Scholars@Duke
Benjamin Aaron Alman

Benjamin Aaron Alman

James R. Urbaniak, M.D., Distinguished Professor of Orthopedic Surgery
Orthopaedic Surgery

Selected Publications

Single cell analysis of Idh mutant growth plates identifies cell populations responsible for longitudinal bone growth and enchondroma formation.

Journal Article Sci Rep · October 31, 2024 Enchondromas are a common tumor in bone that can occur as multiple lesions in enchondromatosis, which is associated with deformity of the affected bone. These lesions harbor somatic mutations in IDH and driving expression of a mutant Idh1 in Col2 expressin ... Full text Link to item Cite

The COMPASS complex maintains the metastatic capacity imparted by a subpopulation of cells in UPS.

Journal Article iScience · July 19, 2024 Intratumoral heterogeneity is common in cancer, particularly in sarcomas like undifferentiated pleomorphic sarcoma (UPS), where individual cells demonstrate a high degree of cytogenic diversity. Previous studies showed that a small subset of cells within U ... Full text Link to item Cite

Evaluating the Outcomes and Trainee Performance of a Canadian Medical Imaging Clinician Investigator Program.

Journal Article Can Assoc Radiol J · February 2024 Purpose: To measure the research productivity of trainees from the University of Toronto's Medical Imaging Clinician Investigator Program (MI-CIP) and comparing it with the research productivity of trainees from MI-non-CIP and General Surgery (GSx) Clinici ... Full text Link to item Cite

Cast or Nail? Using a Preference-Based Tool for Shared Decision-Making in Pediatric Femoral Shaft Fracture Treatment.

Journal Article J Pediatr Orthop · September 1, 2023 BACKGROUND: Femoral shaft fractures are common injuries in children 2 to 7 years of age, with treatments ranging from casting to flexible intramedullary nails (FIN). Each treatment has unique attributes and outcomes are overall similar. Given equivalent ou ... Full text Link to item Cite

Mutant IDH regulates glycogen metabolism from early cartilage development to malignant chondrosarcoma formation.

Journal Article Cell Rep · June 27, 2023 Chondrosarcomas are the most common malignancy of cartilage and are associated with somatic mutations in isocitrate dehydrogenase 1 (IDH1) and IDH2 genes. Somatic IDH mutations are also found in its benign precursor lesion, enchondromas, suggesting that ID ... Full text Link to item Cite

Pro-Con Debate: Interdisciplinary Perspectives on Industry-Sponsored Research.

Journal Article J Arthroplasty · June 2023 PRO: Nearly all new devices and drugs come from industry that provides two-thirds of the funding for medical research, and a much higher fraction of clinical research. Realistically, without corporate-funded studies, perioperative research would stagnate w ... Full text Link to item Cite

Pro-Con Debate: Interdisciplinary Perspectives on Industry-Sponsored Research.

Journal Article Anesth Analg · June 1, 2023 Pro: Nearly all new devices and drugs come from industry that provides two-thirds of the funding for medical research, and a much higher fraction of clinical research. Realistically, without corporate-funded studies, perioperative research would stagnate w ... Full text Link to item Cite

CD142 Identifies Neoplastic Desmoid Tumor Cells, Uncovering Interactions Between Neoplastic and Stromal Cells That Drive Proliferation.

Journal Article Cancer Res Commun · April 2023 UNLABELLED: The interaction between neoplastic and stromal cells within a tumor mass plays an important role in cancer biology. However, it is challenging to distinguish between tumor and stromal cells in mesenchymal tumors because lineage-specific cell su ... Full text Open Access Link to item Cite

Loss of ATRX promotes aggressive features of osteosarcoma with increased NF-κB signaling and integrin binding.

Journal Article JCI Insight · September 8, 2022 Osteosarcoma (OS) is a lethal disease with few known targeted therapies. Here, we show that decreased ATRX expression is associated with more aggressive tumor cell phenotypes, including increased growth, migration, invasion, and metastasis. These phenotypi ... Full text Link to item Cite

Efficacy of auranofin as an inhibitor of desmoid progression.

Journal Article Sci Rep · July 13, 2022 Anticancer drugs and molecular targeted therapies are used for refractory desmoid-type fibromatosis (DF), but occasionally cause severe side effects. The purpose of this study was to identify an effective drug with fewer side effects against DF by drug rep ... Full text Link to item Cite

In desmoid-type fibromatosis cells sorafenib induces ferroptosis and apoptosis, which are enhanced by autophagy inhibition.

Journal Article Eur J Surg Oncol · July 2022 INTRODUCTION: Desmoid-type fibromatosis (DTF) is a rare, soft tissue tumour. Sorafenib, a multikinase inhibitor, has demonstrated antitumour efficacy in DTF patients. Little is known about the underlying molecular mechanisms, which are crucial to know to f ... Full text Link to item Cite

Distinct Roles of Glutamine Metabolism in Benign and Malignant Cartilage Tumors With IDH Mutations.

Journal Article J Bone Miner Res · May 2022 Enchondromas and chondrosarcomas are common cartilage neoplasms that are either benign or malignant, respectively. The majority of these tumors harbor mutations in either IDH1 or IDH2. Glutamine metabolism has been implicated as a critical regulator of tum ... Full text Link to item Cite

The origins and roles of osteoclasts in bone development, homeostasis and repair.

Journal Article Development (Cambridge, England) · April 2022 The mechanisms underlying bone development, repair and regeneration are reliant on the interplay and communication between osteoclasts and other surrounding cells. Osteoclasts are multinucleated monocyte lineage cells with resorptive abilities, forming the ... Full text Cite

The Role of TMIGD1 as a Tumor Suppressor in Colorectal Cancer.

Journal Article Genet Test Mol Biomarkers · April 2022 Background: Colorectal cancer (CRC) is one of the leading causes of cancer-related death, and up to 50% of individuals will suffer relapse. Although transmembrane and immunoglobulin domain-containing protein 1 (TMIGD1) was found to be a protective factor i ... Full text Link to item Cite

CRISPR-SID: Identifying EZH2 as a druggable target for desmoid tumors via in vivo dependency mapping.

Journal Article Proc Natl Acad Sci U S A · November 23, 2021 Cancer precision medicine implies identification of tumor-specific vulnerabilities associated with defined oncogenic pathways. Desmoid tumors are soft-tissue neoplasms strictly driven by Wnt signaling network hyperactivation. Despite this clearly defined g ... Full text Link to item Cite

Tumor-propagating side population cells are a dynamic subpopulation in undifferentiated pleomorphic sarcoma.

Journal Article JCI Insight · November 22, 2021 Sarcomas contain a subpopulation of tumor-propagating cells (TPCs) with enhanced tumor-initiating and self-renewal properties. However, it is unclear whether the TPC phenotype in sarcomas is stable or a dynamic cell state that can derive from non-TPCs. In ... Full text Link to item Cite

Growth Modulation by Stimulating the Growth Plate: A Pilot Study.

Journal Article Ultrasound Med Biol · August 2021 This study investigates the ability of low-intensity pulsed ultrasound (LIPUS) or direct injection of recombinant growth hormone (rGH) to stimulate local growth of long bones. In a randomized controlled animal trial, healthy immature rabbits were allocated ... Full text Link to item Cite

Mutant IDH and non-mutant chondrosarcomas display distinct cellular metabolomes.

Journal Article Cancer Metab · March 24, 2021 BACKGROUND: Majority of chondrosarcomas are associated with a number of genetic alterations, including somatic mutations in isocitrate dehydrogenase 1 (IDH1) and IDH2 genes, but the downstream effects of these mutated enzymes on cellular metabolism and tum ... Full text Link to item Cite

Enchondromatosis and Growth Plate Development.

Journal Article Curr Osteoporos Rep · February 2021 PURPOSE OF REVIEW: Enchondroma is a common cartilage benign tumor that develops from dysregulation of chondrocyte terminal differentiation during growth plate development. Here we provide an overview of recent progress in understanding causative mutations ... Full text Link to item Cite

The Calpain Gene is Correlated With Metal-on-Metal Hip Replacement Failures.

Journal Article J Arthroplasty · January 2021 BACKGROUND: Metal-on-metal (MOM) total hip arthroplasty is associated with unacceptable failure rates secondary to metal ion reactions. Efforts to identify which patients will go on to failure have been limited; recently, there has been a suggestion for a ... Full text Link to item Cite

Parabiosis: Assessing the Effects of Circulating Cells and Factors on the Skeleton.

Chapter · 2021 The circulatory system carries within it numerous types of cells, proteins, and other factors that are able to influence the local biology of tissues. Within this chapter, we present a protocol for parabiosis, a surgical model which results in shared circu ... Full text Link to item Cite

Monocyte/Macrophage Lineage Cells From Fetal Erythromyeloid Progenitors Orchestrate Bone Remodeling and Repair.

Journal Article Front Cell Dev Biol · 2021 A third of the population sustains a bone fracture, and the pace of fracture healing slows with age. The slower pace of repair is responsible for the increased morbidity in older individuals who sustain a fracture. Bone healing progresses through overlappi ... Full text Link to item Cite

Distinct roles of glutamine metabolism in benign and malignant cartilage tumors with IDH mutations

Journal Article · 2021 Enchondromas and chondrosarcomas are common cartilage neoplasms that are either benign or malignant respectively. The majority of these tumors harbor mutations in either IDH1 or IDH2. Glutamine metabolism has been implicated as a critical regulator of tumo ... Full text Cite

Yolk-sac-derived macrophages progressively expand in the mouse kidney with age.

Journal Article Elife · April 17, 2020 Renal macrophages represent a highly heterogeneous and specialized population of myeloid cells with mixed developmental origins from the yolk-sac and hematopoietic stem cells (HSC). They promote both injury and repair by regulating inflammation, angiogenes ... Full text Link to item Cite

The management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients.

Journal Article Eur J Cancer · March 2020 Desmoid tumor (DT; other synonymously used terms: Desmoid-type fibromatosis, aggressive fibromatosis) is a rare and locally aggressive monoclonal, fibroblastic proliferation characterised by a variable and often unpredictable clinical course. Previously su ... Full text Link to item Cite

Complications After Pediatric Hip Fractures: Evaluation and Management.

Journal Article J Am Acad Orthop Surg · January 1, 2020 Pediatric proximal femur fractures are high-energy injuries with predictable and morbid complications. Osteonecrosis of the femoral head is the most common complication with identified risk factors including fracture type, patient's age, degree of displace ... Full text Link to item Cite

Erythromyeloid progenitors give rise to a population of osteoclasts that contribute to bone homeostasis and repair.

