Stroke in Williams syndrome.

BACKGROUND: Williams syndrome is a genetic disorder characterized by a high incidence of heart disease, arterial stenosis, and hypertension. Despite these features, cerebrovascular accidents have been described only recently and only in association with stenoses of the cerebral vasculature. CASE DESCRIPTION: A 19-year-old girl with Williams syndrome developed an acute-onset hemiparesis. MRI demonstrated an infarct involving the internal capsule and putamen. No stenotic areas were seen on angiography. CONCLUSIONS: Stroke should be considered as a possible consequence of Williams syndrome, even in the absence of stenoses of the cerebral vasculature. Comparison of this case with those previously reported in the literature emphasizes the multiplicity of features in Williams syndrome that can contribute to the risk of stroke.

Theophylline does not shorten hospital stay for children admitted for asthma.

OBJECTIVE: To determine if the use of intravenous theophylline, in the form of aminophylline, when added to systemic corticosteroids and aerosolized beta 2-agonists, enhances the improvement of children with acute asthma exacerbations. DESIGN: A double-blind, placebo-controlled, randomized, clinical trial. SETTING: The University of Maryland Medical Center, Baltimore, an urban primary- and tertiary-care pediatric medical center. PATIENTS: Forty-two children, aged 2 to 18 years, admitted to the hospital for acute exacerbations of asthma. METHODS: Patients were randomized to receive either intravenous theophylline to maintain a serum level greater than 55 mumol/L or a placebo infusion. All patients received methylprednisolone and nebulized albuterol. A clinical severity score was assessed twice daily. RESULTS: The mean length of stay for the treatment and control groups was 52.3 +/- 32.3 hours and 48.2 +/- 26.6 hours, respectively (t = 0.45, P = .65). The rate of improvement of clinical scores was similar. CONCLUSION: These data suggest that the addition of theophylline to albuterol and corticosteroids does not enhance improvement of children admitted to the hospital with asthma.