Chronic urticaria (daily or almost daily symptoms lasting for more than six weeks) is characterized by wheals and erythema, with or without itching. A few case reports have shown chronic autoimmune urticaria at the beginning of systemic lupus erythematosus (SLE), particularly in adults. However, the prevalence of this manifestation in a lupus paediatric population was not studied. During 27 consecutive years, 5419 patients were followed up at our University Hospital and 271 (5%) had juvenile SLE (American College of Rheumatology criteria). Two of them (0.7%) had chronic and painless autoimmune urticaria as the first manifestation of juvenile SLE, and were reported herein. One case was a five-year old female with continuous widespread urticaria (duration 120 days), antinuclear antibodies (ANA) 1:640 (dense fine speckled pattern) and elevated complement levels. The juvenile SLE diagnosis was established after one year. The other case was a 13-year old female who had chronic widespread urticaria (lasting 45 days), ANA 1:160 (fine speckled pattern) and normal complement levels. The juvenile SLE diagnosis was established after three years. In conclusion, chronic autoimmune urticaria is very rare and may be the first lupus manifestation, particularly associated with the presence of autoantibodies. This study reinforces the importance of a rigorous follow-up in children and adolescents suffering from autoimmune urticaria due to the possibility of connective tissue disorders, such as paediatric lupus.

Objective: to analyze the morphometric and mechanical characteristics of the soleus and gastrocnemius muscles after immobilization in a shortened position. Methods: 20 Wistar rats (250 ± 20g) were divided equally into immobilized and control groups. The left hind limb was immobilized by means of an acrylic resin orthosis, with the ankle joint at maximum plantar flexion. After seven days of immobilization, the muscle mass, number and length of sarcomeres in series, muscle fiber cross-sectional area, density of the intramuscular connective tissue area and tensile strength of the triceps surae muscle were evaluated. The data were analyzed by the ANOVA and Tukey tests (p< 0.05). Results: The immobilized soleus muscle presented changes in all the morphometric variables analyzed, while some of these changes were not observed in the gastrocnemius muscle. Analysis of the mechanical test showed that the immobilized group presented a 20% decrease in the maximum tensile muscle strength. Conclusion: The results from this study showed that short-term immobilization causes changes to the morphometric parameters of the muscle fibers, with repercussions on muscle mechanics. These results suggest the need for rehabilitation of muscles subjected to immobilization, even if only for a short period, in order to achieve early recovery of normal muscle characteristics.

Infections are an important cause of morbidity and mortality in juvenile systemic lupus erythematosus (JSLE). Among them, invasive aspergillosis (IA), which is usually related to immunosuppressed patients, has been rarely reported in JSLE. From 1983 to 2011, 5604 patients were followed at our institution and 283 (5%) met the American College of Rheumatology (ACR) classification criteria for SLE. Six (2.1%) of our JSLE patients had IA. One of them was previously reported and five will be described herein. Four of them were female. The median age at JSLE diagnosis was 12 years (8-16) and the median interval between diagnosis of JSLE and IA was 6 months (1-38). All had pulmonary involvement and three of them had systemic involvement. The median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) was 19 (7-22). Diagnosis of IA was performed by isolation of Aspergillus spp., two in bronchoalveolar lavage culture and by way of autopsy in the others. All of them were treated with corticosteroids and/or immunosuppressive drugs at IA diagnosis (azathioprine and/or intravenous cyclophosphamide). They all required treatment in the pediatric intensive care unit with mechanical ventilation and antifungal therapy (fluconazole, amphotericin B, itraconazole and/or voriconazole); nonetheless, none of them survived. In conclusion, this was the first report that evaluated the prevalence of IA in a large population of JSLE patients from a tertiary pediatric hospital, and clearly showed the severity of the outcome, especially in patients with active disease and treated with immunosuppressive agents. This study reinforces the importance of early diagnosis and treatment with certain antifungals, especially in critically ill patients.

An increased frequency of varicocele was found in AS patients associated with sperm abnormalities but independent of therapy, anti-sperm antibodies, hormonal alterations, or disease parameters. Investigation for varicocele should be routine in AS patients with fertility problems.

