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Characteristics and outcomes of patients with LAM receiving sirolimus in France based on real-life data.

Front Med (Lausanne)

January 2025

Department of Respiratory Medicine, Reference Centre for Rare Pulmonary Diseases, APHP, Hôpital Avicenne, INSERM U 1272, Université Sorbonne Paris-Nord, Bobigny, France.

LAM is a rare multi-cystic lung disease for which treatment with sirolimus is indicated in cases of moderate or severe lung disease or declining lung function. The aim of this study was to describe patients treated with sirolimus for LAM and their outcomes. This retrospective observational study was based on data from the French national health insurance data system (SNDS).

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Birt-Hogg-Dubé syndrome (BHDS) is a rare autosomal dominant genetic disorder. This case report aims to increase awareness of pulmonary cystic lesions and BHDS in China by providing insights into the clinical features of this syndrome. We present two cases of BHDS from the same family.

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Background: Bedside thoracic ultrasound (US) offers numerous advantages over chest X-ray (CXR) for identification of recurrent pneumothoraces (PTX) after tube thoracostomy (TT) removal. Technologic advancements have led to the development of hand-held devices capable of producing high-quality images termed ultra-portable US (UPUS). We hypothesized that UPUS would be as successful as CXR in detecting post-TT removal PTX and would be preferred by patients.

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Background: Iatrogenic pneumothorax is a common complication of diagnostic and therapeutic pulmonary procedures. New guidelines on primary spontaneous pneumothorax suggest ambulatory approaches may be suitable. However, guidance on iatrogenic pneumothorax occurring in patients with impaired lung function, increased age, comorbidity and frailty is lacking, and the safety profile of ambulatory management is not known.

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