Idiopathic melmomaryhaemosiderosis (IPH) is a rare disease with unknown etiology and intermittent bleeding in the lungs. More than 200 cases were reported internationally and more than 120 cases were reported domestically (by 1994). After the red blood cells in the alveoli are destroyed, globin is absorbed, and hemosiderin is deposited in the lung tissue to cause a response. Iron deficiency anemia can be secondary to repeated bleeding.
Idiopathic hemosiderin disease
Idiopathic hemosiderin
Chinese name: Idiopathic hemosiderinosis
English name: idiopathic pulmomary haemosiderosis
English name: IPH
Idiopathic pulmomary haemosiderosis (IPH) is a rare disease with unknown etiology and intermittent bleeding in the lungs. More than 200 cases were reported internationally and more than 120 cases were reported domestically (by 1994). After the red blood cells in the alveoli are destroyed, globin is absorbed, and hemosiderin is deposited in the lung tissue to cause a response. Iron deficiency anemia can be secondary to repeated bleeding.
Idiopathic hemosiderinosis symptoms
Idiopathic hemosiderin
Detailed description of symptoms
This disease is more common in children, mainly in 1 to 7 years old, 15% over 15 years old, the ratio of male to female is 2: 1, there is no obvious familial. Symptoms depend on the degree of bleeding in the lungs. Mild persistent chronic bleeding can include dry cough, fatigue, pale skin, weight loss, and even clubbing fingers. Bloody sputum, hemoptysis, low fever, and chest pain may occur during acute bleeding. In the later period, there may be symptoms of dyspnea and even heart failure. In the acute phase and secondary infections, obvious wet rales can be heard. Fecal occult blood was positive.
The X-ray showed multiple, fused, spotted shadows in the middle and lower fields of the two lungs with blurred edges. After the pulmonary hemorrhage stopped, the shadows improved within a few weeks. In patients with persistent moderate bleeding, intrapulmonary lesions may be miliary.
The pathogenesis of idiopathic hemosiderin
Idiopathic hemosiderin
unknown. It is presumed to be related to several aspects: abnormal development of lung epithelial cells, mechanical instability of pulmonary interstitial capillaries, and repeated bleeding; immune dysfunction, such as eosinophil infiltration in lungs, mast cells, plasma cells Increasing, some patients have a positive condensation set test. Some patients may be complicated by rheumatoid arthritis, polyarthritis, myocarditis, and Goodpasture syndrome.
Other reasons mentioned are animal protein intake, inhalation of toxic substances (such as organic pesticides).
Pathological changes of idiopathic hemosiderin
Idiopathic hemosiderin
Lung weight increase, diffuse brown pigmentation can be seen on the section, microscopic examination may have alveolar epithelial necrosis, hyperplasia, local capillary dilatation, alveolar and interstitial macrophages phagocytosing heme-containing, and diffuse interstitial Fibrosis. Electron microscopy showed extensive capillary endothelial cell swelling and protein deposition on the inner membrane. After the lung tissue was washed and dried, the iron content in the tissue was still 5 to 200 times higher than that in normal lungs. And proportional to the condition.
Idiopathic hemosiderin diagnosis
Idiopathic hemosiderin
Preliminary diagnosis can be made based on repeated hemoptysis, blood in the sputum, spotted shadows on the edges of the lungs, and secondary iron-deficiency anemia. Phagocytic cells can be found through sputum, bronchoalveolar lavage fluid, and lung biopsy. It can be diagnosed after containing blue hemosiderin and excluding cardiogenic (congestive) factors.
Causes of Idiopathic Hemosiderin
unknown. It is presumed to be related to several aspects: abnormal development of lung epithelial cells, mechanical instability of pulmonary interstitial capillaries, and repeated bleeding; immune dysfunction, such as eosinophil infiltration in lungs, mast cells, and plasma cells in 1/8 of patients Increasing, some patients have a positive condensation set test. Some patients may be complicated by rheumatoid arthritis, polyarthritis, myocarditis, and Goodpasture syndrome.
