Objective To investigate the clinical features with to highlight the main criteria for diagnosis of portopulmonary hypertension(POPH).Methods The clinical and laboratory data of 7 patients with POPH were retrospectively analysed.Results The patients included 2 males and 5 females with median age at diagnosis of 51 years(ranging from 26 to 73 years).The underlying diseases of portal hypertension were post-type B hepatitis cirrhosis(3 cases),autoimmune hepatitis(2 cases),immunologic deficiency disease(1 case)and portal vein thrombosis(1 case).The initial symptom of pulmonary hypertension(PH)in 5 patients was dyspnea on exertion,but 2 patients remain asymptomatic.All patients was assessed by transthoracic echocardiogram(TTE)with the pulmonary arterial systolic pressure(PASP)above 55 mmHg,1 patient accepted right heart catheterization(RHC)with mean pulmonary arterial pressure of 62 mmHg and pulmonary vascular resistance of 8.64 mN/(s?cm5).Immunologic abnormality was found in 6 patients,and the severity of liver function does not appear to correlate with the severity of the PH.Follow-up study demonstrated 3 patients died,2 of whom were due to the cardiac involvement(right heart failure and sudden death).Conclusion PH is a rare but severe complication of portal hypertension.It is the portal hypertension,not the underlying liver disease causing POPH.TTE is an important screening procedure,but the gold standard for diagnosis of POPH is hemodynamic data from RHC.

Objective Pulmonary benign metastasizing leiomyoma (PBML) is a rare entity that leiomyoma of uterus metastasized to the lung.The clinical characteristics of this rare disease were analyzed in this article.Methods The detailed clinical records of 7 patients diagnosed as PBML at Peking Union Medical College Hospital between January 2001 and June 2015 were reviewed.Results All patients were women with median age of 44 years (range 28-62).Symptoms included dyspnea (2/7),chest pain (1/7),cyanosis (1/7),cough (1/7) and bloody sputum (1/7),while 4/7 cases were asymptomatic.Six patients had the past-history of leiomyoma of uterus 20 months to 14 years ago among whom 5 patients received hysterectomy.Chest CT showed bilateral,random-distributed multiple round solid nodules,or diffuse-distributed miliary nodules,or single solid nodule,even some small cavities.Extra-pulmonary metastasis was found in left superclavicular lymph node (1 case) and right heart (1 case).Histological tissues were obtained by video-assisted thoracic surgery lung biopsy (4/7),mass resection on tricuspid valve (1/7),transbronchil lung biopsy (1/7),and CT-guided percutaneous lung biopsy (1/7).Pathology showed an interlacing pattern by spindle cells having elongated nuclei without cellular atypia.Ki-67 index was less than 1％.Molecules such as smooth muscle antibody,estrogen receptor (ER) and progestrone receptor (PR) were positive in immunohistochemistry staining.Neither letrozole nor zoladex was effective.Two patients responded to bilateral adnexectomy,presenting as shrunk nodules.No relapsed disease was seen in one patient with single nodule after resection.There was only one patient with disease-related mortality,whose chest CT showed milliary nodules.Conclusion Although CT findings of PBML are similar to malignancies,the clinical outcome is good.Despite the positive expression of ER and PR,the effectiveness of hormone related treatment is limited.And periodical follow up is suggested even to those uneventful patients.

Objective To analyze the clinical characteristics of large pulmonary vessel involvement in Behcet's diseaseMethods The clinical data of Behcet's disease patients with large pulmonary vessel involvement,who were treated in Peking Union Medical College Hospital from 1983 to 2008,were retrospectively analyzed.Results Thirteen patients had large pulmonary vessel involvement among a total of 418 patients with Behcet's disease.The 13 patients had a mean age of (37.5?12)years old,with 10(69.2%)having hemoptysis,8(61.5%)having dyspnea.Helical CT/CTPA showed that pulmonary vascular involvement included pulmonary arterial aneurysms(53.8%),pulmonary artery occlusion(46.2%),and pulmonary thrombi(46.2%).All the 13 patients were treated with steroid and immunosuppressant;five received steroid pulse therapy;and four received vascular intervention therapy.The mean follow-up was (3?1.6)years,with a follow-up rate of 92.5%.The 3-year survival rate was 83.9% for the 13 patients.Conclusion The manifestation of pulmonary vascular involvement in Behcet's disease is various,and the diagnosis mainly depends on the manifestations of CT pulmonary angiography.

