TABLE 2

Content categories of the standardised pulmonary ciliary dyskinesia (PCD) follow-up form (FOLLOW-PCD version 1.0) and degree of agreement of the working group members for their inclusion

Module and content category	Main included components	Degree of group agreement^# %
Module 1
Demographic information	Date of birth, sex, ethnicity	98–100
Diagnostic status	Current diagnosis and date, other ciliopathies	93–95
Diagnostic tests performed and results	nNO measurement: date, result, information on equipment and manoeuvre EM: date, sample quality, EM phenotype and % of affected cilia, ciliary culture HSVM: date, sample quality, beat frequency, beat pattern, ciliary culture Genetic analysis: date, technique, genes tested, result, variant nomenclature used Immunofluorescence: date, absent proteins, positive proteins	95–100
Differential diagnosis	Immunodeficiency (dates and results of test performed) Cystic fibrosis (dates and results of test performed)	83–100
Baseline medical history	Detailed history of neonatal period (symptoms, management, gestational age, birth weight, information on breastfeeding) Laterality defects (laterality status and cardiovascular malformations, tests and dates of diagnosis) Fertility problems (test performed, date and result, number of own children) Retinitis pigmentosa (date of diagnosis) Hydrocephalus (test performed, date and result) Kidney problems (problem description, date of diagnosis) Gastrointestinal problems (problem description, test performed, date of diagnosis) Upper and lower respiratory symptoms (overview of selected characteristics from the past history, e.g. age of symptom onset, bronchiectasis location and age of diagnosis, pathogens and duration of chronic upper or lower respiratory colonisation, level of hearing impairment and date of evaluation) Previous surgical interventions related to PCD Other PCD-related problems	80–100
Non-PCD-related comorbidities	Information on atopic diseases, e.g. asthma, hay fever, atopic dermatitis, tests performed and results, medication allergies	80–93
Family history	Consanguinity, PCD in the family, family history of other relevant manifestations	98–100
Module 2
Vital signs	Respiratory rate, S_pO₂	93–95
Clinical examination	e.g. signs of dyspnoea, clubbing	95–100
Lung auscultation	Pathological sounds and location	93–95
Heart auscultation	Description of pathological findings	80–93
Module 3
Examination of the nose	Discharge and aspect, mucosa, polyps, other findings	80–95
Examination of the sinuses	Facial pain/sensitivity	93–95
Examination of the ears	Discharge and aspect, otoscopy, audiometry and tympanometry findings	95–100
Module 4
Growth measurements	Height, weight, date of measurement	98–100
Lung function	Spirometry (date and several indices) Plethysmography (date and several indices) Lung diffusion testing (date and D_LCO result) Inert gas washout measurements (date, type of test, type of gas and concertation, number of trials, several indices) Exhaled breath analyses (date and F_eNO result) Information on bronchodilation testing (pre- and post-results) Information on quality of test performed and references used	93–100
Imaging (date and findings)	Chest radiography (date, findings, e.g. atelectasis, infiltrations, bronchial thickening) Chest CT (date, findings, e.g bronchiectasis, atelectasis, infiltrations, lobar collapse, mucus plugging, location of bronchiectasis, Brody II score) Lung MRI (date, findings, e.g bronchiectasis, atelectasis, infiltrations, lobar collapse, mucus plugging, location of bronchiectasis) Sinus radiography (date and findings) Sinus CT (date, findings and location: aplasia, hypoplasia, bone thickening, Lund–Mackay score)	95–100
Microbiology	Date of culture, type of sample, isolated pathogens, antibiotic resistance	95–100
Module 5
Hospitalisations and surgeries	Reason for hospitalisation and numbers of hospitalisation days	93–95
Medication	Antibiotics for acute infection (diagnosis, antibiotic class, route, length of treatment) Prophylactic antibiotics (antibiotic class, route, dosage, start date) Oxygen supplementation/ventilation (type and frequency of ventilation) Other prescribed medication (e.g. bronchodilators, corticosteroids) Relevant vaccinations (type and date of flu and pneumococcal vaccine)	93–100
Physiotherapy	Upper airways management (type and frequency of intervention) Lower airways management (type and frequency of interventions performed routinely and during exacerbations, nebulisation of lower airways, routine visits to professional physiotherapist) Compliance to treatment Exercise recommendations	80–95
Module 6
Symptoms from the upper airways and ears	Description, severity and periodicity: chronic nose symptoms headaches ear symptoms snoring	93–100
Symptoms from the lower airway	Description, severity and periodicity: cough sputum production wheezing shortness of breath chest pain	95–100
General symptoms	e.g. fever	93–95
Module 7
Daily activities	Information on working status, days off work/school because of PCD, sports	80–95
Smoking	Active, passive, type and frequency	93–95
Nutrition	Appetite, dietary supplements	85–93
Environment	Information on neighbourhood traffic, condensation/mould in the house	80–85

nNO: nasal nitric oxide; EM: electron microscopy; HSVM: high-speed video microscopy; S_pO₂: oxygen saturation measured by pulse oximetry; D_LCO: diffusing capacity of the lung for carbon monoxide; F_eNO: exhaled nitric oxide fraction; CT: computed tomography; MRI: magnetic resonance imaging. ^#: range of degree of agreement for the different components of the content category.