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Liposteroid against refractory pulmonary haemorrhage in idiopathic pulmonary haemosiderosis
1Department of Paediatrics, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Insights
Idiopathic pulmonary haemosiderosis (IPH) in children can be refractory to standard treatments. A novel liposteroid treatment effectively ceased bleeding and normalized hemoglobin in two pediatric cases.
Area of Science:
- Pediatric Pulmonology
- Hematology
- Clinical Pharmacology
Background:
- Idiopathic pulmonary haemosiderosis (IPH) is a rare condition causing recurrent pulmonary hemorrhage.
- Management of refractory IPH in children often presents significant challenges.
Observation:
- Two Japanese children presented with IPH symptoms including anemia, reticulocytosis, and pulmonary infiltrates.
- Diagnosis was aided by lung MRI, and initial treatment with methylprednisolone pulse therapy controlled acute bleeding.
Findings:
- Oral prednisolone failed to prevent chronic recurrent hemorrhages in the pediatric IPH patients.
- Intermittent intravenous liposteroid (dexamethasone in lipid emulsion) administration effectively stopped bleeding and normalized hemoglobin levels.
Implications:
- Liposteroid represents a promising therapeutic option for managing refractory pediatric IPH.
- This approach may offer a more effective alternative for children with severe, persistent pulmonary bleeding.
Abstract:
We describe two Japanese children with idiopathic pulmonary haemosiderosis (IPH), whose refractory haemorrhages were treated with an intravenous lipid emulsion containing dexamethasone (liposteroid). A 22-month-old boy and a 14-month-old girl have been observed with similar symptoms; periodic bouts of anaemia, reticulocytosis, diffuse infiltrates on chest X-ray and the finding of siderophages in sputum or gastric lavage fluid. The MRI of the lung was useful for the diagnosis. Methylprednisolone pulse therapy was successful in treating acute massive bleeding. 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 usefulness of liposteroid in the management of refractory IPH.