Minor sperm abnormalities in young male post-pubertal patients with juvenile dermatomyositis

The objective of the present study was to identify sperm abnormalities in young male patients with juvenile dermatomyositis (JDM). In 2005, 18 male JDM patients, diagnosed according to the criteria of Bohan and Peter, were followed at the Pediatric Rheumatology Unit and Rheumatology Division, of our Institution. Of the 18 males, 11 were pre-pubertal and 7 were post-pubertal. Two of 7 post-pubertal JDM male patients were excluded: one for orchidopexy for cryptorchidism and the other for testicular ectopia in the left testis. The remaining 5 post-pubertal JDM patients were prospectively evaluated on the basis of two semen analyses, according to the World Health Organization (WHO), urologic evaluation, testicular Doppler ultrasound hormone profile. The data of the JDM patients were compared with those of 5 age-matched healthy controls. The median age 18, was similar in JDM patients and controls. All JDM patients had teratozoospermia (abnormal sperm morphology), as did 4 (80 percent) of the controls. One of JDM patients had previous oligoasthenoteratozoospermia treated with intravenous cyclophosphamide with normalization of the number and concentration of the sperm after 5 years. All sperm parameters (sperm concentration, total sperm count and total motile sperm count by WHO, and sperm morphology by Kruger strict criteria), testicular volumes by Prader orchidometer and ultrasound, and hormones were similar in JDM patients compared with controls. The frequency of anti-sperm antibodies was similar in both groups. All JDM patients had minor sperm abnormalities in the head, midpiece, and/or tail of spermatozoids. Serial semen analyses in larger study populations are necessary to identify the extent and duration of sperm abnormalities in male patients with idiopathic inflammatory myopathies.

Bone metabolism and vascular calcification

Osteoporosis and atherosclerosis are chronic degenerative diseases which have been considered to be independent and whose common characteristic is increasing incidence with age. At present, growing evidence indicates the existence of a correlation between cardiovascular disease and osteoporosis, irrespective of age. The morbidity and mortality of osteoporosis is mainly related to the occurrence of fractures. Atherosclerosis shows a high rate of morbidity and especially mortality because of its clinical repercussions such as angina pectoris, acute myocardial infarction, stroke, and peripheral vascular insufficiency. Atherosclerotic disease is characterized by the accumulation of lipid material in the arterial wall resulting from autoimmune and inflammatory mechanisms. More than 90 percent of these fatty plaques undergo calcification. The correlation between osteoporosis and atherosclerosis is being established by studies of the underlying physiopathological mechanisms, which seem to coincide in many biochemical pathways, and of the risk factors for vascular disease, which have also been associated with a higher incidence of low-bone mineral density. In addition, there is evidence indicating an action of antiresorptive drugs on the reduction of cardiovascular risks and the effect of statins, antihypertensives and insulin on bone mass increase. The mechanism of arterial calcification resembles the process of osteogenesis, involving various cells, proteins and cytokines that lead to tissue mineralization. The authors review the factors responsible for atherosclerotic disease that correlate with low-bone mineral density.

Desenvolvimento de equação para estimativa da gordura corporal de mulheres idosas com osteoporose e osteopenia através da espessura de dobras cutâneas tendo como referência absorciometria por dupla emissão de raios X / Development of an equation for measurement of bodyfat mass of elderly women with osteoporosis or osteopenia through skin fold thickness using dual energy X-ray absorptiometry as a reference

INTRODUÇÃO E OBJETIVO: A composição corporal vem sendo descrita como importante fator relacionado à densidade mineral óssea. Assim, o presente estudo teve como objetivo desenvolver equação preditiva para percentagem de gordura corporal através da espessura de dobras cutâneas tendo como referência a absorciometria por dupla emissão de raios X (DXA), em mulheres com osteoporose e osteopenia. METODOLOGIA: Foram analisadas 29 mulheres com idade entre 67 e 84 anos, atendidas no Ambulatório de Osteoporose da Disciplina de Reumatologia da Faculdade de Medicina da Universidade de São Paulo. Foram mensuradas as espessuras de quatro dobras cutâneas (bíceps, tríceps, subescapular e supra-ilíaca) e realizada a avaliação da composição corporal pela DXA. A análise estatística constou do teste de Kolmogorov-Smirnov, coeficiente de correlação de Pearson, análise de regressão linear simples, coeficiente de correlação intraclasse, teste t de Student, teste de Bland-Altman e cálculo do erro total da equação de acordo com Lohman (1992). RESULTADOS: O modelo de espessura de dobras cutâneas que melhor explicou o percentual de gordura corporal incluiu a soma das dobras supra-ilíaca, bicipital e tricipital, determinando em até 72 por cento a percentagem de gordura corporal. Os valores médios da gordura corporal em quilograma, estimados pelas dobras e medidos pela DXA, não foram estatisticamente diferentes e estiveram altamente correlacionados (r = 0,82; p < 0,001). Comparando-se o percentual de gordura corporal avaliado pela equação proposta e o medido pela DXA, o erro total foi de 0,7 por cento e 0,4kg. CONCLUSÃO: Em vista dos presentes resultados, a equação resultante do modelo de regressão linear é adequada para as mulheres idosas com osteoporose e osteopenia, podendo ser uma alternativa para a estimativa de gordura corporal nesse tipo de população.

