The disease is characterized by widespread pulmonary capillary hemorrhage, massive deposits of iron-containing hemosiderin in the alveoli, and iron deficiency anemia. The main clinical manifestations are recurrent episodes of hemoptysis, shortness of breath and anemia.
Basic introduction
The syndrome occurs in children and young adults, with clinical symptoms related to pulmonary hemorrhage and duration. Paroxysmal or persistent cough, hemoptysis and shortness of breath. The hemoptysis lasts for hours or days and gradually resolves on its own, at which time it is basically asymptomatic except for pallor and fatigue, but may recur after weeks or months. Chest signs are mostly abnormal. Due to anemia, cyanosis is often masked. Severe cases are often associated with pulmonary heart disease or pestle finger. Hemoptysis is a common cause of death.
Chest X-ray shows scattered small nodular shadows in the hilar or lower middle lung bands, which may fuse into hairy glass lamellar shadows in severe cases. It may improve or even be absorbed when symptoms are relieved.
Treatment with glucocorticoids can control bleeding, but cannot stabilize the disease and prevent recurrence in the long term, and is not effective in chronic cases, and iron can relieve severe anemia.
Idiopathic pulmonary iron-containing hemoglobinosis
This is a reaction caused by repeated bleeding of pulmonary capillaries and deposition of destroyed red blood cells into the alveolar septum as a result of iron-containing hemoglobin. The disease is rare and is characterized by hemoptysis, iron deficiency anemia, and transient pulmonary infiltrates. The disease is aggressive and most patients die from massive pulmonary hemorrhage.
In the past, the disease occurred mostly in children and adolescents, but in recent years, the number of adults with the disease has increased.
There is more than one cause of pulmonary ferritinosis and the cause is not known, so it is called idiopathic pulmonary ferritinosis.
Idiopathic pulmonary ferritinosis
This is called idiopathic pulmonary ferritinosis to distinguish it from other causes. The acute phase is characterized by extensive pulmonary hemorrhage, while the chronic phase is characterized mainly by interstitial pulmonary fibrosis. The iron content of the lungs is 5 to 2000 times higher than normal. Light microscopy reveals marked limited alveolar capillary dilatation, alveolar epithelial degeneration, detachment, and hyperplasia, interstitial infiltration with macrophages that phagocytose iron-containing heme and interstitial fibrosis, and degeneration of elastic fibers in the alveoli, interstitium, and blood vessels. Electron microscopy shows extensive capillary damage, endothelial swelling, and protein deposits in the basement membrane.
The disease is based on pulmonary capillary disorders, but its cause is unknown. There are two main speculations: (i) developmental defects, due to primary alveolar epithelial development and functional abnormalities that impair the mechanical stability of blood vessels, or defects in the development of elastic fibers of the lung, which cannot be confirmed by histological examination. (ii) Caused by an immune response. Patients may have an accumulation of mast cells in the lungs; increased plasma cells in the reticuloendothelial system; eosinophilic leukocytosis in 1/8 to 1/5 of patients; and in a few cases, other immune disorders. It has been found that some infants with confirmed or probable pulmonary ferritinosis have positive skin tests with milk and positive serum milk precipitins, and the symptoms disappear after withdrawal from milk. However, some children with confirmed pulmonary ferritinosis had negative reactions to these tests. Therefore, the etiology of this disease is not clear.
Patients are mostly children aged 1 to 7 years, with about 15% of patients reaching 16 years of age or older. There is no difference in the incidence between the two sexes in childhood, and in adulthood it is about twice as common in males as in females.
Disease etiology
1. abnormal alveolar epithelial cell development and function, destabilizing the alveolar capillaries and causing recurrent bleeding
Viral structure of idiopathic pulmonary iron-containing hemoglobinopathy
However, the etiology of epithelial cell abnormalities is unclear.
2. abnormal pulmonary elastic fibers abnormal presence of acidic mucopolysaccharides in elastic fibers that weaken and dilate the vessel walls.
