Abstract

During inflammation, leukocytes play a key role in maintaining tissue homeostasis by elimination of pathogens and removal of damaged tissues. Leukocytes migrate to the site of inflammation by crawling over and through the blood vessel wall, into the tissue. Leukocyte adhesion deficiencies (LAD 1, LAD 2 and LAD 3) are caused by defects in the adhesion of leukocyte to the blood vessel wall due to mutations in the genes encoding b2 integrin, selectin and kindling-3, respectively. Patients experience recurrent none pus forming bacterial infections and neutrophilia, often preceded by delayed separation of umbilical cord. This case report describes a child who presented with umbilical sepsis followed by separation of the umbilical cord on day 15, and recurrent bacterial meningitis with high neutrophilia.

Highlights

  • The cellular elements of the human immune system are constantly communicating with each other

  • The results showed CD18-0.1%,CD11a0.1%,CD11b-2.3%,CD11c-0.1%, suggestive of severe Leukocyte adhesion defects (LAD) 1

  • Each integrin consists of a distinct heavy (150-180 kDa) polypeptide chain non covalently linked to light (95 kDa) polypeptide chain (CD18)

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Summary

Introduction

The cellular elements of the human immune system are constantly communicating with each other. This network includes endothelial surfaces, as well as extra-cellular matrices and leads to modulation of immune and inflammatory responses and control of cell traffic Some of these interactions depend on cytokines for their regulation, others require firm leucocyte-cell or leucocyte-matrix contact, called adhesion.[1] umbilical cord separation, recurrent severe bacterial infection, periodontitis, and delayed wound healing and often persistent leukocytosis with absent pus formation. It causes reduced inflammatory response in spite of a high neutrophil count. Later she presented with another episode of severe sepsis and died in spite of treatment

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