Neurological Impairment Induced by Hyperventilation in Children With Sickle Cell Anemia

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Abstract
Case reports of 2 children with SS [sickle cell disease] who developed neurologic impairment during hyperventilation with an EEG were presented. Patients with SS have a significantly greater frequency of CNS disorders than the general population. Sickle Hb causes red cells to sickle when the O2 content of the cell drops below a critical level, resulting in intravascular sludging. Such a hypoxic milieu results from hypocapnic-induced cerebral vasospasm. In addition, the presence of an underlying vasculopathy may set the stage for the development of multiple areas of cerebral ischemia or infarction. The presence of lowered O2 content due to the chronic anemia may potentiate the above mechanisms to a critical degree. The occurrence of transient symptoms may signal the presence of the vasculopathy and marginal cerebral blood flow. Experience with strokes induced by hyperventilation during repose in patients with SS is presented to caution against its use in patients with SS.

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