The DOI matches its Crossref record; 37 of the 37 references it cites were found in a registry or an index.
Chronic Granulomatous Disease: The European Experience
Open accessAbstract
CGD is an immunodeficiency caused by deletions or mutations in genes that encode subunits of the leukocyte NADPH oxidase complex. Normally, assembly of the NADPH oxidase complex in phagosomes of certain phagocytic cells leads to a "respiratory burst", essential for the clearance of phagocytosed micro-organisms. CGD patients lack this mechanism, which leads to life-threatening infections and granuloma formation. However, a clear picture of the clinical course of CGD is hampered by its low prevalence (approximately 1:250,000). Therefore, extensive clinical data from 429 European patients were collected and analyzed. Of these patients 351 were males and 78 were females. X-linked (XL) CGD (gp91(phox) deficient) accounted for 67% of the cases, autosomal recessive (AR) inheritance for 33%. AR-CGD was diagnosed later in life, and the mean survival time was significantly better in AR patients (49.6 years) than in XL CGD (37.8 years), suggesting a milder disease course in AR patients. The disease manifested itself most frequently in the lungs (66% of patients), skin (53%), lymph nodes (50%), gastrointestinal tract (48%) and liver (32%). The most frequently cultured micro-organisms per episode were Staphylococcus aureus (30%), Aspergillus spp. (26%), and Salmonella spp. (16%). Surprisingly, Pseudomonas spp. (2%) and Burkholderia cepacia (<1%) were found only sporadically. Lesions induced by inoculation with BCG occurred in 8% of the patients. Only 71% of the patients received antibiotic maintenance therapy, and 53% antifungal prophylaxis. 33% were treated with gamma-interferon. 24 patients (6%) had received a stem cell transplantation. The most prominent reason of death was pneumonia and pulmonary abscess (18/84 cases), septicemia (16/84) and brain abscess (4/84). These data provide further insight in the clinical course of CGD in Europe and hopefully can help to increase awareness and optimize the treatment of these patients.
Overview
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Citations
Cites
Chronic Granulomatous Disease: Report on a National Registry of 368 Patients
Winkelstein, J. A., Marino, M. C., Johnston, R. B., Boyle, J., Curnutte, J., Gallin, J. I., Malech, H. L., Holland, S. M., Ochs, H., Quie, P., et al. (2000) · Medicine · doi:10.1097/00005792-200005000-00003 · Cited by 1,728
Genetic, Biochemical, and Clinical Features of Chronic Granulomatous Disease
Segal, B. H., Leto, T. L., Gallin, J. I., Malech, H. L., & Holland, S. M. (2000) · Medicine · doi:10.1097/00005792-200005000-00004 · Cited by 883
Defective tryptophan catabolism underlies inflammation in mouse chronic granulomatous disease
Romani, L., Fallarino, F., De Luca, A., Montagnoli, C., D’Angelo, C., Zelante, T., Vacca, C., Bistoni, F., Fioretti, M. C., Grohmann, U., et al. (2008) · Nature · doi:10.1038/nature06471 · Cited by 535
Vowells, S. J., Sekhsaria, S., Malech, H. L., Shalit, M., & Fleisher, T. A. (1995) · Journal of Immunological Methods · doi:10.1016/0022-1759(94)00247-t · Cited by 425
Mutations in the X-linked and autosomal recessive forms of chronic granulomatous disease
Roos, D., de Boer, M., Kuribayashi, F., Meischl, C., Weening, R., Segal, A., Ahlin, A., Nemet, K., Hossle, J., Bernatowska-Matuszkiewicz, E., et al. (1996) · Blood · doi:10.1182/blood.v87.5.1663.1663 · Cited by 424
