Hemoglobin (Hb) SD-Los Angeles compound heterozygotes usually have a severe clinical course although the effect of an elevated Hb F on the clinical phenotype has not been reported previously. We describe 5 Kuwaiti children with Hb SD associated with Hb F levels >20%. They all presented with sickling-related symptoms by ≤2 years of age and have been followed for 3–15 years. All had severe clinical courses marked by varying degrees of splenic sequestration crises, acute chest syndrome, vaso-occlusive crises, osteomyelitis and avascular necrosis of the femoral head. This pattern is in contrast with the usually mild presentation in Kuwaiti Hb SS patients with elevated Hb F. It therefore appears that Hb F does not ameliorate the clinical phenotype in Hb SD-Los Angeles. The reasons for this are not quite clear.

Keywords:
Avascular necrosis, Hb F, Hb SD-Los Angeles, Hemoglobin, Sickle cell disease, Vaso-occlusive crises
© 2010 S. Karger AG, Basel
2010
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.