Neonatal Medicine (Nov 2018)

Leukocyte Adhesion Deficiency Associated with Neonatal Septic Hip in a Late Preterm Infant

  • Hye-Eun Kim,
  • Do Hee Kim,
  • Sung-Hoon Chung,
  • Chong-Woo Bae,
  • Yong-Sung Choi

DOI
https://doi.org/10.5385/nm.2018.25.4.191
Journal volume & issue
Vol. 25, no. 4
pp. 191 – 195

Abstract

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Leukocyte adhesion deficiency is a rare primary immunodeficiency and autosomal recessive disorder caused by a mutation in the gene encoding CD18, which is a constituent of leukocyte integrins. Clinical features usually begin with a delay in the separation of the umbilical cord in the neonatal period, and are characterized by marked leukocytosis with infection, delayed wound healing, and repeated bacterial and fungal infections. We experienced a case of leukocyte adhesion deficiency diagnosed in the neonatal period, in which a late preterm infant admitted to neonatal intensive care unit presented with a septic hip. Flow cytometry analysis of whole blood showed a decrease in the expression of CD11b/CD18. This is the first case of leukocyte adhesion deficiency with neonatal septic hip diagnosed in Korea.

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