Advertisement

The Immunocompromised Host: The Patient with Recurrent Infection

  • Andrew R. Gennery
  • Andrew J. Cant
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 549)

Absract

In childhood the immune system is less effective as it is encountering antigens for the first time, learning how to make adequate responses, and acquiring memory. Young children often mix with other children in their family or nursery and so are exposed to many pathogens. As a result recurrent infection is not uncommon, sometimes raising concern that immune function is abnormal. Most children with recurrent infection have a normal immune system but a pattern of recurrent or persistent infection is also a cardinal manifestation of primary immune deficiency (PID). How do we recognize the child with an underlying PID and investigate and treat appropriately? This is an important question as normal children should not be over-investigated yet prompt recognition and early diagnosis of immunodeficiency should ensure treatment before end organ damage has occurred. For example, early institution of replacement intravenous immunoglobulin therapy for antibody deficient patients will prevent bronchiectasis or halt further damage; early diagnosis of Severe Combined Immune Deficiency (SCID) means bone marrow transplantation is more likely to succeed as it can be performed before life threatening infective damage has occurred. Early and accurate diagnosis also helps predict prognosis and facilitates genetic counselling and an accurate prediction of risk to future siblings.

Keywords

Chronic Granulomatous Disease Antibody Deficiency Nijmegen Breakage Syndrome Severe Combine Immune Deficiency Common Variable Immune Deficiency 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Berrington, J.E., Flood, T.J., Abinun, M., Galloway, A., and Cant, A.J. (2000). Unsuspected Pneumocystis carinii pneumonia at presentation of severe primary immunodeficiency. Arch. Dis. Child 82, 144–147.PubMedCrossRefGoogle Scholar
  2. Brown, D.C., Grace, E., Sumner, A.T., Edmunds, A.T., and Ellis, P.M. (1995). ICF syndrome (immunodeficiency, centromeric instability and facial anomalies): Investigation of heterochromatin abnormalities and review of clinical outcome. Hum. Genet. 96, 411–416.PubMedCrossRefGoogle Scholar
  3. Gennery, A.R., Barge, D., O’Sullivan, J.J., Flood, T.J., Abinun, M., and Cant, A.J. (2002). Antibody deficiency and autoimmunity in 22ql 1.2 deletion syndrome. Arch. Dis. Child 86, 422–425.PubMedCrossRefGoogle Scholar
  4. Hague, R.A., Rassam, S., Morgan, G., and Cant, A.J. (1994). Early diagnosis of severe combined immune deficincy syndrome. Arch. Dis. Child 70, 260–263.PubMedCrossRefGoogle Scholar
  5. Hoare, S., El-Shazali, O., Clark, J.E., Fay, A., and Cant, A.J. (2002). Investigation for complement deficiency following meningococcal disease. Arch. Dis. Child 86, 215–217.PubMedCrossRefGoogle Scholar
  6. Hosking, C.S. and Roberton, D.M. (1981). The diagnostic approach to recurrent infections in childhood. Clin. Immunol. Allergy 1, 631–639.Google Scholar
  7. Plebani, A., Soresina, A., Rondelli, R., Amato, G.M., Azzari, C., Cardinale, F. et al., and Italian Pediatric Group for XLA-AIEOP. (2002). Clinical, immunological, and molecular analysis in a large cohort of patients with X-linked agammaglobulinemia: An Italian multicenter study. Clin. Immunol. 104, 221–230.PubMedCrossRefGoogle Scholar
  8. Stephan, J.L., Vlekova, V., Le Deist, F., Blanche, S., Donadieu, J., De Saint-Basile, G. et al. (1993). Severe combined immunodeficiency: A retrospective single-center study of clinical presentation and outcome in 117 patients. J. Pediatr. 123, 564–572.PubMedCrossRefGoogle Scholar

Copyright information

© Science+Business Media New York 2004

Authors and Affiliations

  • Andrew R. Gennery
  • Andrew J. Cant

There are no affiliations available

Personalised recommendations