Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027121 (myositis)
4,538 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Among diagnostic progress over the last three years in internal medicine, Antisynthetase Syndrome is now more easily recognised with the diffusion of laboratory tests for research of antibodies against tRNA synthetases (Anti JO1, anti PL7, Anti PL12). In two third of cases, these antibodies are found despite absence of antinuclear antibodies. Hence, we have to search them specifically in patients with polyarthritis associated with myositis, cutaneous manifestations (Raynaud phenomenom and "mechanic'hands") and interstitial lung disease. Discovery of asymptomatic mutation in the L ferritin coding sequence help us to better understand the "unexplained" hyperferritinemia. Initially described by japonese gastroenterologists, auto immune pancreatitis in fact a part of a systemic sclerosing disease with a biochemical hallmark: in crease of a subclass of immunoglobulins G (IgG4). A new pediatric disease due to a deficiency of the interleukin1 receptor antagonist (multifocal aseptic osteitis, periostitis, stomatitis, disseminated pustulosis) help us to better understand unexplained auto inflammatory diseases. The therapeutic progress is primarily due to an explosion of biological therapies, particularly four of them very useful for internists (in an off label use) : Interleukin 1 inhibitors (anakinra, Canakinumab) to treat some auto inflammatory diseases (cryopirin associated periodic syndromes and deficency of interleukin 1 receptor antagonist), monoclonal antibody against interleukin 5 (mepolizumab) to treat some hypereosinophilic syndromes and Churg and Strauss angiitis, interleukin 6 inhibitiors to treat multifocal Castleman's disease and adult Still disease, a monoclonal antibody against vascular endothelial growth factor (Bevacizumab) to treat hereditary hemorrhagic telangiectasia.
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PMID:[What's new in internal medicine?]. 2011 57

Parosteal osteosarcoma and low-grade central osteosarcoma are two types of low-grade osteosarcoma that show similar clinical behaviors, histological features, and genetic background (ie, amplified sequences of 12q13-15, including MDM2 and CDK4). Low-grade osteosarcoma is often confused with benign lesions, and ancillary techniques to enhance diagnostic accuracy have been awaited. This study explores the use of MDM2 and CDK4 immunohistochemistry for the histological diagnosis of low-grade osteosarcoma. We studied 23 cases of low-grade osteosarcoma from 21 patients (parosteal osteosarcoma (n=14), low-grade central osteosarcoma (n=9)) and 40 cases of benign histological mimics (myositis ossificans (n=11), fibrous dysplasia (n=14), osteochondroma (n=6), desmoplastic fibroma (n=1), florid reactive periostitis (n=4), Nora's lesion (n=3), and turret exostosis (n=1)). Low-grade osteosarcoma labeled for MDM2 in 16 cases (70%) and for CDK4 in 20 cases (87%). All low-grade osteosarcomas expressed one or both markers (100%), with 13 cases (57%) expressing both. Staining pattern was diffuse in most cases, and the majority expressed moderate or strong intensity for either antibody. MDM2/CDK4 immunostaining was shown irrespective of low-grade osteosarcoma histological subtype. In contrast, only 1 Nora's lesion out of the 40 miscellaneous benign processes showed immunoreactivity for MDM2 or CDK4. The combination of these two markers thus shows 100% sensitivity and 97.5% specificity for the diagnosis of low-grade osteosarcoma. MDM2 and CDK4 immunostains therefore reliably distinguish low-grade osteosarcoma from benign histological mimics, and their combination may serve as a useful adjunct in this difficult differential diagnosis.
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PMID:Immunohistochemical analysis of MDM2 and CDK4 distinguishes low-grade osteosarcoma from benign mimics. 2060 38

Bizarre parosteal osteochondromatous proliferation (BPOP) also eponymically called "Nora's lesion", is a rare benign reactive bone lesion first reported in 1983. BPOP occurs classically on the bones of the hands and feet and long bones. This lesion can easily be confused, both clinically and microscopically, with other benign and malignant lesions of bone, including osteochondroma, parosteal osteosarcoma, myositis ossificans and reactive periostitis. BPOP has been reported to have a high rate of recurrence. Only 3 cases of BPOP of the head and neck have been reported in the literature, of which one involved the maxilla. We present a rare case of BPOP involving the mandible in a 10 year old African American male. Microscopically, a fibro-cartilaginous cap giving rise to a proliferation of variably mineralized osteophytic finger-like projections of bone was seen. Multiple trabeculae of "blue bone" were noted as well as numerous atypical appearing chondrocytes. The lesion recurred within 4 months following the initial excision but has not recurred to date after the second local excision. To the best of our knowledge, this is the first report of BPOP arising in the mandible. In addition, we discuss the clinical and microscopic features, differential diagnosis, and prognosis of this rare entity. We present a case of BPOP of the mandible and believe this is the first report of such a case in the mandible.
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PMID:Bizarre parosteal osteochondromatous proliferation (Nora's Lesion) of the mandible. a rare bony lesion. 2209 73

