Demographic, Clinical, and Lab Parameters of Cystic Fibrosis
Table 1 compares the demographic, clinical, and laboratory variables of the patients with CF enrolled in this study in the two above-described decades.
The following markers showed an association between the two decades: digestive symptoms, comorbidities, height and weight, microorganisms, spirometry, SKS, F508del screening, and age range (Table 2, Table 3, Table 4 and Table 5).
An overview of all data analyzed is shown in Figure 1. Comparison of DI with DII revealed the following in DI:
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Figure 1.
Clinical outcome variables the between two decades of data for patients with cystic fibrosis from a Brazilian referral center. SpO2, − transcutaneous hemoglobin saturation by oxygen; SKS, − Shwachman-Kulczycki score; M and NM, − mucoid and nonmucoid.
Figure 2 shows the age at onset of clinical symptoms (Figure 2A) and at diagnosis (Figure 2B).
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Figure 2.
Distribution of patients with cystic fibrosis according to ages at onset of symptoms and diagnosis. A. Comparison of the distribution of patients with cystic fibrosis according to age at onset of symptoms between decades I (n = 95) and II (n = 181). B. Comparison of distribution of patients with cystic fibrosis according to age at diagnosis between decades I (n = 100) and II (n = 180).
Results
Table 1 compares the demographic, clinical, and laboratory variables of the patients with CF enrolled in this study in the two above-described decades.
The following markers showed an association between the two decades: digestive symptoms, comorbidities, height and weight, microorganisms, spirometry, SKS, F508del screening, and age range (Table 2, Table 3, Table 4 and Table 5).
An overview of all data analyzed is shown in Figure 1. Comparison of DI with DII revealed the following in DI:
(Enlarge Image)
Figure 1.
Clinical outcome variables the between two decades of data for patients with cystic fibrosis from a Brazilian referral center. SpO2, − transcutaneous hemoglobin saturation by oxygen; SKS, − Shwachman-Kulczycki score; M and NM, − mucoid and nonmucoid.
fewer patients with digestive disease (OR = 0.295, 95% CI = 0.17–0.51);
fewer patients with MI (OR = 0.35, 95% CI = 0.11–0.91) and DM (OR = 0.228, 95% CI = 0.07–0.61);
fewer patients with an altered fat balance (OR = 0.534, 95% CI = 0.31–0.93);
more patients below the 10th percentile for weight (OR = 4.21, 95% CI = 2.34–7.67) and height (OR = 1.895, 95% CI = 1.08–3.34);
a higher frequency of P. aeruginosa (OR = 2.495, 95% CI = 1.42–4.48) and concurrent mucoid and nonmucoid P. aeruginosa (OR =3.65, 95% CI = 2.11–6.38);
a lower frequency of B. cepacia (OR = 0.302, 95%CI = 0.09–0.84);
a higher frequency of mixed respiratory disorders (OR = 17.58, 95% CI = 4.70–98.87) and a lower frequency of restrictive respiratory disorders (OR = 0.231, 95% CI = 0.09–0.51);
a lower frequency of moderate and severe SKS (OR = 0.491, 95% CI = 0.24–0.96) and a higher frequency of excellent and good SKS (OR = 2.403; 95% CI = 1.40–4.16);
for F508del a heterozygosity mutation was more frequent (OR = 5.445, 95% CI = 3.09–9.74) in CF patients with two CFTR mutations screened and less frequent for patients without an identified CFTR mutation (OR = 0.235, 95% CI = 0.13–0.42); and
a higher frequency of patients in preschool (OR = 2.602, 95% CI = 1.38–4.94) and fewer adult patients (OR = 0.293, 95% CI = 0.13–0.60).
Figure 2 shows the age at onset of clinical symptoms (Figure 2A) and at diagnosis (Figure 2B).
(Enlarge Image)
Figure 2.
Distribution of patients with cystic fibrosis according to ages at onset of symptoms and diagnosis. A. Comparison of the distribution of patients with cystic fibrosis according to age at onset of symptoms between decades I (n = 95) and II (n = 181). B. Comparison of distribution of patients with cystic fibrosis according to age at diagnosis between decades I (n = 100) and II (n = 180).
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