Treatment-Resistant Bacterial Lymphadenitis in an Otherwise Healthy Girl.

Published on Jun 1, 2021in Pediatrics in Review
· DOI :10.1542/PIR.2020-0057
#1Beatriz E. Marciano (NIH: National Institutes of Health)H-Index: 22
#2Christa S. Zerbe (NIH: National Institutes of Health)H-Index: 28
Last. Steven M. Holland (NIH: National Institutes of Health)H-Index: 114
view all 25 authors...
Background Chronic granulomatous disease (CGD) is characterized by recurrent life-threatening bacterial and fungal infections and aberrant inflammation. Mutations in CYBB cause X-linked CGD and account for 65% to 70% of cases in Western countries. Objective We sought to understand the clinical manifestations associated with the X-linked CGD carrier state. Methods We undertook a comprehensive retrospective study of 162 affected female subjects. We examined dihydrorhodamine 123 (DHR) oxidation dat...
74 CitationsSource
#1Danielle E. Arnold (Children's Hospital of Philadelphia)H-Index: 4
#2Jennifer Heimall (Children's Hospital of Philadelphia)H-Index: 22
Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by defects in any of the five subunits of the NADPH oxidase complex responsible for the respiratory burst in phagocytic leukocytes. Patients with CGD are at increased risk of life-threatening infections with catalase-positive bacteria and fungi and inflammatory complications such as CGD colitis. The implementation of routine antimicrobial prophylaxis and the advent of azole antifungals has considerably improved overall surv...
84 CitationsSource
The American Academy of Allergy, Asthma & Immunology (AAAAI) and the American College of Allergy, Asthma & Immunology (ACAAI) have jointly accepted responsibility for establishing the "Practice parameter for the diagnosis and management of primary immunodeficiency." This is a complete and comprehensive document at the current time. The medical environment is a changing environment, and not all recommendations will be appropriate for all patients. Because this document incorporated the efforts of...
265 CitationsSource
#1Beatriz E. Marciano (NIH: National Institutes of Health)H-Index: 22
#2Christine Spalding (NIH: National Institutes of Health)H-Index: 12
Last. Steven M. Holland (NIH: National Institutes of Health)H-Index: 114
view all 29 authors...
Background. Chronic granulomatous disease (CGD) is due to defective nicotinamide adenine dinucleotide phosphate oxidase activity and characterized by recurrent infections with a limited spectrum of bacteria and fungi as well as inflammatory complications. To understand the impact of common severe infections in CGD, we examined the records of 268 patients followed at a single center over 4 decades. Methods. All patients had confirmed diagnoses of CGD, and genotype was determined where possible. M...
181 CitationsSource
#1Dirk Roos (UvA: University of Amsterdam)H-Index: 114
#2M de Boer (UvA: University of Amsterdam)H-Index: 31
Patients with chronic granulomatous disease (CGD) suffer from recurrent, life-threatening bacterial and fungal infections of the skin, the airways, the lymph nodes, liver, brain and bones. Frequently found pathogens are Staphylococcus aureus, Aspergillus species, Klebsiella species, Burkholderia cepacia and Salmonella species. CGD is a rare (approximate to 1:250000 births) disease caused by mutations in any one of the five components of the nicotinamide adenine dinucleotide phosphate (NADPH) oxi...
102 CitationsSource
Cited By0