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Educational paper: Defects in number and function of neutrophilic granulocytes causing primary immunodeficiency.

van den Berg JM, Kuijpers TW - Eur. J. Pediatr. (2011)

Bottom Line: The neutrophilic granulocyte (neutrophil) is the most important cellular component of the innate immune system.A total absence of neutrophils or a significant decrease in their number leads to severe immunodeficiency.In order to be neutralized, this pathogen has to be recognized, phagocytosed, and destroyed by lytic enzymes contained in the neutrophil's granules and reactive oxygen species formed by the enzyme complex NADPH oxidase.

View Article: PubMed Central - PubMed

Affiliation: Dept of Pediatric Hematology, Immunology and Infectious Diseases, Emma Children's Hospital, Academic Medical Centre, University of Amsterdam, Room H7-214, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands. j.m.vandenberg@amc.nl

ABSTRACT
The neutrophilic granulocyte (neutrophil) is the most important cellular component of the innate immune system. A total absence of neutrophils or a significant decrease in their number leads to severe immunodeficiency. A mature neutrophil, released from the bone marrow, should be able to migrate from the blood towards the tissues, following a chemotactic gradient to a pathogen. In order to be neutralized, this pathogen has to be recognized, phagocytosed, and destroyed by lytic enzymes contained in the neutrophil's granules and reactive oxygen species formed by the enzyme complex NADPH oxidase. Rare genetic defects leading to the loss of each one of these biological properties of the neutrophil have been described and are associated with immunodeficiency. This review provides a summary of the normal development and biological functions of neutrophils and describes the diseases caused by defects in neutrophil number and function.

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Related in: MedlinePlus

An ulcerative lesion at the right chest of a formerly healthy 2-year-old boy. The rare fungus Rhizopus oryzae caused this infection which led to the diagnosis auosomal recessive chronic granulomatous disease
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Fig1: An ulcerative lesion at the right chest of a formerly healthy 2-year-old boy. The rare fungus Rhizopus oryzae caused this infection which led to the diagnosis auosomal recessive chronic granulomatous disease

Mentions: In general, a lack of functionally normal neutrophils in an individual may lead to infections with Staphylococcus aureus, gram-negative organisms such as Pseudomonas aeruginosa and Burkholderia cepacia, the gram-positive bacterium Nocardia asteroides, and infections with fungi and yeasts such as Aspergillus spp. and Candida spp. (Fig. 1). In the case of S. aureus, the severity of the disease or the location of the infection (e.g., liver abscess) distinguishes an immunocompromised patient from a healthy individual. The other above-mentioned pathogens, including fungi, do not cause disease in an immunocompetent child. Hence, once suspected or identified, such infections should always lead to scrutinizing neutrophil number and functions.Fig. 1


Educational paper: Defects in number and function of neutrophilic granulocytes causing primary immunodeficiency.

van den Berg JM, Kuijpers TW - Eur. J. Pediatr. (2011)

An ulcerative lesion at the right chest of a formerly healthy 2-year-old boy. The rare fungus Rhizopus oryzae caused this infection which led to the diagnosis auosomal recessive chronic granulomatous disease
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3197933&req=5

Fig1: An ulcerative lesion at the right chest of a formerly healthy 2-year-old boy. The rare fungus Rhizopus oryzae caused this infection which led to the diagnosis auosomal recessive chronic granulomatous disease
Mentions: In general, a lack of functionally normal neutrophils in an individual may lead to infections with Staphylococcus aureus, gram-negative organisms such as Pseudomonas aeruginosa and Burkholderia cepacia, the gram-positive bacterium Nocardia asteroides, and infections with fungi and yeasts such as Aspergillus spp. and Candida spp. (Fig. 1). In the case of S. aureus, the severity of the disease or the location of the infection (e.g., liver abscess) distinguishes an immunocompromised patient from a healthy individual. The other above-mentioned pathogens, including fungi, do not cause disease in an immunocompetent child. Hence, once suspected or identified, such infections should always lead to scrutinizing neutrophil number and functions.Fig. 1

Bottom Line: The neutrophilic granulocyte (neutrophil) is the most important cellular component of the innate immune system.A total absence of neutrophils or a significant decrease in their number leads to severe immunodeficiency.In order to be neutralized, this pathogen has to be recognized, phagocytosed, and destroyed by lytic enzymes contained in the neutrophil's granules and reactive oxygen species formed by the enzyme complex NADPH oxidase.

View Article: PubMed Central - PubMed

Affiliation: Dept of Pediatric Hematology, Immunology and Infectious Diseases, Emma Children's Hospital, Academic Medical Centre, University of Amsterdam, Room H7-214, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands. j.m.vandenberg@amc.nl

ABSTRACT
The neutrophilic granulocyte (neutrophil) is the most important cellular component of the innate immune system. A total absence of neutrophils or a significant decrease in their number leads to severe immunodeficiency. A mature neutrophil, released from the bone marrow, should be able to migrate from the blood towards the tissues, following a chemotactic gradient to a pathogen. In order to be neutralized, this pathogen has to be recognized, phagocytosed, and destroyed by lytic enzymes contained in the neutrophil's granules and reactive oxygen species formed by the enzyme complex NADPH oxidase. Rare genetic defects leading to the loss of each one of these biological properties of the neutrophil have been described and are associated with immunodeficiency. This review provides a summary of the normal development and biological functions of neutrophils and describes the diseases caused by defects in neutrophil number and function.

Show MeSH
Related in: MedlinePlus