Humans ; Pulmonary Alveolar Proteinosis/pathology*

Adult ; Humans ; Male ; Myelodysplastic Syndromes ; complications ; pathology ; Pulmonary Alveolar Proteinosis ; etiology ; pathology

OBJECTIVE: To evaluate the diagnostic value and limits of transbronchial lung biopsy (TBLB) in pulmonary alveolar proteinosis (PAP). METHODS: The complete hospital data from Second Xiangya Hospital, Central South University, between June, 2006 and December, 2012, were analyzed retrospectively in 25 patients with PAP (who were diagnosed pathologically by TBLB or not by TBLB) and in 4 patients with other disease (who were misdiagnosed by TBLB). RESULTS: Among the 25 patients with PAP, 14 patients were confirmed by TBLB in the fi rst time [TBLB positive rate in the first time was 56% (14/25)]; 6 patients who were misdiagnosed by TBLB in the fi rst time were confirmed by TBLB in the second time [the positive rate in the second time was 24% (6/25)]. Th e total positive rate was 80% (20/25). Th e total negative rate was 20% (5/25). Five patients with PAP, who showed negative results in TBLB analysis, were confirmed by the typical CT as well as the whole lung lavage. In addition, 4 patients with other diseases were misdiagnosed as PAP by TBLB. CONCLUSION TBLB is a very good diagnosis method. But the negative results in the first time cannot exclude PAP. TBLB should be repeated if conditions allow. If clinical manifestation and CT results are typical, it can be used for diagnosis of PAP combined with the positive results from bronchoalveolar lavage fluid check. However, for non-typical cases, it needs TBLB pathological diagnosis.
Biopsy ; Diagnostic Errors ; Humans ; Lung ; pathology ; Pulmonary Alveolar Proteinosis ; diagnosis ; pathology ; Retrospective Studies

OBJECTIVE: To explore CT findings and pathologic basis of crazy paving pattern caused by pulmonary alveolar proteinosis. METHODS: Twenty-four patients who were diagnosed pathologically as pulmonary alveolar proteinosis by transbronchial lung biopsy and bronchoalveolar lavage fluid from June 2006 to May 2012 were included in this retrospective study. All patients underwent a 64-slice CT of the lungs. RESULTS: CT findings: crazy paving pattern was observed on CT imaging of all 24 patients. In 23 patients, crazy paving pattern displayed strip-shaped opacities with smooth edges, and there was a clear boundary between the pathological and normal lung tissues. The reticular opacities were connected with peripheral blood vessels and the branches were formed, and their diameters decreased slightly. Microscopically, hemangiectasis were seen in 17 patients. CONCLUSION Crazy paving pattern caused by pulmonary alveolar proteinosis displayed clear edges, and smooth reticular opacities, most of which were due to hemangiectasis of interlobular, interacinar and interalveolar septa. These findings of CT are helpful for the specific diagnosis of pulmonary alveolar proteinosis.
Biopsy ; Humans ; Lung ; pathology ; Pulmonary Alveolar Proteinosis ; pathology ; Retrospective Studies ; Tomography, X-Ray Computed

OBJECTIVETo study high resolution computed tomograghy (HRCT) features and changes of HRCT after whole lung lavage therapy in patients with pulmonary alveolar proteinosis (PAP).

METHODWe retrospectively reviewed the HRCT data of 23 times of lung lavage therapy in 16 PAP patients.

RESULTSHRCT scan showed bilateral clearly-defined patchy areas of ground-glass opacity and interlobular septal thickening, a pattern commonly characterized as "crazy paving". After whole lung lavage therapy, HRCT showed that the diffused ground-glass opacity palliated and the extent of lung opacity shrinked, while the reticular opacity and area of confluence persisted.

CONCLUSIONThe HRCT feature of PAP is relatively characteristic, and may be improved after whole lung lavage therapy.

