Study Of Clinical Diagnosis And Therapy Of Pulmonary Alveolar Proteinosis

[Purposel Pulmonary alveolar proteinosis (PAP) is a rare disease of unknownetiology charcterized pathologically by filling of alveolar sPaces with granlapperiodic acid-Schiff (PAS)-positive material consisting mostly of phospholiPids andproteins. PAP is increasingly being recogIilzed in clinical lecfores wiAnn recot years.The onset of PAP is usually insidious and the presenting syTnPtOms are nonSpecific,often causing a significant delay in the diagnosis or ndsdiagnosis. To imProve thediagnostic rate, the whole lung lavage efficiency and the condition of PAP patients.[Materials and Metkods] @PAS-AB stained smears of BALF obtained frOm l5patiettts with PAP This study was conducted to evaluate the usefulness of PAS-ABstained smears of BALF in aiding a diagnosis of PAP To serve as contfo1s, the smearsof 21 patients with other pulmonary disorders were also examined. The findings ontlie presence and nuxnber of globules were recorded. To differentiate PAP from otherpulmonary disorders, the highest globule value obtained from contTol grouP waschosen as the cutoff point@l5 patients were identified, (age range, 28 to 64 year-old)with histopathologic and bronchial alveolar lavage (BAL)confirmation of pu1monaryalveolar proteinosis (PAP). To analysis the CT and chest radiograPhy of PAP patientS@one patient was trCated with the usual whole lung lavage but seven PAP patientswith the imProved whole lung lavege. The imProved whole lung lavage is the wholelung lavage intervened by the venti1ation.@ The history of 53 PAP cases reported inthe Iectas and 15 patients identified by us are analyzed retrospectively on the basesof the comPlains, symPtoms, lung film, theoretic CT pulmonary driction and bloodanalysis.[ Results ] @ The globules stained green, orange, or orange in the cener coated withgreen rim seen in PAS-AB stained smears ofbronchoalveolar lavage fluid (BALF) aresuggested to be characteristic of pulmonary alveolar proteinosis (PAP). The sensihvityof the globules stained to PAP is 78.9% and specify is 81%(X' test, P<0.0l). After aCT scan, TBLB was conducted under the X-ray, veracity of diagnosis is close tOl00%. @ It was concluded that CT and X-ray film images are like the maP, the"craZy9aving" pattern, lobar or segTnental desity shadow diffese infiltrating and"batWing" shadow sometimes simulating the pattr of acute pulInonary edema. ThetyPical features are stabilization of the shadow (no pneumonia) and that the shadow ismore serious than the symPtOms. Abnormity of CT and X-ray film images is theearliest clinic finding in PAP patients, and Provided with diagnostic value. They arethe imPohant indications which assess cUrative effect and estimate the prognosis ofthe PAP patient.@Treatment with whole lung lavage resulted in imProvements indyspnoea, lung function, and peak cycle ergometry pefformance.@ The delay ofdiagnosis haPpened in all l5 patientS. The time of short delay was 4 month. The timeof long delay was 26 month. The mean time was l4.5f6 month. PAP wasmisdiagnosed as pneumonia(55/68) at the eariy stage and at the later stage asIPF(49/68) or pulmonary carcinoma.(Conclusion] ?PAS-AB stained smears of bronchoalveolar lavage fluid (BALF) are to be characteristic of pulmonary alveolar proteinosis (PAP). After a CT scan, TBLB was conducted under the X-ray and the veracity of diagnosis is close to 100%. (D CT and X-ray film images of PAP are like the map, the "crazy-paving" pattern, lobar or segmental density shadow, diffuse infiltrating and "batwing" shadow sometimes simulating the pattern of acute pulmonary edema. Abnormity of CT and X-ray film images is the earlist clinic finding in PAP patients, and provided with diagnostic value. They are the important indications which assess curative effect and estimate the prognosis of the PAP patient. ㏕reatment with whole lung lavage resulted in improvements in dyspnoea, lung function, and peak cycle ergometry performance.?The delay of diagnosis happened in all 1...