Archives of disease in childhood
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To review the outcome of patients with childhood malignancy requiring intensive care treatment and to assess whether there is any secular trend for improved outcome. ⋯ Compared with previous series, there has been a great improvement in survival of oncology patients admitted to the intensive care unit especially those with either systemic or respiratory infection needing ventilation. Full intensive care treatment should be provided for these patients.
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Feeding difficulties were assessed in 14 children (age range 2-14 years) with merosin deficient congenital muscular dystrophy, a disease characterised by severe muscle weakness and inability to achieve independent ambulation. Twelve of the 14 children were below the 3rd centile for weight. On questioning, all parents thought their child had difficulty chewing, 12 families modified the diet, and 13 children took at least 30 minutes to complete a meal. ⋯ This study shows that children with merosin deficient congenital muscular dystrophy have difficulties at all stages of feeding that progress with age. Appropriate intervention can improve weight gain and reduce chest infections. The severity of the problem has not been previously appreciated in this disease, and the study shows the importance of considering the nutritional status in any child with a primary muscle disorder.
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The design of childhood injury prevention programmes is hindered by a dearth of valid and reliable information on injury frequency, cause, and outcome. A number of local injury surveillance systems have been developed to address this issue. One example is CHIRPP (Canadian Hospitals Injury Reporting and Prevention Program), which has been imported into the accident and emergency department at the Royal Hospital for Sick Children, Glasgow. This paper examines a year of CHIRPP data. ⋯ There are several limitations to the current CHIRPP system in Glasgow: it is not population based, only injuries presented to the accident and emergency department are included, and injury severity is not recorded. Nevertheless, CHIRPP is a valuable source of information on patterns of childhood injury. It offers local professionals a comprehensive dataset that may be used to develop, implement, and evaluate child injury prevention activities.
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This paper describes a Delphi study used to identify and improve areas of concern in the planning of care for children in major incidents. The Delphi was conducted over three rounds and used a multidisciplinary panel of 22 experts. Experts were selected to include major incident, immediate care, emergency medicine, and paediatric specialists. ⋯ Paediatric services may play a vital role in the preparation and response to a major incident involving children. This paper represents a consensus view on how best to plan and respond to major incidents involving children. An accompanying paper describes the practical implementation of this guidance.
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Rett syndrome is a severe neurodevelopmental disorder of unknown aetiology. A prolonged QT interval has been described previously in patients with Rett syndrome. To investigate QT prolongation and the presence of cardiac tachyarrhythmias in Rett syndrome electrocardiography and 24 hour Holter monitoring were performed prospectively in a cohort of 34 girls with Rett syndrome. ⋯ Compared with a group of healthy controls of a similar age range, the patients with Rett syndrome had significantly longer corrected QT values. Clinical severity was not a predictor for prolonged QT intervals in the Rett syndrome cohort. The prolonged QT syndrome is a serious and potentially lethal cardiac disorder and should be considered in all girls with Rett syndrome.