Prognostic markers for idiopathic pulmonary arterial hypertension

Objective The objective of this study is to review the research on the prognostic markers of idiopathic pulmonary arterial hypertension （IPAH）.Date sources We searched literature from PubMed and CNKI databases both in English and Chinese up to 2013.Study selection Data about mortality and cut-off value are from clinical trials and identified by analysis.Results IPAH is an unexplained,progressive,and rare disease characterized by increased pulmonary artery pressure and pulmonary vascular resistance.The diagnosis is difficult,mortality of IPAH is high,and the survival periods are only 2-3 years after diagnosis.Investigations in recent years have identified a range of prognostic markers for IPAH,including the 6-minute walking test,red blood cell distribution width,and platelet levels,as well as imaging findings.Changes in these markers are important sources of information to predict the prognosis of patients with IPAH,which carries significant benefits for treatment planning.Conclusion Even though the prognosis of IPAH has been investigated,the mortality is also high.More accurate and meaningful assessment for the prognosis of IPAH is required.     

手术治疗漏斗胸的疗效和安全性的多中心前瞻性研究

1文献来源 Kelly RE,Mellins RB,Shamberger RC,et al.Multicenter study of pectus excavatum,final report：Complications,static/exercise pulmonary function,and anatomic outcomes[J].J Am Coll Surg,2013,217（6）：1080-1089.2证据水平2b。3背景1998年美国Nuss教授首次报道了不截骨的微创手术（Nuss手术）治疗漏斗胸的成功经验,由于Nuss手术是一种微创且易于掌握的外科技术,从而快速在世界各地广泛开展。     

Evaluation of the Xpert MTB/RIF assay for diagnosis of tuberculosis and rifampin resistance in county-level laboratories in Hunan province, China

Background The Xpert MTB/RIF showed high sensitivity and specificity in previous studies carried out in different epidemiological and geographical settings and patient populations in high-burden tuberculosis （TB） countries.However,there were little data obtained by validation or demonstration study of the assay in China.In this study,the performance of Xpert MTB/RIF was investigated in two county-level laboratories in Hunan Province,China.Methods Consecutive patients with suspected pulmonary tuberculosis （PTB） and suspicion for multidrug-resistant tuberculosis （MDR-TB） were enrolled.For each patient suspected to have PTB,three sputum specimens （one spot sputum,one night sputum,and one morning sputum） were collected and each sputum was tested with smear microscopy,L（o）wenstein-Jensen （LJ） culture,and Xpert MTB/RIF test.For comparison across subgroups and testing methods,95％ confidence intervals were calculated.All analyses were done with SPSS 16.0,and P ＜0.05 was regarded as significant.Results For case detection,the sensitivity of Xpert MTB/RIF was 100％ for smear-and culture-positive TB and 88.6％ for smear-negative and culture-positive TB; the overall sensitivity was 94.5％ for all culture-positive patients.The specificity was 99.8％.The sensitivity of Xpert MTB/RIF assay was 22.0％ in clinical TB patients and the specificity reached 100.0％ in the group of patients who are infected with nontuberculous mycobacteria.For the detection of rifampin resistance,the sensitivity of MTB/RIF RIF-resistance detection was 92.9％,and the specificity was 98.7％.Of the 26 Xpert MTB/RIF-positive and RIF-resistant patients confirmed by LJ proportion tests,20 （76.9％） patients were infected by MDR-TB.Conclusions The Xpert MTB/RIF assay is a highly sensitive and specific method for diagnosis of TB and RIF resistance,which will enable it to have the potential to be used in county-level laboratories and lead to the reduction of the infectious pool and improvements in TB control in China.Furt     

糖尿病患者并发肺结核临床及预后分析

[目的]探讨糖尿病患者患肺结核病人的临床特点及预后情况。[方法]对53例糖尿病患肺结核病进行了观察和追踪，分析其临床特点、实验室检查、治疗过程、预后并与随机选自同期住院及相近的初治肺结核病人60例进行对照。[结果]糖尿病临床表现为发热明显，咯血多见，抗酸杆菌阳性率高，X线胸片病灶广泛，占2个及以上肺野患者多，疗程常延长，病死率高。     

Inaccuracy of doppler echocardiographic estimates of pulmonary artery pressures in adult atrial septal defect patients with pulmonary arterial hypertension

