Pediatric idiopathic pulmonary hemosiderosis

Brief introduction of children with idiopathic pulmonary hemosiderosis Idiopathic pulmonary hemoglobinosis (idiopathic pulmonary hemoseriderosis) is a type of repeated hemorrhage in the alveoli, causing iron accumulation in the interstitial lungs, which eventually leads to progressive pulmonary fibrosis. It is a group of alveolar capillary hemorrhagic diseases, which are more common in children. Clinically, it is a disease characterized by iron deficiency anemia, cough, hemoptysis and progressive shortness of breath. basic knowledge Sickness ratio: 0.001%-0.002% Susceptible people: children Mode of infection: non-infectious Complications: anemia heart failure pulmonary fibrosis hemoptysis

The cause of idiopathic pulmonary hemosiderosis in children

(1) Causes of the disease

The cause of the disease is completely clear.

1. Idiopathic pulmonary hemosiderosis can be divided into 4 subtypes:

(1) Simple type.

(2) Co-morbidity with milk allergy.

(3) Co-occurrence with myocarditis or pancreatitis.

(4) Co-occurrence with hemorrhagic glomerulonephritis (Goodpasture syndrome),

2. Secondary pulmonary hemosiderosis is secondary to the following pathological conditions:

(1) the consequences of left atrial hypertension caused by various reasons,

(2) Complications of collagen vascular disease (such as nodular arteritis).

(3) chemical drug allergy (such as phosphorus-containing pesticides),

(4) Food allergies (such as gliadin, gliadin), which are described here as the idiopathic simple type.

(two) pathogenesis

Still in the process of exploration, at least three completely different situations are known from laboratory studies, suggesting that the pathogenesis may be diverse.

1. No lesions were found to be associated with any immune mechanism.

2. Anti-pulmonary anatomical antibodies, such as lesions of the glomerular basement capillaries in the alveolar basement membrane of Goodpasture syndrome, such as immunoglobulin G (IgG) line-like deposition, immunofluorescence also see complement The anti-base membrane antibody can be found in the serum of 90% of patients with Goodpasture syndrome on the basement membrane of the capillaries of the lungs and kidneys.

3. It may be related to soluble immune complexes. It has been confirmed that diffuse pulmonary hemorrhage can be complicated by immune complex nephritis and systemic lupus erythematosus. Under electron microscope, protein-like necrotic deposits are deposited on the capillary basement membrane, and immunization The deposition of complex components is consistent, and there are many reports on clinical research, but there is no systematic conclusion: since a small number of sick children are determined to be related to fresh milk allergy, it is very important to cause illness due to allergies, but most of them The sick child has no milk allergies. Although autoimmunity may explain some Goodpasture syndrome, only a few patients have this combined pathological process, and this sign is rare in children. Infants and young children have no such disease. The disease is related to abnormal immunity. For example, the IgA in the serum and secretions of some sick children is reduced. On the contrary, the adult patients have the opposite result, and some people are suspected to be related to the virus infection.

Due to the possible increase in condensed antibody titer, viral cultures all have negative results, and genetic factors may be related: two pairs of siblings have been reported in the literature, and one of the grandmothers has a history of hemoptysis and iron deficiency anemia. Reported in 26 children, 13 of them lived in areas with close relatives and marriage customs, which means that there is genetic possibility. Some people have reported that the disease is a fragile defect of congenital pulmonary capillary function, and the poisoning has also been suspected. And: It has been reported that phosphate insecticides have caused pulmonary hemorrhage in 30 children. In the experimental study, mice were injected with imidazole-based chemicals to produce pulmonary hemorrhage, followed by extensive pulmonary hemosiderin deposition.

Prevention of pediatric idiopathic pulmonary hemosiderosis

There is no specific prevention method. Some people think that this disease is related to milk allergy. Because there are many antibodies (blood precipitin) that can precipitate milk in the serum of children, if milk protein is used for intradermal test, it is a positive reaction. After the child stopped taking milk, the symptoms quickly disappeared, and the milk was re-appeared. Symptoms can reappear. Children with increased predruculum should pay attention to finding allergens, avoid contact with allergens, actively prevent lung infection, and enhance the physical condition of children. .

