--- a
+++ b/processing/MACCROBAT/26474553.txt
@@ -0,0 +1,11 @@
+An 8-year-old boy with S549R/1717-1G > A genotype was started on ivacaftor (150 mg b.i.d.) on compassionate use.
+At the age of 9 months he was diagnosed with CF due to failure to thrive.
+His previous history was remarkable for recurring nasal polyposis requiring endoscopic surgery and exocrine pancreatic insufficiency necessitating enzyme replacement therapy.
+He grew with body weight and height along the 10th percentile.
+He suffered from rather mild respiratory symptoms, primarily intermittent productive cough, and had close to normal lung function parameters in previous years as measured by body plethysmography and spirometry (minimal z-score of FEV1: −1.2).
+Sputum cultures grew Haemophilus influenzae and Staphylococcus aureus on several occasions.
+After 6 weeks of ivacaftor treatment, the patient reported clinical improvements in cough frequency, sputum production, physical performance, and less salt cravings.
+He gained 1.4 kg in body weight without changing the dose of his pancreatic enzyme replacement therapy.
+His sweat chloride level (Macroduct®) decreased from 115 mmol/l before ivacaftor to 40 mmol/l after 6 weeks and 52 mmol/l after 41 weeks (normal < 30 mmol/l [11]) of treatment.
+His FEV1 increased from 1.25 L (−1.2 z-score) to 1.65 L (+0.5 z-score) after 41 weeks of ivacaftor therapy.
+The LCI (normal < 8) measured by N2-MBW decreased from 14.5 to 8.3 after 6 weeks and 7.8 after 41 weeks of ivacaftor treatment (Table 1 and Fig.1).