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J. Fungi 2016, 2(2), 15; doi:10.3390/jof2020015

Aspergillosis in Chronic Granulomatous Disease

1
Aberdeen Fungal Group, MRC Centre for Medical Mycology, Institute of Medical Sciences, University of Aberdeen, Aberdeen AB25 2ZD, UK
2
Radboud University Medical Center, Amalia Children’s Hospital, Nijmegen 6500 HB, The Netherlands
*
Author to whom correspondence should be addressed.
Academic Editor: William J. Steinbach
Received: 31 March 2016 / Revised: 19 May 2016 / Accepted: 24 May 2016 / Published: 26 May 2016
(This article belongs to the Special Issue Aspergillus fumigatus: From Diagnosis to Therapy)
View Full-Text   |   Download PDF [258 KB, uploaded 26 May 2016]

Abstract

Patients with chronic granulomatous disease (CGD) have the highest life-time incidence of invasive aspergillosis and despite the availability of antifungal prophylaxis, infections by Aspergillus species remain the single most common infectious cause of death in CGD. Recent developments in curative treatment options, such as haematopoietic stem cell transplantation, will change the prevalence of infectious complications including invasive aspergillosis in CGD patients. However, invasive aspergillosis in a previously healthy host is often the first presenting feature of this primary immunodeficiency. Recognizing the characteristic clinical presentation and understanding how to diagnose and treat invasive aspergillosis in CGD is of utmost relevance to improve clinical outcomes. Significant differences exist in fungal epidemiology, clinical signs and symptoms, and the usefulness of non-culture based diagnostic tools between the CGD host and neutropenic patients, reflecting underlying differences in the pathogenesis of invasive aspergillosis shaped by the nicotinamide adenine dinucleotide phosphate (NADPH)-oxidase deficiency. View Full-Text
Keywords: aspergillosis; chronic granulomatous disease; Aspergillus fumigatus; Aspergillus nidulans; NADPH-oxidase; respiratory burst aspergillosis; chronic granulomatous disease; Aspergillus fumigatus; Aspergillus nidulans; NADPH-oxidase; respiratory burst
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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King, J.; Henriet, S.S.V.; Warris, A. Aspergillosis in Chronic Granulomatous Disease. J. Fungi 2016, 2, 15.

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