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Cited reference with China Academic Journal ago 10 1 Zhang Yong, Pei Li, FORCES, Zhang Yijun, Zhang Linsheng; cyclosporin A treatment of aplastic anemia efficacy [J]; Third Military Medical University; 2001 11 2 shift; cyclosporin A in ophthalmology [J]; foreign medicine. Ophthalmology Volume; 1996 02 3 Chen Xiangdong, Chen Minghua, Wengmeng Wu; cyclosporine A treatment of 1 case of Reiter disease [J]; Journal of Clinical Dermatology; 4 Chenlin Jun 01 1998, fruit was, Lin Peidi; cyclosporin A combined androgen treatment of adults with severe aplastic anemia [J]; Clinical Hematology; 1996 03 5 and Hong; Cyclosporin A treatment of aplastic anemia status and prospects [J]; Clinical Hematology; 1998 05 6 GUO Xiaonan, Xu Shirong, Ren Jinhai, Wang Ying, Lin Fengru, Yang Lin, Yao Seoul solid; with cyclosporin A joint program of treatment factors myelodysplastic syndrome [J]; Clinical Hematology; 2001 04 7 Zhang Mei, Li Han, Lee Moon-cheung; low-dose cyclosporine A treatment of PNH and AA-PNH 15 [J]; Chinese Journal of Clinical Pharmacy; 1999 02 8 Li Dong, Ji Peng; cyclosporine A in the treatment of blood diseases [J]; Chinese Journal of Clinical Pharmacy; 2000 03 9 Lin Jinying; low-dose cyclosporin A the treatment of refractory ITP [J]; Mathematical Medicine Journal; 2000 04 10 Yong Hui Wen, Gu Kim, Chou Hongxia, Wang I-sun, ANS; cyclosporine A treatment of children with aplastic anemia effects of [J]; Suzhou University ( Medical Sciences); 1999 02
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are disease of unknown etiology. Diffuse alveolar hemorrhage in this disease and secondary iron deficiency anemia is characterized. No other devices
Idiopathic pulmonary hemosiderosis disease MRI official involvement. Because pulmonary capillary repeated bleeding, oozing of blood hemolysis, in which globin part is absorbed, hemosiderosis in the lungs react. Pathological see increased lung weight. Section with wide brown pigmentation. Microscopic examination of alveolar and interstitial hemosiderin containing macrophages. There are varying degrees of diffuse pulmonary fibrosis, interstitial lung fibrosis and blood vessel elasticity. Electron microscopy diffuse capillary damage, edema and protein in endothelial cells with calm in the basement membrane. The disease occurs in children and young people, clinical symptoms and pulmonary hemorrhage and the suspension period. Paroxysmal or persistent cough, hemoptysis, and shortness of breath. Hemoptysis for hours or days, and gradually relieve itself, except this time pale, fatigue, the basic symptoms, but a few weeks, months, and then to relapse. No abnormal chest signs and more. Because anemia, cyanosis often hide. Often accompanied by pulmonary heart disease in patients with severe or clubbing. Hemoptysis is a common cause of death. Chest X ray showed hilar or lower two with scattered small nodules in the shadow of severe cases can be integrated into the shadow of ground glass sheet. Symptoms may improve, or even absorbed. Treatment with corticosteroids to control bleeding, but not long-term stability and prevention of disease recurrence, with chronic cases the effect is not significant, iron can alleviate severe anemia. Idiopathic pulmonary hemosiderosis idiopathic pulmonary hemosiderosis patients repeated the pulmonary capillary blood, red blood cells into the damaged hemosiderin deposition in the alveolar septum caused by the reaction. The rare disease, mainly as hemoptysis, iron deficiency anemia and a transient pulmonary infiltrates. Danger of this disease, most patients died of pulmonary bleeding profusely. Over the past more than the disease occurs in children and adolescents, in recent years, adults suffering from the disease compared to those before the increase. Pathological cause pulmonary hemosiderosis more than one cause of disease, etiology unknown, so it is called idiopathic pulmonary iron
Idiopathic pulmonary hemosiderosis flavin PAP blood disorders, and other reasons to show that those who differ. Period of extensive acute pulmonary hemorrhage, chronic phase is mainly interstitial pulmonary fibrosis. Iron lung higher than the normal 5 to 2000 times. Can be seen under the light microscope are obvious limitations to the expansion of alveolar capillaries, alveolar epithelial degeneration, loss, hyperplasia, interstitial hemosiderin phagocytosis of macrophage infiltration and interstitial fibrosis, alveolar, interstitial and vascular elastic fibers degeneration, electron microscopy examination revealed extensive capillary damage, endothelial swelling, basement membrane of protein deposition. The basis of