The term "lung hemosiderosis" is used for the description of a number of seldom found states which are characterized by pathological accumulation of hemosiderin in it. The last is postponed after diffusion alveolar bleeding and can proceed as primary disease or to develop for the second time at heart troubles or vascular system. Children suffer from primary hemosiderosis more often. Distinguish 4 types it: 1) idiopathic 2) connected with an allergy to cow's milk (Geyner's syndrome); 3) the accompanying myocarditis; 4) the complicating progressing glomerulonephritis (Gudpascher's syndrome). The secondary hemosiderosis happens three types: 1) the accompanying mitral stenosis and a chronic left ventricular failure of any etiology; 2) connected with collagenoses; 3) accompanying diseases of blood and vessels.
Idiopathic primary hemosiderosis of lungs.
The cause of illness is not clear, however the messages on family cases which are found sometimes it allow to assume the genetic nature. It begins usually at children's age, it is rare after youthful age. In most cases clinical manifestations are connected with presence of blood at alveoluses and chronic blood loss. The symptomatology consists in recurrent or chronic diseases of lungs and includes cough, a pneumorrhagia, an asthma, goose breathing, sometimes cyanosis, at the same time fatigue increases and pallor of integuments amplifies. Cough can be followed by department of a bloody phlegm, and at children of chest or early age vomiting can join; emetic masses at the same time contains a large amount of blood. During the bad attacks which are usually proceeding within 2 — 4 days at the child body temperature can increase. On a usual clinical picture (fervescence, tachycardia, breath increase, a leukocytosis, breath disturbances, change of radiographic data) the disease can be accepted for bacterial pneumonia, and only long supervision over the patient allows to make the diagnosis correctly. However at some children early displays of a disease are connected with the chronic iron deficiency anemia which is often not giving in to influence of medicines, and the specific pulmonary symptomatology developing is much later. Paradoxically, but sometimes at the child with displays of hard proceeding disease of lungs of change on the roentgenogram can be absent or they can be defined before development of pulmonary symptomatology. Anemia, as a rule, microcytic and hypochromia; iron level in serum low, can raise the level of bilirubin and urobilinigen and to increase number of reticulocytes. In fecal masses the occult blood, apparently, usually is defined swallowed. In the smears prepared from a phlegm or aspirate of a trachea or a stomach, the macrophages loaded with hemosiderin usually find. Radiographic signs can vary from the minimum infiltrates reminding those at pneumonia before massive processes with secondary atelectases, emphysema and radical lymphadenitis. On the basis of these data tuberculosis or hypostasis of a lung can be suspected, and the expressed changes can be observed day by day. Sometimes for the purpose of diagnosis the open biopsy of a lung is required. On a tissue specimen reveal a hemorrhage in a cavity of alveoluses, a large number hemosiderin of the containing macrophages, a hyperplasia of an alveolar epithelium, intersticial fibrosis and a sklerozirovaniye of walls of small vessels. The closed needle biopsy is followed by heavy complications. About a half of patients die during 1 — the 5th year of life usually of acute pulmonary bleeding and the progressing respiratory insufficiency. In these cases before receiving results of the analysis of serum on precipitated calcium superphosphates the diet which is not containing some cow's milk that also serves as diagnostic test at a hemosiderosis of lungs is shown. Corticosteroids (Prednisonum in a daily dose of 1 mg/kg) can cause remission at one patients and cause simplification of disease in others. The supporting treatment by corticosteroids during the mezhpristupny periods is followed by indistinct results. Also immunodepressive drugs and Deferoxaminum were not rather estimated.
Primary hemosiderosis of lungs with hypersensitivity to cow's milk (Geyner's syndrome).
At children the idiopathic hemosiderosis proceeds typically with unusually high level in serum of pretsipitin to many ingredients of cow's milk and positive skin reactions to the proteins which are its part. They can have chronic rhinitis, recurrent average otitis, dysfunction of a gastro intestinal path and a growth inhibition. The symptomatology fades at an exception of a diet of cow's milk and again amplifies at its reception. At some children of improvement of a state does not come, at others it improves against absence in serum of pretsipitin. At a high caption of pretsipitin of milk the pulmonary heart for the second time in relation to a hypertrophy of an adenoid tissue of a nasopharynx can develop. These children need to make operation a tonzilloadenotomiya. In general at patients with a hemosiderosis and presence at serum of pretsipitin to cow's milk the forecast is more favorable, than patients with other forms have diseases, and hypersensitivity to milk at them eventually can disappear. Corticosteroids can have favorable effect, at least during acute bleeding.
Primary hemosiderosis of lungs at myocarditis.
At some patients the hemosiderosis can proceed against myocarditis of different degree. If the expressed changes in a myocardium are defined by the time of emergence of the first symptoms from lungs, happens it is impossible to define, the hemosiderosis is primary or secondary. The clinical picture does not differ from that at an idiopathic form except that at the child the heart sizes can be increased, and on an ECG symptoms of myocarditis come to light.
Primary hemosiderosis of lungs at a glomerulonephritis (Gudpascher's syndrome).
This disease first of all occurs at young men and is rare at children. In the beginning it is shown also as the idiopathic hemosiderosis which is followed by a pneumorrhagia and an iron deficiency anemia, but attentive studying of symptomatology of initial attacks usually allows to diagnose a proliferative or hymenoid glomerulonephritis. Patients have the progressing disease of kidneys with hypertensia, the subsequent insufficiency of their function which is coming to an end with death. In a small number of cases the pulmonary disease proceeded easier after a bilateral nephrectomy.
The heart diseases causing continuous increase of pressure in pulmonary capillaries, for example a mitral stenosis can lead to intra pulmonary bleedings and a secondary hemosiderosis which clinical manifestations meet also at collagenoses. Sometimes changes of vessels at a polyarteritis appear only in lungs in the beginning. Other diseases, for example, a pseudorheumatism, can also cause a hemosiderosis because of development of a generalized diffusion vasculitis. At a small number of patients with an anaphylactoid or Werlhof's disease the hemosiderosis was caused by intra pulmonary bleeding.