| Autor(en) |
Titel |
Quelle |
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| Tazi A |
Adult pulmonary Langerhans' cell histiocytosis. |
The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology 2006, 27: 1272 |
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| Adult pulmonary Langerhans' cell histiocytosis is a rare disorder of unknown aetiology that occurs predominantly in young smokers, with an incidence peak at 20-40 yrs of age. In adults, pulmonary involvement with Langerhans' cell histiocytosis usually occurs as a single-system disease and is characterised by focal Langerhans' cell granulomas infiltrating and destroying distal bronchioles. High-resolution computed tomography (HRCT) of the chest is essential to the diagnosis, typically showing a combination of nodules, cavitated nodules, and thick- and thin-walled cysts. A high macrophage count in bronchoalveolar lavage (BAL) fluid is a common but nonspecific finding that merely reflects exposure to tobacco smoke. BAL is useful for eliminating infections and the other infiltrating lung disorders that can be seen in young adults. Langerhans' cells can be identified in BAL fluid, but, in contrast to what was initially hoped, this test shows a very low sensitivity and is rarely useful in the diagnosis of the disease. The definite diagnosis of pulmonary Langerhans' cell histiocytosis requires identification of Langerhans' cell granulomas, which is usually achieved by surgical lung biopsy at a site selected by chest HRCT. In practice, however, lung biopsy is performed on a case-by-case basis. No effective treatment is available to date, and improved understanding of the mechanisms involved in the pathogenesis of pulmonary Langerhans' cell histiocytosis is urgently needed, and should help in the development of specific therapeutic strategies for patients with this orphan disease. |
| Tazi A, Marc K, Dominique S, de Bazelaire C, Crestani B, Chinet T, Israel-Biet D, Cadranel J, Frija J, Lorillon G, Valeyre D, Chevret S |
Serial CT and lung function testing in pulmonary Langerhans cell histiocytosis. |
The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology 2012 Mar 22; |
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| Little is known about longitudinal lung function variation in patients with pulmonary Langerhans cell histiocytosis (LCH). The contribution of serial lung computed tomography (CT) to managing these patients has not been evaluated.This long-term retrospective study included 49 patients who were serially evaluated by lung CT and pulmonary function tests. The lung function variation was categorised as improvement or deterioration. The extent of the CT lesions was correlated with lung function.Lung function deteriorated in approximately 60% of the patients. FEV1 and DL,CO were the parameters that most frequently deteriorated. A subgroup of the patients experienced a dramatic decline in FEV1 within two years after diagnosis. Airway obstruction was the major functional pattern observed. In a multivariate analysis, FEV1% of predicted at diagnosis was the only factor associated with the incidence of airway obstruction. The increase in cystic lesions on the lung CTs was associated with impaired lung function but did not anticipate the FEV1 or the DL,CO decline.Serial lung function tests are essential for following patients with pulmonary LCH, who frequently develop airway obstruction. A lung CT at diagnosis is informative, but routine sequential CTs seem less useful. A prospective study is needed to characterise those patients with early progressive disease. |
| Tazi A, Soler P, Hance AJ |
Adult pulmonary Langerhans' cell histiocytosis. |
Thorax 2000, 55: 405 |
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| Teng CL, Lin TH, Young JH, Chou G, Young CS |
Rapidly fatal Langerhans' cell histiocytosis in an adult. |
Journal of the Formosan Medical Association = Taiwan yi zhi 2005, 104: 955 |
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| Langerhans' cell histiocytosis (LCH) is a disorder of unknown etiology, characterized by abnormal proliferation of cells of the dendritic cell lineage. The acute disseminated form of the disease characteristically occurs in children younger than 3 years of age. More indolent forms of LCH occur primarily in older children and young adults. The prognosis for patients with LCH is good. We report a 28-year-old man with acute disseminated LCH who initially presented with fever, chills, and dry cough. Diagnosis of LCH was confirmed by bronchoalveolar lavage fluid cytology, and skin and bone marrow biopsies. Conventional chemotherapy was not given, because of the patient's poor clinical condition, including respiratory failure, unstable vital signs, and impaired consciousness. Salvage thalidomide therapy was started on the 12th day of hospitalization. The patient died from multisystem LCH with septic shock and multiple organ failure on the 22nd day of hospitalization. This report illustrates the diagnostic features and treatment of LCH in a rapidly fatal case of adult LCH. |
| Toro JR, Pautler SE, Stewart L, Glenn GM, Weinreich M, Toure O, Wei MH, Schmidt LS, Davis L, Zbar B, Choyke P, Steinberg SM, Nguyen DM, Linehan WM |
Lung cysts, spontaneous pneumothorax, and genetic associations in 89 families with Birt-Hogg-Dubé syndrome. |
American journal of respiratory and critical care medicine 2007 May 15; 175: 1044 |
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| Birt-Hogg-Dubé syndrome (BHDS) is an autosomal, dominantly inherited genodermatosis that predisposes to fibrofolliculomas, kidney neoplasms, lung cysts, and spontaneous pneumothorax. |
| Trouillas P, Takayanagi T, Hallett M, Currier RD, Subramony SH, Wessel K, Bryer A, Diener HC, Massaquoi S, Gomez CM, Coutinho P, Ben Hamida M, Campanella G, Filla A, Schut L, Timann D, Honnorat J, Nighoghossian N, Manyam B |
International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome. The Ataxia Neuropharmacology Committee of the World Federation of Neurology. |
Journal of the neurological sciences 1997 Feb 12; 145: 205 |
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| Despite the involvement of cerebellar ataxia in a large variety of conditions and its frequent association with other neurological symptoms, the quantification of the specific core of the cerebellar syndrome is possible and useful in Neurology. Recent studies have shown that cerebellar ataxia might be sensitive to various types of pharmacological agents, but the scales used for assessment were all different. With the long-term goal of double-blind controlled trials-multicentric and international-an ad hoc Committee of the World Federation of Neurology has worked to propose a one-hundred-point semi-quantitative International Cooperative Ataxia Rating Scale (ICARS). The scale proposed involves a compartimentalized quantification of postural and stance disorders, limb ataxia, dysarthria and oculomotor disorders, in order that a subscore concerning these symptoms may be separately studied. The weight of each symptomatologic compartment has been carefully designed. The members of the Committee agreed upon precise definitions of the tests, to minimize interobserver variations. The validation of this scale is in progress. |
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