Neonatal Med.  2018 Nov;25(4):191-195. 10.5385/nm.2018.25.4.191.

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

• Kim HE
• Kim DH
• Chung SH
• Bae CW
• Choi YS

• KMID: 2436138
• DOI: http://doi.org/10.5385/nm.2018.25.4.191

Abstract

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.

Keyword

Leukocyte adhesion deficiency; Infectious arthritis; Osteomyelitis; Premature infant

MeSH Terms

Arthritis, Infectious
Flow Cytometry
Hip*
Humans
Infant, Newborn
Infant, Premature*
Integrins
Intensive Care, Neonatal
Korea
Leukocytes*
Leukocytosis
Osteomyelitis
Umbilical Cord
Wound Healing
Integrins

Figure

• 

  Figure 1. Hip magnetic resonance imaging shows septic arthritis of the right hip associated with periarticular abscess formation in the T2 coronal section (black arrows).

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  Figure 2. Hip magnetic resonance imaging shows large intramuscular abscess formation in the right anterior thigh, probably vastus intermedius (about 32×28×19 mm in size) in the (A) T2 and (B) T1 coronal section (black arrows).

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  Figure 3. Hip X-ray shows the deformity of the right femoral head (black arrows).

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  Figure 4. Bone scan shows diffuse uptake in the right femur and right iliac bones (arrows).

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  Figure 5. (A, B) Bone scan shows diffuse uptake in the right femur and right iliac bones (arrows).

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