Some children with cow’s milk allergy have a positive intradermal test for cow’s milk, and precipitins reactive to cow’s milk antigens are found in the serum. The clinical symptoms disappeared after discontinuation of milk use. The author Heiner reported 4 cases of confirmed IPH in pediatric milk allergy, which is known as Heinersyndrome, but milk allergy does not explain all of the morbidity in children.
Cassimos investigated 30 cases of children with confirmed IPH in Greece and found that 80% of them grew up in the countryside in poor economic conditions, with a diet low in protein; 50% had a history of continuous exposure to organophosphorus insecticides. The exact basis for this is yet to be confirmed.
Genetic factors are thought to be related to genetic factors, but there is no genetic confirmation.
6. Immunological factors Many scholars believe that the disease is related to immunity. The serum IgG, IgA and IgM are all elevated in patients, with IgA being more pronounced. Some patients have increased eosinophils and mast cell clusters in the lungs. There may be antinuclear antibodies and positive cold globulin antibodies. Some cases are effective with adrenocorticosteroids, immunosuppressants or plasma exchange. These support the association of the disease with abnormal immune function.
Symptoms
Gross examination of the lungs has a solid brown appearance, with increased weight and volume, and scattered hemorrhagic spots visible on the surface and in sections
Blood symptoms of idiopathic pulmonary ferritin-containing hyperpigmentation
The lungs have an enlarged hilar and mediastinal lymph nodes and are brown in color due to ferritin deposition. Hemorrhage is seen during acute attacks, and diffuse interstitial fibrosis in the lungs is more pronounced in the chronic phase.
2. Light microscopic examination showed swelling, degeneration, detachment and hyperplasia of alveolar epithelial cells, phagocytes containing erythrocytes or iron-containing heme in the alveolar lumen, degeneration of elastic fibers in the alveolar wall, capillary hyperplasia, thickening of the basement membrane, degeneration of elastic fibers in the small intrapulmonary arteries, iron-containing heme deposits, endothelial fibrosis and vitellogenic changes. Lymphocytes, plasma cells and iron-containing macrophages infiltrate around the lymphatic vessels, and the lymphatic vessels are dilated. Later stages of the disease may lead to diffuse pulmonary fibrosis, emphysema, and bronchiectasis.
3. Electron microscopy shows extensive alveolar capillary damage, endothelial cell edema, type II alveolar epithelial cell hyperplasia, focal rupture of the basement membrane, protein deposition in the basement membrane, and loss of normal structure of the basement membrane.
Clinical manifestations
The clinical manifestations of the disease depend on the severity and duration of pulmonary hemorrhage. Patients with acute exacerbations have extensive intrapulmonary hemorrhage, manifested by episodes of dyspnea, cyanosis, cough, hemoptysis, and increased heart rate. Many children have persistent small intrapulmonary hemorrhage, intermittent small amounts of blood or blood-stained sputum, but sometimes the blood is only present in the alveolar septum and is not hemorrhaged. Due to repeated pulmonary hemorrhage, a large number of red blood cells are destroyed, and iron-containing heme is deposited in the alveolar septum, which exists in macrophages and cannot be used for heme synthesis, resulting in the depletion of available iron stores in the body and chronic iron deficiency anemia. Part of it is carried by macrophages to the bronchus and coughs up with sputum, so macrophages containing iron-containing heme flavin (iron phagocytes) can often be found in sputum. Agaricus is a traditional blood tonic formula in China, and ferrous lactate is a good bivalent iron tonic preparation, and many blood tonic products on the market use them as separate formulas. Iron Edge tablets, on the other hand, use ferrous lactate, gum and zinc protein all as efficacious ingredients, with the triple effect of iron supplementation, blood production plus nutrition, which can better prevent and improve anemia and enhance human immunity.
In pulmonary hemorrhage X-ray examination shows extensive, fuzzy patchy infiltrates in the middle and lower lung fields of both lungs near the hilum, and some patients have enlarged hilar lymph nodes. The infiltrate may completely subside within 1 to 2 weeks, but after repeated hemoptysis, fibrosis occurs due to the deposition of iron-containing hemoglobin in the alveolar septum, so intermittent X-rays show reticular shadows and small dot-like shadows of deep density.