this disease is pulmonary capillary barrier, but its cause is unknown, there are two main speculation: developmental defects, due to development and function of primary alveolar epithelial abnormalities, so that the mechanical stability of the occurrence of vascular disorder, or lung elasticity fiber developmental defects, but can not be confirmed by histological examination. caused by the immune response. The patient accumulation of mast cells in the lungs may have; plasma cells in the reticuloendothelial system, increased; 1 / 8 ~ 1 / 5 of the patients had eosinophilic leukocytosis; a few cases, while also suffering from other autoimmune diseases. It was found that some have been confirmed or possible pulmonary hemosiderosis as a disease of infants to milk for the skin test was positive, and positive serum precipitin milk, the symptoms disappeared after withdrawal of milk. But some have been confirmed as pulmonary hemosiderosis disease of the sick child, the test was negative. Therefore, the cause of this disease is not yet clear. Patients were mostly children aged 1 to 7, about 15% of patients over 16 years of age or greater. The incidence of childhood gender non-discrimination, adult men about women's double. Cause of a disease. Alveolar epithelial cell development and functional abnormalities, undermining the stability of alveolar capillaries, causing repeated bleeding
Idiopathic pulmonary hemosiderosis syndrome virus structure, but the cause of abnormal epithelial cells is unclear. 2. Abnormal pulmonary elastic fiber elastic fiber abnormalities in the acid mucopolysaccharide, the blood vessel wall to weaken and expand. 3. Some children with milk allergy skin test positive for milk, milk serum antigen found on the precipitin reaction. Disable the milk, the clinical symptoms disappeared. Heiner reports of cases of children with milk allergy were 4 patients who were diagnosed with IPH, of such patients as Heinersyndrome. However, milk allergy can not explain all of the children with disease. 4. With exposure to organophosphorus pesticides on Cassimos confirmed 30 cases in the Greece of children for this disease were investigated and found that 80% of children in rural growth, poor economic conditions, low protein diet; 50% of the children a continuous history of exposure to organophosphorus pesticides. Cassimos that pesticides may induce some children with this disease, more precisely, the basis of need further investigation confirmed. 5. Genetic factors in patients with the disease, Department of Inbreeding their parents, or brothers, the incidence of twins, so that genetic factors, but no gene confirmed. 6. Many scholars believe that the immune factors and immune related disease. Serum IgG, IgA, IgM were increased to IgA is more obvious in some patients increased eosinophils, mast cells in lung concentration. May have anti-nuclear antibodies, cryoglobulin antibodies. In some cases by the adrenal cortex hormones, immunosuppressants or plasmapheresis effective. These are supported by this disease and immune dysfunction. Check the appearance of general symptoms of lung consolidation was brown like the weight and volume increases, surface and scattered hemorrhagic spots section
Idiopathic pulmonary hemosiderosis blood disorder symptoms and diffuse brown pigmentation, hilar and mediastinal lymph node enlargement can be seen, and because of hemosiderosis and brown. Acute exacerbation of visible bleeding, chronic diffuse interstitial pulmonary fibrosis more apparent. 2. Optical microscopy alveolar epithelial cell swelling, degeneration, loss and proliferation, seen alveolar hemosiderin containing red blood cells or phagocytic cells, alveolar wall elastic fiber degeneration, capillary proliferation, basement membrane thickening of small pulmonary arteries elastic fiber degeneration, hemosiderosis, intimal fibrosis, hyalinization. Lymphatic vessels around the lymphocytes, plasma cells and hemosiderin macrophage infiltration, lymphatic expansion. Late disease can lead to diffuse pulmonary fibrosis, emphysema, bronchiectasis, and so on. 3. Electron microscopy extensive alveolar capillary damage, endothelial cell edema, alveolar epithelial cell hyperplasia, focal basement membrane was broken, protein deposition in the basement membrane, loss of normal basement membrane structure. Clinical manifestations of the clinical manifestations of this disease depends on the severity of pulmonary hemorrhage and duration of acute bleeding in patients with extensive lung, showing onset of dyspnea, cyanosis, cough, hemoptysis, tachycardia. Many sick child to a small amount of pulmonary bleeding continued, intermittent small