Nederlands tijdschrift voor geneeskunde
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Ned Tijdschr Geneeskd · Aug 2007
Comment[The silent yet successful revolution of mechanical ventilation in patients with neuromuscular disorders, in particular Duchenne muscular dystrophy].
While developments in molecular biology have been giving rise to breakthroughs in diagnosis, management and promising treatment approaches for neuromuscular disorders, there has been a silent revolution in ventilatory support of patients with a range of neuromuscular disorders with respiratory insufficiency. In a Dutch retrospective study on ventilatory support in 48 boys with Duchenne muscular dystrophy, a 5-year survival rate of 75% was found, with 67% of the patients still living with their relatives. These data are in line with reports from Great Britain. The significant impact of ventilatory support on the natural history of various neuromuscular disorders with respiratory insufficiency is a major breakthrough.
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Ned Tijdschr Geneeskd · Aug 2007
[18 years experience with mechanical ventilation in patients with Duchenne muscular dystrophy].
To find out which patients with Duchenne muscular dystrophy are eligible for starting home mechanical ventilation and what the survival rate is. ⋯ Home mechanical ventilation can be implemented effectively in patients with Duchenne dystrophy, with a 5-year survival of 75%.
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Erythrocytosis is a phenomenon with life-threatening complications and a broad differential diagnosis. Erythrocytosis is usually secondary to a cardiopulmonary condition leading to a low arterial oxygen tension. ⋯ The differential diagnosis can be narrowed down by a determination of the erythropoietin concentration and the JAK2 mutation. If the erythrocytosis is found to be non-physiological, then reduction of the haematocrit via bloodletting and, depending on the diagnosis, treatment with acetylsalicylic acid are indicated.