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
| Demographic and clinical markers | Category | DI (1990–2000) | DII (2000–2010) | p |
|---|---|---|---|---|
| Number of patients | 104 | 181 | ||
| Age | 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 | |||
| Sex | Male | 53.8% | 49.7% | 0.539 |
| Female | 46.2% | 50.3% | ||
| Ethnicity | Caucasian | 93.3% | 92.0% | 0.818 |
| NonCaucasian | 6.7% | 8.0% | ||
| Consanguineous parents | 6.2% | 1.1% | 0.054 | |
| Manifestation | Respiratory | 89.4% | 91.7% | 0.528 |
| Digestive | 59.6% | 83.3% | <0.001 | |
| Onset of symptoms | Mean: 16 months | Mean: 91.75 months | <0.001 | |
| Median: 3 months | Median: 3 months | |||
| Range: 0–20 years | Range: 0–60 years | |||
| Age at diagnosis | 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 | |||
| Meconium ileus | 5.8% | 15.0% | 0.021 | |
| Diabetes mellitus | 4.8% | 18.5% | 0.001 | |
| Nutritional status | Weight below 10th percentile | 69.9% | 35.71% | <0.001 |
| Height below 10th percentile | 56.6% | 40.82% | 0.025 | |
| SpO2 | >95% | 59.5% | 55.5% | 0.713 |
| 91%–95% | 32.9% | 34.7% | ||
| <91% | 7.6% | 9.8% | ||
| Sweat test | <60 mEq/L* | 10.6% | – | |
| 60–100 mEq/L | 28.8% | 40.51% | ||
| >100 mEq/L | 60.6% | 59.49% | ||
| Bacteria | 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 | |
| Spirometry | 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% | ||
| CFTR mutation | 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% | – | ||
| Shwachman-Kulczycki score | Excellent or good | 57.8% | 36.2% | 0.005 |
| Mild | 26.5% | 36.2% | ||
| Moderate or severe | 15.7% | 27.6% | ||
| Deaths | 18 | 31 | 1 | |
| Fecal balance | 67.9% | 80.0% | 0.031 | |
DI – period from 1990 to 2000; DII – period from 2000 to 2010; SpO2 – transcutaneous hemoglobin saturation by oxygen; p – p-value. Statistical analysis was performed by the χ2 test. Statistically significant values are indicated by bold font. *Patients with two identified CFTR mutations.