Encyclopedia of Medical Immunology

Living Edition
| Editors: Ian MacKay, Noel R. Rose

Specific Granule Deficiency

  • Herberto Jose Chong-NetoEmail author
  • Cristine Secco Rosario
  • Nelson Augusto Rosario
Living reference work entry
DOI: https://doi.org/10.1007/978-1-4614-9209-2_146-1

Synonyms

Definition

Neutrophil-specific granule deficiency (SGD) is an autosomal recessive congenital disorder in which the function and structure of neutrophils are altered, resulting in severe and recurrent bacterial infections, mainly by Staphylococcus aureus. SGD patients’ neutrophils lack primary and secondary granule proteins, have abnormal migration and aggregation atypical bilobed nuclei and inadequate bactericidal activity. CEBPE, also known as CCAAT/enhancer binding protein (C/EBP) epsilon, which is a member of the C/EBP family of transcription factors, has been implicated in SGD. Those patients have normal neutrophil count in blood count.

Introduction/Background

Specific granule deficiency (SGD) is a rare congenital disorder provoked by an abnormal formation of peroxidase negative neutrophil granules. SGD patients suffer from recurrent infections, often in the form of abscesses. Their number of neutrophils is...

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References

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Herberto Jose Chong-Neto
    • 1
    Email author
  • Cristine Secco Rosario
    • 2
  • Nelson Augusto Rosario
    • 1
  1. 1.Federal University of ParanáCuritibaBrazil
  2. 2.Department of PediatricsFederal University of ParanáCuritibaBrazil

Section editors and affiliations

  • Antonio Condino-Neto
    • 1
  1. 1.Institute of Biomedical SciencesUniversity of São PauloSão PauloBrasil