Background While echocardiography has been a pivotal screening test in pulmonary arterial hypertension （PAH）, the presence of structural cardiac defects may affect the ability to reliably predict pulmonary artery pressures （PAPs）. This study sought to evaluate the accuracy of Doppler echocardiography （DE） for estimating PAPs in adult atrial septal defect （ASD） patients with PAH. Methods A prospective study was carried out to compare the echocardiographic assessment of PAP with the same pressures obtained by right heart catheterization （RHC） in adult ASD patients with PAH who underwent simultaneous DE and RHC. Bland-Altman analyses were performed to evaluate the agreement between DE and RHC measurements of PAPs. Results Two hundred and fifty-seven patients were included in the study. A significant overestimation of the systolic pulmonary arterial pressure （sPAP） and mean pulmonary artery pressure （mPAP） was reported by echocardiography compared with those by catheterization （（81.8±26.9） mmHg vs. （72.9±26.9） mmHg, P 〈0.01; （51.9±16.4） mmHg vs. （41.4±17.2） mmHg, P 〈0.01, respectively）. Twenty-one percent （55/257） of the patients had PAH when estimated by echocardiography whereas showed normal results in the subsequent catheterization test. Using Bland-Altman analytic methods, the bias for the echocardiographic assessment of the sPAP was 9.1 mmHg with 95% limits of agreement ranging from -24.4 to 42.6 mmHg. For mPAP measurement, the bias was 10.5 mmHg with 95% limits of agreement ranging from -12.4 to 33.4 mmHg. On multiple linear regression analysis, age, gender, body surface area, ASDs＇ diameter, PVR, diastolic blood pressure, and echocardiographic assessment of right atrial pressure （RAP） explained 68.8% of the total variability in the model （P=0.688, P 〈0.01）. Conclusion Inaccuracy was frequently reported in Doppler echocardiographic assessment of the PAP in adult ASD patients with PAH and was often associated with age, gender, body surface a     

Inappropriate antidiuretic hormone secretion in a patient with pulmonary lymphoepithelioma like carcinoma

Antidiuretic hormone （ADH） is produced by an area of he brain called the hypothalamus. The hormone is stored in and released by the pituitary gland. When ADH （also called vasopressin） is produced somewhere other than the hypothalamus, the condition is called syndrome of inappropriate antidiuretic hormone （SIADH）. A variety of conditions can trigger abnormal ADH production, but the main cause is cancer. It is frequently one of the first signs of lung cancer, especially small cell carcinoma, which produces ADH ectopically.l     

Pulmonary embolism and impending paradoxical embolism: a case report

A definitive diagnosis of paradoxical embolism is ,based on the evidence that a thrombus crosses through the right-to-left shunting. We report a case of impending paradoxical embolism in a patient with pulmonary embolism diagnosed by echocardiography and proved by operation later.     

Electroacupuncture at Feishu （BL13） and Zusanli （ST36） down-regulates the expression of orexins and their receptors in rats with chronic obstructive pulmonary disease

Inflammation and lung function decline are the main pathophysiological features of chronic obstructive pulmonary disease （COPD）. Acupuncture can improve lung function in patients with COPD, but the underlying mechanisms are not well understood. Orexins （OXs）, which are found in peripheral plasma, are neuropeptides that regulate respiration and their levels are related to COPD. Therefore, we hypothesized that acupuncture might alter OXs, reduce lung inflammation and improve lung function in COPD. METHODS： COPD was induced in rats by exposure to cigarette smoke for 8 weeks and injecting with lipopolysaccharide twice. Electroacupuncture （EA） was performed at Feishu （BL13） and Zusanli （ST36） for 30 min/d for 2 weeks. Rat lung function and morphology were assessed after EA. The levels of tumor necrosis factor-α （TNF-a） and intedeukin-1β （IL-16） in bronchoalveolar lavage fluid （BALF） and orexin A and B levels in the lung tissue were detected by enzyme-linked immunosorbent assay. OX receptor mRNA levels and immunopositive cells were assessed with real-time polymerase chain reaction and immunohistochemical methods, respectively. The relationships among lung function, cell factors, and OX levels were analyzed by Pearson correlation analyses. RESULTS： Compared with the control group, lung function was significantly decreased in the rats with COPD （P〈0.05）. There were obvious increases in TNF-α and IL-1β levels in BALF （P〈0.05 and P〈0.01, respectively）, orexin A level in lung tissue （P〈0.01; but not orexin B） and mRNA expressions of OX 1 type receptor （OXR1） and OX 2 type receptor （OXR2） in lung tissue （P〈0.05 and P〈0.01, respectively）; the integrative optical densities （IODs） of both receptors were greater in the COPD group （P〈0.05）. For rats with COPD subjected to EA, lung function was improved （P〈0.05）. There were notable decreases in TNF-a and IL-1β levels （P〈0.05 and 〈0.01, respectively） in BALF. Orexin A, but not orexi     

