Recurrent primary spontaneous pneumothorax in a large Chinese family: a clinical and genetic investigation / 中华医学杂志(英文版)

Background@#Primary spontaneous pneumothorax (PSP) is a common manifestation of Birt-Hogg-Dubé (BHD) syndrome, which is an autosomal dominant disorder caused by mutation of the folliculin (FLCN) gene. This study was established to investigate the mutation of the FLCN gene and the phenotype in a family with PSP.@*Methods@#We investigated the clinical and genetic characteristics of a large Chinese family with recurrent spontaneous pneumothorax. Genetic testing was performed by Sanger sequencing of the coding exons (4-14 exons) of the FLCN gene.@*Results@#Among ten affected members in a multi-generational PSP kindred, with a total of 18 episodes of spontaneous pneumothorax, the median age for the initial onset of pneumothorax was 42.5 years (interquartile range: 28.8-57.2 years). Chest computed tomography scan of the proband showed pulmonary cysts and pneumothorax. A novel nonsense mutation (c.1273C>T) in exon 11 of FLCN gene that leads to a pre-mature stop codon (p.Gln425*) was identified in the family. The genetic analysis confirmed the diagnosis of BHD syndrome in this family in the absence of skin lesions or renal tumors.@*Conclusions@#A novel nonsense mutation of FLCN gene was found in a large family with PSP in China. Our results expand the mutational spectrum of FLCN gene in patients with BHD syndrome.

Recurrent primary spontaneous pneumothorax in a large Chinese family: a clinical and genetic investigation / 中华医学杂志(英文版)

BACKGROUND@#Primary spontaneous pneumothorax (PSP) is a common manifestation of Birt-Hogg-Dubé (BHD) syndrome, which is an autosomal dominant disorder caused by mutation of the folliculin (FLCN) gene. This study was established to investigate the mutation of the FLCN gene and the phenotype in a family with PSP.@*METHODS@#We investigated the clinical and genetic characteristics of a large Chinese family with recurrent spontaneous pneumothorax. Genetic testing was performed by Sanger sequencing of the coding exons (4-14 exons) of the FLCN gene.@*RESULTS@#Among ten affected members in a multi-generational PSP kindred, with a total of 18 episodes of spontaneous pneumothorax, the median age for the initial onset of pneumothorax was 42.5 years (interquartile range: 28.8-57.2 years). Chest computed tomography scan of the proband showed pulmonary cysts and pneumothorax. A novel nonsense mutation (c.1273C>T) in exon 11 of FLCN gene that leads to a pre-mature stop codon (p.Gln425*) was identified in the family. The genetic analysis confirmed the diagnosis of BHD syndrome in this family in the absence of skin lesions or renal tumors.@*CONCLUSIONS@#A novel nonsense mutation of FLCN gene was found in a large family with PSP in China. Our results expand the mutational spectrum of FLCN gene in patients with BHD syndrome.

Early removal of the chest tube after lobectomies: a prospective randomized control study / 中华外科杂志

<p><b>OBJECTIVE</b>To evaluate the feasibility and safety of early chest tube removal after lobectomies for lung diseases.</p><p><b>METHODS</b>A prospective randomized control study was performed with data collected from lobectomies between March 2012 and September 2012. Eligible patients (n = 70) were randomized into two groups; early removal group (removal of chest tube when drainage less than 300 ml/24 h, n = 41) and traditional management group (removal of chest tube when drainage less than 100 ml/24 h, n = 29). Criteria for early removal were established and met before chest tube removal. The volume and character of drainage, time of extracting drainage tube and postoperative hospital stay were measured. All patients received standard care during hospital admission and a follow-up visit was performed after 7 days of discharge from hospital.</p><p><b>RESULTS</b>There were no differences between two groups with respect to age, sex, comorbidities, or pathologic evaluation of resection specimens. The median volume of drainage within 24 h after surgery was 300 ml and within 48 h was 250 ml, there was significantly different between two groups (Z = -2.059, P = 0.039). Patients undergoing early removal management had a shorter Chest tube duration (44 hours vs. 67 hours, Z = -2.914, P = 0.004) and a shorter postoperative hospital stay (5.0 days vs. 6.0 days, Z = -3.882, P = 0.000). Analysis of data showed no statistically significant differences between the rate of pleural effusions developed, thoracentesis and complications, one week after discharge from hospital.</p><p><b>CONCLUSIONS</b>Compared to the traditional management group (drainage ≤ 100 ml/24 h), early removal of chest tube after lobectomy (drainage ≤ 300 ml/24 h) is feasible and safe. It could result in a shorter hospital stay, and most importantly, reduces morbidity without the added risk of complications.</p>

