ABSTRACT
OBJECTIVES: Fabry disease (FD) is an X-linked lysosomal disease caused by variants of the GLA gene; the formation of defective alpha-galactosidase A contributes to the accumulation of substrates in several organs. Chronic inflammation is thought to contribute to organ damage in FD patients. METHODS: In total, 36 classic FD patients (15 men/21 women) and 25 healthy controls (20 men/8 women) were assessed. The Mainz Severity Score Index (MSSI) was established after conducting interviews with the patients and chart review. Serum IL-6, IL-1β, and TNF-α levels were evaluated in both groups. RESULTS: The mean age (years) for FD patients was 43.1±15.4 and that for the controls was 47.4±12.2 (p>0.05). Twenty-two patients (59.5%) were treated with enzyme replacement therapy (ERT). Serum IL-6 and TNF-α levels were significantly higher in FD patients than in the controls. Patients treated with ERT had higher serum IL-6 and TNF-α levels than those not treated with ERT. There was no difference in the serum IL-1β levels between patients treated with ERT and those who were not. The MSSI scores in the patients were correlated with serum levels of IL-6 (r=0.60, p<0.001) and TNF-α (r=0.45, p<0.001). CONCLUSION: FD was associated with elevated serum levels of IL-6 and TNF-α in this cohort. The FD patients treated with ERT, particularly, women, exhibited higher levels of serum IL-6 and TNF-α than those not treated with ERT; the serum IL-6 and TNF-α levels were correlated with the MSSI scores reflecting greater disease burden.
Subject(s)
Humans , Male , Female , Tumor Necrosis Factor-alpha , Fabry Disease/drug therapy , Interleukin-6 , Cost of Illness , alpha-GalactosidaseABSTRACT
OBJECTIVE: To evaluate the effect of low dose methotrexate alone or in combination with glucocorticoid treatment on titanium implant osseointegration. METHODS: Groups of 6-8 adult New Zealand White rabbits were treated for 18 weeks with saline (control), methotrexate, glucocorticoid, or methotrexate plus glucocorticoid. The animals received a titanium implant in the tibia at week 6. Lumbar spine and tibia bone mineral densities were analyzed before and after treatment. Histomorphometric analysis of bone cortical thickness, total bone area around the implant, and percent of bone to implant contact was performed. RESULTS: After 18 weeks, the change in the bone mineral density in the lumbar spines and tibias in the methotrexate group was comparable to the control group (0.035 vs. 0.055 g/cm² and 0.021 vs. 0.041 g/cm², respectively). In contrast, both the glucocorticoid group and glucocorticoid plus methotrexate group had significant reductions at both sites. Histomorphometric analysis of the tibia in the control and methotrexate groups revealed no significant changes in cortical thickness (133 vs. 126 μm), total bone area around the implant (33 vs. 30 percent), or bone to implant contact (40 vs. 38 percent). In contrast, glucocorticoid group had significant reductions compared to controls in tibia cortical thickness (99 vs. 133 μm), total bone area around the implant (24 vs. 33 percent), and bone to implant contact (27 vs. 40 percent). Similar reductions were observed in the glucocorticoid plus methotrexate group. CONCLUSIONS: Our results demonstrate that low dose methotrexate treatment does not affect titanium implant osseointegration, suggesting that this therapy is safe for surgical procedures requiring a titanium implant.
Subject(s)
Animals , Male , Rabbits , Antirheumatic Agents/administration & dosage , Methotrexate/administration & dosage , Osseointegration/drug effects , Tibia , Titanium , Absorptiometry, Photon , Bone Density , Glucocorticoids/administration & dosage , Materials Testing , Models, Animal , Time Factors , Treatment OutcomeABSTRACT
A prática de corrida de média e longa distância tem crescido em todo o mundo. Apesar de todos os efeitos benéficos da prática de corrida, tem-se observado uma elevada incidência de lesões, sobretudo em membros inferiores. O mecanismo de lesão relacionada à corrida obedece a um padrão comum a todas as lesões nos diferentes esportes e decorre da sobreposição de dois ou mais fatores. Os objetivos desse estudo foram: 1) relatar prospectivamente a incidência de lesões osteomioarticulares em corredores amadores durante 12 meses de seguimento; e 2) detectar os principais fatores extrínsecos e intrínsecos para as lesões encontradas. Dezoito corredores (13 homens e cinco mulheres) amadores foram selecionados para participarem do estudo. Eles foram submetidos a uma avaliação clínica com exame físico completo e do aparelho locomotor, avaliação nutricional, exames laboratoriais, teste ergométrico, avaliação da densidade mineral óssea e composição corporal e radiografia dos pés no período basal e após um ano de seguimento. Aqueles que apresentaram alguma lesão foram comparados com seus pares que não lesionados, considerando-se as diversas variáveis coletadas. Metade da amostra (50 por cento) apresentou alguma lesão osteomuscular em membros inferiores no período do estudo. Os fatores de risco significantemente associados foram graus de extensão de joelho e flexão plantar diminuídos, frequência cardíaca de repouso menor e velocidade de treino maior. A alta frequência de lesões osteomioartculates nestes corredores de longa distância esteve associada a fatores intrínsecos e extrínsecos. A avaliação clínica deve ser focada nesses parâmetros com intuito de prevenir lesões em corredores.
The practice of middle- and long-distance running has become worldwide popular. Despite the number of benefits associated with this sport, increased incidence of lower limb injury has been observed. The injury mechanisms related to running are similar to those seen in different sports and can be a result of two or more factors. The aims of this study were: 1) to report prospectively the incidence of injuries in non professional runners after a 12-month follow-up; and 2) to determine the main intrinsic and extrinsic factors related to the observed injuries. Eighteen runners (13 males and five females) took part in this study. They were submitted to clinical examination, nutritional and biochemical assessments, VO2max test, bone mineral density and body composition evaluation, and foot radiography at baseline and after one year. The subjects who had injury were compared to those non-injured taken into account the several variables assessed. Fifth percent of the sample presented at least one lower limb injury. The factors significantly associated with the injuries were reduced knee extension and plantar flexion range of motion, lower resting heart rate, and high training speed. The high incidence of injuries observed in this study was associated with intrinsic and extrinsic factors. The clinical assessment should focus on these parameters in order to prevent injuries.