The Clinical Significance Of Lipid Levels In Pulmonary Alveolar Proteinosis Disease

Objective: Pulmonary alveolar proteinosis is a kind of diffuse parenchymal lung,characterized by the accumulation of lipoproteinaceous material in the alveolar andrespiratory bronchioles, which damages gas exchange. The most common spirometricabnormality is restrictive defect, severe cases died of respiratory failure or severeinfection. The onset of clinical disease is insidious, progressive shortness of aerosols asthe main performance. Although the lung is capable of de novo synthesis of cholesterol,the bulk of pulmonary cholesterol is provided by serum lipoproteins. By alveolar lavagefluid and cytological examination, it had confirmed that the disease was caused by theabnormal accumulation of lipids and proteins in alveolar cavity, belonging to metabolicdisease. But it is unclear that whether there is correlation between the PAP with theserum lipid. In this article, through analyzing the7cases of pulmonary alveolarproteinosis disease and combing with related literature review，we summarized theclinical data of the disease, in order to improve clinical knowledge.Methods: The clinical datum that Blood lipids and arterial blood gas of pulmonaryalveolar proteinosis disease were retrospectively analyzed in the first affiliated hospitalof Dalian medical university from2000to2012,and combining the related literatureswhich consisted of pulmonary alveolar proteinosis and serum lipid in Pub Med search.Results: It was indicated that the median age at diagnosis was56.85±8.51,6of7patients were men, and72%had a history of smoking. Cough in5cases (71.4%),expectoration in3patients (42.9%), progressive dyspnea in7cases (100%); the purplein2cases (28.6%); the clubbing in2cases (28.6%), lung wet rale in5cases (71.4%).All7cases existed lipid metabolism disorder, hyper-triglyceridemia in2cases(160.86±66.22mg/dl),5cases were hyper-cholesterolemia（229.14±56.46mg/dl）,hyper-low-density-lipoproteine-cholesterol (hyper-LDL-C) in5cases(143.43±39.11mg/dl), hyper-lipoprotein (a) in3cases(253.5±121.5mg/l),2cases of patientsconfirmed by abdominal ultrasonography had fatty liver disease. Each variable was normal distribution, adopting the Pearman related: The values of lipoprotein (a)correlated with PaO2levels and PA-aDO2levels (r＝-0.792，p＝0.034＜0.05，and r＝0.583，p＝0.169). Serum triglyceride, cholesterol, low-density lipoprotein cholesterol(LDL-C), high-density lipoprotein cholesterol (HDL-C) had no correlation betweenarterial blood gas analysis.Conclusions: Lipoprotein (a) negatively correlated to the degree of the disease.The serum lipid levels, lipoprotein (a) may reflect the severity of the disease in PAP.