The History of Cystic Fibrosis by Dr James Littlewood OBE

1984 Efthimiou J, Smith MJ, Hodson ME, Batten JC. Fatal pulmonary infection Mycobacterium fortuitum in cystic fibrosis. Br J Dis Chest 1984; 78:299-302. [PubMed]
An early, probably the first, report of an atypical Mycobacterium infection in a young adult with cystic fibrosis. The organism was resistant to all antibiotics and the patient died. Atypical mycobacteria were to become an increasing problem in people with CF. In a subsequent paper from Royal Brompton Smith MJ et al (1984 below) made a search to determine the prevalence of atypical mycobacteria in their CF patients – presumably as a result of experience with this patient. (Also Boxerbaum B. Isolation of rapidly growing mycobacteria in patients with cystic fibrosis. J Pediatr 1980; 96:689-691).

1984 Smith MJ, Efthimiou J, Hodson ME, Batten JC. Mycobacterial isolations in young adults with cystic fibrosis. Thorax 1984; 39:369-375. [PubMed]
Seven of 223 patients with CF admitted to the Brompton Hospital over a six year period had Mycobacteria in their sputum. The organisms isolated were Mycobacterium tuberculosis in three patients, M. chelonei in one, M. fortuitum in one, and unidentified Mycobacteria in two. The diagnosis was not suspected on clinical grounds in any of these patients; in one patient, however, night sweats were a prominent feature before diagnosis. In four of the patients direct sputum smear examination did not reveal the organism, which was grown subsequently in culture.

These were early days for appreciating the role of atypical Mycobacteria in CF and the organisms were present in the sputa of patients with cystic fibrosis more often than previously recognised. Therefore the authors recommended that sputum examination and culture for Mycobacteria should be performed periodically in these patients. Subsequently problems with these organisms would become more widely recognised and occur as a problem sometimes after lung transplantation when the patients were on immunosuppressive therapy.
Probably as there were fewer adults at that time and so few CF centres for adults, there were no further reports until one from Dublin in 1990 (Mulherin D, et al. Respir Med 1990; 84:273-276) where the frequency of positive skin reactions was found to be similar as in a control population. One of 43 patients grew an atypical mycobacterium from the sputum.

2009 Radhakrishnan DK. Yau Y. Corey M. Richardson S. Chedore P. Jamieson F. Dell SD. Non-tuberculous mycobacteria in children with cystic fibrosis: isolation, prevalence, and predictors. Pediatr Pulmonol 2009; 44:1100-1106. [PubMed]
Screening for non-tuberculous mycobacteria (NTM) is recommended for adults with cystic fibrosis (CF). The relevance of this organism in North American pediatric CF patients is unclear as there is limited NTM prevalence data for children. 6.1% of 98 children at the hospital for Sick Children Toronto were positive for NTM - 2 M abscessus and 4 m. avium - one with M abscessus required treatment. As the NTM prevalence rate in children with CF is within the range previously reported in adults and there are no reliable clinical predictors for isolation, annual sputum screening is needed to identify NTM in children. Further research is needed to determine the best sputum decontamination method for NTM culture in pediatric patients.

This is a useful study from Toronto detailing the prevelance of NTM in their paediatric CF population - which they found to be similar to that in adult s..

2009 van Ingen J. de Zwaan R. Dekhuijzen RP. Boeree MJ. van Soolingen D. Clinical relevance of Mycobacterium chelonae-abscessus group isolation in 95 patients. J Infect 2009; 59:324-331. [PubMed]
To determine the clinical relevance of Mycobacterium chelonae-abscessus group isolation from clinical samples we retrospectively reviewed medical files of all patients from whom these mycobacteria were isolated between January 1999 and January 2005. We applied the American Thoracic Society (ATS) diagnostic criteria to establish clinical relevance. Ninety-five patients were traced (56 M. chelonae, 25 Mycobacterium abscessus, 8 Mycobacterium massiliense, 6 Mycobacterium bolletii). Most isolates were cultured from pulmonary samples in patients with pre-existing pulmonary disease. Among patients with pulmonary isolates, 27% (20/74) meets ATS criteria; M. abscessus is most relevant (50%; 9/18), followed by M. massiliense (29%; 2/7), M. bolletii (20%; 1/5) and M. chelonae (18%; 8/44). Extrapulmonary disease presented as disseminated skin disease, eye disease specific for M. chelonae and otomastoiditis for M. abscessus. Treatment, especially for pulmonary M. abscessus disease, yielded limited results. One-fourth of the patients with pulmonary M. chelonae-abscessus group isolates met the ATS criteria; this percentage differs by species. Species distribution and clinical relevance differ from other regions. M. abscessus isolation in cystic fibrosis patients warrants special attention. Current ATS criteria might be too lenient to diagnose M. chelonae-abscessus group disease.

Increasing interest in NTM in CF.This survey gives an idea of the general prevalence of the various NTMs.

2009 Roux AL. Catherinot E. Ripoll F. Soismier N. Macheras E. Ravilly S. Bellis G. Vibet MA. Le Roux E. Lemonnier L. Gutierrez C. Vincent V. Fauroux B. Rottman M. Guillemot D. Gaillard JL. Jean-Louis Herrmann for the OMA Group. Multicenter study of prevalence of nontuberculous mycobacteria in patients with cystic fibrosis in france. J Clin Microbiol 2009; 47:4124-4128. [PubMed]
A multicenter prevalence study of nontuberculous mycobacteria (NTM) involving 1,582 patients (mean age, 18.9 years; male/female ratio, 1.06) with cystic fibrosis in France. The overall NTM prevalence (percentage of patients with at least one positive culture) was 6.6% (104/1,582 patients), with prevalences ranging from 3.7% (in the east of France) to 9.6% (in the greater Paris area). Mycobacterium abscessus complex (MABSC; 50 patients) and Mycobacterium avium complex (MAC; 23 patients) species were the most common NTM, and the only ones associated with fulfillment of the American Thoracic Society bacteriological criteria for NTM lung disease. The "new" species, Mycobacterium bolletii and Mycobacterium massiliense, accounted for 40% of MABSC isolates. MABSC species were isolated at all ages, with a prevalence peak between 11 and 15 years of age (5.8%), while MAC species reached their highest prevalence value among patients over 25 years of age (2.2%).

A useful large survey of NTM in France showing an overall prevelance of 6.6%.- very similar to previous reports.