Primárna pľúcna hemosideróza

Primary Pulmonary Haemosiderosis

Primary pulmonary haemosiderosis is a progressive disease. In Slovakia the frequency is about 6.3/million ofthe child population. The group of patients is formed by four girls and two boys. In three patients hypersensitivityto cow’s milk protein was found. In one instance Heiner’s syndrome was associated with myocardititis and inanother patient with coeliac disease. The patient with malabsorption was givne rHuEPO which stimulatederythropoiesis and improved the anaemia only during the period of administration of the preparation. In thebiochemical results during the attack very low values of serum iron and ferritin levels predominated. Theerythropoietin levels (S) in the quiescent stage with mild anaemia did not exceed the reference range. A 13-year-oldgirl was admitted with massive haemorrhage into the lungs and congestive heart failure. In the documentationattention was attracted to a record of sudden heart failure in a brother at the age of two years. Aggressive treatmentdid not affect septic fever with a leukomoid reaction nor abnormalities of the pulmogram with myocardialdysfunction, and did not prevent death. The prognosis and course of the disease are difficult to foresee, and criteriafor its assessment are lacking. A favourable termination of the episode is frequently at variance with long-termpulmonary processes which lead to haemosiderotic fibrosis with subsequent restriction of pulmonary function.

Key words:
pulmonary haemosiderosis, haemosiderophages, abnormal pulmogram