Nora's tumor, also known as bizzarre parosteal osteochondromatous proliferation (POPB), is an exophytic outgrowth arising from the cortical surface of the bone that consists of a mixture of bone, cartilage and fibrous tissue. It is a benign lesion with atipical microscopic features and a tendency to recur. It must be distinguishable from parosteal osteogenic sarcoma, parosteal chondrosarcoma, osteochondroma, florid reactive periostitis, turret exostosis, subungueal exostosis, myositis ossificans. The treatment is surgical, but a high rate of local relapse is described. The diagnosis is histological. We report a case of a patient with POPB involving the foot, underwent surgical excision and with no evidence of recurrence at one year.
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PMID:Bizzarre parosteal osteochondromatous proliferation: case report. 2364 46

A 9-month-old male neutered mixed-breed cat had a history of chronic lameness of the right hind limb, which was non-responsive to antibiotic treatment. Hematologic analysis revealed marked neutrophilia and mild monocytosis. Radiography revealed extensive loss of cortical bone, and replacement with irregular and disorganized bone. There was loss of the normal cortico-medullary distinction, and the medullary cavity had an irregular radiodensity suggestive of osteomyelitis. Surgical curettage and antibiotics did not improve the clinical condition, and amputation was performed. Grossly, the skin over the right tibia was ulcerated with a viscous and granular exudate. At histopathology, there was marked diffuse pyogranulomatous dermatitis, myositis, periostitis and osteomyelitis associated with Splendore-Hoeppli phenomenon. In addition, there was marrow osteoproliferation and multifocal cortical loss, reabsorption, fibroplasia and endosteal bone formation. Gram staining revealed myriad slightly elongated Gram-positive bacteria, arranged in pairs or single chains, confirmed by polymerase chain reaction as Streptococcus species.
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PMID:Splendore-Hoeppli phenomenon in a cat with osteomyelitis caused by Streptococcus species. 2390 7

The 4-component meningococcal serogroup B vaccine 4CMenB (Bexsero) is the first vaccine against this serogroup and has been approved by licensing authorities in Europe, Canada and Australia. Therefore, the vaccine may enter soon nationwide vaccine recommendation schemes. We report on a case of a 5-month-old infant who developed prolonged upper extremity dysfunction after the second injection of the 4CMenB vaccine in the left deltoid muscle and was concomitantly applied with 2 routine vaccinations. Myositis, periostitis, (peri-) vasculitis and axillary inflammation were confirmed by magnetic resonance imaging. Two months after initial initiation of an anti-inflammatory and an antibiotic treatment, symptoms completely resolved. Administration of 3 vaccines requires clear recommendations for the preferred injection site in infants because increased reactogenicity of 4CMenB may lead to local severe adverse events.
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PMID:Severe Upper Extremity Dysfunction After 4CMenB Vaccination in a Young Infant. 2637 62

Pediatric polyarteritis nodosa is rare systemic necrotizing arteritis involving small- and medium-sized muscular arteries characterized by aneurysmal dilatations involving the vessel wall. Aneurysms associated with polyarteritis nodosa are common in visceral arteries; however intracranial aneurysms have also been reported and can be associated with central nervous system symptoms, significant morbidity, and mortality. To our knowledge extracranial involvement of the vertebral arteries has not been reported but has the potential to be deleterious due to fact that they supply the central nervous system vasculature. We present a case of a 3-year-old Haitian boy with polyarteritis nodosa that presented with extracranial vessel involvement of his vertebral arteries. After thorough diagnostic imaging, including a bone scan, ultrasound, Magnetic Resonance Imaging/Angiography, and Computed Tomography Angiography, he was noted to have vertebral artery vasculitis, periostitis, subacute epididymoorchitis, arthritis, and myositis. He met diagnostic criteria for polyarteritis nodosa and was treated with cyclophosphamide, methylprednisolone, and tocilizumab, which resulted in improvement of his inflammatory markers, radiographic findings, and physical symptoms after treatment. To the authors' knowledge, this is the first report of vertebral artery vasculitis in polyarteritis nodosa as well as successful treatment of the condition using the combination cyclophosphamide and tocilizumab for this condition.
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PMID:A Case of Polyarteritis Nodosa Associated with Vertebral Artery Vasculitis Treated Successfully with Tocilizumab and Cyclophosphamide. 2701 80

Bizarre parosteal osteochondromatous proliferation (BPOP) or Nora's lesion, is a rare benign surface-based bone lesion most commonly involving the tubular bones of hands and feet. We report an unusual case of BPOP affecting the distal ulna in a 22-year-old man who presented with a painless wrist mass following injury and was successfully treated with surgical resection. We focus on multi-modality imaging, histopathology, and differential diagnosis (including osteochondroma, florid reactive periostitis, myositis ossificans, and surface-type osteosarcoma), as well as a review of the literature regarding recent concepts on etiology and evolution, spectrum of imaging characteristics and diagnostic overlap, histopathology, as well as treatment options.
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PMID:Bizarre parosteal osteochondromatous proliferation: Rare case affecting distal ulna and review of literature. 3297 95


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