Journal Article Nat Cell Biol · January 2020 Osteoclasts are multinucleated cells of the monocyte/macrophage lineage that degrade bone. Here, we used lineage tracing studies-labelling cells expressing Cx3cr1, Csf1r or Flt3-to identify the precursors of osteoclasts in mice. We identified an erythromye ... Full text Link to item Cite

Tracing Tumor Evolution in Sarcoma Reveals Clonal Origin of Advanced Metastasis.

Journal Article Cell Rep · September 10, 2019 Cellular heterogeneity is frequently observed in cancer, but the biological significance of heterogeneous tumor clones is not well defined. Using multicolor reporters and CRISPR-Cas9 barcoding, we trace clonal dynamics in a mouse model of sarcoma. We show ... Full text Link to item Cite

Pharmacologic targeting of β-catenin improves fracture healing in old mice.

Journal Article Sci Rep · June 21, 2019 β-catenin protein needs to be precisely regulated for effective fracture repair. The pace of fracture healing slows with age, associated with a transient increase in β-catenin during the initial phase of the repair process. Here we examined the ability of ... Full text Link to item Cite

Intracellular cholesterol biosynthesis in enchondroma and chondrosarcoma.

Journal Article JCI Insight · April 30, 2019 Enchondroma and chondrosarcoma are the most common benign and malignant cartilaginous neoplasms. Mutations in isocitrate dehydrogenase 1 and 2 (IDH1/2) are present in the majority of these tumors. We performed RNA-seq analysis on chondrocytes from Col2a1Cr ... Full text Link to item Cite

Unique and overlapping GLI1 and GLI2 transcriptional targets in neoplastic chondrocytes.

Journal Article PLoS One · 2019 Excessive Hedgehog (Hh) signaling in chondrocytes is sufficient to cause formation of enchondroma-like lesions which can progress to chondrosarcoma. To elucidate potential underlying mechanisms, we identified GLI1 and GLI2 target genes in human chondrosarc ... Full text Link to item Cite

The Hip in Fibrous Dysplasia

Chapter · January 1, 2019 Fibrous dysplasia is a benign condition caused by a known genetic mutation leading to dysplastic bone formation, which may be accompanied by extra-osseous manifestations including cafe-au-lait spots, precocious puberty, and hyperthyroidism. Clinically, the ... Full text Cite

Macrophage cells secrete factors including LRP1 that orchestrate the rejuvenation of bone repair in mice.

Journal Article Nat Commun · December 5, 2018 The pace of repair declines with age and, while exposure to a young circulation can rejuvenate fracture repair, the cell types and factors responsible for rejuvenation are unknown. Here we report that young macrophage cells produce factors that promote ost ... Full text Open Access Link to item Cite

Eight-year outcomes of a competency-based residency training program in orthopedic surgery.

Journal Article Med Teach · October 2018 Background: The Division of Orthopaedic Surgery at the University of Toronto implemented a pilot residency training program that used a competency-based framework in July of 2009. The competency-based curriculum (CBC) deployed an innovative, modularized ap ... Full text Link to item Cite

Orthopedic and Surgical Management of the Patient With Duchenne Muscular Dystrophy.

Journal Article Pediatrics · October 2018 Orthopedic care is an important aspect of the overall management of patients with Duchenne muscular dystrophy (DMD). In addition to progressive muscle weakness and loss of function, patients may develop joint contractures, scoliosis, and osteoporosis, caus ... Full text Link to item Cite

Intracellular biosynthesis of lipids and cholesterol by Scap and Insig in mesenchymal cells regulates long bone growth and chondrocyte homeostasis.

Journal Article Development · July 9, 2018 During enchondral ossification, mesenchymal cells express genes regulating the intracellular biosynthesis of cholesterol and lipids. Here, we have investigated conditional deletion of Scap or of Insig1 and Insig2 (Scap inhibits intracellular biosynthesis a ... Full text Link to item Cite

Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management.

Journal Article Lancet Neurol · April 2018 A coordinated, multidisciplinary approach to care is essential for optimum management of the primary manifestations and secondary complications of Duchenne muscular dystrophy (DMD). Contemporary care has been shaped by the availability of more sensitive di ... Full text Link to item Cite

The Role of the Immune Cells in Fracture Healing.

Journal Article Curr Osteoporos Rep · April 2018 PURPOSE OF REVIEW: Bone fracture healing is a complex physiological process relying on numerous cell types and signals. Inflammatory factors secreted by immune cells help to control recruitment, proliferation, differentiation, and activation of hematopoiet ... Full text Link to item Cite

Effects of chondroitin sulfate proteoglycan 4 (NG2/CSPG4) on soft-tissue sarcoma growth depend on tumor developmental stage.

Journal Article J Biol Chem · February 16, 2018 Sarcomas, and the mesenchymal precursor cells from which they arise, express chondroitin sulfate proteoglycan 4 (NG2/CSPG4). However, NG2/CSPG4's function and its capacity to serve as a therapeutic target in this tumor type are unknown. Here, we used cells ... Full text Link to item Cite

A Metabolomics Pilot Study on Desmoid Tumors and Novel Drug Candidates.

Journal Article Sci Rep · January 12, 2018 Desmoid tumors (aggressive fibromatosis) are locally invasive soft tissue tumors that lack the ability to metastasize. There are no directed therapies or standard treatment plan, and chemotherapeutics, radiation, and surgery often have temporary effects. T ... Full text Open Access Link to item Cite

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.

Journal Article Am J Med Genet A · January 2018 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 ... Full text Link to item Cite

Editorial Comment.

Journal Article Urology · August 2017 Full text Link to item Cite

The Fourth Year of Medical School: Time for Reassessment: AOA Critical Issues.

Journal Article J Bone Joint Surg Am · July 5, 2017 Most U.S. medical schools follow the 4-year model, consisting of 2 preclinical years, core clinical experience, and a fourth year intended to permit students to increase clinical competency, to explore specialty areas, and to transition to residency. Altho ... Full text Link to item Cite

Pharmacologically targeting beta-catenin for NF1 associated deficiencies in fracture repair.

Journal Article Bone · May 2017 Patients with Neurofibromatosis type 1 display delayed fracture healing and the increased deposition of fibrous tissue at the fracture site. Severe cases can lead to non-union and even congenital pseudarthrosis. Neurofibromatosis type 1 is caused by a muta ... Full text Link to item Cite

Phenotype Determines Nanoparticle Uptake by Human Macrophages from Liver and Blood.

Journal Article ACS Nano · March 28, 2017 A significant challenge to delivering therapeutic doses of nanoparticles to targeted disease sites is the fact that most nanoparticles become trapped in the liver. Liver-resident macrophages, or Kupffer cells, are key cells in the hepatic sequestration of ... Full text Link to item Cite

Mechanism of hard-nanomaterial clearance by the liver.

Journal Article Nat Mater · November 2016 The liver and spleen are major biological barriers to translating nanomedicines because they sequester the majority of administered nanomaterials and prevent delivery to diseased tissue. Here we examined the blood clearance mechanism of administered hard n ... Full text Link to item Cite

β-Catenin modulation in neurofibromatosis type 1 bone repair: therapeutic implications.

Journal Article FASEB J · September 2016 Tibial pseudarthrosis causes substantial morbidity in patients with neurofibromatosis type 1 (NF1). We studied tibial pseudarthrosis tissue from patients with NF1 and found elevated levels of β-catenin compared to unaffected bone. To elucidate the role of ... Full text Link to item Cite

Mesenchymal Tumors Can Derive from Ng2/Cspg4-Expressing Pericytes with β-Catenin Modulating the Neoplastic Phenotype.

Journal Article Cell Rep · July 26, 2016 The cell of origin for most mesenchymal tumors is unclear. One cell type that contributes to this lineages is the pericyte, a cell expressing Ng2/Cspg4. Using lineage tracing, we demonstrated that bone and soft tissue sarcomas driven by the deletion of the ... Full text Link to item Cite

Hedgehog inhibits β-catenin activity in synovial joint development and osteoarthritis.

Journal Article J Clin Invest · May 2, 2016 Both the WNT/β-catenin and hedgehog signaling pathways are important in the regulation of limb development, chondrocyte differentiation, and degeneration of articular cartilage in osteoarthritis (OA). It is not clear how these signaling pathways interact i ... Full text Link to item Cite

Simulation for Teaching Orthopaedic Residents in a Competency-based Curriculum: Do the Benefits Justify the Increased Costs?

Journal Article Clin Orthop Relat Res · April 2016 BACKGROUND: Although simulation-based training is becoming widespread in surgical education and research supports its use, one major limitation is cost. Until now, little has been published on the costs of simulation in residency training. At the Universit ... Full text Link to item Cite

Adynamic Bone Decreases Bone Toughness During Aging by Affecting Mineral and Matrix.

Journal Article J Bone Miner Res · February 2016 Adynamic bone is the most frequent type of bone lesion in patients with chronic kidney disease; long-term use of antiresorptive therapy may also lead to the adynamic bone condition. The hallmark of adynamic bone is a loss of bone turnover, and a major clin ... Full text Link to item Cite

Regulation of Cholesterol Homeostasis by Hedgehog Signaling in Osteoarthritic Cartilage.

Journal Article Arthritis Rheumatol · January 2016 OBJECTIVE: With no effective therapies to attenuate cartilage degeneration in osteoarthritis (OA), the result is pain and disability. Activation of hedgehog (HH) signaling causes changes related to the progression of OA, with higher levels of Gli-mediated ... Full text Link to item Cite

Mutations Preventing Regulated Exon Skipping in MET Cause Osteofibrous Dysplasia.

Journal Article Am J Hum Genet · December 3, 2015 The periosteum contributes to bone repair and maintenance of cortical bone mass. In contrast to the understanding of bone development within the epiphyseal growth plate, factors that regulate periosteal osteogenesis have not been studied as intensively. Os ... Full text Link to item Cite

Construct validity and reliability of a real-time multidimensional smartphone app to assess pain in children and adolescents with cancer.

Journal Article Pain · December 2015 We evaluated the construct validity (including responsiveness), reliability, and feasibility of the Pain Squad multidimensional smartphone-based pain assessment application (app) in children and adolescents with cancer, using 2 descriptive studies with rep ... Full text Link to item Cite

Identification of CD146 as a marker enriched for tumor-propagating capacity reveals targetable pathways in primary human sarcoma.

Journal Article Oncotarget · November 24, 2015 Tumor-propagating cells (TPCs) are believed to drive cancer initiation, progression and recurrence. These cells are characterized by enhanced tumorigenicity and self-renewal. The ability to identify such cells in primary human sarcomas relies on the dye ex ... Full text Link to item Cite

Bone Marrow Stress Decreases Osteogenic Progenitors.

