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© Therese Couderc, Marc Lecuit
Publication : Clinical infectious diseases : an official publication of the Infectious Diseases Society of America

Chronic Granulomatous Disease in Patients Reaching Adulthood: A Nationwide Study in France

Scientific Fields
Diseases
Organisms
Applications
Technique

Published in Clinical infectious diseases : an official publication of the Infectious Diseases Society of America - 15 Mar 2017

Dunogué B, Pilmis B, Mahlaoui N, Elie C, Coignard-Biehler H, Amazzough K, Noël N, Salvator H, Catherinot E, Couderc LJ, Sokol H, Lanternier F, Fouyssac F, Bardet J, Bustamante J, Gougerot-Pocidalo MA, Barlogis V, Masseau A, Durieu I, Lecuit M, Suarez F, Fischer A, Blanche S, Hermine O, Lortholary O

Link to Pubmed [PMID] – 28362954

Clin. Infect. Dis. 2017 03;64(6):767-775

Background: Although prognosis of Chronic Granulomatous Disease (CGD) has greatly improved, few studies have focused on its long-term outcome. We studied the clinical course and sequelae of CGD patients diagnosed before age 16, at various adult time points.

Method: Cross-sectional French nationwide retrospective study of patients screened through the National Reference Center for Primary Immunodeficiencies (CEREDIH) registry.

Results: Eighty CGD patients (71 males [88.7%], 59 X-linked [73.7%], median age 23.9 years [minimum, 16.6; maximum, 59.9]) were included, Median ages at diagnosis and last follow-up were 2.52 and 23.9 years, respectively. Seven patients underwent hematopoietic stem cell transplantation. A total of 553 infections requiring hospitalization occurred in 2017 patient-years. The most common site of infection was pulmonary (31%). Aspergillus spp. (17%) and Staphylococcus aureus (10.7%) were the commonest pathogens. A total of 224 inflammatory episodes occurred in 71 patients, mainly digestive (50%). Their characteristics as well as their annual frequency did not vary before and after age 16. Main sequelae were a small adult height and weight and mild chronic restrictive respiratory failure. At age 16, only 53% of patients were in high school. After age 30 years, 9/13 patients were working. Ten patients died during adulthood.

Conclusions: Adult CGD patients displayed similar characteristics and rates of severe infections and inflammatory episodes that those of childhood. The high rate of handicap has become a matter of medical and social consideration. Careful follow-up in centers of expertise is strongly recommended and an extended indication of curative treatment by HSCT should be considered.

https://www.ncbi.nlm.nih.gov/pubmed/28362954