Pneumonia relevant to lung transplantation and pathogen distribution / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Pneumonia is the most common cause of morbidity and mortality in lung transplant (LT) recipients. The aim of the present study was to evaluate the incidence, etiology, risk factors and prognosis of pneumonia in LT recipients.</p><p><b>METHODS</b>The LT cohort consisted of 28 recipients receiving LT in Beijing Chao-Yang Hospital from August 2005 to April 2011. Data collected included demographic data, underlying disorders, time and type of transplant, follow-up information, date of last follow-up, and patient status. A retrospective analysis was made of observational data that were prospectively collected.</p><p><b>RESULTS</b>Twenty-two patients of 28 LT recipients had 47 episodes of pneumonia throughout the study period. Thirtyeight episodes of pneumonia in 19 recipients occurred post-LT with a median follow-up of 257.5 days (1-2104 days), the incidence of pneumonia was 192.4 episodes per 100 LT/year and its median time of onset was 100.5 days (0-946 days) post-transplantation. Bacteria, virus and fungi accounted for 62%, 16% and 15% of the microbial pathogens, respectively. The most frequent were Pseudomonas aeruginosa (20%), cytomegalovirus (CMV) (15%), and Aspergillus fumigatus (10%). A total of 29% (11/38) of pneumonias occurred in the first month post-LT, and then the incidence decreased gradually. The incidence of CMV pneumonia was 25% (7/28) with a median time of 97 days (10-971 days). More than one bacterial infection and CMV infection were independent risk factors for aspergillus infection. The incidence of pulmonary tuberculosis (TB) was 18% (5/28), and the history of TB was a risk factor for TB relapse. There were 58% (7/12) of recipients who died of infection, and 71% (5/7) of these died in the first year after LT.</p><p><b>CONCLUSIONS</b>Pneumonia is still a major cause of morbidity and mortality in LT recipients. The most frequent microorganisms were Pseudomonas aeruginosa, CMV, and Aspergillus fumigates. The incidence of CMV pneumonia decreases with a delayed median time of onset. More than one incidence of bacterial infection and CMV infection are independent risk factors for aspergillus infection. LT recipients are at high risk for TB, and the history of TB is a risk factor for TB relapse.</p>

Combined laparoscopic and thoracoscopic Ivor Lewis esophagectomy for esophageal cancer: initial experience from China / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Minimally invasive Ivor Lewis esophagectomy was usually performed with either hand-sewn or circular stapler anastomosis through a small thoracotomy or using a side-to-side stapler anastomotic technique. This study aimed to present our initial results of Ivor Lewis esophagectomy using a circular-stapled anastomosis with transoral anvil technique.</p><p><b>METHODS</b>Six patients with esophageal cancer underwent minimally invasive Ivor Lewis esophagectomy with an intrathoracic circular-stapled end-to-end anastomosis. The abdominal portion was operated on laparoscopically, and the thoracic portion was done using thoracoscopic techniques. A 25 mm anvil connected to a 90 cm long delivery tube was introduced transorally to the esophageal stump in a tilted position, the anvil head was then connected to circular stapler. The anastomosis was completed under direct thoracoscopic view.</p><p><b>RESULTS</b>A total of six patients in this report successfully underwent total laparoscopic and thoracoscopic Ivor Lewis esophagectomy with a circular-stapled anastomosis using a transoral anvil. They were five male and one female patients, and had a mean age of 55 years (range, 38-69 years). The thoracic and abdominal operations were successfully performed without any intraoperative complications or conversion to laparotomy or thoracotomy. The passage of the anvil head was technically easy and successful in all six cases. The mean overall operative time was (260 ± 42) minutes (range, 220-300 minutes), and the mean estimated blood loss was (520 ± 160) ml (range, 130-800 ml). Patients resumed a liquid oral diet on postoperative day seven. The median length of hospital stay was 17 days (range, 9-25 days). The postoperative pathological diagnosis was esophageal squamous cell carcinoma in five patients and esophageal small cell carcinoma in one patient. Tumors were staged as T(2)N(0)M(0) in three cases, T(2)N(1)M(0) in one case, and T(3)N(0)M(0) in two cases. During the mean follow-up of 2.5 months (range, 2-4 months), there were no intraoperative technical failure of the anastomosis or major postoperative complications such as leak or stricture.</p><p><b>CONCLUSIONS</b>The initial results of this small series suggest that minimally invasive Ivor Lewis esophagectomy for malignant esophageal tumor is technically feasible. However, further multi-center prospective studies and thorough evaluation are needed to evaluate the long-term results.</p>

