Porto Alegre, Brazil
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Von Ranke F.M.,Federal University of Rio de Janeiro | Faria I.M.,Federal University of Rio de Janeiro | Zanetti G.,Federal University of Rio de Janeiro | Hochhegger B.,Santa Casa de Porto Alegre | Marchiori E.,Federal University of Rio de Janeiro
Radiologia Brasileira | Year: 2017

Tuberous sclerosis complex (TSC) is a genetically determined hamartomatous neurocutaneous disease with high phenotypic variability. TSC is characterized by widespread hamartomas and benign, or rarely malignant, neoplasms distributed in several organs throughout the body, especially in the brain, skin, retina, kidney, heart, and lung. Common manifestations include cortical tubers, subependymal nodules, white matter abnormalities, retinal abnormalities, cardiac rhabdomyoma, lymphangioleiomyomatosis, renal angiomyolipoma, and skin lesions. The wide range of organs affected by the disease implies that TSC1 and TSC2 genes play important roles in the regulation of cell proliferation and differentiation. Although recent advances in treatment have improved morbidity, the prognosis remains quite poor and nearly 40% of patients die by the age of 35 years. Imaging is important in the evaluation of TSC because of its role not only in presumptive diagnosis, but also in defining the full extent of involvement. This information allows a better understanding of the behavioural phenotype, as related to lesion location. Imaging also contributes to treatment planning. This pictorial review describes common and uncommon imaging manifestations of TSC. © Colégio Brasileiro de Radiologia e Diagnóstico por Imagem.


Hochhegger B.,Santa Casa de Porto Alegre | Hochhegger B.,Federal University of Rio de Janeiro | Marchiori E.,Federal University of Rio de Janeiro | Palermo L.,Federal University of Rio de Janeiro
British Journal of Radiology | Year: 2012

Metastatic pulmonary calcification is a consequence of calcium deposition in the normal pulmonary parenchyma, secondary to abnormal calcium metabolism. The most characteristic radiological manifestation is poorly defined nodular opacities that are mainly seen in the upper lung zone. The aim of this report is to describe the CT and MRI findings observed in two patients with metastatic pulmonary calcification. The disease may present in CT as consolidations with calcification, and with a high lesion/ muscle signal intensity ratio on T 1 weighted imaging without contrast in MRI. The high signal on T 1 weighted imaging probably occurs because the low calcium concentration of the lesion changes the surface effects of diamagnetic calcium particles, causing T 1 shortening of water protons. MRI is a good option for characterising calcium accumulation caused by a metabolic disorder. © 2012 The British Institute of Radiology.


Hochhegger B.,Santa Casa de Porto Alegre | Hochhegger B.,Federal University of Rio de Janeiro | Marchiori E.,Federal University of Rio de Janeiro | Dos Reis D.Q.,Santa Casa de Porto Alegre | And 2 more authors.
American Journal of Roentgenology | Year: 2012

OBJECTIVE. The aim of this study was to show the usefulness of chemical-shift MRI in the diagnosis of intranodular fat in seven patients with pulmonary hamartomas and indeterminate CT findings. CONCLUSION. In the setting of chemical-shift MRI, the average nodule signal intensity index of pulmonary hamartomas was 45.3% (SD = 25.5%). The correlation between average nodule signal intensity and CT attenuation in Hounsfield units was -0.94. Chemical-shift MRI could be an important tool for the detection of fat in pulmonary hamartomas with inconclusive CT findings. © American Roentgen Ray Society.


Belem L.C.,Federal University of Rio de Janeiro | Zanetti G.,Federal University of Rio de Janeiro | Hochhegger B.,Santa Casa de Porto Alegre | Guimaraes M.D.,Camargo Cancer Center | And 4 more authors.
Respiratory Medicine | Year: 2014

Metastatic pulmonary calcification (MPC) is a subdiagnosed metabolic lung disease that is commonly associated with end-stage renal disease. This interstitial process is characterized by the deposition of calcium salts predominantly in the alveolar epithelial basement membranes. MPC is seen at autopsy in 60-75% of patients with renal failure. It is often asymptomatic, but can potentially progress to respiratory failure. Chest radiographs are frequently normal or demonstrate confluent or patchy airspace opacities. Three patterns visible on high-resolution computed tomography have been described: multiple diffuse calcified nodules, diffuse or patchy areas of ground-glass opacity or consolidation, and confluent high-attenuation parenchymal consolidation. The relative stability of these pulmonary infiltrates, in contrast to infectious processes, and their resistance to treatment, in the clinical context of hypercalcemia, are of diagnostic value. Scintigraphy with bone-seeking radionuclides may demonstrate increased radioactive isotope uptake. The resolution of pulmonary calcification in chronic renal failure may occur after parathyroidectomy, renal transplantation, or dialysis. Thus, the early diagnosis of MPC is beneficial. The aim of this review is to describe the main clinical, pathological, and imaging aspects of MPC. © 2014 Elsevier Ltd. All rights reserved.