Pediatric idiopathic pulmonary hemosiderosis complications Complications anemia heart failure pulmonary fibrosis hemoptysis

After a year of constant idiopathic pulmonary hemosiderosis, anemia and chronic pulmonary fibrosis occur, eventually died of heart failure, pulmonary hemorrhage, massive hemoptysis and early death.

Symptoms of children with idiopathic pulmonary hemosiderosis Symptoms Symptoms Increased heart rate, blood stasis, chest pain, fatigue, jaundice, pale heart, lung, atelectasis, toe (toe)

The incidence can be divided into two types, with fulminant onset more common, prominent is repeated cough, shortness of breath and other acute respiratory symptoms with hemoptysis or hematemesis; the other type only with anemia with lethargy, weak and come to the diagnosis, according to our hospital Early 65 cases of statistics: the symptoms were pale (95.2%); fatigue (79.5%); cough (66.7%); hemoptysis or hematemesis (429%); low fever (33.5%); abdominal pain (12.7%); Nose bleeding (6.4%), and signs of hepatosplenomegaly (39.7%); increased heart rate (27%); lung voice (25.4%); jaundice (4.8%); clubbing (1.6%) ) and joint swelling (1.6%).

1. Sudden onset of acute bleeding, common onset pale with fatigue and weight loss, cough, low fever, cough with bloodshot or dark red small blood clots, even a lot of vomiting and abdominal pain, can also see shortness of breath, cyanosis, palpitations and The pulse is accelerated, the lung signs are not the same, there may be no positive body, and the breath sounds may be weakened or bronchial breath sounds. A few may smell dry, wet voice or wheezing sound; severe cases may have heart failure.

2. Most patients with chronic recurrent episodes may enter this period after the acute phase. Symptoms are recurrent episodes. Chronic cough, chest pain, low fever, asthma, etc. caused by foreign body stimulation in the lungs; Bloodshot or old blood clots.

3. In the quiescent period or sequelae period, the intrapulmonary hemorrhage has stopped, no obvious clinical symptoms, and the sequelae period has formed a wide range of pulmonary interstitial fibrosis due to repeated bleeding. The clinical manifestations include a history of multiple attacks and different degrees of lung. Insufficient function, small bronchus has different degrees of stenosis and distortion. Children who have had recurrent episodes for many years still have ventilatory dysfunction; visible liver, splenomegaly, abnormal changes of the finger-toe and electrocardiogram, X-ray chest showed increased texture and roughness, There are small cystic translucent areas or fibrosis, and may have atelectasis, emphysema, bronchiectasis or pulmonary heart disease, etc. This disease can be seen at the time of diagnosis:

1 hemoptysis, hematemesis or oral blood in children's stomach fluid;

2 chronic refractory hypopigmented small cell anemia;

3 lung slices have extensive acute or chronic infiltration, these characteristics can appear successively, the severity can also be disproportionate, and even some children only diagnose with a sign of anemia, although the disease is rare, but not unusual, Beijing Children's Hospital Since deepening the understanding of this disease, it has been found that there are many cases, so missed diagnosis and misdiagnosis are possible. Therefore, the following clinical points should be noted:

1. Repeated anemia with respiratory symptoms. Children with repeated iron deficiency anemia with respiratory irritation such as cough, a small amount of hemoptysis, etc., should raise the vigilance of this disease, because infants can swallow lungs Into the stomach, and then spit or even not spit out, there is no cough; therefore, for children with unexplained vomiting or repeated anemia, X-ray chest X-ray and the disease should be identified.

2. Pulmonary slices show cloud-like shadows or diffuse dot-like shadows such as lung slices showing cloud-like shadows or diffuse dot-like shadows. When pneumonia cannot be fully explained, it should be highly suspected.

3. Look for hemosiderin macrophages in the acute phase should be investigated, looking for hemosiderin macrophages as a basis for diagnosis, the young children will not spit, you can take gastric juice to find, the positive rate is also high, Up to 95%, but sometimes it is necessary to patiently and repeatedly search for positive results. When examining sputum or gastric juice, it should be used for Prussian blue reaction staining. If the smear test is negative and resembles this disease, it has been advocated to use lung puncture to take living tissue. Examination, it is recommended to take bronchoalveolar fluid flushing to take specimens, but there is a risk of severe bleeding, pneumothorax or pneumonia. Therefore, Beijing Children's Hospital repeatedly checks the initial negative results, and never needs to do the above two special operations.

