Inherited primary arrhythmia disorders: cardiac channelopathies and sports activity.

Sudden cardiac death (SCD) in an apparently healthy individual is a tragedy. It is important to identify the cause of death and to prevent SCD in potentially at-risk family members. Inherited primary arrhythmia disorders are associated with exercise-related SCD. Despite the well-known benefits of exercise, exercise restriction has been a historical mainstay of therapy for these conditions. However, since familiarity with inherited arrhythmia conditions has increased and patients are often children and young adults, it is necessary to reassess the treatment guidelines regarding exercise constraints. The aim of this review is to analyze the risk of exercise-induced SCD in patients with inherited cardiac conditions and explore the challenges faced when advising patients about exercise limitations. We searched for publications on cardiac channelopathies in PubMed with the following medical subject headings (MeSH): "long QT syndrome"; "short QT syndrome"; "Brugada syndrome"; and "catecholaminergic polymorphic ventricular tachycardia". The abstracts of these articles were scanned, and articles of relevance, along with pertinent references, were read in full. The analysis was restricted to reports published in English. The findings of this analysis suggest that exercise with low-to-moderate cardiovascular demand may be possible under regular clinical follow-up in inherited primary arrhythmia disorders. Recent data show that patients with inherited primary arrhythmia disorders are at low risk for events once a comprehensive treatment program has been established. Recreational activity is likely safe for these individuals, with personalized management based on individual patient preferences and priorities.

Spontaneous hemothorax and neurofibromatosis type 1: How to explain it, how to explore it and how to treat it?

Neurofibromatosis type 1 (NF1), also known as Von Recklinghausen's disease is an autosomal dominant genetic disorder. It is the most common of phacomatoses. Pulmonary complications have been rarely described in the literature. It is generally a benign disease, but has the potential for rare and fatal complications, such as spontaneous hemothorax. We reported two cases of patients with a history of von Recklinghausen's disease. Both of them suffered sudden chest pain. Chest-X ray revealed a hemi-thoracic opacity. Enhanced chest computed tomography showed massive hemothorax, but no evidence of tumors or an obvious bleeding point in the thorax. After we had ensured a stable hemodynamic condition, we performed video-assisted thoracic surgery to remove the hematoma. No evidence of bleeding was noticed in the first patient whereas an active bleeding was observed in the second patient. We could not determine which vessel was responsible of the hemorrhage. Electrocoagulation and clot removal were performed.  Evolution was favorable for both patients. Spontaneous hemothorax is a rare and potentially life threatening NF1's complication. A tumor like neurofibroma or more rarely vascular involvement of large or small caliber arteries may be at hemothorax's origin.

Interstitial lung diseases: Imaging contribution to diagnosis and elementary radiological lesions.

Interstitial pneumonias comprise a heterogeneous group of disorders in which a multidisciplinary approach is important for accuracy in diagnosis; indeed, one might say, even mandatory. The team of collaborators should include radiologists, because high resolution computed tomography (HRCT) of the thorax is the first, and most of times, the only imaging examination to be prescribed after chest X-ray. Elementary lesions of the interstitium can be accurately described with HRCT, inasmuch as lung windowing with sharp filtering in this technique reproduces the microscopic features of the lung. Guidance of bronchoalveolar lavage and biopsy procedures is also possible with HRCT.

Microscopic polyangiitis associated with primary biliary cirrhosis, Sjogren's syndrome and Hashimoto's thyroiditis.

The association between microscopic polyangiitis (MPA) and primary biliary cirrhosis (PBC) has seldom been reported. We describe here a patient who presented with sensorimotor neuropathy along with hypothyroidism, renal failure and liver dysfunction. Detection of antinuclear antibodies at a titer of 1/800, anti-SSA, anti-SSB, anti-GP210, anti-microsomial and p-ANCA anti-myeloperoxydase antibodies along with renal, salivary and liver biopsy led to a diagnosis of MPA associated with PBC, Sjogren's syndrome and Hashimoto's thyroiditis.