INTRODUCTION AND OBJECTIVE: The body composition has been intensively investigated as a determinant of bone mineral density. The present study was developed in order to propose a predictive equation to calculate body fat percentage by means of skin folds thickness using bone densitometry (DXA) as a reference in a group of elderly women with osteoporosis and osteopenia. METHODOLOGY: Twenty-nine women, mean age 67 to 84 years old, in attendance at the Osteoporosis Clinic at Rheumatology Division, School of Medicine, University of Sao Paulo, were evaluated. Four skin folds thickness were measured (biceps, triceps, subscapular and suprailiac) and body composition by DXA was evaluated. The statistical analysis consisted of Kolmogorov-Smirnov test, Pearson's coefficient correlation, simple linear regression analysis, intra-class correlation coefficient, t Student test, Bland-Altman test and calculus of equation total error according to Lohman (1992). RESULTS: The best skinfold model that explained the percentage of body fat mass included the suprailiac, bicipital and tricipital values, determining up to 72 percent of body fat mass. The fat mass average values in kilograms estimated by the skin folds and measured by DXA were not statistically different and had been highly correlated (r = 0.82; p < 0.001). Comparing the fat mass percentage evaluated by the proposed equation and the percentage measured by DXA, the total error was of 0.7 percent and 0.4 kg. CONCLUSION: In view of the presented results, the resultant equation of the regression model is adequate for elderly women with osteoporosis and osteopenia, and may be an alternative for the body fat mass estimate in this population.

INTRODUCCION Y OBJETIVO: La composición corporal viene siendo descrita como un importante factor relacionado a la densidad mineral ósea. De este modo el presente estudio ha tenido como objetivo desarrollar una ecuación predictiva para el porcentaje de grasa corporal a través de la espesura de los dobleces cutáneos con bases en la absorciometría por doble emisión de rayos X (DXA), en mujeres con osteoporosis y osteopenia. METODOLOGíA: Fueron analizadas 29 mujeres con edades entre 67 y 84 años, atendidas en Ambulatorio de Osteoporosis de la Disciplina de Reumatología de la Facultad de Medicina de la Universidad de San Pablo. Fueron medidas las espesuras de cuatro dobleces cutáneos (bíceps, tríceps, subescapular y suprailiaca) y realizada la evaluación de la composición corporal por DXA. El análisis estadístico constó del test de Kolmogorov-Smirnov, el coeficiente de correlación de Pearson, el análisis de regresión lineal simple, el coeficiente de correlación intraclase, el test t de Student, el test de Bland-Altman y el cálculo del error total de la ecuación de acuerdo a Lohman (1992). RESULTADOS: El modelo de espesura de dobleces cutáneos que mejor explicó el porcentaje de grasa corporal incluyó la suma de los dobleces suprailiaco, bicipital y tricipital, determinando en hasta 72 por ciento el porcentaje de grasa corporal. Los valores medios de grasa corporal en kilogramos, estimados por los dobleces y medidos por el DXA, no fueron estadísticamente diferentes y estuvieron altamente correlacionados (r = 0,82; p < 0,001). Al compararse el porcentaje de grasa corporal evaluado por la ecuación propuesta y el medido por el DXA, el error total fue de 0,7 por ciento y 0,4 kg. CONCLUSION: A la luz de los resultados obtenidos, la ecuación resultante del modelo de regresión lineal es adecuada para las mujeres ancianas con osteoporosis y osteopenia siendo incluso una alternativa para la estimativa de la grasa corporal en este tipo de población.