Hochhegger B.,Santa Casa de Porto Alegre | Ley-Zaporozhan J.,University of Heidelberg | Marchiori E.,Federal University of Fluminense | Irion K.,Liverpool Heart and Chest Hospital | And 4 more authors.
British Journal of Radiology | Year: 2011

Pulmonary embolism (PE) is the third most common acute cardiovascular disease after myocardial infarction and stroke, and results in thousands of deaths each year. Improvements in MRI accuracy are ongoing with the use of parallel imaging for angiography techniques and pulmonary perfusion. This, associated with other potential advantages of MRI (e.g. a radiation free method and better safety profile of MR contrast media), reinforces its use. The aim of this paper is to perform a pictorial review of the principal findings of MRI in acute PE. Acute PE can manifest itself as complete arterial occlusion and the affected artery may be enlarged. We report the main vascular and parenchymal signs, and an overview of current literature regarding accuracy, limitations and technical aspects is provided. © 2011 The British Institute of Radiology.


PubMed | Federal University of Rio de Janeiro, Hospital Sao Lucas PUCRS, Federal University of Paraná, Santa Casa de Porto Alegre and 2 more.
Type: Journal Article | Journal: Lung | Year: 2016

Niemann-Pick disease is a rare autosomal recessive lysosomal storage disease with three subtypes. Types A and B result from a deficiency of acid sphingomyelinase activity, associated with the accumulation of lipid-laden macrophages (so-called Niemann-Pick cells) in various tissues, especially the liver and spleen. Type A is a fatal neurodegenerative disorder of infancy. Type B Niemann-Pick disease is a less severe form with milder neurological involvement, characterized by hepatosplenomegaly, hyperlipidemia, and pulmonary involvement; most patients live into adulthood. Type C Niemann-Pick disease is a complex lipid storage disorder caused by defects in cholesterol trafficking, resulting in a clinical presentation dominated by neurological involvement. Pulmonary involvement occurs in all three types of Niemann-Pick disease, but most frequently in type B. Respiratory manifestations range from a lack of symptoms to respiratory failure. Progression of respiratory disease is slow, but inexorable, due to the accumulation of Niemann-Pick cells in the alveolar septa, bronchial walls, and pleura, potentially leading to a progressively worsening restrictive pattern on pulmonary function testing. Bronchoalveolar lavage has important diagnostic value because it shows the presence of characteristic Niemann-Pick cells. Radiographic findings consist of a reticular or reticulonodular pattern and, eventually, honeycombing, involving mainly the lower lung zones. The most common changes identified by high-resolution computed tomography are ground-glass opacities, mild smooth interlobular septal thickening, and intralobular lines. The aim of this review is to describe the main clinical, imaging, and pathological aspects of Niemann-Pick disease, with a focus on pulmonary involvement.


PubMed | Hospital Ernesto Dornelles, Santa Casa de Porto Alegre and Federal University of Rio Grande do Sul
Type: Journal Article | Journal: Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia | Year: 2016

IgG4-related disease (IgG4RD) is a recent inflammatory process of supposed autoimmune etiology, which is characterized by elevated serum IgG4 levels, dense lymphoplasmacytic infiltration rich in IgG4-positive plasma cells and storiform fibrosis. Tubulointerstitial nephritis is the most common renal manifestation, with different degrees of kidney dysfunction and variable clinical findings. Herein, the authors describe a new case of IgG4 tubulointerstitial nephritis (IgG4TN), and discuss clinic and pathologic criteria. Male patient, 72 years-old, was admitted on hospital service with clinical complaint of asthenia, loss of strength, emaciation, and anosmia. Previous history included type 2 diabetes mellitus. Laboratorial data included normochromic anemia, proteinuria, and creatinine elevation. Bilateral kidney ultrassonography/computed tomography revealed a heterogenous parenchyma, with diffuse irregular dense zones, areas of fibrosis on upper poles, and hydronephrosys. Kidney biopsy showed a dense interstitial lymphoplasmacytic infiltrate, with more than 50 plasma cell per high power field, irregular areas of fibroblastic and collagenous fibrosis, focal tubulitis, and normal glomeruli. Immunofluorescence revealed mild granular deposition of C3c and IgG in the tubular basement membrane. Immunohistochemestry was positive for CD138, lambda and Kappa light chains, and IgG4 (around forty five IgG4 positive plasma cells per high power field). IgG4 serum level was increased. The diagnosis of IgG4TN was then established. The patient received corticotherapy and strict control of glycemia with insulin, with marked improvement of symptoms and creatinine levels.