[The role of imaging in thoracic tuberculosis]. / Apport de l'imagerie dans la tuberculose thoracique.

Tuberculosis is an infectious disease mostly due to Mycobacterium tuberculosis. It is frequent in developing countries and its incidence is rising in developed countries. Lungs are the most involved organs of the chest but other structures can be affected. Imaging is fundamental in the management of the disease. Confirmation of diagnosis can be made only by bacteriologic and/or histologic exams. The first approach of diagnosis is based on clinical symptoms and chest X-ray signs. Radiologic signs depend on patient's age, his immune status and his previous contact with M. tuberculosis. Conventional chest X-ray remains the first-line exam to realize. It can suggest the diagnosis on the appearance and location of the lesions. CT scan is recommended for the positive diagnosis in case of discrepancy between clinical and radiographic signs, as for the diagnosis of parenchymal, vascular, lymph nodes, pleural, parietal or mediastinal complications. It is also essential for the evaluation of parenchyma sequelae. MRI and PET-scan have limited indications. The purpose of this article is to illustrate different radiological forms of chest tuberculosis, its sequelae and complications and to highlight the role of each imaging technique in the patient's management.

[Imaging of pulmonary artery involvement in Takayasu disease]. / Imagerie de l'atteinte artérielle pulmonaire au cours de la maladie de Takayasu.

OBJECTIVES: Illustrate imaging aspects of pulmonary artery involvement in Takayasu's arteritis. PATIENTS AND METHODS: Retrospective study of six patients among 28 patients with Takayasu arteritis whose disease involved the pulmonary arteries and to review their clinical and computed tomography data. RESULTS: Mean patient age among those with pulmonary artery involvement was 34 years. All patients exhibited extensive lesions of systemic arteries. The most common computed tomography angiography sign was wall thickening. Dilatation of the pulmonary artery trunk was observed in one-third of cases. CONCLUSION: Pulmonary arterial involvement in Takayasu's disease is not uncommon. Computed tomography is a reliable imaging technique to establish the diagnosis.

[Cardiac tamponade and myocarditis in Churg-Strauss syndrome]. / Tamponnade et myocardite aiguë au cours d'un syndrome de Churg-Strauss.

INTRODUCTION: The successive occurrence of pericardial tamponade and myocarditis during a Churg-Strauss syndrome is exceptionally described. We report a patient in whom pericardial tamponade and myocarditis were the presenting manifestation of a Churg-Strauss syndrome. CASE REPORT: A 58-year-old woman was admitted because of alteration of the clinical status with eosinophilia. One month ago, she was hospitalized for a pericardial tamponade treated by pericardial drainage. Acute myocarditis was diagnosed on chest pain during the second hospitalization. The etiologic inquiry ended in the diagnosis of Churg-Strauss complicated with a double cardiac involvement. A good response of clinical and biological anomalies was obtained after corticosteroid and immunosuppressive treatment. CONCLUSION: Isolated or multiple involvements of cardiac tunics should lead to make diagnosis of systemic vasculitis. A complete initial assessment and a close observation of the patients followed for Churg-Strauss syndrome is imperative to detect a cardiac achievement and set up an early treatment.

Posterior mediastinal angiomyxolipoma with spinal canal extension.

BACKGROUND: Angiomyxolipoma is a benign tumor considered as a variant of lipoma and that occurs mainly in the subcutis. The mediastinal location hasn't been previously reported. AIM: To describe the radiological features of this tumor in its posterior mediastinal location and to confront them to the pathological features. CASE REPORT: We report the case of a 49-year-old woman who was admitted for chest wall pain and neurologic disturbance of her two lower limbs. The chest X-ray showed a posterior mediastinal opacity. On CT examination, this mass contained some small areas of fat and enhanced intensely. Microscopic examination of the excised mass confirmed the diagnosis of posterior mediastinal angiomyxolipoma. CONCLUSION: Mediastinal location of angiomyxolipoma hasn't been previously reported. Clinicians and radiologists should be aware that this diagnosis should be suggested, among others, when there is a posterior mediastinal mass that contains fat and that intensely enhances with a possible spinal cord extension.

