Hemosiderosis in children

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

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

Symptoms of idiopathic pulmonary hemosiderosis

Children are more often sick in the age of 3-8 years. The onset of the disease is gradual: dyspnea appears at rest, anemia. At the time of the crisis febrile temperature, coughing rusty with blood, respiratory failure increases, anemia (hemoglobin up to 20-30 g / l and lower!). During percussion, the areas of shortening of pulmonary sound are noted. When auscultation - diffusely moist, finely bubbling rales. Characteristic increase in the liver and spleen. The period of the crisis lasts several days (more rarely - longer), gradually turning into remission. The level of hemoglobin increases, the symptoms 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. An increased content of circulating immune complexes, low complement titers, hypergammaglobulinemia, and sometimes a decrease in IgA content are revealed.

In some cases, biopsy of the lung is indicated for the diagnosis of hemosiderosis.

Instrumental methods

On chest radiographs, cranial focal shadows of hemorrhages, sometimes draining, enlarged basal lymph nodes are clearly visible at the time of the crisis. At repeated crises on the roentgenograms, new foci are detected. During the period of remission, the radiologic picture differs: a strengthened interstitial pattern resembling a fine mesh, many small (miliary) shadows forming a "butterfly pattern" that is a characteristic feature of hemosiderosis.

When bronchoscopy in the bronchoalveolar fluid, more than 20% of siderophages are found, the "iron index" is higher than 50 (the norm is up to 25). 

Heiner's syndrome is a kind of hemosiderosis of the lungs with hypersensitivity to cow's milk, the clinical picture does not differ from idiopathic, but patients have antibodies (precipitin) and positive skin tests for milk allergens. In some children, the disease is accompanied by a backlog in physical development, chronic rhinitis, otitis media, adenoiditis.

In the plan of the examination, the determination of antibodies to milk is mandatory.

Treatment of idiopathic pulmonary hemosiderosis

Treatment of hemosiderosis depends on the period of the disease. At the time of the crisis, prednisolone is administered at 1.5-3 mg / kg per day. Thus it is necessary to observe a strict dairy-free diet. When remission occurs, when the iron index of the bronchoalveolar fluid drops to 25 and below, glucocorticoids are canceled. Immunosuppressive drugs 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 injected to remove excess iron.

A dairy-free diet is indicated for all children with hemosiderosis, especially with Hayner syndrome.

The prognosis of the disease is unfavorable. The danger to life is the moment of crisis, the development of pulmonary hemorrhage, respiratory and heart failure.

Goodpasture Syndrome

Goodpasture syndrome acts as a form of hemosiderosis in adolescents. It is more common in boys. Characteristic pulmonary bleeding, anemia, hematuria and other signs of proliferative or membranous glomerulonephritis, which quickly lead to hypertension and chronic renal failure. In the pathogenesis of this form of hemosiderosis play the role of antibodies that affect not only the membrane of the pulmonary alveoli, but also the membrane of the renal glomeruli.

The prognosis is unfavorable, since the disease has a steadily progressing character.

[1], [2], [3], [4], [5], [6], [7], [8], [9]