Extracorporeal membrane oxygenation for pulmonary hemorrhage in microscopic polyangiitis

Microscopic polyangiitis （MPA） is characterized by an inflammatory process of the vessel walls and is classified according to the smallest vessels involved. Diagnosis is based on clinical manifestations, anti-neutrophil cytoplasmic autoantibody （ANCA） testing, and histology. In this disease, pulmonary hemorrhage is seen in 30% of patients and is associated with an eight-fold increase in mortality. Classically, MPA involves the kidneys and upper and lower respiratory tracts. Due to the perceived risk of increased bleeding secondary to the use of systemic anticoagulation, extra corporeal membrane oxygenation （ECMO） is often not used to treat patients with hemorrhage suffered from MPA. Here we report a case of a 50-year-old woman with MPA that was successfully supported with ECMO.     

Primary pulmonary artery chondrosarcoma: the use of different imaging modalities

Fewer than 300 patients with primary pulmonary artery sarcoma have been reported since 1923,when it was first described by Mandelstamm.1 The diagnosis of pulmonary artery sarcoma is difficult,and misdiagnosis as chronic pulmonary embolism often occurs.Herein,we reported a case of primary pulmonary artery chondrosarcoma.So far as we know,this patient served as the first case ever reported to receive computed tomography pulmonary angiography （CTPA）,gadolinium enhanced magnetic resonance imaging （MRI）,and positron emission tomography/computed tomography （PET/CT） before surgery.By these means,the utility of different imaging modalities for pulmonary artery sarcoma was well demonstrated.Keywords：primary pulmonary artery chondrosarcoma; computed tomography pulmonary angiography;magnetic resonance imaging; positron emission tomography     

正常产后38小时发生急性肺栓塞1例

1病历简介患者25岁、孕1产0,孕38^＋1W,住院号58902,因“孕足月,阴道流水9h,阵发腹痛4h”为主诉,于2009年11月3日8Am以“G1P0孕338^＋1w临产、胎膜早破”急诊收住院。患者系G1P0,平素月经规律,孕期过程顺利,     

Trends in conventional mechanical ventilation and pulmonary graphics in the newborn

The optimal treatment for respiratory distress syndrome （RDS） in extremely low birth weight newbom infants now consists of surfactant therapy, ventilator support and aggressive nutritional support. Introduction of surfactant therapy has significantly reduced both the mortality and morbidity in premature infants. However, despite all the preventive efforts the prematurity rate has increased in the United States. As a result of this trend the majority of the infants requiring mechanical ventilation in the current neonatal intensive care units are less than 1000 g.     

Evaluation of the asynchronization and function of the left ventricle in patients with chronic pulmonary hypertension by velocity vector imaging

Background Pulmonary hypertension （PH） is a set of pathophysiological syndromes characterized by increased pulmonary artery pressure and pulmonary vascular resistance,resulting in increased right ventricular afterload.The left and right ventricles interact through hemodynamics.What impact will PH have on synchronization and function of the left ventricle （LV）？ The aim of this study was to evaluate the synchronization of the left ventricular wall motion and left ventricular function in patients with varying degrees of PH using velocity vector imaging （Wl） technology.Methods Sixty patients with chronic PH served as the experimental group,and 20 healthy volunteers served as the control group.According to the different degrees of pulmonary artery systolic pressure,the experimental group was divided into three groups：mild,moderate,and severe PH groups.The time to peak systolic longitudinal velocity （Tvl）,the peak systolic longitudinal velocity （Vsl）,the peak diastolic longitudinal velocity （Vel）,the peak systolic longitudinal strain （SI）,and strain rate （SRI） in 18 segments were measured in each group.Results TvI in the control group and each group with PH was reduced from basal to apical segment,and in control group Tvl in various segments of the same wall and in different walls showed no significant difference （P ＞0.05）.With increase in pulmonary artery pressure,Tvl values measured showed an increasing trend in groups with PH.In groups with PH,Vsl and Vel of each wall were reduced sequentially from basal to apical segments,showing gradient change; Vsl and Vel values measured showed a decreasing trend with increase in pulmonary artery pressure,in which the differences of Vel values measured in the control group and the mild PH group were statistically significant （P ＜0.01）,and the differences between other groups were statistically significant （P ＜0.01）.In groups with PH,SI and SRI in basal segment and the middle segment of each wall were decreased; the difference between groups was statistically significant （P ＜0.01）.Conclusions Asynchronization of the LV and decreased left ventricular function were present in patients with chronic PH;WI technology can accurately evaluate left ventricular function in patients with PH,and indicators such as Tvl,Vsl,and Vel are valuable.     