Bronchoalveolar Lavage ; methods ; Female ; Humans ; Male ; Middle Aged ; Pulmonary Alveolar Proteinosis ; pathology ; therapy ; Retrospective Studies ; Tomography, X-Ray Computed ; methods

OBJECTIVE: To explore characteristic CT findings and pathologic basis of ground glass opacity caused by pulmonary alveolar proteinosis (PAP). METHODS: Retrospective analysis of CT and pathological findings of 24 patients with PAP who were pathologically diagnosed from June 2006 to August 2011. RESULTS: Findings with CT: the lesions of the 24 patients mainly presented ground glass opacities. Local consolidations were seen in 8 patients. In 23 patients part of ground glass opacities bordered strip-shaped opacities with smooth edges, and there was a clear boundary between them and the bordering normal lung tissues, presenting a geographic appearance. Lesions in the 5 cases were mixed with alveoli or lobule aerocele, which made ground glass opacities present curved edges. Crazy paving pattern was detected in the 24 patients. Microscopically, the alveoli were seen to be filled with floccules proteinaceous material in various quantities in the 24 patients; hemangiectasis and congestion were seen in 17 patients, and enlarged alveolar cavities were seen in 5 patients. CONCLUSION PAP usually causes ground glass opacities with clear edges, and different from ground glass opacities with obscure edges caused by other pulmonary diseases. They are relatively specific to the imagining diagnosis to PAP.
Adult ; Aged ; Female ; Humans ; Male ; Middle Aged ; Pulmonary Alveolar Proteinosis ; diagnostic imaging ; pathology ; Retrospective Studies ; Tomography, X-Ray Computed

The male patient was referred to the hospital at 44 days old due to dyspnea after birth and inability to wean off oxygen. His brother died three days after birth due to respiratory failure. The main symptoms observed were respiratory failure, dyspnea, and hypoxemia. A chest CT scan revealed characteristic reduced opacity in both lungs with a "crazy-paving" appearance. The bronchoalveolar lavage fluid (BALF) showed periodic acid-Schiff positive proteinaceous deposits. Genetic testing indicated a compound heterozygous mutation in the ABCA3 gene. The diagnosis for the infant was congenital pulmonary alveolar proteinosis (PAP). Congenital PAP is a significant cause of challenging-to-treat respiratory failure in full-term infants. Therefore, congenital PAP should be considered in infants experiencing persistently difficult-to-treat dyspnea shortly after birth. Early utilization of chest CT scans, BALF pathological examination, and genetic testing may aid in early diagnosis.
Infant ; Infant, Newborn ; Humans ; Male ; Bronchoalveolar Lavage/adverse effects* ; Pulmonary Alveolar Proteinosis/pathology* ; Dyspnea/etiology* ; Respiratory Insufficiency

OBJECTIVETo describe the pathologic features and diagnostic algorithm of pulmonary alveolar proteinosis (PAP).

METHODSThirty-nine biopsy and postmortem cases of PAP were studied by light microscopy and histochemical staining using periodic acid-Schiff (with digestion) (PAS-D), mucicarmine (with digestion) (mucicarmine-D) and alcian blue.

RESULTSHistologically, the affected lung tissue displayed the following characteristic features: (1) alveoli and some of the small bronchioles were filled with eosinophilic and fine granular proteinaceous material with needle-like clefts; (2) proteinaceous material was seen admixed with various numbers of degenerated and sometimes exfoliated pneumocytes; (3) pneumocytes were hyperplastic; (4) alveolar capillaries and alveolar septa had become hyperemic, but pulmonary interstitial inflammation was not obvious; (5) no significant inflammation was identified in the bronchial wall; (6) compensating emphysema was noted in the surrounding lung parenchyma. Fragments of eosinophilic, finely granular proteinaceous material with needle-like clefts were also found in the bronchoalveolar lavage fluid under light microscopy. The proteinaceous material was stained red by PAS-D. The staining for mucicarmine-D was negative, while alcian blue staining was either weakly positive (faint blue staining) or negative. Pathologic examination of lung biopsies and bronchoalveolar lavage fluid thus remaines the gold standard for diagnosis of PAP.