Objective This study was designed to investigate the clinical characteristics of systemic vasculitis with cardiac involvement.Methods Clinical information of 10 patients with systemic vasculitis complicated by myocardial vasculitis,selected from 181 small vessel vasculitis patients and 114 systemic vasculitis patients were analyzed.Results The clinical manifestations were varied significantly dependent on the etiology of small vessel vasculitis.It is usually djfficult to make the diagnosis because of the insidious onset,varied etiology and the undifferentiated manifestations of heart involvement.Echocardiography is commonly used in detecting and monitoring cardiac involvement.Glueocortieoid therapy can improve left ventricular systolic function dramatically when used properly.Conclusions The cardiac involvement of systemic vasculitis is quite rare.Dyspnea of various degrees and left ventricular systolic dysfunction ale the most common clinical findings.The earlier the establishment of diagnosis and institution of appopriate treatment,the better the prognosis.

Objective Type 2 diabetes increases the risk of colorectal cancer and reduces the survival rate of the patients. Therefore, analyzing the characteristics of colorectal cancer in patients with type 2 diabetes may help to develop precise medical measures and to improve the prognosis of patients. Methods The pathological characteristics of 123 colorectal cancer cases with or without type 2 diabetes were analyzed. In addition, PCR and pyrosequencing method were used to test the common gene mutation status of colorectal cancer, including KRAS( codons 12, 13, 61, and 146) , BRAF( codon 600) , and PIK3CA ( exons 9 and 20) , in order to investigate the molecular characteristics of colorectal cancer in diabetic patients which may provide some clues to clinical decision. Results The mean age and body mass index of patients with diabetes were higher than those of patients without diabetes, but there was no difference in the location, the degree of cell differentiation and the grade of colorectal cancer between these 2 groups. The rate of PIK3CA mutation in patients with type 2 diabetes mellitus, especially in patients with long term type 2 diabetes was significantly higher than that in patients without diabetes (28.6％vs 10.3％, P<0.05). There was no significant difference between the two groups in common BRAF and KRAS mutation sites. Conclusion Diabetes does not alter the clinical pathological characteristics of colorectal cancer, but long course type 2 diabetes increases PIK3CA mutation rate. Therefore, using medicine targeting PIK3CA gene mutation may help to improve the prognosis of patients.

Objective To evaluate the role of transbrochial needle aspiration (TBNA) in the diagnosis of patients with enlarged mediastinal and/or hilar lymph node. Methods Patients with mediastinal and/or hilar lymphoadenopathy proven by CT scan were eligible for TBNA as reported. All specimens were directly and instantly smeared for pathological examination. Results From June 1 2004 to December 31 2007, 164 patients were examined: including 80 lung cancers, 69 lung bengin diseases, 2 other malignancy tumor, and 13 without definite diagnosis. Total 260 lymph nodes were punctured. TBNA procedures were successfully carried out in 445/463(96.1%). Sensitivity of TBNA was 82. 5 % (66/80) in patients who had been proven to suffer from bronchogenic carcinoma. There were 25 patients that diagnosis of lung cancer was pathologically determined by TBNA only. A total of 122 lymph nodes in the 80 lung cancer patients were aspirated by TBNA with a positive rate of 65. 6% (80/122). Severe complications were rare except small amount of bleeding at the TBNA site (100/164, 61.0%). From June 1 2006 to December 31 2007, lymph node tissues able to make histology diagnosis were yield in 73.5% (64/87) patients. Through histology pathology, the sensitivities of TBNA were 53. 3% (8/15) for sarcoidosis and 78.6% (33/42) for lung cancer. Conclusion TBNA is quite safe and helpful in diagnosis and staging of bronchogenic carcinoma, and in diagnosis of benign lung diseases.

