Increased Prevalence of Immunoglobulin A Deficiency in Patients with the Chromosome 22q11.2 Deletion Syndrome (DiGeorge Syndrome/Velocardiofacial Syndrome)

This Article

	Full Text
	Full Text (PDF)
	Alert me when this article is cited
	Alert me if a correction is posted
	Citation Map

Services

	E-mail this article to a friend
	Similar articles in this journal
	Similar articles in PubMed
	Alert me to new issues of the journal
	Download to citation manager
	Reprints and Permissions
	Copyright Information
	Books from ASM Press
	MicrobeWorld

Citing Articles

	Citing Articles via HighWire
	Citing Articles via Google Scholar

Google Scholar

	Articles by Smith, C. A.
	Articles by Sullivan, K. E.
	Search for Related Content

PubMed

	PubMed Citation
	Articles by Smith, C. A.
	Articles by Sullivan, K. E.

Increased Prevalence of Immunoglobulin A Deficiency in Patients with the Chromosome 22q11.2 Deletion Syndrome (DiGeorge Syndrome/Velocardiofacial Syndrome)

Christopher A. Smith,¹ Deborah A. Driscoll,² Beverly S. Emanuel,³ Donna M. McDonald-McGinn,³ Elaine H. Zackai,³ and Kathleen E. Sullivan¹^,^*

Division of Immunologic and Infectious Diseases¹ and Division of Human Genetics and Molecular Biology,³ The Children's Hospital of Philadelphia, and Department of Obstetrics and Gynecology, The University of Pennsylvania School of Medicine,² Philadelphia, Pennsylvania

Received 24 October 1997/Returned for modification 26 December 1997/Accepted 2 March 1998

We wished to determine the prevalence of immunoglobulin A (IgA) deficiency in patients with the chromosome 22q11.2 deletionsyndrome. A total of 32 patients with the chromosome 22q11.2 deletionwere examined for IgA deficiency. We report a 13% (n = 4) prevalenceof IgA deficiency in patients with this syndrome. The odds ratioof IgA deficiency in this population is 14.20 (P < 0.0001). Thisconfirms the occurrence of significant humoral deficits in thispredominantly cellular immunodeficiency.

^* Corresponding author. Mailing address: Division of Immunologic and Infectious Diseases, The Children's Hospital of Philadelphia,34th St. and Civic Center Blvd., Philadelphia, PA 19104. Phone:(215) 590-4685. Fax: (215) 590-3044. E-mail: sullivak{at}mail.med.upenn.edu.

This article has been cited by other articles:

Piliero, L. M., Sanford, A. N., McDonald-McGinn, D. M., Zackai, E. H., Sullivan, K. E. (2004). T-cell homeostasis in humans with thymic hypoplasia due to chromosome 22q11.2 deletion syndrome. Blood 103: 1020-1025 [Abstract] [Full Text]
Gennery, A R, Barge, D, O'Sullivan, J J, Flood, T J, Abinun, M, Cant, A J (2002). Antibody deficiency and autoimmunity in 22q11.2 deletion syndrome. Arch. Dis. Child. 86: 422-425 [Abstract] [Full Text]
Perkins, J. A., Sie, K., Gray, S. (2000). Presence of 22q11 Deletion in Postadenoidectomy Velopharyngeal Insufficiency. Arch Otolaryngol Head Neck Surg 126: 645-648 [Abstract] [Full Text]
Sullivan, K. E., McDonald-McGinn, D., Driscoll, D. A., Emanuel, B. S., Zackai, E. H., Jawad, A. F. (1999). Longitudinal Analysis of Lymphocyte Function and Numbers in the First Year of Life in Chromosome 22q11.2 Deletion Syndrome (DiGeorge Syndrome/Velocardiofacial Syndrome). CVI 6: 906-911 [Abstract] [Full Text]
Vorechovsky, I., Blennow, E., Nordenskjold, M., Webster, A. D. B., Hammarstrom, L. (1999). A Putative Susceptibility Locus on Chromosome 18 Is Not a Major Contributor to Human Selective IgA Deficiency: Evidence from Meiotic Mapping of 83 Multiple-Case Families. J. Immunol. 163: 2236-2242 [Abstract] [Full Text]
Urnovitz, H. B., Tuite, J. J., Higashida, J. M., Murphy, W. H. (1999). RNAs in the Sera of Persian Gulf War Veterans Have Segments Homologous to Chromosome 22q11.2. CVI 6: 330-335 [Abstract] [Full Text]