Self-citationGastrointestinal Involvement in Chronic Granulomatous Disease
Marciano, B. E., Rosenzweig, S. D., Kleiner, D. E., Anderson, V. L., Darnell, D. N., Anaya-O'Brien, S., Hilligoss, D. M., Malech, H. L., Gallin, J. I., & Holland, S. M. (2004) · Pediatrics · doi:10.1542/peds.114.2.462 · Cited by 418
Heyworth, P. G., Cross, A. R., & Curnutte, J. T. (2003) · Current Opinion in Immunology · doi:10.1016/s0952-7915(03)00109-2 · Cited by 396
(2012) · SpringerReference · doi:10.1007/springerreference_172807 · Cited by 360
Martire, B., Rondelli, R., Soresina, A., Pignata, C., Broccoletti, T., Finocchi, A., Rossi, P., Gattorno, M., Rabusin, M., Azzari, C., et al. (2008) · Clinical Immunology · doi:10.1016/j.clim.2007.09.008 · Cited by 328
Seger, R. A. (2002) · Blood · doi:10.1182/blood-2002-02-0583 · Cited by 277
Self-citation
Cited by
Patterson, T. F., Thompson, G. R., Denning, D. W., Fishman, J. A., Hadley, S., Herbrecht, R., Kontoyiannis, D. P., Marr, K. A., Morrison, V. A., Nguyen, M. H., et al. (2016) · Clinical Infectious Diseases · doi:10.1093/cid/ciw326 · Cited by 2,926
Regulated necrosis: the expanding network of non-apoptotic cell death pathways
Berghe, T. V., Linkermann, A., Jouan-Lanhouet, S., Walczak, H., & Vandenabeele, P. (2014) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm3737 · Cited by 1,735
2013 IDSA Clinical Practice Guideline for Vaccination of the Immunocompromised Host
Rubin, L. G., Levin, M. J., Ljungman, P., Davies, E. G., Avery, R., Tomblyn, M., Bousvaros, A., Dhanireddy, S., Sung, L., Keyserling, H., et al. (2014) · Clinical Infectious Diseases · doi:10.1093/cid/cit684 · Cited by 1,613
Crump, J. A., Sjölund-Karlsson, M., Gordon, M. A., & Parry, C. M. (2015) · Clinical Microbiology Reviews · doi:10.1128/cmr.00002-15 · Cited by 1,156
Neutrophil extracellular trap cell death requires both autophagy and superoxide generation
Remijsen, Q., Berghe, T. V., Wirawan, E., Asselbergh, B., Parthoens, E., De Rycke, R., Noppen, S., Delforge, M., Willems, J., & Vandenabeele, P. (2011) · Cell Research · doi:10.1038/cr.2010.150 · Cited by 818
Metadata
- Authors
- van den Berg, J. M., van Koppen, E., Åhlin, A., Belohradsky, B. H., Bernatowska, E., Corbeel, L., Español, T., Fischer, A., Kurenko-Deptuch, M., Mouy, R., Petropoulou, T., Roesler, J., Seger, R., Stasia, M.-J., Valerius, N. H., Weening, R. S., Wolach, B., Roos, D., & Kuijpers, T. W.
- Venue
- PLoS ONE
- Publisher
- Public Library of Science (PLoS)
- Published
- 2009-04-21
- Volume
- 4
- Issue
- 4
- Pages
- e5234
- ISSN
- 1932-6203 (ISSN-L)
- OpenAlex
- W2129413349
- Cited by
- 687
- References
- 37
- FWCI
- 15.78
- Type
- journal article
- Language
- en
- Open access
- gold
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
How to cite
Cite CoChat for the checks on this page, and the paper for its own findings.
- This page
- CoChat. (2026). Citation check: Chronic Granulomatous Disease: The European Experience. https://app.cochat.ai/papers/10.1371/journal.pone.0005234
- The paper
- van den Berg, J. M., van Koppen, E., Åhlin, A., Belohradsky, B. H., Bernatowska, E., Corbeel, L., Español, T., Fischer, A., Kurenko-Deptuch, M., Mouy, R., Petropoulou, T., Roesler, J., Seger, R., Stasia, M.-J., Valerius, N. H., Weening, R. S., Wolach, B., Roos, D., & Kuijpers, T. W. (2009). Chronic Granulomatous Disease: The European Experience. PLoS ONE. https://doi.org/10.1371/journal.pone.0005234
Data sources
Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