Journal Article Calcif Tissue Int · November 2015 Age-related bone loss may be a result of declining levels of stem cells in the bone marrow. Using the Col2.3Δtk (DTK) transgenic mouse, osteoblast depletion was used as a source of marrow stress in order to investigate the effects of aging on osteogenic pr ... Full text Link to item Cite

The role of hedgehog signalling in skeletal health and disease.

Journal Article Nat Rev Rheumatol · September 2015 Hedgehog ligands bind to protein patched homologue 1 (PTC), a conserved receptor that activates the GLI family of transcription factors, which are involved in development, disease and skeletal repair processes. During embryonic development, hedgehog signal ... Full text Link to item Cite

Macrophages promote osteoblastic differentiation in-vivo: implications in fracture repair and bone homeostasis.

Journal Article J Bone Miner Res · June 2015 Macrophages are activated in inflammation and during early phases of repair processes. Interestingly, they are also present in bone during development, but their function during this process is unclear. Here, we explore the function of macrophages in bone ... Full text Link to item Cite

Generation of articular chondrocytes from human pluripotent stem cells.

Journal Article Nat Biotechnol · June 2015 The replacement of articular cartilage through transplantation of chondrogenic cells or preformed cartilage tissue represents a potential new avenue for the treatment of degenerative joint diseases. Although many studies have described differentiation of h ... Full text Link to item Cite

Exposure to a youthful circulaton rejuvenates bone repair through modulation of β-catenin.

Journal Article Nat Commun · May 19, 2015 The capacity for tissues to repair and regenerate diminishes with age. We sought to determine the age-dependent contribution of native mesenchymal cells and circulating factors on in vivo bone repair. Here we show that exposure to youthful circulation by h ... Full text Link to item Cite

Macrophage-secreted Factors that Rejuvenate Delayed Fracture Healing Characteristic of Aging

Conference JOURNAL OF THE AMERICAN GERIATRICS SOCIETY · April 1, 2015 Link to item Cite

Mutant IDH is sufficient to initiate enchondromatosis in mice.

Journal Article Proc Natl Acad Sci U S A · March 3, 2015 Enchondromas are benign cartilage tumors and precursors to malignant chondrosarcomas. Somatic mutations in the isocitrate dehydrogenase genes (IDH1 and IDH2) are present in the majority of these tumor types. How these mutations cause enchondromas is unclea ... Full text Link to item Cite

Student-led learning: a new teaching paradigm for surgical skills.

Journal Article Am J Surg · January 2015 BACKGROUND: Competency-based education and simulation are being used more frequently in surgical skills curricula. We explored a novel student-led learning paradigm, which allows trainees to become more active participants in the learning process while mai ... Full text Link to item Cite

Optimal therapy for desmoid tumors: current options and challenges for the future.

Journal Article Expert Rev Anticancer Ther · 2015 Desmoid tumors, or aggressive fibromatosis, are rare, locally infiltrative neoplasms caused by mutations that activate β-catenin. Although these tumors do not metastasize, they are difficult to manage due to variability in tumor presentation and behavior. ... Full text Link to item Cite

WNT activation by lithium abrogates TP53 mutation associated radiation resistance in medulloblastoma.

Journal Article Acta Neuropathol Commun · December 24, 2014 TP53 mutations confer subgroup specific poor survival for children with medulloblastoma. We hypothesized that WNT activation which is associated with improved survival for such children abrogates TP53 related radioresistance and can be used to sensitize TP ... Full text Link to item Cite

Parameters for lithium treatment are critical in its enhancement of fracture-healing in rodents.

Journal Article J Bone Joint Surg Am · December 3, 2014 BACKGROUND: Lithium, a treatment for bipolar disorder, is not clinically indicated for use in fracture management but has been reported to positively influence bone biology. It is hypothesized that lithium dosing for beneficial effects on bone health may b ... Full text Link to item Cite

The immature skeleton

Chapter · December 3, 2014 Cite

Disruption of crosstalk between mesenchymal stromal and tumor cells in bone marrow as a therapeutic target to prevent metastatic bone disease.

Journal Article J Cell Physiol · December 2014 Skeletal metastasis is a serious complication of many primary cancers. A common feature of tumor cells that metastasize to the bone marrow microenvironment is that they initiate a cascade of events, recruiting and presumably/potentially altering the phenot ... Full text Link to item Cite

Development, validation and characterization of a novel mouse model of Adynamic Bone Disease (ABD).

Journal Article Bone · November 2014 The etiology of Adynamic Bone Disease (ABD) is poorly understood but the hallmark of ABD is a lack of bone turnover. ABD occurs in renal osteodystrophy (ROD) and is suspected to occur in elderly patients on long-term anti-resorptive therapy. A major clinic ... Full text Link to item Cite

Prestress in the extracellular matrix sensitizes latent TGF-β1 for activation.

Journal Article J Cell Biol · October 27, 2014 Integrin-mediated force application induces a conformational change in latent TGF-β1 that leads to the release of the active form of the growth factor from the extracellular matrix (ECM). Mechanical activation of TGF-β1 is currently understood as an acute ... Full text Link to item Cite

The immature skeleton

Chapter · October 23, 2014 Cite

The immature skeleton

Chapter · September 9, 2014 Cite

β-Catenin-regulated myeloid cell adhesion and migration determine wound healing.

Journal Article J Clin Invest · June 2014 A β-catenin/T cell factor-dependent transcriptional program is critical during cutaneous wound repair for the regulation of scar size; however, the relative contribution of β-catenin activity and function in specific cell types in the granulation tissue du ... Full text Link to item Cite

Hedgehog pathway inhibition in chondrosarcoma using the smoothened inhibitor IPI-926 directly inhibits sarcoma cell growth.

Journal Article Mol Cancer Ther · May 2014 Hedgehog (Hh) pathway inhibition in cancer has been evaluated in both the ligand-independent and ligand-dependent settings, where Hh signaling occurs either directly within the cancer cells or within the nonmalignant cells of the tumor microenvironment. Ch ... Full text Link to item Cite

Activation of hedgehog signaling during fracture repair enhances osteoblastic-dependent matrix formation.

Journal Article J Orthop Res · April 2014 Fracture repair is a well orchestrated process involving various cell types and signaling molecules. The hedgehog signaling pathway is activated in chondrocytes during fracture repair and is known to regulate chondrogenesis however, its role in osteoblasts ... Full text Link to item Cite

Hedgehog signaling regulates cholesterol homeostasis in osteoarthritis

Conference Osteoarthritis and Cartilage · April 2014 Full text Cite

Percutaneous screw fixation promotes healing of lateral condyle nonunion in children.

Journal Article J Pediatr Orthop · March 2014 BACKGROUND: This retrospective study examined whether pediatric lateral condyle nonunions could be successfully managed by percutaneous screw fixation. We report the outcome of this minimally invasive technique avoiding open reduction and bone grafting ass ... Full text Link to item Cite

Involvement and targeted intervention of dysregulated Hedgehog signaling in osteosarcoma.

Journal Article Cancer · February 15, 2014 BACKGROUND: During development, the Hedgehog pathway plays important roles regulating the proliferation and differentiation of chondrocytes, providing a template for growing bone. In this study, the authors investigated the components of dysregulated Hedge ... Full text Link to item Cite

Patient outcomes in the operative and nonoperative management of high-grade spondylolisthesis in children.

Journal Article J Pediatr Orthop · 2014 BACKGROUND: The optimal management of high-grade spondylolisthesis in the growing child is controversial. Some authors have advocated for surgery in all cases regardless of symptoms. Surgical intervention results in a >10% risk of complications with increa ... Full text Link to item Cite

Letter to the editor response.

Journal Article J Surg Educ · 2014 Full text Link to item Cite

On the shoulders of giants: The future of the Journal of Orthopaedic Research

Journal Article Journal of Orthopaedic Research · January 1, 2014 Full text Cite

Primary cilia attenuate hedgehog signalling in neoplastic chondrocytes.

Journal Article Oncogene · November 21, 2013 Primary cilia can act as either a negative or positive regulator of the hedgehog (Hh) signaling pathway. Many cartilage tumors are characterized by abnormal activation of the Hh pathway. Here, we report that the presence of primary cilia occurs at a low fr ... Full text Link to item Cite

Reflections on current methods for evaluating skills during joint replacement surgery: a scoping review.

Journal Article Bone Joint J · November 2013 Valid and reliable techniques for assessing performance are essential to surgical education, especially with the emergence of competency-based frameworks. Despite this, there is a paucity of adequate tools for the evaluation of skills required during joint ... Full text Link to item Cite

Toronto orthopaedic boot camp III: examining the efficacy of student-regulated learning during an intensive, laboratory-based surgical skills course.

Journal Article Surgery · July 2013 BACKGROUND: Previous studies have presented compelling data that a 1-month "boot-camp"-style course can be a highly effective mechanism for teaching and developing targeted technical skills. In the current study, we examine whether performance of these tar ... Full text Link to item Cite

Targeting stem cell behavior in desmoid tumors (aggressive fibromatosis) by inhibiting hedgehog signaling.

Journal Article Neoplasia · July 2013 Desmoid tumor (also called aggressive fibromatosis) is a lesion of mesenchymal origin that can occur as a sporadic tumor or a manifestation of the preneoplastic syndrome, familial adenomatous polyposis caused by a mutation in adenomatous polyposis coli (AP ... Full text Link to item Cite

Glucocorticoid treatment for the prevention of scoliosis in children with Duchenne muscular dystrophy: long-term follow-up.

Journal Article J Bone Joint Surg Am · June 19, 2013 BACKGROUND: Duchenne muscular dystrophy, a progressive muscle disorder that occurs in males, causes a gradual decline in muscle strength. This progressive decline is associated with the development of scoliosis. Previous studies have shown that the use of ... Full text Link to item Cite

Cutaneous wound healing: recruiting developmental pathways for regeneration.

Journal Article Cell Mol Life Sci · June 2013 Following a skin injury, the damaged tissue is repaired through the coordinated biological actions that constitute the cutaneous healing response. In mammals, repaired skin is not identical to intact uninjured skin, however, and this disparity may be cause ... Full text Link to item Cite

Specification of chondrocytes and cartilage tissues from embryonic stem cells.

Journal Article Development · June 2013 Osteoarthritis primarily affects the articular cartilage of synovial joints. Cell and/or cartilage replacement is a promising therapy, provided there is access to appropriate tissue and sufficient numbers of articular chondrocytes. Embryonic stem cells (ES ... Full text Link to item Cite

Are quantum dots toxic? Exploring the discrepancy between cell culture and animal studies.

Journal Article Acc Chem Res · March 19, 2013 Despite significant interest in developing quantum dots (QDs) for biomedical applications, many researchers are convinced that QDs will never be used for treating patients because of their potential toxicity. The perception that QDs are toxic is rooted in ... Full text Link to item Cite

Development and testing of a multidimensional iPhone pain assessment application for adolescents with cancer.

