Extractions: Ollier disease is a rare skeletal disorder characterized by abnormal bone development (skeletal dysplasia). While this disorder may be present at birth (congenital); it may not become apparent until early childhood when symptoms, such as deformities or improper limb growth, are more obvious. Ollier disease primarily affects the long bones and cartilage of the joints of the arms and legs, specifically the area where the shaft and head of a long bone meet (metaphyses). The pelvis is often involved; and even more rarely, the ribs, breast bone (sternum), and/or skull may also be affected. Ollier disease manifests as greater than normal growth of the cartilage in the long bones of the legs and arms so that growth is abnormal and the outer layer (cortical bone) of the bone becomes thin and more fragile. These masses of cartilage are benign (non-cancerous) tumors known as enchondromas. Enchondromas may occur at anytime. After puberty these growths stabilize as cartilage is replaced by bone. In rare cases, the enchondromas may undergo malignant changes (e.g., chondrosarcomas). The exact cause of Ollier disease is not known, although in some cases it may be inherited as an autosomal dominant genetic trait.
Maffucci Syndrome Dyschondrodysplasia with Hemangiomas; enchondromatosis with Multiple CavernousHemangiomas; Hemangiomatosis Chondrodystrophica; Kast Syndrome http://my.webmd.com/hw/raising_a_family/nord433.asp
Extractions: Maffucci Syndrome is a rare genetic disorder characterized by benign overgrowths of cartilage (enchondromas), skeletal deformities, and dark red irregularly shaped patches of skin (hemangiomas). Enchondromas are most often found in certain bones (phalanges) of the hands and feet. Skeletal malformations may include legs that are disproportionate in length and/or abnormal side-to-side curvature of the spine (scoliosis). In many cases, bones may tend to fracture easily. In most cases, hemangiomas appear at birth or during early childhood and may be progressive. Maffucci Syndrome is inherited as an autosomal dominant genetic trait. Resources
Enchondromatosis Complete online version of The Encyclopaedia of Medical Imaging including textand images from The Encyclopaedia of Medical Imaging s eight book volumes http://www.amershamhealth.com/medcyclopaedia/medical/Volume III 1/ENCHONDROMATOS
Extractions: financial services our commitment our company Search Medcyclopaedia for: Search marked text (mark text before you click) Browse entry words starting with: A B C D ... amershamhealth.com Enchondromatosis, presence of multiple foci of cartilage within bones and subperiosteal deposition of cartilage, involving one side of the body exclusively or predominantly. This disorder is nonhereditary and is also termed Ollier's disease. The tumours occur most frequently in the extremities but may also be found in the flat bones, such as those of the pelvis. Affected bones may be shortened and deformed. Children with enchondromatosis may be susceptible to pathologic fractures, and adults may be at risk of malignant transformation to chondrosarcoma Radiographically, the tumours may appear as linear or columnar radiolucent regions in the metaphyses or flat bones, which represent sites of persistent cartilaginous tissue ( Fig.1 ). Erosion and proliferation of the bone surface are sometimes observed. A nonhereditary enchondromatosis with soft tissue haemangiomas that may undergo malignant transformation is known as Maffuccis syndrome
Multiple Enchondromatosis Complete online version of The Encyclopaedia of Medical Imaging including textand images from The Encyclopaedia of Medical Imaging s eight book volumes http://www.amershamhealth.com/medcyclopaedia/medical/Volume VII/MULTIPLE ENCHOND
Radiology Case 438-3114 The lesions have well demarcated endosteal margins. Diagnosis, enchondromatosis,acroform, distal, expression. More information http://myweb.lsbu.ac.uk/dirt/museum/438-3114.html
Extractions: Web resources for "Enchondromatosis" English French German Spanish = Site with HON description - = Site with a robot description info: enter the site: (click below) domain of the site: HONcode - eMedicine - Maffucci Syndrome : Article by Raymond T Kuwahara, MD www.emedicine.com HONcode - NORD - National Organization for Rare Disorders, Inc. www.rarediseases.org II. Congenital and Hereditary Bone Disorders edcenter.med.cornell.edu HONcode - Virtual Children's Hospital: Paediapaedia: Enchondromatosis www.vh.org