[Small-cell lung carcinoma: CT imaging features]. / Aspects tomodensitométriques du carcinome bronchique à petites cellules.

Small cell lung carcinoma (SCLC) typically is central in location. It is a very aggressive tumor characterized by its propensity for invasion of mediastinal structures, frequently, the ipsilateral pulmonary artery, multifocal nodal metastases and high frequency of distant metastases at initial presentation. CT is very sensitive and effective for local and regional staging. Combined with other diagnostic modalities, especially PET imaging, it allows whole body imaging for accurate staging, which is mandatory for therapeutic management. The different CT imaging features of SCLC and its more specific imaging characteristics will be reviewed in this article.

[Chorea revealing a polycytemia vera]. / Chorée révélant une polyglobulie primitive.

Neurological manifestations in polycytemia vera are common. However, chorea is an exceptionally revealing feature of this disease. We report a 78-year-old man who presented with headache and an abnormal movement disorder corresponding to chorea. Laboratory findings showed increased levels of hemoglobin at 20 g/dl and hematocrit at 62.3%. An elevated erythrocyte mass to twice the normal value demonstrated the absolute erythrocytosis. A JAK2 V617F gene mutation was identified. A diagnosis of polycytemia vera-associated chorea was obtained. Clinical and biological outcomes were favorable after therapeutic phlebotomy and treatment with hydroxyurea. We recommend a complete blood cell count in elderly patient presenting with chorea to eliminate a diagnosis of polycytemia vera.

Imaging of thoracic textiloma.

OBJECTIVE: Intrathoracic textiloma or gossypiboma, a retained surgical sponge in the thoracic cavity, is an exceptional but serious complication following thoracic or abdominal surgery. The purpose of this work is to highlight the topographic features of thoracic textiloma and to describe imaging aspects, and, particularly, computed tomography (CT) features. METHODS: Eight patients have been operated in our thoracic surgery department for thoracic gossypiboma. In the past, three patients had undergone hepatic surgery and the five others had a history of thoracic surgery. All the patients had a chest radiograph, five of them had a thoracic ultrasonography, all had a chest CT, and one patient had a chest magnetic resonance imaging (MRI). RESULTS: In patients with a history of abdominal surgery, the foreign body was located in the parenchyma of the right lower lobe. In the other patients, the foreign body was either intrapleural or mediastinal. Ultrasonography suggested the diagnosis of textiloma in three of the five patients by demonstrating a non-calcified hyperechoic mass with acoustic shadow. At CT, the gossypiboma was a low-attenuating mass containing trapped gas lucencies in six patients and it was a high-attenuating mass in two patients. MRI showed a diaphragmatic defect in one patient with an intrapulmonary gossypiboma that migrated from the abdomen. CONCLUSIONS: The CT aspect of thoracic gossypiboma may be different according to pleural or parenchymal location. The spongiform appearance, characteristic in abdominal gossypiboma, is not the only CT presentation of thoracic gossypiboma. The confrontation of the surgical history with the CT signs helps to have a preoperative diagnosis.

Acute pulmonary embolism: epidemiologic and tomodensitometric study.

BACKGROUND: Acute pulmonary embolism is a common disease with substantial morbidity and mortality in untreated patients. It requires an urgent positive diagnosis. AIM: To assess the prevalence of acute pulmonary embolism and calculate the sensitivity and specificity of multidetector CT for the diagnosis of acute pulmonary embolism in a hospital specialized in cardio-thoracic diseases. METHODS: This is a prospective study conducted at Abderrahmen Mami Hospital, which included 200 consecutive patients suspected of acute pulmonary embolism and explored by a multidetector CT pulmonary angiography (16 slices). RESULTS: Prevalence of acute pulmonary embolism was calculated at 37.5%. The multidetector CT has enabled an alternative diagnosis in 46 patients (40%). The sensitivity and specificity of multidetector CT were calculated respectively 89.6% and 100%. CONCLUSION: The prevalence of acute pulmonary embolism, in a hospital specialized in cardio-thoracic diseases, is higher than that found in general hospitals. High sensitivity and specificity of multidetector CT makes it the gold standard for the diagnosis of pulmonary embolism.

