Chronic granulomatous disease in childhood

Cecilia Barese, W. Goebel

Research output: Contribution to journalArticle

Abstract

Chronic granulomatous disease (CGD) is a genetic immunodeficiency that commonly presents in infancy and early childhood. Currently, life expectancy is beyond the fourth decade of life if the disease is promptly diagnosed and treated. CGD is characterized by increased susceptibility to recurrent and often life-threatening infections caused by bacteria and fungi. The hallmark of the disease is the formation of inflammatory granulomas, which can cause obstruction of the hollow viscera. Therapeutic management of CGD patients relies on the early and aggressive treatment of acute infections, as well as lifelong antibiotic and antifungal prophylaxis. Interferon gamma has a role in the prevention and management of intractable infections. Current efforts for the development of curative therapies focus on allogeneic bone marrow transplantation for CGD patients with HLA-identical sibling donors, and transplantation of autologous genecorrected hematopoietic stem cells for patients who lack suitable marrow donors. Significant recent achievements in these fields provide a realistic hope for a lifelong cure of CGD.

Original languageEnglish
Pages (from-to)63-75
Number of pages13
JournalCurrent Pediatric Reviews
Volume2
Issue number1
StatePublished - Feb 2006

Fingerprint

Chronic Granulomatous Disease
Hope
Infection
Tissue Donors
Viscera
Antibiotic Prophylaxis
Autologous Transplantation
Homologous Transplantation
Hematopoietic Stem Cells
Life Expectancy
Granuloma
Bone Marrow Transplantation
Interferon-gamma
Siblings
Fungi
Therapeutics
Bone Marrow
Bacteria

Keywords

  • CGD
  • Chronic granulomatous disease
  • Inflammation
  • Phagocyte defect
  • Primary immunodeficiency
  • Recurrent infections

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Chronic granulomatous disease in childhood. / Barese, Cecilia; Goebel, W.

In: Current Pediatric Reviews, Vol. 2, No. 1, 02.2006, p. 63-75.

Research output: Contribution to journalArticle

Barese, Cecilia ; Goebel, W. / Chronic granulomatous disease in childhood. In: Current Pediatric Reviews. 2006 ; Vol. 2, No. 1. pp. 63-75.
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