Churg-Strauss syndrome is a vasculitis associated with asthma and eosinophilia. Respiratory involvement is marked by generally severe and often steroid-dependent late-onset asthma associated with allergic rhinitis and sometimes nasal polyposis and recurrent sinusitis. Asthma generally precedes the systemic vasculitis by a few years.
View Article and Find Full Text PDFObjective: Because the optimal cyclophosphamide (CY) treatment duration for severe polyarteritis nodosa (PAN) without virus infection and microscopic polyangiitis (MPA) has not been established, we conducted a trial to compare the effectiveness of 6 versus 12 CY pulses given in combination with corticosteroids (CS).
Methods: Sixty-five (18 PAN, 47 MPA) previously untreated patients were randomized to receive 12 (n = 34) or 6 (n = 31) CY pulses combined with CS. PAN and MPA were histologically proven or met ACR criteria.
Organising pneumonia, defined by intraalveolar buds of connective tissue, may be a disorder secondary to a determined cause (infectious agents, drugs) or occurring in a specific context (as the connective tissue disorders). It may also be a cryptogenic interstitial pneumonia with characteristic clinical and imaging features and especially an excellent response to corticosteroids.
View Article and Find Full Text PDFA series of 90 thymic epithelial tumors were reviewed and classified by histopathologic characteristics into the three major categories (A, B, and C) recognized by the WHO schema. Each tumor type was correlated with patient characteristics and clinical data (age, sex, presence of myasthenia gravis, tumor size and invasiveness, and completeness of resection), and with outcome (survival, recurrence, and metastasis). All tumors were categorized by the WHO schema.
View Article and Find Full Text PDFPrimary cardiac lymphoma is an extranodal non-Hodgkin's lymphoma exclusively located in the heart and/or pericardium, extremely rare in immunocompetent patients, and more frequent in immunodepressed patients. We present 3 retrospectives cases of primary cardiac lymphoma in immunocompetent patients and review 35 cases reported in the literature. Two patients were adults and one was a child.
View Article and Find Full Text PDFBackground: Chest physicians have a limited experience of primary pulmonary sarcomas, which represent a particular entity among rare intrathoracic neoplasms.
Design: Retrospective review of medical records.
Purpose: To study patients with primary sarcomas of the lung diagnosed in our pathology department in order to define their clinical characteristics, treatment, and prognosis.
Am J Respir Crit Care Med
November 2002
Idiopathic acute eosinophilic pneumonia (IAEP) is characterized by acute febrile respiratory failure associated with diffuse radiographic infiltrates and pulmonary eosinophilia. We conducted a multicenter retrospective study to characterize this rare clinical entity further and to improve its diagnostic criteria. A total of 13 male and 9 female patients (mean age: 29 +/- 15.
View Article and Find Full Text PDFA two-step sequence for the asymmetric vicinal acylation of olefins by a [2+2+1] strategy is reported. The key reaction is a [2+2] cycloaddition of an olefin to a chiral keteniminium salt derived from N-tosylsarcosinamide. This is followed by a regioselective Baeyer-Villiger oxidation of the resulting cyclobutanone to yield a lactol derivative that is equivalent to the product of addition of a carboxyl and a carbonyl group to the olefin.
View Article and Find Full Text PDFPulmonary alveolar proteinosis (PAP) is a rare, diffuse infiltrative lung disease of unknown etiology characterized by accumulation of abnormal lung surfactant in the alveoli and distal airways. Although it was first described in 1958, few large scale studies have been published. The diagnosis is conventionally based on lung biopsy and the value of less invasive procedures such as bronchoalveolar lavage (BAL) remains controversial.
View Article and Find Full Text PDFThe case of a right-to-left shunt-induced hypoxemia with an abnormal return of the inferior vena cava (AIVCR) into the left atrium (LA) is reported in a 30-year-old male with cyanosis and polycythemia. The chest X ray and the lung CT scan was normal. Spirometry was normal but the transfert-CO coefficient (KCO) was lowered.
View Article and Find Full Text PDFWegener's granulomatosis is characterized by 3 major lesions: necrosis, granulomatosis, and vasculitis. Although all organs may be involved, chronic rhinosinusitis, pulmonary involvement (especially with the typical imaging pattern of cavitary nodules), and glomerulonephritis are the most common clinical manifestations. Antineutrophil cytoplasmic autoantibodies (characteristically cytoplasmic with anti-proteinase 3 specificity) are of considerable help for diagnosis.
View Article and Find Full Text PDFMedicine (Baltimore)
November 2001
The process leading to irreversible fibrotic constriction of the bronchioles was studied in two cases of bronchiolitis obliterans (BO) after bone marrow transplantation. Because lysyl oxidase (LOX) is the main collagen cross-linking enzyme that might account for irreversible fibrosis, its expression was studied together with expression of extracellular matrix (ECM) proteins. Characteristic types of lesions could be distinguished on the basis of histological and immunohistological criteria.
View Article and Find Full Text PDFSphingosine-1-phosphate (S1P) is a potent lysophospholipid mediator mostly released by activated platelets. It is involved in several functions in peripheral tissues, but its effects in the central nervous system are poorly documented. Therefore, we have examined the effects of S1P on the proliferation of striatal astrocytes from the mouse embryo.
View Article and Find Full Text PDFSphingosine-1-phosphate (S1P) is a potent lysophospholipid mediator mostly released by activated platelets. It is involved in several functions in peripheral tissues, but its effects in the central nervous system are poorly documented. Therefore, we have examined the effects of S1P on the proliferation of striatal astrocytes from the mouse embryo.
View Article and Find Full Text PDFObjectives: To investigate the predictive value of testing for antineutrophil cytoplasmic antibodies (ANCA) in 55 patients with systemic Wegener's granulomatosis (WG) included in a randomized, prospective trial comparing corticosteroids and oral or pulse cyclophosphamide.
Methods: All 55 patients received corticosteroids. A cyclophosphamide pulse of 0.
Metastases are an uncommon cause of tumor in the sellar region. We report two cases of small cell lung cancer presenting with visual loss and hypopituitarism resulting from metastasis to the pituitary area.
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