BACKGROUND: No studies longitudinally, simultaneously assessed body mass index (BMI) and spirometry in primary ciliary dyskinesia (PCD). METHODS: We determined BMI and spirometry in 158 PCD children and adolescents from London, UK (n = 75), Naples, Italy (n = 23) and Copenhagen, Denmark (n = 60) at first presentation and during follow-up. Annual BMI and spirometry were prospectively collected and analyzed over blocks of 2, 4, and 6 consecutive years. Sputum pathogens were recorded. RESULTS: Age at first spirometry was 8.7 years (range, 4.2-17.4). Mean Z scores of first measured BMI, FEV1, FVC, and FEF(25-75) were 0.01, -1.37, -0.84, and -1.68, respectively. First spirometry was not more frequently impaired in patients referred at age ≥6 years than in those referred at preschool age (P = 0.13). There were no differences in slopes for BMI, FEV1, FVC, or FEF(25-75) over any time block. H. influenzae was the most common pathogen, isolated at least once in 65% of patients. P. aeruginosa was found in 58 subjects (37%) of whom 8 (5%) were chronically infected. Neither pathogens was associated with spirometry changes. CONCLUSIONS: Preschool referral to a PCD center was not associated with better spirometry or BMI. PCD children and adolescents receiving centralized care show steady BMI and spirometry during medium term follow-up. There was a high prevalence of Pseudomonas aeruginosa infection, but the evolution of spirometry or BMI was not affected by this microorganism in medium term. Despite our longitudinal analysis showed no differences between the three centers, the assessment of spirometry and BMI over time represents a quality improvement tool. Future studies are needed to highlight the role of spirometry and BMI in long term PCD management and identify subgroups of patients with a higher risk of early lung failure or nutritional problems.
BACKGROUND: No studies longitudinally, simultaneously assessed body mass index (BMI) and spirometry in primary ciliary dyskinesia (PCD). METHODS: We determined BMI and spirometry in 158 PCDchildren and adolescents from London, UK (n = 75), Naples, Italy (n = 23) and Copenhagen, Denmark (n = 60) at first presentation and during follow-up. Annual BMI and spirometry were prospectively collected and analyzed over blocks of 2, 4, and 6 consecutive years. Sputum pathogens were recorded. RESULTS: Age at first spirometry was 8.7 years (range, 4.2-17.4). Mean Z scores of first measured BMI, FEV1, FVC, and FEF(25-75) were 0.01, -1.37, -0.84, and -1.68, respectively. First spirometry was not more frequently impaired in patients referred at age ≥6 years than in those referred at preschool age (P = 0.13). There were no differences in slopes for BMI, FEV1, FVC, or FEF(25-75) over any time block. H. influenzae was the most common pathogen, isolated at least once in 65% of patients. P. aeruginosa was found in 58 subjects (37%) of whom 8 (5%) were chronically infected. Neither pathogens was associated with spirometry changes. CONCLUSIONS: Preschool referral to a PCD center was not associated with better spirometry or BMI. PCDchildren and adolescents receiving centralized care show steady BMI and spirometry during medium term follow-up. There was a high prevalence of Pseudomonas aeruginosa infection, but the evolution of spirometry or BMI was not affected by this microorganism in medium term. Despite our longitudinal analysis showed no differences between the three centers, the assessment of spirometry and BMI over time represents a quality improvement tool. Future studies are needed to highlight the role of spirometry and BMI in long term PCD management and identify subgroups of patients with a higher risk of early lung failure or nutritional problems.
Authors: Stephanie D Davis; Thomas W Ferkol; Margaret Rosenfeld; Hye-Seung Lee; Sharon D Dell; Scott D Sagel; Carlos Milla; Maimoona A Zariwala; Jessica E Pittman; Adam J Shapiro; Johnny L Carson; Jeffrey P Krischer; Milan J Hazucha; Matthew L Cooper; Michael R Knowles; Margaret W Leigh Journal: Am J Respir Crit Care Med Date: 2015-02-01 Impact factor: 21.405
Authors: Katharina Schütz; Diana Alecsandru; Bodo Grimbacher; Jamanda Haddock; Annemarie Bruining; Gertjan Driessen; Esther de Vries; Peter M van Hagen; Ieneke Hartmann; Francesco Fraioli; Cinzia Milito; Milica Mitrevski; Isabella Quinti; Goffredo Serra; Peter Kelleher; Michael Loebinger; Jiri Litzman; Vera Postranecka; Vojtech Thon; Judith Babar; Alison M Condliffe; Andrew Exley; Dinakantha Kumararatne; Nick Screaton; Alison Jones; Maria P Bondioni; Vassilios Lougaris; Alessandro Plebani; Annarosa Soresina; Cesare Sirignano; Giuseppe Spadaro; Nermeen Galal; Luis I Gonzalez-Granado; Sabine Dettmer; Robert Stirling; Helen Chapel; Mary Lucas; Smita Patel; Claire-Michele Farber; Isabelle Meyts; Arpan K Banerjee; Scott Hackett; John R Hurst; Klaus Warnatz; Benjamin Gathmann; Ulrich Baumann Journal: J Clin Immunol Date: 2018-12-13 Impact factor: 8.317
Authors: Stephanie D Davis; Margaret Rosenfeld; Hye-Seung Lee; Thomas W Ferkol; Scott D Sagel; Sharon D Dell; Carlos Milla; Jessica E Pittman; Adam J Shapiro; Kelli M Sullivan; Keith R Nykamp; Jeffrey P Krischer; Maimoona A Zariwala; Michael R Knowles; Margaret W Leigh Journal: Am J Respir Crit Care Med Date: 2019-01-15 Impact factor: 30.528
Authors: Virginia Mirra; Carlo Caffarelli; Marco Maglione; Rossella Valentino; Giuseppe Perruolo; Claudia Mazzarella; Laida Lisa Di Micco; Silvia Montella; Francesca Santamaria Journal: Ital J Pediatr Date: 2015-02-22 Impact factor: 2.638
Authors: Odin Joensen; Tamara Paff; Eric G Haarman; Ib M Skovgaard; Peter Ø Jensen; Thomas Bjarnsholt; Kim G Nielsen Journal: PLoS One Date: 2014-12-26 Impact factor: 3.240