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Review Article
Advances in Immunology
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Volume 343:1703-1714 December 7, 2000 Number 23
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Immunodeficiency Diseases Caused by Defects in Phagocytes
Julie A. Lekstrom-Himes, M.D., and John I. Gallin, M.D.

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Primary phagocytic defects must be included in the differential diagnosis of recurrent infection and fever in a child and occasionally in an adult. Early diagnosis is essential, because manifestations of infection are usually blunted and rapid intervention can be lifesaving. In general, patients are identified at a young age on the basis of their susceptibility to normally nonpathogenic bacteria or fungi. In some cases, the infectious agents point to the disorder (Table 1): catalase-positive microorganisms and aspergillosis species are characteristic of chronic granulomatous disease,1 and atypical mycobacteria suggest a defect in the interferon-{gamma}–interleukin-12 axis.2 These bacterial infections . . . [Full Text of this Article]

Congenital Neutropenias

Cyclic Neutropenia

Severe Congenital Neutropenia

The Shwachman–Diamond Syndrome

Treatment of Congenital Neutropenias

Defects of Adhesion

Defects of Signaling

Defects of Intracellular Killing

Defects in the Formation and Function of Neutrophil Granules

Myeloperoxidase Deficiency

The Chédiak–Higashi Syndrome

Neutrophil-Specific Granule Deficiency

Conclusions


Source Information

From the Laboratory of Host Defenses, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Md.

Address reprint requests to Dr. Gallin at Bldg. 10, Rm. 2C146, 10 Center Dr., MSC 1504, Bethesda, MD 20892-1504, or at jgallin@cc.nih.gov.

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