Other mentioned reasons are animal protein intake, inhalation of toxic substances (such as organic pesticides).
Lung weight increase, diffuse brown pigmentation can be seen on the section, microscopic examination may have alveolar epithelial necrosis, hyperplasia, local capillary dilatation, alveolar and interstitial macrophages phagocytosing heme-containing, and diffuse interstitial Fibrosis. Electron microscopy showed extensive capillary endothelial cell swelling and protein deposition on the inner membrane. After the lung tissue was washed and dried, the iron content in the tissue was still 5 to 200 times higher than that in normal lungs. And proportional to the condition.
Clinical manifestations of idiopathic hemosiderin
1, the first attack: the onset is more sudden, typical manifestations are fever, cough, hemoptysis and anemia. Cough is generally severe, with a few having difficulty breathing and cyanosis. Mucus and sputum are common, with pink blood in it, and severe hemoptysis may occur in severe cases. At the same time, the child developed anemia and fatigue. Examination of the lungs showed no specific manifestations, and there may be reduced breath sounds or a small amount of dry rales and fine wet rales.
2, recurrent period: children with intermittent recurrent attacks after the first episode, which can last up to several years. The above manifestations occurred during the attack. There is also a cough during the intermittent period, and small brown particles can be seen in the sputum. Anemia is mild to severe. Most of the children did not pay attention to the blood in the sputum, and the infants and young children swallowed more sputum. Parents mostly complained of anemia and cough, and the misdiagnosis rate was high.
3. Sequelae period: Repeated seizures cause pulmonary fibrosis and affect respiratory function. Hypoxic cyanosis is common and can cause pulmonary heart disease. On examination, hepato-splenomegaly and clubbing fingers and toes were also found. Pulmonary hemorrhage stopped in some patients, but most patients still had intermittent attacks.
This disease is more common in children, mainly in 1 to 7 years old, 15% over 15 years old, the ratio of male to female is 2: 1, there is no obvious familial. Symptoms depend on the degree of bleeding in the lungs. Mild persistent chronic bleeding can include dry cough, fatigue, pale skin, weight loss, and even clubbing fingers. Bloody sputum, hemoptysis, low fever, and chest pain may occur during acute bleeding. In the later period, there may be symptoms of dyspnea and even heart failure. In the acute phase and secondary infections, obvious wet rales can be heard. Fecal occult blood was positive.
The X-ray showed multiple, fused, spotted shadows in the middle and lower fields of the two lungs with blurred edges. After the pulmonary hemorrhage stopped, the shadows improved within a few weeks. In patients with persistent moderate bleeding, intrapulmonary lesions may be miliary.
Idiopathic hemosiderin cases
Case 1, male, 6 years old, had recurrent anemia with cough, had been hospitalized 5 times, and was suspected of IPH, but did not find hemosiderin-containing macrophages after multiple sputum checks, so he was diagnosed with nutritional anemia and bronchitis She was discharged after treatment. For the sixth time, he was admitted to the hospital with respiratory distress due to exacerbation of the condition, cough, severe dyspnea, audible lungs and wet rales.
Examination: T 38.5 ° C, R 92 times / min, severe anemia face, cyanosis of lips, nasal crests, and three concave signs. The respiratory sounds in the lower lungs of both lungs were weakened, dullness was percussive, and the heart circle was enlarged. The heart rate was 180 beats / min. The heart sounds were dull and regular. Chest CT: The light transmittance of both lungs is reduced, and the lung parenchyma is "ground-glass-like". The density of miliary small nodules can be seen in both lungs, and the lung texture is increased, thickened, and blurred. Blood routine: RBC 2.09 × 1012 / L, Hb 47g / L, MCV 80.9fl, MCH 22.5pg, MCHC 267g / L. A large number of inflammatory cells, bronchial epithelial cells and a large number of phagocytic cells were found in the sputum. The phagocytosed hemosiderin particles were found in the phagocytes, which was clearly diagnosed as IPH. After comprehensive treatment such as strengthening the heart, correcting hypoxemia and acidosis, and effective nursing, the symptoms were obviously relieved. The child improved and was discharged from the hospital. He was still maintained with a small amount of hormones. He was followed up by telephone and no recurrence was seen.