Journal Article J Med Internet Res · March 8, 2013 BACKGROUND: Pain is one of the most common and distressing symptoms reported by adolescents with cancer. Despite advancements in pain assessment and management research, pain due to cancer and/or its treatments continues to be poorly managed. Our research ... Full text Link to item Cite

Patients' views on surgeons' financial conflicts of interest.

Journal Article J Bone Joint Surg Am · January 16, 2013 BACKGROUND: The U.S. Department of Justice's investigations into financial relationships between surgical device manufacturers and orthopaedic surgeons have raised the question as to whether surgeons can continue to collaborate with industry and maintain p ... Full text Link to item Cite

Primary cilia attenuate hedgehog signalling in neoplastic chondrocytes

Journal Article Oncogene · January 1, 2013 Primary cilia can act as either a negative or positive regulator of the hedgehog (Hh) signaling pathway. Many cartilage tumors are characterized by abnormal activation of the Hh pathway. Here, we report that the presence of primary cilia occurs at a low fr ... Full text Cite

Competency-based education: a new model for teaching orthopaedics.

Journal Article Instr Course Lect · 2013 The current methods used to train residents to become orthopaedic surgeons are based on tradition, not evidence-based models. Educators have only a limited ability to assess trainees for competency using validated tests in various domains. The reduction in ... Link to item Cite

Suppressor of fused (Sufu) mediates the effect of parathyroid hormone-like hormone (Pthlh) on chondrocyte differentiation in the growth plate.

Journal Article J Biol Chem · October 19, 2012 Growth plate chondrocytes undergo a coordinated process of differentiation, regulating long bone growth. Parathyroid hormone-like hormone (Pthlh) inhibits hypertrophic differentiation in the growth plate chondrocytes and reduces Hedgehog (Hh) signaling. In ... Full text Link to item Cite

RNA extraction from human articular cartilage by chondrocyte isolation.

Journal Article Anal Biochem · October 1, 2012 We report an optimized method for RNA extraction from human articular cartilage that does not require the use of specialized equipment or column purification. To maximize RNA yield while minimizing degradation and contamination, chondrocytes are isolated f ... Full text Link to item Cite

Abnormal fatty acid metabolism in spinal muscular atrophy may predispose to perioperative risks.

Journal Article Eur J Paediatr Neurol · September 2012 A 15 year old boy with SMA type II underwent spinal fusion and suffered a mitochondrial Reye-like catabolic crisis 4 days postop with hypoketotic hypoglycemia, lactic acidaemia, hyperammonemia and liver failure, with 90% coagulative necrosis and diffuse ma ... Full text Link to item Cite

Open reduction and internal fixation of unstable slipped capital femoral epiphysis by means of surgical dislocation does not decrease the rate of avascular necrosis: A preliminary study

Journal Article Journal of Children's Orthopaedics · August 1, 2012 Purpose: The treatment of unstable slipped capital femoral epiphysis (SCFE) remains controversial. Surgical dislocation and open reduction has the potential to significantly reduce the rate of avascular necrosis (AVN) by allowing direct preservation of the ... Full text Cite

Orthopaedic Boot Camp II: examining the retention rates of an intensive surgical skills course.

Journal Article Surgery · June 2012 BACKGROUND: We examined retention rates for basic surgical skills taught through a 1-month intensive laboratory boot camp-style course at the onset of residency. METHODS: We present data from 3 groups, each composed of 6 residents. The first group consiste ... Full text Link to item Cite

The Canadian experience with long-term deflazacort treatment in Duchenne muscular dystrophy.

Journal Article Acta Myol · May 2012 Deflazacort is the most commonly prescribed corticosteroid for the treatment of Duchenne muscular dystrophy in Canada. We review the long-term experience with deflazacort treatment at two centers in Canada; Montreal and Toronto. Deflazacort has benefitted ... Link to item Cite

The Canadian experience with long term deflazacort treatment in Duchenne muscular dystrophy

Journal Article Acta Myologica · May 1, 2012 Deflazacort is the most commonly prescribed corticosteroid for the treatment of Duchenne muscular dystrophy in Canada. We review the long term experience with deflazacort treatment at two centers in Canada; Montreal and Toronto. Deflazacort has benefitted ... Cite

A mechanism for gene-environment interaction in the etiology of congenital scoliosis.

Journal Article Cell · April 13, 2012 Congenital scoliosis, a lateral curvature of the spine caused by vertebral defects, occurs in approximately 1 in 1,000 live births. Here we demonstrate that haploinsufficiency of Notch signaling pathway genes in humans can cause this congenital abnormality ... Full text Link to item Cite

Hedgehog and Notch signaling regulate self-renewal of undifferentiated pleomorphic sarcomas.

Journal Article Cancer Res · February 15, 2012 Like many solid tumors, sarcomas are heterogeneous and include a small fraction of the so-called side population (SP) cells with stem-like tumor-initiating potential. Here, we report that SP cells from a soft tissue tumor of enigmatic origin termed undiffe ... Full text Link to item Cite

Desmoid tumors: Are they benign or malignant?

Chapter · January 1, 2012 The distinction between benign and malignant tumors is classically based on the metastatic potential of a tumor type. While desmoid tumors do not metastasize and as such are classified as benign lesions, their clinical behavior, cellular biology, and molec ... Full text Cite

A high throughput screen identifies Nefopam as targeting cell proliferation in β-catenin driven neoplastic and reactive fibroproliferative disorders.

Journal Article PLoS One · 2012 Fibroproliferative disorders include neoplastic and reactive processes (e.g. desmoid tumor and hypertrophic scars). They are characterized by activation of β-catenin signaling, and effective pharmacologic approaches are lacking. Here we undertook a high th ... Full text Link to item Cite

A new Cre driver mouse line, Tcf21/Pod1-Cre, targets metanephric mesenchyme.

Journal Article PLoS One · 2012 Conditional gene targeting in mice has provided great insight into the role of gene function in kidney development and disease. Although a number of Cre-driver mouse strains already exist for the kidney, development of additional strains with unique expres ... Full text Link to item Cite

T-lymphocytes enable osteoblast maturation via IL-17F during the early phase of fracture repair.

Journal Article PLoS One · 2012 While it is well known that the presence of lymphocytes and cytokines are important for fracture healing, the exact role of the various cytokines expressed by cells of the immune system on osteoblast biology remains unclear. To study the role of inflammato ... Full text Link to item Cite

The first successful lower extremity transplantation: 6-year follow-up and implications for cortical plasticity.

Journal Article Am J Transplant · December 2011 Vascularized composite allotransplantation as a viable reconstructive option is gaining recognition and new cases are being reported with increasing frequency including hand, face and laryngeal transplantation. However, only one successful complete lower l ... Full text Link to item Cite

Heal thyself: using endogenous regeneration to repair bone.

Journal Article Tissue Eng Part B Rev · December 2011 Bone has the capacity to repair itself after an injury, and this occurs in normal fracture repair. This reparative process can be harnessed to regenerate segments of bone using distraction osteogenesis, in which the healing bone is slowly stretched. The us ... Full text Link to item Cite

Osteoid osteoma and osteoblastoma.

Journal Article J Am Acad Orthop Surg · November 2011 Osteoid osteoma and osteoblastoma are commonly seen benign osteogenic bone neoplasms. Both tumors are typically seen in the second decade of life, with a notable predilection in males. Histologically, these tumors resemble each other, with characteristical ... Full text Link to item Cite

Pax7 expressing cells contribute to dermal wound repair, regulating scar size through a β-catenin mediated process.

Journal Article Stem Cells · September 2011 During skin wound healing, fibroblast-like cells reconstitute the dermal compartment of the repaired skin filling the wound gap. A subset of these cells are transcriptionally active for β-catenin/T-cell factor (TCF) signaling during the proliferative phase ... Full text Link to item Cite

Kif7 promotes hedgehog signaling in growth plate chondrocytes by restricting the inhibitory function of Sufu.

Journal Article Development · September 2011 Proper regulation of Indian hedgehog (Ihh) signaling is vital for chondrocyte proliferation and differentiation in the growth plate. Its dysregulation causes skeletal dysplasia, osteoarthritis or cartilaginous neoplasia. Here, we show that Suppressor of fu ... Full text Link to item Cite

Fibronectin and beta-catenin act in a regulatory loop in dermal fibroblasts to modulate cutaneous healing.

Journal Article J Biol Chem · August 5, 2011 β-Catenin is an important regulator of dermal fibroblasts during cutaneous wound repair. However, the factors that modulate β-catenin activity in this process are not completely understood. We investigated the role of the extracellular matrix in regulating ... Full text Link to item Cite

Don't hedge your bets: hedgehog signaling as a central mediator of endochondral bone development and cartilage diseases.

Journal Article J Orthop Res · June 2011 Cell differentiation and patterning are vital processes in the development of the appendicular skeleton. The hedgehog (Hh) signaling pathway plays a central role in regulating the anterior-posterior axis of the distal limb as well as the length of endochon ... Full text Link to item Cite

Orthopedic boot camp: examining the effectiveness of an intensive surgical skills course.

Journal Article Surgery · June 2011 BACKGROUND: Changes in health care across the globe have had a profound impact on the number of hands-on surgical training opportunities that are available to residents. In the current study, we examine whether an intensive laboratory-based skills course a ... Full text Link to item Cite

Testosterone regulates cell proliferation in aggressive fibromatosis (desmoid tumour).

Journal Article Br J Cancer · April 26, 2011 BACKGROUND: Aggressive fibromatosis (desmoid tumour) is a locally invasive tumour caused by mutations resulting in β-catenin protein stabilisation. Apc1638N mice are predisposed to developing aggressive fibromatosis tumours, and male mice develop greater n ... Full text Link to item Cite

Familial adenomatous polyposis-associated desmoids display significantly more genetic changes than sporadic desmoids.

Journal Article PLoS One · 2011 Desmoid tumours (also called deep or aggressive fibromatoses) are potentially life-threatening fibromatous lesions. Hereditary desmoid tumours arise in individuals affected by either familial adenomatous polyposis (FAP) or hereditary desmoid disease (HDD) ... Full text Link to item Cite

Genetic Diagnosis of Skeletal Dysplasias

Journal Article · December 1, 2010 This chapter discusses the molecular genetic diagnosis of skeletal dysplasia. Skeletal dysplasias are mentioned to be a distinct group of disorders in which the skeletal abnormalities are caused by an intrinsic derangement in bone development. Among the la ... Full text Cite

A novel fibrotic disorder associated with increased dermal fibroblast proliferation and downregulation of genes of the microfibrillar network.

Journal Article Br J Dermatol · November 2010 Clinical evaluation of a young woman with subcutaneous fibrotic nodules, progressive distal joint contractures and marfanoid stature revealed a previously unrecognized fibrotic disorder characterized by several unique phenotypic features and some features ... Full text Link to item Cite

Aggressive fibromatosis (desmoid tumor) is derived from mesenchymal progenitor cells.

Journal Article Cancer Res · October 1, 2010 The cellular origins from which most tumors arise are poorly defined, especially in mesenchymal neoplasms. Aggressive fibromatosis, also known as desmoid tumor, is a locally invasive soft tissue tumor that has mesenchymal characteristics. We found that agg ... Full text Link to item Cite

Ultrafast mid-IR laser scalpel: protein signals of the fundamental limits to minimally invasive surgery.

Journal Article PLoS One · September 28, 2010 Lasers have in principle the capability to cut at the level of a single cell, the fundamental limit to minimally invasive procedures and restructuring biological tissues. To date, this limit has not been achieved due to collateral damage on the macroscale ... Full text Link to item Cite

Cartilage tumours and bone development: molecular pathology and possible therapeutic targets.

Journal Article Nat Rev Cancer · July 2010 As a group, cartilage tumours are the most common primary bone lesions. They range from benign lesions, such as enchondromas and osteochondromas, to malignant chondrosarcoma. The benign lesions result from the deregulation of the hedgehog signalling pathwa ... Full text Link to item Cite

Robert Bruce Salter, C.C., MD, FRCSC. Dec 15, 1924-May 10, 2010

Journal Article Journal of Children's Orthopaedics · June 29, 2010 Full text Cite

Pilot assessment of a radiologic classification system for segmentation defects of the vertebrae.

Journal Article Am J Med Genet A · June 2010 Existing nomenclature systems for describing and reporting congenital segmentation defects of the vertebrae (SDV) are confusing, inconsistently applied, and lack molecular genetic advances. Our aim was to develop and assess a new classification system for ... Full text Link to item Cite

Pathogenesis of radiation-induced capsular contracture in tissue expander and implant breast reconstruction.

Journal Article Plast Reconstr Surg · February 2010 BACKGROUND: Capsular contracture is the main complication in postmastectomy tissue expander and implant breast reconstruction in patients requiring radiotherapy. There is evidence that the wingless signaling pathway plays a central role in the pathogenesis ... Full text Link to item Cite

Protecting the hedgerow: p53 and hedgehog pathway interactions.

Journal Article Cell Cycle · February 1, 2010 A common environment for the Hedgehog (Subfamily: erinaceinae) is a row of shrubs and trees often used on farms for enclosing or separating fields, called a hedgerow. Maintenance of a continuous shrub border is important for shielding crops from weather da ... Full text Link to item Cite

Overview and comparison of idiopathic, neuromuscular, and congenital forms of scoliosis

Journal Article · January 1, 2010 Scoliosis is really a physical finding, a lateral curvature of the spine. There are a number of potential causes, not all of which are related to a primary spinal deformity. For instance, if a patient has one leg longer than another, they will display a cu ... Full text Cite

Modulating hedgehog signaling can attenuate the severity of osteoarthritis.

Journal Article Nat Med · December 2009 Osteoarthritis is associated with the irreversible degeneration of articular cartilage. Notably, in this condition, articular cartilage chondrocytes undergo phenotypic and gene expression changes that are reminiscent of their end-stage differentiation in t ... Full text Link to item Cite

Gli2 and p53 cooperate to regulate IGFBP-3- mediated chondrocyte apoptosis in the progression from benign to malignant cartilage tumors.

Journal Article Cancer Cell · August 4, 2009 Clinical evidence suggests that benign cartilage lesions can progress to malignant chondrosarcoma, but the molecular events in this progression are unknown. Mice that develop benign cartilage lesions due to overexpression of Gli2 in chondrocytes developed ... Full text Link to item Cite

Beta-catenin and transforming growth factor beta have distinct roles regulating fibroblast cell motility and the induction of collagen lattice contraction.

Journal Article BMC Cell Biol · May 11, 2009 BACKGROUND: beta-catenin and transforming growth factor beta signaling are activated in fibroblasts during wound healing. Both signaling pathways positively regulate fibroblast proliferation during this reparative process, and the effect of transforming gr ... Full text Link to item Cite

Beta-catenin mediates soft tissue contracture in clubfoot.

Journal Article Clin Orthop Relat Res · May 2009 The contracted tissues from clubfeet resemble tissues from other fibroproliferative disorders such as palmar fibromatosis. Beta-catenin-mediated signaling is a crucial pathway controlling the fibroproliferative response in many fibroproliferative disorders ... Full text Link to item Cite

Oligodontia is caused by mutation in LTBP3, the gene encoding latent TGF-beta binding protein 3.

Journal Article Am J Hum Genet · April 2009 We have identified a consanguineous Pakistani family where oligodontia is inherited along with short stature in an autosomal-recessive fashion. Increased bone density was present in the spine and at the base of the skull. Using high-density single-nucleoti ... Full text Link to item Cite

Wnt pathway, an essential role in bone regeneration.

Journal Article J Cell Biochem · February 15, 2009 Fracture repair is a complex regenerative process initiated in response to injury, resulting in optimal restoration of skeletal function. Although histology characteristics at various phases of fracture repair are clear and well established, much remains t ... Full text Link to item Cite

Progress in the understanding of the genetic etiology of vertebral segmentation disorders in humans.

Journal Article Ann N Y Acad Sci · January 2009 Vertebral malformations contribute substantially to the pathophysiology of kyphosis and scoliosis, common health problems associated with back and neck pain, disability, cosmetic disfigurement, and functional distress. This review explores (1) recent advan ... Full text Link to item Cite

Beta-catenin is a mediator of the response of fibroblasts to irradiation.

Journal Article Am J Pathol · January 2009 Radiation causes soft tissue complications that include fibrosis and deficient wound healing. beta-Catenin, a key component in the canonical Wnt-signaling pathway, is activated in fibrotic processes and wound repair and, as such, could play a role in media ... Full text Link to item Cite

Skin-derived precursors differentiate into skeletogenic cell types and contribute to bone repair.

Journal Article Stem Cells Dev · 2009 Skin-derived precursors (SKPs) are multipotent dermal precursors that share similarities with neural crest stem cells and that can give rise to peripheral neural and some mesodermal cell types, such as adipocytes. Here, we have asked whether rodent or huma ... Full text Link to item Cite

Parathyroid hormone-related protein regulates glioma-associated oncogene transcriptional activation: lessons learned from bone development and cartilage neoplasia.

Journal Article Ann N Y Acad Sci · November 2008 Hedgehog and parathyroid hormone-related protein (PTHrP) signaling play important roles regulating the differentiation of chondrocytes, which form the template for growing bone. By studying the interaction of the pathways in normal and neoplastic growth-pl ... Full text Link to item Cite

Radiation effects and radioprotection in MC3T3-E1 mouse calvarial osteoblastic cells.

Journal Article Plast Reconstr Surg · October 2008 BACKGROUND: Little is known about the mechanisms and treatment of radiation-induced inhibition of craniofacial bone growth. In an earlier study, the radioprotector amifostine (WR-2721) administered to rabbits before irradiation radioprotected cultured orbi ... Full text Link to item Cite

Molecular diagnosis of vertebral segmentation disorders in humans.

Journal Article Expert Opin Med Diagn · October 2008 BACKGROUND: Vertebral malformations contribute substantially to the pathophysiology of kyphosis and scoliosis, common health problems associated with back and neck pain, disability, cosmetic disfigurement and functional distress. OBJECTIVE: To provide an o ... Full text Link to item Cite

Side population cells in human cancers.

Journal Article Cancer Lett · September 8, 2008 Cancer stem cells (CSCs) are found in multiple tumor types. While the presence of surface markers selectively expressed on CSCs are used to isolate these cells, no marker or pattern of makers are known to prospectively identify CSCs in many tumor types. In ... Full text Link to item Cite

Patched-one or smoothened gene mutations are infrequent in chondrosarcoma.

Journal Article Clin Orthop Relat Res · September 2008 Constitutive hedgehog signaling has been implicated in the tumorigenesis of cartilaginous neoplasia; however, a common mutational mechanism remains unknown. Some tumors exhibiting hedgehog pathway activation such as basal cell cancer frequently harbor PATC ... Full text Link to item Cite

Beta-catenin in the race to fracture repair: in it to Wnt.

Journal Article Nat Clin Pract Rheumatol · August 2008 The Wnt/beta-catenin pathway regulates multiple biological events during embryonic development, including bone formation. Fracture repair recapitulates some of the processes of normal bone development, such as the formation of bone from a cartilaginous tem ... Full text Link to item Cite

Diagnosis of limb-girdle muscular dystrophy 2A by immunohistochemical techniques.

Journal Article Neuropathology · June 2008 The Western blot technique is currently the standard detection method for suspected limb girdle muscular dystrophy (LGMD) 2A (calpainopathy). This is the first report in the English literature of the successful application of immunohistochemical techniques ... Full text Link to item Cite

Allograft airway fibrosis in the pulmonary milieu: a disorder of tissue remodeling.

Journal Article Am J Transplant · March 2008 Obliterative bronchiolitis (OB) is thought to be a form of chronic allograft rejection. However, immunosuppressive therapy is not effective once fibrosis has developed. We hypothesize that disordered tissue remodeling is a mechanism for the pathogenesis of ... Full text Link to item Cite

Skeletal dysplasias and the growth plate.

Journal Article Clin Genet · January 2008 Skeletal dysplasias are disorders in which there is derangement in the growth or shape of the skeleton. Long bone grows from cartilage that persists near the ends until skeletal maturity as the growth plate. Developmental biology work has identified the ma ... Full text Link to item Cite

Are surgeons' preferences for instrumentation related to patient outcomes? A randomized clinical trial of two implants for idiopathic scoliosis.

Journal Article J Bone Joint Surg Am · December 2007 BACKGROUND: Although many techniques for the surgical treatment of scoliosis have been described, we are not aware of any randomized trials that have compared implant systems. The relationship between surgeons' preferences for implants and patient outcomes ... Full text Link to item Cite

Improvement in quality of life following surgery for adolescent idiopathic scoliosis.

Journal Article Spine (Phila Pa 1976) · November 15, 2007 STUDY DESIGN: We used the Climent Quality of Life for Spinal Deformities Scale prospectively in a nonrandomized prospective comparative cohort of operative versus observational management of adolescent idiopathic scoliosis. OBJECTIVE: To compare the change ... Full text Link to item Cite

Side population cells isolated from mesenchymal neoplasms have tumor initiating potential.

Journal Article Cancer Res · September 1, 2007 Although many cancers are maintained by tumor-initiating cells, this has not been shown for mesenchymal tumors, in part due to the lack of unique surface markers that identify mesenchymal progenitors. An alternative technique to isolate stem-like cells is ... Full text Link to item Cite

Does spinal fusion influence quality of life in neuromuscular scoliosis?

