26474553 Visualization

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