Completely minimally invasive Ivor Lewis esophagectomy: the preliminary experience of circular-stapled anastomosis with the trans-oral anvil / 中华外科杂志

<p><b>OBJECTIVE</b>to present the preliminary results of minimally invasive Ivor Lewis esophagectomy using a circular-stapled anastomosis with trans-oral anvil technique.</p><p><b>METHODS</b>six patients with esophageal cancer received minimally invasive Ivor Lewis oesophagectomy from April 2010 to June 2010. There were 5 males and 1 female with mean age of 55 years (ranging 38 to 69 years). The lesion located in cardiac in 1 case, in lower third of the esophagus in 4 cases and in middle third in 1 case. The abdominal portion was operated laparoscopically. The thoracic portion was done using thoracoscopic techniques. The esophago-gastric anastomosis was created using a 25 mm anvil passed trans-orally and connected to a 90 cm long polyvinyl chloride delivery tube through an opening in the esophageal stump. The anastomosis was completed by joining the anvil to a circular stapler (end-to-end anastomosis stapler) inserted into the gastric conduit.</p><p><b>RESULTS</b>six patients with esophageal squamous cell cancer (n = 5) and small-cell cancer (n = 1) underwent an Ivor Lewis esophagectomy. All the operation was successfully performed without intra-operative technical failures of the anastomosis. There was no severe postoperative complications. The mean operation time was 380 min. The mean blood loss was 300 ml. pTNM staging: T2N0M0 in 3 cases, T2N1M0 in 1 case and T3N0M0 in 2 cases.</p><p><b>CONCLUSIONS</b>the circular-stapled anastomosis with the trans-oral anvil is an efficient and safe technique for esophago-gastric anastomosis.</p>

The expression of galectin-3 and osteopontin in occult metastasis of non-small cell lung cancer / 中华外科杂志

<p><b>OBJECTIVE</b>To study the relationship between expression of galectin-3 (Gal-3) and osteopontin (OPN) in occult metastasis in non-small cell lung cancer.</p><p><b>METHODS</b>Forty-six patients of non-small cell lung cancer (NSCLC) from January 2006 to October 2007 were selected. There were 28 males and 18 females, aged from 33 to 77 years old. The levels of lung tissues Gal-3 and OPN were detected by RT-PCR, and the levels of blood plasma's were measured by ELISA.</p><p><b>RESULTS</b>There were 12 patients who had metastasized. In un-metastasis group the Gal-3 and OPN mRNA expression levels were significantly lower than that in metastasis group: mean value were 0.07 +/- 0.17 and 0.17 +/- 0.25 in un-metastasis group, while 0.73 +/- 0.23 and 0.79 +/- 0.24 in metastasis group. Blood plasma levels of Gal-3 (18.8 +/- 7.9) microg/L and OPN (153.5 +/- 63.5) microg/L in NSCLC which were detected from metastasis group were higher than un-metastasis group of (9.2 +/- 5.6) microg/L and (89.2 +/- 24.0) microg/L.</p><p><b>CONCLUSIONS</b>High serum levels of Gal-3 and OPN and high expression of Gal-3 and OPN mRNA in NSCLC are closely related to the occurrence and metastasis of NSCLC. They may be indexes of evaluating the occult metastasis in NSCLC.</p>

