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Cervical Lymphadenitis

  • Laura Marques
  • Lidia Branco
  • Julia Vasconcelos
  • Esmeralda Neves
Chapter

Abstract

Chronic granulomatous disease (CGD) is caused by a defect in phagocytes NADPH oxidase complex that is responsible for the phagocytes respiratory burst

The screening diagnostic test is the oxidative burst test

Patients with X-linked forms usually present in the first years of life but autosomal recessive forms may present later and be milder

The first-line curative treatment in CGD is hematopoietic stem cell transplantation

Keywords

Chronic granulomatous disease NADPH oxidase Oxidative burst test Leishmania donovani Kala Azar 

References

  1. 1.
    Greenberg DE, Goldberg JB, Stock F, Murray PR, Holland SM, LiPuma JJ. Recurrent Burkholderia infection in patients with chronic granulomatous disease: 11-year experience at a large referral center. Clin Infect Dis. 2009;48(11):1577–9.CrossRefGoogle Scholar
  2. 2.
    Rosenzweig SD, Holland S. Chronic granulomatous disease: pathogenesis, clinical manifestations, pathogenesis. In: UpToDate. Wolters Kluwer. 2017. https://www.uptodate.com/contents/chronic-granulomatous-disease-pathogenesis-clinical-manifestations-and-diagnosis. Accessed 27 Nov 2017.
  3. 3.
    Meischl C, Roos D. The molecular basis of chronic granulomatous disease. Springer Semin Immunopathol. 1998;19(4):417–34.CrossRefGoogle Scholar
  4. 4.
    Asensi V, Tricas L, Meana A, Roos D, Carton J, Maradona J, Fresno M, Valle E, Fierer J, Arribas J. Visceral leishmaniasis and other severe infections in an adult patient with p47-phoxdeficient chronic granulomatous disease. Infection. 2000;28(3):171–4.CrossRefGoogle Scholar
  5. 5.
    Roos D, de Boer M. Molecular diagnosis of chronic granulomatous disease. Clin Exp Immunol. 2014;175(2):139–49.CrossRefGoogle Scholar
  6. 6.
    Martín A, Marques L, Soler-Palacín P, Caragol I, Hernandez M, Figueras C, Español T. Visceral leishmaniasis associated hemophagocytic syndrome in patients with chronic granulomatous disease. Pediatr Infect Dis J. 2009;28(8):753–4.CrossRefGoogle Scholar
  7. 7.
    Roos D. Chronic granulomatous disease. Br Med Bull. 2016;118(1):50–63.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Laura Marques
    • 1
  • Lidia Branco
    • 2
  • Julia Vasconcelos
    • 2
  • Esmeralda Neves
    • 2
    • 3
  1. 1.Paediatric Infectious Diseases and Immunodeficiencies Unit, Centro Materno-Infantil do Norte, Centro Hospitalar do PortoPortoPortugal
  2. 2.Immunology DepartmentCentro Hospitalar do PortoPortoPortugal
  3. 3.Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN)PortoPortugal

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