Postgraduate medical journal
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Lymphocyte adenosine deaminase (L-ADA) activity, a measure of lymphocyte activity, was estimated in 10 healthy controls and 30 patients with typhoid fever (20 uncomplicated and 10 complicated) at the time of admission, at onset of complications and weekly until recovery. Mean L-ADA activity in healthy controls was 20.49 +/- 3.62 mU/10(6) cells. In uncomplicated patients L-ADA activity was 36.33 +/- 5.09 mU/10(6) cells at time of admission, which is significantly raised as compared to controls. ⋯ Serial L-ADA activity can be of prognostic significance. A cut-off value of 24 mU/10(6) cells is suggested to predict prognosis and severity of disease. Activity below this indicates a probability of a severe, prolonged course and may help in instituting early and energetic treatment.
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The case is reported of a young woman with the Maroteaux-Lamy syndrome (mucopolysaccharidosis type VI) who presented with rapidly progressive dyspnoea due to mitral stenosis. Mitral valve replacement was performed and the appearance of the valve was typical of mucopolysaccharide infiltration. Dyspnoea in patients with the Maroteaux-Lamy syndrome may be due primarily to cardiac valve involvement, and in this setting, valve surgery is safe and effective.