Mortality and Maldevelopment Part II: The Saga of Neural Tube Defect

Congenital malformations are worldwide occurrences striking in every condition of society. These severe physical abnormalities which are present at birth and affecting every part of the body happen more often than usually realized, once in every 33 births

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Mortality and Maldevelopment Part II: The Saga of Neural Tube Defects

Harold Kalter Children’s Hospital Medical Center College of Medicine University of Cincinnati Cincinnati, Ohio, USA

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Author Dr. Harold Kalter 5 Lenox Lane Cincinnati OH 45229 USA [email protected]

ISBN 978-1-4020-9605-1

e-ISBN 978-1-4020-9606-8

Library of Congress Control Number: 2009920466

© 2009 Springer Science+Business Media B.V. No part of this work may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, microfilming, recording or otherwise, without written permission from the Publisher, with the exception of any material supplied specifically for the purpose of being entered and executed on a computer system, for exclusive use by the purchaser of the work. Printed on acid-free paper 9 8 7 6 5 4 3 2 1 springer.com

The Children’s Hospital Research Foundation A great place to work

Contents

1

Introduction: Basic Stuff . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . To Begin with, Infant Death . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Early Death Matters . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Perinatal Mortality . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

1 1 2 2

2

Neural Tube Defects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Neural Tube Defects in History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Normal Neural Tube Development . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Failure of Closure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Defining Neural Tube Defects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Definition Differences . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . A Word about Etiology, etc . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . The Frequency of Neural Tube Defects . . . . . . . . . . . . . . . . . . . . . . . . . . Death and Malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

5 5 7 7 8 10 11 13 15

3

Setting the Stage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Malformations in Early 20th Century USA . . . . . . . . . . . . . . . . . . . . . . .

17 17

4

Studies in the Old World . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . United Kingdom and Ireland . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Liverpool . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Belfast . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Other North Ireland Areas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Dublin . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .