Table 1.
Patients | Age | Mutations | History of infections | PA culture | Shwachman score | Additional Data |
MGV | 15 | ΔF508/ΔF508 | PA chronic colonization | Pos | 95 | |
MGP | 14 | ΔF508/ΔF508 | PA chronic colonization, occasional SA | Pos | 76 | |
AAS | 17 | ΔF508/? | PA chronic colonization | Pos | 60 | |
AGH | 15 | ΔF508/? | PA chronic colonization | Pos | 80 | |
MCC | 14 | ΔF508/? | SA and PA chronic colonization | Pos | 65 | DMID |
LGS | 5 | ΔF508/ΔF508 | PA chronic colonization | Pos | 85 | |
EML | 29 | R347H/ΔF508 | PA chronic colonization | Pos | 55 | |
DNP | 12 | ΔF508/2789+ 563A | PA chronic colonization | Pos | 95 | |
YML | 23 | R347H/ΔF508 | PA chronic colonization | Pos | 55 | |
MVG | 8 | ΔF508/ΔF508 | occasional SA | Neg | 90 | |
VMM | 8 | ΔF508/G542X | # | Neg | 85 | |
ASA | 7 | ΔF508/L206W | SA chronic colonization, occasional HI | Neg | 95 | |
GCF | 10 | ΔF508/ΔF508 | occasional SA | Neg | 85 | |
MSP | 21 | ΔF508/? | * PA chronic colonization | * | 71 | DMID |
PA: Pseudomonas aeruginosa, SA: Staphylococcus aureus, HI: Haemophilus influenzae. Pos: sputum culture positive for PA, Neg: sputum culture negative for PA, DMID:Diabetes mellitus insulin-dependent. # PA colonization until 2 years before blood extraction, negative from this date until now * PA negative only for one year, blood extraction was made during this year Schwachman score rates severity based on history, physical examination and chest roentgenogram