Massive pulmonary hemorrhage can occur at any time, and most patients die from severe pulmonary hemorrhage; only a few patients survive and develop pulmonary heart disease.
Laboratory tests
Tests Diagnosis Diagnosis.
The patient has recurrent hemoptysis (especially in children), unexplained iron deficiency anemia, and diffuse small nodular or lamellar on the chest radiograph
Idiopathic pulmonary iron-containing hemoglobinopathies examination
The disease should be suspected if there are nodular or reticular shadows. Further examination or lung biopsy is required, and a definitive diagnosis can be made if typical iron-containing macrophages are found.
Laboratory tests.
Blood picture shows iron-deficient microcytic hypochromic anemia with reticulocytosis. Serum iron and iron saturation are significantly reduced due to iron deposition in alveolar macrophages, which cannot be transported for hemoglobin synthesis, and a normal erythrocyte saline fragility test. Eosinophils in the peripheral blood may be increased and the sedimentation may be increased. Serum bilirubin may be increased because of the destruction of hemoglobin in the alveoli, serum IgA may be increased, direct test, condensation set test and phagocytic agglutination test may be positive, serum lactate dehydrogenase may be increased, and electrocardiogram may be abnormal if the heart is involved. Typical iron-containing macrophages found in sputum, gastric fluid, bronchoalveolar lavage fluid or lung biopsy tissue are important for diagnosis. Lung biopsy and fiberoptic bronchoscopy: When there is much alveolar bleeding, blood can be seen in the bronchi by fiberoptic bronchoscopy, and pathological observation by light and electron microscopy can be done by fiberoptic lung biopsy or transthoracic lung biopsy to clarify the cause of alveolar bleeding. Blood gas analysis: Patients are mostly normal in the early stage, when there is more alveolar bleeding or extensive interstitial lung fibrosis, PaO2 decreases, normal or decreases, heavy cases may present as type I respiratory failure, in the late stage when emphysema, pulmonary heart disease and respiratory failure appear, PaO2 decreases outside PaCO2 may increase, and blood gas analysis may present as type II respiratory failure. Pulmonary function tests: In the acute stage, due to alveolar hemorrhage, erythrocyte hemoglobin can take up a certain amount of CO, so the pulmonary diffusion function Dco measured by CO increases instead. In chronic pulmonary fibrosis, pulmonary diffusion function is reduced, and pulmonary compliance, total lung volume and residual air volume decrease, showing restrictive ventilation dysfunction. In late stage combined with emphysema and pulmonary heart disease, a decrease in maximum ventilation and expiratory force in one second (FEV1) indicates mixed ventilatory dysfunction.
Other auxiliary examinations.
1. X-ray examination
(1) Acute exacerbation: Chest X-ray may be normal or may show a variety of manifestations. Mostly, we see increased texture in both lungs, diffuse patchy and speckle-like shadow in both lungs, which is obvious in the middle and lower lung fields and the intrapulmonary band, and sometimes it may fuse into a large patchy or cloudy flocculent shadow, a few patients show limited or unilateral lung lesions, and the hilar and mediastinal lymph nodes may be enlarged. In most cases, the lung lesions are significantly absorbed within 1 to 2 weeks, and some may continue for several months or recur.
(2) Chronic interictal phase: Extensive interstitial fibrotic changes are seen, and in severe cases, cystic hyaline areas may be present in the lung films.
2. CT examination of the chest can detect diffuse small nodular shadows in the lower and middle lungs bilaterally at an early stage.
Differential diagnosis
1. Secondary pulmonary ferritinosis is most commonly secondary to heart disease, especially mitral stenosis and chronic heart failure from various causes. Due to pulmonary stasis, prolonged increase in intrapulmonary capillary pressure, extravasation of blood and hemorrhage, patients may present with recurrent hemoptysis, deposition of iron-containing heme in the lung, phagocytosis by macrophages, visible iron-containing macrophages, microscopic examination of heart failure cells, i.e. iron-containing macrophages, positive Prussian blue reaction. Based on the history of heart disease, cardiac signs and pleural effusion examination, it is generally not difficult to differentiate.
