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T1 Age 3 13 8-year-old T2 Sex 14 17 boy T4 Medication 65 74 ivacaftor E2 Medication:T4 T5 Dosage 76 89 150 mg b.i.d. R1 MODIFY Arg1:T5 Arg2:E2 T6 Date 120 135 age of 9 months E3 Date:T6 T7 Disease_disorder 158 160 CF E4 Disease_disorder:T7 #1 AnnotatorNotes E4 cystic fibrosis T8 Disease_disorder 168 185 failure to thrive E5 Disease_disorder:T8 T9 History 227 252 recurring nasal polyposis T10 History 286 319 exocrine pancreatic insufficiency T11 History 365 423 grew with body weight and height along the 10th percentile T12 Sign_symptom 454 474 respiratory symptoms E6 Sign_symptom:T12 T13 Severity 449 453 mild R2 MODIFY Arg1:T13 Arg2:E6 T14 Sign_symptom 510 515 cough E7 Sign_symptom:T14 T15 Detailed_description 499 509 productive T16 Detailed_description 486 498 intermittent R3 MODIFY Arg1:T15 Arg2:E7 R4 MODIFY Arg1:T16 Arg2:E7 T17 Diagnostic_procedure 541 554 lung function E8 Diagnostic_procedure:T17 T18 Lab_value 525 540 close to normal R5 MODIFY Arg1:T18 Arg2:E8 T19 Diagnostic_procedure 599 619 body plethysmography E9 Diagnostic_procedure:T19 T20 Diagnostic_procedure 624 634 spirometry E10 Diagnostic_procedure:T20 T21 Lab_value 636 665 minimal z-score of FEV1: −1.2 R6 MODIFY Arg1:T21 Arg2:E10 T22 Diagnostic_procedure 668 683 Sputum cultures E11 Diagnostic_procedure:T22 T23 Lab_value 689 711 Haemophilus influenzae T24 Lab_value 716 737 Staphylococcus aureus T25 Detailed_description 741 758 several occasions R7 MODIFY Arg1:T25 Arg2:E11 R8 MODIFY Arg1:T23 Arg2:E11 R9 MODIFY Arg1:T24 Arg2:E11 T26 Duration 766 773 6 weeks E12 Duration:T26 T27 Medication 777 786 ivacaftor E13 Medication:T27 T28 Sign_symptom 844 849 cough E14 Sign_symptom:T28 A1 TREND E14 DEC T29 Lab_value 819 840 clinical improvements R10 MODIFY Arg1:T29 Arg2:E14 T30 Sign_symptom 911 924 salt cravings E15 Sign_symptom:T30 A2 TREND E15 DEC T31 Diagnostic_procedure 880 900 physical performance E16 Diagnostic_procedure:T31 T32 Sign_symptom 861 878 sputum production E17 Sign_symptom:T32 R11 MODIFY Arg1:T29 Arg2:E17 R12 MODIFY Arg1:T29 Arg2:E16 T33 Diagnostic_procedure 946 957 body weight E18 Diagnostic_procedure:T33 T34 Lab_value 929 942 gained 1.4 kg R13 MODIFY Arg1:T34 Arg2:E18 T35 Medication 991 1020 pancreatic enzyme replacement E19 Medication:T35 T36 Diagnostic_procedure 1034 1054 sweat chloride level E20 Diagnostic_procedure:T36 T37 Lab_value 1083 1093 115 mmol/l R14 MODIFY Arg1:T37 Arg2:E20 T38 Lab_value 1114 1123 40 mmol/l R15 MODIFY Arg1:T38 Arg2:E20 T39 Detailed_description 1094 1110 before ivacaftor R16 MODIFY Arg1:T39 Arg2:T37 T40 Date 1124 1137 after 6 weeks E21 Date:T40 R17 MODIFY Arg1:E21 Arg2:T38 T41 Lab_value 1142 1151 52 mmol/l R18 MODIFY Arg1:T41 Arg2:E20 T42 Date 1152 1166 after 41 weeks E22 Date:T42 R19 MODIFY Arg1:E22 Arg2:T41 T43 Diagnostic_procedure 1211 1215 FEV1 E23 Diagnostic_procedure:T43 T44 Lab_value 1231 1237 1.25 L R20 MODIFY Arg1:T44 Arg2:E23 T45 Lab_value 1239 1251 −1.2 z-score T46 Lab_value 1256 1262 1.65 L T47 Lab_value 1264 1276 +0.5 z-score R21 MODIFY Arg1:T46 Arg2:E23 R22 MODIFY Arg1:T45 Arg2:T44 R23 MODIFY Arg1:T47 Arg2:T46 T48 Date 1278 1292 after 41 weeks E24 Date:T48 R24 MODIFY Arg1:E24 Arg2:T46 T49 Medication 1296 1305 ivacaftor E25 Medication:T49 T50 Diagnostic_procedure 1319 1322 LCI E26 Diagnostic_procedure:T50 T51 Detailed_description 1336 1354 measured by N2-MBW R25 MODIFY Arg1:T51 Arg2:E26 T52 Lab_value 1370 1374 14.5 T53 Lab_value 1378 1381 8.3 R26 MODIFY Arg1:T52 Arg2:E26 R27 MODIFY Arg1:T53 Arg2:E26 T54 Date 1382 1395 after 6 weeks E27 Date:T54 R28 MODIFY Arg1:E27 Arg2:T53 T55 Lab_value 1400 1403 7.8 R29 MODIFY Arg1:T55 Arg2:E26 T56 Date 1404 1418 after 41 weeks E28 Date:T56 R30 MODIFY Arg1:E28 Arg2:T55 T57 Medication 1422 1431 ivacaftor E29 Medication:T57 T3 History 23 49 S549R/1717-1G > A genotype R31 AFTER Arg1:E2 Arg2:E3 * OVERLAP E3 E4 E5 R32 BEFORE Arg1:E5 Arg2:E6 * OVERLAP E6 E7 E8 E1 E9 E10 E11 T58 Date 569 583 previous years E1 Date:T58 R33 BEFORE Arg1:E11 Arg2:E12 * OVERLAP E12 E13 E14 E17 E16 E15 E18 E19 E20 E23 E25 E26 E29 T59 History 334 360 enzyme replacement therapy T60 History 263 281 endoscopic surgery