amount of blood or blood-stained sputum slightly, but sometimes the blood exists only in the alveolar septum, but not spit up, the patient had fever, paleness, fatigue, weakness, chronic dry cough, not gain weight. Due to repeated pulmonary hemorrhage, a large number of red blood cell destruction, into hemosiderin deposition in the alveolar septum, there are macrophages, not for heme synthesis, resulting in the availability of iron storage in vivo depletion, chronic iron deficiency anemia . Part is carried to the bronchial macrophages with phlegm spit up, so can often be found in sputum hemosiderin containing macrophages (macrophage iron cells), sputum and throat in children is not the stomach, the gastric juice can also be found macrophage iron cells. Gelatin is a traditional recipe blood, lactic acid is a good divalent ferrous iron preparations, blood products on the market a lot of recipes to use them separately as. The edge of the sheet iron is ferrous lactate, gelatin and protein effects of zinc are used as ingredients, iron, nutrition and blood plus one three, to better prevention and improved anemia, enhance human immunity. Lung X-ray examination showed bleeding lungs at the middle and lower lung field near the hilar broad, fuzzy patchy infiltrates, and some patients hilar lymph nodes. Infiltration can be dissipated 1 to 2 weeks, but after repeated hemoptysis hemosiderin deposition in the alveolar septum, fibrosis occurs, it can be seen intermittent network of X-ray density of deep shadow and the shadow of the small spots. Massive pulmonary hemorrhage can occur at any time, most of the patients died due to severe pulmonary hemorrhage, only a minority of patients survive and develop Cor. Laboratory diagnosis of diagnosis: patients with recurrent hemoptysis (especially children), unexplained iron deficiency anemia, chest X-ray diffuse small nodules appeared on or in pieces
Idiopathic pulmonary hemosiderosis Disease Tests like, reticular shadows, should be suspected of the disease. Need for further examination or lung biopsy, such as found in typical hemosiderin macrophages can confirm the diagnosis. Laboratory tests: blood showed hypochromic iron-deficiency anemia of small cells, reticulocytes increased. Because iron deposition in the alveolar macrophages, can not use transit as a synthesis of hemoglobin, serum iron and iron saturation was significantly reduced red blood cell fragility test normal saline. Peripheral blood eosinophils may be increased ESR. The destruction of hemoglobin in the alveoli, it can increase serum bilirubin, serum IgA increased, direct test, agglutination test, macrophage differentiation can be positive agglutination test, serum lactate dehydrogenase may be increased, involving the heart by ECG may be abnormal. Sputum, gastric juice, bronchial lavage fluid or lung biopsy to find the typical hemosiderin macrophages are important for diagnosis. Lung biopsy and bronchoscopy: alveolar hemorrhage for a long time, fiberoptic bronchoscopy can be seen in the endobronchial blood, lung biopsy through bronchoscopy or open lung biopsy and other methods to do light and electron microscopy of the pathology observed in order to clear the alveolar causes of bleeding. Blood gas analysis: patients with early and more normal, alveolar hemorrhage, or extensive pulmonary interstitial fibrosis, PaO2 decreased, normal or decreased, and might appear as type respiratory failure, post-emphysema, pulmonary heart disease and respiratory failure, downward trend can be increased PaCO2 PaO2, blood gas analysis can be expressed as type respiratory failure. Pulmonary function test: the acute phase due to alveolar hemorrhage, red blood cell hemoglobin intake of a certain amount of CO, so the CO measured pulmonary diffusion Dco actually increased, anemia with a hemoglobin value of Dco be corrected. Chronic pulmonary fibrosis, decreased lung diffusion capacity, lung compliance, total lung capacity and residual volume decreased, showing a restrictive ventilatory dysfunction. Complicating emphysema, pulmonary heart disease, the maximum voluntary ventilation, forced expiratory one second (FEV1) decline, the show mixed ventilation dysfunction. Other laboratory examinations: 1.X-ray examination (1) acute attack: chest X ray may be normal, also display a wide range of performance. More common two markings, both lungs diffuse plaques, spots like shadow, to the middle and lower lung fields and lung with clear, sometimes flaky or goes convergence flocculent shadows National Cheng Kung University, a small number of patients show limited or unilateral lung lesions, hilar and mediastinal lymph nodes may be swollen. Most cases of lung disease in 1 to 2 weeks was absorbed, and some may last a few months or repeatedly. (2) chronic intermittent episodes of: See the extensive interstitial fibrosis, severe lung transparent film may have cystic areas. 