Ischemic femoral head osteonecrosis in a piglet model causes three dimensional decrease in acetabular coverage.

Legg-Calve-Perthes disease (LCPD) is a childhood form of ischemic osteonecrosis marked by development of severe femoral head deformity and premature osteoarthritis. The pathogenesis of femoral head deformity has been studied extensively using a piglet model of ischemic osteonecrosis, however, accompanying acetabular changes have not been investigated. The purpose of this study was to determine if acetabular changes accompany femoral head deformity in a well-established piglet model of LCPD and to define the acetabular changes using three dimensional computed tomography (3-D CT) and modeling.

Safety and Efficacy of Repeat Open-Label AbobotulinumtoxinA Treatment in Pediatric Cerebral Palsy.

This was a prospective, repeat-treatment, open-label study (NCT01251380) of abobotulinumtoxinA for the management of lower limb spasticity in children who had completed a double-blind study. Children (2-17 years) received injections into the gastrocnemius-soleus complex, and other distal and proximal muscles as required (maximum total dose per injection cycle: 30 U/kg or 1000U). A total of 216 of the 241 double-blind patients entered the extension study and 207 received ≥1 open label injection into the gastrocnemius-soleus; 17-24% of patients also had injections into the hamstrings.

Characterization of the Polish Primitive Horse (Konik) maternal lines using mitochondrial D-loop sequence variation.

The Polish Primitive Horse (PPH, Konik) is a Polish native horse breed managed through a conservation program mainly due to its characteristic phenotype of a primitive horse. One of the most important goals of PPH breeding strategy is the preservation and equal development of all existing maternal lines. However, until now there was no investigation into the real genetic diversity of 16 recognized PPH dam lines using mtDNA sequence variation.

Quengel Casting for the Management of Pediatric Knee Flexion Contractures: A 26-Year Single Institution Experience.

Background: Quengel casting was introduced in 1922 for nonsurgical treatment of knee flexion contractures (KFC) associated with hemophilic arthropathy. It consists of an extension-desubluxation hinge fixed to a cast allowing for gradual correction of a flexion deformity while preventing posterior tibial subluxation. The purpose of this study is to report 1 center's experience with this technique for the treatment of pediatric KFC.

A Brief History of Limb Lengthening.

In the last 35 years, orthopaedic surgeons have witnessed 3 major advances in the technique of limb lengthening: "distraction osteogenesis" facilitated by Gavriil Ilizarov method and infinitely-adaptable circular fixator with fine-wire bone fragment fixation; the introduction of the "6-strut" computer program-assisted circular fixators to effect complex deformity correction simultaneously; and the development of motorized intramedullary lengthening nails. However, the principles and associated complications of these techniques are on the basis of observations by Codivilla, Putti, and Abbott from as much as 110 years ago. This review notes the contribution of these pioneers in limb lengthening, and the contribution of Thor Heyerdahl principles of tolerance and diversity to the dissemination of Ilizarov principles to the Western world.

Redefining Optimal Nail to Medullary Canal Diameter Ratio in Stainless Steel Flexible Intramedullary Nailing of Pediatric Femur Fractures.

Purpose: The ideal canal fill for flexible intramedullary fixation of pediatric femoral shaft fractures is considered to be 80% based upon relatively few clinical studies. The purpose of this study is to assess the relationship between the summed nail to intramedullary canal diameter (ND/MCD) ratio and alignment at radiographic union following flexible intramedullary nailing (FIMN) of pediatric femoral shaft fractures.

Methods: An Internal Review Board approved, retrospective review of a consecutive series of patients who sustained a femoral shaft fracture treated by retrograde, stainless steel FIMN was performed at a single level 1 pediatric trauma center from 2005 to 2012.

Genome-Wide Association Meta-Analysis Reveals Novel Juvenile Idiopathic Arthritis Susceptibility Loci.

Objective: Juvenile idiopathic arthritis (JIA) is the most common childhood rheumatic disease and has a strong genomic component. To date, JIA genetic association studies have had limited sample sizes, used heterogeneous patient populations, or included only candidate regions. The aim of this study was to identify new associations between JIA patients with oligoarticular disease and those with IgM rheumatoid factor (RF)-negative polyarticular disease, which are clinically similar and the most prevalent JIA disease subtypes.

