Phenotypes and outcome of diffuse pulmonary non-amyloid light chain deposition disease

François Lestelle, Catherine Beigelman,David Rotzinger, Salim Si-Mohamed,Mouhamad Nasser, Lidwine Wemeau,Sandrine Hirschi, Grégoire Prevot,Antoine Roux, Vincent Bunel, Emmanuel Gomez, Laurent Sohier, Helene Morisse Pradier,Martine Reynaud Gaubert, Anne Gondouin, Romain Lazor,Jean-Charles Glerant, Françoise Thivolet Bejui, Magali Colombat,Vincent Cottin

Respiratory Research（2024）

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摘要

Light chain deposition disease (LCDD) is a very rare entity. Clinical manifestations of LCDD vary according to the organs involved. Data on pulmonary LCDD are scarce and limited to small series or case reports. This study aimed to describe the characteristics and outcome of diffuse pulmonary non-amyloid LCDD localized to the lungs. A multicenter retrospective cohort study was conducted. Clinical characteristics were collected, and chest CTs were centrally reviewed. The diagnosis of pulmonary non-amyloid LCDD was confirmed by immunohistochemistry. Thirty-one cases were identified (68

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Light chain deposition disease,Lung cysts,Bronchiectasis,Lung transplantation

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