(-)-CGP 12177 increases contractile force and hastens relaxation of human myocardial preparations through a propranolol-resistant state of the beta1-adrenoceptor

Two forms of the activated beta(1)-adrenoceptor exist, one that is stabilized by (-)-noradrenaline and is sensitive to blockade by (-)-propranolol and another which is stabilized by partial agonists such as (-)-pindolol and (-)-CGP 12177 but is relatively insensitive to (-)-propranolol. We investigated the effects of stimulation of the propranolol-resistant PI-adrenoceptor in the human heart. Myocardium from non-failing and failing human hearts were set up to contract at 1 Hz. In right atrium from non-ailing hearts in the presence of 200 nM (-)-propranolol, (-)-CGP 12177 caused concentration-dependent increases in contractile force (-logEC(50)[M] 7.3+/-0.1, E-max 23+/-1% relative to maximal (-)-isoprenaline stimulation of beta(1)- and beta(2)-adrenoceptors, n=86 patients), shortening of the time to reach peak force (-logEC(50)[M] 7.4+/-0.1, E-max 37+/-5%, n=61 patients) and shortening of the time to reach 50% relaxation (t(50%), -logEC(50)[M] 7.3+/-0.1, E-max 33+/-2%, n=61 patients). The potency and maxima of the positive inotropic effects were independent of Ser49Gly- and Gly389Arg-beta(1)-adrenoceptor polymorphisms but were potentiated by the phosphodiesterase inhibitor 3-isobutyl-1-methylxanthine (-logEC(50)[M] 7.7+/-0.1, E-max 68+/-6%, n=6 patients, P

Effect of alcohol on iron storage diseases of the liver

The consumption of excess alcohol in patients with liver iron storage diseases, in particular the iron-overload disease hereditary haemochromatosis (HH), has important clinical consequences. HH, a common genetic disorder amongst people of European descent, results in a slow, progressive accumulation of excess hepatic iron. If left untreated, the condition may lead to fibrosis, cirrhosis and primary hepatocellular carcinoma. The consumption of excess alcohol remains an important cause of hepatic cirrhosis and alcohol consumption itself may lead to altered iron homeostasis. Both alcohol and iron independently have been shown to result in increased oxidative stress causing lipid peroxidation and tissue damage. Therefore, the added effects of both toxins may exacerbate the pathogenesis of disease and impose an increased risk of cirrhosis. This review discusses the concomitant effects of alcohol and iron on the pathogenesis of liver disease. We also discuss the implications of co-existent alcohol and iron in end-stage liver disease.

Finite element analysis of pectus carinatum surgical correction via a minimally invasive approach

Pectus carinatum (PC) is a chest deformity caused by a disproportionate growth of the costal cartilages compared to the bony thoracic skeleton, pulling the sternum towards, which leads to its protrusion. There has been a growing interest on using the ‘reversed Nuss’ technique as minimally invasive procedure for PC surgical correction. A corrective bar is introduced between the skin and the thoracic cage and positioned on top of the sternum highest protrusion area for continuous pressure. Then, it is fixed to the ribs and kept implanted for about 2–3 years. The purpose of this work was to (a) assess the stresses distribution on the thoracic cage that arise from the procedure, and (b) investigate the impact of different positioning of the corrective bar along the sternum. The higher stresses were generated on the 4th, 5th and 6th ribs backend, supporting the hypothesis of pectus deformities correction-induced scoliosis. The different bar positioning originated different stresses on the ribs’ backend. The bar position that led to lower stresses generated on the ribs backend was the one that also led to the smallest sternum displacement. However, this may be preferred, as the risk of induced scoliosis is lowered.

Crescer com saúde. Perspectiva dos professores do 1.º ciclo acerca da postura dos alunos e análise postural de crianças com 6 a 7 anos inseridas na comunidade escolar

O presente estudo teve como objectivos conhecer a percepção dos professores do ensino básico acerca da postura corporal dos alunos na sala de aula e analisar a postura de alunos do 1º ano do ensino básico. Este estudo foi realizado numa escola do 1º Ciclo, com 6 professores voluntários e 40 alunos do 1º ano. Na I parte referente ao estudo qualitativo, realizou-se uma discussão por grupo focal, com um roteiro de perguntas que os professores foram discutindo livremente expondo as suas opiniões. Concluiu-se que há por parte dos professores noção de como é a postura corporal dos alunos na sala de aula, como essa postura corporal, pode influenciar de forma, positiva ou negativa o estado de saúde do aluno na sala de aula e comprometer a qualidade de vida dos alunos. Na II parte quantitativa os alunos foram avaliados através do registo fotográfico, num programa de avaliação postural, demonstrando os resultados que na amostra em estudo não foi encontrado desvio vertical significativo entre a cintura escapular e a cintura pélvica. No entanto, foi encontrado desvio horizontal significativo do alinhamento das omoplatas entre si e das espinhas ilíacas postero-superiores entre si, já indicando um início de desvio postural com carácter escoliótico. Conclui-se pois que os alunos avaliados apresentavam problemas posturais com necessidade de serem acompanhados por Fisioterapeutas.

