ArticleClinical infectious diseases : an official publication of the Infectious Diseases Society of America2015
Common severe infections in chronic granulomatous disease.
Article in Clinical infectious diseases : an official publication of the Infectious Diseases Society of America, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 222 papers, 2 of them syntheses that pooled it.
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Who cites it
222 citing papers in PubMed, 2 syntheses or guidelines pooled it, 410 citations in OpenAlex.
- When the Good Syndrome Goes Bad: A Systematic Literature Review.Frontiers in immunology · 2021Pooled it
- Nocardiosis Associated with Primary Immunodeficiencies (Nocar-DIP): an International Retrospective Study and Literature Review.Journal of clinical immunology · 2020Pooled it
- Antiviral cellular therapy for enhancing T-cell reconstitution before or after hematopoietic stem cell transplantation (ACES): a two-arm, open label phase II interventional trial of pediatric patients with risk factor assessment.Nature communications · 2024Trial
- Severe hematopoietic stem cell inflammation compromises chronic granulomatous disease gene therapy.Cell reports. Medicine · 2023Trial
- High Levels of IL-18 and IFN-γ in Chronically Inflamed Tissue in Chronic Granulomatous Disease.Frontiers in immunology · 2019Trial
- Thalidomide for CGD-related inflammatory bowel disease: A randomized, double-blind trial.Journal of human immunity · 2026Article
- Clinical pearls and pitfalls in inborn errors of immunity: An expert-derived framework for diagnostic pattern recognition.Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology · 2026Review
- Chronic granulomatous disease: Clinical, microbial, and genetic findings in 39 Colombian patients.Journal of human immunity · 2026Article
- Recurrent and unusual infections unmasking a rare inborn error of immunity: a case report RAS-associated Autoimmune Lymphoproliferative Disease (RALD).BMC pediatrics · 2026Article
- Concurrent Burkholderia cepacia detected by plasma mcfDNA sequencing in a child with MRSA liver abscess and chronic granulomatous disease: a case report.BMC pediatrics · 2026Article
- How I Treat: Chronic granulomatous disease.Journal of human immunity · 2026Review
- Antibiotic-induced microbiota disruption impairs neutrophil-mediated immunity to respiratorymBio · 2026Article
- Adeno-associated virus delivery of anti-alpha toxin monoclonal antibodies confers protection against Staphylococcus aureus infections.PLoS pathogens · 2026Article
- NLRP3 inflammasome blockade treats intestinal inflammation associated with chronic granulomatous disease.Blood · 2026Article
- Chronic granulomatous disease with suspected secondary hemophagocytic lymphohistiocytosis: a case report and hypothesis-generating rationale for neuroimmune modulation.Critical care (London, England) · 2026Review
- Primary Immunodeficiency Diseases with BCG-Induced Diseases: A 15-Year Longitudinal Cohort Study.Journal of clinical immunology · 2026Article
- Bursting at the Seams.Clinical infectious diseases : an official publication of the Infectious Diseases Society of America · 2026Article
- Clinical Spectrum of Cutaneous, Ocular, and Hair Manifestations in Patients With Inborn Errors of Immunity: Insights From a Single Center in Turkey.Immunity, inflammation and disease · 2026Article
- Misleading presentation of tuberculosis in a child with CGD revealing invasive aspergillosis: A molecularly confirmed case.IDCases · 2026Article
- Cerebral Aspergillosis: Diagnostic Challenges, Therapeutic Strategies, and Future Research.CNS drugs · 2026Review
162 more citing papers are in PubMed but not listed here.
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29 authors at 5 institutions in 1 country.
Funding
Abstract
backgroundChronic 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.
methodsAll patients had confirmed diagnoses of CGD, and genotype was determined where possible. Medical records were excerpted into a standard format. Microbiologic analyses were restricted to Staphylococcus, Burkholderia, Serratia, Nocardia, and Aspergillus.
resultsAspergillus incidence was estimated at 2.6 cases per 100 patient-years; Burkholderia, 1.06 per 100 patient-years; Nocardia, 0.81 per 100 patient-years; Serratia, 0.98 per 100 patient-years, and severe Staphylococcus infection, 1.44 per 100 patient-years. Lung infection occurred in 87% of patients, whereas liver abscess occurred in 32%. Aspergillus incidence was 55% in the lower superoxide-producing quartiles (quartiles 1 and 2) but only 41% in the higher quartiles (rate ratio, <0.0001). Aspergillus and Serratia were somewhat more common in lower superoxide producing gp91phox deficiency. The median age at death has increased from 15.53 years before 1990 to 28.12 years in the last decade. Fungal infection carried a higher risk of mortality than bacterial infection and was the most common cause of death (55%). Gastrointestinal complications were not associated with either infection or mortality.
conclusionsFungal infections remain a major determinant of survival in CGD. X-linked patients generally had more severe disease, and this was generally in those with lower residual superoxide production. Survival in CGD has increased over the years, but infections are still major causes of morbidity and mortality.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.