Idiopathic hemosiderosis

Introduction to idiopathic hemosiderinosis Idiopathic pulmo- serotoninosis (IPH) is a rare disease in which the cause is unknown and intermittent bleeding occurs 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. basic knowledge The proportion of illness: the incidence rate is about 0.003% - 0.006% Susceptible people: no specific population Mode of infection: non-infectious Complications: iron deficiency anemia

Idiopathic hemosiderosis

Unexplained, speculation is related to several aspects, abnormal development of lung epithelial cells, mechanical instability of the interstitial capillaries and repeated bleeding; immune dysfunction, such as 1 / 8 patients with pulmonary eosinophil infiltration, and mast cells, There are more plasma cells, some patients have positive condensing test, some patients can 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 suggests a wide range of capillary endothelial cell swelling, protein deposition in the intima, lung tissue washing, dry, tissue iron content is still 5 to 200 times higher than normal lung, and is proportional to the disease.

Idiopathic hemosiderin prevention

The course of the disease varied greatly. There were 68 cases reported, 20 cases (29%) died within 3 years, 17 cases (25%) were active, 12 cases (18%) were stable, and 19 cases (28%) returned to normal.

Some people think that milk may be one of the causes of the disease, and it is best for children to stop using milk.

Idiopathic hemosiderin complications Complications iron deficiency anemia

Diffuse pulmonary interstitial fibrosis, diffuse pulmonary interstitial fibrosis is an inflammatory disease of the interstitial lung caused by a variety of causes, the lesion mainly involving the pulmonary interstitial, but also involving alveolar epithelial cells and pulmonary vasculature. The cause is clear and some are unknown. The clear causes are inhalation of inorganic dust such as asbestos and coal; organic dust such as mildew dust, cotton dust; gases such as smoke, sulfur dioxide, etc.; viruses, bacteria, fungi, parasitic infections; drug effects and radiation damage.

Idiopathic hemosiderosis symptoms common symptoms skin pale fatigue, low heat, dry cough, skin, metal color... hemosiderin, internal bleeding, chest pain, dyspnea, hemoptysis

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, the symptoms depend on the degree of intrapulmonary hemorrhage, mild persistent chronic bleeding can be There are dry cough, fatigue, pale skin, weight loss and even clubbing. During acute bleeding, there may be blood in the sputum, hemoptysis, low fever, chest pain, etc. In the later stage, there may be difficulty in breathing, and even the performance of cardiac insufficiency in the acute phase. And secondary infection can have obvious wet Luo sound and stool occult blood positive.

The X-ray showed a fusion of spotted shadows in the lower lungs with multiple unclear edges. After the pulmonary hemorrhage stopped, the shadows improved within a few weeks. In the case of persistent moderate hemorrhage, 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.

Examination of idiopathic hemosiderosis

First, laboratory inspection

1, different degrees of small cell hypochromic anemia, more common with moderate anemia. Serum ferritin is reduced.

2, the attack period may have a slight jaundice, mainly indirect bilirubin increased. A few Combs tests can be positive.

3, sputum smear Prussian blue staining shows that there are blue particles in the cells, which are hemosiderin particles, according to which the diagnosis can be confirmed. For infants and young children, gastric juice should be taken for the above staining.

Second, microscopic examination of alveolar and interstitial macrophages containing red blood cells and hemosiderin. There are varying degrees of diffuse fibrosis in the lungs. Electron microscopy showed diffuse capillary damage with endothelial edema, type II alveolar epithelial hyperplasia, and protein deposition on the basement membrane.

Third, 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.

Diagnosis and diagnosis of idiopathic hemosiderin

Identification with cardiogenic (congestive) pulmonary hemorrhage:

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.

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