Table 1.
Patient | TNF-α genotype | CFTR genotype | Shwachman-Kulczycki | Kanga | Oxygen saturation | FEV1% | |
---|---|---|---|---|---|---|---|
| |||||||
-238G>A | -308G>A | ||||||
MVG | G/G | G/A | F508del/F508del | Moderate | Normal | Normal | Normal |
JPD | G/G | G/A | F508del/F508del | **** | Normal | Normal | Mild |
RSR | G/A | A/A | F508del/F508del | Excellent | Normal | Mild | Normal |
EVM | G/G | A/A | F508del/R1162X | Excellent | Normal | Mild | Severe |
IFM | G/G | A/A | F508del/F508del | **** | **** | **** | **** |
MLA | G/G | A/A | F508del/F508del | **** | Exacerbate | Normal | Moderate |
VLPC | G/G | A/A | F508del/F508del | Moderate | Normal | Normal | Normal |
MAB | G/G | A/A | F508del/F508del | Severe | Exacerbate | Mild | Severe |
EVSMS | G/G | A/A | F508del/F508del | **** | Normal | Normal | Mild |
YBK | G/G | A/A | F508del/F508del | Moderate | Normal | Normal | Normal |
HB | G/A | A/A | F508del/G542X | **** | Normal | Severe | Severe |
WSP | G/G | A/A | F508del/G542X | Moderate | Normal | Mild | Severe |
DRG | G/G | A/A | F508del/F508del | **** | **** | **** | **** |
BBK | G/G | G/A | F508del/N1303K | **** | **** | **** | **** |
AO | G/G | A/A | F508del/F508del | **** | Exacerbate | Mild | Mild |
LSM | G/A | A/A | F508del/F508del | Excellent | Normal | Normal | Moderate |
VAL | G/G | A/A | F508del/F508del | Good | Normal | Mild | Mild |
LFSA | G/G | A/A | F508del/F508del | **** | Normal | Mild | Mild |
IBN | G/G | A/A | F508del/R553X | Excellent | Normal | Normal | Normal |
CAQ | G/G | G/G | F508del/G542X | **** | Normal | Normal | Mild |
MEMZ | G/A | A/A | F508del/F508del | **** | Normal | Normal | Normal |
TMG | G/G | G/G | F508del/F508del | Moderate | Normal | Normal | Normal |
JMGR | G/G | G/A | F508del/N1303K | **** | Normal | Normal | **** |
MAP | G/A | G/G | F508del/F508del | **** | **** | **** | **** |
AX | G/G | A/A | F508del/F508del | Good | Normal | Normal | Normal |
LPOL | G/G | G/A | F508del/N1303K | Good | Normal | Normal | **** |
EG | G/G | A/A | F508del/R1162X | Good | Normal | Normal | Normal |
NCB | G/G | G/A | F508del/R553X | **** | Normal | Mild | Severe |
FVV | G/G | A/A | F508del/G542X | Excellent | Normal | Normal | Normal |
LSS | G/G | A/A | F508del/F508del | **** | Exacerbate | Moderate | **** |
RNC | G/G | A/A | F508del/F508del | **** | Normal | Normal | **** |
FEL | G/G | A/A | F508del/G542X | Moderate | Normal | Normal | Moderate |
GOV | G/G | G/A | F508del/F508del | **** | Normal | Normal | **** |
CVAR | G/G | A/A | F508del/G542X | **** | Normal | Normal | **** |
EAG | G/G | A/A | F508del/F508del | **** | Normal | Mild | **** |
AVM | G/G | G/G | DF508/G542X | Excellent | Normal | Normal | Normal |
BSG | G/G | A/A | G542X/R1162X | Good | Exacerbate | Mild | Mild |
GPNS | G/A | A/A | F508del/F508del | **** | Normal | Normal | **** |
CAL | G/G | A/A | DF508/G542X | Good | Normal | Normal | Normal |
MOS | G/G | G/G | F508del/F508del | Moderate | Normal | Normal | **** |
FSC | G/G | A/A | F508del/F508del | Good | Normal | Normal | Normal |
ITVS | G/G | G/A | F508del/F508del | **** | Normal | Normal | Normal |
JRL | G/G | G/G | F508del/F508del | Mild | Normal | Normal | **** |
GPT | G/A | A/A | F508del/F508del | Excellent | Normal | Normal | Normal |
ASR | G/A | G/A | F508del/R1162X | **** | **** | **** | **** |
VAF | G/G | A/A | F508del/F508del | **** | Normal | Normal | **** |
IRO | G/A | G/G | F508del/F508del | **** | Normal | Normal | **** |
BMSL | G/G | A/A | R1162X/R1162X | **** | **** | **** | **** |
BSC | G/A | A/A | F508del/F508del | Moderate | Normal | Normal | Mild |
****: no data; CFTR = Cystic Fibrosis Transmembrane Regulator; TNF-α = tumor necrosis factor.