RANK, RANKL and osteoprotegerin in arthritic bone loss

Rheumatoid arthritis is characterized by the presence of inflammatory synovitis and destruction of joint cartilage and bone. Tissue proteinases released by synovia, chondrocytes and pannus can cause cartilage destruction and cytokine-activated osteoclasts have been implicated in bone erosions. Rheumatoid arthritis synovial tissues produce a variety of cytokines and growth factors that induce monocyte differentiation to osteoclasts and their proliferation, activation and longer survival in tissues. More recently, a major role in bone erosion has been attributed to the receptor activator of nuclear factor kappa B ligand (RANKL) released by activated lymphocytes and osteoblasts. In fact, osteoclasts are markedly activated after RANKL binding to the cognate RANK expressed on the surface of these cells. RANKL expression can be upregulated by bone-resorbing factors such as glucocorticoids, vitamin D3, interleukin 1 (IL-1), IL-6, IL-11, IL-17, tumor necrosis factor-alpha, prostaglandin E subscrito 2, or parathyroid hormone-related peptide. Supporting this idea, inhibition of RANKL by osteoprotegerin, a natural soluble RANKL receptor, prevents bone loss in experimental models. Tumor growth factor-ß released from bone during active bone resorption has been suggested as one feedback mechanism for upregulating osteoprotegerin and estrogen can increase its production on osteoblasts. Modulation of these systems provides the opportunity to inhibit bone loss and deformity in chronic arthritis.

Effect of 17á-estradiol or alendronate on the bone densitometry, bone histomorphometry and bone metabolism of ovariectomized rats

The objective of the present study was to evaluate the effect of 17á-estradiol or alendronate in preventing bone loss in 3-month-old ovariectomized Wistar rats. One group underwent sham ovariectomy (control, N = 10), and the remaining three underwent double ovariectomy. One ovariectomized group did not receive any treatment (OVX, N = 12). A second received subcutaneous 17á-estradiol at a dose of 30 æg/kg for 6 weeks (OVX-E, N = 11) and a third, subcutaneous alendronate at a dose of 0.1 mg/kg for 6 weeks (OVX-A, N = 8). Histomorphometry, densitometry, osteocalcin and deoxypyridinoline measurements were applied to all groups. After 6 weeks there was a significant decrease in bone mineral density (BMD) at the trabecular site (distal femur) in OVX rats. Both alendronate and 17á-estradiol increased the BMD of ovariectomized rats, with the BMD of the OVX-A group being higher than that of the OVX-E group. Histomorphometry of the distal femur showed a decrease in trabecular volume in the untreated group (OVX), and an increase in the two treated groups, principally in the alendronate group. In OVX-A there was a greater increase in trabecular number. An increase in trabecular thickness, however, was seen only in the OVX-E group. There was also a decrease in bone turnover in both OVX-E and OVX-A. The osteocalcin and deoxypyridinoline levels were decreased in both treated groups, mainly in OVX-A. Although both drugs were effective in inhibiting bone loss, alendronate proved to be more effective than estradiol at the doses used in increasing bone mass

Vasculite urticariforme hipocomplementêmica como primeira manifestaçäo do lúpus eritematoso sistêmico / Hypocomplementaemic urticarial vasculitis first manifestation of systemic lupus erythematosus

A síndrome vasculite urticariforme hipocomplementêmica é uma vasculite leucocitoclástica que se apresenta com lesöes urticariformes, associada a febre, artralgias, artrite e cólica abdominal. Outras manifestaçöes sistêmicas incluem a presença de glomerulonefrite, uveíte, episclerite, doença pulmonar obstrutiva e alteraçöes neurológicas. Alguns casos associados ao lúpus eritematoso sistêmico (LES) têm sido descritos, com o diagnóstico baseando-se na presença de critérios bem definidos de LES prévia ou concomitantemente ao aparecimento de vasculite urticariforme. A apresentaçäo de vasculite urticariforme precedendo o diagnóstico de LES é rara, o que motivou o relato destes dois casos. Enfatiza-se a positivaçäo do anticorpo anti-Ro/SS-A por ocasiäo do diagnóstico de LES, alertando para a necessidade de avaliaçäo periódica nos casos de vasculite urticariforme.

Eritromelalgia primária / Primary erythromelalgia

Eritromelalgia é uma afecçäo rara que se caracteriza por intenso rubor, dor em queimaçäo e aumento da temperatura das extremidades. Formas primárias e secundárias têm sido descritas, em particular com trombocitemia essencial e policitemia vera. Os autores fazem o relato de um paciente de 15 anos, portador de eritromelalgia primária, e discutem os aspectos patogênicos, clínicso e tarapêuticso dessa entidade