2. Chest CT, chest CT examination can be detected earlier and lower bilateral diffuse small pulmonary nodular shadows. Complications 1. Secondary pulmonary hemosiderosis is a disease most commonly secondary to heart disease, especially mitral stenosis and the various causes of chronic heart failure. Because pulmonary congestion, long-term increase in pulmonary capillary pressure, blood extravasation and bleeding, the patient may appear repeated hemoptysis, hemosiderin deposition in the lungs, macrophages, showing hemosiderin macrophages, microscopic examination heart cells can be seen that hemosiderin macrophages, Prussian blue-positive. According to history of heart disease, cardiac symptoms and pleural effusion examination, generally not difficult to identify. 2. Hematogenous disseminated tuberculosis, chest X-ray of the disease can also be found diffuse small nodules, the shadow on the lung field is more than a two. TB patients with symptoms of poisoning, rarely hemoptysis, and no anemia, sputum macrophages hemosiderin-negative, anti-TB treatment is effective. 3. Pulmonary hemorrhage - nephritis syndrome (Goodpasturesyndrome) This syndrome is characterized by recurrent hemoptysis, chest X-ray showed small lung nodules scattered in the shadow or flake. Performance to clinical glomerulonephritis. Can be detected in serum anti-glomerular basement membrane (anti-GBM) antibody; immunofluorescence of glomerular and alveolar capillary basement membrane, IgG and C3 are calm, there are important differences between this and the IPH. Diagnosis of children under the age of 10, if repeated episodes of hemoptysis, dyspnea, or unexplained iron deficiency anemia, growth retardation, X-ray chest showed diffuse bilateral lung and reticular shadows fuzzy spots, several investigations should be further sputum or gastric juice, if many cells found in macrophage iron, diagnosis is established, subject with mitral stenosis, the lungs of connective tissue disease, hematogenous disseminated tuberculosis and 's syndrome and other identification, In particular the latter, the pulmonary manifestations of the disease with the same, but the disease is not complicated by glomerulonephritis, and immunofluorescence staining of anti-glomerular basement membrane antibodies were negative. Etiology of this disease is unknown, so no specific treatment, the incidence and milk were the withdrawal of milk related symptoms can disappear, the prognosis is good. Iron deficiency anemia on the good response, severe pulmonary hemorrhage may transfusion, corticosteroids in the acute attack might help, but long-term use can improve the prognosis. Immunosuppressive agents, the efficacy of splenectomy and other hard to sure. Child poor prognosis, with age, the prognosis is slightly better, the general course of three years or so, there are those who live longer. As currently incurable diseases, I made some life care recommendations, from my personal experience: healthy living, adjusting the rest, away from the place where the air is poor, usually have to avoid eating spicy things, fried, and junk food , you can eat tomatoes, strawberries and other fruits. Cordyceps can be used occasionally to drink soup, the effect can not match any Cordyceps capsule, the drug less Sibu. The respiratory tract is very vulnerable, in fact, pay attention to normal life, a good long run but also will improve the condition. Personally think that using hormone therapy alone is far from ideal. Always pay attention to, if possible, stick with the movement to increase the amount of the lung activities, such as qigong, tai chi practice. But certainly not excessive, sweating paint dry in time, not cold, because the general disease in patients with low immunity, you can make some improvement in the immune system drugs. For children, parents, care should be comprehensive, seeking to enhance immunity and lung activity, away from dust and smoke, to eliminate the disease in the developing Shiyou possible. In addition to treatment, although is no cure, but no one would give up the hope of treatment, if treatment is recommended to use traditional Chinese medicine, as there is not conclusive improvement in my condition is not because of the tiny Chinese eat, so only recommend, but do not trying everything, looking for experience in the treatment of Chinese medicine have been better. Finally, mental health is particularly important, human emotions can affect the