RESUMOObjetivo: Avaliar o efeito da estimulação elétrica (EE) sobre o perfil metabólico e morfométrico dos músculos do membro posterior de ratos submetidos à imobilização durante 15 dias. Método: Ratos Wistar foram divididos em 3 grupos (n=5): controle, imobilizado por 15 dias e imobilizado associado à EE por 15 dias. Foram avaliados: reserva de glicogênio (RG) dos músculos sóleo (S), extensor longo dos dedos (ELD), gastrocnêmio branco (GB), gastrocnêmio vermelho (GV) e tibial anterior (TA), além do peso do sóleo, área das fibras e tecido conjuntivo do S. A análise estatística foi feita pelos testes ANOVA e Kruskal-Wallis (p<0,05). Resultados: A imobilização promoveu alterações significativas (p<0,05) como: redução nas RG (S: 44,73%, GB: 47,82%, GV: 46,34%, ELD: 41,66%, TA: 48,38%), no peso (7,2%) e na área das fibras (35%) do S, além do aumento da densidade do tecido conjuntivo (160%). A EE promoveu aumento significativo (p<0,05) nas RG de todos os músculos imobilizados (S: 90,47%, GB: 62,5%, GV: 95,45%, ELD: 76,19%, TA: 56,25%), no peso (20,94%) e na área das fibras (19,65%) do S e também promoveu redução significativa (15,38%, p<0,05) na densidade do tecido conjuntivo. Conclusões: A EE minimizou a redução das RG, preveniu a redução da área das fibras e a proliferação do tecido conjuntivo nos músculos submetidos à imobilização.Palavras-chave: estimulação elétrica, imobilização, morfometria, metabolismo, fisioterapia. ABSTRACT Effects of Neuromuscular Electrical Stimulation on Rat Hind Limbs Immobilized for 15 Days: Metabolic and Morphometric AnalysesObjective: To evaluate the effect of electrical stimulation on the metabolic and morphometric profile of rat hind limb muscles subjected to immobilization for 15 days. Method: Wistar rats were divided into three groups (n=5): control; immobilized for 15 days; and immobilized for 15 days with electrical stimulation. The glycogen reserves of the soleus, extensor digitorum longus (EDL), white gastrocnemius (WG), red gastrocnemius (RG) and tibialis anterior (TA) muscles were evaluated, along with the weight, fibrous area and conjunctive tissue of the soleus.

Normal testicular function has been identified in PAPS patients, in spite of morphofunctional penile abnormalities. Previous arterial thrombosis may underlie penile anthropometry alteration.

RESUMOObjetivo: Avaliar o conteúdo de glicogênio dos músculos respiratórios e o registro eletrocardiográfico (ECG) de ratos submetidos à estimulação diafragmática elétrica transcutânea (EDET).Método: Ratos adultos Wistar foram divididos em 2 grupos (n=6): controle (C) e tratado com EDET (f=50Hz; T ON / T OFF = 2/2 seg.; T= 400μs, i= 5mA com acréscimo de 1mA a cada 3 min.; t= 20 minutos) durante 5 dias. Após o período experimental, a análise do ECG foi realizada seguida do sacrifício dos animais para a obtenção das amostras dos músculos peitoral, intercostal, diafragma e abdominal, que foram encaminhadas para a análise do conteúdo de glicogênio. A análise estatística foi feita através do teste de normalidade e teste t de Student (p<0,05). Resultados: A EDET promoveu elevação no conteúdo de glicogênio em 42,85% no diafragma (C: 0,21 ± 0,008 x EDET: 0,30 ± 0,04, p>0,05), 81,2% no intercostal (C: 0,32 ± 0,08 x EDET: 0,58 ± 0,17, p>0,05), 96,7% no peitoral (C: 0,30 ± 0,05 x EDET: 0,59 ± 0,06, p<0,05) e 104,5% no abdominal (C: 0,22 ± 0,01 x EDET: 0,45 ± 0,03, p<0,05) quando comparado ao C. Os resultados da análise do ECG mostraram que não houve alteração nos parâmetros analisados (freqüência cardíaca, intervalos QR, QT e QTc) no grupo que recebeu a EDET. Conclusão: Além do trabalho sugerir um protocolo de EDET em animais, os resultados mostraram sua eficácia na melhora das condições energéticas da musculatura respiratória sem interferir na dinâmica elétrica cardíaca.Palavras-chave: estimulação elétrica, glicogênio, diafragma, ECG. ABSTRACT Transcutaneous Electrical Stimulation of the Diaphragm Improves the Metabolic Conditions of Respiratory Muscles in RatsObjective: To evaluate the respiratory muscle glycogen content and electrocardiogram (ECG) records of rats subjected to transcutaneous electric stimulation of the diaphragm (TESD). Method: Two groups (n=6) of male adult Wistar rats were utilized over a five-day period: controls (C) and TESD treatment (f = 50Hz; T ON / T OFF = 2/2 sec.; T = 400μs, i = 5mA with 1mA increase every three min.; t = 20 minutes). After this period, ECG analysis was done, followed by sacrificing the animals to obtain samples of the pectoral, intercostal, diaphragm and abdominal (A) muscles, which were used for glycogen content analysis. Statistical analysis was performed using the normality and Student t tests (p<0.05). Results: In comparison with the C group, TESD presented 42.85% glycogen increase in the diaphragm muscle (C: 0.21 ± 0.008 vs. TESD: 0.30 ± 0.04, p>0.05); 81.2% in the intercostal muscle (C: 0.32 ± 0.08 vs. TESD: 0.58 ± 0.17, p>0.05); 96.7% in the pectoral muscle (C: 0.30 ± 0.05 vs. TESD: 0.59 ± 0.06, p<0.05); and 104.5% in the abdominal muscles (C: 0.22 ± 0.01 vs. TESD: 0.45 ± 0.03, p<0.05). The ECG analysis showed that there were no alterations in the parameters analyzed (heart rate and QR, QT and QTc intervals) in the TESD group. Conclusion: This study suggests a protocol for TESD using an animal model. The results showed the efficacy of the protocol for improving the ener...

The clinical features of 2 new cases of Sjögren-Larsson syndrome are described. Histopathological examination of the skin in 1 of the cases revealed an ichthyosis vulgaris, which does not agree with the clinical and genetic classification. In the same case, a neuroblastoma was the cause of death, and neuropathological examination showed a symmetrical demyelination of some ascending and descending long tracts in the medulla and spinal cord.