Parameter | Adult PCD patients (n = 31) |
---|
Demographic information |
Current Agea | 33.6 (22.2–50.9) | |
Gender (female) | 18/31 | 58.1% |
Age at Presentationa | 23.79 (17.8–45.1) | |
Situs Abnormalities | 14/31 | 45.2% |
Diagnostic characteristics |
Nasal Nitric Oxide (nl/min)a | 24.9 (13.0–48.0) | |
TEM result |
Normal TEM | 8/31 | 25.8% |
ODA + IDA | 9/31 | 29.0% |
ODA only | 6/31 | 19.4% |
CP/ IDA + MD | 8/31 | 25.8% |
Other | 0/31 | 0% |
HSVM result |
Normal HSVM | 0/31 | 0% |
Immotile/Almost Immotile | 13/31 | 41.9% |
Extremely stiff due to reduced ciliary bending | 8/31 | 25.8% |
Stiff Beating pattern | 3/31 | 9.7% |
Circular Pattern | 7/31 | 22.6% |
Other | 0/31 | 0% |
Clinical Characteristics |
Chronic Rhinorrhoea | 31/31 | 100% |
Chronic wet cough | 31/31 | 100% |
History of NRDS | 14/31 | 45.2% |
History of nasal polyps | 8/30 | 26.7% |
History of Pneumonia | 12/29 | 41.4% |
History of Heamoptysis | 3/31 | 9.7% |
History of lung resection | 5/31 | 16.1% |
- aMedian and Interquartile Range TEM Transmission Electron Microscopy, HSVM High Speed Video Microscopy, ODA + IDA Combined Outer Dynein Arm defect and Inner Dynein Arm defect, ODA Isolated Outer Dynein Arm defect, CP Central Pair defect, IDA + MD Inner Dynein Arm and Microtubular Disorganisation defect, NRDS Neonatal Respiratory Distress Syndrome