Midterm results of diagnostic treatment and repair strategy in older patients presenting with nonrestrictive ventricular septal defect and severe pulmonary artery hypertension

Background Congenital heart disease with severe pulmonary arterial hypertension （SPAH）,previously thought to have irreversible pulmonary vascular disease （PVD）,has been recently successfully corrected using diagnostic treatment and repair strategy by our surgery team.This study aimed to evaluate the midterm results of a selected cohort of older patients with nonrestrictive ventricular septal defect （VSD） and SPAH using diagnostic treatment and repair strategy.Methods The records of 56 patients older than six years with nonrestrictive VSD and SPAH undergoing diagnostic treatment and repair strategy from 2006 to 2012 were retrospectively reviewed.All patients received advanced pulmonary arterial hypertension （PAH） therapy and radical repairs were performed when transcutaneous oxygen saturation （SPO2） increased up to 93％.Results There were no operative deaths.SPO2 and baseline six-minute walk test （SMWT） distance of all selected patients increased significantly and mean pulmonary artery pressure （MPAP） regressed significantly after diagnostic treatment and at late follow-up （P ＜0.01）.The incidence of late postoperative PAH was seen in six （10.7％） patients and by Logistic regression analysis,early postoperative PAH was an independent risk factor related to late postoperative PAH.Conclusions Diagnostic treatment and repair strategy was effective and safe for treatment of nonrestrictive VSD and SPAH and the midterm results were excellent.Diagnostic treatment strategy was effective in assessing the reversibility of SPAH in older patients associated with nonrestrictive VSD and the PVD in these selective patients is generally reversible.     

An adult case of isolated unilateral pulmonary agenesis under general anesthesia

Pulmonary agenesis is a rare congenital malformation of unknown etiology that is defined as the complete absence of the bronchus,lung parenchyma,and pulmonary vasculature.The exact incidence of this rare abnormality is not known but is believed to range from 0.003 4% to 0.009 7%.1 Pulmonary agenesis affects men and women almost equally.Left sided agenesis is more common.More than half of the reported cases are associated with anomalies involving cardiovascular,gastrointestinal,skeletal,and urogenital systems and patients die within the first five years of life.Although some cases are compatible with long-term survival and normal growth,completely symptom free and well tolerated surgery is extremely rare.Here we present a case of congenital left lung agenesis in an adult woman tolerating general anesthesia well for modified radical mastectomy.     

The St. George's Respiratory Questionnaire in Lymphangioleiomyomatosis

Objective To examine the correlation between the health-related quality of life measured by the St. George＇s Respiratory Questionnaire （SGRQ） and the commonly used physiological measures in lymphangioleiomyomatosis （LAM）. Methods This study retrospectively analyzed the SGRQ scores and other measures （the Borg scale of breathlessness at rest, 6-minute walking distance, blood oxygen levels, and pulmonary function） of patients diagnosed and confirmed with LAM. Altogether 38 patients between June 2007 and November 2009 were included. Results The mean values of the SGRQ three components （symptoms, activity, and impacts） and total scores in the LAM patients were 46.95±28.90, 58.47±25.41,47.89±29.66, and 51.11±26.35, respectively. The SGRQ total or component scores were correlated well with the Borg scale of breathlessness, 6-minute walking distance, partial pressure of oxygen in arterial blood, spirometry and diffusion capacity of lung. There were poor correlations between SGRQ score and residual volume or total lung capacity. In our preliminary observation, sirolimus improved the SGRQ total and three component scores and the Borg scale of breathlessness significantly after 101-200 days of treatment （n=6）. Conclusions The SGRQ score in LAM is correlated well with physiological measures （Borg scale of breathlessness, 6-minute walking distance, blood oxygen levels, and pulmonary function tests）. The SGRQ could therefore be recommended in baseline and follow-up evaluation of patients with LAM. Treatment with sirolimus, an inhibitor of mammalian target of rapamycin, may improve the quality of life and patient＇s perception of breathlessness in LAM.     

