Table 1 Comparison of data (demographic, clinical, and laboratory markers) of patients with cystic fibrosis from a Brazilian referral center during the decades of 1990 to 2000 and 2000 to 2010

104

181

Mean: 10 years 9 months ± 6.33 months

Mean :16 years 7 months ± 13.16 months

<0.001

Median: 9 years

Median: 12 years 10 months

Range: 11 months to 31 years 2 months

Range: 6 months to 73 years 10 months

Male

53.8%

49.7%

0.539

Female

46.2%

50.3%

Caucasian

93.3%

92.0%

0.818

NonCaucasian

6.7%

8.0%

6.2%

1.1%

0.054

Respiratory

89.4%

91.7%

0.528

Digestive

59.6%

83.3%

<0.001

Mean: 16 months

Mean: 91.75 months

<0.001

Median: 3 months

Median: 3 months

Range: 0–20 years

Range: 0–60 years

Mean: 4 years 2 months

Mean: 2 years 10 months

<0.001

Median: 2 years 4 months

Median: 2 years

Range: 0 to 29 years 11 months

Range: 0–60 years

5.8%

15.0%

0.021

4.8%

18.5%

0.001

Weight below 10th percentile

69.9%

35.71%

<0.001

Height below 10th percentile

56.6%

40.82%

0.025

>95%

59.5%

55.5%

0.713

91%–95%

32.9%

34.7%

<91%

7.6%

9.8%

<60 mEq/L*

10.6%

–

60–100 mEq/L

28.8%

40.51%

>100 mEq/L

60.6%

59.49%

Staphylococcus aureus

80.2%

78.5%

0.880

Pseudomonas aeruginosa

76.0%

55.8%

0.001

Mucoid P. aeruginosa

53.1%

42.0%

0.085

Burkholderia cepacia

5.2%

14.4%

0.016

Mucoid and nonmucoid P. aeruginosa

51.0%

21.85%

<0.001

Normal

27.3%

34.4%

<0.001

Restrictive ventilatory disorder

18.2%

48.9%

Obstructive lung disorder

25.4%

14.5%

Mixed respiratory disorder

29.1%

2.3%

F508del homozygotes

18.75%

26.5%

<0.001

F508del heterozygotes

62.5%

22.7%

G542X

4.17%

6.45%

N1303K

2.08%

1.1%

G551D

1.04%

–

R553X

0.52%

0.3%

W1282X

0.52%

–

Excellent or good

57.8%

36.2%

0.005

Mild

26.5%

36.2%

Moderate or severe

15.7%

27.6%

18

31

1

67.9%

80.0%

0.031