomyelitis (AHO) demonstrates regional variability in incidence and severity. In this study, we evaluated seasonal variations of AHO and assessed the effects of weather trends on the occurrence and severity of illness in affected children.

Methods: National Weather Service data from the dates of symptom onset and of admission of children with AHO were gathered.

Biallelic mutations in the ferredoxin reductase gene cause novel mitochondriopathy with optic atrophy.

Iron-sulfur (Fe-S) clusters are ubiquitous cofactors essential to various cellular processes, including mitochondrial respiration, DNA repair, and iron homeostasis. A steadily increasing number of disorders are being associated with disrupted biogenesis of Fe-S clusters. Here, we conducted whole-exome sequencing of patients with optic atrophy and other neurological signs of mitochondriopathy and identified 17 individuals from 13 unrelated families with recessive mutations in FDXR, encoding the mitochondrial membrane-associated flavoprotein ferrodoxin reductase required for electron transport from NADPH to cytochrome P450.

The reduced osteogenic potential of Nf1-deficient osteoprogenitors is EGFR-independent.

Neurofibromatosis type 1 (NF1) is a common genetic disorder caused by mutations in the NF1 gene. Recalcitrant bone healing following fracture (i.e.

Pooled analysis of menstrual irregularities from three major clinical studies evaluating everolimus for the treatment of tuberous sclerosis complex.

Objectives: To determine the impact of everolimus on female fertility, including menstrual irregularities, secondary amenorrhea, and luteinizing and follicle stimulating hormone levels in female patients.

Design: A pooled analysis from 3 prospective studies consisting of a core phase (≥6 months) and a long-term follow-up open-label extension.

Setting: One phase 2 single-center and two phase 3 multicenter studies.

Functional Outcomes Following Operative Treatment of Tibial Tubercle Fractures.

Background: Quantitative evaluation of the functional results of surgically managed tibial tubercle fractures in adolescents is unreported in the orthopaedic literature.

Methods: All patients treated surgically for unilateral tibial tubercle fractures at a single institution from 2007 to 2011 were invited to return for functional evaluation. Fractures were classified using the Ogden classification system.

The effect of everolimus on renal angiomyolipoma in pediatric patients with tuberous sclerosis being treated for subependymal giant cell astrocytoma.

Background: Patients with tuberous sclerosis complex (TSC) often have multiple TSC-associated hamartomas, particularly in the brain and kidney.

Methods: This was a post hoc analysis of pediatric patients being treated for subependymal giant cell astrocytomas (SEGAs) during the phase 3, randomized, double-blind, placebo-controlled EXIST-1 trial. Patients were initially randomly assigned to receive everolimus 4.

In Vitro and In Vivo Characterization of Premixed PMMA-CaP Composite Bone Cements.

Acrylic bone cements, although successful in the field of orthopedics, suffer from a lack of bioactivity, not truly integrating with surrounding bone. Bioactive fixation is expected to enhance cement performance because of the natural interlocking and bonding with bone, which can improve the augmentative potential of the material in applications such as vertebroplasty (VP). In a recent study, two composite cements (PMMA-hydroxyapatite and PMMA-brushite) showed promising results demonstrating no deterioration in rheological and mechanical properties after CaP filler addition.

Comparison of a fast 5-min knee MRI protocol with a standard knee MRI protocol: a multi-institutional multi-reader study.

Purpose: To compare diagnostic performance of a 5-min knee MRI protocol to that of a standard knee MRI.

Materials And Methods: One hundred 3 T (100 patients, mean 38.8 years) and 50 1.

A multidimensional blood stimulation assay reveals immune alterations underlying systemic juvenile idiopathic arthritis.

The etiology of sporadic human chronic inflammatory diseases remains mostly unknown. To fill this gap, we developed a strategy that simultaneously integrates blood leukocyte responses to innate stimuli at the transcriptional, cellular, and secreted protein levels. When applied to systemic juvenile idiopathic arthritis (sJIA), an autoinflammatory disease of unknown etiology, this approach identified gene sets associated with specific cytokine environments and activated leukocyte subsets.

Comparative Effectiveness of Tumor Necrosis Factor Agents and Disease-modifying Antirheumatic Therapy in Children with Enthesitis-related Arthritis: The First Year after Diagnosis.

Objective: To characterize the effect of anti-tumor necrosis factor (TNF) therapy compared to conventional synthetic disease-modifying antirheumatic drugs (csDMARD) in children with enthesitis-related arthritis (ERA) over the first year after diagnosis.

Methods: We conducted a multicenter retrospective comparative effectiveness study of children diagnosed with ERA. We estimated the effect of anti-TNF therapy on clinical variables (active joint count, tender entheses count) and patient-reported pain and global assessment of disease activity over the first year after diagnosis using state-of-the-art comparative effectiveness analytic methods.