Basic and clinical research into chronic obstructive pulmonary

Chronic obstructive pulmonary disease （COPD）, one of the most common public health problemsworldwide, is the fourth leading cause of death in the world. In China, respiratory diseases （of which COPD is a major component） are the third leading cause of death in rural areas and the fourth leading cause of death in urban area, accounting for 1 million deaths and over 5 million disabilities each year. COPD ranks first among the burdens in China.1 Hence, medical workers and governments at different levels pay close attention to this disease~ The Respiratory Group under the Chinese Medical Association published the Guidelines for Diagnosis and Management of COPD in 2002^2 and the revised version in 2007^3. This publication serves as a milestone of the development of basic and clinical investigation in China. The guidelines are likely to affect the way for the treatment of COPD in addition to reflect the current knowledge about the underlying causes of COPD and recommend the proper diagnosis and management of COPD.     

A diagnosis neglected for 6 years: report of a misdiagnosed case of pulmonary mucormycosis and review of the literature

Pulmonary mucormycosis is an uncommon and often fatal opportunistic fungal infection caused by fungi in the class zygomycetes of the order mucorales. As a rare clinical entity characterized by acute onset, rapid progression and high mortality rate, pulmonary mucormycosis often results in fatal consequences within two years after the onset, and can be easily misdiagnosed. Here we report a case of pulmonary mucormycosis for which a definite diagnosis had not been established until 6 years after the initial onset.     

Clinical profiles of pulmonary lymphangioleiomyomatosis in the mainland of China

Pulmonary lymphangioleiomyomatosis （PLAM） is a rare disease primarily occurring in women at reproductive age, and characterized by abnormal proliferation of immature smooth muscle cells named lymphangioleiomyomatosis （LAM） cells in the pulmonary lymphatics, blood vessels and airways, resulting in respiratory failure and death.     

Original article：A novel two-stage complete repair method for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries

Background Pulmonary atresia with ventricular septal defect （PA-VSD） and major aortopulmonary collateral arteries （MAPCAs） remains a challenging complex congenital heart disease nowadays. In the present study, we aimed to develop a two-stage surgical method and to evaluate outcomes of this method in managing PA-VSD and MAPCAs. Methods Between December 2003 and December 2008, 7 female and 4 male patients between the age of 5 and 10 years who were suffering from PA-VSD and MAPCAs were selected and recruited. The native pulmonary artery trunks were absent in all patients; the lungs were solely supplied by major aortopulmonary collaterals, and the numbers of supplied lung segments ranged from 15 to 20 （17.9±1.6）. There were a total of 43 MAPCAs in all the patients （3-5 （3.9~0.7） MAPCAs per patient）. The accumulated Nakata index was （222.9＋_29.9） mm2/m2 （ranged from 182 to 272）. All the patients underwent two sequential operations. Stage one included left major aortopulmonary collateral unifocalization and modified Blalock-Taussig shunt from left posterior lateral thoracotomy; stage two comprised right unifocalization, ligation of the shunt, followed by ventricular septal defect closure and right ventricular outflow tract reconstruction assisted with cardiopulmonary bypass from midline sternotomy. Results All the patients survived the initial surgery, but one of them died of low cardiac output syndrome on the third day after the second operation. Postoperative complications included pneumonia in one case and capillary leak syndrome in another. Postoperative oxygen saturation maintained about 95%-100%, which was significantly higher than pre-operation （P 〈0.01）. During the follow-up period of 3-51 （25.4±15.2） months, there were no late death and no need for reintervention. All the patients enjoyed their lives with good conditions. Conclusions This two-stage complete repair strategy was well-tolerated and effective with good outcome, thus offering an alternative surgical approach in the treatment of PA-VSD and MAPCAs.