Objective:To analyze the differences in clinical characteristics, microbiologic distributions, in vitro susceptibility, treatment and outcomes between elderly and young/middle-aged patients with nocardiosis.Methods:A total of 27 patients diagnosed with nocardiosis by bacteria culture and gene sequencing in our hospital from May 2007 to September 2011 were included.Patients were separated into two groups according to their ages.Clinical characteristics, laboratory tests, treatment and prognosis were compared between the two groups.Results:Of the 27 patients, 11 were male and 16 were female, with a median age of 50 years(range, 20-84 years old)and a median course of 3.0 months(range, 0.5-60.0 months). There were 6 cases in the elderly group and 21 cases in the non-elderly group.The common underlying diseases were chronic pulmonary disease(4/6, 66.7%), metabolic disease(2/6, 33.3%)and connective tissue disease(2/6, 33.3%)in the elderly group, and connective tissue disease(9/21, 42.9%), chronic pulmonary disease(6/21, 28.6%)and hematological disease(2/21, 9.5%)in the non-elderly group.Results of laboratory tests revealed that white blood cell counts were normal or elevated, lymphocyte percentages decreased and neutrophils percentages were elevated in both two groups.All patients in the elderly group had pulmonary infection, while 71.4%(15/21)had pulmonary infection and 28.6%(6/21)had disseminated infection in the non-elderly group.The proportion of patients with pulmonary consolidation on CT was higher in the elderly patients than in the non-elderly patients(100% vs.42.9%, P=0.02). The detection rates of N. cyriacigeorgica, N. farcinica, N. abscessus, N. Africana and N. wallacei were 50.0%(3/6), 16.7%(1/6), 16.7%(1/6), 16.7%(1/6)and 0%(0/6)in the elderly group and 23.8%(5/21), 42.9%(9/21), 42.9%(9/21), 4.8%(1/21)and 14.3%(3/21)in the non-elderly group, respectively.The mortality rate was 16.7% in the elderly patients and 28.6% in the non-elderly patients.The 5-year survival rates in both two groups were 100%. Conclusions:Elderly patients with nocardiosis often have underlying pulmonary disease with a long disease course. N. farcinica is the main observed strain.Multiple nodules and plaques and consolidation are common on CT.Multi-drug combinations are recommended in treatment.Concomitant fungal or other bacterial infections are factors for poor prognosis.The long-term prognosis is good in patients with nocardiosis.

Objective: Pulmonary metastatic angiosarcoma is a rare, fatal disease that often presents as diffuse alveolar hemorrhage(DAH). In this report, clinical characteristics of pulmonary metastatic angiosarcoma were retrospectively reviewed. Methods: A total of 9 patients with angiosarcoma who presented as DAH were enrolled. Clinical data included age, gender, symptoms, smoking status, physical exam findings, pulmonary function tests, and radiology. Results: All patients were male with median age 41 years(range, 22 to 57 years). The most common symptom was hemoptysis(9/9). Other symptoms included dyspnea (5/9), cough(3/9), chest pain(3/9), fever(2/9,) and edema of the lower extremity and oliguria(4/9).The common misdiagnoses were tuberculosis(4/9), vasculitis(3/9) and other infection(1/9). Chest computed tomography showed bilateral,random distributed different-sized nodules(9/9),as well as ground-glass areas (9/9).The hearts, mainly right atrium, were the most common primary locations(7/9).Cardiac mass was the first manifestation in five patients by echocardiography(5/8).Two atrial masses were identified by computer tomography pulmonary angiography and magnetic resonance imaging respectively. Transbronchial lung biopsy failed to find malignancy. Computer tomography guided transthoracic needle biopsy was difficult to perform in most patients. Eight patients were diagnosed by surgical biopsy, either by lung biopsy(4/8) or cardiac biopsy(4/8).The median survival period was only 3 months after surgery. Conclusion Metastatic pulmonary angiosarcoma should be considered in patients with DAH and multiple glass ground opacity and nodules on chest CT. Careful cardiologic monitoring is necessary. Surgical biopsy is reliable for diagnosis.

