To describe the clinical,radiological and pathological characteristics of idiopathic pulmonary alveolar proteinosis(I-PAP)and to evaluate the methods of diagnosis and treatment.Three patients were successfully diagnosed and treated in our hospital and the literature on the subject was reviewed.Three patients,two males and one female(mean age 46 years),were diagnosed averagely in 4 months.Two severe patients presented with progressive dyspnea and type I respiratory failure,and one mild patient only with dry cough and hypoxemia.Chest X-ray radiographs all showed perihilar "butterfly" shadow and chest CT scans showed diffused ground-glass opacities(GGO),typically with "map" changes and "crazy paving" patterns.All the patients underwent bronchoscope,branchoalveolar lavage fluid(BALF)had grossly opaque and/or milky appearance and its sediment was periodic acid-Schiff stain positive.Transbronchoscopic lung biopsy(TBLB)specimens were obtained and under light microscopy alveoli and some of the small bronchioles were filled with eosinophilic proteinaceous material with needle-like clefts.By electron microscopy numerous cellular debris and extracellular multilamellated bodies were found.Two severe patients were successfully treated with sequential whole-lung lavage and one required repeated lavages.I-PAP is rare and prone to be misdiagnosed.The radiological features may indicate the diagnosis and examinations of TBLB and BALF can make the accurate diagnosis.Whole-lung lavage is the most effective therapy by now and granulocyte-macrophage colony-stimulating factor(GM-CSF)may be beneficial in some patients.

To explore the clinical manifestation of lymphomatoid granulomatosis (LG) and the early diagnosis. Retrospective analyses of the clinical features of LG and pathology features by open lung bio-psies were conducted. The patient was a 42-year-old female with irregular fever,and her chest X-ray and computed tomography showed nodules with cavity and pleural effusion. Open lung biopsy proved LG. LG is seldom seen in clinic. Open lung biopsy is very important for pathology diagnosis. Early diagnosis and treatment are the key to improving survival in these patients. The therapeutic effect is good.

SUMMARY Marginal zone B-cell lymphoma of the pulmonary mucosa-associated lymphoid tissue type (pulmonary MALT-MZL), a common kind of primary pulmonary lymphoma, is rare in pulmonary malignant tumors. One patient in our hospital was diagnosed by bronchoscope and the literatures on the subject were reviewed. The patient presented with periodical fever, cough and chest pain, and antibiotic therapy had no use. Chest CT scan showed the consolidation of right middle lobe and left lower lobe with CT angiogram signs, air bronchograms and distended bronchi. Pleural effusion in the left thorax mainly consisted of monocytes. Monoclonal protein was found in the electrophoresis of serum protein. Bronchial stenosis and swollen mucosa were seen with bronchoscope. The tissue section of transbronchial lung biopsy (TBLB) specimens showed diffusedly infiltrated small lymphocytes and a lymphoepithelial lesion. CD20 was positive and CD3, CD5, CD10, CD21, CD23, bcl2, bcl6 were negative in immunohistochemical stain. The clinical manifestations of pulmonary MALT-MZL are nonspecific and misdiagnosis is common, Appropriate invasive biopsy procedures are necessary for early diagnosis. Presentations such as periodical fever, distended bronchi in pulmonary consolidation, monoclonal protein might indicate diagnosis. Treatment includes surgical resection, radiotherapy and chemotherapy. Pulmonary MALT-MZL belongs to inert lymphoma and prognosis is relatively good.

Objective To improve the diagnosis and treatment of pulmonary cryptococcosis.Methods The clinical data of 3 cases of pulmonary cryptococcosis were analyzed and reviewed.Results The cases were tested by percutaneous lung biopsy and were confirmed by histopathologic examination.The sputum cultures were negative and serum latex cryptococcal antigen agglutination tests were positive.Two of them had mild to moderate symptoms and were treated by fluconazol;the other with meningitis had severe symptoms and was treated by amphotericin B.All of them were clinically cured.Conclusion Percutaneous lung biopsy combined with latex cryptococcal antigen agglutination tests is helpful for diagnosis of pulmonary cryptococcosis.Patients with mild to moderate symptoms should be firstly treated by fluconazol and those with severe symptoms or meningitis,by amphotericin B.

To investigate the clinical characteristics, diagnosis and therapy of influenza pneumonia with staphylococcal infection. One patient in our hospital was diagnosed and the literatures on the subject were reviewed. The patient presented with high fever and dyspnea. Arterial gas analysis indicated type 1 respiratory failure. Chest X ray photographs showed bilateral infiltrations and bilatera encapsulated pleural effusions. Viral separation and culture of pharyngeal swab indicated H3N2 subtype of human influenza virus. Blood, sputum and bronchoalveolar lavage fluid (BALF) cultures showed Staphylococcus aureus. Pleural effusion was complex parapneumonic pleural effusion. After the administration of anti-virus, anti-staphylococcal antibiotics and pleural cavity drainage, the patient was cured. The infection of staphylococcus aureus is a typical characteristic of influenza pneumonia, and anti-staphylococcal antibiotic therapy (with MRSA activity in MRSA endemic regions) should be initiated in hospitalized cases of influenza pneumonia. If complex parapneumonic pleural effusion or empyema complicated, we should perform pleural cavity drainage in time. The oral neuraminidase inhibitor (oseltamivir) could significantly improve prognosis.

Objective:Organizing pneumonia (OP) is a clinical syndrome manifested by granulation tissue plug in respiratory bronchioles, alveolar ducts and alveolar spaces pathologically.With advent of lung biopsy techniques, increasing pathological diagnosis of organizing pneumonia was made, most of which were confirmed secondary organizing pneumonia (SOP) either at time of diagnosis or on follow-up.The secondary etiology is related to the choice of treatment and prognosis.First of all, we need to rule out infection, find evidence of connective tissue disease, or previous medication, radiotherapy and even transplantation.The coexistence of multiple pathological features and simultaneous involvement of pulmonary vessels and pleura suggest possible secondary causes.The pathological diagnosis of organic pneumonia by empty core needle puncture or transbronchial lung biopsy needs to be combined with imaging to avoid omitting the tumor, granuloma and necrosis around organic pneumonia.In general, organizing pneumonia, no matter cryptogenic or secondary, is mostly responsive to corticosteroids compared with other interstitial lung diseases, although relapses are frequent.In summary, real cryptogenic organized pneumonia is actually relatively uncommon and should be diagnosed with caution.Long term follow up is not only crucial to assess the evolution of lung disease but also for looking into the underlying etiologies behind an organizing pneumonia.

Scoliosis is a common deformity in neuromuscular disease, which usually has the characteristics of early onset age, severe degree of deformity, and rapid progression.Neuromuscular scoliosis often leads to serious damages to the quality of life, and results in the loss of walking, standing and sitting, and cardiopulmonary insufficiency.Surgical treatment can improve the quality of life for children with neuromuscular scoliosis, but surgical treatment is still challenging due to the complex surgery and many complicated diseases.The complications are much higher than those of idiopathic scoliosis.A multidisciplinary team is necessary in the surgical treatment of neuromuscular scoliosis to promptly and effectively reduce the complications.