Moyamoya syndrome should be considered as a potential consequence in children with HbE/beta-thalassemia.
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May warrant Moyamoya evaluation in HbE/beta-thalassemia with growth hormone deficiency; single case leaves association and screening open.
Moyamoya syndrome is a rare diagnosis that has been linked to a small number of hemoglobinopathies. Children with Moyamoya syndrome tend to present with transient ischemic attacks, mental deficiency, and/or neurological deficits. We describe a case of a 15-year-old Cambodian male with HbE/beta-thalassemia who was found to have left Moyamoya syndrome as part of an evaluation for growth hormone deficiency. The link between Moyamoya syndrome and HbE/beta-thalassemia may be multifactorial, but Moyamoya syndrome is an important consequence to consider in children with HbE/beta-thalassemia.
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Parker et al. (2008) studied this question.
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