British Paediatric Orphan Lung Diseases (BPOLD)

Idiopathic Pulmonary Haemosiderosis Checklist

• Full blood count, reticulocyte count, blood film, extended coagulation profile.
• Iron and Ferritin stores
• Chest X-ray
• Spirometry (Low FEV1, Low FVC, normal FEV/FVC ratio)
• Carbon monoxide transfer factor (raised during acute bleed)
• CT chest
• Bronchoscopy and Bronchoalveolar lavage (haemosiderin in macrophages from 3-14 days post bleed)

Secondary causes

Pulmonary infection (including CF/bronchiectasis)
Bronchoalveolar lavage culture (including moulds)

Gastro-eosophageal reflux and aspiration
Barium swallow/pH probe

Goodpastures Syndrome
Anti Basement Membrane Antibody

Pulmonary Haemorrhage associated with immune-complex-mediated Glomerulonephritis (PHIMG)
Consider Renal Biopsy (lumpy immune deposits on basement membrane)

Heiner’s Syndrome (Cow’s milk protein allergy)
Total IgE, Specific RAST IgE Cow’s Milk

Other autoimmune disease:
ESR, RhF, Antinuclear antibody, c ANCA + pANCA (to exclude poly-arteritis nodosa, Wegener’s granulomatosis, rheumatoid arthritis, systemic lupus erythematosis, mixed connective tissue disease).

Cardiac assessment:
ECG, ECHO (to exclude heart failure, myocarditis, mitral stenosis with pulmonary venous obstruction).

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