hemosiderosis
Introduction
Introduction Idiopathic pulmomaryhae mosiderosis (IPH) is a rare disease with unclear etiology and intermittent bleeding in the lungs. More than 200 cases were reported internationally and more than 120 cases were reported domestically (until 1994). After the destruction of red blood cells in the alveoli, globin is absorbed, and hemosiderin is deposited in the lung tissue to cause a reaction. Iron deficiency anemia can be secondary to repeated bleeding.
Cause
Cause
unknown. It is speculated that there are several aspects related to abnormal development of lung epithelial cells, mechanical instability of pulmonary interstitial capillaries and repeated hemorrhage; immune dysfunction, such as 1/8 patients with pulmonary eosinophil infiltration, mast cells, plasma cells Increased, some patients have positive test of condensation. Some patients may have rheumatoid, polyarthritis, myocarditis, and Goodpasture syndrome.
Other reasons mentioned are animal protein intake, inhalation of toxic substances (such as organic pesticides).
As the lung weight increases, the diffuse brown pigmentation can be seen on the cut surface. The microscopic examination can have alveolar epithelial necrosis, hyperplasia, local telangiectasia, macrophages that phagocytose hemosiderin in the alveolar and interstitial, and diffuse interstitial in the later stage. Fibrosis. Electron microscopy suggested extensive swelling of capillary endothelial cells and protein deposition in the intima. After washing and drying the lung tissue, the iron content in the tissue is still 5 to 200 times higher than that in the normal lung. And in direct proportion to the condition.
Examine
an examination
Related inspection
Blood test blood analyzer check
The disease is more common in children, mainly in the 1 to 7 years old, 15% over 15 years old, the ratio of male to female is 2:1, no obvious familial. Symptoms depend on the extent of intrapulmonary bleeding. Mild persistent chronic bleeding can have dry cough, fatigue, pale skin, weight loss, and even clubbing. During acute bleeding, blood in the sputum, hemoptysis, low fever, chest pain, etc. may occur. In the later stages, there may be difficulty in breathing, and even insufficiency of cardiac function. In the acute phase and secondary infections, there may be obvious wet rales. Fecal occult blood is positive.
The X-ray showed a fusion of spotted shadows in the middle and lower parts of the lungs. After the pulmonary hemorrhage stopped, the shadows improved within a few weeks. In patients with persistent moderate bleeding, the lesions in the lungs may be miliary.
According to repeated hemoptysis, bloody sputum, spotted shadows on the inner edge of the lung, and secondary iron deficiency anemia can be diagnosed by phagocytic cells found in sputum, bronchoalveolar lavage fluid and lung biopsy. It can be diagnosed after containing blue hemosiderin and excluding cardiogenic (congestive) factors.
Diagnosis
Differential diagnosis
Different from cardiogenic (congestive) pulmonary hemorrhage.
The disease is more common in children, mainly in the 1 to 7 years old, 15% over 15 years old, the ratio of male to female is 2:1, no obvious familial. Symptoms depend on the extent of intrapulmonary bleeding. Mild persistent chronic bleeding can have dry cough, fatigue, pale skin, weight loss, and even clubbing. During acute bleeding, blood in the sputum, hemoptysis, low fever, chest pain, etc. may occur. In the later stages, there may be difficulty in breathing, and even insufficiency of cardiac function. In the acute phase and secondary infections, there may be obvious wet rales. Fecal occult blood is positive.
The X-ray showed a fusion of spotted shadows in the middle and lower parts of the lungs. After the pulmonary hemorrhage stopped, the shadows improved within a few weeks. In patients with persistent moderate bleeding, the lesions in the lungs may be miliary.
According to repeated hemoptysis, bloody sputum, spotted shadows on the inner edge of the lung, and secondary iron deficiency anemia can be diagnosed by phagocytic cells found in sputum, bronchoalveolar lavage fluid and lung biopsy. It can be diagnosed after containing blue hemosiderin and excluding cardiogenic (congestive) factors.
