Dragosavac, D.

Gonçcalves, J.R.

Metze, K.

Moreira, M.M.

Paschoal, I.A.

Pereira, M.C.

Piza, S.T.

Terzi, R.G.

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155* Noninvasive evaluation of pulmonary disease using volumetric capnography in adult patients with cystic fibrosis 

S38 6. Pulmonology
155* Noninvasive evaluation of pulmonary disease using volumetric
capnography in adult patients with cystic ﬁbrosis
I.A. Paschoal, M.M. Moreira, M.C. Pereira, S.T. Piza, J.R. Gonc¸alves, K. Metze,
D. Dragosavac, R.G. Terzi. Internal Medicine, UNICAMP, Campinas, Sa˜o Paulo,
Brazil
Volumetric capnography is a technique that analyses the pattern of CO2 elim-
ination as a function of expired volume. Expired gas receives CO2 from three
sequential compartments forming three recognizible phases on the capnogram.
Phase 1 contains gas from proximal conducting airways. Phase 2 represents
the transitional region characterized by an increasing CO2 concentration, and
phase 3 represents alveolar gas (alveolar plateau). Here we present the results of
volumetric capnography in 114 normal controls (Group 1) and 11 adult patients
with cystic ﬁbrosis (Group 2) with two abnormal sweat chloride concentrations.
Age was 33.6±11.4 (1) and 34.3±11.7 (2). All patients had severe pulmonary
involvement: FVC= 57.9±25.9; FEV1= 41.9±23.4; FEV1%=58.4±10.6. All of
them had diffuse bronchiolitis and bronchiectasis on HRCT. Capnography revealed
in Groups 1 and 2, respectively: respiratory rate (RR) = 12.9±3.5 and 20.6±8.4,
p = 0.001; expiratory volume (Ve): 693.2±245.9 and 537.1±151.5, p = 0.03. Peak
expiratory ﬂow (PEF): 26.5±8.9 and 30.1±7.8, p = 0.11. Phase3 slope (P3Slp):
7.80±2.36 and 43.56±31.95, p< 0.001. P3Slp/Ve = 0.014±0.007 and 0.124±0.144,
p< 0.001. Expiratory time (Te): 3.1±1.1 and 2.1±1.5, p< 0.001. Patients had
obstructive defects with concomitant low vital capacities. Values of capnography
in patients suggest a restrictive respiratory pattern. A large phase 3 slope was
found in patients. Gas transport by diffusion is the dominant mechanism in acinar
spaces. Steepened phase 3 slopes may represent increased diffusional resistance in
peripheral lung. Small tidal volumes also produce steeper slopes. Phase 3 slope/Ve
was still signiﬁcantly greater in patients. These alterations can be expected in lungs
with diffuse bronchiectasis.
Supported by: UNICAMP.
156* Lung function in children with CF – from infants to seven years
old
A. Geborek1,2, L. Hjelte1,2, L. Lagerstrand3. 1Stockholm CF Center, Karolinska
Univ Hospital Huddinge, Stockholm, Sweden; 2Dep of Clinical Science,
Intervention & Technology, Karolinska Institutet, Stockholm, Sweden; 3Dep of
Clinical Physiology, Karolinska Univ Hospital Huddinge, Stockholm, Sweden
Aim: To investigate if changes in infant lung function testing (IFLT) correlate with
changes later in childhood measured with pulmonary function test (PFT).
Patients: Thirty ﬁve patients (15 girls) from Stockholm CF Center, born between
1992 and 2001, who in infancy had undergone IFLT and later in childhood
performed a PFT were included in the study. V’max FRC (maximal ﬂow at
functional residual capacity) was measured in infancy, and FEV1, FVC and FEF75%
(forced expiratory ﬂow) with PFT.
Results: Infant lung function testing (mean age 2.2 yrs (range 3m–3 yrs 3m)): Mean
V’max FRC in % of predicted 80 (29–152)%. 71% of the ﬂow volume charts had
a normal appearance.
PTF (mean age 6.1 (3.5−8.7) yrs): Mean FEV1 87 (30–116)%, mean FVC 92 (34–
121)%, and mean FEF75% 88 (30–201)%.
When comparing the result from each patient’s IFLT with their PFT at the age
of 7, or their latest spirometry if they were <7 yrs old, we found no signiﬁcant
differences between V’max FRC and FEV1, FVC or FEF75%. 11/13 patients with
V’max FRC <70% in infancy had a FEF75% below the PFT’s normal mean value.
Discussion: FEF75% has in previous studies been found to show the earliest signs
of pulmonary detoriation. The function of V’max FRC corresponds to FEF75% and
