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Purine nucleoside phosphorylase deficiency (PNP-def) presenting with lymphopenia and developmental delay: successful correction with umbilical cord blood transplantation.

Publication ,  Journal Article
Myers, LA; Hershfield, MS; Neale, WT; Escolar, M; Kurtzberg, J
Published in: J Pediatr
November 2004

Purine nucleoside phosphorylase deficiency is a primary immunodeficiency syndrome characterized by the triad of recurrent infection, neurologic dysfunction, and autoimmunity. This patient presented atypically with few infections and normal T-cell function. Progressive lymphopenia, ataxia, and developmental delay led to diagnosis. Umbilical cord blood transplantation corrected the immunodeficiency.

Duke Scholars

Published In

J Pediatr

DOI

ISSN

0022-3476

Publication Date

November 2004

Volume

145

Issue

5

Start / End Page

710 / 712

Location

United States

Related Subject Headings

  • Purine-Nucleoside Phosphorylase
  • Pediatrics
  • Male
  • Lymphopenia
  • Infant
  • Immunologic Deficiency Syndromes
  • Humans
  • Developmental Disabilities
  • Cord Blood Stem Cell Transplantation
  • 3213 Paediatrics
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Myers, L. A., Hershfield, M. S., Neale, W. T., Escolar, M., & Kurtzberg, J. (2004). Purine nucleoside phosphorylase deficiency (PNP-def) presenting with lymphopenia and developmental delay: successful correction with umbilical cord blood transplantation. J Pediatr, 145(5), 710–712. https://doi.org/10.1016/j.jpeds.2004.06.075
Myers, Laurie A., Michael S. Hershfield, Wirt T. Neale, Maria Escolar, and Joanne Kurtzberg. “Purine nucleoside phosphorylase deficiency (PNP-def) presenting with lymphopenia and developmental delay: successful correction with umbilical cord blood transplantation.J Pediatr 145, no. 5 (November 2004): 710–12. https://doi.org/10.1016/j.jpeds.2004.06.075.
Myers, Laurie A., et al. “Purine nucleoside phosphorylase deficiency (PNP-def) presenting with lymphopenia and developmental delay: successful correction with umbilical cord blood transplantation.J Pediatr, vol. 145, no. 5, Nov. 2004, pp. 710–12. Pubmed, doi:10.1016/j.jpeds.2004.06.075.
Journal cover image

Published In

J Pediatr

DOI

ISSN

0022-3476

Publication Date

November 2004

Volume

145

Issue

5

Start / End Page

710 / 712

Location

United States

Related Subject Headings

  • Purine-Nucleoside Phosphorylase
  • Pediatrics
  • Male
  • Lymphopenia
  • Infant
  • Immunologic Deficiency Syndromes
  • Humans
  • Developmental Disabilities
  • Cord Blood Stem Cell Transplantation
  • 3213 Paediatrics