Very rare. Affection of unknown origin characterized by recurrent episodes of pulmonary intraalveolar bleeding. It appears generally between 1 and 7 years of age, affecting more boys than girls. It can occur at any age. 

Variable clinical manifestations:

-         anemia of varying importance

-         lung symptoms: chronic cough, episodes of dyspnea, hemoptoic sputum; sometimes major hemoptysis, catastrophic pulmonary hemorrhage; finger clubbing

-         infiltrates or a miliary image on the chest XRay or CT.

Diagnosis: bronchoscopy, bronchoalvelolar lavage .

The repetition of alveolar bleeding can cause pulmonary fibrosis.

Treatment: exclude causes of secondary hemosiderosis; corticosteroid therapy.

Anesthetic implications: 

check hemoglobin levels; chest XRay or CT; SpO2 at room air; corticosteroid therapy.

References : 

-         Tripi P, Thomas S, Dearborn DG.
Management of general anaesthesia in infants and children with a history of idiopathic pulmonary haemorrhage.
Pediatr Anesth 2002; 12: 243-7.

-        Gutierrez S, Shaw S, Huseni S, Sachdeva S et al. 
Extracorporeal life support for a 5-week-old infant with idiopathic pulmonary hemosiderosis. 
Eur J Pediatr 2014; 173:1573-6. 

Updated: August 2019