[Imaging features of primary pulmonary lymphomas]. / Imagerie des lymphomes pulmonaires primitifs.

INTRODUCTION: Primitive pulmonary lymphomas (PPL) are rare tumors, often misdiagnosed by radiologists. METHODS: In order to illustrate the various radiological presentations of PPL, we report a retrospective series of nine cases of PPL collected in our service over a period of four years. A mucosa-associated lymphoid tissue (MALT) lymphoma was found in six patients, a diffuse large B-cell lymphoma in two patients and lymphomatoid granulomatosis in one patient. All diagnoses were proven histologically by bronchial or surgical biopsies. RESULTS: Among the six cases of MALT lymphoma, computed tomography (CT) demonstrated one or more areas of alveolar consolidation in four patients, progressing with a chronic course over 2 years in two patients. Other CT features were nodular opacities associated with a mass or consolidation and diffuse "ground glass" opacities. In the two cases of diffuse large B-cell lymphoma, CT showed one or more masses spreading locally, mimicking primary bronchial carcinoma. In lymphomatoid granulomatosis the CT showed diffuse interstitial disease with fibrosis. CONCLUSION: The imaging features of PPL are very polymorphic. The diagnosis of MALT lymphoma is often difficult because its radiological appearance is often falsely reassuring.

CMOS/microfluidic Lab-on-chip for cells-based diagnostic tools.

We describe in this paper cells sensing and manipulation methods, as well as platforms based on Lab-on-chip devices. Among other contributions, new circuit and microfluidic techniques, and packaging methods are proposed for efficient cells manipulation and detection. The proposed devices include high-sensitivity sensing circuits (200 mV/fF), low-pressure liquid injection interfaces (< 0.65 psi), low-voltage manipulation signals, direct-write microfluidic fabrication technique on top of CMOS based capacitive sensors. In addition, several types of electrode arrays (square and L-shaped) are used for the manipulation of various types of cells and particles.

[Pseudotumoral mediastinal amyloidosis]. / Amylose ganglionnaire médiastinale pseudotumorale.

PURPOSE: Amyloidosis involvement of mediastinal nodes is rare. Isolated pseudotumoral involvement without extra-thoracic disease is a diagnostic challenge and typically raises concern for underlying malignancy. We present 3 cases of pseudotumoral mediastinal amyloidosis. METHODS: We report the cases of 3 patients presenting with recent onset of respiratory symptoms. Bronchoscopy showed mucosal infiltration suspicious for lymphangitic spread of tumor. The patients underwent chest radiography complemented by CT of the chest and abdomen, and laboratory and immunological work-up. A diagnosis of pseudotumoral mediastinal amyloidosis was confirmed by mediastinoscopic biopsy in all cases. RESULTS: CT showed a pulmonary and mediastinal tumor process in 2 cases and pericarinal tumor in 1 case. Diffuse bronchial wall thickening was present in all cases. Review of biopsy material showed tracheobronchial amyloidosis in 1 case. Patient work-up showed no evidence of extra-thoracic amyloidosis. Rapid progression of bronchial obstruction was observed in 1 case. CONCLUSION: The imaging features of mediastinal amyloidosis are non-specific. Pseudotumoral involvement of mediastinal nodes associated with pulmonary amyloidosis accelerates the degree of airway obstruction.

[Barraquer-Simons syndrome in a patient with systemic lupus erythematosus]. / Syndrome de Barraquer-Simons au cours d'un lupus érythémateux systémique.

Barraquer-Simons syndrome is a rare disorder characterized by a partial lipodystrophy. It is often associated with positive C3 nephritic factor and various glomerular nephropathy. Its association with some autoimmune diseases has also been reported. We report a 30-year-old woman with partial lipodystrophy, lupus erythematosus, hypothyroidism and vitiligo.