Castro M.,University of Washington | Rubin A.,Santa Casa de Porto Alegre | Laviolette M.,Laval University | Hanania N.A.,Baylor College of Medicine | And 2 more authors.
Annals of Allergy, Asthma and Immunology | Year: 2011

Background: Bronchial thermoplasty (BT) has been demonstrated to be safe and effective in the treatment of severe persistent asthma out to at least 1 year. Preclinical studies have demonstrated that the reduction in airway smooth muscle after bronchial thermoplasty persists out to at least 3 years. Objectives: To examine the persistence of effectiveness of BT 2 years posttreatment in subjects with severe asthma. Methods: Subjects participating in the long-term safety follow-up phase of the Asthma Intervention Research 2 (AIR2) Trial were evaluated by comparing the proportion of subjects who experienced exacerbations, adverse events, or healthcare utilization during the first year (year 1) after BT treatment with the proportion of subjects who experienced the same during the subsequent 12 months (year 2). Results: Severe exacerbations, respiratory adverse events, emergency department visits for respiratory symptoms, and hospitalizations for respiratory symptoms (proportion of subjects experiencing and rates of events), and stability of pre- and post-bronchodilator forced expiratory volume in 1 second (FEV 1), were comparable between years 1 and 2. The proportion of subjects experiencing severe exacerbations in year 2 after BT was 23.0%, compared with 30.9% in year 1. Conclusions: The reduction in the proportion of subjects experiencing severe exacerbations after BT is maintained for at least 2 years. Bronchial thermoplasty provides beneficial long-term effects on asthma outcomes in patients with severe asthma. © 2011 American College of Allergy, Asthma & Immunology.


PubMed | Federal University of Health Sciences, Porto Alegre, Santa Casa de Porto Alegre and Federal University of Rio Grande do Sul
Type: Journal Article | Journal: Brazilian journal of cardiovascular surgery | Year: 2017

Oxidative stress seems to be a role in the atherosclerosis process, but research in human beings is scarce.To evaluate the role of oxidative stress on human aortas of patients submitted to surgical treatment for advanced aortoiliac occlusive disease.Twenty-six patients were divided into three groups: control group (n=10) formed by cadaveric organ donors; severe aortoiliac stenosis group (patients with severe aortoiliac stenosis; n=9); and total aortoiliac occlusion group (patients with chronic total aortoiliac occlusion; n=7). We evaluated the reactive oxygen species concentration, nicotinamide adenine dinucleotide phosphate-oxidase, superoxide dismutase and catalase activities as well as nitrite levels in samples of aortas harvested during aortofemoral bypass for treatment of advanced aortoiliac occlusive disease.We observed a higher level of reactive oxygen species in total aortoiliac occlusion group (48.39.56 pmol/mg protein) when compared to severe aortoiliac stenosis (33.57.4 pmol/mg protein) and control (4.910.8 pmol/mg protein) groups (P<0.05). Nicotinamide adenine dinucleotide phosphate oxidase activity was also higher in total aortoiliac occlusion group when compared to the control group (3.811.7 versus 1.050.31 mol/min.mg protein; P<0.05). Furthermore, superoxide dismutase and catalase activities were significantly higher in the severe aortoiliac stenosis and total aortoiliac occlusion groups when compared to the control cases (P<0.05). Nitrite concentration was smaller in the severe aortoiliac stenosis group in comparing to the other groups.Our results indicated an increase of reactive oxygen species levels and nicotinamide adenine dinucleotide phosphate-oxidase activity in human aortic samples of patients with advanced aortoiliac occlusive disease. The increase of antioxidant enzymes activities may be due to a compensative phenomenon to reactive oxygen species production mediated by nicotinamide adenine dinucleotide phosphate oxidase. This preliminary study offers us a more comprehensive knowledge about the role of oxidative stress in advanced aortoiliac occlusive disease in human beings.


PubMed | Federal University of Health Sciences, Porto Alegre and Santa Casa de Porto Alegre
Type: Journal Article | Journal: Revista brasileira de ortopedia | Year: 2016

Paralysis or nerve injury associated with fractures of forearm bones fracture is rare and is more common in exposed fractures with large soft-tissue injuries. Ulnar nerve paralysis is a rare condition associated with closed fractures of the forearm. In most cases, the cause of paralysis is nerve contusion, which evolves with neuropraxia. However, nerve lacerations and entrapment at the fracture site always need to be borne in mind. This becomes more important when neuropraxia appears or worsens after reduction of a closed fracture of the forearm has been completed. The importance of diagnosing this injury and differentiating its features lies in the fact that, depending on the type of lesion, different types of management will be chosen.

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