Factors associated with response to methotrexate in systemic-onset juvenile chronic arthritis

We retrospectively investigated, in 19 children with systemic-onset juvenile chronic arthritis (SoJCA), the possible influence on the outcome of methotrexate (MTX) therapy of several independent variables, including age at onset of juvenile chronic arthritis, disease duration and severity of the disease at baseline. The dosage of MTX ranged from 7.5 to 11.O mg/m*/week (median 9.3 mg/m²/week) and was given as a single, oral weekly dose. After 6 months of treatment, 12 (63%) patients were judged as responders on grounds of a 50% reduction in the number of joints with active arthritis and/or an articular severity score; 7 (37%) did not respond to therapy. When the baseline values of the selected variables were compared, we found that the responder group had, with respect to the non-responder group, a lower percentage of radiographic lesions ( p < 0.005), a shorter duration of the disease ( p < 0.05) and a lower number of joints with limitation of motion ( p < 0.01), functional limitation score ( p < 0.05) and articular severity score ( p < 0.05). A threshold value of disease duration of two years and the presence/absence of radiographic lesions gave a correct classification with respect to the treatment outcome of 73.7% and 83.3%, respectively. The predictive value of these two variables was confirmed by a multivariate analysis. We conclude that earlier treatment with MTX, possibly before the appearance of radiographic changes, may favourably influence the outcome of MTX treatment in those patients with SoJCA who require a second-line drug.     

Early physiotherapy in the respiratory intensive care unit

Physiotherapy is an integral part of the management of patients in respiratory intensive care units (RICUs). The most important aim in this area is to enhance the overall patient's functional capacity and to restore his/her respiratory and physical independence, thus decreasing the risks of bed rest associated complications. This article is a review of evidence-based effectiveness of weaning practices and physiotherapy treatment for patients with respiratory insufficiency in a RICU. Literature searches were performed using general and specialty databases with appropriate keywords. The evidence for applying a weaning process and physiotherapy techniques in these patients has been described according to their individual rationale and efficacy. The growing number of patients treated in RICUs all over the world makes this non pharmacological approach both welcome and interesting. However, to date, there are only strong recommendations concerning the evidence-based strategies to speed weaning. Early physiotherapy may be effective in ICU: however, most techniques (postures, limb exercise and percussion/vibration in particular) need to be further studied in a large population. Evidence supporting physiotherapy intervention is limited as there are no studies examining the specific effects of interventions on long-term outcome.     

Expression of alpha2a-2b neuregulin-1 is associated with early peripheral nerve repair along muscle-enriched tubes

Using RT-PCR, we have investigated expression of isoforms beta1 (the axonal isoform) and alpha2a-2b (the mesenchymal isoform) of neuregulin-1, one of the most important known trophic factors for Schwann cells, in the rat sciatic nerve repaired by muscle-enriched non-nervous conduits (made by a vein filled with fresh skeletal muscle). Repaired nerves were harvested 2, 6 and 13 days post-operatively. Results showed that while muscle-vein combined grafts were enriched in mRNA coding for alpha2a-2b since the very early regeneration stages, isoform beta1 mRNA was not detectable inside the tubes at day 2 and 6 post-operatively while its expression at day 13 was very slight. These results suggest that Schwann cell survival and activity inside a fresh muscle-enriched non-nervous conduit graft (a key factor for successful nerve regeneration along the graft) may be supported by the mesenchymal isoform of neuregulin-1 during very early repair phases, i.e. when axons are still not present along the tube.     

Agreement between physicians and parents in rating functional ability of children with juvenile idiopathic arthritis

Objective  

To investigate concordance between physicians and parents in rating the degree of functional ability of children with juvenile idiopathic arthritis (JIA).     

Bilateral anorchia in infancy: occurence of micropenis and the effect of testosterone treatment

OBJECTIVE: To analyze the clinical and histological findings in boys with bilateral anorchia and the response to testosterone treatment on penis length. STUDY DESIGN: Patients were divided into two groups according to the absence (group A, n = 29) or the presence (group B, n = 26) of palpable intrascrotal or inguinal mass at first clinical examination. RESULTS: A micropenis was found in 46% of patients (n = 24) with a similar proportion in both groups. Testosterone treatment induced a mean penis length gain of 1.9 +/- 1.3 SDS (standard deviation score). However, micropenis persisted in six patients. Histological examination (n = 18) confirmed the absence of any testicular structure with deferent ducts being present unilaterally or bilaterally in all but three patients. In these three patients, a hemorrhagic testis, probably as a result of a mechanical torsion, was found. CONCLUSIONS: The presence of isolated micropenis in almost half of patients with bilateral anorchia strongly suggests that the testicular damage frequently occurs during the second half of gestation after male sexual differentiation. In most cases, testosterone treatment stimulates the penile growth. Although the pathogenesis of bilateral anorchia may be heterogeneous, our study suggests that gonads may have been functionally abnormal before they disappeared, and suggests that some patients have an intrinsic endocrine disorder.     

The effect of dietary omega-3 fatty acids (fish oil) on azoxymethanol-induced focal areas of dysplasia and colon tumor incidence

MaxEPA (MA), a fish oil high in omega-3 fatty acids, was combined with various levels of corn oil (CO), rich in omega-6 fatty acids, and fed to female CF1 mice. The three fish oil blends with CO and the two CO levels of the diets studied were as follows: 16.0% CO + 4.4% MA (Diet 1); 10.2% CO + 10.2% MA (Diet 2); 4.4% CO + 16.0% MA (Diet 3); 20.4% CO (Diet 4); and 4.4% CO (Diet 5). The diets were provided 2 weeks before weekly subcutaneous injection of saline or azoxymethanol (AOM). Studies of epithelial cell proliferation and the incidence of focal areas of dysplasia (FAD) involved six weekly AOM injections. One week after the last AOM injection and 1 hour before killing, mice were injected with tritiated thymidine (3HTdR). No differences in any proliferative parameters were found among the five groups of saline-treated mice. Among the AOM-treated animals, those fed Diet 3 showed significantly fewer cells per crypt and significantly fewer labeled cells/gland than CO Diets 4 and 5. Additionally, the distribution of S-phase cells in crypts of AOM-treated mice fed Diet 3 most closely resembled that of the saline controls. The greatest alteration in the distribution of proliferative cells was observed in the high-CO diet (Diet 4) and the lowest MA level (Diet 1). Mice fed Diets 2 and 3 had significantly fewer FAD/500 microns of distal colonic serial sections than those fed the high CO diet (Diet 4). Mice involved in chronic tumor incidence studies received only three weekly injections of the same dose of AOM. Regardless of diet, approximately 88% of all tumors arose in the distal colon. A significantly larger tumor-bearing population was observed in both the high-CO Diet 4 and the lowest MaxEPA (MA) diet (Diet 1) compared with the incidence in MA Diets 2 and 3 and the low-CO Diet 5. A diet with a ratio of omega-6 to omega-3 fatty acids of approximately 1.0 apparently prevented the development of an adenoma-type proliferative pattern thereby reducing FAD numbers and subsequent tumor incidence.