A case of chronic recurrent multifocal osteomyelýtýs successfully treated with etanercept

PubMe

MEFV mutations in systemic JIA

Background: Systemic form of juvenile idiopathic arthritis (JIA) is regarded as an autoinflammatory disease. Certain genetic polymorphisms in genes coding inflammatory proteins have been associated with the disease. On the other hand mutations of the MEFV gene cause a monogenic autoinflammatory disease, Familial Mediterranean Fever (FMF). In a previous study in adult rheumatoid arthritis 3 out of the 25 British patients who developed secondary amyloidosis had a mutation/polymorphism in the MEFV gene. Aim: To analyse whether mutaions in the MEFV gene had an association with systemic JIA. Patients and methods: MEFV mutations were screened in a total of 32 systemic JIA patients. All had been classified as systemic JIA according to the Durban JIA criteria. None had disease characteristics that met the Tel Hashomer criteria for the diagnosis of FMF. Results: 2 carrier for M694V and two patients who were homozygote for MEFV mutations. Both of these patients were among the most severe patients in the group. One had an excellent response to etanercept whereas the other was resistant to anti-TNF and other conventional treatments and had only a partial response to thalidomide. Although the number of severe mutations were increased in this small group of patients with systemic JIA the difference with the Turkish population did not reach statistical significance, but the disease causing mutation (M694V) was significantly high in the patients with systemic JIA(p = 0.02). Conclusion: However, the severe disease course in the aforementioned patients suggest that MEFV mutations may be a modifying genetic factor in systemic JIA.PubMe

Does unity of Familial Mediterranean fever with juvenile idiopathic arthritis affect the outcome?

PubMe

Experimental Study on the Low-velocity Impact Behavior of Foam-core Sandwich Panels

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/97064/1/AIAA2012-1701.pd

Time to focus on outcome assessment tools for childhood vasculitis

Childhood systemic vasculitides are a group of rare diseases with multi-organ involvement and potentially devastating consequences. After establishment of new classification criteria (Ankara consensus conference in 2008), it is now time to establish measures for proper definition of activity and damage in childhood primary vasculitis. By comparison to adult vasculitis, there is no consensus for indices of activity and damage assessment in childhood vasculitis. Assessment of disease activity is likely to become a major area of interest in pediatric rheumatology in the near future. After defining the classification criteria for primary systemic childhood vasculitis, the next step was to perform a validation study using the original Birmingham vasculitis activity score as well as the disease extent index to measure disease activity in childhood vasculitis. Presently, there are efforts in place to develop a pediatric vasculitis activity score. This paper reviews the current understanding about the assessment tools (i.e., clinical features, laboratory tests, radiologic assessments, etc.) widely used for evaluation of the disease activity and damage status of the children with vasculitis

TC-99M NANOCOLLOID AND TC-99M MDP 3-PHASE BONE IMAGING IN OSTEOMYELITIS AND SEPTIC ARTHRITIS - A COMPARATIVE-STUDY

The aim of this study was to compare Tc-99m nanocolloid scintigraphy with Tc-99m MDP bone imaging in the diagnosis of osteomyelitis and septic arthritis. Overall, 31 Tc-99m MDP bone scans and 39 Tc-99m nanocolloid studies were performed in 34 patients (15 female, 19 male; mean age, 14.88 years +/- 19.00 years) who were suspected of osteomyelitis and/or septic arthritis. The final diagnoses were established by needle aspiration, cultures, radiography, clinical course, and, in some patients, with CT, ultrasonography, and biopsy. The sensitivity, specificity, and accuracy were 100%, 75%, and 92%, respectively for both Tc-99m MDP and Tc-99m nanocolloid scans in detecting osteomyelitis. For septic arthritis, Tc-99m MDP bone imaging showed 100%, 85%, and 94%, and Tc-99m nanocolloid scans showed 90%, 59%, and 76%, sensitivity, specificity, and accuracy, respectively. These results show that, although both scans are in good agreement for osteomyelitis, for septic arthritis nanocolloid is not specific enough to recommend it to be a complementary method to MDP bone scans

FALSE-POSITIVE UPTAKE OF TC-99M PENTA-DMSA IN FIBROUS DYSPLASIA OF BREAST IN A PATIENT WITH MEDULLARY CARCINOMA OF THYROID

Tc-99m penta-DMSA has been established as a tumor-seeking agent in the detection of primary and metastatic medullary carcinoma of thyroid. In a case with Sipple's syndrome (pheochromocytoma and medullary carcinoma of thyroid) the Tc-99m penta-DMSA scan showed increased accumulation in the right breast, in which there was a mass shown by MRI