OBJECTIVE: To identify cystic fibrosis (CF) patients with a history of meconium ileus (MI) and to compare long-term outcome of these patients with CF patients without MI. PATIENTS: 370 CF patients who were treated at the Munich CF centre in 2006, mean age 17.1 years (range: 0-48), were classified with regard to a history of MI. METHODS: Retrospective chart analysis regarding history of MI, age, and lung function and first detection of Pseudomonas aeruginosa as the primary outcome variables. RESULTS: We identified 60 patients with MI (16.2% of all patients with a mean age of 16.2 +/- 9 years (0-41) who did not differ in age from patients without MI (17.3 +/- 10; 0-48), whereas the mean age of CF diagnosis was different (0.4 vs. 21.8 months, P < 0.001). Cross sectional analysis of best Forced expiratory volume (FEV% predicted) in 2006 was not different between patients with MI (89 +/- 23%, 17-134) and those without (88 +/- 27%, 21-148, P = 0.73). Longitudinal analysis using FEV1 values of more than 9,000 lung function tests also showed no difference in FEV1 although first detection of P. aeruginosa in patients with MI was significantly earlier. CONCLUSION: MI was associated with hospitalization, surgery, and early infection with P. aeruginosa. However, the long-term outcome of CF patients with or without MI was the same. This finding might be explained by the considerably earlier CF diagnosis in CF patients with MI including the benefit of an early initiation of appropriate CF therapy.
OBJECTIVE: To identify cystic fibrosis (CF) patients with a history of meconium ileus (MI) and to compare long-term outcome of these patients with CFpatients without MI. PATIENTS: 370 CFpatients who were treated at the Munich CF centre in 2006, mean age 17.1 years (range: 0-48), were classified with regard to a history of MI. METHODS: Retrospective chart analysis regarding history of MI, age, and lung function and first detection of Pseudomonas aeruginosa as the primary outcome variables. RESULTS: We identified 60 patients with MI (16.2% of all patients with a mean age of 16.2 +/- 9 years (0-41) who did not differ in age from patients without MI (17.3 +/- 10; 0-48), whereas the mean age of CF diagnosis was different (0.4 vs. 21.8 months, P < 0.001). Cross sectional analysis of best Forced expiratory volume (FEV% predicted) in 2006 was not different between patients with MI (89 +/- 23%, 17-134) and those without (88 +/- 27%, 21-148, P = 0.73). Longitudinal analysis using FEV1 values of more than 9,000 lung function tests also showed no difference in FEV1 although first detection of P. aeruginosa in patients with MI was significantly earlier. CONCLUSION: MI was associated with hospitalization, surgery, and early infection with P. aeruginosa. However, the long-term outcome of CFpatients with or without MI was the same. This finding might be explained by the considerably earlier CF diagnosis in CFpatients with MI including the benefit of an early initiation of appropriate CF therapy.
Authors: Weili Li; David Soave; Melissa R Miller; Katherine Keenan; Fan Lin; Jiafen Gong; Theodore Chiang; Anne L Stephenson; Peter Durie; Johanna Rommens; Lei Sun; Lisa J Strug Journal: Hum Genet Date: 2013-09-22 Impact factor: 4.132
Authors: Lindsay B Henderson; Vishal K Doshi; Scott M Blackman; Kathleen M Naughton; Rhonda G Pace; Jackob Moskovitz; Michael R Knowles; Peter R Durie; Mitchell L Drumm; Garry R Cutting Journal: PLoS Genet Date: 2012-03-15 Impact factor: 5.917
Authors: Arul Earnest; Farhad Salimi; Claire E Wainwright; Scott C Bell; Rasa Ruseckaite; Tom Ranger; Tom Kotsimbos; Susannah Ahern Journal: Sci Rep Date: 2020-10-15 Impact factor: 4.379