Case 2, female, 1 year old. The child was pale, feverish, coughed, and had blood in his sputum two months ago. He was hospitalized twice, diagnosed with heart failure and anemia, and treated with antibiotics and blood transfusion. Hemostasis improved and was discharged. She has been pale since then, and this time with the same symptoms as above, there was an exacerbation for 3 days, accompanied by shortness of breath, vomiting and dyspnea, bloodshot and vomiting in the sputum and entered the station.
Examination: T 38.5 , R 84 times / min, normal development, poor nutrition, indifferent expression, severe anemia face, cyanosis of lips, pale face, cold limbs, thickened lung texture, audible noise, liver The costal margin and xiphoid were 3 cm below each other, and the spleen could be touched. RBC 1.44 × 1012 / L, Hb 36g / L, serum iron 9mol / L, suspected to be pulmonary hemosiderin. The sputum was collected twice within half a day, and hematocrine-containing macrophages were found and diagnosed as IPH. After transfusion, antibacterial, anti-heart failure, and hormone application and careful care, his condition improved and he was discharged from hospital. No recurrence has been observed during telephone follow-up.
Essentials of nursing for idiopathic hemosiderin
1. Bed placement and ward management: Place children in non-infectious wards to avoid sharing the same room with children with infectious diseases such as pneumonia and diarrhea to prevent cross-infection. The indoor sunlight should be sufficient, the air should be fresh, and the suffocation temperature should be maintained at 18 22 , and the relative humidity should be 50% 60%. Strictly implement the isolation and disinfection system in the ward, and disinfect the ultraviolet radiation once / d, and open the window twice. / d. Avoid air convection during ventilation, prevent cold, strictly control visits and prevent various infections.
2. Diagnosis points and differential diagnosis: First, carefully observe the clinical performance and actively provide diagnostic evidence to help clear the diagnosis; then, master the differential diagnosis:
IPH is similar to acute miliary tuberculosis, but acute miliary tuberculosis is a miliary shadow with uniform distribution and density throughout the lung field, with obvious symptoms of tuberculosis, and can be checked for sputum and other tests.
Pulmonary hemorrhage at rest, chronic prolonged evidence period and rheumatoid mitral valve disease caused by rheumatoid deposits: Rheumatoid mitral valve disease caused by pulmonary hemosiderin patients often have rheumatism The history of mitral valve disease and the corresponding changes in heart shadow, so it can be identified in combination with clinical.
3. Observation and nursing of complications: The course and prognosis of the disease are related to the number and degree of pulmonary hemorrhage, and there is no obvious respiratory symptoms and X-ray manifestations when there is a small amount of bleeding. Excessive pulmonary hemorrhage can cause respiratory failure and heart failure. Patients should closely monitor the amount and frequency of hemoptysis, report to the physician in time to control the development of bleeding, and pay attention to the presence or absence of heart failure and be prepared for rescue.
4. Clinical observation and nursing
Nursing care when applying digitalis drugs: Most of the patients have heart failure, and digitalis drugs are preferred for correcting heart failure, but the amount of this drug is close to the amount of poisoning, and the safety factor is small. For example, using rapid digitalis method, More prone to toxic reactions. Therefore, the medication should be accurate and the dosage strictly. When the shift is handed over, the medication time should be clear, and the response after the medication should be closely observed. Pay attention to observe the BP, P, R, heart rate and urine output.
