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Skeletal Embryology and Limb Growth
Published in Manoj Ramachandran, Tom Nunn, Basic Orthopaedic Sciences, 2018
Rick Brown, Anish Sanghrajka, Deborah Eastwood
Hereditary multiple osteochondromatosis (HMO, diaphyseal aclasia) is the most common skeletal tumour-like dysplasia, with an incidence of 1 in 50,000. It is characterized by the growth of cartilage-capped benign bone tumours arising from the metaphysis towards the diaphysis. HMO is an autosomal dominant disorder, with 96% penetrance, linked to mutations in the EXT1 and EXT2 genes (the EXT1 gene is associated with greater involvement). The risk of sarcomatous transformation has been reported as 0.57%.
Orthopaedic oncology
Published in Pankaj Sharma, Nicola Maffulli, Practice Questions in Trauma and Orthopaedics for the FRCS, 2017
Pankaj Sharma, Nicola Maffulli
Hereditary multiple osteochondromatosis is inherited in an auto-somal dominant manner, and most commonly occurs in the first decade of life. The risk of malignant transformation is 25–30%, and sessile lesions carry a higher risk of malignant transformation. A cartilage cap greater than 2 cm in diameter raises the suspicion of malignant transformation.
Musculoskeletal
Published in Vincent Helyar, Aidan Shaw, The Final FRCR, 2017
Rare, also known as hereditary multiple osteochondromatosis; it is inherited by autosomal dominance. Most patients are diagnosed by 5 years of age. There is short stature in 40%. About 5% become malignant.
Chondrosarcoma in Norway 1990–2013; an epidemiological and prognostic observational study of a complete national cohort
Published in Acta Oncologica, 2019
Joachim Thorkildsen, Ingeborg Taksdal, Bodil Bjerkehagen, Hans Kristian Haugland, Tom Børge Johannesen, Trond Viset, Ole-Jacob Norum, Øyvind Bruland, Olga Zaikova
Table 2 depicts the patient demographics of the studied cohort. It shows an overall equal gender split with mean age 55 y.o.a. The exception to this was peripheral subtype with a male:female ratio of 1.8:1 and mean age of 35 y.o.a. Both peripheral and dedifferentiated subtypes have on average larger tumors than central CS and overall. While central CS appeared equally as often in the extremities as in axial skeleton, peripheral and dedifferentiated subtypes appeared approx. 90% in the extremities and only 10% in the axial skeleton. Peripheral CS presented mostly as grade 1 malignancy grade (56%) while central CS presented more evenly as 33% grade 1, 40% grade 2 and 24% grade 3 disease, respectively. Extremity central CS presented as 46% grade 1, 34% grade 2 and 20% grade 3 while axial central CS as 21%, 49% and 30%, respectively. While 41% of the peripheral CS population had an underlying syndrome (multiple osteochondromatosis), this was much more seldom (approx. 5%) for central CS (Ollier/Maffucci) and dedifferentiated types. Grade 1 CS was most common in early adulthood with a gradual decline with age. Dedifferentiated disease conversely, presented first in the third decade of life and gradually increased in occurrence.
PEG-PLGA- hybrid nanoparticles loaded with etoricoxib – phospholipid complex for effective treatment of inflammation in rat model
Published in Journal of Microencapsulation, 2019
Vivek Dave, Prarthana Srivastava, Kajal Tak, Swapnil Sharma
Rheumatoid arthritis is an auto-immune disorder which involves joint inflammation, synovial proliferation (Synovial proliferation is the proliferation in the synovium or joint-lining tissue which changes to bone-forming cartilages and cause the disease known as synovial osteochondromatosis) and destruction of articular cartilage. IgM immune complex activates complement and liberates TNF-α and IL-1 which act as chemotactic for neutrophils. Affected inflammatory cell secretes lysozomal enzyme which causes cartilage damage, erode bone and vasodilation is caused due to release of prostaglandins. NSAIDS (Non-steroidal anti-inflammatory drugs which inhibit the prostaglandin synthesis by blocking COX-1 and COX-2 enzymes) are the 1st line of drugs generally opted for symptomatic relief in pain, swelling, morning stiffness, immortality. NSAIDs are mainly used to inhibit the COX mediated synthesis of prostaglandins, which are considered as an important intermediate in the development of inflammation and pain. Glucocorticoids and disease modifying anti-rheumatic drugs (Diseases modifying anti-rheumatic drugs are used for the treatment of rheumatoid arthritis. They are of two types: Traditional (methotrexate, leflunomide, sulfasalazine etc.) and Biologics (cyclophosphamide, cyclosporine, and tacrolimus) along with the NSAIDs are part of initial drug therapy in rheumatoid arthritis. It helps in the management of other rheumatic disease caused by chronic musculo-skeleton pain, acute pain and provides symptomatic relief. Etoricoxib is a potent COX-2 inhibitor chemically known as 5-chloro-6′-methyl-3-[4-(methylsulfonyl) phenyl]-2, 3′bipyridine. It is primarily indicated in the management of inflammation, pain, fever, acute and chronic osteoarthritis and rheumatoid arthritis. It exhibits poor aqueous solubility which results in delayed therapeutic action with low bioavailability. It exhibits greater selectivity for COX-2 over COX-1.