can thus be an early and sensitive predictor of lung function changes. In our study
V’max FRC showed a concordance with FEF75%, especially if V’max FRC was
below 70%, though the reproducibility of FEF75% is not comparable with that of
FEV1.
Conclusion: In our study infant lung function testing was comparable to PFT in
preschool children.
157 Monitoring of Dornase alfa treatment efﬁcacy for children with
cystic ﬁbrosis
S. Dumcius, D. Kazakevicius, I. Gasiunaite. Dpt. Children diseases, Vilnius
University Hospital, Vilnius, Lithuania
Objective: Dornase alfa has become an established treatment in patients with cystic
ﬁbrosis.
Clinical trials have shown that Dornase alfa (Pulmozyme) may improve pulmonary
functions, reduce infective episodes and change life style.
The aim of our study was to evaluate effect of Dornase alfa treatment on quality
of life and pulmonary functions in CF patiens.
Medical records of 24 children (mean age 9.6 years, FVC> 40%) were revised. All
patients received 2.5mg dornase alfa daily for 3 month by nebulisation. Recordings
of FVC and FEV1 were taken at the start and end of Dornase alfa therapy. The
number of respiratory exacerbation episodes was compared during last 12 month
before and after treatment.
Results: Prior to every-day Dornase alfa treatment our patients were hospitalised
averagely 2.1 yearly, as compared to 0.9 times after it (p< 0.05). Improvement of
FEV1 after 3 month by mean was 9.97%, FVC by 7.03%. This improvement was
maintained during 12 month after treatment.
Conclusion: Dornase alfa treatment signiﬁcantly reduced the number of infections
and hospitalisation rate among our patients. Inhalation therapy has a positive impact
on pulmonary functions and children s well being. This treatment reduces hospital
cost of CF patients treatment.
158 Nocturnal cough frequency in children with cystic ﬁbrosis
L.J. van der Giessen1, M. Loeve2, W.C. Hop3, H.A. Tiddens2. 1Department
of Pediatric Physiotherapy, Erasmus MC – Sophia, Rotterdam, Netherlands;
2Department of Pediatrics, Respiratory Medicine and Allergology, Erasmus MC –
Sophia, Rotterdam, Netherlands; 3Department of Epidemiology & Biostatistics,
Erasmus MC, Rotterdam, Netherlands
Introduction: To date no studies have been published on cough frequency during
sleep in children with stable CF.
Aim: To assess the cough frequency during sleep in children with CF. In addition
cough frequency was correlated to parameters of disease severity
Methods: During two nights cough was recorded with a digital audio recorder at the
patient’s home in twenty-ﬁve patients (mean age 13; range 6−19) with clinically
stable CF. In addition oxygen saturation was measured. The day following the
recording spirometry was carried out. CT scores were obtained from the most
recent routine CT scan done at the annual evaluation. Cough was expressed in
cough seconds (cs) and in cough seconds per hour (cs/h) and as median values
and interquartile range (IQR). Spearman rank correlation coefﬁcient (rs) and the
Wilcoxon Rank test were used.
Results: First night: 8 cs (IQR 3−52); 0.9 cs/h (IQR 0.3−6.1) Second night: 6 cs
(IQR 2−32); 0.6 cs/h (IQR 0.1−3.4). Cs and the cs/h from the ﬁrst night did not
correlate with the cs (rs = 0.29, p = 0.20) or the cs/h (rs = 0.35, p = 0.124) from the
second night. For the 2nd night we found a strong correlation between cs/h and
the FEV1%pred (rs = −0.75, p< 0.000), MEF25%pred (rs = −0.71, p< 0.000) and
the HRCT score (rs = 0.48, p< 0.027). During both nights cough was signiﬁcantly
worse in the ﬁrst hour of sleep (p 0.037).
Conclusion: In conclusion the cough frequency in children with clinically stable
CF is more severe in patients with more advanced disease. Cough is worse in the
ﬁrst hour of sleep and varies from night-to-night.


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155* Noninvasive evaluation of pulmonary disease using volumetric capnography in adult patients with cystic fibrosis

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