Alterações da curvatura da coluna vertebral: influência da fisioterapia, a nível neuromuscular

Mestrado em Fisioterapia

Nephrotic syndrome associated with hepatointestinal schistosomiasis

Schistosomal nephropathy has long been related to the hepatosplenic form of schistosomiasis. In the last few years, 24 patients with hepatointestinal schistosomiasis and the nephrotic syndrome were studied. Aiming at evaluating a possible etiologic participation of schistosomiasis in the development of the nephropathy, this group was comparatively studied with a group of 37 patients with idiopathic nephrotic syndrome. Both groups had a different distribution of the histologic lesions. In the group with schistosomiasis there was a statistically significant prevalence of proliferative mesangial glomerulonephritis (33.3%), whereas in the control group there was prevalence of membranous glomerulonephritis (32.4%). On immunofluorescence, IgM was positive in 94.4% of the patients with schistosomiasis versus 55.0% in the control group (p<0.01). In the group with schistosomiasis, 8 patients evidenced mesangial proliferative glomerulonephritis and 5, membra-noproliferative glomerulonephritis. In both histological types immunofluorescence showed IgM and C3 granular deposits in the glomeruli. The data in this study suggests that mesangial proliferative and membranoproliferative glomerulonephritis, with glomerular granular IgM and C3 deposits, represent the renal lesions of the schistosomiasis associated nephropathy.

Auditory event-related potentials in children with benign epilepsy with centro-temporal spikes

Benign focal epilepsy in childhood with centro-temporal spikes (BECTS) is one of the most common forms of idiopathic epilepsy, with onset from age 3 to 14 years. Although the prognosis for children with BECTS is excellent, some studies have revealed neuropsychological deficits in many domains, including language. Auditory event-related potentials (AERPs) reflect activation of different neuronal populations and are suggested to contribute to the evaluation of auditory discrimination (N1), attention allocation and phonological categorization (N2), and echoic memory (mismatch negativity – MMN). The scarce existing literature about this theme motivated the present study, which aims to investigate and document the existing AERP changes in a group of children with BECTS. AERPs were recorded, during the day, to pure and vocal tones and in a conventional auditory oddball paradigm in five children with BECTS (aged 8–12; mean = 10 years; male = 5) and in six gender and age-matched controls. Results revealed high amplitude of AERPs for the group of children with BECTS with a slight latency delay more pronounced in fronto-central electrodes. Children with BECTS may have abnormal central auditory processing, reflected by electrophysiological measures such as AERPs. In advance, AERPs seem a good tool to detect and reliably reveal cortical excitability in children with typical BECTS.

Aracnoidite Adesiva Lombar

Spinal arachnoiditis, an inflammatory process involving all three meningeal layers as well as the nerve roots, is a cause of persistent symptoms in 6% to 16% of postoperative patients. Although spinal surgery is the most common antecedent associated with arachnoiditis, multiple causes have been reported, including infection, intrathecal steroids or anesthetic agents, trauma, subarachnoid hemorrhage and ionic myelographic contrast material--both oil soluble and water soluble. In the past, oil-based intrathecal contrast agents (Pantopaque) were associated with arachnoiditis especially when this material was introduced into the thecal sac and mixed with blood. Arachnoiditis is apparently rarely idiopathic. The pathogenesis of spinal arachnoiditis is similar to the repair process of serous membranes, such as the peritoneum, with a negligible inflammatory cellular exudate and a prominent fibrinous exudate. Chronic adhesive arachnoiditis of the lower spine is a myelographic diagnosis. The myelographic findings of arachnoiditis were divided into two types by Jorgensen et al. In type 1, "the empty thecal sac" appearance, there is homogeneous filling of the thecal sac with either absence of or defects involving nerve root sleeve filling. In type 2 arachnoiditis, there are localized or diffuse filling defects within the contrast column. MRI has demonstrated a sensitivity of 92% and a specificity of 100% in the diagnosis of arachnoiditis. The appearance of arachnoiditis on MRI can be assigned to three main groups. The MRI findings in group I are a conglomeration of adherent roots positioned centrally in the thecal sac. Patients in group II show roots peripherally adherent to the meninges--the so called empty sac. MRI findings in group III are a soft tissue mass within the subarachnoid space. It corresponds to the type 2 categorization defined by Jorgensen et al, where as the MRI imaging types I and II correspond to the myelographic type 1.

