PEDIATRICS Vol. 45 No. 1 January 1970, pp. 60-70
This Article
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Fabia, J.
Right arrow Articles by Drolette, M.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Fabia, J.
Right arrow Articles by Drolette, M.

MALFORMATIONS AND LEUKEMIA IN CHILDREN WITH DOWN'S SYNDROME

Jacqueline Fabia M.D.1 and Margaret Drolette Ph.D1

1 Departments of Epidemiology and Biostatistics, Harvard School of Public health, Boston, and the International Children's Centre, Paris

Among 2,421 children with Down's syndrome born alive to residents of Massachusetts from 1950 through 1966, approximately one-third had at least one malformation other than the characteristic diagnostic criteria of the syndrome itself. After congenital heart defect, the most common were duodenal obstruction, clubfoot, cataract, imperforate anus, syndactyly or webbed fingers and/or toes, cleft palate and/or lip, and congenital megacolon.

Males predominated among children with cataract, syndactyly, and congenital megacolon. Somewhat more females than males had congenital heart defect, although the excess was not significant. The mean maternal age was strikingly low for mongols with cleft palate and/or lip and for males with congenital megacolon. The mean birth weight was significantly lower for mongols with malformations, due primarily to a low birth weight of the children with duodenal obstruction.

Most malformations occurred more often in combination with other malformations than expected by chance. The exceptions were duodenal obstruction, congenital megacolon, and imperforate anus.

Among 23 mongols with leukemia, 5 died in the neonatal period—a very high age-specific leukemia mortality rate. The sex distribution of cases shifted over time from predominantly males to predominantly females. Leukemic mongols weighed somewhat more at birth than other mongols, although not significantly so. The risk of leukemia among mongols was not related to the presence or absence of congenital malformations.

Submitted on July 31, 1969
Accepted on October 7, 1969




This article has been cited by other articles:


Home page
 J. Med. Genet.Home page
R H Scott, C A Stiller, L Walker, and N Rahman
Syndromes and constitutional chromosomal abnormalities associated with Wilms tumour
J. Med. Genet., September 1, 2006; 43(9): 705 - 715.
[Abstract] [Full Text] [PDF]

Home page
 BloodHome page
M. Ahmed, A. Sternberg, G. Hall, A. Thomas, O. Smith, A. O'Marcaigh, R. Wynn, R. Stevens, M. Addison, D. King, et al.
Natural history of GATA1 mutations in Down syndrome
Blood, April 1, 2004; 103(7): 2480 - 2489.
[Abstract] [Full Text] [PDF]

Home page
 CLIN PEDIATRHome page
P. Vesterhus and P.J. Moe
Bone Marrow Dysfunction in a Newborn Twin with Down's Syndrome: Report of a Transitory Leukemia-Like Blood Reaction
Clinical Pediatrics, April 1, 1974; 13(4): 347 - 349.
[PDF]

Home page
 CLIN PEDIATRHome page
T. Kushnick
Immunologic Interreactions in Neoplastic Diseases: A Brief Review
Clinical Pediatrics, July 1, 1971; 10(7): 372 - 376.
[PDF]