Journal Article Spine (Phila Pa 1976) · September 1, 2007 STUDY DESIGN: : Systematic literature review of articles pertaining to quality of life (QOL) in neuromuscular scoliosis patients that underwent spinal fusion. OBJECTIVE: : To determine if QOL is improved by scoliosis surgery in neuromuscular patients. SUMM ... Full text Link to item Cite

IFN-{beta} signaling positively regulates tumorigenesis in aggressive fibromatosis, potentially by modulating mesenchymal progenitors.

Journal Article Cancer Res · August 1, 2007 Aggressive fibromatosis (also called desmoid tumor) is a benign, locally invasive, soft tissue tumor composed of cells with mesenchymal characteristics. These tumors are characterized by increased levels of beta-catenin-mediated T-cell factor (TCF)-depende ... Full text Link to item Cite

Beta-catenin signaling plays a disparate role in different phases of fracture repair: implications for therapy to improve bone healing.

Journal Article PLoS Med · July 31, 2007 BACKGROUND: Delayed fracture healing causes substantial disability and usually requires additional surgical treatments. Pharmacologic management to improve fracture repair would substantially improve patient outcome. The signaling pathways regulating bone ... Full text Link to item Cite

Surgical decision making in adolescent idiopathic scoliosis.

Journal Article Spine (Phila Pa 1976) · June 15, 2007 STUDY DESIGN: Cross-sectional survey. OBJECTIVE: The objective of this paper was to assess the reliability of surgeons' decision-making in adolescent idiopathic scoliosis (AIS) based on patient photographs and clinical and radiographic data. SUMMARY OF BAC ... Full text Link to item Cite

Opportunities for improving the therapeutic ratio for patients with sarcoma.

Journal Article Lancet Oncol · June 2007 Sarcomas are mesenchymal cancers, which, in many cases, have distinctive molecular features. Limb-sparing surgery delivered at specialised sarcoma centres as part of a multidisciplinary approach has become the standard treatment for most patients and usual ... Full text Link to item Cite

Abnormal vertebral segmentation and the notch signaling pathway in man.

Journal Article Dev Dyn · June 2007 Abnormal vertebral segmentation (AVS) in man is a relatively common congenital malformation but cannot be subjected to the scientific analysis that is applied in animal models. Nevertheless, some spectacular advances in the cell biology and molecular genet ... Full text Link to item Cite

A randomized, controlled trial of a removable brace versus casting in children with low-risk ankle fractures.

Journal Article Pediatrics · June 2007 OBJECTIVES: Isolated distal fibular ankle fractures in children are very common and at very low risk for future complications. Nevertheless, standard therapy for these fractures still consists of casting, a practice that carries risks, inconveniences, and ... Full text Link to item Cite

Inhibition of notch signaling induces neural differentiation in Ewing sarcoma.

Journal Article Am J Pathol · May 2007 Cells from Ewing sarcoma exhibit cellular features and express markers, suggesting that the tumor is of neuroectodermal origin. Because Notch signaling regulates the differentiation of neuroectodermal cells during development, we examined the role of Notch ... Full text Link to item Cite

PTHrP regulates growth plate chondrocyte differentiation and proliferation in a Gli3 dependent manner utilizing hedgehog ligand dependent and independent mechanisms.

Journal Article Dev Biol · May 1, 2007 Growth plate chondrocytes undergo a tightly regulated process of differentiation, allowing for the longitudinal growth of bones. Although it is known that parathyroid hormone related protein (PTHrP) and Indian hedgehog regulate the differentiation of growt ... Full text Link to item Cite

Human sterile alpha motif domain 9, a novel gene identified as down-regulated in aggressive fibromatosis, is absent in the mouse.

Journal Article BMC Genomics · April 3, 2007 BACKGROUND: Neoplasia can be driven by mutations resulting in dysregulation of transcription. In the mesenchymal neoplasm, aggressive fibromatosis, subtractive hybridization identified sterile alpha motif domain 9 (SAMD9) as a substantially down regulated ... Full text Link to item Cite

Surgeon reliability in rating physical deformity in adolescent idiopathic scoliosis.

Journal Article Spine (Phila Pa 1976) · February 1, 2007 STUDY DESIGN: Cross sectional survey. OBJECTIVES: To compare pediatric spine surgeons' relative rankings of the importance of surgical considerations, and their reliability of ratings of the physical deformity of patients with adolescent idiopathic scolios ... Full text Link to item Cite

Beta-catenin signaling pathway is crucial for bone morphogenetic protein 2 to induce new bone formation.

Journal Article J Biol Chem · January 5, 2007 Endochondral ossification is recapitulated during bone morphogenetic protein (BMP)-induced ectopic bone formation. Although BMP and beta-catenin have been investigated in bone development and in mesenchymal cells, how they interact in this process is not c ... Full text Link to item Cite

An association between the 4G polymorphism in the PAI-1 promoter and the development of aggressive fibromatosis (desmoid tumor) in familial adenomatous polyposis patients.

Journal Article Fam Cancer · 2007 Aggressive fibromatosis is a mesenchymal neoplasm associated with mutations resulting in beta-catenin mediated transcriptional activation. Plasminogen activator inhibitor-1 (PAI-1) is expressed at a high level in aggressive fibromatosis, and using transgen ... Full text Link to item Cite

Scoliosis: Review of diagnosis and treatment

Journal Article Paediatrics and Child Health · January 1, 2007 Scoliosis is a spinal deformity consisting of lateral curvature and rotation of the vertebrae. The causes of scoliosis vary and are classified broadly as congenital, neuromuscular, syndrome-related, idiopathic and spinal curvature due to secondary reasons. ... Full text Cite

Parents' and patients' perceptions of postoperative appearance in adolescent idiopathic scoliosis.

Journal Article Spine (Phila Pa 1976) · September 15, 2006 STUDY DESIGN: A cross-sectional evaluation of patients after surgery for adolescent idiopathic scoliosis (AIS). OBJECTIVES: To determine the agreement between patients' and parents' perceptions of the patient's postoperative appearance and to compare those ... Full text Link to item Cite

First successful lower-extremity transplantation: technique and functional result.

Journal Article J Reconstr Microsurg · May 2006 Composite tissue transplantation has emerged as a viable alternative to prosthetics and complex reconstructive surgery. Thus far it is reserved for cases which cannot be effectively reconstructed and where it offers some benefits over prostheses. It has be ... Full text Link to item Cite

Beta-catenin regulates wound size and mediates the effect of TGF-beta in cutaneous healing.

Journal Article FASEB J · April 2006 After cutaneous injury, a variety of cell types are activated to reconstitute the epithelial and dermal components of the skin. beta-Catenin plays disparate roles in keratinocytes and fibroblasts, inhibiting keratinocyte migration and activating fibroblast ... Full text Link to item Cite

Long-term benefits of deflazacort treatment for boys with Duchenne muscular dystrophy in their second decade.

Journal Article Neuromuscul Disord · April 2006 We compare the clinical course of 74 boys 10-18 years of age with Duchenne muscular dystrophy (DMD) treated (40) and not treated (34) with deflazacort. Treated boys were able to rise from supine to standing, climb stairs and walk 10 m without aids, 3-5 yea ... Full text Link to item Cite

Constitutive hedgehog signaling in chondrosarcoma up-regulates tumor cell proliferation.

Journal Article Am J Pathol · January 2006 Chondrosarcoma is a malignant cartilage tumor that may arise from benign precursor lesions, such as enchondromas. Some cases of multiple enchondromas are caused by a mutation that results in constitutive activation of Hedgehog-mediated signaling. We found ... Full text Link to item Cite

Dysregulation of hedgehog signalling predisposes to synovial chondromatosis.

Journal Article J Pathol · June 2005 Synovial chondromatosis is a condition affecting joints in which metaplastic cartilage nodules arise from the synovium, causing pain, joint dysfunction, and ultimately joint destruction. Because dysregulation of hedgehog signalling is a feature of several ... Full text Link to item Cite

Prolonged beta-catenin stabilization and tcf-dependent transcriptional activation in hyperplastic cutaneous wounds.

Journal Article Lab Invest · March 2005 Mesenchymal cells that accumulate during the proliferative phase of wound healing and that are present in hyperplastic wounds share cytologic similarities with the cells from fibroproliferative lesions in which there is activation of beta-catenin-mediated ... Full text Link to item Cite

Plasminogen activator inhibitor-1 (PAI-1) modifies the formation of aggressive fibromatosis (desmoid tumor).

Journal Article Oncogene · February 24, 2005 Aggressive fibromatosis is a mesenchymal neoplasm associated with mutations, resulting in beta-catenin-mediated transcriptional activation. We found that plasminogen activator inhibitor-1 (PAI-1) was upregulated fourfold in aggressive fibromatosis. We inve ... Full text Link to item Cite

Should foot surgery be performed for children with Duchenne muscular dystrophy?

Journal Article J Pediatr Orthop · 2005 The authors conducted a retrospective study to determine the outcome of foot surgery in full-time wheelchair users with Duchenne muscular dystrophy. Medical records on all 88 teenaged boys with Duchenne muscular dystrophy treated at the authors' institutio ... Full text Link to item Cite

The effect of surgical delay on acute infection following 554 open fractures in children.

Journal Article J Bone Joint Surg Am · January 2005 BACKGROUND: Traditional recommendations hold that open fractures in both children and adults require urgent surgical debridement for a number of reasons, including the preservation of soft-tissue viability and vascular status as well as the prevention of i ... Full text Link to item Cite

Bone Morphogenetic Proteins Are Expressed by Both Bone-Forming and Non-Bone-Forming Lesions.

Journal Article Arch Pathol Lab Med · November 2004 Context.-Bone morphogenetic proteins (BMPs) are thought to be responsible for bone formation; they cause bone to form in soft tissues and are clinically used in helping fracture union or tumor reconstructions. Skeletal metastases from epithelial tumors may ... Full text Link to item Cite

Deflazacort in Duchenne muscular dystrophy: a comparison of two different protocols.

Journal Article Neuromuscul Disord · September 2004 We compare the long-term benefits and side effects of deflazacort using two treatment protocols from Naples (N) and Toronto (T). Boys with Duchenne muscular dystrophy between the ages of 8 and 15 years and who had four or more years of deflazacort treatmen ... Full text Link to item Cite

Matrix metalloproteinase activity modulates tumor size, cell motility, and cell invasiveness in murine aggressive fibromatosis.

