Clinical characteristics of cystic fibrosis patients
Patient . | Age, y . | Vitamin E, mg/L* . | FEV1, % predicted† . | Inpatient or outpatient‡ . | Genotype . | Platelet studies§ . |
|---|---|---|---|---|---|---|
| 1 | 20 | 6.6 | 25 | In | δF508/unk | A |
| 2 | 20 | 3.6 | 70 | In | δF508/G542X | A |
| 3 | 11 | 16.8 | 92 | Out | δF508/dF508 | A |
| 4 | 16 | 5.4 | 101 | Out | δF508/G542X | A |
| 5 | 9 | 3.9 | 124 | Out | δF508/dF508 | A,F |
| 6 | 6 | 5.1 | 118 | Out | δF508/dF508 | A,F |
| 7 | 13 | 8.1 | 119 | Out | δF508/dF508 | A,F |
| 8 | 10 | 9.7 | 104 | Out | δF508/dF508 | A,F |
| 9 | 22 | 9.0 | 58 | In | δF508/dF508 | A |
| 10 | 19 | 8.0 | 57 | Out | δF508/N1303K | A |
| 11 | 17 | 7.0 | 24 | Out | δF508/dF508 | A,D,E |
| 12 | 20 | 3.2 | 55 | Out | δF508/dF508 | A,D |
| 13 | 15 | 5.8 | 41 | In | δF508/dF508 | A,D,E |
| 14 | 26 | 12.7 | 88 | Out | δF508/dF508 | A,D |
| 15 | 11 | 16.3 | 72 | Out | δF508/W1282X | A,D |
| 16 | 18 | 10.0 | 58 | In | δF508/dF508 | A,D |
| 17 | 22 | 10.5 | 50 | Out | δF508/dF508 | A,D |
| 18 | 35 | 8.6 | 87 | Out | δF508/C276X | A,E |
| 19 | 17 | 16.2 | 62 | In | δF508/dF508 | B,E |
| 20 | 14 | 4.1 | 85 | In | δF508/dF508 | B |
| 21 | 22 | 2.3 | 62 | In | δF508/G542X | B |
| 22 | 21 | 7.7 | 54 | In | δF508/N1303K | B |
| 23 | 19 | 2.4 | 69 | In | δF508/Y1092X | B |
| 24 | 19 | 4.6 | 87 | In | δF508/dF508 | B, C, E |
| 25 | 21 | 8.2 | 58 | In | R334W/unk | C |
| 26 | 22 | 5.8 | 85 | In | δF508/dF508 | C,E |
| 27 | 22 | 2.9 | 67 | In | unk/unk | C |
| 28 | 20 | 6.7 | 77 | In | δF508/dF508 | E |
| 29 | 18 | 13.3 | 92 | In | δF508/dF508 | E |
| 30 | 22 | 8.8 | 71 | In | δF508/394delTT | E |
| 31 | 15 | 13.0 | 68 | In | δF508/dF508 | E |
| 32 | 14 | unk | 97 | Out | δF508/dF508 | E |
Patient . | Age, y . | Vitamin E, mg/L* . | FEV1, % predicted† . | Inpatient or outpatient‡ . | Genotype . | Platelet studies§ . |
|---|---|---|---|---|---|---|
| 1 | 20 | 6.6 | 25 | In | δF508/unk | A |
| 2 | 20 | 3.6 | 70 | In | δF508/G542X | A |
| 3 | 11 | 16.8 | 92 | Out | δF508/dF508 | A |
| 4 | 16 | 5.4 | 101 | Out | δF508/G542X | A |
| 5 | 9 | 3.9 | 124 | Out | δF508/dF508 | A,F |
| 6 | 6 | 5.1 | 118 | Out | δF508/dF508 | A,F |
| 7 | 13 | 8.1 | 119 | Out | δF508/dF508 | A,F |
| 8 | 10 | 9.7 | 104 | Out | δF508/dF508 | A,F |
| 9 | 22 | 9.0 | 58 | In | δF508/dF508 | A |
| 10 | 19 | 8.0 | 57 | Out | δF508/N1303K | A |
| 11 | 17 | 7.0 | 24 | Out | δF508/dF508 | A,D,E |
| 12 | 20 | 3.2 | 55 | Out | δF508/dF508 | A,D |
| 13 | 15 | 5.8 | 41 | In | δF508/dF508 | A,D,E |
| 14 | 26 | 12.7 | 88 | Out | δF508/dF508 | A,D |
| 15 | 11 | 16.3 | 72 | Out | δF508/W1282X | A,D |
| 16 | 18 | 10.0 | 58 | In | δF508/dF508 | A,D |
| 17 | 22 | 10.5 | 50 | Out | δF508/dF508 | A,D |
| 18 | 35 | 8.6 | 87 | Out | δF508/C276X | A,E |
| 19 | 17 | 16.2 | 62 | In | δF508/dF508 | B,E |
| 20 | 14 | 4.1 | 85 | In | δF508/dF508 | B |
| 21 | 22 | 2.3 | 62 | In | δF508/G542X | B |
| 22 | 21 | 7.7 | 54 | In | δF508/N1303K | B |
| 23 | 19 | 2.4 | 69 | In | δF508/Y1092X | B |
| 24 | 19 | 4.6 | 87 | In | δF508/dF508 | B, C, E |
| 25 | 21 | 8.2 | 58 | In | R334W/unk | C |
| 26 | 22 | 5.8 | 85 | In | δF508/dF508 | C,E |
| 27 | 22 | 2.9 | 67 | In | unk/unk | C |
| 28 | 20 | 6.7 | 77 | In | δF508/dF508 | E |
| 29 | 18 | 13.3 | 92 | In | δF508/dF508 | E |
| 30 | 22 | 8.8 | 71 | In | δF508/394delTT | E |
| 31 | 15 | 13.0 | 68 | In | δF508/dF508 | E |
| 32 | 14 | unk | 97 | Out | δF508/dF508 | E |
unk indicates unknown.
Measured as alpha-tocopherol. Lower limit of normal = 5.5 mg/L.
Normal: 80% to 120% of predicted.
Outpatients were seen for routine CF Clinic visit; inpatient were hospitalized for therapy of an exacerbation of CF.
Studies done on each subject's platelets: A indicates leukocyte-platelet aggregates (Figure 1); B, whole blood studies (Figures 2, 3); C, washed platelet-P-selectin measurements (Figure 4); D, inhibition of P-selectin expression by PGE1 (Figure 5); E, measurement of fatty acids (Figure 6); and F, AA and thromboxane release.