Document Detail

Moyamoya syndrome associated with hemolytic anemia due to Hb Alesha.
MedLine Citation:
PMID:  16096527     Owner:  NLM     Status:  MEDLINE    
Moyamoya angiopathy is a well-known complication of sickle cell disease but has rarely been observed in other hemoglobinopathies. The authors describe a previously unreported association of hemolytic anemia due to a rare unstable hemoglobinopathy with abnormal oxygen affinity (Hb Alesha) and moyamoya syndrome in a 10-year-old girl. At age 4 she had recurrent migraine-with-aura-like symptoms. Cranial MRI, Doppler, and EEG examinations were not conclusive. Deterioration of her neurologic symptoms prompted a renewed EEG examination at 10 years of age, which revealed a re-buildup phenomenon. MRI and MR angiography now showed moyamoya angiopathy with stenotic and occlusive lesions of both internal carotid and middle cerebral arteries. Conventional angiography confirmed these findings. Reperfusion with three extra-intracranial bypasses terminated the transient ischemic attacks. The authors suggest that chronic hypoxemia may be the cause of occlusive moyamoya angiopathy in Hb Alesha and possibly other unstable hemoglobinopathies with altered oxygen affinity.
Knut Brockmann; Stephan Stolpe; Carsten Fels; Nadia Khan; Andreas E Kulozik; Arnulf Pekrun
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of pediatric hematology/oncology     Volume:  27     ISSN:  1077-4114     ISO Abbreviation:  J. Pediatr. Hematol. Oncol.     Publication Date:  2005 Aug 
Date Detail:
Created Date:  2005-08-12     Completed Date:  2005-09-20     Revised Date:  2011-10-06    
Medline Journal Info:
Nlm Unique ID:  9505928     Medline TA:  J Pediatr Hematol Oncol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  436-40     Citation Subset:  IM    
Department of Pediatrics and Neuropediatrics, University Hospital Göttingen, Göttingen, Germany.
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MeSH Terms
Anemia, Hemolytic / complications*,  etiology*
Cognition Disorders / etiology
Hemoglobins, Abnormal*
Hypoxia, Brain / complications,  etiology
Magnetic Resonance Angiography
Magnetic Resonance Imaging
Moyamoya Disease / etiology*,  pathology
Reg. No./Substance:
0/Hemoglobins, Abnormal; 149531-76-0/hemoglobin Alesha

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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