Paediatric respiratory reviews
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Paediatr Respir Rev · Mar 2010
ReviewCardiac manifestations of neuromuscular disorders in children.
Cardiac abnormalities occur in association with many of the neuromuscular disorders that present in childhood. Genetic defects involving the cytoskeleton, nuclear membrane, and mitochondrial function have all been described in patients with skeletal myopathy and cardiac involvement. The most common classes of neuromuscular disorders with cardiac manifestations are the muscular dystrophies- Duchenne, Becker, limb-girdle and Emery Dreifuss. ⋯ The most common cardiac findings include dilated or hypertrophic cardiomyopathy, atrioventricular conduction defects, atrial fibrillation and ventricular arrhythmias. Screening for cardiac involvement should be performed in all children with neuromuscular disorders that have the potential for cardiac involvement. This review discusses the cardiac findings associated with specific neuromuscular disorders and outlines the indications for evaluation and treatment.
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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.
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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.
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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.