[Caregivers faced with painful events, testimonials]. / Les soignants face aux événements douloureux, témoignages.

Whatever the profession of caregivers, their activity involves supporting patients and families who are suffering. Doctors and nurses, in particular, talk of the strong emotions which they feel but which they find difficult to express. Being aware of them is essential for being able to accept them and limit their consequences. This article shares different insights.

[Jeune'disease (asphyxiating thoracic dystrophy) and respiratory failure: importance of early respiratory management with periodic hyperinsufflation]. / Maladie de Jeune (dystrophie thoracique asphyxiante) et insuffisance respiratoire: intérêt de la ventilation précoce par hyperinsufflations périodiques.

Asphyxiating thoracic dystrophy (ATD) is a rare autosomal recessive form of chondrodysplasia characterized by short ribs. Respiratory failure is due to the reduced volume and complete immobility of the thoracic cage. There is no consensus on the treatment of this restrictive pulmonary disease. Surgical attempts to enlarge the thoracic cage are disappointing. We report the cases of nine children with ATD treated by periodic respiratory hyperinsufflation. Their clinical outcome was related to the severity of their respiratory distress and their age at the beginning of this treatment. It is possible to use periodic hyperinsufflation very early after birth to prevent secondary respiratory failure. Periodic insufflation can also be used to treat older children with severe restrictive respiratory insufficiency requiring tracheostomy and endotracheal management. This treatment promotes alveolar multiplication and thoracic growth. Four children had laboratory and/or clinical evidence of hepatic dysfunction that improved on ursodeoxycholic acid therapy. Three children who had muscle weakness at birth improved during childhood.