Clinical study of inflammatory factors in sputum induced early after lung volume reduction surgery / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>The aim of this study was to prospectively study the changes in neutrophil elastase (NE), fibroblast growth factor 9 (Fgf9), matrix metalloproteinase-9 (MMP-9), tissue inhibitor of metalloproteinase 1 (TIMP-1) in sputum induced during the early period after lung volume reduction surgery (LVRS).</p><p><b>METHODS</b>From April to October 2005, ten consecutive patients with chronic obstructive pulmonary disease (COPD) underwent LVRS. Ten non-small cell lung cancer patients (stage II - IIIa) received lobectomy as a control group. The induced sputum was collected from both groups at six different times (two weeks before operation and postoperatively at 1, 2, 4, 6 and 10 days). The level of NE, Fgf9, MMP-9 and TIMP-1 were measured using enzyme-linked immunosorbent assay.</p><p><b>RESULTS</b>The pulmonary function (FEV(1)%) and arterial blood gases (PaO(2) and PaCO(2)) were significantly different between the groups. There were no significant differences in age, ejection fraction (EF), and operation duration, but hemoglobin in the LVRS group was statistically higher than in the controls. At certain times, there were significant differences in NE, MMP-9, TIMP-1 and MMP-9/TIMP-1 (P < 0.05) but not in Fgf9 between the two groups. The levels of NE and TIMP-1 were maximal at 2 days postoperatively and that of MMP-9 and MMP-9/TIMP-1 at 4 days postoperatively in the LVRS group. In the control group, maximal levels of NE and TIMP-1 occurred at 2 days postoperatively and that of MMP-9 and MMP-9/TIMP-1 at 1 day postoperatively. Ten days after surgery, all values of the control group were not significantly different from the baseline. In the LVRS group, the levels were significantly different from the pre-operative values (P < 0.05) apart from TIMP-1.</p><p><b>CONCLUSION</b>The levels of NE, MMP-9, TIMP-1 and MMP-9/TIMP-1 of the LVRS group were different from those of the control group. The time course of these changes may be related to LVRS and the underlying process of COPD.</p>

Comparison of changes in early pulmonary function and hemodynamic between unilateral and bilateral lung volume reduction for chronic obstructive pulmonary disease / 中华外科杂志

<p><b>OBJECTIVE</b>To compare changes in early pulmonary function and hemodynamics between unilateral and bilateral lung volume reduction (LVRS) for severe chronic obstructive pulmonary disease (COPD).</p><p><b>METHODS</b>Eighty-six patients with severe COPD underwent LVRS, 61 underwent unilateral LVRS and 25 underwent lateral LVRS. The results of lung function (FEV(1), RV, TLC), arterial blood gas analysis (PaO(2), PaCO(2)) and color Doppler echocardiography (CO, CI, EF, PAP) were evaluated preoperatively and 3, 6 months postoperatively.</p><p><b>RESULTS</b>Six patients died. FEV(1), RV and TLC were improved significantly after (P < 0.05). PaO(2) increased (P < 0.05) and PaCO(2) decreased postoperatively (P < 0.05). According to the Doppler echocardiography there were no statistic difference in cardia functions (CO, CI, EF, PAP) between unilateral and bilateral LVRS preoperatively and 3, 6 months postoperatively.</p><p><b>CONCLUSIONS</b>Unilateral and bilateral LVRS is safe and effective in the treatment of patients with severe COPD, the pulmonary function significantly improved postoperatively, but the results of bilateral LVRS is better than unilateral. Both unilateral and bilateral LVRS showed no significant deterioration in hemodynamics, there were no significant difference between preoperatively and postoperatively.</p>