Long-term Follow-up of Neonatal Brachial Plexopathy: Psychological and Physical Function in Adolescents and Young Adults.

Purpose: The prevalence of neonatal brachial plexus palsy (NBPP) has been increasing since the early 1980s. No known studies have examined long-term psychological health and quality of life (QOL) in young adults. The purpose of this study was to investigate the psychosocial and intellectual aspects of NBPP during adolescence into young adulthood.

Bone marrow adipocytes promote the regeneration of stem cells and haematopoiesis by secreting SCF.

Endothelial cells and leptin receptor (LepR) stromal cells are critical sources of haematopoietic stem cell (HSC) niche factors, including stem cell factor (SCF), in bone marrow. After irradiation or chemotherapy, these cells are depleted while adipocytes become abundant. We discovered that bone marrow adipocytes synthesize SCF.

A genome-wide transcriptomic analysis of articular cartilage during normal maturation in pigs.

Objective: The articular cartilage undergoes dramatic changes in structure and composition during post-natal maturation, but the associated transcriptional changes are not well characterized. Compared to a mature stage, the immature articular cartilage shows developmental features such as increased thickness, presence of blood vessels, and the presence of a deep layer of growth cartilage which undergoes endochondral ossification. These features decrease during normal development.

Functional Assessment of Children and Adolescents with Symbrachydactyly: A Unilateral Hand Malformation.

Background: We studied children and adolescents with symbrachydactyly to determine whether hand function depends on digit opposability and whether scores for function and quality-of-life measures differ from population norms.

Methods: Participants were grouped on the basis of hand morphology: Group A lacked opposable digits, and Group B had ≥2 digits that were opposable. The groups were compared with each other and with norms with respect to pinch strength, the performance of bimanual activities and in-hand manipulation, and questionnaires regarding psychosocial status and the ability to perform activities of daily living (ADLs).

"On-Top Plasty" for Radial Polydactyly Reconstruction.

Purpose: To introduce the "on-top plasty" technique and report our long-term outcomes.

Methods: We evaluated 5 thumbs in 5 patients who underwent radial polydactyly reconstruction using the "on-top plasty" technique between 1998 and 2003. This technique was used when it was felt that neither thumb possessed adequate proximal and distal structures to provide a functional and aesthetically pleasing thumb.

In-depth investigations of adolescents and adults with holoprosencephaly identify unique characteristics.

PurposeWith improved medical care, some individuals with holoprosencephaly (HPE) are surviving into adulthood. We investigated the clinical manifestations of adolescents and adults with HPE and explored the underlying molecular causes.MethodsParticipants included 20 subjects 15 years of age and older.

Treatment of the Upper Extremity Contracture/Deformities.

Patients with arthrogryposis multiplex congenita have a characteristic upper extremity resting posture consisting of internal rotation of the shoulders, elbow extension, flexed wrists, thumb-in palm deformities, and variable degrees of finger contractures. Treatment of these patients is aimed at improving independence and performance of activities of daily living. Although each area needs to be assessed independently for the most appropriate surgical procedure, often multiple areas can be addressed at the same operative setting.

The health and healthcare impact of providing insurance coverage to uninsured children: A prospective observational study.

Background: Of the 4.8 million uninsured children in America, 62-72% are eligible for but not enrolled in Medicaid or CHIP. Not enough is known, however, about the impact of health insurance on outcomes and costs for previously uninsured children, which has never been examined prospectively.

Interval setting selection affects ambulatory activity outputs in children with cerebral palsy.

Introduction: Accelerometer based devices have been widely used to assess the ambulatory activity of children with and without functional disabilities. Many researchers who utilize the StepWatch Activity Monitor (SAM) collect at a 60second (60sec) interval setting. The purpose of this study was to assess the effect of SAM interval settings on ambulatory activity outputs in children with cerebral palsy (CP) and typically developing youth.

Event centrality as a unique predictor of posttraumatic stress symptoms and perceived disability following spinal cord injury.

Study Design: We conducted a cross-sectional study involving completion of self-report measures.

