[Follow-up strategies for lung transplant recipients in France]. / Suivi partagé des patients transplantés pulmonaires.

BACKGROUND: Lung transplantation (LT) requires sustained care for a frequently polypathological condition. Follow-up is focused on three main issues: 1/stability of respiratory function; 2/comorbidity management; 3/preventive medicine. About 3000 LT patients in France are treated in 11 LT centers. Given the increased size of the LT recipient cohort, follow-up might be partially shared with peripheral centers. METHODS: This paper presents the suggestions of a working group of the SPLF (French-speaking respiratory medicine society) on possible modalities of shared follow-up. RESULTS: While the main LT center is tasked with centralizing follow-up, particularly the choice of optimal immunosuppression, an identified peripheral center (PC) may serve as an alternative to deal with acute events, comorbidities and routine assessment. Communication between the different centers should be free-flowing. Shared follow-up may be offered from the 3rd postoperative year to stable and consenting patients, whereas unstable and non-observant patients are poor candidates. CONCLUSION: These guidelines may serve as a reference for any pneumologist wishing to effectively contribute to follow-up, even and especially subsequent to lung transplant.

[Secondary pulmonary alveolar proteinosis in a transplant patient]. / Protéinose pulmonaire secondaire chez un patient greffé.

INTRODUCTION: Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by alveolar accumulation of lipoproteinaceous material, caused by a macrophagic clearance disorder. We present a case of PAP in a patient taking the immunosuppressant drug mycophenolate mofetil (MMF) in the context of invasive pulmonary aspergillosis, of which we discuss the pathophysiology and treatment as reported in the literature. CASE REPORT: A 43-year-old man with cardiomyopathy received a heart transplant and was treated by MMF, tacrolimus and corticosteroids. Three months after the transplant, he presented with acute oxygen-dependent respiratory failure. The diagnosis of PAP seemed likely on the CT scan and was confirmed by bronchoalveolar lavage, as was the diagnostic of invasive pulmonary aspergillosis (IPA). However, GM-CSF autoantibodies were not found. As there existed a suspicion of MMF imputability, the treatment was discontinued and an antifungal treatment was started. The patient was reassessed one month after discontinuation of MMF and found to have clinically and radiologically improved. Four other cases of MMF-induced PAP have been reported in the literature. CONCLUSIONS: MMF and IPA could be predisposing cofactors for the occurrence of secondary PAP.

Evaluation of a flexible bronchoscope prototype designed for bronchoscopy during mechanical ventilation: a proof-of-concept study.

Bronchoscopy during mechanical ventilation of patients' lungs significantly affects ventilation because of partial obstruction of the tracheal tube, and may thus be omitted in the most severely ill patients. It has not previously been possible to reduce the external diameter of the bronchoscope without reducing the diameter of the suction channel, thus reducing the suctioning capacity of the device. We believed that a better-designed bronchoscope could improve the safety of bronchoscopy in patients whose lungs were ventilated. We designed a flexible bronchoscope prototype with a drumstick-shaped head consisting of a long, thin proximal portion; a short and large distal portion for camera docking; and a large suction channel throughout the length of the device. The aims of our study were to test the impact of our prototype on mechanical ventilation when inserted into the tracheal tube, and to assess suctioning capacity. We first tested the efficiency of the suction channel, and demonstrated that the suction flow of the prototype was similar to that of conventional adult bronchoscopes. We next evaluated the consequences of bronchoscopy when using the prototype on minute ventilation and intrathoracic pressures during mechanical ventilation: firstly, in vitro using a breathing simulator; and secondly, in vivo using a porcine model of pulmonary ventilation. The insertion of adult bronchoscopes into the tracheal tube immediately impaired the protective ventilation strategy employed, whereas the prototype preserved it. For the first time, we have developed an innovative flexible bronchoscope designed for bronchoscopy during invasive mechanical ventilation, that both preserved the protective ventilation strategy, and enabled efficient suction flow.

[Ecchymosis as the presenting manifestation of Wilson disease: A case report]. / Ecchymoses diffuses révélant une maladie de Wilson : à propos d'un cas.

INTRODUCTION: The presence of a psychiatric disorder during the course of an organic disease is a common cause of delayed diagnosis. CASE REPORT: We report a 16-year-old girl who was admitted with thrombocytopenia and had a two-year history of neuropsychiatric disorder which was attributed to a difficult family situation. Neurological examination showed a frontal lobe disorder and extrapyramidal manifestations. The thrombocytopenia was attributed to liver cirrhosis. These neurological and hepatic injuries were consistent with a metabolic disease, most likely Wilson disease. This was supported by the presence of a corneal Kayser-Fleischer ring and a decreased ceruloplasmin concentration. An urgent liver transplantation was required due to worsening neurological symptoms. CONCLUSION: Wilson disease is a rare genetic disease caused by copper toxicity. It is characterized by combined hepatic and neurologic damage to varying degrees and can develop at any age. Urgent treatment is required, but the diagnosis may be delayed by prevailing psychiatric symptoms.