Utilidade de Ecocardiografia da Sobrecarga Farmacológica com Dobutamina nas Cardiomiopatias Dilatadas não Isquémicas

INTRODUCTION: Low-dose dobutamine stress echocardiography is a common and useful technique to assess myocardial viability in patients with ischemic cardiomyopathy. OBJECTIVE: To evaluate the use of low-dose dobutamine stress echocardiography in determining the functional status of patients with idiopathic dilated cardiomyopathy (IDCM). METHODS: Prospective study of 28 patients with IDCM by transthoracic echocardiography (2D), low-dose dobutamine stress echocardiography, cardiopulmonary exercise testing (CPET) and measurement of pro-BNP. RESULTS: The mean age of the population was 50.3 +/- 11.5 years, 9 female and 19 male. Mean ejection fraction was 32.1 +/- 9.8%. All were in sinus rhythm. The following parameters were analyzed in 2D echocardiography and after dobutamine: dimensions of left atrium (LA) and of left ventricle in diastole and systole, shortening fraction (%), left ventricular end-diastolic (EDV) and end-systolic volumes (ESV), ejection fraction (EF), and mitral inflow (E, A, E/A ratio and deceleration time). In CPET, we considered the following parameters: peak VO2 and % maximal peak VO2 attained. We compared echo results with CPET. There was a correlation between age and peak VO2 (r = -0.38 with p = 0.049). In 2D echo, there was a correlation between baseline EF and LA dimensions and peak VO2 (r = 0.45 / p = 0.004 and r = -0.49 / p = 0.014, respectively). After dobutamine echo, there was a correlation between some echo parameters and peak VO2: EF - r = 0.59 / p = 0.001, LA dimensions - r = 0.56 / p = 0.007, and ESV - r = -0.45 / p = 0.026. Percentage maximal peak VO2 attained correlated with LA dimensions measured in 2D echo and after dobutamine (r = -0.398 / p = 0.036 and r = -0.674 / p = 0.02 respectively) and EF after dobutamine (r = -0.389 / p = 0.04). The value of pro-BNP correlated with LA dimensions and baseline EF (r = 0.44 / p = 0.02 and r = -0.57 / p = 0.002, respectively), and the correlation was maintained after inotropic stimulation with dobutamine (r = 0.57 / p = 0.001 and r = -0.55 / p = 0.0039). CONCLUSION: Low-dose dobutamine stress echocardiography showed stronger correlations with cardiopulmonary exercise testing than the parameters evaluated by conventional echocardiography and could be used to determine the functional status of patients with dilated cardiomyopathy; patients with greater ejection fraction after inotropic stimulation had better cardiopulmonary tests.

Quilopericárdio Primário Idiopático

O quilopericárdio é uma entidade rara, que é encontrada mais frequentemente associada a traumatismos (com lesão do canal torácico), neoplasias e infiltrações por filária. As formas primárias são mais raras e associam-se a malformações da circulação linfática. Apresenta-se o caso clínico de um doente de 52 anos, sexo masculino, com o diagnóstico de quilopericárdio primário, sendo discutida a abordagem diagnóstica, assim como a evolução clínica favorável com tratamento conservador.

Úlceras Esofágicas Idiopáticas em Mulher VIH+: Terapêutica com Talidomida

Apresenta-se o caso clínico de uma doente seropositiva para o vírus da imunodeficiência humana (VIH+) com um quadro de esofagite idiopática com excelente resposta à terapêutica com talidomida, associando-se regressão do síndrome febril que se manifestara concomitantemente. O mecanismo de acção da talidomida nestes casos é desconhecido, mas poderá estar relacionado com os efeitos do fármaco sobre o sistema imunitário ou dever-se a um efeito anti-retroviral directo.

