Hemosiderosis in children

Idiopathic pulmonary hemosiderosis (ICD-10 code: J84.8) develops as a primary disease and is related to interstitial lung diseases of unknown etiology. Since glucocorticoid and immunosuppressant therapy is effective in hemosiderosis, the current hypothesis of this disease today remains immunoallergic, i.e. associated with the formation of autoantibodies. The role of a hereditary factor is not excluded. The pathogenesis is based on the process of hemorrhage into the alveoli with subsequent impregnation of iron salts into the hemorrhage zone, thickening of the interstitium, development of fibrosis, pulmonary hypertension and formation of pulmonary heart.

In the sputum of patients with hemosiderosis, macrophages (hemosiderophages) phagocytizing hemosiderin are found. In children, hemosiderosis is rare, more often in girls.

Symptoms of idiopathic pulmonary hemosiderosis

Children aged 3-8 years are most often affected. The onset of the disease is gradual: dyspnea at rest and anemia appear. During the crisis, the temperature is febrile, sputum when coughing is rusty, with blood, respiratory failure increases, anemia (hemoglobin up to 20-30 g / l and below!). Percussion reveals areas of shortening of the pulmonary sound. Auscultation reveals diffusely moist fine bubbling rales. Enlargement of the liver and spleen is characteristic. The crisis period lasts for several days (less often - longer), gradually turning into remission. The hemoglobin level increases, the symptoms of the disease disappear.

Diagnosis of idiopathic pulmonary hemosiderosis

Laboratory diagnostics

Characteristic changes in the form of microcytic hypochromic anemia, low serum iron, reticulocytosis, erythroblastic bone marrow reaction, as well as moderate leukocytosis, increased ESR, thrombocytopenia. The Coombs reaction (direct and indirect) is rarely positive. Increased levels of circulating immune complexes, low complement titers, hypergammaglobulinemia, and sometimes decreased IgA levels are detected.

In some cases, a lung biopsy is indicated to diagnose hemosiderosis.

Instrumental methods

On chest X-rays, multiple focal hemorrhage shadows, sometimes confluent, and enlarged hilar lymph nodes are clearly visible during a crisis. During repeated crises, new foci are revealed on X-rays. During remission, the X-ray picture is different: an enhanced interstitial pattern resembling a fine mesh, many small (miliary) shadows forming a "butterfly pattern", which is a characteristic sign of hemosiderosis.

During bronchoscopy, more than 20% of siderophages are detected in the bronchoalveolar fluid, the “iron index” is higher than 50 (the norm is up to 25).

Heiner's syndrome is a type of pulmonary hemosiderosis with hypersensitivity to cow's milk, the clinical picture is no different from idiopathic, but patients have antibodies (precipitins) and positive skin tests to milk allergens. In some children, the disease is accompanied by delayed physical development, chronic rhinitis, otitis media, and adenoiditis.

The examination plan necessarily includes determination of antibodies to milk.

Treatment of idiopathic pulmonary hemosiderosis

Treatment of hemosiderosis depends on the period of the disease. During a crisis, prednisolone is prescribed at 1.5-3 mg/kg per day. A strict dairy-free diet should be followed. When remission occurs, when the iron index of the bronchoalveolar fluid drops to 25 or lower, glucocorticoids are discontinued. Immunosuppressants are added to the therapy in a maintenance dose: cyclophosphamide (2 mg/kg per day) or azathioprine (3 mg/kg per day). After massive bleeding, desferoxamine is administered to remove excess iron.

A dairy-free diet is indicated for all children with hemosiderosis, especially those with Heiner's syndrome.

The prognosis of the disease is unfavorable. The moment of crisis, development of pulmonary hemorrhage, respiratory and cardiac failure are life-threatening.

Goodpasture's syndrome

Goodpasture's syndrome is one of the forms of hemosiderosis in adolescents. It is more common in boys. It is characterized by pulmonary hemorrhages, anemia, hematuria and other signs of proliferative or membranous glomerulonephritis, which quickly lead to hypertension and chronic renal failure. Antibodies play a role in the pathogenesis of this form of hemosiderosis, affecting not only the membrane of the pulmonary alveoli, but also the membrane of the renal glomeruli.

The prognosis is unfavorable, since the disease is steadily progressive.

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