Primary phagocytic disorders of childhood.
Primary phagocytic disorders are rare and usually first manifest during childhood. A phagocytic disorder should be considered in patients with unusually severe or recurrent infections by common pathogens or an infection by certain opportunistic pathogens. Common manifestations of primary phagocytic disorders include recurrent soft-tissue infections requiring incision and drainage, severe dental infections leading to premature tooth loss, recurrent pneumonias, and perirectal infections. Primary phagocytic disorders are caused by defects of neutrophil number or function, and the latter, in turn, can be divided into disorders of oxidative and nonoxidative pathways. Certain phagocytic disorders have unique characteristics apart from the immune defect that may facilitate diagnosis. Early diagnosis of phagocytic disorders can be life-saving or lead to a significant reduction in morbidity and relies on a compatible clinical (or family) history and appropriate laboratory diagnostic studies. Key principles of management of such patients involve early recognition and aggressive treatment of infections and appropriate surgical débridement of localized disease. Prophylactic antibiotics, BMT, and the use of exogenous cytokines, such as IFN-gamma and G-CSF, are appropriate for specific phagocytic disorders. Gene therapy is a promising strategy for several of the phagocytic disorders.
Segal
B H
BH
Divisions of Infectious Diseases and Allergy, Immunology, and Rheumatology, State University of New York at Buffalo, Roswell Park Cancer Institute, Buffalo, New York, USA. Brahm.Segal@roswellpark.org
Holland
S M
SM
D001424:Bacterial Infections / Q000453:epidemiology / Q000276:immunology*; D002675:Child, Preschool; D005260:Female; D006105:Granulomatous Disease, Chronic / Q000175:diagnosis* / Q000276:immunology* / Q000628:therapy; D006801:Humans; D007154:Immune System Diseases / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D007155:Immunologic Factors; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010587:Phagocytosis / Q000276:immunology*; D011379:Prognosis; D012008:Recurrence; D018570:Risk Assessment
D007155:Immunologic Factors