肺泡微结石症三例(英文)

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Introduction Pulmonary alveolar microlithiasis (PAM) is a rare diffuse lung disease characterized by the accumulation of calcium phosphate microliths within the alveoli.The etiology and pathogenesis of the disease are still unknown.Consequently,there is no known effective treatment[1].In this report,we present the clinical,radiological,pathological findings of three patients with PAM,emphasizing the diagnostic and therapeutic views. Case report Case 1 A 16-year-old boy was admitted to our hospital on Jul 3,2008,for evaluation of abnormalities noted on previous chest X-ray films.His local physician had suggested that he could have far-advanced pulmonary tuberculosis.The patient was totally asymptomatic,denying dyspnea,cough,hemoptysis,chest pain,loss of weight,or night sweats.The patient had no known exposure to toxic dusts.His family history disclosed no pulmonary disease;he had one younger healthy sister.Physical examination was unremarkable.There was no clubbing and auscultation showed normal breath sounds with no adventitious sounds.The laboratory data,including urine analysis,sweat chloride,blood glucose,calcium and phosphate blood levels,renal and liver function tests,and thyroid hormone and uric acid blood levels were within normal ranges.Results of skin tests for tuberculosis,and studies for acid-fast bacilli,routine pathogens,and malignant cells were negative.Pulmonary function tests showed a mild restrictive respiratory defeat with low diffusion capacity.Chest X-ray film revealed extensive uniform,bilateral sand-like miliary densities of both lungs(sandstorm lung) obscuring the cardiac outlines,most marked in both hilar regions and fading out toward the apices and peripheral portions of the lungs.The minor fissure was thickened.High resolution CT revealed diffuse ground glass opacities throughout both lungs with micronodular densities.Interlobular septal thickening,more prominent in the anterior parts of both lungs,and linear calcifications along the pleura were also seen on HRCT scans (Figure 1,2).The case was authenticated by lung biopsy after video-assisted thoracoscopic surgery (VATS).The cut surface was hard,and demonstrated numerous sand-like concretions,making cutting difficult (Figure 3).Microliths were formed in the alveolar space.They ranged from 0.01 to 3 mm in size and they were usually irregular or bosselated,and there was interstitial fibrosis (Figure 4).He is asymptomatic after one year of follow-up. Introduction Pulmonary alveolar microlithiasis (PAM) is a rare diffuse lung disease characterized by the accumulation of calcium phosphate microliths within the alveoli. The etiology and pathogenesis of the disease are still unknown. Conclusion, there is no known effective treatment [1]. report, we present the clinical, radiological, pathological findings of three patients with PAM, emphasizing the diagnostic and therapeutic views. Case report Case 1 A 16-year-old boy was admitted to our hospital on Jul 3, 2008, for evaluation of abnormalities noted on previous chest X-ray films. His local physician had suggested that he could have far-advanced pulmonary tuberculosis. The patient was totally asymptomatic, denying dyspnea, cough, hemoptysis, chest pain, loss of weight, or night sweats. patient had no known exposure to toxic dusts.His family history disclosed no pulmonary disease; he had one younger healthy sister. Physical examination was unremarkable .here was no clubbing and auscultation show ed normal breath sounds with no adventitious sounds.The laboratory data, including urine analysis, sweat chloride, blood glucose, calcium and phosphate function of blood, renal and liver function tests, and thyroid hormone and uric acid blood levels were within normal range. Results of skin tests for tuberculosis, and studies for acid-fast bacilli, routine pathogens, and malignant cells were negative. Pulmonary function tests showed a mild restrictive respiratory defeat with low diffusion capacity. Chest X-ray film revealed extensive uniform, bilateral sand-like miliary densities of both lungs (sandstorm lung) obscuring the cardiac outlines, most marked in both hilar regions and fading out toward toward the apices and peripheral portions of the lungs.The minor fissure was thickened. High resolution CT revealed diffuse ground glass opacities micronodular densities.Interlobular septal thickening, more prominent in the anterior parts of both lungs, and linear calcifications along the pleuraThe case was authenticated by lung biopsy after video-assisted thoracoscopic surgery (VATS). The cut surface was hard, and demonstrated numerous sand-like concretions, making cutting difficult (Figure 3 ). Microliths were formed in the alveolar space. The range of from 0.01 to 3 mm in size and they were usually irregular or bosselated, and there was interstitial fibrosis (Figure 4). He is asymptomatic after one year of follow-up.
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