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1 Kathy and Lee Graub Cystic Fibrosis Center and Pulmonary Unit, and 3 Infectious Disease Unit, Schneider Children's Medical Center of Israel, Petah Tikva and the Sackler School of Medicine, Tel Aviv University, Tel Aviv, and 2 Cystic Fibrosis Center and Paediatric Pulmonary Unit, and 4 Dept of Paediatrics, Carmel Medical Center, Faculty of Medicine, Technion, Haifa, Israel
CORRESPONDENCE: H. Blau, Graub CF Center and Pulmonary Unit, Schneider Children's Medical Center of Israel, 14 Kaplan Street, Petah Tikva, 49202, Israel. Fax: 972 39253147. E-mail: hblau@post.tau.ac.il
Keywords: Allergic bronchopulmonary aspergillosis, cystic fibrosis, Mycobacterium abscessus, nontuberculous mycobacteria, pulmonary infection, steroid therapy
Received: May 16, 2004
Accepted October 9, 2004
Nontuberculous mycobacterial (NTM) infection, particularly due to Mycobacterium abscessus, is an emerging disease that can be relentlessly progressive, particularly in cystic fibrosis (CF) patients. The risk factors that were associated with this increasingly symptomatic infection in a group of CF patients were investigated.
A total of 139 CF patients aged 2–52 yrs were reviewed. Sputum was cultured for NTM annually or whenever clinical deterioration was unexplained.
In total, 12 patients (8.6%) had positive cultures and six (4.3%) met the criteria for NTM pulmonary disease (five with M. abscessus). Five had allergic bronchopulmonary aspergillosis (ABPA) compared with one out of 133 patients without NTM disease. Five had received systemic steroids (four as a treatment for ABPA) compared with only one out of 133 without NTM lung disease. All six NTM patients deteriorated markedly following mycobacterial infection, and forced expiratory volume in one second dropped 18–46%. Despite prolonged triple antibiotic therapy, M. abscessus was not eradicated, and four out of six did not return to baseline clinically.
In conclusion, severe nontuberculous mycobacterial lung disease, particularly with Mycobacterium abscessus, is becoming a perplexing challenge in cystic fibrosis patients. Allergic bronchopulmonary aspergillosis and systemic steroids appear to be risk factors, although small patient numbers limit this to a descriptive observation. When pulmonary condition deteriorates, increased surveillance for mycobacteria would enable prompt diagnosis and treatment.
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