Miosite Orbitária Idiopática

A miosite orbitária idiopática é uma doença inflamatória de etiologia desconhecida, que deve constituir sempre um diagnóstico de exclusão, cujo conhecimento tem evoluído com as novas técnicas de imagem. Os autores descrevem o caso clínico de um doente do sexo masculino, 69 anos, que inicia subitamente um quadro clínico caracterizado por dor peri-orbitária à direita e diplopia, ao que se seguiu, passado uma semana, diminuição da acuidade visual homolateral. À observação, era de realçar marcada diminuição da acuidade visual, oftalmoplegia, proptose e hipostesia álgica no território do V par do mesmo lado. Faz-se referência aos aspectos tomodensitométricos e à resposta à corticoterapia, devido à sua importância no diagnóstico desta patologia.

Annular Lichen Planus in Association with Crohn Disease

BACKGROUND: Lichen planus is an idiopathic inflammatory disease of the skin and mucous membranes. Although the etiology is not established, it has been associated with autoimmune diseases, viral infections, drugs and dental restoration materials. However, the association with inflammatory bowel disease has been very rarely reported in the literature. CASE REPORT: A 19-year-old female patient presented with annular lesions on her upper body and limbs, with a sharply defined border and non-atrophic skin in the center. The lesions were hyperpigmented and had been stable for over one year. The histopathology confirmed the diagnosis of annular lichen planus. She had weight loss, occasional diarrhea, and a severe anemia. The investigation of these symptoms led to the diagnosis of Crohn disease and a sickle cell trait. Therapy with systemic corticosteroids and mesalazine controlled the intestinal disease, with concomitant improvement of the skin lesions. CONCLUSIONS: As lichen planus can be associated with other immunological disorders, the association with inflammatory bowel disease should be considered in the evaluation of the patient.

Tranexamic Acid for Major Spinal Surgery in Children. A Retrospective Study

Introduction: Paediatric patients who undergo posterior spinal fusion surgery to correct scoliosis often require multiple blood transfusions. Tranexamic acid is a synthetic antifibrinolytic drug that reduces transfusion requirements in scoliosis surgery (1),(2),(3). Methods: To evaluate the efficacy of prophylactic tranexamic acid (TA) (initial dose of 10mg/kg and infusion of 1mg.kg(-1).h(-1)) in reducing perioperative blood transfusion requirements, we reviewed patients files and compared the amount of blood lost and blood transfused in the perioperative period of 12 patients (54.5%) that received TA and 10 patients (45.5%) who did not received TA. T-Student test was applied. Results: The average difference of blood losses (2,67 +/- 6,06ml) and blood transfused (212,9 +/- 101,1ml) between the two groups was not statistically significant (p>0.05). No thrombotic complications were detected in either group. Discussion: Results of the current study showed that prophylactic low dose of TA did not have a significant effect in the management of intraoperative blood loss and transfusion requirements in children undergoing scoliosis surgery. It is important to emphasize that our study is retrospective and that the size of the sample is small. Further studies are needed to evaluate the efficacy and safety of TA on paediatric scoliosis surgery.

McCune Albright Syndrome: A Diagnosis to be Kept in Mind

Precocious puberty, defined as the development of secondary sexual characteristics before the age of 8, often leads to anxiety in patients and their families but also in clinicians searching for the final diagnosis. After adequate investigation, the majority of the cases in girls turn out to be idiopathic. The authors present a case of McCune Albright syndrome in order to call attention to a rare cause of sexual precocity and the value of ultrasound in the evaluation of these situations. 10 years old infant girl admitted in our department due to irregular menstrual bleeding. She experienced a vaginal bleeding by the age of 3 which led to the diagnosis of McCune Albright Syndrome after a complete evaluation. Pubertal assessment revealed a reversed sequence in the remaining events with adrenarche at 5 and thelarche at 8. Hormonal evaluation demonstrated low FSH and LH levels (11,2 and 6,72 respectively) with high estrogen (204). Pelvic ultrasound showed a normal sized uterus (73x 29x32 mm), endometrial thickness of 5 mm and ovaries with several microfollicles and a copus luteum measuring 23 mm in the right ovary. McCune Albright syndrome is a very uncommon cause of sexual precocity that should, however, be suspected in all infant girls who present with vaginal bleeding. It is characterized by a triad: polyostotic fibrous dysplasia, gonadotropin-independent precocious puberty and café-au-lait skin spots. Due to autonomous production of estrogen by the ovaries, ultrasound image of the female reproductive tract is inconsistent with chronologic age. Pelvic ultrasound demonstrates a normal sized uterus with a well defined cervix and clearly identified ovaries with several follicles, similar to adult women of reproductive age. Ultrasonography of the pelvis has also an important role excluding other causes of GnRH-independent precocious puberty conditions like ovarian cysts or tumors.