Pediatrics in Review
HOME HELP CONTACT US SUBSCRIPTIONS CME ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Full Text (PDF)
Right arrow Rapid Responses: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when Rapid Responses are posted
Right arrow Alert me if a correction is posted
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Boxer, L. A.
Right arrow Articles by Blackwood, R. A.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Boxer, L. A.
Right arrow Articles by Blackwood, R. A.

Leukocyte Disorders: Quantitative and Qualitative Disorders of the Neutrophil, Part 2

Laurence A. Boxer MD1
R. Alexander Blackwood MD, PhD2
 1 Professor and Director, Pediatric Hematology/Oncology, University of Michigan, Ann Arbor, MI. Dr Boxer participated in Amgen Corporation's phase III trial evaluating the efficacy of rhG-CSF in the treatment of patients who have severe congenital neutropenia. He also participated in Genentech's trial evaluating the efficacy of gamma-interferon in the treatment of patients who have chronic granulomatous disease
2 Assistant Professor of Pediatrics and Communicable Diseases, Division of Pediatric Infectious Diseases, University of Michigan, Ann Arbor, MI.

The differential diagnosis for a patient presenting with recurrent infections is formidable, given the complexity of the immune system. The clinical presentation of a patient who has a qualitative neutrophil abnormality may be similar to that of one who has an antibody or complement disorder. In general, evaluation for phagocytic cell disorders (Table) should be initiated among those patients who have at least one of the following clinical features: 1) Two or more systemic bacterial infections; 2) Frequent, serious respiratory infections, such as pneumonia or sinusitis, or frequent bacterial infection, such as cellulitis, draining otitis media, or lymphadenitis; 3) Infections presenting at unusual sites (hepatic or brain abscess); and 4) Infections associated with unusual pathogens (ie, Aspergillus pneumonia, disseminated candidiasis, or infections with Serratia marcescens, Nocardia sp, and Pseudomonas cepacia).

Disorders of Neutrophil Function

HYPERIMMUNOGLOBULIN E SYNDROME (JOB SYNDROME)

Hyperimmunoglobulin E (hyper-IgE) syndrome is an autosomal dominant disorder with incomplete penetrance that is characterized by elevated levels of serum IgE and recurrent staphylococcal abscesses (Table). Neutrophils in these children exhibit impaired chemotaxis, although phagocytic function is normal. Clinically, hyper-IgE syndrome must be distinguished from severe atopic dermatitis. Both disorders are characterized by the development of severe dermatitis in the presence of elevated IgE levels.




This article has been cited by other articles:


Home page
 J. Immunol.Home page
T. O. Hirche, R. Benabid, G. Deslee, S. Gangloff, S. Achilefu, M. Guenounou, F. Lebargy, R. E. Hancock, and A. Belaaouaj
Neutrophil Elastase Mediates Innate Host Protection against Pseudomonas aeruginosa
J. Immunol., October 1, 2008; 181(7): 4945 - 4954.
[Abstract] [Full Text] [PDF]


HOME HELP CONTACT US SUBSCRIPTIONS CME ARCHIVE SEARCH TABLE OF CONTENTS
Pediatrics Pediatrics in Review
Copyright © 1996 by the American Academy of Pediatrics.