The effect of fibreoptic bronchoscopy in acute respiratory distress syndrome: experimental evidence from a lung model.

Flexible bronchoscopy is essential for appropriate care during mechanical ventilation, but can significantly affect mechanical ventilation of the lungs, particularly for patients with acute respiratory distress syndrome. We aimed to describe the consequences of bronchoscopy during lung-protective ventilation in a bench study, and thereby to determine the optimal diameter of the bronchoscope for avoiding disruption of the protective-ventilation strategy during the procedure. Immediately following the insertion of the bronchoscope into the tracheal tube, either minute ventilation decreased significantly, or positive end-expiratory pressure increased substantially, according to the setting of the inspiratory pressure limit. The increase in end-expiratory pressure led to an equivalent increase in the plateau pressure, and lung-protective ventilation was significantly altered during the procedure. We showed that a bronchoscope with an external diameter of 4 mm (or less) would allow safer bronchoscopic interventions in patients with severe acute respiratory distress syndrome.

An evaluation of a new single-use flexible bronchoscope with a large suction channel: reliability of bronchoalveolar lavage in ventilated piglets and initial clinical experience.

A single-use flexible bronchoscope with a large suction channel has become available recently and we have evaluated this innovative device. Firstly, bronchoalveolar lavage was performed and quantified in ventilated piglets. Next, the bronchoscope was evaluated in three intensive care units and a satisfaction questionnaire was carried out. Sixteen bronchoalveolar lavages were performed in piglets with a recovery rate of 83 (79-86 [72-89])% of the instilled volume. Quality and performance of all devices tested was identical. The medical satisfaction questionnaire was as follows: 'acceptable' to 'very good' for quality of aspiration, manoeuvrability and quality of vision; 'very good' to 'perfect' for setting up and insertion. This encouraging preliminary evaluation demonstrates the effectiveness of this new single-use device, which may obviate the need for disinfection procedures and, thereby, eradicate a potential vector of patient cross-contamination.

Initial characteristics and outcome of hospitalized patients with amiodarone pulmonary toxicity.

UNLABELLED: Amiodarone-induced pulmonary toxicity (APT) is a serious adverse event that can lead to death. The aims of our study are to determine factors associated with mortality and to describe outcome and sequelae of patients with APT. METHODS: Forty-six patients with APT were divided into two groups according to survival at day 90 for a clinical, functional, biological and radiological comparaison. We then evaluated the evolution of 15 survivors at a median of three months [1-6 months] and/or 12 months [8-36 months]. RESULTS: Mortality of APT at day 90 was 37% (17 patients) and was linked to the speed of onset of symptoms and a high HRCT alveolar score. Angiotensin system antagonist treatment was prescribed significantly more in the survival group (p = 0.042, HR 0.34 (95% CI 0.12-0.96)). In surviving patients, dyspnea, vital capacity and HRCT alveolar score improved significantly while HRCT fibrosis score deteriorated gradually during the first six months. At the end of the study, all the surviving patients presented functional and/or radiological sequelae. CONCLUSIONS: Severity of APT is linked to the extent and speed of onset of pulmonary damage. After the initial episode, the patients who survived improved slowly but with persistent sequelae.

Pulmonary toxicity associated with the use of lenalidomide: case report of late-onset acute respiratory distress syndrome and literature review.

Lenalidomide is an immunomodulating drug structurally similar to thalidomide. It is indicated for patients with relapsing or refractory multiple myeloma in combination with dexamethasone, and for patients with myelodysplastic syndromes associated with a deletion 5q cytogenetic abnormality. It is also used to treat other myelodysplastic syndromes such as myelofibrosis and lymphoma. We report a case of organizing pneumonia leading to acute respiratory distress syndrome (ARDS) after long-term administration of lenalidomide, along with a review of the literature.

[Respimat®, first Soft Mist™ inhaler: new perspectives in the management of COPD]. / Respimat(®), premier inhalateur « Soft Mist ¼ : intérêt dans la prise en charge de la BPCO.

INTRODUCTION: In COPD, inhaler choice should be based on the likelihood that the patient will be able to use the device correctly in order to favour adherence and compliance, and therefore treatment efficacy. SATE OF THE ART: Performances of usual inhalers are limited by the necessity of a good coordination of patient inspiration and inhaler activation (pressurized metered dose inhalers), or a sufficient inspiratory flow (dry powder inhalers). Respimat®, the first "Soft Mist™ inhaler" (SMI), releases the drug solution as a low and sustained soft mist, so that lung deposition is both improved and reproducible. PERSPECTIVES: In clinical studies, Respimat® has been shown to allow equivalent bronchodilator response and tolerability to metered dose or dry powder inhalers, but with lower doses of active drugs. Furthermore, studies assessing inhaler preference in COPD showed that patients preferred Respimat® to usual inhalers. CONCLUSION: Respimat® SMI offers new perspectives for the management of chronic respiratory diseases, particularly in newly diagnosed or poorly compliant patients.