4. Regular pulmonary function test for children with chronic recurrent episodes should be regularly measured for lung function, combined with the progress of the lung lobe results, the maximum ventilation and time lung capacity decreased in severe cases, pulmonary fibrosis may have dispersion Functional impairment and hypoxemia, according to clinical manifestations, laboratory findings and abnormal shadows displayed by X-ray films, can make a correct diagnosis, but some cases have clinical and X-ray findings are not obvious, sometimes clinical lack of hemoptysis However, anemia is very significant, so in the case of children with chronic anemia, after eliminating common causes, you should be alert to the possibility of suffering from this disease.

Examination of children with idiopathic pulmonary hemosiderosis

The key is to find hemosiderin macrophages in the sputum or in the gastric juice of children. Other aspects are:

1. The acute phase of blood picture shows different degrees of small cell hypochromic anemia. Children with Beijing Children's Hospital have severe anemia (hemoglobin 30 ~ 60g / L) about 1/3, moderate anemia (hemoglobin 60 ~ 90g) /L) accounted for 45%, reticulocytes in peripheral blood samples increased, up to 23%, more than 3% accounted for 70%, eosinophils increased in some cases, more than 3% accounted for about 1/3 The platelets are normal and the erythrocyte sedimentation rate is increased.

2. sputum (or bronchial lavage) or gastric juice test hemosiderin cells positive, the positive rate of about 95% is the main method to diagnose the disease, suspicious people should be found multiple times.

3. Other examinations showed an increase in serum bilirubin during acute attack. Direct Coombs test, condensation set test, heterophilic agglutination test may be occasionally positive, fecal occult blood is mostly positive, although a large amount of iron is accumulated in the lung, but it is imprisoned in giant In phagocytes, it can not be used for hematopoiesis, so the serum iron concentration is still low, there are different degrees of pulmonary dysfunction, erythrocyte sedimentation rate increases, blood T cell subclass detection abnormal, CD3 decreased, CD4/CD8 inverted.

4. X-ray lung slices with acute onset of X-ray can be seen in the lung field with unclear edges and cloud-like shadows of different density. The lesions can be fused from rice size to small pieces, mostly involving bilateral sides, generally more on the right side; It can also be changed by the ground glass with reduced transparency. The tip of the lung is not tired. In the follow-up observation, the patchy shadow can be dissipated within 2 to 4 days, but it can be reproduced in the short term and the lung texture is increased. , coarse and chaotic, chronic recurrent episodes, manifested as diffuse granules or fine mesh spots in the lungs, severe interstitial fibrosis changes, emphysema, pulmonary atelectasis with pulmonary heart disease, about half of the cases visible lung The door is enlarged. In the 2/3 cases, the right interlobular membrane is thickened due to the obstruction of lymphatic drainage. In the chest radiograph, 2/3 cases have enlarged heart, and the chronic recurrent X-ray lung slices show heavy lung texture on both sides. The texture can be seen in the fine mesh of unclear boundary, granules or miliary shadows, mostly bilateral, more common in the middle of the two lungs, the lung tip and the rib angle area are rarely affected, but also coexist with fresh hemorrhage This typical X-ray shows that the disease has been long-lived. Like in 6 to 12 months, the duration of this period and even up to 10 years.

5.B super liver, splenomegaly.

6. ECG has abnormal changes in ECG.

7. Lung tissue biopsy see phagocytic cells containing hemosiderin particles, can be diagnosed.

Diagnosis and differentiation of children with idiopathic pulmonary hemosiderosis

In the differential diagnosis, patients with such hemoptysis, fever, respiratory distress and anemia should pay attention to the identification of pneumonia, sepsis, tuberculosis, bronchiectasis, Gaucher disease, histiocytosis X, intrapulmonary mass and Wegner granulomatosis, such as merger Those with urinary blood, collagen vascular disease, blood disease, etc. should be considered, anemia and reticulocyte increase, should be differentiated from iron deficiency anemia and hemolytic anemia.

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