Pulmonary alveolar proteinosis

Overview of pulmonary alveolar proteinosis (pulmonary alveolar proteinosis) means unexplained diffuse alveolar protein calmly, first by Rose in the 1958 report, So far the literature about 400 ~ 500 cases, 1,965 cases in the country report. Clinical for sexual characteristics of shortness of breath, bronchoalveolar lavage diagnosis and treatment is the principal means. Prognosis big difference, self-healing, but also found that after a year or two deaths. Cause unknown etiology, and to speculate on several factors : If a large number of respirable dust (aluminum, silica, etc.) immune function decline (especially in infants), genetic factors, alcohol, microbial infection. For infections, is sometimes difficult to identify the primary factors causing or secondary to pulmonary alveolar proteinosis. For example, cytomegalovirus, Pneumocystis carinii, histoplasmosis infections, etc. are found within the alveolar protein calmly. Although the initiating factor is not yet clear, but basically agreed to the pathogenesis of lipid metabolism disorders caused. As a body that is, external factors induced alveolar surfactant in abnormal metabolism. So far, more studies are alveolar macrophage cells, Animal experiments proved macrophage phagocytosis of dust after its viability decreased significantly, and the patient fluid macrophages particles normal cell activity could decline by bronchoalveolar lavage treatment, alveolar macrophage activity can be increased. And the study found no type II cell protein, lipid metabolism body nor abnormal. It is generally considered that the disease and decrease the ability to remove. Pathological changes in the lungs showed huge seizure can significantly stiffen, weight increase. Real change and pulmonary emphysema compensatory coexist. Optical microscope normal alveolar structure, alveolar were filled with fine granular structure of the non - PAS-positive protein-like substance, type II cell hyperplasia and hypertrophy, intracytoplasmic lamellar body increased, lamellar bodies were arranged in rings. Macrophage number and volume also increased significantly. Pulmonary interstitial fibrosis may have been made, the author suggests supraclavicular lymph nodes to be checked and protein-like substance. Diagnostic chest X-ray findings of pulmonary infiltration concentric without heart failure when the suspect and the performance of the disease. Bronchoalveolar lavage found to be dirtier, creamy white or yellow liquid, which precipitated a large number of unstructured eosinophilic body Diagnosis will be clear. If combining bronchoscopy lung biopsy is a more definite diagnosis. Currently very few open lung biopsy required. Reports that the sputum of surfactant protein A is a simple means of diagnosis. Clinical performance ratio of male to female was 2 ~ 3:1, can occur at any age, mostly in the 20 ~ 50 years of age. Misprision of onset, symptoms of sexual shortness of breath. About 1 / 3 cases with low heat, the majority of cough, sputum was milky white bubble-like, sometimes massive, pipe-type morphology or cough. Medical respiratory movement may have weakened mildly voiced spontaneous delivery, pulmonary obvious pathological bronchial breath sounds, occasionally wet rales. Severe have clubbed fingers, cyanosis and pulmonary hypertension performance. X-ray showed perihilar diffuse alveolar infiltration, the butterfly was accused of, bilateral symmetry is not necessarily very, sublimity pulmonary edema. Treatment has not yet found an effective drug. At present, effective and widely used for bronchial lavage, conditional should in general anesthesia whole lung lavage. Bronchoscopy is also feasible under lobes, sections lavage. Effects similar, but in patients with more difficult for law Branchofiberoscope tolerance, and should not be great capacity lavage. Epidemiological sex ratio of 2 ~ 3:1, can occur at any age, mostly in the 20 ~ 50 years of age. Misprision of onset, symptoms of sexual shortness of breath. About 1 / 3 cases with low heat, the majority of cough, sputum was milky white bubble-like, sometimes massive, pipe-type morphology or cough. Medical respiratory movement may have weakened mildly voiced spontaneous delivery, pulmonary obvious pathological bronchial breath sounds, occasionally wet rales. Severe have clubbed fingers, cyanosis and pulmonary hypertension performance. X-ray showed perihilar diffuse alveolar infiltration, the butterfly was accused of, bilateral symmetry is not necessarily very, sublimity pulmonary edema. Children prognosis in patients with poor prognosis, died a few years, adults are self-healing cases, there are more patients after relapse. About half of patients progressing or concurrent infections, respiratory failure in 5 ~ 10 deaths.