Bronchogenic cysts with infection in the chest wall skin of a 64-year-old asymptomatic patient: A case report.

BACKGROUND: Skin bronchogenic cysts are extremely rare congenital bronchocystic changes caused by the abnormal development of the trachea, bronchial trees or lung buds during the embryonic period. The first case of skin bronchogenic cysts was reported in 1945. Since then, this disease has attracted increasing attention, but due to the low incidence, its pathogenesis is still not clear. CASE SUMMARY: Here, we report another case of skin bronchogenic cysts with infection in a 64-year-old female patient. The patient had no symptoms for more than 60 years until her chest wall was recently found to be swollen, and she felt pain and discomfort. At the same time, secretions were found on the surface of the swelling. Color Doppler ultrasound examination showed abnormal echoes in the soft tissue under the frontal chest wall, suggesting the presence of cysts. Cytological puncture resulted in about 2 mL of pus and showed the presence of more acute inflammatory cells. The final clinical diagnosis was skin cyst with infection, and surgery was carried out. The pathological results obtained after surgery showed that the cystic wall was covered with column-like cilia epithelial cells, and the interstitial structure was partially inundated with inflammatory cells. After a variety of examinations and clinical diagnoses, we finally confirmed that the patient was suffering from bronchogenic cyst. CONCLUSION: This article not only describes the case of an elderly patient with rare skin bronchogenic cysts with infection but also provides a detailed and correct diagnosis and a successful treatment process, which is of great value for the diagnosis and treatment of the disease.

[Temporal and spatial distribution of Gli1+ cells and their function during periodontal development].

OBJECTIVE: This study aimed to investigate the distribution of Gli1+ cells in the periodontal ligament (PDL) and to evaluate their contribution in the development of periodontal tissue by using transgenic mouse lines. METHODS: Gli1lacZ/+ mice were harvested at different ages (3, 6, and 8 weeks), and the temporal and spatial distribution patterns of Gli1+ PDL cells were revealed by X-gal staining. Afterward, 3-week-old Gli1-CreERT2/+;R26RtdTomato/+ mice were administered with tamoxifen, and the fates of Gli1+ cells and their descendants were traced during periodontal development. RESULTS: A large number of Gli1+ cells were detected in the PDL of the 3-week-old mice; however, their number significantly decreased from 3 weeks to 8 weeks (P<0.05). Cell lineage tracing data showed that the descendants of Gli1+ cells dramatically increased from 3 weeks to 8 weeks (P<0.05) and gradually differentiated into fibroblasts, cementocytes, and osteocytes. CONCLUSIONS: The multi-differentiation potential of Gli1+ PDL cells was revealed, indicating that Gli1+ cells are an important cell source for periodontal development.

α-adrenoceptor-mediated enhanced inducibility of atrial fibrillation in a canine system inflammation model.

The exact mechanism associated with inflammation and atrial fibrillation (AF) remains unknown. The aim of the present study was to investigate the roles of connexin 43 (Cx43) and a1­adrenergic receptor (α1­AR) activation in the pathogenesis of system inflammation­induced AF. A canine model of chronic low­grade system inflammation was established by administrating a low dose of lipopolysaccharide (LPS; 0.1 µg/kg) for 2 weeks. Programmed stimulation was applied on the right atrial appendage to determine the effective refractory periods (ERP) and the window of vulnerability (WOV). Tumor necrosis factor α (TNF­α) and interleukin 6 (IL­6) levels in plasma and atrial tissue were measured by ELISA. Cx43, Toll­like receptor 4 (TLR4) and nuclear factor κB (NF­κB) proteins were analyzed using western blotting or immunohistochemistry. Administration of LPS for 2 weeks increased the concentration of TNF­α and IL­6 in the plasma and right atrium. ERP was markedly shortened and cumulative WOV was significantly widened in the LPS group. Following treatment with LPS, the amount of Cx43 protein in the area of intercalated disk increased. In addition, a high­density of Cx43 in the lateral connection was identified. LPS also induced the activation of NF­κB in the canine atrium. Administration with the α1­AR blocker doxazosin prevented the production of LPS­induced inflammatory cytokine and reversed the enhanced vulnerability to atrial fibrillation. Doxazosin inhibited the LPS­induced increase in Cx43 protein and heterogeneous distribution, and prevented the activation of NF­κB. These results indicated that chronic low­grade system inflammation may increase the inducibility of AF in a canine model. The underlying mechanism may be involved in the LPS­induced activation of NF­κB, and the increase in Cx43 expression and lateral distribution via an α1-AR-dependent pathway.

c-Met identifies a population of matrix metalloproteinase 9-producing monocytes in peritumoural stroma of hepatocellular carcinoma.

Macrophages (Mϕ) are prominent components of solid tumours and exhibit distinct phenotypes in different microenvironments. Previously, we found that tumours could alter the normal developmental process of Mϕ to trigger transient activation of monocytes in the peritumoural stroma of human hepatocellular carcinoma (HCC). In the present study, we showed that a fraction of monocytes in the peritumoural stroma, but not in HCC cancer nests, expressed surface c-Met molecules. Monocytes exposed to tumours strongly expressed c-Met proteins with kinetics similar to their activation status, and significant correlations were found between c-Met levels and HLA-DR expression on tumour-infiltrating monocytes. NF-κB-mediated autocrine TNF-α stimulated the expression of c-Met on activated monocytes, and by interacting with its ligand hepatocyte growth factor (HGF), c-Met increased the motility and matrix metalloproteinase (MMP) 9-producing capacity of tumour-associated monocytes. The intensity of c-Met expression on tumour-infiltrating monocytes was associated with high mortality and reduced survival of patients with HCC. Therefore, the expression of c-Met on activated monocytes/Mϕ may represent a novel mechanism by which a tumour actively and precisely regulates the distribution and functions of these cells to facilitate disease progression.

