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Previous Volume 335:1806-1810 December 12, 1996 Number 24 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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Severe combined immunodeficiency is a congenital syndrome due to various genetic abnormalities that cause susceptibility to infection, failure to thrive, lymphoid hypoplasia, very low levels of T lymphocytes, and hypogammaglobulinemia.^1,2 Untreated, the disorder is usually fatal within the first year of life. We report the successful treatment of a fetus with the X-linked variant of severe combined immunodeficiency by the in utero transplantation of paternal bone marrow that was enriched with hematopoietic cell progenitors.

Case Report

The patient, 11 months old at this writing, is the second son of a 28-year-old woman known to carry a mutation found in X-linked severe combined . . . [Full Text of this Article]

Methods

Ethical Considerations

Prenatal Genetic Evaluation

Donor-Cell Processing

Injection Procedure

Detection of Donor-Cell Engraftment

Proliferation Assays

Results

Enrichment of Donor Marrow with CD34+ Cells

Engraftment

Hematologic Findings

Cellular Immune Function

Humoral Immune Function

Immunologic Tolerance

Clinical Course

Discussion

Source Information

From the Department of Pediatric Surgery (A.W.F.), the Department of Obstetrics and Gynecology, Center for Molecular Medicine and Genetics (M.I.E., M.P.J.), and the Department of Pediatrics (E.M.A., D.D.H.), Wayne State University, Detroit; the Division of Human Immunology, DNAX, Palo Alto, Calif. (M.-G.R.); the National Center for Human Genome Research, National Institutes of Health, Bethesda, Md. (J.M.P.); and the Department of Medicine, Veterans Affairs Medical Center, University of Nevada, Reno (G.A.-P., E.D.Z.).

Address reprint requests to Dr. Flake at the Department of Surgery, Children's Hospital of Philadelphia, 34th St. and Civic Center Blvd., Philadelphia, PA 19104-4318.

References

Related Letters:

Treatment of Severe Combined Immunodeficiency
Flake A. W., Zanjani E. D., Buckley R. H., Myers L. A.
Extract | Full Text  
N Engl J Med 1999; 341:291-292, Jul 22, 1999. Correspondence

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