Histidine Rich Carboxyl Terminus 1 (HRCT1)

Since 1996, Sophia Children"""s Hospital, a tertiary academic hospital has monitored CF patients using annual PFT and biennial HRCT scans. All children with CF who had two HRCT scans (HRCT1 and HRCT2) in combination with two routine PFT (PFT1 and PFT2) were selected for this follow-up study. CF was diagnosed by a positive sweat test and/or genotyping for known CF mutations and/or an abnormal potential difference measured across the nasal mucosa. The ethical review board of the hospital approved the study. Data obtained at the first evaluation are reported as HRCT1 and PFT1, and data obtained at the second evaluation are reported as HRCT2 and PFT2. DHRCT is the annual change for a scoring system ((HRCT2-HRCT1)/time interval). DHRCT and DPFT were also evaluated as a function of age and baseline disease severity (HRCT1 and FEV1 % pred).