Paediatric respiratory reviews
-
Airway clearance consists of two linked processes: mucociliary clearance and cough clearance. Patients with neuromuscular weakness are at risk for impaired cough clearance and therefore the development of pneumonia and atelectasis. Aiding airway clearance in the patient with neuromuscular weakness is critical to the maintenance of health and the prevention of significant respiratory morbidity. ⋯ This review will discuss the physiology of cough and the mechanics of aiding cough clearance in the patient with neuromuscular weakness. In addition, technologies and techniques used to improve mucociliary clearance will also be discussed. Newer technologies such as mechanical insufflation-exsufflation have gained widespread acceptance in the management of airway clearance in the patient with neuromuscular weakness.
-
Paediatr Respir Rev · Mar 2010
Sleep, sleep disordered breathing, and nocturnal hypoventilation in children with neuromuscular diseases.
Sleep disordered breathing (SDB) is now well recognized in children with neuromuscular diseases (NMD) and may lead to significant morbidity and increased mortality. Predisposing factors to SDB in children with NMD include reduced ventilatory responses, reduced activity of respiratory muscles during sleep and poor lung mechanics due to the underlying neuro-muscular disorder. SDB may present long before signs of respiratory failure emerge. ⋯ Polysomnography is the preferred method to evaluate for SDB in children with NMD. When the diagnosis of SDB is confirmed, treatment by non-invasive ventilation (NIV) is usually recommended. However, other modalities of mechanical ventilation do exist and may be indicated in combination with or without other supportive measures.
-
Subjects with progressive neuromuscular diseases undergo a typical sequence of respiratory compromise, leading from normal unassisted gas exchange to nocturnal hypoventilation with normal daytime gas exchange, and eventually to respiratory failure requiring continuous ventilatory support. Several different abnormalities in respiratory pump function have been described to explain the development of respiratory failure in subjects with neuromuscular weakness. ⋯ The likely beneficial effects of mechanical ventilatory support include resting fatigue-prone respiratory muscles and resetting of the central chemoreceptors to PaCO(2). Recent experience shows that select patients who require daytime ventilation can be supported with non-invasive ventilation continuously to correct gas exchange abnormalities while avoiding detrimental aspects of tracheostomy placement.
-
Paracoccidioidomycosis is a systemic fungal infection caused by Paracoccidoides brasiliensis. The infection is endemic in subtropical areas of Latin America and has a high prevalence in Brazil. The disease is acquired by airborne inhalation of conidia and is frequently observed in adult male rural workers. ⋯ Sulfonamides, ketoconazole, itraconazole, fluconazole and amphotericin B have been successfully used in the treatment of paracoccidioidomycosis. Itraconazole is the treatment of choice, being effective in more than 95% of cases. Co-trimoxazole is still frequently used especially in chronic progressive disease and as maintenance after a course of amphotericin B in severe cases of this mycosis.