Objective To analyze the clinical characteristics of IgG4-related disease (IgG4-RD)so as to improve the understanding of IgG4-RD in China.Methods IgG4-RD patients were recruited from Peking Union Medical College Hospital between January 2011 and January 2016.All patients were followedup for more than 6 months.The demographic characteristics,symptoms,organ involvements,laboratory examinations and treatment efficacy were evaluated and analyzed.Results A total of 346 patients were finally enrolled,including 230 males (66.5％) and 116 females (33.5％).The mean age of disease onset was (53.8 ± 14.2) years old.The mostly common involved organs were lymph nodes (56.4％) and submandibular glands (52.6％).Other affected organs and manifestations included:swelling of the lacrimal glands (46.5％),autoimmune pancreatitis (38.4％),pulmonary involvement (28.0％),sclerosing cholangitis (25.4％),naso-sinusitis (23.4％),parotid gland swelling (21.7％),retroperitoneal fibrosis (19.9％),large arteries involvement (9.5％),kidney involvement (obstructive nephropathy caused by retroperitoneal fibrosis was excluded) (6.9％),skin lesions (6.4％).Rare features consisted of thyroid glands,pituitary glands,gastrointestinal tract,pachymeningitis,pericardium,sclerosing mediastinitis and orchitis.The majority of patients had multi-organ involvement,such as 74.3％ patients with 3 and more,18.2％ and 7.5％ patients with 2 and single organ involvement respectively.The average IgG4-RD responder index (IgG4-RD RI) was 13.21 ±5.70.History of allergy was found in 172 (49.7％) patients.As to the laboratory tests,elevated serum IgG4 levels were confirmed in 285 (94.1％) patients,which was positively correlated with IgG4-RD RI.There were 33.5％ patients receiving monotherapy of glucocorticoid,52.6％ treated with glucocorticoids combined with immunosuppressive agents,4.9％ patients with immunosuppressant only,and 9.0％ patients with mild disease not receiving medication.The majority (336,97.1％) patients improved the above regimens.Conclusion IgG4-RD is a systemic fibro-inflammatory disease with multiple organ involvement.The mostly common involved organs include lymph node,submandibular glands,and pancreas.Glucocorticoids and immunosuppressive agents were effective for IgG4-RD.

Objective: To investigate the characteristics and prognostic value of peripheral blood T lymphocyte subsets in patients with severe influenza. Methods: This was a single-center cross-sectional study in influenza patients admitted to Peking Union Medical College Hospital from August 2017 to April 2018. Peripheral blood lymphocyte subsets were detected by flow cytometry in both patients and 108 healthy controls. Influenza patients were divided into mild group and severe group. Severe patients were further classified into alive and fatal subgroups. Results: A total of 42 influenza patients were recruited in this study, including 24 severe cases (6 deaths). The remaining 18 cases were mild. The peripheral blood lymphocyte counts and lymphocyte subset counts (B, NK, CD4⁺T, CD8⁺T) in either mild patients[795 (571,1 007), 43 (23,144), 70 (47,135), 330 (256,457), 226 (148,366) cells/μl respectively] or severe patients[661 (474,1 151),92 (52,139), 54 (34,134), 373 (235,555), 180 (105,310) cells/μl respectively] were both significantly lower than those of healthy controls [1 963 (1 603,2 394),179 (119,239), 356 (231,496), 663 (531,824), 481 (341,693) cells/μl respectively]. Meanwhile, the T cells and CD8⁺T counts in fatal patients [370 (260,537) cells/μl and 87 (74,105) cells/μl] were significantly lower than those in severe and alive patients [722 (390,990) cells/μl and 222 (154,404) cells/μl]. CD8⁺HLA-DR/CD8⁺and CD8⁺CD38⁺/CD8⁺T cell activating subgroups in mild cases[(53.7±19.2)% and 74.8% (64.1%,83.7%) respectively] were significantly higher than those in severe cases[(38.5±21.7)% and 53.3% (45.3%,67.2%) respectively].Moreover,CD8⁺HLA-DR/CD8⁺count in severe and alive group was higher than that in fatal group [(46.1±19.1)% vs. (18.2±14.6)%, P<0.01]. Logistic regression analysis showed that CD8⁺T cell count (OR=0.952, 95%CI 0.910-0.997, P=0.035) and CD8⁺HLA-DR/CD8⁺T (OR=0.916, 95%CI 0.850-0.987, P=0.022) were both negatively correlated with mortality.Peripheral blood lymphocyte counts in mild cases rapidly decreased within 1 day after diagnosis, and returned to the basic level one week later. Conclusions All peripheral blood lymphocyte subsets (T,B,NK) in patients with influenza are significantly reduced. These findings are consistent with the immunological characteristics of respiratory viral infections, in which peripheral lymphocytes (especially T cells) migrate to respiratory tract in the early stage and circulate to the peripheral blood after recovery. The activated CD8⁺T cell counts in peripheral blood are negatively correlated with the severity of disease, which could be considered as a prognostic indicator of severe influenza.