Extractions: @import "/ng/style.css"; NATURE.COM NEWS@NATURE.COM NATUREJOBS NATUREEVENTS ... Help E-MAIL: PASSWORD: Save password Forgotten password? SEARCH JOURNAL Advanced search Journal home Archive Table of Contents ... For librarians NPG Resources Nature Nature Cell Biology Nature Medicine Nature Reviews Genetics ... Browse all publications Letter Nature Genetics Sevan Hopyan , Nalan Gokgoz , Raymond Poon , Robert C. Gensure , Chunying Yu , William G. Cole , Robert S. Bell , Irene L. Andrulis , Jay S. Wunder Program in Developmental Biology, Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada. Program in Molecular Biology and Cancer, Samuel Lunenfeld Research Institute, Mount Sinai Hospital, Toronto, Ontario M5G1X5, Canada. Department of Surgery, Division of Orthopaedics, University of Toronto, Toronto, Ontario, Canada. Institute of Medical Science, University of Toronto, Toronto, Ontario, Canada. Endocrine and Pediatric Endocrine Units, Departments of Medicine and Pediatrics, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, USA. Division of Orthopaedics, The Hospital for Sick Children, Toronto, Ontario, Canada.
Extractions: It is possible that the main title of the report Ollier Disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report. Ollier disease is a rare skeletal disorder characterized by abnormal bone development (skeletal dysplasia). While this disorder may be present at birth (congenital); it may not become apparent until early childhood when symptoms, such as deformities or improper limb growth, are more obvious. Ollier disease primarily affects the long bones and cartilage of the joints of the arms and legs, specifically the area where the shaft and head of a long bone meet (metaphyses). The pelvis is often involved; and even more rarely, the ribs, breast bone (sternum), and/or skull may also be affected. Ollier disease manifests as greater than normal growth of the cartilage in the long bones of the legs and arms so that growth is abnormal and the outer layer (cortical bone) of the bone becomes thin and more fragile. These masses of cartilage are benign (non-cancerous) tumors known as enchondromas. Enchondromas may occur at anytime. After puberty these growths stabilize as cartilage is replaced by bone. In rare cases, the enchondromas may undergo malignant changes (e.g., chondrosarcomas). The exact cause of Ollier disease is not known, although in some cases it may be inherited as an autosomal dominant genetic trait.
Enchondromatosis - Talk Medical Humanfriendly medical definition of enchondromatosis. http://www.talkmedical.com/medical-dictionary/4846/Enchondromatosis
Extractions: Tell a friend Enchondromatosis: A condition characterized by multiple enchondromas benign masses of cartilage growing within bones. The enchondromas can deform and shorten a limb and predispose to a fracture. The condition can be caused by a mutation in the gene for the parathyroid hormone receptor (PTHR1). Also known as Ollier disease. Print this page About Talk Medical Help Contact Us ... Terms and Conditions
Extractions: This Article Full Text Full Text (PDF) Letters to the Editor: Submit a response ... Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Reprints and Permissions PubMed PubMed Citation Articles by GABOS, P. G. Articles by BOWEN, J. R. Related Collections Pediatric Disease The Journal of Bone and Joint Surgery, Inc. PETER G. GABOS, M.D. and J. RICHARD BOWEN, M.D. , WILMINGTON, DELAWARE Investigation performed at the Alfred I. duPont Hospital for Children, Wilmington We reviewed the cases of eight unrelated children who had an unusual form of enchondromatosis characterized by unilateral enchondromas arising within the epiphyseal and metaphyseal regions of the long tubular bones of the lower extremity. Unlike previously
Entrez PubMed enchondromatosis (Ollier disease, Maffucci syndrome) is a rare developmentaldisorder characterized by multiple enchondromas. Not much is known about its http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=1