[Effects of angiotensin converting enzyme inhibitor on the expression of angiotensin converting enzyme 2 in atrium of patients with atrial fibrillation].

OBJECTIVE: To investigate the expression of angiotensin converting enzyme 2 (ACE2) and the changes treated with angiotensin converting enzyme inhibitor (ACEI), and its signal transduction pathway. METHODS: Atrial tissues were obtained from 47 patients with RHD undergoing cardiac surgery. The mRNA of ACE2 and ACE were semi-qualified by RT-PCR and normalized to the gene beta-actin. Western blot analysis was employed to examine the expressions of ACE2, ACE, ERK1/2 and phosphorylated ERK (pERK1/2). The atrial tissue angiotensin II (Ang II) content was determined by radioimmunoassay detection. RESULTS: The expression of ACE2 was significantly decreased (P < 0.05), the expression of ACE and pERK1/2 were significantly increased (P < 0.05), and the level of atrial tissue Ang II was significantly increased in patients with chronic atrial fibrillation group (CAF) compared with sinus rhythm group (SR) (P < 0.05). Compared with CAF patients treated without ACEI, the expression of ACE2 significantly increased (P < 0.01), and the relative activity of ERK1/2 significantly decreased (P < 0.05), whereas the expression of ACE and the level of atrial tissue Ang II remained unchanged in CAF patients treated with ACEI. CONCLUSIONS: The study suggested that the dysequilibrium of ACE/ACE2 might play an important role in the process of atrial fibrillation, which may be related to the activation of ERK1/2 pathway. The clinical effect of long-term treatment of ACEI maybe associated with elevated ACE2 expression but not ACE expression.

Transplantation of autologous endothelial progenitor cells may be beneficial in patients with idiopathic pulmonary arterial hypertension: a pilot randomized controlled trial.

OBJECTIVES: The goal of this study was to investigate the feasibility, safety, and initial clinical outcome of intravenous infusion of autologous endothelial progenitor cells (EPCs) in patients with idiopathic pulmonary arterial hypertension (IPAH). BACKGROUND: Experimental data suggest that transplantation of EPCs attenuates monocrotaline-induced pulmonary hypertension in rats and dogs. In addition, clinical studies suggest that autologous progenitor cell transplantation is feasible and safe in patients with ischemic diseases. METHODS: We conducted a prospective, randomized trial comparing the effects of EPC transplantation plus conventional therapy with those of conventional therapy alone in patients with IPAH. The primary end point was change in the 6-min walk distance using a standardized protocol. The secondary end points were changes in hemodynamic variables as assessed by right heart catheterization. RESULTS: After 12 weeks of follow-up, the mean distance walked in 6 min increased by 48.2 m in the cell infusion group (from 263 +/- 42 m to 312 +/- 34 m), and an increase of 5.7 m occurred in the conventional therapy group (from 264 +/- 42 m to 270 +/- 44 m). The mean difference between the 2 groups was 42.5 m (95% confidence interval 28.7 to 56.3 m, p < 0.001). The patients in the cell infusion group also had significant improvement in mean pulmonary artery pressure, pulmonary vascular resistance, and cardiac output. There were no severe adverse events with cell infusion. CONCLUSIONS: This preliminary study showed that intravenous infusion of autologous EPCs seemed to be feasible and safe, and might have beneficial effects on exercise capacity and pulmonary hemodynamics in patients with IPAH. (Safety and Efficacy Study of Transplantation of EPCs to Treat Idiopathic Pulmonary Arterial Hypertension; http://www.clinicaltrials.gov/ct/show/NCT00257413?order=1; NCT00257413).

A novel splice mutation of HERG in a Chinese family with long QT syndrome.

Congenital long QT syndrome (LQTS) is a genetically heterogeneous disease in which six ion-channel genes have been identified. The phenotype-genotype relationships of the HERG (human ether-a-go-go-related gene) mutations are not fully understood. The objective of this study is to identify the underlying genetic basis of a Chinese family with LQTS and to characterize the clinical manifestations properties of the mutation. Single strand conformation polymorphism (SSCP) analyses were conducted on DNA fragments amplified by polymerase chain reaction from five LQT-related genes. Aberrant conformers were analyzed by DNA sequencing. A novel splice mutation in C-terminus of HERG was identified in this Chinese LQTS family, leading to the deletion of 11-bp at the acceptor splice site of Exon9 [Exon9 IVS del (-12-->-2)]. The mutation might affect, through deficient splicing, the putative cyclic nucleotide binding domain (CNBD) of the HERG K(+) channel. This mutation resulted in a mildly affected phenotype. Only the proband had a history of syncopes, while the other three individuals with long QT interval had no symptoms. Two other mutation carriers displayed normal phenotype. No sudden death occurred in the family. The 4 affected individuals and the two silent mutation carriers were all heterozygous for the mutation. It is the first splice mutation of HERG reported in Chinese LQTS families. Clinical data suggest that the CNBD mutation may be less malignant than mutations occurring in the pore region and be partially dominant over wild-type function.