Observation and nursing of glucocorticoid therapy:
The use of high-dose hormones for the prevention and control of infection can reduce the immune function, and it is easy to get infected. For this reason, prevention should be the main factor to prevent all cross-infection factors. Once the infection occurs, control it with antibacterial drugs in time;
Preventing fractures: After the application of large doses of hormones, the body showed negative nitrogen balance, increased urine calcium excretion, and prone to osteoporosis. Therefore, in addition to supplementing vitamin D and calcium, intramuscular injection of nandrolone phenylpropionate should be used to promote protein synthesis;
prevent electrolyte disorders and diabetes. Since such drugs can reduce sodium and potassium, intake of sodium salts should be controlled. Patients can be instructed to eat more potassium-rich foods, such as oranges, red dates, celery, etc., and take blood regularly to detect blood potassium;
Do a good job of psychological care for children and their families, adhere to medication and consolidate treatment. For young children, you can make a funny hug. For older children, you can tell stories and look at pictures to make them receive treatment and care smoothly.
Correcting anemia: Generally speaking, iron supplementation is not recommended to correct anemia, and the condition is aggravated because iron supplementation increases the iron content of the lungs. If serum iron is below normal, blood transfusions can be used to correct anemia.
5. Diet Nursing: The causes of the disease may include:
There is mechanical instability of pulmonary interstitial capillaries and repeated bleeding;
Immune disorders, some patients have infiltration of eosinophils and mast cells in the lung, and some have a condensation test positive;
Other reasons, such as ingestion of animal protein and inhalation of toxic substances. Therefore, increase immune function and avoid contact with allergens, such as milk and other foreign proteins are not suitable for consumption. Eat more nutritious and digestible foods and limit sodium salts appropriately.
Idiopathic hemosiderin treatment
Hormonal therapy can be tried in the acute phase. Prednisone is usually used at a dose of 1 to 2 mg / (kg · d). After 2 to 3 weeks, the dose is gradually reduced to a maintenance dose. Symptomatic treatment includes iron treatment for iron deficiency anemia, and antibiotic treatment after secondary infection. The course of the disease is very different, with 68 cases reported, 20 deaths (29%) within 3 years, 17 cases of activity (25%), 12 cases of stability (18%), and 19 cases (28%) of normal. Corticosteroids are currently the first choice for treatment, and can be administered intravenously during the onset period, followed by oral prednisone 1-2mg / kg.d for 4 weeks, which is gradually reduced and maintained for 6 months to 2 years. If not, try other immunosuppressive agents, such as 6-MP, cyclophosphamide, etc. It is currently believed that low-dose hormones and long-term maintenance therapy can reduce the number and extent of attacks and improve long-term prognosis. Some people have tried deferoxamine in an attempt to expel iron from the lungs, but the effect is still uncertain. In recent years, some people have used lung lavage during the remission period to remove hemosiderin particles, which is believed to reduce the incidence of pulmonary fibrosis.
Idiopathic hemosiderinosis is safe to use
1. There is no special treatment for idiopathic pulmonary hemosiderin. Anemia is effective with iron. Treatment with corticosteroids and immunosuppressants such as cyclophosphamide and azathioprine can temporarily reduce symptoms in some cases.
2. The main symptoms of idiopathic pulmonary hemosiderin include cough, hemoptysis, shortness of breath, fatigue, pale, and no increase or decrease in weight. Among them, hemoptysis is prominent, and the amount of hemoptysis varies. The few who have bloody sputum in their sputum and the ones with bloody sputum in their mouth. Large mouth hemoptysis is rare, but it can be fatal. However, there are a few patients without hemoptysis. Exacerbation of dyspnea in the later stages, more often with club-like fingers and hepatosplenomegaly, may cause heart failure symptoms due to pulmonary hypertension.
3. The course of disease is inconsistent, and the survival time ranges from several weeks to many years. In some cases, spontaneous remission can occur, and small bleeding in the lungs can continue during remission. Most of the causes of death were heart failure or massive pulmonary hemorrhage.
Idiopathic hemosiderin