Published in Revue des maladies respiratoires
Interstitial lung diseases in children of genetic origin. Interstitial lung disease (ILD) in children (chILD) encompasses a heterogeneous group of rare respiratory disorders, most of which are chronic and severe. In more and more of these cases, a genetic cause has been identified. As of now, the main mutations have been localized in the genes enco...
Objectif : le syndrome d’apnées obstructives du sommeil (SAOS) est fréquent chez les patients adultes atteints de pneumopathie interstitielle diffuse (PID). L’objectif de l’étude est d’évaluer la prévalence du SAOS et la qualité du sommeil chez les enfants et les jeunes adultes atteints de PID. Méthodes : une polysomnographie (PSG) a été réalisée c...
INTRODUCTION: Auto-immune pulmonary alveolar proteinosis is a rare disorder characterized by the accumulation of surfactant proteins in the alveoli. CASE REPORT: We report a case of a 41-year-old smoker, presenting initially with acute respiratory failure. Whole lung lavages were effective initially but only for a few weeks. GM-CSF subcutaneous inj...
Published in Revue des maladies respiratoires
Alveolar proteinosis (AP) is a rare disease characterized by alveolar accumulation of surfactant components, which impairs gas exchange. AP is classified into three groups: auto-immune AP defined by the presence of plasma autoantibodies anti-GM-CSF, the most frequent form (90% of all AP); secondary AP, mainly occurring as a consequence of haematolo...
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Contexte : L intolérance aux protéines dibasiques (IPD) est une maladie rare, autosomique récessive, due à un défaut de transport des acides aminés dibasiques. Elle responsable d une atteinte multisystémique mais son pronostic dépend essentiellement de l atteinte respiratoire, dont la physiopathologie est encore mal comprise et dont l évolution est...
REIMS-BU Santé (514542104) / Sudoc / PARIS-BIUM (751062103) / Sudoc / Sudoc / France / FR
RENNES1-BU Santé (352382103) / Sudoc / PARIS-BIUM (751062103) / Sudoc / Sudoc / France / FR
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