This disease can also be seen as diffuse small nodules on chest radiograph, with shadows in both upper lung fields. Patients have symptoms of tuberculosis toxicity, rarely hemoptysis, no anemia, sputum negative for ferritin-containing macrophages, and effective anti-tuberculosis treatment.
This syndrome is characterized by recurrent hemoptysis, and the chest radiograph shows small nodular or lamellar shadows scattered in both lungs. There are clinical manifestations of glomerulonephritis. Anti-glomerular basement membrane (anti-GBM) antibodies can be detected in the patient’s serum; immunofluorescence examination of the basement membrane of glomeruli and alveolar capillaries shows immunoglobulin IgG and C3 deposits, which is an important distinction from IPH.
Diagnosis
In children under 10 years of age, if there are recurrent episodes of hemoptysis, dyspnea or iron deficiency anemia of unknown origin, developmental delay, and X-ray chest radiographs showing diffuse faint dotted and reticular shadows in both lungs, sputum or gastric fluid should be further examined several times, and if iron phagocytosis can be found several times, the diagnosis is established. The latter, in particular, has the same pulmonary manifestations as the disease, but is not complicated by nephritis and is negative for anti-glomerular basement membrane antibodies by immunofluorescence staining.
The etiology of this disease is unknown, so there is no specific treatment. The symptoms of milk-related disease can disappear after the withdrawal of milk, and the prognosis is good. Iron deficiency anemia responds well to iron, and blood transfusion is available for severe pulmonary hemorrhage. Corticosteroids may be helpful in acute attacks, but long-term application does not improve the prognosis. The efficacy of immunosuppressive agents and splenectomy is not yet certain.
The prognosis is not good in young children, but slightly better with age, with a general duration of about three years.
As the disease is still incurable, I would like to suggest some suggestions for care in life, from my own experience.
Healthy life, adjust the work and rest, away from the poor air, usually diet must avoid spicy things, fried and other junk food, you can eat more tomatoes, strawberries and other fruits. Occasionally you can drink soup with cordyceps, the effect is any cordyceps capsules can not compare, medicine is better than food. As the patient’s respiratory tract is very fragile, in fact, usually pay attention to good life, the condition will also improve in the long run.
Personally, I think it’s not ideal to rely on hormone therapy alone. If you can, insist on doing exercises that increase lung activity, such as practicing qigong and tai chi. However, you must not overdo it, sweat should be dried in time, not to be cold, because generally this disease patients immune system is low, you can supplement some drugs to improve immunity. For children, parents should take good care of them and try to improve immunity and lung activity, stay away from dust and smoke, and the disease may be eliminated at the time of development.
In addition, as far as treatment is concerned, although it is untreatable, but no one will give up hope of treatment, if treatment, it is recommended to use herbal medicine, because it is not conclusive that the tiny improvement in my condition is not because of eating herbal medicine, so it is also just a suggestion, but do not throw yourself at doctors, it is better to find a Chinese medicine doctor who has had experience in treatment.
Lastly, mental health is especially important. A person’s state of mind emotionally affects immunity, but maintaining a good state of mind is much more than that. Because of the disease, it is even more important to stay happy and optimistic. Adult patients or those with aggravated conditions should learn to be open. After the disease becomes serious, even if it does not affect other organ lesions, due to the lack of oxygen supply and so on, will naturally bring a lot of emotional problems, especially at night, easy to irritable depression, etc., with hobbies, or find something happy to distract, if there is a good circle of life, its happiness is also possible. Life is just a matter of trouble and care, nothing special. Treatment options
Drug treatment
There is no specific treatment. Early control of acute attacks is the key to avoid interstitial lung fibrosis.
1. Symptomatic treatment during acute attack should be bed rest, oxygen, stop taking cow’s milk, give hemostatic agents, and for anemia
Drug treatment for idiopathic pulmonary iron-containing hemoglobinopathies
Iron supplementation, blood transfusion if necessary.