CONCLUSIONSIdentification of homogeneous, eosinophilic, finely granular and PAS-D-positive proteinaceous material with needle-like clefts in alveolar spaces or bronchoalveolar lavage fluid is of diagnostic importance in PAP. Bronchoalveolar lavage, being a relatively safe and non-invasive procedure, can be a useful adjunct in arriving at the final conclusion.

Adult ; Bronchoalveolar Lavage ; Bronchoalveolar Lavage Fluid ; cytology ; Female ; Humans ; Lung ; pathology ; Male ; Middle Aged ; Periodic Acid-Schiff Reaction ; Pulmonary Alveolar Proteinosis ; pathology ; therapy ; Pulmonary Alveoli ; pathology

OBJECTIVETo discuss the clinical features of Indium-related lung diseases.

METHODSWe searched database of Chinese and Pubmed, Embase, Web of Science to collect research data of indium-related lung diseases from Jan. 1998 to Aprl. 2014. Case reports, exposure histories and lab results were analysed and summarized.

RESULTS1998 to Mar 2010, ten cases of indium-related lung diseases were published. Seven cases of interstitial pneumonia were reported in Japan, two cases of pulmonary alveolar proteinosis (PAP) were reported in the USA and one case of PAP reported in China. Chest computer tomography (CT) showed diffuse or local ground glass appearance (GGA) in 8 cases, 3 of which also showed centrilobular nodules; Pulmonary function test were normal only in one out of 8 cases. Cholesterol clefts were found in 4 cases of interstitial pneumonia. 3 cases died among 6 cases who were followed-up.

CONCLUSIONSOccupational exposure to indium compounds are contributory to different pulmonary diseases, which are composed of interstitial pneumonia and pulmonary alveolar proteinosis. The relationships between In-C, In-S and these pulmonary diseases are unclear.

China ; Glass ; Humans ; Indium ; toxicity ; Japan ; Lung Diseases, Interstitial ; etiology ; pathology ; Occupational Exposure ; adverse effects ; Pulmonary Alveolar Proteinosis ; etiology ; pathology ; Respiratory Function Tests ; Tomography, X-Ray Computed

Idiopathic pulmonary alveolar proteinosis (PAP) is a rare disorder in which lipoproteinaceous material accumulates within alveoli. There were few reports on Asian populations with idiopathic PAP. We retrospectively reviewed 38 patients with idiopathic PAP in Korea. We assessed clinical features, therapeutic efficacy and outcomes of whole lung lavage in patients with idiopathic PAP. The mean age at diagnosis was 52 yr. Eighty six percent of patients were symptomatic at diagnosis. Dyspnea and cough were the most common symptoms. Crackles were the most common physical examination finding. On pulmonary function test, a mild restrictive ventilatory defect was common, with a predicted mean forced vital capacity (FVC) of 77% and forced expiratory volume in one second (FEV1) of 84.6%. Diffusing capacity was disproportionately reduced at 67.7%. Arterial blood gas analysis revealed hypoxemia with a decreased PaO2 of 69.0 mmHg and an increased D(A-a)O2 of 34.2 mmHg. After whole lung lavage, PaO2, D(A-a)O2 and DLCO were significantly improved, but FVC and total lung capacity (TLC) were not different. This is the first multicenter study to analyze 38 Korean patients with idiopathic PAP. The clinical features and pulmonary parameters of Korean patients with idiopathic PAP are consistent with reports in other published studies. Whole lung lavage appears to be the most effective form of treatment.
Adult ; Biopsy ; Bronchoalveolar Lavage ; Female ; Humans ; Korea ; Male ; Middle Aged ; Pulmonary Alveolar Proteinosis/diagnosis/pathology/*physiopathology/*therapy ; Respiratory Function Tests ; Retrospective Studies ; Survival Rate ; Treatment Outcome