Journal Article Cancer Res · August 15, 2004 Matrix metalloproteinases (MMPs) and tissue inhibitors of MMPs (TIMPs) regulate the degradation of extracellular matrix components and play important roles in the progression of select neoplastic processes. The locally invasive soft tissue tumor, aggressiv ... Full text Link to item Cite

Invasion and MMP expression profile in desmoid tumours.

Journal Article Br J Cancer · April 5, 2004 Desmoid tumours are locally invasive soft tissue tumours in which beta-catenin mediated TCF-dependent transcription is activated. The role of soluble factors secreted by the myofibroblastic desmoid tumour, which could stimulate tumour invasiveness, was inv ... Full text Link to item Cite

Orthopaedic manifestations of Brachmann-de Lange syndrome: a report of 34 patients.

Journal Article J Pediatr Orthop B · March 2004 Brachmann-de Lange syndrome (BDLS) is a disorder of unknown cause that is recognized on the basis of characteristic facies in association with growth retardation, mental retardation and, in many cases, upper limb anomalies. Because of its association with ... Full text Link to item Cite

Steroid treatment and the development of scoliosis in males with duchenne muscular dystrophy.

Journal Article J Bone Joint Surg Am · March 2004 BACKGROUND: Scoliosis due to progressive muscle weakness occurs in almost all males with Duchenne muscular dystrophy, and it progresses relentlessly. Previous studies have shown that corticosteroid treatment slows the decline in muscle strength and stabili ... Full text Link to item Cite

Growth factors regulate beta-catenin-mediated TCF-dependent transcriptional activation in fibroblasts during the proliferative phase of wound healing.

Journal Article Exp Cell Res · February 15, 2004 Beta-catenin is a critical regulator of cell behavior during embryogenesis and neoplastic processes. It also plays a crucial role in repair by modulating dermal fibroblast activity during the proliferative phase of cutaneous wound healing. We hypothesize t ... Full text Link to item Cite

Identification of IGFBP-6 as a significantly downregulated gene by beta-catenin in desmoid tumors.

Journal Article Oncogene · January 22, 2004 Desmoid tumors (aggressive fibromatosis) are locally invasive soft tissue tumors in which beta-catenin-mediated TCF-3-dependent transcription is activated. To provide more insight into the pathophysiology of these tumors, expression profiles were generated ... Full text Link to item Cite

Complications of elastic stable intramedullary nail fixation of pediatric femoral fractures, and how to avoid them.

Journal Article J Pediatr Orthop · 2004 Flexible intramedullary nailing has become a popular method of fixation of pediatric femoral fractures. The authors analyzed their first 5-year experience with titanium elastic stable intra-medullary nailing, specifically to report the complications associ ... Full text Link to item Cite

Defective Limb Embryology

Chapter · December 1, 2003 Full text Cite

NOV (CCN3) regulation in the growth plate and CCN family member expression in cartilage neoplasia.

Journal Article J Pathol · December 2003 Growth plate chondrocytes undergo a coordinated differentiation process resulting in terminal differentiation and new bone formation. Enchondromas are pre-malignant, benign cartilaginous lesions that arise from growth plate chondrocytes that fail to underg ... Full text Link to item Cite

Genetic deletion of receptor for hyaluronan-mediated motility (Rhamm) attenuates the formation of aggressive fibromatosis (desmoid tumor).

Journal Article Oncogene · October 9, 2003 Aggressive fibromatosis (desmoid tumor) is a locally invasive soft tissue neoplasm associated with mutations resulting in beta-catenin-mediated transcriptional activation. This tumor is composed of cells with histological and molecular characteristics comm ... Full text Link to item Cite

Elevated levels of beta-catenin and fibronectin in three-dimensional collagen cultures of Dupuytren's disease cells are regulated by tension in vitro.

Journal Article BMC Musculoskelet Disord · July 16, 2003 BACKGROUND: Dupuytren's contracture or disease (DD) is a fibro-proliferative disease of the hand that results in the development of scar-like, collagen-rich disease cords within specific palmar fascia bands. Although the molecular pathology of DD is unknow ... Full text Link to item Cite

Beta-catenin expression in Dupuytren's disease: potential role for cell-matrix interactions in modulating beta-catenin levels in vivo and in vitro.

Journal Article Oncogene · June 12, 2003 Dupuytren's disease (DD) is a superficial fibromatosis of the hand. Although the molecular mechanisms responsible for this disease are unknown, recent studies suggest that beta-catenin may be a key factor involved in fibromatosis. In this study, we analyse ... Full text Link to item Cite

Developmental pathways in musculoskeletal neoplasia: involvement of the Indian Hedgehog-parathyroid hormone-related protein pathway.

Journal Article Pediatr Res · April 2003 There are many crucial genes and signaling pathways in the proper development of an organism. Pathologies may arise from a deregulation of these pathways. The Indian Hedgehog-PTH-related protein (Ihh-PTHrP) pathway is vital in the proper development of end ... Full text Link to item Cite

Eosinophilic granuloma. A different behaviour in children than in adults.

Journal Article J Bone Joint Surg Br · August 2002 Localised Langerhans-cell histiocytosis of bone (eosinophilic granuloma) is a benign tumour-like condition with a variable clinical course. Different forms of treatment have been reported to give satisfactory results. However, previous series all contain p ... Full text Link to item Cite

A classification for genetic disorders of interest to orthopaedists.

Journal Article Clin Orthop Relat Res · August 2002 The genetic etiology of many disorders of interest to the orthopaedist now is known. Based on this information, these disorders can be grouped broadly based on the function of the causative gene into five categories: structural, tumor and cell regulatory, ... Full text Link to item Cite

Idiopathic scoliosis in families of children with congenital scoliosis.

Journal Article Clin Orthop Relat Res · August 2002 Although most cases of congenital scoliosis are thought to be of sporadic etiology, it is not known whether other types of spinal deformity occur in families of individuals with this type of scoliosis. Children with congenital scoliosis were identified thr ... Full text Link to item Cite

beta-Catenin stabilization dysregulates mesenchymal cell proliferation, motility, and invasiveness and causes aggressive fibromatosis and hyperplastic cutaneous wounds.

Journal Article Proc Natl Acad Sci U S A · May 14, 2002 Fibroproliferative processes are a group of disorders in which there is excessive proliferation of spindle (mesenchymal fibroblast-like) cells. They range from hypertrophic scars to neoplasms such as aggressive fibromatosis. Cells from these disorders shar ... Full text Link to item Cite

A mutant PTH/PTHrP type I receptor in enchondromatosis.

Journal Article Nat Genet · March 2002 Enchondromas are common benign cartilage tumors of bone. They can occur as solitary lesions or as multiple lesions in enchondromatosis (Ollier and Maffucci diseases). Clinical problems caused by enchondromas include skeletal deformity and the potential for ... Full text Link to item Cite

Sensitivity of a clinical examination to predict need for radiography in children with ankle injuries: a prospective study.

Journal Article Lancet · December 22, 2001 BACKGROUND: Radiographs are ordered routinely for children with ankle trauma. We assessed the predictive value of a clinical examination to identify a predefined group of low-risk injuries, management of which would not be affected by absence of a radiogra ... Full text Link to item Cite

Suppressor of fused negatively regulates beta-catenin signaling.

Journal Article J Biol Chem · October 26, 2001 Suppressor of fused (Su(fu)) is a negative regulator of the Hedgehog signaling pathway that controls the nuclear-cytoplasmic distribution of Gli/Ci transcription factors through direct protein-protein interactions. We show here that Su(fu) is present in a ... Full text Link to item Cite

Tcf-3 expression and beta-catenin mediated transcriptional activation in aggressive fibromatosis (desmoid tumour).

Journal Article Br J Cancer · July 6, 2001 Aggressive fibromatosis harbours mutations resulting in beta-catenin protein stabilization. Primary cell cultures demonstrate constitutive tcf activation in aggressive fibromatosis. Expression and co-immunoprecipitation studies suggest that beta-catenin bi ... Full text Link to item Cite

Short stature as a screening test for endocrinopathy in slipped capital femoral epiphysis.

Journal Article J Bone Joint Surg Br · March 2001 Slipped capital femoral epiphysis may be associated with hypothyroidism and other endocrinopathies. Routine screening for such abnormalities is unlikely to be cost-effective since the overall incidence of these disorders, in association with slipped capita ... Full text Link to item Cite

Local complications after limb-salvage surgery for pediatric bone tumours: a pictorial essay.

Journal Article Can Assoc Radiol J · February 2001 The prognosis for patients with bone sarcoma treated with LSS has improved considerably over the past 2 decades, but this has also lead to an increase in the number of complications requiring treatment. Imaging plays an important role, not only in assessin ... Link to item Cite

Cyclooxygenase-two (COX-2) modulates proliferation in aggressive fibromatosis (desmoid tumor).

Journal Article Oncogene · January 25, 2001 Aggressive fibromatosis is a locally invasive soft tissue lesion. Seventy-five per cent of cases harbor a somatic mutation in either the APC or beta-catenin genes, resulting in beta-catenin protein stabilization. Cyclooxygenase-2 (COX-2) is an enzyme invol ... Full text Link to item Cite

Potential treatment for clubfeet based on growth factor blockade.

Journal Article J Pediatr Orthop · 2001 Select soft tissues in clubfeet are contracted, resulting in stiffness. These contracted tissues share ultrastructural characteristics with palmar fibromatosis (Dupuytren contracture), in which the growth factors transforming growth factor-beta (TGF-beta) ... Link to item Cite

Congenital clubfoot

Journal Article Current Paediatrics · January 1, 2001 Congenital clubfoot, one of the most common musculoskeletal anomalies, varies considerably in severity and its response to treatment. The aetiology, pathogenesis and treatment have been subjects of wide debate since antiquity. Even today, there is little c ... Full text Cite

A germline mutation at the extreme 3' end of the APC gene results in a severe desmoid phenotype and is associated with overexpression of beta-catenin in the desmoid tumor.

Journal Article Clin Genet · March 2000 Desmoid tumors arise sporadically or as part of the extraintestinal manifestations of familial adenomatous polyposis (FAP). In FAP, two distinct clinical presentations of the desmoid phenotype are seen: 1) one or a few desmoid tumors present predominantly ... Full text Link to item Cite

Prominent expression of mRNA for proinflammatory cytokines in synovium in patients with juvenile rheumatoid arthritis or chronic Lyme arthritis.

Journal Article J Rheumatol · February 2000 OBJECTIVE: To examine the cytokine profiles in synovium of patients with juvenile rheumatoid arthritis (JRA) or Lyme arthritis, 2 chronic inflammatory arthritides that affect children. METHODS: We used in situ hybridization with specific riboprobes to dete ... Link to item Cite

A shortened course of parenteral antibiotic therapy in the management of acute septic arthritis of the hip.