2. AdrenocorticosteroidsAdrenocorticosteroids are more effective in controlling the symptoms in the acute phase. In the acute stage, hydrocortisone 4-5mg/(kg?d) is commonly used, later it can be changed to oral prednisone 1-2mg/(kg?d), and the dosage is gradually reduced to the lowest maintenance dosage 2-3 weeks after the symptoms are relieved, and the dosage is continued for six months, if it is repeated, the maintenance dosage can be used for 1-2 years.
3. Immunosuppressant adrenocorticotropic hormone treatment is ineffective, the immunosuppressant azathioprine 1.2-2.5mg/(kg?d) can be added, the dosage for adult patients is 50-100mg/d, no side effects can be continued for more than 1 year. The treatment course is 1.5 years with good results.
4. Plasma replacement plasma replacement can remove the persistent immune damage produced by immune complexes, so that patients’ clinical symptoms, chest X-ray and lung function can be improved.
5. Iron removal method To prevent excessive iron deposition in the lung causing lung tissue damage, iron complexing agents can be used to remove the iron deposited in the lung and stop the development of pulmonary fibrosis. Deferoxamine (deferoxmine) can be used for treatment at a dose of 25 mg/(kg?d), intramuscularly, and the amount of iron excreted from the urine can be increased significantly after the use of the drug. Iron complexation has certain toxic effects, so it is not widely used.
6. Symptomatic treatment is required for patients with combined pulmonary infection, pulmonary hypertension, pulmonary heart disease and respiratory failure.
Food therapy
Shredded rabbit and mushroom 1. celery and carp stir-fry
Ingredients carp 250 grams, fresh celery
50 grams, starch, ginger, garlic, soy sauce, sugar, vinegar, salt, monosodium glutamate, yellow wine, pickled sour pepper, vegetable oil appropriate amount.
Make the carp cut into shreds, celery cut into sections, the soy sauce, sugar, vinegar, monosodium glutamate, yellow wine, salt, starch, soup to make the sauce. Put a frying pan on a high fire, add oil and heat to 50%, put the shredded fish to stir-fry, drain the remaining oil, put ginger and pickled chili. Stir-fry celery fragrance, and then cook into the gravy, put in the bright oil, pot can be.
2. shredded rabbit meat and mushrooms
Ingredients cooked rabbit meat 100 grams, mushrooms
50 grams, 25 grams of white onion, chili oil, soy sauce, vinegar, sugar, sesame oil, sesame paste, pepper powder, monosodium glutamate appropriate amount.
Preparation: Cut the cooked rabbit meat and white onion into shreds, and cook the mushrooms. Put the green onion and mushroom on the bottom, cover the rabbit with shredded rabbit and put it on the plate. Mix the sesame sauce with soy sauce and sesame oil to make a flavorful sauce, then pour it over the rabbit and serve.
3. Quail and lily soup
Ingredients quail 1, lily 25 grams, ginger, onion, monosodium glutamate, fine salt appropriate amount.
Make the quail killed, remove the hair, claws, gutted and washed, put in boiling water, blanch, take out and cut; break the lily, wash, spare. Wash the ginger and onion, ginger patted, onion cut. Pot placed on a high fire, pour in an appropriate amount of water, put the quail, boil, the next lily, ginger, scallions, change to low heat stew until the quail cooked, add salt, monosodium glutamate stew for a few minutes, into the soup bowl can be served.
4. Lean meat and cabbage soup
Ingredients lean meat, cabbage heart 100 grams each, ginger, garlic, salt, monosodium glutamate, chicken oil a little.
Production of lean meat shredded, cleaned, shredded cabbage, put into boiling water, just cooked, put clear water rinse, drain the water for use; pot placed on a high fire, put the chicken oil burned five mature, put people garlic, fried golden brown, then add lean meat stir-fry, add fine salt, people soup cooked, then add cabbage heart boiling, put MSG can be served.