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Juvenile hyaline fibromatosis (JHF) is a rare autosomal recessive hereditary disorder (less than 80 cases reported), characterized by multiple nodular lesions on the skin and musculoskeletal involvement, very debilitating because most adolescents and adults become bedridden. Only 10 cases have been reported on JHF in adulthood. We report the case of a 34-year-old male patient in whom clinical and histological findings were consistent with a mild JHF and focus on the radiographic features. The main purpose of this report is to increase the information available related to the radiographic manifestations and prognosis of JHF.  相似文献   
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Rheumatoid arthritis (RA) is a chronic autoimmune disease. The pathophysiology of RA implicates several mediators such as nitric oxide (NO) and cytokines such as interleukin-6 (IL-6), which is deeply involved in the main characteristics of RA. Furthermore, all-trans retinoic acid (ATRA) is an active vitamin A derivative well-known to have diverse immunomodulatory actions. In our study, we investigated first, the ex vivo immunomodulatory potential of ATRA on NO pathway by peripheral blood mononuclear cells (PBMCs) from Algerian RA patients. Then, we assessed the possible regulatory effect of ATRA on NO production induced by IL-6. PBMCs isolated from active and inactive RA patients and healthy controls were cultured with different concentrations of IL-6 or/with ATRA. NO production was assessed using the Griess method. Inducible nitric oxide synthase expression and NF-κB activity were analyzed by immunofluorescence test. Our results revealed a high NO production during active RA. We noticed that while IL-6 induced a high NO production and iNOS expression, ATRA downregulated both. ATRA also inhibited nuclear NF-κB translocation. Interestingly, it seems that NO production mediated by IL-6 on PBMCs of RA patients is downregulated by ATRA. Taken together, our results highlight the immunomodulatory effect of ATRA on NO pathway in RA patients and its possible role in regulating IL-6-mediated NO production. All these findings suggest its potential therapeutic role during RA.  相似文献   
4.

Background

The management of septic arthritis without bacteriological evidence is not well codified.

Aim of the work

To compare the features of septic arthritis with and without isolated germs.

Patients and Methods

This is a retrospective study including all patients with septic arthritis, discharged from the Rheumatology Department of Charles Nicolle Hospital, Tunisia over a period of 17?years [1998–2014]. The epidemiological and clinical data were evaluated. Patients were grouped according to the presence and absence of isolated germs.

Results

Fifty-nine septic arthritis patients were collected with an average of 3.5?cases/year. The mean age of the patients was 54.6?±?19?years [15–95] without sex predominance: 28 were male and 31 were female. At least one risk factor for SA was founded in 41 patients (69.5%). It was monoarticular in 50 cases (84.7%), oligoarticular in 6 (10.2%) and polyarticular in 3 (5.1%). The knee was the most often affected (49.2%). Germ was isolated in cultures and/or synovial fluids in 27 patients (45.8%). The age tended to be older in those with isolated germs and the elderly were more frequently infected compared to the non-elderly (51.8% versus 21.9%) (p?=?.01). The synovial fluid analysis, clinical and laboratory characteristics were comparable but the functional disability was significant higher in those without isolated germs (p?=?.024). Sternoclavicular joint was more common in patients with isolated germs (p?=?.016). There was no difference between the two groups regarding the course of the infection.

Conclusion

Patients with isolated and non-isolated germs have similar epidemiologic, clinical, biological and radiological characteristics.  相似文献   
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Background

Septic arthritis of the costovertebral thoracic joint is a rare site infection. We report an isolated case of septic arthritis of the 10th costo-vertebral right joint with osteitis due to Staphylococcus aureus.

Case presentation

A 59 year old Tunisian man presented with a 2 months history of dorsal spinal pain with fever, associated with asthenia, anorexia and loss of weight. There was a raised C-reactive protein (176 mg/L) and erythrocyte sedimentation rate (100 mm/1st h). Tests for tuberculosis and brucellosis were negative. In the present patient, the clinical symptoms were unspecific with lack of obvious predisposing factors. He had neither history of taking immunosuppressors nor of any disease indicative of immunodeficiency. Thoraco-abdominal computed tomography (CT) showed a lytic lesion centered on the 10th costo-vertebral right joint and histo-pathologic exam of the costo-vertebral puncture confirmed chronic active osteitis and bacteriologic culture allowed identifying methicillin-sensitive Staphylococcus aureus. The patient was treated with ciprofloxacin 1500 mg/day, associated with daily rifampin (20 mg/kg) for total treatment duration of 12 weeks after consulting infectious disease specialists. After a follow-up of 6 months, the patient remained asymptomatic and the markers of inflammation negative.

Conclusion

Septic arthritis of costovertebral joints should be considered when a patient presents with back pain, fever and elevated inflammatory markers. The diagnosis of septic arthritis of costovertebral joints remain a challenge to clinicians. CT is important to confirm a diagnosis and guide costovertebral biopsy and culture. Early and appropriate antibiotic therapy is important for a required outcome.  相似文献   
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Background

Supportive care as a bridge to transplant or recovery remains challenging in children suffering from acute liver failure (ALF). We report our experience in children using the Molecular Absorbent Recirculating System (MARS®).

Methods

Retrospective data from children receiving therapy using MARS® from October 2009 to October 2012 were included in this single-center retrospective study. Patient characteristics, clinical presentation and complications of ALF, clinical and biological data before and after each MARS® session, technical modalities and adverse events were recorded.

Results

A total of six children underwent 17 MARS® sessions during the study period. Two adolescents were treated with the adult filter MARSFLUX® and four infants were treated with the MiniMARS® filter. The mean PEdiatric Logistic Dysfunction (PELOD) score at admission was 19 (range 11–33). All patients were mechanically ventilated, and four had acute kidney injury. The neurological course improved in one case, judged as stable in two cases and worsened in one case; data were unavailable in two cases. Mean serum ammonia levels decreased significantly following treatment with MARS® from an initial 89?±?29 to 58?±?35 mcmol/L (p?=?0.02). No other significant biological improvement was observed. Hemodynamic status improved/remained unchanged in the adolescent group, but in the infants four of the seven sessions were poorly tolerated and two sessions were aborted. Three patients died, two were successfully transplanted and one recovered without transplantation.

Conclusion

In our experience, treatment with MARS® is associated with encouraging results in adolescents, but it needs modification for very sick infants to improve tolerance.  相似文献   
9.
Malignancy is one of the comorbidities linked to golimumab, a biological TNF-α blocker. In this systematic review and meta-analysis, we searched different databases and analyzed original publications to elucidate the remaining open question about the real association of malignancies with golimumab therapy. The most frequent cancer in patients treated with golimumab, in association or not with methotrexate, is the lung adenocarcinoma. However, lymphoma is not very commonly represented in these patients. We show that there is no major and evident risk of malignancies associated with golimumab in current scientific literature. An increased risk of malignancies may be associated with golimumab, but this warrants further clinical confirmation. Also, this risk mentioned in different studies must be taken with caution because of number of limits and biases.  相似文献   
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