From: Clinical expression of cystic fibrosis in a large cohort of Italian siblings
Concordance within sib-pairs for PI (%)95.1 | ||||
---|---|---|---|---|
PI/PI | PI/PS | PS/PS | ||
Number of sib-pairs | 58 | 3 | 40 | |
Meconium ileus: | MI/MI | 4 | 0 | 0 |
MI/N | 5 | 0 | 2 | |
N/N | 49 | 3 | 38 | |
Lung disease: | Severe/severe | 6 | 0 | 0 |
Severe/mild | 15 | 1 | 5 | |
Mild/mild | 37 | 2 | 35 | |
CF liver disease: | CFLD/CFLD | 3 | 2 | 0 |
CFLD/N | 11 | 0 | 2 | |
N/N | 44 | 1 | 38 | |
CF related diabetes: | CFRD/CFRD | 5 | 0 | 1 |
CFRD/N | 11 | 3 | 2 | |
N/N | 42 | 0 | 37 | |
Pancreatitis: | RP/RP | 1 | 0 | 1 |
RP/N | 1 | 0 | 6 | |
N/N | 56 | 3 | 33 |