[Central control malfunction in Wolfram syndrome: Report of one case of difficult weaning]. / Dysfonction de la commande centrale dans le syndrome de Wolfram : à propos d'un cas de sevrage ventilatoire difficile.

INTRODUCTION: Wolfram syndrome is a rare autosomal recessive genetic disease characterized by diabetes insipidus, diabetes mellitus, optic atrophy and deafness. A neurodegenerative syndrome is usually associated, including cerebellar ataxia. A few cases of central apnea have been reported in the literature. Here, we report a case of Wolfram syndrome with respiratory symptoms that led to the discovery of central apneas as well as complicated and delayed weaning in an intensive care unit (ICU). OBSERVATION: The patient is a 39-year-old woman diagnosed with Wolfram syndrome who was admitted to an ICU for septic shock. She experienced difficult weaning before central apneas were observed while spontaneous ventilation was being attempted. After two extubation failures, cerebral MRI was performed and revealed parenchymatous atrophy of the posterior brain fossa involving the cerebral trunk, cerebellar peduncles, as well as both cerebellum hemispheres and the cerebellar vermis. Even after the patient was tracheotomized, central apneas persisted when the patient breathed spontaneously with her tracheotomy, necessitating nocturnal ventilation. CONCLUSION: While central apneas Wolfram syndrome remain rare, they should be systematically investigated due to their association with severe morbimortality.

[Noninvasive ventilation. The 2015 guidelines from the Groupe Assistance Ventilatoire (GAV) of the Société de Pneumologie de Langue Française (SPLF)]. / Ventilation non invasive. Les conseils 2015 du Groupe assistance ventilatoire (GAV) de la Société de pneumologie de langue française (SPLF).

A task force issued from the Groupe Assistance Ventilatoire (GAV) of the Société de Pneumologie de Langue Française (SPLF) was committed to develop a series of expert advice concerning various practical topics related to long-term non invasive ventilation by applying the Choosing Wisely® methodology. Three topics were selected: monitoring of noninvasive ventilation, the interpretation of data obtained from built-in devices coupled to home ventilators and the role of hybrid modes (target volume with variable pressure support. For each topic, the experts have developed practical tips based on a comprehensive analysis of recent insights and evidence from the literature and from clinical experience.

[Necrotizing pneumonia due to Staphylococcus aureus producing Panton-Valentine toxin]. / Pneumonie nécrosante à Staphylococcus aureus secréteur de la toxine de panton valentine.

BACKGROUND: Necrotizing pneumonia caused by Staphylococcus aureus producing Panton Valentine Leukocidine (PVL) has been described recently and is associated with a high mortality (75%). OBSERVATION: We report a case of pneumonia due to PV secreting Staphylococcus aureus in a healthy young adult, complicated by multiple necrotizing lung lesions and major cystic changes, with a favourable final outcome. Acute respiratory failure with haemodynamic failure and ARDS developed a few days after an influenza-like illness. The appearances at fibreoptic bronchoscopy were atypical, consisting of a purulent, necrotic tracheo- bronchitis with desquamation. The initial unfavourable progress despite broad spectrum antibiotic therapy and, finally, the identification of PV leukotoxin in blood cultures and tracheal aspirate, led to the diagnosis. During the clinical course there were repeated pneumothoraces (8 drains) due to multiple bilateral cystic lesions. Ultimately progress was favourable following treatment with antibiotics (flucloxacillin and clindamycin) and steroids. CONCLUSION: It is important to consider pulmonary complications of the Panton-Valentine leukotoxin in a healthy young adult who presents with necrotizing pneumonia and an unfavourable outcome in spite of broad spectrum antibiotics. Treatment is difficult on account of the extent of the necrotizing lesions and the need to use antibiotics effective against both the bacterium and its toxin.

Fatal haemoptysis in a case of lymphomatoid granulomatosis treated with rituximab.

Lymphomatoid granulomatosis is a rare angiocentric and angiodestructive disease, which commonly involves the lungs but also the brain, kidneys, liver and skin. This report describes the case of a 33-yr-old female with an aggressive form of lymphoid granulomatosis treated with an anti-CD20 antibody. Dramatic radiological improvement was seen at the fourth week. However, the patient died at home 1 month after the last rituximab administration from a massive haemoptysis.

[Keratinocyte growth factor and Hepatocyte growth factor: their roles in alveolar epithelial repair]. / Keratinocyte growth factor et Hepatocyte growth factor: rôles dans la réparation épithéliale alvéolaire.

INTRODUCTION: The alveolar epithelium is the principal target in the course of acute lung injury (ALI) and the acute respiratory distress syndrome (ARDS) and to a lesser extent in chronic reactions like pulmonary fibrosis. STATE OF ART: Keratinocyte growth factor (KGF) and Hepatocyte growth factor (HGF) are potent growth factors for type II pneumocytes and seem to play a specific role in the process of alveolar repair. PERSPECTIVES: The studies conducted by our group have demonstrated 1) that KGF and HGF are present in biologically active concentrations in human pulmonary alveoli in ALI and ARDS, 2) that in these patients as well as those with pulmonary fibrosis circulating neutrophils are an important source of HGF. HGF and KGF act within a system involving other factors such as parathyroid hormone related protein (PTHrP). PTHrP acts in an autocrine and paracrine manner to regulate the balance between proliferation and apoptosis of alveolar epithelial cells. CONCLUSIONS: Our results obtained in humans suggest a beneficial role for neutrophils in the alveolar repair after acute or chronic lung injury. The experimental data suggest that use of KGF and HGF might be considered in the future in the treatment of human acute or chronic lung injury.