Journal Article J Pediatr Orthop · 2000 We reviewed 20 consecutive patients with a culture-proven acute septic arthritis of the hip who were treated with a shortened course of parenteral antibiotic therapy after a surgical drainage. Patients were switched over to an oral antibiotic when they sho ... Link to item Cite

Predominance of beta-catenin mutations and beta-catenin dysregulation in sporadic aggressive fibromatosis (desmoid tumor).

Journal Article Oncogene · November 11, 1999 Aggressive fibromatosis (also called desmoid tumor) occurs as a sporadic lesion or as part of Familial Adenomatous Polyposis, which is caused by germ line mutations in the Adenomatous polyposis Coli (APC) gene. APC is involved in the regulation of the cell ... Full text Link to item Cite

Expression of osteocalcin and its transcriptional regulators core-binding factor alpha 1 and MSX2 in osteoid-forming tumours.

Journal Article J Orthop Res · September 1999 Osteosarcoma, fibrous dysplasia, and myositis ossificans contain osteoid-producing cells that are not necessarily morphologically typical osteoblasts. Nevertheless, these pathologic cells may share differentiation steps with osteoblasts at the molecular le ... Full text Link to item Cite

Pelvic obliquity after fusion of the spine in Duchenne muscular dystrophy.

Journal Article J Bone Joint Surg Br · September 1999 Spinal fusion, ending caudally at L5 rather than at the sacrum, is recommended for selected patients with scoliosis due to Duchenne muscular dystrophy. We present a retrospective review of 48 patients operated on for this condition. Patients having spinal ... Full text Link to item Cite

Congenital clubfoot

Journal Article Current Orthopaedics · January 1, 1999 Full text Cite

Adenomatous polyposis coli gene mutation alters proliferation through its beta-catenin-regulatory function in aggressive fibromatosis (desmoid tumor).

Journal Article Am J Pathol · September 1998 Aggressive fibromatosis is a monoclonal proliferation of spindle (fibroblast-like) cells. A subset of lesions contain somatic truncating adenomatous polyposis coli (APC) gene mutations, and all of the lesions contain an elevated beta-catenin protein level. ... Full text Link to item Cite

Increased beta-catenin protein and somatic APC mutations in sporadic aggressive fibromatoses (desmoid tumors).

Journal Article Am J Pathol · August 1997 Sporadic aggressive fibromatosis (also called desmoid tumor) is a monoclonal proliferation of spindle (fibrocyte-like) cells that is locally invasive but does not metastasize. A similarity to abdominal fibromatoses (desmoids) in familial adenomatous polypo ... Link to item Cite

Differential collagen I gene expression in fetal fibroblasts.

Journal Article J Pediatr Surg · July 1997 PURPOSE: Fetal wound healing is characterized by the regeneration of normal dermis and the absence of scar. Transforming growth factor beta-1 (TGF-beta1) is a ubiquitous cytokine with potent fibrogenic effects in both postnatal and fetal wounds. Supplement ... Full text Link to item Cite

Aggressive fibromatosis (desmoid tumor) is a monoclonal disorder.

Journal Article Diagn Mol Pathol · April 1997 Aggressive fibromatosis (also called deep fibromatosis or desmoid tumor) is a proliferation of cytologically benign-appearing fibrocytes, often resulting in significant functional loss. The nature of the lesion is controversial: some evidence suggests that ... Full text Link to item Cite

APC mutations are present in some sporadic aggressive fibromatosis.

Journal Article LABORATORY INVESTIGATION · 1997 Cite

Etiology and treatment of fibrous dysplasia

Journal Article Current Opinion in Orthopaedics · January 1, 1997 Fibrous dysplasia is a benign bone lesion composed of a proliferation of spindle cells producing dysplastic bone. It occurs in a monostotic form, a polyostic form, or as part of McCune-Albright syndrome (polyostotic form with endocrinopathies). In all thre ... Full text Cite

Regulation of proliferation and platelet-derived growth factor expression in palmar fibromatosis (Dupuytren contracture) by mechanical strain.

Journal Article J Orthop Res · September 1996 Palmar fibromatosis (Dupuytren contracture) causes fibrosis of specific palmar fascial bands. These bands are subjected to repetitive mechanical strain in situ. Primary cell cultures were derived from (a) palmar fibromatosis from eight patients, (b) uninvo ... Full text Link to item Cite

Molecular genetic and immunohistochemical analysis of the tumor suppressor genes Rb and p53 in palmar and aggressive fibromatosis.

Journal Article Diagn Mol Pathol · September 1996 This pilot project analyzed the tumor suppressor genes p53 and Rb in 13 cases of aggressive fibromatoses and six cases of palmar fibromatoses (Dupuytren contracture). Immunohistochemistry, reverse transcription polymerase chain reaction, polymerase chain r ... Full text Link to item Cite

Function of dislocated hips in children with lower level spina bifida.

Journal Article J Bone Joint Surg Br · March 1996 We reviewed 52 children, born between 1974 and 1985 with spina bifida affecting L3 and L4, who had dislocated hips. Their motor function was stable and they were able to walk at the time of dislocation. They were interviewed and examined physically and rad ... Link to item Cite

Activating mutations of Gs protein in monostotic fibrous lesions of bone.

Journal Article J Orthop Res · March 1996 Activating mutations of the alpha chain of the heterotrimeric signal transducer Gs disrupt the inherent guanosine triphosphatase activity of the alpha chain, stimulate adenylyl cyclase, and can result in independent cell proliferation. Such mutations are i ... Full text Link to item Cite

Function of dislocated hips in children with lower level spina bifida.

Journal Article The Journal of bone and joint surgery. British volume · March 1996 We reviewed 52 children, born between 1974 and 1985 with spina bifida affecting L3 and L4, who had dislocated hips. Their motor function was stable and they were able to walk at the time of dislocation. They were interviewed and examined physically and rad ... Cite

Activating Gs mutations in monostotic fibrous lesions of bone

Journal Article LABORATORY INVESTIGATION · 1996 Cite

Growth factor and cytokine expression in osteolytic bone metastases

Journal Article LABORATORY INVESTIGATION · 1996 Cite

Prenatal diagnosis and the pediatric surgeon: the impact of prenatal consultation on perinatal management.

Journal Article J Pediatr Surg · January 1996 PURPOSE: Pediatric surgeons are increasingly called on by obstetrical colleagues to counsel parents about the implications of a prenatal ultrasound finding. Our understanding of the natural history of many prenatally diagnosed surgical conditions has grown ... Full text Link to item Cite

Proximal femoral focal deficiency: results of rotationplasty and Syme amputation.

Journal Article J Bone Joint Surg Am · December 1995 We reviewed the results of treatment of sixteen patients who had had an isolated unilateral proximal femoral focal deficiency; nine were managed with a rotationplasty and seven, with a Syme amputation combined with an arthrodesis of the knee. We evaluated ... Full text Link to item Cite

Amniotic band syndrome in fetal lambs. I: Fetoscopic release and morphometric outcome.

Journal Article J Pediatr Surg · July 1995 A fetal lamb model of amniotic band syndrome (ABS) was developed to study the pathophysiology of banded extremities and evaluate the possibility of in utero treatment with the potential for functional recovery. Eight fetal lambs underwent banding of their ... Full text Link to item Cite

Massive allografts in the treatment of osteosarcoma and Ewing sarcoma in children and adolescents.

Journal Article J Bone Joint Surg Am · January 1995 A retrospective review was performed of the results of all allograft reconstructions that had been done after the resection of an osteosarcoma or an Ewing sarcoma in a skeletally immature patient between 1982 and 1989 at The Hospital for Sick Children in T ... Full text Link to item Cite

Platelet-derived growth factor in fibrous musculoskeletal disorders: a study of pathologic tissue sections and in vitro primary cell cultures.

Journal Article J Orthop Res · January 1995 Despite the great variability in the clinical behavior of fibrous lesions of the musculoskeletal system, they are composed of cytologically similar fibrocytes. Receptors for estrogen or progesterone, or both, are present in some of these lesions and some i ... Full text Link to item Cite

The expression of platelet-derived growth-factor gene in Dupuytren contracture.

Journal Article J Bone Joint Surg Am · January 1995 Dupuytren contracture is a disease of the palmar fascia characterized by nodular fibroblastic proliferation; its etiology and pathogenesis are poorly understood. Growth factors are polypeptides that regulate cell growth and differentiation and extracellula ... Full text Link to item Cite

Giant marginal ulcer.

Journal Article Surg Endosc · February 1994 Marginal ulcer is a well-known complication of gastroenterostomy. It occurs in 3% of patients post-Billroth II subtotal gastrectomy; it occurs in less than 1% if truncal vagotomy is included but in up to 30% of patients with gastroenterostomy without vagot ... Full text Link to item Cite

Digital nerves of the foot: anatomic variations and implications regarding the pathogenesis of interdigital neuroma.

Journal Article Foot Ankle · May 1993 Seventy-one cadaveric feet were dissected, with attention to communicating branches of the digital nerves, the diameters of the digital nerves, the distance between the metatarsal heads, and the presence or absence of interdigital neuromas. A communicating ... Full text Link to item Cite

Subtalar arthrodesis for stabilization of valgus hindfoot in patients with cerebral palsy.

Journal Article J Pediatr Orthop · 1993 All patients with spastic cerebral palsy who underwent correction of valgus hindfoot by Grice extraarticular subtalar arthrodesis between 1971 and 1986 performed by two surgeons using an identical technique were reviewed. Twenty-nine patients (53 feet) wer ... Link to item Cite

Femur fractures in institutionalized patients after hip spica immobilization.

Journal Article J Pediatr Orthop · 1993 The charts of 77 severely handicapped institutionalized children and young adults were retrospectively reviewed to assess the incidence of femur fractures. No fractures occurred in ambulatory patients. Four of 37 nonambulatory patients who did not have hip ... Link to item Cite

Aggressive fibromatosis.

Journal Article J Pediatr Orthop · January 1992 Ten patients with aggressive fibromatosis of the extremities were prospectively followed for 2-6 years. Results of treatment methods were compared. Five patients underwent three-dimensional imaging with and without intravenous contrast, and the images were ... Link to item Cite

Solitary osteochondroma of the clavicle.

Journal Article J Pediatr Orthop · 1991 There are no clinicopathologic reports of solitary osteochondroma of the clavicle other than listings in tumor registries. Two boys had solitary asymptomatic osteochondroma, in one at the medial and in the other at the lateral aspect of the clavicle. One u ... Full text Link to item Cite

Fracture failure mechanisms in patients with osteogenesis imperfecta.

Journal Article J Orthop Res · 1987 This investigation examines the failure mechanism of bone from patients with osteogenesis imperfecta (OI). Mechanical testing and ultrastructural analysis of surgically obtained bone specimens, from patients with OI and from approximately age matched norma ... Full text Link to item Cite