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Study objectives:
Study objectives To estimate the incidence of pulmonary deterioration in adult pulmonary Langerhans cell histiocytosis To assess the impact of tobacco discontinuation Study Design Multicentric prospective cohort study Main endpoint: Pulmonary deterioration Sample size : 40 patients
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Histocytosis | Adult with isolated pulmonary Langerhans cell histiocytosis (pulmonary LCH) |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Tabacco discontinuation | Behavioral | Tabacco discontinuation |
|
| Measure | Description | Time Frame |
|---|---|---|
| Pulmonary deterioration | decrease in Forced expiratory volume in 1 second (FEV1), vital capacity (VC), or Diffusing capacity of the lung for carbon monoxide (DLCO) of at least 15% as compared to baseline values and/or progression of clinical symptoms (general symptoms) and/or occurrence of pneumothorax | 2 years |
| Measure | Description | Time Frame |
|---|---|---|
| Evolution of pulmonary volumes (FEV1) | 6 months | |
| Evolution of pulmonary lesions in High Resolution Computed Tomography (HRCT) | 6 months |
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Inclusion Criteria:
Exclusion Criteria:
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Adult Pulmonary Langerhans cell histiocytosis
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| Name | Affiliation | Role |
|---|---|---|
| Abdellatif TAZI, MD PhD | Assistance Publique - Hôpitaux de Paris | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hôpital Saint Louis | Paris | Paris | 75010 | France |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 25887097 | Result | Tazi A, de Margerie C, Naccache JM, Fry S, Dominique S, Jouneau S, Lorillon G, Bugnet E, Chiron R, Wallaert B, Valeyre D, Chevret S. The natural history of adult pulmonary Langerhans cell histiocytosis: a prospective multicentre study. Orphanet J Rare Dis. 2015 Mar 14;10:30. doi: 10.1186/s13023-015-0249-2. | |
| 26833097 | Derived |
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| ID | Term |
|---|---|
| D006646 | Histiocytosis, Langerhans-Cell |
| ID | Term |
|---|---|
| D017563 | Lung Diseases, Interstitial |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
| D015614 | Histiocytosis |
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| Tazi A, de Margerie-Mellon C, Vercellino L, Naccache JM, Fry S, Dominique S, Jouneau S, Lorillon G, Bugnet E, Chiron R, Wallaert B, Valeyre D, Chevret S. Extrathoracic investigation in adult patients with isolated pulmonary langerhans cell histiocytosis. Orphanet J Rare Dis. 2016 Feb 2;11:11. doi: 10.1186/s13023-016-0387-1. |
| D008206 |
| Lymphatic Diseases |
| D006425 | Hemic and Lymphatic Diseases |