Objectives: Individuals who acquire a spinal cord injury (SCI) face numerous physical and psychological challenges, with the former receiving considerable less attention during the rehabilitation process. In this article, we examined event centrality as a unique predictor of psychological outcomes in a sample of individuals receiving rehabilitation for SCI.

Open-Label, Phase II Study to Assess the Efficacy and Safety of Canakinumab Treatment in Active Hyperimmunoglobulinemia D With Periodic Fever Syndrome.

Objective: To evaluate the efficacy and safety of canakinumab treatment in active hyperimmunoglobulinemia D with periodic fever syndrome (HIDS).

Methods: This was a 3-part open-label study with an initial 6-month treatment period in which patients with HIDS (n = 9) received canakinumab subcutaneously at a dose of 300 mg (or 4 mg/kg for those weighing ≤40 kg) every 6 weeks (period 1 [P1]), followed by a 6-month withdrawal period (period 2 [P2]), and then a 24-month extension treatment period with canakinumab at the same dose (period 3 [P3]). The primary end point was reduction in the frequency of attacks during treatment periods as compared to the historical period (HP; defined as the period in which patients did not receive drugs other than nonsteroidal antiinflammatory drugs and/or steroids).

ACR Appropriateness Criteria Urinary Tract Infection-Child.

Urinary tract infection (UTI) is common in young children and may cause pyelonephritis and renal scarring. Long-term complications from renal scarring are low. The role of imaging is to evaluate for underlying urologic abnormalities and guide treatment.

ACR Appropriateness Criteria Suspected Physical Abuse-Child.

The youngest children, particularly in the first year of life, are the most vulnerable to physical abuse. Skeletal survey is the universal screening examination in children 24 months of age and younger. Fractures occur in over half of abused children.

Reliability of Radiographic Assessments of Adolescent Midshaft Clavicle Fractures by the FACTS Multicenter Study Group.

Objectives: There is a recent trend toward increased surgical treatment of displaced midshaft clavicle fractures in adolescents. The primary purpose of this study was to evaluate the intrarater and interrater reliability of clavicle fracture classification systems and measurements of displacement, shortening, and angulation in adolescents. The secondary purpose was to compare 2 different measurement methods for fracture shortening.

Spectrum of Diagnosis and Disposition of Patients Referred to a Pediatric Orthopaedic Center for a Diagnosis of Intoeing.

Background: Orthopaedic surgeons frequently evaluate otherwise healthy children for concern of intoed gait. Intoeing in otherwise healthy young children due to metatarsus adductus, internal tibial torsion, and increased femoral anteversion do not typically require orthopaedic treatment. This study reviewed the actual diagnosis, management, and disposition of patients referred to a pediatric orthopaedic specialty hospital for a diagnosis of intoeing; the efficacy of an Advanced Practice Provider (APP) assessment program to screen and triage patients with a primary complaint of intoeing; and parental satisfaction with that program.

Targeted Disruption of NF1 in Osteocytes Increases FGF23 and Osteoid With Osteomalacia-like Bone Phenotype.

Neurofibromatosis type 1 (NF1, OMIM 162200), caused by NF1 gene mutations, exhibits multi-system abnormalities, including skeletal deformities in humans. Osteocytes play critical roles in controlling bone modeling and remodeling. However, the role of neurofibromin, the protein product of the NF1 gene, in osteocytes is largely unknown.

Clubfoot Infants Initially Thought to be Idiopathic, But Later Found Not to Be. How do They do With Nonoperative Treatment?

Background: Infants thought to be normal with idiopathic clubfeet when nonoperative treatment begins may later be found to have other complicating diagnoses. The purpose of this study was to determine the incidence of this occurrence, and to compare the clinical outcomes of these "nonidiopathic" patients with idiopathic clubfoot patients.

Methods: Infants below 3 months old with clubfeet who were thought to be normal (idiopathic) at presentation and had ≥2-year follow-up were studied.

A successful approach to minimizing attrition in racial/ethnic minority, low-income populations.

Background: Recruiting and retaining minority participants in clinical trials continue to be major challenges. Although multiple studies document lower minority trial enrollment, much less is known about effective minority retention strategies. Our objectives were to evaluate an innovative approach to high RCT retention of minority children, and identify child/caregiver characteristics predicting attrition.