mentality of immunity, but to maintain a good attitude is far more than that. Because of illness, more needs to be happy optimistic. Sicker patients or adults who must learn to calm point. After the disease becomes severe, even without affecting other organ damage, due to lack of oxygen, etc., will naturally bring a lot of emotional problems, especially at night, easily agitated depression, with hobbies, or find something fun to do to divert attention, if there is a good living area, enjoyable is also possible. Life is but a point to trouble carefully, nothing special. Drug treatment regimens currently no specific treatment. Early control of acute attacks is to avoid the key to pulmonary fibrosis. 1. Symptomatic treatment of acute exacerbation should be bed rest, oxygen, stop taking the milk to give hemostatic agents, anemia should be
Idiopathic pulmonary hemosiderosis Disease drug therapy iron supplements, if necessary, require blood transfusions. 2. Adrenocorticotropic hormone adrenocorticotropic hormone control acute symptoms more positive effect. Common acute hydrocortisone 4 ~ 5mg / (kg? D), can be changed after oral prednisone 1 ~ 2mg / (kg? D), relief of symptoms after 2 to 3 weeks tapered to the lowest maintenance dose, continuous medication six months, if repeated, can be used to maintain the amount to 1 to 2 years. 3. Immunosuppressive drugs ineffective glucocorticoid therapy, immunosuppressive agents can be added with azathioprine 1.2 ~ 2.5mg / (kg? D), the amount of adult patients 50 ~ 100mg / d, no side effects, drug use more than 1 year of sustainable . 1.5 years of treatment effect is good. 4. Plasmapheresis plasmapheresis to remove immune complexes generated by the persistence of immune injury, the clinical symptoms, chest X ray, pulmonary function improved. 5. Iron removal method to prevent excessive iron deposition in the lungs causing lung injury, can get rid of iron chelators of iron deposition in the lungs, preventing the development of pulmonary fibrosis. Available deferoxamine (deferoxamine, deferoxmine) treatment, the dose 25mg / (kg? D), intramuscular injection, after treatment can make the iron from the urine excretion increased significantly. Due to the toxicity of iron chelators have a certain effect, it is not widely used. 6. Symptomatic treatment of pulmonary infection, pulmonary hypertension, pulmonary heart disease, respiratory failure, need for appropriate treatment. Therapeutic method of rabbit meat mushrooms wire 1. Lei Lei leavened raw celery 250 grams, 50 grams of fresh celery, starch, ginger, garlic, silk, soy sauce, sugar, vinegar, salt, MSG, rice wine, hot acid, pepper, vegetable oil amount. Production of the carp cut wire, celery cut into sections, the soy sauce, sugar, vinegar, MSG, rice wine, salt, starch, soup transferred into the juice. Wok stir the upper and lower into hot oil till 5, release the silk powder leavened, Lek to the remaining oil, put ginger, sour hot pepper. Paragraph Stir celery flavor, and then cooked into the sauce, put people light oil, can clean wok. 2. Rabbit meat, rabbit meat cooked mushrooms 100 grams of raw silk, 50 grams of mushrooms, 25 grams of light blue, chili oil, soy sauce, vinegar, sugar, sesame oil, sesame paste, pepper powder, MSG amount. Production will be cooked rabbit meat, very light blue strips, respectively, mushrooms cooked. Onions, mushrooms, bottom, cover up rabbit wire, poured into a tray. Soy sauce, sesame graded by adjusting the powder, sesame oil and mix thoroughly into the sauce, pour over rabbit Sibu can be eaten. 3. Tom raw quail quail 1 Lily, Lily 25 grams, ginger, onion, monosodium glutamate, thin salt amount. After killing the quail made to the hair, to paw, to the viscera washed in water, placed in boiled, remove and cut into pieces; the lily breaking off valve, wash and set aside. The ginger, spring onions washed ginger film break, spring onions and cut into sections. Placed each on a pan, pour the amount of water, release quail, boil, lower Lily, Ginger, spring onion, cooked quail Dunzhi low heat, add salt, MSG stew a few minutes into the soup bowl that is edible. 4. Lean meat, lean meat, raw cabbage soup, cabbage heart of the 100 grams, ginger, garlic, salt, monosodium glutamate, chicken fat little. Production of lean meat into strips, washed cabbage, shredded, placed in boiling water, remove when just cooked, put clean water rinse and drain water stand; pot placed each on five mature under Cantharellus burning, put people garlic, fried golden brown, plus lean meat fried together, add thin salt, one soup cooked, add boiled cabbage, can eat into the MSG


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