LIPOSTEROID AGAINST REFRACTORY PULMONARY HEMORRHAGE IN IDIOPATHIC PULMONARY HEMOSIDEROSIS

We describe two Japanese children with idiopathic pulmonary haemosider osis (IPH), whose refractory haemorrhages were treated with an intrave nous; lipid emulsion containing dexamethasone (liposteroid). A 22-mont h-old boy and a 14-month-old girl have been observed with similar symp toms; periodic bouts of anaemia, reticulocytosis, diffuse infiltrates on chest X-ray and the finding of siderophages in sputum or gastric la vage fluid. The MRI of the lung was useful for the diagnosis. Methylpr ednisolone pulse therapy was successful in treating acute massive blee ding. Subsequent oral prednisolone could not prevent chronic recurrent haemorrhages. However, the intermittent administration of liposteroid (0.05 mg/kg/dose IV) led to a cessation of bleeding; the haemoglobin concentration rose to normal levels. This observation emphasizes the u sefulness of liposteroid in the management of refractory IPH.